ShortTexet Lang Ophthalmology © 2000 Thieme.pdf

ShortTexet Lang Ophthalmology © 2000 Thieme

1. I
Ophthalmology
AShortTextbook
GerhardK.Lang,M.D.
ProfessorandChairman
DepartmentofOphthalmologyand
UniversityEyeHospitalUlm
Germany
Withcontributionsby
J.Amann,M.D.
O.Gareis,M.D.
GabrieleE.Lang,M.D.
DorisRecker,M.D.
C.W.Spraul,M.D.
P.Wagner,M.D.
305Illustrations
Thieme
Stuttgart·NewYork2000
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

2. II
LibraryofCongressCataloging-in-Publica-
tionData
Lang,GerhardK.
[Augenheilkunde.English]
Ophthalmology:ashorttextbook/
GerhardK.Lang;withcontributionsby
J.Amann...[etal.].p.;cm.Includesbiblio-
graphicalreferencesandindex.
ISBN3131261617
1.Eye-Diseases.2.Ophthalmology.
I.Amann,J.(Josef)II.Title.
[DNLM:1.EyeDiseases.
WW40L269a2000a]
RE46.L34132000
617.7–dc21 00-032597
Studentcontributors:
ChristopherDedner,Tübingen
UtaEichler,Karlsruhe
HeidiJaneczek,Göttingen
BeateJentzen,Husberg
MathisKayser,Freiburg
KerstinLipka,Kiel
MarenMolkewehrum,Kiel
AlexandraOgilvie,Munich
PatriciaOgilvie,Würzburg
StefanRose,Oldenburg
TranslatedbyJohnGrossman,Berlin,
Germany
Thisbookisanauthorizedtranslationofthe
Germaneditionpublishedandcopyrighted
1998byGeorgThiemeVerlag,Stuttgart,
Germany.
DrawingsbyMarkusVoll,Fürstenfeldbruck
ImportantNote:Medicineisanever-
changingscienceundergoingcontinual
development.Researchandclinical
experiencearecontinuallyexpandingour
knowledge,inparticularourknowledgeof
propertreatmentanddrugtherapy.Insofar
asthisbookmentionsanydosageorappli-
cation,readersmayrestassuredthatthe
authors,editors,andpublishershavemade
everyefforttoensurethatsuchreferences
areinaccordancewiththestateofknowl-
edgeatthetimeofproductionofthebook.
Neverthelessthisdoesnotinvolve,
imply,orexpressanyguaranteeor
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ciesnoticed.
!2000GeorgThiemeVerlag
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TypesettingbyDruckhausGötzGmbH,
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Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

3. III
TheConceptoftheBookinBrief...
Definition:Theconceptbehindthisbookwastoorganizecontentandlayout
accordingtoauniformstructure.Thisenhancestheclarityofthepresenta-
tionandallowsthereadertoaccessinformationquickly.Eachchapterhasits
ownheadericon,whichisshownoneverypageofthechapter.
Figureheadingssummarizethekeyinformationpresentedintherespective
figure,eliminatingtheneedforthereadertoreadthroughtheentirelegend.
Epidemiology:Intheabsenceofpreciseepidemiologicdata,theauthors
statewhetherthedisorderiscommonorrarewhereverpossible.
Etiology:Thissectionusuallycombinesinformationabouttheetiologyand
pathogenesisofadisorderandinsodoinghelpstoilluminateimportantrela-
tionships.
Symptomsanddiagnosticconsiderations:Theseitemsareusuallydis-
cussedseparately.Thesectiononsymptomsincludesonlythephenomena
withwhichthepatientpresents.Howandbywhichmethodstheexaminer
proceedsfromthesesymptomstoadiagnosisisonlydiscussedunderdiag-
nosticconsiderations.
Sectionshighlightedwithanexclamationmarkcontainimportant
facts.Thesemaybefactsthatoneisoftenrequiredtoknowforexami-
nations,ortheymaybepracticaltipsthatarehelpfulindiagnosingand
treatingthedisorder.
Differentialdiagnosis:Whereverpossible,thissectiondiscussesnotonly
otherpossiblediagnosesbutalsoimportantcriteriafordifferentiatingthe
disorderfromothers.
Treatment:Thissectiongoesbeyondmerelydocumentingallpossiblether-
apeuticoptions.Italsoexplainswhichtherapeuticmeasuresareadvisable
andofferaprospectofsuccess.Thediscussionofmedicaltreatmentocca-
sionallyincludesdosageinformationandexamplesofpreparationsused.This
isdonewheresuchinformationisrelevanttocasesstudentswillencounterin
practice.Thetradenamesspecifieddonotrepresentacomprehensivelisting.
Prognosisandclinicalcourse:Thefurtherdevelopmentofthebook
dependsinnosmallmeasureonyourcriticism.Wearehappytoreceiveany
suggestionsforimprovementsasthiswillhelpustailorthenexteditionto
bettersuityorneeds.Pleaseusetheenclosedpostcard.
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

4. IV
Authors
GerhardK.Lang,M.D.
ProfessorandChairman,UniversityEyeHospital,Ulm,
Germany
J.Amann,M.D.
Researchassistant,UniversityEyeHospital,Ulm
O.Gareis,M.D.
Seniorphysician,UniversityEyeHospital,Ulm
GabrieleE.Lang,M.D.
Director,DepartmentofMedicalRetinaandLaserSurgery,
UniversityEyeHospital,Ulm
DorisRecker
Orthoptist,UniversityEyeHospital,Ulm
C.W.Spraul,M.D.
Seniorphysician,UniversityEyeHospital,Ulm
P.Wagner,M.D.
Chiefofmedicalstaff,UniversityEyeHospital,Ulm
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

5. V
Preface
WhenmycoworkersandIfirsttookupthetaskofwritingatextbookof
ophthalmologythatwasaimedatmedicalstudentsbutwouldalsobesuita-
bleforinternsandophthalmologyresidents,wedidnotknowexactlywhat
weweregettingourselvesinto.Thenextfouryearsweredevotedtointensive
studyofthissubject.Wedidnotmerelyintendtodesignabookaccordingto
themaxims“understanditinmedicalschool,”“learnitfortheexamination,”
and“useitduringyourinternship.”Ourbroadergoalwastogivestudentsa
textbookthatwouldkindletheirinterestandindeedtheirenthusiasmfora
“small”specialtylikeophthalmologyandthatwouldsustainthisenthusiasm
allthewaythroughasuccessfulexamination.Inanageinwhichteachingis
undergoingevaluation,wefeltthiswasparticularlyimportant.Inpursuing
thisadmittedlyambitiousgoal,wewereabletodrawuponmanyyearsof
teachingexperience.Thisexperiencehasshapedtheeducationalconcept
behindthisbookandmanifestsitselfindetailssuchasthelayout,whichis
characterizedbynumerousphotographsandillustrativedrawings.Wehave
placedspecialemphasisonthefiguresinparticular.Theseillustrationsmake
ophthalmologycomealiveandhopefullywillbeabletoimbuethereader
withsomeoftheenthusiasmthattheauthorsthemselveshavefortheir
specialty.
Iwouldliketotakethisopportunitytooffermyheartfeltthankstomy
teacher,Prof.Dr.Dr.hcG.O.H.Naumann,Erlangen,Germany,forhissugges-
tionsandfortheslidesfromthecollectionoftheDepartmentofOphthal-
mologyandUniversityEyeHospital,Erlangen.Iwouldalsoliketooffer
specialthankstomycoauthors,Dr.JosefAmann,Dr.OskarGareis,Prof.Dr.
GabrieleE.Lang,DorisRecker,Dr.ChristophSpraul,andDr.PeterWagnerfor
theirharmoniouscooperationandexceptionalinitiativeinwritingthisbook.
IalsothankDr.EckhardWeingärtnerforhisassistanceincompilingthe
Appendix.
IwouldalsoliketoextendspecialthankstoDr.JürgenLüthjeandSabine
BartlofGeorgThiemeVerlag,whoseprofessionalismandactiveandtireless
supportwereaconstantsourceofinspirationtousall.Iwouldagainliketo
thankMarkusVoll,Fürstenfeldbruck,Germany,forhissplendidillustrations.
Ulm,Germany,Summer2000 GerhardK.Lang
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

6. VI
TableofContents
1 TheOphthalmicExamination...
1
(GabrieleE.Lang,GerhardK.Lang)
1.1 Equipment
...
1
1.2 History
...
3
1.3 VisualAcuity
...
4
1.4 OcularMotility
...
5
1.5 BinocularAlignment
...
6
1.6 ExaminationoftheEyelidsandNasolacrimalDuct
...
7
1.7 ExaminationoftheConjunctiva
...
7
1.8 ExaminationoftheCornea
...
10
1.9 ExaminationoftheAnteriorChamber
...
11
1.10 ExaminationoftheLens
...
12
1.11 Ophthalmoscopy
...
13
1.12 ConfrontationFieldTesting
...
14
1.13 MeasurementofIntraocularPressure
...
15
1.14 Eyedrops,Ointment,andBandages
...
15
2 TheEyelids...
17
(PeterWagner,GerhardK.Lang)
2.1 BasicKnowledge
...
17
2.2 ExaminationMethods
...
19
2.3 DevelopmentalAnomalies
...
20
2.3.1Coloboma
...
20
2.3.2EpicanthalFolds
...
21
2.3.3Blepharophimosis
...
21
2.3.4Ankyloblepharon
...
22
2.4 Deformities
...
22
2.4.1Ptosis
...
22
2.4.2Entropion
...
24
2.4.3Ectropion
...
28
2.4.4Trichiasis
...
30
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

7. VII
2.4.5Blepharospasm
...
30
2.5 DisordersoftheSkinandMarginoftheEyelid
...
30
2.5.1ContactEczema
...
30
2.5.2Edema
...
31
2.5.3SeborrheicBlepharitis
...
33
2.5.4HerpesSimplexoftheEyelids
...
34
2.5.5HerpesZosterOphthalmicus
...
35
2.5.6EyelidAbscess
...
36
2.5.7TickInfestationoftheEyelids
...
37
2.5.8LouseInfestationoftheEyelids
...
37
2.6 DisordersoftheEyelidGlands
...
38
2.6.1Hordeolum
...
38
2.6.2Chalazion
...
39
2.7 Tumors
...
40
2.7.1 BenignTumors
...
40
2.7.1.1DuctalCysts
...
40
2.7.1.2Xanthelasma
...
40
2.7.1.3MolluscumContagiosum
...
42
2.7.1.4CutaneousHorn
...
42
2.7.1.5Keratoacanthoma
...
42
2.7.1.6Hemangioma
...
43
2.7.1.7Neurofibromatosis(Recklinghausen’sDisease)
...
44
2.7.2MalignantTumors
...
45
2.7.2.1BasalCellCarcinoma
...
45
2.7.2.2SquamousCellCarcinoma
...
47
2.7.2.3Adenocarcinoma
...
47
3 LacrimalSystem...
49
(PeterWagner,GerhardK.Lang)
3.1 BasicKnowledge
...
49
3.2 ExaminationMethods
...
52
3.2.1EvaluationofTearFormation
...
52
3.2.2EvaluationofTearDrainage
...
53
3.3 DisordersoftheLowerLacrimalSystem
...
57
3.3.1Dacryocystitis
...
57
3.3.1.1AcuteDacryocystitis
...
57
3.3.1.2ChronicDacryocystitis
...
60
3.3.1.3NeonatalDacryocystitis
...
60
3.3.2Canaliculitis
...
61
3.3.3TumorsoftheLacrimalSac
...
61
3.4 LacrimalSystemDysfunction
...
62
3.4.1KeratoconjunctivitisSicca
...
62
TableofContents
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

8. VIII
3.4.2Illacrimation
...
64
3.5 DisordersoftheLacrimalGland
...
64
3.5.1AcuteDacryoadenitis
...
64
3.5.2ChronicDacryoadenitis
...
65
3.5.3TumorsoftheLacrimalGland
...
66
4 Conjunctiva...
67
(GerhardK.Lang,GabrieleE.Lang)
4.1 BasicKnowledge
...
67
4.2 ExaminationMethods
...
68
4.3 ConjunctivalDegenerationandAgingChanges
...
69
4.3.1Pinguecula
...
69
4.3.2Pterygium
...
69
4.3.3Pseudopterygium
...
71
4.3.4SubconjunctivalHemorrhage
...
72
4.3.5CalcareousInfiltration
...
72
4.3.6ConjunctivalXerosis
...
72
4.4 Conjunctivitis
...
74
4.4.1GeneralNotesontheCauses,Symptoms,andDiagnosisof
Conjunctivitis
...
74
4.4.2InfectiousConjunctivitis
...
82
4.4.2.1BacterialConjunctivitis
...
82
4.4.2.2ChlamydialConjunctivitis
...
83
4.4.2.3ViralConjunctivitis
...
93
4.4.2.4NeonatalConjunctivitis
...
95
4.4.2.5ParasiticandMycoticConjunctivitis
...
98
4.4.3NoninfectiousConjunctivitis
...
98
4.5 Tumors
...
104
4.5.1EpibulbarDermoid
...
104
4.5.2ConjunctivalHemangioma
...
104
4.5.3EpithelialConjunctivalTumors
...
105
4.5.3.1ConjunctivalCysts
...
105
4.5.3.2ConjunctivalPapilloma
...
106
4.5.3.3ConjunctivalCarcinoma
...
107
4.5.4MelanocyticConjunctivalTumors
...
108
4.5.4.1ConjunctivalNevus
...
108
4.5.4.2ConjunctivalMelanosis
...
108
4.5.4.3CongenitalOcularMelanosis
...
112
4.5.5ConjunctivalLymphoma
...
113
4.5.6Kaposi’sSarcoma
...
113
4.6 ConjunctivalDeposits
...
114
TableofContents
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

9. IX
5 Cornea...
117
(GerhardK.Lang)
5.1 BasicKnowledge
...
117
5.2 ExaminationMethods
...
120
5.2.1SlitLampExamination
...
120
5.2.2DyeExaminationoftheCornea
...
120
5.2.3CornealTopography
...
121
5.2.4DeterminingCornealSensitivity
...
121
5.2.5MeasuringtheDensityoftheCornealEpithelium
...
121
5.2.6MeasuringtheDiameteroftheCornea
...
124
5.2.7CornealPachymetry
...
125
5.2.8ConfocalCornealMicroscopy
...
125
5.3 DevelopmentalAnomalies
...
125
5.3.1ProtrusionAnomalies
...
125
5.3.1.1Keratoconus
...
125
5.3.1.2Keratoglobus
...
127
5.3.2CornealSizeAnomalies(Microcorneaand
Megalocornea)
...
127
5.4 InfectiousKeratitis
...
127
5.4.1ProtectiveMechanismsoftheCornea
...
127
5.4.2CornealInfections:PredisposingFactors,Pathogens,and
Pathogenesis
...
128
5.4.3GeneralNotesonDiagnosingInfectiousFormsof
Keratitis
...
130
5.4.4BacterialKeratitis
...
130
5.4.5ViralKeratitis
...
132
5.4.5.1HerpesSimplexKeratitis
...
132
5.4.5.2HerpesZosterKeratitis
...
134
5.4.6MycoticKeratitis
...
134
5.4.7AcanthamoebaKeratitis
...
136
5.5 NoninfectiousKeratitisandKeratopathy
...
137
5.5.1SuperficialPunctateKeratitis
...
138
5.5.2ExposureKeratitis
...
140
5.5.3NeuroparalyticKeratitis
...
141
5.5.4ProblemswithContactLenses
...
141
5.5.5BullousKeratopathy
...
143
5.6 CornealDeposits,Degenerations,andDystrophies
...
145
5.6.1CornealDeposits
...
145
5.6.1.1ArcusSenilis
...
145
5.6.1.2CornealVerticillata
...
145
5.6.1.3ArgyrosisandChrysiasis
...
146
5.6.1.4IronLines
...
146
5.6.1.5Kayser-FleischerRing
...
146
TableofContents
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10. X
5.6.2CornealDegeneration
...
146
5.6.2.1CalcificBandKeratopathy
...
146
5.6.2.2PeripheralFurrowKeratitis
...
147
5.6.3CornealDystrophies
...
148
5.7 CornealSurgery
...
150
5.7.1 CurativeCornealProcedures
...
152
5.7.1.1PenetratingKeratoplasty(Fig.5.18a)
...
152
5.7.1.2LamellarKeratoplasty(Fig.5.18b)
...
153
5.7.1.3PhototherapeuticKeratectomy(Fig.5.18c)
...
154
5.7.2RefractiveCornealProcedures
...
155
5.7.2.1PhotorefractiveKeratectomy(Fig.5.18d)
...
155
5.7.2.2RadialKeratotomy(Fig.5.18e)
...
155
5.7.2.3PhotorefractiveKeratectomyCorrectionof
Astigmatism
...
156
5.7.2.4HolmiumLaserCorrectionofHyperopia
...
156
5.7.2.5EpikeratophakicKeratoplasty(Epikeratophakia)
...
156
5.7.2.6ExcimerLaserInSituKeratomileusis(LASIK)
...
156
6 Sclera...
157
(GerhardK.Lang)
6.1 BasicKnowledge
...
157
6.2 ExaminationMethods
...
157
6.3 ColorChanges
...
157
6.4 StaphylomaandEctasia
...
158
6.5 Trauma
...
158
6.6 Inflammations
...
158
6.6.1Episcleritis
...
159
6.6.2Scleritis
...
161
7 Lens...
165
(GerhardK.Lang)
7.1 BasicKnowledge
...
165
7.2 ExaminationMethods
...
168
7.3 DevelopmentalAnomaliesoftheLens
...
169
7.4 Cataract
...
170
7.4.1 AcquiredCataract
...
173
7.4.1.1SenileCataract
...
173
7.4.2CataractinSystemicDisease
...
179
7.4.3ComplicatedCataracts
...
180
7.4.4CataractafterIntraocularSurgery
...
180
TableofContents
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11. XI
7.4.5TraumaticCataract
...
180
7.4.6ToxicCataract
...
182
7.4.7CongenitalCataract
...
182
7.4.7.1HereditaryCongenitalCataracts
...
183
7.4.7.2CataractfromTransplacentalInfectionintheFirstTrimester
ofPregnancy
...
185
7.4.8 TreatmentofCataracts
...
185
7.4.8.1MedicalTreatment
...
185
7.4.8.2SurgicalTreatment
...
185
7.4.8.3SecondaryCataract
...
192
7.4.8.4SpecialConsiderationsinCataractSurgeryinChildren
...
192
7.5 LensDislocation
...
195
8 UvealTract
(VascularPigmentedLayer)...
199
(GabrieleE.Lang,GerhardK.Lang)
8.1 BasicKnowledge
...
199
8.1.1 Iris
...
199
8.1.2 CiliaryBody
...
201
8.1.3 Choroid
...
201
8.2 ExaminationMethods
...
201
8.3 DevelopmentalAnomalies
...
202
8.3.1 Aniridia
...
202
8.3.2Coloboma
...
203
8.4 PigmentationAnomalies
...
206
8.4.1Heterochromia
...
206
8.4.2Albinism
...
206
8.5 Inflammation
...
208
8.5.1 AcuteIritisandIridocyclitis
...
208
8.5.2ChronicIritisandIridocyclitis
...
212
8.5.3Choroiditis
...
213
8.5.4SympatheticOphthalmia
...
214
8.6 NeovascularizationintheIris:RubeosisIridis
...
215
8.7 Tumors
...
216
8.7.1 MalignantTumors(UvealMelanoma)
...
216
8.7.2BenignChoroidalTumors
...
217
TableofContents
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12. XII
9 Pupil...
219
(OskarGareis,GerhardK.Lang)
9.1 BasicKnowledge
...
219
9.2 ExaminationMethods
...
221
9.2.1TestingtheLightReflex(Table9.1)
...
221
9.2.2EvaluatingtheNearReflex
...
223
9.3 InfluenceofPharmacologicAgentsonthePupil
(Table9.2)
...
224
9.4 PupillaryMotorDysfunction
...
226
9.4.1IsocoriawithNormalPupilSize
...
227
9.4.2AnisocoriawithDilatedPupilintheAffectedEye
...
228
9.4.3AnisocoriawithaConstrictedPupilintheAffectedEye
...
229
9.4.4IsocoriawithConstrictedPupils
...
230
9.3.5IsocoriawithDilatedPupils
...
231
10 Glaucoma...
233
(GerhardK.Lang)
10.1 BasicKnowledge
...
233
10.2ExaminationMethods
...
238
10.2.1ObliqueIlluminationoftheAnteriorChamber
...
238
10.2.2Slit-LampExamination
...
238
10.2.3Gonioscopy
...
238
10.2.4MeasuringIntraocularPressure
...
240
10.2.5OpticDiskOphthalmoscopy
...
244
10.2.6VisualFieldTesting
...
246
10.2.7ExaminationoftheRetinalNerveFiberLayer
...
250
10.3PrimaryGlaucoma
...
251
10.3.1PrimaryOpenAngleGlaucoma
...
251
10.3.2PrimaryAngleClosureGlaucoma
...
265
10.4SecondaryGlaucomas
...
270
10.4.1SecondaryOpenAngleGlaucoma
...
271
10.4.2SecondaryAngleClosureGlaucoma
...
271
10.5ChildhoodGlaucomas
...
273
11 VitreousBody...
279
(ChristophW.Spraul,GerhardK.Lang)
11.1 BasicKnowledge
...
279
11.2 ExaminationMethods
...
281
11.3 AgingChanges
...
282
TableofContents
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13. XIII
11.3.1Synchysis
...
282
11.3.2VitreousDetachment
...
282
11.4 AbnormalChangesintheVitreousBody
...
284
11.4.1PersistentFetalVasculature(Developmental
Anomalies)
...
284
11.4.1.1Mittendorf’sDot
...
284
11.4.1.2Bergmeister’sPapilla
...
285
11.4.1.3PersistentHyaloidArtery
...
285
11.4.1.4PersistentHyperplasticPrimaryVitreous(PHPV)
...
285
11.4.2AbnormalOpacitiesoftheVitreousBody
...
287
11.4.2.1AsteroidHyalosis
...
287
11.4.2.2SynchysisScintillans
...
287
11.4.2.3VitreousAmyloidosis
...
287
11.4.3VitreousHemorrhage
...
287
11.4.4VitritisandEndophthalmitis
...
290
11.4.5VitreoretinalDystrophies
...
293
11.4.5.1JuvenileRetinoschisis
...
293
11.4.5.2Wagner’sDisease
...
293
11.5 TheRoleoftheVitreousBodyinVariousOcularChangesand
FollowingCataractSurgery
...
293
11.5.1RetinalDetachment
...
293
11.5.2RetinalVascularProliferation
...
293
11.5.3CataractSurgery
...
294
11.6 SurgicalTreatment:Vitrectomy
...
294
12 Retina...
299
(GabrieleE.Lang,GerhardK.Lang)
12.1 BasicKnowledge
...
299
12.2ExaminationMethods
...
304
12.2.1ExaminationoftheFundus
...
304
12.2.2NormalandAbnormalFundusFindingsinGeneral
...
308
12.2.3ColorVision
...
311
12.2.4ElectrophysiologicExaminationMethods
(electroretinogram,electrooculogram,andvisualevoked
potentials;seeFig.12.2a)
...
312
12.3VascularDisorders
...
314
12.3.1DiabeticRetinopathy
...
314
12.3.2RetinalVeinOcclusion
...
318
12.3.3RetinalArterialOcclusion
...
320
12.3.4HypertensiveRetinopathyandScleroticChanges
...
323
12.3.5Coats’Disease
...
325
12.3.6RetinopathyofPrematurity
...
326
TableofContents
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14. XIV
12.4DegenerativeRetinalDisorders
...
328
12.4.1RetinalDetachment
...
328
12.4.2DegenerativeRetinoschisis
...
333
12.4.3PeripheralRetinalDegenerations
...
334
12.4.4CentralSerousChorioretinopathy
...
335
12.4.5Age-RelatedMacularDegeneration
...
337
12.4.6DegenerativeMyopia
...
339
12.5RetinalDystrophies
...
340
12.5.1MacularDystrophies
...
340
12.5.1.1Stargardt’sDisease
...
340
12.5.1.2Best’sVitelliformDystrophy
...
341
12.5.2RetinitisPigmentosa
...
343
12.6ToxicRetinopathy
...
345
12.7RetinalInflammatoryDisease
...
346
12.7.1RetinalVasculitis
...
346
12.7.2PosteriorUveitisDuetoToxoplasmosis
...
348
12.7.3AIDS-RelatedRetinalDisorders
...
349
12.7.4ViralRetinitis
...
351
12.7.5RetinitisinLymeDisease
...
351
12.7.6ParasiticRetinalDisorders
...
352
12.8RetinalTumorsandHamartomas
...
353
12.8.1Retinoblastoma
...
353
12.8.2Astrocytoma
...
355
12.8.3Hemangiomas
...
356
13 OpticNerve...
359
(OskarGareis,GerhardK.Lang)
13.1 BasicKnowledge
...
359
13.1.1IntraocularPortionoftheOpticNerve
...
360
13.1.2TheIntraorbitalandIntracranialPortionoftheOptic
Nerve
...
361
13.2ExaminationMethods
...
362
13.3DisordersthatObscuretheMarginoftheOpticDisc
...
363
13.3.1CongenitalDisordersthatObscuretheMarginoftheOptic
Disc
...
363
13.3.1.1ObliqueEntryoftheOpticNerve
...
363
13.3.1.2TiltedDisc
...
364
13.3.1.3Pseudopapilledema
...
364
13.3.1.4MyelinatedNerveFibers
...
365
13.3.1.5Bergmeister’sPapilla
...
366
13.3.1.6OpticDiscDrusen
...
366
13.3.2AcquiredDisordersthatObscuretheMarginoftheOptic
Disc
...
367
TableofContents
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15. XV
13.3.2.1Papilledema
...
368
13.3.2.2OpticNeuritis
...
370
13.3.2.3AnteriorIschemicOpticNeuropathy(AION)
...
374
13.3.2.4InfiltrativeOpticDiscEdema
...
379
13.4DisordersinwhichtheMarginoftheOpticDiscisWell
Defined
...
380
13.4.1AtrophyoftheOpticNerve
...
380
13.4.2OpticNervePits
...
383
13.4.3OpticDiscColoboma(MorningGloryDisc)
...
385
13.5 Tumors
...
385
13.5.1IntraocularOpticNerveTumors
...
385
13.5.2RetrobulbarOpticNerveTumors
...
387
14 VisualPathway...
389
(OskarGareis,GerhardK.Lang)
14.1 BasicKnowledge
...
389
14.2ExaminationMethods
...
391
14.3DisordersoftheVisualPathway
...
394
14.3.1PrechiasmalLesions
...
394
14.3.2ChiasmalLesions
...
396
14.3.3RetrochiasmalLesions
...
400
15 OrbitalCavity...
403
(ChristophW.Spraul,GerhardK.Lang)
15.1 BasicKnowledge
...
403
15.2ExaminationMethods
...
405
15.3DevelopmentalAnomalies
...
409
15.3.1CraniofacialDysplasia
...
409
15.3.1.1Craniostenosis
...
409
15.3.2MandibulofacialDysplasia
...
410
15.3.2.1OculoauriculovertebralDysplasia
...
410
15.3.2.2MandibulofacialDysostosis
...
410
15.3.2.3OculomandibularDysostosis
...
410
15.3.2.4Rubinstein–TaybiSyndrome
...
410
15.3.3Meningoencephalocele
...
410
15.3.4Osteopathies
...
411
15.4OrbitalInvolvementinAutoimmuneDisorders:
Graves’Disease
...
411
15.5OrbitalInflammation
...
413
15.5.1OrbitalCellulitis
...
414
TableofContents
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16. XVI
15.5.2CavernousSinusThrombosis
...
415
15.5.3OrbitalPseudotumor
...
416
15.5.4Myositis
...
416
15.5.5OrbitalPeriostitis
...
417
15.5.6Mucocele
...
417
15.5.7Mycoses(MucormycosisandAspergillomycosis)
...
417
15.6 VascularDisorders
...
418
15.6.1PulsatingExophthalmos
...
418
15.6.2IntermittentExophthalmos
...
419
15.6.3OrbitalHematoma
...
419
15.7 Tumors
...
420
15.7.1OrbitalTumors
...
420
15.7.1.1Hemangioma
...
420
15.7.1.2DermoidandEpidermoidCyst
...
420
15.7.1.3NeurinomaandNeurofibroma
...
420
15.7.1.4Meningioma
...
420
15.7.1.5HistiocytosisX
...
421
15.7.1.6LeukemicInfiltrations
...
421
15.7.1.7Lymphoma
...
421
15.7.1.8Rhabdomyosarcoma
...
421
15.7.2Metastases
...
421
15.7.3OpticNerveGlioma
...
422
15.8OrbitalSurgery
...
422
16 OpticsandRefractiveErrors...
423
(ChristophW.Spraul,GerhardK.Lang)
16.1 BasicKnowledge
...
423
16.1.1UncorrectedandCorrectedVisualAcuity
...
423
16.1.2Refraction:EmmetropiaandAmetropia
...
423
16.1.3Accommodation
...
425
16.1.4AdaptationtoDifferencesinLightIntensity
...
428
16.2ExaminationMethods
...
429
16.2.1RefractionTesting
...
429
16.2.2.TestingthePotentialResolvingPoweroftheRetinainthe
PresenceofOpacifiedOpticMedia
...
431
16.3RefractiveAnomalies(Table16.2)
...
432
16.3.1Myopia(Shortsightedness)
...
432
16.3.2Hyperopia(Farsightedness)
...
436
16.3.3Astigmatism
...
440
16.3.4Anisometropia
...
444
16.4ImpairedAccommodation
...
445
16.4.1AccommodationSpasm
...
445
TableofContents
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17. XVII
16.4.2AccommodationPalsy
...
446
16.5CorrectionofRefractiveErrors
...
447
16.5.1EyeglassLenses
...
447
16.5.2ContactLenses
...
451
16.5.2.1AdvantagesandCharacteristicsofContactLenses
...
451
16.5.3Prisms
...
455
16.5.4MagnifyingVisionAids
...
455
16.5.5AberrationsofLensesandEyeglasses
...
456
17 OcularMotilityandStrabismus...
459
(DorisRecker,JosefAmann,GerhardK.Lang)
17.1 BasicKnowledge
...
459
17.2 ConcomitantStrabismus
...
465
17.2.1FormsofConcomitantStrabismus
...
467
17.2.1.1Esotropia
...
467
17.2.1.2AbnormalAccommodativeConvergence/Accommodation
Ratio
...
470
17.2.1.3Exotropia
...
471
17.2.1.4VerticalDeviations(HypertropiaandHypotropia)
...
471
17.2.2DiagnosisofConcomitantStrabismus
...
471
17.2.2.1EvaluatingOcularAlignmentwithaFocusedLight
...
471
17.2.2.2DiagnosisofInfantileStrabismicAmblyopia(Preferential
LookingTest)
...
472
17.2.2.3DiagnosisofUnilateralandAlternatingStrabismus(Unilateral
CoverTest)
...
473
17.2.2.4MeasuringtheAngleofDeviation
...
474
17.2.2.5DeterminingtheTypeofFixation
...
476
17.2.2.6TestingBinocularVision
...
476
17.2.3TherapyofConcomitantStrabismus
...
477
17.2.3.1EyeglassPrescription
...
477
17.2.3.2TreatmentandAvoidanceofStrabismicAmblyopia
...
477
17.2.3.3Surgery
...
479
17.3 Heterophoria
...
480
17.4 Pseudostrabismus
...
481
17.5 OphthalmoplegiaandParalyticStrabismus
...
481
17.6 Nystagmus
...
494
TableofContents
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18. XVIII
18 OcularTrauma...
497
(GerhardK.Lang)
18.1 ExaminationMethods
...
497
18.2ClassificationofOcularInjuriesbyMechanismof
Injury
...
498
18.3MechanicalInjuries
...
498
18.3.1EyelidInjury
...
498
18.3.2InjuriestotheLacrimalSystem
...
499
18.3.3ConjunctivalLaceration
...
499
18.3.4CornealandConjunctivalForeignBodies
...
503
18.3.5CornealErosion
...
505
18.3.6BluntOcularTrauma(OcularContusion)
...
506
18.3.7BlowoutFracture
...
507
18.3.8Open-GlobeInjuries
...
514
18.3.9ImpalementInjuriesoftheOrbit
...
515
18.4ChemicalInjuries
...
517
18.5InjuriesDuetoPhysicalAgents
...
523
18.5.1UltravioletKeratoconjunctivitis
...
523
18.5.2Burns
...
523
18.5.3RadiationInjuries(IonizingRadiation)
...
524
18.6IndirectOcularTrauma:Purtscher’sRetinopathy
...
525
19 CardinalSymptoms...
527
(GerhardK.Lang)
Index...
563
TableofContents
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19. 1
1 TheOphthalmicExamination
GabrieleE.LangandGerhardK.Lang
1.1 Equipment
Thebasicequipmentfortheophthalmicexaminationincludesthefollowing
instruments:
!Directophthalmoscopeforexaminingthefundus(Fig.1.1).
!Focusedlight(Fig.1.1)forexaminingthereactionofthepupilandtheante-
riorchamber.
!Asphericlens(Fig.1.1)forexaminingtheanteriorchamber.
!Eyechartfortestingvisualacuityatadistanceof5meters(20feet)
(Fig.1.2).
Basicdiagnosticinstrumentsforthefundus,pupil,andanteriorchamber.
Fig.1.1Fromlefttoright:direct
ophthalmoscope,asphericlens,and
focusedlight.
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20. 2
Eyechartsfortestingvisualacuityatadistanceof5meters.
Fig.1.2From
lefttoright:Snel-
lenletterchart,
Arabicnumber
chart,Egame,
Landoltbroken
rings,children’s
pictograph.
!Binocularloupesforremovingcornealandconjunctivalforeignbodies.
!Desmarreseyelidretractorandglassrodorsterilecottonswabforeyelid
eversion(Fig.1.3).
Foreign-bodyneedleforremovingsuperficialcornealforeignbodies(Fig.1.3).
Recommendedmedications:
!Topicalanesthetic(suchasoxybuprocaine0.4%eyedrops)toprovidelocal
anesthesiaduringremovalofconjunctivalandcornealforeignbodiesand
superficialanesthesiapriortoflushingtheconjunctivalsacinchemical
injuries.
!Sterilebuffersolutionforprimarytreatmentofchemicalinjuries.
!Antibioticeyedropsforfirstaidtreatmentofinjuries,sterileeyecompresses,
anda1cmadhesivebandageforprotectivebandaging.
Anophthalmologistshouldbeconsultedfollowinganyemergency
treatmentofeyeinjuries.
1TheOphthalmicExamination
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21. 3
Basicdiagnosticequipmentforremovingcornealforeignbodiesandeyelid
eversion.
Fig.1.3Fromlefttoright:Foreign
bodyneedle,glassspatula,andDes-
marreseyelidretractor.
1.2 History
Acompletehistoryincludesfouraspects:
1.Familyhistory.Manyeyedisordersarehereditaryorofhigherincidencein
membersofthesamefamily.Examplesincluderefractiveerrors,stra-
bismus,cataract,glaucoma,retinaldetachment,andretinaldystrophy.
2.Medicalhistory.Asocularchangesmayberelatedtosystemicdisorders,
thispossibilitymustbeexplored.Conditionsaffectingtheeyesinclude
diabetesmellitus,hypertension,infectiousdiseases,rheumaticdisorders,
skindiseases,andsurgery.Eyedisorderssuchascorticosteroid-induced
glaucoma,corticosteroid-inducedcataract,andchloroquine-induced
maculopathycanoccurasaresultoftreatmentwithmedicationssuchas
steroids,chloroquine,Amiodarone,Myambutol,orchlorpromazine(see
tableinAppendix).
3.Ophthalmichistory.Theexaminershouldinquireaboutcorrectivelenses,
strabismusoramblyopia,posttraumaticconditions,andsurgeryoreye
inflammation.
1.2History
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22. 4
4.Currenthistory.Whatsymptomsdoesthepatientpresentwith?Doesthe
patienthaveimpairedvision,pain,rednessoftheeye,ordoublevision?
Whendidthesesymptomsoccur?Areinjuriesorassociatedgeneralized
symptomspresent?
1.3 VisualAcuity
Visualacuity,thesharpnessofnearanddistancevision,istestedseparately
foreacheye.Oneeyeiscoveredwithapieceofpaperorthepalmofthehand
placedlightlyovertheeye.Thefingersshouldnotbeusedtocovertheeye
becausethepatientwillbeabletoseebetweenthem(Fig.1.4).
Thegeneralpractitionerorstudentcanperformanapproximatetestof
visualacuity.Thepatientisfirstaskedtoidentifycertainvisualsymbols
referredtoasoptotypes(seeFig.1.2)atadistanceof5metersor20feet(testof
distancevision).Thesevisualsymbolsaredesignedsothatoptotypesofacer-
tainsizecanbarelyberesolvedbythenormaleyeataspecifieddistance(this
standarddistanceisspecifiedinmetersnexttotherespectivesymbol).The
eyechartsmustbecleanandwellilluminatedfortheexamination.The
sharpnessofvisionmeasuredisexpressedasafraction:
Examiningvisualacuity.
Fig.1.4Thepalmofthehandis
placedlightlyovertheeyetocoverit
toallowtestingofthedistanceand
nearvisionintheoppositeeye.
1TheOphthalmicExamination
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23. 5
actualdistance
standarddistance
!visualacuity.
Normalvisualacuityis5/5(20/20),or1.0asadecimalnumber,wherethe
actualdistanceequalsthestandarddistance.
Anexampleofdiminishedvisualacuity(seeFig.1.2):Thepatientsees
onlythe“4”andnoneofthesmallersymbolsonthelefteyechartatadistance
of5meters(20feet)(actualdistance).Anormal-sightedpersonwouldbeable
todiscernthe“4”atadistanceof50metersor200feet(standarddistance).
Accordingly,thepatienthasavisualacuityof5/50(20/200)or0.1.
Theophthalmologisttestsvisualacuityafterdeterminingobjective
refractionusingtheintegrallenssystemofaPhoroptor,oraboxofindividual
lensesandanimageprojectorthatprojectsthevisualsymbolsatadefined
distanceinfrontoftheeye.Visualacuityisautomaticallycalculatedfromthe
fixedactualdistanceandisdisplayedasadecimalvalue.Pluslenses(convex
lenses)areusedforfarsightedness(hyperopiaorhypermetropia),minus
lenses(concavelenses)fornearsightedness(myopia),andcylindricallensesfor
astigmatism.
Ifthepatientcannotdiscernthesymbolsontheeyechartatadistanceof5
meters(20feet),theexaminershowsthepatientthechartatadistanceof1
meteror3feet(boththeophthalmologistandthegeneralpractitioneruse
eyechartsforthisexamination).Ifthepatientisstillunabletodiscernany
symbols,theexaminerhasthepatientcountfingers,discernthedirectionof
handmotion,anddiscernthedirectionofapointlightsource.
1.4 OcularMotility
Withthepatient’sheadimmobilized,theexaminerasksthepatienttolookin
eachoftheninediagnosticpositionsofgaze:1,straightahead;2,right;3,
upperright;4,up;5,upperleft;6,left;7,lowerleft;8,down;and9,lower
right(Fig.1.5).Thisallowstheexaminertodiagnosestrabismus,paralysisof
ocularmuscles,andgazeparesis.
Evaluatingthesixcardinaldirectionsofgaze(right,left,upperright,
lowerright,upperleft,lowerleft)issufficientwhenexaminingparalysisof
theoneofthesixextraocularmuscles.Themotionimpairmentoftheeye
resultingfromparalysisofanocularmusclewillbemostevidentinthese
positions.Onlyoneoftherectusmusclesisinvolvedineachoftheleftand
rightpositionsofgaze(lateralormedialrectusmuscle).Allotherdirectionsof
gazeinvolveseveralmuscles.
1.4OcularMotility
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24. 6
Evaluatingtheninediagnosticpositionsofgaze.
Fig.1.5Thisexaminationallowstheexaminertodiagnosestrabismus,paralysisof
ocularmuscles,andgazeparesis.
1.5 BinocularAlignment
Binocularalignmentisevaluatedwithacovertest.Theexaminerholdsa
pointlightsourcebeneathhisorherowneyesandobservesthelightreflec-
tionsinthepatient’scorneasinthenearfield(40cm)andatadistance(5m).
Thereflectionsarenormallyinthecenterofeachpupil.Ifthecornealreflection
isnotinthecenterofthepupilinoneeye,thenatropiaispresentinthateye.
Thentheexaminercoversoneeyewithahandoranoccluder(Fig.1.6)and
testswhethertheuncoveredeyemakesacompensatorymovement.Compen-
satorymovementoftheeyeindicatesthepresenceoftropia.However,there
willalsobealackofcompensatorymovementiftheeyeisblind.Thecover
testisthenrepeatedwiththeothereye.
Iftropiaispresentinanewbornwithextremelypoorvision,thebabywill
nottoleratethegoodeyebeingcovered.
1TheOphthalmicExamination
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25. 7
Evaluationofbinocularalignment.
Fig.1.6Theex-
aminercovers
oneeyeofthe
patientwiththe
handtotestif
theuncovered
eyemakesa
compensatory
movementindi-
catingpresence
oftropia.
1.6 ExaminationoftheEyelidsandNasolacrimalDuct
Theuppereyelidcoversthesuperiormarginofthecornea.Afewmillimeters
ofthesclerawillbevisibleabovethelowereyelid.Theeyelidsareindirectcon-
tactwiththeeyeball.
Stenosisofthenasolacrimalductproducesapooloftearsinthemedial
angleoftheeyewithlacrimation(epiphora).Ininflammationofthelacrimal
sac,pressureonthenasolacrimalsacfrequentlycausesarefluxofmucusor
pusfromtheinferiorpunctum.Patencyofthenasolacrimalductistestedby
instillinga10%fluoresceinsolutionintheconjunctivalsacoftheeye.Ifthe
dyeispresentinnasalmucusexpelledintopapertissueaftertwominutes,
thelacrimalductisopen(seealsop.53).
Duetothedangerofinfection,anyprobingorirrigationofthe
nasolacrimalductshouldbeperformedonlybyanophthalmologist.
1.7 ExaminationoftheConjunctiva
Theconjunctivaisexaminedbydirectinspection.Thebulbarconjunctivais
directlyvisiblebetweentheeyelids;thepalpebralconjunctivacanonlybe
examinedbyevertingtheupperorlowereyelid.Thenormalconjunctivais
smooth,shiny,andmoist.Theexaminershouldbealerttoanyreddening,
secretion,thickening,scars,orforeignbodies.
Eversionofthelowereyelid.Thepatientlooksupwhiletheexaminerpulls
theeyeliddownwardclosetotheanteriormargin(Fig.1.7).Thisexposesthe
conjunctivaandtheposteriorsurfaceofthelowereyelid.
1.7ExaminationoftheConjunctiva
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26. 8
Examinationofthelowereyelidandinferiorfornix.
Fig.1.7The
lowereyelidmust
beevertedfor
thisexamination.
Thepatientlooks
upwhiletheex-
aminerpullsthe
eyeliddownward
closetotheante-
riormargin.
Examinationoftheuppereyelid(simpleeversion).
Fig.1.8Thepatientrelaxesand
looksdown.Theexaminerplacesa
swabsuperiortothetarsalregionof
theuppereyelid,graspstheeyelashes
oftheuppereyelidbetweenthe
thumbandforefinger,andevertsthe
eyelidusingtheswabasafulcrum.
Eversionoftheuppereyelid.Simpleeversion(Fig.1.8).Thepatientisaskedto
lookdown.Thepatientshouldrepeatedlybetoldtorelaxandtoavoidtightly
shuttingtheoppositeeye.Thisrelaxesthelevatorpalpebraesuperiorisand
orbicularisoculimuscles.Theexaminergraspstheeyelashesoftheupper
eyelidbetweenthethumbandforefingerandevertstheeyelidagainstaglass
rodorswabusedasafulcrum.Eversionshouldbeperformedwithaquick
leveringmotionwhileapplyingslighttraction.Thepalpebralconjunctivacan
thenbeinspectedandcleanedifnecessary.
1TheOphthalmicExamination
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27. 9
Fulleversionwithretractor.Toexposethesuperiorfornix,theuppereyelidis
fullyevertedaroundaDesmarreseyelidretractor(Figs.1.9aandb).This
methodisusedsolelybytheophthalmologistandisonlydiscussedherefor
thesakeofcompleteness.Thiseversiontechniqueisrequiredtoremovefor-
eignbodiesor“lost”contactlensesfromthesuperiorfornixortocleanthe
conjunctivaoflimeparticlesinachemicalinjurywithlime.
Examinationoftheuppereyelidandsuperiorfornix(fulleversionwith
retractor).
Figs.1.9aandbInthiscase,theex-
aminerevertstheeyelidaroundaDes-
marreseyelidretractor.Incontrastto
simpleeversion,thisprocedureallows
examinationofthesuperiorfornixin
additiontothepalpebralconjunctiva.
1.7ExaminationoftheConjunctiva
a
b
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28. 10
Examinationoftheanteriorportionoftheeye.
Fig.1.10The
examinerevalu-
atestheeye
usingafocallight
sourceandloupe
magnification.
1TheOphthalmicExamination
Blepharospasmcanrendersimpleandfulleversionverydifficult
especiallyinthepresenceofchemicalinjury.Inthesecases,thespasm
shouldfirstbeeliminatedbyinstillingatopicalanestheticsuchasoxy-
buprocainehydrochlorideeyedrops.
1.8 ExaminationoftheCornea
Thecorneaisexaminedwithapointlightsourceandaloupe(Fig.1.10).The
corneaissmooth,clear,andreflective.Thereflectionisdistortedinthepres-
enceofcornealdisorders.Epithelialdefects,whicharealsoverypainful,will
takeonanintensegreencolorafterapplicationoffluoresceindye;corneal
infiltratesandscarsaregrayishwhite.Evaluatingcornealsensitivityisalso
important.Sensitivityisevaluatedbilaterallytodetectpossibledifferencesin
thereactionofbotheyes.Thepatientlooksstraightaheadduringtheexami-
nation.Theexaminerholdstheuppereyelidtopreventreflexiveclosingand
touchesthecorneaanteriorly(Fig.1.11).Decreasedsensitivitycanprovide
informationabouttrigeminalorfacialneuropathy,ormaybeasignofaviral
infectionofthecornea.
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29. 11
Evaluationofcornealsensitivity.
Fig.1.11Cornealsensitivitycanbe
evaluatedwithadistendedcotton
swab.Thepatientlooksstraight
aheadwhiletheexaminerholdsthe
uppereyelidandtouchesthecornea
anteriorly.
1.9ExaminationoftheAnteriorChamber
1.9 ExaminationoftheAnteriorChamber
Theanteriorchamberisfilledwithclearaqueoushumor.Cellularinfiltration
andcollectionofpusmayoccur(hypopyon).Bleedingintheanteriorcham-
berisreferredtoashyphema.
Itisimportanttoevaluatethedepthoftheanteriorchamber.Inacham-
berofnormaldepth,theiriscanbewellilluminatedbyalaterallightsource
(Fig.1.12).Inashallowanteriorchambertherewillbeamedialshadowonthe
iris.Thepupillarydilationshouldbeavoidedinpatientswithshallowante-
riorchambersbecauseoftheriskofprecipitatingaglaucomaattack.Older
patientswith“small”hyperopiceyesareaparticularriskgroup.
Dilationofthepupilwithamydriaticiscontraindicatedinpatientswitha
shallowanteriorchamberduetotheriskofprecipitatingangleclosure
glaucoma.
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30. 12
Evaluationofthedepthoftheanteriorchamber.
Illuminated area
a
Shadow
b
Fig.1.12aNormalanteriorchamberdepth:theiriscanbewellilluminatedbya
laterallightsource.bShallowanteriorchamber:amedialshadowisvisibleonthe
iris.
1TheOphthalmicExamination
1.10 ExaminationoftheLens
Theophthalmologistusesaslitlamptoexaminethelens.Theeyecanalsobe
examinedwithafocusedlightifnecessary.
Directilluminationwillproducearedreflectionofthefundusifthelensisclear
andgrayshadowsiflensopacitiesarepresent.Theexaminerthenilluminates
theeyelaterallywithafocusedlightheldasclosetotheeyeaspossibleand
inspectstheeyethrougha+14diopterloupe(seeFig.1.10).Thisexamination
permitsbetterevaluationofchangesintheconjunctiva,cornea,andanterior
chamber.Withsevereopacificationofthelens,agraycolorationwillbevis-
ibleinthepupillaryplane.Anysuchlight-scatteringopacityisreferredtoasa
cataract.
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31. 13
Ophthalmoscopy.
Fig.1.13Adirectophthalmoscopeproducesanerectimageofthefundus.Theex-
aminerviewsthepatient’srighteyewithhisorherownrighteyesothattheirnoses
donotinterferewiththeexamination.Theexaminer’srighthandrestsonthedialof
theophthalmoscopetobringtheretinaintofocus.
1.11Ophthalmoscopy
1.11 Ophthalmoscopy
Indirectophthalmoscopyisusuallyperformedbytheophthalmologist(see
p.306)andproducesalaterallyreversedimageofthefundus.Less
experiencedexaminerswillpreferdirectophthalmoscopy.Here,theoph-
thalmoscopeisheldasclosetothepatientaspossible(Fig.1.13;seealsoFigs.
12.4bandc).Refractiveerrorsinthepatient’seyeandtheexaminer’seyeare
correctedbyselectingtheophthalmoscopelensrequiredtobringtheretina
intofocus.Theexaminerseesanerect,16powermagnifiedimageoftheret-
ina.Theexaminationshouldbeperformedinaslightlydarkenedroomwith
thepatient’spupilsdilated.Studentsshouldbeabletoidentifytheopticdisk.
Inanormaleye,itissharplydefinedstructurewithvitalcoloration(i.e.,yel-
lowishorange)attheleveloftheretinaandmayhaveacentralexcavation.
Thecentralveinlieslateraltotheartery;venousdiameterisnormally1.5
timesgreaterthanarterialdiameter.Eachvascularstructureshouldbeofuni-
formdiameter,andthereshouldbenovascularconstrictionwherevessels
overlap.Aspontaneousvenouspulseisnormal;anarterialpulseisabnormal.
Youngerpatientswillhaveafovealandmacularlightreflex,andtheretina
willhaveareddishcolor(seeFig.12.8).Anophthalmologistshouldbecon-
sultedifthereareanyabnormalfindings.
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32. 14
Confrontationfieldtesting.
Fig.1.14Confrontationtest:thepatientfacestheexamineratadistanceof1m
withhisorhereyesatthesamelevelastheexaminer’s.Eachfocusesontheother’s
oppositeeyewhilecoveringtheircontralateraleyewiththepalmofthehand.The
examinermovesapenfromtheperipherytowardthemidlineinallfourquadrantsin
thenasalandtemporalfieldsandinthesuperiorandinferiorfields.
1TheOphthalmicExamination
1.12 ConfrontationFieldTesting
Confrontationtestingprovidesgrossscreeningofthefieldofvisionwhere
perimetrytestsarenotavailable(seep.391).
Thepatientfacestheexamineratastandarddistanceof1mwithhisorher
eyesatthesamelevelastheexaminer’s(Fig.1.14).Bothfocusontheother’s
oppositeeye(i.e.,thepatient’slefteyefocusesontheexaminer’srighteye)
whilecoveringtheircontralateraleyewiththepalmofthehand.The
examinermovesanobjectsuchasapen,cottonswab,orfingerfromthe
peripherytowardthemidlineinallfourquadrants(inthesuperiorandinfe-
riornasalfieldsandsuperiorandinferiortemporalfields).Apatientwitha
normalfieldofvisionwillseetheobjectatthesametimeastheexaminer;a
patientwithanabnormalorrestrictedfieldofvisionwillseetheobjectlater
thantheexaminer.
Confrontationtestingisagrossmethodofassessingthefieldofvision.
Itcanbeusedtodiagnoseaseverelyrestrictedfieldofvisionsuchas
homonymoushemianopsiaorquadrantanopsia.
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33. 151.14Eyedrops,Ointment,andBandages
Measurementofintraocularpressure.
Fig.1.15The
examineruses
bothindexfin-
gerstopalpate
theeyethrough
theuppereyelid.
1.13 MeasurementofIntraocularPressure
Withthepatient’seyesclosed,theexaminerplaceshisorherhandsonthe
patient’sheadandpalpatestheeyethroughtheuppereyelidwithbothindex
fingers(Fig.1.15).Thetestisrepeatedonthecontralateraleyeforcomparison.
A“rockhard”eyeballonlyoccursinacuteangleclosureglaucoma.
Slightincreasesinintraocularpressuresuchasoccurinchronicglau-
comawillnotbepalpable.
1.14 Eyedrops,Ointment,andBandages
Eyedropsandointmentshouldbeadministeredposteriortotheevertedlower
eyelid.Onedroporstripofointmentapproximately1cmlongshouldbe
administeredlaterallytotheinferiorconjunctivalsac.Toavoidinjurytothe
eye,dropsshouldbeadministeredwiththepatientsupine(Fig.1.16)orseated
withtheheadtiltedbackandsupported.Thepersonadministeringthemedi-
cationplaceshisorherhandonthepatient’sfaceforsupport.Bottlesand
tubesmustnotcomeincontactwiththepatient’seyelashesastheymight
otherwisebecomecontaminated.Allowthedropsorstripofointmentto
dropintotheconjunctivalsac.
Eyeointmentshouldnotbeadministeredfollowingoculartraumaas
thismaycomplicatesubsequentexaminationorsurgery.Dilationofthe
pupilswithamydriaticinunconsciouspatientsshouldbeavoidedas
thiscomplicatesneurologicexamination.
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34. 16 1TheOphthalmicExamination
Administrationofeyedropswiththepatientsupine.
Fig.1.16Eye-
dropsshouldbe
administered
posteriortothe
evertedlower
eyelid.
Eyebandage.Asterileswaborcommerciallyavailablebandage(twooval
layersofbandagematerialwithalayercottonbetweenthem)maybeused.
Careshouldbetakentoavoidtouchingthesideincontactwiththeeye.The
bandageisfixedtotheforeheadandcheekwithstripsofadhesivetape.
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35. 17
2 TheEyelids
PeterWagnerandGerhardK.Lang
2.1 BasicKnowledge
Protectivefunctionoftheeyelids:Theeyelidsarefoldsofmuscularsoft
tissuethatlieanteriortotheeyeballandprotectitfrominjury.Theirshapeis
suchthattheeyeballiscompletelycoveredwhentheyareclosed.Strong
mechanical,optical,andacousticstimuli(suchasaforeignbody,blinding
light,orsuddenloudnoise)“automatically”elicitaneyeclosingreflex.The
corneaisalsoprotectedbyanadditionalupwardmovementoftheeyeball
(Bell’sphenomenon).Regularblinking(20–30timesaminute)helpstouni-
formlydistributeglandularsecretionsandtearsovertheconjunctivaandcor-
nea,keepingthemfromdryingout.
Structureoftheeyelids:Theeyelidsconsistofsuperficialanddeeplayers
(Fig.2.1).
!Superficiallayer:
–Thin,wellvascularizedlayerofskin.
–Sweatglands.
–Modifiedsweatglandandsebaceousglands(ciliaryglandsorglandsof
Moll)andsebaceousglands(glandsofZeis)inthevicinityoftheeye-
lashes.
–Striatedmusclefibersoftheorbicularisoculimusclethatactively
closestheeye(suppliedbythefacialnerve).
!Deeplayer:
–Thetarsalplategivestheeyelidfirmnessandshape.
–Smoothmusculatureofthelevatorpalpebraethatinsertsintothetarsal
plate(tarsalmuscle).Thetarsalmuscleissuppliedbythesympathetic
nervoussystemandregulatesthewidthofthepalpebralfissure.High
sympathetictonecontractsthetarsalmuscleandwidensthepalpebral
fissure;lowsympathetictonerelaxesthetarsalmuscleandnarrowsthe
palpebralfissure.
–Thepalpebralconjunctivaisfirmlyattachedtothetarsalplate.Itforms
anarticularlayerfortheeyeball.Everytimetheeyeblinks,itactslikea
windshieldwiperanduniformlydistributesglandularsecretionsand
tearsovertheconjunctivaandcornea.
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36. 18
Sagittalsectionthroughtheuppereyelid.
Accessory
lacrimal
gland
Accessory
lacrimal
gland
Superior
palpebral
furrow
M. tarsalis Müller
Meibomian gland
Palpebral
conjunctiva
Gland of Zeis
Gland of Moll
Eyelash
Orbital septumOrbicularis oculi muscle Orbital fatLevator palpebrae muscle
Fig.2.1Thesuperficiallayeroftheeyelidconsistsoftheskin,glandsofMolland
Zeis,andtheorbicularisoculiandlevatorpalpebraemuscles.Thedeeplayerconsists
ofthetarsalplate,tarsalmuscle,palpebralconjunctiva,andmeibomianglands.
–Sebaceousglands(tarsalormeibomianglands),tubularstructuresin
thecartilageoftheeyelid,whichlubricatethemarginoftheeyelid.
Theirfunctionistopreventtheescapeoftearfluidpastthemarginsof
theeyelids.ThefibersofRiolan’smuscleattheinferioraspectofthese
sebaceousglandssqueezeouttheductsofthetarsalglandseverytime
theeyeblinks.
2TheEyelids
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37. 19
Dimensionsofthenormalpalpebralfissure.
2 mm
9 mm
28–30 mm
3 mm
Fig.2.2The
widthofthe
palpebralfissureis
animportantin-
dicatorforanum-
berofpathologic
changesintheeye
(seeTable2.1).
Theeyelashesprojectfromtheanterioraspectofthemarginoftheeyelid.On
theuppereyelid,approximately150eyelashesarearrangedinthreeorfour
rows;onthelowereyelidthereareabout75intworows.Liketheeyebrows,
theeyelasheshelppreventdustandsweatfromenteringtheeye.Theorbital
septumislocatedbetweenthetarsalplateandthemarginoftheorbit.Itisa
membranoussheetofconnectivetissueattachedtothemarginoftheorbit
thatretainstheorbitalfat.
2.2 ExaminationMethods
Theeyelidsareexaminedbydirectinspectionunderabrightlight.Aslitlamp
maybeusedforthispurpose.Bilateralinspectionoftheeyelidsincludesthe
followingaspects:
!Eyelidposition:Normallythemarginsoftheeyelidsareincontactwiththe
eyeballandthepunctaaresubmergedinthelacuslacrimalis.
!Widthofthepalpebralfissure:Whentheeyeisopenandlookingstraight
ahead,theupperlidshouldcoverthesuperiormarginofthecorneaby
about2mm.Occasionallyathinstripofsclerawillbevisiblebetweenthe
corneaandthemarginofthelowerlid.Thewidthofthepalpebralfissureis
normally6–10mm,andthedistancebetweenthelateralandmedial
anglesoftheeyeis28–30mm(Fig.2.2).Varyingwidthsofthegaps
betweentheeyelidsmaybeasignofprotrusionoftheeyeball,enophthal-
mos,oreyeballsofvaryingsize(Table2.1).
!Skinoftheeyelid:Theskinoftheeyelidisthinwithonlyaslightamountof
subcutaneousfattytissue.Allergicreactionandinflammationcanrapidly
causeextensiveedemaandswelling.Inolderpatients,theskinofthe
uppereyelidmaybecomeincreasinglyflaccid(cutislaxasenilis).Occa-
sionallyitcanevenhangdownovertheeyelashesandrestrictthefieldof
vision(dermatochalasisorblepharochalasis).
2.2ExaminationMethods
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38. 20
Table2.1Possiblecausesofabnormalwidthofthepalpebralfissure
Increasedpalpebralfissure Decreasedpalpebralfissure
!Peripheralfacialparesis
(lagophthalmos)
!Grave’sdisease
!Perinaud’ssyndrome
!Buphthalmos
!High-grademyopia
!Retrobulbartumor
!Congenitalptosis
!Ptosisinoculomotornervepalsy
!Ptosisinmyastheniagravis
!Sympatheticptosis(withHorner’ssyndrome,
seepp.23–24)
!Progressiveophthalmoplegia(Graefe’ssign)
!Microphthalmos
!Enophthalmos
!Shrinkageoftheorbitalfat(asinsenile
enophthalmos)
Thepalpebralconjunctivaisexaminedbysimpleeversionoftheuppereye-
lid(seeFigs.1.7and1.8).Thenormalpalpebralconjunctivaissmoothand
shinywithoutanyscarstricturesorpapilliformprojections.
FulleversionoftheuppereyelidwithaDesmarreseyelidretractor(see
Fig.1.9,p.9)allowsexaminationofthesuperiorfornix(fornormalappear-
ance,seepalpebralconjunctiva).
2.3 DevelopmentalAnomalies
2.3.1 Coloboma
Definition
Anormallyunilateraltriangulareyeliddefectwithitsbaseatthemarginofthe
eyelidoccurringmostoftenintheuppereyelid(Fig.2.3).
Epidemiologyandetiology.Colobomasareraredefectsresultingfroma
reductionmalformation(defectiveclosureoftheopticcup).Theyareonly
rarelytheresultofaninjury.
Diagnosticconsiderations:Thedisorderisoftenaccompaniedbyadditional
deformitiessuchasdermoidcystsoramicrophthalmos.Congenitaldefectsof
thefirstembryonicbranchialarchthatcanresultincolobomainclude
Franceschetti’ssyndrome(mandibulofacialdysostosis)orGoldenhar’ssyn-
drome(oculoauriculovertebraldysplasia).Dependingontheextentofthe
coloboma,desiccationsymptomsontheconjunctivaandcorneawith
incipientulcerationmayarisefromthelackofregularanduniformmoisten-
ingoftheconjunctivaandcornea.
2TheEyelids
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39. 21
Treatment:Defectsareclosedbydirectapproximationorplasticsurgery
withaskinflap.
2.3.2EpicanthalFolds
Acrescenticfoldofskinusuallyextendingbilaterallybetweentheupperand
lowereyelidsandcoveringthemedialangleoftheeye.Thisrarecongenital
anomalyisharmlessandtypicalineasternAsians.However,italsooccurs
withDown’ssyndrome(trisomy21syndrome).Thirtypercentofnewborns
haveepicanthalfoldsuntiltheageofsixmonths.Whereonefoldismorepro-
nounced,itcansimulateesotropia.Thenasalbridgebecomesmorepro-
nouncedasthechildgrows,andmostepicanthalfoldsdisappearbytheageof
four.
2.3.3Blepharophimosis
Thisreferstoshorteningofthehorizontalpalpebralfissurewithoutpatho-
logicchangesintheeyelids.Thepalpebralfissure,normally28–30mm
wide,maybereducedtohalfthatwidth.Blepharophimosisisararedisorder
thatiseithercongenitaloracquired(forexample,fromscarcontractureor
aging).Aslongasthecenterofthepupilremainsunobstructeddespitethe
decreasedsizeofthepalpebralfissure,surgicalenlargementofthepalpebral
fissure(bycanthotomyorplasticsurgery)hasapurelycosmeticpurpose.
Congenitalcoloboma.
Fig.2.3Thetri-
angulareyelidde-
fectwithitsbase
atthemarginof
theeyelidresults
fromareduction
malformation
duringclosureof
theopticcupin
theembryonic
stage.
2.3DevelopmentalAnomalies
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40. 22
2.3.4Ankyloblepharon
Thisreferstohorizontalshorteningofthepalpebralfissurewithfusionof
theeyelidsatthelateralandmedialanglesoftheeye.Usually,thepartialor
totalfusionbetweentheupperandlowereyelidswillbebilateral,andthe
palpebralfissurewillbepartiallyorcompletelyoccludedasaresult.Posterior
totheeyelids,theeyeballitselfwillbedeformedortotallyabsent.Ankylo-
blepharonisfrequentlyassociatedwithotherskulldeformities.
2.4 Deformities
2.4.1 Ptosis
Definition
Paralysisofthelevatorpalpebraemusclewithresultingdroopingofoneor
bothuppereyelids(fromtheGreekptosis,afalling).Thefollowingformsare
differentiatedaccordingtotheirorigin(seealsoEtiology):
!Congenitalptosis(Fig.2.4).
!Acquiredptosis:
–Paralyticptosis.
–Sympatheticptosis.
–Myotonicptosis.
–Traumaticptosis.
Epidemiology.Onthewholeptosisisararedisorder.
Etiology:Ptosismaybecongenitaloracquired.
Congenitalptosis.
Fig.2.4Con-
genitalptosisof
thelevatorpalpe-
braemuscle
causestheupper
eyelidtodroop;
usuallythede-
formityisuni-
lateral.Ambly-
opiawillresultif
thecenterofthe
pupiliscovered.
2TheEyelids
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41. 23
Congenitalptosis.Thedisorderisusuallyhereditaryandisprimarilyauto-
somaldominantasopposedtorecessive.Thecauseisfrequentlyaplasiain
thecoreoftheoculomotornerve(neurogenic)thatsuppliesthelevatorpalpe-
braemuscle;lessfrequentlyitisattributabletoanunderdevelopedlevator
palpebraemuscle(myogenic).
Acquiredptosis:
!Neurogeniccauses:
–Oculomotorpalsy(paralyticptosis).
–Lesionsinthesympatheticnerve(sympatheticptosis)isHorner’spalsy
(ptosis,miosis,andenophthalmos).
!Myogenicptosis:myastheniagravisandmyotonicdystrophy.
!Traumaticptosiscanoccurafterinjuries.
Symptoms.Thedroopingoftheuppereyelidmaybeunilateral(usuallyasign
ofaneurogeniccause)orbilateral(usuallyasignofamyogeniccause).A
characteristicfeatureoftheunilateralformisthatthepatientattemptsto
increasethepalpebralfissurebyfrowning(contractingthefrontalismuscle).
Congenitalptosis(Fig.2.4)generallyaffectsoneeyeonly;bilateralsymptoms
areobservedfarlessfrequently(7%).
Diagnosticconsiderations:Congenitalptosis.Theaffectedeyelidingeneral
isunderdeveloped.Theskinoftheuppereyelidissmoothandthin;thesupe-
riorpalpebralfurrowisabsentorill-defined.Atypicalsymptomis“lidlag”in
whichtheuppereyeliddoesnotmovewhenthepatientglancesdown.This
importantdistinguishingsymptomexcludesacquiredptosisindifferentialdiag-
nosis.Inabout3%ofallcases,congenitalptosisisassociatedwithepicanthal
foldsandblepharophimosis(Waardenburgsyndrome).
Congenitalptosiscanoccurinvaryingdegreesofseverityandmaybecom-
plicatedbythepresenceofadditionaleyelidandocularmuscledisorders
suchasstrabismus.
Congenitalptosisinwhichtheuppereyeliddroopsoverthecenterof
thepupilalwaysinvolvesanincreasedriskofamblyopia.
Acquiredptosis:
!Paralyticptosisinoculomotorpalsy(seealsoChap.17)isusuallyunilateral
withthedroopingeyelidcoveringthewholeeye.Oftentherewillbeother
signsofpalsyintheareasuppliedbytheoculomotornerve.Inexternal
oculomotorpalsy,onlytheextraocularmusclesareaffected(mydriasiswill
notbepresent),whereasincompleteoculomotorpalsy,theinnerciliary
muscleandthesphincterpupillaemusclearealsoaffected(internaloph-
thalmoplegiawithlossofaccommodation,mydriasis,andcompletelossof
pupillarylightreflexes).
!Myastheniagravis(myogenicptosisthatisoftenbilateralandmaybe
asymmetrical)isassociatedwithabnormalfatigueofthestriated
2.4Deformities
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42. 24
extraocularmuscles.Ptosistypicallybecomesmoresevereasthedaygoes
on.
!SympatheticptosisoccursinHorner’spalsy(ptosis,miosis,andenophthal-
mos).
Rapidlyopeningandclosingtheeyelidsprovokesptosisinmyasthenia
gravisandsimplifiesthediagnosis.
Treatment:
!Congenitalptosis:Thisinvolvessurgicalretractionoftheuppereyelid
(Fig.2.5a–c),whichshouldbeundertakenasquicklyaspossiblewhen
thereisariskoftheaffectedeyedevelopingavisualimpairmentasaresult
oftheptosis.
!Acquiredptosis:Treatmentdependsonthecause.Aspalsiesoftenresolve
spontaneously,thepatientshouldbeobservedbeforeresortingtosurgical
intervention.Conservativetreatmentwithspecialeyeglassesmaybesuffi-
cienteveninirreversiblecases.
Becauseoftheriskofovercorrectingorundercorrectingthedisorder,several
operationsmaybenecessary.
Prognosisandcomplications:Promptsurgicalinterventionincongenital
ptosiscanpreventamblyopia.Surgicalovercorrectionoftheptosiscanleadto
desiccationoftheconjunctivaandcorneawithulcerationasaresultof
incompleteclosureoftheeyelids.
2.4.2Entropion
Definition
Entropionischaracterizedbyinwardrotationoftheeyelidmargin.Themargin
oftheeyelidandeyelashesoreventheouterskinoftheeyelidareincontact
withtheglobeinsteadofonlytheconjunctiva.Thefollowingformsarediffer-
entiatedaccordingtotheirorigin(seeEtiology):
!Congenitalentropion(Fig.2.6).
!Spasticentropion(Fig.2.7).
!Cicatricialentropion.
Epidemiology:CongenitalentropionoccursfrequentlyamongAsiansbutis
rareamongpeopleofEuropeandescent,inwhomthespasticandcicatricial
formsaremorecommonlyencountered(seealsoChap.18).
Etiology:
!Congenitalentropion:Thisresultsfromfleshythickeningoftheskinand
orbicularisoculimusclenearthemarginoftheeyelid.Usuallythelower
eyelidisaffected.Thisconditionmaypersistintoadulthood.
2TheEyelids
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43. 25
Methodsofsurgicalretractionoftheuppereyelid.
c
b
a
Fig.2.5aThe
Fasanella-Servatpro-
cedure,indicatedfor
correctionofminimal
ptosis,involvesresec-
tionofaportionof
thetarsus(2mmor
less)tovertically
shortentheeyelid.
bTheamountof
muscleremovedina
levatorresectionde-
pendsonlevatorfunc-
tion(rangingfromap-
proximately10mm
withslightptosis,up
to22mmwithmod-
erateptosis).cWhere
levatorfunctionis
poor(lessthan5mm),
theuppereyelidcan
beconnectedtotissue
intheeyebrowregion.
Thefrontalissuspen-
siontechniquemay
employautogenous
fascialataorplastic
suture.
2.4Deformities
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44. 26
Congenitalentropion.
Fig.2.6Con-
genitalinwardro-
tationofthemar-
ginsoftheupper
andlowereyelids
isafrequentfind-
inginAsian
populationsand
isusuallyasymp-
tomatic.
Spasticentropion.
Fig.2.7Dis-
placedfibersof
theorbicularis
oculimuscle
causetheeye-
lashesofthe
lowereyelidto
turninward.Sur-
gicalintervention
isindicatedto
correctthelaxity
ofthelowereye-
lid.
!Spasticentropion:Thisaffectsonlythelowereyelid.Acombinationof
severalpathogeneticfactorsofvaryingseverityisusuallyinvolved:
–Thestructuressupportingthelowereyelid(palpebralligaments,tarsus,
andeyelidretractor)maybecomelaxwithage,causingthetarsustotilt
inward.
–Thiscausesthefibersoftheorbicularisoculimuscletooverridethe
normallysuperiormarginoftheeyelid,intensifyingtheblepharospasm
resultingfromthepermanentcontactbetweentheeyelashesandthe
eyeball.
2TheEyelids
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45. 27
–Senileenophthalmos,usuallyoccurringinoldageasaresultofatrophy
oftheorbitfattytissue,furthercontributestoinstabilityofthelower
eyelid.
!Cicatricialentropion:Thisformofentropionisfrequentlytheresultof
postinfectiousorpost-traumatictarsalcontracture(suchastrachoma;
burnsandchemicalinjuries).Causescanalsoincludeallergicandtoxic
reactions(pemphigus,Stevens-Johnsonsyndrome,andLyell’ssyndrome).
Symptomsanddiagnosticconsiderations(seealsoetiology):Constant
rubbingoftheeyelashesagainsttheeyeball(trichiasis)representsaper-
manentforeign-bodyirritationoftheconjunctivawhichcausesablepharo-
spasm(p.93)thatinturnexacerbatestheentropion.Thechronicallyirritated
conjunctivaisreddened,andtheeyefillswithtears.Onlycongenital
entropionisusuallyasymptomatic.
Treatment:
!Congenitalentropion:Totheextentthatanytreatmentisrequired,itcon-
sistsofmeasured,semicircularresectionofskinandorbicularisoculi
muscletissuethatcanbesupplementedbyevertingsutureswhereindi-
cated.
!Spasticentropion:Surgicalmanagementmustbetailoredtothespecific
situation.Usuallytreatmentcombinesseveraltechniquessuchasshorten-
ingtheeyelidhorizontallycombinedwithweakeningordivertingthepre-
tarsalfibersoftheorbicularisoculimuscleandshorteningtheskinverti-
cally.
!Cicatricialentropion:Thesurgicalmanagementofthisformisidenticalto
thatofspasticentropion.
Anadhesivebandagemaybeappliedtoincreasetensionontheeyelid
fortemporaryreliefofsymptomspriortosurgery.
Prognosisandcomplications:
Congenitalentropionisusuallyasymptomaticandoftenresolveswithin
thefirstfewmonthsoflife.
!Spasticentropion:Theprognosisisfavorablewithpromptsurgicalinter-
vention,althoughthedisordermayrecur.Leftuntreated,spastic
entropionentailsariskofdamagetothecornealepitheliumwithsuperin-
fectionwhichmayprogresstothecompleteclinicalsyndromeofaserpigi-
nouscornealulcer(seep.29).
!Cicatricialentropion:Theprognosisisfavorablewithpromptsurgical
intervention(i.e.,beforeanycornealchangesoccur).
2.4Deformities
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46. 28
2.4.3Ectropion
Definition
Ectropionreferstotheconditioninwhichthemarginoftheeyelidisturned
awayfromtheeyeball.Thisconditionalmostexclusivelyaffectsthelowereye-
lid.Thefollowingformsaredifferentiatedaccordingtotheirorigin(seealso
Etiology):
!Congenitalectropion.
!Senileectropion.
!Paralyticectropion.
!Cicatricialectropion.
Epidemiology:Senileectropionisthemostprevalentform;theparalyticand
cicatricialformsoccurlessfrequently.Congenitalectropionisveryrareandis
usuallyassociatedwithotherdevelopmentalanomaliesoftheeyelidandface
suchasFranceschetti’ssyndrome.
Etiology:
!Congenitalectropion:SeeEpidemiology.
!Senileectropion:Thepalpebralligamentsandtarsusmaybecomelax
withage,causingthetarsustosagoutward(Fig.2.8).
!Paralyticectropion:Thisiscausedbyfacialparalysiswithresultinglossof
functionoftheorbicularisoculimusclethatclosestheeyelid.
!Cicatricialectropion:Likecicatricialentropion,thisformisusuallya
sequelaofinfectionorinjury.
Senileectropion.
Fig.2.8The
structures
supportingthe
eyelidarelax,
causingthelower
eyelidsagout-
ward.
2TheEyelids
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47. 29
Symptomsanddiagnosticconsiderations:Leftuntreated,incompleteclo-
sureoftheeyelidscanleadtosymptomsassociatedwithdesiccationofthe
corneaincludingulcerationfromlagophthalmos.Atthesametime,theever-
sionofthepunctumcausestearstoflowdownacrossthecheekinsteadof
drainingintothenose.Wipingawaythetearsincreasestheectropion.This
resultsinchronicconjunctivitisandblepharitis.
Treatment:
!Congenitalectropion:Surgery.
!Senileectropion:Surgeryisindicated.Aprovenprocedureistotightenthe
lowereyelidviaatarsalwedgeresectionfollowedbyhorizontaltightening
oftheskin.
!Paralyticectropion:Dependingontheseverityofthedisorder,artificial
tearsolutions,eyeglasseswithananatomiclateralprotectivefeature,ora
“watchglass”bandage(Fig.2.9)maybesufficienttopreventdesiccationof
thecornea.Insevereorirreversiblecases,thelagophthalmosistreated
surgicallyviaalateraltarsorrhaphy.
!Cicatricialectropion:Plasticsurgeryisoftenrequiredtocorrecttheeyelid
deformity.
Prognosis:Theprognosisisfavorablewhenthedisorderistreatedpromptly.
Sometimesseveraloperationswillberequired.Surgeryismoredifficult
wherescarringispresent.
Watchglassbandageforparalyticectropion.
Fig.2.9Inpatientswithlagoph-
thalmosresultingfromfacialparaly-
sis,awatchglassbandagecreatesa
moistchamberthatprotectsthe
corneaagainstdesiccation.
2.4Deformities
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48. 30
2.4.4Trichiasis
Trichiasisreferstotherarepostinfectiousorpost-traumaticinwardturning
oftheeyelashes.Thedeformitycausestheeyelashestorunagainstthecon-
junctivaandcornea,causingapermanentforeign-bodysensation,increased
tearsecretion,andchronicconjunctivitis.Theeyelashfolliclescanbeoblit-
eratedbyelectrolysis.Thedisordermayalsobesuccessfullytreatedbycryo-
cauteryepilationorsurgicalremovalofthefolliclebed.
2.4.5Blepharospasm
Definition
Thisreferstoaninvoluntaryspasmodiccontractionoftheorbicularisoculi
musclesuppliedbythefacialnerve.
Etiology:Inadditiontophotosensitivityandincreasedtearproduction,
blepharospasmwillalsoaccompanyinflammationorirritationoftheanterior
chamber.(Photosensitivity,epiphora,andblepharospasmformatriadof
reactiveclinicalsymptoms.)Causesofthedisorderincludeextrapyramidal
diseasesuchasencephalitisormultiplesclerosis.Trigeminalneuralgiaorpsy-
chogeniccausesmayalsobepresent.
Symptoms:Clinicalsymptomsincludespasmodicallynarrowedorclosed
palpebralfissuresandloweredeyebrows.
Treatment:Thisdependsonthecauseofthedisorder.Mildcasescanbecon-
trolledwellwithmusclerelaxants.Severecasesmayrequiretransectionofthe
fibersofthefacialnervesupplyingtheorbicularisoculimuscle.Thedisorder
mayalsobesuccessfullytreatedwithrepeatedlocalinjectionsofbotulinum
toxin.
Prognosis:Theprognosisisgoodwhereacause-relatedtreatmentispossible.
Essentialblepharospasmdoesnotrespondwelltotreatment.
2.5 DisordersoftheSkinandMarginoftheEyelid
2.5.1 ContactEczema
Epidemiology:Light-skinnedpatientsandpatientssusceptibletoallergyare
frequentlyaffected.
Etiology:Contacteczemaiscausedbyanantigen–antibodyreactionin
patientswithintolerancetocertainnoxioussubstances.Cosmetics,adhesive
bandages,oreyedropsandeyeointmentsareoftenresponsible,particularly
thepreservativesusedinthemsuchasbenzalkoniumchloride.
2TheEyelids
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49. 31
Symptoms:Reddening,swelling,lichenification,andsevereitchingofthe
skinoftheeyelidoccurinitially,followedbyscalingoftheinduratedskinwith
asensationoftension(Fig.2.10).
Treatment:Thisconsistsofeliminatingthecausativeagent.(Allergytesting
maybenecessary.)Limiteduseofcorticosteroidsusuallybringsquickreliefof
symptoms.
Prognosis:Theprognosisisgoodifthecausecanbeidentified.
2.5.2Edema
Definition
Thisreferstoswellingoftheeyelidduetoabnormalcollectionoffluidinthe
subcutaneoustissue.
Epidemiology:Edemaisafrequentlyencounteredclinicalsymptom.
Etiology:Theskinoftheeyelidisaffectedintensivelybyinfectiousandaller-
gicprocesses.Withtheuppereyelid’srelativelythinskinandtheloosestruc-
Contacteczema.
Fig.2.10Thisdisorderisfrequently
causedbypreservativessuchasthose
usedineyedrops.Theycausetypical
reddening,swelling,andlichenifica-
tionoftheskinoftheeyelid.
2.5DisordersoftheSkinandMarginoftheEyelid
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50. 32
tureofitssubcutaneoustissue,watercaneasilyaccumulateandcause
edema.
Symptoms:Dependingonthecause(Table2.2),theintensityofswellingin
theeyelidwillvary.Thelocationofswellingisalsoinfluencedbygravityand
canvaryinintensity.Forexample,itmaybemoreintenseintheearlymorn-
ingafterthepatientrisesthanintheevening(Fig.2.11).
Fig.2.2Differentialdiagnosisofedema
Criteria Inflammatoryedema Noninflammatoryedema
Symptoms !Swelling
!Reddening
!Sensationofheat
!Painful
!Usuallyunilateral
!Swelling
!Paleskin
!Coolskin
!Painless
!Usuallybilateral
Possiblecauses!Hordeolum(p.38)
!Abscess(p.36)
!Erysipelas
!Eczema(p.104)
!Associatedwith:
–paranasalsinusdisorders
–orbitalcellulitis
–dacryoadenitis
–dacryocystitis
!Systemicdisorder:
–heart
–kidneys
–thyroidgland
!AllergysuchasQuincke’s
edema
Edema.
Fig.2.11With
itsrelativelythin
skinanditssub-
cutaneoustissue
thatcontains
littlefat,the
uppereyelidis
particularlysus-
ceptibletorapid
fluidaccumula-
tionsfrompatho-
logicprocesses.
2TheEyelids
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51. 33
Table2.2showsthecausesanddifferentialdiagnosisforinflammatory
andnoninflammatoryedemas.
Treatment:Thisdependsonthecauseofthedisorder.
Clinicalcourseandprognosis:Thisdependsontheunderlyingdisorder.
2.5.3SeborrheicBlepharitis
Definition
Thisrelativelyfrequentdisorderischaracterizedbyscalyinflammationofthe
marginsoftheeyelids.Usuallybotheyesareaffected.
Etiology:Thereareoftenseveralcontributingcauses.Theconstitutionofthe
skin,seborrhea,refractiveanomalies,hypersecretionoftheeyelidglands,and
externalstimulisuchasdust,smoke,anddryairinair-conditionedrooms
oftencontributetopersistentchronicinflammation.
Symptomsanddiagnosticconsiderations:Themarginsoftheeyelidsusu-
allyexhibitslightinflammatorychangessuchasthickening.Theeyelashes
adhereduetotheincreasedsecretionfromtheglandsoftheeyelids,andscaly
depositsform(Fig.2.12).Thedisorderwilloftenbeaccompaniedbychronic
conjunctivitis.
Treatment:Thisdependsonthecauseofthedisorder(seeEtiology).The
scalesandcrustscanusuallybesoftenedwithwarmoliveoilandtheneasily
removedwithacotton-tippedapplicator.Inmoreseverecases,recom-
Seborrheicblepharitis.
Fig.2.12The
marginsofthe
eyelidsare
slightlyreddened
withadhesionof
theeyelashes.
Scalydeposits
formalongthe
marginsofthe
eyelids.
2.5DisordersoftheSkinandMarginoftheEyelid
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52. 34
mendedtreatmentincludesexpressingtheglandsoftheeyelidandlocal
applicationofantibioticointment.Treatmentwithtopicalsteroidsmaybe
indicatedundercertainconditions.
Prognosis:Theprognosisisgoodalthoughtheclinicalcourseofthedisorder
isoftenquiteprotracted.
2.5.4HerpesSimplexoftheEyelids
Definition
Acute,usuallyunilateraleyeliddisorderaccompaniedbyskinandmucous
membranevesicles.
Etiology:Infectionoftheskinoftheeyelidsresultswhenlatentherpessim-
plexvirusespresentinthetissueareactivatedbyultravioletradiation.The
virusspreadsalongsensorynervefibersfromthetrigeminalgangliontothe
surfaceoftheskin.
Symptoms:Typicalclusterederuptionsofpainfulvesiclesfilledwithserous
fluidfrequentlyoccuratthejunctionofmucousmembranesandskin
(Fig.2.13).Laterthevesiclesdryandcrustsform.Lesionshealwithoutscar-
ring.Thedisorderisusuallyunilateral.
Treatment:Topicaluseofvirostaticagentsisindicated.Thepatientshould
avoidintenseultravioletradiationasaprophylacticmeasureagainstrecur-
rence.
Prognosis:Theprognosisisgood,althoughthedisorderfrequentlyrecurs.
Herpessimplexoftheeyelids.
Fig.2.13Painful
vesiclesfilled
withserousfluid
eruptinclusters
attheangleof
theeye.
2TheEyelids
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53. 35
2.5.5HerpesZosterOphthalmicus
Definition
Facialrashcausedbythevaricella-zostervirus.
Epidemiology:Thedisorderusuallyaffectsimmunocompromisedpersons
betweentheagesof40and60whohaveunderlyingdisorders.
Etiology:Thedisorderiscausedbythevaricella-zostervirus,whichinitially
manifestsitselfaschickenpox.Ifactivationorreinfectionoccurs,thelatent
neurotropicvirusespresentinthebodycanleadtotheclinicalsyndromeof
herpeszosterophthalmicus(Fig.2.14).
Symptoms:Theincubationperiodis7–18days,afterwhichseverepain
occursintheareasuppliedbythefirstbranchofthetrigeminalnerve(the
ophthalmicnervewithitsfrontal,lacrimal,andnasociliarybranches).Prodro-
malsymptomsoferythema,swelling,photosensitivity,andlacrimationmay
occurbeforethecharacteristicclearwateryvesiclesappear.Thevesiclesburst
andbrownishscabsform,whicharelatershed.Blepharitis(seep.33)isalso
presentin50–70%ofallcases.Asherpeszosterusuallyaffectsimmunocom-
Herpeszosterophthalmicus.
Fig.2.14Thefacialrashofherpes
zosteriscausedbytheneurotropic
varicella-zostervirus.Aftertheclear
wateryvesiclesburst,brownishscabs
form,whicharelatershed.
2.5DisordersoftheSkinandMarginoftheEyelid
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54. 36
promisedpersons,thepatientshouldbeexaminedforapossibleunderlying
disorder.
Theskinsensitivityatthetipofthenoseshouldbeevaluatedonboth
sidesintheinitialstageofthedisorder.Decreasedsensitivitytotouch
suggestsinvolvementofthenasociliarybranchoftheophthalmic
nerve,whichcanleadtosevereintraocularinflammation.
Treatment:Thisincludestopicalvirostaticagentsandsystemicacyclovir.
Complications:Involvementofthenasociliarybranchoftheophthalmic
nervecanleadtosevereintraocularinflammation.
Prognosis:Theskinlesionshealwithinthreetofourweeks;scarsmay
remain.Oftenneuralgiformpainandhypesthesiamaypersist.
2.5.6EyelidAbscess
Definition
Circumscribedcollectionofpuswithsevereinflammation,swelling,andsub-
sequentfluctuation.
Etiology:Anabscessoftheupperorlowereyelidcanformasasequelaof
minortrauma,insectsting,orspreadofinflammationfromtheparanasal
sinuses.
Symptoms:Thesevereinflammationandswellingoftenmakeitimpossible
activelytoopentheeye(Fig.2.15).Thecontentsoftheabscesscanfluctuate
Eyelidabscess.
Fig.2.15Severe
inflammationand
swellingmakeit
impossibleac-
tivelytoopenthe
eye.
2TheEyelids
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55. 37
duringtheclinicalcourseofthedisorder.Spontaneousperforationwithpus
drainagecanoccur.
Treatment:Oralorintravenousantibioticsanddryheatareindicated.Astab
incisioncanrelievetensionattheonsetoffluctuation.
Prognosis:Theprognosisisgenerallygood.
Orbitalcellulitisorcavernoussinusthrombosiscanoccasionallyoccur
asasequelaofeyelidabscess,especiallywhenlocatedatthemedial
angleoftheeye.Thisrepresentsalife-threateningcomplication.
2.5.7TickInfestationoftheEyelids
Tickshavebeenknowntoinfesttheeyelids.Theyarethoughttobevectorsof
borreliosisandcancauseencephalitis.Treatmentconsistsofmechanical
removaloftheparasites.
2.5.8LouseInfestationoftheEyelids
Thisreferstoinfestationofthemarginoftheeyelidwithcrabliceasaresult
ofpoorhygienicconditions.Thesmallovalnitsfrequentlyhangfromtheeye-
lashes(Fig.2.16),causinginflammationofthemarginoftheeyelidwith
severeitching.Mechanicalremovalwithforcepsisatimeconsumingbuteffec-
tivetreatment.Applicationofa2%mercuryprecipitateointmentoveran
extendedperiodoftimeisalsoeffective.
Louseinfestationoftheeyelids.
Fig.2.16Under
poorhygienic
conditions,crab
licecaninfestthe
basesoftheeye-
lashes.
2.5DisordersoftheSkinandMarginoftheEyelid
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56. 38
2.6 DisordersoftheEyelidGlands
2.6.1 Hordeolum
Definition
Ahordeolumistheresultofanacutebacterialinfectionofoneormoreeyelid
glands.
Epidemiologyandetiology:Staphylococcusaureusisacommoncauseof
hordeolum.ExternalhordeoluminvolvesinfectionoftheglandsofZeisor
Moll.Internalhordeolumarisesfrominfectionofthemeibomianglands.
Hordeolumisoftenassociatedwithdiabetes,gastrointestinaldisorders,or
acne.
Symptomsanddiagnosticconsiderations:Hordeolumpresentsaspainful
noduleswithacentralcoreofpus.Externalhordeolumappearsonthemargin
oftheeyelidwherethesweatglandsarelocated(Fig.2.17).Internalhorde-
olumofasebaceousglandisusuallyonlyrevealedbyevertingtheeyelidand
usuallyaccompaniedbyamoreseverereactionsuchasconjunctivitisorche-
mosisofthebulbarconjunctiva.Pseudoptosisandswellingofthepreauricu-
larlymphnodesmayalsooccur.
Differentialdiagnosis:Chalazion(tendertopalpation)andinflammationof
thelacrimalglands(rarerandmorepainful).
Treatment:Antibioticointmentsandapplicationofdryheat(redheatlamp)
willrapidlyhealthelesion.
Externalhordeolum.
Fig.2.17The
painfulinflamed
hordeolumis
usuallycausedby
Staphylococcus
aureusinfection
ofaneyelid
gland.
2TheEyelids
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57. 39
Clinicalcourseandprognosis:Aftereruptionanddrainageofthepus,the
symptomswillrapidlydisappear.Theprognosisisgood.Anunderlyinginter-
naldisordershouldbeexcludedincasesinwhichthedisorderfrequently
recurs.
2.6.2Chalazion
Definition
Firmnodularbulbwithinthetarsus.
Epidemiologyandetiology:Chalaziaoccurrelativelyfrequentlyandare
causedbyachronicgranulomatousinflammationduetobuildupofsecretion
fromthemeibomiangland.
Symptoms:Thefirmpainlessnoduledevelopsveryslowly.Asidefromthe
cosmeticflaw,itisusuallyasymptomatic(Fig.2.18).
Differentialdiagnosis:Hordeolum(tendertopalpation)andadenocarci-
noma.
Treatment:Surgicalincisionisusuallyunavoidable(Fig.2.19).
Afterintroducingthechalazionclamp,thelesionisincisedeithermedi-
ally,perpendiculartothemarginoftheeyelid,orlaterally,perpendicu-
lartothemarginoftheeyelid(thisisimportanttoavoidcicatricial
ectropion).Thefattycontentsarethenremovedwithacuret.
Prognosis:Goodexceptforthechanceoflocalrecurrence.
Chalazion.
Fig.2.18Painful
topalpation,the
chalazionis
causedbya
chronicbuildup
ofsecretionfrom
themeibomian
glands.
2.6DisordersoftheEyelidGlands
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58. 40
Surgicalremovalofachalazion.
Fig.2.19Afterthechalazionclamp
hasbeenintroducedandthelesionin-
cisedwithascalpel,thefattycontents
areremovedwithacuret.
2.7 Tumors
2.7.1 BenignTumors
2.7.1.1DuctalCysts
TheroundcystsoftheglandsorMollareusuallylocatedintheangleofthe
eye.Theircontentsareclearandwateryandcanbetransilluminated.Gravity
canresultinectropion(Fig.2.20).Therapyconsistsofmarsupialization.The
prognosisisgood.
2.7.1.2Xanthelasma
Definition
Localfatmetabolismdisorderthatproduceslipoproteindeposits.Theseare
usuallybilateralinthemedialcanthus.
2TheEyelids
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59. 41
Ductalcyst.
Fig.2.20The
roundcystsof
theglandsof
Mollareusually
locatedinthe
angleoftheeye.
Theweight
causestemporary
ectropion.
Epidemiology:Postmenopausalwomenaremostfrequentlyaffected.A
higherincidencehasalsobeenobservedinpatientswithdiabetes,increased
levelsofplasmalipoprotein,orbileductdisorders.
Symptoms:Thesoftyellowwhiteplaquesaresharplydemarcated.Theyare
usuallybilateralanddistributedsymmetrically(Fig.2.21).Asidefromthecos-
meticflaw,thepatientsareasymptomatic.
Xanthelasma.
Fig.2.21The
fattydepositsare
oftensymmetri-
callydistributed
inthemedial
canthus.
2.7Tumors
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60. 42
Treatmentandprognosis:Theplaquescanonlyberemovedsurgically.The
incidenceofrecurrenceishigh.
2.7.1.3MolluscumContagiosum
ThenoninflammatorycontagiousinfectioniscausedbyDNAviruses.The
diseaseusuallyaffectschildrenandteenagersandistransmittedbydirect
contact.Thepinhead-sizedlesionshavetypicalcentraldepressionsandare
scatteredneartheupperandlowereyelids(Fig.2.22).Theselesionsarere-
movedwithacuret.(Inchildrenthisisdoneundershort-actinganesthesia.)
2.7.1.4CutaneousHorn
Theyellowishbrowncutaneousprotrusionsconsistofkeratin(Fig.2.23).
Olderpatientsaremorefrequentlyaffected.Thecutaneoushornshouldbe
surgicallyremovedas25%ofkeratosiscasescandevelopintomalignant
squamouscellcarcinomasyearslater.
2.7.1.5Keratoacanthoma
Arapidlygrowingtumorwithacentralkeratinmassthatopensontheskin
surface,whichcansometimesbeexpressed(Fig.2.24).Thetumormay
resolvespontaneously,formingasmallsunkenscar.
Differentialdiagnosisshouldexcludeabasalcellcarcinoma(seethatsection);
themarginofakeratoacanthomaischaracteristicallyavascular.Likewise,a
squamouscellcarcinomacanonlybeexcludedbyabiopsy.
Molluscumcontagiosum.
Fig.2.22The
pinhead-sized
molluscumle-
sionshavetypical
centraldepres-
sions.
2TheEyelids
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61. 43
CutaneousHorn.
Fig.2.23The
yellowishbrown
cutaneouspro-
trusionsconsist
ofkeratin.They
frequently(25%
ofallcases)
developintoa
malignant
squamouscell
carcinomain
lateryearsifthey
arenotsurgically
removed.
Keratoacanthoma.
Fig.2.24The
rapidlygrowing
benigntumorhas
acentralkeratin
massthatopens
ontheskinsur-
face.
2.7.1.6Hemangioma
Definition
Congenitalbenignvascularanomalyresemblinganeoplasmthatismost
frequentlynoticedintheskinandsubcutaneoustissues.
Epidemiology:Girlsaremostoftenaffected(approximately70%ofallcases).
Faciallesionsmostcommonlyoccurintheeyelids(Fig.2.25).
2.7Tumors
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62. 44
Cavernoushemangioma.
Fig.2.25Thecongenitalvascular
anomalyoccursasafaciallesionmost
commonlyoccurintheeyelids.Thele-
sionregressesspontaneouslyinap-
proximately70%ofallcases.
Symptoms:Hemangiomasincludecapillaryorsuperficial,cavernous,and
deepforms.
Diagnosticconsiderations:Hemangiomascanbecompressed,andtheskin
willthenappearwhite.
Differentialdiagnosis:Nevusflammeus:Thisischaracterizedbyasharply
demarcatedbluishredmark(“port-wine”stain)resultingfromvascular
expansionundertheepidermis(notagrowthortumor).
Treatment:Awatch-and-waitapproachisjustifiedinlightofthehighrateof
spontaneousremission(approximately70%).Wherethereisincreasedriskof
amblyopiaduetothesizeofthelesion,cryotherapy,intralesionalsteroid
injections,orradiationtherapycanaccelerateregressionofthehemangioma.
Prognosis:Generallygood.
2.7.1.7Neurofibromatosis(Recklinghausen’sDisease)
Definition
Acongenitaldevelopmentaldefectoftheneuroectodermgivesrisetoneural
tumorsandpigmentspots(caféaulaitspots).
Neurofibromatosisisregardedasaphacomatosis(adevelopmentaldisorder
involvingthesimultaneouspresenceofchangesintheskin,centralnervous
system,andectodermalportionsoftheeye).
2TheEyelids
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63. 45
Symptomsanddiagnosticconsiderations:Thenumeroustumorsaresoft,
broad-based,orpediculate,andoccureitherintheskinorinsubcutaneous
tissue,usuallyinthevicinityoftheuppereyelid.
Theycanreachmonstrousproportionsandpresentaselephantiasisofthe
eyelids(Fig.2.26).
Treatment:Smallerfibromascanbeeasilyremovedbysurgery.Larger
tumorsalwaysentailariskofpostoperativebleedingandrecurrence.Onthe
whole,treatmentisdifficult.
2.7.2MalignantTumors
2.7.2.1BasalCellCarcinoma
Definition
Basalcellcarcinomaisafrequent,moderatelymalignant,fibroepithelialtumor
thatcancauseseverelocaltissuedestructionbutveryrarelymetastasizes.
Neurofibroma.
Fig.2.26Largerfibromascanleadto
elephantiasisoftheeyelids.
2.7Tumors
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64. 46
Epidemiology:Approximately90%ofallmalignanteyelidtumorsarebasal
cellcarcinomas.Theirincidenceincreaseswithage.Inapproximately60%of
allcasestheyarelocalizedonthelowereyelid.Morbidityinsunnycountries
is110casesper100000persons(incentralEuropeapproximately20per
100000persons).Dark-skinnedpeopleareaffectedsignificantlylessoften.
Genderisnotapredisposingfactor.
Etiology:Causesofbasalcellcarcinomamayincludeageneticdisposition.
Increasedexposuretothesun’sultravioletradiation,carcinogenicsubstances
(suchasarsenic),andchronicskindamagecanalsoleadtoanincreasedinci-
dence.Basalcellcarcinomasarisefromthebasalcelllayersoftheepidermis
andthesebaceousglandhairfollicles,wheretheirgrowthlocallydestroys
tissue.
Symptoms:Typicalcharacteristicsincludeafirm,slightlyraisedmargin(a
haloresemblingastringofbeads)withacentralcraterandsuperficialvascular-
izationwithanincreasedtendencytobleed(Fig.2.27).
Ulcerationwith“gnawing”peripheralproliferationisoccasionally
referredtoasanulcusrodens;anulcusterebansreferstodeepinfiltrationwith
invasionofcartilageandbone.
Diagnosticconsiderations:Thediagnosiscanveryoftenbemadeonthe
basisofclinicalevidence.Abiopsyisindicatedifthereisanydoubt.
Lossoftheeyelashesinthevicinityofthetumoralwayssuggestsmalig-
nancy.
Treatment:Thelesionistreatedbysurgicalexcisionwithinamarginof
healthytissue.Thisisthesafestmethod.Ifaradicalprocedureisnotfeasible,
Basalcellcarcinoma.
Fig.2.27Ahalo
resemblinga
stringofbeads,
superficialvascu-
larization,anda
centralcrater
withatendency
tobleedare
characteristic
signsofthismod-
eratelymalignant
tumor.
2TheEyelids
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65. 47
theonlyremainingoptionsareradiationtherapyorcryotherapywithliquid
nitrogen.
Prognosis:Thechangesofsuccessfultreatmentbysurgicalexcisionarevery
good.Frequentfollow-upexaminationsareindicated.
Theearlierabasalcellcarcinomaisdetected,theeasieritistoremove.
2.7.2.2SquamousCellCarcinoma
Thisisthesecondmostfrequentlyencounteredmalignanteyelidtumor.The
carcinomaarisesfromtheepidermis,growsrapidlyanddestroystissue.Itcan
metastasizeintotheregionallymphnodes.Remotemetastasesarerarer.The
treatmentofchoiceiscompletesurgicalremoval.
2.7.2.3Adenocarcinoma
Therareadenocarcinomaarisesfromthemeibomianglandsortheglandsof
Zeis.Thefirm,painlessswellingisusuallylocatedintheuppereyelidandis
mobilewithrespecttotheskinbutnotwithrespecttotheunderlyingtissue.
Initsearlystagesitcanbemistakeneasilyforachalazion(seep.39).The
lesioncanmetastasizeintolocallymphnodes.
Anapparentchalazionthatcannotberemovedbytheusualsurgical
procedurealwayssuggestsasuspectedadenocarcinoma.
Thetreatmentofchoiceiscompletesurgicalremoval.
2.7Tumors
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67. 49
3 LacrimalSystem
PeterWagnerandGerhardK.Lang
3.1 BasicKnowledge
Thelacrimalsystem(Fig.3.1)consistsoftwosections:
!Structuresthatsecretetearfluid.
!Structuresthatfacilitateteardrainage.
Anatomyofthelacrimalsystem.
Orbital part of the
lacrimal gland
Plica semilunaris
Superior punctum lacrimale
Lacrimal sac
Nasolacrimal
duct
Superior lacrimal
canaliculus
Inferior concha
Fundus of the
lacrimal sac
Inferior punctum lacrimale
Fig.3.1Thelacrimalsystemconsistsoftearsecretionstructuresandteardrainage
structures.
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68. 50
Structureofthetearfilm.
Oily layer (approx. 0.1 µm)
– cholesteryl esters
– cholesterol
– triglyceride
– phospholipids
Water layer (approx. 8 µm)
– 98–99% water
– approx. 1% inorganic salts
– approx. 0.2–0.6% proteins,
globulins, and albumin
– approx. 0.02–0.06%
lysozyme
– Rest: glucose, urea, neutral
mucopolysaccharides
(mucin), and acidic
mucopolysaccharides
Mucin layer (approx. 0.8 µm)
Epithelium with microvilli
and folds
Oily layer,
0.1 µm
Meibomian glands
Lacrimal
gland
Water
layer,
8 µm
Conjunctival
goblet cells
Mucin layer,
0.8 µm
Fig.3.2Thetearfilmiscomposedofthreelayers:
!Anoilylayer(preventsrapiddesiccation).
!Awaterylayer(ensuresthatthecornearemainscleanandsmoothforoptimal
transparency).
!Amucinlayer(liketheoilyouterlayer,itstabilizesthetearfilm).
Position,structure,andnervesupplyofthelacrimalgland:Thelacrimal
glandisaboutthesizeofawalnut;itliesbeneaththesuperiortemporalmar-
ginoftheorbitalboneinthelacrimalfossaofthefrontalboneandisneither
visiblenorpalpable.Apalpablelacrimalglandisusuallyasignofapathologic
changesuchasdacryoadenitis.Thetendonofthelevatorpalpebraemuscle
dividesthelacrimalglandintoalargerorbitalpart(two-thirds)andasmaller
palpebralpart(one-third).Severaltinyaccessorylacrimalglands(glandsof
KrauseandWolfring)locatedinthesuperiorfornixsecreteadditionalserous
tearfluid.
Thelacrimalglandreceivesitssensorysupplyfromthelacrimalnerve.Its
parasympatheticsecretomotornervesupplycomesfromthenervusinterme-
dius.Thesympatheticfibersarisefromthesuperiorcervicalsympathetic
ganglionandfollowthecourseofthebloodvesselstothegland.
Tearfilm:Thetearfilm(Fig.3.2)thatmoistenstheconjunctivaandcorneais
composedofthreelayers:
1.Theouteroilylayer(approximately0.1µmthick)isaproductofthemei-
bomianglandsandthesebaceousglandsandsweatglandsofthemarginof
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69. 51
theeyelid.Theprimaryfunctionofthislayeristostabilizethetearfilm.
Withitshydrophobicproperties,itpreventsrapidevaporationlikealayer
ofwax.
2.Themiddlewaterylayer(approximately8µmthick)isproducedbythe
lacrimalglandandtheaccessorylacrimalglands(glandsofKrauseand
Wolfring).Itstaskistocleanthesurfaceofthecorneaandensuremobility
ofthepalpebralconjunctivaoverthecorneaandasmoothcornealsurface
forhigh-qualityopticalimages.
3.Theinnermucinlayer(approximately0.8µmthick)issecretedbythe
gobletcellsoftheconjunctivaandthelacrimalgland.Itishydrophilicwith
respecttothemicrovilliofthecornealepithelium,whichalsohelpstosta-
bilizethetearfilm.Thislayerpreventsthewaterylayerfromformingbeads
onthecorneaandensuresthatthewaterylayermoistenstheentiresurface
ofthecorneaandconjunctiva.
Lysozyme,beta-lysin,lactoferrin,andgammaglobulin(IgA)aretear-specific
proteinsthatgivethetearfluidantimicrobialcharacteristics.
Teardrainage:Theshingle-likearrangementofthefibersoftheorbicularis
oculimuscle(suppliedbythefacialnerve)causestheeyetocloseprogress-
ivelyfromlateraltomedialinsteadoftheeyelidssimultaneouslyclosing
alongtheirentirelength.Thiswindshieldwipermotionmovesthetearfluid
mediallyacrosstheeyetowardthemedialcanthus(Figs.3.3a–c).
Thesuperiorandinferiorpunctalacrimalescollectthetears,whichthen
drainthroughthesuperiorandinferiorlacrimalcanaliculiintothelacrimal
sac.Fromtheretheypassthroughthenasolacrimalductintotheinferior
concha(seeFig.3.1).
Combinedfunctionoftheorbicularisoculimuscleandthelowerlacrimal
system.
Opening the eye
Levator palpebrae
superioris muscle
(oculomotor nerve)
Closing the eye
Orbicularis oculi
muscle (facial
nerve)
Figs.3.3a–cAstheeyelidsclose,theyactlikeawindshieldwipertomovethetear
fluidmediallyacrosstheeyetowardthepunctaandlacrimalcanaliculi.
a b c
3.1BasicKnowledge
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70. 52
MeasuringtearsecretionwithSchirmerteartesting.
Fig.3.4Astrip
oflitmuspaperis
foldedoverand
insertedintothe
conjunctivalsac
ofthetemporal
thirdofthelower
eyelid.Normally,
atleast15mmof
thepapershould
turnbluewithin
fiveminutes.
3.2 ExaminationMethods
3.2.1 EvaluationofTearFormation
Schirmerteartesting:Thistest(Fig.3.4)providesinformationonthequan-
tityofwaterycomponentintearsecretion.
!Test:Astripoflitmuspaperisinsertedintotheconjunctivalsacofthetem-
poralthirdofthelowereyelid.
!Normal:Afteraboutfiveminutes,atleast15mmofthepapershouldturn
blueduetothealkalinetearfluid.
!Abnormal:Valueslessthan5mmareabnormal(althoughtheywillnot
necessarilybeassociatedwithclinicalsymptoms).
Thesamemethodisusedafterapplicationofatopicalanesthetictoevaluate
normalsecretionwithoutirritatingtheconjunctiva.
Tearbreak-uptime(TBUT):Thistestevaluatesthestabilityofthetearfilm.
!Test:Fluoresceindye(10µlofa0.125%fluoresceinsolution)isaddedtothe
precornealtearfilm.Theexaminerobservestheeyeunder10–20power
magnificationwithslitlampandcobaltbluefilterandnoteswhenthefirst
signsofdryingoccur(i)withoutthepatientclosingtheeyeand(ii)withthe
patientkeepingtheeyeopenasheorshewouldnormally.
!Normal:TBUTofatleast10secondsisnormal.
Rosebengaltest:Rosebengaldyesdeadepithelialcellsandmucin.Thistest
hasprovenparticularlyusefulinevaluatingdryeyes(keratoconjunctivitis
sicca)asitrevealsconjunctivalandcornealsymptomsofdesiccation.
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71. 53
Impressioncytology:AMilliporefilterisfastenedtoatonometerand
pressedagainstthesuperiorconjunctivawith20–30mmHgofpressurefor
twoseconds.Thedensityofgobletcellsisestimatedunderamicroscope
(normaldensityis20–45gobletcellspersquaremillimeterofepithelialsur-
face).Thenumberofmucus-producinggobletcellsisreducedinvariousdis-
orderssuchaskeratoconjunctivitissicca,ocularpemphigoid,andxeroph-
thalmia.
3.2.2EvaluationofTearDrainage
Conjunctivalfluoresceindyetest:Normalteardrainagecanbedemon-
stratedbyhavingthepatientblowhisorhernoseintoafacialtissuefollowing
applicationofa2%fluoresceinsodiumsolutiontotheinferiorfornix.
Probingandirrigation:Theseexaminationmethodsareusedtolocateste-
noses.Afterapplicationofatopicalanesthetic,aconicalprobeisusedto
dilatethepunctum.Thenthelowerlacrimalsystemisflushedwithaphysio-
logicsalinesolutionintroducedthroughabluntcannula(Figs.3.5aandb).If
thepassageisunobstructed,thesolutionwilldrainfreelyintothenose.
Canalicularstenosiswillresultinrefluxthroughtheirrigatedpunctum.
Ifthestenosisisdeeper,refluxwilloccurthroughtheoppositepunctum
(Fig.3.6).
Aprobecanbeusedtodeterminethesiteofthestricture,andpossiblyto
eliminateobstructions(Fig.3.7).
Radiographiccontraststudies:Radiographiccontrastmediumisinstilledin
thesamemannerasthesalinesolution.Thesestudiesdemonstratetheshape,
position,andsizeofthepassageandpossibleobstructionstodrainage.
Digitalsubstractiondacryocystography:Thesestudiesdemonstrateonly
thecontrastmediumandimagethelowerlacrimalsystemwithoutsuperim-
posedbonystructures.Theyareparticularlyusefulaspreoperativediagnos-
ticstudies(Fig.3.8).
Lacrimalendoscopy:Fineendoscopesnowpermitdirectvisualizationof
themucousmembraneofthelowerlacrimalsystem.Untilrecently,endo-
scopicexaminationofthelowerlacrimalsystemwasnotaroutineprocedure.
3.2ExaminationMethods
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72. 54
Irrigationofthelowerlacrimalsystemundertopicalanesthesia.
Figs.3.5aandb
Firstthepunc-
tumisdilatedby
rotatingaconical
probe.Thenthe
lacrimalpassage
isflushedwitha
physiologicsaline
solution.Theex-
aminershouldbe
particularlyalert
togooddrainage
orpossiblereflux.
a
b
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73. 55
Localizinganobstructionbyirrigatingthelowerlacrimalsystem.
No obstruction
Stenosis of the
inferior canaliculus
Stenosis of the inferior
common punctum
Stenosis within the
lacrimal sac
Fig.3.6Thelowerlacrimalsystem
shouldbeirrigatedwithcarebyanex-
periencedophthalmologist.Failureto
locatethepassagewillinflatetheeyelid
andprovidenodiagnosticinformation.
3.2ExaminationMethods
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74. 56
Openingastenosisofthelowerlacrimalsystemwithaprobe.
Figs.3.7a–cAfterapplicationofatopicalanesthetic,theprobeiscarefullyintro-
ducedintothelowerlacrimalsystem.Thepunctaaredilatedandthenthevalveof
Hasnerisopened(aandb).Adyesolutioncanthenbeintroducedtoverifypatency
ofthelowerlacrimalsystem(c).Ininfantssixmonthsorolder,theprocedureisbest
performedundershort-actinggeneralanesthesia.
a
c
b
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75. 57
Radiographicimageofthelowerlacrimalsystem.
Fig.3.8Digital
substraction
dacryocystogra-
phyimagesthe
lowerlacrimal
systemandcan
demonstratea
possiblestenosis
(arrow)without
superimposed
bonystructures.
3.3 DisordersoftheLowerLacrimalSystem
3.3.1 Dacryocystitis
Inflammationofthelacrimalsacisthemostfrequentdisorderofthelower
lacrimalsystem.Itisusuallytheresultofobstructionofthenasolacrimalduct
andisunilateralinmostcases.
3.3.1.1AcuteDacryocystitis
Epidemiology:Thedisordermostfrequentlyaffectsadultsbetweentheages
of50and60.
Etiology:Thecauseisusuallyastenosiswithinthelacrimalsac.Theretention
oftearfluidleadstoinfectionfromstaphylococci,pneumococci,Pseudo-
monas,orotherpathogens.
Symptoms:Clinicalsymptomsincludehighlyinflamed,painfulswellingin
thevicinityofthelacrimalsac(Fig.3.9)thatmaybeaccompaniedbymalaise,
fever,andinvolvementoftheregionallymphnodes.Thepainmaybereferredas
farastheforeheadandteeth.Anabscessinthelacrimalsacmayformin
advanceddisorders;itcanspontaneouslyrupturetheskinandformadrain-
ingfistula.
3.3DisordersoftheLowerLacrimalSystem
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76. 58
Acutedacryocystitis.
Fig.3.9Typical
symptomsin-
cludehighlyin-
flamed,painful
swellinginthevi-
cinityofthelacri-
malsac.
Acuteinflammationthathasspreadtothesurroundingtissueofthe
eyelidsandcheekentailsariskofsepsisandcavernoussinusthrombo-
sis,whichisalife-threateningcomplication.
Diagnosticconsiderations:Radiographiccontraststudiesordigitalsub-
stractiondacryocystographycanvisualizetheobstructionforpreoperative
planning.Thesestudiesshouldbeavoidedduringtheacutephaseofthedis-
orderbecauseoftheriskofpathogendissemination.
Differentialdiagnosis:
!Hordeolum(small,circumscribed,nonmobileinflamedswelling).
!Orbitalcellulitis(usuallyassociatedwithreducedmotilityoftheeyeball).
Treatment:Acutecasesaretreatedwithlocalandsystemicantibiotics
accordingtothespecificpathogensdetected.Disinfectantcompresses(suchas
a1:1000Rivanolsolution)canalsopositivelyinfluencetheclinicalcourseof
thedisorder.Pusfromafluctuatingabscessisbestdrainedthroughastabinci-
sionfollowingcryoanesthesiawitharefrigerantspray.
Treatmentafteracutesymptomshavesubsidedoftenrequiressurgery
(dacryocystorhinostomy;Figs.3.10a–c)toachievepersistentrelief.Also
knownasalowersystembypass,thisoperationinvolvesopeningthelateral
wallofthenoseandbypassingthenasolacrimalducttocreateadirectcon-
nectionbetweenthelacrimalsacandthenasalmucosa.
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77. 59
Dacryocystorhinostomy.
Orbital rima
b
c
Figs.3.10a–cAskinincisionismade,
andtheorbitalrimisexposed.Thena
windowisopenedtoexposethenasal
mucosa.Thenasalmucosaandthelacri-
malsacarebothincisedinanH-shape
anddoor-likeflapsareraised.Theante-
riorandposteriormucosalflapsarethen
suturedtogether.Thiscreatesanew
drainagerouteforthetearfluidthatby-
passesthenasolacrimalduct.
3.3DisordersoftheLowerLacrimalSystem
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78. 60
3.3.1.2ChronicDacryocystitis
Etiology:Obstructionofthenasolacrimalductisoftensecondarytochronic
inflammationoftheconnectivetissueornasalmucosa.
Symptomsanddiagnosticconsiderations:Theinitialcharacteristicof
chronicdacryocystitisisincreasedlacrimation.Signsofinflammationarenot
usuallypresent.Applyingpressuretotheinflamedlacrimalsaccauseslarge
quantitiesoftransparentmucoidpustoregurgitatethroughthepunctum.
Chronicinflammationofthelacrimalsaccanleadtoaserpiginouscor-
nealulcer.
Treatment:Surgicalinterventionistheonlyeffectivetreatmentinthevast
majorityofcases.Thisinvolveseitheradacryocystorhinostomy(creationofa
directconnectionbetweenthelacrimalsacandthenasalmucosa;seeFigs.
3.10a–c)orremovalofthelacrimalsac.
3.3.1.3NeonatalDacryocystitis
Etiology:Approximately6%ofnewbornshaveastenosisofthemouthofthe
nasolacrimalductduetoapersistentmucosalfold(lacrimalfoldorvalveof
Hasner).Theresultingretentionoftearfluidprovidesidealgrowthconditions
forbacteria,particularlystaphylococci,streptococci,andpneumococci.
Symptomsanddiagnosticconsiderations:Shortlyafterbirth(usually
withintwotofourweeks),pusissecretedfromthepuncta.Thediseasecon-
tinuessubcutaneouslyandpuscollectsinthepalpebralfissure.Theconjunc-
tivaisnotusuallyinvolved.
Differentialdiagnosis:
!Gonococcalconjunctivitisandinclusionconjunctivitis(seeFig.4.3).
!Silvercatarrh(harmlessconjunctivitiswithslimymucosalsecretionfol-
lowingCredé’smethodofprophylaxiswithsilvernitrate).
Treatment:Duringthefirstfewweeks,theinfantshouldbemonitoredfor
spontaneousopeningofthestenosis.Duringthisperiod,antibioticandanti-
inflammatoryeyedropsandnosedrops(suchaserythromycinandxylo-
metazoline0.5%forinfants)areadministered.
Ifsymptomspersist,irrigationorprobingundershort-actinggeneralanes-
thesiamaybeindicated(seeFigs.3.7a–c).
Oftenmassagingtheregionseveraltimesdailywhilecarefullyapplying
pressuretothelacrimalsacwillbesufficienttoopenthevalveofHasner
andeliminatetheobstruction.
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79. 61
3.3.2Canaliculitis
Definition
Thisusuallyinvolvesinflammationofthecanaliculus.
Epidemiologyandetiology:Genuinecanaliculitisisrare.Usuallyastricture
willbepresentandtheactualinflammationproceedsfromtheconjunctiva.
Actinomycetes(fungoidbacteria)oftencausepersistentpurulentgranular
concrementsthataredifficulttoexpress.
Symptomsanddiagnosticconsiderations:Thecanaliculusregionisswol-
len,reddened,andoftentendertopalpation.Pusorgranularconcrements
canbeexpressed.
Treatment:Thedisorderistreatedwithantibioticeyedropsandointments
accordingtothespecificpathogensdetectedincytologicsmears.Successful
treatmentoccasionallyrequiressurgicalincisionofthecanaliculus.
3.3.3TumorsoftheLacrimalSac
Epidemiology:Tumorsofthelacrimalsacarerarebutareprimarilymalig-
nantwhentheydooccur.Theyincludepapillomas,carcinomas,andsar-
comas.
Symptomsanddiagnosticconsiderations:Usuallythetumorscauseuni-
lateralpainlessswellingfollowedbydacryostenosis.
Diagnosticconsiderations:Theirregularandoccasionallybizarreformof
thestructureinradiographiccontraststudiesistypical.Ultrasound,CT,MRI,
andbiopsyallcontributetoconfirmingthediagnosis.
Differentialdiagnosis:Chronicdacryocystitis(seeabove),mucoceleofthe
ethmoidcells.
Treatment:Theentiretumorshouldberemoved.
3.3DisordersoftheLowerLacrimalSystem
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80. 62
3.4 LacrimalSystemDysfunction
3.4.1 KeratoconjunctivitisSicca
Definition
Noninfectiouskeratopathycharacterizedbyreducedmoisteningofthecon-
junctivaandcornea(dryeyes).
Epidemiology:Keratoconjunctivitissiccaasaresultofdryeyesisoneofthe
mostcommoneyeproblemsbetweentheagesof40and50.Asaresultofhor-
monalchangesinmenopause,womenarefarmorefrequentlyaffected(86%)
thanmen.Therearealsoindicationsthatkeratoconjunctivitissiccaismore
prevalentinregionswithhigherlevelsofenvironmentalpollution.
Etiology:Keratoconjunctivitissiccaresultsfromdryeyes,whichmaybedue
tooneoftwocauses:
!Reducedtearproductionassociatedwithcertainsystemicdisorders(such
asSjögren’ssyndromeandrheumatoidarthritis)orasaresultofatrophyor
destructionofthelacrimalgland.
!Alteredcompositionofthetearfilm.Thecompositionofthetearfilmcan
alterduetovitaminAdeficiency,medications(suchasoralcontraceptives
andretinoids),orcertainenvironmentalinfluences(suchasnicotine,
smog,orairconditioning).Thetearfilmbreaksuptooquicklyandcauses
cornealdrying.
Dryeyescanrepresentadisorderinandofitself.
Symptoms:Patientscomplainofburning,reddenedeyes,andexcessivelacri-
mation(reflexlacrimation)fromonlyslightenvironmentalcausessuchas
wind,cold,lowhumidity,orreadingforanextendedperiodoftime.Aforeign
bodysensationisalsopresent.Thesesymptomsmaybeaccompaniedby
intensepain.Eyesightisusuallyminimallycompromisedifatall.
Diagnosticconsiderations:Oftenthereisadiscrepancybetweenthemini-
malclinicalfindingsthattheophthalmologistcanestablishandtheintense
symptomsreportedbythepatient.ResultsfromSchirmerteartestingusually
showreductionsofthewaterycomponentoftears,andthetearbreak-up
time(whichprovidesinformationaboutthemucincontentofthetearfilm
whichisimportantforitsstability)isreduced.Valuesofatleast10seconds
arenormal;thetearbreak-uptimeinkeratoconjunctivitissiccaislessthan5
seconds.
Slitlampexaminationwillrevealdilatedconjunctivalvesselsandminimal
pericornealinjection.Atearfilmmeniscuscannotbedemonstratedonthe
lowereyelidmargin,andthelowereyelidwillpushtheconjunctivaalongin
foldsinfrontofit.
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81. 63
Inseverecasestheeyewillbereddened,andthetearfilmwillcontainthick
mucusandsmallfilamentsthatproceedfromasuperficialepitheliallesion
(filamentarykeratitis;seeFig.5.11).Thecorneallesioncanbedemonstrated
withfluoresceindye.Inlessseverecasestheeyewillonlybereddened,
althoughapplicationoffluoresceindyewillrevealcorneallesions(superficial
punctatekeratitis;seep.138).Therosebengaltest(seep.52)andimpression
cytology(seep.53)areadditionaldiagnosticteststhatareusefulinevaluat-
ingpersistentcases.
Treatment:Dependingontheseverityoffindings,artificialtearsolutionsin
varyingviscositiesareprescribed.Theserangefromeyedropstohigh-viscos-
itylong-actinggelsthatmaybeappliedeveryhouroreveryhalfhour,
dependingontheseverityofthedisorder.Inpersistentcases,thepunctacan
betemporarilyclosedwithsiliconepunctalplugs(Fig.3.11)toatleastretain
thefewtearsthatarestillproduced.Surgicalobliterationofthepunctamay
beindicatedinseverecases.
Patientsshouldalsobeinformedaboutthepossibilityofinstallinganair
humidifierinthehomeandredirectingblowersinautomobilestoavoid
furtherdryingoftheeyes.Dryeyesinwomenmayalsobeduetohormonal
changes,andagynecologistshouldbeconsultedregardingthepatient’shor-
monalstatus.
Prognosis:Theprognosisisgoodforthosetreatmentsdiscussedhere.
However,thedisordercannotbecompletelyhealed.
Treatmentofdryeyes.
Fig.3.11Treat-
mentcanbeaug-
mentedbytem-
porarilyclosing
thepunctawith
siliconepunctal
plugs.
3.4LacrimalSystemDysfunction
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82. 64
3.4.2Illacrimation
Illacrimationorepiphoramaybeduetohypersecretionfromthelacrimal
gland.However,itismoreoftencausedbyobstructeddrainagethroughthe
lowerlacrimalsystem.
Causesofhypersecretion:
!Emotionaldistress(crying).
!Increasedirritationoftheeyes(bysmoke,dust,foreignbodies,injury,or
intraocularinflammation)leadstoexcessivelacrimationinthecontextof
thedefensivetriadofblepharospasm,photosensitivity,andepiphora.
Causesofobstructeddrainage:
!Strictureorstenosisinthelowerlacrimalsystem.
!Eyeliddeformity(eversionofthepunctumlacrimale,ectropion,or
entropion).
3.5 DisordersoftheLacrimalGland
3.5.1 AcuteDacryoadenitis
Definition
Acuteinflammationofthelacrimalglandisararedisordercharacterizedby
intenseinflammationandextremetendernesstopalpation.
Etiology:Thedisorderisoftenattributabletopneumococciandstaphylo-
cocci,andlessfrequentlytostreptococci.Theremaybearelationship
betweenthedisorderandinfectiousdiseasessuchasmumps,measles,scar-
letfever,diphtheria,andinfluenza.
Symptomsanddiagnosticconsiderations:Acutedacryoadenitisusually
occursunilaterally.Theinflamedswollenglandisespeciallytendertopalpa-
tion.
TheuppereyelidexhibitsacharacteristicS-curve(Fig.3.12).
Differentialdiagnosis:
!Internalhordeolum(smallerandcircumscribed).
!Eyelidabscess(fluctuation).
!Orbitalcellulitis(usuallyassociatedwithreducedmotilityoftheeyeball).
Treatment:Thiswilldependontheunderlyingdisorder.Moistheat,disinfect-
antcompresses(Rivanol),andlocalantibioticsarehelpful.
Clinicalcourseandprognosis:Acuteinflammationofthelacrimalglandis
characterizedbyarapidclinicalcourseandspontaneoushealingwithineight
3LacrimalSystem
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83. 65
totendays.Theprognosisisgood,andcomplicationsarenotusuallytobe
expected.
3.5.2ChronicDacryoadenitis
Etiology:Thechronicformofinflammationofthelacrimalglandmaybethe
resultofanincompletelyhealedacutedacryoadenitis.Diseasessuchastuber-
culosis,sarcoidosis,leukemia,orlymphogranulomatosiscanbecausesof
chronicdacryoadenitis.
Bilateralchronicinflammationofthelacrimalandsalivaryglandsis
referredtoasMikulicz’ssyndrome.
Symptomsanddiagnosticconsiderations:Usuallythereisnopain.The
symptomsarelesspronouncedthanintheacuteform.However,theS-curve
deformityofthepalpebralfissureresultingfromswellingofthelacrimal
glandisreadilyapparent(seeFig.3.12).
Differentialdiagnosis:
!Periostitisoftheupperorbitalrim(rare).
!Lipodermoid(nosignsofinflammation).
Treatment:Thiswilldependontheunderlyingdisorder.Systemiccorti-
costeroidsmaybeeffectiveintreatingunspecificforms.
Prognosis:Theprognosisforchronicdacryoadenitisisgoodwhentheunder-
lyingdisordercanbeidentified.
Acutedacryoadenitis.
Fig.3.12
Characteristic
S-curveofthe
uppereyelid.
3.5DisordersoftheLacrimalGland
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84. 66
3.5.3TumorsoftheLacrimalGland
Epidemiology:Tumorsofthelacrimalglandaccountfor5–7%oforbitalneo-
plasms.Lacrimalglandtumorsaremuchrarerinchildren(approximately2%
oforbitaltumors).Therelationofbenigntomalignanttumorsofthelacrimal
glandspecifiedintheliteratureis10:1.Themostfrequentbenignepithelial
lacrimalglandtumoristhepleomorphicadenoma.Malignanttumors
includetheadenoidcysticcarcinomaandpleomorphicadenocarcinoma.
Etiology:TheWHOclassificationof1980divideslacrimalglandtumorsinto
thefollowingcategories:
I.Epithelialtumors.
II.Tumorsofthehematopoieticorlymphatictissue.
III.Secondarytumors.
IV.Inflamedtumors.
V.Otherandunclassifiedtumors.
Symptoms:Tumorsusuallygrowveryslowly.Afterawhile,theydisplacethe
eyeballinferiorlyandmedially,whichcancausedoublevision.
Diagnosticconsiderations:Testingmotilityprovidesinformationaboutthe
infiltrationofthetumorintotheextraocularmusclesormechanicalchanges
intheeyeballresultingfromtumorgrowth.Theechogenicityofthetumorin
ultrasoundstudiesisanindicationofitsconsistency.CTandMRIstudies
showtheexactlocationandextentofthetumor.Abiopsywillconfirm
whetheritismalignantandwhattypeoftumoritis.
Treatment:Totheextentthatthisispossible,theentiretumorshouldbe
removed;orbitalexenteration(removaloftheentirecontentsoftheorbit)
mayberequired.Systemicadministrationofcorticosteroidsisindicatedfor
unspecifictumors.
Prognosis:Thisdependsonthedegreeofmalignancyofthetumor.Adenoid
cysticcarcinomashavethemostunfavorableprognosis.
3LacrimalSystem
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85. 67
4 Conjunctiva
GerhardK.LangandGabrieleE.Lang
4.1 BasicKnowledge
Structureoftheconjunctiva(Fig.4.1):Theconjunctivaisathinvascular
mucousmembranethatnormallyofshinyappearance.Itformstheconjunc-
tivalsactogetherwiththesurfaceofthecornea.Thebulbarconjunctivais
looselyattachedtothescleraandismorecloselyattachedtothelimbusofthe
cornea.Theretheconjunctivalepitheliumfuseswiththecornealepithelium.
Thepalpebralconjunctivalinestheinnersurfaceoftheeyelidandisfirmly
attachedtothetarsus.Theloosepalpebralconjunctivaformsafoldinthe
conjunctivalfornix,whereitjoinsthebulbarconjunctiva.Ahalf-moon-
shapedfoldofmucousmembrane,theplicasemilunaris,islocatedinthe
medialcornerofthepalpebralfissure.Thisbordersonthelacrimalcaruncle,
whichcontainshairsandsebaceousglands.
Functionoftheconjunctivalsac:Theconjunctivalsachasthreemaintasks:
1.Motilityoftheeyeball.Thelooseconnectionbetweenthebulbarconjunc-
tivaandthescleraandthe“spare”conjunctivaltissueinthefornicesallow
theeyeballtomovefreelyineverydirectionofgaze.
2.Articulatinglayer.Thesurfaceoftheconjunctivaissmoothandmoistto
allowthemucousmembranestoglideeasilyandpainlesslyacrosseach
other.Thetearfilmactsasalubricant.
3.Protectivefunction.Theconjunctivamustbeabletoprotectagainst
pathogens.Follicle-likeaggregationsoflymphocytesandplasmacells(the
lymphnodesoftheeye)arelocatedbeneaththepalpebralconjunctivaand
inthefornices.Antibacterialsubstances,immunoglobulins,interferon,
andprostaglandinshelpprotecttheeye.
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86. 68
Anatomyoftheconjunctiva.
Glands of Krause
Glands of Wolfring
Accessory lacrimal
glands:
Meibomian
gland
Bulbar
conjunctiva
Conjunctival
fornix
Palpebral
conjunctiva
Surface of the
cornea (functions
as a part of the
conjunctival sac)
Fig.4.1Theconjunctivaconsistsofthebulbarconjunctiva,theconjunctivalfor-
nices,andthepalpebralconjunctiva.Thesurfaceofthecorneafunctionsasthefloor
oftheconjunctivalsac.
4.2 ExaminationMethods
Inspection:Thebulbarconjunctivacanbeevaluatedbydirectinspection
underafocusedlight.Normallyitisshinyandtransparent.Theotherpartsof
theconjunctivawillnotnormallybevisible.Theycanbeinspectedbyevert-
ingtheupperorlowereyelid(seeeyelideversionbelow).
4Conjunctiva
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87. 69
Dyestaining:Defectsandtearsintheconjunctivaorcorneacanbevisualized
byapplyingadropoffluoresceindyeorrosebengalandinspectingtheeye
underilluminationwithacobaltbluefilter(seeFig.5.11,p.139).
Eyelideversion:Eventhenon-ophthalmologistmustbefamiliarwiththe
techniqueofevertingtheupperorlowereyelid.Thisisanimportantexami-
nationmethodincasesinwhichtheconjunctivalsacrequirescleaningor
irrigation,suchasremovingaforeignbodyorrenderingfirstaidaftera
chemicalinjury.SeeChapter1foradetaileddescriptionoftheexamination
method.
4.3 ConjunctivalDegenerationandAgingChanges
4.3.1 Pingueculum
Definition
Harmlessgrayishyellowthickeningoftheconjunctivalepitheliuminthepalpe-
bralfissure.
Epidemiology:Pingueculaarethemostfrequentlyobservedconjunctival
changes.
Etiology:Theharmlessthickeningoftheconjunctivaisduetohyaline
degenerationofthesubepithelialcollagentissue.Advancedageandexposure
tosun,wind,anddustfostertheoccurrenceofthedisorder.
Symptoms:Pingueculumdoesnotcauseanysymptoms.
Diagnosticconsiderations:Inspectionwillrevealgrayishyellowthickening
at3o’clockand9o’clockonthelimbus.Thebaseofthetriangularthickening
(oftenlocatedmedially)willbeparalleltothelimbusofthecornea;thetip
willbedirectedtowardtheangleoftheeye(Fig.4.2).
Differentialdiagnosis:Apingueculumisanunequivocalfinding.
Treatment:Notreatmentisnecessary.
4.3.2Pterygium
Definition
Triangularfoldofconjunctivathatusuallygrowsfromthemedialportionofthe
palpebralfissuretowardthecornea.
Epidemiology:Pterygiumisespeciallyprevalentinsoutherncountriesdue
toincreasedexposuretointensesunlight.
4.3ConjunctivalDegenerationandAgingChanges
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88. 70
Pinguecula.
Fig.4.2Harm-
lesstriangular
pingueculum
whosebaseis
paralleltothe
cornea(arrow).
Etiology:Histologically,apterygiumisidenticaltoapinguecula.However,it
differsinthatitcangrowontothecornea;thegrayheadofthepterygiumwill
growgraduallytowardthecenterofthecornea(Fig.4.3a).Thisprogressionis
presumablytheresultofadisorderofBowman’slayerofthecornea,whichpro-
videsthenecessarygrowthsubstrateforthepterygium.
Symptomsanddiagnosticconsiderations:Apterygiumonlyproduces
symptomswhenitsheadthreatensthecenterofthecorneaandwithitthe
visualaxis(Fig.4.3b).Tensileforcesactingonthecorneacancausesevere
cornealastigmatism.Asteadilyadvancingpterygiumthatincludesscarred
conjunctivaltissuecanalsograduallyimpairocularmotility;thepatientwill
thenexperiencedoublevisioninabduction.
Differentialdiagnosis:Apterygiumisanunequivocalfinding.
Treatment:Treatmentisonlynecessarywhenthepterygiumproducesthe
symptomsdiscussedabove.Surgicalremovalisindicatedinsuchcases.The
headandbodyofthepterygiumarelargelyremoved,andthescleraisleft
openatthesite.Thecorneaisthensmoothedwithadiamondreameroran
excimerlaser(aspeciallaserthatoperatesintheultravioletrangeata
wavelengthof193nm).
Clinicalcourseandprognosis:Pterygiatendtorecur.Keratoplastyisindi-
catedinsuchcasestoreplacethediseasedBowman’slayerwithnormal
tissue.OtherwisethediseasedBowman’slayerwillcontinuetoprovidea
growthsubstrateforarecurrentpterygium.
4Conjunctiva
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89. 71
Pterygium.
Fig.4.3aTriangularfoldofconjunctivagrowingfromthemedialportionofthe
palpebralfissuretowardthecornea.bPterygiumthathasgrownontothecornea
andthreatenstheopticalaxis.
a
b
4.3.3Pseudopterygium
Apseudopterygiumduetoconjunctivalscarringdiffersfromapterygiumin
thatthereareadhesionsbetweenthescarredconjunctivaandthecorneaand
sclera.Causesincludecornealinjuriesand/orchemicalinjuriesandburns.
Pseudopterygiacausepainanddoublevision.Treatmentconsistsoflysisof
theadhesions,excisionofthescarredconjunctivaltissue,andcoverageofthe
defect(thismaybeachievedwithafreeconjunctivalgraftharvestedfromthe
temporalaspect).
4.3ConjunctivalDegenerationandAgingChanges
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90. 72
4.3.4SubconjunctivalHemorrhage
Extensivebleedingundertheconjunctiva(Fig.4.4)frequentlyoccurswith
conjunctivalinjuries(forobtainingahistoryintraumacases,seeChapter18,
conjunctivallaceration).Subconjunctivalhemorrhagingwillalsooftenoccur
spontaneouslyinelderlypatients(asaresultofcompromisedvascularstruc-
turesinarteriosclerosis),oritmayoccuraftercoughing,sneezing,pressing,
bendingover,orliftingheavyobjects.Althoughthesefindingsareoftenvery
unsettlingforthepatient,theyareusuallyharmlessandresolvespon-
taneouslywithintwoweeks.Thepatient’sbloodpressureandcoagulation
statusneedonlybecheckedtoexcludehypertensionorcoagulationdisorders
whensubconjunctivalhemorrhagingoccursrepeatedly.
4.3.5CalcareousInfiltration
Aforeign-bodysensationintheeyeisoftencausedbywhitepunctateconcre-
mentsonthepalpebralconjunctiva.Theseconcrementsarethecalcifiedcon-
tentsofgobletcells,accessoryconjunctivalandlacrimalglands,ormei-
bomianglandswherethereisinsufficientdrainageofsecretion.Thesecal-
careousinfiltratescanberemovedwithascalpelundertopicalanesthesia.
4.3.6ConjunctivalXerosis
Definition
DesiccationoftheconjunctivaduetoavitaminAdeficiency.
Subconjunctivalhemorrhage.
Fig.4.4Exten-
sivebleeding
underthecon-
junctiva.
4Conjunctiva
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91. 73
Epidemiology:Duetothehighgeneralstandardofnutrition,thisdisorderis
veryrareinthedevelopedworld.However,itisoneofthemostfrequent
causesofblindnessindevelopingcountries.
Etiology:VitaminAdeficiencyresultsinkeratinizationofthesuperficial
epithelialcellsoftheeye.Degenerationofthegobletcellscausesthesurface
oftheconjunctivatoloseitluster(Fig.4.5a).Thekeratinizedepithelialcells
dieandaresweptintothepalpebralfissurebyblinking,wheretheyaccumu-
lateandcreatecharacteristicwhiteBitot’sspots(Fig.4.5b).Xerosisbacteria
frequentlyproliferate.
Conjunctivalxerosisduetovitaminadeficiency.
Fig.4.5aKerat-
inizationofthe
superficial
epithelialcells
causesthesur-
faceofthecon-
junctivatolose
itsluster.
bThekeratinized
epithelialcellsdie
andcreate
characteristic
Bitot’sspotsin
thepalpebralfis-
sure.
4.3ConjunctivalDegenerationandAgingChanges
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92. 74
Treatmentandprognosis:Thechangesdisappearafterlocalandsystemic
vitaminAsubstitution.WithoutvitaminAsubstitution,thedisorderwilllead
toblindnesswithinafewyears.
4.4 Conjunctivitis
4.4.1 GeneralNotesontheCauses,Symptoms,andDiagnosisof
Conjunctivitis
Definition
Conjunctivitisisaninflammatoryprocessinvolvingthesurfaceoftheeyeand
characterizedbyvasculardilation,cellularinfiltration,andexudation.Two
formsofthedisorderaredistinguished:
!Acuteconjunctivitis.Onsetisabruptandinitiallyunilateralwithinflamma-
tionofthesecondeyewithinoneweek.Durationislessthanfourweeks.
!Chronicconjunctivitis.Durationislongerthanthreetofourweeks.
Epidemiology:Conjunctivitisisoneofthemostfrequenteyedisorders.
Etiology:Causesofconjunctivitismaybefallintotwobroadcategories:
!Infectious(seeFig.4.2)
–bacterial
–viral
–parasitic
–mycotic
!Noninfectious(seeFig.4.4)
-fromapersistentirritation(suchaslackoftearfluidoruncorrected
refractiveerror;seeFig.4.4)
-allergic
-toxic(duetoirritantssuchassmoke,dust,etc.)
-asaresultofanotherdisorder(suchasStevens–Johnsonsyndrome).
Symptoms:Typicalsymptomsexhibitedbyallpatientsincludereddened
eyesandstickyeyelidsinthemorningduetoincreasedsecretion.Anycon-
junctivitisalsocausesswellingoftheeyelid,whichwillappearpartially
closed(pseudoptosis).Foreign-bodysensation,asensationofpressure,anda
burningsensationareusuallypresent,althoughthesesymptomsmayvary
betweenindividualpatients.Intenseitchingalwayssuggestsanallergicreac-
tion.Photophobiaandlacrimation(epiphora)mayalsobepresentbutcan
varyconsiderably.Simultaneouspresenceofblepharospasmsuggestscor-
nealinvolvement(keratoconjunctivitis).
4Conjunctiva
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93. 75
Diagnosticconsiderations:Therearemanycausesofconjunctivitis,andthe
clinicalpictureandsymptomscanvaryconsiderablybetweenindividual
patients.Thismakesitallthemoreimportanttonotecertaincharacteristic
findingsthatpermitanaccuratediagnosis,suchasthetypeofexudation,con-
junctivalfindings,orswollenpreauricularlymphnodes(Table4.1).
Hyperemia.Reddenedeyesareatypicalsignofconjunctivitis.Theconjuncti-
valinjectionisduetoincreasedfillingoftheconjunctivalbloodvessels,
whichoccursmostprominentlyintheconjunctivalfornices.Hyperemiais
presentinallformsofconjunctivitis.However,thevisibilityofthehyperemic
vesselsandtheirlocationandsizeareimportantcriteriafordifferentialdiag-
nosis.Onecanalsodistinguishconjunctivitisfromotherdisorderssuchas
scleritisorkeratitisaccordingtotheinjection(Fig.4.6).Thefollowingtypesof
injectionaredistinguished.
!Conjunctivalinjection(brightred,clearlyvisibledistendedvesselsthat
movewiththeconjunctiva,decreasingtowardthelimbus;Fig.4.7).
!Pericornealinjection(superficialvessels,circularorcircumscribedinthe
vicinityofthelimbus).
Formsofconjunctivalinjection.
Conjunctival
Conjunctival
disorders;
conjunctivitis
Mixed
Corneal disorders
with intraocular
irritation;
corneal ulcerations
CiliaryPericorneal
Conjunctival
disorders near
the cornea:
– rosacea
– corneal lesions
near the limbus
– foreign body
– herpetic keratitis
Disorders of deeper
tissues and intraocular
structures:
– episcleritis
– scleritis
– disciform keratitis
– iritis
– cyclitis
Fig.4.6
4.4Conjunctivitis
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94. 76
T
able
4.
1
Symp
t
oms
and
findings
in
conjunctivitis
as
the
y
relat
e
t
o
v
arious
f
o
rms
o
f
t
he
disorder
Sympt
om
or
f
inding
Bacterial conjunctivitis
Chlam
ydial
conjunctivitis
V
i
r
a
l
conjunctivitis
Allergic conjunctivitis
T
o
xic
conjunctivitis
Itc
hing
–
–
–
+
+
–
Hyperemia
(reddened
e
y
e)
+
+
+
+
+
+
Bleeding
+
–
+
–
–
Disc
harge
Pur
ulent;
y
ello
w
cr
usts
Mucopurulent
W
a
t
e
r
y
R
o
p
y
whit
e,
viscous
–
Chemosis
++
–
!
++
!
Lacrimation
(epiphor
a)
+
+
+
+
+
+
F
ollicles
–
+
+
+
+
+
P
apillae
+
!
–
+
–
Pseudomembr
anes,
membr
anes
!
–
!
–
–
Sw
ollen
lymph
nodes
+
+
+
+
–
–
P
annus
formation
–
+
–
–
!
4Conjunctiva
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95. 77
Concurrent
k
e
r
atitis
!
+
!
–
!
F
e
v
e
r
o
r
angina
!
–
!
–
–
R
esults
of
cyt
ologic
smear
Granulocyt
es,
bact
eria
Intracyt
oplasmic
inclusions
in
epi-
thelial
cells,
leuk
o-
cyt
es,
plasma
cells,
lymphocyt
es
L
y
mphocyt
es,
monocyt
es
Eosinophilic g
r
a
n
u
loc
y
t
e
s
,
lymphocyt
es
Epithelial
cells,
g
r
a
n
u
loc
y
t
e
s
,
lymphocyt
es
++
=
S
e
v
ere
+
=
Moderat
e
!
=
Occasional
–
=
R
a
re
or
absent
4.4Conjunctivitis
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96. 78
Conjunctivalinjection.
Fig.4.7Clearly
visible,bright
red,distended
conjunctivalves-
sels,decreasing
towardthelim-
busofthecor-
nea.
!Ciliaryinjection(notclearlydiscernible,brightlycolorednonmobileves-
selsintheepiscleranearthelimbus).
!Compositeinjection(frequent).
Discharge.Thequantityandnatureoftheexudate(mucoid,purulent,watery,
ropy,orbloody)dependontheetiology(seeTable4.1).
Chemosis(Fig.4.8).Thismayrangefromtheabsenceofanyconjunctival
thickeningtoawhiteglassyedemaandswellingoftheconjunctivaprojecting
fromthepalpebralfissure(chemosisthissevereoccurswithbacterialand
allergicconjunctivitis).
Epiphora(excessivetearing).Illacrimationshouldbedistinguishedfrom
exudation.Illacrimationisusuallyreflexlacrimationinreactiontoaconjunc-
tivalorcornealforeignbodyortoxicirritation.
Follicle.Lymphocytesinthepalpebralandbulbarconjunctivaaccumulatein
punctatemassesoflymphtissuecellsthathaveagranularappearance.Fol-
liclesoccurtypicallyinviralandchlamydialinfections(Fig.4.9).
Papillae.Papillaeappearaspolygonal“cobblestone”conjunctivalprojections
withacentralnetworkoffinelybranchingvessels.Theyareatypicalsignof
allergicconjunctivitis(Fig.4.10).
Membranesandpseudomembranes.Theseareconjunctivalreactionsto
severeinfectiousortoxicconjunctivitis.Theyformfromnecroticepithelial
tissueandeithercanbeeasilyremovedwithoutbleeding(pseudomem-
branes)orleavebehindableedingsurfacewhentheyareremoved(mem-
branes;Figs.4.11a,b).
4Conjunctiva
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97. 79
Conjunctivalchemosis.
Fig.4.8White
glassyedemaand
swellingofthe
conjunctiva.
Follicularconjunctivitis.
Fig.4.9Punc-
tatemassesof
lymphtissuecells
ofagranularap-
pearance.
Swollenlymphnodes.Lymphfromtheeyeregiondrainsthroughthe
preauricularandsubmandibularlymphnodes.Swollenlymphnodesarean
importantandfrequentlyencountereddiagnosticsignofviralconjunctivitis.
Pannusformation.ConjunctivalorvascularingrowthbetweenBowman’s
layerandthecornealepitheliumintheuppercircumference.
Thecombinationandseverityofindividualsymptomsusuallyprovide
essentialinformationthathelpstoidentifytherespectivepresenting
formofconjunctivitis.
4.4Conjunctivitis
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98. 80
Papillaryconjunctivitis.
Fig.4.10Ever-
sionoftheupper
eyelidreveals
“cobblestone”
conjunctivalpro-
jections.
Granulomas.Theseareinflamednodesofconjunctivalstromawithcircum-
scribedareasofreddeningandvascularinjection.Theycanoccurwithsys-
temicdisorderssuchastuberculosisorsarcoidosisormaybeexogenous,such
aspostoperativesuturegranulomasorotherforeign-bodygranulomas.
Granulomasoccurinconjunctionwithswollenpreauricularandsubman-
dibularlymphnodesindisorderssuchasParinaud’soculoglandularsyn-
drome.Granulomasarenotasignofconjunctivitisinthestrictsenseandfor
thatreasonhavenotbeenincludedassymptomsorfindingsinTable4.1.
Examinationmethods:Slitlampexamination.
Thenatureandextentofvascularinjections,discharge,conjunctivalswelling,
etc.areevaluatedusingaslitlamp.
Eyelideversion.Thisisperformedtoexaminetheupperandlowereyelidsfor
thepresenceoffollicles,papillae,membranes,andforeignbodies.
Ifthediagnosisisuncertainorwhatappearstobebacterialconjunctivitis
doesnotrespondtoantibiotics,aconjunctivalsmear(Fig.4.12)shouldbe
obtainedformicrobiologicalexaminationtoidentifythepathogen.Cotton
swabswithsterileshippingtubesarecommerciallyavailable;specialtestkits
withspecificculturesareavailablefordetectingchlamydiae.
Anantibioticthatisnoteffectiveintreatingwhatappearstobebacte-
rialconjunctivitisshouldbediscontinued.Aconjunctivalsmearshould
thenbeobtained24hourslater.Microbiologicalexaminationtoiden-
tifythepathogenisindicatedforanyconjunctivitisinchildren.
Epithelialsmear.Thisisusedtodetectchlamydiaeinparticularandtomore
clearlyidentifythepathogeningeneral.Ascrapingofconjunctival
4Conjunctiva
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99. 81
Membranousconjunctivitis.
Figs.4.11a,b
Genuinemem-
branes(a)leave
behindableed-
ingsurfacewhen
heyareremoved
(b).
epitheliumissmearedonaslideanddyedwithGiemsaandGramstain.Cyto-
logicfindingsprovideimportantinformationabouttheetiologyofthecon-
junctivitis.
!bacterialconjunctivitis:granulocyteswithpolymorphousnucleiandbac-
teria;
!viralconjunctivitis:lymphocytesandmonocytes;
!chlamydialconjunctivitis(specialformofbacterialconjunctivitis):com-
positefindingsoflymphocytes,plasmacells,andleukocytes;characteris-
ticintracytoplasmicinclusionbodiesinepithelialcellsmayalsobepresent
(seeFig.4.13);
a
b
4.4Conjunctivitis
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100. 82
Conjunctivalsmearformicrobiologicalexamination.
Fig.4.12The
lowereyelidis
slightlyeverted
andasmearof
theconjunctival
secretionisob-
tainedwithacot-
tonswab.
!allergicconjunctivitis:findingsprimarilyincludeeosinophilicgranulo-
cytesandlymphocytes;
!mycoticconjunctivitis(veryrare):theGiemsaorGramstainwillrevealthe
hyphae.
Irrigation.Conjunctivitiswilloccuroccasionallyinasymptomaticdacryocys-
titis(seep.60)orcanaliculitis(seep.61)asaresultofcontinuousscattered
spreadofbacteria.Thelowerlacrimalsystemshouldalwaysbeirrigatedin
thepresenceofinflammationthatrecursorresiststreatmenttoverifyor
excludethatitisthesourceoftheinflammation.
4.4.2InfectiousConjunctivitis
Thenormalconjunctivacontainsmicroorganisms.Inflammationusually
occursasaresultofinfectionfromdirectcontactwithpathogens(suchasfrom
afinger,towel,orswimmingpool)butalsofromcomplicatingfactors(suchas
acompromisedimmunesystemorinjury).Therearesignificantregional
differencesinthespectrumofpathogens.Table4.2providesanoverviewof
pathogens,symptoms,andtreatments.
4.4.2.1BacterialConjunctivitis
Epidemiology:Bacterialconjunctivitisisveryfrequentlyencountered.
Etiology:Staphylococcus,streptococcus,andpneumococcusinfectionsare
mostcommonintemperatecountries.
4Conjunctiva
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101. 83
Symptoms:Typicalsymptomsincludeseverereddening,swellingofthecon-
junctiva,andpurulentdischargethatleadstoformationofyellowishcrusts.
Diagnosticconsiderations:Bacterialconjunctivitiscanusuallybereliably
diagnosedfromthepresenceoftypicalsymptoms.Laboratorytests(conjunc-
tivalsmear)areusuallyonlynecessarywhentheconjunctivitisfailsto
respondtoantibiotictreatment.
Bacterialconjunctivitisisdiagnosedonthebasisofclinicalsymptoms.
Smearsareobtainedonlyinsevere,uncertain,orpersistentcases.
Treatment:Bacterialconjunctivitisusuallyrespondsverywelltoantibiotic
treatment.Awiderangeofwelltolerated,highlyeffectiveantibioticagentsis
availabletoday.Mostofthesearesuppliedasointments(whicharelonger
actingandsuitableforovernighttherapy)andaseyedropsfortopicaltherapy.
Substancesincludegentamicin,tobramycin,Aureomycin,chloramphenicol,
1
neomycin,polymyxinBincombinationwithbacitracinandneomycin,Ter-
ramycin,kanamycin,fusidicacid,ofloxacin,andacidamphenicol.
1
1
SeeAppendixforsideeffectsofmedications
Preparationsthatcombineanantibioticandcortisonecanmorerapidly
alleviatesubjectivesymptomswhenfindingsarecloselymonitored.These
includemedicationssuchasgentamicinanddexamethasone;neomycin,
polymyxinB,anddexamethasone;ortetracycline,polymyxinB,andhydro-
cortisone.
Insevere,uncertain,orpersistentcasesrequiringmicrobiologicalexami-
nationtoidentifythepathogen,treatmentwithbroad-spectrumantibiotics
ortopicalantibioticcombinationpreparationsthatcoverthefullrangeof
Gram-positiveandGram-negativepathogensshouldbeginimmediately.This
methodisnecessarybecausemicrobiologicalidentificationofthepathogen
andresistancetestingoftheantibioticarenotalwayssuccessfulandmay
requireseveraldays.Itisnotadvisabletoleavetheconjunctivitisuntreated
forthisperiod.
Inthepresenceofsevere,uncertain,orpersistentconjunctivitis,treat-
mentwithbroad-spectrumantibioticsortopicalantibioticcombination
preparationsshouldbeinitiatedimmediately,evenbeforethelabora-
toryresultsareavailable.
Clinicalcourseandprognosis:Bacterialconjunctivitisusuallyrespondswell
toantibiotictreatmentandremitswithinafewdays.
4.4.2.2ChlamydialConjunctivitis
ChlamydiaareGram-negativebacteria.
4.4Conjunctivitis
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102. 84
T
able
4.
2
Ov
er
view
o
f
inf
ectious
conjunctivitis
Cause
Clinical cour
se
Sympt
oms
and
f
indings
P
a
thogen
T
r
eatment
Bact
eria
S
t
aph
ylococcal
conjunctivitis
Subacut
e
Pur
ulent
disc
harge,
blepharitis,
super
ficial
punct
at
e
k
eratitis,
thic
k
ening
of
the
con-
junctivitis
at
the
lim-
bus
S
t
aph
ylococci:
Gram-posi-
t
iv
e
c
lu
s
t
e
r
f
o
r
m
S
t
rep
t
ococcal
conjunctivitis
Subacut
e
W
at
er
y
mucoid
dis-
c
harge,
conjunctiv
al
sw
elling,
pseudomem-
b
r
a
n
e
s
S
t
r
e
p
t
o
c
o
c
c
i
:
Gram-posi-
t
iv
e
c
h
a
in
f
o
r
m
T
opical:
broad-spec-
tr
um
antibio
tic
(suc
h
as
neom
y
cin,
k
anam
y-
cin,
t
e
tracy
cline,
gen-
t
amicin,
or
c
hloram-
phenicol)*
Pneumococcal conjunctivitis
A
cut
e
Moderat
ely
purulent
disc
harge,
c
hemosis,
multiple
subconjuncti-
v
a
l
hemorrhages,
cor-
neal
ulceration
Pneumococci:
B
r
ig
h
t
ly
encapsulat
ed
Gram-positiv
e
lance
t-shaped
diplococci
!"""""""""""""""""#"""""""""""""""""$
4Conjunctiva
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103. 85
Bact
eria
Diphtheric conjunctivitis (must
b
e
repor
t
ed)
A
cut
e
Moderat
ely
purulent
disc
harge,
adhesiv
e
co
v
e
rings
(mem-
branes)
dominat
e,
conjunctiv
al
necrosis,
e
y
elid
edema
Gr
am-positiv
e
diplobacilli
!
T
opical:
broad-spec-
tr
um
antibio
tic
(see
abo
v
e
)
!
Syst
emic:
300
–
500
units
per
k
g
of
diph-
theria
antit
o
xin
IV
;
antibio
tics:
penicillin,
t
e
tracy
cline
Gonococcal conjunctivitis
Hyper- acut
e
Cream
y
purulent
dis-
c
harge,
b
right
red
con-
junctiv
a,
sw
ollen
e
y
e-
lids
and
conjunctiv
a
Gonococci
(
N
eisseria
gonor-
rhoeae
):
intracellular
Gram-
neg
ativ
e
diplococci
!
T
opical:
broad-spec-
tr
um
antibio
tic
(gen-
t
amicin,
k
anam
y
cin,
t
e
tracy
cline,
c
hloram-
phenicol)*
!
Syst
emic:
penicillin
f
o
r
4
–
5
d
a
y
s
:
–
N
ewborn:
1
meg
a-
unit
per
da
y
–
Children:
2
meg
a-
units
per
da
y
–
A
dults:
4
–
5
meg
a-
units
per
da
y
Continued
!
4.4Conjunctivitis
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104. 86
T
able
4.
2
(Continued)
Cause
Clinical cour
se
Sympt
oms
and
f
indings
P
a
thogen
T
r
eatment
Bact
eria
Pseudomonas conjunctivitis
Hyper- acut
e
Pur
ulent
disc
harge,
of
t
e
n
with
corneal
in
v
o
lv
ement.
F
ulmi-
nant
cour
se:
inf
ection
ma
y
b
e
spread
b
y
u
n
s
t
e
r
ile
e
y
e
d
r
o
p
bo
ttles
and
cont
act
lens
holder
s.
The
bac-
t
e
rium
emits
an
enzyme
(pro
t
eogly-
can)
that
can
pene-
trat
e
t
he
cornea
within
2
4
hour
s.
Gram-neg
ativ
e
Pseudo-
monas
aeruginosa
(
Bacillus
p
y
ocy
aneus
)
T
opical:
broad-spectr
um
antibio
tic
(gent
amicin,
polym
yxin
B,
c
hloram-
phenicol)*
Haemophilus influenzae conjunctivitis
Subacut
e
Serous,
mucopurulent
disc
harge;
especially
common
in
c
hildren.
C
o
r
n
e
a
l
in
v
o
lv
e
m
e
n
t
is
rare.
Haemophilus
inf
luenzae
:
Gram-neg
ativ
e
r
ods
T
opical:
broad-spectr
um
antibio
tic
(see
abo
v
e
)
4Conjunctiva
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105. 87
Bact
eria
Haemophilus aegyp
tius
(K
oc
h-
W
eek
s)
conjunctivitis
A
cut
e
Highly
inf
ectious
con-
junctivitis
pre
v
alent
in
w
a
r
m
c
o
u
n
t
r
ie
s
,
r
a
r
e
in
t
e
m
p
e
r
a
t
e
c
ou
n
-
tries;
e
y
elid
sw
elling,
c
hemosis,
subconjunc-
tiv
al
hemorrhaging,
pseudomembranes, corneal
ulceration
Haemophilus
aegyp
tius
(K
oc
h-
W
eek
s):
fine
Gram-
neg
ativ
e
r
ods
T
opical:
broad-spectr
um
antibio
tic
(t
e
t
racy
cline,
k
anam
y
cin,
gent
amicin)
Morax
ella
conjunctivitis
Subacut
e
Minimal
disc
harge,
moderat
e
irrit
ation
(circumscribed
in
the
a
n
g
le
o
f
t
h
e
e
y
e
w
it
h
accompan
ying
blepharoconjunctivi- tis).
Corneal
ulceration
ma
y
occur
.
Morax
ella
lacunat
a
(Morax-
Ax
enf
eld
diplobacillus):
la
r
g
e
G
r
a
m
-
n
e
g
a
t
iv
e
diplobacilli
T
opical:
broad-spectr
um
antibio
tic
0.25
–
0.5
%
zinc
sul-
fat
e
e
y
edrops
are
considered
t
o
be
an
ef
f
ectiv
e
specific
treatment
Continued
!
4.4Conjunctivitis
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106. 88
T
able
4.
2
(Continued)
Cause
Clinical cour
se
Sympt
oms
and
f
indings
P
a
thogen
T
r
eatment
Chlam
ydia
Inclusion conjunctivitis
A
cut
e
t
o
c
h
r
o
n
ic
Moderat
ely
r
eddened
e
y
e,
typical
viscous
disc
harge,
s
tic
ky
e
y
es,
t
a
r
sal
f
ollicles
on
the
upper
and
lo
w
e
r
e
y
e
-
lids,
super
ficial
punc-
t
a
t
e
k
e
rat
oconjunctivi-
tis,
spread
of
pannus
across
the
limbus
of
the
cornea,
occasional
peripheral
sub-
epithelial
corneal
infil-
t
r
a
t
e
s
Chlam
ydia
trac
homatis
(sero
type
D-K)
!
T
opical:
er
ythrom
y
cin
or
t
e
tracy
cline
f
o
r
2
–
3
w
eek
s
!
Syst
emic:
er
ythrom
y-
cin
or
t
e
tracy
cline
f
o
r
at
least
3
w
eek
s
Bew
a
re:
disorder
will
recur
if
medication
is
dis-
c
o
n
t
in
u
e
d
t
o
o
e
a
r
ly
T
r
ac
homa
Chronic
R
a
re
in
t
e
mperat
e
countries
but
endemic
in
w
a
r
m
c
lim
a
t
e
s
.
L
y
mph
f
ollicles
on
the
palpebral
conjunctiv
a
of
the
upper
e
y
elid,
cicatricial
entropion,
p
t
osis,
tric
hiasis,
cor-
n
e
a
l
s
c
a
r
r
in
g
,
x
e
r
o
s
is
of
the
conjunctiv
a.
F
our
st
ages
of
the
dis-
order
are
distin-
guished.
Chlam
ydia
trac
homatis
(sero
type
A
-C)
As
in
inclusion
conjunctiv-
itis
4Conjunctiva
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107. 89
V
ir
u
s
e
s
!
Epidemic k
e
r
a
t
o
-
conjunctivitis
A
cut
e
Highly
cont
agious
con-
junctivitis.
W
a
t
e
r
y
mucoid
disc
harge,
c
hemosis,
e
y
elid
edema,
reddening
and
sw
elling
of
the
plica
semilunaris
and
lacri-
mal
car
uncle
(
c
harac-
t
e
r
is
t
ic
s
ig
n
),
sw
ollen
preauricular
lymph
nodes;
of
t
e
n
t
here
will
be
a
moderat
e
influenza
inf
ection.
Nummular
k
e
ratitis
will
appear
af
t
e
r
8
–
1
5
d
a
y
s
(
c
haract
eristic
sign
).
!
A
deno
virus
(adenoid
phar
yngeal
conjuncti-
v
al);
types
1
8
and
1
9
are
most
f
requent.
!
No
specific
treatment
is
possible.
Symp
t
o
-
matic
moist
ening
treatment.
!
Proph
ylaxis:
me
ticu-
lous
h
y
giene.
Human
int
er
f
e
ron
(Berof
or)
pre
v
ents
inf
ection
in
e
xposed
patients
(e
xtremely
e
xpensiv
e).
!
Herpes
simple
x
conjunctivitis
!
H
e
r
p
e
s
z
o
s
t
e
r
ophthalmicus
A
cut
e,
mild
K
e
r
a
t
it
is
a
n
d
k
e
r
a
t
o
-
conjunctivitis
alw
a
y
s
accompanied
b
y
crops
of
v
esicles
on
an
er
ythemat
ous
base
on
the
e
y
elids
!
Herpes
vir
us
!
V
a
ricella-zost
e
r
vir
us
T
opical:
acy
clo
vir
oint-
ment !
Syst
emic:
acy
clo
vir
IV
if
necessar
y
Continued
!
0,
1
µ
m
0,
1
µ
m
4.4Conjunctivitis
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108. 90
T
able
4.
2
(Continued)
Cause
Clinical cour
se
Sympt
oms
and
f
indings
P
a
thogen
T
r
eatment
P
a
rasit
es
Onc
hocerciasis
(riv
er
blindness)
Chronic
Conjunctivitis
from
m
ic
r
o
f
ila
r
ia
,
p
r
o
g
-
ressing
t
o
k
e
ratitis,
iridocy
clitis,
uv
eitis,
and
conjunctiv
al
scar-
ring.
This
is
most
frequent
cause
of
blindness
in
Africa.
Onc
hocerca
v
o
lvulus
(trans-
mitt
ed
b
y
the
f
lies
of
the
genus
Simulium
)
Syst
emic
treatment
with
Iv
ermectin
no
w
a
v
ailable
(treatment
t
a
k
e
s
y
ear
s)
Loa
loa
Chronic
Conjunctivitis
from
microfilaria.
The
para-
sit
es
are
visible
with
the
nak
ed
e
y
e
under
the
conjunctiv
a
and
will
flee
the
light
of
the
slit
lamp).
The
dis-
order
is
endemic
in
w
e
s
t
A
f
r
ic
a
Loa
loa
(f
emale
5
–
7
"
0.5
cm;
male
3
–
3.5
"
0.3
cm)
Surgical
remo
v
a
l
o
f
t
he
w
o
rms
f
rom
t
he
conjunc-
tiv
a
2,0 – 4,5 cm 23 – 50 cm 3,3 – 3,4 cm 5,0 – 7
,0 cm
4Conjunctiva
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109. 91
P
a
rasit
es
Nodose conjunctivitis
Chronic
V
e
r
y
rare
conjunctivi-
tis.
Cat
erpillar
hair
s
accident
ally
f
ind
their
w
a
y
int
o
t
he
conjuncti-
v
a
l
sac.
The
hair
s
h
a
v
e
barbs
and
w
o
rk
their
w
a
y
deep
int
o
t
he
tissue.
Granulomas
de
v
elop
on
the
con-
junctiv
a.
Blindness
can
result
when
these
hair
s
pene
trat
e
int
o
the
int
erior
of
the
e
y
e
.
Caterpillar
hair
s
Surgical
remo
v
a
l
o
f
t
he
cat
erpillar
hair
s,
t
opical
st
eroid
therap
y
F
ungi
My
co
tic
conjunctivitis
A
cut
e
F
requently
associat
ed
with
m
y
c
o
tic
k
e
ratitis
or
secondar
y
t
o
m
y
co
tic
canaliculitis
Hyphae
As
with
m
y
c
o
tic
k
e
ratitis:
syst
emic
and
t
opical
antim
y
c
o
tic
therap
y
*
See
Appendix
f
o
r
side
ef
f
ects
of
medications
4.4Conjunctivitis
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110. 92
Chlamydialconjunctivitis.
Fig.4.13Cyto-
logicsmear
showingtypical
basophiliccyto-
plasmicinclusion
bodies.
InclusionConjunctivitis
Epidemiology:Inclusionconjunctivitisisveryfrequentintemperatecoun-
tries.Theincidenceinwesternindustrializedcountriesrangesbetween1.7%
and24%ofallsexuallyactiveadultsdependingonthespecificpopulation
studied.
Etiology:Oculogenitalinfection(ChlamydiatrachomatisserotypeD–K)is
alsocausedbydirectcontact.Inthenewborn(seeneonatalconjunctivitis),
thisoccursatbirththroughthecervicalsecretion.Inadults,itisprimarily
transmittedduringsexualintercourse,andrarelyfrominfectioninpoorly
chlorinatedswimmingpools.
Symptoms:Theeyesareonlymoderatelyredandslightlystickyfromviscous
discharge.
Diagnosticconsiderations:Tarsalfolliclesareobservedtypicallyonthe
upperandlowereyelids,andpannuswillbeseentospreadacrossthelimbus
ofthecornea.Asthisisanoculogenitalinfection,itisessentialtodetermine
whetherthemotherhasanyhistoryofvaginitis,cervicitis,orurethritisif
thereisclinicalsuspicionofneonatalinfection.Gynecologicorurologic
examinationisindicatedinappropriatecases.Chlamydiamaybedetectedin
conjunctivalsmears,byimmunofluorescence,orintissuecultures.Typical
cytologicsignsincludebasophiliccytoplasmicinclusionbodies(Fig.4.13).
Treatment:Inadults,thedisorderistreatedwithtetracyclineorerythromy-
cineyedropsorointmentoveraperiodoffourtosixweeks.Theoculogenital
modeofinfectionentailsariskofreinfection.Therefore,patientsandsexual
4Conjunctiva
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111. 93
Trachoma(StageII–III).
Fig.4.14Promi-
nenttarsalfol-
liclesandpapillae
ontheupperand
lowereyelids.
partnersoftreatedpatientsshouldallbetreatedsimultaneouslywithoral
tetracycline.Childrenshouldbetreatedwitherythromycininsteadoftetracy-
cline(seethetableintheAppendixforsideeffectsofmedications).
Prognosis:Theprognosisisgoodwhenthesexualpartnerisincludedinther-
apy.
Trachoma
Trachoma(ChlamydiatrachomatisserotypeA–C)israreintemperatecoun-
tries.Inendemicregions(warmclimates,poorstandardofliving,andpoor
hygiene),itisamongthemostfrequentcausesofblindness(seeTable4.2for
symptoms,findings,andtherapy).Leftuntreated,thedisorderprogresses
throughfourstages(Fig.4.14):
!StageI:Hyperplasiaofthelymphfolliclesontheuppertarsus.
!StageII:Papillaryhypertrophyoftheuppertarsus,subepithelialcorneal
infiltrates,pannusformation,folliclesonthelimbus.
!StagesIIIandIV:Increasingscarringandsymptomsofkeratoconjunctivi-
tissicca.Theprogressionisentropion,trichiasis,keratitis,superinfection,
ulceration,perforation,andfinallylossoftheeye.
4.4.2.3ViralConjunctivitis
Epidemiology:Theincidenceofepidemickeratoconjunctivitisishighin
general,anditisbyfarthemostfrequentlyencounteredviralconjunctivitis
(seeTable4.2).
4.4Conjunctivitis
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112. 94
Etiology:Thishighlycontagiousconjunctivitisisusuallycausedbytype18or
19adenovirusandisspreadbydirectcontact(seealsoprophylaxis;Figs.4.15
aandb).Theincubationperiodiseighttotendays.
Symptoms:Onsetisusuallyunilateral.Typicalsignsincludesevereillacri-
mationanditchingaccompaniedbyawaterymucoiddischarge.Theeyelid
andoftentheconjunctivitisareswollen.Patientsoftenalsohaveamoderate
influenzainfection.
Diagnosticconsiderations:Characteristicfindingsincludereddeningand
swellingoftheplicasemilunarisandlacrimalcaruncleandnummularker-
atitis(Fig.4.15b)after8–15days,duringthehealingphase.
Epidemickeratoconjunctivitis(viralconjunctivitis).
Fig.4.15
aAcuteunilateral
reddeningofthe
conjunctivaac-
companiedby
pseudoptosis.
bAfter8–10
dayscoin-likein-
filtrates(nummu-
larkeratitis)ap-
pearinthesuper-
ficialcorneal
stroma.These
maypersistfor
monthsoryears.
4Conjunctiva
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113. 95
Differentialdiagnosis:Thediseaserunsawelldefinedclinicalcoursethatis
nearlyimpossibletoinfluenceandresolvesaftertwoweeks.Nospecificther-
apyispossible.Treatmentwithartificialtearsandcoolcompresseshelps
relievesymptoms.Cortisoneeyedropsshouldusuallybeavoidedastheycan
compromisetheimmunesystemandprolongtheclinicalsymptoms.
Prophylaxis:Thisisparticularlyimportant.Becausethediseaseisspreadby
contact,thepatientshouldrefrainfromrubbinghisorhereyesdespitea
severeitchingsensationandavoiddirectcontactwithotherpeoplesuchas
shakinghands,sharingtools,orusingthesametowelsorwashcloths,etc.
Specialhygieneprecautionsshouldbetakenwhenexaminingpatients
withepidemickeratoconjunctivitisinophthalmologiccarefacilitiesanddoc-
tors’officestominimizetheriskofinfectingotherpatients.Patientswithepi-
demickeratoconjunctivitisshouldnotbeseatedinthesamewaitingroomas
otherpatients.Theyshouldnotbegreetedwithahandshake,andtheyshould
berequestedtorefrainfromtouchingobjectswherepossible.Examination
shouldbebyindirectmeansonly,avoidingapplanationtonometry,contact
lensexamination,orgonioscopy.Afterexamination,theexaminershould
cleanhisorherhandsandtheworksitewithasurfacedisinfectant.
4.4.2.4NeonatalConjunctivitis
Epidemiology:Approximately10%ofthenewborncontractconjunctivitis.
Etiology(Table4.3):ThemostfrequentpathogensareChlamydia,followed
bygonococci.Neonatalconjunctivitisislessfrequentlyattributabletoother
bacteriasuchasPseudomonasaeruginosa,Haemophilus,Staphylococcus
aureusandStreptococcuspneumoniae,ortoherpessimplex.Theinfection
occursatbirth.Chlamydiainfectionsareparticularlyimportantbecausethey
areamongthemostcommonundetectedmaternalgenitaldiseasesin
Europe,affecting5%ofallpregnantwomen.Neonatalconjunctivitissome-
timesoccursasaresultofCredé’smethodofprophylaxiswithsilvernitrate,
requiredbylawinEuropetopreventbacterialinfection.
Symptoms:Dependingonthepathogen,theinflammationwillmanifest
itselfbetweenthesecondandfourteenthdayoflife(Table4.3).Thespectrum
rangesfrommildconjunctivalirritationtolife-threateninginfection
(especiallywithgonococcalinfection).ConjunctivitisasaresultofCredé’s
methodofprophylaxisappearswithhoursbutonlyleadstomildconjunctival
irritation.
Acutepurulentconjunctivitisinthenewborn(gonococcalconjunctivi-
tis)isconsideredamedicalemergency.Thepatientshouldbereferred
toanophthalmologistforspecificdiagnosis.
4.4Conjunctivitis
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114. 96
Table4.3Differentialdiagnosisofneonatalconjunctivitis(ophthalmianeonatorum)
Cause Onset Findings Cytologyand
laboratorytests
Toxic(AgNO3:silver
nitrate;Credé’spro-
phylaxis)
Withinhours!Hyperemia
!Slightwateryto
mucoiddischarge
Negativeculture
Gonococci(gono-
coccalconjunctivitis)
2nd–4thday
oflife
!Acutepurulent
conjunctivitis
IntracellularGram-
negativediplococci;
positivecultureon
bloodagarandchoc-
olateagar
Otherbacteria(Pseu-
domonasaeruginosa,
Staphylococcus
aureus,Streptococcus
pneumoniae,
Haemophilus)
4th–5thday
oflife
!Mucopurulent
conjunctivitis
Gram-positiveor
Gram-negative
organisms;positive
cultureonblood
agar
Chlamydia(inclusion
conjunctivitis)
5th–14thday
oflife
!Mucopurulent
conjunctivitis,
lessfrequently
purulent
!Viscousmucus
Giemsa-positive
cytoplasmicinclu-
sionbodiesin
epithelialcells;nega-
tiveculture
Herpessimplexvirus5th–7thday
oflife
!Wateryblepharo-
conjunctivitis
!Cornealinvolve-
ment
!Systemicmanifes-
tations
Multinucleatedgiant
cells,cytoplasmic
inclusionbodies;
negativeculture
Diagnosticconsiderations:Thetentativeclinicaldiagnosisismadeonthe
basisoftheonsetofthedisease(Table4.3)andtheclinicalsyndrome.For
example,gonococcalinfections(gonococcalconjunctivitis)aretypifiedby
particularlysevereaccumulationsofpus(Figs.4.16aandb).Thenewborn’s
eyelidaretightandswollenbecausethepusaccumulatesunderthem.When
thebaby’seyesareopened,thepuscansquirtoutunderpressureandcause
dangerousconjunctivitisintheexaminer’sowneyes.
Theexaminershouldalwaysweareyeprotectioninthepresenceofsus-
pectedgonococcalconjunctivitistoguardagainstinfectionfrompus
issuingfromthenewborn’seyes.Gonococcicanpenetratetheeyeeven
intheabsenceofacornealdefectandleadtolossoftheeye.
4Conjunctiva
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115. 97
Neonatalconjunctivitis(gonococcalconjunctivitis).
Fig.4.16aHighlyinfectiousconjuncti-
vitiswithswellingoftheeyelidsand
creamypurulentdischargeissuingfrom
thepalpebralfissure.
bTheGramstainoftheconjunctival
smearrevealscharacteristicGram-nega-
tiveintracellulardiplococci(gonococci).
Thediagnosisshouldbeconfirmedbycytologicandmicrobiologicalstudies.
However,thesestudiesoftenfailtoyieldunequivocalresults,sothattreat-
mentmustproceedonthebasisofclinicalfindings.
Differentialdiagnosis:Theonsetofthediseaseiscrucialtodifferentialdiag-
nosis(Table4.3).Neonatalconjunctivitismustbedistinguishedfrom
neonataldacryocystitis.Thisdisorderdiffersfromthespecificformsofcon-
junctivitisinitonlybecomessymptomatictwotofourweeksafterbirth,with
reddeningandswellingoftheregionofthelacrimalsacandpurulentdis-
chargefromthepuncta.Itcanbereadilydistinguishedfromneonatalcon-
junctivitisbecauseofthesesymptoms.
Treatment:Toxicconjunctivitis(Credé’smethodofprophylaxis):Whenthe
eyeisregularlyflushedandtheeyelidscleaned,symptomswillabatespon-
taneouslywithinoneortwodays.
Gonococcalconjunctivitis:Topicaladministrationofbroad-spectrumanti-
biotics(gentamicineyedropseveryhour)andsystemicpenicillin(penicillinG
4.4Conjunctivitis
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116. 98
IV2mill.IUdaily)orcephalosporininthepresenceofpenicillinase-produc-
ingstrains.
Chlamydialconjunctivitis:Systemicerythromycinandtopicalerythromy-
cineyedropsfivetimesdaily.Thereisariskofrecurrencewherethedosageor
durationoftreatmentisinsufficient.Itisessentialtoexaminetheparentsand
includethemintherapy.
Herpessimplexconjunctivitis:Therapyinvolvesapplicationofacyclovir
ointmenttotheconjunctivalsacandeyelidsasherpesvesicleswillusuallybe
presentthere,too.Systemicacyclovirtherapyisonlyrequiredinseverecases.
Prophylaxis:Credé’smethodofprophylaxis(applicationof1%silvernitrate
solution)preventsbacterialinflammationbutnotchlamydialorherpesinfec-
tion.Prophylaxisofchlamydialinfectionconsistsofregularexaminationof
thewomanduringpregnancyandtreatmentinappropriatecases.
4.4.2.5ParasiticandMycoticConjunctivitis
Parasiticandmycoticformsofconjunctivitis(seeTable4.2)arelessimportant
intemperateclimates.Theyareeitherveryrareoroccurprimarilyasco-
morbiditiesassociatedwithaprimarycornealdisorder,suchasmycotic
infectionsofcornealulcers.
4.4.3NoninfectiousConjunctivitis
Table4.4providesanoverviewofpathogens,symptoms,andtreatmentsof
noninfectiousconjunctivitis.
Acuteconjunctivitisisfrequentlyattributabletoaseriesofexternalirri-
tantsortodryeyes(conjunctivitissicca).Thedisorderisunpleasantbut
benign.Primarysymptomsincludeforeign-bodysensation,reddeningofthe
eyesofvaryingseverity,andepiphora.Therapyshouldfocusoneliminating
theprimaryirritantandtreatingthesymptoms.
Acuteconjunctivitisshouldbedistinguishedfromthegroupofallergic
formsofconjunctivitis,whichcanbeduetoseasonalinfluencesandoften
affectthenasalmucosa.Examplesincludeallergicconjunctivitis(hayfever;
Fig.4.17)andvernalconjunctivitis.Ingiantpapillaryconjunctivitis,theinflam-
mationistriggeredbyaforeignbody(hardorsoftcontactlenses.Theremay
alsobeanadditionalchronicmicrobialirritationsuchmicrobialcontamina-
tionofcontactlenses.Phlyctenularkeratoconjunctivitisisadelayedallergic
reactiontomicrobialproteinsortoxins(staphylococcalinflammation).This
diseaseoccursfrequentlyinatopicindividualsandispromotedbypoor
hygiene.Thecardinalruleinallergicconjunctivitisistoavoidthecausative
agent.Desensitizationshouldbeperformedasaprophylacticmeasurebya
dermatologistorallergist.Long-termtreatmentincludescromoglycicacid
eyedropstopreventmastcelldegranulation.Treatmentofacuteallergiccon-
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117. 99
junctivitisconsistsofadministeringcoolingcompresses,artificialtearswith
preservatives,astringenteyedrops(tetryzolineandnaphazoline),and,ifnec-
essary,surface-actingcortisoneeyedrops(fluorometholone).
Ocular-mucocutaneoussyndromessuchasStevens–Johnsonsyndrome
(erythemamultiforme),Lyell’ssyndrome(toxicepidermalnecrolysis),and
ocularpemphigoid(progressiveshrinkageoftheconjunctiva)areclinicalsyn-
dromesthatinvolvemultipletoxicandimmunologiccausativemechanisms.
Theclinicalcourseofthedisorderissevere,therapeuticoptionsarelimited,
andtheprognosisforeyesightispoor(Fig.4.18).
Seasonalallergicconjunctivitis.
Fig.4.17Con-
junctivalswelling
(chemosis)ina
patientwithhay
fever.
Stevens–Johnsonsyndrome(erythemamultiforme).
Fig.4.18After
severalyearsthe
conjunctivalsac
hasfused
completely(total
symblepharon),
effectivelycaus-
ingblindness.
4.4Conjunctivitis
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118. 100
T
able
4.
4
Ov
er
view
o
f
noninf
ectious
conjunctivitis
Cause
and
form
of
conjunctivitis
Clinical cour
se
Sympt
oms
and
f
indings
Other
c
har
acteristic
features
T
r
eatment
I
r
r
it
a
n
t
A
c
u
t
e
c
o
n
-
junctivitis
A
cut
e
t
o
c
h
r
o
n
ic
F
o
reign
body
sensation,
conjunctiv
al
reddening,
epiphora,
blepharitis
!
Lac
k
o
f
t
ear
s
(
k
e
rat
o
-
conjunctivitis
sicca)
!
E
x
t
e
r
n
a
l
ir
r
it
a
n
t
s
:
smok
e,
heat,
cold,
wind
(car
windo
w
o
r
open
con
v
e
r
tible
t
op),
ultra
viole
t
light
(w
elding,
high-alti-
tude
sunlight).
!
P
ositional
anomalies
of
the
e
y
elids
or
e
y
e-
lashes
!
Uncorrect
ed
refrac-
t
iv
e
e
r
r
o
r
(
u
s
u
a
lly
h
yperopia)
!
Dysfunction
of
binocular
vision
(uncompensat
ed
h
e
t
e
r
o
p
h
o
r
ia
)
!
I
m
p
r
o
p
e
r
ly
c
e
n
t
e
r
e
d
e
y
eglasses
or
wrong
c
o
r
r
e
c
t
io
n
!
Ov
ere
x
er
tion,
lac
k
o
f
sleep
(burnout
syn-
d
r
o
m
e
)
!
Ar
tificial
t
ear
s
!
A
v
oiding
s
p
e
c
if
ic
ir
r
i-
t
ants
!
Correction
of
anomaly
o
r
e
y
elash
epila-
tion
!
Ey
eglasses
!
Prism
lenses
!
Cent
er
or
replace
e
y
e-
glass
lenses
!
R
e
s
t
!"""""""""""""""""#"""""""""""""""""$
Specific elimina- tion
of
u
n
d
e
r
ly
-
ing
cause
4Conjunctiva
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119. 101
Allergy
Allergic
con-
junctivitis
(ha
y
f
e
v
er)
A
cut
e
(seasonal)
Se
v
e
re
t
earing,
c
hemo-
sis
(can
be
e
xtremely
se
v
e
re),
w
a
t
e
r
y
dis-
c
harge,
f
oreign
body
sensation,
sneezing
T
ypically
accompanied
b
y
rhinitis;
seasonal
allergy
t
o
pollen,
grasses,
and
plant
aller-
gens.
!
Desensitization
!
Astringent
e
y
edrops
(t
e
t
r
y
-
zoline,
naphazoline),
if
nec-
essar
y
with
sur
face-acting
cor
tisone
e
y
edrops
(fluorome
tholone)
V
e
rnal
con-
junctivitis
A
cut
e
(seasonal)
!
T
a
r
sal
and
conjuncti-
v
a
l
f
orm:
“cobble-
st
one”
conjunctiv
al
projections
on
the
palpebral
conjunc-
tiv
a
o
f
t
he
upper
e
y
elid,
pseudop
t
osis,
f
o
reign
body
sensa-
tion,
epiphora
!
Limbic
f
o
rm:
Sw
elling
of
the
bulbar
con-
junctiv
a
is
t
he
pri-
mar
y
symp
t
om,
accompanied
b
y
a
ring
of
nodules
on
the
limbus
of
the
cornea,
f
o
reign
body
sensation,
and
epi-
p
h
or
a
.
!
Corneal
in
v
o
lv
ement:
W
idespread
corneal
erosion
t
o
whic
h
mucus
adheres
(plaques),
def
ensiv
e
triad
of
pain,
blepharo-
spasm,
and
epiphora.
Occur
s
in
b
o
y
s
and
male
adolescents
during
spring,
either
isolat
ed
in
the
e
y
e
s
o
r
in
combination
with
generalized
asthma;
IgE-mediat
ed
reaction.
!
Brief
treatment
with
cor
ti-
sone
e
y
edrops
t
o
control
sw
elling
!
A
c
e
t
ylcyst
eine
gel
t
o
liquify
the
mucus
!
Cromogly
cic
acid
e
y
edrops
as
proph
ylaxis
during
the
asymp
t
omatic
int
er
v
a
l
!
Le
v
ocabastine
h
ydroc
hloride
Continued
!
4.4Conjunctivitis
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120. 102
T
able
4.
4
(Continued)
Cause
and
form
of
conjunctivitis
Clinical cour
se
Sympt
oms
and
f
indings
Other
c
har
acteristic
features
T
r
eatment
Allergy
Giant
papillar
y
conjunctivitis
Chronic
Conjunctiv
al
reddening
a
n
d
ir
r
it
a
t
io
n
w
it
h
p
r
o
-
nounced
papillar
y
h
yper
troph
y
,
similar
t
o
the
f
indings
and
symp-
t
oms
in
v
e
rnal
con-
junctivitis
F
r
equently
due
t
o
o
v
er-
w
earing
cont
act
lenses
(especially
sof
t
lenses);
microbial
component
is
probable
(smear
should
be
obt
ained)
Use
of
cont
act
lenses
should
be
discontinued
until
the
inflam-
mation
abat
es.
Cont
act
lenses
should
be
replaced
or
refitt
ed;
if
the
disorder
recur
s,
the
y
should
be
discontinued.
Phly
c
t
enular
k
er-
at
oconjunctivitis
Chronic
Discre
t
e
nodular
areas
of
inflammation
of
the
cornea
or
conjunctiv
a
(phly
c
t
enules),
pho
t
o
-
phobia,
epiphora,
itc
h-
ing,
rarely
f
o
reign
body
sensation,
no
pain
Usually
occur
s
in
c
hildren
and
y
oung
adults
living
in
poor
h
y
gienic
conditions
and
in
countries
c
haract
er-
ized
b
y
a
high
rat
e
of
tuberculosis.
The
dis-
ease
is
uncommon
in
w
e
s
t
e
r
n
c
o
u
n
t
r
ie
s
.
T
opical
broad-spectr
um
antibi-
o
tics
combined
with
cor
tisone
or
cor
tisone
e
y
edrops
alone
pro
vide
rapid
relief
of
symp-
t
oms.
Ocular
-muco-
cut
aneous
syndrome
S
t
e
v
ens-Johnson
syndrome (er
ythema
multi-
f
o
r
m
e
)
Chronic
Allergic,
membranous
conjunctivitis
with
blis-
t
e
ring
and
increasing
symblepharon;
of
t
e
n
the
skin
is
also
in
v
o
lv
e
d
.
T
o
xic
immunologic
dis-
order
,
usually
g
eneral-
ized
as
a
r
eaction
t
o
medications
(generally
an
antibio
tic);
lif
e-
threat
ening
!
Bland
ointment
therap
y
(suc
h
a
s
Bepanthen)
!
R
a
rely
cor
tisone
e
y
e
oint-
ment
!
Clean
conjunctiv
a
o
f
f
ibrin
d
a
ily
!
L
y
sis
of
symblepharon
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121. 103
T
o
xic
epidermal
necrolysis
(L
y
ell’
s
syndrome)
Hyper- acut
e
Generalized
blist
e
ring
and
shedding
or
necro
tic
skin,
mucous
membrane,
and
con-
junctivitis
Extremely
acut
e,
lif
e-
threat
ening
disorder
S
t
e
v
ens-Johnson
and
L
y
ell’
s
syn-
dromes
ha
v
e
a
similar
clinical
cour
se
and
similar
treatment
as
w
ell.
Ocular pemphigoid
Chronic
Chronic
bilat
eral
con-
junctivitis
per
sisting
f
o
r
y
ear
s;
leads
t
o
in
c
r
e
a
s
e
d
s
c
a
r
r
in
g
,
symblepharon,
and
increasingly
shallo
w
conjunctiv
al
f
o
rnix
that
ma
y
p
rogress
t
o
t
o
t
a
l
oblit
eration
of
the
con-
junctiv
al
sac
be
tw
een
the
bulbar
conjunctiv
a
and
the
palpebral
con-
junctiv
a.
A
u
t
oimmune
process
with
c
hronic
episodic
cour
se;
e
y
edrops
and
preser
v
ativ
es
used
in
them
e
x
acerbat
e
t
he
process.*
!
Sym
p
t
omatic:
Ar
tificial
t
ear
s
without
preser
v
ativ
es
–
T
opical
br
oad-spectrum
antibio
tics
in
case
of
bact
erial
superinf
ection
–
T
opical
st
er
oid
therap
y
relie
v
e
s
symp
t
oms.
No
t
e
:
this
increases
intraocular
pressure
(
risk
of
cat
aract)
–
S
y
s
t
e
m
ic
s
t
e
r
o
id
s
in
an
acut
e
episode
–
Immunosuppressiv
e
agents:
cy
closporin
A
4.4Conjunctivitis
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122. 104
ConjunctivalirritationsymptomscanoccurwithGraves’orbitopathy,
gout,rosacea,neurodermatitis,erythemamultiforme,Sjögren’ssyndrome,and
Reiter’ssyndrome(triad:conjunctivitisoriridocyclitis,urethritis,andpolyar-
thritis).Parinaud’soculoglandularsyndromedescribesaclinicalsyndrome
ofwidelyvariedetiology.Granulomatousconjunctivitisalwaysoccursuni-
laterallyandinconjunctionwithswollenpreauricularandsubmandibular
lymphnodesinthepresenceoftuberculosis,syphilis,viruses,bacteria,fungi,
andparasites.Theexcisionalbiopsyoftheconjunctivalgranulomaisitself
partofthetreatmentofgranulomatousconjunctivitis.Thespecificmedica-
tionswilldependontheunderlyingdisorder.
4.5 Tumors
Primarybenignconjunctivaltumors(nevi,dermoids,lymphangiomas,
hemangiomas,lipomas,andfibromas)occurfrequently,asdotumor-like
inflammatorychanges(viralpapillomas,granulomassuchassuture
granulomasaftersurgerytocorrectstrabismus,cysts,andlymphoidhyper-
plasia).Malignantconjunctivaltumors(carcinomasinsitu,carcinomas,
Kaposi’ssarcomas,lymphomas,andprimaryacquiredmelanosis)arerare.
Benignlesionsmaybecomemalignant;forexample,anevusoracquired
melanosismaydevelopintoamalignantmelanoma.Thissectionpresents
onlythemostimportanttumors.
4.5.1 EpibulbarDermoid
Epibulbardermoidisarounddome-shapedgrayishyelloworwhitishcon-
genitaltumor.Itisgenerallylocatedonthelimbusofthecornea,extending
intothecornealstromatoavaryingdepth.Epibulbardermoidscanoccuras
isolatedlesionsorasasymptomofoculoauriculovertebraldysplasia(Golden-
har’ssyndrome).Additionalsymptomsofthatdisorderincludeouterear
deformitiesandpreauricularappendages(Figs.4.19aandb).Dermoidscan
containhairandminorskinappendages.Ophthalmologistsareoftenaskedto
removethemforcosmeticreasons.Surgicalexcisionshouldremainstrictly
superficial;completeexcisionmayriskperforatingtheglobeasdermoids
oftenextendthroughtheentirewalloftheeyeball.
4.5.2ConjunctivalHemangioma
Conjunctivalhemangiomasaresmall,cavernousproliferationsofbloodves-
sels.Theyarecongenitalanomaliesandusuallyresolvespontaneouslybythe
ageofseven.Wherethisisnotthecase,theycanbesurgicallyremoved
(Fig.4.20).
4Conjunctiva
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123. 105
Epibulbardermoidinoculoauriculovertebraldysplasia
(Goldenhar’ssyndrome).
Fig.4.19
aEpibulbarder-
moidonthe
limbusofthe
cornea.
bAdditional
preauricular
appendages.
4.5.3EpithelialConjunctivalTumors
4.5.3.1ConjunctivalCysts
Conjunctivalcystsareharmlessandbenign.Occurrenceismostoftenpost-
operative(forexampleaftersurgerytocorrectstrabismus),post-traumatic,
orspontaneous.Theyusuallytaketheformofsmallclearfluid-filledinclu-
sionsofconjunctivalepitheliumwhosegobletcellssecreteintothecystand
notontothesurface(Fig.4.21).Cystscanleadtoaforeign-bodysensationand
areremovedsurgicallybymarsupialization(removaloftheupperhalfofthe
cyst).
4.5Tumors
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124. 106
Conjunctivalhemangioma.
Fig.4.20Small
cavernouspro-
liferationsof
bloodvesselson
theconjunctiva.
Conjunctivalcyst.
Fig.4.21Small,
clear,fluid-filled
inclusionsofcon-
junctival
epithelium.
4.5.3.2ConjunctivalPapilloma
Papillomasareofviralorigin(humanpapillomavirus)andmaydevelopfrom
thebulbarorpalpebralconjunctiva.Theyarebenignanddonotturnmalig-
nant.Asintheskin,conjunctivalpapillomascanoccurasbranchingpediculate
tumorsorasbroad-basedlesionsonthesurfaceoftheconjunctiva(Fig.4.22).
Papillomasproduceapermanentforeign-bodysensationthatisannoyingto
thepatient,andtheentirelesionshouldbesurgicallyremoved.
4Conjunctiva
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125. 107
Conjunctivalpapilloma.
Fig.4.22Broad-
basedpapilloma
originatingfrom
thesurfaceofthe
palpebralcon-
junctiva.
4.5.3.3ConjunctivalCarcinoma
Conjunctivalcarcinomasareusuallywhitish,raised,thickenedareasof
epithelialtissuewhosesurfaceformsaplateau.Theselesionsareusuallyker-
atinizingsquamouscellcarcinomasthatdevelopfromepithelialdysplasia
(precancer)andprogresstoacarcinomainsitu(Fig.4.23).Conjunctivalcarci-
nomasmustbeexcisedandacytologicdiagnosisobtained,andthepatient
mustundergopostoperativeradiationtherapytopreventgrowthdeepinto
theorbit.
Conjunctivalsquamouscellcarcinoma.
Fig.4.23Typi-
calfeaturesin-
cludethewhitish,
raised,thickened
areaofepithelial
tissue.
4.5Tumors
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126. 108
4.5.4MelanocyticConjunctivalTumors
4.5.4.1ConjunctivalNevus
Birthmarkscanoccurontheconjunctivaasontheskin.Theyareusually
locatednearthelimbusinthetemporalportionofthepalpebralfissure,less
frequentlyonthelacrimalcaruncle.Thesebenign,slightlyraisedepithelialor
subepithelialtumorsarecongenital.Fiftypercentofnevicontainhollowcys-
ticspaces(pseudocysts)consistingofconjunctivalepitheliumandgoblet
cells.Conjunctivalnevimaybepigmented(Fig.4.24a)orunpigmented
(Fig.4.24b),andtheymayincreaseinsizeasthepatientgrowsolder.Increas-
ingpigmentationofthenevusasaresultofhormonalchangesduringpreg-
nancyorpubertyorfromexposuretosunlightcansimulateanincreaseinthe
sizeofthenevus,ascanproliferationofthepseudocysts.Conjunctivalnevican
degenerateintoconjunctivalmelanomas(50%ofconjunctivalmelanomas
developfromanevus).Therefore,completeexcisionandhistologicdiagnostic
studiesareindicatedifthenevussignificantlyincreasesinsizeorshowssigns
ofinflammation.
Photographsshouldalwaysbetakenduringfollow-upexaminationsof
conjunctivalnevi.Smallclearwateryinclusioncystsarealwaysasignof
aconjunctivalnevus.
4.5.4.2ConjunctivalMelanosis
Definition
Conjunctivalmelanosisisapigmentedthickeningoftheconjunctivalepi-
thelium(Fig.4.24c).
Epidemiology:Conjunctivalmelanosisisrarelikeallpotentiallymalignant
ormalignanttumorsoftheconjunctiva.
Etiology:Unclear.
Symptoms:Acquiredconjunctivalmelanosisusuallyoccursaftertheageof
40.Typicalsymptomsincludeirregulardiffusepigmentationandthickening
oftheepitheliumthatmay“comeandgo.”
Diagnosticconsiderations:Acquiredconjunctivalmelanosisismobilewith
theconjunctiva(animportantcharacteristicthatdistinguishesitfromcon-
genitalmelanosis).Itrequirescloseobservationwithfollow-upexaminations
everysixmonthsasitcandevelopintoamalignantmelanoma.
Differentialdiagnosis:Thisdisordershouldbedistinguishedfrombenign
congenitalmelanosis(seebelow),whichremainsstablethroughoutthe
patient’slifetimeandappearsmorebluishgraythanbrownish.Incontrastto
acquiredmelanosis,itisnotmobilewiththeconjunctiva.
4Conjunctiva
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127. 109
Differentialdiagnosisofpigmentedconjunctivalchanges.
Figs.4.24a–j
aPigmentedcon-
junctivalnevus.
bUnpigmented
conjunctival
nevus.
cPrimary
acquiredmelano-
sis.
Continued!
4.5Tumors
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128. 110
Differentialdiagnosisofpigmentedconjunctivalchanges.
Fig.4.24dCon-
genitalmelanosis.
eMalignantcon-
junctival
melanoma.
fMalignant
melanomaofthe
ciliarybodypene-
tratingbeneath
theconjunctiva.
4Conjunctiva
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129. 111
Differentialdiagnosisofpigmentedconjunctivalchanges.
Fig.4.24g
Metallicforeign
bodythathas
healedwithinthe
conjunctiva.
hAdrenochrome
deposits(from
eyedropscontain-
ingepinephrine).
iIrondeposits
frommake-up
(mascara)
Continued!
4.5Tumors
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130. 112
Differentialdiagnosisofpigmentedconjunctivalchanges.
Fig.4.24j
Ochronosis
(alkaptonuria).
Treatment:Becausethedisorderoccursdiffuselyoverabroadarea,treat-
mentisoftendifficult.Usuallyitcombinesexcisionoftheprominentdeeply
pigmentedportions(forhistologicconfirmationofthediagnosis)withcryo-
coagulationoftheadjacentmelanosisandinsomecaseswithpostoperative
radiationtherapy.
Clinicalcourseandprognosis:About50%ofconjunctivalmelanomas
developfromconjunctivalmelanosis(theother50%developfromaconjunc-
tivalnevus;seeabove).Conjunctivalmelanomasarenotusuallyasaggres-
sivelymalignantasskinmelanomas.Theradicalresectionrequiredtoremove
thetumorcanbeaproblem.Multiplerecurrenceswillproducesignificant
conjunctivalscarringthatcanresultinsymblepharonwithfusionoftheeye-
lidskinandconjunctiva.Wherethetumorhasinvadedtheeyelidsorthe
deeperportionsoftheorbit,orbitalexenterationwillbeunavoidableto
completelyremovethetumor.
4.5.4.3CongenitalOcularMelanosis
Benigncongenitalmelanosis(Fig.4.24d)issubepithelialintheepisclera.The
conjunctivalepitheliumisnotinvolved.Pigmentationisbluishgray.Incon-
trasttoacquiredmelanosis,congenitalmelanosisremainsstableandstation-
arythroughoutthepatient’slifetime.Incontrasttoneviandacquiredmela-
nosis,congenitalmelanosisremainsstationarywhentheconjunctivaaboveit
ismovedwithforceps.Congenitalocularmelanosiscanoccurasanisolated
anomalyoftheeyeorinassociationwithskinpigmentations(oculodermal
melanosisorOta’snevus).Althoughthetumorisbenign,evidencesuggests
4Conjunctiva
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131. 113
thatmalignantmelanomasinthechoroidoccurmorefrequentlyinpatients
withcongenitalmelanosis.
4.5.5ConjunctivalLymphoma
Prominentareasofsalmon-coloredconjunctivalthickeningfrequentlyoccur-
ringintheinferiorfornix(Fig.4.25)areoftenthefirstsignoflymphaticdis-
ease.Identifyingthespecificformsanddegreeofmalignancyrequiresbiopsy
andhistologicexamination.Lesionsmayrangefrombenignlymphoidhyper-
plasiatomalignantlymphomasthataremoderatelytohighlymalignant.
Becauselymphomasrespondtoradiation,acombinationofradiationtherapy
andchemotherapyisusuallyprescribedaccordingtothespecifichistologic
findings.
4.5.6Kaposi’ssarcoma
Thisisaprominent,lighttodarkredtumorintheconjunctivalfornixorpro-
ceedingfromthepalpebralconjunctiva.Itconsistsofmalignantspindlecells
andnestsofatypicalendothelialcells.TodayKaposi’ssarcomasareseenmost
frequentlyasopportunisticdiseaseinpatientswithAIDS(AcquiredImmune
DeficiencySyndrome).Theophthalmologistcanmakeatentativediagnosis
ofAIDSonthebasisoftypicalclinicalsignsontheconjunctivaandorder
furtherdiagnosticstudies(Fig.4.26).Recentlytherehasbeenevidencethat
herpesvirus(HHV-8)isinvolvedinthedevelopmentofKaposi’ssarcoma.
Conjunctivallymphoma.
Fig.4.25Typi-
calsalmon-
coloredconjunc-
tivaltumorinthe
inferiorfornix.
4.5Tumors
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132. 114
4.6 ConjunctivalDeposits
Thesecanoccurinboththeconjunctivaandcornea.Some,likesometumors,
leadtopigmentedchangesintheconjunctiva.However,theirtypicalappear-
anceusuallyreadilydistinguishesthemfromtumors(Fig.4.24).Thefollow-
ingconjunctivalandcornealdepositsanddiscolorationsmayoccur:
Adrenochromedeposit(Fig.4.24h).Prolongeduseofepinephrineeyedrops
(asinglaucomatherapy)producesbrownishpigmentedchangesintheinfe-
riorconjunctivalfornixandonthecorneaasaresultofepinephrineoxidation
products(adrenochrome).Thiscansimulateamelanocyticconjunctival
tumor.Therefore,thephysicianshouldalwaysascertainwhetherthepatient
hasahistoryofprolongeduseofepinephrineeyedrops.Notherapyisindi-
cated.
Irondeposits(Fig.4.24i).Inwomen,irondepositsfromeyemake-upand
mascaraarefrequentlyseentoaccumulateintheconjunctivalsac.Notherapy
isindicated.
Argyrosis.Prolongedusedofsilver-containingeyedropscanproduce
brownishblacksilverdepositsintheconjunctiva.
Ochronosis(alkaptonuria:aninheritedautosomalrecessivedeficiencyofthe
enzymehomogentisate1,2-dioxygenase).Approximately70%ofallpatients
withochronosisexhibitbrownishpigmenteddepositsintheskinoftheeye-
lids,conjunctiva,sclera,andlimbusofthecornea(Fig.4.24j).Thedeposits
increasewithtime.Thedisordercannotbetreatedintheeye.
Kaposi’ssarcoma.
Fig.4.26Promi-
nentdarkred
tumorinthecon-
junctivalfornixin
apatientwith
AIDS.
4Conjunctiva
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133. 115
Metallicforeignbodiesintheconjunctiva.Ametallicforeignbodythatisnot
removedimmediatelywillhealintotheconjunctiva,whereitwillsimulatea
pigmentedchangeintheconjunctiva(Fig.4.24g).Obtainingameticuloushis-
tory(theexaminershouldalwaysenquireaboutoculartrauma)willquickly
revealthecauseoftheanomaly.Theforeignbodycanberemovedundertopi-
calanesthesia.
Jaundice.Thiswillleadtoyellowingoftheconjunctivaandsclera.
4.6ConjunctivalDeposits
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135. 117
5 Cornea
GerhardK.Lang
5.1 BasicKnowledge
Fundamentalimportanceofthecorneafortheeye:Thecorneaistheeye’s
opticalwindowthatmakesitpossibleforhumanstosee.Theophthalmolo-
gistisonlyabletodiscernstructuresintheinterioroftheeyebecausethecor-
neaistransparent.At43diopters,thecorneaisthemostimportantrefractive
mediumintheeye.
Shapeandlocation:Thecornea’scurvatureisgreaterthanthesclera’scurva-
ture.Itfitsintothescleralikeawatch-glasswithashallowsulcus(thelimbus
ofthecornea)markingthejunctionofthetwostructures.
Embryology:Thecornealtissueconsistsoffivelayers.Thecorneaandthe
scleraareformedduringthesecondmonthofembryonicdevelopment.The
epitheliumdevelopsfromectoderm,andthedeepercorneallayersdevelop
frommesenchyme.
Morphologyandhealing(Fig.5.1):
!Thesurfaceofthecorneaisformedbystratifiednonkeratinized
squamousepitheliumthatregeneratesquicklywheninjured.Withina
hour,epithelialdefectsareclosedbycellmigrationandrapidcelldivision.
However,thisassumesthatthelimbusstemcellsinthelimbusofthecor-
neaareundamaged.Regularcornealregenerationwillnolongerbe
possiblewhenthesecellsarecompromised.Anintactepitheliumprotects
againstinfection;adefectintheepitheliummakesiteasyforpathogensto
entertheeye.
!Athinbasementmembraneanchorsthebasalcellsofthestratified
squamousepitheliumtoBowman’slayer.Thislayerishighlyresistantbut
cannotregenerate.Asaresult,injuriestoBowman’slayerusuallyproduce
cornealscarring.
!BeneathBowman’slayer,manylamellaeofcollagenfibrilsformthecor-
nealstroma.Thestromaisahighlybradytrophictissue.Asavascular
tissue,itonlyregeneratesslowly.However,itsavascularitymakesitan
immunologicallyprivilegedsiteforgrafting.Routinecornealtransplants
maybeperformedwithoutpriortissuetyping.Anincreasedriskofrejec-
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136. 118
Anatomyofthecornea.
Fig.5.1Seediscussionintext.
tionneedonlybefearedwheretherecipient’scorneaishighlyvascularized
asmaybethecasefollowingchemicalinjuryorinflammation.Suchcases
requireeitheratissue-typeddonorgraftorimmunosuppressivetherapy
withcyclosporin.
!Descemet’smembraneandthecornealendotheliumlieontheposterior
surfaceofthecornealstromaadjacenttotheanteriorchamber.
Descemet’smembraneisarelativelystrongmembrane.Itwillcontinueto
definetheshapeoftheanteriorchamberevenwherethecornealstroma
hascompletelymelted(seeDescemetocele).Becauseitisagenuinebase-
mentmembrane,losttissueisregeneratedbyfunctionalendothelialcells.
Thecornealendotheliumisresponsibleforthetransparencyofthecornea
(seealsoTransparencybelow).Ahighdensityofepithelialcellsisneces-
sarytoachievethis.Thecornealendotheliumdoesnotregenerate;defects
intheendotheliumareclosedbycellenlargementandcellmigration.
Diameter:Thenormalaveragediameteroftheadultcorneais11.5mm
(10–13mm).Acongenitallysmallcornea(microcornea,diameterlessthan
10.0mm)oracongenitallylargecornea(megalocornea,diameterfrom13to
15mm)isalwaysanabnormalfinding(seeCornealSizeAnomalies).
5Cornea
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137. 119
Nourishment:Thefivelayersofthecorneahavefewcellsandareunstruc-
turedandavascular.Likethelens,sclera,andvitreousbody,thecorneaisa
bradytrophictissuestructure.Itsmetabolismisslow,whichmeansthatheal-
ingisslow.Thecorneaisnourishedwithnutritivemetabolites(aminoacids
andglucose)fromthreesources:
1.Diffusionfromthecapillariesatitsedge.
2.Diffusionfromtheaqueoushumor.
3.Diffusionfromthetearfilm.
Significanceofthetearfilmforthecornea:Thethree-layerprecornealtear
filmensuresthatthesurfaceofthecornearemainssmoothandhelpstonour-
ishthecornea(seeabove).Withoutatearfilm,thesurfaceoftheepithelium
wouldberough,andthepatientwouldseeablurredimage.Theenzyme
lysozymecontainedinthetearfilmalsoprotectstheeyeagainstinfection
(seep.50,forcompositionofthetearfilm).
Transparency:Thisisduetotwofactors.
1.Theuniformarrangementofthelamellaeofcollagenfibrilsinthecor-
nealstromaandthesmoothendothelialandepithelialsurfaceproduced
bytheintraocularpressure.
2.Thewatercontentofthecornealstromaremainsconstantat70%.The
combinedactionoftheepitheliumandendotheliummaintainsaconstant
watercontent;theepitheliumsealsthestromaofffromtheoutside,while
theendotheliumactsasanionpumptoremovewaterfromthestroma.
Thisrequiresasufficientlyhighdensityofendothelialcells.Endothelialcell
densityisage-dependent;normallyitisapproximately2500cellsper
mm
2
.Atcelldensitiesbelow300endothelialcellspermm
2
,the
endotheliumisnolongerabletopumpwateroutofthecornea,resultingin
edemaofthecornealstromaandendothelium.
Protectionandnervesupply:Thecorneaisavitalstructureoftheeyeandas
aresultextremelysensitive.Itreceivesitsamplesensorysupplyfromtheoph-
thalmicdivisionofthetrigeminalnerve.Theslightesttactilesensationcauses
aneyeclosingreflex.Anyinjurytothecornea(erosion,foreignbodypenetra-
tion,orultravioletkeratoconjunctivitis)exposessensorynerveendingsand
causesintensepainwithreflexivetearingandinvoluntaryeyeclosing.
Thetriadofinvoluntaryeyeclosing(blepharospasm),reflexivetearing
(epiphora),andpainalwayssuggestsapossiblecornealinjury(see
Chapter18).
5.1BasicKnowledge
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138. 120
5.2 ExaminationMethods
Non-ophthalmologistscanevaluatethetransparencyofthecornea(opaci-
tiesofthestromaandepitheliumsuggestscarringorinfiltrationofthe
epithelium),itssurfaceluster(lackoflustersuggestsanepithelialdefect),
andpossiblesuperficialcornealinjuries(seeFig.19.1).Asimplerulermaybe
usedtomeasurethesizeofthecornea(seeAnatomy),andsensitivitymay
betestedwithacottonswab(seeFig.1.11,p.11).
Theophthalmologistusesinstrumentstoevaluatecornealmorphology
andfunctioningreaterdetail.
5.2.1 SlitLampExamination
Theslitlampistheprimaryinstrumentusedinevaluatingthecornea.The
ophthalmologistchoosesbetweeneightandforty-powermagnificationfor
examiningalllevelsofthecorneawithanarrowbeamofcollimatedlight
(Fig.5.2).
5.2.2DyeExaminationoftheCornea
Defectsinthesurfaceofthecorneacanbevisualizedwithfluoresceinorrose
bengalsolution(ineithercase,administeronedropof1%solution).Since
thesedyesarenotusuallyabsorbedbytheepithelium,theymaybeusedto
visualizelossofepitheliumoverawidearea(suchascornealerosion)and
extremelyfinedefects(asinsuperficialpunctatekeratitis).Illumination
withacobaltbluefilterenhancesthefluorescenteffect.
Slitlampexaminationofthecornea.
Fig.5.2Theslit
lamp(slitaper-
ture)maybe
usedtoexamine
alllevelsofthe
corneawitha
narrowbeamof
collimatedlight.
5Cornea
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139. 121
Thesedyemethodscanrevealcornealepithelialdefects(cornealero-
sion)evenwithouttheuseofaslitlamp,whichishelpfulinexamining
infants.
5.2.3CornealTopography
Thekeratoscope(Placido’sdisk)permitsgrossevaluationoftheuniformity
ofthesurfaceofthecornea.Thisinstrumentconsistsofarounddiskmarked
withconcentricblackandwhiteringsaroundacentralaperture.The
examinerholdsthediskinhisorherhandandlooksthroughtheaperture.
Themirrorimagesoftheringsonthepatient’scorneaindicatethepresenceof
astigmatism(inwhichcasetheyappeardistorted).However,thisinexact
evaluationmethodlackstheprecisionrequiredformodernapplicationssuch
asrefractivesurgery.Therefore,thesurfaceofthecorneaisnownormally
evaluatedbycomputerizedcornealtopography(videokeratoscopy).Inthis
examination,thecontoursofthecorneaaremeasuredbyacomputerinthe
samemannerasthekeratoscope.Therefractivevaluesofspecificcorneal
regionsarethenrepresentedinacolor-codeddioptricmap.Brightred,for
example,representsasteepcurvaturewithahighrefractivepower.Thistech-
niqueprovidesacontourmapofthedistributionoftherefractivevaluesover
theentirecornea(Figs.5.3aandb).
5.2.4DeterminingCornealSensitivity
Non-ophthalmologistsmayperformasimplepreliminaryexaminationof
cornealsensitivitywithadistendedcottonswab(seeFig.1.11,p.11).This
examinationalsohelpstheophthalmologistconfirmthediagnosisinthe
presenceofasuspectedviralinfectionofthecorneaortrigeminalorfacial
neuropathyasthesedisordersareassociatedwithreducedcornealsensitiv-
ity.OphthalmologistsmayuseanautomaticDrägeresthesiometerforprecise
testingofcornealsensitivityandforfollow-upexaminations.Thisinstru-
mentcanincrementallyraisethesensitivitystimulus.Thismakesitpossible
todetermineifandhowrapidlycornealsensitivityincreasesfollowingacor-
nealtransplant.
5.2.5MeasuringtheDensityoftheCornealEpithelium
Asufficientlyhighdensityofendothelialcellsisveryimportantforthetrans-
parencyofthecornea(seeTransparency).Grossestimationoftheendothelial
celldensityispossibleforacircumscribedareaofthecorneausingaslitlamp
andindirectillumination.Boththeviewingaxisandilluminationaxisareoff-
setfromthevisualaxis.Precisequantificationandmorphologicevaluation
ofendothelialcellsoverlargeareasisonlypossiblebymeansofspecular
microscopy,atechniquedesignedespeciallyforthispurpose(Fig.5.4).Exact
5.2ExaminationMethods
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140. 122
Computerizedcornealtopography(videokeratoscopy).
Fig.5.3aRegularcornealastigmatisminanormalcornea.
5Cornea
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141. 123
Computerizedcornealtopography(videokeratoscopy).
Fig.5.3bIrregularcornealastigmatisminkeratoconus.ThePlacidodiskimageis
shownabove;therespectivecolormappingofrefractivevaluesindioptersisshown
below.
5.2ExaminationMethods
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142. 124
analysisisnecessarywhenthenumberofcellsappearsextremelylowunder
slitlampexaminationandthepatientisacandidateforcataractsurgery.If
exactanalysisthenverifiesthatthenumberofcellsisextremelylow(below
300–400cellspermm
2
),cataractsurgeryiscombinedwithacornealtrans-
plant.Thisisdonetoensurethatthepatientwillbeabletoseeevenaftercat-
aractsurgery,whichsacrificesadditionalendothelialcells.
5.2.6MeasuringtheDiameteroftheCornea
Anabnormallylargeorsmallcornea(megalocorneaormicrocornea)willbe
apparentfromsimplevisualinspection.Asuspectedsizeanomalycanbe
easilyverifiedbymeasuringthecorneawitharuler.Cornealdiametermay
bedeterminedmoreaccuratelywithcalipers(usuallydoneundergeneral
anesthesia,seeFig.10.21)orwiththeWesselykeratometer.Thisisatypeof
tubewithacondensinglenswithmillimetergraduationsatoneend.The
examinerplacesthisendonthepatient’seyeandlooksthroughtheother
end.
Megalocorneainaninfantalwaysrequiresfurtherdiagnosticinvestigation
todeterminewhetherbuphthalmosispresent.Microcorneamaybeasignof
congenitaldefectsinotheroculartissuesthatcouldresultinimpairedfunc-
tion(microphthalmos).
Automaticmeasurementofendothelialcelldensity.
Fig.5.4Specu-
larmicroscopy
permitsaprecise
endothelialcell
count(CD=2159
endothelialcells
permm
2
)while
simultaneously
measuringthe
thicknessofthe
cornea(pachy-
metry;
pachy=572µm).
5Cornea
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143. 125
5.2.7CornealPachymetry
Precisemeasurementofthethicknessofthecorneaiscrucialinrefractive
surgery(seeradialkeratotomyandcorrectionofastigmatism,p.155).
Improvingrefractionoftenrequiresmakingincisionsthrough90%ofthe
thicknessofthecorneawhilemeticulouslyavoidingfullpenetrationofthe
cornea.Therearetwopachymetrytechniquesformeasuringcornealthick-
nesswiththehighdegreeofprecisionthatthissurgeryrequires:
!Opticalpachymetrywithaslitlampandmeasuringattachmentmaybe
performedonthesittingpatient.
!Ultrasonicpachymetry;thishastheadvantageofgreaterprecisionand
canalsobeperformedwiththepatientsupine.
Recentdevelopmentsnowpermitpachymetrybymeansofspecularmicros-
copy(see5.2.8andFig.5.4).
5.2.8ConfocalCornealMicroscopy
Confocalcornealmicroscopyisarecentlydevelopedexaminationtechnique
thatmakesitpossibletoscanthecorneaoverawideareafromtheouterlayer
totheinnerlayer.Itdiffersinthisregardfromslitlampexamination,which
tendstobeafocalexaminationalongashaftoflightperpendiculartotheeye.
Confocalcornealmicroscopyvisualizescellstructuresatmaximummagnifi-
cationthatcannotbeobservedindetailwithaslitlamp.Theseincludecor-
nealnerves,amebas,andhyphae.Althoughnotyetroutinelyusedinclinical
practice,confocalcornealmicroscopyappearstobeapromisingexamination
methodforthefuture.
5.3 DevelopmentalAnomalies
5.3.1 ProtrusionAnomalies
5.3.1.1Keratoconus
Definition
Conical,usuallybilateralcentraldeformationofthecorneawithparenchymal
opacificationandthinningofthecornea.
Epidemiology:Keratoconusisthemostfrequentlyencountereddeformation
ofthecornea.Occurrenceisfamilial,althoughwomenaremorelikelytobe
affectedthanmen.
Etiology:Keratoconusisprobablyageneticdisorder.Itcanoccurinfamilies
withvaryingpathsofhereditarytransmission.Occasionallykeratoconusis
associatedwithtrisomy21syndrome(Downsyndrome)aswellaswith
5.3DevelopmentalAnomalies
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144. 126
atopicdermatitisandotherconnective-tissuedisorderssuchasMarfan’ssyn-
drome.
Symptoms:Theclinicalcourseofthedisorderisepisodic;theincreasingpro-
trusionofthecorneausuallyproducesbilateralirregularmyopicastigmatism
(seeFig.5.3b).Leftuntreated,inrarecaseskeratoconuscancausetearsof
Descemet’smembraneduetothecontinuousstretching.Theentirecornea
canthenbulgeoutatthissite.Thisisreferredtoasacutekeratoconus.Symp-
tomsofacutekeratoconusincludesuddenlossofvisualacuityaccompanied
byintensepain,photophobia,andincreasedtearing.
Diagnosticconsiderations:Thediagnosisisusuallymadewithakerato-
scopeorophthalmometer(refleximageswillbeirregular).Theexaminercan
alsodetectkeratoconuswithoutdiagnosticaidsbystandingbehindthe
patientandpullingthepatient’suppereyelidsdownward.Theconicalprotru-
sionofthesurfaceofthecornea(Fig.5.5)willthenbereadilyapparentdueto
thedeformationofthemarginoftheeyelid(Munson’ssign).
Treatment:Degenerationofvisualacuitycanusuallybecorrectedinitially
witheyeglasses;hardcontactlenseswillberequiredasthedisorderprog-
resses.However,afteracertainpoint,thepatientrepeatedlywilllosethecon-
tactlenses.Thentheonlypossibletreatmentispenetratingkeratoplasty
(transplantationofacornealgraftfromadonorintothepatient’scornea).
Prognosis:Theprognosisforpenetratingkeratoplastyintreatingkerato-
conusisgoodbecausethecorneaisavascularinkeratoconus.
Keratoconus.
Fig.5.5The
conicaldeforma-
tionofthecor-
neaisepisodic
andusuallypro-
ducesbilateralir-
regularmyopic
astigmatism(see
alsoFig.5.3b).
5Cornea
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145. 127
5.3.1.2Keratoglobus
Veryraredisordersincludekeratoglobus,acongenitaldeformationresulting
inhemisphericalprotrusion(Fig.5.6)thattendstoproducemyopia,andflat-
teningofthecornea(corneaplana)thattendstoproducehyperopia.
5.3.2CornealSizeAnomalies(MicrocorneaandMegalocornea)
Cornealsizeanomaliesareusuallycongenitalandonthewholearerare.An
abnormallysmallcornea(microcornea)hasadiameterlessthan10.0mm).It
usuallycausesseverehyperopiathatinadvancedageoftenpredisposesthe
patienttoangleclosureglaucoma(seeTable10.2,p.236).Anabnormally
largecornea(megalocornea)maybeaslargeas13–15mm.Cornealenlarge-
mentinthenewbornandinfantsmaybeacquiredduetoincreasedintraocu-
larpressure(buphthalmos).Combinationsofmicrocorneaandmegalocor-
neatogetherwithotheroculardeformitiesmayalsooccur.
5.4 InfectiousKeratitis
5.4.1 ProtectiveMechanismsoftheCornea
Aswasdiscussedabove,thecorneahascertaindefensivemechanisms
requiredbecauseofitsconstantexposuretomicrobesandenvironmentalin-
fluences.Themechanismsinclude:
!Reflexiveeyeclosing.
!Flushingeffectoftearfluid(lysozyme).
Keratoglobus.
Fig.5.6The
congenitaldefor-
mationresultsin
hemispherical
protrusionthat
canleadtomy-
opia.
5.4InfectiousKeratitis
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146. 128
!Itshydrophobicepitheliumformsadiffusionbarrier.
!Epitheliumcanregeneratequicklyandcompletely.
5.4.2CornealInfections:PredisposingFactors,Pathogens,and
Pathogenesis
Whencertainpathogenssucceedinbreachingthecornealdefensesthrough
superficialinjuriesorminorepithelialdefects,thebradytrophiccorneal
tissuewillrespondtothespecificpathogenwithcharacteristickeratitis.
Predisposingfactorsthatpromoteinflammationare:
!Blepharitis.
!Infectionoftheocularappendages(forexample,dacryostenosisaccom-
paniedbybacterialinfestationofthelacrimalsac).
!Changesinthecornealepithelialbarrier(bullouskeratopathyordryeyes).
!Contactlenses.
!Lagophthalmos.
!Neuroparalyticdisorders.
!Trauma.
!Topicalandsystemicimmunosuppressiveagents.
Pathogenscausingcornealinfectionsmayinclude:
!Viruses.
!Bacteria.
!Acanthamoeba.
!Fungi.
Pathogenesis:Oncethesepathogenshaveinvadedthebradytrophictissue
throughasuperficialcorneallesion,atypicalchainofeventswillensue:
!Corneallesion.
!Pathogensinvadeandcolonizethecornealstroma(redeye).
!Antibodieswillinfiltratethesite.
!Asaresult,thecorneawillopacifyandthepointofentrywillopenfurther,
revealingthecornealinfiltrate.
!Irritationoftheanteriorchamberwithhypopyon(typicallypuswill
accumulateontheflooroftheanteriorchamber;seeFig.5.7).
!Thepathogenswillinfesttheentirecornea.
!AsaresultthestromawillmeltdowntoDescemet’smembrane,whichis
relativelystrong.Thisisknownasadescemetocele;onlyDescemet’s
membraneisstillintact.Descemet’smembranewillbeseentoprotrude
anteriorlywhenexaminedunderaslitlamp.
!Asthedisorderprogresses,perforationofDescemet’smembraneoccurs
andtheaqueoushumorwillbeseentoleak.Thisisreferredtoasaper-
foratedcornealulcerandisanindicationforimmediatesurgicalinterven-
tion(emergencykeratoplasty;seep.152).Thepatientwillnoticepro-
gressivelossofvisionandtheeyewillbesoft.
5Cornea
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147. 129
Bacterialcornealulcer.
Fig.5.7aClini-
calfindingsin-
cludeacentral
bacterialcorneal
ulcerwithhy-
popyon.
bHistologicfindingsincludeGram-
positiverodbacteriainthecorneal
stroma.
!Prolapseoftheiris(theiriswillprolapseintothenewlycreateddefect)
closingthecornealperforationposteriorly.Adhesionoftheiriswillpro-
duceawhitecornealscar.
Thissequenceofeventscanvaryinspeedandseverity.Dependingonthe
voracityofthepathogensandthestateofthepatient’simmunesystem,an
infiltratecanformwithinafewhoursordaysandquicklyprogresstoacorneal
ulcer,meltingofthestroma,andevenadescemetocele.Thisrapidlyprog-
ressingformofinfectiouscornealulcer(usuallybacterial)isreferredtoasa
serpiginouscornealulcer.Itpenetratesthecorneaparticularlyrapidlyand
soonleadstointraocularinvolvement(thepathogenswillbeactivebeyond
5.4InfectiousKeratitis
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148. 130
thevisiblerimoftheulcer).Aserpiginouscornealulcerisoneofthemost
dangerousclinicalsyndromesasitcanrapidlyleadtolossoftheeye.
5.4.3GeneralNotesonDiagnosingInfectiousFormsofKeratitis
Promptdiagnosisandtreatmentofcornealinfectionsarecrucialinavoiding
permanentimpairmentofvision.Thediagnosisofanytypeofinfectiousker-
atitisessentiallyincludesthefollowingsteps:
!Identifyingthepathogenandtestingitsresistance.Thisisdonebytakinga
smearfromthebaseoftheulcertoobtainsamplematerialandinoculating
culturemediaforbacteriaandfungi.Wearersofcontactlensesshouldalso
haveculturestakenfromthelensestoensurethattheyarenotthesource
ofthebacteriaorfungus.
!Slidesofsmears,unstainedandtreatedwithGramandGiemsastains,are
examinedtodetectbacteria.
!Whereaviralinfectionissuspected,testingcornealsensitivityisindicated
asthiswillbediminishedinviralkeratitis.
5.4.4BacterialKeratitis
Epidemiology:Over90%ofallcornealinflammationsarecausedbybacteria.
Etiology:ThepathogenslistedinTable5.1areamongthemostfrequent
causesofbacterialkeratitisintheurbanpopulationintemperateclimates.
Table5.1Themostcommonbacterialpathogensthatcausekeratitis
Bacterium Typicalcharacteristicsofinfection
StaphylococcusaureusInfectionprogressesslowlywithlittlepain.
Staphylococcus
epidermidis
AsinStaphylococcusaureusinfection.
Streptococcus
pneumoniae
Typicalserpiginouscornealulcer:thecorneaisrapidly
perforatedwithearlyintraocularinvolvement;verypain-
ful.
PseudomonasaeruginosaBluishgreenmucoidexudate,occasionallywitharing-
shapedcornealabscess.Progressionisrapidwithaten-
dencytowardmeltingofthecorneaoverawidearea;
painful.
Moraxella Painlessovalulcerintheinferiorcorneathatprogresses
slowlywithslightirritationoftheanteriorchamber.
5Cornea
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149. 131
Mostbacteriaareunabletopenetratethecorneaaslongastheepi-
theliumremainsintact.Onlygonococcianddiphtheriabacteriacan
penetrateanintactcornealepithelium.
Symptoms:Patientsreportmoderatetoseverepain(exceptinMoraxella
infections;seeTable5.1),photophobia,impairedvision,tearing,andpurulent
discharge.Purulentdischargeistypicalofbacterialformsofkeratitis;viral
formsproduceawaterydischarge.
Diagnosticconsiderations:Positiveidentificationofthepathogensiscru-
cial.Serpiginouscornealulcersarefrequentlyassociatedwithseverereac-
tionoftheanteriorchamberincludingaccumulationofcellsandpusinthe
inferioranteriorchamber(hypopyon,Fig.5.7a)andposterioradhesionsof
theirisandlens(posteriorsynechia).
Differentialdiagnosis:Fungi(positiveidentificationofthepathogenis
requiredtoexcludeafungusinfection).
Treatment:
Becauseoftheriskofperforation,anytypeofcornealulcerisanemer-
gencyrequiringtreatmentbyanophthalmologist.
Conservativetherapy.Treatmentisinitiatedwithtopicalantibiotics(suchas
ofloxacinandpolymyxin)withaverybroadspectrumofactivityagainstmost
Gram-positiveandGram-negativeorganismsuntiltheresultsofpathogen
andresistancetestingareknown.Immobilizationoftheciliarybodyandiris
bytherapeuticmydriasisisindicatedinthepresenceofintraocularirritation
(manifestedbyhypopyon).Bacterialkeratitiscanbetreatedinitiallyonan
outpatientbasiswitheyedropsandointments.
Anadvancedulcer,i.e.,aprotractedclinicalcourse,suggestsindolenceand
poorcomplianceonthepartofthepatient.Hospitalizationisindicatedin
thesecases.Subconjunctivalapplicationofantibioticsmayberequiredto
increasetheeffectivenessofthetreatment.
Surgicaltreatment.Emergencykeratoplastyisindicatedtotreatadesceme-
toceleoraperforatedcornealulcer(seeemergencykeratoplasty,p.152).
Broadareasofsuperficialnecrosismayrequireaconjunctivalflaptoaccel-
eratehealing.Stenosisorblockageofthelowerlacrimalsystemthatmay
impairhealingoftheulcershouldbesurgicallycorrected.
Assoonastheresultsofbacteriologicandresistancetestingareavail-
able,thephysicianshouldverifythatthepathogenswillrespondtocur-
renttherapy.
Failureofkeratitistorespondtotreatmentmaybeduetooneofthefollow-
ingcauses,particularlyifthepathogenhasnotbeenpositivelyidentified.
5.4InfectiousKeratitis
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150. 132
1.Thepatientisnotapplyingtheantibiotic(poorcompliance).
2.Thepathogenisresistanttotheantibiotic.
3.Thekeratitisisnotcausedbybacteriabutbyoneofthefollowingpatho-
gens:
!Herpessimplexvirus.
!Fungi.
!Acanthamoeba.
!RarespecificpathogenssuchasNocardiaormycobacteria(astheseare
veryrare,theynotdiscussedinfurtherdetailinthischapter).
5.4.5ViralKeratitis
Viralkeratitisisfrequentlycausedby:
!Herpessimplexvirus.
!Varicella-zostervirus.
!Adenovirus.
Otherrarecausesincludecytomegalovirus,measlesvirus,orrubellavirus.
5.4.5.1HerpesSimplexKeratitis
Epidemiologyandpathogenesis:Herpessimplexkeratitisisamongthe
morecommoncausesofcornealulcer.About90%ofthepopulationarecar-
riersoftheherpessimplexvirus.Atypicalfeatureoftheubiquitousherpes
simplexvirusisanunnoticedprimaryinfectionthatoftenhealsspon-
taneously.Manypeoplethenremaincarriersoftheneurotropicvirus,which
canleadtorecurrentinfectionatanytimeproceedingfromthetrigeminal
ganglion.Acornealinfectionisalwaysarecurrence.Aprimaryherpessimplex
infectionoftheeyewillpresentasblepharitisorconjunctivitis.Recurrences
maybetriggeredexternalinfluences(suchasexposuretoultravioletlight),
stress,menstruation,generalizedimmunologicdeficiency,orfebrileinfec-
tions.
Symptoms:Herpessimplexkeratitisisusuallyverypainfulandassociated
withphotophobia,lacrimation,andswellingoftheeyelids.Visionmaybe
impaireddependingonthelocationoffindings,forexampleinthepresence
ofcentralepitheliitis.
Formsanddiagnosisofherpessimplexkeratitis:Thefollowingformsof
herpessimplexkeratitisaredifferentiatedaccordingtothespecificlayerof
thecorneainwhichthelesionislocated.Recurrencesaremorefrequentin
thestromaandendothelium.
Dendritickeratitis.Thisischaracterizedbybranchingepitheliallesions
(necroticandvesicularswollenepithelialcells,Fig.5.8).Thesefindingswill
bevisiblewiththeunaidedeyeafterapplicationoffluoresceindyeandare
5Cornea
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151. 133
characteristicofdendritickeratitis.Cornealsensitivityisusuallyreduced.
Dendritickeratitismayprogresstostromalkeratitis.
StromalKeratitis.Purelystromalinvolvementwithoutpriordendriticker-
atitisischaracterizedbyanintactepitheliumthatwillnotshowanydefects
afterapplicationoffluoresceindye.Slitlampexaminationwillrevealcentral
diskiformcornealinfiltrates(diskiformkeratitis)withorwithoutawhitish
stromalinfiltrate.Dependingonthefrequencyofrecurrence,superficialor
deepvascularizationmaybepresent.Reactionoftheanteriorchamberwill
usuallybeaccompaniedbyendothelialplaques(proteindepositsonthepos-
teriorsurfaceofthecorneathatincludephagocytizedgiantcells).
Endotheliitis.Endotheliitisorendothelialkeratitisiscausedbythepresence
ofherpesvirusesintheaqueoushumor.Thiscausesswellingofthe
endothelialcellsandopacificationoftheadjacentcornealstroma.Involve-
mentoftheendothelialcellsintheangleoftheanteriorchambercausesa
secondaryincreaseinintraocularpressure(secondaryglaucoma).Otherfind-
ingsincludeinflamedcellsandpigmentcellsintheanteriorchamber,and
endothelialplaques;involvementoftheiriswithsegmentallossofpig-
mentedepitheliumisdetectablebyslitlampexamination.
Acuteretinalnecrosissyndrome.Involvementoftheposterioreyeball(see
herpeticretinitis)forallpracticalpurposesisseenonlyinimmunocom-
promisedpatients(e.g.,recipientsofbonemarrowtransplantsandAIDS
patients).
Treatment:Infectionsinvolvingtheepitheliumaretreatedwithtrifluridine
asasuperficialvirostaticagent.Stromalandintraocularherpessimplex
Herpessimplexkeratitis:dendritickeratitis.
Fig.5.8Charac-
teristicfindings
includebranch-
ingepithelialle-
sions.
5.4InfectiousKeratitis
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152. 134
infectionscanbetreatedwithacyclovir,whichisavailablefortopicaluse(in
ointmentform)andsystemicuse.
Corticosteroidsarecontraindicatedinepithelialherpessimplexinfec-
tionsbutmaybeusedtotreatstromalkeratitiswheretheepitheliumis
intact.
5.4.5.2HerpesZosterKeratitis
Definition
Keratitisduetoendogenousrecurrenceofchickenpox(causedbythevaricella-
zostervirus;seeherpeszosterophthalmicus).
Etiology:Proceedingfromthetrigeminalganglion,thevirusreinfectsthe
regionsuppliedbythetrigeminalnerve.Theeyeisonlyaffectedwherethe
ophthalmicdivisionofthetrigeminalnerveisinvolved.Inthiscase,thenaso-
ciliarynervesupplyingtheinterioroftheeyewillalsobeaffected.Hutchin-
son’ssign,vesicularlesionsonthetipofthenose,willbepresent(seeFig.2.14).
Diagnosticconsiderations:Herpeszosterophthalmicusalsooccursin
superficialanddeepforms,whichinpartaresimilartoherpessimplexinfec-
tionofthecornea(redeyewithdendritickeratitis,stromalkeratitis,andker-
atouveitis).Cornealsensitivityisusuallydecreasedorabsent.
Treatment:Theeyeistreatedwithacyclovirointmentinconsultationwitha
dermatologist,whowillusuallytreatskinchangeswithsystemicacyclovir(in
theformofinfusionsortablets).Ifthecornealepitheliumisintact,theirrita-
tionoftheanteriorchambercanbecarefullytreatedwithsteroidsandimmo-
bilizationofthepupilandciliarybodybytherapeuticmydriasis.
5.4.6MycoticKeratitis
Epidemiology:Mycotickeratitiswasonceveryrare,occurringalmostexclu-
sivelyinfarmlaborers(seeEtiologyforcontactwithpossiblecausative
agents).However,thisclinicalsyndromehasbecomefarmoreprevalent
todayasaresultoftheincreasedandoftenunwarranteduseofantibioticsand
steroids.
Etiology:ThemostfrequentlyencounteredpathogensareAspergillusand
Candidaalbicans.Themostfrequentcausativemechanismisaninjurywith
fungus-infestedorganicmaterialssuchasatreebranch.
Symptoms:Patientsusuallyhaveonlyslightsymptoms.
Diagnosticconsiderations:Theredeyeisapparentuponinspection(nor-
mallythedisorderisunilateral),asisacornealulcerwithanundermined
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153. 135
margin(Fig.5.9).Theulcerwillcontinuetoexpandbeneaththevisiblemar-
gins(serpiginouscornealulcer).Hypopyonmayalsobepresent(asshownin
Fig.5.9a).Slitlampexaminationwillrevealtypicalwhitishstromalinfil-
trates,especiallywithmycotickeratitisduetoCandidaalbicans.Theinfil-
tratesandulcerspreadveryslowly.Satellitelesions,severaladjacentsmaller
infiltratesgroupedaroundalargercenter,arecharacteristicbutwillnotnec-
essarilybepresent.
Identificationofthepathogen.Microbiologicalidentificationoffungiisdiffi-
cultandcanbetimeconsuming(forhistologicidentification,seeFig.5.9b).Itis
importanttoobtainsamplesfrombeyondthevisiblemarginoftheulcer.Fun-
galculturesshouldalwaysbeobtainedwherebacterialculturesarenegative.
Mycotickeratitis.
Fig.5.9aClini-
calfindingsin-
cludeacorneal
ulcerthatex-
tendsbeyondthe
visiblemargin
andhypopyon.
bHistologicfindingsincludehyphaein
thecornealstroma.
5.4InfectiousKeratitis
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154. 136
Treatment:
Conservativetreatment.Hospitalizationisrecommendedwhenbeginning
treatmentasthedisorderrequiresprotractedtherapy.Systemictherapyis
onlyindicatedinthecaseofanintraocularinvolvement.Othercaseswill
respondwelltotopicaltreatmentwithantimycoticagentssuchasnatamycin,
nystatin,andamphotericinB.Ingeneral,thetopicalantimycoticagentswill
havetobespeciallypreparedbythepharmacist.
Surgicaltreatment.Emergencykeratoplasty(seep.152)isindicatedwhen
thedisorderfailstorespondorrespondstooslowlytoconservativetreatment
andfindingsworsenundertreatment.
5.4.7AcanthamoebaKeratitis
Epidemiology:Thisisararetypeofkeratitisandonewhichmayhavebeen
diagnosedtoorarelyinthepast.
Etiology:Acanthamoebaisasaprophyticprotozoon.Infectionsusuallyoccur
inwearersofcontactlenses,particularlyinconjunctionwithtraumaand
moistenvironmentssuchassaunas.
Symptoms:Patientscomplainofintensepain,photophobia,andlacrimation.
Diagnosticconsiderations:Thepatientwilloftenhaveahistoryofseveral
weeksormonthsofunsuccessfulantibiotictreatment.
Inspectionwillrevealaunilateralreddeningoftheeye.Usuallytherewill
benodischarge.Theinfectioncanpresentasasubepithelialinfiltrate,asan
intrastromaldisciformopacificationofthecornea,orasaring-shapedcor-
nealabscess(Fig.5.10a).
Thedisorderisdifficulttodiagnose,andevenimmunofluorescencestud-
iesinspecializedlaboratoriesoftenfailtoprovidediagnosticinformation.
Amebiccystscanbereadilydemonstratedonlybyhistologicandpathologic
studiesofexcisedcornealtissue(Fig.5.10b).Recentlyithasbecomepossible
todemonstrateamebiccystswiththeaidofconfocalcornealmicroscopy
(seep.125).Patientswhowearcontactlensesshouldhavethemsentinfor
laboratoryexamination.
Treatment:
Conservativetreatment.Topicalagentscurrentlyincludepropamidine(only
availablethroughinternationalpharmaciesasProlene)andpentamidine,
whichmustbepreparedbyapharmacist.Usuallybroad-spectrumantibiotic
eyedropsarealsoadministered.Cycloplegia(immobilizationofthepupiland
ciliarybody)isusuallyrequiredaswell.
Surgicaltreatment.Emergencykeratoplasty(seep.152)isindicatedwhen
conservativetreatmentfails.
5Cornea
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155. 137
Acanthamoebakeratitis.
Fig.5.10aClini-
calfindingsin-
cludekeratitis
witharing-
shapedcorneal
abscess.
bHistologicfindingsafterkerato-
plastyincludetypicaldouble-walled
amebiccystsinthecornealstroma
aboveDescemet’smembrane(arrow).
5.5 NoninfectiousKeratitisandKeratopathy
Thiscategoryencompassesawidevarietyofcornealdisorders,someof
which,suchaskeratoconjunctivitissicca,occurveryfrequently.Causes
include:
!Inflammations(blepharitisandconjunctivitis).
!Injuries(rubbingtheeyes,foreignbodiesbeneaththeuppereyelid,con-
tactlensincompatibility,exposuretointenseultravioletirradiation).
!Age-relatedchanges(senileectropionwithtrichiasis;spasticentropion;
keratoconjunctivitissicca).
!Surgery(cataractorglaucoma).
5.5NoninfectiousKeratitisandKeratopathy
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156. 138
!Endogenousfactors(facialneuropathy).
!Exogenousfactors(medicationsorpreservatives).
5.5.1 SuperficialPunctateKeratitis
Definition
Superficialpunctatecorneallesionsduetolacrimalsystemdysfunctionfroma
numbercauses(seeetiology).
Epidemiologyandetiology:Superficialpunctatekeratoconjunctivitisisa
veryfrequentfindingasitcanbecausedbyawidevarietyofexogenousfactors
suchasforeignbodiesbeneaththeuppereyelid,contactlenses,smog,etc.It
mayalsoappearasasecondarysymptomofmanyotherformsofkeratitis
(seetheformsofkeratitisdiscussedinthefollowingsection).Itcanalsooccur
inassociationwithanendogenousdisordersuchasThygeson’sdisease.
Symptoms:Dependingonthecauseandseverityofthesuperficialcorneal
lesions,symptomsrangefromanearlyasymptomaticclinicalcourse(suchas
inneuroparalytickeratitisinwhichthecornealosesitssensitivity)toan
intenseforeignbodysensationinwhichthepatienthasasensationofsandin
theeyewithtypicalsignsofepiphora,severepain,burning,andblepharo-
spasm.Visualacuityisusuallyonlyminimallycompromised.
Diagnosticconsiderationsanddifferentialdiagnosis:Fluoresceindyeis
appliedandtheeyeisexaminedunderaslitlamp.Thisvisualizesfine
epithelialdefects.Thespecificdyepatternsthatemergegivetheophthalmol-
ogistinformationabouttheetiologyofthepunctatekeratitis(Figs.5.11a–i).
Treatmentandprognosis:Dependingonthecause,thesuperficialcorneal
changeswillrespondrapidlyorlesssototreatmentwithartificialtears,
wherebyeveryeffortshouldbemadetoeliminatethecausativeagents(Fig.
5.11).Dependingontheseverityoffindings,artificialtearsofvaryingviscos-
ity(rangingfromeyedropstohigh-viscositygels)areprescribedandapplied
withvaryingfrequency.Inexposurekeratitis,ahigh-viscositygelorointment
isusedbecauseofitslongretentiontime;superficialpunctatekeratitisis
treatedwitheyedrops.
KeratoconjunctivitisSicca
Thisisoneofthemostfrequentcausesofsuperficialkeratitis.Thesyndrome
itselfisattributabletodryeyesduetolackoftearfluidandisdiscussedin
Chapter3.
5Cornea
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157. 139
Superficialpunctatekeratitis.
Superficial punctate
keratitis
a b c
d e f
g h i
Filamentary keratitis Entropion
Thygeson's disease Contact lens lesions Foreign bodies beneath
the upper eyelid
Exposure to intense
ultraviolet irradiation
Toxic conjunctivitis Exposure or ectropion
Fig.5.11Typicaldyepatternsinthevariousformsofsuperficialpunctatekeratitis.
Thecauseofthedisordercanbeinferredfromthespecificpatternofcorneallesions.
5.5NoninfectiousKeratitisandKeratopathy
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158. 140
5.5.2ExposureKeratitis
Definition
Keratitisresultingfromdryingofthecorneainthecaseoflagophthalmos.
Epidemiology:Exposurekeratitisisarelativelyfrequentclinicalsyndrome.
Forexample,itmayoccurinassociationwithfacialparalysisfollowinga
stroke.
Etiology:Duetofacialnervepalsy,thereisinsufficientclosureoftheeyelids
overtheeyeball(lagophthalmos),andtheinferiorthirdtohalfofthecornea
remainsexposedandunprotected(exposurekeratitis).Superficialpunctate
keratitis(seeabove)initiallydevelopsinthisregionandcanprogresstocor-
nealerosion(seeFig.18.5)orulcer.
Othercausesforexposurekeratitiswithoutfacialnervepalsyinclude:
!UncompensatedexophthalmosinGraves’disease.
!Insufficienteyelidclosurefollowingeyelidsurgerytocorrectptosis.
!Insufficienteyecareinpatientsreceivingartificialrespirationonthe
intensivecareward.
Symptoms:Similartosuperficialpunctatekeratitis(althoughusuallymore
severe)butunilateral.
Diagnosticconsiderations:Applicationoffluoresceindyewillrevealatypi-
calpatternofepitheliallesions(Fig.5.11i).
Treatment:Applicationofartificialtearsisusuallynotsufficientwhereeye-
lidmotorfunctionisimpaired.Insuchcases,high-viscositygels,ointment
packings(forantibioticprotection),andawatchglassbandagearerequired.
Thewatchglassbandagemustbeappliedsoastocreateamoistairtight
chamberthatpreventsfurtherdesiccationoftheeye(seeFig.2.9).Inthepres-
enceofpersistentfacialnervepalsythatshowsnosignsofremission,lateral
tarsorrhaphyisthetreatmentofchoice.Thesameappliestotreatmentof
exposurekeratitisduetoinsufficienteyelidclosurefromothercauses(see
Etiology).
Poorcornealcareinexposurekeratitiscanleadtosuperficialpunctate
keratitis,erosion,bacterialsuperinfectionwithcornealulcer,andfinally
tocornealperforation.
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159. 141
5.5.3NeuroparalyticKeratitis
Definition
Keratitisassociatedwithpalsyoftheophthalmicdivisionofthetrigeminal
nerve.
Epidemiology:Palsyoftheophthalmicdivisionofthetrigeminalnerveisless
frequentthatfacialnervepalsy.
Etiology:Thetrigeminalnerveisresponsibleforthecornea’ssensitivityto
exogenousinfluences.Aconductiondisturbanceinthetrigeminalnerveis
usuallyasequelaofdamagetothetrigeminalganglionfromtrauma,radiation
therapyofanacousticneurinoma,orsurgery.Itwillleadtolossofcornealsen-
sitivity.Asaresultofthislossofsensitivity,thepatientwillnotfeelanysensa-
tionofdryingintheeye,andtheblinkingfrequencydropsbelowthelevel
requiredtoensurethatthecornearemainsmoist.Asinexposurekeratitis,
superficialpunctatelesionswillforminitially,followedbylargerepithelial
defectsthatcanprogresstoacornealulcerifbacterialsuperinfectionoccurs.
Symptoms:Becausepatientswithlossoftrigeminalfunctionarefreeofpain,
theywillexperienceonlyslightsymptomssuchasaforeignbodysensationor
aneyelidswelling.
Diagnosticconsiderations:Cornealdamage,usuallycentralorslightly
belowthecenterofthecornea,mayrangefromsuperficialpunctatekeratitis
(visibleafterapplicationoffluoresceindye)toadeepcornealulcerwithper-
foration.Theeyewillberedandinextremecasesmaybeleakingaqueous
humor.
Differentialdiagnosis:Cornealulcerduetoherpesvirusinfection.
Treatment:Thisisessentiallyidenticaltotreatmentofexposurekeratitis.It
includesmoisteningthecornea,antibioticprotectionasprophylaxisagainst
infection,and,ifconservativemethodsareunsuccessful,tarsorrhaphy.
PrimaryandRecurrentCornealErosion
Thesechangesaregenerallytheresultofacornealtraumaandaredealtwith
inthechapteronoculartrauma.
5.5.4ProblemswithContactLenses
Etiology:Theseproblemsoccureitherwithpoorlyseatedrigidcontactlenses
thatrubonthesurfaceofthecorneaorfromoverwearingsoftcontactlenses.
5.5NoninfectiousKeratitisandKeratopathy
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160. 142
Ifcontactlensesarewornforextendedperiodsoftimedespitesymptoms,
severeinflammation,cornealulceration,andvascularizationofthecorneal
peripherymayresult.
Symptoms:Patientsfindthecontactlensesincreasinglyuncomfortableand
noticeworseningoftheirvision.Thesesymptomsareespeciallypronounced
afterremovingthecontactlensesasthelensesmaskthedefectinthecorneal
epithelium.
Diagnosticconsiderations:Theophthalmologistwilldetecttypicalcorneal
changesafterapplyingfluoresceindye(Fig.5.11e).Keratoconjunctivitison
thesuperiorlimbuswithformationofgiantpapillae,wart-likeprotrusionsof
connectivetissuefrequentlyobservedonthesuperiortarsus(Fig.5.12),are
signsofcontactlensorpreservativeincompatibility.
Treatment:Thepatientshouldtemporarilydiscontinuewearingthecontact
lenses,andinflammatorychangesshouldbecontrolledwithsteroidsuntil
theirritationoftheeyehasabated.
Protractedtherapywithtopicalsteroidsshouldbemonitoredregularly
byanophthalmologistassuperficialepithelialdefectshealpoorly
understeroidtherapy.Protractedhigh-dosagesteroidtherapycausesa
secondaryincreaseinintraocularpressureandcataractinone-thirdof
allpatients.
Thespecificophthalmologicfindingswilldeterminewhetherthepatient
shouldbeadvisedtopermanentlydiscontinuewearingcontactlensesor
whetherchangingcontactlensesandcleaningagentswillbesufficient.
Giantpapillaefromcontactlensincompatibility.
Fig.5.12Wart-
likeprotrusions
ofconnective
tissueonthepal-
pebralconjunc-
tivaduetocon-
tactlensorpre-
servativeincom-
patibility(with
simpleeversion
oftheuppereye-
lid).
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161. 143
5.5.5BullousKeratopathy
Definition
Opacificationofthecorneawithepithelialbullaeduetolossoffunctionofthe
endothelialcells.
Epidemiology:Bullouskeratopathyisamongthemostfrequentindications
forcornealtransplants.
Etiology:Thetransparencyofthecornealargelydependsonafunctioning
endotheliumwithahighdensityofendothelialcells(seeTransparency).
Wheretheendotheliumhasbeenseverelydamagedbyinflammation,
trauma,ormajorsurgeryintheanterioreye,thefewremainingendothelial
cellswillbeunabletopreventaqueoushumorfromenteringthecornea.This
resultsinhydrationofthecorneawithstromaledemaandepithelialbullae
(seeFigs.5.13aandb).Lossofendothelialcellsmayalsohavegeneticcauses
(seeFuchs’endothelialdystrophy).
Symptoms:Thegraduallossofendothelialcellscausesslowdeteriorationof
vision.Thepatienttypicallywillhavepoorervisioninthemorningthaninthe
evening,ascornealswellingisgreaterduringthenightwiththeeyelids
closed.
Diagnosticconsiderations:Slitlampexaminationwillrevealthickeningof
thecornea,epithelialedema,andepithelialbullae.
Differentialdiagnosis:Bullouskeratopathycanalsooccurwithglaucoma.
However,inthesecasestheintraocularpressureistypicallyincreased.
Treatment:Wherethedamagetotheendothelialcellsisnottoofaradvanced
andonlyoccasionalperiodsofopacificationoccur(suchasinthemorning),
hyperosmolarsolutionssuchas5%Adsorbonaccanimprovethepatient’seye-
sightbyremovingwater.However,thisisgenerallyonlyatemporarysolu-
tion.Beyondacertainstageacornealtransplant(penetratingkeratoplasty;
seep.152)isindicated.
5.5NoninfectiousKeratitisandKeratopathy
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162. 144
Bullouskeratopathy.
Fig.5.13
aCornealedema
duetoalackof
endothelialcells.
bImageobtained
byspecularmi-
croscopyshows
destructionof
theendothelial
cells(rightsideof
image).Incom-
parison,theleft
side(awide-
angleview)and
themiddle(mag-
nifiedview)of
theimageshow
anintacten-
dotheliumwitha
clearlyvisible
honeycomb
structure.Theac-
tualsizeofthe
areashownon
theleftsideof
theimageis
about0.5mm
2
.
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163. 145
5.6 CornealDeposits,Degenerations,andDystrophies
Asbradytrophicavasculartissue,thecorneaisparticularlysusceptibleto
depositsofforeignmaterialanddegeneration(see5.6.2).
5.6.1 CornealDeposits
5.6.1.1ArcusSenilis
Thisisagrayish-whitering-shapedfattydepositnearthelimbusthatcan
occuratanyagebutusuallyappearsinadvancedage(Fig.5.14).Arcussenilis
isusuallybilateralandisafrequentlyencounteredphenomenon.Itoccursasa
resultoflipiddepositsfromthevesselsofthelimbusalongtheentireperiph-
eryofthecornea,whichnormallyincreasewithadvancedage.Alipid-free
clearzonealongthelimbuswillbediscernible.Patientsyoungerthan50
yearswhodeveloparcussenilisshouldbeexaminedtoexcludehyper-
cholesteremiaasacause.Arcussenilisrequiresnotreatmentasitdoesnot
causeanyvisualimpairments.
Thedepositsandpigmentationsdiscussedinthefollowingsectiondonot
generallyimpairvision.
5.6.1.2CornealVerticillata
Bilateralgrayorbrownishepithelialdepositsthatextendinaswirlingpattern
fromapointinferiortothepupil.Thiscornealchangetypicallyoccurswith
theuseofcertainmedications,mostfrequentlywithchloroquineandamio-
Arcussenilis.
Fig.5.14Typical
grayish-white,
ring-shapedfatty
depositsnearthe
limbus.
5.6CornealDeposits,Degenerations,andDystrophies
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164. 146
darone.Fabry’sdisease(glycolipidlipidosis)canalsoexhibitthesekindsof
cornealchanges,whichcanhelptoconfirmthediagnosis.
5.6.1.3ArgyrosisandChrysiasis
Topicalmedicationscontainingsilverandhabitualexposuretosilverinelec-
troplatingoccupationsleadtosilverdepositsintheconjunctivaandthedeep
layersofthecornea(argyrosis).Systemicgoldtherapy(morethan1–2g)will
leadtogoldcolorationoftheperipheralcornealstroma(chrysiasis).
5.6.1.4IronLines
Anyirregularityinthesurfaceofthecorneacausestheeyelidtodistributethe
tearfilmirregularlyoverthesurfaceofthecornea;asmallpuddleoftearfluid
willbepresentatthesiteoftheirregularity.Irondepositsforminacharac-
teristicmanneratthissiteinthecornealepithelium.Themostfrequently
observedironlinesarethephysiologicirondepositsatthesitewherethe
eyelidsclose(theHudson-Stähliline),Stocker-Busaccalinewithpterygium,
Ferry’slinewithafilteringblebafterglaucomasurgery,andFleischerring
withkeratoconus.Ironlineshavealsobeendescribedfollowingsurgery
(radialkeratotomy;photorefractivekeratectomy;keratoplasty)andinthe
presenceofcornealscars.
5.6.1.5Kayser-FleischerRing
Thisgoldenbrowntoyellowishgreencornealringiscausedbycopper
depositsatthelevelofDescemet’smembraneinWilson’sdisease(liverand
lensdegenerationwithdecreasedserumlevelsofceruloplasmin).Thisringis
socharacteristicthattheophthalmologistoftenisthefirsttodiagnosethis
rareclinicalsyndrome.
5.6.2CornealDegeneration
5.6.2.1CalcificBandKeratopathy
Aftermanyyearsofchronicinflammationoftheanteriorchamber(chronic
uveitisandkeratitis)withshrinkageoftheeyeballorinpatientswithjuvenile
polyarthritis,calcificdepositsoccurinBowman’slayer,causingatransverse
zoneofopacificationintheregionofthepalpebralfissure.Thelimbus
regionwillremainclear(Fig.5.15).Thischangesignificantlyimpairsvision.
Theopacificationcanbecompletelyremovedandvisionrestoredbychelat-
ingthecalcificationswithasodiumEDTAsolution.
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165. 147
5.6.2.2PeripheralFurrowKeratitis
Thisincludesaheterogeneousgroupofdisordersintermsofmorphologyand
etiology.Allarenoninfectiousandleadtothinningandmeltingofthe
peripheralcorneathatmayprogresstoperforation.Etiologicfactorsinclude:
!Autoimmuneprocesses(collagenosis,marginalkeratitis,andscleroker-
atitis).
!Trophicdysfunctions(pittingduetolackoftearfilm).
!Unknowndegenerativeprocesses(Terrien’smarginaldegenerationor
Mooren’sulcer).
Calcificbandkeratopathy.
Fig.5.15
aBrownish-white
calcificdeposits
occurinBow-
man’slayer,
severelyimpair-
ingthepatient’s
vision.
bFindingsafter
chelationofthe
calcificdeposits
withanEDTAso-
lution.
5.6CornealDeposits,Degenerations,andDystrophies
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166. 148
Thesecornealchangesaremostfrequentlyobservedinpatientswithrheuma-
toidarthritis.Treatingtheunderlyingdisorderisessentialinthesecases.
Otherwisethechangesarerare.Keratomalaciaisaspecialformofthedis-
orderinwhichvitaminAdeficiencycausesxerosisoftheconjunctivacom-
binedwithnightblindness.Thisdisorderremainsoneofthemostfrequent
causesofblindnessinthedevelopingcountriesinwhichmalnutritionisprev-
alent.
5.6.3CornealDystrophies
Definition
Thistermreferstoagroupofcornealmetabolicdysfunctionsthatalwayslead
tobilateralopacificationofthevariouslayersofthecornea(seeClassification
below).
Epidemiology:Cornealdystrophytendstoberare.Themostfrequentformis
Fuchs’endothelialdystrophy,followedbydystrophyinthecornealstroma.
Etiology:Thevariouscornealdystrophiesaregeneticdisorders.Theyusually
manifestthemselvesinthefirstorseconddecadeoflifeexceptforFuchs’
endothelialdystrophy,whichonlybecomessymptomaticbetweentheages
of40and50.
Classification:Thefollowingformsofdystrophyaredifferentiatedaccording
totheindividuallayersofthecorneainwhichtheyoccur:
!Epithelialcornealdystrophy.
!Stromalcornealdystrophy.Themostprevalentformsinclude:
–Granulardystrophy(hyalinedeposits,Fig.5.16).
–Latticedystrophy(amyloiddeposits).
–Maculardystrophy(depositsofacidicmucopolysaccharides,Fig.5.17).
!Endothelialdystrophy,suchas:
-Fuchs’endothelialdystrophy(themostfrequentlyencounteredformof
cornealdystrophy).
Symptomsanddiagnosticconsiderations:Allpatientssufferfroma
steadilyincreasinglossofvisualacuityduetothegenerallygradualopacifica-
tionofthecornea.Thislossofvisualacuitymayprogresstothepointwherea
cornealtransplantbecomesnecessary.
Maculardystrophyisthemostrapidlydebilitatingformofthestromaldys-
trophies,resultinginaseverelossofvisualacuityintheseconddecadeoflife.
Epithelialandstromalcornealdystrophiesarealsooftenaccompaniedby
painfulandrecurrentcornealerosion.Fuchs’endothelialdystrophyinvolves
agraduallossofendothelialcellsthatintimeleadstobullouskeratopathy
(hydrationofthecorneawithstromaledemaandepithelialbullae).The
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167. 149
Granularstromalcornealdystrophy.
Fig.5.16aClini-
calfindingsin-
cludefragmented
opacitiessur-
roundedbyareas
ofclearcornea
betweenthe
deposits.
bHistologicfindingsdemonstrating
hyalinedepositsunderMasson’s
trichromestain.
patienttypicallywillhavepoorervisioninthemorningthanintheevening,
ascornealswellingisgreaterduringthenightwiththeeyelidsclosed.
Treatment:Dependingontheseverityofthelossofvisualacuity(seeabove),
acornealtransplant(penetratingkeratoplasty;seep.152)maybeindicated.
Becausethecornearemainsavascularinthesedisorders,theprognosisis
good.
InFuchs’endothelialdystrophy,acornealtransplantisthetreatmentof
choice.Wherethesymptomsarenottoofaradvanced,frequentapplicationof
hyperosmolarsolutionscanremovewaterfromthecornea.However,thisis
generallyonlyatemporarysolution.Thecornealtransplantisperformedin
combinationwithacataractextraction;patientswithFuchs’endothelialdys-
5.6CornealDeposits,Degenerations,andDystrophies
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168. 150
Macularstromalcornealdystrophy.
Fig.5.17aClini-
calfindingsin-
cludenodular
opacitiessur-
roundedbyareas
ofclearcornea
betweenthe
deposits.
bHistologicfindingsdemonstrating
depositsofacidicmucopolysac-
charidesunderAMPstain.
trophythataffectstheirvisionareusuallyolderandalsohaveacataract.The
twoproceduresarecombinedbecausecornealdecompensationoftenresults
fromFuchs’endothelialdystrophyfollowingthesurgicaltraumaofcataract
extraction(see5.2.5).
5.7 CornealSurgery
Cornealsurgeryincludescurativeortherapeuticproceduresandrefractive
procedures(Fig.5.18).
!Curativecornealproceduresareintendedtoimprovevisionbyeliminating
cornealopacification.
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169. 151
Therapeuticandrefractivecornealprocedures.
46.OD
42.5D
43.5D
42.5D
Therapeutic procedures
Refractive procedures
Epikeratophakia
Excimer laser in situ keratomileusis (LASIK)
a b
Penetrating keratoplasty Lamellar keratoplasty
Phototherapeutic keratectomy
c
Photorefractive keratectomy
d e
Radial keratotomy
f
Keratotomy correction of astigmatism
g
Holmium laser correction of hyperopia
Epikeratophakic keratoplasty
h
i
= Excimer laser
Figs.5.18a–iSeeexplanationsintext.
5.7CornealSurgery
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170. 152
!Refractivecornealprocedureschangetherefractivepowerofaclearcor-
nea.
5.7.1 CurativeCornealProcedures
5.7.1.1PenetratingKeratoplasty(Fig.5.18a)
Principle:Thisinvolvesreplacementofdiseasedcornealtissuewithafull-
thicknessdonorgraftofcornealtissueofvaryingdiameter.Aclear,regularly
refractingbuttonofdonorcorneaisplacedinanopacifiedorirregularly
refractingcornea.Thecornealbuttonissuturedwithacontinuoussingleor
doublesuture(Fig.5.19)orwithinterruptedsutures.(Forspecialconsidera-
tionsincornealtransplants,seealsoMorphologyandhealing.)
Penetratingkeratoplastycanbeperformedasanelectiveprocedureto
improvevisualacuityorasanemergencyprocedure(emergencykerato-
plasty).Emergencykeratoplastyisindicatedtotreataperforatedornonheal-
ingcornealulcertoremovetheperforationsiteandsavetheeye(tectonicker-
atoplasty).
Indications:Cornealdiseasesthataffectthefullthicknessofthecorneal
stroma(cornealscars,dystrophy,ordegeneration)orprotrusionanomalies
suchaskeratoconusorkeratoglobuswithorwithoutcentralcornealopacifi-
cation.
AllograftRejection(Complications):Thebody’simmunesystemcan
respondwithachronicfocalallograftrejection(Fig.5.20)oradiffuseallograft
rejection(Fig.5.21).Thegraftwillbebecomeopacified.
Penetratingkeratoplasty.
Fig.5.19The
donorcorneal
buttonissutured
withacontinuous
doublesuture.
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171. 153
5.7.1.2LamellarKeratoplasty(Fig.5.18b)
Principle:Thisinvolvesreplacementofasuperficialstromalopacification
withapartial-thicknessdonorgraftofclearcornealtissue.
Thissurgeryrequiresthecornealepithelium,Descemet’smembrane,and
thedeeperlayersofthecorneatobeintactandhealthyasitisonlysuitablefor
removingsuperficialopacificationsdowntoaboutthemiddleofthecornea.
Thedonorcornealbuttonisthensuturedwithoneortwocontinuoussutures
orwithinterruptedsutures.
Chronicfocalallograftrejection.
Fig.5.20aThe
reactionpro-
ceedsfromvas-
cularbranches
extendingtothe
graft(arrows).
Thegraftshows
focalopacifica-
tion(leftimage)
andisthickened
(rightimage)
withaprogress-
ivefrontalline
(Khodadoust’s
line).
bThesameeye
aftertwoweeks
oftopicaland
systemicsteroid
therapy.The
graftisagain
clearandofnor-
malthickness.
5.7CornealSurgery
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172. 154
Acutediffuseallograftrejection.
Fig.5.21aThe
graftisopacified
andthickened.
bTheslitlamp
imagereveals
precipitatespos-
teriortothecor-
nea.
Indications:Cornealopacificationsandscarsaffectingthesuperficialcorneal
stroma(post-traumatic,degenerative,dystrophic,orpostinflammatory
opacifications).Thismethodisnotsuitablefortreatingcornealulcers.
AllograftRejection(Complications):Allograftrejectionislessfrequentthan
inthecaseofpenetratingkeratoplasty.Thereisalsolessdangerofinfectionas
lamellarkeratoplastydoesnotinvolveopeningtheglobe.
5.7.1.3PhototherapeuticKeratectomy(Fig.5.18c)
Principle:Superficialcornealscarscanbeablatedwithanexcimerlaser
(wavelengthof193nm).Thelesionisexcisedparalleltothesurfaceofthecor-
neatoavoidrefractiveeffects.Theedgesoftheablatedareaaremerged
smoothlywiththerestofthecornealsurface,eliminatinganyirregularities.
Indications:Indicationsareidenticaltothoseforlamellarkeratoplasty.
However,thismethodisonlysuitableforablationofrelativelysuperficialcor-
nealopacifications,i.e.,intheupper20%ofthecornealstroma.
Disadvantage:Despiteattemptingablationparalleltothesurfaceofthecor-
nea,phototherapeutickeratectomyoftencreatesahyperopiceffect.
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173. 155
5.7.2RefractiveCornealProcedures
5.7.2.1PhotorefractiveKeratectomy(Fig.5.18d)
Principle:Tissueisablatedtochangethecornealcurvatureandtoachievea
refractivecorrection.Flatteningthecornealcurvaturecorrectsmyopia,
whereassteepeningthecurvaturecorrectshyperopia.Theamountoftissue
removedatdifferentsitescanbevariedwithlayer-by-layerexcimerlaser
ablationandtheuseofapertures.Thismakesitpossibletocorrectformyopia,
byremovingmoretissuefromthecenterofthecornea,orforhyperopia,by
removingmoretissuefromtheperiphery.
Indications:Bestresultsareachievedincorrectingmyopiaoflessthan6
diopters.Atpresentstablecorrectioncanbeachievedin85–95%ofallcases
ofmyopiaupto!6diopters,withdeviationof!1diopterfromthetarget
withinoneyear.Correctionofhyperopiahasalsobeenattempted.
5.7.2.2RadialKeratotomy(Fig.5.18e)
Principle:Correctionofmyopiabyflatteningthecentraldomeofthecornea
withfourtosixteenradialincisionsextendingthroughasmuchas90%ofthe
thicknessofthecornea.Thisincreasesthesteepnessofthecornealperiphery
andlowersthecenterofthecornea,reducingitsrefractivepower.This
methoddoesnotinfluencetheopticalcenterofthecornea(Fig.5.22).
Indicationsandprognosis:Themethodissuitableformoderatemyopia
(lessthan6diopters).Theeffectachievedisinfluencedbytheinitialrefrac-
tion,intraocularpressure,cornealthickness,andthepatient’sageandsex.A
Radialkeratotomy.
Fig.5.22My-
opiaiscorrected
withradialinci-
sionsinthecor-
nea(twelvein
thiscase).This
flattensthedome
ofthecentral
corneaandin-
creasesthe
steepnessofthe
cornealperiph-
ery.
5.7CornealSurgery
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174. 156
disadvantageisrefractivefluctuationsofupto1.5dioptersduringthecourse
oftheday.Inone-fifthofallcasesrefractionbecomesunstablewithinayear.
5.7.2.3PhotorefractiveKeratectomyCorrectionofAstigmatism
Principle:Surgicalreductionofsevereregularastigmatismbyflatteningthe
steepmeridianinthecenterofthecorneabyincreasingthesteepnessofthe
cornealperiphery.Irregularastigmatismcannotbecorrected.
Indication:Severeregularastigmatism.
5.7.2.4HolmiumLaserCorrectionofHyperopia
Principle:Thelaserisfocusedonthecornealstromatocreateshrinkage
effects.Placingtheseareassymmetricallysteepensthecentralcornea,which
cancorrectseverehyperopia.
Indication:Hyperopiacorrectionupto8diopters.
5.7.2.5EpikeratophakicKeratoplasty(Epikeratophakia)
Principle:Severemyopiaandhyperopiaarecorrectedbysuturingspecially
preparedhyperopicormyopicpartial-thicknesscornealgraftsontothe
recipient’scornea.Thisinvolvesspecialtrephinationandpreparationofthe
recipient’scornea.Thedonorgraftisthenfittedintothepreparedcorneaand
suturedinplace.Thedonorcornealbuttonispreparedasafrozensectionand
shapedtotherequiredrefractivepower;theseimplantscanbeorderedfrom
eyebanks.
Indications:Anyseverityofhyperopiaormyopiacanbecorrected.
5.7.2.6ExcimerLaserinsituKeratomileusis(LASIK)
Principle:MyopiaiscorrectedwithpreservationofBowman’slayer.Asuper-
ficialcornealflap(approx.160µm)iscreatedwithamicrokeratome.Theker-
atomeiswithdrawn,theflapisreflected,andtheexposedunderlyingcorneal
stromaisablatedwithanexcimerlasertocorrectthemyopia.Thentheflapis
repositionedonthecornealbedandfixedinplacebyforceofitsownadhe-
sion.
Indication:Evenseveremyopia(upto10–12diopters)canbecorrectedwith
thismethod.
5Cornea
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175. 157
6 Sclera
GerhardK.Lang
6.1 BasicKnowledge
Function:Thescleraandthecorneaformtherigidoutercoveringoftheeye.
Allsixocularmusclesinsertintothesclera.
Morphology:Thescleraisfibrous,whitishopaque,andconsistsofnearly
acellularconnectivetissuewithahigherwatercontentthanthecornea.The
scleraisthickest(1mm)anteriorlyatthelimbusofthecorneawhereitjoins
thecornealstromaandatitsposteriorpole.Itisthinnest(0.3mm)atthe
equatorandbeneaththeinsertionsoftherectusmuscles.Thesitewherethe
fibersoftheopticnerveenterthescleraisknownasthelaminacribrosa.In
theangleoftheanteriorchamber,thescleraformsthetrabecularnetwork
andthecanalofSchlemm.Theaqueoushumordrainsfromthereintothe
intrascleralandepiscleralvenousplexusthroughabout20canaliculi.
Neurovascularsupply:Vortexveinsandtheshortanteriorandposteriorcili-
aryarteriespenetratethesclera.Theciliarynervescoursethroughthesclera
fromposteriortoanterior.
6.2 ExaminationMethods
Theanteriorportionofthescleraaboutasfarbackastheequatorcanbe
examineddirectlywithaslitlamp.Evaluationofthescleraposteriortothe
equatorrequiresindirectmethodssuchasultrasound.
Transilluminationcanprovideevidenceofpossibleabnormalchangesin
theposteriorsclera.However,thismethodisnotaspreciseasanultrasound
study.
6.3 ColorChanges
Thescleraisnormallydullwhitelikeporcelain.Alteredcolorsuggestsoneof
thefollowingchanges:
!Conjunctivaland/orciliaryinjectionandinflammationwillgivethesclera
aredappearance.
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176. 158
!Asclerathatisverythinwillappearbluebecauseoftheunderlying
choroid(thisoccursinthenewborn,inosteogenesisimperfecta,andfol-
lowinginflammation;seeFig.6.4).
!Injaundice,thescleraturnsyellow.
!Inochronosis(alkaptonuria),thesclerawilltakeonbrownishcolor.This
shouldbedistinguishedfrompigmentedchangesintheconjunctiva.
6.4 StaphylomaandEctasia
Staphylomareferstoabulgingofthesclerainwhichtheunderlyinguveal
tissueinthebulgeisalsothinnedordegenerated.Byfarthemostcommon
formisposteriorstaphylomainseveremyopia,abulgingoftheentireposterior
poleoftheeyeball(Fig.6.1).Staphylomacanalsooccursecondarytoscleritis
(seeFig.6.4).
Ectasiaisathinningandbulgingofthesclerawithoutuvealinvolvement,
ascanoccursecondarytoinflammation.
Bothstaphylomaandectasiaaresecondaryorincidentalfindings.No
treatmentisavailable.
6.5 Trauma
Thescleraisfrequentlyinvolvedinpenetratingtrauma.Deepinjuriesthat
extendfarposteriorlyusuallyalsoinvolvethechoroidandretina.Surgeryto
treatlargerinjuriesextending8mmpastthelimbusshouldalsoincludearet-
inalrepair(retinalcryopexyorretinaltamponade.
6.6 Inflammations
Inflammationsarethemostclinicallysignificantscleralchangesencountered
inophthalmologicpractice.Theymoreofteninvolvetheanteriorsclera(epis-
cleritisandanteriorscleritis)thantheposteriorsclera(posteriorscleritis).
Classification:Formsofscleralinflammationaredifferentiatedasfollows:
!Location:anteriororposterior,i.e.,anteriororposteriortotheequatorof
theglobe.
!Depth:
–Superficial(episcleritis).
–Deep(scleritis).
!Nature:
–Diffuse(usuallyscleritis).
–Circumscribedorsegmental(episcleritis).
–Nodular,withformationofsmallmobilenodules(scleritisandepis-
cleritis).
6Sclera
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177. 159
Posteriorstaphylomainahighlymyopiceye.
Fig.6.1aOphthalmologic
imageofposteriorstaphy-
lomaofthesclera.
bUltrasoundimageshowing
theposteriorscleralbulgeand
obliquecourseoftheoptic
nervethroughthesclera.
–Necrotizing(scleritisonly).
–Non-necrotizing(scleritisonly).
6.6.1 Episcleritis
Definition
Circumscribed,usuallysegmental,andgenerallynodularinflammationofthe
episclera(connectivetissuebetweenscleraandconjunctiva).
Epidemiology:Episcleritisisthemostcommonformofscleralinflammation.
6.6Inflammations
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178. 160
Etiology:Episcleritisisrarelyattributabletooneofthesystemicunderlying
disorderslistedinTable6.1,andisonlyoccasionallyduetobacterialorviral
inflammation.Oftenepiscleritiswillhavenoreadilydiscerniblecause.
Symptoms:Episcleritiscanbeunilateralorbilateral.Itisusuallyassociated
withsegmentalreddeningandslighttendernesstopalpation.
Findings:Theepiscleralvesselsliewithinthefascialsheathoftheeyeball
(Tenon’scapsule)andarearrangedradially.Inepiscleritis,thesevesselsand
theconjunctivalvesselsabovethembecomehyperemic(Fig.6.2).Tenon’s
capsuleandtheepiscleraareinfiltratedwithinflammatorycells,butthe
scleraitselfisnotswollen.Thepresenceofsmallmobilenodulesistypicalof
nodularepiscleritis.
Differentialdiagnosis:Thedisordershouldbedistinguishedfromcon-
junctivitis(seenextparagraph)andscleritis(6.6.2).
Theconjunctivalbloodvesselsarethemostsuperficial;theepiscleral
vesselsliewithinTenon’scapsuleandarearrangedradially.Whenvaso-
constrictiveeyedropsareapplied,theconjunctivalinjectionwilldisap-
pearbutnottheepiscleralinjection.Thismakesitpossibletodistin-
guishconjunctivitisfromepiscleritis.
Treatmentandprognosis:Episcleritisusuallyresolvesspontaneously
withinonetotwoweeks,althoughthenodularformcanpersistforextended
periodsoftime.Severesymptomsaretreatedwithtopicalsteroids(eyedrops)
orwithanonsteroidalanti-inflammatoryagent.
Segmentalepiscleritis.
Fig.6.2Typical
hyperemiaand
inflammationof
theradial
episcleralblood
vessels.
6Sclera
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179. 161
Table6.1Systemicdiseasesthatcancausescleritis
Frequentcauses Rarecauses
!Rheumatoidarthritis
!Polymyositis
!Dermatomyositis
!Ankylosingspondylitis
!Spondylarthritis
!Vasculitis
!Wegener’sgranulomatosis
!Herpeszosterophthalmicus
!Syphilis
!Gout
!Tuberculosis
!Lues
!Borreliosis
!Reiter’ssyndrome
6.6.2Scleritis
Definition
Diffuseorlocalizedinflammationofthesclera.Scleritisisclassifiedaccording
tolocation:
!Anterior(inflammationanteriortotheequatoroftheglobe).
!Posterior(inflammationposteriortotheequatoroftheglobe).
Anteriorscleritisisfurtherclassifiedaccordingtoitsnature:
!Non-necrotizinganteriorscleritis(nodularordiffuse).
!Necrotizinganteriorscleritis(withorwithoutinflammation).
Epidemiology:Scleritisisfarlessfrequentthanepiscleritis.Patientsaregen-
erallyolder,andwomenareaffectedmoreoftenthanmen.
Etiology:Approximately50%ofscleritiscases(whichtendtohavesevere
clinicalcourses)areattributabletosystemicautoimmuneorrheumaticdis-
ease(Table6.1),oraretheresultofimmunologicprocessesassociatedwith
infection.Thisappliesespeciallytoanteriorscleritis.Posteriorscleritisisnot
usuallyassociatedwithanyspecificdisorder.Aswithepiscleritis,scleritisis
onlyoccasionallyduetobacterialorviralinflammation.
Symptomsandfindings:Allformsexceptforscleromalaciaperforansare
associatedwithseverepainandgeneralreddeningoftheeye.
Anteriornon-necrotizingscleritis(nodularform).Thenodulesconsistof
edematousswollenscleraandarenotmobile(incontrasttoepiscleritis).
6.6Inflammations
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180. 162
Anteriornecrotizingscleritis(diffuseform).Theinflammationismore
severethaninthenodularform.Itcanbelimitedtoacertainsegmentormay
includetheentireanteriorsclera(Fig.6.3).
Anteriornecrotizingscleritiswithinflammation.Circumscribedreddening
oftheeyesisatypicalsign.Theremaybedeviationorinjectionoftheblood
vesselsoftheaffectedregion,accompaniedbyavascularpatchesinthe
episcleraltissue.Asthedisorderprogresses,thesclerathinsasthescleral
lamellaeofcollagenfibrilsmelt,sothattheunderlyingchoroidshows
through(Fig.6.4).Theinflammationgraduallyspreadsfromitsprimary
focus.Usuallyitisassociatedwithuveitis.
Anteriornecrotizingscleritiswithoutinflammation(scleromalaciaper-
forans).Thisformofscleritistypicallyoccursinfemalepatientswithalong
historyofseropositiverheumatoidarthritis.Theclinicalcourseofthedis-
orderisusuallyasymptomaticandbeginswithayellownecroticpatchonthe
sclera.Asthedisorderprogresses,thescleraalsothinssothattheunderlying
choroidshowsthrough.Thisistheonlyformofscleritisthatmaybepainless.
Posteriorscleritis.Sometimestherewillbenoabnormalfindingsintheante-
rioreye,andpainwillbetheonlysymptom.Associatedinflammationofthe
orbitmayresultinproptosis(exophthalmos)andimpairedocularmotility
duetomyositisoftheocularmuscles.Intraocularfindingsmayinclude
exudativeretinaldetachmentand/orchoroiddetachment.Macularandoptic
diskedemaarefrequentlypresent.
6Sclera
Diffusenon-necrotizingscleritis.
Fig.6.3Typical
signsinclude
thickeningand
edemaofthe
scleraanddeep,
diffuseredden-
ing.
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181. 163
Circumscribedscleralstaphylomasecondarytoscleritis.
Fig.6.4Theun-
derlyingchoroid
showsthroughat
thebulgewhere
thesclerais
thinned,givingit
abluishtinge.
Thereddeninginscleritisisduetoinjectionofthedeepervascular
plexusonthescleraandtoinjectionoftheepisclera.Inspectingtheeye
indaylightwillbestrevealthelayerofmaximuminjection.
Differentialdiagnosis:Conjunctivitisandepiscleritis(seethatsection).
Treatment:
Anteriornon-necrotizingscleritis.Topicalorsystemicnonsteroidalanti-
inflammatorytherapy.
Anteriornecrotizingscleritiswithinflammation.Systemicsteroidtherapyis
usuallyrequiredtocontrolpain.Ifcorticosteroidsdonothelporarenot
tolerated,immunosuppressiveagentsmaybeused.
Anteriornecrotizingscleritiswithoutinflammation(scleromalaciaper-
forans).Asnoeffectivetreatmentisavailable,graftsofpreservedscleraor
lyophilizedduramayberequiredtopreservetheglobeifthecourseofthe
disorderisfulminant.
Posteriorscleritis.Treatmentisthesameasforanteriornecrotizingscleritis
withinflammation.
6.6Inflammations
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183. 165
7 Lens
GerhardK.Lang
7.1 BasicKnowledge
Functionofthelens:Thelensisoneoftheessentialrefractivemediaofthe
eyeandfocusesincidentraysoflightontheretina.Itaddsavariableelement
totheeye’stotalrefractivepower(10–20diopters,dependingonindividual
accommodation)tothefixedrefractivepowerofthecornea(approximately
43diopters).
Shape:Thefullydevelopedlensisabiconvex,transparentstructure.The
curvatureoftheposteriorsurface,whichhasaradiusof6mm,isgreaterthan
thatoftheanteriorsurface,whichhasaradiusof10mm.
Weight:Thelensisapproximately4mmthick,anditsweightincreaseswith
agetofivetimesitsweightatbirth.Anadultlensweighsabout220mg.
Positionandsuspension:Thelensliesintheposteriorchamberoftheeye
betweentheposteriorsurfaceoftheirisandthevitreousbodyinasaucer-
shapeddepressionofthevitreousbodyknownasthehyaloidfossa.Together
withtheirisitformsanopticaldiaphragmthatseparatestheanteriorand
posteriorchambersoftheeye.Radiallyarrangedzonulefibersthatinsertinto
thelensarounditsequatorconnectthelenstotheciliarybody.Thesefibers
holdthelensinposition(Fig.7.1)andtransferthetensileforceoftheciliary
muscle(seeAccommodation).
Embryologyandgrowth:Thelensisapurelyepithelialstructurewithout
anynervesorbloodvessels.Itmovesintoitsintraocularpositioninthefirst
monthoffetaldevelopmentassurfaceectoderminvaginatesintotheprimi-
tiveopticvesicle,whichconsistsofneuroectoderm.Apurelyectodermalstruc-
ture,thelensdifferentiatesduringgestationintocentralgeometriclens
fibers,ananteriorlayerofepithelialcells,andanacellularhyalinecapsule
(Figs.7.2aandb).Thenormaldirectionofgrowthofepithelialstructuresis
centrifugal;fullydevelopedepithelialcellsmigratetothesurfaceandare
peeledoff.However,thelensgrowsintheoppositedirection.Theyoungest
cellsarealwaysonthesurfaceandtheoldestcellsinthecenterofthelens.
Thegrowthofprimarylensfibersformstheembryonicnucleus.Attheequa-
tor,theepithelialcellsfurtherdifferentiateintolensfibercells(Fig.7.2).
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184. 166
Embryologyofthelens.
Fig.7.2aFirstmonthoffetaldevelopment:Theectoderminvaginatesandisiso-
latedinwhatbecomestheopticcup.bThelensvesicleiscompletelyinvaginated.
Theprimarylensfibersgrowandbegintoformtheembryonicnucleus.
Shapeofthelensanditspositionintheeye.
Anterior chamber
Posterior chamber
Vitreous body
Lens
Iris
Zonule fibers
Ciliary body
Hyaloid fossa
Fig.7.1Thelensisabiconvexstructuresuspendedonthezonulefibers.Itliesin
thehyaloidfossaandseparatestheanteriorandposteriorchambersoftheeye.
7Lens
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185. 167
Surface
Embryonic nucleus
Fetal nucleus
Infantile nucleus
Adult nucleus
Cortex
Epithelium
Capsule
Anterior aspect Lateral aspect
Anatomyofthelens.
Fig.7.3
Thesenewsecondaryfibersdisplacetheprimaryfiberstowardthecenterof
thelens.Formationofafetalnucleusthatenclosestheembryonicnucleusis
completeatbirth.Fiberformationattheequator,whichcontinuesthrough-
outlife,producestheinfantilenucleusduringthefirstandseconddecadesof
life,andtheadultnucleusduringthethirddecade.Completelyenclosedby
thelenscapsule,thelensneverlosesanycellssothatitstissueiscontinuously
compressedthroughoutlife(Fig.7.3aandb).Thevariousdensityzones
createdasthelensdevelopsarereadilydiscernibleasdiscontinuityzones
(Fig.7.4).
Metabolismandagingofthelens:Thelensisnourishedbydiffusionfrom
theaqueoushumor.Inthisrespectitresemblesatissueculture,withthe
aqueoushumorasitssubstrateandtheeyeballasthecontainerthatprovides
aconstanttemperature.
Themetabolismanddetailedbiochemicalprocessesinvolvedinaging
arecomplexandnotcompletelyunderstood.Becauseofthis,ithasnot
beenpossibletoinfluencecataractdevelopment(seeCataract,p.170)
withmedications.
Themetabolismandgrowthofthelenscellsareself-regulating.Metabolic
activityisessentialforthepreservationoftheintegrity,transparency,and
opticalfunctionofthelens.Theepitheliumofthelenshelpstomaintainthe
ionequilibriumandpermittransportationofnutrients,minerals,andwater
intothelens.Thistypeoftransportation,referredtoasa“pump-leaksys-
tem,”permitsactivetransferofsodium,potassium,calcium,andaminoacids
7.1BasicKnowledge
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186. 168
Slit beam of light
Cross section
of cornea
Anterior chamber
Anterior capsule
Cortex
Discontinuity zones identifying
the adult , infantile ,
fetal , and embryonic nuclei
Posterior lens capsule
Vitreous chamber
Slit beam on the anterior
surface of the iris
2
4
3
1
1 2
3 4
Slit-lampexaminationofthelens.
Fig.7.4Thevariousdensityzones(1–4)createdasthelensdevelopsarediscern-
ibleasdiscontinuityzones.
fromtheaqueoushumorintothelensaswellaspassivediffusionthroughthe
posteriorlenscapsule.Maintainingthisequilibrium(homeostasis)isessen-
tialforthetransparencyofthelensandiscloselyrelatedtothewaterbalance.
Thewatercontentofthelensisnormallystableandinequilibriumwiththe
surroundingaqueoushumor.Thewatercontentofthelensdecreaseswith
age,whereasthecontentofinsolublelensproteins(albuminoid)increases.
Thelensbecomesharder,lesselastic(seeLossofaccommodation),andless
transparent.Adecreaseinthetransparencyofthelenswithageisas
unavoidableaswrinklesintheskinorgrayhair.Manifestlyreducedtrans-
parencyispresentin95%ofallpersonsovertheageof65,althoughindividual
exceptionsarenotuncommon.Thecentralportionornucleusofthelens
becomessclerosedandslightlyyellowishwithage.
7.2 ExaminationMethods
Cataracts:Retroilluminationofthelens(Brückner’stest)isthequickestpre-
liminaryexaminationmethodforlensopacities(Cataracts,seesection7.4).
Underalightsourceorophthalmoscope(setto10diopters),opacitieswill
appearblackintheredpupil(Fig.7.5).Thelenscanbeexaminedingreater
detailandinthreedimensionsunderfocalilluminationwithaslitlampwith
thepupilmaximallydilated.Theextent,type,location,anddensityofopaci-
tiesandtheirrelationtothevisualaxismaybeevaluated.Maturelensopaci-
7Lens
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187. 169
Retroilluminationofthelens(Brückner’stest).
Fig.7.5Opacitiesappearblackinthe
redpupil.
tiesmaybediagnosedwiththeunaidedeyebythepresenceofawhitepupil
(leukocoria).
Wherethefundusisnotvisibleinthepresenceofamaturelensopacity,
ultrasoundstudies(one-dimensionalA-scanandtwo-dimensionalB-
scanstudies)areindicatedtoexcludeinvolvementofthedeeperstruc-
turesoftheeye.
Iridodonesisandphacodonesis:Tremulousmotionoftheirisandlens
observedduringslit-lampexaminationsuggestssubluxationofthelens(see
p.195).
7.3 DevelopmentalAnomaliesoftheLens
Anomaliesoflensshapeareveryrare.Lenticonusisacircumscribedconical
protrusionoftheanteriorpole(anteriorlenticonus)orposteriorpole(poste-
riorlenticonus).Ahemisphericalprotrusionisreferredtoaslentiglobus.
Symptomsincludemyopiaandreducedvisualacuity.Somepatientswith
Alport’ssyndrome(kidneydiseaseaccompaniedbysensorineuralhearing
lossandanomaliesoflensshape)haveanteriorlenticonus.Posteriorlenti-
conusmaybeassociatedwithalensopacity(Fig.7.6).Treatmentisthesame
asforcongenitalorjuvenilecataract.
Microphakiareferstoalensofabnormallysmalldiameter.Anyinterrup-
tionofthedevelopmentoftheeyegenerallyleadstomicrophakia.Thiscan
occurforexampleinWeill-Marchesanisyndrome(seeTable7.5).
7.3DevelopmentalAnomaliesoftheLens
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188. 170
Posteriorlenticonus.
Fig.7.6Conicalprotrusionoftheposteriorpole,hereassociatedwithaposterior
subcapsularopacity.
7.4 Cataract
Definition
Acataractispresentwhenthetransparencyofthelensisreducedtothepoint
thatthepatient’svisionisimpaired.ThetermcataractcomesfromtheGreek
wordkatarraktes(downrushing;waterfall)becauseearlieritwasthoughtthat
thecataractwasacongealedfluidfromthebrainthathadflowedinfrontof
thelens.
Generalsymptoms:Developmentofthecataractanditssymptomsisgen-
erallyanoccultprocess.Patientsexperiencethevarioussymptomssuchas
seeingonlyshadesofgray,visualimpairment,blurredvision,distorted
vision,glareorstarbursts,monoculardiplopia,alteredcolorperception,etc.
tovaryingdegrees,andthesesymptomswillvarywiththespecifictypeof
cataract(seeTable7.3andFigs.7.7aandb).
Diagnosisofacataractisgenerallyveryunsettlingforpatients,who
immediatelyassociateitwithsurgery.Oneshouldthereforereferonly
toacataractwhenithasbeenestablishedthatsurgeryisindicated.If
thecataracthasnotprogressedtoanadvancedstageorthepatientcan
copewellwiththevisualimpairment,oneshouldreferinsteadtoa“lens
opacity.”
7Lens
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189. 171
Cataractsymptoms.
Fig.7.7aVisual
imagewithouta
cataract.
bVisualimage
withacataract:
grayareasand
partiallossof
imagepercep-
tion.
Classification:Cataractsmaybeclassifiedaccordingtoseveraldifferentcri-
teria.
!Timeofoccurrence(acquiredorcongenitalcataracts).
!Maturity.
!Morphology.
Nooneclassificationsystemiscompletelysatisfactory.Wepreferthesystem
inTable7.1.
7.4Cataract
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190. 172
Table7.1Classificationofcataractsaccordingtotimeofoccurrence
Acquiredcataracts
(over99%ofallcataracts)
!Senilecataract(over90%ofallcataracts)
!Cataractwithsystemicdisease
–Diabetesmellitus
–Galactosemia
–Renalinsufficiency
–Mannosidosis
–Fabry’sdisease
–Lowe’ssyndrome
–Wilson’sdisease
–Myotonicdystrophy
–Tetany
–Skindisorders
!Secondaryandcomplicatedcataracts
–Cataractwithheterochromia
–Cataractwithchroniciridocyclitis
–Cataractwithretinalvasculitis
–Cataractwithretinitispigmentosa
!Postoperativecataracts
–Mostfrequentlyfollowingvitrectomyand
siliconeoilretinaltamponade
–Followingfilteringoperations
!Traumaticcataracts
–Contusionorperforationrosette
–Infraredradiation(glassblower’scataract)
–Electricalinjury
–Ionizingradiation
!Toxiccataract
–Corticosteroid-inducedcataract(most
frequent)
–Lessfrequentlyfromchlorpromazine,miotic
agents,orbusulfan
Congenitalcataracts
(lessthan1%ofallcataracts)
!Hereditarycataracts
–Autosomaldominant
–Recessive
–Sporadic
–X-linked
!Cataractsduetoearlyembryonic(trans-
placental)damage
–Rubella(40–60%)
–Mumps(10–22%)
–Hepatitis(16%)
–Toxoplasmosis(5%)
7Lens
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191. 173
7.4.1 AcquiredCataract
7.4.1.1SenileCataract
Epidemiology:Senilecataractisbyfarthemostfrequentformofcataract,
accountingfor90%ofallcataracts.About5%ofall70-year-oldsand10%ofall
80-year-oldssufferfromacataractrequiringsurgery.
Ninetypercentofallcataractsaresenilecataracts.
Etiology:Theprecisecausesofsenilecataracthavenotbeenidentified.As
occurrenceisoftenfamilial,itisimportanttoobtainadetailedfamilyhistory.
Classificationandformsofsenilecataracts:Theclassificationaccordingto
maturity(Table7.2)followsthedegreeofvisualimpairmentandthematur-
ity,whichearlierwasimportanttodeterminethetimeofsurgery.Wefollowa
morphologicclassificationasmorphologicaspectssuchasthehardnessand
thicknessofthenucleusnowinfluencethesurgicalprocedure(Table7.3):
Nuclearcataract.Inthefourthdecadeoflife,thepressureofperipherallens
fiberproductioncauseshardeningoftheentirelens,especiallythenucleus.
Thenucleustakesonayellowishbrowncolor(brunescentnuclearcataract).
Thismayrangefromreddishbrowntonearlyblackdiscolorationoftheentire
lens(blackcataract).Becausetheyincreasetherefractivepowerofthelens,
nuclearcataractsleadtolenticularmyopiaandoccasionallyproduceasecond
focalpointinthelenswithresultingmonoculardiplopia(Fig.7.8).
Nuclearcataractsdevelopveryslowly.Duetothelenticularmyopia,
nearvision(evenwithouteyeglasses)remainsgoodforalongtime.
Corticalcataract.Nuclearcataractsareoftenassociatedwithchangesinthe
lenscortex.Itisinterestingtonotethatpatientswithcorticalcataractstend
tohaveacquiredhyperopiaincontrasttopatientswithnuclearcataracts,who
tendtobemyopic(seeabove).
Table7.2Classificationofcataractsaccordingtomaturity
Cataractform Visualacuity
Developingcataract Stillfull(0.8–1.0)
Immaturecataract Reduced(0.4–0.5)
Developedcataract Severelyreduced(1/50–0.1)
Maturecataract
Hypermaturecataract
Lightanddarkperception,perceptionofhand
movementsinfrontoftheeye
7.4Cataract
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192. 174
Table7.3Overviewofformsofsenilecataract
Cataractform Morphology Incidence Symptoms
Nuclear
cataract
About30%,
particularlyin
moresevere
myopia
–Shadesofgray(like
lookingthrough
frostedglass)
–Blurredvision
–Distortedvision
–Intenseglarein
brightlight
–Diminishedcon-
trast
–Changesincolor
perception(rare)
anterior
Subcapsular
cataract
posterior
About50%
Posteriorsub-
capsularcataract
About20%
Maturecataract Finalstage –Objectsnolongerdis-
cernible
–Patientswithbilateral
cataractsarepracti-
callyblindand
dependentonothers
ineverydaylife
Hypermature
cataract
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193. 175
Visualacuity ProgressionPeculiarities,glare,
eyesightintwilight
Diagnosisandprog-
nosisforvision
–Impairmentis
relativelylate
–Increasingpoor
distancevision
–Nearvision
remainsdueto
myopiceffect
ofcataract
Slow –Eyesightintwilight
isoftenbetterthan
indaylightbecause
themydriasisin
darknessallows
lightpasttheopac-
ity
–Glareislesspro-
nounced
–Monoculardiplopia
duetotwofocal
pointsinthelens.
–Earlylossof
visualacuity
–Hyperopic
effectofcata-
ractcompro-
misesdistance
visionlessthan
nearvision
Rapid(tem-
poraryimpro-
vementin
visualacuity
dueto
stenopeic
effect)
–Patientisseverely
hamperedbyglare
(sun,snow,head-
lights).Patients
typicallypreferdark
glassesandwide-
brimmedhats.
–Markedimprove-
mentofvisionin
twilightandatnight
(nyctalopia)
–Morphologyby
transillumina-
tion(Brück-
ner’stest)
–Detaileddiag-
nosisinslit-
lampexamina-
tion
–Predictionof
expectedpost-
operativevisual
acuity:laser
interference
visualacuity
testing
–Earlylossof
visualacuity
–Nearvisionpar-
ticularlyaffect-
ed,distance
visionlessso
Rapid
Visualacuity
reducedtoper-
ceptionoflight
anddark;percep-
tionofhand
movementsin
frontoftheeyeat
best.
Allcataract
formswill
progresstoa
matureor
hypermature
formgiven
enoughtime.
Inintenselight,patient
willperceivegross
movementsandper-
sonsassilhouettes.
–Leukocoria(white
pupil)detectable
withunaidedeye.
–Slit-lamppermits
differentiation.
–Retinoscopyto
determinevisual
acuityisoften
inneffectivewith
denseopacities.
7.4Cataract
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194. 176
Whereaschangesinnuclearcataractsareduetohardening,cortical
changesarecharacterizedbyincreasedwatercontent.Severalmorphologic
changeswillbeapparentuponslit-lampexaminationwithmaximummydri-
asis:
!Vacuoles:Fluidaccumulationswillbepresentintheformofsmallnarrow
corticalvesicles.Thevacuolesremainsmallandincreaseinnumber.
!WaterFissures:Radialpatternsoffluid-filledfissureswillbeseenbetween
thefibers.
!Separationofthelamellae:Notasfrequentaswaterfissures,theseconsist
ofazoneoffluidbetweenthelamellae(oftenbetweentheclearlamellae
andthecorticalfibers).
!Cuneiformcataract:Thisisafrequentfindinginwhichtheopacities
radiatefromtheperipheryofthelenslikespokesofawheel.
Corticalcataractsprogressmorerapidlythannuclearcataracts.Visual
acuitymaytemporarilyimproveduringthecourseofthedisease.Thisis
duetoastenopeiceffectaslightpassesthroughaclearareabetween
tworadialopacities.
Posteriorsubcapsularcataract.Thisisaspecialformofcorticalcataractthat
beginsinthevisualaxis.Beginningasasmallclusterofgranularopacities,
thisformofcataractexpandsperipherallyinadisk-likepattern.Asopacity
increases,therestofthecortexandthenucleusbecomeinvolved(theusual
spectrumofsenilecataract).
Nuclearcataract.
Fig.7.8Thenu-
cleusofthelens
hasayellowish
browncolordue
tothepressureof
peripherallens
fiberproduction.
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195. 177
Posteriorsubcapsularcataractleadstoearly,rapid,andseverelossof
visualacuity.Nearvisionisusuallysignificantlyworsethandistance
vision(near-fieldmiosis).Dilatingeyedropscanimprovevisualacuityin
thisformofcataract.
Maturecataract.Thelensisdiffuselywhiteduetocompleteopacificationof
thecortex.Ayellowlensnucleusisoftenfaintlydiscernible(Fig.7.9).Where
watercontentisincreased,alenswithamaturecataractcanswelland
acquireasilkyluster(intumescentcataractinwhichthecapsuleisunder
pressure).Theincreasingthicknessofthelensincreasestheresistanceofthe
pupilandwithittheriskofangleclosureglaucoma.
Visionisreducedtoperceptionoflightanddark,andtheinteriorofthe
eyeisnolongervisible.Cataractsurgeryisindicatedtorestorevisual
acuity.
Hypermaturecataract.Ifamaturecataractprogressestothepointof
completeliquificationofthecortex,thedensebrownnucleuswillsubside
withinthecapsule.Itssuperiormarginwillthenbevisibleinthepupilasa
darkbrownsilhouetteagainstthesurroundinggrayishwhitecortex.The
pressureinthelenscapsuledecreases.Thecontentsofthelimpandwrinkled
capsularbaggravitatewithinthecapsule.Thiscondition,referredtoas
Maturecataract.
Fig.7.9
!Thereisdiffuse,completeopacificationofthelens.Abrownishnucleusisfaintlyvis-
ibleposteriortothecorticallayer.
!Interioroftheeyeisnolongervisible.
!Visualacuityisreducedtoperceptionoflightanddark.
7.4Cataract
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196. 178
Morgagni’scataract,isthefinalstageinacataractthathasusuallydeveloped
overthecourseoftwodecades.Theapproximateonsetofthecataractcan
usuallybeinferredfromsuchfindings(Figs.7.10aandb).
Promptcataractextractionnotonlyrestoresvisualacuitybutalsopre-
ventsdevelopmentofphacolyticglaucoma.
Hypermaturecataract.
Fig.7.10aThe
brownnucleus
hassubsidedin
theliquifiedcor-
tex.
bHistologicimageobtainedatau-
topsyshowsthepositionofthesub-
sidednucleusandtheshrunkencapsu-
larbag.
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197. 179
Whenthelenscapsulebecomespermeableforliquifiedlenssubstances,it
willlosevolumeduetoleakage.Thecapsulewillbecomewrinkled.The
escapinglensproteinswillcauseintraocularirritationandattractmacro-
phagesthatthencausecongestionofthetrabecularnetwork(phacolyticglau-
coma:seeSecondaryopenangleglaucoma).
Emergencyextractionofthehypermaturecataractisindicatedinpha-
colyticglaucomatosavetheeye.
7.4.2CataractinSystemicDisease
Epidemiology.Lensopacitiescanoccasionallyoccurasasignofsystemicdis-
ease.
Formsofcataractsinsystemicdisease:
Diabeticcataract.Thetypicaldiabeticcataractisrareinyoungdiabetic
patients.Transientmetabolicdecompensationpromotestheoccurrenceofa
typicalradialsnowflakepatternofcorticalopacities(snowflakecataract).
Transienthyperopiaandmyopiacanoccur.
Diabeticcataractprogressesrapidly.Senilecataractsareobserved
aboutfivetimesasofteninolderdiabeticsasinpatientsthesameage
withnormalmetabolism.Thesecataractsusuallyalsooccurtwoto
threeyearsearlier.
Galactosemiccataract.Thisdeepposteriorcorticalopacitybeginsafterbirth.
Galactosemiaisararecauseofearlycataractinchildrenlackinganenzyme
requiredtometabolizegalactose.Thenewbornreceivesampleamountsof
galactoseinthemother’smilk.Duealackofuridyltransferase,orless
frequentlygalactokinase,galactosecannotbemetabolizedtoglucose,andthe
bodybecomesinundatedwithgalactoseorwithgalactoseandgalactose-1-
phosphate.Ifthedisorderisdiagnosedpromptlyandthechildismaintained
onagalactose-freediet,theopacitiesofthefirstfewweeksoflifewillbe
reversible.
Galactosemiccataractistheonlyformofcataractthatrespondstocon-
servativetherapy.
Dialysiscataract.Hemodialysistoeliminatemetabolicacidosisinrenal
insufficiencycandisturbtheosmoticequilibriumoflensmetabolismand
causeswellingofthecortexofthelens.
Otherraremetabolicdiseasesthatcancausecataractincludemannosido-
sis,Fabry’sdisease,Lowe’ssyndrome(oculocerebrorenalsyndrome),and
Wilson’sdisease(hepatolenticulardegeneration).
Cataractwithmyotonicdystrophy.Opacitiesfirstoccurbetweentheagesof
30and50,initiallyinathinlayeroftheanteriorcortexandlateralsointhe
7.4Cataract
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198. 180
subcapsularposteriorcortexintheformofrosettes.Detectingtheseopacities
isimportantfordifferentialdiagnosisascataractsdonotoccurinThomsen’s
disease(myotoniacongenita)orErb’sprogressivemusculardystrophy.
Symptomsthatconfirmthediagnosisincludecataract,activesignsofmyo-
tonia(delayedopeningofthefist),andpassivesignsofmyotonia(decreased
relaxationofmusclesintheextremitiesfollowingdirectpercussionofthe
muscleandabsenceofreflexes).
Tetanycataract.Theopacitylieswithinabroadzoneinferiortotheanterior
lenscapsuleandconsistsofaseriesofgraypunctatelesions.Symptomsthat
confirmthediagnosisincludelowbloodcalciumlevels,apositivehyperventi-
lationtest,andsignsoftetany:positiveChvostek,Trousseau,andErbsigns.
Dermatogenouscataract.Thismayoccurwithchronicneurodermatitis,less
frequentlywithotherskindisorderssuchasscleroderma,poikiloderma,
andchromiceczema.Characteristicsignsincludeananteriorcrest-shaped
thickeningoftheprotrudingcenterofthecapsule(Fig.7.11).
7.4.3ComplicatedCataracts
Thisformofcataractcanoccurasacomplicationofanyprotractedintraocular
inflammation,especiallyheterochromia,chroniciridocyclitis,retinalvasculi-
tis,andretinitispigmentosa.Theresultisapumice-likeposteriorsubcapsular
cataractthatprogressesaxiallytowardthenucleus.Thisformofcataractpro-
ducesextremelightscattering(Fig.7.12).
7.4.4CataractafterIntraocularSurgery
Cataractsusuallydevelopearlierintheoperatedeyeascomparedtothe
opposite,non-operatedeyeafterintraocularsurgery.Thisappliesespecially
tofilteringoperations.Asecondarycataractwillgenerallyoccurfollowing
vitrectomyandsiliconeoiltamponade.
7.4.5TraumaticCataract
Theincidenceoftheselensopacitiesishigherinmenthaninwomendueto
occupationalandsportsinjuries.Thefollowingtypesoftraumaticcataracts
aredifferentiated:
Frequenttraumaticcataracts:
!Contusioncataract:Contusionoftheeyeballwillproducearosette-
shapedsubcapsularopacityontheanteriorsurfaceofthelens.Itwillnor-
mallyremainunchangedbutwillmigrateintothedeepercortexovertime
duetotheappositionofnewfibers(Fig.7.13).
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199. 181
Dermatogenouscataract.
Fig.7.11Typical
symptomsin-
cludeacrest-
shapedwhitish
opacitybeneath
theanteriorlens
capsulealongthe
visualaxis.
Complicatedcataractinchroniciridocyclitis.
Fig.7.12Thisdiffuseopacityproceedsfromtheposteriorsubcapsularcataract.In-
flammatoryprecipitatesindicativeofchronicuveitisarealsovisibleontheposterior
surfaceofthecornea(arrow).
7.4Cataract
"
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200. 182
Contusioncataract.
Fig.7.13Acontusionrosetteposteriortotheanteriorlenscapsuledevelopsfol-
lowingsevereblunttraumatotheeyeball.
Rarertraumaticcataracts:
!Infraredradiationcataract(glassblower’scataract):Thistypeofcataract
occursafterdecadesofprolongedexposuretotheinfraredradiationoffire
withouteyeprotection.Characteristicfindingsincludesplittingofthe
anteriorlenscapsule,whoseedgeswillbeobservedtocurlupandfloatin
theanteriorchamber.Occupationalsafetyregulationshavedrastically
reducedtheincidenceofthistypeofcataract.
!Electricalinjury:Thisdensesubcapsularcataractcanbecausedby
lightningorhigh-voltageelectricalshock.
!Cataractfromionizingradiation:SeeChapter18.
7.4.6ToxicCataract
Steroidcataract.Prolongedtopicalorsystemictherapywithcorticosteroids
canresultinaposteriorsubcapsularopacity.Theexactdose-responserela-
tionshipisnotknown(Fig.7.14).
Othertoxiccataractscanresultfromchlorpromazine,mioticagents
(especiallycholinesteraseinhibitors),andbusulfan(Myleran)usedinthe
treatmentofchronicmyelocyticleukemia.
7.4.7CongenitalCataract
Therearemanycongenitalcataracts.Theyareeitherhereditaryoracquired
throughtheplacenta.
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201. 183
Cortisonecataract.
Fig.7.14Adensepumice-likeopacitydevelopsintheposteriorcapsulefollowing
prolongedsystemicsteroidtherapyforbronchialasthma.
7.4.7.1HereditaryCongenitalCataracts
Familialformsofcongenitalcataractsmaybeautosomaldominant,auto-
somalrecessive,sporadic,orX-linked.Theyareeasilydiagnosedonthebasis
oftheircharacteristicsymmetricmorphology.
Formsofhereditarycongenitalcataract:
Lamellarorzonularcataract.Opacitiesarelocatedinonelayeroflensfibers,
oftenas“riders”onlyintheequatorialregion(Fig.7.15).
Nuclearcataract.Thisisavariantofthelamellarcataractinwhichinitially
onlytheouterlayeroftheembryonicnucleusisaffected(Fig.7.16).
Coronarycataract.Thisischaracterizedbyfineradialopacitiesintheequa-
torialregion.
Ceruleancataract.Thisischaracterizedbyfineroundorclub-shapedblue
peripherallensopacities.
Mostfamiliallensopacitiesdonotimpairvisionandarenotprogressive.
Thisalsoappliestorarelensopacitiesinvolvingthecapsulesuchasanterior
andposteriorpolarcataracts,anteriorpyramidalcataract,andMittendorf’s
dot(remnantoftheembryonichyaloidarteryontheposteriorcapsuleofthe
lens;seeChapter11).
7.4Cataract
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202. 184
Lamellarcataract.
Fig.7.15Thelensopacities(“riders”)arelocatedinonlyonelayeroflensfibers,
oftenonlyintheequatorialregionasshownhere.
Nuclearcataract.
Fig.7.16Thisvariantofthelamellarcataractaffectsonlytheouterlayerofthe
embryonicnucleus,seenhereasasuturalcataract.
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203. 185
7.4.7.2CataractfromTransplacentalInfectionintheFirstTrimesterof
Pregnancy
AstatisticalstudybyPau(1986)citesthefollowingincidencesofcongenital
cataractwithrespecttosystemicdiseasecontractedbythemotherduringthe
firsttrimesterofpregnancy:
!Rubella40–60%.
!Mumps10–22%.
!Hepatitis16%.
!Toxoplasmosis5%.
Mostofthesecasesinvolvedtotalcataractsduetovirusinfectioncontracted
bythemotherduringearlypregnancy.Thisinfectionoccurredduringthefifth
toeighthweekofpregnancy,thephaseinwhichthelensdevelops.Because
theprotectivelenscapsulehasnotyetbeenformedatthistime,virusescan
invadeandopacifythelenstissue.
Themostfrequentcauseofcataractisarubellainfectioncontractedbythe
mother,whichalsoproducesotherdevelopmentalanomalies(Gregg’ssyn-
dromeinvolvinglensopacity,anopenductusarteriosus,andsensorineural
hearingloss).Thecataractisbilateralandtotalandmaybediagnosedbythe
presenceofleukocoria(whitepupil)andchorioretinalscarringsecondaryto
choroiditis.
7.4.8TreatmentofCataracts
7.4.8.1MedicalTreatment
Inspiteoftheoreticalapproachesinanimalresearch,theeffectivenessofcon-
servativecataracttreatmentinhumanshasnotbeendemonstrated.
Atpresenttherearenoavailableconservativemethodstoprevent,
delay,orreversethedevelopmentofacataract.Galactosemiccataracts
(seep.179)aretheonlyexceptiontothisrule.
7.4.8.2SurgicalTreatment
Cataractsurgeryisthemostfrequentlyperformedprocedureinophthal-
mology.
Whenissurgeryindicated?
Earliersurgicaltechniquesweredependentuponthematurityofthecataract.
Thisisnolongerthecaseinmoderncataractsurgery.
!Inthepresenceofbilateralcataracts,theeyewiththeworsevisualacuity
shouldundergosurgerywhenthepatientfeelsvisuallyhandicapped.
7.4Cataract
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204. 186
However,thisthresholdwillvarydependingonthepatient’soccupational
requirements.
!Inthepresenceofaunilateralcataract,thepatientisofteninclinedto
postponesurgeryaslongasvisioninthehealthyeyeissufficient.
!Inthepresenceofamaturecataract,itisimportanttoadvisethepatientto
undergosurgeryassoonaspossible.
Willtheoperationbesuccessful?
Theprospectofasuccessfuloutcomeisimportantforthepatient.Most
patientsdefineasuccessfuloutcomeintermsofasignificantimprovementin
vision.Therefore,itisimportantthatthepatientundergoesathoroughpre-
operativeeyeexaminationtoexcludeanyoculardisorders,asidefromthe
cataract,thatmayworsenvisualacuityandcompromisethesuccessofthe
cataractoperation.Suchdisordersincludeuncontrolledglaucoma,uveitis,
maculardegeneration,retinaldetachment,atrophyoftheopticnerve,and
amblyopia.
Adetailedhistoryofthepatient’sotheroculardisordersandvisionprior
todevelopmentofthecataractshouldbeobtainedbeforesurgery.
Severalmethodsaidinmakingaprognosiswithrespecttoexpectedvisual
acuity(retinalresolution)followingcataractsurgery.Theseinclude:
!Retinoscopytodeterminevisualacuity.
!Evaluationofthechoroidfigure(insevereopacificationssuchasamature
cataract).
Reliabilityofcataractsurgery
Cataractsurgeryisnowperformedasamicrosurgicaltechniqueunderan
operatingmicroscope.Moderntechniques,microsurgicalinstruments,atrau-
maticsuturematerial(30µmthinnylonsuturethread),andspeciallytrained
surgeonshavemadeitpossibletosuccessfullyperformcataractsurgery
withoutseriouscomplicationsin98%ofallpatients.Theprocedurelastsabout
30minutesand,likethepostoperativephase,ispainless.
Durationofhospitalization
Thepatientmaybehospitalizedfor3days,dependingontheadequacyof
postoperativecareathome.Olderpatientswholivealonemaybeunableto
careadequatelyforthemselvesandmaintaintheregimeofprescribedmedi-
cationsfortheoperatedeyeintheimmediatepostoperativephase.The
operationmaybeperformedasanoutpatientprocedureiftheophthalmolo-
gist’spracticeisabletoensureadequatecare.
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205. 187
Possibletypesofanesthesia
Cataractextractionmaybeperformedunderlocalanesthesiaorgeneral
anesthesia.Today,mostoperationsareperformedunderlocalanesthesia.
Asidefromthepatient’swishes,therearemedicalreasonsforpreferringone
formofanesthesiaoveranother:
Generalanesthesia:Thisisrecommendedforpatientswhoareextremely
apprehensiveandnervous,deaf,ormentallyretarded;itisalsoindicatedfor
patientswithParkinson’sdiseaseorrheumatism,whoareunabletoliestill
withoutpain.
Localanesthesia(retrobulbar,peribulbar,ortopicalanesthesia):Thisisrec-
ommendedforpatientswithincreasedanesthesiarisks.
Preoperativeconsultationregardingoptionsforachievingrefractive
correction(Table7.4)
Intraocularlens:In95–98%ofallcataractextractions,anintraocularlens
(IOL)isimplantedinplaceofthenaturallens(posteriorchamberlens).Aneye
withanartificiallensisreferredtoasapseudophakia.Thepowerofthelens
requiredisdeterminedpreoperativelybybiometry.TheIOLrefractivepower
isdeterminedbyultrasonicmeasurementofaxislength,IOLrefractioncon-
stants,andtherefractivepowerofthecornea.Therearetwotypesof
intraocularlenses:
!MonofocalIOLs.Thepatientcanselectwhetherthestrengthoftheartifi-
ciallensissuitablefordistancevisionornearvision.
!BifocalormultifocalIOLs.Theseallowcloseandremoteobjectstoappear
infocus.However,itshouldbenotedthatbifocalandmultifocallensesdo
notachievetheopticalimagingqualityofmonofocallenses.
Cataracteyeglasses:Thedevelopmentoftheintraocularlenshaslargely
supplantedcorrectionofpostoperativeaphakiawithcataractlenses.Longthe
standard,thismethodisnowonlynecessaryinexceptionalcases.Cataracteye-
glassescannotbeusedforcorrectingunilateralaphakiabecausethediffer-
enceinthesizeoftheretinalimagesistoogreat(aniseikonia).Therefore,cat-
aracteyeglassesareonlysuitableforcorrectingbilateralaphakia.Cataract
eyeglasseshavethedisadvantageoflimitingthefieldofvision(peripheraland
ringscotoma).
Contactlenses(soft,rigid,andoxygen-permeable):Theselensespermita
nearnormalfieldofvisionandaresuitableforpostoperativecorrectionofuni-
lateralcataractsasthedifferenceinimagesizeisnegligible.However,many
olderpatientshavedifficultylearninghowtocopewithcontactlenses.
7.4Cataract
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206. 188
Table7.4Comparisonofnormaleye(1),correctionofcataractwithposteriorcham-
berintraocularlens(2),contactlens(3),cataracteyeglasses(4)
Monocular
image size
Binocular vision:
combination
Advantage/
disadvantage
Correction
5
26 4
4 8 7 6
4 5 3 1
9
2
8
7
5
5 3 1 8
1 4 9 3
3 6 4 1
9 6 5 2
5
26 4
4 8 7 6
4 5 3 1
9
2
8
7
5
5 3 1 8
1 4 9 3
3 6 4 1
9 6 5 2
5
26 4
4 8 7 6
4 5 3 1
9
2
8
7
5
5 3 1 8
1 4 9 3
3 6 4 1
9 6 5 2
5
26 4
4 8 7 6
4 5 3 1
9
2
8
7
5
5 3 1 8
1 4 9 3
3 6 4 1
9 6 5 2
Normal
2% larger
than 1
8–10% larger
than 1
25% larger
than 1
3
1 can be combined
with and .
Difference in image
size is small enough
for the brain to fuse
the images.
can be combined
with , , and
.
32
Normal eye
Posterior chamber
IOL
Contact lens
Cataract eyeglasses
4
2
1
Field of vision: full
Normal vision
Field of vision: full
IOL: No care
necessary
Visual acuity: (even
without eye-
glasses) good
visual acuity,
good orientation
Field of vision: full
Contact lenses:
Care and handling
often difficult for
older patients
Visual acuity: Good
with contact lenses,
poor orientation
without them.
Irritation possible;
dry eyes preclude
contact lenses.
Field of vision:
Limited (peripheral
scotoma)
Cataract eyeglasses:
Simple to use,
heavy, unsatis-
factory cosmetic
appearance
Visual acuity: good
with eyeglasses,
poor orientation
without eyeglasses
2
12
3
can be combined
with , , and
.
3
12
3
can only be
combined with .
4
4
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207. 189
Intracapsularcataractextraction.
Fig.7.17The
lensisfrozenin
itscapsulewitha
cryophakeand
removedfrom
theeyethrougha
largesuperior
cornealincision.
Thephotograph
isfromthesur-
geon’sperspec-
tive.
SurgicalTechniques
Theoperationisperformedononlyoneeyeatatime.Theprocedureonthe
felloweyeisperformedafteraboutaweekifoncethefirsteyehasstabilized.
Historicalmilestones:
!Couching(reclination):For2000yearsuntilthe19thcentury,apointed
instrumentwasusedtodisplacethelensintothevitreousbodyoutofthe
visualaxis.
!1746:J.Davielperformedthefirstextracapsularcataractextractionby
removingthecontentsofthelensthroughaninferiorapproach.
!1866:A.vonGraefeperformedthefirstremovalofacataractthrougha
superiorlimbalincisionwithcapsulotomy.
Intracapsularcataractextraction:Untilthemid1980s,thiswasthemethodof
choice.Todayintracapsularcataractextractionisusedonlywithsubluxation
ordislocationofthelens.Theentirelensisfrozeninitscapsulewithacryo-
phakeandremovedfromtheeyethroughalargesuperiorcornealincision
(Fig.7.17).
Extracapsularcataractextraction:Procedure(Figs.7.18a–c):Theanterior
capsuleisopened(capsulorrhexis).Thenonlythecortexandnucleusofthe
lensareremoved(extracapsularextraction);theposteriorcapsuleand
zonulesuspensionremainintact.Thisprovidesastablebaseforimplanta-
tionoftheposteriorchamberintraocularlens.
Extracapsularcataractextractionwithimplantationofaposterior
chamberintraocularlensisnowthemethodofchoice.
7.4Cataract
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208. 190
Extracapsularcataractextraction.
Fig.7.18aThe
anteriorcapsuleof
thelensisopened
withacontinuous
curvilinearcap-
sulorrhexis.
bThenucleusisde-
stroyedbyultra-
sound(phacoemul-
sification),andthe
fragmentsofthe
nucleusandcortex
areaspirated.
cAposteriorcham-
berintraocularlens
isimplantedinthe
capsularbag.
7Lens
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209. 191
Todayphacoemulsification(emulsifyingandaspiratingthenucleusofthelens
withahigh-frequencyultrasonicneedle)isthepreferredtechniquefor
removingthenucleus.Wherethenucleusisveryhard,theentirenucleusis
expressedoraspirated.Thenthesofterportionsofthecortexareremovedby
suctionwithanaspirator/irrigatorattachmentinanaspiration/irrigation
maneuver.Theposteriorcapsuleisthenpolished,andanintraocularlens
(IOL)isimplantedintheemptycapsularbag(Fig.7.19aandb).Phacoemulsi-
ficationandIOLimplantationrequireanincisiononly3–6mminlength.
Whereatunneltechniqueisusedtomakethisincision,nosuturewillbenec-
essaryasthewoundwillcloseitself.
Patientwithposteriorchamberintraocularlens.
Fig.7.19aThe
IOLisnotnotice-
ableinanormal
pupilthatisnot
underthein-
fluenceofmedi-
cation.
bThesame
patientafterdila-
tionofthepupils
withamydriatic.
TheIOLisdis-
cernibleunder
retroillumination.
7.4Cataract
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210. 192
Advantagesoverintracapsularcataractextraction.Extracapsularcataract
extractionusuallydoesnotachievethesamebroadexposureoftheretina
thatintracapsularcataractextractiondoes,particularlywhereasecondary
cataractispresent.However,theextracapsularcataractextractionmaintains
theintegrityoftheanteriorandposteriorchambersoftheeye,andthevit-
reousbodycannotprolapseanteriorlyasafterintracapsularcataractextrac-
tion.At0.1–0.2%,theincidenceofretinaldetachmentafterextracapsularcat-
aractextractionisabouttentimeslessthanafterintracapsularcataract
extraction,whichhasanincidenceof2–3%.
7.4.8.3SecondaryCataract
Epidemiology:Approximately30%ofallcataractpatientsdevelopasecond-
arycataractafterextracapsularcataractextraction.
Etiology:Extracapsularcataractextractionremovesonlytheanteriorcentral
portionofthecapsuleandleavesepithelialcellsofthelensintactalongwith
remnantsofthecapsule.Theseepithelialcellsarecapableofreproducingand
canproduceasecondarycataractoffibrousorregenerativetissueinthepos-
teriorcapsulethatdiminishesvisualacuity(Fig.7.20a).
Treatment:Aneodymium:yttrium-aluminum-garnet(Nd:YAG)lasercan
incisetheposteriorcapsuleinthevisualaxiswithoutrequiringinvasiveeye
surgery.Thisimmediatelyimprovesvision(Fig.7.20b).
7.4.8.4SpecialConsiderationsinCataractSurgeryinChildren
Observechangesinthechild’sbehavior:Childrenwithcongenital,trau-
matic,ormetaboliccataractwillnotnecessarilycommunicatetheirvisual
impairmentverbally.However,itcanbediagnosedfromthesesymptoms:
!Leukocoria.
!Oculodigitalphenomenon:Thechildpresseshisorherfingeragainstthe
eyeoreyesbecausethiscanproducelightpatternsthechildfindsinterest-
ing.
!Strabismus:thefirstsignofvisualimpairment(Fig.7.21).
!Thechildcrieswhenthenormaleyeiscovered.
!Thechildhasdifficultywalkingorgrasping.
!Erraticeyemovementispresent.
!Nystagmus.
Operateasearlyaspossible:Retinalfixationandcorticalvisualresponses
developwithinthefirstsixmonthsoflife.Thismeansthatchildrenwho
undergosurgeryaftertheageofoneyearhavesignificantlypoorerchancesof
developingnormalvision.
7Lens
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211. 193
Secondarycataract.
Fig.7.20aRegenerativesecondarycataractsleadtodiminishedvisualacuityand
increasedglare.bNd:YAGlasercapsulotomy:theposteriorcapsulotomyremoves
theobstructionofthevisualaxis,andimmediatelyimprovesvision.
Childrenwithcongenitalcataractshouldundergosurgeryasearlyas
possibletoavoidamblyopia.
Theprognosisforsuccessfulsurgeryislessfavorableforunilateralcataracts
thanforbilateralcataracts.Thisisbecausetheamblyopiaofthecataracteye
putsitatanirreversibledisadvantageincomparisonwiththefelloweyeas
thechildlearnshowtosee.
Planforthefuturewhenperformingsurgery:Afteropeningtheextremely
elasticanteriorlenscapsule,onecanaspiratethesoftinfantilecortexand
7.4Cataract
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212. 194
Congenitalcataract.
Fig.7.21One-
and-one-half-
year-oldchild
withcongenital
cataract(leuko-
coria)andeso-
tropiaoftheright
eye.
nucleus.Secondarycataractsarefrequentcomplicationsininfants.Therefore,
theprocedureshouldincludeaposteriorcapsulotomywithanteriorvit-
rectomytoensureanunobstructedvisualaxis.Theoperationpreservesthe
equatorialportionsofthecapsuletopermitsubsequentimplantationofapos-
teriorchamberintraocularlensinlateryears.
Refractionchangesconstantly:Therefractivepoweroftheeyechanges
dramaticallywithinashortperiodoftimeastheeyegrows.Therefractionin
theeyeofanewbornis30–35dioptersanddropsto15–25diopterswithin
thefirstyearoflife.Refractivecompensationforaunilateralcataractis
achievedwithasoftcontactlens(Fig.7.22).Theuseofsoftcontactlensesin
infantsisdifficultandrequirestheparents’intensivecooperation.Refractive
correctionofbilateralcataractsisachievedwithcataracteyeglasses.
Refractionshouldbeevaluatedbyretinoscopy(seeChapter16)every
twomonthsduringthefirstyearoflifeandeverythreetofourmonths
duringthesecondyear,andcontactlensesandeyeglassesshouldbe
changedaccordingly.
Implantationofposteriorchamberintraocularlensesforcongenitalcata-
ractisnotyetrecommendedinchildrenunderthreeyearsofage.Thisis
becauseexperiencewiththeposteriorchamberintraocularlensandpresent
follow-upperiodsaresignificantlylessthanthelifeexpectancyofthe
children.Inaddition,thereisnowaytoadapttherefractivepowerofthelens
tochangingrefractionoftheeyeasthechildgrows.
Orthopticpostoperativetherapyisrequired:Unilateralcataractsinpartic-
ularrequireorthopticpostoperativetherapyintheoperatedeyetoclosethe
7Lens
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213. 195
Refractivecompensationwithsoftcontactlens.
Fig.7.22Ina
unilateralcata-
ract,acontact
lensprovidere-
fractivecompen-
sation(thear-
rowsindicatethe
edgeofthecon-
tactlens).
gapwithrespecttothenormalfelloweye.Regularevaluationofretinalfixa-
tionisindicated,asisamblyopiatreatment(seepatching).
7.5 LensDislocation
Definition
!Subluxation(partialdislocation):Thesuspensionofthelens(thezonule
fibers)isslackened,andthelensisonlypartiallywithinthehyaloidfossa
(Fig.7.23).
!Luxation(completedislocation):Thelensistorncompletelyfreeand
hasmigratedintothevitreousbodyor,lessfrequently,intotheanterior
chamber.
Etiology:Thereareseveralcausesoflensdislocation(Table7.5).Most
frequently,itisduetotrauma(seecontusionoftheeyeball).Laterinlife,
pseudoexfoliationmayalsoleadtosubluxationorluxationofthelens.
Hereditarycausesandmetabolicdiseaseproducelensdisplacementearly
yetonthewholearerare.Additionalrarecausesincludehyperlysinemia
(characterizedbyretardedmentaldevelopmentandseizures)andsulfiteoxi-
dasedeficiency(whichleadstomentalretardationandexcretionofcysteine
intheurine).
ThemostfrequentatraumaticcausesoflensdislocationareMarfan’s
syndrome,homocystinuria,andWeill-Marchesanisyndrome.
7.5LensDislocation
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214. 196
Table7.5Etiologyoflensdisplacement
Causes Lensdisplacement
!Hereditarycauses(rare)
–Ectopialentis:isolatedandmono-
symptomatic.
–Completeorpartialdisplacementof
thelens(forexample,intotheante-
riorchamber).
–Marfan’ssyndrome:characterized
byarachnodactyly,longlimbs,and
laxnessofjoints.
–Lensisabnormallyround;lensdis-
placementisusuallysuperiorand
temporal;zonulefibersareelon-
gatedbutfrequentlyintact.
–Weill-Marchesanisyndrome:symp-
tomsincludeshortstatureand
brachydactyly.
–Lensisabnormallyroundandoften
toosmall;lensisusuallyeccentric
anddisplacedinferiorly.
–Homocystinuria(metabolicdis-
ease):characterizedbyoligo-
phrenia,osteoporosis,andskeletal
deformities.
–Lensdisplacementisusuallymedial
andinferior;tornzonulefibers
appearasa“permanentwave”on
thelens.
!Acquiredcauses
–Trauma(probablythemost
frequentcause).
–Zonuledefectsduetodeformation
cancausesubluxationorluxationof
thelens.
–Pseudoexfoliation(inadvanced
age).
–Zonuleweaknessduetolooseningof
theinsertionofthefibersonthelens
cancauselensdisplacement.
–Ciliarybodytumor(rare). –Lensisdisplacedbytumor.
–Largeeyeswithseveremyopiaand
buphthalmos(rare).
–Zonuledefectsduetoexcessive
longitudinalgrowthcancauselens
displacement.
Symptoms:Slightdisplacementmaybeofnofunctionalsignificancetothe
patient.Morepronounceddisplacementproducessevereopticaldistortion
withlossofvisualacuity.
Diagnosticconsiderations:Cardinalsymptomsincludetremulousmotionof
theirisandlenswhentheeyemoves(iridodonesisandphacodonesis).These
symptomsaredetectableunderslit-lampexamination.
Treatment:Opticalconsiderations(seesymptoms)andtheriskofsecondary
angleclosureglaucomafromprotrusionoftheirisanddislocationofthelens
intotheanteriorchamberareindicationsforremovalofthelens.
7Lens
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215. 197
SubluxationofthelensinMarfan’ssyndrome.
Fig.7.23The
lensisdisplaced
superiorlyand
medially.Asthe
zonulefibersare
intact,acertain
measureofac-
commodationis
stillpossible.
7.5LensDislocation
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217. 199
8 UvealTract
(Vascularpigmentedlayer)
GabrieleE.LangandGerhardK.Lang
8.1 BasicKnowledge
Structure:Theuvealtract(alsoknownasthevascularpigmentedlayer,
vasculartunic,anduvea)takesitsnamefromtheLatinuva(grape)because
thedarkpigmentationandshapeofthestructurearereminiscentofagrape.
Theuvealtractconsistsofthefollowingstructures:
!Iris,
!Ciliarybody,
!Choroid.
Position:Theuvealtractliesbetweenthescleraandretina.
Neurovascularsupply:Arterialsupplytotheuvealtractisprovidedbythe
ophthalmicartery.
!Theshortposteriorciliaryarteriesentertheeyeballwiththeopticnerve
andsupplythechoroid.
!Thelongposteriorciliaryarteriescoursealongtheinteriorsurfaceofthe
scleratotheciliarybodyandtheiris.Theyformthemajorarterialcircleat
therootoftheirisandtheminorarterialcircleinthecollaretteoftheiris.
Theanteriorciliaryarteriesoriginatefromthevesselsoftherectusmuscles
andcommunicatewiththeposteriorciliaryvessels.
Venousblooddrainsthroughfourtoeightvorticoseorvortexveinsthat
penetratethescleraposteriortotheequatorandjointhesuperiorandinferior
ophthalmicveins(Fig.8.1).Sensorysupplyisprovidedbythelongandshort
ciliarynerves.
8.1.1 Iris
Structureandfunction:Theirisconsistsoftwolayers:
!Theanteriormesodermalstromallayer.
!Theposteriorectodermalpigmentedepitheliallayer.
Theposteriorlayerisopaqueandprotectstheeyeagainstexcessiveincident
light.Theanteriorsurfaceofthelensandthepigmentedlayeraresoclose
togethernearthepupilthattheycaneasilyformadhesionsininflammation.
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218. 200
Vascularsupplytotheuvealtract.
Minor arterial circle of the iris
(collarette of the iris)
Major arterial circle
of the iris
Anterior ciliary artery
Vorticose vein
Long posterior
ciliary artery
Short posterior
ciliary artery
Fig.8.1Seediscussionintext.
Thecollaretteoftheiriscoveringtheminorarterialcircleoftheiris
dividesthestromaintopupillaryandciliaryportions.Thepupillaryportion
containsthesphinctermuscle,whichissuppliedbyparasympatheticnerve
fibers,andthedilatorpupillaemuscle,suppliedbysympatheticnervefibers.
Thesemusclesregulatethecontractionanddilationofthepupilsothatthe
irismayberegardedastheapertureoftheopticalsystemoftheeye.
Pupildilationissometimessluggishinpreterminfantsandthenewborn
becausethedilatorpupillaemuscledevelopsrelativelylate.
Surface:Thenormalirishasarichlytexturedsurfacestructurewithcrypts
(tissuegaps)andinterlinkedtrabeculae.Afadedsurfacestructurecanbea
signofinflammation(seeiridocyclitis).
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219. 201
Color:Thecoloroftheirisvariesintheindividualaccordingtothemelanin
contentofthemelanocytes(pigmentcells)inthestromaandepitheliallayer.
Eyeswithahighmelanincontentaredarkbrown,whereaseyeswithless
melaninaregrayish-blue.Caucasiansatbirthalwayshaveagrayish-blueiris
asthepigmentedlayeronlydevelopsgraduallyduringthefirstyearoflife.
Eveninalbinos(seeimpairedmelaninsynthesis),theeyeshaveagrayish-
blueirisbecauseofthemelanindeficiency.Underslitlampretroillumination
theyappearreddishduetothefundusreflex.
8.1.2 CiliaryBody
Positionandstructure:Theciliarybodyextendsfromtherootoftheiristo
theoraserrata,whereitjoinsthechoroid.Itconsistsofanteriorparsplicata
andtheposteriorparsplana,whichlies3.5mmposteriortothelimbus.
Numerousciliaryprocessesextendintotheposteriorchamberoftheeye.The
suspensoryligament,thezonule,extendsfromtheparsplanaandtheinter-
valsbetweentheciliaryprocessestothelenscapsule.
Function:Theciliarymuscleisresponsibleforaccommodation.Thedouble-
layeredepitheliumcoveringtheciliarybodyproducestheaqueoushumor.
8.1.3 Choroid
Positionandstructure:Thechoroidisthemiddletunicoftheeyeball.Itis
boundedontheinteriorbyBruch’smembrane.Thechoroidishighlyvascu-
larized,containingavessellayerwithlargebloodvesselsandacapillarylayer.
Thebloodflowthroughthechoroidisthehighestintheentirebody.
Function:Thechoroidregulatestemperatureandsuppliesnourishmentto
theouterlayersoftheretina.
8.2 ExaminationMethods
Theslitlampisusedtoexaminethesurfaceoftheirisunderafocusedbeam
oflight.Normallynovesselswillbevisible.
Irisvesselsareonlyvisibleinatrophyoftheiris,inflammation,oras
neovascularizationinrubeosisiridis(seeFig.8.12).
Wherevesselsarepresent,theycanbevisualizedbyirisangiographyafter
intravenousinjectionoffluoresceinsodiumdye.
Defectsinthepigmentedlayeroftheirisappearredunderretroillumina-
tionwithaslitlamp(seeFig.8.6).Slitlampbiomicroscopyvisualizesindi-
vidualcellssuchasmelanincellsat40-powermagnification.
Theanteriorchamberisnormallytransparent.Inflammationcanincrease
thepermeabilityofthevesselsoftheirisandcompromisethebarrier
8.2ExaminationMethods
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220. 202
betweenbloodandaqueoushumor.Opacificationoftheaqueoushumorby
proteinsmaybeobservedwiththeaidofaslitlampwhentheeyeisillumi-
natedwithalateralfocalbeamoflight(Tyndalleffect).Thismethodcanalso
beusedtodiagnosecellsintheanteriorchamberinthepresenceofinflamma-
tion.
Directinspectionoftherootoftheirisisnotpossiblebecauseitdoesnot
liewithinthelineofsight.However,itcanbeindirectlyvisualizedbygonios-
copy.Inspectionoftheposteriorportionoftheparsplanarequiresathree-
mirrorlens.Theglobeisalsoindentedwithametalrodtopermitvisualization
ofthispartoftheciliarybody(forexampleinthepresenceofasuspected
malignantmelanomaoftheciliarybody).
Thepigmentedepitheliumoftheretinapermitsonlylimitedevaluationof
thechoroidbyophthalmoscopyandfluoresceinangiographyorindocyanine
greenangiography.Changesinthechoroidsuchastumorsorhemangiomas
canbevisualizedbyultrasoundexamination.Whereatumorissuspected,
transilluminationoftheeyeisindicated.Afteradministrationoftopicalanes-
thesia,afiberopticlightsourceisplacedontheeyeballtovisualizetheshadow
ofthetumorontheredofthefundus.
8.3 DevelopmentalAnomalies
8.3.1 Aniridia
Aniridiaistheabsenceoftheiris.Thisgenerallybilateralconditionistrans-
mittedasanautosomaldominanttraitoroccurssporadically.Aniridiamay
alsobetraumaticandcanresultfrompenetratinginjuries.However,periph-
eralremnantsoftheirisareusuallystillpresentsothatciliaryvilliandzonule
fiberswillbevisualizedunderslit-lampexamination(Fig.8.2).
Insporadicaniridia,aWilms’tumorofthekidneyshouldbeexcluded.
Visionisseverelycompromisedasaresultofthefovealhypoplasia.Thedis-
orderisfrequentlyassociatedwithnystagmus,amblyopia,buphthalmos,and
cataract.
Visualacuitywillgenerallybereducedinthepresenceofnystagmus.
8UvealTract(Vascularpigmentedlayer)
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221. 203
Aniridia.
Fig.8.2Thecili-
aryvilli(arrow)
andthelensare
visibleunderslit-
lampretroillumi-
nation.
8.3.2Coloboma
Anothercongenitalanomalyresultsfromincompletefusionoftheembry-
onicopticcup,whichnormallyoccursinaboutthesixthweekofpregnancy.
Theseanomaliesareknownascolobomas.Theyaredirectedmediallyand
inferiorlyandcaninvolvetheiris(Fig.8.3),ciliarybody,zonulefibers,
choroid,andopticnerve(Fig.8.4).Bridgecolobomasexhibitremnantsofthe
irisorchoroid.Involvementofthechoroidandopticnervefrequentlyleadsto
reducedvisualacuity.
Surgicaliriscolobomasincataractandglaucomasurgeryareusually
openedsuperiorly.Inthismanner,theyarecoveredbytheuppereyelidsothe
patientwillnotusuallyexperienceblindingglare.
Traumaticiriscolobomasarecausedbyavulsionoftheiris(iridolysis;
Fig.8.5).
8.3DevelopmentalAnomalies
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222. 204
Congenitaliriscoloboma.
Fig.8.3The
congenitaliris
colobomaislo-
catedmedially
andinferiorly.
Thepupilmerges
withthecolo-
bomawithout
anysharpdemar-
cation.
Colobomaoftheretina,choroid,andopticnerve.
Fig.8.4The
colobomaofthe
retina,choroid,
andopticnerve
exposestheun-
derlyingwhite
sclera.
8UvealTract(Vascularpigmentedlayer)
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223. 205
Variousirischanges.
Congenital medial and
inferior iris coloboma
Traumatic iris
avulsion (iridolysis)
Cloverleaf pupil due
to posterior synechiae
Surgical basal
iris coloboma
Surgical segmental
iris coloboma
Ando's surgical
iridectomy
Fig.8.5Seediscussiononp.203.
8.3DevelopmentalAnomalies
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224. 206
8.4 PigmentationAnomalies
8.4.1 Heterochromia
Impaireddevelopmentofthepigmentationoftheiriscanleadtoacongenital
differenceincolorationbetweentheleftandrightiris(heterochromia).
Oneiriscontainingvaryingpigmentationisreferredtoasirisbicolor.Iso-
latedheterochromiaisnotnecessarilyclinicallysignificant(simplehetero-
chromia),yetitcanbeasignofabnormalchanges.Thefollowingtypesare
differentiated:
!Fuchs’heterochromiccyclitis(etiologyunclear):Thisreferstorecurrent
iridocyclitis(simultaneousinflammationofseveralportionsoftheuveal
tract)inadults,withprecipitatesontheposteriorsurfaceofthecornea
withoutformationofposteriorsynechiae(adhesionsbetweentheirisand
lens).Theeyeisfreeofexternalirritation.Thisdisorderisoftenassociated
withcomplicatedcataractandincreasedintraocularpressure(glaucoma).
!Sympatheticheterochromia:Inunilateralimpairmentofthesympathetic
nervesupply,theaffectedirisissignificantlylighter.Heterochromiawith
unilaterallylighterpigmentationoftheirisalsooccursiniridocyclitis,
acuteglaucoma,andanteriorchamberhemorrhage(hyphema).
!Melanosisoftheiris:Thisreferstodarkpigmentationofoneiris.
Asidefromthedifferenceincolorationbetweenthetwoirises,neithersym-
patheticheterochromianormelanosisleadstofurthersymptoms.Theonly
formofheterochromiathatleadstoabnormalchangesisFuchs’hetero-
chromiccyclitis.Thepossiblecomplicationsinvolvedrequirespecifictreat-
ment.
8.4.2Albinism
Albinism(fromtheLatinalbus=white)isacongenitalmetabolicdisease
thatleadstohypopigmentationoftheeye.Thefollowingtypesaredifferen-
tiated:
!ocularalbinism(involvingonlytheeyes)and
!oculocutaneousalbinism(involvingtheeyes,skin,andhair).
Inalbinismtheirisislightbluebecauseofthemelanindeficiencyresulting
fromimpairedmelaninsynthesis.Underslit-lampretroillumination,theiris
appearsreddishduetofundusreflex(Fig.8.6).Ophthalmoscopywilldetect
choroidalvessels(Fig.8.7).Associatedfovealaplasiaresultsinsignificant
reductioninvisualacuityandnystagmus.Mostpatientsarealsophotophobic
becauseofthemissingfilterfunctionofthepigmentedlayeroftheiris.
8UvealTract(Vascularpigmentedlayer)
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225. 207
Ocularalbinism.
Fig.8.6Thepe-
ripheralirisap-
pearsredunder
retroillumination.
Fundusinocularalbinism.
Fig.8.7Typical
featuresinclude
thechoroidalves-
sels,whichare
visualizedbyoph-
thalmoscopy
(choroidalvessel,
thickarrowhead;
retinalvessel,
arrow).
8.4PigmentationAnomalies
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226. 208
8.5 Inflammation
Inflammationsoftheuvealtractareclassifiedaccordingtothevariouspor-
tionsoftheglobe:
!Anterioruveitis(iritis).
!Intermediateuveitis(cyclitis).
!Posterioruveitis(choroiditis).However,someinflammationsinvolvethe
middleportionsoftheuvealtractsuchasiridocyclitis(inflammationof
theirisandciliarybody)orpanuveitis(inflammationinvolvingallseg-
ments).
8.5.1 AcuteIritisandIridocyclitis
Epidemiology:Iritisisthemostfrequentformofuveitis.Itusuallyoccursin
combinationwithcyclitis.Aboutthree-quartersofalliridocyclitiscaseshave
anacuteclinicalcourse.
Etiology:Iridocyclitisisfrequentlyattributabletoimmunologiccausessuch
asallergicorhyperergicreactiontobacterialtoxins.Insomerheumaticdis-
ordersitisknowntobefrequentlyassociatedwiththeexpressionofspecific
humanleukocyteantigens(HLA)suchasHLA-B27.Iridocyclitiscanalsobea
symptomofsystemicdiseasesuchasankylosingspondylitis,Reiter’ssyn-
drome,sarcoidosis,etc.(Table8.1).Infectionsarelessfrequentandoccursec-
ondarytopenetratingtraumaorsepsis(bacteria,viruses,mycosis,orpara-
sites).Phacogenicinflammation,possiblywithglaucoma,canresultwhenthe
lensbecomesinvolved.
Symptoms:Patientsreportdullpainintheeyeorforeheadaccompaniedby
impairedvision,photophobia,andexcessivetearing(epiphora).
Incontrasttochoroiditis,acuteiritisoriridocyclitisispainfulbecauseof
theinvolvementoftheciliarynerves.
Diagnosticconsiderations:Typicalsignsinclude:
!Ciliaryinjection:Theepiscleralandperilimbalvesselsmayappearblue
andred.
!Combinedinjection:Theconjunctivaisalsoaffected.
Theirisishyperemic(theirisvesselswillbevisibleinalight-colorediris).
Thestructureappearsdiffuseandreactivemiosisispresent.
Visionisimpairedbecauseofcellularinfiltrationoftheanteriorchamber
andproteinorfibrinaccumulation(visibleasaTyndalleffect).Theprecipi-
tatesaccumulateontheposteriorsurfaceofthecorneainatriangularcon-
figurationknownasArlt’striangle.Exudateaccumulationonthefloorofthe
anteriorchamberisreferredtoashypopyon(Fig.8.8).Viralinfectionsmaybe
accompaniedbybleedingintotheanteriorchamber(hyphema;Fig.8.9).Cor-
nealedemacanalsodevelopinrarecases.
8UvealTract(Vascularpigmentedlayer)
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227. 209
Table8.1Causesofuveitisaccordingtolocation
Formofuveitis Possiblecauses
HLA-B27-associatedirido-
cyclitis
!Idiopathic
!Ankylosingspondylitis
!Reiter’ssyndrome
!Regionalenteritis
!Ulcerativecolitis
!Psoriasis
Non-HLA-B27-associated
iridocyclitis
!Idiopathic
!Viral
!Tuberculosis
!Sarcoidosis
!Syphilis
!Leprosy
!Rheumatoidarthritis(Still-Chauffardsyndrome)
!Heterochromiccyclitis
!Phacogenicuveitis
!Trauma
Iridocyclitisandchoroiditis !Toxoplasmosis
!Sarcoidosis
!Tuberculosis
!Syphilis
!Behçet’sdisease
!Sympatheticophthalmia
!Borreliosis
!Brucellosis
!Yersiniosis
!Listeriosis
!Malignanttumors
Choroiditis !Toxoplasmosis
!Sarcoidosis
!Syphilis
!Behçet’sdisease
!Histoplasmosis
!Toxocara
8.5Inflammation
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228. 210
Hypopyoninacuteiridocyclitis.
Fig.8.8The
purulentexudate
accumulatesasa
poolonthefloor
oftheanterior
chamber.
Hyphema.
Fig.8.9Bleed-
ingintotheante-
riorchambercan
occurinrubeosis
iridis,trauma,or,
inrarecases,ir-
idocyclitis.
CornealedemasandTyndalleffects(accumulationsofproteininthe
anteriorchamber)canbediagnosedwhentheeyeisilluminatedwitha
lateralbeamoflightfromafocusedlightorslitlamp.
Differentialdiagnosis:SeeTable8.2.
Inacuteiritis,thedepthoftheanteriorchamberisnormalandreactive
miosisispresent.Incontrast,inacuteglaucomatheanteriorchamberis
shallowandthepupilisdilated(Table8.2).
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229. 211
Table8.2Differentialdiagnosisofiritisandacuteglaucoma
Differentialcriteria Acuteiritis Acuteglaucoma
Symptoms DullpainandphotophobiaIntensepainandvomiting
Conjunctiva Combinedinjection Combinedinjection
Cornea Clear Opacified,edematous
Anteriorchamber Normaldepth;cellsand
fibrinarepresent
Shallow
Pupil Narrowed(reactivemiosis)Dilated,notround
Globe Normalpressure Rockhard
Complications:Theseinclude:
!Secondaryopenangleglaucomawithanincreaseinintraocularpressure.
!Adhesionsbetweentheirisandposteriorsurfaceofthecornea(anterior
synechiae).
!Adhesionsbetweentheirisandlens(posteriorsynechiae;Fig.8.10).
Treatment:Topicaland,inappropriatecases,systemicantibioticorantiviral
therapyisindicatedforiridocyclitisduetoapathogen(withacornealulcer,
penetratingtrauma,orsepsis).
Posteriorsynechiaesecondarytoiridocyclitis(cloverleafpupil).
Fig.8.10Acute
iridocyclitispro-
ducesadhesions
betweentheiris
andlens(seealso
Fig.8.5).
8.5Inflammation
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230. 212
Aconjunctivalsmear,orabloodcultureinsepticcases,isobtainedto
identifythepathogen.Antibiotictherapyshouldbeginimmediatelyas
microbiologicalidentificationofthepathogenisnotalwayssuccessful.
Therapeuticmydriasisincombinationwithsteroidtherapyisindicatedto
minimizetheriskofsynechiae.
Wherenopathogencanbeidentified,high-dosetopicalsteroidtherapy
(prednisoloneeyedropseveryhourincombinationwithsubconjunctival
injectionsofsolubledexamethasone)isadministered.Tominimizetheriskof
posteriorsynechiae,thepupilmustbemaximallydilated(atropine,
scopolamine,cyclopentolate,andpossiblyepinephrineandepinephrineeye-
drops).
Themydriaticeffectofdilatingeyedropsmaybereducediniritis.This
maynecessitatetheuseoflonger-actingmedicationssuchasatropine,
whichmayhavetobeappliedseveraltimesdaily.
Occasionallyitispossibletobreakoffexistingsynechiaeinthismanner,and
patchesofiristissuewillremainontheanteriorsurfaceofthelens.Second-
aryopenangleglaucomaistreatedbyadministeringbetablockersineye-
dropformand,inapplicablecases,carbonicanhydraseinhibitors(aceta-
zolamide;seeTable10.3).
Prognosis:Symptomsusuallyimprovewithinafewdayswhenproperther-
apyisinitiated.Thedisordercanprogresstoachronicstage.
8.5.2ChronicIritisandIridocyclitis
Epidemiology:Aboutonequarterofalliridocyclitiscaseshaveachronic
clinicalcourse.
Etiology:SeeTable8.1.
Symptoms:Seeacuteiridocyclitis.Chroniciridocyclitismayexhibitminimal
symptoms.
Diagnosticconsiderations:Seeacuteiridocyclitis.
Differentialdiagnosis:Thedisordershouldbedistinguishedfromacute
glaucoma,conjunctivitis,andkeratitis.
Complications:Totalobliterationofthepupilbyposteriorsynechiaeis
referredtoapupillaryblock.Becausetheaqueoushumorcannolongercircu-
late,secondaryangleclosureglaucomawithirisbombéoccurs.Occlusionof
thepupilalsoresultsinfibrousscarringinthepupil.Thiscanleadtothe
developmentofposteriorsubcapsularopacitiesinthelens(secondarycata-
ract).Recurrentiridocyclitiscanalsoleadtocalcificbandkeratopathy.
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231. 213
Treatment:Inpupillaryblockwithasecondaryangleclosureglaucoma,a
Nd:YAGlaseriridotomymaybeperformedtocreateashunttoallowthe
aqueoushumorfromtheposteriorchambertocirculateintotheanterior
chamber.Inthepresenceofasecondarycataract,acataractextractionmay
beperformedwhentheinflammationhasabated.
Prognosis:Becauseofthechronicrecurrentcourseofthedisorder,it
frequentlyinvolvescomplicationssuchassynechiaeorcataractthatmay
progresstoblindnessfromshrinkageoftheeyeball.
8.5.3Choroiditis
Epidemiology:Therearefewepidemiologicstudiesofchoroiditis.The
annualincidenceisassumedtobefourcasesper100000people.
Etiology:SeeTable8.1.
Symptoms:Patientsarefreeofpain,althoughtheyreportblurredvisionand
floaters.
Choroiditisispainlessasthechoroidisdevoidofsensorynervefibers.
Diagnosticconsiderations:Ophthalmoscopyrevealsisolatedormultiple
choroiditisfoci.Inacutediseasetheyappearasill-definedwhitedots
(Fig.8.11).Oncescarringhasoccurredthefociaresharplydemarcatedwitha
yellowish-browncolor.Occasionallythemajorchoroidalvesselswillbevis-
iblethroughtheatrophicscars.
Multifocalchoroiditis.
Fig.8.11The
fociofacutein-
flammationare
yellowishandill-
defined;olderle-
sionsareyel-
lowish-brownand
sharplydemar-
cated.
8.5Inflammation
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232. 214
Nocellswillbefoundinthevitreousbodyinaprimarychoroidalprocess.
However,inflammationproceedingfromtheretina(retinochoroiditis)will
exhibitcellularinfiltrationofthevitreousbody.
Differentialdiagnosis:Thisdisordershouldbedistinguishedfromretinal
inflammations,whichareaccompaniedbycellularinfiltrationofthevitreous
bodyandaremostfrequentlycausedbyvirusesorToxoplasmagondii.
Treatment:Choroiditisistreatedeitherwithantibioticsorsteroids,depend-
ingonitsetiology.
Prognosis:Theinflammatoryfociwillhealwithintwotosixweeksandform
chorioretinalscars.Thescarswillresultinlocalizedscotomasthatwillreduce
visualacuityifthemaculaisaffected.
8.5.4SympatheticOphthalmia
Definition
Specificbilateralinflammationoftheuvealtractduetochronicirritationof
oneeye,causedbyaperforatingwoundtotheeyeorintraocularsurgery,pro-
ducestransferreduveitisinthefelloweye.
Epidemiology:Sympatheticophthalmiaisveryrare.
Etiology:Sympatheticuveitiscanoccurinanotherwiseunaffectedeyeeven
yearsafterpenetratinginjuriesorintraocularsurgeryinthefelloweye,
especiallywheretherewaschronicirritation.Tissuesintheinjuredeye
(uvealtract,lens,andretina)actasantigensandprovokeanautoimmunedis-
orderintheunaffectedeye.
Symptoms:Theearliestsymptomsincludelimitedrangeofaccommodation
andphotophobia.Laterthereisdiminishedvisualacuityandpain.
Diagnosticconsiderations:Clinicalsymptomsincludecombinedinjections,
cellsandproteinintheanteriorchamberandvitreousbody,papillaryandret-
inaledema,andgranulomatousinflammationofthechoroid.
Differentialdiagnosis:Thedisordershouldbedistinguishedfromiridocycli-
tisandchoroiditisfromothercauses(seeTable8.1).
Treatment:Theinjuredeye,whichisusuallyblind,mustbeenucleatedto
eliminatetheantigen.High-dosetopicalandsystemicsteroidtherapyisindi-
cated.Concurrenttreatmentwithimmunosuppressives(cyclophosphamide
andazathioprine)maybenecessary.
Clinicalcourseandcomplications:Thedisorderhasachronicclinicalcourse
andmayinvolveseverecomplicationsofuveitissuchassecondaryglaucoma,
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233. 215
secondarycataract,retinaldetachment,andshrinkageoftheeyeball.Sympa-
theticophthalmiacanleadtoblindnessinparticularlyseverecases.
Whentheinjuredeyeisblind,prophylacticenucleationisindicated
beforetheonsetofsympatheticophthalmiainthefelloweye.Anearly
signofsympatheticophthalmiaisalimitedrangeofaccommodation
withphotophobia.
8.6 NeovascularizationintheIris:RubeosisIridis
Definition:
Rubeosisiridisisneovascularizationintheiristhatoccursinvariousretinaldis-
orders.
Etiology:Themostfrequentcausesofrubeosisiridis(Fig.8.12)areprolifera-
tivediabeticretinopathyandretinalveinocclusion.Retinalperiphlebitisisa
lessfrequentcauseofneovascularizationintheiris.
Symptomsanddiagnosticconsiderations:Neovascularizationinthe
stromaoftheirisisasymptomaticforthepatient.Neovascularizationinthe
angleoftheanteriorchamberisirreversibleandproducessecondaryangle
closureglaucomawiththetypicalsymptomsofacuteglaucoma:lossofvisual
acuity,intensepain,conjunctivalandciliaryinjection,anda“rockhard”eye-
balluponpalpation(seeFig.10.17).
Differentialdiagnosis:Acuteglaucomaduetoothercausessuchasacute
angleclosureglaucomashouldbeexcluded.
Neovascularizationintheiris:rubeosisiridis.
Fig.8.12Protru-
sionofthepig-
mentedlayer
(arrow)indicates
thattherubeosis
iridishasbeen
presentforat
leastseveral
weeks.
8.6NeovascularizationintheIris:RubeosisIridis
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234. 216
Treatment,prognosis,andprophylaxis:Rubeosisiridisisessentiallytanta-
mounttothelossofaneye.Usuallyitleadstoirreversibleblindness.Prompt
lasertreatmentofretinaldisordersiscrucialtopreventrubeosisiridis.Sec-
ondaryangleclosureglaucomaistreatedbytransscleralfreezingofthecili-
arybody(cyclocryotherapy)toreduceintraocularpressure.Wherethisfails
ortheeyeshrinks(phthisisbulbi)andthepatientexperiencesintensepain,
enucleationoftheeyeisindicated.
Promptlasertreatmentisimportantinproliferativediabeticreti-
nopathytopreventrubeosisiridis.
8.7 Tumors
8.7.1 MalignantTumors(UvealMelanoma)
Withanincidenceofonepertenthousand,malignantuvealmelanomaisthe
mostcommonprimaryintraoculartumor.Itusuallyoccursasachoroidal
melanoma,andisalmostalwaysunilateral.Tumorsintheirisaredetected
earlierthantumorslocatedintheciliarybodyandchoroid(Fig.8.13).
!Irismelanomas:Thesetumorsareofteninitiallyasymptomatic.However,
metastaticmelanomacellsintheangleoftheanteriorchambercanleadto
secondaryglaucoma.Circumscribedirismelanomasareremovedbyseg-
mentaliridectomy.
Choroidalmelanoma.
Fig.8.13A
prominentyel-
lowish-brown
choroidaltumor
(thickarrow-
heads)accom-
paniedbyserous
retinaldetach-
ment(arrows).
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235. 217
!Ciliarybodymelanomas:Symptomsincludechangesinaccommodation
andrefractionresultingfromdisplacementofthelens.Ciliarybody
melanomasareresectedenbloc.
!Choroidalmelanomas:Thesetumorsbecomeclinicallysymptomatic
wheninvolvementofthemaculareducesvisualacuityorthepatient
noticesashadowinhisorherfieldofvisionasaresultofthetumorandthe
accompanyingretinaldetachment.Thediagnosisisconfirmedwiththeaid
oftransillumination,ultrasound,andfluoresceinangiography.Choroidal
tumorsaretreatedwithradioactiveisotopesdeliveredbyplaquesof
radioactivematerial(brachytherapy).Enucleationisindicatedfortumors
whosediameterexceeds8mmandwhoseprominenceexceeds5mm.
!Uvealmetastasesmostfrequentlydevelopfromcarcinomasofthebreast
orlung.Theyareusuallyflatwithlittlepigmentation.
8.7.2BenignChoroidalTumors
Choroidalnevioccurin11%ofthepopulation.Theycanleadtosecondary
neovascularizationwithretinaledema.Inveryrarecaseswherethemaculais
involved,choroidalnevicanleadtoimpairedvision.However,benign
choroidaltumorsarenormallyasymptomatic.
8.7Tumors
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237. 219
9 Pupil
OskarGareisandGerhardK.Lang
9.1 BasicKnowledge
Function:Thepupilreferstothecentralopeningintheiris.Itactsasanaper-
turetoimprovethequalityoftheresultingimagebycontrollingtheamount
oflightthatenterstheeye.
Pupillarylightreflex:Thisreflexarcconsistsofanafferentpaththatdetects
andtransmitsthelightstimulusandanefferentpaththatsuppliesthe
musclesoftheiris(Fig.9.1).
Parasympatheticpupillaryreflexpathway.
Sphincter
pupillae
muscle
Optic chiasma
Optical tract
Lateral
geniculate
body
Pretectal
nucleus
Retina
Ciliary
ganglion
Oculomotor
nerve
Edinger-
Westphal
nucleus
Optic nerve
Visual cortex
(area 17)
Afferents Efferents
A
I
C
D
E
F
B
J
H
G
Fig.9.1Seediscussionintext.
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238. 220
Afferentpath.Thispathbeginsatthelightreceptorsoftheretina(Fig.9.1,A),
continuesalongtheopticnerve(B),theopticchiasma(C)wheresomeofthe
fiberscrosstotheoppositeside.Thepathcontinuesalongtheopticaltracts
(D)untilshortlybeforethelateralgeniculatebody(E).Theretheafferent
reflexpathseparatesfromthevisualpathwayandcontinuestothepretectal
nuclei(F)andfromtheretobothEdinger-Westphalnuclei(G).Eachofthetwo
pretectalnucleiconductimpulsestobothEdinger-Westphalnuclei.This
bilateralconnectionhasseveralconsequences:
!Bothpupilswillnormallybethesamesize(isocoria)evenwhenoneeyeis
blind.Deviationsupto1mmarenormal
!Bothpupilswillnarrowevenwhenonlyoneeyeisilluminated(consensual
lightreflex).
Efferentparasympatheticpath.ThispathbeginsintheEdinger-Westphal
nucleus(G).Itsnervefibersformtheparasympatheticpartoftheoculomotor
nerve(H)andtraveltotheciliaryganglion(I)intheorbit.Postganglionic
nervefiberspassthroughtheshortciliarynervestotheeffectororgan,the
sphincterpupillaemuscle(J).
Perlia’snucleusandtheEdinger-Westphalnucleiarealsoresponsiblefor
thenearreflex,whichconsistsofaccommodation,convergence,andmiosis.
Efferentsympatheticnervesupplytothepupil.Threeneuronsconnectedby
synapsessupplythepupil(Fig.9.2):
!Thecentralfirstneuronbeginsintheposteriorhypothalamus(A),passes
thebrainstemandthemedullaoblongatatotheciliospinalcenter
(Budge’scenter;B)inthecervicalspinalcord(C8–T2).
!Thepreganglionicsecondneuronextendsfromtheciliospinalcenter
throughthewhiteramicommunicantesandsympathetictrunk(C)tothe
superiorcervicalganglion(D).Itisvulnerabletocertainlesionssuchas
Pancoasttumorsbecauseitisimmediatelyadjacenttothetipofthelung.
!Thepostganglionicthirdneuronextendsfromthesuperiorcervicalgan-
glionasaneuralplexusalongtheinternalcarotidartery,ophthalmic
artery,andlongciliarynervestotheeffectororgan,thedilatorpupillae
muscle(E).
Normalpupilsize:Pupilsizerangesfromapproximately1mm(miosis)to
approximately8mm(mydriasis).
!Pupilstendtobewiderinteenagersandindarkness.Theyarealsowider
withjoy,fear,orsurpriseduetoincreasedsympathetictone,andwhenthe
personinhalesdeeply.
!Pupilstendtobenarrowerinthenewbornduetoparasympathetictone,in
theelderlyduetodecreasedmesencephalicinhibitionandsympathetic
diencephalicactivity,inlight,duringsleep,andwhenthepersonis
fatigued(duetodecreasedsympatheticactivity).
9Pupil
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239. 221
Sympatheticsupplytotheeye.
A
E
C
B
Posterior
hypothalamus
Dilator
pupillae
muscle
Superior cervical
ganglion
Sympathetic
trunk
Ciliospinal center
(Budge's center)
Rami communicantes
Internal carotid artery
D
Fig.9.2Seediscussionintext.
9.2 ExaminationMethods
Completeexaminationofthepupilincludestestingdirectandindirectlight
reflexes,theswingingflashlighttest,testingthenearreflex,andmorphologic
evaluationoftheiris.Asynopsisofallfindingsisrequiredtodetermine
whetheradisorderisduetoocularorcerebralcauses(see9.4).
9.2.1 TestingtheLightReflex(Table9.1)
Lightreflexistestedinsubdueddaylightwherethepupilisslightlydilated.
Thepatientgazesintothedistancetoneutralizenear-fieldmiosis.
Directlightreflex:Theexaminerfirstcoversbothofthepatient’seyes,then
uncoversoneeye.Normallythepupilwillconstrictafteralatencyperiodof
about0.2seconds.Theothereyeistestedinthesamemanner.
Indirectorconsensuallightreflex:Theexaminerseparatesthepatient’s
eyesbyplacinghisorherhandonthebridgeofthepatient’snose.Thispre-
9.2ExaminationMethods
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240. 222
T
able
9.
1
Charact
eristic
pupil
findings
in
unilat
eral
lesions
of
the
pupillar
y
r
efle
x
pathw
a
y
Localization
of
the
lesion
(unilater
al)
Direct
light
r
e
f
l
e
x
Ind
irect
lig
ht
ref
l
ex
Swing
ing
f
lashlight
t
e
s
t
Findings
ipsilater
al
contr
alater
al
Af
f
e
rent
pupil-
lar
y
pathw
a
y
(op
tic
ner
v
e
,
re
tina)
Slight
lesion
S
e
v
e
r
e
lesion
+ –
++ ++
+ –
Slight constrictions, q
u
ic
k
e
r
dilation Dilation
I
s
o
c
o
r
ia
Ef
f
e
rent
pupil-
lar
y
pathw
a
y
Oculomo
t
o
r
lesion Ciliar
y
g
a
n
g
lion
lesion
– +
– +
++ ++
No
response
Dela
y
e
d
constriction, dela
y
e
d
dilation
Anisocoria
Legend:
–
=
response
absent,
+
=
w
eak
response,
++
=
s
trong
response
9Pupil
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241. 223
ventsincidentlightfromdirectlystrikingtheeyebeingexamined,which
wouldelicitadirectlightreflex.Theexaminerthenilluminatestheothereye
whileobservingthereactionofthecovered,non-illuminatedeye.Normally
bothpupilswillconstrict,eveninthenon-illuminatedeye.
Swingingflashlighttest:Thistestisusedtodiagnoseadiscreteunilateralor
unilaterallymorepronouncedsensorydeficitintheeye(opticnerveand/orret-
ina).Oftendamagetotheopticnerveorretinaisonlypartial,suchasinpartial
atrophyoftheopticnerve,maculopathy,orperipheralretinaldetachment.In
thesecases,theremaininghealthyportionsoftheafferentpathwayaresuffi-
cienttotriggerconstrictionofthepupilduringtestingofthedirectlight
reflex.Thisconstrictionwillbelessthaninthehealthyeyebutmaybediffi-
culttodiagnosefromdiscretepupillaryreflexfindingsalone.Therefore,the
reflexivebehaviorofbotheyesshouldbeevaluatedinadirectcomparisonto
detectdifferencesintherapidityofconstrictionandsubsequentdilation.This
isdonebymovingalightsourcealternatelyfromoneeyetotheotherinwhat
isknownasaswingingflashlighttest.
Reproducibleresultscanonlyobtainediftheexaminerstrictlyadheresto
thistestprotocol:
!Thepatientfocusesonaremoteobjectinaroomwithsubduedlight.This
neutralizesconvergencemiosis,andthepupilsareslightlydilated,making
thepupillaryreflexmoreeasilydiscernible.
!Theexamineralternatelyilluminatesbotheyeswitharelativelybright
light,takingcaretomaintainaconstantdistance,durationofillumination,
andlightintensitysothatbotheyesmustadapttothesameconditions.
!Theexaminerevaluatestheinitialconstrictionuponilluminationandthe
subsequentdilationofthepupil.
Wherethepupilconstrictsmoreslowlyanddilatesmorerapidlythaninthe
felloweye,onereferstoarelativeafferentpupillarydefect.Thedefectis“rela-
tive”becausethedifferenceinpupillaryreflexonlyoccurswhenthereisa
differenceinthesensorydefecttotheleftandrighteyes.
9.2.2EvaluatingtheNearReflex
Thenearreflextriadconsistsof:
1.Convergenceofthevisualaxes.
2.Accommodation.
3.Constrictionofthepupils(miosis).
Thenearreflexistestedbyhavingthepatientfocusonadistantobjectand
thenonanobjectinthenearfield.Usuallythisisthepatient’sfinger,whichis
broughttowithin10cmoftheeyes.Thenearreflexisintactifbotheyescon-
tinuouslyconvergewithaccommodationandmiosisappropriateforthe
patient’sageastheobjectismovedtowithin10cmoftheeyes.Theexaminer
9.2ExaminationMethods
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242. 224
shouldtakecaretoavoidilluminatingthepupil,whichwillproducealight
reflexwithmiosis.
9.3 InfluenceofPharmacologicAgentsonthePupil
(Table9.2)
Table9.2Influenceofpharmacologicagentsonthepupil
Substancegroupand
individualactiveingre-
dients
Mechanismandduration
ofaction
Indicationandspecial
considerations
Miotics
Parasympathomimetics
!Directparasym-
pathomimetics
–Actonacetylcholine
receptorsofthesphinc-
terpupillaemuscle
(miosis)andtheciliary
muscle(increased
accommodation)
Glaucomatherapy
–Acetylcholine –Extremelyshortdura-
tionofaction(several
minutes)
Intraocularapplication
only(cataractsurgery);
ineffectiveaseyedrops
(rapidbreakdown)
–Pilocarpine –Effectivefor5–7hoursStandardmedicationin
glaucomatherapy
–Aceclidine –Effectivefor5–7hours
–Weakermioticeffect
thanpilocarpine
Standardmedicationin
glaucomatherapy
–Carbachol –Effectivefor7–9hours
–Strongermioticeffect
thanpilocarpine
Standardmedicationin
glaucomatherapy
!Indirectparasym-
pathomimetics
–Actbyinhibitingacetyl-
choline
Glaucomatherapy
Sideeffects:cataract,iris
cysts,mayincreaseriskof
retinaldetachment;
thereforenotthemedica-
tionoffirstchoicein
glaucomatherapy
–Physostigmine –Effectivefor2–3days
–Prostigmin –Effectivefor1day
9Pupil
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243. 225
Table9.2(Continued)
Substancegroupand
individualactiveingre-
dients
Mechanismandduration
ofaction
Indicationandspecial
considerations
Mydriatics
Parasympatholytics –Actbyblockingacetyl-
cholinereceptorsofthe
sphincterpupillae
muscle(mydriasis)and
theciliarymuscle
(accommodationpara-
lysis)
–Tropicamide –Effectiveforapproxi-
mately4–6hours
(shortestactingmydri-
atic)
Usedfordiagnosticpur-
poses
–Cyclopentolate –Effectiveforapproxi-
mately12–24hours
–Morecycloplegicthan
mydriatic
Useddiagnosticallyfor
objectivemeasurement
ofrefraction
Usedtherapeuticallyto
relaxtheciliarybody(in
iritis)
–Homatropine –Effectiveforapproxi-
mately1–2days
Usedtherapeutically(in
iritis)
–Scopolamine –Effectiveforapproxi-
mately1week
Usedtherapeuticallyfor
protractedmydriasis,for
examplefollowingsurgi-
calrepairofretinal
detachmentoriniridocy-
clitis
–Atropine –Effectiveforlessthan
oneweek(longestact-
ingmydriatic)
Foralltherapyrequiring
protractedmydriasis,for
examplefollowingsurgi-
calrepairofretinal
detachmentandin
iridocyclitis
Sympathomimetics
!Directsympatho-
mimetics
–Actontheadrenaline
receptorsofthedilator
pupillaemuscle
Primarilyfordiagnostic
purposes
Continued!
9.3InfluenceofPharmacologicAgentsonthePupil
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244. 226
Table9.2(Continued)
Substancegroupand
individualactiveingre-
dients
Mechanismandduration
ofaction
Indicationandspecial
considerations
Sympathomimetics
–Epinephrine –Onlyslightlyeffective;
rapidlybrokendownby
aminooxidases
Usedinthediagnosisof
Horner’ssyndromeandin
intraocularapplicationfor
bettermydriasisduring
surgery
–Phenylephrine –Effectiveforapproxi-
matelysixhours(onset
anddurationofaction
identicaltotropic-
amide;seeparasym-
patholytics)
–Advantage:doesnot
causeaccommodation
paralysis
Usedfordiagnosticpur-
posesduetoitsshort
durationofaction
!Indirectsympatho-
mimetics
–Inhibitreabsorptionof
norepinephrine
Fordiagnosticpurposes
–Cocaine4%
!Drug-induced
mydriasisiscontrain-
dicatedinpatients
withashallowante-
riorchamberdueto
theriskofacute
angleclosureglau-
coma.
–Effectiveforapproxi-
matelysixhours
Todayusedaseyedrops
onlyfordiagnosticpur-
posesandinHorner’s
syndrome
9.4 PupillaryMotorDysfunction
Pupillarymotordysfunctionmustbedistinguishedfromanumberofdiffer-
entialdiagnosesthatincludenotonlyoculardisordersbutneurologicand
internaldisorders.Diagnosisisdifficultbecauseisocoriaoranisocoriaare
unspecificclinicalsymptoms.Therefore,functionaltestsareindicatedtocon-
firmthediagnosis.Thefollowingsectionusesdiagramsoftheinitiallypres-
entingclinicalsymptomstoillustratethevarioustypesofpupillarydysfunc-
tion.Thetextpresentsthedifferentialdiagnoseswiththefunctionalstudies
usedtoconfirmtherespectivediagnosis.
9Pupil
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245. 227
Isocoriawithconstrictedordilatedpupilsisprimarilyofinteresttotheneu-
rologistandlesssotheophthalmologist.Thesedisordersarethereforedis-
cussedattheendofthesection.
9.4.1 IsocoriawithNormalPupilSize
RelativeAfferentPupillaryDefect
Causes:Unilateralsensorydisordersuchasretinaldetachment,neuritisof
theopticnerve,atrophyoftheopticnerve,orretinalvascularocclusion.
Diagnosticconsiderations:
!Directlightreflexisdecreasedorabsent(relativeafferentpupillarydefect)
intheaffectedeye.
!Theconsensuallightreflexintheaffectedeyeisweakorabsentbutnormal
intheunaffectedeye.
!Theswingingflashlighttestrevealsdilationintheaffectedeyewhen
illuminated(MarcusGunnpupil)orreducedconstrictionandearlierdila-
tioninthepresenceoflesserlesions(afferentpupillarydefect).
!Nearreflexisnormal.
!Unilaterallyreducedvisualacuityand/orfieldofvision.
Unilateralblindness(afferentdefect)doesnotproduceanisocoria.
BilateralAfferentPupillaryDefect
Causes:Bilateralsensorydisordersuchasmaculopathyoratrophyofthe
opticnerve.
Diagnosticconsiderations:
!Delayeddirectandconsensuallightreflexes.
!Theswingingflashlighttestproducesidenticalresultsinbotheyes(where
disorderaffectsbothsidesequally).
!Nearreflexisnormal.
!Bilaterallyreducedvisualacuityand/orfieldofvision.
9.4PupillaryMotorDysfunction
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246. 228
9.4.2AnisocoriawithDilatedPupilintheAffectedEye
CompleteOculomotorPalsy
Causes:
!Processesinthebaseoftheskullsuchastumors,aneurysms,inflamma-
tion,orbleeding.
Diagnosticconsiderations:
!Directandconsensuallightreflexeswithoutconstrictionintheaffected
eye(fixedpupil).
!Nearreflexmiosisisabsent.
!Impairedmotilityanddoublevision.
Suddencompleteoculomotorpalsy(lossofmotorandparasympa-
theticfunction)isasignofapotentiallylife-threateningdisorder.In
unconsciouspatients,unilateralmydriasisisoftentheonlyclinicalsign
ofthis.
TonicPupil
Causes:Postganglionicdamagetotheparasympatheticpathway,pre-
sumablyintheciliaryganglion,thatfrequentlyoccurswithdiabetesmellitus,
alcoholism,viralinfection,andtrauma.
Diagnosticconsiderations:
!Directandconsensuallightreflexesshowabsentordelayedreaction,
possiblywithworm-likesegmentalmuscularcontractions.
!Dilationisalsosignificantlydelayed.
!Nearreflexisslowbutclearlypresent;accommodationwithdelayed
relaxationispresent.
!Motilityisunimpaired.
!Pharmacologictestingwith0.1%pilocarpine.
–Significantmiosisintheaffectedeye(denervationhypersensitivity).
–Nochangeinthepupiloftheunaffectedeye(tooweak).
!Adie’stonicpupilsyndrome:Thetonicpupilisaccompaniedbyabsenceof
theAchillesandpatellartendonreflexes.
Tonicpupilisarelativelyfrequentandcompletelyharmlesscauseof
unilateralmydriasis.
9Pupil
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247. 229
IrisDefects
Causes:
!Trauma(aniridiaorsphinctertears).
!Secondarytoacuteangleclosureglaucoma.
!Synechiae(post-iritisorpostoperative).
Diagnosticconsiderations:Patienthistoryandslit-lampexamination.
FollowingEyedropApplication(UnilateralAdministrationofa
Mydriatic)
Simpleanisocoria
Causes:Presumablyduetoasymmetricalsupranuclearinhibitionofthe
Edinger-Westphalnucleus.
Diagnosticconsiderations:
!Directandconsensuallightreflexesandswingingflashlighttestshowcon-
stantdifferenceinpupilsize.
!Nearreflexisnormal.
!Pharmacologictesting:Cocainetest(4%cocaineeyedropsareappliedto
botheyesandpupilsizeismeasuredafteronehour):bilateralpupildila-
tionindicatesanintactneuronchain.
9.4.3AnisocoriawithaConstrictedPupilintheAffectedEye
Horner’sSyndrome
Causes:Damagetothesympatheticpathway.
!Central(firstneuron):
–Tumors.
–Encephalitis.
–Diffuseencephalitis.
!Peripheral(secondneuron):
–Syringomyelia.
–Diffuseencephalitis.
–Trauma.
–Rhinopharyngealtumors.
–Goiter.
9.4PupillaryMotorDysfunction
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248. 230
–Aneurysm.
–Processesinthetipofthelung.
!Peripheralinthestrictsense(thirdneuron):
–Vascularprocesses.
–Internalcarotidaneurysm.
ClinicalPicture:
!Miosis(approximately1–2mmdifference)duetofailureofthedilator
pupillaemuscle.
!Ptosis(approximately1–2mmdifference)duetofailureofthemuscleof
Müller.
!Enophthalmosduetofailureoftherudimentarylowereyelidretractors.
Thismakesthelowereyelidprojectsothattheeyeappearssmaller.This
conditiononlyrepresentsatypeofpseudoenophthalmos.
!Decreasedsweatglandsecretion(onlypresentinpreganglionicdisorders
asthesweatglandsreceivetheirneuralsupplyviatheeternalcarotid).
Diagnosticconsiderations:
!Directandconsensuallightreflexesareintact,whichdistinguishesthis
disorderfromaparasympatheticlesion);thepupildilatesmoreslowly
(dilationdeficit).
!Nearreflexisintact.
!Pharmacologictestingwithcocaineeyedrops:
–PeripheralHorner’ssyndrome:Ontheaffectedside,thereisslight
mydriasis(decreaseinnorepinephrineduetonervelesion).Onthe
unaffectedside,thereissignificantmydriasis.
–CentralHorner’ssyndrome:Ontheaffectedside,thepupilisdilated.On
theunaffectedside,thepupilisalsodilated(thenorepinephrineinthe
synapsesisnotinhibited).
FollowingEyedropApplication(UnilateralAdministrationofaMioticas
inGlaucomaTherapy)
9.4.4IsocoriawithConstrictedPupils
Argyll-RobertsonPupil
Causes:Thepreciselocationofthelesionisnotknown;presumablythedis-
orderisduetoalesionisinthepretectalregionandtheEdinger-Westphal
nucleussuchastabesdorsalis(Argyll-Robertsonphenomenon),encephalitis,
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249. 231
diffuseencephalitis,syringomyelia,trauma,bleeding,tumors,andalco-
holism.
Diagnosticconsiderations:
!Directandconsensuallightreflexesareabsent.
!Nearreflexisintactorthereisovercompensation(theEdinger-Westphal
nucleusisbeingcontrolledviatheconvergencecenter).
!Thepupilisnotround,andconstrictionisnotalwayssymmetrical.
!Thereisnoreactiontodarknessorpharmacologicstimuli.
BilateralPupillaryConstrictionduetoPharmacologicAgents
Causes:
!Morphine.
!Deepgeneralanesthesia.
!Pilocarpineeyedrops.
ToxicBilateralPupillaryConstriction
Causes:Mushroompoisoning.
InflammatoryBilateralPupillaryConstriction
Causes:
!Encephalitis.
!Meningitis.
9.3.5IsocoriawithDilatedPupils
Parinaud’sOculoglandularSyndrome
Causes:Tumorssuchaspinealglandtumorsthatselectivelydamagefibers
betweenthepretectalnucleiandtheEdinger-Westphalnucleus.
Diagnosticconsiderations:
!Fixeddilatedpupilsthatdonotrespondtolight.
!Normalnearreflex.
!Limitedupwardgaze(duetodamagetotheverticalgazecenter)and
retractionnystagmus.
9.4PupillaryMotorDysfunction
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250. 232
Intoxication
Causes:Atropine,spasmolyticagents,anti-parkinsonagents,antidepres-
sants,botulism(veryrarebutimportant),carbonmonoxide,cocaine.
Disorders
!Migraine.
!Schizophrenia.
!Hyperthyreosis.
!Hysteria.
!Epilepticseizure.
!Increasedsympathetictone(Bumke’sanxietypupils).
!Coma.
!Agony.
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251. 233
10Glaucoma
GerhardK.Lang
10.1BasicKnowledge
Definition
Glaucomaisadisorderinwhichincreasedintraocularpressuredamagesthe
opticnerve.Thiseventuallyleadstoblindnessintheaffectedeye.
!Primaryglaucomareferstoglaucomathatisnotcausedbyotherocular
disorders.
!Secondaryglaucomamayoccurastheresultofanotheroculardisorderor
anundesiredsideeffectofmedicationorothertherapy.
Epidemiology:Glaucomaisthesecondmostfrequentcauseofblindnessin
developingcountriesafterdiabetesmellitus.Fifteentotwentypercentofall
blindpersonslosttheireyesightasaresultofglaucoma.InGermany,approxi-
mately10%ofthepopulationover40hasincreasedintraocularpressure.
Approximately10%ofpatientsseenbyophthalmologistssufferfromglau-
coma.OftheGermanpopulation,8millionpersonsareatriskofdeveloping
glaucoma,800000havealreadydevelopedthedisease(i.e.,theyhaveglau-
comathathasbeendiagnosedbyanophthalmologist),and80000facethe
riskofgoingblindiftheglaucomaisnotdiagnosedandtreatedintime.
Earlydetectionofglaucomaisoneofthehighestprioritiesforthepub-
lichealthsystem.
Physiologyandpathophysiologyofaqueoushumorcirculation(Fig.10.1):
Theaveragenormalintraocularpressureof15mmHginadultsissignificantly
higherthantheaveragetissuepressureinalmosteveryotherorganinthe
body.Suchahighpressureisimportantfortheopticalimagingandhelpsto
ensureseveralthings:
!Uniformlysmoothcurvatureofthesurfaceofthecornea.
!Constantdistancebetweenthecornea,lens,andretina.
!Uniformalignmentofthephotoreceptorsoftheretinaandthepigmented
epitheliumonBruch’smembrane,whichisnormallytautandsmooth.
Theaqueoushumorisformedbytheciliaryprocessesandsecretedintothe
posteriorchamberoftheeye(Fig.10.1[A]).Atarateofabout2–6µlper
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252. 234
Physiologyofaqueoushumorcirculation.
A
E
D
C
B
Ciliary body
Lens
Iris
CorneaCanal of Schlemm
Trabecular meshwork
Collecting channel
Conjunctiva
Episcleral venous plexus
Fig.10.1AsitflowsfromthenonpigmentedcellsoftheciliaryepitheliaAto
beneaththeconjunctivaD,theaqueoushumorovercomesphysiologicresistance
fromtwosources:theresistanceofthepupilBandtheresistanceofthetrabecular
meshworkC.
minuteandatotalanteriorandposteriorchambervolumeofabout
0.2–0.4ml,about1–2%oftheaqueoushumorisreplacedeachminute.
Theaqueoushumorpassesthroughthepupilintotheanteriorchamber.As
theirisliesflatalongtheanteriorsurfaceofthelens,theaqueoushumorcan-
notovercomethispupillaryresistance(firstphysiologicresistance;Fig.10.1
[B])untilsufficientpressurehasbuiltuptolifttheirisoffthesurfaceofthe
lens.Therefore,theflowoftheaqueoushumorfromtheposteriorchamber
intotheanteriorchamberisnotcontinuousbutpulsatile.
Anyincreaseintheresistancetopupillaryoutflow(pupillaryblock)leadsto
anincreaseinthepressureintheposteriorchamber;theirisinflatesanteri-
orlyonitsrootlikeasailandpressesagainstthetrabecularmeshwork(Table
10.2).Thisisthepathogenesisofangleclosureglaucoma.
Variousfactorscanincreasetheresistancetopupillaryoutflow(Table
10.1).Theaqueoushumorflowsoutoftheangleoftheanteriorchamber
throughtwochannels:
!Thetrabecularmeshwork(Fig.10.1[C])receivesabout85%oftheout-
flow,whichthendrainsintothecanalofSchlemm.Fromhereitiscon-
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253. 235
Table10.1Factorsthatincreaseresistancetopupillaryoutflowandpredisposetoangle
closureglaucoma
Increasedcontactbetween
themarginofthepupiland
lenswith:
!Smalleyes
!Largelens(increasedlensvolume)dueto:
–Age(lensvolumeincreaseswithagebya
factorofsix)
–Diabetesmellitus(osmoticswellingofthe
lens)
!Miosis
–Age(atrophyofthesphincteranddilator
muscles)
–Medications(mioticagentsinglaucoma
therapy)
–Iritis(reactivemiosis)
–Diabeticiridopathy(thickeningoftheiris)
!Posteriorsynechiae(adhesionsbetweenlens
andiris)
Increasedviscosityofthe
aqueoushumorwith:
!Inflammation(protein,cells,orfibrininthe
aqueoushumor)
!Bleeding(erythrocytesintheaqueoushumor)
ductedby20–30radialcollectingchannelsintotheepiscleralvenous
plexus(D).
!Auveoscleralvascularsystemreceivesabout15%oftheoutflow,which
joinsthevenousblood(E).
Thetrabecularmeshwork(C)isthesecondsourceofphysiologicresistance.
Thetrabecularmeshworkisabodyofloosesponge-likeavasculartissue
betweenthescleralspurandSchwalbe’sline.Increasedresistanceinpresent
inopenangleglaucoma.
Classification:Glaucomacanbeclassifiedaccordingtothespecific
pathophysiology(Table10.2).
Themanyvarioustypesofglaucomaarenearlyallattributableto
increasedresistancetooutflowandnottoheightenedsecretionof
aqueoushumor.
10.1BasicKnowledge
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254. 236
Table10.2Classificationofglaucoma
Formof
glaucoma
Incidence
Openangle
glaucoma
Primary Over90%ofall
glaucomas
Secondary 2–4%ofall
glaucomas
Angleclosure
glaucoma
Primary
(pupillary
block
glaucoma)
About5%ofall
glaucomas
Secondary 2–4%ofall
glaucomas
Juvenile
glaucoma
1%ofall
glaucomas
Absolute
glaucoma
Thisisnotaseparateformofglaucoma,ratheritdescribesan
oftenpainfuleyeblindedbyglaucoma
Linse
Linse
Linse
Linse
Linse
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255. 237
Angle
(anatomic)
Angle
(gonioscopy)
Outflowimpediment
Open Completelyopen.Structures
appearnormal.
Inthetrabecularmeshwork
Open Completelyopen.Trabecular
meshworksandsecondaryocclud-
ingcellsvisible.
Erythrocytes,pigment,and
inflammatorycellsocclude
thetrabecularmeshwork.
Blocked Occluded.Noanglestructures
visible
Iristissueoccludesthe
trabecularmeshwork.
Blocked Occluded.Noanglestructures
visible.Occludingstructures
visible.
Displacementofthe
trabecularmeshworkpro-
ducesanteriorsynechiae,
scarring,andneovasculari-
zation(rubeosisiridis)
UndifferentiatedOpen.Occludingembryonictissue
andlackofdifferentiationvisible.
Inthetrabecularmeshwork
(whichisnotfullydifferen-
tiatedand/orisoccludedby
embryonictissue)
10.1BasicKnowledge
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256. 238
10.2ExaminationMethods
10.2.1ObliqueIlluminationoftheAnteriorChamber
Theanteriorchamberisilluminatedbyabeamoflighttangentialtotheplane
oftheiris.Ineyeswithananteriorchamberofnormaldepth,theirisisuni-
formlyilluminated.Thisisasignofadeepanteriorchamberwithanopen
angle(seeFig.1.12).
Ineyeswithashallowanteriorchamberandananglethatispartiallyor
completelyclosed,theirisprotrudesanteriorlyandisnotuniformlyillumi-
nated(seeFig.1.12).
10.2.2Slit-LampExamination
Thecentralandperipheraldepthoftheanteriorchambershouldbeevaluated
onthebasisofthethicknessofthecornea.Ananteriorchamberthatisless
thanthreetimesasdeepasthethicknessofthecorneainthecenterwitha
peripheraldepthlessthanthethicknessofthecorneasuggestsanarrow
angle(Fig.10.2).Gonioscopyisessentialforfurtherevaluation.
Toevaluatethedepthoftheanteriorchamberwithaslit-lampbio-
microscope,selectanarrowsettingforthelightbeam.Thebeam
shouldstriketheeyeataslightangletotheexaminer’slineofsight.
10.2.3Gonioscopy
Theangleoftheanteriorchamberisevaluatedwithagonioscopeplaced
directlyonthecornea(Fig.10.3aandb).
Slit-lampexaminationtoevaluatethedepthoftheanteriorchamber.
Fig.10.2Thedepthof
theanteriorchamberis
lessthanthethicknessof
thecorneaonitsperiph-
ery.Thecornealreflex
andirisreflectiontouch
eachother(arrow),indi-
catingashallowanterior
chamber.Gonioscopyis
indicated.
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257. 239
Gonioscopyandmorphologyoftheanglestructures.
Line of sight into the angle
of the anterior chamber
from the slit lamp
*
*
Mirror
Fig.10.3
aSchematicdiagramof
gonioscopy.Theangleof
theanteriorchambercan
bevisualizedwithagonio-
scopeplacedonthecor-
nea.
bGonioscopicimageof
theangle.
a
b
10.2ExaminationMethods
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258. 240
Gonioscopycandifferentiatethefollowingconditions:
!Openangle:openangleglaucoma.
!Occludedangle:angleclosureglaucoma.
!Angleaccessisnarrowed:configurationwithimminentriskangleofan
acuteclosureglaucoma.
!Angleisoccluded:secondaryangleclosureglaucoma,forexampledueto
neovascularizationinrubeosisiridis.
!Angleopenbutwithinflammatorycellulardeposits,erythrocytes,orpig-
mentinthetrabecularmeshwork:secondaryopenangleglaucoma.
Gonioscopyistheexaminationofchoiceforidentifyingtherespective
presentingformofglaucoma.
10.2.4MeasuringIntraocularPressure
Palpation(Fig.1.15,p.15):Comparativepalpationofbotheyeballsisapre-
liminaryexaminationthatcandetectincreasedintraocularpressure.
!Iftheexaminercanindenttheeyeball,whichfluctuatesunderpalpation,
pressureislessthan20mmHg.
!Aneyeballthatisnotresilientbutrockhardisasignofabout60–70mm
Hgofpressure(acuteangleclosureglaucoma).
Schiøtzindentationtonometry(Figs.10.4aandb):Thisexaminationmeas-
uresthedegreetowhichthecorneacanbeindentedinthesupinepatient.The
lowertheintraocularpressure,thedeeperthetonometerpinsinksandthe
greaterdistancetheneedlemoves.
Indentationtonometryoftenprovidesinexactresults.Forexamplethe
rigidityofthescleraisreducedinmyopiceyes,whichwillcausethetonome-
terpintosinkmoredeeplyforthatreasonalone.Becauseofthis,indentation
tonometryhasbeenlargelysupplantedbyapplanationtonometry.
Applanationtonometry:Thismethodisthemostcommonmethodofmeas-
uringintraocularpressure.Itpermitstheexaminertoobtainameasurement
onasittingpatientwithinafewseconds(Goldmann’smethod,seeFig.10.5
a–c)oronasupinepatient(Draeger’smethod).Aflattonometertiphasa
diameterof3.06mmforapplanationofthecorneaoveracorrespondingarea
(7.35mm
2
).Thismethodeliminatestherigidityofthescleraasasourceof
error(seealsotonometricself-examination).
Intraocularpressureof22mmHgisregardedassuspicious.Caution:
Infectionispossibleinthepresenceofconjunctivitis.
Pneumaticnon-contacttonometry:Theelectronictonometerdirectsa
3msblastofairagainstthecornea.Thetonometerrecordsthedeflectionof
thecorneaandcalculatestheintraocularpressureonthebasisofthisdefor-
mation.
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259. 241
Schiøtzindentationtonometry.
Fig.10.4aThetonometerisplacedon
theanesthetizedcornea.Theexaminerre-
tractsbotheyelidsandthepatientfocuses
onhisorherthumbwiththeothereye.
bDetailviewofthetonometerpinin-
dentingthecornea.Theharderthe
eyeball,theshallowertheindentation
andthesmallerthemovementofthe
indicatorneedle.
Advantages:
!Doesnotrequiretheuseofatopicalanesthetic.
!Non-contactmeasurementeliminatesriskofinfection(maybeusedto
measureintraocularpressureinthepresenceofconjunctivitis).
Disadvantages:
!Calibrationisdifficult.
!Precisemeasurementsarepossibleonlywithinlowtomiddlerangepres-
sures.
!Cannotbeusedinthepresenceofcornealscarring.
!Examinationisunpleasantforthepatient.
!Airflowisloud.
!Theinstrumentismoreexpensivetopurchasethananapplanation
tonometer.
Tonometer
pin
Base plate
of the
tonometer
10.2ExaminationMethods
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260. 242
Goldmannapplanationtonometry.
Fig.10.5
aSlit-lampmeasurement
ofintraocularpressure:
Afterapplicationofanes-
thetizingeyedropscontain-
ingfluorescein,the
tonometertipisplacedon
thecornea.
bThecorneaisapplanated
(flattened)overanarea
measuringprecisely
7.35mm
2
.Theexternal
pressurerequiredisdirectly
proportionaltointraocular
pressure.
cViewthroughaslitlamp:
Thepressurereadingis
takenwhenthetwoinner
menisciofthefluorescein
arcstouch(arrow).
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261. 243
Measuringthetwenty-four-hourpressurecurve(Fig.10.6):Thisexamina-
tionisperformedtoanalyzefluctuationsofthepressurelevelovera24-hour
periodinpatientswithsuspectedglaucoma.
Asinglemeasurementmaynotberepresentative.Onlya24-hourcurve
providesreliableinformationaboutthepressurelevel.
Intraocularpressurefluctuatesinarhythmicpattern.Thehighestvalues
frequentlyoccuratnightorintheearlymorninghours.Innormalpatients,
thesefluctuationsinintraocularpressurerarelyexceed4–6mmHg.
Pressureismeasuredonthewardat6:00a.m.,noon,6:00p.m.,
9:00p.m.,andmidnight.Outpatient24-hourpressurecurveswithoutnight-
timeandearlymorningmeasurementsarelessreliable.
Inglaucomapatientsmaintainedoneyedrops,specialattentionshould
begiventothetimeofapplication.Pressureismeasuredimmediately
priortoapplyingtheeyedrops.Inthismanner,measurementsare
obtainedwhentheeffectoftheeyedropsisweakest.
Tonometricself-examination:Recentdevelopmentshavemadeitpossible
forpatientstomeasureintraocularpressurethemselvesathomeinamanner
similartoself-monitoringofbloodpressureandbloodglucose(Fig.10.7).The
patienttonometermakesitpossibletoobtaina24-hourpressurecurvefrom
10.2ExaminationMethods
Twenty-four-hourpressurecurve.
50
45
40
35
30
25
20
15
10
5
mmHg
369121518213691215182136912151821hours
Normal limit
Abnormal
Normal
Beta blockers right and left, twice daily
Pressure curve, left eye
Pressure curve, right eye
Fig.10.6Thecoloreddotsrepresentthetimesofthemeasurements.Thetimeof
theinitialapplicationofanti-glaucomaeyedropsismarked(arrow).Thetime,
frequency,andeyeofeyedropapplicationarealsoidentified.
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262. 244
Tonometricself-examination.
Fig.10.7Thepatientplacesthetonometeronhisorherforeheadandusesthe
fixationlighttoalignitintheproperposition.Theheadofthetonometerthenauto-
maticallypressesagainstthecornea,measuresintraocularpressure,andretracts.
Pressureisindicatedinadigitaldisplay.
anynumberofmeasurementsobtainedundernormaleverydayconditions.A
patienttonometermaybeprescribedinapplicablecases(suchasincreased
riskofacuteglaucoma).However,usingthedevicerequiresacertaindegree
ofskillonthepartofthepatient.Patientswhohaveproblemsapplyingeye-
dropsarebestadvisednottoattempttouseapatienttonometer.Youngerand
wellmotivatedpatientsarethebestcandidatesfortonometricself-examina-
tion.
10.2.5OpticDiskOphthalmoscopy
Theopticdiskhasaphysiologicindentationknownastheopticcup.Inthe
presenceofpersistentlyelevatedintraocularpressure,theopticcupbecomes
enlargedandcanbeevaluatedbyophthalmoscopy.
Stereoscopicexaminationoftheopticdiskthroughaslit-lampbiomicroscope
fittedwithacontactlensprovidesathree-dimensionalimage.Theopticcup
maybeexaminedstereoscopicallywiththepupildilated.
Theopticnerveistheeye’s“glaucomamemory.”Evaluatingthisstruc-
turewilltelltheexaminerwhetherdamagefromglaucomaispresent
andhowfaradvanceditis.
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263. 245
Normalopticcup(Fig.10.8):Thenormalanatomycanvarywidely.Large
normalopticcupsarenearlyalwaysroundanddifferfromtheverticalelonga-
tionoftheopticcupseenineyeswithglaucoma.
Documentingtheopticdisk:Recordingfindingsinsketchesissuitablefor
routinedocumentationandfollow-upexaminationoftheopticdisk.Photo-
graphingtheopticdiskwithafunduscamerapermitslonger-termfollow-
up.Stereoscopicphotographyalsoprovidesathree-dimensionalimage.Optic
diskmeasurementandtomographycanprovideprecisemeasurementsofthe
opticnerve.
Opticdiskmeasurement.Theareaoftheopticdisk,opticcup,andneuroreti-
nalrim(vitalopticdisktissue)canbemeasuredbyplanimetryontwo-dimen-
sionalphotographsoftheopticnerve.
Normalopticdisk.
"
Fig.10.8Theopticdisk
issharplydemarcated.It
islevelwiththeretina,
anditscolorindicates
vitaltissue.Thesmall
centralopticcup(arrow)
isdiscernibleasbrighter
area.
10.2ExaminationMethods
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264. 246
Opticdisktomography.Modernlaserscanningophthalmoscopespermit
three-dimensionaldocumentationoftheopticnerve(Fig.10.9).
Glaucomatouschangesintheopticnerve:Glaucomaproducestypical
changesintheshapeoftheopticcup.Progressivedestructionofnervefibers,
fibrousandvasculartissue,andglialtissuewillbeobservable.Thistissue
atrophyleadstoanincreaseinthesizeoftheopticcupandtopalediscoloration
oftheopticdisk(Fig.10.10).
Progressiveglaucomatouschangesintheopticdiskareclosely
associatedwithincreasingvisualfielddefects(Figs.10.11a–d).
10.2.6VisualFieldTesting
Detectingglaucomaasearlyaspossiblerequiresdocumentingglaucomatous
visualfielddefectsattheearliestpossiblestage.Weknowthatglaucomatous
visualfielddefectsinitiallymanifestthemselvesinthesuperiorparacentral
nasalvisualfieldor,lessfrequently,intheinferiorfield,asrelativescotomas
thatlaterprogresstoabsolutescotomas(Fig.10.11a–d).
Opticdisktomography.
0.000.501.001.502.002.50
x (mm)
1.80
1.60
1.40
1.20
1.00
0.80
0.60
0.40
0.20
0.00
-0.20
-0.40
-0.60
-0.80
-1.00
z
(mm)
2.50
2.00
1.50
1.00
0.50
0.00
0.000.501.001.502.002.50
y
(mm)
x (mm)a1
1.501.000.500.00-0.50-1.00
2.80
2.60
2.40
2.20
2.00
1.80
1.60
1.40
1.20
1.00
0.80
0.60
0.40
0.20
0.00
y
(mm)
a2b
z (mm)
Stereometric Analysis OHN:
Disk Area: 2.103 mm
2
Cup Area: 0.576 mm
2
Cup/Disk Area Ratio:0.274
Rim Area: 1.527 mm
2
Rim Volume: 0.322 mm
3
Mean Cup Depth:0.262 mm
Maximum Cup Depth:0.843 mm
c
d
Fig.10.9Alaserbeamscanstheopticdisk(a1anda2)toproduceaverticalmap(b)
andhorizontalmap(c)oftheheightanddepthoftheopticdisk.Thecomputerthen
calculatescrucialdatafortheopticdiskandpresentsastereometricanalysis(d).
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265. 247
Glaucomatouslesionsintheopticnerve.
"
Fig.10.10Theoptic
diskissharplydemar-
catedandpale(asignof
tissueatrophy).Theoptic
cupisenlargedandal-
mostcompletelycovers
thedisk.Thebloodves-
selsabruptlyplungeinto
thedeepcup,indicated
bytheirtypicalbayonet-
shapedkinksinthe
image(arrow).
Computerizedstaticperimetry(measurementofthesensitivitytodiffer-
encesinlight)issuperiortoanykineticmethodindetectingtheseearly
glaucomatousvisualfielddefects.Computer-controlledsemiautomaticgrid
perimetrydevicessuchastheOctopusorHumphreyfieldanalyzerareused
toexaminethecentral30degreefieldofvision(moderncampimetry;
Fig.10.12).
Reproduciblevisualfieldfindingsareimportantinfollow-uptoexclude
anyenlargementofthedefects.
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266. 248
Overviewofglaucomatousvisualfielddefects.
Fig.10.11
Peripheral optic cup in a temporal and inferior
location (with damage to the optic nerve
fibers in this area).
Increase in the size of the optic cup with thinning
of the vital rim. The lamina cribrosa is visible.
Advanced generalized thinning of the
neuroretinal rim with an increasingly visible
lamina cribrosa and nasal displacement of
the blood vessels.
Total glaucomatous atrophy of the optic nerve:
Complete atrophy of the neuroretinal rim, kettle-
shaped optic cup, bayonet kinks in the blood
vessels on the margin of the optic disk, some of
which disappear. The lamina cribrosa is diffusely
visible. Only remnants of the atrophic tissue of
the optic disk remain. The optic disk is surrounded
by a ring of chorioretinal atrophy (glaucomatous
halo) due to pressure atrophy of the choroid and
lysis of the retinal pigmented epithelium.

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267. 249
An enlarged blind spot and a superior
paracentral nasal scotoma. The paracentral
scotomas precede the enlargement of the
blind spot.
Narrowing of the peripheral superior
paracentral visual field. The insular
paracentral scotomas converge and
extend to the blind spot.
Further loss of superior nasal visual field.
Circumscribed horizontal penetration of
the Bjerrum's scotoma into the nasal half of
the field of vision. A new inferior nasal
scotoma is a sign of a superior temporal
nerve fiber lesion.
A small central and peripheral residual field
of vision remains. The arc-shaped scotoma
has expanded into a ring-shaped scotoma
surrounding the focal point. As the focal
point degenerates, the center of vision
disappears and only a peripheral residual
field of vision remains.
10.2ExaminationMethods
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268. 250
Thirtydegreevisualfieldtestforglaucomascreening.
Fig.10.12Thecentralfieldofvisionisexaminedforscotomaswithanautomatic
perimeterasstudiesofearlyglaucomahaveshownthattheinitialdefectsoccurin
thisarea(seeFig.10.11a–d).Thefigureshowsthevisualfielddefectintheearly
stagesofglaucoma.Theblindspotisslightlyenlarged(arrow),andanarc-shaped
paracentralBjerrum’sscotomaispresent(arrowhead).Thestandardizedexamina-
tionconditionsinautomaticperimetrynotonlypermitearlydetectionofglaucoma;
thereproducibleresultsalsoaidinthepromptdiagnosisofworseningfindings.
10.2.7ExaminationoftheRetinalNerveFiberLayer
Theretinalnervefibershaveacharacteristicarrangement,whichexplainsthe
typicalvisualfielddefectsthatoccurinprimaryopenangleglaucoma.In
additiontotheearlyprogressiveopticnerveandvisualfielddefects,arc-
shapeddefectsalsooccurinthenervefiberlayer.Thesedefectsmaybe
observedinlightwithredcomponents(Fig.10.13).
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269. 251
Examinationoftheretinalnervefiberlayer.
Fig.10.13Thearc-
shapednervefiberdefect
(betweenthearrows)isa
signofanearlyglauco-
matousopticnervele-
sion.
10.3PrimaryGlaucoma
10.3.1PrimaryOpenAngleGlaucoma
Definition
Primaryopenangleglaucomabeginsinmiddle-agedandelderlypatientswith
minimalsymptomsthatprogressivelyworsen.Theangleoftheanteriorcham-
bercharacteristicallyremainsopenthroughouttheclinicalcourseofthedis-
order.
Epidemiology:Primaryopenangleglaucomaisbyfarthemostcommonform
ofglaucomaandaccountsforover90%ofadultglaucomas.Theincidenceof
thedisordersignificantlyincreasesbeyondtheageof40,reachingapeak
betweentheagesof60and70.Itsprevalenceamong40-year-oldsis0.9%as
comparedto4.7%amongpatientsovertheageof50.
10.3PrimaryGlaucoma
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270. 252
Thereappearstobeageneticpredispositionforprimaryopenangleglau-
coma.Overone-thirdofglaucomapatientshaverelativeswiththesamedis-
order.
Patientswithapositivefamilyhistoryareatgreaterriskofdeveloping
thedisorder.
Etiology(Seealsophysiologyandpathophysiologyofaqueoushumorcircu-
lation):Thecauseofprimaryopenangleglaucomaisnotknown,althoughit
isknownthatdrainageoftheaqueoushumorisimpeded.Theprimary
lesionoccursintheneuroretinaltissueoftheopticnerveascompression
neuropathyoftheopticnerve.
Symptoms:Themajorityofpatientswithprimaryopenangleglaucomado
notexperienceanysubjectivesymptomsforyears.However,asmallnumber
ofpatientsexperienceoccasionalunspecificsymptomssuchasheadache,a
burningsensationintheeyes,orblurredordecreasedvisionthatthepatient
mayattributetolackofeyeglassesorinsufficientcorrection.Thepatientmay
alsoperceiveringsofcoloraroundlightsourcesatnight,whichhastradition-
allybeenregardedasasymptomofangleclosureglaucoma.
Primaryopenangleglaucomaoftendoesnotexhibittypicalsymptoms
foryears.Regularexaminationbyanophthalmologistiscrucialforearly
diagnosis.
Primaryopenangleglaucomacanbefaradvancedbeforethepatientnotices
anextensivevisualfielddefectinoneorbotheyes.
Itiscrucialtodiagnosethedisorderasearlyaspossiblebecausetheprog-
nosisforglaucomadetectedinitsearlystagesisfarbetterthanforadvanced
glaucoma.Whereincreasedintraocularpressureremainsundiagnosedor
untreatedforyears,glaucomatousopticnervedamageandtheassociated
visualfielddefectwillincreasetothepointofblindness.
Diagnosticconsiderations:Measurementofintraocularpressure.Elevated
intraocularpressureinaroutineophthalmicexaminationisanalarmingsign.
Twenty-four-hourpressurecurve.Fluctuationsinintraocularpressureof
over5–6mmHgmayoccurovera24-hourperiod.
Gonioscopy.Theangleoftheanteriorchamberisopenandappearsasnormal
astheangleinpatientswithoutglaucoma.
Ophthalmoscopy.Examinationoftheopticnerverevealswhetherglaucoma-
touscuppinghasalreadyoccurredandhowfaradvancedtheglaucomais.
Wheretheopticdiskandvisualfieldarenormal,ophthalmoscopicexamina-
tionoftheposteriorpoleundergreenlightmayrevealfascicularnervefiber
defectsasearlyabnormalfindings.
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271. 253
Perimetry.Noisefieldperimetryissuitableasascreeningtestasitmakesthe
patientawareofscotomasandmakesitpossibletodetectanddescribethem.
Thepatientisshownaflickeringmonitordisplayingwhatresemblesimage
noiseonatelevisionset.Thepatientwillnotseetheflickeringpointsinthe
regionofthescotoma.Afterthistest,thedefectshouldbequantifiedbymore
specificmethods.Automaticgridperimetryissuitablefortheearlystagesof
glaucoma.Specialprograms(suchastheG1programontheOctopusperime-
terandthe30–2programontheHumphreyperimeterdevices)revealthe
earliestglaucomatouschanges.Inadvancedglaucoma,kinetichandpe-
rimetrywiththeGoldmannperimeterdeviceisausefulpreliminaryexami-
nationtoevaluatetheremainingfieldofvision.
Differentialdiagnosis:Twodisordersareimportantinthiscontext:
Ocularhypertension.Patientswithocularhypertensionhavesignificantly
increasedintraocularpressureoveraperiodofyearswithoutsignsofglauco-
matousopticnervedamageorvisualfielddefects.Somepatientsinthis
groupwillcontinuetohaveelevatedintraocularpressurebutwillnotdevelop
glaucomatouslesions;theotherswilldevelopprimaryopenangleglaucoma.
Theprobabilitythatapatientwilldevelopdefinitiveglaucomaincreasesthe
highertheintraocularpressure,theyoungerthepatient,andthemorecom-
pellingtheevidenceofahistoryofglaucomainthefamily.
Low-tensionglaucoma.Patientswithlow-tensionglaucomaexhibittypical
progressiveglaucomatouschangesintheopticdiskandvisualfieldwithout
elevatedintraocularpressure.Thesepatientsareverydifficulttotreat
becausemanagementcannotfocusonthecontrolofintraocularpressure.
Oftenthesepatientswillhaveahistoryofhemodynamiccrisessuchas
gastrointestinaloruterinebleedingwithsignificantlossofblood,lowblood
pressure,andperipheralvascularspasms(coldhandsandfeet).Patientswith
glaucomamayalsoexperiencefurtherworseningofthevisualfieldduetoa
dropinbloodpressure.
Cautionshouldbeexercisedwhenusingcardiovascularandanti-hyper-
tensionmedicationsinpatientswithglaucoma.
Treatment:Indicationsforinitiatingtreatment.
!Glaucomatouschangesintheopticcup:Medicaltreatmentshouldbe
initiatedwheretherearesignsofglaucomatouschangesintheopticcupor
wherethereisadifferenceofmorethan20%betweentheopticcupsofthe
twoeyes.
!Anyintraocularpressureexceeding30mmHgshouldbetreated.
!Increasingglaucomatouschangesintheopticcuporincreasingvisualfield
defects:Regardlessofthepressuremeasured,thesechangesshowthatthe
currentpressurelevelistoohighfortheopticnerveandthatadditional
medicaltherapyisindicated.Thisalsoappliestopatientswithadvanced
10.3PrimaryGlaucoma
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272. 254
glaucomatousdamageandthresholdpressurelevels(around22mmHg).
Thestrongestpossiblemedicationsareindicatedinthesecasestolower
pressureasmuchaspossible(10–12mmHg).
!Earlystages:Itisoftendifficulttodeterminewhethertherapyisindicated
intheearlystages,especiallywhereintraocularpressureiselevated
slightlyabovethresholdvalues.Patientswithlow-tensionglaucoma
exhibitincreasingcuppingoftheopticaldiskevenatnormalpressures
(lessthan22mmHg),whereaspatientswithelevatedintraocularpressure
(25–33mmHg)mayexhibitanunchangedopticnerveforyears.
Patientswithsuspectedglaucomaandriskfactorssuchasafamilyhistoryof
thedisorder,middlemyopia,glaucomaintheothereye,ordifferences
betweentheopticcupinthetwoeyesshouldbemonitoredclosely.Follow-up
examinationsshouldbeperformedthreetofourtimesayear,especiallyfor
patientsnotundergoingtreatment.
Medicaltherapy.Availableoptionsinmedicaltreatmentofglaucoma(seealso
Fig.10.1):
!Inhibitaqueoushumorproduction.
!Increasetrabecularoutflow.
!Increaseuveoscleraloutflow.
Fig.10.14andTable10.3listthevariousactiveingredientsandsubstance
groupsavailableformedicaltreatmentofglaucoma.Forthesakeof
completeness,Fig.10.14alsoliststraditionalsubstancesthatarenolonger
usedtoday;theseincludesubstancesthathavetoomanysideeffectsorhave
beenreplacedbymoreefficientmedications.Table10.3listsonlythosemedi-
cationsthatareactuallyusedtoday.
Principlesofmedicaltreatmentofprimaryopenangleglaucoma:
Medicaltherapyisthetreatmentofchoiceforprimaryopenangleglau-
coma.Surgeryisindicatedonlywheremedicaltherapyfails.
Thereisnoonegenerallyapplicabletherapyplan.However,severalprinciples
maybeformulated:
!Wheremiosisisundesirable,therapyshouldbeginwithbetablockers
(Table10.3).
!Wheremiosisisnotaproblem(asisthecasewithaphakia),therapybegins
withmioticagents.
!Mioticagentsmaybesupplementedwithbetablockers,epinephrine
derivatives,guanethidine,dorzolamideand/orlatanoprostmaximum
topicaltherapy).
!Osmoticagentsorcarbonicanhydraseinhibitors(administeredorallyor
intravenously)inhibittheproductionofaqueoushumor.Theycanbe
administeredtemporarilyinadditiontotopicalmedications.Theirside
effectsusuallymakethemunsuitableforprolongedtreatment.The
generalruleistotrytousetheweakestpossiblemedicationsrequiredto
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273. 255
Optionsinmedicaltreatmentofglaucoma.
Systemic medication
Direct
(cholinergic agents)
Pilocarpine
Carbachol
Aceclidine
Parasympatho-
mimetic agents
Indirect
(cholinesterase
inhibitors)
Reversible
Irreversible
Physostigmine (Eserine)
Neostigmine
Demecarium bromide
Echothiophate iodide
Diisopropyl fluorophosphate
Sympatho-
mimetic
agents
Direct
sympatho-
mimetic
agents
Epinephrine (!- und "-agonist)
Dipivefrin (clonidine central
!
2-agonist)
Apraclonidine, Brimonidine
Sympatho-
lytic agents
Direct
sympatho-
lytic agents
Indirect
sympatho-
lytic agents
Beta blockers
Guanethidine
6-hydroxy dopamine
Carbonic anhydrase
inhibitors
Dorzolamide (eyedrops)
Acetazolamide (systemic)
Dichlorphenamide
Osmotic
agents
Mannitol
Glycerine
Ethyl alcohol
Inhibit
production
of aqueous
humor
Reduce
ocular
volume via
osmotic
gradient
Improve
drainage of
aqueous
humor
Topical eyedrops and ointments
Prostaglandin
analogues
Latanoprost
Fig.10.14
achievenormalpressureovera24-hourperiod:asmuchasnecessary,and
aslittleaspossible.
!Theeffectivenessofanypressure-reducingtherapyshouldbeverifiedby
pressureanalysisonthewardoronanoutpatientbasis.
!Theeffectoftheeyedropsshouldnotinterferewiththepatient’sabilityto
work.Tolerance,effects,andsideeffectsoftheeyedropsshouldbe
repeatedlyverifiedonanindividualbasisduringthecourseoftreatment.
10.3PrimaryGlaucoma
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274. 256
Table10.3Medicaltreatmentofglaucoma
Activeingredientsand
preparations(examples)
ModeofactionIndicationsSideeffects
Parasympathomimetic
agents
!Directparasym-
pathomimetic
agents:
Cholinergicagents
–Pilocarpine
–Carbachol
–Aceclidine
!Improve
drainageof
aqueoushumor
inprimary
openangle
glaucoma.The
effectisprob-
ablypurely
mechanicalvia
contractionof
theciliary
muscleandten-
siononthe
trabecular
meshworkand
scleralspur.
!Inacuteangle
closureglau-
coma,the
forcednarrow-
ingofthepupil
andtheextrac-
tionoftheiris
fromtheangle
oftheanterior
chamberare
mostimpor-
tant.
!Primary
openangle
glaucoma
!Acuteangle
closure
glaucoma
!Youngerpa-
tientsfrequent-
lydonot
toleratethe
temporary
myopiadueto
contractionof
theciliary
muscle.
!Miosiswith
worseningof
thenightvision
andnarrowing
oftheperi-
pheralfieldof
vision.
Continued!
Surgicaltreatmentofprimaryopenangleglaucoma.Indications:
!Medicaltherapyisinsufficient.
!Thepatientdoesnottoleratemedicaltherapy.Reactionsincludeallergy,
reducedvisionduetonarrowingofthepupil,pain,andciliaryspasms,and
ptosis.
!Thepatientisnotasuitablecandidateformedicaltherapyduetolackof
complianceordexterityinapplyingeyedrops.
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275. 257
Table10.3(Continued)
Activeingredientsand
preparations(examples)
ModeofactionIndicationsSideeffects
!Indirectparasym-
pathomimetic
agents:cholin-
esteraseinhibitors
–Neostigmine
!Improve
drainage.Con-
tractionofthe
ciliarymuscle
andsphincter
pupillaemuscle
ismorepro-
nouncedthan
withother
mioticagents.
!Primary
openangle
glaucoma
ifother
miotic
agentsare
nolonger
effective.
!Cholinesterase
inhibitorsare
nolongerrou-
tinelyused
todaybecause
oftheirsignifi-
cantocularand
systemicside
effects.They
areonlyusedin
isolatedcases
suchaswhen
othermedica-
tionsfailto
controlintra-
ocularpressure.
Directsympathomi-
meticagents
–Dipivefrin
(epinephrinederiva-
tive)
!Improve
drainageof
aqueoushumor
andreducepro-
ductionof
aqueoushu-
mor.
!Usedincombi-
nationwith
pilocarpineand
carbonicanhy-
draseinhibitors,
theseagents
alsoreduce
intraocular
pressure.
!Primary
openangle
glaucoma
!10–15%ofpa-
tientsdevelop
anallergy.
!Paradoxicalin-
creaseinintra-
ocularpressure
occasionally
occurs.
!Epinephrinede-
rivativeshave
beenshownto
causecystoid
maculopathyin
patientswith
aphakia.
!Oxidationprod-
uctsofepine-
phrinederiva-
tivesformde-
positsinthe
conjunctiva
(adrenochrome
deposits)and
canleadtoob-
structionofthe
canaliculus(see
Fig.4.24h).
Continued!
10.3PrimaryGlaucoma
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276. 258
Table10.3(Continued)
Activeingredientsand
preparations(examples)
ModeofactionIndicationsSideeffects
Clonidine: !Reduces
intraocular
pressureby
about20%,pri-
marilybyvaso-
constriction
withoutin-
fluencingthe
sizeofthepupil
andaccommo-
dation.
!Particularly
suitablefor
young
patients
withpri-
maryopen
angleglau-
coma.
!Lowersblood
pressure.
Shouldbeused
onlyinlowcon-
centrations
(1/16%and
1/8%)because
theeffecton
intraocular
pressureisthe
sameaswith
higherconcen-
trationsbutthe
sideeffectsare
significantly
less.
Apraclonidine: !Alsoreduces
aqueoushumor
production.
Incontrastto
clonidine,this
agentdoesnot
reducesys-
temicblood
pressure.
!Verygood
reduction
ofintraocu-
larpressure
indecom-
pensated
glaucoma.
!Bewareofcar-
diovasculardis-
ease.
Brimonidine: !Improves
drainageof
aqueoushumor
byreducing
episcleralve-
nouspressure
andreducing
aqueoushumor
productionby
decreasingcili-
arybodyperfu-
sion.
Aswithapra-
clonidine.
Aswithapra-
clonidine.
Continued!
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277. 259
Table10.3(Continued)
Activeingredientsand
preparations(examples)
ModeofactionIndicationsSideeffects
Sympatholyticagents
!Directsympatholytic
agents:betablockers
–Timolol:
–Betaxolol:
–Carteolol:
–Levobunolol:
–Metipranolol:
!Reducepres-
surebyde-
creasingpro-
ductionof
aqueoushumor
withoutin-
fluencingpupil
sizeandaccom-
modation.
!Primary
openangle
glaucoma
!Secondary
openangle
glaucoma
!Secondary
angleclo-
sureglau-
coma
!Reduceheart
rateandin-
creasebron-
chiospasmsin
asthmapa-
tients.
Contraindica-
tions:Beta
blockersshould
usedwithcau-
tioninpatients
withobstruc-
tivelungdis-
ease,cardiac
insufficiency,or
cardiacarrhyth-
miaandonly
afterconsult-
inganinternist.
Absorption
fromtopical
applicationcan
producesys-
temicside
effects.
!Indirectsympatho-
lyticagents:
–Guanethidine:
!Decrease
aqueoushumor
production.
!Reduce
pressure
only
slightly.
!Redeyes.
Continued!
10.3PrimaryGlaucoma
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278. 260
Table10.3(Continued)
Activeingredientsand
preparations(examples)
ModeofactionIndicationsSideeffects
Prostaglandinana-
logues:
–Latanoprost:
!Increase
uveoscleral
aqueoushumor
drainage.
!Suitablefor
allpatients
withpri-
maryopen
angleglau-
coma.
!Adjunctive
therapy
withbeta
blockers,
epine-
phrinederi-
vatives,
pilocarpine,
andcar-
bonicanhy-
draseinhib-
itors.
!Noknownsys-
temicside
effects.
!Localchanges
inthecolorof
theirisin16%
ofallpatients.
Carbonicanhydrase
inhibitors:
–Dorzolamide:
!Reduces
aqueoushumor
production.The
enzymecar-
bonicanhy-
drasecon-
tributestothe
productionof
aqueoushumor
viaactivesecre-
tionofbicar-
bonate.
!Acuteglau-
coma.
!Surgical
procedures
thatcan
increase
intraocular
pressure.
!Prolongedther-
apycauses
malaise,nau-
sea,depres-
sion,anorexia,
weightloss,
anddecreased
libidoin
40–50%of
glaucoma
patients.
–Acetazolamide:
–Dichlorphenamide:
Continued!
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279. 261
Table10.3(Continued)
Activeingredientsand
preparations(examples)
ModeofactionIndicationsSideeffects
Osmoticagents:
–Mannitol:
–Glycerine:
!Decrease
intraocular
pressurepre-
sumablyby
producingan
osmoticpres-
suregradient
bymeansof
thehyper-
osmoticsub-
stancesre-
leasedintothe
bloodstream.
Thisdraws
waterfromthe
fluid-filled
spaces,
especiallyfrom
thevitreous
bodyand
aqueous
humor.
!Exclusively
indicatedin
acutein-
creasesof
intraocular
pressure
suchas
angleclo-
sureglau-
comadue
toitsshort
durationof
action(only
afew
hours).
Argonlasertrabeculoplasty:
!Principle:Laserburnsinthetrabecularmeshworkcausetissuecontrac-
tionthatwidenstheinterveningspacesandimprovesoutflowthroughthe
trabecularmeshwork.
!Technique:Fiftyto100focallaserburnsareplacedintheanteriortrabecu-
larmeshwork(Fig.10.15).
!Comment:Lasersurgeryintheangleofanteriorchamberispossibleonlyif
theangleisopen.Thesurgeryitselfislargelypainless,maybeperformed
asanoutpatientprocedure,andinvolvesfewpossiblecomplications.
Thesemayincludebleedingfromvascularstructuresneartheangleand
synechiaebetweentheirisandindividuallaserburns.Argonlaser
trabeculoplastycanbringimprovementwithintraocularpressuresupto
30mmHg.Itdecreasesintraocularpressurebyabout6–8mHgforabout
twoyears.Argonlasertrabeculoplastyisonlyeffectiveinabouteverysec-
ondpatient.Thefulleffectoccursaboutfourtosixweekspostoperatively.
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280. 262
Argonlasertrabeculoplasty.
**
Iris
Cornea
Trabecular
meshwork
Canal of Schlemm
Ciliary body Lens
Argon laser beam
Trabecularmeshwork
Fig.10.15Anargonlaserbeamisfocusedonthetrabecularmeshworkthrougha
gonioscopeandslitlamp.Approximately100laserburnsareplacedinacircleinthe
trabecularmeshworktoimproveaqueoushumordrainage.
Filtrationsurgery:
!Principle:Theaqueoushumorisdrainedthroughtheanteriorchamber
throughasubconjunctivalscleralopening,circumventingthetrabecular
meshwork.Formationofathin-walledfiltrationblebisasignofsufficient
drainageofaqueoushumor.
!Technique(Fig.10.16a–c):Firstaconjunctivalflapisraised,whichmaybe
eitherfornix-basedorlimbal-based.Thenapartial-thicknessscleralflapis
raised.Accesstotheanteriorchamberisgainedviaagoniotomyperformed
witha1.5mmtrephineatthesclerocornealjunctionorviaarectangular
trabeculectomyperformedwithascalpelanddissectingscissors.Aperiph-
eraliridectomyisthenperformedthroughthisopening.Thescleralflapis
thenlooselyclosedandcoveredwithconjunctiva.
!Comment:Apermanentreductioninintraocularpressureisachievedin
80–85%oftheseoperations.
Cyclodialysis:
!Principle:Theaqueoushumorisdrainedthroughanopeningintothe
suprachoroidalspace.
!Technique:Afull-thicknessscleralincisionismadedowntotheciliary
body4mmposteriortothelimbus.Thescleraisthenseparatedfromthe
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281. 263
Filtrationsurgery.
Fig.10.16
aThetrabecular
meshworkisex-
cisedwithdis-
sectingscissors.
bThepartial-
thicknessscleral
flapisclosedwith
twosutures.
cThepostopera-
tivephotograph
showsapromi-
nentblebbe-
neaththecon-
junctiva.
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282. 264
ciliarybodywitharetractorandretractedanteriorlyintotheanterior
chamber.Theciliarybodyatrophiesintheareaoftheincision,whichalso
helpstodecreasetheproductionofaqueoushumor.
!Comment:Thisprocedureislesscommontodaythanitwasinthe1980s.
Onereasonforthisisthatitisdifficulttogaugeaccuratelytheatrophyto
theciliarybody.Occasionallyseverehypotoniaoftheglobewillresult,
whichthenrequiressurgicalinterventiontoclosethedialysisopening.
Cycloablation:
!Principle:Atrophyisinducedinportionsoftheciliarybodythroughthe
intactscleratoreduceintraocularpressurebydecreasingtheamountof
tissueproducingaqueoushumor.
!Technique:
–Cyclocryotherapy:Acryoprobeisusedtofreezetheciliarybodyat
severalpointsthroughthesclera.Thisprocedurecanberepeatedifnec-
essary;theinterventionshaveacumulativeeffect.
–Cyclodiathermy:Thismethodissimilartocyclocryotherapyexceptthat
adiathermyneedleisadvancedthroughthescleraintotheciliarybody
tocauterizeitwithheat.Theproceduremaybeperformedwithor
withoutpriordissectionofapartial-thicknessscleralflap.
–LasercycloablationinducesatrophyintheciliarybodyusingYAGlaser
orhigh-energydiodelaserpulses.
–Ultrasounddisruptioninducesatrophyintheciliarybodywithhigh-
frequencyultrasoundwaves.Theselasttwoformsoftherapyhavebeen
developedtoinduceatrophymoreeffectively,moreaccurately,andin
morecontrolleddoses,whichislesstraumaticfortheeye.
!Comment:Alltheseformsofcycloablationareirreversibleandcauseper-
manenthypotonia.Therefore,theyrepresentthelastlineoftreatment
options.
Prophylaxis:Noprophylacticactioncanbetakentopreventprimaryopen
angleglaucoma.
Earlydiagnosisiscrucialandcanonlybemadebyanophthalmologist.
Bytheageof40atthelatest,patientsshouldhavetheirintraocular
pressuremeasuredregularly.Theophthalmologistperformsregular
glaucomascreeningexaminationsofintraocularpressureandpupil.
Therefore,thefirstpairofreadingeyeglassesshouldalwaysbepre-
scribedbyanophthalmologist.
Prognosis:Theprognosisdependsgreatlyonthestageatwhichprimary
openangleglaucomaisdiagnosed.Asageneralrule,therapyismoreeffective
theearlieritcanbeinitiated.
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283. 265
10.3.2PrimaryAngleClosureGlaucoma
Definition
Acuteepisodicincreaseinintraocularpressuretoseveraltimesthenormal
value(10–20mmHg)duetosuddenblockageofdrainage.Productionof
aqueoushumorandtrabecularresistancearenormal.
Epidemiology:Theincidenceamongpersonsovertheageof60isoneper
thousand.Womenarethreetimesaslikelytobeaffectedasmen.Inuitare
morefrequentlyaffectedthanotherethnicgroups,whereasthedisorderis
rareinblacks.
Etiology:(Seealsophysiologyandpathophysiologyofaqueoushumorcircu-
lation):Anatomicallypredisposedeyeswithshallowanteriorchambers(see
Fig.10.1)posearelativeimpedimenttotheflowofaqueoushumorthrough
thepupil.Thispupillaryblockincreasesthepressureintheposteriorcham-
ber(Fig.10.18a).Thepressuredisplacestheirisanteriorlytowardthe
trabecularmeshwork,suddenlyblockingtheoutflowofaqueoushumor
(angleclosure).Atypicalglaucomaattackoccursunilaterallydueto
wideningofthepupileitherindarksurroundingsand/orunderemotional
stress(dismayorfear).Atypicalsituationistheeveningmysterymovieon
television.Iatrogenicpharmacologicmydriasisandsystemicpsychotropic
drugscanalsotriggeraglaucomaattack.
Bearinmindthatmydriaticagentsentailariskoftriggeringaglaucoma
attackbywideningthepupil.Therefore,itisimportanttoevaluatethe
depthoftheanteriorchamberineverypatientevenpriortoaroutine
fundusexamination.
Symptoms:Acuteonsetofintensepain.Theelevatedintraocularpressure
actsonthecornealnerves(theophthalmicnerveorfirstbranchofthe
trigeminalnerve)tocausedullpain.Thispainmaybereferredtothetemples,
backofthehead,andjawsviathethreebranchesofthetrigeminalnerve,
whichcanmaskitsocularorigin.
Nauseaandvomitingoccurduetoirritationofthevagusnerveandcansimu-
lateabdominaldisorders.Thegeneralizedsymptomssuchasheadache,
vomiting,andnauseamaydominatetotheextentthatthepatientfailsto
noticelocalsymptoms.
Diminishedvisualacuity.Patientsnoticeobscuredvisionandcoloredhalos
aroundlightsintheaffectedeye.Thesesymptomsarecausedbythecorneal
epithelialedemaprecipitatedbytheenormousincreaseinpressure.
Prodromalsymptoms.Patientsreporttransitoryepisodesofblurredvision
ortheappearanceofcoloredhalosaroundlightspriortotheattack.These
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284. 266
prodromalsymptomsmaygounnoticedormaybedismissedasunimportant
bythepatientinmildepisodeswheretheeyereturnstonormal.Earlyidenti-
ficationofthoseriskpatientswithshallowanteriorchambersandgonio-
scopicfindingsisimportantasdamagetothestructuresoftheanglemaybe
welladvancedbeforeclinicalsymptomsappear.
Thefullclinicalsyndromeofacuteglaucomawillnotalwaysbepresent.
Thediminishedvisualacuitymaygounnoticediftheothereyehasnor-
malvision.Patients’subjectiveperceptionofpainintensitycanvary
greatly.
Diagnosticconsiderations(Fig.10.17):
Thediagnosisismadeonthebasisofatriadofsymptoms:
!Unilateralredeyewithconjunctivalorciliaryinjection.
!Fixedanddilatedpupil.
!Hardeyeballonpalpation.
Acuteglaucomaattack:pupillaryblock.
Fig.10.17Typicalsymptomsinclude:
!Conjunctivalandciliaryinjection(redeye).
!Cornealedema.
!Dull,non-reflectingsurfacewithdullcornealreflex.
!Opacificationofthecornealstromathatobscurestheviewofthefundus.Theiris
appearsfaded,andtheanteriorchamberisshallow.
!Thepupilisovalinsteadofround,andisfixedandmoderatelydilated.
!Intraocularpressureiselevated;theeyeisrockhardtopalpation.
!Severeheadacheandgastrointestinalsymptomsarepresent.
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285. 267
Otherfindings.
!Thecorneaisdullandsteamywithepithelialedema.
!Theanteriorchamberisshalloworcompletelycollapsed.Thiswillbe
apparentwhentheeyeisilluminatedbyafocusedlaterallightsource
(Fig.1.12,p.12)anduponslit-lampexamination.Inspectionoftheshallow
anteriorchamberwillbedifficult.Detailsofthesurfaceoftheiriswillbe
visible,andtheiriswillappearfaded.
!Thefundusisgenerallyobscuredduetoopacificationofthecorneal
epithelium.Whenthefunduscanbevisualizedassymptomssubsideand
thecorneaclears,thespectrumofchangestotheopticdiskwillrangefrom
anormalvitalopticdisktoanill-definedhyperemicopticnerve.Inthelat-
tercase,venouscongestionwillbepresent.Thecentralarteryoftheretina
willbeseentopulseontheopticdiskasbloodcanonlyentertheeye
duringthesystolicphaseduetothehighintraocularpressure.
!Visualacuityisreducedtoperceptionofhandmotions.
Differentialdiagnosis:Misdiagnosisispossibleasthewidevarietyofsymp-
tomscansimulateotherdisorders.
!Generalsymptomssuchasheadache,vomiting,andnauseaoftenpredom-
inateandcaneasilybemistakenforappendicitisorabraintumor.
!Iniritisandiridocyclitis,theeyeisalsoredandtheirisappearsfaded.
However,intraocularpressuretendstobedecreasedratherthanelevated.
Treatment:
Anacuteglaucomaattackisanemergency,andthepatientrequires
immediatetreatmentbyanophthalmologist.Theunderlyingcausesof
thedisorderrequiresurgicaltreatment,althoughinitialtherapyiscon-
servative.
Medicaltherapy.Goalsofconservativetherapy:
!Decreaseintraocularpressure.
!Allowthecorneatoclear(importantforsubsequentsurgery).
!Relievepain.
Timefactorinreducingintraocularpressure:
Conservativetreatment
Withinsixhours Notwithinsixhours
Surgerythenextday Immediatesurgery
Principlesofmedicaltherapyinprimaryangleclosureglaucoma(seeFig.10.3):
!Osmoticreductioninthevolumeofthevitreousbodyisachievedviasys-
temichyperosmoticsolutions(oralglycerin,1.0–1.5g/kgofbodyweight,or
intravenousmannitol,1.0–2.0g/kgofbodyweight).
10.3PrimaryGlaucoma
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286. 268
!Productionofaqueoushumorisdecreasedbyinhibitingcarbonicanhy-
drase(intravenousacetazolamide,250–500mg).Bothstepsaretakenini-
tiallytoreduceintraocularpressuretobelow50–60mmHg.
!Theirisiswithdrawnfromtheangleoftheanteriorchamberbyadminis-
teringtopicalmioticagents.Pilocarpine1%eyedropsshouldbeapplied
every15minutes.Ifthisisnoteffective,pilocarpinecanbeappliedmore
often,everyfiveminutes,andinconcentrationsupto4%.Mioticagentsare
notthemedicationsoffirstchoicebecausethesphincterpupillaemuscle
isischemicatpressuresexceeding40–50mmHgandwillnotrespondto
mioticagents.Mioticagentsalsorelaxthezonulefibers,whichcauses
anteriordisplacementofthelensthatfurthercompressestheanterior
chamber.Thismakesitimportanttofirstinitiatetherapywithhyper-
osmoticagentstoreducethevolumeofthevitreousbody.
!Symptomatictherapywithanalgesicagents,antiemeticagents,andseda-
tivesmaybeinitiatedwherenecessary.
Mechanicalindentationofthecornea:Simplerepetitiveindentationofthe
centralcorneawithamusclehookorglassrodforapproximately15–30sec-
ondspressestheaqueoushumorintotheperipheryoftheangleoftheante-
riorchamber,whichopenstheangle.Ifthismanipulationsucceedsinkeep-
ingthetrabecularmeshworkopenforafewminutes,itwillpermitaqueous
humortodrainandreduceintraocularpressure.Thisimprovestheresponse
topilocarpineandhelpsclearupthecornea.
Surgicalmanagement(shuntbetweentheposteriorandanteriorcham-
bers).Oncethecorneaisclear,theunderlyingcausesofthedisorderaretreated
surgicallybycreatingashuntbetweentheposteriorandanteriorchambers.
Neodymium:yttrium-aluminum-garnetlaseriridotomy(nonincisionalpro-
cedure):TheNd:YAGlasercanbeusedtocreateanopeningintheperipheral
iris(iridotomy)bytissuelysiswithouthavingtoopentheglobe(Figs.10.18
a–c).Theoperationcanbeperformedundertopicalanesthesia(Fig.10.19).
Peripheraliridectomy(incisionalprocedure):Wherethecorneaisstillswollen
withedemaortheirisisverythick,anopenproceduremayberequiredto
createashunt.Alimbalincisionismadeat12o’clockundertopicalanesthesia
orgeneralanesthesia,throughwhichabasaliridectomyisperformed.Today
peripheraliridectomyisrarelyperformed,inonlyin1–2%ofallcases.
Prophylaxis:Whenthepatientreportsclearprodromalsymptomsandthe
angleoftheanteriorchamberappearsconstricted,thesafestprophylaxisisto
performaNd:YAGlaseriridotomyorperipheraliridectomy.Ifoneeyehas
alreadysufferedanacuteattack,thefelloweyeshouldbetreatedinitially
every4–6hourswithpilocarpine1%tominimizetheriskofaglaucoma
attack.ThesecondeyeshouldthenbetreatedwithaNd:YAGlasertoprevent
glaucomaoncesurgicalstabilizationofthefirsteyehasbeenachieved.
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287. 269
Acuteangleclosureglaucoma:etiologyandtreatment.
**
Fig.10.18aThepupillaryblock(asterisk)preventstheoutflowofaqueoushumor
intotheanteriorchamber.Thepressureintheposteriorchamberincreases(redar-
rows),andtheperipheralirisispressedagainstthetrabecularmeshwork.Thisblocks
drainageoftheaqueoushumorandcreatesanacuteangleclosure(arrow).
bANd:YAGlaserbeamfocusedthroughacontactlensburnsacircumscribedholein
thetissueoftheiristocreateashuntbetweentheposteriorandanteriorchambers
(arrow).Thispermitstheaqueoushumortoflowintotheanteriorchamberdespite
thepersistingpupillaryblock(asterisk).
cTheaqueoushumortrappedintheposteriorchambernowflowsthroughthis
newlycreatedopeningintheiris,equalizingthepressureinthetwochambersand
circumventingthepupillaryblock.Theirisrecedesintoitsnormalposition,the
trabecularmeshwork(arrow)isopenedagain,theaqueoushumorcandrainnor-
mally,andnormalintraocularpressureisrestored.Nofuturepupillaryblockcan
formfollowingNd:YAGlaseriridotomy.
**
Laser beam
**
a
b
c
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288. 270
Nd:YAGlaseriridotomy.
Fig.10.19TheNd:YAG
laseropeningintheiris
(arrow)createsashunt
betweentheposterior
andanteriorchambers.
Prognosis:Onecanusuallyreadilyreleaseapupillaryblockandlower
intraocularpressureinaninitialattackwithmedicationandpermanently
preventfurtherattackswithsurgery.However,recurrentacuteangleclosure
glaucomaorangleclosurepersistinglongerthan48hourscanproduce
peripheralsynechiabetweentherootoftheirisandthetrabecularmeshwork
oppositeit.Thesepersistingcasesofangleclosureglaucomacannotbecured
byNd:YAGlaseriridotomyoriridectomy,andtheangleclosurewillpersist
despitesurgery.Filtrationsurgeryisindicatedinthesecases.
Whereintraocularpressureiscontrolledandthecorneaisclear,gonios-
copyisindicatedtodemonstratethattheangleisopenagainandto
excludepersistentangleclosure.
10.4SecondaryGlaucomas
Definition
Theseglaucomasarecausedbyotheroculardiseasesoffactorssuchasinflam-
mation,trauma,bleeding,tumors,medication,andphysicalorchemical
influences(seeTable10.1).
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289. 271
10.4.1SecondaryOpenAngleGlaucoma
Definition
Theanatomicrelationshipsbetweentherootoftheiris,thetrabecularmesh-
work,andperipheralcorneaarenotdisturbed.However,thetrabecularmesh-
workiscongestedandtheresistancetodrainageisincreased.
Themostimportantforms:Pseudoexfoliativeglaucoma.Thisformoccurs
particularlyfrequentlyinScandinaviancountries.Depositsofamorphous
acellularmaterialformthroughouttheanteriorchamberandcongestthe
trabecularmeshwork.
Pigmentaryglaucoma.Youngmyopicmentypicallyareaffected.Thedis-
orderischaracterizedbyreleaseofpigmentgranulesfromthepigmentary
epitheliumoftheiristhatcongestthetrabecularmeshwork.
Cortisoneglaucoma.Thirty-fivetofortypercentofthepopulationreactto
three-weektopicalorsystemicsteroidtherapywithelevatedintraocular
pressure.Increaseddepositsofmucopolysaccharidesinthetrabecularmesh-
workpresumablyincreaseresistancetooutflow;thisisreversiblewhenthe
steroidsarediscontinued.
Inflammatoryglaucoma.Twomechanismscontributetotheincreasein
intraocularpressure:
1.Theviscosityoftheaqueoushumorincreasesasaresultoftheinfluxofpro-
teinfrominflamedirisvessels.
2.Thetrabecularmeshworkbecomescongestedwithinflammatorycellsand
cellulardebris.
Phacolyticglaucoma.Thisisacuteglaucomaineyeswithmatureorhyper-
maturecataracts.Denaturedlensproteinpassesthroughtheintactlenscap-
suleintotheanteriorchamberandisphagocytized.Thetrabecularmeshwork
becomescongestedwithprotein-bindingmacrophagesandtheproteinitself.
10.4.2SecondaryAngleClosureGlaucoma
Definition
Insecondaryangleclosureglaucomaasinprimaryangleclosureglaucoma,
theincreaseinintraocularpressureisduetoblockageofthetrabecularmesh-
work.However,theprimaryconfigurationoftheanteriorchamberisnotthe
decisivefactor.
Themostimportantcauses:Rubeosisiridis.Neovascularizationdrawsthe
angleoftheanteriorchambertogetherlikeazipper(neovascularglaucoma).
10.4SecondaryGlaucomas
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290. 272
Ischemicretinaldisorderssuchasdiabeticretinopathyandretinalveinocclu-
sioncanleadtorubeosisiridiswithprogressiveclosureoftheangleofthe
anteriorchamber.Otherformsofretinopathyorintraoculartumorscanalso
causerubeosisiridis.Theprognosisforeyeswithneovascularglaucomais
poor(seeFig.10.20aandb).
Trauma.Post-traumaticpresenceofbloodorexudateintheangleoftheante-
riorchamberandprolongedcontactbetweentheirisandtrabecularmesh-
workinacollapsedanteriorchamber(followinginjury,surgery,orinsuffi-
cienttreatmentofprimaryangleclosure)canleadtoanteriorsynechiaeand
angleclosurewithoutrubeosisiridis.
Neovascularglaucoma:secondaryangleclosureglaucomawith
rubeosisiridis.
Fig.10.20
aRubeosisiridis:
Neovascularization
(arrow)isvisibleonthe
surfaceoftheiris.Con-
tractionevertstheposte-
riorpigmentedepithe-
liumoftheirisontothe
anteriorsurfaceoftheiris
(arrow)inacondition
knownasectropion
uveae.
bGonioscopy:Theangle
oftheanteriorchamberis
closed,andthetrabecular
meshworkisnolonger
visible(arrow).Rubeosis
iridishasdrawntheangle
oftheanteriorchamber
togetherlikeazipper.
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291. 273
Treatmentofsecondaryglaucomas:
Medicaltherapyofsecondaryglaucomasisusuallyidenticaltothe
treatmentofprimarychronicopenangleglaucoma.
Secondaryglaucomasmaybecausedbymanydifferentfactors,andtheangle
maybeopenorclosed.Therefore,treatmentwilldependontheetiologyof
theglaucoma.Theunderlyingdisorderisbesttreatedfirst.Glaucomaswith
uveitis(suchasiritisoriridocyclitis)initiallyaretreatedconservativelywith
anti-inflammatoryandantiglaucomaagents.Surgeryisindicatedwherecon-
servativetreatmentisnotsufficient.
Theprognosisforsecondaryglaucomasisgenerallyworsethanforpri-
maryglaucomas.
10.5ChildhoodGlaucomas
Definition
Anyabnormalincreaseinintraocularpressureduringthefirstyearsoflifewill
causedilatationofthewalloftheglobe,andespeciallyofthecornea.The
resultisacharacteristic,abnormallylargeeye(buphthalmos)withaprogress-
iveincreaseincornealdiameter.Thisisalsoreferredtoashydrophthalmosor
hydrophthalmia.
Epidemiology:Glaucomasinchildrenoccuronceevery12000–18000
birthsandaccountforabout1%ofallglaucomas.Primarycongenitalglau-
comaisaninheritedautosomalrecessivedisorder.Itisbilateralinapproxi-
mately70%ofallcases;boysareaffectedinapproximately70%ofallcases;
andglaucomamanifestsitselfbeforetheageofsixmonthsinapproximately
70%ofallcases.
Todaythereiswidespreadpublicawarenessofglaucomainadults.
Unfortunately,thisdoesnotyetapplytoglaucomainchildren.
Etiology:(Seealsophysiologyandpathophysiologyofaqueoushumorcircu-
lation):Theirisinsertsanteriorlyfarinthetrabecularmeshwork(Fig.10.2).
Embryonicmesodermaltissueintheformofathintransparentmembrane
(Barkan’smembrane)coversthetrabecularmeshworkandimpedestheflow
ofaqueoushumorintothecanalofSchlemm.Otherabnormalocularorsys-
temicfindingsarelacking.
Asidefromisolatedbuphthalmos,otherocularchangescanleadtosec-
ondaryhydrophthalmos.Theseinclude:
!Hydrophthalmiawithoculardevelopmentalanomalies.
!Hydrophthalmiawithsystemicdisease.
!Secondarybuphthalmosresultingfromacquiredeyedisorders.
10.5ChildhoodGlaucomas
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292. 274
Regardlessofthecauseoftheincreaseinintraocularpressure,theobjective
signsandclinicalsymptomsofchildhoodformsofglaucomaareidenticaland
shouldbeapparenttoanyexaminingphysician.
Symptoms:Classicsignsincludephotophobia,epiphora,cornealopacifica-
tion,andunilateralorbilateralenlargementofthecornea.Thesechangesmay
bepresentfrombirth(incongenitalglaucoma)ormaydevelopshortlyafter
birthorduringthefirstfewyearsoflife.
Childrenwiththisdisorderareirritable,pooreaters,andrubtheireyes
often.Thebehaviorofsomechildrenmayleadonetosuspectmentalretarda-
tion.
Physiciansshouldbealerttoparentswhoboastabouttheirchild’s“big
beautifuleyes”andshouldmeasureintraocularpressure.
Itisessentialtodiagnosethedisorderasearlyinthechild’slifeaspossibleto
minimizetheriskoflossoforirreparabledamagetothechild’svision.
Diagnosticconsiderations:Theseexaminationsmaybeperformedwithout
generalanesthesiainmanychildren.However,generalanesthesiawillocca-
sionallybenecessarytoconfirmthediagnosisespeciallyinolderchildren
(Fig.10.21).
10Glaucoma
Congenitalglaucoma.
Fig.10.21Examinationofathree-
month-oldinfantwithbuphthalmos
undergeneralanesthesia.Findingsin-
cludeacornealdiameterof14.0mm
(normaldiameterisapproximately
9.5mm)andstromalopacification.
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293. 275
Measurementofintraocularpressure.Oneshouldgenerallyattemptto
measureintraocularpressurebyapplanationtonometry(tonometrywitha
hand-heldtonometer).
Measurementisfacilitatedbygivingthehungryinfantabottleduring
theexamination.Feedingdistractsthebaby,andameasurementusu-
allycanbeobtainedeasily.Suchameasurementisusuallyfarmore
accuratethanoneobtainedundergeneralanesthesiaasnarcotics,
especiallybarbituratesandhalothane,reduceintraocularpressure.
Opticdiskophthalmoscopy.Theopticcupisaverysensitiveindicatorof
intraocularpressure,particularlyinthephaseinwhichpermanentvisual
fielddefectsoccurs.Asymmetryintheopticcupcanbehelpfulindiagnosing
thedisorderandinfollow-up.
Specialconsiderations:Aglaucomatousopticcupinchildrenmaywellbe
reversible.Oftenitwillbesignificantlysmallerwithinseveralhoursofa
successfultrabeculotomy.
Inspectionofthecornea.Thecorneawillappearwhitishandopacifieddueto
epithelialedema.BreaksinDescemet’smembranecanexacerbatean
epithelialorstromaledema.Theselesions,knownasHaab’sstriae,will
exhibitatypicalhorizontalorcurvilinearconfiguration.
Theenlargedcornealdiameterisacharacteristicfinding.Thecorneanor-
mallymeasures9.5mmonaverageinnormalnewborninfants.Enlargement
tomorethan10.5mmsuggestschildhoodglaucoma.Chronicallyelevated
intraocularpressureinchildrenundertheageofthreewillleadtoenlarge-
mentoftheentireglobe.
Gonioscopyoftheangleoftheanteriorchamber.Examinationoftheangle
oftheanteriorchamberprovidescrucialetiologicinformation.Theanglewill
notbefullydifferentiated.Embryonictissuewillbeseentooccludethe
trabecularmeshwork.
Differentialdiagnosis:Largeeyes.Alargecornealdiametercanoccurasa
harmlessanomaly(megalocornea).
Cornealopacification.Diffusecornealopacificationwithepithelialedema
occursincongenitalhereditaryendothelialdystrophy.Opacificationwith-
outepithelialedemaoccursinmucopolysaccharidosis(Hurler’ssyn-
drome,Scheie’ssyndrome,Morquio’ssyndrome,andMaroteaux-Lamysyn-
drome).
StriaeinDescemet’smembrane.IncontrasttothehorizontalHaab’sstriaein
congenitalglaucoma,endothelialbreakscanalsooccurasaresultofinjury
duringaforcepsdelivery(verticalstriae),inkeratoconus,andindeepker-
atitis.
10.5ChildhoodGlaucomas
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294. 276
Noneofthesedifferentialdiagnosesareaccompaniedbyelevated
intraocularpressure.
Treatment:Childhoodglaucomasaretreatedsurgically.Theprognosis
improvestheearliersurgeryisperformed.
Principleandprocedureofgoniotomy.Withagonioscopeinplaceonthe
eye,thegoniotomyscalpelisadvancedthroughtheanteriorchambertothe
trabecularmeshwork.Thetrabecularmeshworkcannowbeincisedasfarthe
canalofSchlemmoveranarcofabout120degreestopermitdrainageofthe
aqueoushumor.Oftentwoorthreegoniotomiesatdifferentlocationsare
requiredtocontrolintraocularpressure.Theseoperationscanonlybeper-
formedwhenthecorneaisclearenoughtoallowvisualizationofthestruc-
turesoftheanteriorchamber.
Principleandprocedureoftrabeculotomy.Afteraconjunctivalflapand
split-thicknessscleralflaphavebeenraised,accesstothecanalofSchlemmis
gainedthrougharadialincision,andthecanalisprobedwithatrabeculo-
tome.Thenthetrabeculotomeisrotatedintotheanteriorchamber
(Fig.10.22).Thistearsthroughtheinnerwallofthecanal,thetrabecular
meshwork,andanyembryonictissuecoveringittoopenadrainageroutefor
theaqueoushumor.
Ahigherrateofsuccessisattributedtotrabeculotomywhenperformedas
aninitialprocedure.Thisoperationcanalsobeperformedwhenthecorneais
largelyopacified.
Prognosis:Goniotomiesandtrabeculotomiesarenotalwayssuccessful.
Evenafterapparentlysuccessfulinitialtrabecularsurgery,thesechildren
requirealifetimeoffollow-upexaminations(initiallyseveraltimesayearand
lateronceeveryyear)aselevatedintraocularpressurecanrecur,inwhich
caserepeatgoniotomyortrabeculotomyisindicated.
10Glaucoma
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295. 277
Trabeculotomy.
Fig.10.22
aA12o’clockincision
ismadetoexposethe
canalofSchlemm,
whichisthenprobed
withatrabeculo-
tome.Thenthe
trabeculotomeisro-
tatedintotheante-
riorchamber,tearing
throughtheembry-
onictissueoccluding
theangle.The
aqueoushumorcan
nowreadilydraininto
thecanalof
Schlemm.
bThesurgeoncan
observetherota-
tionofthe
trabeculotome
directlythrougha
gonioscopeplaced
ontheeyeduring
theoperation.
cRightandlefteyes
followingsuccessful
trabeculotomy
(photographshows
thesamechildasin
Fig.10.21).Both
eyesexhibitaclear
cornea(normalcor-
neallightreflex)
andnormalin-
traocularpressure.
Cornea
Lens
Ciliary
body
Trabecularmeshwork
10.5ChildhoodGlaucomas
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297. 279
11VitreousBody
ChristophW.SpraulandGerhardK.Lang
11.1 BasicKnowledge
Importanceofthevitreousbodyfortheeye:Thevitreousbodystabilizes
theglobealthoughtheeyecanremainintactwithoutthevitreousbody(see
vitrectomy).Italsopreventsretinaldetachment.
Embryology:Thedevelopmentofthevitreousbodycanbedividedintothree
phases:
!Firstphase(firstmonthofpregnancy;fetusmeasures5–13mmcranium
tococcyx):Theprimaryvitreousformsduringthisperiod.Thisphaseis
characterizedbytheentryofmesenchymeintotheopticcupthroughthe
embryonicchoroidalfissure.Themainfunctionoftheprimaryvitreousisto
supplythedevelopinglenswithnourishment.Inkeepingwiththisfunc-
tion,itconsistsmainlyofavascularplexus,theanteriorandposterior
tunicavasculosalentis,thatcoverstheanteriorandposteriorsurfacesof
thelens.Thisvascularplexusissuppliedbythehyaloidarteryandits
branches(Fig.11.1).Thisvascularsystemandtheprimaryvitreousregress
astheposteriorlenscapsuledevelopsattheendofthesecondmonthof
pregnancy.
!Secondphase(secondmonthofpregnancy;fetusmeasures14–70mm
craniumtococcyx):Thesecondaryvitreousformsduringthisperiod.This
avascularvitreousbodyconsistingoffineundulatingcollagenfibers
developsfromwhatlaterbecomestheretina.Innormaldevelopmentit
expandstocompressthecentralprimaryvitreousintoaresidualcentral
canal(hyaloidcanalorCloquet’scanal).
!Thirdphase(thirdmonthofpregnancy;fetusmeasures71–110mm
craniumtococcyx):Thetertiaryvitreousdevelopsfromexistingstruc-
turesinthesecondaryvitreous.Thesecondaryvitreousremains.The
zonulefibersthatformthesuspensoryligamentofthelensdevelopduring
thisperiod.
Compositionofthevitreousbody:Thegelatinousvitreousbodyconsistsof
98%waterand2%collagenandhyaluronicacid.Itfillsthevitreouschamber,
whichaccountsforapproximatelytwo-thirdsofthetotalvolumeoftheeye.
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298. 280
Transitoryembryonicvascularsupply.
LensIridohyaloid
vessels
Anterior tunica
vasculosa lentis
and pupillary
membrane
Posterior tunica
vasculosa lentis
Trunk of the
hyaloid artery
and envelope
of glial tissue
Long posterior
ciliary arteries
Fig.11.1Theanteriortunicavasculosalentis(darkred)formsanastomoseswith
theposteriortunicavasculosalentis(lightred)throughtheiridohyaloidvessels.
Stabilizationandconfinesofthevitreousbody:Withtheirhighnegative
electrostaticpotential,thehyaluronicacidmoleculesfillthethree-dimen-
sionalcollagenfibernetworkandprovidemechanicalstability.Condensation
ofperipheralcollagenfibrilscreatesaboundarymembrane(hyaloidmem-
brane),whichisnotabasementmembrane.Itisattachedtoadjacentstruc-
turesatthefollowinglocations(Fig.11.2):
!AttheligamentofWiegeralongtheposteriorcapsuleofthelens.
!Atthevitreousbaseattheoraserrata.
!AtthefunnelofMartegiani(approximately10µmwide)surroundingthe
peripheryoftheopticdisk.
Theconnectionsbetweenthevitreousbodyandretinaaregenerallyloose
althoughtheremaybefirmfocaladhesions.Thesefirmerfocalattachments
causeproblemsduringvitreousdetachmentbecausetheydonotpermitthe
vitreousbodytobecomecompletelydetached.Thefocaladhesionsbetween
thevitreousbodyandretinaproducefocaltractionforcesthatactontheret-
inaandcancauseretinaltearsanddetachment.
11VitreousBody
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299. 281
Attachmentsofthevitreousbodyandadjacentspaces.
Martegiani's funnel
(attachment at the
optic disc)
Hannover's canal
Vitreous
base
(attachment
to the ora
serrata)
Wieger's band
(attachment to
the posterior
lens capsule)
Garnier's space
Zonular
spaces
(Petit's
canals)
Berger's
space
Hyaloid
canal
Egger's line
Fig.11.2Attachmentsofthevitreousbodyareidentifiedbythickredlinesand
listedontheleft.Spacesadjacenttothevitreousbodyareshowningreenandlisted
ontheright.
Neurovascularsupply:Thevitreousbodycontainsneitherbloodvesselsnor
nerves.Asaresult,pathogenscanmultiplyundisturbedforarelativelylong
timebeforetheonsetofanimmuneresponsefromadjacentstructures.
11.2ExaminationMethods
Theanteriorthirdofthevitreousbodycanbereadilyexaminedwithaslit
lamp.Anadditionalcontactlensorhand-heldcondensinglens(+60,+78,
and+90diopters)isrequiredtoexaminetheposteriorportions.Indirect
ophthalmoscopyorretroillumination(Brückner’stest)isusuallyusedto
examinethevitreousbodyinitsentirety.Opacitieswillappearasdark
shadows.Ultrasoundexaminationofthevitreousbodyisperformedincases
suchasamaturecataractwherevisualizationbyothermethodsisnot
possible.
11.2ExaminationMethods
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300. 282
11.3 AgingChanges
11.3.1Synchysis
Theregulararrangementofcollagenfibersgraduallydeterioratesinmiddle
age.Thefiberscondensetoflattenedfilamentousstructures.Thisprocess,
knownasliquefaction,createssmallfluid-filledlacunaeinthecentralvit-
reousbodythatinitiallyarelargelyasymptomatic(patientsmayreport
floaters).However,onceliquefactionhasprogressedbeyondacertainpoint,
thevitreousbodycancollapseanddetachfromtheretina.
11.3.2VitreousDetachment
Definition
Completeorpartialdetachmentofthevitreousbodyfromitsunderlying
tissue.Themostcommonformisposteriorvitreousdetachment(see
Fig.11.3a);anteriororbasalvitreousdetachmentismuchrarer.
Epidemiology:Sixpercentofpatientsbetweentheagesof54and65and65%
ofallpatientsbetweentheagesof65and85haveposteriorvitreousdetach-
ment.Patientswithaxialmyopiahaveapredispositiontoearlyvitreous
detachment.Presumablythevitreousbodycollapsesearlierinthesepatients
becauseitmustfilla“longer”eyewithalargervolume.
Etiology:Liquefactioncausescollapseofthevitreousbody.Thisusually
beginsposteriorlywheretheattachmentstotheunderlyingtissueareleast
welldeveloped.Detachmentintheanteriorregion(anteriorvitreousdetach-
ment)orintheregionofthevitreousbase(basalvitreousdetachment)usually
onlyoccurswherestrongforcesactontheglobeasinoculartrauma.
Symptomsandfindings:Collapseofthevitreousbodyleadstovitreousdensi-
tiesthatthepatientperceivesasmobileopacities.Thesefloaters(alsoknown
asfliesorcobwebs)maytaketheformofcircularorserpentinelinesorpoints.
Thevitreousbodymaydetachpartiallyorcompletelyfromtheretina.An
increasedriskofretinaldetachmentispresentonlywithpartialvitreous
detachment.Inthiscase,thevitreousbodyandretinaremainattached,with
theresultthateyemovementsinthisregionwillplacetractionontheretina.
Thepatientperceivesthisphenomenonasflashesoflight.Ifthetractionon
theretinabecomestoostrong,itcantear(seeretinaltearsinposteriorvit-
reousdetachment,Fig.11.3b–c).Thisincreasestheriskofretinaldetachment
andvitreousbleedingfrominjuredvessels.
Floatersandespeciallyflashesoflightrequirethoroughexaminationof
theocularfundustoexcludearetinaltear.
11VitreousBody
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301. 283
Retinaltearsinposteriorvitreousdetachment.
Fig.11.3aCompleteposteriorvit-
reousdetachment(arrows).bThiscan
producetractionattheposteriorat-
tachmentofthebaseofthevitreous
bodytotheretina,causingretinal
tears.cAutopsyfindingofamanifest
retinaltearwithtractionofthevit-
reousbodyattheedgeoftheopening
(arrow).
Attachment
of vitreous
body to retina
Horseshoe tear
Round or
oval hole in
the retina
b
a b
c
Diagnosticconsiderations:Thesymptomsofvitreousdetachmentrequire
examinationoftheentirefundusoftheeyetoexcludearetinaldefect.Incases
suchaslensopacificationorvitreoushemorrhagewherevisualizationisnot
possible,anultrasoundexaminationisrequiredtoevaluatethevitreousbody
andretina.
Vitreousdetachmentintheregionoftheattachmentattheopticdisk
(funnelofMartegiani)willappearasasmokeyring(Weiss’ring)under
ophthalmoscopy.
11.3AgingChanges
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302. 284
Treatment:Thesymptomsofvitreousdetachmentresolvespontaneously
oncethevitreousbodyiscompletelydetached.However,thecomplications
thatcanaccompanypartialvitreousdetachmentrequiretreatment.These
includeretinaltears,retinaldetachment(fortreatmentseeChapter12,Ret-
ina),andvitreoushemorrhage.
11.4 AbnormalChangesintheVitreousBody
11.4.1PersistentFetalVasculature(DevelopmentalAnomalies)
Theembryonicvascularsysteminthevitreousbodyandlensnormallydisap-
pearscompletely,leavingonlythehyaloidcanal.Persistenceofthevascular
systemisreferredtoaspersistentfetalvasculature.Thefollowingsection
describesthevaryingdegreesofseverityofthissyndromeastheyrelateto
thevitreousbody.Persistenceoftheanteriortunicavasculosalentisleadstoa
persistentpupillarymembrane.
11.4.1.1Mittendorf’sDot
Mittendorf’sdotisasmallvisuallyasymptomaticopacityintheposteriorlens
capsulelocatedapproximately0.5mmmedialtothecenter.Thisisthesite
wherethehyaloidarteryenterstheembryoniclens.Thisharmlesschange
occursinupto2%ofthetotalpopulation.Normallensfiberdevelopmentcan
bedisturbedwherelargeportionsofthehyaloidarterialsystemremain,
althoughthisoccursveryrarely.Thesepatientsdevelopposteriorpolarcata-
racts.
Weiss’ring.
Fig.11.4The
smokeyringap-
pearswhenthe
vitreousbody
detachesfromits
attachmentat
theopticdiskat
thefunnelof
Martegiani
(arrow).
11VitreousBody
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303. 285
11.4.1.2Bergmeister’sPapilla
11.4.1.3PersistentHyaloidArtery
Isolatedpersistenceofthehyaloidarteryisrare.Usuallythisphenomenonis
accompaniedbypersistenceofthehyperplasticprimaryvitreous(seenext
section).Apersistenthyaloidarterywillappearasawhitishcordinthe
hyaloidcanalproceedingfromtheopticdiskandextendingtotheposterior
capsuleofthelens.Isolatedpersistenceofthehyaloidarteryisasymptomatic
anddoesnotrequiretreatment.
11.4.1.4PersistentHyperplasticPrimaryVitreous(PHPV)
Definition
Persistenceoftheembryonicprimaryvitreous(hyaloidarterialsysteminclud-
ingtheposteriortunicavasculosalentis).
Epidemiology:Thisdevelopmentalanomalyisalsoveryrare.
Symptomsandfindings:Usuallythedisorderisunilateral.
AnteriorvariantofPHPV.Withthismorefrequentvariant,awhitepupil
(leukocoriaoramauroticcat’seye)typicallywillbediscoveredshortlyafter
birth.Thisiscausedbythewhitishplateofconnectivetissueposteriortothe
lens.Dependingontheseverity,itwillbeaccompaniedbymoreorlesssevere
changesinthelensleadingtomoreorlessseverelyimpairedvision.In
extremecases,thelensresemblesanopacifiedmembrane(membranouscat-
aract).Inrarecases,fattytissuewilldevelop(lipomatouspseudophakia),and
evenmorerarelycartilagewilldevelopinthelens.Retrolenticularscarring
drawstheciliaryprocessestowardthecenter,andtheywillbevisibleinthe
pupil.Growthoftheeyeisretarded.Thisresultsinmicrophthalmosunless
drainageoftheaqueoushumorisalsoimpaired,inwhichcasebuphthalmos
(hydrophthalmos)willbepresent.
PosteriorvariantofPHPV.Retinaldetachmentandretinaldysplasiacanoccur
whereprimarilyposteriorembryonicstructurespersist.Thewhitishplateof
connectivetissuewillonlybevisiblewhereanteriorchangesassociatedwith
persistenthyperplasticprimaryvitreousarealsopresent.Thereductionin
visualacuitywillvarydependingontheseverityoftheretinalchanges.
Diagnosticconsiderations:Adefinitivediagnosisisusuallypossibleonthe
basisofthecharacteristicclinicalpicture(seesymptomsandfindings)and
additionalultrasoundstudies(whentheposteriorsegmentisobscuredby
lensopacities).
11.4AbnormalChangesintheVitreousBody
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304. 286
Differentialdiagnosis:Othercausesofleukocoria(Table11.1)shouldbe
excluded.Retinoblastoma,themostimportantdifferentialdiagnosis,can
usuallybeexcludedonthebasisofultrasoundorCTstudies.Inthepresence
ofaretinoblastoma,thesestudieswillrevealanintraocularmasswithcalcifi-
cations.IncontrasttoPHPV,microphthalmoswillnotbepresent.
Leukocoriashouldberegardedasaretinoblastomauntilprovenother-
wise.
Treatment:Thedisorderisnotusuallytreatedasneitherconservativether-
apynorsurgerycanimprovevisualacuity.Surgeryisindicatedonlywhere
complicationssuchasprogressivecollapseoftheanteriorchamber,second-
aryincreaseinintraocularpressure,vitreoushemorrhage,andretinaldetach-
mentarepresentorimminent.Theonlygoalistosavetheeyeandmaintain
existingvisualacuity.
Table11.1Differentialdiagnosisofleukocoria
Possiblecauses Differentialcriteria
Congenitalcataract
(4–8:20000)
Earlyinfancy,unilateralorbilateral,normalglobe
size.
Retinoblastoma(1:20000) Infancy,normalglobesize,unilateral(two-thirds)or
bilateral(one-third),calcificationsintumor.
Retinopathyofprematurity,
gradeV(1:20000)
Earlyinfancy,usuallybilateral,nomicrophthalmos,
pretermbirthwithoxygentherapy.
Exudativeretinitis
(Coats’disease)
Childhood,unilateral.
Persistenthyperplasticprimary
vitreous
Usuallyunilateral,usuallymicrophthalmos,con-
natal,centrallydisplacedciliaryprocesses.
Tumors Astrocytoma,medulloepithelioma.
Exudativeretinaldetachment Intoxocariasis,angiomatosisretinae(vonHippel-
Lindautumor),diffusechoroidalhemangioma.
Othercauses Norrie’sdisease,incontinentiapigmenti(Bloch-
Sulzbergerdisease),juvenileretinoschisis,retinal
dysplasia,vitreousabscess,myelinizednervefibers,
colobomaoftheopticdisk(morningglorydisk),
foreignbodiesinthevitreouschamber.
11VitreousBody
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305. 287
Clinicalcourseandprognosis:Theclinicalcourseandprognosisdependpri-
marilyontheseverityofthedisorder.However,adequatesurgicalinterven-
tioncanoftensavetheeyeandstabilizevisualacuityevenifataverylowlevel.
11.4.2AbnormalOpacitiesoftheVitreousBody
11.4.2.1AsteroidHyalosis
Theseusuallyunilateralopacitiesofthevitreousbody(75%ofallcases)are
notallthatinfrequent.Theyarethoughttobelinkedtodiabetesmellitusand
hypercholesterolemia.Thedisorderischaracterizedbywhitecalcific
depositsthatareassociatedwiththecollagenfibersofthevitreousbodyand
thereforearenotverymobile.Mostpatientsarenotbotheredbytheseopaci-
ties.However,theexaminer’sviewofthefunduscanbesignificantly
obscuredby“snowflurries”ofwhiteopacities.Interestingly,theseopacities
donotinterferewithfluoresceinangiography.Vitrectomytoremovethe
opacitiesisrarelynecessaryandisperformedonlywhentheopacities
adverselyaffectthepatient,i.e.,whenvisualacuityisdiminished.
11.4.2.2SynchysisScintillans
Theseveryrareopacitiesofthevitreousbodyusuallyoccurunilaterallyfol-
lowingrecurrentintraocularinflammationorbleeding.Incontrastto
asteroidhyalosis,theseopacitiesarefreefloatingcholesterolcrystalsinthe
vitreouschamberthatrespondtogravity.Fractilecrystalsaretypical.Surgery
isonlyindicatedinrarecasesinwhichtheopacitiesimpairvisualacuity.
11.4.2.3VitreousAmyloidosis
Thisrareinheritedautosomaldominantdisorderbeginsatabouttheageof
20,progressesfordecades,andfinallyleadstodiminishedvisualacuity.Amy-
loidosiscausescharacteristicamyloiddepositsaroundthecollagenfibersof
thevitreousbodyexceptforthehyaloidcanal,whichremainsunaffected.The
amyloidexhibitshistologicallytypicalstaining.Thedisordercanbetreated
byvitrectomy.
11.4.3VitreousHemorrhage
Definition
Bleedingintothevitreouschamberoraspacecreatedbyvitreousdetachment.
Epidemiology:Theannualincidenceofthisdisorderissevencasesper
100000.
11.4AbnormalChangesintheVitreousBody
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306. 288
Etiology:Avitreoushemorrhagemayinvolveoneofthreepossiblepatho-
geneticmechanisms(Fig.11.5):
!1.Bleedingfromnormalretinalvesselsascanoccurasaresultofmechani-
calvasculardamageinacutevitreousdetachmentorretinaltear.
!2.Bleedingfromretinalvesselswithabnormalchangesascanoccurasa
resultofretinalneovascularizationinischemicretinopathyorretinalmac-
roaneurysms.
!3.Influxofbloodfromtheretinaorothersourcessuchasthesubretinal
spaceortheanteriorsegmentsoftheeye.
Morefrequentcausesofvitreoushemorrhageinclude:
!Posteriorvitreousdetachmentwithorwithoutretinaltears(38%).
!Proliferativediabeticretinopathy(32%).
!Branchretinalveinocclusion(11%).
!Age-relatedmaculardegeneration(2%).
!Retinalmacroaneurysm(2%).
Lessfrequentcausesofvitreoushemorrhageinclude:
!Arteriosclerosis.
!Retinalperiphlebitis.
Pathogeneticmechanismsofvitreoushemorrhage.
Choroid
Influx of blood from
adjacent structures
(here: bleeding
in the anterior
segment)
Retina
Bleeding
from normal
retinal vessels
(here: retinal
tear)
Breakthrough
of retinal or
subretinal bleeding
Bleeding from
abnormally
changed
retinal
vessels
(here:
neovas-
culariza-
tion)
Fig.11.5
11VitreousBody
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307. 289
!Terson’ssyndrome(subarachnoidhemorrhage,increaseinintraocular
pressure,acutelyimpaireddrainageofbloodfromtheeye,dilationand
ruptureofretinalvessels,retinalandvitreoushemorrhage).
!Penetratingtrauma.
!Retinalvasculartumors.
Symptoms:Patientsoftenreportthesuddenoccurrenceofblackopacities
thattheymaydescribeas“swarmsofblackbugs”or“blackrain.”Thesearedis-
tinctfromthebrighterandlessdensefloatersseeninsynchysisandvitreous
detachment.Severevitreoushemorrhagecansignificantlyreducevisualacu-
ity.Approximately10µlofbloodaresufficienttoreducevisualacuitytoper-
ceptionofhandmovementsinfrontoftheeye.
Diagnosticconsiderations:Hemorrhagesintothevitreousbodyitselfdonot
exhibitanycharacteristiclimitationsbutspreaddiffusely(thebloodcannot
formafluidmeniscusinthegelatinousvitreousbody)andcoagulationoccurs
quickly(Fig.11.6).Vitreoushemorrhagesrequireexaminationwithanoph-
thalmoscopeorcontactlens.Thecontactlensalsopermitsexaminationofthe
retinaatahigherresolutionsothattheexaminerisbetterabletodiagnose
smallretinaltearsthanwithanophthalmoscope.Ultrasoundstudiesareindi-
catedwhereseverebleedingsignificantlyobscuresthefundusexamination.
Bleedinginthetissuesadjacenttothevitreousbody,i.e.,intheretrohyaloid
space,Berger’sspace,orPetit’sspace(Fig.11.2),canproduceacharacteristic
fluidmeniscus.Thismeniscuswillbevisibleunderslit-lampexamination
(Fig.11.6b).
Treatment:Patientswithacutevitreoushemorrhageshouldbeplacedinan
uprightrestingposition.Thishastwobeneficialeffects:
!1.Thebleedingusuallydoesnotcontinuetospreadintothevitreousbody.
!2.Thebloodintheretrohyaloidspacewillsettlemorequickly.
Nextthecauseofthevitreoushemorrhageshouldbetreated,forexamplearet-
inaltearmaybetreatedwithalaser.Vitrectomywillberequiredtodrainany
vitreoushemorrhagethatisnotabsorbed.
Clinicalcourseandprognosis:Absorptionofavitreoushemorrhageisalong
process.Theclinicalcoursewilldependonthelocation,cause,andseverityof
thebleeding.Bleedinginthevitreousbodyitselfisabsorbedparticularly
slowly.
11.4AbnormalChangesintheVitreousBody
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308. 290
Formsofvitreoushemorrhage.
Figs.11.6aandb
aDiffusevitreous
hemorrhage.The
viewofthefundus
isobscuredbythe
vitreoushemor-
rhage;detailsare
cloudedorcom-
pletelyobscured.
Thestarindicates
thecenterofthe
vitreoushemor-
rhage;thearrow
indicatestheoptic
disk.
bRetrohyaloid
bleedingwithfor-
mationofafluid
meniscus.The
imageshows
bleedingintoa
spacecreatedby
acircularvitreous
detachment.
Gravityhascaus-
edtheerythro-
cytestosinkand
formahorizontal
surface.
11.4.4VitritisandEndophthalmitis
Definition
Thisreferstoacuteorchronicintraocularinflammationduetomicrobialor
immunologiccauses.Inthestrictsense,anyintraocularinflammationis
endophthalmitis.However,inclinicalusageandthroughoutthisbook,
endophthalmitisrefersonlytoinflammationcausedbyamicrobialactionthat
alsoinvolvesthevitreousbody(vitritis).Ontheotherhand,isolatedvitritis
withoutinvolvementoftheotherintraocularstructuresisinconceivabledueto
theavascularityofthevitreouschamber.
11VitreousBody
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309. 291
Epidemiology:Microbialvitritisorendophthalmitisoccursmostfrequently
asaresultofpenetratingtraumatotheglobe.Rarely(in0.5%ofallcases)itisa
complicationofincisiveintraocularsurgery.
Etiology:Becausethevitreousbodyconsistsofonlyafewcellularelements
(hyalocytes),inflammationofthevitreousbodyisonlypossiblewhenthe
inflammatorycellscangainaccesstothevitreouschamberfromtheuveal
tractorretinalbloodvessels.Thismayoccurviaoneofthefollowingmecha-
nisms:
!Microbialpathogens,i.e.,bacteria,fungi,orviruses,enterthevitreous
chambereitherthroughdirectcontamination(forexampleviapenetrat-
ingtraumaorincisiveintraocularsurgery)ormetastaticallyasaresultof
sepsis.Thevirulenceofthepathogensandthepatient’sindividual
immunestatusdeterminewhetheranacute,subacute,orchronicinflam-
mationwilldevelop.Bacterialinflammationisfarmorefrequentthanviral
orfungalinflammation.However,themetastaticformofendophthalmitis
isobservedinimmunocompromisedpatients.Usuallytheinflammationis
fungal(mycoticendophthalmitis),andmostoftenitiscausedbyoneofthe
Candidaspecies.
!Inflammatory(microbialorautoimmune)processes,instructuresadja-
centtothevitreousbody,suchasuveitisorretinitiscanprecipitateasec-
ondaryreactioninthevitreouschamber.
Acuteendophthalmitisisaseriousclinicalsyndromethatcanresultin
lossoftheeyewithinafewhours.
Symptoms:Acutevitreousinflammationorendophthalmitis.Characteris-
ticsymptomsincludeacutelossofvisualacuityaccompaniedbydeepdull
ocularpainthatrespondsonlyminimallytoanalgesicagents.Severeredden-
ingoftheconjunctivaispresent.Incontrasttobacterialorviralendophthal-
mitis,mycoticendophthalmitisbeginsasasubacutedisordercharacterized
byslowlyworseningchronicvisualimpairment.Daysorweekslater,thiswill
alsobeaccompaniedbyseverepain.
Chronicvitreousinflammationorendophthalmitis.Theclinicalcourseisfar
lesssevere,andthelossofvisualacuityisoftenmoderate.
Diagnosticconsiderations:Thepatient’shistoryandthepresenceoftypical
symptomsprovideimportantinformation.
Acutevitreousinflammationorendophthalmitis.Slit-lampexamination
willrevealmassiveconjunctivalandciliaryinjectionaccompaniedby
hypopyon(collectionofpusintheanteriorchamber).Ophthalmoscopywill
revealyellowish-greendiscolorationofthevitreousbodyoccasionally
referredtoasavitreousbodyabscess.Iftheviewisobscured,ultrasoundstud-
iescanhelptoevaluatetheextentoftheinvolvementofthevitreousbodyin
endophthalmitis.Roth’sspots(whiteretinalspotssurroundedbyhemor-
11.4AbnormalChangesintheVitreousBody
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310. 292
rhage)andcircumscribedretinochoroiditiswithavitreousinfiltratewillbe
observedintheinitialstages(duringthefirstfewdays)ofmycoticendoph-
thalmitis.Inadvancedstages,thevitreousinfiltratehasacreamywhitish
appearance,andretinaldetachmentcanoccur.
Chronicvitreousinflammationorendophthalmitis.Inspectionwillusually
revealonlymoderateconjunctivalandciliaryinjection.Slit-lampexamina-
tionwillrevealinfiltrationofthevitreousbodybyinflammatorycells.
Aconjunctivalsmear,asampleofvitreousaspirate,and(wheresepsisis
suspected)bloodculturesshouldbeobtainedformicrobiologicalexamina-
tiontoidentifythepathogen.Negativemicrobialresultsdonotexclude
possiblemicrobialinflammation;theclinicalfindingsaredecisive.SeeChap-
ter12fordiagnosisofretinitisanduveitis.
Differentialdiagnosis:Thediagnosisismadebyclinicalexaminationin
mostpatients.Intraocularlymphomashouldbeexcludedinchronicformsof
thedisorderthatfailtorespondtoantibiotictherapy.
Treatment:Microbialinflammationsrequirepathogen-specificsystemic,
topical,andintravitrealtherapy,wherepossibleaccordingtothestrain’s
documentedresistancetoantibiotics.Mycoticendophthalmitisisusually
treatedwithamphotericinBandsteroids.Immediatevitrectomyisather-
apeuticoptionwhoseindicationshaveyettobeclearlydefined.
Secondaryvitreousreactionsinthepresenceofunderlyingretinitisor
uveitisshouldbeaddressedbytreatingtheunderlyingdisorder.
Prophylaxis:Intraocularsurgeryrequiresextremecaretoavoidintraocular
contaminationwithpathogens.Immunocompromisedpatients(suchasAIDS
patientsorsubstanceabusers)andpatientswithindwellingcathetersshould
undergoregularexaminationbyanophthalmologist.
Decreasedvisualacuityandeyepaininsubstanceabusersandpatients
withindwellingcatheterssuggestCandidaendophthalmitis.
Clinicalcourseandprognosis:Theprognosisforacutemicrobialendoph-
thalmitisdependsonthevirulenceofthepathogenandhowquicklyeffective
antimicrobialtherapycanbeinitiated.Extremelyvirulentpathogenssuchas
Pseudomonasanddelayedinitiationoftreatment(notwithinafewhours)
worsentheprognosisforvisualacuity.Withpostoperativeinflammationand
poorinitialvisualacuity,animmediatevitrectomycanimprovetheclinical
courseofthedisorder.Theprognosisisusuallyfarbetterforchronicforms
andsecondaryvitritisinuveitis/vitritis.
11VitreousBody
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311. 293
11.4.5VitreoretinalDystrophies
11.4.5.1JuvenileRetinoschisis
JuvenileretinoschisisisaninheritedX-linkedrecessivedisorderthataffects
onlymales.Aretinalschisisatthemaculasometimesreferredtoclinicallyasa
“spokephenomenon”usuallydevelopsbetweentheagesof20and30.Thisis
associatedwithasignificantlossofvisualacuity.Aperipheralretinalschisis
isalsopresentinabouthalfofthesecases.Thissplittingoftheretinaispre-
sumablyduetotractionofthevitreousbody.Thissplittingoccursinthenerve
fiberlayerincontrasttotypicalsenileretinoschisis,inwhichsplittingoccurs
intheouterplexiformlayer.
11.4.5.2Wagner’sDisease
Thisdisorderisalsoinherited(autosomaldominant)andinvolvescentral
liquefactionofthevitreousbody.This“visualvoid”inthevitreouschamber
andfibrillarycondensationofthevitreousstromaassociatedwithacataract
characterizevitreoretinaldegenerationinWagner’sdisease.
11.5 TheRoleoftheVitreousBodyinVariousOcular
ChangesandFollowingCataractSurgery
11.5.1RetinalDetachment
Thecloseconnectionbetweenthevitreousbodyandretinacanresultinreti-
naltearsinvitreousdetachment,whichinturncanleadtorhegmatogenous
retinaldetachment(fromtheGreekword“rhegma,”breakage.
Theseretinaldefectsprovideanopeningforcellsfromtheretinalpigment
epitheliumtoenterthevitreouschamber.Thesepigmentcellsmigratealong
thesurfaceoftheretina.Astheydoso,theyactsimilarlytomyofibroblastsand
leadtotheformationofsubretinalandepiretinalmembranesandcausecon-
tractionofthesurfaceoftheretina.Thisclinicalpictureisreferredtoaspro-
liferativevitreoretinopathy(PVR).Therigidretinalfoldsandvitreousmem-
branesinproliferativevitreoretinopathysignificantlycomplicatereattach-
mentoftheretina.Usuallythisrequiresmoderntechniquesofvitreoussurgery.
11.5.2RetinalVascularProliferation
Retinalvascularproliferationcanoccurinretinalischemiaindisorderssuch
asdiabeticretinopathy,retinopathyinpreterminfants,centralorbranchreti-
nalveinocclusion,andsickle-cellretinopathy.Growthofthisretinalneovascu-
larizationintothevitreouschamberusuallyoccursonlywherevitreous
detachmentisabsentorpartialbecausetheseproliferationsrequireasub-
stratetogrowon.Preretinalproliferationsoftenleadtovitreoushemorrhage.
11.5TheRoleoftheVitreousBodyinVariousOcularChanges
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312. 294
Fibroticchangesproducetractionoftheretinaresultinginatractionalretinal
detachment.
11.5.3CataractSurgery
Increasedpostoperativeinflammationintheanteriorsegmentcanprogress
throughthehyaloidcanaltotheposteriorpoleoftheeyeandacystoidmacu-
laredemacandevelop.Thiscomplicationoccursparticularlyfrequentlyfol-
lowingcataractsurgeryinwhichtheposteriorlenscapsulewasopenedwith
partiallossofvitreousbody.(Hruby-Irvine-Gasssyndromeisthedevelop-
mentofcystoidmacularedemafollowingintracapsularcataractextraction
withincarcerationofthevitreousbodyinthewound).
11.6 SurgicalTreatment:Vitrectomy
Definition
SurgicalremovalandreplacementofthevitreousbodywithRinger’ssolution,
gas,orsiliconeoil.
Indication:Theprimaryindicationsinclude:
!Unabsorbedvitreoushemorrhage.
!Tractionalretinaldetachment.
!Proliferativevitreoretinopathy.
!Removalofintravitrealdisplacedlensesorforeignbodies.
!Severepostoperativeorpost-traumaticinflammatoryvitreouschanges.
Procedure:Thevitreousbodycannotsimplybeaspiratedfromtheeyeasthe
vitreoretinalattachmentswouldalsocauseretinaldetachment.Thepro-
cedurerequiressuccessive,piecemealcuttingandaspirationwithavitrectome
(aspecializedcuttingandaspiratinginstrument).Cuttingandaspirationof
thevitreousbodyisperformedwiththeaidofsimultaneousinfusiontopre-
venttheglobefromcollapsing.Thesurgicalsiteisilluminatedbyafiberoptic
lightsource.Thethreeinstruments(infusioncannula,lightsource,andvit-
rectome),all1mmindiameter,areintroducedintotheglobethroughthe
parsplana,whichiswhytheprocedureisreferredtoasaparsplanavit-
rectomy(PPV).Thissiteentailstheleastriskofiatrogenicretinaldetachment
(Fig.11.7).Thesurgeonholdsthevitrectomeinonehandandthelightsource
intheother.Theprocedureisperformedunderanoperatingmicroscopewith
specialcontactlensesplacedonthecornealsurface.Oncethevitreousbody
andanyvitreousmembraneshavebeenremoved(Fig.11.7),theretinacanbe
treatedintraoperativelywithalaser(forexample,totreatproliferativedia-
beticretinopathyorrepairaretinaltear).Inmanycases,suchaswithan
unabsorbedvitreoushemorrhage,itissufficienttofilltheeyewithRinger’s
solutionfollowingvitrectomy.
11VitreousBody
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313. 295
Parsplanavitrectomy.
Retina
Cerclage
(encircling
band)
Light source
Vitrectome
Infusion cannula
Fig.11.7Theillustrationdepictstheinfusioncannula,lightsource,andvitrectome
(cuttingandaspiratinginstrument).Acerclageisusuallyplacedaroundtheequator
toreleaseresidualtractionandpreventretinaldetachment.Itisleftinplaceafter
surgery.
FillingtheeyewithRinger’ssolutionisnotsufficienttotreatacompli-
catedretinaldetachmentwithepiretinalorsubretinalmembranesandcon-
tractionofthesurfaceoftheretina(seeproliferativevitreoretinopathy).In
thesecases,thedetachedretinamustbeflattenedfromanteriortoposterior
andheldwithatamponadeoffluidwithaveryhighspecificgravitysuchasa
perfluorocarbonliquid(Fig.11.8a).These“heavy”liquidscanalsobeusedto
floatartificallensesthathavebecomedisplacedinthevitreousbody.The
artificiallenseshavealowerspecificgravitythantheseliquidsandwillfloat
onthem(Fig.11.8b).Attheendoftheoperation,theseheavyliquidsmustbe
replacedwithgases,suchasamixtureofairandsulfurhexafluoride,thatare
spontaneouslyabsorbedwithinafewdaysorwithsiliconeoil(whichmustbe
removedinasecondoperation).Postoperativepatientpositioningshould
reflectthefactthatmaximumgaspressurewillbeinthesuperiorregion
(Fig.11.9a)duetoitsbuoyancy.Complicatedretinaldetachmentswillrequire
aprolongedinternaltamponade.Siliconeoilhasproveneffectiveforthispur-
11.6SurgicalTreatment:Vitrectomy
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314. 296
Perfluorocarbon
liquid
11VitreousBody
Useof“heavy”liquidsinvitreoretinalsurgery.
Retina
Cerclage
(encircling
band)
Perfluorocarbon
liquid
Retinotomy
Removal of
epiretinal
membranes
Fig.11.8aRepair-
ingtheretinaina
complicatedretinal
detachmentusing
aliquidwithahigh
specificgravity.
Thehighspecific
gravityoftheliquid
flattensoutthe
retina.Theliquid
actsasa“third
hand”when
manipulatingthe
retina,simplifying
maneuverssuchas
removalofepireti-
nalmembranes
andretinotomies.
bFloatingadis-
placedintraocular
lens.
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315. 297
Useofgasandsiliconeoilinvitreoretinalsurgery.
Fig.11.9aAn
intraoculargas
bubbleexerts
pressurepri-
marilyinthesu-
periorarea(blue
arrows)dueto
itsbuoyancy.
Thismustbe
considered
whenposition-
ingthepatient
postoperatively;
thepatient
shouldbeposi-
tionedsothat
theforamenlies
inthisregion.
bCompletely
fillingtheglobe
withsiliconeoil
fixestheretina
toitsunderlying
tissueatpracti-
callyeveryloca-
tion(arrows).
Gas bubble
Silicone oil
11.6SurgicalTreatment:Vitrectomy
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316. 298
poseasitcompletelyfillsthevitreouschamberandexertspermanentpres-
sureontheentireretina(Fig.11.9b).However,siliconeoilinevitablycauses
cataractformationandoccasionallycornealchangesandglaucoma.There-
fore,itmustberemovedinasecondoperation.
Complications:Vitrectomynearlyalwaysleadstosubsequentlensopacifica-
tion,andrarelytoretinaltears,bleeding,orendophthalmitis.
11VitreousBody
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317. 299
12Retina
GabrieleK.LangandGerhardK.Lang
12.1BasicKnowledge
Theretinaistheinnermostofthreesuccessivelayersoftheglobe.Itcomprises
twoparts:
!Aphotoreceptivepart(parsopticaretinae),comprisingthefirstnineof
the10layerslistedbelow.
!Anonreceptivepart(parscaecaretinae)formingtheepitheliumofthecil-
iarybodyandiris.
Theparsopticaretinaemergeswiththeparscecaretinaeattheoraserrata.
Embryology:Theretinadevelopsfromadiverticulumoftheforebrain(proen-
cephalon).Opticvesiclesdevelopwhichtheninvaginatetoformadouble-
walledbowl,theopticcup.Theouterwallbecomesthepigmentepithelium,
andtheinnerwalllaterdifferentiatesintotheninelayersoftheretina.The
retinaremainslinkedtotheforebrainthroughoutlifethroughastructure
knownastheretinohypothalamictract.
Thicknessoftheretina(Fig.12.1)
Layersoftheretina:Movinginwardalongthepathofincidentlight,the
individuallayersoftheretinaareasfollows(Fig.12.2):
1.Innerlimitingmembrane(glialcellfibersseparatingtheretinafromthe
vitreousbody).
2.Layerofopticnervefibers(axonsofthethirdneuron).
3.Layerofganglioncells(cellnucleiofthemultipolarganglioncellsofthe
thirdneuron;“dataacquisitionsystem”).
4.Innerplexiformlayer(synapsesbetweentheaxonsofthesecondneuron
anddendritesofthethirdneuron).
5.Innernuclearlayer(cellnucleiofthebipolarnervecellsofthesecond
neuron,horizontalcells,andamacrinecells).
6.Outerplexiformlayer(synapsesbetweentheaxonsofthefirstneuron
anddendritesofthesecondneuron).
7.Outernuclearlayer(cellnucleioftherodsandcones=firstneuron).
8.Outerlimitingmembrane(sieve-likeplateofprocessesofglialcells
throughwhichrodsandconesproject).
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318. 300
Thicknessoftheretina.
Close to the ora
serrata: 0.12 mm
At the equator: 0.18 mm
Around the fovea:
0.23 mm
Fovea centralis:
0.10 mm
At the optical disk:
0.56 mm
Fig.12.1Retinaltearsmostoftenoccurclosetotheoraserrata.
9.Layerofrodsandcones(theactualphotoreceptors).
10.Retinalpigmentepithelium(asinglecubiclayerofheavilypigmented
epithelialcells).
11.Bruch’smembrane(basalmembraneofthechoroidseparatingtheretina
fromthechoroid).
Maculalutea:Themaculaluteaisaflattenedovalareainthecenterofthe
retinaapproximately3–4mm(15degrees)temporaltoandslightlybelowthe
opticdisk.Itsdiameterisroughlyequaltothatoftheopticdisk(1.7–2mm).
Themaculaappearsyellowwhenexaminedundergreenlight,hencethe
namemaculalutea(yellowspot).Locatedinitscenteristheavascularfovea
!Fig.12.2aLayersoftheretinaandexaminationmethodsusedtodiagnoseabnormal
processesintherespectivelayers(EOG=electro-oculogram;ERG=electroretinogram;
VEP=visualevokedpotential).bHistologicimageofthe10layersoftheretina.
12Retina
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319. 301
Histologyandfunctionofthelayersoftheretina.
Fig.12.2
Light
VEP
Optic nerve
Pattern
ERG
ERG
EOG
1. Inner limiting membrane
2. Layer of optic nerve fibers
3. Layer of ganglion cells
4. Inner plexiform layer
5. Inner nuclear layer
6. Outer plexiform layer
7. Outer nuclear layer
8. Outer limiting membrane
9. Layer of rods and cones
10. Retinal pigment
epithelium
11. Bruch's membrane
Amacrine cells
Bipolar cells
Horizontal cells
Photoreceptors
Supporting cells of Müller
1
2
3
4
5
6
7
8
9
10
12.1BasicKnowledge
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320. 302
centralis,thepointatwhichvisualperceptionissharpest.Thefoveacentralis
containsonlycones(norods)eachwithitsownneuralsupply,whichexplains
whythisregionhassuchdistinctvision.Lightstimuliinthisregioncan
directlyactonthesensorycells(firstneuron)becausethebipolarcells(sec-
ondneuron)andganglioncells(thirdneuron)aredisplacedperipherally.
Vascularsupplytotheretina:Theinnerlayersoftheretina(theinnerlim-
itingmembranethroughtheinnernuclearlayer)aresuppliedbythecentral
arteryoftheretina.Thisoriginatesattheophthalmicartery,enterstheeye
withtheopticnerve,andbranchesontheinnersurfaceoftheretina.Thecen-
tralarteryisagenuinearterywithadiameterof0.1mm.Itisaterminalartery
withoutanastomosesanddividesintofourmainbranches(seeFig.12.8).
Becausethecentralarteryisaterminalartery,occlusionwillleadtoreti-
nalinfarction.
Theouterlayers(outerplexiformlayerthroughthepigmentepithelium)con-
tainnocapillaries.Theyarenourishedbydiffusionprimarilyfromtherichly
suppliedcapillarylayerofthechoroid.Theretinalarteriesarenormally
brightred,havebrightredreflexstrips(seeFig.12.8)thatbecomepalerwith
advancingage,anddonotshowapulse.Theretinalveinsaredarkredwitha
narrowreflexstrip,andmayshowspontaneouspulsationontheopticdisk.
Pulsationintheretinalveinsisnormal;pulsationintheretinalarteriesis
abnormal.
Thewallsofthevesselsaretransparentsothatonlythebloodwillbevisible
onophthalmoscopy.Intermsoftheirstructureandsize,theretinalvessels
arearteriolesandvenules,althoughtheyarereferredtoasarteriesandveins.
Venousdiameterisnormally1.5timesgreaterthanarterialdiameter.Capil-
lariesarenotvisible.
Nervesupplytotheretina:Theneurosensoryretinahasnosensorysupply.
Disordersoftheretinaarepainlessbecauseoftheabsenceofsensory
supply.
Lightpaththroughtheretinallayers:Whenelectromagneticradiationin
thevisiblelightspectrum(wavelengthsof380–760nm)strikestheretina,it
isabsorbedbythephotopigmentsoftheouterlayer.Electricsignalsare
createdinamulti-stepphotochemicalreaction.Theyreachthephotoreceptor
synapsesasactionpotentialswheretheyarerelayedtothesecondneuron.
Thesignalsarerelayedtothethirdandfourthneuronsandfinallyreachthe
visualcortex.
Lightmustpassthroughthreelayersofcellnucleibeforeitreachesthe
photosensitiverodsandcones.Thisinvertedpositionofthephotore-
ceptorsisduetothemannerinwhichtheretinadevelopsfromadiver-
ticulumoftheforebrain.
12Retina
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321. 303
Sensitivityoftheretinatolightintensity:Theretinahastwotypesofpho-
toreceptors,therodsandthecones.The110–125millionrodspermitmesopic
andscotopicvision(twilightandnightvision).Theyareabout500timesmore
photosensitivethantheconesandcontainthephotopigmentrhodopsin.
Twilightvisiondecreasesaftertheageof50,particularlyinpatients
withadditionalage-relatedmiosis,cataract,anddecreasedvisualacu-
ity.Therefore,glaucomapatientsundergoingtreatmentwithmiotic
agentsshouldbeadvisedofthedangerofoperatingmotorvehiclesin
twilightoratnight.
Thesixtosevenmillionconesinthemaculaareresponsibleforphotopic
vision(daytimevision),resolution,andcolorperception.Therearethree
typesofcones:
!bluecones,
!greencones,
!redcones.
Theirphotopigmentscontainthesameretinalbutdifferentopsins.Beyonda
certainvisualfieldluminance,atransitionfromdarkadaptationtolight
adaptationoccurs.Luminancereferstotheluminousfluxperunitsolidangle
perunitprojectedarea,measuredincandelaspersquaremeter(cd/m
2
).The
conesareresponsibleforvisionuptoaluminanceof10cd/m
2
,therodsupto
0.01cd/m
2
(twilightvisionis0.01–10cd/m
2
;nightvisionislessthan0.01
cd/m
2
).
Adaptationistheadjustmentofthesensitivityoftheretinatovarying
degreesoflightintensity.Thisisdonebydilationorcontractionofthepupil
andshiftingbetweenconeandrodvision.Inthismanner,thehumaneyeis
abletoseeindaylightandatnight.Inlightadaptation,therhodopsinis
bleachedoutsothatrodvisionisimpairedinfavorofconevision.Lightadap-
tationoccursfarmorequicklythandarkadaptation.Indarkadaptation,the
rhodopsinquicklyregenerateswithinfiveminutes(immediateadaptation),
andwithin30minutestoanhourthereisafurtherimprovementinnight
vision(long-termadaptation).Anadaptometermaybeusedtodeterminethe
lightintensitythreshold.Firstthepatientisadaptedtobrightlightfor10
minutes.Thentheexaminingroomisdarkenedandthelightintensity
thresholdismeasuredwithlighttestmarkers.Thesemeasurementscanbe
usedtoobtainanadaptationcurve(Fig.12.3).
Sensitivitytoglare:Glarereferstodisturbingbrightnesswithinthevisualfield
sufficientlygreaterthantheluminancetowhichtheeyesareadaptedsuchas
theheadlightsofoncomingtrafficorintensereflectedsunlight.Becausethe
retinaisadaptedtoalesserluminance,visionisimpairedinthesecases.Often
theglarewillcauseblinkingorelicitaneyeclosingreflex.Sensitivitytoglare
canbemeasuredwithaspecialdevice.Patientsareshownaseriesofvisual
symbolsinrapidsuccessionthattheymustrecognizedespiteintenseglare.
12.1BasicKnowledge
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322. 304
Normalandabnormaldarkadaptationcurves.
3,2
.
10
–3
3,2
.
10
–5
3,2
3,2
.
10
–7
0 10 20 30 40 50min
cd/m
2
Fig.12.3Xaxis:adaptationtimeinminutes.Yaxis:luminanceoftherespective
testmarkerincandelaspersquaremeter.Thebluecurveshowsnormalprogression
withKohlrausch’stypicaldiscontinuityindicatingthetransitionfromconetorodvi-
sion.Theredcurveinretinitispigmentosaisconsiderablylesssteep.
Thesensitivitytoglareorthespeedofadaptationandreadaptationoftheeye
isimportantindeterminingwhetherthepatientisfittooperateamotor
vehicle.
12.2ExaminationMethods
VisualAcuityseeChapter1.
12.2.1ExaminationoftheFundus
Directophthalmoscopy(Fig.12.4a;seealsoFig.1.13):Adirectophthalmo-
scopeispositionedclosetothepatient’seye.Theexaminerseesa16-power
magnifiedimageofthefundus.
Advantages.Thehighmagnificationpermitsevaluationofsmallretinalfind-
ingssuchasdiagnosingretinalmicroaneurysms.Thedialoftheophthalmo-
scopecontainsvariousdifferentplusandminuslensesandcanbeadjustedas
necessary.Theselensescompensateforrefractiveerrorsinboththepatient
!Fig.12.4
aDirectophthalmoscopy:theexaminerseesanerectfundusimageofthepatient.bIn-
directophthalmoscopy:theexaminerseesavirtualinvertedfundusimage.cPositionof
examinerandpatientforindirectophthalmoscopy.
12Retina
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323. 305
Ophthalmoscopy.
Light source
Examiner Patient
Light source
Examiner PatientLoupe
a
b
Fig.12.4c
12.2ExaminationMethods
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324. 306
andtheexaminer.Theymayalsobeusedtomeasuretheprominenceofretinal
changes,suchastheprominenceoftheopticdiskinpapilledemaortheprom-
inenceofatumor.Thebaseofthelesionisbroughtintofocusfirstandthen
thepeakofthelesion.Adifferenceof3dioptersfrombasetopeakcorre-
spondstoaprominenceof1mm.Directophthalmoscopyproducesanerect
imageofthefundus,whichissignificantlyeasiertoworkwiththanan
invertedimage,andisthereforeasuitabletechniqueevenforless
experiencedexaminers.
Disadvantages.Theimageofthefundusishighlymagnifiedbutshowsonlya
smallportionofthefundus.Rotatingtheophthalmoscopecanonlypartially
compensateforthisdisadvantage.Directophthalmoscopyalsoproducesonly
atwo-dimensionalimage.
Indirectophthalmoscopy(Figs.12.4bandc):Acondensinglens(+14to+30
diopters)isheldapproximately13cmfromthepatient’seye.Thefundus
appearsintwotosix-powermagnification;theexaminerseesavirtual
invertedimageofthefundusatthefocalpointoftheloupe.Lightsourcesare
availableformonocularorbinocularexamination.
Advantages.Thistechniqueprovidesagoodstereoscopic,optimallyillumi-
natedoverviewoftheentirefundusinbinocularsystems.
Disadvantages.Magnificationissignificantlylessthanindirectophthalmos-
copy.Indirectophthalmoscopyrequirespracticeandexperience.
Contactlensexamination:Thefundusmayalsobeexaminedwithaslit
lampwhenanadditionalmagnifyinglenssuchasathree-mirrorlens(see
Fig.12.5)ora78to90diopterlensisused.
ExaminationofthefunduswithaGoldmannthree-mirrorlens.
Slit-lamp light
Examiner Patient
a b
Retinal
tear
1
23
4
Three-mirror
lens
4
3
2
1
Figs.12.5aandbPrincipleoftheexamination:Thelensisplaceddirectlyonthe
eyeafterapplicationofatopicalanesthetic.ThevariousmirrorsofGoldmannthree-
mirrorlensvisualizedifferentareasoftheretina:1)posteriorpole,2)centralpartof
theperipheralretina,3)outerperipheralretina(importantindiagnosingretinal
tears),4)gonioscopymirrorforexaminationofthechamberangle.
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325. 307
Advantages.Thistechniqueproducesahighlymagnifiedthree-dimensional
imageyetstillprovidestheexaminerwithagoodoverviewoftheentirefun-
dus.Thethree-mirrorlensalsovisualizes“blindareas”oftheeyesuchasthe
angleoftheanteriorchamber.Contactlensexaminationcombinestheadvan-
tagesofdirectophthalmoscopyandindirectophthalmoscopyandisthere-
forethegoldstandardfordiagnosingretinaldisorders.
Wheresignificantopacificationoftheopticmedia(asinamaturecata-
ract)preventsdirectvisualizationoftheretinawiththetechniques
mentionedabove,theexaminercanevaluatethepatternoftheretinal
vasculature.Thescleraisdirectlyilluminatedinallfourquadrantsby
movingalightsourcebackandforthdirectlyoverthesclera.Patients
withintactretinaswillbeabletoperceivetheshadowoftheirown
vasculatureontheretina(entopticphenomenon).Theywillseewhat
lookslike“veinsofaleafinautumn”.Patientswhoareabletoperceive
thisphenomenonhavepotentialretinalvisionofatleast20/200.
Ultrasonography:Ultrasoundstudiesareindicatedwhereopacificationof
theopticmediasuchascataractorvitreoushemorrhagepreventdirect
inspectionofthefundusorwhereretinalandchoroidalfindingsareinconclu-
sive.Intraoculartissuesvaryinhowtheyreflectultrasonicwaves.Theretina
ishighlyreflective,whereasthevitreousbodyisnormallynearlyanechoic.
Ultrasoundstudiescanthereforedemonstrateretinaldetachmentanddistin-
guishitfromachangeinthevitreousbody.Opticdiskdrusenarealsohighly
reflective.Ultrasoundisalsohelpfulindiagnosingintraoculartumorswitha
prominenceofatleast1.5mm.Thespecificechogenicityofthetissuealso
helpstoevaluatewhetheratumorismalignant,forexampleindistinguishing
achoroidalnevusfromamalignantmelanoma(Fig.12.6).
Ultrasoundstudiescandemonstrateretinaldetachmentwherethe
opticmediaoftheeyeareopacified(duetocausessuchascataractor
vitreoushemorrhage).Thisisbecausetheretinaishighlyreflectivein
contrasttothevitreousbody.Ultrasoundcanalsobeusedtoconfirm
thepresenceofmalignantchoroidalprocesses.
Fundusphotography:Abnormalchangescanberecordedwithasingle-lens
reflexcamera.Thispermitsprecisedocumentationoffollow-upfindings.
Photographsobtainedwithafunduscameraingreenlightprovidehigh-con-
trastimagesofabnormalchangestotheinnermostlayersoftheretinasuchas
changesinthelayerofopticnervefibers,bleeding,ormicroaneurysms.
Fluorescenceangiography(withfluoresceinorindocyaninegreen):In
fluoresceinangiography,10mlof5%fluoresceinsodiumareinjectedintoone
ofthepatient’scubitalveins.Blueandyellow-greenfiltersarethenplaced
alongtheopticalaxisofasingle-lensreflexcamera.Thebluefilterensures
thatonlybluelightfromthelightsourcereachestheretina.Theyellow-green
12.2ExaminationMethods
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326. 308
Ultrasoundexaminationofthefundus.
Fig.12.6Ultra-
soundfindingsin
malignant
melanoma
(arrow).
filterblocksthebluecomponentsofthereflectedlightsothatthecamerarec-
ordsonlytheimageofthefluorescentdye(Fig.12.7).
Fluoresceinangiographyisusedtodiagnosevascularretinaldisorders
suchasproliferativediabeticretinopathy,venousocclusion,age-
relatedmaculardegeneration,andinflammatoryretinalprocesses.
Wheretheblood-retinabarrierformedbythezonulaeoccludentesis
disturbed,fluoresceinwillleakfromtheretinalvessels.Disordersofthe
choroidsuchaschoroiditisortumorscanalsobediagnosedbythis
method;inthesecasesindocyanineisbetterthanfluorescein.
12.2.2NormalandAbnormalFundusFindingsinGeneral
Normalfundus:Theretinaisnormallycompletelytransparentwithoutany
intrinsiccolor.Itreceivesitsuniformbrightredcolorationfromthevascula-
tureofthechoroid(Fig.12.8).Thevesselsofthechoroidthemselvesare
obscuredbytheretinalpigmentepithelium.Lossoftransparencyoftheretina
isasignofanabnormalprocess(forexampleinretinaledemas,theretina
appearswhitishyellow).Theopticdiskisnormallyasharplydefined,yel-
lowishorangestructure(inteenagersitispalepink,andinyoungchildrensig-
nificantlypaler)thatmayexhibitacentraldepressionknownastheopticor
physiologiccup.Lightreflectionontheinnerlimitingmembranewillnor-
mallyproducemultiplelightreflexesonthefundus.Teenagerswillalsoexhibit
anormalfovealreflexandwallreflexsurroundingthemacula,whichis
causedbythetransitionfromthedepressionofthemaculartothehigher
leveloftheretina(Fig.12.9).
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327. 309
Fluoresceinangiographyofthefundus.
White
light
Blue
light
Yellow-green
light reaches
the camera
Yellow-green light
is emitted; blue light
is reflected
Light
source
a Blue filter
Yellow-green filterCamera
b
Fig.12.7Blueandyellow-greenfiltersareplacedalongtheopticalaxisofasingle-
lensreflexcamera.aFirstthebluefilterensuresthatonlybluelightfromthelight
sourcereachestheretina.Thisexcitesthepreviouslyinjectedfluoresceindyeinthe
vesselsofthefundus.bTheexcitedfluoresceinemitsyellow-greenlight,andthe
bluelightisreflected.Theyellow-greenfilterblocksthebluecomponentsofthere-
flectedlightsothatthecamerarecordsonlytheimageofthefluorescentdye.
Normalfundus.
Fig.12.8The
maculalutealies
about3–4mm
temporaltoand
slightlybelowthe
opticdisk.The
fundusreceives
itsuniformbright
redcoloration
fromthevessels
ofthechoroid.
Venousdiameter
isnormally1.5
timesgreaterthan
arterialdiameter.
12.2ExaminationMethods
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328. 310
Wallreflexsurroundingthemacula.
Fig.12.9Typical
highlyreflective
fundusina
teenager(see
wallreflex).
Age-relatedchanges:Theopticdiskturnspaleyellowwithage,andoften
theopticcupwillbecomeshallowandwillbesurroundedbyaregionof
choroidalatrophy.Thefunduswillbecomedullandnonreflective.Drusen
willbevisibleintheretinalpigmentepitheliumandmiddleperipheralreticu-
larproliferationsofpigmentepitheliumwillbepresent.Thearterioleswillbe
elongatedduetolossofelasticitywithirregularfillingduetothickeningofthe
vascularwalls.Meanderingofthevenuleswillbepresentwithcrossingsigns,
i.e.,thescleroticarterywillbeseentocompresstheveinatthearteriovenous
crossing,reducingthediameterofthecolumnofvenousblood.Inextreme
casesvenousbloodflowwillbecutoffcompletely.
Abnormalchangesinthefundus:Asarule,lossoftransparencyoftheretina
isasignofanabnormalprocess.Forexampleinaretinaledema,theretina
appearswhitishyellow(seeFig.12.19).Adistinctivefeatureofabnormalreti-
nalandchoroidalchangesisthatthetypeandappearanceofthesechanges
permitprecisetopographiclocalizationoftherespectiveabnormalprocess
whenthediagnosisismade.Theophthalmoscopicimagewillusuallyallow
onetodetermineinwhichofthelayersshowninFig.12.2theprocessis
occurring.Forexample,inFig.12.27(nonexudativeage-relatedmacular
degeneration)onemayseethatthedrusenandatrophyarelocatedintheret-
inalpigmentepithelium;thestructuresaboveitarenotaffected,asis
apparentfromtheintactvascularstructures.
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329. 311
12.2.3ColorVision
Colorvisiondefectsmaybecongenital(especiallyinmenastheyare
inheritedandX-linkedrecessive)oracquired,forexampleinmaculardis-
orderssuchasStargardt’sdisease.Qualitativered-greenvisiondefectsare
evaluatedwithpseudoisochromaticplatessuchastheIshiharaorStilling-
Velhagenplates.Theycontainnumeralsorletterscomposedofsmallcolor
dotssurroundedbyconfusioncolors(Fig.12.10)thatpatientswithcolor
visiondefectscannotread.TheFarnsworth-Munselltests(Fig.12.11)can
detectblue-yellowcolorvisiondefects.
Pseudoisochromaticplatescontainnumeralsthatpatientswithcolor
visiondefectscannotread.IntheFarnsworth-Munselltest,patients
withacolorvisiondefectcannotsortmarkerswithdifferenthues
(accordingtothecolorsoftherainbow)intherightorder.
TheNagelanomaloscopepermitsquantitativeevaluationofcolorvision
defects.Thetestplateconsistsofaloweryellowhalfwhosebrightnesscanbe
adjusted,andanupperhalfthatthepatienttriestomatchtotheloweryellow
colorbymixingredandgreen.Theanomalyratioiscalculatedfromthefinal
adjustment.Green-blindpatientswillusetoomuchgreen,andred-blind
patientstoomuchredwhenmixingthecolors.
Perimetry
Ishiharaplatesfordiagnosingred-greenvisiondefects.
Fig.12.10Patientswithnormalcolorvisionwillrecognizethenumber26onthe
leftand42ontheright.
12.2ExaminationMethods
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330. 312
Farnsworth-Munselltestofred-greenandblue-yellowcolorvisiondefects.
Fig.12.11The
patientmustsort
markersof
varioushuesin
therightorder
accordingtothe
colorsoftherain-
bow.
12.2.4ElectrophysiologicExaminationMethods
(electroretinogram,electro-oculogram,andvisualevoked
potentials;seeFig.12.2a)
Electroretinogram(ERG):Thisexaminationmethoduseselectrodestorec-
ordtheelectricalresponseoftheretinatoflashesoflight(Fig.12.12a).Photopic
(light-adapted)andscotopic(dark-adapted)electroretinogramsareobtain-
ed.Theelectroretinogram(ERG)consistsofanegativeAwaveindicatingthe
responseofthephotoreceptorsandapositiveBwaveprimarilyindicatingthe
responseofthebipolarcellsandthesupportingcellsofMüller(Fig.12.12b).A
flickerERG(repeatedflashes)isolatespureconeresponse;apatternERG
(suchasacheckerboard)andoscillatingpotentialscanbeusedtoevaluatethe
innerlayersoftheretina.TheERGrepresentsasummationresponseoftheret-
ina.AfocalERGcanrecordtheresponseofisolatedareasoftheretina.
Theclassicindicationforanelectroretinogramisretinitispigmentosa
withearlylossofscotopicandphotopicpotentials.
Electro-oculogram(EOG):Theelectro-oculogramdetectsabnormalchanges
intheretinalpigmentepitheliumsuchasmacularvitelliformdystrophy.This
examinationmethodutilizesthedipoleoftheeyeinwhichthecorneaforms
thepositivepoleandtheretinalpigmentepitheliumthenegativepole.The
standingpotentialacrosscorneaandretinaincomparisontothecorneais
measuredindirectlywithtwotemporalelectrodes(Fig.12.13).Duringthe
measuringprocess,thepatientperformsregulareyemovementsbyalter-
natelyfocusingontwolights.Thestandingpotentialisnormallyhigherinthe
light-adaptedeyethaninthedark-adaptedeye.Theratiooflight-adapted
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331. 313
Electroretinogram(ERG).
Fig.12.12
aRetinalpoten-
tialsarerecorded
withacorneal
contactlenselec-
trodeandskin
electrode.
bNormalelectroretinogram.
potentialtodark-adaptedpotential(Ardenratio)isobtainedtoevaluatethe
eye;thisratioisnormallygreaterthan1.8.Theratiowillbedecreasedinthe
presenceofabnormalchanges.
Thetypicalindicationforanelectro-oculogramismacularvitelliform
dystrophy(Best’svitelliformdystrophy)withasignificantlydecreased
Ardenratio.
Visualevokedpotential(VEP):Thisexaminationisusedtodiagnosedam-
agealongthevisualpathway.TheVEPisnotaspecificexaminationoftheret-
inasuchasanelectroretinogramorelectro-oculogram.Thismethodisbriefly
discussedinChapter13,OpticNerve.
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332. 314
Electro-oculogram(EOG).
+
–
– –
–
+
+
+
left eye
EOG
potential
Right gaze
EOG
potential
Dark Light
Normal
Subnormal
Abnormal
0 15 20 Time (min.)
Straight aheadLeft gaze
Fig.12.13Theeye
formsadipolein
whichtheanterior
poleispositiveand
theposteriorpole
isnegative.The
EOGrecordsthe
changeinposition
ofthestanding
potentialoftheret-
inawithtwotem-
poralelectrodes.
12.3VascularDisorders
12.3.1DiabeticRetinopathy
Definition
Diabeticretinopathyisanocularmicroangiopathy.
Epidemiology:Diabeticretinopathyisoneofthemaincausesofacquired
blindnessintheindustrializedcountries.Approximately90%ofalldiabetic
patientshaveretinopathyaftertwentyyears.
Pathogenesisandindividualstagesofdiabeticretinopathy:Diabetesmel-
lituscanleadtochangesinalmosteveryoculartissue.Theseincludesymp-
tomsofkeratoconjunctivitissicca,xanthelasma,mycoticorbitalinfections,
transitoryrefractorychanges,cataract,glaucoma,neuropathyoftheoptic
nerve,oculomotorpalsy.However,90%ofallvisualimpairmentsindiabetic
patientsarecausedbydiabeticretinopathy.Themostcommoninternational
nomenclatureusedtodescribethevariouschangesindiabeticretinopathy
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333. 315
Table12.1Changesindiabeticretinopathy
Stageofretinopathy Retinalchanges
Nonproliferativediabetic
retinopathy
!Microaneurysms.
!Intraretinalhemorrhages
!Lipiddepositsintheretina(hardexudates)
!Retinaledema
!Venousbeading
!Excessivehemorrhages
!Cotton-woolspots(nervefiberinfarctionswithsoft
exudates)
!Intraretinalmicrovascularanomalies
Proliferativediabetic
retinopathy
!Preretinalneovascularization
!Vitreoushemorrhage
!Tractionalretinaldetachment(duetotractionofvit-
reousscarring)
!Rubeosisiridis(neovascularizationoftheiristhatcan
occludetheangleoftheanteriorchamber;this
entailstheriskofacutesecondaryangleclosureglau-
coma)
(Table12.1)isbasedontheclassificationoftheDiabeticRetinopathyStudy.A
distinctionismadebetweennonproliferativestages(1.mild,2.moderate,3.
severe;Fig.12.14)andproliferativestages(1.non-high-risk2.high-risk;
Fig.12.15–12.17).
Moderatenonproliferativediabeticretinopathy.
Fig.12.14
Microaneurysms,
intraretinal
hemorrhages,
hardexudates
(arrow),andcot-
ton-woolspots
(arrowheads).
12.3VascularDisorders
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334. 316
Proliferativediabeticretinopathy.
Fig.12.15
aPreretinalneo-
vascularization
(arrows)isatypi-
calsign.
bCorresponding
angiographic
image.Fluo-
resceindyeleak-
ageisseeninthe
neovascularized
area(arrows).
Symptoms:Diabeticretinopathyremainsasymptomaticforalongtime.Only
inthelatestageswithmacularinvolvementorvitreoushemorrhagewillthe
patientnoticevisualimpairmentorsuddenlygoblind.
Diagnosticconsiderations:Diabeticretinopathyanditsvariousstages(see
Table12.1)arediagnosedbystereoscopicexaminationofthefunduswith
thepupildilated.Ophthalmoscopyandevaluationofstereoscopicfundus
photographsrepresentthegoldstandard.Fluoresceinangiographyisusedto
determineiflasertreatmentisindicated.Thepresenceofrubeosisiridisis
confirmedorexcludedinslit-lampexaminationwithamobilepupil,i.e.,
withouttheuseofamydriatic,andbygonioscopyoftheangleoftheanterior
chamber.
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335. 317
High-riskproliferativediabeticretinopathy.
Fig.12.16The
clearlyvisiblevit-
reoushemor-
rhageseenhere
(arrow)isatypi-
calsignofthis
stageofdiabetic
retinopathy.The
patientwillonly
noticedeteriora-
tionofvisionin
thislaterstage.
Differentialdiagnosis:Adifferentialdiagnosismustexcludeothervascular
retinaldiseases,primarilyhypertonicchangesofthefundus(thisisdoneby
excludingtheunderlyingdisorder).
Treatment:Clinicallysignificantmacularedema,i.e.,macularedemathat
threatensvision,ismanagedwithfocallasertreatmentattheposteriorpole.
Proliferativediabeticretinopathyistreatedwithscatterphotocoagulation
performedinthreetofivesessions.
Prophylaxis:Failuretoperformregularophthalmologicscreeningexamina-
tionsinpatientswithdiabetesmellitusisanegligentomissionthatexposes
patientstotheriskofblindness.Therefore,alltypeIIdiabeticsshould
undergoophthalmologicexaminationupondiagnosisofthedisorder,and
typeIdiabeticsshouldundergoophthalmologicexaminationwithinfive
yearsofthediagnosis.Thereafter,diabeticpatientsshouldundergoophthal-
mologicexaminationonceayear,ormoreoftenifdiabeticretinopathyis
present.Pregnantpatientsshouldbeexaminedonceeverytrimester.
Clinicalcourseandprognosis:Optimumcontrolofbloodglucosecanpre-
ventordelayretinopathy.However,diabeticretinopathycanoccurdespite
optimumtherapy.Rubeosisiridis(neovascularizationintheiris)inprolifera-
tivediabeticretinopathyistantamounttolossoftheeyeasrubeosisiridisisa
relentlessandirreversibleprocess.
Theriskofblindnessduetodiabeticretinopathycanbereducedby
optimumcontrolofbloodglucose,regularophthalmologicexamina-
tion,andtimelytherapy,butitcannotbecompletelyeliminated.
12.3VascularDisorders
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336. 318
Proliferativediabeticretinopathybeforeandafterlasertreatment.
Fig.12.17
aProliferative
diabeticreti-
nopathywith
clinicallysignifi-
cantmacular
edemabefore
lasertherapy.
bFindingsafter
successfullaser
treatment(laser
burnsappear
whitishbrown).
12.3.2RetinalVeinOcclusion
Definition
Veinocclusionoccursasaresultofcirculatorydysfunctioninthecentralveinor
oneofitsbranches.
Epidemiology:Retinalveinocclusionisthesecondmostfrequentvascular
retinaldisorderafterdiabeticretinopathy.Themostfrequentunderlyingsys-
temicdisordersarearterialhypertensionanddiabetesmellitus;themost
frequentunderlyingoculardisorderisglaucoma.
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337. 319
Frequentunderlyingsystemicdisordersofretinalveinocclusioninclude
arterialhypertensionanddiabetesmellitus.Frequentunderlyingocular
disordersincludeglaucomaandretinalvasculitis.
Etiology:Occlusionofthecentralveinoftheretinaoritsbranchesis
frequentlyduetolocalthrombosisatsiteswherescleroticarteriescompress
theveins.Incentralretinalveinocclusion,thethrombusliesatthelevelof
thelaminacribrosa;inbranchretinalveinocclusion,itisfrequentlyatan
arteriovenouscrossing.
Symptoms:Patientsonlynoticealossofvisualacuityifthemaculaoroptic
diskareinvolved.
Diagnosticconsiderationsandfindings:Centralretinalveinocclusioncan
bediagnosedwherelinearorpunctiformhemorrhagesareseentooccurinall
fourquadrantsoftheretina(Fig.12.18a).Oftenonewillfinddistendedand
increasinglymeanderingveins.Inbranchretinalveinocclusion,intraretinal
hemorrhageswilloccurintheareaofvascularsupply;thisbleedingmay
occurinonlyonequadrant(Fig.12.18b)orintwoquadrants(hemispheric
veinocclusion).Cotton-woolspotsandretinaloroptic-diskedemamayalso
bepresent(simultaneousretinalandoptic-diskedemaisalsopossible).
Chronicocclusionsmayalsobeaccompaniedbylipiddeposits.Onedifferen-
tiatesbetweennon-ischemicandischemicocclusiondependingonthe
extentofcapillaryocclusion.Ischemicocclusionisdiagnosedwiththeaidof
fluoresceinangiography.
Differentialdiagnosis:Otherformsofvascularretinaldiseasemustbe
excluded,especiallydiabeticretinopathy.Aninternistshouldbeconsultedto
verifyorexcludethepossiblepresenceofanunderlyingdisorder.
Treatment:Intheacutestageofveinocclusion,hematocritshouldbe
reducedto35–38%byhemodilution.Lasertreatmentisperformedin
ischemicocclusionthatprogressestoneovascularizationorrubeosisiridis.
Focallasertreatmentisperformedinbranchretinalveinocclusionwith
macularedemawhenvisualacuityisreducedto20/40orlesswithinthree
monthsofocclusion.
Prophylaxis:Earlydiagnosisandprompttreatmentofunderlyingsystemic
andoculardisordersisimportant.
Clinicalcourseandprognosis:Visualacuityimprovesinapproximatelyone-
thirdofallpatients,remainsunchangedinone-third,andworsensinone-
thirddespitetherapy.Complicationsincludepreretinalneovascularization,
retinaldetachment,andrubeosisiridiswithangleclosureglaucoma.
12.3VascularDisorders
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338. 320
12.3.3RetinalArterialOcclusion
Definition
Retinalinfarctionduetoocclusionofanarteryinthelaminacribrosaora
branchretinalarteryocclusion.
Epidemiology:Retinalarteryocclusionsoccursignificantlylessoftenthan
veinocclusions.
Etiology:Emboli(Table12.2)arefrequentlythecauseofretinalarteryand
branchretinalarteryocclusions.Lessfrequentcausesincludeinflammatory
processessuchastemporalarteritis(Horton’sarteritis).
Retinalveinocclusion.
Fig.12.18
aCentralretinal
veinocclusion:in-
traretinalhemor-
rhagesarevisible
ineveryretinal
quadrant.
bOcclusionof
thetwomainin-
feriorbranches.
Bleedingoccurs
onlyintheaf-
fectedareasof
theretinain
branchretinal
veinocclusion.
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339. 321
Table12.2Causesofembolusinretinalarteryocclusion
Typeofembolus Sourceofembolus
Calciumemboli Atheromatousplaquesfromthecarotidarteryor
heartvalves
Cholesterolemboli Atheromatousplaquesfromthecarotidartery
Thrombocyte-fibrinemboli
(gray)
Inatrialfibrillation,myocardialinfarction,ordueto
heartsurgery
Myxomaemboli Inatrialmyxoma(youngpatients)
Bacterialormycoticemboli
(Rothspots)
Inendocarditisandsepticemia
Horton’sarteritisshouldbeexcludedwhereretinalarteryocclusionis
accompaniedbyheadache.
Symptoms:Incentralretinalarteryocclusion,thepatientgenerallycom-
plainsofsudden,painlessunilateralblindness.Inbranchretinalarteryocclu-
sion,thepatientwillnoticealossofvisualacuityorvisualfielddefects.
Diagnosticconsiderations:Thediagnosisismadebyophthalmoscopy.In
theacutestageofcentralretinalarteryocclusion,theretinaappearsgrayish
whiteduetoedemaofthelayerofopticnervefibersandisnolongertrans-
parent.Onlythefoveacentralis,whichcontainsnonervefibers,remainsvis-
ibleasa“cherryredspot”becausetheredofthechoroidshowsthroughatthis
site(Fig.12.19a).Thecolumnofbloodwillbeseentobeinterrupted.Rarely
onewillobserveanembolus.Patientswithacilioretinalartery(arteryorigi-
natingfromtheciliaryarteriesinsteadofthecentralretinalartery)will
exhibitnormalperfusionintheareaofvascularsupply,andtheirlossofvisual
acuitywillbeless.Atrophyoftheopticnervewilldevelopinthechronicstage
ofcentralretinalarteryocclusion.
Intheacutestageofcentralretinalarteryocclusion,thefoveacentralis
appearsascherryredspotonophthalmoscopy.Thereisnotedemaof
thelayerofopticnervefibersinthisareabecausethefoveacontainsno
nervefibers.
Inbranchretinalarteryocclusion,aretinaledemawillbefoundinthe
affectedareaofvascularsupply(Fig.12.19b).Perimetry(visualfieldtesting)
willrevealatotalvisualfielddefectincentralretinalarteryocclusionanda
partialdefectinbranchocclusion.
Differentialdiagnosis:Lipid-storagediseasesthatcanalsocreateacherry
redspotsuchasTay-Sachsdisease,Niemann-Pickdisease,orGaucher’sdis-
easeshouldbeexcluded.Thesediseasescanbeclearlyidentifiedonthebasis
12.3VascularDisorders
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340. 322
Retinalarteryocclusion.
Fig.12.19
aCentralretinal
arteryocclusion.
Thepaper-thin
vesselsandex-
tensiveretinal
edemainwhich
theretinaloses
itstransparency
aretypicalsigns.
Onlythefoveais
spared,whichap-
pearsasacherry
redspot.
bBranchretinal
arteryocclusion.
Multipleemboli
arevisibleinthe
affectedarterial
branches(ar-
rows).
oftheirnumerousadditionalsymptomsandthefactthattheyafflictyounger
patients.
Treatment:Emergencytreatmentisoftenunsuccessfulevenwheninitiated
immediately.Ocularmassage,medicationsthatreduceintraocularpressure,
orparacentesisareappliedinanattempttodraintheembolusinaperipheral
retinalvessel.Calciumantagonistsorhemodilutionareappliedinanattempt
toimprovevascularsupply.Lysistherapyisnolongerperformedduetothe
poorprognosis(itisnotabletopreventblindness)andtherisktovitaltissue
involved.
Prophylaxis:Excludingorinitiatingprompttherapyofpredisposingunder-
lyingsystemicdisordersiscrucial(seeTable12.2).
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341. 323
Clinicalcourseandprognosis:Theprognosisispoorbecauseirreparable
damagetotheinnerlayersoftheretinaoccurswithinonehour.Blindnessusu-
allycannotbepreventedincentralretinalarteryocclusion.Theprognosisis
betterwhereonlyabranchofthearteryisoccludedunlessamacularbranch
isaffected.
12.3.4HypertensiveRetinopathyandScleroticChanges
Definition
Arterialchangesinhypertensionareprimarilycausedbyvasospasm;inarterio-
sclerosistheyaretheresultofthickeningofthewallofthearteriole.
Epidemiology:Arterialhypertensioninparticularfiguresprominentlyin
clinicalsettings.
Vascularchangesduetoarterialhypertensionarethemostfrequent
causeofretinalveinocclusion.
Pathogenesis:Highbloodpressurecancausebreakdownoftheblood-retina
barrierorobliterationofcapillaries.Thisresultsinintraretinalbleeding,cot-
ton-woolspots,retinaledema,orswellingoftheopticdisk.
Symptoms:Patientswithhighbloodpressurefrequentlysufferfromhead-
acheoreyepain.ImpairedvisionorlossofvisualacuityonlyoccursinstageIII
orIVhypertensivevascularchanges.Arteriosclerosisdoesnotexhibitany
ocularsymptoms.
Diagnosticconsiderations:Hypertensiveandarterioscleroticchangesinthe
fundusarediagnosedbyophthalmoscopy,preferablywiththepupildilated
(Tables12.3and12.4).Changesintheretinalvasculaturearefrequentfind-
ings;choroidalinfarctionsarerareinacutehypertension(Elschnig’sspots:
circumscribedatrophyandproliferationofpigmentepitheliuminthe
infarctedarea).
Differentialdiagnosis:Ophthalmoscopyshouldbeperformedtoexclude
othervascularretinaldisorderssuchasdiabeticretinopathy.Diabeticreti-
nopathyisprimarilycharacterizedbyparenchymalandvascularchanges;a
differentialdiagnosisismadebyconfirmingorexcludingthesystemicunder-
lyingdisorder.
Treatment:Treatingtheunderlyingdisorderiscrucialwherefunduschanges
duetoarterialretinopathyarepresent.Bloodpressureshouldbereducedto
below140/90mmHg.Funduschangesduetoarteriosclerosisareuntreat-
able.
12.3VascularDisorders
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342. 324
Table12.3Stagesofhypertensivevascularchanges(asdescribedbyKeith,Wagener,
andBarker)
Stage Characteristics
StageI: Constricted,tortuousarterioles.
StageII: SeverevascularconstrictionandGunn’scrossingsign.Thecolumn
ofvenousbloodisconstrictedbythescleroticarteryatanarterio-
venouscrossing.
StageIII: Retinalhemorrhages,hardexudates,cotton-woolspots,retinal
edema(Fig.12.20)
StageIV: Papilledema
TheWHOdistinguishesbetweenhypertensiveretinopathy(stagesIandII)andmalignant
hypertensiveretinopathy(stagesIIIandIV)
Table12.4Stagesofarterioscleroticvascularchanges(asdescribedbyScheie)
Stage Characteristics
StageI: Wideningofarteriolereflexes
StageII: Arteriovenouscrossingsign
StageIII: Copper-wirearteries(coppercoloredarterialreflex)
StageIV: Silver-wirearteries(silvercoloredarterialreflex)
Hypertensiveretinopathy(StageIII).
Fig.12.20Typi-
calfindingsin
thisstageinclude
hemorrhages
(herereadilyvis-
ible)andcotton-
woolspots.
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343. 325
Prophylaxis:Regularbloodpressuremonitoringandophthalmoscopic
examinationofthefundusarerequiredtominimizetheriskofcomplications
(seebelow).
Clinicalcourseandcomplications:Sequelaeofarterioscleroticandhyper-
tensivevascularchangesincluderetinalarteryandveinocclusionandthefor-
mationofmacroaneurysmsthatcanleadtovitreoushemorrhage.Inthepres-
enceofpapilledema,thesubsequentatrophyoftheopticnervecanproduce
lastingandoccasionallyseverelossofvisualacuity.
Prognosis:Insomecases,thecomplicationsdescribedaboveare
unavoidabledespitewellcontrolledbloodpressure.
12.3.5Coats’Disease
Definition
Congenitalretinaltelangiectasiawithvascularanomaliesthatnearlyalways
presentsunilaterallyandcanleadtoexudationandeventuallytoexudativereti-
naldetachment.
Epidemiology:Thisraredisordermanifestsitselfinyoungchildrenand
teenagers.Boysareusuallyaffected(inabout90%ofallcases).
Coats’diseaseusuallyoccursinyoungandteenageboys.Itisnearly
alwaysunilateral.
Pathogenesis:Telangiectasiaandaneurysmsleadtoexudationandeventu-
allytoretinaldetachment.
Symptoms:Theearlystagesarecharacterizedbylossofvisualacuity,the
laterstagesbyleukocoria(whitepupil;seeFig.12.36)orunilateralstra-
bismus,althoughthecombinationofleukocoriaandstrabismusisalso
possible.
Diagnosticconsiderationsandfindings:Ophthalmoscopywillrevealtelan-
giectasia,subretinalwhitishexudatewithexudativeretinaldetachmentand
hemorrhages(Fig.12.21).
Differentialdiagnosis:Intheadvancedstagesofthedisorder,retinoblas-
tomashouldbeexcludedbyophthalmoscopyandretinopathyofprematurity
onthebasisofthepatient’shistory.Bothdisordersmayalsocauseleukocoria.
Treatment:Thetreatmentofchoiceislaserphotocoagulationorcryotherapy
todestroyanomalousvasculature.
12.3VascularDisorders
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344. 326
Coats’disease.
Fig.12.21Typi-
calvascular
changesoftelan-
giectasia(arrow)
accompaniedby
exudativeretinal
detachmentwith
numerouslipid
deposits(arrow-
heads).
Prognosis:Leftuntreated,thediseasewilleventuallycauseblindnessdueto
totalretinaldetachment.Treatmentiseffectiveinpreventingblindnessin
about50%ofallpatients.
12.3.6RetinopathyofPrematurity
Definition
Aretinaldisorderattributabletodisruptionofnormaldevelopmentofthereti-
nalvasculatureinpreterminfantswithbirthweightlessthan2500g.
Epidemiology:Thedisorderisrare.Infantswithbirthweightbelow1000g
areatincreasedriskofdevelopingthedisorder.Retinopathyofprematurityis
notalwayspreventabledespiteoptimumcareandstrictmonitoringofpartial
pressureofoxygen.
Etiology:Pretermbirthandexposuretooxygendisturbsthenormaldevelop-
mentoftheretinalvasculature.Vesselobliterationoccurs,followedbypro-
liferativeneovascularization.Thisresultsinvitreoushemorrhage,retinal
detachment,and,inthelatescarringstage,retrolenticularfibroplasiaasves-
selsandconnectivetissuefusewiththedetachedretina.
Findingsandsymptoms:Afteraninitiallyasymptomaticclinicalcourse,vit-
reoushemorrhageorretinaldetachmentwillbeaccompaniedbysecondary
strabismus.Leukocoriacanoccurintheretrolenticularfibroplasiastage.
Table12.5showstheclassificationofthevariousstages.
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345. 327
Asiscustomaryinophthalmology,theextentoftherespectiveabnormal
changeisspecifiedbyanalogytoaclockface.Forexampleademarcationline
maybesaidtoextendfromonetosixo’clock.Aplusstageincludesdilated
andtortuousvasculatureoftheposteriorpoleinadditiontotheother
changes.
Diagnosticconsiderations:Theretinashouldbeexaminedwiththepupil
dilatedfourweeksafterbirthatthelatest.Thismaybedoneaspartofthe
routineexaminationofthenewborn.Follow-upexaminationswilldependon
thedegreeofretinalvascularization.
Differentialdiagnosis:Othercausesofleukocoriasuchasretinoblastomaor
cataract(seeTable11.1)shouldbeconsidered.
Treatment:SurgeryisrarelysuccessfulinstagesIVandV.InstageIII,laser
photocoagulationorcryotherapyisperformedinthenonvascularizedpor-
tionoftheretina.
Prophylaxis:Partialpressureofoxygenshouldbekeptaslowaspossible,and
ophthalmologicscreeningexaminationsshouldbeperformed.
Earlydetectionofretinopathyofprematurityisparticularlyimportant.
Clinicalcourseandprognosis:StageIandIIretinopathyresolvesspon-
taneouslyin85%ofallaffectedchildren.
Table12.5Classificationofretinopathyofprematurity
Stage Characteristics
StageI: Demarcation(borderbetweenvascularizedandnonvascularized
retina)
StageII: Formationofaridge(developmentofintraretinalproliferative
tissue)
StageIII: Ridgewithextraretinalproliferation
StageIV: Subtotalretinaldetachment
StageV: Totalretinaldetachment
12.3VascularDisorders
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346. 328
12.4DegenerativeRetinalDisorders
12.4.1RetinalDetachment
Definition
Retinaldetachmentreferstotheseparationoftheneurosensoryretina(see
Fig.12.2a)fromtheunderlyingretinalpigmentepithelium,towhichnormally
itislooselyattached.Thiscanbeclassifiedintofourtypes:
!Rhegmatogenousretinaldetachmentresultsfromatear,i.e.,abreakin
theretina.
!Tractionalretinaldetachmentresultsfromtraction,i.e.,fromvitreous
strandsthatexerttensileforcesontheretina(seeproliferativevitreoreti-
nopathyandcomplicatedretinaldetachment).
!Exudativeretinaldetachmentiscausedbyfluid.Blood,lipids,orserous
fluidaccumulatesbetweentheneurosensoryretinaandtheretinalpig-
mentepithelium.Coats’diseaseisatypicalexample.
!Tumor-relatedretinaldetachment.
Primaryretinaldetachmentusuallyresultsfromatear.Inrarecases,second-
aryretinaldetachmentmayalsoresultfromatearduetootherdisordersor
injuries.Combinationsofbotharealsopossiblebutrare.Proliferativevitreore-
tinopathyfrequentlydevelopsfromachronicretinaldetachment(seeChapter
11,VitreousBody).
Epidemiology:Althoughretinaldetachmentsarerelativelyrarelyencoun-
teredinophthalmologicpractice,theyareclinicallyhighlysignificantasthey
canleadtoblindnessifnottreatedimmediately.
Rhegmatogenousretinaldetachment(mostfrequentform):Approxi-
mately7%ofalladultshaveretinalbreaks.Theincidenceofthisfinding
increaseswithadvancedage.Thepeakincidenceisbetweenthefifthand
seventhdecadesoflife.Thisindicatesthesignificanceofposteriorvitreous
detachment(separationofthevitreousbodyfrominnersurfaceoftheretina;
alsoage-related)asacauseofretinaldetachment.Theannualincidenceof
retinaldetachmentisoneper10000persons;theprevalenceisabout0.4%in
theelderly.Thereisaknownfamilialdisposition,andretinaldetachmentalso
occursinconjunctionwithmyopia.Theprevalenceofretinaldetachment
withemmetropia(normalvision)is0.2%comparedwith7%inthepresence
ofseveremyopiaexceedingminus10diopters.
Exudative,tractional,andtumor-relatedretinaldetachmentsare
encounteredfarlessfrequently.
Etiology:Rhegmatogenousretinaldetachment.Thisdisorderdevelopsfrom
anexistingbreakintheretina.Usuallythisbreakisintheperipheralretina,
rarelyinthemacula(Fig.12.22).Twotypesofbreaksaredistinguished:
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347. 329
Horseshoetear(arrow)andretinaldetachment(whitishretina).
Fig.12.22The
imageshowsa
typicalreddish
horseshoetearin
theretina(arrow)
withbullousreti-
naldetachment.
!Roundbreaks:Aportionoftheretinahasbeencompletelytornoutduetoa
posteriorvitreousdetachment.
!Horseshoetears:Theretinaisonlyslightlytorn.
Noteveryretinalbreakleadstoretinaldetachment.Thiswilloccuronly
wheretheliquifiedvitreousbodyseparates,andvitreoushumorpenetrates
beneaththeretinathroughthetear.Theretinaldetachmentoccurswhenthe
forcesofadhesioncannolongerwithstandthisprocess.Tractionalforces
(tensileforces)ofthevitreousbody(usuallyvitreousstrands)canalsocause
retinaldetachmentwithorwithoutsynchysis.Inthisandeveryothertypeof
retinaldetachment,thereisadynamicinterplayoftractionalandadhesive
forces.Whethertheretinawilldetachdependsonwhichoftheseforcesis
stronger.
Tractionalretinaldetachment.Thisdevelopsfromthetensileforcesexerted
ontheretinabypreretinalfibrovascularstrands(seeproliferativevitreoreti-
nopathy)especiallyinproliferativeretinaldiseasessuchasdiabeticreti-
nopathy.
Exudativeretinaldetachment.Theprimarycauseofthistypeisthebreak-
downoftheinnerorouterblood–retinabarrier,usuallyasaresultofavascu-
lardisordersuchasCoats’disease.Subretinalfluidwithorwithouthardexu-
dateaccumulatesbetweentheneurosensoryretinaandtheretinalpigment
epithelium.
Tumor-relatedretinaldetachment.Eitherthetransudatefromthetumor
vasculatureorthemassofthetumorseparatestheretinafromitsunderlying
tissue.
12.4DegenerativeRetinalDisorders
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348. 330
Symptoms:Retinaldetachmentcanremainasymptomaticforalongtime.In
thestageofacuteposteriorvitreousdetachment,thepatientwillnotice
flashesoflight(photopsia)andfloaters,blackpointsthatmovewiththe
patient’sgaze.Aposteriorvitreousdetachmentthatcausesaretinaltearmay
alsocauseavulsionofaretinalvessel.Bloodfromthisvesselwillthenenter
thevitreousbody.Thepatientwillperceivethisas“blackrain,”numerous
slowlyfallingsmallblackdots.Anothersymptomisadarkshadowinthe
visualfield.Thisoccurswhentheretinadetaches.Thepatientwillperceivea
fallingcurtainorarisingwall,dependingonwhetherthedetachmentissupe-
riororinferior.Abreakinthecenteroftheretinawillresultinasuddenand
significantlossofvisualacuity,whichwillincludemetamorphopsia(image
distortion)ifthemaculaisinvolved.
Diagnosticconsiderations:Thelesionisdiagnosedbystereoscopicexami-
nationofthefunduswiththepupildilated.Thedetachedretinawillbewhite
andedematousandwillloseitstransparency.Ophthalmoscopywillreveala
bullousretinaldetachment;inrhegmatogenousretinaldetachment,a
brightredretinalbreakwillalsobevisible(seeFig.12.22).Thetearsinrheg-
matogenousretinaldetachmentusuallyoccurinthesuperiorhalfoftheret-
inainaregionofequatorialdegeneration.Intractionalretinaldetachment,
thebullousdetachmentwillbeaccompaniedbypreretinalgraystrands.In
exudativeretinaldetachment,onewillobservethetypicalpictureofserous
detachment;theexudativeretinaldetachmentwillgenerallybeaccom-
paniedbymassivefattydepositsandoftenbyintraretinalbleeding.
Thetumor-relatedretinaldetachment(ascanoccurwithamalignant
melanoma)eitherleadstosecondaryretinaldetachmentoverthetumororat
somedistancefromthetumorintheinferiorperipheralretina.Ultrasound
studiescanhelpconfirmthediagnosiswhereretinalfindingsareequivocalor
atumorissuspected.
Aninferiorretinaldetachmentatsomedistancefromthetumorisa
signthatthetumorismalignant.
Differentialdiagnosis:Degenerativeretinoschisisistheprimarydisorder
thatshouldbeexcludedasitcanalsoinvolverhegmatogenousretinaldetach-
mentsinrarecases.Aretinaldetachmentmayalsobeconfusedwitha
choroidaldetachment.Fluidaccumulationinthechoroid,duetoinflam-
matorychoroidaldisorderssuchasVogt-Koyanagi-Haradasyndrome,causes
theretinalpigmentepitheliumandneurosensoryretinatobulgeoutward.
Theseformsofretinaldetachmenthaveagreenishdarkbrowncolorincon-
trasttotheotherformsofretinaldetachmentdiscussedhere.
Treatment:Retinalbreakswithminimalcircularretinaldetachmentcanbe
treatedwithargonlasercoagulation(Fig.12.23).Theretinasurroundingthe
breakisfusedtotheunderlyingtissuewhereasthebreakitselfisleftopen.
Thescarsresultingfromargonlasertherapyaresufficienttopreventany
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349. 331
Retinalbreakimmediatelyafterargonlaserphotocoagulation.
Fig.12.23
Circularwhite
laserburnsare
visiblearoundthe
break.
furtherretinaldetachment.Moreextensiveretinaldetachmentsareusually
treatedwitharetinaltamponadewithanelasticsiliconespongethatis
suturedtotheoutersurfaceofthesclera,aso-calledbuddingprocedure
(Fig.12.24a–c).Itcanbesuturedeitherinaradialposition(perpendicularto
thelimbus)orparalleltothelimbus.Thisindentsthewalloftheglobeatthe
retinalbreakandbringstheportionoftheretinainwhichthebreakislocated
backintocontactwiththeretinalpigmentepithelium.Theindentationalso
reducesthetractionofthevitreousbodyontheretina.Anartificalscaris
createdtostabilizetherestoredcontactbetweentheneurosensoryretinaand
retinalpigmentepithelium.Thisisachievedwithacryoprobe.Aftera
successfuloperation,thisscarpreventsrecurringretinaldetachment.Where
thereareseveralretinalbreaksorthebreakcannotbelocated,asiliconecer-
clageisappliedtotheglobeasacircumferentialbucklingprocedure.Thepro-
ceduresdescribedupuntilnowapplytouncomplicatedretinaldetachments,
i.e.,withoutproliferativevitreoretinopathy.Suturingaretinaltamponade
withsiliconespongemayalsobeattemptedinitiallyinacomplicatedretinal
detachmentwithproliferativevitreoretinopathy.Ifthistreatmentis
unsuccessful,thevitreoretinalproliferationsareexcised,andavitrectomyis
performedinwhichthevitreousbodyisreplacedwithRinger’ssolution,gas,
orsiliconeoil.Thesefluidstamponadetheeyefromwithin.
Prophylaxis:High-riskpatientsabovetheageof40withapositivefamily
historyandseveremyopiashouldberegularlyexaminedbyanophthalmolo-
gist,preferablyonceayear.
Clinicalcourseandprognosis:About95%ofrhegmatogenousretinal
detachmentscanbetreatedsuccessfullywithsurgery.Wheretherehasbeen
12.4DegenerativeRetinalDisorders
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350. 332
Reattachingadetachedretinawithasiliconespongetamponade.
ba
Fig.12.24aTheocularmusclesareretractedandtheeyeisbroughtintothe
properpositionfortheoperation.Thetamponadeissuturedtotheoutersurfaceof
thesclera.bCrosssectionoftheeyewiththetamponadeinplace:Theglobeisin-
dentedatthesiteofthetamponade.cWedgedbeneaththehorseshoetear(arrow)
isaradialtamponade(arrowhead).Theretinaisagainincontactwiththeunderlying
tissue.
c
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351. 333
macularinvolvement(i.e.,theinitialdetachmentincludedthemacula),aloss
ofvisualacuitywillremain.Theprognosisfortheotherformsofretinal
detachmentisusuallypoor,andtheyareoftenassociatedwithsignificant
lossofvisualacuity.
12.4.2DegenerativeRetinoschisis
Definition
Afrequentlybilateralsplitinaninnerandouterlayeroftheretina.Thesplitis
usuallyattheleveloftheouterplexiformlayer(Fig.12.25).
Epidemiology:About25%ofallpeoplehaveretinoschisis.Thetendency
increaseswithage.
Pathogenesis:Idiopathicretinalsplittingoccurs,usuallyintheouterplexi-
formlayer.
Symptoms:Retinoschisisisprimarilyasymptomatic.Thepatientwillusually
noticeareductionofvisualacuityandseeshadowsonlywhentheretinalsplit
issevereandextendstotheposteriorpole.
Diagnosticconsiderations:Ophthalmoscopicexaminationwillreveal
bullousseparationofthesplitinnerlayeroftheretina.Theinnersurfacehas
theappearanceofhammeredmetal.Rarelybreakswilloccurintheinnerand
outerretinallayers.
Retinoschisis.
Fig.12.25Split
intheretinawith
bulloussepara-
tionoftheinner
layersoftheret-
ina(arrows).
12.4DegenerativeRetinalDisorders
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352. 334
Differentialdiagnosis:Rhegmatogenousretinaldetachmentshouldbeex-
cluded.Ophthalmoscopywillrevealacontinuousbreakintheretinainareti-
naldetachment,andtheretinawillnotappearastransparentasinretino-
schisis.However,retinalbreakscanalsooccurinretinoschisis.Intheinner
layeroftheretina,thesebreakswillbeverysmallandhardlydiscernible.In
theouterlayer,theywillbeverylarge.Completerhegmatogenousretinal
detachmentcanoccurinretinoschisisonlywherethereisabreakinboth
layers.
Treatment:Usuallynotreatmentisrequired.Therarecasesinwhichretinal
detachmentoccursaretreatedsurgicallyusingthestandardproceduresfor
retinaldetachment.
Degenerativeretinoschisisdiffersfromretinaldetachmentinthatit
usuallyrequiresnotreatment.
Clinicalcourseandprognosis:Theprognosisfordegenerativeretinoschisis
isverygood.Progressiveretinalsplittingorretinaldetachmentwithasub-
sequentreductioninvisualacuityisrare.
12.4.3PeripheralRetinalDegenerations
Definition
Peripheralretinaldegenerationsrefertodegenerativechangesthatlieparallel
totheoraserrataintheperipheralportionsoftheretina.Theseincludetwo
basictypes:
!Harmlessretinalchangessuchasparsplanacystsoftheposteriorciliary
bodyorperipheralchorioretinalatrophy(cobblestonedegeneration).
!Precursorsofretinaldetachmentsuchaslocalthinningoftheretina
referredtoassnailtrackorlatticedegeneration.
Epidemiology:Theprevalenceofthelesionsis6–10%.
Pathogenesis:Unknown.
Symptoms:Peripheralretinaldegenerationsareasymptomatic.
Diagnosticconsiderations:Thediagnosisismadebyophthalmoscopic
examinationoftheperipheralretinawiththepupildilated.Theretinamaybe
examinedbyindirectbinocularophthalmoscopyorusingathree-mirrorlens.
Cobblestonedegenerationsappearaswhitishsharplydefinedlocalized
areasofextensiveatrophyoftheretina,pigmentepithelium,andchoriocapil-
laristhatliebetweentheoraserrataandtheequator.Snailtrackdegenera-
tionpresentswithyellowish,whitishradiantdotsconsistingofmicrogliaand
astrocytes.Latticedegenerationpresentswiththinnedretinalareaswith
whitishscleroticvessels.Thisresultsinreactivefocalatrophyandhypertro-
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353. 335
phyoftheretinalpigmentepitheliumintheregionofequatorialdegenera-
tionandliquefactionoftheoverlyingvitreousbody.
Differentialdiagnosis:Thefindingsarehighlycharacteristicandeasilydiag-
nosedclinically.Rarely,vascularprocessesorinflammatorychangesand
scarsfromothercausesmustbeconsideredinadifferentialdiagnosis.
Treatment:Treatmentiseithernotrequiredornotrecommendedaslaser
therapydoesnotreducetheriskofretinaldetachment.Ophthalmoscopicfol-
low-upexaminationsshouldbeperformedatregularintervals.
Prophylaxis:Noprophylaxisispossible.
Clinicalcourseandprognosis:Theclinicalcourseisusuallybenign.Round
atrophicretinalbreakscandevelopintheareasofsnailtrackandlattice
degeneration.However,thelong-termriskofretinaldetachmentisonly1%.
12.4.4CentralSerousChorioretinopathy
Definition
Serousdetachmentoftheretinaand/orretinalpigmentepithelium.
Etiology:Serousdetachmentoccursthroughadefectintheouterblood–ret-
inabarrier(“tightjunctions”intheretinalpigmentepithelium).Localfactors
thatmayberelatedtophysicalorpsychologicalstressarepresumably
involved.
Epidemiology:Thedisorderprimarilyaffectsmeninthethirdandfourth
decadeoflife.
Symptoms:Patientspresentwithalossofvisualacuity,arelativecentralsco-
toma(darkspot),imagedistortion(metamorphopsia),orperceptionof
objectsaslargerorsmallerthantheyare(macropsiaormicropsia).
Diagnosticconsiderations:Ophthalmoscopywillrevealaserousretinal
detachment,usuallyatthemacula.Inchroniccases,afinebrownandwhite
pigmentepithelialscarwilldevelopatthesiteofthefluideffusion.Swelling
inthecentralretinashortensthevisualaxisandproduceshyperopia.Thesite
offluideffusioncanbeidentifiedduringtheactivephasewiththeaidof
fluoresceinangiography(Fig.12.26aandb).
Treatment:Usuallynotreatmentisrequiredforthefirstoccurrenceofthe
disorder.Retinalswellingresolvesspontaneouslywithinafewweeks.Recur-
rencesmaybetreatedwithlasertherapyprovidedthesiteoffluideffusion
liesoutsidethefoveacentralis.Corticosteroidtherapyiscontraindicatedas
thetherapyitselfcanleadtodevelopmentofcentralserouschorioreti-
nopathyinrarecases.
12.4DegenerativeRetinalDisorders
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354. 336
Centralserouschorioretinopathy.
Fig.12.26
aBullousfluidac-
cumulation
beneaththeret-
ina(arrows).
bAngiogramofthesamepatient.The
siteoffluideffusionappearsasahy-
perfluorescentspot(arrow).
Clinicalcourseandprognosis:Theprognosisisusuallygood.However,
recurrencesorchronicformscanleadtoapermanentlossofvisualacuity.
Localstress-relatedfactorsandsteroidscanleadtomacularedemain
predisposedpatients.
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355. 337
12.4.5Age-RelatedMacularDegeneration
Definition
Progressivedegenerationofthemaculainelderlypatients.
Epidemiology:Age-relatedmaculardegenerationisthemostfrequentcause
ofblindnessbeyondtheageof65years.
Pathogenesis:Drusendevelopintheretinalpigmentepitheliumdueto
accumulationofmetabolicproducts.
Symptoms:Patientsnoticeagraduallossofvisualacuity.Wheremacular
edemaispresent,patientscomplainofimagedistortion(metamorphopsia),
macropsia,ormicropsia.
Findingsanddiagnosticconsiderations:Ophthalmoscopicexamination
candistinguishtwoseparatestagesthatoccurinchronologicalorder(Table
12.6).
Differentialdiagnosis:Othervasculardiseasesoftheretinasuchasbranch
retinalveinocclusionshouldbeexcludedbyophthalmoscopy.Malignant
melanomashouldbeexcludedbyultrasoundstudies.
Treatment:Noreliablyeffectivemedicaltherapyisavailable.Lasertherapy
maybeperformedintheexudativestageinabout5–10%ofallpatients
withoutneovascularizationinvolvingthefoveacentralis.Useofprogressively
strongernearvisionaidssuchasahandmagnifierorbinocularmagnifier
shouldbetried.
Clinicalcourseandprognosis:Thecourseofthedisorderischronicand
leadstoprogressivelossofvisualacuity.
Lasertherapymaybeperformedintheexudativestageoflateage-
relatedmaculardegenerationin10%ofallpatientsprovidedthedis-
orderisdiagnosedearly.
Table12.6Stagesofage-relatedmaculardegeneration(ARM)
Stage Characteristics
1.EarlyARM: Drusen,atrophy,andproliferationofretinalpigmentepithelium
(!175µm)
2.LateARM: Geographicatrophy(Fig.12.27a)oftheretinalpigment
epithelium.Serousdetachmentoftheretinaand/orretinalpig-
mentepithelium;hemorrhages(Fig.12.27b).Fibrousscar
(Fig.12.27c)
12.4DegenerativeRetinalDisorders
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356. 338
Stagesoflateage-relatedmaculardegeneration.
Fig.12.27
aLateage-re-
latedmacular
degeneration:
Typicalsignsin-
cludedrusen
(arrow)andgeo-
graphicallycen-
tralatrophy(ar-
rowhead).
bLateage-
relatedmacular
degeneration:
Intraretinal
bleeding(arrow)
isatypicalsign.
cLateage-related
macular
degeneration:
Thefibrousscar
isatypicalsign.
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12.4.6DegenerativeMyopia
Definition
Thefundusindegenerativemyopiaischaracterizedbyabnormalchorioretinal
atrophy.
Epidemiology:Chorioretinalatrophyduetomyopiaisrare.
Pathogenesis:Theatrophyusuallyoccursinthepresenceofseveremyopia
exceedingminus6diopters.Thecausesincludestretchingchangesintheret-
ina,choroid,andBruch'smembraneduetotheelongatedglobeinaxial
myopia.
Symptoms:Lossofvisualacuityoccurswherethereismacularinvolvement.
Findingsanddiagnosticconsiderations:Typicalsignsincludechorioretinal
atrophyaroundtheopticdiskandattheposteriorpoleanddefectsinBruch’s
membraneknownaslacquercracks(Fig.12.28).Thesecrackscanprovide
openingsforvascularinfiltrationwithresultingsubretinalneovasculariza-
tionthatcanleadtoretinaledemaandbleeding(Fuchs’blackspot).Thefinal
stageofthedisorderischaracterizedbyadiskiformscar.Thediagnosisis
madebyophthalmoscopy.Fluoresceinangiographyisindicatedwheresub-
retinalneovascularizationissuspected.
Differentialdiagnosis:Choroidalscarsandangioidstreaks(breaksinBruch’s
membrane)inpseudoxanthomaelasticummustbeexcludedbyophthalmos-
copy.Thediagnosisisunequivocalwheremyopiaispresent.
Degenerativemyopia.
Fig.12.28
Extensiveareasof
chorioretinal
atrophy(arrows).
12.4DegenerativeRetinalDisorders
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358. 340
Treatment:Thecausesofthedisordercannotbetreated.Itisimportantto
correctmyopiaoptimallywitheyeglassesorcontactlensestoavoidfostering
progressionofthedisorder.Subretinalneovascularizationoutsidethefovea
orclosetoitsbordercanbetreatedbylaserphotocoagulation.
Clinicalcourseandprognosis:Chronicprogressivemyopiawillresultin
increasinglossofvisualacuity.Theprognosisforsubretinalneovasculariza-
tionispoor.Theincidenceofretinaldetachmentishigherinmyopiceyes.
12.5RetinalDystrophies
12.5.1MacularDystrophies
Definition
Maculardystrophiesaredisordersofthemaculathatusuallyoccurbilaterally
andmanifestthemselvesbetweentheagesof10and30.
12.5.1.1Stargardt’sDisease
Definition
Thisisamaculardystrophythatproceedsfromtheretinalpigmentepithelium.
Inheritance:Autosomalrecessivedisorder.
Epidemiology:Stargardt’sdiseaseisrare.
Symptoms:Progressivelossofvisualacuityoccursbetweentheagesof10
and20years.
Findingsanddiagnosticconsiderations:Initialfindingsareslightwith
white“fleck”lesionsinthemacularregion(Fig.12.29),whichmayoccurin
combinationwithlesionsintheentirefundus(fundusflavimaculatus).The
electroretinogramandelectro-oculogramwillbenormalorreduced.Inthe
laterstage,thewhitelesionssignificantlyincreaseinsizeandnumber.This
willnotnecessarilybereflectedintheERGorEOG.
Differentialdiagnosis:Otherdisordersinvolvingwhite“fleck”lesionssuch
asinheritedautosomaldominantdrusenmustbyexcludedbyophthalmos-
copy.Thediagnosisisconfirmedbyfluoresceinangiography.Blockageofthe
choroidalfluoresceinisacharacteristicfeatureofStargardt’sdisease.
Treatment:Notreatmentisavailable.Edge-filteredeyeglassesandmagnify-
ingnearvisionaidscanhelpmakebetteruseofthepatient’sremaining
vision.
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359. 341
Stargardt’sdisease.
Fig.12.29
Typical“fleck”le-
sionsoftheret-
ina(arrow)and
extensiveatrophy
ofthepigment
epitheliuminthe
macularregion.
Prophylaxis:Noprophylaxisispossible.Examinationofsiblingsandgenetic
counselingareindicated.
Clinicalcourseandprognosis:Thedisorderischronicallyprogressive.
Visioninthefinalstagesisusually0.1(20/200)orless.
12.5.1.2Best’sVitelliformDystrophy
Epidemiology:Thedisorderisrare,withanincidencesimilartoStargardt’s
disease.
Inheritance:Thedisorderisinheritedasanautosomaldominanttraitwith
variablepenetranceandexpressivity.Thegenelocusisonchromosome11
(11q13).
Symptoms:Clinicalmanifestationoccursbetweentheagesof5and15years.
Initiallythereisasubjectivelyslightdecreaseinvisualacuity.Inthelater
stagesofthedisorder,visionisreducedtoabout20/200.
Findingsanddiagnosticconsiderations:Atypicalfeatureofthisformof
maculardystrophyisthatvisualacuityisnegligiblydiminishedattheonsetof
thedisorder.However,themorphologicfindingsareremarkable.Ophthalmos-
copywillrevealyellowishroundvitelliformlesionsinthemacularregion
(Fig.12.30)thatlookliketheyolkofafriedegg.(TheLatinwordvitellusmeans
eggyolk.)Usuallytheselesionsarebilateralandsymmetrical,although
eccentriclesionsmayalsooccur.Table12.7liststhevariousmanifestations.
12.5RetinalDystrophies
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360. 342
Best’svitelliformdystrophy.
Fig.12.30
Theyellow,
sharplydemar-
catedlesionap-
pearslikethe
yolkofafried
egg.
Table12.7StagesofBest’svitelliformdystrophy
Stage Characteristics
Previtelliformstage Yellowishcentralpigmentchanges
Vitelliformstage Sharplydemarcatedyellowyolk-likelesion(see
Fig.12.30)
Pseudohypopyonstage Settlingoftheyellowmaterial
Vitelliruptivestage “Scrambling”oftheyolk-likelesionswithirregular
yellowdeposits
Scarstage Transitiontoscar
Themacularchangeresemblinganeggyolkgaverisetothenamevitel-
liformdystrophy.
Differentialdiagnosis:Anunequivocaldiagnosiscanusuallybemadeonthe
basisoftheclinicalpicturealone.Sharplyreducedorabsentlightresponsein
theEOGandERGconfirmsthepresenceofBest’svitelliformdystrophy.
Treatment:Thecausesofthedisordercannotbetreated.
Prophylaxis:Examinationofsiblingsandgeneticcounselingareindicated.
Clinicalcourseandprognosis:TheprognosisismorefavorablethanforStar-
gardt’sdisease.Thedisorderischronicallyprogressive.Visualacuityinthe
bettereyeusuallyremainsabout20/40.Secondarylossofvisualacuitycan
resultfromsubretinalneovascularization.
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361. 343
12.5.2RetinitisPigmentosa
Definition
Thistermisusedtorefertoaheterogeneousgroupofretinaldisordersthat
leadtoprogressivelossofvisualacuity,visualfielddefects,andnightblind-
ness.Thenameretinitispigmentosacomesfromthepigmentdepositsthat
characterizethesedisorders.Intheirclassicform(seefindingsanddiagnostic
considerations)ofsuchdisorders,thesedepositsprogressfromtheperiphery
tothecenteroftheretina.
Epidemiology:Theworldwideincidenceofretinitispigmentosaisestimated
atbetweenoneper35000andoneper70000persons.Theestimatedinci-
denceofmutatedallelesisoneper80persons.
Formsofretinitispigmentosa:
1.Rod-conedystrophy(classicretinitispigmentosa,byfarthemostfrequent
form).
2.Cone-roddystrophy(inverseretinitispigmentosa).
3.Sectoralretinitispigmentosa.
4.Retinitispigmentosasinepigmento(formwithoutpigment).
5.Unilateralretinitispigmentosa.
6.Leber’samaurosis(formoccurringinearlychildhood).
7.Retinopathypunctataalbescens(punctateretinitis).
8.Incombinationwithotherdisordersinsyndromesandmetabolicdis-
orderssuchasmucopolysaccharidoses,Fanconi’ssyndrome,mucolipido-
sisIV,peroxisomaldisorders,Cockayne’ssyndrome,mitochondrial
myopathies,Usher’ssyndrome,neuronalandceroidlipofuscinoses,renal
tubulardefectsyndromes,etc.
Retinitispigmentosaoccursalmostexclusivelyasrod-conedystrophy.There-
fore,theotherextremelyrareformsarenotdiscussedhereexceptforthe
inverseformofclassicretinitispigmentosa,whichispresentedforpurposes
ofcomparison.
Inheritance:Individualgeneticformsmaybeidentifiedfromamongthehet-
erogeneousgroupofdisorderscomprisingretinitispigmentosa.Thisgroupof
disorderscaninvolvevariousgenotypesaswellasvariablephenotypic
expressionordifferentstagesofadisorderwithonespecificgenotype.There
areover15purelyocularformsofretinitispigmentosa.Themostcommon
formofinheritanceisautosomalrecessive(60%),followedbyautosomal
dominant(upto25%),andX-linked(15%).Rhodopsingenemutations(chro-
mosome3)and“retinaldegenerationslow”(RTS)genemutations(chromo-
some6)havealsobeendescribed.
12.5RetinalDystrophies
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362. 344
Symptoms:Initialsymptomsofretinitispigmentosaincludeglare,night
blindness,progressivevisualfielddefects,lossofvisualacuity,andcolor
visiondefects.Theageofmanifestationdependsonthetypeofinheritance.
Findingsanddiagnosticconsiderations:Thediagnosisismadebyophthal-
moscopyonthebasisofaclassicpicture.
Rod-conedystrophy(primarilytherodsareaffectedfirst).“Bone-spicule”
proliferationofretinalpigmentepitheliumisobservedinthemiddleperiphery
oftheretina.Thiswillgraduallyspreadtowardthecenterandfartherperiph-
erally(Fig.12.31).Earlydeficitsincludecolorvisiondefectsanddisturbed
contrastperception.Atrophyoftheopticnerve,discernibleasawaxyyellow
appearanceoftheopticdisk,willoccurintheadvancedstages.Thearteries
willappearnarrowed,andthefundusreflexwillbeextremelymuted.The
patientwilltypicallyhavea“gun-barrel”visualfieldwithgoodvisualacuity
forasurprisinglylongtimebutwithprogressivelossoftheperipheralvisual
field.
Cone-roddystrophy(primarilytheconesareaffectedfirst).Here,thereis
earlylossofvisualacuitywithgradualprogressivelossofvisualfield.Inboth
formsofretinitispigmentosa,thediagnosisisconfirmedbyelectroretinogra-
phy.Lightresponseintheelectroretinogramwillbesharplyreducedor
absentearlyintheclinicalcourseofthedisease.
Differentialdiagnosis:Differentialdiagnosisshouldconsiderchangescol-
lectivelyreferredtoaspseudoretinitispigmentosabecausetheysimulatethe
clinicalpictureofretinitispigmentosa.Themostcommoncausesthatshould
beexcludedinthiscontextare:
Advancedretinitispigmentosa.
Fig.12.31
Typicalsignsin-
cludenarrowed
retinalvessels,
waxyyellowap-
pearanceofthe
opticdiskdueto
atrophyofthe
opticnerve,and
“bone-spicule”
proliferationof
retinalpigment
epithelium.
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363. 345
!Posttraumaticchanges.
!Postinflammatoryorpostinfectiouschanges.Thesemayincludedegener-
ativeretinalpigmentepithelialdiseasesecondarytorubellawith“saltand
pepper”fundusofpunctateareasofatrophyandproliferationofretinal
pigmentepithelium.Othercausesincludesyphilis,whichmaypresent
withplacoidlesionsofpigmentepithelialatrophyandproliferations.
!Tumors.
!Medications,suchaschloroquine,Myambutol(ethambutol),andthiori-
dazine.
Treatment:Thecausesofthedisordercannotbetreated.Edge-filteredeye-
glasses(eyeglasseswithorangeorbluecoloredlensesthatfilteroutcertain
wavelengths)andmagnifyingnearvisionaidscanhelpmakebetteruseofthe
patient’sremainingvision.
Prophylaxis:Noprophylaxisispossible.
Clinicalcourseandprognosis:Retinitispigmentosaischronicallyprogress-
ive.Theclinicalcoursedependsonthespecificformofthedisorder;severe
formsleadtoblindness.
12.6ToxicRetinopathy
Definition
Retinalchangesresultingfromuseofmedications.
Epidemiology:Toxicretinopathyisrare.
Pathogenesis:Toxicretinopathycanremainasymptomaticforalongtime.
Lossofvisualacuityoccursifthemaculaisaffected.
Chloroquineindosesexceeding250gcausesretinaldamage.Macular
edemacanoccurinitially.Later,punctatepigmentepithelialchanges
develop,whichmayprogresstobull’seyemaculopathywithconcentricrings
ofhypopigmentationandhyperpigmentationinthemacularregion
(Fig.12.32).Thesefindingsareusuallybilateralandsymmetrical.Othertoxic
retinalchangesarelistedintheappendix.
Diagnosticconsiderations:Thediagnosisismadebybinocularophthalmos-
copywiththepupildilatedandconfirmedbyelectrophysiologicstudiesthat
includeanelectroretinogram,electro-oculogram,andvisualevokedpoten-
tials(seeFig.12.2a).
Differentialdiagnosis:Retinalpigmentepitheliumorretinalbleedingcan
resultfrommanyotherretinaldisorders,andmayalsobeassociatedwiththe
underlyingdiseaseforwhichthemedicationwasprescribed.
12.6ToxicRetinopathy
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364. 346
Chloroquinetoxicity(bull’seyemaculopathy).
Fig.12.32
Chronicuseof
thismedication
causesconcentric
ringsofatrophy
andproliferation
ofretinalpig-
mentepithelium
(arrows).
Treatment:Themedicationshouldbediscontinuedifpossible.
Prophylaxis:Regularophthalmologicfollow-upexaminationsareindicated
beforeandduringtreatmentthatinvolvesmedicationswithknownocular
sideeffects.
Clinicalcourseandprognosis:Theclinicalcoursedependsonthespecific
medicationanddose.Findingsmayimproveafterthemedicationisdiscon-
tinued.However,withchloroquineinparticular,findingsmaycontinueto
worsenevenyearslater.
12.7RetinalInflammatoryDisease
12.7.1RetinalVasculitis
Definition
Retinalvasculitisisaninflammationoftheretinalvasculature.Typicalfindings
includecellsinthevitreousbody.
Epidemiology:Retinalvasculitisisoneofthemorefrequentclinicalsyn-
dromes.
Etiology:Thecauseofretinalvasculitisoftenremainsobscure.Itcanbe
causedbyapathogenoroccurinassociationwithimmunologicprocesses
(Table12.8).
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365. 347
Table12.8Themostimportantcausesofretinalvasculitis
!Idiopathic
!Eales’disease
!Behçet’sdisease
!Multiplesclerosis
!Lupuserythematosus
!Wegener’sgranulomatosis
!Polyarteritisnodosa
!Horton’sarteritis
!Sarcoidosis
!Tuberculosis
!Borreliosis(Lymedisease)
!Listeriosis
!Brucellosis
!Syphilis
!Viruses
Symptoms:Patientsreportlossofvisualacuityorblackdotsintheirvisual
field.Theseareduetothepresenceofcellsinthevitreousbody.
Diagnosticconsiderations:Theophthalmologicdiagnosticwork-up
includesclinicalexamination,ophthalmoscopy,andslit-lampexamination.
Theslit-lampexaminationwillrevealcellsinthevitreousbody.Ophthalmo-
scopicfindingswillincludewhitishpreretinalinfiltrates(Fig.12.33),vascular
constriction(usuallyinvolvingtheveins),vascularocclusion,intraretinal
bleeding,andretinaledema.Fluoresceinangiographymaybeusedtoeval-
uatethepresenceandactivityofneovascularization.Underlyingsystemic
disease,immunologicprocesses,andinfections(seeTable12.8)mustbe
excluded.
Differentialdiagnosis:Othervasculardiseasesoftheretinasuchasvein
occlusionshouldbeexcluded.Thesevasculardiseasesmaybedistinguished
fromvascularretinitisbytheabsenceofcellsinthevitreousbody.
Retinalvasculitis.
Fig.12.33
Ophthalmoscopy
revealswhitish
preretinalvit-
reousinfiltrates
(arrow).
12.7RetinalInflammatoryDisease
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366. 348
Treatment:Thecausesofknownunderlyingdisordersshouldbetreated.
Symptomsaretreatedwithtopicalsteroidsandsystemicsteroidsinthe
absenceofcontraindications.Neovascularizationistreatedwithlaserther-
apy.
Prophylaxis:Noprophylaxisispossibleexceptforpossibletreatmentofan
underlyingdisorder.
Clinicalcourseandprognosis:Vascularocclusioncanresultinneovasculari-
zationthatmayleadtovitreoushemorrhage.Tractionalretinaldetachmentis
anotherpossiblecomplication.
12.7.2PosteriorUveitisDuetoToxoplasmosis
Definition
Focalchorioretinalinflammationcausedbyinfection.
Epidemiology:Thisclinicalsyndromeisencounteredfrequently.
Pathogenesis:Thepathogen,Toxoplasmagondii,istransmittedbyingestion
oftissuecystsinraworundercookedmeatorbyoocystsfromcatfeces.Incon-
genitaltoxoplasmosis,thechildacquiresthepathogenthroughtransplacen-
taltransmission.
Symptomsanddiagnosticconsiderations:Asageneralrule,anegative
complement-fixationtestdoesnotexcludeToxoplasmainfectionwhereclas-
sicclinicalsymptomsarepresent.Bothformsofthedisorderpresentwith
characteristicgrayishwhitechorioretinalfocallesionssurroundedbyvitreous
infiltrationandassociatedvasculitis(Fig.12.34).Incongenitaltoxoplasmosis,
theaffectedchildrenhaveamacularscarthatsignificantlyimpairsvisualacu-
ity.Thisoftenleadstosecondarystrabismus.Intracerebralinvolvementcan
alsoresultinhydrocephalusandintracranialcalcifications.Intheacquired
form,visualacuityisimpairedonlywherethemaculaisinvolved.Thisis
rarelythecase.
Congenitaltoxoplasmosisresultsinamacularscarthatsignificantly
impairsvisualacuity.
Differentialdiagnosis:Chorioretinitiswithtuberculosis,sarcoidosis,bor-
reliosis(Lymedisease),orsyphilisshouldbeexcludedbyserologicstudies.
Treatment:Thetreatmentofchoiceconsistsofacombinationof
pyrimethamine,sulfonamide,folinicacid,andsteroidsintheirrespective
standarddoses.
Prophylaxis:Avoidrawmeatandcatfeces.
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367. 349
Recurrenttoxoplasmosis.
Fig.12.34
Acutegrayish
whitechorioreti-
nalfocallesion
(arrow)and
brownishwhite
chorioretinal
scars(arrow-
head).Lesions
usuallyrecurat
themarginofthe
originalscar,the
”motherspot”.
Clinicalcourseandprognosis:Posterioruveitisduetotoxoplasmosisusu-
allyhealswithoutseverelossofvisualacuitywherethemaculaisnot
involved.However,itcanrecuratanytime.Thereisnocureforthecongenital
form.
12.7.3AIDS-RelatedRetinalDisorders
Definition
RetinaldisordersinAIDSinvolveeitherAIDS-associatedmicroangiopathyor
infection.
Epidemiology:Upto80%ofallAIDSpatientshaveretinaldisordersasa
resultofthedisease.Otherocularinvolvementisrare.
Pathogenesis:Thepathogenesisofmicroangiopathyisstillunclear.Oppor-
tunisticinfectionsarefrequentlycausedbyviruses.
Symptoms:Microangiopathyisusuallyasymptomatic.Patientswithinfec-
tiousretinaldisordersreportlossofvisualacuityandvisualfielddefects.
Diagnosticconsiderations:OphthalmoscopicfindingsinAIDS-associated
microangiopathyincludehemorrhages,microaneurysms,telangiectasia,
andcotton-woolspots.DirectinvolvementofvascularendothelialcellsinHIV
infectionorimmune-complex-mediateddamagetoendothelialcellsand
vascularstructuresisthoughttoplayarole.
12.7RetinalInflammatoryDisease
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368. 350
Cytomegalovirusretinitisoccursin20–40%ofolderpatients.Peripheral
retinalnecrosisandintraretinalbleeding(Fig.12.35)arefrequentlyobserved.
Vascularocclusionisrare.Secondaryrhegmatogenousretinaldetachment
maydevelop.Theselesionshealtoproducefinegranularpigmentepithelial
scars.
Lessfrequently,AIDSmayinvolveretinalinfectioncausedbyherpessim-
plexandvaricella-zosterviruses,Toxoplasmagondii,orPneumocystis
carinii.ThediagnosisofaviralretinalinfectioninAIDSisconfirmedby
attemptingtoobtainpositiveserumculturesandbyresistancetesting.
Differentialdiagnosis:Inflammatoryretinalchangesduetoothercauses
shouldbeexcludedbyserologicstudies.
Treatment:Microangiopathydoesnotrequiretreatment.Viralretinitisis
treatedwithganciclovirorfoscarnet.Herpessimplexandvaricella-zoster
virusesaretreatedwithacyclovir.
Prophylaxis:Ophthalmologicscreeningexaminationsareindicatedinthe
presenceofknownviralinfection.
Clinicalcourseandprognosis:Theprognosisformicroangiopathyisvery
good.Infectiousretinitiswillleadtoblindnessifleftuntreated.Visualacuity
canoftenbepreservedifapromptdiagnosisismade.
Cytomegalovirusretinitis.
Fig.12.35
Typicalsignsin-
cludeextensive
whiteareasof
retinalnecrosis
andhemor-
rhages.
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369. 351
12.7.4ViralRetinitis
Definition
Retinaldisordercausedbyviralinfection.
Epidemiology:Viralretinitisisararedisorder
Pathogenesis:Infectionoftheretinaandretinalvasculaturecausedby
cytomegalovirus,herpessimplex,varicella-zoster,orrubellaviruses.Viral
retinitisfrequentlyoccursinimmunocompromisedpatients.
Symptoms:Patientsreportlossofvisualacuityandvisualfielddefects.
Diagnosticconsiderations:Slit-lampexaminationwillrevealcellsinthevit-
reousbody.Ophthalmoscopicfindingswillincluderetinalnecrosiswith
intraretinalbleeding(seeFig.12.35).Necrosiscanoccurasacutelesionsand
spreadovertheentireretinalikeagrassfirewithinafewdays.Whenthereti-
nitisheals,itleavesbehindwide-areascarring.
Duringpregnancy,rubellaviruscancauseembryopathyinthechild.Oph-
thalmicexaminationwillrevealtypicalfinegranularpigmentepithelialscars
onthefundusthatareoftenassociatedwithacongenitalcataract.Thediag-
nosisisconfirmedbymeasuringtheserumvirustiter.Thepossibilityofcom-
promisedimmunocompetenceshouldbeverifiedorexcluded.
Differentialdiagnosis:Posterioruveitisandvasculitisshouldbeexcluded.
Thesedisordersmaybedistinguishedfromviralretinitisbytheabsenceof
necrosis.
Treatment:Thedisorderistreatedwithhighdosesofanantiviralagent(acy-
clovir,ganciclovir,orfoscarnet)accordingtothespecificpathogen.
Prophylaxis:Ophthalmologicscreeningexaminationsareindicatedin
immunocompromisedpersonswithsuspectedviralinfection.
Clinicalcourseandprognosis:Viralretinitiscanbearrestedifdiagnosed
early.However,recurrencesarefrequentinimmunocompromisedpatients.
Blindnessusuallycannotbepreventedinretinalnecrosissyndrome.
12.7.5RetinitisinLymeDisease
Definition
InflammationoftheretinausuallycausedbyBorreliaburgdorferi.
Epidemiology:Theincidenceofthisretinaldisorderhasincreasedinrecent
years.
12.7RetinalInflammatoryDisease
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370. 352
Etiology:Theinflammationiscausedbyspirochetesusuallytransmittedby
bitesfrominfectedticks.
Findingsandsymptoms:Lymediseasecanleadtomanyinflammatoryocu-
larchangeswiththeirrespectivesymptoms.Theseincludeconjunctivitis,
keratitis,andiridocyclitis.Retinalvasculitis,retinalarteryocclusion,neu-
roretinitis,opticneuritis,andchoroiditishavealsobeendescribed.
Lymediseaseshouldbeexcludedasapossiblecauseofposterioruveitis
ofuncertainetiology.
Diagnosticconsiderations:Thediagnosisismadebyophthalmoscopyand
serologicstudiestoidentifythepathogen.
Differentialdiagnosis:Inflammatoryocularchangesduetoothercauses
(suchastoxoplasmosisortuberculosis)shouldbeexcluded.
Treatment:Antibiotictreatmentwithtetracycline,penicillinG,orthird-
generationcephalosporinsisindicated.
Clinicalcourseandprognosis:RetinalchangesduetoLymediseasetendto
recur.
12.7.6ParasiticRetinalDisorders
Definition
Inflammationoftheretinacausedbyinfectionwithparasitessuchas
Onchocercavolvulus(thepathogenthatcausesonchocerciasis),Toxocaracanis
orToxocaracati(nematodelarvaethatarenormallyintestinalparasitesofdogs
andcats),Taeniasolium,(porktapeworm),andotherparasites.
Epidemiology:Onchocerciasis,liketrachomaandleprosy,isoneofthemost
frequentcausesofblindnessworldwide.However,liketheotherparasiticdis-
easesdiscussedhere,itisrareinEuropeandNorthAmerica.
Etiology:Onchocercavolvulusistransmittedbythebiteofblackflies.This
allowsthelarvae(microfilaria)topenetratetheskin,wheretheyformfibrous
subcutaneousnodules.Theretheyreachmaturityandproduceothermicro-
filaria,whichmigrateintosurroundingtissue.Thedangerofocularinfiltra-
tionisparticularlygreatwheretherearefibrousnodulesclosetotheeye.
ToxocaracanisorToxocaracati(eggsofnematodesinfestingdogsand
cats)aretransmittedtohumansbyingestionofsubstancescontaminated
withthefecesoftheseanimals.Theeggshatchinthegastrointestinaltract,
wheretheygainaccesstothecirculatorysystemandmaybespreadthrough-
outtheentirebody.Thechoroidcanbecomeinfestedinthismanner.
12Retina
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371. 353
Taeniasolium:Theporktapeworminfestationcanoccurfromeatingpork
contaminatedwithlarvaeorothersubstancescontaminatedwithtapeworm
eggs.Maturetapewormscanalsoreleaseeggsintotheintestine.Thelarvae
travelthroughthebloodstreamtovariousorgansandcanalsoinfesttheeye.
Diagnosticconsiderationsandfindings:Ophthalmoscopywillreveal
intraocularinflammation.Onchocerciasishasbeenknowntobeassociated
withposterioruveitisaswellaskeratitisandiritis.Histologicexamination
willdemonstratemicrofilariaintheretina.Viscerallarvamigrans,Toxocara
canis,orToxocaracaticancausecomplicationsinvolvingendophthalmitis
andretinaldetachment.Subretinalgranulomasandlarvalinflammationof
theretinahavebeenknowntooccur.Thelarvaeofdifferentspeciesofworms
canproducediffuseunilateralsubacuteneuroretinitiswiththetypicalclini-
calpictureofgrayishwhiteintraretinalandsubretinalfocallesions.Flylarvae
canalsoinvadethesubretinalspaceinophthalmomyiasis.
Differentialdiagnosis:Othercausesofretinalinflammationandsubretinal
granulomasshouldbeexcluded.
Treatment:Laserphotocoagulationorsurgicalremovalofthewormlarvae
maybeindicated.
Clinicalcourseandprognosis:Itisnotuncommonforthesedisordersto
leadtoblindness.
12.8RetinalTumorsandHamartomas
12.8.1Retinoblastoma
Definition
Aretinoblastomaisamalignanttumorofearlychildhoodthatdevelopsfrom
immatureretinalcells.
Epidemiology:Retinoblastomaisthemostcommonmalignantoculartumor
inchildren,occurringinapproximatelyoneof20000births.In30%ofall
cases,itisbilateral.
Pathogenesis:Asomaticmutationisdetectedinabout95%ofallpatients.In
theotherpatients,itisinheritedasanautosomaldominanttrait.Changeson
chromosome13qhavebeenobservedingerm-cellmutations.Retinoblas-
tomasmaythenoccuratseverallocationsintheretinaorbilaterally.
Whereretinoblastomaisinheritedasanautosomaldominanttrait,the
siblingsoftheaffectedchildshouldberegularlyexaminedbyanoph-
thalmologist.
12.8RetinalTumorsandHamartomas
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372. 354
Symptoms:Retinoblastomamanifestsitselfbeforetheageofthreein90%of
affectedchildren.Parentsobserveleukocoria(awhitishyellowpupil;
Fig.12.36)in60%ofthesechildren,strabismusin20%,andareddenedeyein
10%.
Everychildpresentingwithstrabismusshouldundergoexaminationof
thefunduswiththepupildilatedtoexcludearetinoblastoma.
Findingsanddiagnosticconsiderations:Agrayishwhite,vascularizedreti-
naltumorwillbeobservedonophthalmoscopy.Initsadvancedstages,this
tumorwasformerlyreferredtoasanamauroticcat'seye.Infiltrationofthe
vitreousbody,anteriorchamber(pseudohypopyon),andorbitmayoccur.A
retinoblastomathatalsoinvolvesthefelloweyeandpinealbodyisreferredto
asatrilateralretinoblastoma.
Atrilateralretinoblastomaisdefinedasadditionalmanifestationofthe
tumorinthepinealbody.
Calcificationsfrequentlyoccurinthesetumors.RadiographsorCTimages
thatshowcalcificationscanthereforehelptoconfirmthediagnosisinuncer-
taincases.
Differentialdiagnosis:Severalotherdisordersshouldbeexcludedbyoph-
thalmoscopy.Theseinclude:
!Cataract(withleukocoria).
!Primarystrabismus(withstrabismus).
!Infection(withareddenedeye).
Leukocoriainthelefteyeduetoaretinoblastoma.
Fig.12.36
Thewhitish
gleamofthe
pupiloftheleft
eyeisatypical
findinginretino-
blastoma.
12Retina
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373. 355
Retinaldetachment,persistenthyperplasticprimaryvitreous(PHPV),and
Coats’diseaseshouldalsobeexcluded.
Treatment:Tumorslessthanfourpupildiametersmaybemanagedwith
radiationtherapydeliveredbyplaquesofradioactiverutheniumoriodine
(brachytherapy)andcryotherapy.Largertumorsrequireenucleationofthe
eye.
Prophylaxis:Followingthediagnosis,thefelloweyeshouldbeexamined
withthepupildilatedeverythreemonthsforfiveyears.Afterthat,follow-up
examinationsmaybeperformedatgreaterintervals.
Clinicalcourseandprognosis:Leftuntreated,aretinoblastomawilleventu-
allymetastasizetothebrainandcausedeath.Patientsfrequentlydevelopa
secondmalignanttumorsuchasanosteosarcoma.
12.8.2Astrocytoma
Definition
Anastrocytomaorastrocytichamartomaisabenigntumorthatdevelopsfrom
theastrocytesoftheneuroglialtissue.
Epidemiology:Astrocytomasarerare.
Etiology:Astrocytomasbelongtothephakomatosesandarepresumably
congenitaldisordersthatdevelopfromthelayerofopticnervefibers.They
maymanifestthemselvesaspurelyoculardisordersorinassociationwith
tuberoussclerosis(Bourneville’sdisease).
Symptoms:Patientsusuallyhavenoocularsymptoms.Calcifyingastrocytic
hamartomasintheregionofthebasalgangliaorventriclescancauseepilepsy
andmentaldeficiency.AnastrocytomainBourneville’sdiseasewillbe
associatedtypicallywithanadenomasebaceuminthefacialskin.
Findingsanddiagnosticconsiderations:Astrocytomasareeitherincidental
findingsinophthalmicexaminationsperformedforotherreasons,ortheyare
diagnosedinpatientspresentingwithreducedvisualacuity.Ophthalmos-
copywillrevealsingleormultiple“mulberry”tumorsonetotwopupildiame-
tersinsize.Thesewillappearwhiteandareoftencalcified.Thetumorsare
inherentlyfluorescentwhenobservedinbluelightinfluoresceinangiogra-
phywithabluefilter.
Differentialdiagnosis:Aretinoblastomashouldbeexcludedinchildren.
Thatisusuallylargerthananastrocytomaonophthalmoscopy.Apossible
Toxocaracanisgranulomashouldbeconfirmedorexcludedbyserologic
studies.
12.8RetinalTumorsandHamartomas
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374. 356
Treatment:Noophthalmologictreatmentisrequired.Thepatientshouldbe
referredtoaneurologisttoexcludecerebralinvolvement.
Clinicalcourseandprognosis:Thesetumorsrarelyincreaseinsize.
12.8.3Hemangiomas
Definition
Capillaryhemangiomasorhemangioblastomasoccurinangiomatosisretinae
(vonHippel-Lindaudisease).
12Retina
VonHippel-Lindaudisease.
Fig.12.37
aAhemangio-
blastoma(arrow)
invonHippel-Lin-
daudiseasewith
enlargedretinal
arteriesandveins
andretinal
detachmentwith
hardexudate(ar-
rowhead).
bCorrespondingfluoresceinangio-
gram.
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375. 35712.8RetinalTumorsandHamartomas
Epidemiology:Hemangiomasarerare.
Etiology:Thesearebenigncongenitalchanges.Theremaybeanautosomal
dominantinheritance.
Symptoms:Lossofvisualacuitywillresultwhereexudativeretinaldetach-
mentdevelops.
Findingsanddiagnosticconsiderations:Retinalhemangiomasarecharac-
terizedbythickenedtortuousarteriesandveins(Figs.12.37aandb).Bilateral
changesarepresentin50%ofallpatients.
Differentialdiagnosis:Coats’disease,branchingretinalhemangiomasin
Wyburn-Masonsyndrome,andcavernoushemangiomasshouldbecon-
sidered.Cerebralhemangiomas,renalcysts,hypernephromas,andpheochro-
mocytomasshouldalsobeexcluded.
Treatment:Retinalhemangiomasmaybetreatedbylaserorcryocautery
therapy.However,exudativeretinaldetachmentwilldevelopasthetreat-
mentincreasesthisrisk.
Clinicalcourseandprognosis:Thedisorderisgraduallyprogressive.The
prognosisforvisualacuityispoorinthedisorderwhereretinaldetachment
develops.
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377. 359
13OpticNerve
OskarGareisandGerhardK.Lang
13.1BasicKnowledge
Theopticnerveextendsfromtheposteriorpoleoftheeyetotheopticchiasm
(Fig.13.1).Afterthischaracteristiccrossing,thefibersoftheopticnervetravel
astheoptictracttothelateralgeniculatebody.Dependingontheshapeofthe
skull,theopticnervehasatotallengthof35–55mm.Thenerveconsistsof:
!Anintraocularportion.
!Anintraorbitalportion.
!Anintracranialportion.
Pathoftheopticnerve.
Globe
Optic nerve
Optic canal
Optic chiasm
Fig.13.1CT
imageshowing
theintraorbital
andintracranial
portionsofthe
opticnerve.
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378. 360
13.1.1IntraocularPortionoftheOpticNerve
Theintraocularportionoftheopticnerveisvisibleonophthalmoscopyasthe
opticdisk.Alltheretinalnervefibersmergeintotheopticnervehere,andthe
centralretinalvesselsenterandleavetheeyehere.Thecompleteabsenceof
photoreceptorsatthissitecreatesagapinthevisualfieldknownastheblind
spot.
Shapeandsize:Theopticdisk(Fig.13.2)isnormallyslightlyverticallyoval
withanaverageareaofapproximately2.7mm
2
andahorizontaldiameterof
approximately1.8mm.Thereisawiderangeofphysiologicvariabilityinthe
sizeoftheopticdisk;itsareamayvarybyafactorofseven,anditshorizontal
diameterbyafactoroftwoandone-half.
Color:Thenormalphysiologiccolorisyellowishorange.Thetemporalhalfof
theopticdiskisusuallyslightlypaler.
Margin:Themarginoftheopticdiskissharplydefinedandreadilydistin-
guishedfromthesurroundingretinaltissue.Onthenasalside,thegreater
densityofthenervefibersmakesthemarginslightlylessdistinctthanonthe
temporalside.Acommonclinicalobservationisacrescentofpigmentor
irregularpigmentationclosetotheopticdiskonthetemporalside;some-
timesthesclerawillbevisiblethroughthiscrescent.
Prominenceoftheopticdisk:Thenormalopticdiskisnotprominent.The
nervefibersarepracticallyflushwiththeretina.
Normalopticdisk.
Cilioretinal
vessel
Vein
Artery
Optic cupNeuroretinal
rim
Fig.13.2Typicalsignsofanormalpupilincludeayellowishorangeneuroretinal
rimsharplysetofffromtheretina.
13OpticNerve
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379. 361
Neuroretinalrim(Fig.13.2):Thisconsistsofthebundlesofalltheopticnerve
fibersastheyexitthroughthescleralcanal.Therimhasacharacteristiccon-
figuration:Thenarrowestportionisinthetemporalhorizontalregionfol-
lowedbythenasalhorizontalarea;thewidestareasaretheverticalinferior
andsuperiorareas.
Opticcup:Thisistheslightlyeccentriccavitationoftheopticnervethathasa
slightlyflattenedovalshapecorrespondingtothatoftheneuroretinalrim.It
isthebrightestpartoftheopticdisk.Nonervefibersexitfromit(Fig.13.2).
Thesizeoftheopticcupcorrelateswiththesizeoftheopticdisk;thelarger
theopticdisk,thelargertheopticcup.Becauseenlargementoftheopticcup
meansalossofnervefibersintherim,itisparticularlyimportanttodocument
thesizeoftheopticcup.Thisisspecifiedasthehorizontalandverticalratiosof
cuptodiskdiameter(cup/diskratio).Duetothewiderangeofvariabilityin
opticdisksize,itisnotpossibletospecifyabsolutecup/diskratiosthatindi-
catethepresenceofabnormalprocesses.
Centralretinalarteryandvein:Thesestructuresusuallyentertheeye
slightlynasaltothecenteroftheopticdisk.Visiblepulsationintheveinis
normal.However,arterialpulsationisalwaysabnormalandoccurswithdis-
orderssuchasincreasedintraocularpressureandaorticstenosis.
Cilioretinalvesselsareaberrantvesselsoriginatingdirectlyfromthechoroid
(shortposteriorciliaryarteries).Resemblingacane,theyusuallycoursealong
thetemporalmarginoftheopticdiskandsupplytheinnerlayersoftheretina
(Fig.13.2).
Bloodsupplytotheopticdisk(Fig.13.3):Theopticdiskreceivesitsblood
supplyfromtheringofZinn,ananastomoticringofsmallbranchesofthe
shortposteriorciliaryarteriesandthecentralretinalartery.Bothgroupsof
vesselsoriginatefromtheophthalmicartery,whichbranchesoffoftheinter-
nalcarotidarteryandenterstheeyethroughtheopticcanal.Thecentralreti-
nalarteryandveinbranchintotheopticnerveapproximately8mmbefore
thepointatwhichtheopticnerveexitstheglobe.Approximately10short
posteriorciliaryarteriespenetratethescleraaroundtheopticnerve.
13.1.2TheIntraorbitalandIntracranialPortionoftheOpticNerve
Theintraorbitalportionbeginsafterthenervepassesthroughasieve-like
plateofscleralconnectivetissue,thelaminacribrosa.Insidetheorbit,theoptic
nervedescribesanS-shapedcoursethatallowsextremeeyemovements.
Aftertheopticnervepassesthroughtheopticcanal,theshortintracranial
portionbeginsandextendsasfarastheopticchiasm.Likethebrain,the
intraorbitalandintracranialportionsoftheopticnervearesurroundedby
sheathsofduramater,pia,andarachnoid(seeFig.13.3).Thenervereceivesits
bloodsupplythroughthevascularpiasheath.
13.1BasicKnowledge
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380. 362
Vascularstructuressupplyingtheheadoftheopticnerve.
Dura mater sheath
Arachnoid sheath
Pigment epithelium
Retina
Choroid
Sclera
Lamina cribrosa
Pia mater sheath
Posterior ciliary artery
Ring of Zinn
Vascular plexus
of the pia sheath
Central retinal vein
Central retinal artery
Short posterior
ciliary arteries
Fig.13.3Theopticnerveissuppliedwithbloodfromboththeshortposteriorcili-
aryarteriesandthecentralretinalartery.
13.2ExaminationMethods
Theseinclude:
!Ophthalmoscopy(seeChapter1).
!Visualacuitytesting(seeChapter1).
!Perimetrytest(seeChapter14).
!Pupillarylightreflex(seeChapter9).
!Testingcolorvision(forexamplewiththepanelD15test).
!Visualevokedpotential(VEP).
PanelD15testofcolorvision:Thisisacolormarkersortingtest.Thepatient
ispresentedwith15smallcolormarkersthatheorshemustselectandsort
accordingtoafixedbluecolormarker.Patientswithcolorvisiondefectswill
typicallyconfusecertainmarkerswithinthecolorseries.Thespecificcolor
visiondefectcanbediagnosedfromthesemistakes.
13OpticNerve
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381. 363
VisualEvokedPotential(VEP):TheVEPmayberegardedasanisolated
occipitalEEG.Theelectricalresponsesinthebraintoopticalstimuliaretrans-
mittedbyelectrodesplacedovertheoccipitallobe.Measurementsinclude
thespeedofconduction(i.e.,latency;normalvaluesrangebetween90and110
ms)andthevoltagedifferentialbetweentheoccipitallobeandskinelectrodes
(i.e.,amplitude;normalvaluesdependonthelaboratorysetting).Themost
importantindicationforVEPtestingisretrobulbaropticneuritistodemon-
strateanextendedlatencyperiodindemyelinization,suchasindiffuse
encephalitis.
13.3DisordersthatObscuretheMarginoftheOpticDisk
13.3.1CongenitalDisordersthatObscuretheMarginoftheOptic
Disk
Therearenormalvariantsoftheopticdiskinwhichthemarginappearsfully
orpartiallyblurred.Careshouldbetakentodistinguishthemfromabnormal
findings.
13.3.1.1ObliqueEntryoftheOpticNerve
Wheretheopticnerveexitstheeyeinanobliqueandnasaldirection
(Fig.13.4),thenervefibersonthenasalcircumferencewillbeelevated.The
tightlycompressednasalnervefiberswillobscurethemarginoftheopticdisk.
Accordingly,temporalnervefibersarestretched,andtheneuroretinalrimcan-
notbeclearlydistinguished.Oftenanadjacentcrescenticwhitisharea,
Obliqueentryoftheopticnerve.
Fig.13.4Tightly
compressednasal
nervefibers
causeslight
elevationofthe
opticdisk,and
themarginofthe
diskisobscured.
13.3DisordersthatObscuretheMarginoftheOpticDisk
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382. 364
knownasatemporalcrescent,willbeobservedonthetemporalside.This
crescentisfrequentlyseeninmyopiaandisreferredtoasamyopiccrescent.
Itcanalsobecircular.
13.3.1.2TiltedDisk
Anopticnervethatexitstheeyesuperiorly(Fig.13.5)isreferredtoasatilted
disk.Thesuperiorcircumferenceofthemarginoftheopticdiskwillbeobscured
inamannersimilartoobliqueentryoftheopticnerve.Anumberofother
changesmayalsobeobserved,includinganinferiorcrescent,situsinversusof
theretinalvessels,ectasiaofthefundus,myopia,andvisualfielddefects.
Thesefindingsmayoccurinvariouscombinationsandarereferredtocollec-
tivelyastilted-disksyndrome.Thisisclinicallyhighlysignificantasnasal
inferiorectasiaofthefunduscanproducetemporalsuperiorvisualfield
defects.Wherethesefindingsarebilateral,careshouldbetakentodistin-
guishthemfrompituitarytumors.Thisclinicalpictureisregardedasaform
ofrudimentarycoloboma.
13.3.1.3Pseudopapilledema
Pseudopapilledema(Fig.13.6)isduetoanarrowscleralcanal.Becauseofthe
constriction,thenervefibersaretightlycompressed.Theopticdiskisele-
vatedandthefullcircleofthemarginobscured.Theopticcupisabsent,and
theretinalvesselsappeartortuous.Therearenoabnormalmorphologic
changessuchasbleeding,nervefiberedema,andhyperemia;visualacuity
andvisualfieldarenormal.Pseudopapilledemacanoccurwithhyperopia,
Tilteddisk.
Fig.13.5
Obliqueentryof
theopticnerve
superiorlywithan
inferiorcrescent
andinferiorseg-
mentalectasiaof
thefundus.
13OpticNerve
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383. 365
Pseudopapilledema.
Fig.13.6Circu-
larblurringofthe
marginofthe
opticdiskwith
absenceofthe
opticcup.
althoughitisencounteredequallyfrequentlyinemmetropicorslightly
myopiceyes.
Differentialdiagnosis:opticdiskedema,opticdiskdrusen(seeTable13.1).
13.3.1.4MyelinatedNerveFibers
Normallyretinalnervefibersarenotmyelinated.However,myelinatedareas
occasionallyoccurintheretina(Fig.13.7).Theyoccurmostfrequentlyatthe
Myelinatednervefibers.
Fig.13.7Be-
causetheyare
myelinated,the
nervefibersap-
pearwhitishand
striatedandcan
simulateseg-
mentalblurring
ofthemargin.
13.3DisordersthatObscuretheMarginoftheOpticDisk
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384. 366
marginoftheopticdisk.Whitishandstriated,theysimulatesegmentalor
circularblurringofthemargin.Myelinatednervefiberscanalsooccuronthe
peripheryoftheretina.Becauseoftheirlocationintheinnermostlayerofthe
retina,theytendtoobscuretheretinalvessels.Myelinatednervefibersnor-
mallycausenolossoffunction.Onlyextensivefindingscanleadtosmallsco-
tomas.
13.3.1.5Bergmeister’sPapilla
Thefetalhyaloidarteryemergesfromtheopticdisktosupplythevitreous
bodyandlens.Glialandfibroustissuemaypersistifthestructureisnotfully
absorbed.Thisvestigialtissue,usuallyonthenasalsideoftheopticdisk,is
knownasBergmeister’spapilla.Whenthistissuetakestheformofveil-like
membraneoverlyingthesurfaceoftheopticdisk,itisalsoreferredtoasan
epipapillarymembrane(Fig.13.8).Usuallythisconditionisasymptomatic.
13.3.1.6OpticDiskDrusen
Drusenareyellowishlobularbodiesinthetissueoftheopticdiskthatare
usuallybilateral(in70%ofallcases).Ophthalmoscopycanrevealsuperficial
drusenbutnotdrusenlocateddeepinthescleralcanal.Inthepresenceof
opticdiskdrusen,thediskappearsslightlyelevatedwithblurredmarginsand
withoutanopticcup(Fig.13.9).Abnormalmorphologicsignssuchashyper-
emiaandnervefiberedemawillnotbepresent.However,bleedinginlines
alongthediskmarginorsubretinalperipapillarybleedingmayoccurinrare
cases.
Bergmeister'sPapilla.
Fig.13.8Rem-
nantsofthehy-
aloidarteryform-
ingaveil-likeepi-
papillarymem-
braneoverlying
thesurfaceofthe
opticdiskare
seenonnasal
side.
13OpticNerve
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385. 367
Opticdiskdrusen.
Fig.13.9The
yellowishlobular
deposits(drusen)
maketheoptic
diskappearele-
vatedwith
blurredmargins
andwithoutan
opticcup.
Asmalllaminacribrosaappearstobeafactorintheetiologyofthedis-
order.Thisimpedesaxonalplasmaflow,whichpredisposesthepatientto
axonaldegeneration.Thisinturnproducescalcificationsexteriortotheaxons
(drusen).RetinaldrusenarehyalinedepositsinBruch’smembraneandarea
completelyunrelatedprocess.
Drusenusuallydonotcauseanylossoffunction.Deepdrusencancause
compressiveatrophyofnervefiberswithresultingsubsequentvisualfield
defects.
Opticdiskdrusenmaybediagnosedonthebasisofcharacteristicultra-
soundfindingsofhighlyreflectivepapillarydeposits.Fluoresceinangiogra-
phyfindingsofautofluorescencepriortodyeinjectionarealsocharacteristic.
SeeTable13.1fordifferentialdiagnosis.
13.3.2AcquiredDisordersthatObscuretheMarginoftheOpticDisk
Thenormalvariantsandcongenitalchangesdiscussedintheprevioussection
mustbedistinguishedfromabnormalchangestotheopticdiskduetonerve
fiberedema.Thetermopticdiskedemaisusedinagenericsensetodescribe
anysuchchange.However,thistermshouldbefurtherspecifiedwhenever
possible:
!Opticdiskedemawithoutprimaryaxonaldamage:
–Papilledema.
–Hypotensionpapilledema.
!Opticdiskedemawithdirectaxonaldamage:
–Inflammation:papillitisorretrobulbaropticneuritis.
–Infarctionwithischemicopticneuropathy(arterioscleroticorarteritic).
13.3DisordersthatObscuretheMarginoftheOpticDisk
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386. 368
!Opticdiskedemaduetoinfiltration:
–Forexampleduetoanunderlyinghematologicdisorder.
13.3.2.1Papilledema
Definition
Bilateralopticdiskedemasecondarytoincreasedintracranialpressure.
Epidemiology:Epidemiologicdatafromthe1950sdescribepapilledemain
asmanyas60%ofpatientswithbraintumors.Sincethen,advancesinneu-
roradiologyhavesignificantlyreducedtheincidenceofpapilledema.The
diagnosticimportanceofthedisorderhasdecreasedaccordingly.
Etiology:Anadequatetheorytofullyexplainthepathogenesisofpapil-
ledemaislacking.Currentthinkingcentersaroundamechanicalmodelin
whichincreasedintracranialpressureandimpededaxonalplasmaflow
throughthenarrowedlaminacribrosacausenervefiberedema.However,
thereisnodefinitecorrelationbetweenintracranialpressureandpromi-
nenceofthepapilledema.Noristhereadefinitecorrelationbetweenthe
timesatwhichthetwoprocessesoccur.However,severepapilledemacan
occurwithinafewhoursofincreasedintracranialpressure,suchasinacute
intracranialhemorrhage.Therefore,papilledemaisaconditional,unspecific
signofincreasedintracranialpressurethatdoesnotprovideconclusiveevi-
denceofthecauseorlocationofaprocess.
Inapproximately60%ofallcases,theincreasedintracranialpressurewith
papilledemaiscausedbyanintracranialtumor;40%ofallcasesaredueto
othercauses,suchashydrocephalus,meningitis,brainabscess,encephalitis,
malignanthypertension,orintracranialhemorrhages.Thepatientshouldbe
referredtoaneurologist,neurosurgeon,orinternistfordiagnosisofthe
underlyingcauses.
Everyincidenceofpapilledemarequiresimmediatediagnosisofthe
underlyingcausesasincreasedintracranialpressureisalife-threatening
situation.
Theincidenceofpapilledemainthepresenceofabraintumordecreaseswith
increasingage;inthefirstdecadeoflifeitis80%,whereasintheseventhdec-
adeitisonly40%.Papilledemacannotoccurwherethereisatrophyofthe
opticnerve,aspapilledemarequiresintactnervefiberstodevelop.
Specialforms:
!FosterKennedysyndrome:Thisreferstoisolatedatrophyoftheopticnerve
duetodirecttumorpressureononesideandpapilledemaduetoincreased
intracranialpressureontheotherside.Possiblecausesmayincludea
meningiomaofthewingofthesphenoidorfrontallobetumor.
13OpticNerve
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387. 369
!Hypotensionpapilledema:Thisreferstoanervefiberedemaduetoocular
hypotension.Possiblecausesmayincludepenetratingtraumaorfistula
secondarytointraocularsurgery.
Symptomsanddiagnosticconsiderations:Visualfunctionremainsunim-
pairedforlongtime.Thissignificantdiscrepancybetweenmorphologicand
functionalfindingsisanimportantcharacteristicindifferentialdiagnosis.
Earlyfunctionalimpairmentscanincludereversibleobscurations.Perimetry
testingmayrevealanincreaseinthesizeoftheblindspot(Fig.13.10c).Cen-
tralvisualfielddefectsandconcentricnarrowingofthevisualfieldarelate
functionalimpairmentsthatoccurwithexistingcomplexatrophyofthe
opticnerve.
Papilledemaischaracterizedbysignificantmorphologicfindingsand
onlyslightvisualimpairment.
Thefollowingphasesmaybedistinguishedbyophthalmoscopy:
Earlyphase(Fig.13.10a):Firstthenasalmarginandthenthesuperiorand
inferiormarginsoftheopticdiskareobscuredbecauseofthedifferenceinthe
relativedensitiesofthenervefibers(seeopticdisk).Theopticcupisinitially
preserved.Thisisimportantinadifferentialdiagnosistoexcludepseudo-
papilledemaandopticdiskdrusen.Theopticdiskishyperemicduetodilata-
tionofthecapillaries,andthereisnopulsationinthecentralretinalvein.
EdemacanproduceconcentricperipapillaryretinalfoldsknownasPaton’s
folds.
Acutephase(Fig.13.10b):Thisischaracterizedbyincreasingelevationofthe
opticdisk,radialhemorrhagesaroundthemarginoftheopticdiskandgray-
ishwhiteexudates.Theopticcupisoftennolongerdiscernible.Thecolorofthe
opticdiskwillberedtograyishred.
Chronicphase.Significantopticdiskedemaispresent.Theopticcupisoblit-
erated,andthehyperemiawillbeseentosubside.
Atrophicphase.Proliferationofastrocytesresultsincomplexorsecondary
atrophyoftheopticnerve.
Differentialdiagnosis:Thisincludespseudopapilledema,opticdiskdrusen
(Table13.1),abnormalitiesoftheopticdiskwithoutfunctionalimpairment,
opticdiskedemawithhypertension,andopticneuritis.
Treatment:Intracranialpressureshouldbereducedbytreatingtheunderly-
ingdisorder(seeEtiology).Onceintracranialpressurehasbeennormalized,
thepapilledemawillresolvewithinafewweeks.Usuallycomplexatrophyof
theopticnervewillremain.Theseveritywillvaryaccordingtothedurationof
thepapilledema.
13.3DisordersthatObscuretheMarginoftheOpticDisk
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388. 370
Papilledema.
Fig.13.10
aEarlyphaseof
papilledema:The
nasalmarginof
theopticdiskis
partiallyobscur-
ed.Theopticdisk
ishyperemicdue
todilatationof
thecapillaries,
andtheopticcup
isstillvisible.
bAcutestage:
Theopticdiskis
increasingelevat-
edandhasagray
tograyishred
color.Radial
hemorrhages
aroundthemar-
ginoftheoptic
diskandgrayish
whiteexudates
areobserved.The
opticdiskcanno
longerbeclearly
distinguished.
Continued!
13.3.2.2OpticNeuritis
Definition
Opticneuritisisaninflammationoftheopticnervethatmayoccurwithinthe
globe(papillitis)orposteriortoit(retrobulbaropticneuritis).
Epidemiology:Opticneuritisoccursmostfrequentlyinadultsbetweenthe
agesof20and45.Womenaremorefrequentlyaffectedthanmen.Twentyto
fortypercentofallpatientswithopticneuritisdevelopdiffuseencephalitis
(multiplesclerosis).
13OpticNerve
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389. 371
Papilledema(continued).
F
ig.
1
3
.
1
0
c
F
unctional
find-
ings.
The
enlarged
blind
spo
t
(indicat
ed
b
y
hatc
hing)
is
an
e
a
r
ly
f
u
n
c
t
io
n
a
l
c
o
r
r
e
la
t
e
t
o
ophthalmoscopic
findings.
The
blind
spo
t
is
a
n
absolut
e
sco
t
oma
(indicat
ed
b
y
cross-
hatc
hing),
meaning
that
the
patient
canno
t
discern
mark
e
r
V/4.
The
enlargement
of
the
blind
spo
t
(indicat
ed
b
y
hatc
hing)
is
a
r
elativ
e
sco
t
oma,
meaning
that
the
patient
canno
t
discern
mark
e
r
I/4.
The
mark
e
r
s
used
in
the
t
est
a
re
light
mark
e
r
s
of
v
a
r
ying
size
(indicat
ed
b
y
R
oman
numerals)
and
v
a
r
ying
light
int
ensity
(indicat
ed
b
y
Arabic
numerals
and
le
tt
er
s).
The
larger
t
he
number
,
t
he
larger
t
he
size
and
great
er
the
light
int
ensity
of
the
r
espectiv
e
mark
e
r
.
The
t
able
at
the
lo
w
e
r
r
ight
sho
ws
whic
h
mark
e
r
s
w
e
re
used
in
the
t
est.
The
t
able
at
the
lo
w
er
lef
t
sho
ws
the
v
alues
corresponding
t
o
the
numerals
and
le
tt
er
s.
13.3DisordersthatObscuretheMarginoftheOpticDisk
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390. 372
Table13.1Differentialdiagnosisofpseudopapilledema,opticdiskdrusen,andpapill-
edema
DifferentialcriterionPseudopapill-
edema
OpticdiskdrusenPapilledema
!Sizeofopticdisk Small Small Unaffected
!Opticcup Absent Absent Initiallypresent
!Spontaneous
venouspulse
PossiblypresentPossiblypresentAbsent
!Veinsandpapillary
capillaries
Normal Normal Obstructed
!ColorofopticdiskNormal Pale Hyperemic
!Peripapillarybleed-
ing
Absent Absent Present
!Peripapillarynerve
fibers
Normal Normal Edematous
!Angiography Normal Intrinsicfluores-
cence
Earlyleakage
!Ultrasound Atypical Highlyreflective
deposits
Atypical
Etiology:
Papillitis.
!Inflammatoryprocesses:TheseincludeinfectiousdiseasessuchasLyme
disease,malaria,andsyphilis,andmanifestationsintheopticnerveof
inflammationoftheorbit,paranasalsinuses,orbaseoftheskull.
!Autoimmunedisorders:Theseincludelupuserythematosus,polychon-
dritis,regionalenteritis(Crohn’sdisease),ulcerativecolitis,nodular
panarteritis,andWegener’sgranulomatosis.
!Toxicdamageduetoagentssuchasmethanol,lead,Myambutol(ethambu-
tolhydrochloride),andchloramphenicol.In70%ofthesecases,thecauseis
notdetermined.
Retrobulbaropticneuritis.Theprimarycausesofthisdisorderaredemyeli-
natingdiseasesofthecentralnervoussystemsuchasdiffuseencephalitis.In
20%ofallcases,retrobulbaropticneuritisisanisolatedearlysymptomofdif-
fuseencephalitis.However,adifferentialdiagnosisshouldalwaysalsocon-
sidertheothercausesofpapillitismentionedabove.
Symptoms:Thecardinalsymptomissuddenlossofvision,whichmayocca-
sionallybeaccompaniedbyfever(Uhthoffsymptom).Thefieldofvisionis
typicallyimpairedbyacentralscotoma(Fig.13.11b),paracentralscotomas,a
13OpticNerve
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391. 373
centrocecalscotomainvolvingthemaculaandblindspot,andwedge-shaped
visualfielddefectsuptoandincludingcompleteblindness.
Othersymptomsincludepainthatincreasesinextremepositionsofgaze
andwhenpressureisappliedtotheglobe,andreducedperceptionofcolor
intensity.
Diagnosticconsiderations:Ophthalmoscopicfindingsinpapillitis(Fig.
13.11a)includeedemaandhyperemiaoftheheadoftheopticnerve.Thisflat-
tenstheopticcupandobscuresthemarginoftheopticdisk.Bleedingatthe
marginoftheopticdiskmayormaynotbepresent.Theelevationoftheoptic
diskisconsiderablylessthaninpapilledema.
Theopticdiskwillappearnormalinretrobulbaropticneuritis.
Inretrobulbaropticneuritis,thepatientseesnothing(duetoacentral
scotoma),andthephysicianseesnothing(thefundusappearsnormal).
Otherfindingsuponexaminationincludeanafferentpupillarydefect(thisis
regularlyencountered;seeChapter9),red-greencolorvisiondefect,and
delayedlatencyinthevisualevokedpotential.
Differentialdiagnosis:
Papilledema:Initiallythereisnolossoffunction.
Ischemicopticneuropathy:Thecentralscotomaislacking,andpatientsare
usuallyovertheageof60.
Treatment:Thisdependsontheunderlyingdisorder.Retrobulbaroptic
neuritiswithseverelossofvision(lessthan0.1)maybetreatedwithhigh
dosesofsteroids,i.e.,1000mgoforalprednisolonedailyforthreedaysand
1mgoforalprednisoloneperkilogramofbodyweightondaysfourthrough
fourteen.However,thistreatmentonlyleadstomorerapidrestorationof
vision.Finalvisualacuityafteroneyearisidenticalwithorwithouthigh-dose
steroidtherapy.
Prognosis:Thisdependsontheunderlyingdisorder.Severepermanent
lossesofvisualacuityarepossible,asaresignificantspontaneousimprove-
ments.Retrobulbaropticneuritisindiffuseencephalitisusuallyexhibitsa
strongtendencytowardspontaneousimprovementwithinfourweeks
withoutanytreatment.However,discretefunctionaldefectssuchasreduced
visualcontrastandreducedperceptionofcolorintensitywillalwaysremain.
Morphologicfindingsalwaysincludeapaleopticdiskasaresultofcomplex
atrophyoftheopticnervefollowingpapillitisorpartialisolatedatrophyof
theopticnervefollowingretrobulbaropticneuritis.
13.3DisordersthatObscuretheMarginoftheOpticDisk
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392. 374
Papillitis.
Fig.13.11
aPapillitisin
Lymedisease:
Themarginof
theopticdiskis
slightlyobscured
byedemaandhy-
peremiaofthe
headoftheoptic
nerve.Theoptic
cupisobscured.
Continued!
13.3.2.3AnteriorIschemicOpticNeuropathy(AION)
Thefollowingformsofanteriorischemicopticneuropathy(AION)aredistin-
guishedaccordingtothecauseofthedisorder:
!Arterioscleroticanteriorischemicopticneuropathy.
!Arteriticanteriorischemicopticneuropathy.
ArterioscleroticAnteriorIschemicOpticNeuropathy
Definition
Anacutedisruptionofthebloodsupplytotheopticdisk,i.e.,opticdiskinfarc-
tion,resultingfromvascularchangesinarteriosclerosis.
Epidemiology:ArterioscleroticAIONisacommoncauseofsuddenlossof
visualacuity.Thegreatestincidenceofthisdisorderisbetweentheagesof60
and70.IncontrasttoarteriticAION,itcanalsooccurinadultsbelowtheage
of60.
Etiology:Thecausesofthedisorderlieinacutedisruptionofthebloodflow
throughthelateralbranchesoftheshortposteriorciliaryarteriesandthering
ofZinninthesettingofseverearteriosclerosis.Anarrowscleralcanal,i.e.,a
smallopticdisk,isapredisposingfactor.Thedisorderknownasdiabetic
papillopathyalsobelongstothisgroupofdisorders,althoughithasabetter
prognosisintermsofvision.
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393. 375
Papillitis(continued).
F
ig.
1
3
.
1
1
b
Central
sco
t
oma
in
papillitis:
A
central
sco
t
oma
is
a
typical
functional
finding
in
re
trobulbar
op
tic
neuritis
but
on
that
ma
y
also
be
obser
v
e
d
in
papillitis.
In
this
case,
a
r
elativ
e
sco
t
oma
is
present
(indicat
ed
b
y
single
hatc
hing),
i.e.,
the
patient
is
only
unable
t
o
discern
mark
e
r
s
I/1
and
w
eak
er
in
central
area
whereas
larger
mark
e
r
s
are
visible
(see
also
F
ig.
1
3
.
1
0
).
The
blind
spo
t
is
also
locat
ed
ne
xt
t
o
this
area.
13.3DisordersthatObscuretheMarginoftheOpticDisk
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394. 376
Symptoms:Patientsreportasuddenunilaterallossofvisualacuity.Thisisdue
tosegmentalorcompleteinfarctionoftheanteriorportionoftheopticnerve.
Severityisvariable.Thepatientmaypresentwithwedge-shapedvisualfield
defects(Fig.13.12b)orhorizontalvisualfielddefectsthatcorrelatewithseg-
mentalnervefiberedemas.However,severeconcentricdefectsprogressing
tototalblindnesscanalsooccur.Visionmayornotbeimpaired.Anafferent
pupillarydefectisalwayspresent.
Diagnosticconsiderations:Thepatientwillfrequentlyhaveahistoryof
hypertension,diabetesmellitus,orhyperlipidemia.
Ophthalmoscopywillrevealedemaoftheopticdisk,whosemarginwill
beaccordinglyobscured.Themarginisoftenobscuredinasegmentalpat-
tern,whichisanimportantcriterionindifferentialdiagnosis(Fig.13.12a).
Theheadoftheopticnerveisalsohyperemicwithmarginalbleeding.
Obscuredsegmentsofthemarginoftheopticdiskthatcorrelatewith
visualfielddefectsareasignofAION.
Treatment:Anteriorischemicopticneuropathyisnearlyimpossibletotreat.
Attemptedmethodsincludehemodilution(pentoxifyllineinfusions,acetyl-
salicylicacid,andbloodlettingdependingonhematocritlevels)andsystemic
administrationofsteroidstocontroltheedema.Diagnosisoftheunderlying
causeisimportant;examinationbyaninternistandDopplerultrasoundstud-
iesofthecarotidarterymaybehelpful.Underlyingdisorderssuchasdiabetes
mellitusorarterialhypertensionshouldbetreated.
Anteriorischemicopticneuropathy(AION).
Fig.13.12
aSuperiorandin-
feriorsegments
ofthemarginof
theopticdiskare
obscured(ar-
rows)dueto
edema.Thisisa
typicalmorpho-
logicsignof
AION.
Continued!
13OpticNerve
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395. 377
Anteriorischemicopticneuropathy(continued).
F
ig.
1
3
.
1
2
b
Superior
and
inf
erior
w
edge-shaped
visual
field
def
ects
correlat
e
with
obscured
segments
of
the
margin
of
the
o
p
tic
disk.
A
s
t
hese
are
absolut
e
sco
t
omas
the
y
are
indicat
ed
b
y
crosshatc
hing.
13.3DisordersthatObscuretheMarginoftheOpticDisk
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396. 378
Prognosis:Theprognosisisusuallypoorevenwheretherapyisinitiated
early.Isolatedatrophyoftheopticnervewillappearwithinthreeweeks,
complexatrophyoftheopticnerveislessfrequentbutmayalsobeobserved.
ArteriticAnteriorIschemicOpticNeuropathy
Definition
Anacutedisruptionofthebloodsupplytotheopticdiskduetoinflammation
ofmedium-sizedandsmallarterialbranches.
Epidemiology:Theannualincidenceisapproximatelythreecasesper
100000.Thedisorderoccursalmostexclusivelyaftertheageof60.Women
areaffectedslightlymoreoftenthanmen,accountingfor55%ofallcases.
Fiftypercentofallpatientssufferfromocularinvolvementwithinafewdays
uptoapproximatelythreemonthsoftheonsetofthedisorder.
Etiology:Giantcellarteritisisafrequentlybilateralgranulomatousvasculitis
thatprimarilyaffectsthemedium-sizedandsmallarteries.Commonsites
includethetemporalarteries,ophthalmicartery,shortposteriorciliaryarter-
ies,centralretinalartery,andtheproximalportionofthevertebralarteries,
whichmaybeaffectedinvaryingcombinations.
Symptoms:Patientsreportsuddenunilateralblindnessorseverevisual
impairment.Othersymptomsincludeheadaches,painfulscalpintheregion
ofthetemporalarteries,tendernesstopalpationintheregionofthetemporal
arteries,painwhilechewing(acharacteristicsign),weightloss,reduced
generalhealthandexercisetolerance.Patientsmayhaveahistoryofamauro-
sisfugaxorpolymyalgiarheumatica.
Diagnosticconsiderations:Theophthalmoscopicfindingsarethesameas
inarterioscleroticAION(seeFig.13.12a).Otherfindingsincludeasignifi-
cantlyincreasederythrocytesedimentationrate(precipitoussedimentation
isthemostimportanthematologicfinding),anincreasedlevelofC-reactive
protein,leukocytosis,andiron-deficiencyanemia.
Erythrocytesedimentationrateshouldbemeasuredineverypatient
presentingwithanteriorischemicopticneuropathy.
Thetemporalarteriesareprominent(Fig.13.13),painfultopalpation,and
havenopulse.Thediagnosisisconfirmedbyabiopsyofthetemporalartery.
Becauseofthesegmentalpatternofvascularinvolvement,negativehistologic
findingscannotexcludegiantcellarteritis.
Giantcellarteritisshouldbeconsideredineverypatientpresenting
withanteriorischemicopticneuropathy.
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397. 379
Prominenttemporalarteriesintemporalarteritis.
Fig.13.13The
prominenttem-
poralarteriesare
painfultopalpa-
tionandhaveno
pulse.
Differentialdiagnosis:ArterioscleroticAIONshouldbeconsidered.
Treatment:Immediatehigh-dosagesystemicsteroidtherapy(initialdosesup
to1000mgofintravenousprednisone)isindicated.Steroidsarereducedas
theerythrocytesedimentationratedecreases,C-reactiveproteinlevelsdrop,
andclinicalsymptomsabate.However,amaintenancedosewillberequired
forseveralmonths.Vasculartreatmentsuchaspentoxifyllineinfusionsmay
beattempted.
High-dosagesystemicsteroidtherapy(forexample250mgof
intravenousprednisone)isindicatedtoprotectthefelloweyeevenifa
giantcellarteritisisonlysuspected.
Prognosis:Theprognosisfortheaffectedeyeispoorevenwheretherapyis
initiatedearly.Immediatesteroidtherapyisabsolutelyindicatedbecausein
approximately75%ofallcasesthefelloweyeisaffectedwithinafewhours
andcerebralarteriesmayalsobeatrisk.
13.3.2.4InfiltrativeOpticDiskEdema
Infiltrationoftheopticdiskoccursinaboutoneinthreecasesofleukosisor
otherblooddyscrasias.Thisinfiltrationresultsinopticdiskedemathatis
usuallyassociatedwithinfiltrationofthemeninges.Theopticdiskedemacan
thereforeoccurfrombothdirectleukemicinfiltrationandsecondaryto
increasedpressureinthemeningesoftheopticnerve.Theprognosisforboth
visionandsurvivalispoor.
13.3DisordersthatObscuretheMarginoftheOpticDisk
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398. 380
13.4DisordersinwhichtheMarginoftheOpticDiskisWell
Defined
13.4.1AtrophyoftheOpticNerve
Definition
Irreversiblelossofaxonsintheregionofthethirdneuron(fromtheretinal
layerofganglioncellstothelateralgeniculatebody).
Morphologyandpathologicclassification:Atrophyoftheopticnerveis
classifiedaccordingtoitsmorphologyandpathogenesis.Thefollowingforms
aredistinguishedonthebasisofophthalmoscopicfindings:
!Primaryatrophyoftheopticnerve.
!Secondaryatrophyoftheopticnerve.
!Glaucomatousatrophyoftheopticnerve.
Formsofprimaryatrophyoftheopticnervemaybefurtherclassifiedaccord-
ingtotheirpathogenesis:
!Ascendingatrophyinwhichthelesionislocatedanteriortothelaminacri-
brosaintheocularportionoftheopticnerveorretina.
!Descendingatrophyinwhichthelesionislocatedposteriortothelamina
cribrosainaretrobulbarorcraniallocation.
Etiology:
Etiologyofprimaryatrophyoftheopticnerve.
Themostimportantcausesareasfollows:
!Ascendingatrophy(aftertwotofourweeks):
–Usuallyvascular,suchascentralretinalarteryocclusionoranterior
ischemicopticneuropathy.
!Descendingatrophy(afterfourtosixweeks):
–Compressive,suchasfromanorbitalorintracranialmassorhydro-
cephalus.
–Traumatic,suchasavulsion,compressionoftheopticnerveinafrac-
ture,orhematomaintheopticnervesheath.
–Inflammatory,suchasretrobulbaropticneuritis,arachnoiditisofthe
opticchiasm,orsyphilis.
!Toxic:
–Chronicabuseoflow-gradetobaccoandalcoholintobaccoandalcohol
amblyopia.
–Lead,arsenic,orthallium.
–Methylalcohol.
–Medications,suchasethambutol,chloramphenicol,gentamicin,isoni-
azid,vincristine,penicillamine,etc.
13OpticNerve
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399. 381
!Congenitalorhereditary:
–Infantilehereditaryopticatrophy(anautosomaldominantdisorder
withslowprogressivelossofvisualacuity,colorvisiondefects,and
visualfielddefects.
–Juvenilehereditaryopticatrophy(similartotheinfantileformonlythe
onsetisusuallylater,intheseconddecadeoflife).
–Leber’sopticatrophy.
–Behr’sinfantilerecessiveopticatrophy.
!Systemicdisorders:
–Hemorrhagicanemiaorperniciousanemia.
–Leukosis.
Etiologyofsecondaryatrophyoftheopticnerve.
Themostimportantcausesareasfollows:
!Papilledema.
!Anteriorischemicopticneuropathy.
!Papillitis.
Theetiologyofanyatrophyoftheopticnerveshouldbedeterminedto
excludepossiblelife-threateningintracerebralcausessuchasatumor.
Symptoms:Thespectrumoffunctionaldefectsinopticatrophyisbroad.
Theserangefromsmallperipheralvisualfielddefectsinpartialopticatrophy
tosevereconcentricvisualfielddefectsorblindnessintotalopticatrophy.
Diagnosticconsiderations:Themostimportantexaminationsareadetailed
history,ophthalmoscopy,andperimetrytesting.Colorvisiontestingand
visualevokedpotentialmaybeusefulasfollow-upexaminationsinbegin-
ningopticatrophy.
Primaryatrophyoftheopticnerve.Ophthalmoscopywillrevealawell
defined,paleopticdisk(Fig.13.14).Thepallorcancovertheentireopticdisk
(itwillappearchalkwhiteintotalopticatrophy),oritmaybepartialorseg-
mental.Theneuroretinalrimisatrophied,whichcausestheopticdisktoflat-
tenout.Thediameteroftheretinalvesselswillbedecreased.
Secondaryatrophyoftheopticnerve.Ophthalmoscopywillrevealapale
opticdisk.Thediskisslightlyelevatedduetoproliferationofastrocytes,and
themarginisblurred(Fig.13.15).Theopticcupwillbepartiallyorcompletely
obscured.Theretinalvesselswillbeconstricted.
Treatment:Thedisorderinvolvesirreversibledamagetothenervefibers.Asa
result,noeffectivetreatmentisavailable.
Prognosis:Earlyidentificationandtimelymanagementofatreatablecause
suchasatumororperniciousanemiacanarresttheprogressionofthedis-
order.Wherethisisnotthecase,theprognosisforvisionispoor.
13.4DisorderswithWell-DefinedOpticDiskMargin
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400. 382
Primaryatrophyoftheopticnerve.
Fig.13.14The
opticdiskiswell
definedandpale.
Theneuroretinal
rimisatrophied
resultinginaflat-
tenedopticdisk.
Secondaryatrophyoftheopticnerve.
Fig.13.15The
opticdiskisele-
vatedandpale
duetoprolifera-
tionofastro-
cytes.
SpecialFormsofAtrophyoftheOpticNerve
Leber’satrophy.Herethereisinvolvementofbothopticnerveswithoutaddi-
tionalneurologicsymptoms.In85%ofallcases,menbetweentheagesof20
and30areaffected.Thedisorderisduetomutationsinthemitochondrial
DNA.
Ophthalmoscopywillrevealopticdiskedemaasinpapillitisfollowedby
primaryopticnerveatrophy.Initialretrobulbaropticneuritisisalsopossible.
13OpticNerve
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401. 383
Functionalsymptomsincludealargecentralscotomawithaperipherally
limitedvisualfield.Thiswillleadtosignificantlossofvisionwithinafew
months,althoughtheremainingvisionwillnotdecreaseanyfurther.
Thereisnotreatment.
Behr’sdisease(infantilerecessiveopticatrophy).Thisisalsoadisorder
involvingbothopticnerves.However,incontrasttoLeber’satrophythereare
additionalneurologicsymptoms.Thesemayincludeataxiaandmentalretar-
dation.Thediseaseisaninheritedautosomalrecessivedisorderand
manifestsitselfinearlychildhood.
Ophthalmoscopywillrevealprogressiveopticatrophywithseverelossof
visualacuitybutwithoutcompleteblindness.
Thereisnotreatment.
Waxypalloropticatrophy.Thisdisorder(Fig.13.16)isassociatedwithtape-
toretinaldegeneration,suchasretinitispigmentosa.
Ophthalmoscopywillrevealanopticdiskwithawax-likepallorthatisshal-
lowwithawelldefinedmargin.Therewillbeseverethinningofthecentral
retinalvessels.Thecauseofthewax-likeyellowcolorisnotknown.
Thereisnotreatment.
13.4.2OpticNervePits
Anopticnervepit(Fig.13.17)ischaracterizedbyaroundorovalgrayish
depressioninthepapillarytissuethatdoesnotcompromisethemarginof
theopticdisk.Thesepitsareusuallyfoundinaninferiortemporallocation,
althoughtheydooccurelsewhere.In85%ofallcases,oneeyeisaffected.
Waxypalloropticatrophy.
Fig.13.16Waxy
palloropticatro-
phyisassociated
withtapetoreti-
naldegeneration.
13.4DisorderswithWell-DefinedOpticDiskMargin
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402. 384
Opticnervepits.
Fig.13.17These
areovalgrayish
temporaldepres-
sionsinthepapil-
larytissue
(arrow).
Severalpitsinoneopticdiskhavebeendescribed.Serousretinaldetachment
occursin25%ofallcases,dependingonthelocationofthepit.Wherethe
detachmentaffectsthemacula,asignificantlossofvisualacuitywillresult
thatwillproveverydifficulttomanagewithlasersurgery.Otherwiseoptic
nervepitsareanincidentalfindingwithoutanyfunctionaldeficit.Theyare
consideredtoberudimentarycolobomas.
Opticdiskcoloboma.
Fig.13.18The
opticdiskisen-
largedwithafun-
nel-shapedde-
pressionwith
whitishtissueand
aperipapillary
pigmentring.
Theretinalves-
selsdonot
branchfroma
centralvenousor
arterialtrunk.
13OpticNerve
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403. 385
13.4.3OpticDiskColoboma(MorningGloryDisk)
Anopticdiskcoloboma(Fig.13.18)istheresultofincompleteclosureofthe
embryonicopticcup.Theopticdiskisenlargedwithafunnel-shapeddepres-
sionwithwhitishtissueandaperipapillarypigmentring.Theretinalvessels
extendoutwardacrossthemarginofthediskinaradialpatternwithouta
centraltrunkvessel.Patientswithopticdiskcolobomaoftenhavedecreased
visualacuityandvisualfielddefects.
13.5Tumors
Opticnervetumorsareclassifiedasintraocularorretrobulbartumors.
Intraoculartumorsarerare.
13.5.1IntraocularOpticNerveTumors
Melanocytoma(Fig.13.19):Thesearebenignpigmentedtumorsthatpri-
marilyoccurinblacks.Thecolorofthetumorvariesfromgraytopitch
black.Itisofteneccentricandextendsbeyondthemarginoftheopticdisk.In
50%ofallcases,onewillalsoobserveaperipapillarychoroidalnevus.Visual
acuityisusuallynormal,althoughdiscretechangesinthevisualfieldmybe
present.
Astrocytoma(Fig.13.20):Astrocytomasappearaswhitereflecting“mul-
berry”massesthatcancalcify.Theirsizecanrangeuptoseveraldiskdiame-
ters.Thetumorishighlyvascularized.Visualfielddefectscanresultwhere
thetumorissufficientlylargetocompresstheopticnerve.Astrocytomas
Melanocytoma.
Fig.13.19
Benigntumorof
theopticdisk
thatrepresentsa
specialformof
nevus(arrow).
13.5Tumors
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404. 386
Astrocytomaintuberoussclerosis(Bourneville’sdisease).
Fig.13.20
Whitish,“mul-
berry”tumoron
thesuperiormar-
ginoftheoptic
disk(arrow).
CapillaryhemangiomainvonHippeldisease.
Fig.13.21Ec-
centriccapillary
vasculardeform-
ityontheoptic
disk(arrow).
occurintuberoussclerosis(Bourneville’sdisease)andneurofibromatosis
(Recklinghausen’sdisease).
Hemangioma (Fig.13.21):Capillaryhemangiomasareeccentric,round
orange-coloredvasculardeformitiesontheopticdisk(vonHippeldisease).
Theymayoccurinassociationwithotherangiomas,forexampleinthecere-
bellum(invonHippel-Lindaudisease).
13OpticNerve
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405. 387
13.5.2RetrobulbarOpticNerveTumors
Themostcommonretrobulbaropticnervetumorsaregliomasandmening-
iomas.Symptomsincludeausuallyslowlossofvisualacuitywithexophthal-
mos.Ophthalmoscopywillrevealdescendingprimaryatrophyoftheoptic
nerve.Meningiomaofthesheathoftheopticnerveistypicallyaccompaniedby
theformationofopticociliaryshuntvesselswithcompressionofthecentral
retinalvessels.
13.5Tumors
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407. 389
14VisualPathway
OskarGareisandGerhardK.Lang
14.1BasicKnowledge
Theanatomyofthevisualpathwaymaybedividedintosixseparateparts
(Fig.14.1):
1.Opticnerve:Thisincludesalloftheopticnervefiberbundlesoftheeye.
2.Opticchiasm:Thisiswherethecharacteristiccrossoverofthenervefibers
ofbothopticnervesoccurs.Thecentralandperipheralfibersfromthetem-
poralhalvesoftheretinasdonotcrossthemidlinebutcontinueintotheoptic
tractoftheipsilateralside.Thefibersofthenasalhalvescrossthemidlineand
thereenterthecontralateraloptictract.Alongtheway,theinferiornasal
fiberstravelinasmallarcthroughtheproximalendofthecontralateraloptic
nerve(theanteriorarcofWilbrand).Thesuperiornasalfiberstravelinasmall
arcthroughtheipsilateraloptictract(theposteriorarcofWilbrand).
3.Optictract:Thisincludesalloftheipsilateralopticnervefibersandthose
thatcrossthemidline.
4.Lateralgeniculatebody:Theoptictractendshere.Thethirdneuroncon-
nectstothefourthhere,whichiswhyatrophyoftheopticnervedoesnot
occurinlesionsbeyondthelateralgeniculatebody.
5.Opticradiations(geniculocalcarinetracts):Thefibersoftheinferiorretinal
quadrantspassthroughthetemporallobes;thoseofthesuperiorquadrants
passthroughtheparietallobestotheoccipitallobeandfromtheretothe
visualcortex.
6.Primaryvisualarea(striatecortexorBrodmann’sarea17ofthevisualcor-
tex):Thenervefibersdivergewithintheprimaryvisualarea;themaculalutea
accountsformostofthesefibers.Themaculaisrepresentedonthemostpos-
teriorportionoftheoccipitallobe.Thecentralandintermediateperipheral
regionsofthevisualfieldarerepresentedanteriorly.Thetemporalcrescentof
thevisualfield,onlypresentunilaterally,isrepresentedfarthestanteriorly.
Otherconnectionsextendfromthevisualcortextoassociatedcentersand
oculomotorareas(parastriateandperistriateareas).Asidefromtheoptic
tractthereisalsoanothertractknownastheretinohypothalamictract.This
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408. 390
Anatomyofthevisualpathway.
Layer of optic
nerve fibers
Optic chiasm
Optic tract
Lateral
geniculate
body
Optic nerve
Visual cortex
(area 17)
Left eye Right eye
Optic radiations
(fourth neuron)
Temporal
fibers
Superior nasal
fibers
Inferior
nasal fibers
Anterior arc
of Wilbrand
Posterior arc
of Wilbrand
b
Light
Pigment
epithelium
a
1st neuron
(cones and rods)
2nd neuron
(bipolar cells)
3rd neuron
(ganglion cells)
Fig.14.1aOverviewofthecourseofthevisualpathway.bStructureoftheretina.
cCourseofthenervefibersintheopticchiasm.
14VisualPathway
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409. 391
tractisolderinevolutionarytermsanddivergesfromtheopticchiasm.It
transmitslightimpulsesformetabolicandhormonalstimulationtothedien-
cephalonandpituitaryglandsystemandinfluencesthecircadianrhythm.
14.2ExaminationMethods
Visualfieldtesting(perimetry):Thisisthemostimportanttestforvisual
pathwaylesions.Becauseitpermitsonetodiagnosethelocationofthelesion,
itisalsoofinterestfromaneurologicstandpoint.The“visualfield”isdefined
asthefieldofperceptionoftheeyeatrestwiththegazedirectedstraightahead.
Itincludesallpoints(objectsandsurfaces)inspacethataresimultaneously
visiblewhentheeyefocusesononepoint.
Theexaminationisperformedononeeyeatatime.Theprincipleofthetest
istohavethepatientfocusonacentralpointinthedevicewhiletheeyeisina
definedstateofadaptationwithcontrolledambientlighting(seebelow).
Lightmarkersappearinthehemisphereofthedevice.Thepatientsignalsthat
heorsheperceivesthemarkersbypressingabuttonthattriggersanacoustic
signal.
Therearetwotypesofperimetry.
1.Kineticperimetry.HemisphericGoldmannorRodenstockperimetersare
usedforthistest(Fig.14.2).Kineticperimetryinvolvesmovingpointsoflight
thattravelintothehemispherefromtheperiphery.Lightmarkersofidentical
sizeandintensityproduceconcentricringsofidenticalperceptionreferredto
asisopters.Thepointsoflightdecreaseinsizeandlightintensityastheymove
towardthecenterofthevisualfield,andtheisoptersbecomecorrespond-
inglysmaller(Fig.14.2b).Thiscorrespondswiththesensitivityoftheretina,
whichincreasesfromtheperipherytothecenter.
Theadvantageofkineticperimetryisthepersonalinteractionbetween
physicianandpatient.Thismethodisespeciallysuitableforolderpatients
whomayhavedifficultieswithastereotypedinteractionrequiredbyacom-
puterprogram.Specificindicationsforkineticperimetryincludevisualfield
defectsduetoneurologiccausesandexaminationstoestablishadisability
(suchashemianopsiaorquadranticanopsia).
2.Staticperimetry.Thisisusuallyperformedwithcomputerizedequipment
suchastheHumphreyfieldanalyzer(Fig.14.3)orOctopus2000,althougha
GoldmannorRodenstockhemisphericperimetercanalsobeusedforstatic
testingofthevisualfield.Instaticperimetry,thelightintensityofimmobile
lightmarkersisincreaseduntiltheyareperceived.Theintensitythreshold
continuouslyincreasesfromthemacula,withthehighestsensitivity,tothe
periphery.Avarietyofdifferentcomputerprogramscanbeselecteddepend-
ingonthespecificclinicalsetting.Theseincludetheoutermarginsorthe30
degreevisualfieldinglaucoma(Fig.14.3b).
14.2ExaminationMethods
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410. 392
Goldmannhemisphericperimeterandvisualfieldfindings.
Fig.14.2aThepatientfocuses
withoneeyeonablackdotinthe
middleofthehemisphere.Assoon
asthepatientnoticesthelight
markermovinginfromtheperiph-
ery,heorshepressesabuttonthat
triggersanacousticsignal.Theex-
aminersitsbehindthehemisphere.
Fromthere,theexaminercontrols
thelightmarkerandrecordswhich
pointsthepatientrecognizes.
Continued!
Otherexaminationmethods:
!Pupillaryfindings.
!Pupillarylightreflex.
!Visualevokedpotential.
!CTorMRItodiagnosecauses.
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411. 393
Goldmannhemisphericperimeterandvisualfieldfindings.
F
ig.
1
4
.
2
b
Normal
visual
field.
Due
t
o
the
anat
om
y
o
f
t
he
bridge
o
f
t
he
nose
and
roof
of
the
orbit,
the
visual
field
is
ph
ysiologi-
cally
limit
ed
in
the
nasal
and
superior
regions.
The
blind
spo
t
(op
tic
disk)
normally
lies
1
0
t
o
20
degrees
of
f
cent
er
in
the
horizont
al
plane,
on
the
r
ight
in
the
r
ight
e
y
e
and
on
the
lef
t
in
t
he
lef
t
e
y
e
.
14.2ExaminationMethods
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412. 394
Humphreyfieldanalyzerandvisualfieldfindings.
Fig.14.3aInstaticperimetry,the
patientalsofocusesonablackdotin
themiddleofthehemisphere.As
soonasthepatientperceivesalight
marker,heorshepressesabutton
thattriggersanacousticsignal.The
resultisshownonthemonitoron
theright.
Continued!
14.3DisordersoftheVisualPathway
Lesionsofthevisualpathwaymaybeclassifiedaccordingtothreemainloca-
tions.
1.Prechiasmallesions(lesionsoftheopticnerve)involvevisualfielddefects
onthesameside.
2.Chiasmallesions(disordersoftheopticchiasm)typicallycausebilateral
temporalhemianopsiabutcanalsocauseunilateralorbilateralvisualfield
defects(seebelow).
3.Retrochiasmallesions(disordersofvisualpathwayposteriortotheoptic
chiasm,i.e.,fromtheoptictracttothevisualcortex)causehomonymous
visualfielddefects.
14.3.1PrechiasmalLesions
Disordersoftheopticnerveleadtoanipsilateraldecreaseinvisualacuity
and/orvisualfieldsdefects(seeChapter13).
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413. 395
Humphreyfieldanalyzerandvisualfieldfindings.
F
ig.
1
4
.
3
b
Normal
visual
field
in
the
lef
t
and
right
e
y
es
in
the
3
0
degree
r
ange.
The
se
v
e
rity
of
the
visual
field
def
ect
is
depict
ed
with
increasing
gra
y
scales.
These
correspond
t
o
specific
light
int
ensities
(ASB
st
ands
f
o
r
apostilb)
and
are
g
raphed
as
log
arithmic
v
alues
(DB
st
ands
f
o
r
decibel)
t
o
be
tt
er
visualize
the
r
e
tinal
sensitivity
.
The
dark
a
rea
in
b
o
t
h
g
raphs
represents
the
blind
spo
t.
14.3DisordersoftheVisualPathway
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414. 396
14.3.2ChiasmalLesions
Anatomy:Theopticchiasmandtheopticnerves(Fig.14.4)lieonthedia-
phragmasellae,aduralfoldthatformstheroofofthesellaturcica.
Thepituitaryglandinthesellaturcicaliesinferiortothechiasm.The
internalcarotidarterydefinesthelateralborderofthechiasm.The
hypothalamusandanteriorlobeofthecerebrumarelocatedsuperiortothe
chiasm.Withinthechiasm,theinferiornasalfiberscrossinferiorlyandante-
riorly,andarethereforemostlikelytobeaffectedbypituitarytumors.The
superiornasalfiberscrossposteriorlyandsuperiorlywithinthechiasmand
arethereforemostlikelytobeaffectedbycraniopharyngiomas.Themacular
fiberscrossinvariouslocationsthroughoutthechiasm,includingposteriorly
andsuperiorly.
Etiologyandcorrespondingvisualfielddefects:
Pituitaryadenomas:Thesearetumorsthatproceedfromthehormone-
secretingcellsoftheanteriorlobeofthepituitarygland.Astheyincreasein
sizesuperiorly,theyreachtheanteriormarginofthechiasmwheretheycom-
presstheinferiorandnasalfibersthatcrossthere(Fig.14.5).Thisleadstoan
initialvisualfielddefectinthesuperiortemporalquadrantthatmaylater
progresstocompletebilateraltemporalhemianopsia.Thevisualfielddefect
usuallyspreadsinanasymmetricalpattern.Theeyewiththemoresevere
visualfielddefectoftenexhibitsthelessercentralvisualacuity.
Craniopharyngiomas.Theseslow-growingtumorsdevelopfromtissueofthe
pouchofRathke(thepituitarydiverticulum)alongthestemofthepituitary
gland.Craniopharyngiomascompresstheopticchiasmposteriorlyandsupe-
Anatomicrelationshipsoftheopticchiasm.
Superior and
nasal fibers
Macular fibers
Inferior and
nasal fibers
Anterior
clinoid
process
Pituitary
gland
Third ventricle
Optic chiasm
Diaphragma sellae
Dorsum sellae
Posterior clinoid
process
Fig.14.4Sagittal
sectionofthe
opticchiasm.See
textfordetails.
14VisualPathway
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415. 397
riorlyandthereforeprimarilyaffectthesuperiornasalfibersthatcrossthere
(Fig.14.6).Thecorrespondingvisualfielddefectbeginsintheinferiortem-
poralquadrantsandthenspreadsintothesuperiortemporalquadrants.
Inferiorcompressionoftheopticchiasmbyapituitaryadenoma.
Left eye Right eye
Hand motion
Finger counting
Chromophobic
adenomas spread
anterior to the chiasm
Bony contour of
the optic canal
Fig.14.5The
visualfielddefect
beginsasabi-
lateralsuperior
temporaldefect
andmayprogress
tocompletebi-
lateraltemporal
hemianopsia.The
terms“finger
counting”and
“handmotion”de-
scribethepatient’s
visualperception.
Superiorcompressionoftheopticchiasmbyacraniopharyngioma.
Left eye Right eye
Hand motion
Finger counting
The fibers crossing
posteriorly are the
most sensitive
Craniopharyngioma
Bony contour of
the optic canal
Fig.14.6The
visualfielddefect
beginsbilaterally
intheinferiortem-
poralquadrants
andcanprogress
tocompletebi-
lateraltemporal
hemianopsia.
14.3DisordersoftheVisualPathway
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416. 398
Meningiomas.Thesearetumorsthatproceedfromthearachnoid.Theymay
affectvariousdifferentpartsofthechiasmdependingonthesiteoftheir
origin(Fig.14.7).Whentheyoccuronthetuberculumsellae,theycancom-
presseithertheopticnerveorthechiasm.Tumorsthatcompressthejunction
oftheopticnerveandchiasmsimultaneouslycompressthefibersinthearcof
Wilbrand.Inadditiontotheipsilateralcentralscotoma,thisproducesacon-
tralateralvisualfielddefectinthesuperiortemporalquadrants.Meningiomas
canalsoproceedfromthemarginofthesphenoidandcompresstheoptic
nerve.Thosethatoriginatealongtheolfactorytractcanleadtoalossofsense
ofsmellandtocompressionoftheopticnerve.
Aneurysms.Dilationoftheinternalcarotidarteryduetoananeurysmcan
resultinlateralcompressionofopticchiasm(Fig.14.8).Theresultingvisual
fielddefectbeginsunilaterallybutcanbecomebilateralifthechiasmis
pressedagainstthecontralateralinternalcarotidartery.Initiallythereis
ipsilateralhemianopsiaextendingnasally.Thisisfollowedbycompressionof
thecontralateralsidewithcontralateralhemianopsiathatalsoextends
nasally.
Otherchangesinthechiasm.Asidefromtheexternaleffectsonthechiasm,
changescanoccurwithinthechiasmitself.Theseincludegliomas,demyeli-
nation,andtrauma.Thechiasmcanalsobeinvolvedininfiltrativeorinflam-
matorychangesofthebasalleptomeninges(arachnoiditisoftheopticchi-
asm).Theresultingvisualfielddefectsarehighlyvariable.
Possiblecompressionoftheopticnervebyameningioma.
Left eye Right eye
Scotoma in the superior
temporal quadrants
Meningioma along the
olfactory tract
Bony contour of
the optic canal
Meningioma
on the margin
of the sphenoid
Meningioma on the
tuberculum sellae
Fig.14.7Inaddi-
tiontovisualfield
defectsonthe
sideoftheaf-
fectedopticnerve,
thecontralateral
eyemayalsobe
affectediffibersin
thearcofWil-
brandarecom-
pressed.
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417. 399
Lateralcompressionoftheopticchiasmbyananeurysmintheinternal
carotidartery.
Left eye Right eye
Bony contour of
the optic canal
Lateral
compression
of the chiasm
by an aneurysm
Fig.14.8The
visualfielddefect
beginsonthe
samesideas
hemianopsiaex-
tendingnasally
andcanprogress
tobilateralnasal
hemianopsia.
Symptoms,diagnosticconsiderations,andclinicalpicture:Thecompres-
sionoftheopticnerveproducesprimarydescendingatrophyoftheoptic
nerve.Thisisassociatedwithamoreorlessseveredecreaseinvisualacuity
andvisualfielddefects(seeEtiology).Avisualfielddefectconsistingofhet-
eronymousbilateraltemporalhemianopsiaisreferredtoaschiasmsyn-
drome.Thevisualfielddefectsinthesecasesarefrequentlyincongruent.
Chiasmsyndromedevelopsslowlyandusuallyrepresentsthelatestageofa
pituitaryadenomaorcraniopharyngioma.
Heteronymousbilateraltemporalhemianopsiawithdecreasedvisual
acuityandunilateralorbilateralopticnerveatrophyisreferredtoas
chiasmsyndrome.
Bilateraltemporalvisualfielddefectsaretypicalforchiasmalprocesses.
However,themanypossiblelocationsoflesionsintheregionofthechiasm
producewidelyvaryingvisualfielddefectsdependingonthespecificeti-
ology.
Bilateraltemporalvisualfielddefectsareduetochiasmallesions.A
chiasmallesionshouldalwaysbeconsideredinthepresenceofany
uncertainvisualfielddefect.
Furtherdiagnosticstudiesmaybeperformedaftervisualacuitytesting,pupil-
larylightreactiontesting,perimetry,andophthalmoscopyofthefundusand
opticdisk.Suchstudiesincluderadiographsofthesellaturcica(todetect
14.3DisordersoftheVisualPathway
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418. 400
enlargementordestructionofthesellaturcicaduetoapituitaryadenoma),CT,
MRI,carotidarteriography,and,inapplicablecases,endocrinologicstudies.
Treatment:Thisdependsontheunderlyingcause.Neurosurgerymaybe
indicatedormedication,suchasbromocriptineforapituitarytumor.
Prognosis:Thisalsodependsontheunderlyingdisorder.Ocularfunctional
deficitsmaysubsidewhenthedisorderispromptlydiagnosedandtreated.
14.3.3RetrochiasmalLesions
Etiology:Retrochiasmallesionsmayresultfromawidevarietyofneurologic
disorderssuchastumors,vascularinsults,basalmeningitis,aneurysmsofthe
posteriorcommunicatingartery,abscesses,injuries(suchasacontrecoup
injurytotheoccipitallobe),andvasospasms(inanocularmigraine).
Symptoms,diagnosticconsiderations,andclinicalpicture:Visualfield
testinginparticularwillprovideinformationonthelocationofthelesion.
Perimetryisthereforeacrucialdiagnosticstudy.Bilateralsimultaneousvisual
fielddefectsarecommontoallretrochiasmallesionsofthevisualpathway.
Oftenthesedefectswillbeincongruent.
Homonymousvisualfielddefectsaretheresultofaretrochiasmal
lesion.
Lesionsoftheoptictractandthelateralgeniculatebody.Becausethenerve
fibersareconcentratedinaverysmallspace,thevisualfielddefectthat
occurstypicallyintheselesionsishomonymoushemianopsia.Lesionsonthe
rightsideproducevisualfielddefectsinthelefthalfofthevisualfieldand
viceversa.Partialprimaryatrophyoftheopticnervemayoccurasthethird
neuronisaffected,whichextendsfromtheretinatothelateralgeniculate
body.Anafferentpupillarydefectonthesideoppositethelesionwillbepresent.
Thecauseofthisdefectisnotknown.
Lesionsoftheopticradiations.Thevisualfielddefectsassumemanydifferent
formsduetothewidespreadoftheopticradiations.Injuriestoboththetem-
poralandparietallobestypicallyproducehomonymoushemianopsia.Injuries
primarilyinvolvingthetemporallobeproducehomonymoussuperiorquad-
ranticanopsia;injuriesprimarilyinvolvingtheparietallobeproducehomo-
nymousinferiorquadranticanopsia.Pupillaryfindingsarenormalbecause
thelesionaffectsthefourthneuron.Approximately30%ofallcasesinvolvean
afferentpupillarydefectonthesideoppositethelesion.Thecauseofthisdefect
isnotknown.
Lesionsofthevisualcortex.Thevisualfielddefects,likethelesionsofthe
visualpathway,arehomonymousandhemianoptic.Themaculamayormay
notbeaffecteddependingontheextentofthelesion.
14VisualPathway
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419. 401
Specialforms.
Corticalblindness.Bilaterallesionsofthevisualcortex,especiallyinjuries,
canproducebothtemporalandnasalvisualfielddefectswithnormalpupil-
larylightreactionandnormalopticdiskfindings.
Visualagnosia.Wheretheassociationareasofthebrainaredamaged,as
oftenoccursinlesionsoftheparietallobeormarginalvisualcortex,the
patientcanseebutisunabletointerpretorclassifyvisualinformation.
Examplesofthisincludealexia(acquiredinabilitytocomprehendwritten
words)andcoloragnosia(inabilitytodistinguishcolors).
Othersymptomsandfindings.Dependingontheunderlyingdisorder,these
mayincludeheadache,nausea,vomiting,andpapilledema.Adifferential
diagnosisrequiresCTandMRIstudies.
Visualfielddefectsassociatedwiththemajorlesionsofthevisualpathway.
Optic chiasm
Optic tract
Lateral
geniculate
body
Optic nerve
Visual cortex
(area 17)
1
2
3
4
5
6
7
8
1
2
3
4
5
6
7
8
Left eye Right eye
Optic
radiations
Fig.14.9
14.3DisordersoftheVisualPathway
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420. 402
Treatment:Dependingontheunderlyingdisorder,thepatientisreferredto
eitheraneurologistorneurosurgeonfortreatment.
Prognosis:Theprognosisisgenerallypoor,andthevisualfielddefectsusu-
allydonotsubside.
OcularMigraine
Thisisduetoatransientvasospasmoftheposteriorcerebralarterythat
suppliesthevisualcortex.Symptomsvary.Typicallytherewillbeaunilateral
homonymousandinitiallyparacentralscintillatingscotoma,aseriesof
flashesofbrightlight(fortificationspectra),andperceptionsofdazzling
colors.Headache,nausea,andvertigoalsooccur.Paresisoftheocularmuscles
(ophthalmoplegicmigraine)mayalsooccur.Treatment:Patientsshouldbe
referredtoaneurologist.
Fig.14.9providesaschematicoverviewofallmajorlesionsofthevisual
pathwaywiththeirassociatedvisualfielddefects.
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421. 403
15OrbitalCavity
ChristophW.SpraulandGerhardK.Lang
15.1BasicKnowledge
Importanceoftheorbitalcavityfortheeye:Theorbitalcavityistheprotec-
tivebonysocketfortheglobetogetherwiththeopticnerve,ocularmuscles,
nerves,bloodvessels,andlacrimalgland.Thesestructuresaresurroundedby
orbitalfattytissue.Theorbitalcavityisshapedlikeafunnelthatopensanteri-
orlyandinferiorly.Thesixocularmusclesoriginateattheapexofthefunnel
aroundtheopticnerveandinsertintotheglobe.Theglobemoveswithinthe
orbitalcavityasinajointsocket.
Bonysocket:Thisconsistsofsevenbones(Fig.15.1):
!Frontal.
!Ethmoid.
!Lacrimal.
!Sphenoid.
!Maxillary.
!Palatine.
!Zygomatic.
Thebonyrimoftheorbitalcavityformsastrongring.Itsotherbonysurfaces
includeverythinplatesofbone(seeadjacentstructures).
Adjacentstructures:Thecloseproximityoftheorbitalcavitytoadjacent
structuresisclinicallysignificant.Themaxillarysinusinferiortotheorbital
cavityisseparatedfromitbyaplateofbone0.5mmthick.Theethmoidal
aircellslocatedmedialandposteriortotheorbitalcavityareseparated
fromitbyaplateofboneonly0.3mmthickorbyperiosteumalone.Thefol-
lowingotherstructuresarealsolocatedimmediatelyadjacenttotheorbital
cavity.
!Sphenoidalsinus.
!Middlecranialfossa.
!Regionoftheopticchiasm.
!Pituitarygland.
!Cavernoussinus.
Superioradjacentstructuresincludetheanteriorcranialfossaandthefron-
talsinus.Table15.1liststhevariousbonyopeningsintotheorbitalcavityand
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422. 404
Anterioraspectoftheleftorbitalcavity.
Anterior and posterior
ethmoidal foramina
Supraorbital notch
Orbital plate of
the ethmoid
Frontomaxillary
suture
Nasal
Anterior
lacrimal crest
Posterior
lacrimal crest
Infraorbital suture
Zygomaticomaxillary suture Infraorbital sulcus
Inferior orbital
fissure
Zygomaticofacial
foramen
Orbital plate
of the zygomatic
Zygomatic
Greater wing of
the sphenoid
Superior orbital fissueOptic canal
Fig.15.1Diagramofthesevenorbitalbonesandtheopeningsintotheorbital
cavity.
theanatomicstructuresthatpassthroughthem.Becauseofthisanatomicsit-
uation,theorbitalcavityisfrequentlyaffectedbydisordersofadjacentstruc-
tures.Forexample,inflammationsoftheparanasalsinusescanresultinorbi-
talcellulitis.
Thewallsoftheorbitalcavityarelinedwithperiosteum,whichisalso
referredtoasperiorbita.Itsanteriorboundaryisformedbytheorbitalsepta
extendingfromtheorbitalrimtothesuperiorandinferiortarsalplates,the
lateralandmedialpalpebralligaments,andtheeyelids.
Arterialsupply:Theorbitalcavityissuppliedbytheophthalmicartery,a
branchoftheinternalcarotidartery.Theophthalmicarterycommunicates
withtheangularartery,abranchoftheexternalcarotidartery,viathe
supraorbitalandsupratrochleararteries.
Stenosisoftheinternalcarotidarterycanresultinreversedbloodflow
throughthesupraorbitalandsupratrochleararteries.Thiscanbedem-
onstratedbyDopplerultrasoundstudies.
15OrbitalCavity
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423. 405
Table15.1Openingsintotheorbitalcavityandthestructuresthatpassthroughthem
Orbitalopenings Structures
Opticcanal !Opticnerve
!Ophthalmicartery
Superiororbitalfissure !Oculomotornerve
!Trochlearnerve
!Abducentnerve
!Ophthalmicnerve:
–Lacrimalnerve
–Frontalnerve
–Nasociliarynerve
!Superiorophthalmicveins
Inferiororbitalfissure !Infraorbitalnerve
!Zygomaticnerve
!Inferiorophthalmicvein
Infraorbitalcanal !Infraorbitalnerve
Venousdrainagefromtheorbitalcavity:Theorbitalcavitydrainsthrough
theinferiorophthalmicveinintothepterygoidplexus,throughthesuperior
ophthalmicveinintothecavernoussinus,andthroughtheangularveininto
thefacialveins.
15.2ExaminationMethods
Cardinalsymptoms:Unilateralorbilateralenophthalmos(recessionofthe
eyeballwithintheorbitalcavity)orexophthalmos(protrusionoftheeyeball)
arecharacteristicofmanyorbitaldisorders(Table15.2).Theseconditions
shouldbedistinguishedfrompseudoexophthalmosduetoalongeyeballin
severemyopia,andpseudoenophthalmosduetoasmalleyeball,suchasin
microphthalmosorphthisisbulbi.
Thefollowinglistofexaminationtechniquesbeginswiththesimplestan-
dardtechniquesandprogressestothedifficult,moreelaboratemethods.Asa
generalrule,orbitaldisordersrequireinterdisciplinarycooperationbetween
ENTspecialists,neurologists,neurosurgeons,neuroradiologists,internists,
nuclearmedicinespecialists,andoncologists.
15.2ExaminationMethods
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Table15.2Causesforexophthalmosandenophthalmoslistedaccordingtoclinicalsyn-
dromesofsimilaretiology.Boththegroupsthemselvesandthedisorderswithineach
grouparelistedindescendingorderofincidence
Changeinposition Causes
Exophthalmos
(protrusionoftheeyeball)
!Graves’disease(mostfrequentcause)
Inflammatoryorbitaldisorders
!Orbitalcellulitis(mostfrequentcausein
children)
!Orbitalpseudotumor(autoimmunedisorder)
!Myositisoftheocularmuscles(specialformof
pseudotumor)
!Orbitalabscess
!Cavernoussinusthrombosis(seriousclinical
syndrome)
!Severetenonitis(inflammationofTenon’scap-
sule)
!Mucocele
!Mycosis(inimmunocompromisedpatients)
!Parasiticinfestationoftheorbitalcavity(rare)
Vascularorbitaldisorders
!Arteriovenousfistulas(pulsating)
!Orbitalhematomas(usuallypost-traumatic)
!Orbitalvarices(intermittentexophthalmos)
Orbitaltumors(slowlyprogressive)
Developmentalanomalies
!Craniosynostosis(prematurefusionofcranial
sutures)
!Meningoencephalocele(veryrare)
!Osteopathy(rare)
Enophthalmos Orbitalfractures(mostfrequentcause)
!Blowoutfracturenotassociatedwithanorbital
hematoma
Neurogeniccauses
!Horner’ssyndrome(sympatheticpalsy)
!Paresisoftheobliqueocularmuscles
Atrophyoforbitaltissue(symmetrical)
!Senileatrophyoftheorbitalfat
!Dehydration
15OrbitalCavity
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425. 407
Visualacuity:SeeChapter1.
Ocularmotility:Thepatternofdisturbedocularmotilitycanbeasignofthe
causeofthedisorder.Causesmaybeneurogenic,myogenic,ormechanical
(seeChapter17).
Examinationofthefundus:Retrobulbarprocessescanpresstheglobe
inward.Thisoftenproduceschoroidalfoldsthatarevisibleuponophthal-
moscopy.Compressionoftheopticnervebyatumormayresultinoptic
nerveatrophyoredema.Meningiomasinthesheathoftheopticnervelead
tothedevelopmentofshuntvesselsontheopticdisk.
Exophthalmometry:TheHertelmirrorexophthalmometer(Figs.15.2aandb)
measurestheanteriorprojectionoftheglobebeyondtheorbitalrim.A
FunctionandapplicationoftheHertelmirrorexophthalmometer.
15
d20
c
15
d20
c
100
15
20
C
15
D20
100
C
D
FF
E
B
E
B
Fig.15.2aThedevicemeasurestheextraorbitalprominenceoftheeyefromthe
anteriorsurfaceofthecornea(dashedline)tothetemporalbonyrimoftheorbit(F).
Theexaminer(B)viewstheanteriorsurfaceofthecorneathroughamirror(C).The
extraorbitalprominenceinmillimetersisthenreadofftheintegralscale(D).Toob-
tainreproducibleresults,itisimportanttomaintainaconstantbasesettinginmm
(E)everytimetheexophthalmometerisapplied.
Continued!
15.2ExaminationMethods
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426. 408
FunctionandapplicationoftheHertelmirrorexophthalmometer(continued).
Fig.15.2bTheexophthalmometerisplacedonthelowestpointofthetemporal
zygomatic.Toavoidparallacticmeasurementerrors,theexaminermoveshisorher
owneyehorizontallyuntilthetwointegralgraduations(blackarrowheadsonthe
right)alignintheprojection(blackleftarrow).Oncethegraduationsarealigned,
theexaminerreadsthevalueoftheextraorbitalprominenceoftheanteriorsurface
ofthecornea(longwhitearrow)onthescale(shortwhitearrows).Theexaminer
readsthemeasurementwithonlyoneeye.Theexamineruseshisorherlefteyeto
readthevalueforthepatient’srighteyeandviceversa.
changeinthepositionoftheglobewithrespecttotheorbitalrimisacardi-
nalsymptomofmanyorbitaldisorders(seeTable15.2).
Thedifferencebetweenthetwosidesismoreimportantthantheabso-
lutevalue.Adifferencegreaterthan3mmbetweenthetwoeyesis
abnormal.Unilateralexophthalmosisrecognizablewithoutanexoph-
thalmometer.Todoso,theexaminerstandsbehindthepatient,slightly
liftsthepatient’suppereyelids,andlooksdownoverthepatient’sfore-
headtowardthecheek.
Visualfieldtesting:Thisisusedtodocumentdamagetotheopticnervein
orbitaldisorders.
Ultrasoundstudies:Twotechniquesareavailableforthisnoninvasive
examination.
1.TheB-modescan(Bstandsforbrightness)providesatwo-dimensional
imageoforbitalstructures.Thisexaminationisindicatedinthepresenceof
suspectedorbitalmasses.
15OrbitalCavity
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427. 409
2.TheA-modescan(Astandsforamplitude)permitsprecisemeasurementof
opticnerveandmusclethickness.Thisexaminationisindicatedasafollow-
upstudyinthepresenceofGraves’disease(endocrineorbitopathy).
ThesestudiesmayalsobecombinedwithDopplerscanstoevaluateblood
flow.
Conventionalradiographicstudies:Thesestudiesusuallyonlyprovide
informationaboutthenatureofbonestructures,i.e.,whetherafractureis
presentandwhereitislocated.Smallerfracturesoftencannotbediagnosed
byconventionalradiographyandrequireCTscans.
Computedtomographyandmagneticresonanceimaging:Thesemodern
examinationmodalitiescanpreciselyvisualizeorbitalstructuresinvarious
planes.Theyarestandardmethodsfordiagnosingtumors.
Inthepresenceoforbitaltrauma,initialCTstudiesshouldbeper-
formedasthismethodcanbettervisualizebonystructures.InitialMRI
scansshouldbeperformedwheresoft-tissuelesionsaresuspected.
Angiography:Thisisindicatedinthepresenceofsuspectedarteriovenous
fistulas.
15.3DevelopmentalAnomalies
Congenitaldevelopmentalanomaliesaffectingtheorbitalcavityarevery
rare.
15.3.1CraniofacialDysplasia
15.3.1.1Craniostenosis
Thisclinicalpictureinvolvesprematurefusionofthecranialsutures.Clinical
signsoftenincludebilateralexophthalmosassociatedwithocularhyper-
telorismandexotropia(divergentstrabismus).Themechanicalimpairmentof
theopticnerveisevidencedbydevelopmentofpapilledemaandrequiressur-
gicaldecompressiontopreventatrophyoftheopticnerve.
15.3.1.1.1Oxycephaly
Prematurefusionofthecoronalsuturecausestheorbitstobecomeele-
vated,flattened,andsmallerthannormal.
15.3DevelopmentalAnomalies
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428. 410
15.3.1.1.2CraniofacialDysostosis
Prematurefusionofthecoronalandsagittalsuturesalsoresultsinahigh
skullandabnormallysmallorbits.Thisconditionisalsocharacterizedbya
widerootofthenoseandaprominentchin.
Enucleationinearlychildhoodcanresultinorbitalhypoplasiaasthe
globeprovidesagrowthstimulusfortheorbitalcavity.Thereforethe
patientshouldpromptlyreceiveaprosthesis.
15.3.2MandibulofacialDysplasia
15.3.2.1OculoauriculovertebralDysplasia
Epibulbardermoidsnearthelimbusarepresentinadditiontoouterear
anomaliesandrudimentsofabranchialpassageinthecheek(seeFig.4.19).
15.3.2.2MandibulofacialDysostosis
AlsoknownasTreacherCollins’syndrome(incompletetype)orFrances-
chetti’ssyndrome(completetype),thisanomalyofthefirstbranchialarchis
characterizedbyorbitaldeformitieswithantimongoloidpalpebralfissures,
colobomaofthelowereyelid,low-setears,andahypoplasticmandiblewith
dentaldeformities.
15.3.2.3OculomandibularDysostosis
Inadditiontothetypicalbird-likeface,thisanomalymaybeaccompaniedby
bilateralmicrophthalmosassociatedwithcataract,nystagmus,andstra-
bismus.
15.3.2.4Rubinstein–TaybiSyndrome
Thiscraniomandibulofacialdysplasiaisprimarilycharacterizedbyantimon-
goloidpalpebralfissures,ocularhypertelorism,epicanthalfolds,andenoph-
thalmos.Cataracts,iriscolobomas,andinfantileglaucomahavealsobeen
described.
15.3.3Meningoencephalocele
Incompletefusionofthecranialsuturesintheorbitalregioncanleadto
evaginationsofduralsacwithbraintissue.Clinicalfindingsoccasionally
includepulsatingexophthalmosor,inextremecases,atumorousprotrusion.
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429. 411
15.3.4Osteopathies
Manyofthesedisorderscanproduceorbitalchanges.Themostcommonof
thesediseasesincludePaget’sdiseaseofbone,dysostosismultiplex(Hurler’s
syndrome),andmarble-bonediseaseofAlbers-Schönberginwhichcompres-
siveopticneuropathyalsooccurs.
15.4OrbitalInvolvementinAutoimmuneDisorders:
Graves’Disease
Definition
Autoimmunedisorderwithorbitalinvolvementfrequentlyassociatedwiththy-
roiddysfunction.Histologicexaminationrevealsinflammatoryinfiltrationof
theorbitalcavity.
Epidemiology:Womenareaffectedeighttimesasoftenasmen.Sixtyper
centofallpatientshavehyperthyroidism.Tenpercentofpatientswiththy-
roiddisordersdevelopGraves’diseaseduringthecourseoftheirlife.
Graves’diseaseisthemostfrequentcauseofbothunilateraland
bilateralexophthalmos.
Etiology:Thepreciseetiologyofthisautoimmunedisorderisnotclear.Histo-
logicexaminationrevealslymphocyticinfiltrationoftheorbitalcavity.The
ocularmusclesareparticularlyseverelyaffected.Fibrosisdevelopsafterthe
acutephase.
Anautonomousadenomaofthethyroidglandisnotassociatedwith
Graves’disease.SomepatientswithGraves’diseaseneverexhibitany
thyroiddysfunctionduringtheirentirelife.
Symptoms:Theonsetofthisgenerallypainlessdisorderisusuallybetween
theagesof20and45.Patientscomplainofreddeneddryeyeswithasensa-
tionofpressure(symptomsofkeratoconjunctivitissicca)andofcosmetic
problems.Ocularmotilityisalsolimited,andpatientsmayexperiencedouble
vision.
Diagnosticconsiderations:Cardinalsymptomsincludeexophthalmos,
whichisunilateralinonly10%ofallcases,andeyelidchangesthatinvolve
developmentofacharacteristiceyelidsign(Table15.3andFig.15.3).Thicken-
ingofthemuscles(primarilytherectusinferiorandmedialis)andsub-
sequentfibrosisleadtolimitedmotilityanddoublevision.Elevationis
impaired;thiscanleadtofalsehighvalueswhenmeasuringintraocular
pressurewiththegazeelevated.
15.4AutoimmuneDisordersandtheOrbit:Graves’Disease
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430. 412
Table15.3EyelidsignsinGraves’disease
Eyelidsign Explanation
!Dalrymple’ssign Uppereyelidisretractedwithvisiblesclerasuperior
tothelimbusandwidenedpalpebralfissurewith
developingexposurekeratitis(overactivemuscleof
Müller).
!vonGraefe’ssign Uppereyelidretractswhentheeyedepresses(over-
activemuscleofMüller).
!Gifford’ssign Uppereyelidisdifficulttoevert(duetoeyelid
edema).
!Stellwag’ssign Rareblinking.
!Kocher’ssign Fixedgaze.
!Eyelidflutterswhenclosed
PatientwithGraves’disease,moresevereintheleftthanintherighteye.
Fig.15.3Typical
signsincludeex-
ophthalmos,
whichhereis
readilyapparent
inthelefteye,re-
tractionofthe
uppereyelidwith
visiblesclerasu-
periortothelim-
bus(Dalrymple’s
sign),conjuncti-
valinjection,and
fixedgaze
(Kocher’ssign).
ThetentativeclinicaldiagnosisofGraves’diseaseissupportedbythicken-
ingoftheextraocularmusclesidentifiedinultrasoundorCTstudies
(Fig.15.4).Thefurtherdiagnosticwork-uprequiresthecooperationofan
internist,endocrinologist,andradiologist.
15OrbitalCavity
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431. 413
CTimageofapatientwithGraves’disease.
Fig.15.4Theimage
showsobviousthickening
oftheextraocular
musclesintherightorbit,
primarilytherectusmedi-
alis(1)andrectuslateralis
(2),andoftherectusme-
dialis(3)intheleftorbit.
Differentialdiagnosis:Rarerclinicalsyndromessuchasorbitaltumorsand
orbitalpseudotumorsmustbeexcluded.
Treatment:Themainprinciplesintreatingthediseaseinitsacutestage
includemanagementofthethyroiddysfunction,systemiccortisone(initially
60–100mgofprednisone)andradiationtherapyoftheorbitalcavity.Surgical
decompressionoftheorbitalcavityisindicatedinrecurrentcasesthatdonot
respondtotreatmenttoavoidcompressiveopticneuropathy.Exposureker-
atitis(keratitisduetoinabilitytoclosetheeye)shouldbetreatedwithartifi-
cialtearsortarsorrhaphy(partialorcompletesutureclosureoftheupperand
lowereyelidtoshortenorclosethepalpebralfissure).Inthepostinflam-
matorystageofthedisease,eyemusclesurgerymaybeperformedtocorrect
strabismus.
Clinicalcourseandprognosis:Visualacuitywillremaingoodiftreatmentis
initiatedpromptly.Inthepostinflammatoryphase,exophthalmosoftenper-
sistsdespitethefactthattheunderlyingdisorderiswellcontrolled.
15.5OrbitalInflammation
Becauseofthecloseproximityoftheorbitalcavitytotheparanasalsinuses,
whichareparticularlysusceptibletoinflammation,orbitalinflammation
representsthesecondmostfrequentgroupoforbitaldisordersafterGraves’
disease.Orbitalcellulitisisthemostsevereofthese.
15.5OrbitalInflammation
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432. 414
15.5.1OrbitalCellulitis
Definition
Acuteinflammationofthecontentsoftheorbitalcavitywiththecardinal
symptomsoflimitedmotilityandgeneralmalaise.
Orbitalcellulitisisthemostfrequentcauseofexophthalmosinchildren.
Etiology:Acuteorbitalinflammationposteriortotheorbitalseptumisusu-
allyaninflammationthathasspreadfromsurroundingtissue.Over60%ofall
cases(ashighas84%inchildren)maybeclassifiedasoriginatinginthe
sinuses,especiallytheethmoidalaircellsandthefrontalsinus.Ininfants,
toothgerminflammationsmaybethecause.Lessfrequently,thisclinicalpic-
tureoccursinassociationwithfacialfuruncles,erysipelas,hordeolum,
panophthalmitis,orbitalinjuries,andsepsis.
Symptoms:Patientsreportseveremalaise,occasionallyaccompaniedby
feverandpainexacerbatedbyeyemovement.
Diagnosticconsiderations:Typicalsymptomsincludeexophthalmoswith
severechemosis(conjunctivalswelling),eyelidswelling,andsignificantly
limitedocularmotility(“cemented”globe;seeFig.15.5).Patientsmay
exhibitleukocytosisandanincreasederythrocytesedimentationrate.
Wherethereisclinicalevidenceofsuspectedinvolvementoftheparanasal
sinuses,anENTspecialistshouldbeconsultedtoevaluatethesinusesand
initiateanynecessarytreatment.
Patientwithorbitalcellulitis.
Fig.15.5Typical
symptomsin-
cludechemosis
(conjunctival
swelling),exoph-
thalmos,andsig-
nificantlylimited
ocularmotility
(therighteye
doesnotmove
withthelefteye).
15OrbitalCavity
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433. 415
Differentialdiagnosis:Preseptalcellulitis,whichismorefrequentlyencoun-
tered,shouldbeexcluded.Theinflammationinpreseptalcellulitisisanterior
totheorbitalseptum;chemosisandlimitedmotilityareabsent.Rarerclinical
syndromesthatshouldalsobeconsideredinadifferentialdiagnosisinclude
anorbitalpseudotumor,orbitalperiostitiswhichmaybeaccompaniedbya
subperiostealabscess,andanorbitalabscess.
Thecrucialcharacteristicfeatureoforbitalcellulitisfordifferentialdiag-
nosisisthesignificantlylimitedocularmotility(“cemented”globe).A
rhabdomyosarcomashouldalsobeconsideredinchildren.
Treatment:Thisconsistsofhigh-doseintravenousantibiotictherapywith
1.5gofoxacillineveryfourhourscombinedwithonemillionunitsofpenicil-
linGeveryfourhours.Infantsaretreatedwithceftriaxoneandschool-age
childrenwithoxacillincombinedwithcefuroximeintheappropriatedoses.
Treatmentofunderlyingsinusitisisindicatedinapplicablecases.
Clinicalcourseandcomplications:Orbitalinflammationcanleadtooptic
neuritiswithsubsequentatrophyandlossofvision.Purulentthrombophle-
bitisoftheorbitalveinscanresultincavernoussinusthrombosiswithmenin-
gitis,cerebralabscess,orsepsis.
Orbitalcellulitiscanprogresstoalife-threateningsituation(cavernous
sinusthrombosis).
15.5.2CavernousSinusThrombosis
Definition
Rarebutsevereacuteclinicalsyndromeinwhichthespacesofthecavernous
sinusposteriortotheorbitalcavitybecomethrombosed,usuallyinthepres-
enceofadjacentpurulentprocesses.Thisisnotanorbitaldisorderinthestrict
sense.
Etiology:Thesearepurulentinflammationsthathavespreadfromthe
middleear,petrousbone,orbitalcavities,orfromthefacialskinviatheangu-
larvein.
Symptoms:Patientspresentwithanacuteclinicalpicturewithheadache,
stupor,fever,andvomiting.
Clinicalfindings:Theophthalmologistwillusuallydiagnosebilateralexoph-
thalmosandepiscleralandconjunctivalvenousstasisincombinationwith
multipleparesesofthecranialnerves.Neurogenicparalysisofallocular
musclesisreferredtoastotalophthalmoplegia.Wheretheopticnerveisalso
involved,theconditionisreferredtoasorbitalapexsyndrome.
15.5OrbitalInflammation
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434. 416
Thelimitedmotilityoftheglobeisprimarilyneurogenicanddueto
damagetothenervesinthecavernoussinusasopposedtothe
mechanicallimitationofmotilityduetotheorbitalinflammationin
orbitalcellulitis.
Diagnosticconsiderationsandtreatment:Thisliesprimarilyinthehands
ofENTspecialists,neurosurgeons,andinternists.High-dosesystemicanti-
biotictherapyandanticoagulationareindicated.
15.5.3OrbitalPseudotumor
Definition
Lymphocyticorbitaltumorofunknownorigin.
Symptomsandfindings:Painful,moderatelysevereinflammatoryreaction
witheyelidswelling,chemosis,andunilateralorbilateralexophthalmos.
Involvementoftheocularmusclesresultsinlimitedmotilitywithdiplopia.
Diagnosticconsiderations:TheCTandMRimageswillshowdiffusesoft-
tissueswelling.Abiopsyisrequiredtoconfirmthediagnosis.
OccasionallytheCTimagewillsimulateaninfiltrativetumor.
Differentialdiagnosis:Variousdisordersshouldbeexcluded.Theseinclude
Graves’diseaseandorbitalcellulitis,whichisusuallybacterial.Specialforms
oforbitalpseudotumorincludemyositisandTolosa–Huntsyndrome(pain-
fultotalophthalmoplegiaproducedbyanidiopathicgranulomaattheapexof
theorbit).
Treatment:High-dosesystemiccortisone(initially100mgofprednisone)
usuallyleadstoremission.Orbitalradiationtherapyorsurgicalintervention
maybeindicatedincasesthatfailtorespondtotreatment.
15.5.4Myositis
Thisaspecialformoforbitalpseudotumorinwhichthelymphaticinfiltra-
tionprimarilyinvolvesoneormoreocularmuscles.Asidefromsignificantpain
duringmotion,symptomsincludelimitedocularmotilitywithdoublevision
(diplopia).Dependingontheextentofthemyositicchanges,exophthalmos
withchemosisandeyelidswellingmayalsobepresent.Ultrasoundstudies
(Fig.15.6)willrevealthickeningoftheocularmuscleswithtenonitis(inflam-
mationofTenon’scapsule).
InGraves’disease,onlythemusclebellyisthickened.Inmyositis,the
entiremuscleisthickened.
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435. 417
15.5.5OrbitalPeriostitis
Thisisaninflammationoftheperiosteumliningtheorbitalcavity,usually
duetobacterialinfectionsuchasactinomycosis,tuberculosis,orsyphilis.Less
frequently,thedisorderisduetoosteomyelitisor,ininfants,toothgerm
inflammations.Theclinicalsymptomsaresimilartoorbitalcellulitisalthough
significantlylesssevereandwithoutlimitationofocularmotility.Liquefac-
tionoftheprocesscreatesanorbitalabscess;largeabscessesmayprogressto
orbitalcellulitis.
15.5.6Mucocele
Thesemucus-filledcystsmayinvadetheorbitalcavityinchronicsinusitis.
Theydisplaceorbitaltissueandcauseexophthalmos.
Treatmentisrequiredinthefollowingcases:
!Displacementoftheglobecausescosmeticorfunctionalproblems,suchas
lagophthalmosorlimitedmotility.
!Compressionneuropathyoftheopticnerveresults.
!Themucocelebecomesinfected(pyocele).
15.5.7Mycoses(MucormycosisandAspergillomycosis)
Theseraredisordersoccurprimarilyinimmunocompromisedpatients,such
asthosewithdiabetesmellitusorAIDS.Thedisorderoftenspreadsfrom
infectedparanasalsinuses.Theclinicalpictureissimilartothoseofinflam-
matoryorbitaldisorders.
Diagnosisofmyositis.
Fig.15.6The
ultrasoundimage
(B-modescan)
showsthickening
oftheentire
hypoechoicrec-
tusmedialis
(arrow).
15.5OrbitalInflammation
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15.6VascularDisorders
Thesechangesarerare.Themostimportantandmostfrequentlyencountered
disorderinthisgroupispulsatingexophthalmos.
15.6.1PulsatingExophthalmos
Definition
Acuteexophthalmoswithpalpableandaudiblepulsationssynchronouswith
thepulseinthepresenceofacavernoussinusfistulaorarteriovenousaneurysm.
Etiology:Anabnormalcommunicationbetweenthecavernoussinusandthe
internalcarotidartery(adirectshunt)oritsbranches(indirectshunt)results
indistentionoftheorbitalvenousnetwork.Eightypercentofallcasesare
attributabletotrauma;lessfrequentlythedisorderisduetosyphilisorarte-
riosclerosis.
Symptoms:Patientsreportanunpleasantsoundintheheadthatisreminis-
centofamachineandsynchronouswiththeirpulse.
Diagnosticconsiderations:Theincreasedvenouspressureleadstodilation
oftheepiscleralandconjunctivalvessels(Fig.15.7),retinalsignsofvenous
stasiswithbleeding,exudation,andpapilledema.Intraocularpressureis
alsoincreased.Theincreasedpressureinthecavernoussinuscanalsoresult
inoculomotorandabducentnervepalsy.
Soundsnearthedirectfistulaareclearlyaudiblewithastethoscope.
Fistulabetweenthecarotidarteryandcavernoussinus.
Fig.15.7The
episcleraland
conjunctivalves-
selsaresignifi-
cantlydilatedand
describetortuous
corkscrew
courses.
15OrbitalCavity
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437. 419
Dopplerultrasoundstudiescanconfirmaclinicalsuspicion.However,only
angiographycandeterminetheexactlocationoftheshunt.
Treatment:Selectiveembolizationmaybeperformedincooperationwitha
neuroradiologistoncetheshunthasbeenlocated.
Smallshuntsmayclosespontaneouslyinresponsetopressurefluctua-
tionssuchascanoccurinairtravel.
15.6.2IntermittentExophthalmos
Thisrareclinicalpicturecharacterizedbyintermittentunilateralorbilateral
exophthalmosiscausedbyvaricosedilationoftheorbitalveins,suchascan
occurfollowingtraumaorinOsler’sdisease(polycythemiavera).Patients
reportprotrusionoftheeyeballofvaryingseverity.Exophthalmosisusually
unilateralandisespeciallypronetooccurwhentheresistancetovenous
drainageisincreased,ascanoccurwhenthepatientpresses,bendsover,
screams,orcompressesthevesselsoftheneck.Occasionallytheexophthal-
moswillbeassociatedwithincreasedfillingoftheepiscleraland/orconjunc-
tivalvessels.Thedisordercanbediagnosedinultrasoundstudiesusingthe
Valsalvamaneuver.Adifferentialdiagnosisshouldexcludeafistulabetween
thecarotidarteryandcavernoussinusoranarteriovenousaneurysm,which
isusuallyaccompaniedbyadramaticclinicalpicturewithpulsationand
increasedintraocularpressure.Intheseclinicalpictures,theultrasound
examinationwillrevealgeneralizeddilationoftheorbitalveins.Surgical
removaloforbitalvaricesentailsahighriskofdamagingcrucialdelicateneu-
rovascularstructuresintheorbitalcavity.However,itmaybeindicatedin
rarecasessuchascosmeticallyunacceptableexophthalmosorwheresymp-
tomsofkeratoconjunctivitissiccaoccurduetoexposurethatfailstorespond
totreatment.
15.6.3OrbitalHematoma
Orbitalbleedingisusuallypost-traumaticbutmayoccurlessfrequentlydue
tocoagulopathyresultingfromvitaminCdeficiency,anticoagulants,or
leukemia.Retrobulbarinjectionspriortoeyesurgeryandacutevenousstasis
suchasmayoccurincoughingfits,asphyxia,orchildbirthcanalsocauseorbi-
talhematomas.Exophthalmosmaybeaccompaniedbymonocleoreyeglass
hematoma,eyelidswelling,andsubconjunctivalhemorrhage;limitedmotilityis
rare.Surgicaldecompressionoftheorbitalcavity(transfornixorbitaldecom-
pressionororbitotomy)isindicatedwheredamagetotheopticnerveor
blockageofthecentralretinalarteryisimminent.
15.6VascularDisorders
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438. 420
15.7Tumors
15.7.1OrbitalTumors
Allorbitaltumorsdisplacetheglobeandcauseexophthalmosthatis
frequentlyassociatedwithlimitedocularmotility.Sometumorsalsocause
specificadditionalsymptomsandfindings.Thesearediscussedseparatelyfor
eachofthetumorspresentedinthefollowingsection.
TumorsofthelacrimalglandarediscussedinChapter3,LacrimalSystem.
15.7.1.1Hemangioma
Hemangiomasarethemostcommonbenignorbitaltumorsinbothchildren
andadults.Theyusuallyoccurinanasalsuperiorlocation.Capillaryheman-
giomasaremorecommoninchildren(theyswellwhenthechildscreams),
andcavernoushemangiomasaremorecommoninadults.Treatmentisonly
indicatedwherethetumorthreatenstooccludethevisualaxiswithresulting
amblyopiaorwherethereisariskofcompressiveopticneuropathy.Capillary
hemangiomasinchildrenmaybetreatedwithcortisoneorlow-doseradia-
tiontherapy.
15.7.1.2DermoidandEpidermoidCyst
Theselesionsarethemostcommonorbitaltumorsinchildren.Etiologically,
theyarechoristomas,i.e.,dermalorepidermalstructuresthathavebeendis-
placedintodeeperlayers.However,theyusuallyarelocatedanteriortothe
orbitalseptum(andthereforearenotintheactualorbititself).Lesionslocated
posteriortotheorbitalseptumusuallybecomeclinicallysignificantonlyin
adults.Treatmentconsistsofcompleteremoval.
15.7.1.3NeurinomaandNeurofibroma
ThesetumorsareoftenassociatedwithRecklinghausen’sdisease(neurofibro-
matosis).Iftheyoccurintheopticcanal,theymustberemovedbeforethey
causecompressiveopticneuropathy.
15.7.1.4Meningioma
Ameningiomacanproceedfromtheopticnerve(meningiomaoftheoptic
nervesheath)orfromwithinthecranium(sphenoidmeningioma).Symp-
tomsvarydependingonthelocationofthetumor.Exophthalmos,limited
motility,andcompressiveopticneuropathycanresult.Hyperostosesare
frequentfindingsinradiographicstudies.Treatmentconsistsofneurosurgi-
calremovalofthetumor.Likeneurinomas,16%ofallmeningiomasare
15OrbitalCavity
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439. 421
associatedwithneurofibromatosis(Recklinghausen’sdisease).Meningiomas
oftheopticnervesheathareusuallyhistologicallybenignbutcanrecurifnot
completelyremoved.Interestingly,theaverageageofpatientsis32;20%are
youngerthan20.
15.7.1.5HistiocytosisX
ThisisagenerictermfortheproliferationofLangerhans’cellsofundeter-
minedetiology;allthreeofthefollowingtypescancauseexophthalmos
wherethereisorbitalinvolvement:
!Letterer-Siwedisease(malignant).
!Hand-Schüller-Christiandisease(benign).
!Eosinophilicgranuloma(rareandbenign).
15.7.1.6LeukemicInfiltrations
Leukemicinfiltrationsoccurespeciallyinacutelymphoblasticleukemiaand
inaspecialformofmyeloidleukemia(granulocyticsarcomaorchloroma).
Inflammationispresentinadditiontoexophthalmos.
15.7.1.7Lymphoma
Lymphomascanoccurinisolationorinsystemicdisease.Cooperationwith
anoncologistisrequired.Thedisordermaybetreatedbyradiationtherapyor
chemotherapy.Usuallythesetumorsareonlyslightlymalignant.Thehighly
malignantBurkitt’slymphoma,whichhasahighaffinityfortheorbitalcav-
ity,isanotableexception.
15.7.1.8Rhabdomyosarcoma
Thisisthecommonestprimarymalignanttumorinchildren.Thetumoroften
growsveryrapidly.Becauseoftheaccompanyinginflammation,adifferential
diagnosisshouldexcludeorbitalcellulitis.Otherindicateddiagnosticstudies
includeaCTscanandpossiblyabiopsy.Withmoderntherapeuticregimes
suchaschemotherapyandradiationtherapy,curativetreatmentispossiblein
manycases.
15.7.2Metastases
Inchildren,theincidenceofmetastasisishigherintheorbitalcavitythanin
thechoroid.Inadults,itisexactlytheopposite.Themostcommonorbital
metastasesinchildrenoriginatefromneuroblastomas.Malignanttumors
fromadjacenttissuecanalsoinvadetheorbitalcavity.
15.7Tumors
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440. 422
15.7.3OpticNerveGlioma
Inchildren,thisisthesecondmostcommonpotentiallymalignantorbital
tumor.In25%ofallpatients,theopticnervegliomaisassociatedwithneuro-
fibromatosis(Recklinghausen’sdisease).Fifteenpercentofallpatientswith
neurofibromatosisdevelopopticnervegliomas.Theprognosisisgoodonly
wherethetumoriscompletelyresected.
Injuries
SeeChapter18.
15.8OrbitalSurgery
Accesstotheorbitalcavityisgainedprimarilythroughananteriorapproach
(transconjunctivalortranspalpebralapproachesyieldgoodcosmeticresults)
orthroughalateralapproach.ThelateralKrönleinapproachprovidesbetter
intraoperativeexposure.Transantral,transfrontal,transcranial,andtrans-
nasalorbitotomiesareusedlessfrequently.
Orbitalexenterationisindicatedwithadvancedmalignanttumors.This
involvesremovaloftheentirecontentsoftheorbitalcavityincludingtheeye-
lids.
15OrbitalCavity
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441. 423
16OpticsandRefractiveErrors
ChristophW.SpraulandGerhardK.Lang
16.1BasicKnowledge
16.1.1UncorrectedandCorrectedVisualAcuity
Uncorrectedvisualacuity:Thisreferstotheresolvingpoweroftheeye
withoutcorrectivelenses.
Correctedvisualacuity:Thisreferstotheresolvingpoweroftheeyewithan
optimalcorrectionprovidedbycorrectivelenses(determinedbyvisualacu-
itytesting).
Bothuncorrectedvisualacuityandcorrectedvisualacuityprovideinfor-
mationonhowfaraparttwoobjectsmustbefortheeyetoperceivethemas
distinctobjects(minimumthresholdresolution).Fortheeyetoperceivetwo
objectsasdistinct,atleastoneunstimulatedconemustliebetweentwo
stimulatedconesontheretina.Theconedensityisgreatestinthecenterof
theretinaandcentralvisualacuityishighest.Theretheconesarespaced
only2.5µmapart.Thisintervalincreasestowardtheperipheryoftheretina,
andbothuncorrectedvisualacuityandcorrectedvisualacuitydecrease
accordingly.Conespacingandphysicaleffectssuchasdiffractionandoptical
aberrationslimittheaverageminimumthresholdresolution,theminimum
visualangletooneminuteofarc(theindividualmaximumvalueisapproxi-
mately30secondsofarc).Oneminuteofarcis1/60ofadegreeorapproxi-
mately0.004mm,whichissomewhatmorethanthewidthofacone.This
correspondstothemaximumresolvingpoweroftheretina(Fig.16.1).
16.1.2Refraction:EmmetropiaandAmetropia
Refractionisdefinedastheratiooftherefractivepowerofthelensandcornea
(therefractivemedia)totheaxiallengthoftheglobe.Emmetropiaisdistin-
guishedfromametropia.
Emmetropia(normalsight):Theratiooftheaxiallengthoftheeyetothe
refractivepowerofthecorneaandlensisbalanced.Parallellightraysthat
entertheeyethereforemeetatafocalpointontheretina(Figs.16.2and
16.6a)andnotanteriororposteriortoit,asisthecaseinametropia.
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442. 424
k
k
0.5–1 minutes of arc
(approx. 0.5–1/60
degrees)
Photoreceptors
O
1
O
2
k
k
y
x
z
Resolutionoftheeye(minimumthresholdresolution).
Fig.16.1Twopoints(O1andO2)canonlybeperceivedasdistinctifatleastoneun-
stimulatedcone(z)liesbetweentwostimulatedcones(xandy)ontheretina.Dueto
opticalaberrationsanddiffraction,apunctiformobjectisreproducedasacircle(k).
Thisresultsinamaximumresolutionoftheeyeof0.5–1minutesofarcor0.5/60–
1/60ofadegree.Thedrawingisnottoscale.
Focalpointinemmetropiaandametropia.
Fig.16.2Parallelraysoflightenteringtheeyefromanopticallyinfinitedistance
meetatafocalpointontheretinainemmetropia(blacklines).Inhyperopia,this
focalpoint(II)liesposteriortotheretina(greenlines).Inmyopia(I),itliesanteriorto
theretina(redlines).
Ametropia(refractiveerror):Thereisamismatchbetweentheaxiallength
oftheeyeandtherefractivepowerofthelensandcornea.Theametropiais
eitheraxial,whichiscommon,orrefractive,whichislessfrequentlyencoun-
tered.Themostcommondisordersarenearsightedness,farsightedness,and
astigmatism.
16OpticsandRefractiveErrors
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443. 425
Veryfewpeoplehaverefractionofexactly!0.0diopters.Approximately
55%ofpersonsbetweentheagesof20and30haverefractionbetween+1and
–1diopters.
Emmetropiaisnotnecessarilyidenticaltogoodvisualacuity.Theeye
mayhaveotherdisordersthatreducevisualacuity,suchasatrophyof
theopticnerveoramblyopia.
Therefractivepowerofanopticallenssystemisspecifiedindiopters,which
aretheinternationalunitsofmeasure.Refractivepoweriscalculatedaccord-
ingtothelawsofgeometricoptics.AccordingtoSnell’slaw,therefractionof
theincidentlightrayisdeterminedbytheangleofincidenceanddifference
intherefractiveindicesnofthetwomedia(Table16.1).
Themaximumtotalrefractivepowerofanemmetropiceyeis63diopters
withanaxiallengthoftheglobemeasuring23.5mm.Thecorneaaccountsfor
43dioptersandthelensfor10–20diopters,dependingonaccommodation.
However,therefractivepoweroftheeyeisnotsimplythesumofthesetwo
values.Theopticmediathatsurroundtheeye’slenssystemandthedistance
betweenthelensandcornearenderthetotalsystemmorecomplex.
TherefractivepowerD(specifiedindiopters)ofanopticalsystemisthe
reciprocalofthefocallengthofalensf(specifiedinmeters).Thisyields
theequation:D=1/f.
Example:Wherealensfocusesparallelincidentlightrays0.5mbehindthe
lens,therefractivepoweris1/0.5m=+2diopters.Thisisaconverginglens.
Wherethevirtualfocalpointisinfrontofthelens,therefractivepoweris
1/–0.5m=–2diopters.Thisisadiverginglens(Fig.16.3).
16.1.3Accommodation
Therefractivepoweroftheeyedescribedintheprevioussectionisnotacon-
stantvalue.Theeye’srefractivepowermustaltertoallowvisualizationof
Table16.1Importantrefractiveindicesnofthevarioustissuesoftheeye(fromKrause,
K.MethodenderRefraktionsbestimmung.Biermann,Münster,Germany,1985)
Eyetissue Refractiveindexn
Cornea
Aqueoushumor
Lensatthepoles
Lensatthecore
Vitreousbody
1.376
1.336
1.385
1.406
1.336
16.1BasicKnowledge
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444. 426
Refractionoflightraystravelingthroughconverginganddiverginglenses.
a b
Fig.16.3aTheconverginglens(biconvex)concentratesincidentlightraysata
focalpointbehindthelens.bAdiverginglens(biconcave)ensuresthatthelightrays
donotmeetatall.Thelightraysappeartooriginateatavirtualfocalpointinfrontof
thelens.
bothnearanddistantobjectswithsharpcontours.Thisaccommodationis
madepossiblebytheelasticityofthelens.
Accommodationmechanisms:Accommodationinvolvesthelens,zonule
fibers,andciliarymuscle.
!Lens:Thesolubleproteinsofthelensaresurroundedbyathinelasticcap-
sule.Thecurvatureoftheposteriorcapsuleofthelensisgreaterthanits
anteriorcurvature,withaposteriorradiusof6.0mmasopposedtoan
anteriorradiusof10.0mm.Theintrinsicelasticityofthelenscapsuletends
tomakethelensassumeasphericalshape.However,intheunaccommo-
datedstatethisispreventedbythepullofthezonulefibers.Theelasticity
oftheinnertissueofthelensprogressivelydecreaseswithagedueto
depositsofinsolubleproteins.
!Zonulefibers:Theradiatingzonulefibersinsertintotheequatorofthe
lensandconnectittotheciliarybody.Theyholdthelenssecurelyinposi-
tionandtransmitthepulloftheciliarymuscletothelens.
!Ciliarymuscle:Contractionofthering-shapedciliarymuscledecreasesthe
tensioninthezonulefibers.Thelenscanthenapproachthespherical
shape(witharadiusofcurvatureof5.3mm)thatitsphysicalconfiguration
andchemicalcompositionwouldotherwisedictate.Thischangeinthe
curvatureofthelensisespeciallypronouncedinitsanteriorsurface.The
deformationincreasestherefractivepower;thefocusoftheeyeshiftstothe
nearfield(Fig.16.4),andcloseobjectstakeonsharpcontours.Astheciliary
musclerelaxes,thetensiononthelensincreasesandthelensflattens.The
resultingdecreaseinrefractivepowershiftsthefocusoftheeyeintothedis-
tance(Fig.16.4),anddistantobjectstakeonsharpcontours.
Theciliarymuscleisinnervatedbytheshortciliarynerves,postganglionic
parasympatheticfibersoftheoculomotornerve.Parasympatholyticssuchas
atropine,scopolamine,andcyclopentolateinhibitthefunctionoftheciliary
muscleandthereforepreventaccommodation.Referredtoascycloplegics,
16OpticsandRefractiveErrors
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445. 427
Morphologicchangesinaccommodation.
Ciliary muscle
Accommodation
No accommodation
Fig.16.4Upperhalfoffigure:Inaccommodation,thelensbecomesincreasingly
globular.Thecurvatureoftheanteriorsurfaceinparticularincreases.Theciliary
muscleisshiftedslightlyanteriorly,andtheanteriorchamberbecomesshallower.
Objectsinthenearfield(continuousline)arerepresentedontheretinawithsharp
contours.
Lowerhalfoffigure:Withtheciliarybodyrelaxed,parallelincidentlightrays
(dottedline)arefocusedontheretina.Distantobjectsarerepresentedontheretina
withsharpcontours.
thesemedicationsalsocausemydriasisbyinhibitingthesphincterpupillae.
Parasympathomimeticssuchaspilocarpinecausetheciliarymuscleand
sphincterpupillaetocontract,producingmiosis.
Whentheciliarymuscleisatrest,thezonulefibersareundertension
andtheeyefocusesondistantobjects.
Accommodationisregulatedbyacontrolloop.Thecontrolvariableisthe
sharpnessoftheretinalimage.Thesystempresumablyusesthecolordisper-
sionoftheretinalimagetodeterminethedirectioninwhichaccommodation
shouldbecorrected.
Rangeofaccommodation:Thisspecifiesthemaximumincreaseinrefractive
powerthatispossiblebyaccommodationindiopters(Fig.16.5).Inmathemati-
calterms,therangeofaccommodationisobtainedbysubtractingnear-point
refractivepowerfromfar-pointrefractivepower.Thenearpointisshortest
distancethatallowsfocusedvision;thefarpointdescribesthefarthestpoint
thatisstilldiscernibleinfocus.Thenearandfarpointsdefinetherangeof
accommodation;itsspecificlocationinspaceisafunctionoftherefractive
poweroftheeye.
Example:Inonepatient,thenearpointliesat0.1mandthefarpointat1m.
Thispatient’srangeofaccommodationisthen10diopters–1diopter=9
diopters.
16.1BasicKnowledge
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446. 428
Inanemmetropiceye,thefarpointisatopticalinfinity.However,accom-
modationcanalsobringnear-fieldobjectsintofocus(Fig.16.6b).Theelastic-
ityofthelensdecreaseswithincreasingage,andtherangeofaccommodation
decreasesaccordingly(Fig.16.5).Presbyopia(physiologiclossofaccommo-
dationinadvancingage)beginswhentherangeofaccommodationfallsbelow
3diopters.Thegraduallossofaccommodationcausesthenearpointto
recede;thatpatient’sarmsbecome“tooshortforreading”.Dependingonage
andlimitationofaccommodation,presbyopiacanbecompensatedforwith
converginglensesof0.5–3diopters(seeFig.16.6candd).
16.1.4AdaptationtoDifferencesinLightIntensity
Likeacamera,theeye’sapertureandlenssystemalsoautomaticallyadaptsto
differencesinlightintensitytoavoid“overexposure”.Thisadjustmentis
effectedbytwomechanisms.
1.Theirisactsasanaperturetocontroltheamountoflightenteringthe
eye.Thisregulationtakesaboutonesecondandcanchangethelightinten-
sityontheretinaoverarangeofaboutapoweroften.
2.Thesensitivityoftheretinachangestoadapttodifferencesinlightinten-
sity.Thesensitivityoftheretinatolightisafunctionoftheconcentrationof
photopigmentinthephotoreceptorsandoftheneuronalactivityofthereti-
nalcells.Thechangeinneuronalactivityisarapidprocessthattakesonlya
fewmillisecondsandcanalterthelightsensitivityoftheretinaovera
rangeofthreepowersoften.Thechangeintheconcentrationofphotopig-
menttakesseveralminutesbutcancoverawiderangeofretinallightsen-
sitivity,asmuchaseightpowersoften.
Rangeofaccommodationindioptersasafunctionofage.
Age in years
Range of accommodation
!
D max
16
dpt
12
10
8
6
4
2
0
010203040506070
Fig.16.5Whentherangeofaccommoda-
tionfallsbelow3diopters,apreviousem-
metropicpatientwillrequireeyeglassesfor
reading(adaptedfromGoersch1987).
16OpticsandRefractiveErrors
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447. 429
Refractionintheemmetropiceye.
a
c
b
d
Fig.16.6aParallellightraysenteringtheeyefromopticalinfinityarefocusedon
theretinainanunaccommodatedeye.
bAccommodationfocusesthelightraysfromacloseobjectontheretina,andthe
objectisvisualizedwithsharpcontours.
cWhereaccommodationisinsufficient,asinadvancedage,closeobjectsappear
blurred.
dAconverginglensisrequiredtocorrectinsufficientaccommodationfornearvision
inadvancingage.
16.2ExaminationMethods
Visualacuity:seeChapter1.
16.2.1RefractionTesting
Refractiontestingmeansmeasuringtheadditionalrefractivepowerrequired
toproduceasharpimageontheretina.Subjectiveandobjectivemethodsare
used.Subjectivemethodsrequireinformationfromthepatient.
Subjectiverefractiontesting:Thisconsistsofsuccessivelyplacingvarious
combinationsoflensesbeforethepatient’seyeuntilthemaximumvisual
acuityisreached(seeCorrectionofRefractiveErrors).
Objectiverefractiontesting:Objectivetestingisunavoidablewhenthe
patientisunabletoprovidesubjectiveinformation(forexamplewithinfants)
orwhenthisinformationisunreliable.Thismethodalsogreatlyaccelerates
subjectiverefractivetesting.
16.2ExaminationMethods
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448. 430
Retinoscopy(shadowtesting):Theretinaisilluminatedthroughthepupil.
Theexaminerobservestheopticalphenomenainthepatient’spupilwhile
movingthelightsource(Fig.16.7).
Objectivedeterminationofrefractivepowerwitharetinoscope.
Retinoscope
Retinoscope
Fig.16.7
16OpticsandRefractiveErrors
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449. 431
Refractometry.Themeasuringprincipleisbasedonophthalmoscopicobser-
vationofatestimageprojectedontothepatient’sretina.Thedistance
betweenthetestfigureandtheeyeischangeduntiltheimageappearsin
focusontheretina.Refractioncanthenbecalculatedfromthemeasured
values.Analternativetochangingofthedistanceistoplacevariouslensesin
thepathofthelightbeam.
Automatedrefractometry.Themethodmeasuresrefractionautomatically
withtheaidoflight-sensitivedetectorsandacomputeruntilafocusedimage
appearsontheretina.Thesesystemsoperatewithinfraredlight.
Anyobjectivemeasurementsofrefractionshouldbeverifiedbysubjec-
tivetestingwheneverpossible.
16.2.2.TestingthePotentialResolvingPoweroftheRetinainthe
PresenceofOpacifiedOcularMedia
Specialexaminationmethodsareindicatedinthepresenceofopacificationof
theocularmediaoftheeye(suchasacataract)todeterminethepotential
visualacuityoftheretina.Thispermitstheophthalmologisttoestimate
whetheroptimizingtherefractivemediawithtechniquessuchascataract
surgeryorcornealtransplantationwouldachievethedesiredimprovement.
Laserinterferencevisualacuitytesting:Lasersareusedtoprojectinference
stripsofvaryingwidthsontotheretina.Thepatientmustspecifythedirec-
tioninwhichtheseincreasingnarrowerstripsarealigned.Thisexamination
cannolongerbeperformedwherethereissevereopacificationoftheoptic
mediasuchasinamaturecataract.Thepreliminaryexaminationthencon-
sistsofevaluatingthepatternofthetransilluminatedretinalvasculature.
!Fig.16.7Withtheretinoscope,theexaminermovesalightsource(abeamofyellow
light)acrossthepupil(darkspot)atadistanceofabout50cmfromthepatient.Thispro-
ducesalightreflex(redspot)inthepatient’seye.Itisimportanttonotehowthislightre-
flex(redspot)behavesasthelightsourceoftheretinoscopeismoved.Therearetwo
possibilities:
a“With”motion:thelightreflexinthepupil(redspot)movesinthesamedirection(red
arrows)asthelightsourceoftheretinoscope(yellowarrows).Thismeansthatthefar
pointoftheeyeisbehindthelightsource.b“Against”motion.Thelightreflexinthe
pupilmovesintheoppositedirection(redarrows)tothelightsourceoftheretinoscope
(yellowarrows).Thismeansthatthefarpointoftheeyeliesbetweentheeyeandthelight
source.Theexaminerplacesappropriatelensesinfrontofthepatient’seyes(pluslenses
for“with”motionandminuslensesfor“against”motion)untilnofurthermotionofthe
lightreflexisobserved.Themotionoftheretinoscopewillthenonlyelicitaninfinitely
fastreflex(neutralpoint).Thismethodisusedtodeterminetheproperlensforcorrect-
ingtherefractiveerror.
16.2ExaminationMethods
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450. 432
16.3RefractiveAnomalies(Table16.2)
16.3.1Myopia(Shortsightedness)
Definition
Adiscrepancybetweentherefractivepowerandaxiallengthoftheeyesuch
thatparallelincidentlightraysconvergeatafocalpointanteriortotheretina
(Fig.16.8a).
Epidemiology:Approximately25%ofpersonsbetweentheagesof20and30
haverefractionlessthan–1diopters.
Refractioninmyopia.
ba
e
c
d
Fig.16.8aThefocalpointofparallellightraysenteringtheeyeliesanteriortothe
retina.bOnlycloseobjectsfromwhichthelightraysdivergeuntiltheyentertheeye
arefocusedontheretinaandappearsharplydefined.Thefarpointisafinitedis-
tancefromtheeye.cAxialmyopia:normalrefractivepowerinanexcessivelylong
globe.dRefractivemyopia:excessiverefractivepowerinanormal-lengthglobe.
eNuclearcataractwithasecondaryfocalpoint(patientseesdouble).
16OpticsandRefractiveErrors
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451. 433
T
able
1
6
.
2
Ov
er
view
o
f
t
he
most
impor
t
ant
refractiv
e
anomalies
R
efr
activ
e
anomaly
F
ocal
point
of
par-
allel
incident
light
r
a
y
s
Causes
Vision
P
ossible
complica-
tions
Optical
correction
My
opia
(near
sight
edness)
Ant
erior
t
o
the
r
e
t
in
a
!
Ey
eball
t
o
o
long
(axial
m
y
opia).
!
Ex
cessiv
e
r
efrac-
tiv
e
p
o
w
er
(refractiv
e
m
y
opia).
!
V
e
r
y
good
near
vision.
!
P
oor
dist
ance
vision.
!
Increased
risk
of
re
tinal
de
t
a
c
h
-
ment.
!
See
p.
434
f
o
r
complications specific
t
o
patho-
logic
m
y
opia.
Div
erging
lenses
(minus
or
conca
v
e
lenses).
Hyperopia (far
sight
edness)
P
o
s
t
e
r
io
r
t
o
t
h
e
r
e
t
in
a
!
Ey
eball
t
o
o
shor
t
(axial
h
yperopia).
!
Insuf
ficient
refractiv
e
p
o
w
er
(refractiv
e
h
yper-
opia).
!
P
oor
near
vision.
!
A
ccommodation
usually
permits
normal
dist
ance
vision
(in
y
oung
patients
and
in
slight
t
o
mod-
erat
e
h
yperopia).
!
Disposition
t
o
acut
e
angle
clo-
sure
g
laucoma
(shallo
w
ant
erior
c
hamber).
Cau-
tion
is
advised
with
diagnostic
and
therapeutic
m
ydriasis.
!
Eso
tropia
Con
v
e
rging
lenses
(plus
or
con
v
e
x
lenses).
Astigmatism
Lac
k
o
f
a
f
ocal
point
Anomalies
in
the
cur
v
ature
o
f
t
he
n
o
r
m
a
lly
s
p
h
e
r
ic
a
l
s
u
r
f
a
c
e
s
o
f
t
h
e
refractiv
e
media
(cornea
and
lens).
P
atients
see
e
v
er
y-
thing
dist
o
r
t
ed.
Risk
of
refractiv
e
ambly
opia.
Cylindrical
lenses;
e
y
eglass
correction
is
only
possible
where
a
stigmatism
is
regular
.
16.3RefractiveAnomalies
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452. 434
Etiology:Theetiologyofmyopiaisnotclear.Familialpatternsofincreased
incidencesuggesttheinfluenceofgeneticfactors.
Pathophysiology:Whereasparallelincidentlightraysconvergeatafocal
pointontheretinainemmetropiceyes,theyconvergeatafocalpointanterior
totheretinainmyopiceyes(Fig.16.8a).Thismeansthatnosharplydefined
imagesappearontheretinawhenthepatientgazesintothedistance
(Fig.16.8a).Themyopiceyecanonlyproducesharplydefinedimagesofclose
objectsfromwhichthelightraysdivergeuntiltheyentertheeye(Fig.16.8b).
Thefarpointmovescloser;inmyopiaof–1diopteritliesatadistanceof1m.
Inmyopia,thefarpoint(distancefromtheeye=A)canbecalculated
usingtheformula:A(m)=1/D,whereDismyopiaindiopters.
Possiblecausesincludeanexcessivelylongglobewithnormalrefractivepower
(axialmyopia;Fig.16.8c)and,lessfrequently,excessiverefractivepowerina
normal-lengthglobe(refractivemyopia;Fig.16.8d).
Adifferenceinglobelengthof1mmwithrespecttoanormaleyecorre-
spondstoadifferenceofabout3dioptersinrefractivepower.
Specialformsofrefractivemyopia:
!Myopicsclerosisofthenucleusofthelens(cataract)inadvancedage(see
p.!).Thiscausesasecondaryfocalpointtodevelop,whichcanleadto
monoculardiplopia(doublevision).
!Keratoconus(increaseintherefractivepowerofthecornea).
!Spherophakia(sphericallyshapedlens).
Forms:Theseinclude:
!Simplemyopia(school-agemyopia):Onsetisattheageof10–12years.
Usuallythemyopiadoesnotprogressaftertheageof20.Refractionrarely
exceeds6diopters.However,abenignprogressivemyopiaalsoexists,
whichstabilizesonlyaftertheageof30.
!Pathologicmyopia:Thisdisorderislargelyhereditaryandprogressescon-
tinuouslyindependentlyofexternalinfluences.
Symptomsanddiagnosticconsiderations:Thediagnosisismadeonthe
basisofatypicalclinicalpictureandrefractiontesting.Myopicpatientshave
verygoodnearvision.Whengazingintothedistance,theysquintinan
attempttoimprovetheiruncorrectedvisualacuitybyfurthernarrowingthe
opticapertureofthepupil.Theterm“myopia”comesfromthissquinting;the
Greekword“myein”meanstosquintorclosetheeyes.Oldermyopicpatients
canreadwithoutcorrectivelensesbyholdingthereadingmaterialatabout
thedistanceofthefarpoint.
Thetypicalmorphologicchangesoccurringinmyopiaarereferredtoas
myopiasyndrome.Progressivemyopiainparticularischaracterizedbythin-
ningofthesclera.Theelongationoftheglobecausesashiftintheaxesoftheeye.
16OpticsandRefractiveErrors
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453. 435
Correctionofmyopia.
a b c
Fig.16.9aCorrectionwithdiverginglenses(minuslenses).bCorrectionwithcon-
tactlens.cCorrectionbyremovingthelenstoreducerefractivepoweroftheeye.
Thisoftensimulatesesotropia.Theanteriorchamberisdeep.Atrophyofthe
ciliarymuscleispresentasitishardlyused.Thevolumeofthevitreousbodyis
toosmallforthelargeeye,anditmaycollapseprematurely.Thisresultsinvit-
reousopacificationsthatthepatientperceivesasfloaters.
Morphologicfunduschangesinmyopia,suchasmaculopathyandFuchs’
spot,arediscussedinSection12.4.6.
Theriskofretinaldetachmentisincreasedinmyopia.However,itdoesnot
increaseinproportiontotheseverityofthemyopia.
Becauseoftheincreasedriskofretinaldetachment,patientswith
myopiashouldbeexaminedparticularlythoroughlyforprodromal
signsofretinaldetachment,suchasequatorialdegenerationorretinal
tears.Therefore,examinationofthefunduswiththepupildilatedis
indicatedbothwhenthefirstpairofeyeglassesisprescribedandatreg-
ularintervalsthereafter.
Glaucomaismoredifficulttodiagnoseinpatientswithmyopia.Measure-
mentsofintraocularpressureobtainedwithaSchiøtztonometerwillbe
lowerthannormalduetothedecreasedrigidityofthesclera.
Applanationtonometryyieldsthemostaccuratevaluesinpatientswith
myopiabecausetherigidityofthescleraonlyslightlyinfluencesresults.
Theopticcupisalsodifficulttoevaluateinpatientswithmyopiabecausethe
opticnerveenterstheeyeobliquely.Thisalsomakesglaucomamoredifficult
todiagnose.
Treatment:Theexcessiverefractivepoweroftherefractivemediamustbe
reduced.Thisisachievedthroughtheuseofdiverginglenses(minusorcon-
cavelenses;Fig.16.9a).Theselensescauseparallelincidentlightraysto
divergebehindthelens.Thedivergentraysconvergeatavirtualfocalpointin
frontofthelens.Therefractivepower(D)isnegative(hencetheterm“minus
16.3RefractiveAnomalies
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454. 436
lens”)andisequalto1/f,wherefisthefocallengthinmeters.Previously,
biconcaveorplanoconcavelensblankswereusedinthemanufactureofcor-
rectivelenses.However,theseentailedanumberofopticaldisadvantages.
Todaylensesaremanufacturedinapositivemeniscusshapetoreducelens
aberrations.
Correctionwithcontactlenses(Fig.16.9b)offersopticaladvantages.The
reductioninthesizeoftheimageislessthanwitheyeglasscorrection.Aber-
rationsarealsoreduced.Theseadvantagesareclinicallyrelevantwithmyopia
exceeding3diopters.
Thecloserthe“minus”lensistotheeye,theweakeritsrefractivepower
mustbetoachievethedesiredopticeffect.
Minuslensestobeusedtocorrectmyopiashouldbenostrongerthanabso-
lutelynecessary.Althoughaccommodationcouldcompensateforanovercor-
rection,patientsusuallydonottoleratethiswell.Accommodativeasthenopia
(rapidocularfatigue)resultsfromtheexcessivestresscausedbychroniccon-
tractionoftheatrophicciliarymuscle.
Myopicpatientshave“lazy”accommodationduetoatrophyofthecili-
arymuscle.Averyslightundercorrectionisoftenbettertoleratedthan
aperfectlysharpimagewithminimalovercorrection.
Incertainspecialcases,removalofthecrystallinelens(Fig.16.9c)maybe
performedtoreducetherefractivepowerofthemyopiceye.However,this
operationisassociatedwithahighriskofretinaldetachmentandisrarely
performed.Thereisalsothepossibilityofimplantingananteriorchamber
intraocularlens(diverginglens)anteriortothenaturallenstoreducerefrac-
tivepower.SeeChapter5foradditionalsurgicaloptions.
Popularhealthbooksdescribeexercisesthatcanallegedlytreatrefractive
errorssuchasnearsightednesswithouteyeglassesorcontactlenses.Such
exercisescannotinfluencethesharpnessoftheretinalimage;theycanonly
seeminglyimproveuncorrectedvisualacuitybytrainingthepatienttomake
betteruseofadditionalvisualinformation.However,afterpubertynolate
sequelaeofchronicallyuncorrectedvisionaretobeexpected.
16.3.2Hyperopia(Farsightedness)
Definition
Inhyperopia,thereisadiscrepancybetweentherefractivepowerandaxial
lengthoftheeyesuchthatparallelincidentlightraysconvergeatafocalpoint
posteriortotheretina(Fig.16.10a).
Epidemiology:Approximately20%ofpersonsbetweentheagesof20and30
haverefractionexceeding+1diopters.Mostnewbornsexhibitslighthyper-
16OpticsandRefractiveErrors
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455. 437
opia(newbornhyperopia).Thisdecreasesduringthefirstfewyearsoflife.In
advancedage,refractiontendstoshifttowardthemyopicsideduetoscleros-
ingofthenucleusofthelens.
Etiology:Themechanismsthatcoordinatethedevelopmentoftheeyeballso
astoproduceopticmediaofagivenrefractivepowerarenotyetfullyunder-
stood.
Refractioninhyperopia.
a b
d
e f
c
Fig.16.10aThefocalpointofparallellightraysenteringtheeyeliesposteriorto
theretina.bDivergentlightraysarefocusedontheretina.Thevirtualfarpointlies
posteriortotheeye(dottedline).cTobringthefocalpointontotheretina,a
farsightedpersonmustaccommodateevenwhengazingintothedistance.dAxial
hyperopia:Refractivepowerisnormalbuttheglobeistooshort(morecommon).
eRefractivehyperopia:Theglobeisofnormallengthbutrefractivepowerisinsuffi-
cient(lesscommon).fAspecialformofrefractivehyperopiaisaphakia(absenceof
thelens).
16.3RefractiveAnomalies
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456. 438
Pathophysiology:Infarsightedpatients,thevirtualfarpointoftheeyelies
posteriortotheretina(Fig.16.10b).Onlyconvergentincidentlightrayscanbe
focusedontheretina(Fig.16.10b).Thisisdueeithertoanexcessivelyshort
globewithnormalrefractivepower(axialhyperopia;Fig.16.10d)or,less
frequently,toinsufficientrefractivepowerinanormal-lengthglobe(refrac-
tivehyperopia;Fig.16.10e).Axialhyperopiaisusuallycongenitalandis
characterizedbyashallowanteriorchamberwithathickscleraandwell
developedciliarymuscle.
Hyperopiceyesarepredisposedtoacuteangleclosureglaucoma
becauseoftheirshallowanteriorchamber.Thiscanbeprovokedby
diagnosticandtherapeuticmydriasis.
Specialformsofrefractivehyperopia:
!Absenceofthelens(aphakia)duetodislocation.
!Postoperativeaphakiafollowingcataractsurgerywithoutplacementofan
intraocularlens(seeFig.16.10).
Tobringthefocalpointontotheretina,afarsightedpersonmustaccommo-
dateevenwhengazingintothedistance(Fig.16.10c).Closeobjectsremain
blurredbecausetheeyeisunabletoaccommodateanyfurtherinnearvision.
Asaccommodationislinkedtoconvergence,thisprocesscanresultin
esotropia(accommodativeesotropiaoraccommodativeconvergentstra-
bismus).
Symptoms:Inyoungpatients,accommodationcancompensateforslightto
moderatehyperopia.However,thisleadstochronicoveruseoftheciliary
muscle.Readinginparticularcancauseasthenopicsymptomssuchaseye
painorheadache,burningsensationintheeyes,blepharoconjunctivitis,
blurredvision,andrapidfatigue.Esotropiacanalsooccur,aswasmentioned
above.Asaccommodationdecreaseswithadvancingage,nearvision
becomesincreasinglydifficult.Forthisreason,hyperopicpersonstendto
becomepresbyopicearly.
Diagnosticconsiderations:Ophthalmoscopicexaminationofthefundus
mayrevealaslightlyblurredopticdiskthatmaybeelevated(hyperopic
pseudoneuritis).However,thisisnotassociatedwithanyfunctionalimpair-
mentssuchasvisualfielddefects,lossofvisualacuity,orcolorvisiondefects.
Theretinaistoolargeforthesmalleye,whichleadstotortuousretinalvascu-
larstructures.Transitionstoabnormalformsofaxialshortening,suchasin
microphthalmos,arenotwelldefined.
Theciliarymuscleischronicallyundertensioninslightormoderate
hyperopiatocompensateforthehyperopia.Thisoveruseoftheciliarymuscle
leadstoaconditionofresidualaccommodationinwhichthemuscleisunable
torelaxevenafterthehyperopiahasbeencorrectedwithpluslenses.This
residualorlatenthyperopiamaybeoverlookedifrefractiontestingisper-
formedwithoutfirstcompletelyparalyzingtheciliarybodywithcycloplegic
16OpticsandRefractiveErrors
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457. 439
agentssuchascyclopentolateoratropine.Thefullextentofhyperopia
includesboththisresidualhyperopiaandclinicallymanifesthyperopia.
Inthepresenceofasthenopicsymptomsofuncertainorigin,refraction
testingundercycloplegiaisindicatedtoruleoutlatenthyperopia.
Treatment:Theinsufficientrefractivepowermustbeaugmentedwithcon-
verginglenses(plusorconvexlenses;Fig.16.11a).Awatch-and-wait
approachisindicatedwithasymptomaticyoungpatientswithslighthyper-
opia.Sphericalpluslensesconvergeparallelincidentlightraysatafocalpoint
behindthelens.Therefractivepower(D)inpluslensesispositive.Itisequal
to1/f,wherefisthefocallengthinmeters.Previously,biconvexorplanocon-
vexlensblankswereusedinthemanufactureofcorrectivelenses.However,
theseentailedanumberofopticaldisadvantages.Theopticalaberrationsof
thepositivemeniscuslensesusedtodayarecomparativelyslight.
Theclinicianshoulddeterminethetotaldegreeofhyperopiapresent(see
Diagnosticconsiderations)priortoprescribingcorrectivelenses.Thesecond
stepistoprescribethestrongestpluslensthatthepatientcantolerate
withoutcompromisingvisualacuity.Careshouldbetakentoavoidovercor-
rection.Thiswillcompensateforthemanifestcomponentofthehyperopia.If
thepatientwearsthesecorrectivelensespermanently,thenwithtimeitwill
alsobecomepossibletocorrectthelatentcomponent(seeDiagnosticcon-
Correctionofhyperopia.
a b
dc
Fig.16.11aCorrectionwithconverginglenses(pluslenses).b–dCorrectionof
aphakiawithcataractlens(b),contactlens(c),anteriorchamberintraocularlens
(d,blue)orposteriorchamberintraocularlens(d,red).
16.3RefractiveAnomalies
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458. 440
siderations).Thisisbecausethepermanenttensioninciliarybodyisno
longernecessary.
Priortoanycorrectionofhyperopia,refractiontestingshouldbeper-
formedafteradministeringcycloplegicstothepatient.Thecorrection
isthenmadewiththestrongestpluslensthatthepatientcansubjec-
tivelytoleratewithoutcompromisingvisualacuity.
Incontrast,refractiontestingtocorrectaphakiadoesnotrequirecycloplegia.
Here,too,pluslensesarerequiredtocorrectthehyperopia.Theclosertheplus
lensistotheretina,thestrongeritsrefractivepowermustbetoconvergeinci-
dentlightsatapointontheretina.Forthisreason,acataractlens(Fig.16.11b)
hasarefractivepowerofabout12diopters,acontactlens(Fig.16.11c)about
14diopters,ananterior-chamberintraocularlensabout20diopters
(Fig.16.11d),andaposterior-chamberlensabout23diopters.
16.3.3Astigmatism
Definition
AstigmatismisderivedfromtheGreekwordstigma(point)andliterallymeans
lackofafocalpoint.Thedisorderischaracterizedbyacurvatureanomalyof
therefractivemediasuchthatparallelincidentlightraysdonotconvergeata
pointbutaredrawnaparttoformaline.
Epidemiology:Forty-twopercentofallhumanshaveastigmatismgreater
thanorequalto0.5diopters.Inapproximately20%,thisastigmatismis
greaterthan1diopterandrequiresopticalcorrection.
Pathophysiology:Therefractivemediaoftheastigmaticeyearenotspheri-
calbutrefractdifferentlyalongonemeridianthanalongthemeridianperpen-
diculartoit(Fig.16.12).Thisproducestwofocalpoints.Therefore,apunc-
tiformobjectisrepresentedasasharplydefinedlinesegmentatthefocalpoint
ofthefirstmeridianbutalsoappearsasasharplydefinedlinesegment
rotated90degreesatthefocalpointofthesecondmeridian.Midway
betweenthesetwofocalpointsiswhatisknownasthe“circleofleastconfu-
sion.”Thisreferstothelocationatwhichtheimageisequallydistortedin
everydirection,i.e.,thelocationwiththeleastlossofimagedefinition.
Theaggregatesystemlacksafocalpoint.
Thecombinedastigmaticcomponentsofalloftherefractivemediacom-
prisethetotalastigmatismoftheeye.Thesemediainclude:
!Anteriorsurfaceofthecornea.
!Posteriorsurfaceofthecornea.
!Anteriorsurfaceofthelens.
!Posteriorsurfaceofthelens.
16OpticsandRefractiveErrors
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459. 441
Rarely,nonsphericalcurvatureoftheretinamayalsocontributetoastig-
matism.
Classificationandcauses:Astigmatismcanbeclassifiedasfollows:
!Externalastigmatism:astigmatismoftheanteriorsurfaceofthecornea.
!Internalastigmatism:thesumoftheastigmaticcomponentsoftheother
media.
Astigmatismcanalsobeclassifiedaccordingtothelocationofthemeridianof
greaterrefraction:
!With-the-ruleastigmatism(mostcommonform):Themeridianwiththe
greaterrefractivepowerisvertical,i.e.,between70and110degrees.
!Against-the-ruleastigmatism:Themeridianwiththegreaterrefractive
powerishorizontal,i.e.,between160and20degrees.
!Obliqueastigmatism:Themeridianwiththegreaterrefractivepoweris
oblique,i.e.,between20and70degreesorbetween110and160degrees.
Thediscussionuptothispointhasproceededfromtheassumptionthatthe
anomalyisaregularastigmatisminvolvingonlytwomeridiansapproxi-
matelyperpendiculartoeachother(Fig.16.12).Thisispresumablycausedby
excessiveeyelidtensionthatleadstoastigmaticchangesinthesurfaceofthe
cornea.
Theconditionaboveshouldbedistinguishedfromirregularastigmatism.
Here,thecurvatureandtherefractivepoweroftherefractivemediaare
completelyirregular(Fig.16.13a).Therearemultiplefocalpoints,whichpro-
ducesacompletelyblurredimageontheretina.Thisconditionmaybecaused
bythefollowingdiseases:
!Cornealulcerationswithresultingscarringofthecornea.
!Penetratingcornealtrauma.
Imageformationwithanastigmaticcornea.
Y'
Y'
Kr
Fig.16.12Thetwomainmeridians(IandII)areperpendiculartoeachother.A
punctiformobject(o)isrepresentedasalinesegmentY'IIandY'Iatthefocalpoints
ofthetwomeridians.Midwaybetweenthesetwofocalpointsisthe“circleofleast
confusion”(Kr),thelocationwiththeleastlossofimagedefinition.
16.3RefractiveAnomalies
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460. 442
!Advancedkeratoconus.
!Cataract.
!Lenticonus.
Symptoms:Patientswithastigmatismseeeverythingdistorted.Attemptsto
compensatefortherefractiveerrorbyaccommodationcanleadtoasthenopic
symptomssuchasaburningsensationintheeyesorheadache.
Diagnosticconsiderations:Thekeratoscope(Placidodisk)permitsgross
estimationofastigmatism.Theexaminerevaluatesthemirrorimagesofthe
ringsonthepatient’scornea.Inregularastigmatism,theringsareoval;in
irregularastigmatism,theyareirregularlydistorted.Computerizedcorneal
topography(videokeratoscopy)canbeusedtoobtainanimageofthedis-
tributionofrefractivevaluesovertheentirecornea(seeFig.5.3).AHelmholtz
orJavalophthalmometercanbeusedtomeasurethecentralcornealcurva-
ture,whichdeterminestherefractivepowerofthecornea(Fig.16.14).
Treatment:Earlycorrectioniscrucial.Untreatedastigmatisminchildrenwill
eventuallyleadtouncorrectablerefractiveamblyopiabecauseasharpimage
isnotprojectedontheretina.
Treatmentofregularastigmatism:Thepurposeofthecorrectionistobring
the“focallines”oftwomainmeridianstogetheratonefocalpoint.This
requiresalensthatrefractsinonlyoneplane.Cylinderlensesarerequiredfor
thisapplication(Fig.16.15a).Oncethetwo“focallines”havebeenconverged
intoafocalpoint,additionalsphericallensescanbeusedtoshiftthisfocal
pointontotheretinaifnecessary.
Irregularcornealastigmatism.
b
a
b
a
Fig.16.13aCurvatureandrefractivepoweroftherefractivemediaaretotallyir-
regular,resultinginmultiplefocalpoints.bCorrectionofirregularcornealastig-
matismwitharigidcontactlens.
16OpticsandRefractiveErrors
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461. 443
Diagnosisofcornealastigmatismwithanophthalmometer.
1 2
1 2
Fig.16.14Thediagramshowsthecornealrefleximages(outlinecross[1]andsolid
cross[2])oftheZeissophthalmometer.Theseimagesareprojectedontothecor-
nea;thedistancebetweenthemwillvarydependingonthecurvatureofthecornea.
Theexaminermustaligntheimagesbychangingtheirangleofprojection.After
aligningthem,theexaminerreadstheaxisofthemainmeridian,thecornealcurva-
tureinmillimeters,andtheappropriaterefractivepowerindioptersonascaleinthe
device.Thismeasurementisperformedinbothmainmeridians.Thedifference
yieldstheastigmatism.Inirregularastigmatism,theimagesaredistorted,andoften
ameasurementcannotbeobtained.
Correctionofregularastigmatismwithcylinderlenses.
0
0
90°
90°
180
180
a
b
c
d
Fig.16.15aCyl-
inderlensesrefract
lightonlyinthe
planeperpendicu-
lartotheaxisof
thecylinder.The
axisofthecylinder
definesthenonre-
fractingplane.
b–dCylinder
lensescanbeman-
ufacturedasplus
cylinders(c)or
minuscylinders
(d).
16.3RefractiveAnomalies
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462. 444
Treatmentofirregularastigmatism:Thisformcannotbecorrectedwitheye-
glasses.Externalastigmatismmaybemanagedwitharigidcontactlens
(Fig.16.13b),keratoplasty,orsurgicalcorrectionoftherefractiveerror.
Irregularinternalastigmatismisusuallylens-related.Inthiscase,removalof
thelenswithimplantationofanintraocularlensisindicated.
Onlyregularastigmatismcanbecorrectedwitheyeglasses.
16.3.4Anisometropia
Definition
Inanisometropia,thereisadifferenceinrefractivepowerbetweenthetwo
eyes.
Epidemiology:Anisometropiaofatleast4dioptersispresentinlessthan1%
ofthepopulation.
Etiology:Thereasonforthevaryingdevelopmentofthetwoeyesisnotclear.
Thisprimarilycongenitaldiseaseisknowntoexhibitafamilialpatternof
increasedincidence.
Pathophysiology:Inanisometropia,thereisadifferenceinrefractivepower
betweenthetwoeyes.Thisrefractivedifferencecanbecorrectedseparately
foreacheyewithdifferentlensesaslongasitliesbelow4diopters.Wherethe
differenceinrefractionisgreaterthanorequalto4diopters,thesizediffer-
enceofthetworetinalimagesbecomestoogreatforthebraintofusethetwo
imagesintoone.Knownasaniseikonia,thisconditionjeopardizesbinocular
visionbecauseitcanleadtodevelopmentofamblyopia(anisometropic
amblyopia).Theaniseikonia,ordifferingsizeoftheretinalimages,depends
notonlyonthedegreeofrefractiveanomalybutalsodependssignificantlyon
thetypeofcorrection.Theclosertothesiteoftherefractiondeficitthecor-
rectionismade,thelesstheretinalimagechangesinsize.Correctionwith
intraocularlensesresultsinalmostnodifferenceinimagesize.Contactlenses
produceaslightandusuallyirrelevantdifferenceinimagesize.However,eye-
glasscorrectionresultinginadifferenceofmorethan4dioptersleadstointol-
erableaniseikonia(seeTable7.4).
Symptoms:Anisometropiaisusuallycongenitalandoftenasymptomatic.
Childrenarenotawarethattheirvisionisabnormal.However,thereisaten-
dencytowardstrabismusasbinocularfunctionsmayremainunder-
developed.Wherethecorrectionoftheanisometropiaresultsinunac-
ceptableaniseikonia,patientswillreportunpleasantvisualsensationsof
doublevision.
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463. 445
Diagnosticconsiderations:Anisometropiaisusuallydiagnosedduring
routineexaminations.Thediagnosisismadeonthebasisofrefractiontesting.
Treatment:Therefractiveerrorshouldbecorrected.Anisometropiaexceed-
ing4diopterscannotbecorrectedwitheyeglassesbecauseoftheclinically
relevantaniseikonia.Contactlensesand,inrarecases,surgicaltreatmentare
indicated.Patientswithunilateralaphakiaorwhodonottoleratecontact
lenseswillrequireimplantationofanintraocularlens.
Correctionofunilateralaphakiawithunilateralglassesisusuallycon-
traindicatedbecauseitresultinaniseikoniaofapproximately25%.
16.4ImpairedAccommodation
16.4.1AccommodationSpasm
Definition
Anaccommodationspasmisdefinedasinadequateprotractedcontractionof
theciliarymuscle.
Etiology:Accommodationspasmsarerare.Theymayoccurasfunctional
impairmentortheymayoccuriatrogenicallywhentreatingyoungpatients
withparasympathomimeticagents(mioticagents).Thefunctionalimpair-
mentsarefrequentlyattributabletoheightenedsensitivityoftheaccommo-
dationcenter,whichespeciallyinchildren(oftengirls)canbepsychogenic.
Rarelythespasmisduetoorganiccauses.Inthesecases,itismostoften
attributabletoirritationintheregionoftheoculomotornuclei(fromcerebral
pressureorcerebraldisorders)ortochangeintheciliarymusclesuchasinan
ocularcontusion.
Symptoms:Patientscomplainofdeepeyepainandblurreddistancevision
(lenticularmyopia).
Diagnosticconsiderationsanddifferentialdiagnosis:Thediagnosisis
madeonthebasisofpresentingsymptomsandrefractiontesting,including
measurementoftherangeofaccommodation.Thisisdonewithanaccom-
modometer,whichdeterminesthedifferenceinrefractivepowerbetween
thenearpointandfarpoint.Adifferentialdiagnosisshouldexcludelatent
hyperopia.Inchildren,thiswillfrequentlybeassociatedwithaccommoda-
tiveesotropiaandaccommodativepupilnarrowing.
Treatment:Thisdependsontheunderlyingdisorder.Cycloplegictherapy
withagentssuchastropicamideorcyclopentolatemaybeattemptedinthe
presenceofrecurrentaccommodationspasms.
16.4ImpairedAccommodation
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464. 446
Prognosis:Iatrogenicspasmsarecompletelyreversiblebydiscontinuingthe
parasympathomimeticagents.Theprognosisisalsogoodforpatientswith
functionalcauses.Spasmsduetoorganiccausesrequiretreatmentoftheun-
derlyingdisorderbutoncetreatmentisinitiatedtheprognosisisusuallygood.
16.4.2AccommodationPalsy
Definition
Failureofaccommodationduetopalsyoftheciliarymuscle.
Etiology:Thisraredisorderisprimarilytooneofthefollowingcauses:
!Iatrogenicdrug-inducedpalsyduetoparasympatholyticagentssuchas
atropine,cyclopentolatescopolamine,homatropine,andtropicamide.
!Peripheralcauses:Oculomotorpalsy,lesionsoftheciliaryganglion,orthe
ciliarymuscle.
!Systemiccauses:Damagetotheaccommodationcenterindiphtheria,dia-
betesmellitus,chronicalcoholism,meningitis,cerebralstroke,multiple
sclerosis,syphilis,leadorergotaminepoisoning,medicationssuchas
isoniazidorpiperazine,andtumors.
Symptoms:Thefailureofaccommodationleadstoblurrednearvisionand
maybeassociatedwithmydriasiswherethesphincterpupillaemuscleisalso
involved.Theclinicalsyndromeslistedbelowexhibitaspecificconstellation
ofclinicalsymptomsandthereforewarrantfurtherdiscussion.
!Post-diphtheriaaccommodationpalsy:Thistransitorypalsyisatoxic
reactionandoccurswithoutpupillarydysfunctionapproximatelyfour
weeksafterinfection.Sometimesitisassociatedwithpalsyofthesoftpal-
ateand/orimpairedmotorfunctioninthelowerextremities.
!Accommodationpalsyinbotulism:Thisisalsoatoxicpalsy.Itdoes
involvethepupil,producingmydriasis,andcanbethefirstsymptomof
botulism.Itisassociatedwithspeech,swallowing,andocularmuscledys-
functionaccompaniedbydoublevision.
!Tonicpupillarycontractionisassociatedwithtonicaccommodation.
!Sympatheticophthalmiaischaracterizedbyadecreaseintherangeof
accommodation,evenintheunaffectedeye.
Measurementoftherangeofaccommodationisindicatedwhenever
sympatheticophthalmiaissuspected.
Diagnosticconsiderations:Inadditiontomeasuringtherangeofaccommo-
dationwithanaccommodometer,theexaminershouldinquireaboutother
ocularandgeneralsymptoms.
Treatment:Thisdependsontheunderlyingdisorder.
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465. 447
Prognosis:Theclinicalcourseoftonicpupillarycontractionischronicand
resultsinirreversiblelossofaccommodation.Thetoxicaccommodationpal-
siesarereversibleoncetheunderlyingdisorderiscontrolled.
16.5CorrectionofRefractiveErrors
16.5.1EyeglassLenses
MonofocalLenses
Therearetwobasictypes.
!Sphericallensesrefractlightequallyalongeveryaxis.
!Toriclenses(knownascylindricallenses)refractlightonlyalongoneaxis.
Sphericalandtoriclensescanbecombinedwhereindicated.
Therefractivepowerofthelensesismeasuredmanuallyorautomatically
withanopticalinterferometer.Themeasuredrefractionisspecifiedas
spherocylindricalcombination.Byconvention,thespecifiedaxisofthecylin-
dricallensisperpendiculartoitsaxisofrefraction(Fig.16.15candd).The
orientationofthisaxiswithrespecttotheeyeisspecifiedonastandardized
form(Fig.16.16).
Example:+4.00diopters–2.00diopters/90degreesmeansthatthelensrep-
resentsacombinationofconverginglens(+4diopters)andcylindricallens
(–2diopters)withitsaxisat90degrees.
Eyeglasslensesexhibittypicalcharacteristicswhenmovedbackand
forthafewinchesinfrontofone’seye.Objectsviewedthroughminus
lensesappeartomoveinthesamedirectionasthelens;objectsviewed
throughpluslensesmoveintheoppositedirection.Acylindricallens
producesimagedistortionswhenturned.
MultifocalLenses
Multifocallensesdifferfromthemonofocallensesofuniformrefractive
powerdiscussedintheprevioussectioninthatdifferentareasofthelens
havedifferentrefractivepowers.Theselensesarebestunderstoodascombi-
nationsoftwoormorelensesinasinglelens.
Bifocals:Theupperandmiddleportionofthelensisgroundforthedistance
correction;thelowerportionisgroundforthenear-fieldcorrection
(Fig.16.17aandb).Patientsareabletoviewdistantobjectsinfocusandread
usingonepairofeyeglasses,eliminatingtheneedtoconstantlychange
glasses.Thegazeisloweredandconvergedtoread.Thisportionofthelens
containsthenear-fieldcorrection.Thisnear-fieldcorrectioncanbeplacedin
adifferentpartofthelensforspecialapplications.
16.5CorrectionofRefractiveErrors
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466. 448
Eyeglassprescription.
R
Eyeglass prescription
for Mr./Mrs./M.
Spherical AxisPrism.Base
Vertex
distance
R
L
R
L
F
N
Typ of spectacles::
Comments:
Date
Signature
1
8
0
1
7
0
1
6
0
1
5
0
1 4
0
13 0
120
110
10090 80
7
0
6
0
5
0
4
0
3
0
2
0
1
0
0R
1
8
0
1
7
0
1
6
0
1
5
0
1 4
0
13 0
120
110
10090 80
7
0
6
0
5
0
4
0
3
0
2
0
1
0
0L
Cylindrical
Fig.16.16Therefractionvaluesfortherighteyehavebeenfilledin.Thecylindrical
axishasalsobeenentered(redline).Thediagramspecifiesthepositionofthecylin-
dricalaxiswithrespecttotheeye.Aperpendicularcylindricalaxis(redline)corre-
spondsto90degreesonthestandardform.
16OpticsandRefractiveErrors
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467. 449
Multifocallenses.
a b c
Fig.16.17aandbBifocals.cTrifocals.
Trifocals:Theselensesincludeathirdrefractivecorrectionbetweenthedis-
tanceandnear-fieldportions.Thisintermediateportionsharplyimagesthe
intermediatefieldbetweendistancevisionandreadingrangewithoutanyneed
foraccommodation(Fig.16.17c).
Progressiveadditionlenses:Theselensesweredevelopedtominimize
abruptimagechangeswhenthegazemovesthroughthedifferentcorrection
zonesofthelenswhilemaintainingasharpfocusateverydistance(Fig.16.18).
Theseeyeglassesalsooffercosmeticadvantages.Theyproducewell
focusedimagesinthecentralregionbuthaveahighdegreeofperipheralastig-
matism.However,manypatientslearntotoleratethisperipheraldistortion.
Presbyopicpatientstolerateprogressiveadditionlensesbetterwhen
theystillhaveonlyslightpresbyopiaandhavenotpreviouslywornbifo-
cals.
Progressiveadditionlenses.
Distance
correction
Intermediate
correction
Near-
field
correction
Fig.16.18Theselensesprovidea
smoothtransitionbetweenthedistance
correction(upperportion),interme-
diatecorrection(middleportion),and
near-fieldcorrection(lowerportion),
withcontinuouslyincreasingrefractive
power.Clearvisionislimitedtoonlythe
lightbluearea.ThestrongertheAddin
thenearfield,thenarrowerthislight
bluecorridorbecomes.Atthesame
time,thisincreasestheperipheralopti-
caldistortion(darkblue)outsidethe
corridor.
16.5CorrectionofRefractiveErrors
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468. 450
SpecialLenses
Thefollowingtypesoflenseshavebeendevelopedforspecialapplications:
Plasticlenses:Theselensesreducetheweightofeyeglasseswheresevere
ametropiamustbecorrected.Anotheradvantageisthattheselensesare
largelyshatterproof,whichiswhytheyarepreferredforchildren.However,
theyareeasilyscratched.
Absorptionlenses:Theselensesareindicatedinpatientswithincreasedsen-
sitivitytoglare.
Operatingmotorvehiclesintwilightoratnightwitheyeglassesthat
absorbmorethan20%ofincidentlightisdangerousbecauseofthe
resultingreductioninvisualacuity.
Photochromaticlenses:Theselensesdarkeninresponsetotheintensityof
ultravioletlight.Thelensesbecomedarkeratlowtemperaturesthanathigh
temperatures;theylightenmoreslowlyatlowtemperaturesandmore
rapidlyathightemperatures.Lightattenuationrangesbetween15and50%in
somelensesandbetween30and65%inothers.
Photochromaticlensesposeproblemsforpatientsoperatingmotor
vehicles.Thelensesdarkenonlyslightlyinawarmcarwiththewindows
closedduetothelackofultravioletlight.Darklenseslightentooslowly
whenthecarentersatunnel.
Coatedlenses:Extremelythincoatingsofmagnesiumfluoridecanbe
appliedtolensestoreducesurfacereflectiononthefrontandbackofthelens.
SubjectiveRefractionTestingforEyeglasses
Whilethepatientlooksatvisioncharts,theexaminerplacesvariouscombi-
nationsoflensesinfrontofthepatient’seye.Thepatientreportswhichoftwo
lensesproducesthesharperimage.Thebetterofthetwoisthencompared
withthenextlens.Thisincrementalmethodidentifiestheoptimalcorrec-
tion.Itisexpedienttousethepatient’sobjectiverefractionasthestarting
pointforsubjectivetesting.Refractiontestingisperformedeitherwitha
seriesoftestlensesfromacaseorwithaPhoroptor,whichcontainsmany
lensesthatcanbeautomaticallyormanuallyplacedbeforethepatient’seye.
Theexaminationproceedsinthreestages:
!Monoculartesting:Theoptimalrefractionforachievingbestvisualacuity
isdeterminedseparatelyforeacheye.Theweakestpossibleminuslensis
usedinmyopicpatients,andthestrongestpossiblepluslensinhyperopic
patients.Thered-greenchromaticaberrationtestcanbeusedforfine
refraction.Inthistest,thepatientcomparesoptotypesongreenandred
backgrounds.Fineadjustmentofrefractionpermitspreciseshiftingofthe
16OpticsandRefractiveErrors
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469. 451
focalpointofthelightontheretina.Optotypesonbothredandgreen
backgroundsthenappearequallysharplydefined.
!Binoculartesting:Theobjectiveofthisstageistoachieveabalance
betweenbotheyes.
!Nearpointtesting:Thefinalstageoftheexaminationdeterminesthe
patient’snearvisualacuity,and,ifnecessary,thepresbyopicaddition
(“Add”).Allowanceismadeforthepatient’spreferredreadingandworking
position.
Thevaluesdeterminedbythisexaminationareenteredintheeyeglasspre-
scription(seeFig.16.16).Thevertexdistanceatwhichrefractionwasper-
formedisanimportantadditionalparameterfortheoptician.Thisisthe
distancebetweenthebacksurfaceofthetestlensandtheanteriorsurfaceof
thecornea.Ifthemanufacturedeyeglasseshaveadifferentvertexdistance,
thenthestrengthofthelensesshouldbealteredaccordingly.Thisisbecause
theopticaleffectofeyeglasslensesvariesaccordingtothedistancefromthe
eye.
Beforethelensesarefittedintotheframe,thedistancebetweenthepupils
mustbemeasuredtoensurethatthelensesareproperlycentered.Thecenter
ofthelensshouldbeinfrontofthepupil.Theprismaticeffectsofeccentric
lensesmightotherwisecauseasthenopicsymptomssuchasheadacheora
burningsensationintheeyes.
Tofacilitateearlydetectionofglaucoma,intraocularpressureshouldbe
measuredinanypatientovertheageof40presentingforrefraction
testingforeyeglasses.
16.5.2ContactLenses
16.5.2.1AdvantagesandCharacteristicsofContactLenses
Contactlensesareinimmediatecontactwiththecornea.Althoughtheyare
foreignbodies,mostpatientsadapttoproperlyfittedcontactlenses.Contact
lensesdifferfromeyeglassesinthattheycorrecttherefractiveerrorcloserto
thelocationofitsorigin.Forthisreason,thequalityoftheopticalimage
viewedthroughcontactlensesishigherthanthatviewedthrougheyeglasses.
Contactlenseshavesignificantlylessinfluenceonthesizeoftheretinal
imagethandoescorrectionwitheyeglasses.Lensesdonotcloudupinrainy
weatherorsteam,andperipheraldistortionisminimized.Thecosmeticdis-
advantageofthickeyeglassesinsevereametropiaisalsoeliminated.Severe
anisometropiarequirescorrectionwithcontactlensesforopticalreasons,i.e.,
tominimizeaniseikonia.
Contactlensesaredefinedbythefollowingcharacteristics:
!Diameterofthecontactlens.
!Radiusofcurvatureoftheposteriorsurface.
16.5CorrectionofRefractiveErrors
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470. 452
!Geometryoftheposteriorsurface,i.e.,spherical,aspherical,complexcur-
vature,ortoric.
!Refractivepower.
!Material.
!Oxygenpermeabilityofthematerial(Dkvalue).
Thecornearequiresoxygenfromtheprecornealtearfilm.Toensurethis
supply,contactlensmaterialsmustbeoxygen-permeable.Thisbecomesallthe
moreimportantthelessthecontactlensmovesandpermitscirculationof
tearfluid.Contactlensesmaybemanufacturedfromrigidorflexiblemateri-
als.
RigidContactLenses
Thesecontactlenseshaveastable,nearlyunchangingshape.Patientstake
sometimetobecomeusedtothemandshouldthereforewearthemoften.The
goalistoachievethebestpossibleintimacyoffitbetweentheposteriorsur-
faceofthelensandtheanteriorsurfaceofthecornea(Fig.16.19).Thisallows
thecontactlenstofloatontheprecornealtearfilm.Everytimethepatient
blinks,thelensisdisplacedsuperiorlyandthenreturnstoitscentralposition.
Thispermitscirculationofthetearfilm.
Previously,polymethylmethacrylate(PMMA)wasusedasamaterial.
However,thisispracticallyimpermeabletooxygen.Thelenseswerefittedin
smalldiameterswithaveryshallowcurvature;thecentralareamaintained
contactwiththecorneawhiletheperipheryprojected.Thisallowedexcellent
tearfilmcirculation,andpatientswereabletowearthelensesforsurpris-
inglylongperiods.Today,highlyoxygen-permeablematerialssuchassilicone
Fitofarigidcontactlens.
Fig.16.19A
tearfilmliesbe-
tweentheante-
riorsurfaceofthe
corneaandthe
posteriorsurface
ofthelens
(visualizedby
fluoresceindye).
16OpticsandRefractiveErrors
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471. 453
copolymersareavailable.Thiseliminatesthetimelimitfordailywearing.
Theselensesmayalsoremainintheeyeovernightinspecialcases,suchas
aphakicpatientswithpoorcoordination(prolongedwearing).
Rigidcontactlensescanbemanufacturedassphericallensesandtoric
lenses.Sphericalcontactlensescanalmostcompletelycompensateforcor-
nealastigmatismoflessthan2.5diopters.Thisispossiblebecausethespace
betweentheposteriorsurfaceofthesphericalcontactlensandtheanterior
surfaceoftheastigmaticcorneaisfilledwithtearfluidthatformsa“tearlens.”
Tearfluidhasnearlythesamerefractiveindexasthecornea.Moreseverecor-
nealastigmatismorinternalastigmatismrequirescorrectionwithtoriccon-
tactlenses.Rigidcontactlensescanevencorrectseverekeratoconus.
SoftContactLenses
Thematerialofthecontactlens,suchashydrogel,issoftandpliable.Patients
findtheselensessignificantlymorecomfortable.Theoxygenpermeabilityof
thematerialdependsonitswatercontent,whichmayrangefrom36%to85%.
Thehigherthewatercontent,thebettertheoxygenpermeability.However,it
istypicallylowerthanthatofrigidlenses.Thematerialismorepermeableto
foreignsubstances,whichcanaccumulateinit.At12.5–16mm,flexible
lensesarelargerindiameterthanrigidlenses.Flexiblelensesareoftensup-
portedbythelimbus.Thelensisoftendisplacedonlyafewtenthsofamilli-
meterwhenthepatientblinks.Thisgreatlyreducesthecirculationoftearfilm
underthelenses.Thislimitsthemaximumdailyperiodthatpatientsareableto
wearthemandrequiresthattheyberemovedatnighttoallowregeneration
ofthecornea.Deviationfromthisprincipleisonlypossibleinexceptional
casesunderthestrictsupervisionofaphysician.
Asthelensesarealmostcompletelyincontactwiththesurfaceofthecor-
nea,cornealastigmatismcannotbecorrectedwithsphericalsoftlenses.This
requirestoricsoftlenses.
SpecialLenses
Thefollowingtypesofspeciallensesareavailableforspecificsituations:
Therapeuticcontactlenses:Inthepresenceofcornealerosion,softultra-
thin(0.05mm)contactlensesactasabandageandtherebyacceleratere-
epithelializationofthecornea.Theyalsoreducepain.Softcontactlensesmay
alsobeusedinpatientsreceivingtopicalmedicationastheystoremedication
andonlyreleaseitveryslowly.
Cornealshields:Thesearecollagendevicesthatresemblecontactlenses.
Theseshieldsaregraduallybrokendownbythecollagenaseinthetearfilm.
Theyareusedasbandagesandsubstratesfortopicalmedicationinthetreat-
mentofanteriordisorders,suchaserosionorulcer.
16.5CorrectionofRefractiveErrors
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472. 454
Irisprintlenses:Thesecoloredcontactlenseswithaclearcentralpupilare
usedinpatientswithaniridiaandalbinism.
Theyproducegoodcosmeticresults,reduceglare,andcancorrectarefractive
errorwhereindicated.
Bifocalcontactlenses:Theselensesweredevelopedtoallowtheuseofcon-
tactlensesinpresbyopicpatients.Asineyeglasses,anear-fieldcorrectionis
groundintothelens.Thisnear-fieldportionisalwayslocatedatthebottomof
thelensbecausethelensisheavierthere.Whenthepatientgazesdownward
toread,theimmobilelowereyelidpushesthisnear-fieldportionsuperiorly
whereitalignswiththepupilandbecomesopticallyeffective.Anotherpossi-
bilityisdiffraction(bendingoflightraysasopposedtorefraction)through
concentricringsontheposteriorsurfaceofthecontactlens.Thisproduces
twoimages,adistantrefractiveimageandanear-fielddiffractiveimage.The
patientchoosestheimagethatisimportantatthemoment.Itisalsopossible
tocorrectoneeyefordistancevisionandthefelloweyefornearvision
(monocularvision).
DisadvantagesofContactLenses
Contactlensesexertmechanicalandmetabolicinfluencesonthecornea.
Therefore,theyrequiretheconstantsupervisionofanophthalmologist.
Mechanicalinfluencesonthecorneacanleadtotransientchangesinrefrac-
tion.“Spectacleblur”canresultwheneyeglassessuddenlynolongerprovide
thepropercorrectionafterremovingthelens.Contactlensesrequirecareful
dailycleaninganddisinfection.Thisismoredifficult,time-consuming,and
moreexpensivethaneyeglasscareandisparticularimportantwithsoft
lenses.
Metabolicinfluencesonthecornea:Themacromolecularmeshofmaterial
absorbsproteins,proteinbreakdownproducts,low-molecular-weightsub-
stancessuchasmedicationsanddisinfectants,andbacteriaandfungi.Serious
complicationscanoccurwheredailycareofthecontactlensesisinadequate.
Withtheirthresholdoxygenpermeability,softcontactlensesinterferewith
cornealmetabolism.Contactlensesarelesssuitableforpatientswithsymp-
tomsofkeratoconjunctivitissicca.
ContactLensComplications
Complicationshavebeenobservedprimarilyinpatientswearingsoftcontact
lenses.Theseinclude:
Infectiouskeratitis(cornealinfiltrationsandulcers)causedbybacteria,
fungi,andprotozoans.
16OpticsandRefractiveErrors
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473. 455
Acanthamoebakeratitisisaseriouscomplicationaffectingwearersof
softcontactlensesandoftenrequirespenetratingkeratoplasty.
Giantpapillaryconjunctivitis:Thisisanallergicreactionofthepalpebral
conjunctivaoftheuppereyelidtodenaturedproteins.Itresultsinprolifera-
tive“cobblestone”conjunctivallesions.
Cornealvascularizationmaybeinterpretedastheresultofinsufficient
supplyofoxygentothecornea.
Severechronicconjunctivitis:Thisusuallymakesitimpossibletocontinue
wearingcontactlenses.
16.5.3Prisms
Prismscanchangethedirectionofparallellightrays.Theopticalstrengthofa
prismisspecifiedinprismdiopters.Prismlensescanbecombinedwith
sphericalandtoriclenses.Whenprescribingeyeglasses,theophthalmologist
specifiesthestrengthandthepositionofthebaseoftheprism.Prismlenses
areusedtocorrectheterophoria(latentstrabismus)andocularmusclepal-
sies,andinpreparationforsurgerytocorrectstrabismus.
A1diopterprismdeflectsarayoflight1cmatadistanceof1mfrom
thebaseoftheprism.
16.5.4MagnifyingVisionAids
Thereductionincentralcorrectedvisualacuityasaresultofdestructionof
thefoveawithacentralscotomarequiresmagnifyingvisionaids.However,
magnificationisalwaysassociatedwithareductioninthesizeofthevisual
field.Asaresult,thesevisionaidsrequirepatience,adaptation,motivation,
anddexterity.Cooperationbetweenophthalmologistandopticianisoften
helpful.Thefollowingsystemsareavailableinorderofmagnification.
Increasednear-fieldcorrections:Thestrongerthenear-fieldcorrection,the
shorterthereadingdistance.Magnification(V)isafunctionoftherefractive
powerofthenear-fieldcorrection(D)andisdeterminedbytheequation
V=D/4.
Example:Eyeglasseswitha10diopternear-fieldcorrectionmagnifythe
imagetwoandone-halfpowers.However,theobjectmustbebroughtto
within10cmoftheeye.
Magnifyingglassesareavailableinvariousstrengths,withorwithout
illumination.
16.5CorrectionofRefractiveErrors
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474. 456
Monocularandbinocularloupes,telescopes,andprismloupes:Anoptical
magnifyingsystemismountedononeorbotheyeglasslenses.Theoptical
systemfunctionsontheprincipleofGalileanorKeplerianoptics.
Closed-circuitTVmagnifier:Thisdevicedisplaystextatupto45power
magnification.
16.5.5AberrationsofLensesandEyeglasses
Opticallenssystems(eyeglassesorlenses)alwayshaveminoraberrations.
Theseaberrationsarenotmaterialflaws,rathertheyareduetothelawsof
physics.Expensiveopticalsystemscanreducetheseaberrationsbyusing
manydifferentlensesinaspecificorder.
ChromaticAberration(Dispersion)
Thismeansthattherefractivepowerofthelensvariesaccordingtothe
wavelengthofthelight.
Lightconsistsofablendofvariouswavelengths.Lightwithashort
wavelengthsuchasblueisrefractedmorethanlightwithalongwavelength
suchasred(Fig.16.20).Thisiswhymonochromaticlight(lightofasingle
wavelength)producesasharperimageontheretina.
Chromaticaberrationisthebasisofthered-greentestusedforfine
refractiontesting.
Chromaticaberration.
Fig.16.20Chro-
maticaberration
splitswhitelight
intoitscomponent
spectralcolors.Red
isrefractedleast,
andblueisre-
fractedmost.
16OpticsandRefractiveErrors
SphericalAberration
Thismeansthattherefractivepowerofthelensvariesaccordingtotheloca-
tionatwhichthelightraystrikesthelens.
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475. 457
Patientsmayreportbeingabletoseebetterwhenlookingthrougha
diskwithapinhole(astenopeicaperture)thanwithoutit.Thisusuallyis
asignofanuncompensatedrefractiveerrorintheeye.
Thefurtherperipherallythelightraystrikesthelens,themoreitwillbe
refracted(Fig.16.21).Theirisinterceptsalargeshareoftheseperipherallight
rays.Anarrowpupilwillinterceptaparticularlylargeshareofperipheral
lightrays,whichimprovesthedepthoffield.Conversely,depthoffieldissignif-
icantlypoorerwhenthepupilisdilated.
Patientswhohavereceivedmydriaticagentsshouldrefrainfromdriv-
ing.
Sphericalaberration.
Fig.16.21Dueto
sphericalaberration
therefractionof
lightraysincreases
thefurtherperiph-
erallytheystrikethe
lens.
16.5CorrectionofRefractiveErrors
AstigmaticAberration
Apunctiformobjectviewedthroughasphericallensappearsasaline.
Ifonelooksthroughalensobliquelytoitsopticalaxis,itwillactasaprism
(Fig.16.22a).Aprismrefractsalightraytowarditsbase(Fig.16.22b).Inaddi-
tiontothis,thelightissplitintoitscomponentspectralcolors.Lightwitha
shortwavelength(blue)isrefractedmorethanlightwithalongwavelength
(red).Astigmaticaberrationisanundesiredsideeffectthatispresent
wheneveronelooksthroughalensatanobliqueangle.
Thisphenomenonshouldbedistinguishedfromastigmaticortoriclenses,
whichcorrectforastigmatismoftheeyewhenthepatientslooksthrough
themalongtheopticalaxis.
CurvatureofField
Thismeansthatthemagnificationoftheimagechangesasoneapproaches
theperiphery.Theresultisasharpimagewithperipheralcurvature.Convex
orpluslensesproducepincushiondistortion;concaveorminuslensespro-
ducebarreldistortion(Fig.16.23).
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476. 458
Astigmaticaberration.
Apex
a b Base
Fig.16.22aLensesmayberegardedascomposedofmanyprisms,whichexplains
manyoftheopticalphenomenaoflensessuchasdispersion.bAprismrefractsa
lightraytowarditsbasetwice(solidline).However,itappearstotheobserverthat
theobjectisshiftedtowardtheapexoftheprism(dottedline).
Curvatureoffield.
Fig.16.23Viewinganobjectthroughpluslensesproducespincushiondistortion
oftheimage,whereasminuslensesproducebarreldistortion.
16OpticsandRefractiveErrors
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477. 459
17OcularMotilityandStrabismus
DorisRecker,JosefAmann,andGerhardK.Lang
Definition
Strabismusisdefinedasdeviationofaneye’svisualaxisfromitsnormalposi-
tion.
Therearetwomajortypesofmanifeststrabismusorheterotropia:
1.Concomitantstrabismus(fromtheLatin“comitare”,accompany).The
deviatingeyeaccompaniestheleadingeyeineverydirectionofmovement.
Theangleofdeviationremainsthesameinalldirectionsofgaze.Thisform
ofstrabismusmayoccurasmonocularstrabismus,inwhichonlyoneeye
deviates,orasalternatingstrabismus,inwhichbotheyesdeviatealter-
nately.
2.Paralyticstrabismusresultsfromparalysisofoneormoreeyemuscles.
Thisformdiffersfromconcomitantstrabismusinthattheangleofdevia-
tiondoesnotremainconstantineverydirectionofgaze.Forthisreason,
thisformisalsoreferredtoasincomitantstrabismus.
Epidemiology:Theincidenceofstrabismusisabout5–7%.Esotropia(con-
vergentstrabismus)occursfarmorefrequentlythanexotropia(divergent
strabismus)inEuropeandNorthAmerica.Concomitantstrabismususually
occursinchildren,whereasparalyticstrabismusprimarilyaffectsadults.
Thisisbecauseconcomitantstrabismusisgenerallycongenitaloracquired
withinthefirstfewyearsoflife,whereasparalyticstrabismusisusually
acquired,forexampleasapost-traumaticcondition.
17.1 BasicKnowledge
Ocularmotility:Themovementsoftheeyeballsareproducedbythefollow-
ingextraocularmuscles(Fig.17.1):
!Thefourrectusmuscles:thesuperior,inferior,medial,andlateralrectus
muscles.
!Thetwoobliquemuscles:thesuperiorandinferiorobliquemuscles.
Allofthesemusclesoriginateatthetendinousringexceptfortheinferior
obliquemuscle,whichhasitsoriginnearthenasolacrimalcanal.Therectus
musclesenvelopetheglobeposteriorly,andtheirrespectivetendonsinsert
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478. 460
Extraocularmusclesoftherighteye.
Superior rectus
Lateral
rectus
Medial
rectus
Inferior
rectus
Inferior
oblique
Superior
oblique
Inferior oblique
Optic
nerve
Tendinous ring
Trochlea
Tendon of the
superior oblique
Superior
oblique
Inferior rectus
Lateral rectus
Superior rectus
Medial rectus
Figs.17.1aandbThetwoobliqueocularmusclesinsertonthetemporalaspect
posteriortotheequator.Thefourrectusmusclesinsertonthesuperior,inferior,
nasal,andtemporalsclera.
intothesuperior,inferior,nasal,andtemporalsclera.Theobliquemuscles
insertintothetemporalglobeposteriortotheequator.Theinsertionofthe
musclesdeterminesthedirectionoftheirpull(seeTable17.1).
Theconnectivetissuebetweentheindividualocularmusclesisincor-
poratedintothefascialsheathoftheeyeball(Tenon’scapsule).Otherimpor-
tantanatomicstructuresincludethelateralandmedialcheckligaments
comprisingthelateralconnectionsoftheorbitalconnectivetissueandthe
ligamentofLockwood.Thisiscomprisedoftheligamentousstructures
betweentheinferiorrectusandinferiorobliquethatspreadoutlikeaham-
mocktothemedialandlateralrectusmuscles.
Theseanatomicstructuresandtheuniformnervesupplytotheextraocu-
larmuscles(likeactingmuscleshavelikenervesupply)ensureocular
balance.Changesthatdisturbthisbalance,suchasocularmuscleparalysis
thatlimitsordestroystheaffectedmuscle’sabilitytocontract,causestra-
bismus.Theangleofdeviationisasignofabnormalimbalance.
17OcularMotilityandStrabismus
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479. 461
Directionofpulloftheextraocularmuscles:Thehorizontalocularmuscles
pulltheeyeinonlyonedirection:Thelateralrectuspullstheeyeoutward
(abduction);themedialrectuspullsitinward(adduction).Allotherextraocu-
larmuscleshaveasecondarydirectionofpullinadditiontotheprimaryone.
Dependingonthepathofthemuscle,whereitinsertsontheglobe,andthe
directionofgaze(Fig.17.1),thesemusclesmayelevateordepresstheeye,
adductorabductit,orrotateitmedially(intorsion)orlaterally(extorsion).
Theprimaryactionofthesuperiorrectusandsuperiorobliqueiselevation;
theprimaryactionoftheinferiorrectusandinferiorobliqueisdepression.
Table17.1showstheprimaryandsecondaryactionsofthesixextraocular
muscles.Aknowledgeoftheseactionsisimportanttounderstandingpara-
lyticstrabismus.
Nervesupplytotheextraocularmuscles:Theoculomotornerve(third
cranialnerve)suppliesalloftheextraocularmusclesexceptthesuperior
oblique,whichissuppliedbythetrochlearorfourthcranialnerve,andthe
lateralrectus,whichissuppliedbytheabducentorsixthcranialnerve(see
Table17.1).Theextraocularmusclenucleiarelocatedinthebrainstemon
thefloorofthefourthventricleandareinterconnectedviathemediallongi-
tudinalfasciculus,anervefiberbundleconnectingtheextraocularmuscles,
neckmuscles,andvestibularnucleiforcoordinatedmovementsofthehead
andglobe(Fig.17.2).Variousvisualareasinthebraincontroleyeandgaze
movements.Thelocationofthemusclenucleiandknowledgeofthevisual
areasareimportantprimarilyingazeparalysisandparalyticstrabismusand
ofparticularinteresttotheneurologist.Forexample,thetypeofgazeparaly-
siswillallowonetodeducetheapproximatelocationofthelesioninthe
brain.
Allextraocularmusclesexceptforthesuperiorobliqueandlateralrec-
tusaresuppliedbytheoculomotornerve.
Physiologyofbinocularvision:Strictlyspeaking,we“see”withourbrain.
Theeyesaremerelytheorgansofsensoryreception.Theirimagesarestored
bycodingthestimulireceivedbytheretina.Theopticnerveandvisualpath-
waytransmitthisinformationincodedformtothevisualcortex.
Thesensorysystemproducesaretinalimageandtransmitsthisimageto
thehigher-ordercenters.Themotorsystemaidsinthisprocessbydirecting
botheyesattheobjectsothatthesameimageisproducedoneachretina.The
braincanthenprocessthisinformationintobinocularvisualimpression.A
personhasnosubjectiveawarenessofthisinterplaybetweensensoryand
motorsystems.
Therearethreedistinctlevelsofqualityofbinocularvision:
1.Simultaneousvision:Theretinasofthetwoeyesperceivetwoimages
simultaneously.Innormalbinocularvision,botheyeshavethesamepoint
offixation,whichlandsonthefoveacentralisineacheye.Theimageofan
17.1BasicKnowledge
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480. 462
Table17.1Functionoftheextraocularmuscleswiththegazedirectedstraightahead
Muscle Primary
action
Secondary
action
Example
(righteye)
Nerve
supply
Lateralrectus AbductionNone Abducent
nerve
Medialrectus AdductionNone Oculomotor
nerve
SuperiorrectusElevationIntorsionand
adduction
Oculomotor
nerve
InferiorrectusDepressionExtorsionand
adduction
Oculomotor
nerve
SuperiorobliqueIntorsionDepression
andabduc-
tion
Trochlear
nerve
Continued!
17OcularMotilityandStrabismus
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481. 463
Table17.1(Continued)
Muscle Primary
action
Secondary
action
Example
(righteye)
Nerve
supply
InferiorobliqueExtorsionElevationand
abduction
Oculomotor
nerve
Locationoftheextraocularmusclenucleiandgazecenters.
Frontal gaze center
(area 8)
Vertical eye movement
Occipital gaze center
(areas 17, 18, and 19)
III
IV
VI
RIMLF
PPRFHorizontal eye movement
Midbrain
Pons
Medulla oblongata
Cerebellum
Medial longitudinal fasciculus
Fig.17.2Theoculomotororthirdcranialnervesuppliesalloftheextraocular
musclesexceptthesuperioroblique(suppliedbythetrochlearorfourthcranial
nerve)andthelateralrectus(suppliedbytheabducentorsixthcranialnerve).The
rostralinterstitialnucleusofthemediallongitudinalfasciculus(RIMLF)isresponsible
forverticaleyemovementandphasesofrapidnystagmus.Theparamedianpontine
reticularformation(PPRF)isresponsibleforhorizontaleyemovement.
17.1BasicKnowledge
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482. 464
objectalwayslandsonidenticalareasoftheretina,referredtoascorre-
spondingpointsontheretina.Objectslyingonanimaginarycircleknown
asthegeometrichoropter(Fig.17.3a)areprojectedtothesepointsonthe
retina.Adifferenthoropterwillapplyforanygivenfixationdistance.The
imagesofbothretinasarethereforeidenticalinnormalbinocularvision.
Thisphenomenonmaybeexaminedbypresentingdifferentimagesto
eachretina;normallybothimageswillbeperceived.Thisisknownas
physiologicdiplopia.
Physiologicdiplopiacanbedemonstratedbyplacingtwoverticalpen-
cilsinalinealongthesubject’svisualaxis,withthesecondpencil
approximatelytwiceasfarfromthesubjectasthefirst.Whenthesub-
jectfocusesononepencil,theotherwillappeardouble.
2.Fusion:Onlywherebothretinasconveythesamevisualimpression,i.e.,
transmitidenticalimagestothebrain,willthetworetinalimagesblend
intoasingleperception.Impairedfusioncanresultindoublevision(horror
fusionisordiplopia).
Geometricandphysiologichoropters.
Panum's area
a b
A
B
Fixation point
Fovea Fovea
Horopter
Fovea Fovea
Fixation point
A
B
Fig.17.3aGeometrichoropter.Thelightraysfromthefixationpointstrikethe
foveacentralisinbotheyesinnormalsimultaneousvision.Therefore,objectsAand
Bonthegeometrichoropterareprojectedtocorrespondingpointsontheretina.
bPhysiologichoropter.Inanarrowrangeinfrontofandbehindthehoropter
(Panum’sarea),tworetinalimagescanstillbefusedintoone.PointsAandB,which
lieoutsidePanum’sarea,areprojectedtononcorrespondingpointsontheretina.
17OcularMotilityandStrabismus
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483. 465
3.Stereoscopicvision(perceptionofdepth):Thisisthehighestlevelofquality
ofbinocularvisionandispossibleonlywhereseveralconditionsaremet.
Forobjectstobeprojectedtocorrespondingoridenticalpointsontheret-
ina,theymustlieonthesamegeometrichoropter.Objectslyinginfrontof
orbehindthiscirclewillnotbeprojectedtocorrespondingpointsbutto
noncorrespondingordisparatepointsontheretina.Theresultisthatthese
objectsareperceivedasdoubleimages(diplopia).However,objectswithin
anarrowrangeinfrontofandbehindthehoropterarefusedintoasingle
image.ThisareaisreferredtoasPanum’sarea.Thebrainprocessesnoncor-
respondingretinalimageswithinPanum’sareaintoasinglethree-dimen-
sionalvisualperceptionanddoesnotinterpretthemasdoubleimages
(Fig.17.3b).Onthecontrary,thebrainusesthedoubleimagestodistin-
guishdifferencesindepth.
17.2 ConcomitantStrabismus
Definition
Concomitantstrabismusdiffersfromparalyticstrabismusinthattheangleof
deviationremainsthesameineverydirectionofgaze.Thedeviatingeyefol-
lowsthenormalfelloweyeataconstantangle.
Epidemiology:Concomitantstrabismusoccursalmostexclusivelyin
children.Approximately5.3–7.4%ofallchildrenareaffected.In60–70%of
allcases,thedisorderinitiallymanifestsitselfwithinthefirsttwoyearsoflife.
Etiology:Visionatbirthisneitherfocusednorbinocular,andbothsen-
sorimotorcoordinationandbinocularvisionareveryunstableduringthefirst
fewyearsoflife.Impairmentsofthesensoryormotorsystemsorcentralpro-
cessingofvisualperceptionsthatoccurduringthistimecandisturbthe
coordinationbetweentheeyesandleadtostrabismus.However,thecausesof
concomitantstrabismusareoftenunclear.Thefollowingcauseshavebeen
identifiedtodate:
!Geneticfactors:Approximately60%ofchildrenwithstrabismushavea
familyhistoryofincreasedincidence.
!Uncorrectedrefractiveerrorsarepartiallyresponsiblefortheoccurrence
ofstrabismus.Childrenwithhyperopia(farsightedness)tendtohave
esotropia.Thisisbecauseconvergenceandaccommodationarecoupled.
Childrenwithhyperopiahavetoaccommodatewithoutconvergingwhen
gazingintothedistancetocompensatefortheirrefractiveerror.However,
accommodationalwaystriggersaconvergenceimpulsethatcancause
esotropia.
!Insufficientfusion:Thiscanoccurinconjunctionwithanisometropia
(unequalrefractivepowerinthetwoeyes)andaniseikonia(unequalreti-
17.2ConcomitantStrabismus
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484. 466
nalimagesize).Itcanalsooccurinheterophoria(latentstrabismus)after
oneeyehasbeencoveredwithabandageforaprolongedperiod.
!Unilateralvisualimpairment:Severenearsightedness,cornealscarring,
lensopacities(cataract),macularchanges,andretinaldisorderscancause
secondarystrabismus.Retinalcausesincluderetinoblastoma,Coats’dis-
ease,retinopathyofprematurity,retinaldetachment,orcentralretinal
scarringincongenitaltoxoplasmosis.
Anyinitialexaminationofapatientwithstrabismusmustinvariably
includeexaminationofthefundusofbotheyesundermydriasisinaddi-
tiontoexaminationoftheanteriorsegmentsoftheeye
!Otherpossiblecausesofconcomitantstrabismusinclude:
–Perinatallesionssuchaspretermbirthandasphyxia.
–Cerebraltraumaandencephalitis.
Pathophysiology:Deviationofthevisualaxisofthedeviatingeyecauses
objectstobeprojectedtononcorrespondingpointsontheretina.Onewould
expectthesepatientssufferfromconstantdoublevisionbecausetheleftand
righteyessupplydifferentinformationtothebrain.However,thecentral
nervoussystemutilizestwomechanismstohelpavoiddoublevisionincon-
comitantstrabismus:
1.Suppression:Acentralinhibitingmechanismsuppressesthevisual
stimulifromthedeviatingeye.Therearetwodifferenttypesofsuppres-
sion:
Centralscotoma:Thisvisualfielddefectoccurswhentheperceivedobject
isprojectedtothesamelocationonthefoveainbotheyesbutstrabismus
causestheeyestoperceiveitasseparateobjects.Asthiswouldcausecon-
fusion,theobjectprojectedonthefoveaofthedeviatingeyeissuppress-
ed.
Fixationpointscotoma:Thisvisualfielddefectoccurswhentheimageper-
ceivedbytheleadingeyeisprojectedtoapointnexttothefoveainthe
deviatingeye.Thisresultsindiplopiaasthefixationpointdoesnotlie
withinthefoveaasitwouldinphysiologicsight.Thescotomaoccursat
thisnoncorrespondingpointnexttothefoveatosuppressthediplopia.
2.Sensoryadaptation:Inbinocularvisionwiththegazedirectedstraight
ahead,thefixationpointofthedeviatingeyecanfallbeyondthefovea.This
producesanomalousretinalcorrespondenceasthefixationpointinthe
nondeviatingeyealwaysfallsonthefovea.Thismeansthattheimage
createdinthedeviatingeyeisnotassharplyfocusedastheimageinthe
leadingeyeandissuppressed.
Amblyopiasecondarytosuppression.Constantsuppressioninstrabismusin
theformofacentralandfixationscotomascanleadtosevereamblyopia,
especiallyinchildrenbelowtheageofsix.Theprospectsforsuccessfultreat-
mentdecreasewithage,andamblyopiabecomesirreversiblebeyondtheage
17OcularMotilityandStrabismus
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485. 467
ofsixtoeight.Amblyopiaonlyoccursinunilateralstrabismus.Inalternating
strabismus,fixationordeviationalternatesbetweenbotheyessothatboth
eyeslearntosee.Adifferentialdiagnosismustdistinguishamblyopiainstra-
bismusfromotherformsofamblyopia.ThesearelistedinTable17.2.
Strabismusoccurringbeforetheageofsixwillfrequentlyleadtoambly-
opia.Earlyexaminationandtreatmentbyanophthalmologistarecru-
cial.
17.2.1FormsofConcomitantStrabismus
Theseessentiallyincludethefollowingforms:
!Esotropia:Inwarddeviationofthevisualaxis.
!Exotropia:Outwarddeviationofthevisualaxis.
!Hypertropiaandhypotropia:Oculardeviationwithoneeyehigheror
lowerthantheother.
!Cyclotropia:Thisreferstotherotationofoneeyearounditsvisualaxis.An
isolatedformofstrabismus(i.e.,onethatdoesnotoccurincombination
withparalyticstrabismus),thisdisorderisextremelyrareandtherefore
willnotbediscussedingreaterdetail.
17.2.1.1Esotropia
Epidemiology:Esotropiaisoneofthemostcommonlyencounteredformsof
strabismus.
Table17.2Formsofamblyopia
FormsofamblyopiaCause Treatment
Amblyopiawith
strabismus
Suppressionofthedeviat-
ingeye
Occlusiontherapy
DeprivationamblyopiaOrganicdisease,suchas
ptosisorcataract
Earlysurgeryandocclusion
therapyinapplicablecases
RefractiveamblyopiaDifferentrefractiveerrorsCorrectionwitheyeglassesor
contactlensesandocclusion
therapyinapplicablecases
Bilateralamblyopia Nystagmus,astigmatism,
latecorrectionofrefractive
errors
None
17.2ConcomitantStrabismus
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486. 468
Symptomsanddiagnosticconsiderations:Therearethreeformsof
esotropia:
1.Congenitalorinfantileesotropia:Strabismusispresentatbirthor
developswithinthefirstsixmonthsoflife.
Thisformischaracterizedbyalargealternatingangleofdeviation
(Fig.17.4aandb),lackofbinocularvision,latentnystagmus(involuntary
oscillationoftheeyeballsthatonlyoccursorbecomesmorepronounced
whenoneeyeiscovered),intermittentinclinationoftheheadinthedirec-
tionoftheleadingeye,andadditionalhypertropia(primaryoblique
muscledysfunctionanddissociatedverticaldeviation).
Alternatingesotropia.
Fig.17.4Inthis
formofstrabis-
mus,theeyes
taketheleadal-
ternately.
aEyeposition
whenfixatingan
objectonthe
right.
bEyeposition
whenfixatingan
objectonthe
left.
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487. 469
Anothermotilitydisorderthatalwaysoccursininfantilestrabismussyn-
dromeistheAorVpatterndeviation.Thisistheresultofanomalouscen-
tralcontrol,i.e.,anomaliesinthepatternofnervesupplytotherectusand
obliquemuscles.
!“Apatterndeviation”referstoaninwardangleofdeviationthat
increasesinupgazeanddecreasesindowngaze.
!“Vpatterndeviation”referstoaninwardangleofdeviationthat
decreasesinupgazeandincreasesindowngaze.
2.Acquiredstrabismus:Twoformsaredistinguished:
!1.Strabismusbeginsattheageofincompletesensorydevelopment,i.e.,
betweentheagesofoneandthree.Usuallythedisordermanifestsitself
attheageoftwoandleadstosensoryadaptationsyndromesintheform
ofunilateralstrabismus.Amblyopiaisusuallyalreadypresent,andcor-
respondenceisprimarilyanomalous.
!2.Strabismusmanifestsitselfbetweentheagesofthreeandseven.This
formofacutelatestrabismuswithnormalsensorydevelopmentis
encounteredfarlessfrequentlythanotherforms.Asbinocularvisionis
alreadywelldeveloped,affectedchildrencannotimmediatelysuppress
thevisualimagesofthedeviatingeye.Asaresult,theysufferfromsud-
dendoublevisionattheonsetofstrabismus,whichtheyattemptto
suppressbyclosingoneeye.Immediatetreatmentisindicatedtopre-
servebinocularvision.Thisconsistsofthefollowingsteps:
–Objectiveexaminationofrefractionwiththepupilsdilatedwith
atropineorcyclopentolateisperformedtodeterminewhethera
refractiveerrorispresent.Clinicalexperiencehasshownthatmod-
erateandseverehyperopiawillbedetectedmorefrequentlythanin
thecongenitalform.
–Theangleofdeviationispreciselydeterminedandcorrectedwith
prismeyeglasses.
–Surgeryisindicatedifeyeglasscorrectionfailstoimprovetheangle
ofdeviationwithinafewweeksortheeyesareemmetropic.
Binocularvisioniswelldevelopedinlatestrabismuswithnormal
sensorydevelopment.Surgerywithinthreetosixmonthswillallowthe
patienttomaintainorregainstereoscopicvision.
3.Microstrabismus:Thisisdefinedasunilateralesotropiawithaminimal
cosmeticeffect,i.e.,anangleofdeviationof5degreesorless.Asaresult,
microstrabismusisoftendiagnosedtoolate,i.e.,onlyattheageoffourto
six.Bythattimetheresultingamblyopiainthedeviatingeyemaybe
severe.Anothersequelaofmicrostrabismusisanomalousretinalcorre-
spondence.Binocularvisionispartiallypreserveddespiteanomalousreti-
nalcorrespondenceandamblyopia.However,itcannolongerbeimproved
bytreatment.Forthisreason,treatmentislimitedtoocclusiontherapyto
correcttheamblyopia.
17.2ConcomitantStrabismus
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488. 470
Accommodativeesotropiaintherighteye.
Fig.17.5aGaze
isdirected
throughthedis-
tanceportionof
bifocals.
bGazeisdirected
throughthenear-
fieldportionof
thebifocals.The
arrowindicates
thedividingline
betweenthedis-
tanceandnear-
fieldportions.
17.2.1.2AbnormalAccommodativeConvergence/AccommodationRatio
Whentheaccommodativeconvergence/accommodationratioisabnormal,
theangleofdeviationmayfluctuatedependingonwhetherthefixatedobject
isfarornear.Forexampleinaccommodativeesotropia,theangleofdeviation
islargerwithcloseobjectsthanwithdistantobjects.Thedisorderiscorrected
withbifocaleyeglasses,whichinthecaseofaccommodativeesotropiahavea
strongnear-fieldcorrection(Fig.17.5aandb).Aresidualangleofdeviation
mayremaindespitetheeyeglasscorrection.However,theangleofdeviation
mayalsoimprovetothepointthatthevisualaxesareparallelwithgood
binocularvision.
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489. 471
Anabnormalaccommodativeconvergence/accommodationratiowill
causefluctuationsinoculardeviationinnearanddistancefixation.
17.2.1.3Exotropia
Exotropia(divergentstrabismus)islesscommonthanesotropia.Asitisusu-
allyacquired,thedisorderisencounteredmoreofteninadultsthanin
children,whomorefrequentlyexhibitesotropia.Exotropialessfrequently
leadstoamblyopiabecausethestrabismusisoftenalternating.Occasionally
whatisknownas“panoramavision”willoccur,inwhichcasethepatienthas
anexpandedbinocularfieldofvision.Thefollowingformsaredistinguished:
!Intermittentexotropia.Thisisthemostcommonformofdivergentstra-
bismus.Inintermittentexotropia,anangleofdeviationispresentonly
whenthepatientgazesintothedistance;thepatienthasnormalbinocular
visioninnearfixation(Figs.17.6aandb).Theimagefromthedeviatingeye
issuppressedinthedeviationphase.Thisformofstrabismuscanoccuras
alatentdisorderinmildcases,meaningthattheintermittentexotropia
onlybecomesmanifestundercertainconditions,suchasfatigue.
!Secondaryexotropiaoccurswithreducedvisualacuityinoneeyeresult-
ingfromdiseaseortrauma.
!Consecutiveexotropiaoccursafteresotropiasurgery.Oftenthedisorderis
overcorrected.
17.2.1.4VerticalDeviations(HypertropiaandHypotropia)
LikeApatternandVpatterndeviations,verticaldeviationsarealsotypically
causedbyanomaliesinthepatternofnervesupplytotherectusandoblique
muscles.Verticaldeviationsareusuallyassociatedwithesotropiaor
exotropia,forexampleininfantilestrabismus.Primaryobliquemuscledys-
functionanddissociatedverticaldeviationarecommoninthissetting.
Primaryobliquemuscledysfunctionischaracterizedbyupwardvertical
deviationoftheadductingeyeduringhorizontaleyemovements.
Dissociatedverticaldeviationisalternatingupwarddeviationoftheeyes.
Therespectivenon-fixatingeyeortheeyeoccludedinthecovertestwillbe
elevated.
17.2.2DiagnosisofConcomitantStrabismus
17.2.2.1EvaluatingOcularAlignmentwithaFocusedLight
Thisisafundamentalexaminationandisusuallythefirstoneperformedby
theophthalmologistinpatientswithsuspectedconcomitantstrabismus.The
examinerholdsthelightbeneathandclosetohisorherowneyesand
observesthelightreflexesonthepatient’scorneas(Hirschberg’smethod)in
17.2ConcomitantStrabismus
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490. 472
Intermittentexotropiaintherighteye.
Fig.17.6aThe
righteyedeviates
indistancefixa-
tion.
bNodeviationis
presentinnear
fixation.
nearfixationatadistanceof30cm.Normally,thesereflexesaresymmetrical.
Strabismusispresentifthecornealreflexdeviatesinoneeye.Thecorneal
reflexesaresymmetricalinnormalbinocularvisionorpseudostrabismus;in
esotropia,exotropia,andverticaldeviation,theyareasymmetrical.
17.2.2.2DiagnosisofInfantileStrabismicAmblyopia(Preferential
LookingTest)
Strabismusoccursmostfrequentlyinthenewbornandinfantsandmustalso
betreatedatthisagetominimizetheriskofvisualimpairment.Asthe
examinercannotrelyonpatientcooperationatthisage,examinationtech-
niquesrequiringminimalpatientcooperationarenecessary.Thepreferential
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491. 473
lookingtestcanbeusedforearlyevaluationofvisionbeginningattheageof
fourtosixmonths.Thistestcannotreliablydetectstrabismicamblyopia.
However,Telleracuitycards(Fig.17.7)aresufficientlysensitiveforearly
detectionofdeficitsinthepresenceofdefectsoftheentirevisualsystem.
Procedure:Theinfantisshownacard(Telleracuitycard)withthesame
backgroundbrightness.Theexaminerishiddenbehindaviewingcasethat
covershimorherfromthefrontandside.Anobservationpinholeinthe
middleofthecardpermitstheexaminertoobserveonlytheinfant’seyesand
determineuponwhichsideofthecardtheinfantisfixating.Infantswhopre-
ferthestripedsidehavegoodfixation.
17.2.2.3DiagnosisofUnilateralandAlternatingStrabismus(Unilateral
CoverTest)
Aunilateralcovertestcandistinguishbetweenmanifestunilateralstra-
bismusandalternatingstrabismus.Thepatientisrequestedtofixateona
point.Theexaminerthancoversoneeyeandobservestheuncoveredeye
(Fig.17.8a–c).
!Inunilateralstrabismus,thesameeyealwaysdeviates.Whenthedeviat-
ingeyeiscovered,theuncoveredeye(theleading,nondeviatingeye)
remainsfocusedonthepointoffixation.Whenthenondeviatingeyeis
covered,theuncovereddeviatingeyehastotakethelead.Todoso,itwill
firstmakeavisibleadjustment.Inesotropia,thisadjustmentisfrom
medialtolateral;inexotropia,itisfromlateraltomedial.
!Inbilateralalternatingstrabismus,botheyeswillalternatelyfixateand
deviate.
DiagnosisofstrabismusinchildrenwiththeTelleracuitycard.
Fig.17.7The
Telleracuitycard
islocatedina
viewingcasebe-
hindwhichthe
examinersits.
Thispermitsthe
examinertosee
uponwhichhalf
ofthecardthe
infantfixates.In-
fantswhoprefer
thestripedside
havegoodfixa-
tion.
17.2ConcomitantStrabismus
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492. 474
Responseofthedeviatingeyetoaunilateralcovertest.
a
b
c
Fig.17.8aUnilateralesotropiaoftherighteye.bUnilateralcovertest:Whenthe
leadinglefteyeiscovered,thedeviatingrighteyeadjustswithamovementfrom
medialtolateralandthentakesthelead.Thecoveredlefteyedeviates.cWhenthe
leadinglefteyeisuncoveredagain,therighteyerevertstoitsdeviation.Theleading
lefteyeisrealignedwiththefixationpoint.
17.2.2.4MeasuringtheAngleofDeviation
Exactmeasurementoftheangleofdeviationiscrucialtoprescribingthe
properprismcorrectiontocompensatefortheangleofdeviationandtothe
correctivesurgerythatusuallyfollows.Ameasurementerrormayleadto
undercorrectionorovercorrectionoftheangleofdeviationduringtheopera-
tion.Example:Esotropiaof+15degreesiscorrectedbyshiftingthemedial
rectus4.0mmposteriorlyandshorteningthelateralrectus5.0mm.
Theangleofdeviationismeasuredwithacovertestincombinationwith
theuseofprismlensofvariousrefractivepowers.Thepatientfixatesona
certainpointwiththeleadingeyeatadistanceof5mor30cm,dependingon
whichangleofdeviationistobemeasured.Theexaminerplaceprismlenses
ofdifferentrefractivepowerbeforethepatient’sdevianteyeuntiltheeyeno
longermakesanyadjustment.Thisisthecasewhentheangleofdeviation
correspondstothestrengthoftherespectiveprismandisfullycompensated
forbythatprism.Thetipoftheprismmustalwayspointinthedirectionof
deviationduringtheexamination.
17OcularMotilityandStrabismus
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493. 475
Prismbarssimplifytheexamination.Thesebarscontainaseriesofprisms
ofprogressivelyincreasingstrengtharrangedoneabovetheother.
Maddox’scross(Fig.17.9)isadeviceoftenusedtomeasuretheangleof
deviation.Alightsourcemountedinthecenterofthecrossservesasafixa-
tionpoint.Thepatientfixatesthelightsourcewithhisorherleadingeye.The
objectiveangleofdeviationismeasuredwithprismsasdescribedabove.In
children,oftenonlytheobjectiveangleofdeviationismeasuredasthis
measurementdoesnotrequireanyactiononthepartofthepatientexceptfor
fixatingacertainpoint,inthiscasethelightsourceatcenterofthecross.In
adults,theexaminercanaskthepatienttodescribethelocationoftheareaof
doublevision(doublevisionmaybeasequelaofparalyticstrabismus,which
isthemostcommonformencounteredinadults).Thisusesthegraduations
ontheMaddox’scross.Thecrosshastwoscales,alargenumberedscalefor
testingatfivemetersandafinescalefortestingatonemeter(seeFig.17.9).
Thepatientdescribesthelocationoftheareaofdoublevisionaccordingtoa
certainnumberonthisscale.Theexaminerselectstheappropriateprismcor-
rectionaccordingtothepatient’sdescriptiontocorrecttheangleofdeviation
oftheparalyzedeye.Thissuperimposestheimagesseenbythedeviatingeye
andthenondeviatingeyetoeliminatethedoublevision.
Maddox’scross.
Fig.17.9AMaddoxcrossisfrequent-
lyusedonlyasafixationobjectwhen
examiningchildren.Thepatientfix-
atesonthelightsourceinthecenter.
Thetwoscales(alargenumbered
scalefortestingatfivemetersanda
finescalefortestingatonemeter)are
onlyrelevantforverbalpatientsasked
todescribethelocationoftheareaof
doublevision,forexampleinparalytic
strabismus.(Seetextforexamination
procedure.)
17.2ConcomitantStrabismus
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494. 476
Theangleofdeviationcanbemeasuredinprismdioptersordegrees.
Oneprismdiopterrefractslightraysapproximatelyhalfadegreesothat
twoprismdiopterscorrespondtoonedegree.
17.2.2.5DeterminingtheTypeofFixation
Thisexaminationisusedtoascertainwhichpartoftheretinaofthedeviating
eyetheimageofthefixatedpointfallson.Thepatientlooksthroughaspecial
ophthalmoscopeandfixatesonasmallstarthatisimagedonthefundusof
theeye.Theexaminerobservesthefundus.
!Incentralfixation,theimageofthestarfallsonthefoveacentralis.
!Ineccentricfixation,theimageofthestarfallsonanareaoftheretinaout-
sidethefovea(Fig.17.10).Usuallythispointliesbetweenthefoveaandthe
opticdisk.
Asidefromthetypeoffixation,onecanalsoestimatepotentialvisualacuity.
Thegreaterthedistancebetweenwherethepointoffixationliesandthe
fovea,thelowertheresolvingpoweroftheretinaandthepoorervisualacuity
willbe.Initialtreatmentconsistsofocclusiontherapytoshiftaneccentric
pointoffixationontothefoveacentralis.
Ophthalmoscopicexaminationoffixation.
5
x 3
x
6
x
2
x
1
x
4
x
Fig.17.10
1!fovealfixa-
tion;2!para-
fovealfixation;
3!macularfixa-
tion;4!para-
macularfixation;
5and6!eccen-
tricfixation.
17.2.2.6TestingBinocularVision
Bagolinitest:Thistestusesflatlenseswithfineparallelstriations.Thestria-
tionsspreadlightfromapointsourceintoastrip.Thelensesaremountedin
theexaminationeyeglassesinsuchamannerthatthestripsoflightforma
diagonalcrossinpatientswithintactbinocularvision.Thepatientisaskedto
17OcularMotilityandStrabismus
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495. 477
describethepatternofthestripsoflightwhilelookingatthepointsource.
Patientswhodescribeacrosshavenormalsimultaneousvision.Patientwho
seeonlyonediagonalstripoflightaresuppressingtheimagereceivedbythe
respectivefelloweye.
Lang’stest:Thistestmaybeusedtodeterminedepthperceptionininfants.A
carddepictsvariousobjectsthatthechildonlyseesifitcanperceivedepth.
17.2.3TherapyofConcomitantStrabismus
Therapyofconcomitantstrabismusinchildren:Treatmentisgenerally
long-term.Thedurationoftreatmentmayextendfromthefirstmonthsoflife
toabouttheageoftwelve.Specifictreatmentsandtherapeuticsuccessare
determinednotonlybytheclinicalcoursebutalsobythechild’soverallper-
sonalityandtheparents’abilitytocooperate.Theentirecourseoftreatment
maybedividedintothreephaseswithcorrespondinginterimgoals.
1.Theophthalmologistdetermineswhetherthecauseofthestrabismusmay
betreatedwitheyeglasses(suchashyperopia).
2.Ifthestrabismuscannotbefullycorrectedwitheyeglasses,thenextstepin
treatment(paralleltoprescribingeyeglasses)istominimizetheriskof
amblyopiabyocclusiontherapy.
3.Oncetheocclusiontherapyhasproducedsufficientvisualacuityinboth
eyes,thealignmentofoneorbotheyesiscorrectedbysurgery.Latestra-
bismuswithnormalsensorydevelopmentisanexceptiontothisrule(for
furtherinformation,seeSurgery).Thealignmentcorrectionisrequiredfor
normalbinocularvisionandhastheaddedbenefitofcosmeticimprove-
ment.
Therapyofconcomitantstrabismusinadults:Theonlypurposeofsurgery
iscosmeticimprovement.Afunctionalimprovementinbinocularvisioncan
nolongerbeachieved.
17.2.3.1EyeglassPrescription
Wherethestrabismusisduetoacausethatcanbetreatedwitheyeglasses,
theneyeglassescaneliminateatleasttheaccommodativecomponentofthe
disorder.Oftenresidualstrabismusrequiringfurthertreatmentwillremain
despiteeyeglasscorrection.
17.2.3.2TreatmentandAvoidanceofStrabismicAmblyopia
Strictocclusiontherapybyeyepatchingoreyeglassocclusionisthemost
effectivemethodofavoidingortreatingstrabismicamblyopia.Primarilythe
leadingeyeispatched.
17.2ConcomitantStrabismus
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496. 478
Eyepatching:Severeamblyopiawitheccentricfixationrequiresaneyepatch
(Fig.17.11).Eyeglassocclusion(seenextsection)entailstheriskthatthechild
mightattempttocircumventtheocclusionofthegoodeyebylookingover
therimoftheeyeglasseswiththeleadingeye.Thiswouldcompromisethe
effectivenessofocclusiontherapy,whosepurposeistotraintheamblyopic
eye.
Eyeglassocclusion:Mildcasesofamblyopiausuallymaybetreatedsuccess-
fullybycoveringtheeyeglasslensoftheleadingeyewithanopaquematerial.
Insuchcases,thechildusuallydoesnotattempttolookovertherimofthe
eyeglassesbecausethedeviatingeyehassufficientvisualacuity.
Procedure:Thedurationofocclusiontherapymustbebalancedsoastoavoid
alossofvisualacuityintheleadingeye.Theleadingeyeisoccludedfor
severalhoursatatimeinmildamblyopia,andforseveraldaysatatimein
severeamblyopiadependingtothepatient’sage.Forexample,thenondeviat-
ingeyeinafour-year-oldpatientispatchedforfourdayswhilethedeviating
eyeisleftuncovered.Botheyesarethenleftuncoveredforoneday.Thistreat-
mentcycleisrepeatedbeginningonthefollowingday.
Amblyopiamustbetreatedinearlychildhood.Theyoungerthechildis,
themorefavorableandrapidtheresponsetotreatmentwillbe.The
upperagelimitforocclusiontherapyisapproximatelytheageofnine.
Theearliertherapyisinitiated,thesooneramblyopiacanbeeliminated.
Occlusiontherapyofamblyopia.
Fig.17.11Theleadingeyeis
patchedforseveralhoursor
daysatatimetoimprove
visualacuityinthedeviating
amblyopiceye.
17OcularMotilityandStrabismus
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497. 479
Thegoaloftreatmentininfantilestrabismusistoachievealternatingstra-
bismuswithfullvisualacuityandcentralfixationinbotheyes.Binocular
visionislessimportantinthissetting.Itisnotnormallydevelopedanywayin
patientswhodevelopstrabismusatanearlyageandcannotbefurther
improved.
17.2.3.3Surgery
Surgeryininfantilestrabismussyndrome:Surgeryshouldbepostponed
untilafteramblyopiahasbeensuccessfullytreated(seeprevioussection).Itis
alsoadvisabletowaituntilthepatienthasreachedacertainage.Adequate
follow-upincludesprecisemeasurementofvisualacuityatregularintervals
inteststhatrequirethepatient’scooperation,andsuchcooperationisdiffi-
culttoensureinyoungpatientsbelowtheageoffour.Surgicalcorrectionina
veryyoungpatientpriortosuccessfultreatmentofamblyopiainvolvesarisk
thatadecreaseinvisualacuityinoneeyemaygounnoticedafterthestra-
bismushasbeencorrected.However,thechildshouldundergosurgeryprior
toenteringschoolsoastoavoidthesocialstigmaofstrabismus.Insuchacase,
surgeryachievesonlyacosmeticcorrectionofstrabismus.
Surgeryinlatestrabismuswithnormalsensorydevelopment:Inthis
case,surgeryshouldbeperformedasearlyaspossiblebecausetheprimary
goalistopreservebinocularvision,whichisnecessarilyabsentininfantile
strabismussyndrome.
Procedure:Theeffectofsurgeryislesstoalterthepulloftheextraocular
musclesthantoalterthepositionoftheeyesatrest.Esotropiaiscorrectedby
acombinedprocedureinvolvingamedialrectusrecessionandalateralrectus
resection.Themedialrectusisreleasedbecauseitspullis“toostrong”(see
Fig.17.1),whereasthelateralrectusisshortedtoincreaseitspull.Thedegree
ofcorrectiondependsontheangleofdeviation.Primaryobliquemuscledys-
functioniscorrectedbyinferiorobliquerecessionandifnecessarybydou-
blingthesuperiorobliquetoreinforceit.Exotropiaiscorrectedbyposteriorly
alateralrectusrecessionincombinationwithamedialrectusresection.
17.2ConcomitantStrabismus
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498. 480
17.3 Heterophoria
Definition
Heterophoriareferstoamuscularimbalancebetweenthetwoeyesthatleads
tomisalignmentofthevisualaxesonlyundercertainconditions(seebelow).
Thisisincontrasttoorthophoria,muscularbalancewithparallelvisualaxes.
Heterophoriaistypifiedbyinitiallyparallelvisualaxesandfullbinocularvision.
Thefollowingformsaredistinguishedanalogouslytomanifeststrabismus:
!Esophoria:latentinwarddeviationofthevisualaxis.
!Exophoria:latentoutwarddeviationofthevisualaxis.
!Hyperphoria:latentupwarddeviationofoneeye.
!Hypophoria:latentdownwarddeviationofoneeye.
!Cyclophoria:latentrotationofoneeyearounditsvisualaxis.
Epidemiology:Thisdisorderoccursin70–80%ofthepopulation.Theinci-
denceincreaseswithage.
Etiologyandsymptoms:Heterophoriadoesnotmanifestitselfaslongas
imagefusionisunimpaired.Wherefusionisimpairedasaresultofalcohol
consumption,stress,fatigue,concussion,oremotionaldistress,themuscular
imbalancecancauseintermittentoroccasionallypermanentstrabismus.This
isthentypicallyassociatedwithsymptomssuchasheadache,blurredvision,
diplopia,andeasilyfatiguedeyes.
Diagnosticconsiderations:Heterophoriaisdiagnosedbytheuncovertest.
Thistestsimulatesthespecialconditionsunderwhichheterophoriabecomes
manifest(decreasedimagefusionsuchascanoccurduetoextremefatigueor
consumptionofalcohol)andeliminatestheimpetustofuseimages.Incontrast
tothecovertest,theuncovertestfocusesontheresponseofthepreviously
coveredeyeimmediatelyafterbeinguncovered.Onceuncovered,theeye
makesavisibleadjustmenttopermitfusionandrecoverbinocularvision.
Treatment:Heterophoriarequirestreatmentonlyinsymptomaticcases.
Convergencedeficienciescanbeimprovedbyorthopticexercises.The
patientfixatesasmallobjectateyelevel,whichisslowlymovedtoapoint
veryclosetotheeyes.Theobjectmaynotappearasadoubleimage.Prism
eyeglassestocompensateforalatentangleofdeviationhelponlytem-
porarilyandarecontroversialbecausetheyoccasionallyresultinanincrease
inheterophoria.Strabismussurgeryisindicatedonlywhenheterophoria
deterioratesintoclinicallymanifeststrabismus.
17OcularMotilityandStrabismus
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499. 481
17.4 Pseudostrabismus
Abroaddorsumofthenosewithepicanthalfoldsthroughwhichthenasal
aspectofthepalpebralfissureappearsshortenedcanoftensimulatestrabis-
musinsmallchildren(Fig.17.12).Thechild’seyesappearesotropicespecially
whengazingtotheside.Testingwithafocusedlightwillrevealthatthecor-
nealreflexesaresymmetrical,andtherewillbenoeyeadjustmentsinthe
covertest.Usuallytheepicanthalfoldswillspontaneouslydisappearduring
thefirstfewyearsoflifeasthedorsumofthenosedevelops.
17.5 OphthalmoplegiaandParalyticStrabismus
Definitions
Ophthalmoplegiacanaffectoneormoreocularmusclesatthesametime.The
conditionmaybepartial(paresis,morecommon)orcomplete(paralysis,less
common).Theresultiseithergazepalsyorstrabismus(paralyticstrabismus),
dependingonthecause(seenextsection)andseverity.
!Gazepalsy:Impairmentorfailureofcoordinatedeyemovements.For
exampleincycloverticalmuscularpalsy,theupwardanddownwardgaze
movementsareimpairedorabsent.
!Paralyticstrabismus:Strabismusdueto:
–Isolatedlimitedmotilityinoneeye.
–Asymmetricallimitedmotilityinbotheyes.
Theangleofdeviationdoesnotremainconstantineverydirectionofgaze(as
inconcomitantstrabismus)butincreasesinthedirectionofpullofthepara-
lyzedmuscle.Thisisreferredtoasanincomitantangleofdeviation.
Pseudostrabismus.
Fig.17.12
Esotropiaofthe
lefteye(arrow)is
onlysimulatedby
abroaddorsum
ofthenose.The
cornealreflexes
demonstratepar-
allelvisualaxes.
17.5OphthalmoplegiaandParalyticStrabismus
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500. 482
Etiologyandformsofocularmotilitydisturbances:Twoformsaredistin-
guished.
!Congenitalocularmotilitydisturbancesmaybeduetothefollowing
causes:
–Prenatalencephalitis.
–Aplasiaoftheocularmuscles.
–Birthtrauma.
!Acquiredocularmotilitydisturbancesmaybeduetothefollowing
causes:
–Diabetesmellitus.
–Multiplesclerosis.
–Intracranialtumors.
–Arteriosclerosis.
–Centralischemia(apoplexy).
–AIDS.
–Traumaandothercauses.
Ocularmotilitydisturbancesareeitherneurogenic,myogenic,ordueto
mechanicalcauses.
Neurogenicocularmotilitydisturbances(seealsoophthalmoplegiasecond-
arytocranialnervelesions)aredistinguishedaccordingtothelocationofthe
lesion(Table17.3):
!Lesionsofthenervessupplyingtheocularmuscles.Thisconditionisreferred
toasaninfranuclearocularmotilitydisturbanceandisthemostcommon
causeofparalyticstrabismus.Thefollowingnervesmaybeaffected:
–Oculomotornervelesionsarerareandcauseparalysisofseveral
muscles.
–Trochlearnervelesionsarecommonandcauseparalysisofthesuperior
oblique.
–Abducentnervelesionsarecommonandcauseparalysisofthelateral
rectus.
!Lesionsoftheocularmusclenuclei.Thisconditionisreferredtoasanuclear
ocularmotilitydisturbance(seeFig.17.2).
Theoculomotornucleisupplybothsidesbutthenervesarenotclose
together.Therefore,bilateralpalsysuggestsanuclearlesion,whereas
unilateralpalsysuggestsalesionofonenerve.
!Lesionsofthegazecenters.Thisconditionisreferredtoasasupranuclear
ocularmotilitydisturbance(seegazecenters,Fig.17.2).Itveryoftencauses
gazepalsy.
!Anotherpossiblebutrareconditionisalesionofthefibersconnectingtwo
nuclei.Thisconditionisreferredtoasaninternuclearocularmotilitydis-
turbanceandmayoccurasaresultofalesionofthemediallongitudinal
fasciculus(seeFigs.17.2and17.13,Internuclearophthalmoplegia).
17OcularMotilityandStrabismus
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501. 483
Table17.3Classificationofneurogenicophthalmoplegiaaccordingtothelocationof
thelesion(seeFig.17.2)
Ocularmotility
disturbance
Causes LocationoflesionEffects
Infranuclear
ocularmotility
disturbance
!Inyoungerpa-
tients:
–Trauma
–Multiple
sclerosis
–Infectiousdis-
ease
–Braintumors
!Inolderpatients:
–Vasculardis-
ease
–Diabetes
–Hyperten-
sion
–Arterioscle-
rosis
!Lesioninoneof
thenervessup-
plyingtheocular
muscles:
–Oculomotor
nerve
–Trochlear
nerve
–Abducent
nerve
Palsyofoneorseveral
extraocularmusclesof
oneorbotheyes
resultinginstrabismus
orcompletegaze
palsy.
Nuclearocular
motilitydistur-
bance
!Multiplesclero-
sis
!Myasthenia
gravis
!Meningo-
encephalitis
!Syphilis
!AIDS
Lesionoftheocular
musclenucleus
Palsyoftheextraocu-
larmusclesofboth
eyesinvarying
degreesofseverity.
Supranuclear
ocularmotility
disturbance
!Horizontal
gazepalsy
!Diabetes
!Apoplexy
!Tumor
!Encephalitis
!Vascularinsult
!Multiplesclero-
sis
Lesioninthepara-
medianpontine
reticularformation
(PPRF;seeFig.17.2)
!Allconjugateeye
movementsonthe
sideofthelesion
areimpaired.
!Peripheralfacial
paresisisoftenalso
present.
!Botheyesare
affected.
Continued!
17.5OphthalmoplegiaandParalyticStrabismus
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502. 484
Table17.3(Continued)
Ocularmotility
disturbance
Causes LocationoflesionEffects
!Verticalgaze
palsy(Pari-
naud’ssyn-
drome)
!Midbraininfarc-
tions
!Tumorsofthe
quadrigeminal
regionsuchas
pinealgland
tumorsandger-
minomas.
Lesioninthemedial
longitudinalfasci-
culus(MLF;see
Fig.17.2)
!Isolatedupwardor
downwardgaze
palsy(common).
!Combinedupward
anddownward
gazepalsy(rare).
!Moderatelywide
pupils.
!Impairedaccom-
modation.
!Convergencenys-
tagmus.
!Jerkyuppereyelid
retraction.
Internuclear
ocularmotility
disturbance
(INO)
!Younger
patientswith
bilateralINO:
multiplesclero-
sis
!Olderpatients
withunilateral
INO:brainstem
infarction
Lesioninthemedial
longitudinalfasci-
culus(seeFig.17.2)
!Medialnervepalsy
orimpairedadduc-
tioninoneeyein
sidegazewith
intactnearreflex
convergence(see
Fig.17.13).
!Jerknystagmusin
theabductedeye
aslongasthepalsy
persists.
!InbilateralINO,fine
verticalnystagmus
inthedirectionof
gaze.
Myogenicocularmotilitydisturbancesarerare.Theseincludepalsiesdueto
thefollowingcauses:
!Graves’diseaseisthemostcommoncauseofmyogenicocularmotilitydis-
turbances.Becauseitaltersthecontractilityandductilityoftheocular
muscles,itcanresultinsignificantmotilitydisturbances(seeChapter15).
!Ocularmyastheniagravisisadisorderofneuromusculartransmission
characterizedbythepresenceofacetylcholinereceptorantibodies.Typi-
calsymptomsofocularmyastheniagravisincludefluctuatingweakness
17OcularMotilityandStrabismus
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503. 485
Rightinternuclearophthalmoplegia.
a
b
c
d
Fig.17.13aParal-
lelvisualaxes.
bNormalright
gaze.cInleftgaze,
therighteyecan-
notbeadducted
becausethemedial
longitudinalfasci-
culusisinterrupted.
dConvergenceis
preservedinboth
eyes.
thatisclearlyattributabletoanyonecranialnerve.Theweaknesstypically
increasesinseverityduringthecourseofthedaywithfatigue.
Importantdiagnosticaidsincludethefollowingtests.
–Simpsontest:Thepatientisaskedtogazeupwardforoneminute.
Gradualdroopingofoneofthepatient’seyelidsduringthetestdueto
fatigueofthelevatorpalpebraestronglysuggestsmyastheniagravis.
–Tensilon(edrophoniumchloride)test:Thistestisusedtoconfirmthe
diagnosis.Thepatientisgiven1–5mgofintravenousTensilon(edro-
phoniumchloride).Wheremyastheniagravisispresent,theparesis
willdisappearwithinafewseconds.(Refertoatextbookofneurology
foradetaileddescriptionofthistest.)
17.5OphthalmoplegiaandParalyticStrabismus
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504. 486
!Chronicprogressiveexternalophthalmoplegia(CPEO)isausuallybilateral,
graduallyprogressiveparalysisofoneormoreextraocularmuscles.Inthe
finalstagesitresultsincompleteparalysisofbotheyes.Becausetheparal-
ysisissymmetricthepatientdoesnotexperiencestrabismusordouble
vision.
!Ocularmyositisisinflammationofoneormoreextraocularmuscles.The
pathogenesisisuncertain.Ocularmotilityisoftenlimitednotsomuchin
thedirectionofpulloftheinflamedmuscleasintheoppositedirection.
Whilethereisparesisofthemuscle,itischaracterizedprimarilyby
insufficientductility.Oftenadditionalsymptomsarepresent,suchaspain
duringeyemovement.
Mechanicalocularmotilitydisturbancesincludepalsiesduetothefollowing
causes:
!Fractures.Inablowoutfractureforexample,thefracturedflooroftheorbit
canimpingetheinferiorrectusandoccasionallytheinferioroblique.This
caninterferewithupwardgazeandoccasionallyproducestrabismus.
!Hematomas.
!Swellingintheorbitorfacialbones,suchascanoccurinanorbitalabscess
ortumor.
Symptoms:Strabismus:Paralysisofoneormoreocularmusclescancauseits
respectiveantagonisttodominate.Thisresultsinatypicalstrabismusthat
allowswhichmuscleisparalyzedtobedetermined(seeDiagnosticcon-
siderations).Thisisreadilydoneespeciallyinabducentortrochlearnerve
palsyastheabducentnerveandthetrochlearnerveeachsupplyonlyone
extraocularmuscle(seeFig.17.1).
Example:abducentnervepalsy(Fig.17.14).Alesionoftheabducentnervepar-
alyzesthelateralrectussothattheeyecannolongerbyabducted.Thisparaly-
sisalsocausesthemuscle’santagonist,themedialrectus,todominate.
Becausethismuscleisresponsibleforadduction,theaffectedeyeremains
mediallyrotated.
Gazepalsy.Symmetricalparalysisofoneormoremusclesofbotheyeslimits
ocularmotilityinacertaindirection.Forexample,verticalgazepalsyorPari-
naud’ssyndrome,whichprimarilyoccursinthepresenceofapinealgland
tumor,involvesalesionoftherostralinterstitialnucleusofthemediallongi-
tudinalfasciculus(seeFig.17.12).Paralysisofallextraocularmusclesleadsto
completegazepalsy.Gazepalsysuggestsasupranuclearlesion,i.e.,alesionin
thegazecenters.Examinationbyaneurologistisindicatedinthesecases.
Doublevision.Lossofbinocularcoordinationbetweenthetwoeyesdueto
ophthalmoplegialeadstodoublevision.Normalvisionmaybeexpectedin
patientswithonlymoderateparesis.Astheonsetofparesisisusuallysudden,
doublevisionisthetypicalsymptomthatinducespatientstoconsultaphys-
17OcularMotilityandStrabismus
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505. 487
Leftabducentnervepalsy.
Fig.17.14The
lefteyeremains
immobileinleft
gaze(arrow).
ician.Somepatientslearntosuppressoneofthetwoimageswithinafew
hours,days,orweeks.Otherpatientssufferfrompersistentdoublevision.
Childrenusuallylearntosuppresstheimagequickerthanadults.
Causes.Doublevisionoccurswhentheimageofthefixatedobjectonlyfalls
onthefoveainoneeyewhilefallingonapointontheperipheralretinainthe
felloweye.Asaresult,theobjectisperceivedintwodifferentdirectionsand
thereforeseendouble(Fig.17.15aandb).Thedoubleimageofthedeviating
eyeisusuallysomewhatoutoffocusastheresolvingpoweroftheperipheral
retinaislimited.Despitethis,thepatientcannottellwhichisrealandwhich
isavirtualimageandhasdifficultyinreachingtograspanobject.
Thedistancebetweenthedoubleimagesisgreatestinophthalmoplegiain
theoriginaldirectionofpulloftheaffectedmuscle.
Example:trochlearnervepalsy(Fig.17.16).Thesuperiorobliquesuppliedby
thetrochlearnerveisprimarilyanintorteranddepressorinadduction(see
Table17.1);itisalsoanabductorwhenthegazeisdirectedstraightahead.
Therefore,thelimitedmotilityandupwarddeviationoftheaffectedeyeis
mostapparentindepressionandintorsionaswhenreading.Thedistance
betweenthedoubleimagesisgreatestandthediplopiamostirritatinginthis
directionofgaze,whichisthemaindirectionofpulloftheparalyzedsuperior
oblique.
Compensatoryheadposture.Thepatientcanavoiddiplopiaonlybyattempt-
ingtoavoidusingtheparalyzedmuscle.Thisisdonebyassumingatypical
compensatoryheadpostureinwhichthegazelieswithinthebinocularvisual
field;thepatienttiltshisorherheadandturnsittowardtheshoulder
oppositetheparalyzedeye.
17.5OphthalmoplegiaandParalyticStrabismus
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506. 488
Crossedanduncrosseddiplopia.
a
LE
P
LE
F
Uncrossed double images
Esotropic eye bExotropic eye
RE
F
LE
P
LE
F
Crossed double images
Virtual imageReal image
RE
F
Virtual imageReal image
Fig.17.15aEsotropiainthelefteye(LE)
withuncrossedimages.Therighteye(RE)
istheleadingeye,andthelefteyeiseso-
tropic.Thevisualimagefallingonthe
foveaintheleadingeyefallsonthenasal
retinanexttothefovea(PLE)intheeso-
tropiceyeandisperceivedinspaceina
temporallocation.Theobjectisseenas
twouncrossedorhomonymousimages.
bExotropiainthelefteye(LE)with
crossedimages.Therighteye(RE)isthe
leadingeye,andthelefteyeisex-
otropic.Thevisualimagefallingonthe
foveaintheleadingeyefallsonthetem-
poralretinanexttothefovea(PLE)inthe
exotropiceyeandisperceivedinspace
inanasallocation.Theobjectisseenas
twocrossedorheteronymousimages.
TheBielschowskyheadtilttestusesthisposturetoconfirmthediagnosisof
trochlearorfourthcranialnervepalsy(Fig.17.17).Inthistest,theexaminer
tiltsthepatient’sheadtowardthesideoftheparalyzedeye.Ifthepatientthen
fixateswiththenormaleye,theparalyzedeyewilldeviate.Whenthe
patient’sheadistiltedtowardthenormalside,therewillbenoverticaldevia-
tion(seeDiagnosticconsiderationsforfurtherdiagnosticprocedures).
Oculartorticollis.Thecompensatoryheadpostureintrochlearnervepalsyis
themostpronouncedandtypicalofallcranialnervepalsies.Congenital
trochlearnervepalsycanleadtowhatisknownasoculartorticollis.
Incomitantangleofdeviation.Theangleofdeviationinparalyticstrabismus
alsovarieswiththedirectionofgazeandisnotconstantasinconcomitant
strabismus.Likethedistancebetweenthedoubleimages,theangleofdevia-
tionisgreatestwhenthegazeisdirectedinthedirectionofpullofthepara-
17OcularMotilityandStrabismus
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507. 489
Righttrochlearnervepalsy.
Fig.17.16Verti-
caldeviationof
therighteyein
leftdownward
gaze(arrow).
Bielschowskyheadtilttest.
Fig.17.17aWhenthepatienttiltsher
headtotheleft(towardthenormalside),
therighteyedoesnotdeviateupward
whenthenormallefteyefixates.
bWhenthepatienttiltsherheadtothe
right(towardthesideoftheparalyzed
muscle),therighteyedeviatesupward
whenthenormallefteyefixates.
17.5OphthalmoplegiaandParalyticStrabismus
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508. 490
lyzedmuscle.Theangleofdeviationmaybeclassifiedaccordingtothewhich
eyefixates.
–Aprimaryangleofdeviationistheangleofdeviationwhenfixatingwith
thenormaleye.Thisangleissmall.
–Asecondaryangleofdeviationistheangleofdeviationwhenfixatingwith
theparalyzedeye.Thisangleislarge.
Thesecondaryangleofdeviationisalwayslargerthantheprimary
angle.Thisisbecauseboththeparalyzedmuscleanditssynergistinthe
felloweyereceiveincreasedimpulseswhentheparalyzedeyefixates.
Forexamplewhentherighteyefixatesinrightabducentnervepalsy,
theleftmedialrectuswillreceiveincreasedimpulses.Thisincreasesthe
angleofdeviation.
Cranialnervepalsies:Thecommonestpalsiesarethoseresultingfrom
cranialnervelesions.Therefore,thissectionwillbedevotedtoexamining
thesepalsiesingreaterdetailthantheothermotilitydisturbanceslisted
underEtiology.Itbecomesevidentfromtheexamplesofcauseslistedhere
thatadiagnosisofophthalmoplegiawillalwaysrequirefurtherdiagnostic
procedures(oftenbyaneurologist)toconfirmorexcludethepresenceofa
tumororacertainunderlyingdisordersuchasdiabetesmellitus.
Abducentnervepalsy:
Causes:Themaincausesofthisrelativelycommonpalsyincludevasculardis-
ease(diabetesmellitus,hypertension,orarteriosclerosis)andintracerebral
tumors.Oftenatumorwillcauseincreasedcerebrospinalfluidpressure,
whichparticularlyaffectstheabducentnervebecauseofitslongcoursealong
thebaseoftheskull.Inchildren,thesetransientisolatedabducentnervepal-
siescanoccurininfectiousdiseases,febriledisorders,orsecondarytoinocu-
lations.
Effects:Thelateralrectusisparalyzed,causingitsantagonist,themedialrec-
tus,todominate.Abductionisimpairedorabsentaltogether,andtheaffected
eyeremainsmediallyrotated(seeFig.17.14).Horizontalhomonymous
(uncrossed)diplopiaispresent(seeFig.17.15).Theimagesarefarthestapart
inabduction.
Example:rightabducentnervepalsy.
!Compensatoryheadposturewithrighttilt.
!Esotropiawhenthegazeisdirectedstraightahead.
!Largestangleofdeviationanddistancebetweenimagesinrightgaze.
!Noangleofdeviationordiplopiainleftgaze.
Retractionsyndrome(specialformofabducentnervepalsy):
Causes:Retractionsyndromeisacongenitalunilateralmotilitydisturbance
resultingfromalesiontotheabducentnerveacquiredduringpregnancy.
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509. 491
Effects:Thelateralrectusisnolongersuppliedbytheabducentnervebutby
fibersfromtheoculomotornervethatbelongthemedialrectus.Thishas
severalconsequences.Asinabducentnervepalsy,abductionislimitedand
slightesotropiaisusuallypresent.Incontrasttoabducentnervepalsy,the
globerecedesintotheorbitalcavitywhenadductionisattempted.Thisnar-
rowsthepalpebralfissure.Thisretractionoftheglobeinattemptedadduction
resultsfromthesimultaneousoutwardandinwardpulloftwoantagonistson
theglobebecausetheyaresuppliedbythesamenerve(oculomotornerve).
Trochlearnervepalsy:
Causes:Thecommonestcauseistrauma;lesscommoncausesincludevascu-
lardisease(diabetesmellitus,hypertension,andarteriosclerosis).Trochlear
nervepalsyisarelativelycommonphenomenon.
Effects:Thesuperiorobliqueisprimarilyanintorterandadepressorinadduc-
tion.Thisresultsinupwardverticaldeviationoftheparalyzedeyeinadduc-
tionandverticalstrabismus(seeFig.17.16).Patientsexperiencevertical
diplopia;theimagesarefarthestapartindepressionandintorsion.Compen-
satoryheadpostureisdiscussedinthesectiononsymptoms.Diplopiais
absentinelevation.
Oculomotornervepalsy:
Causes:
!Completeoculomotornervepalsy:Everyintraocularandalmostevery
extraocularmuscleisaffected,withlossofbothaccommodationandpupil-
larylightreaction.Thefailureoftheparasympatheticfibersintheoculo-
motornerveproducesmydriasis.Ptosisispresentbecausethelevatorpal-
pebraeisalsoparalyzed.Theparalyzedeyedeviatesinextorsionand
depressionasthefunctionofthelateralrectusandsuperiorobliqueispre-
served.Patientsdonotexperiencediplopiabecausetheptoticeyelid
coversthepupil.
!Partialoculomotornervepalsy:
–Externaloculomotornervepalsy(isolatedparalysisoftheextraocular
musclessuppliedbytheoculomotornerve;seeFig.17.1)ischaracter-
izedbydeviationinextorsionanddepression.Iftheptoticeyeliddoes
notcoverthepupil,thepatientwillexperiencediplopia.
–Internaloculomotornervepalsyisisolatedparalysisoftheintraocular
musclessuppliedbytheoculomotornerve.Thisischaracterizedbyloss
ofaccommodation(duetoparalysisoftheciliarymuscle)andmydria-
sis(duetoparalysisofthesphincterpupillae).Patientsdonotex-
periencediplopiaasthereisnostrabismicdeviation(seealsotonic
pupilandAdiesyndrome).
Combinedcranialnervepalsies.Thethird,fourth,andsixthcranialnerves
canbesimultaneouslyaffected,forexampleinalesionattheapexoftheorbi-
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510. 492
talcavityorinthecavernoussinus.Clinicalsuspicionofcombinedlesionmay
besupportedbyacornealsensitivitytestastheophthalmicdivisionofthe
trigeminalnerve,whichprovidessensorysupplytothecornea,courses
throughthecavernoussinus.Wherethereislossofcornealsensitivity,
whetherthelesionislocatedinthecavernoussinusmustbedetermined.
Diagnosisofophthalmoplegia:Examinationoftheninediagnosticposi-
tionsofgaze(seeChapter1).Thepatientisaskedtofollowthemovementsof
theexaminer’sfingerorapencilwithhisorhereyesonly.Thesixcardinal
directionsofgaze(right,upperright,lowerright,left,upperleft,lowerleft)
providethemostinformation;upwardanddownwardmovementsareper-
formedwithseveralmusclesandthereforedonotallowpreciseidentification
oftheactionofaspecificmuscle.Immobilityofoneeyewhenthepatient
attemptsacertainmovementsuggestsinvolvementofthemuscle
responsibleforthatmovement.
TheBielschowskyheadtilttestisperformedonlywheretrochlearnerve
palsyissuspected(seesymptoms).
Measuringtheangleofdeviation.Measuringthisangleintheninediagnos-
ticdirectionsofgazeprovidesinformationabouttheseverityofthepalsy,
whichisimportantforsurgicalcorrection.ThisisdoneusingaHarmstangent
MeasuringtheangleofdeviationwiththeHarmstangenttable.
Fig.17.18Thepatientsitsatadis-
tanceof2.5metersfromthetable
andfixatesonthelightinthecenter.
Theexaminerevaluatestheninediag-
nosticpositionsofgaze.Thegridpro-
videsthecoordinatesformeasuring
thehorizontalandverticaldeviations,
andthediagonalsareusedto
measuretheangleofdeviationata
headtiltof45degrees(Bielschowsky
headtilttestintrochlearnervepalsy).
Asmallprojectorwithpositioning
crosshairsmountedonthepatient’s
foreheadpermitstheexaminertode-
terminethepatient’sheadtiltwitha
relativelyhighdegreeofprecision.
Thetiltoftheimage(paralyticstra-
bismusoftenleadstoimagetilting)
canalsobemeasuredwiththeHarms
tangenttable.Todoso,thefixation
lightinthecenterofthetableis
spreadintoabandoflight.
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511. 493
Table17.4Differentialdiagnosisbetweenconcomitantstrabismusandparalyticstra-
bismus
DifferentialcriterionConcomitantstrabismus Paralyticstrabismus
Onset Atanearlyage,initiallyonly
periodically.
Atanyage,suddenonset.
Cause Hereditary,uncorrected
refractiveerror,perinatal
injury.
Diseaseoforinjurytoocular
muscles,supplyingnerves,
ornuclei.
Diplopia None;imagesuppressed
(exceptinlatestrabismus
withnormalsensory
development).
Diplopiaispresent.
Compensatoryhead
posture
None. Constant.
Depthperception Notpresent. Onlypresentwhenpatient
assumescompensatory
headposture(seesymp-
toms).
Visualacuity Usuallyunilaterallyreduced
visualacuity.
Nochangeinvisualacuity.
Angleofdeviation Constantineverydirection
ofgaze.
Variable,increasinginthe
directionofpullofthepara-
lyzedmuscle.
table(Fig.17.18).Inadditiontotheverticalandhorizontalgraduationsofthe
Maddox’scross,theHarmstablealsohasdiagonals.Thesediagonalspermit
theexaminertomeasuretheangleofdeviationeveninpatientswithacom-
pensatoryheadtilt,suchascanoccurintrochlearnervepalsy.
Differentialdiagnosis:Table17.4showsthemostimportantdifferences
betweenparalyticstrabismusandconcomitantstrabismus.
Treatmentofophthalmoplegia:Surgeryforparalyticstrabismusshouldbe
postponedforatleastoneyeartoallowforpossiblespontaneousremission.
Preoperativediagnosticstudiestodeterminetheexactcauseareindicatedto
permittreatmentofapossibleunderlyingdisorder,suchasdiabetesmellitus.
Severediplopiamaybetemporarilymanagedbyalternatelypatchingthe
eyesuntilsurgery.Alternatively,aneyeglasslenswithaprismcorrectionfor
theparalyzedeyemaybeusedtocompensatefortheangleofdeviationand
eliminatediplopia.Eyeglasseswithnonrefractinglensesmaybeusedfor
patientswhodonotnormallywearcorrectivelenses.Prismlensesmaynot
alwaysbeabletocorrectextremestrabismus.Ifsurgeryisindicated,care
17.5OphthalmoplegiaandParalyticStrabismus
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512. 494
mustbetakentocorrectlygaugetheangleofdeviation.Thegoalofsurgeryis
toeliminatediplopiainthenormalvisualfield,i.e.,withheaderect,inboth
nearanddistancevision.Itwillnotbepossibletosurgicallyeliminate
diplopiaineveryvisualfield.
Procedure:Theantagonistoftherespectiveparalyzedmusclecanbeweakened
byrecession.Resectingordoublingtheparalyzedmusclecanadditionally
reducetheangleofdeviation.
Strabismussurgeryforophthalmoplegiaispossibleonlyafteraone-
yearregenerationperiod.
17.6 Nystagmus
Definition
Nystagmusreferstobilateralinvoluntaryrhythmicoscillationoftheeyes,
whichcanbejerkyorpendular(jerknystagmusandpendularnystagmus).
ThevariousformsofnystagmusarelistedinTable17.5.
Etiology:Theetiologyandpathogenesisofnystagmusremainunclear.Nys-
tagmusisalsoaphysiologicphenomenonthatmaybeelicitedbygazingat
rapidlymovingobjects.Anexampleofthisisoptokineticnystagmus,ajerk
nystagmusthatoccursinsituationssuchasgazingoutofamovingtrain.
Treatment:Wherenystagmuscanbereducedbyconvergence,prismswith
anoutwardfacingbasemaybeprescribed.Inspecialcases,suchaswhenthe
patientassumesacompensatoryheadposturetocontrolthenystagmus,
Kestenbaum’soperationmaybeindicated.Thisprocedureinvolvesparallel
shiftsinthehorizontalextraocularmusclessoastoweakenthemusclesthat
arecontractedinthecompensatorypostureandstrengthenthosethatare
relaxedinthisposture.
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513. 495
Table17.5Formsofnystagmus
Forms Onset Characteristics Typeofnystagmus
Ocular
nystagmus
Congenital
oracquired
inearly
childhood
!Occursinorganicdis-
ordersofbotheyes,such
asalbinism,cataract,
colorblindness,vitreous
opacification,ormacular
scarring.
!Significantvisualimpair-
ment.
!Secondarystrabismus
mayalsobepresent.
!Pendularnystagmus.
Congenital
nystagmus
Congenital
oracquired
inearly
childhood
(attheage
ofthree
months)
!Nystagmusisnotcurbed
byfixationbutexacer-
bated.
!Oscillationisusuallyhori-
zontal.
!Intensityvarieswiththe
directionofgaze(usually
lessinnearfixationthan
indistancefixation).
!Constantalternation
betweenpendular
andjerknystagmus.
Latent
nystagmus
Congenital
oracquired
inearly
childhood
!Alwaysassociatedwith
congenitalstrabismus.
!Manifestedonlybyspon-
taneouslyuncovering
oneeyewhenfixation
changes.
!Directionofoscillation
changeswhenfixation
changes(seeright
column).
!Rightoscillatingnys-
tagmusinrightfixa-
tion.
!Leftoscillatingnys-
tagmusinleftfixa-
tion.
!Nystagmusoccursas
jerknystagmus.
Fixation
nystagmus
Acquired !Occursindisordersofthe
brainstemorcerebellum
duetovascularinsults,
multiplesclerosis,
trauma,ortumors.
!Pendularorother
abnormalformof
oscillation.
Gazepalsy
nystagmus
AcquiredSeefixationnystagmus. !Jerkyoscillation.This
nystagmusis
especiallyapparentat
theonsetofmuscular
paralysiswhenthe
patientattemptsto
usethemusclethatis
becomingparalyzed.
17.6Nystagmus
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515. 497
18OcularTrauma
GerhardK.Lang
18.1ExaminationMethods
Theincidenceofocularinjuriesremainshighdespitetheincreaseinsafety
regulationsinrecentyears,suchasmandatoryseatbeltsandprotectiveeye-
wearforpersonsoperatinghigh-speedrotarymachinery.Thereforeitis
importantthateverygeneralpractitionerandhealthcarestaffmemberis
abletorecognizeanocularinjuryandprovideinitialtreatment.Thepatient
shouldthenbereferredtoanophthalmologist,whoshouldbesolely
responsibleforevaluationoftheinjuryanddefinitivetreatment.Thefollow-
ingdiagnosticoptionsareavailabletodeterminethenatureoftheinjury
moreprecisely.
Patienthistory:Obtainingathoroughhistorywillprovideimportantinfor-
mationaboutthecauseoftheinjury.
!Workwithahammerandchiselnearlyalwayssuggestsanintraocularfor-
eignbody.
!Cuttingandgrindingworksuggestscornealforeignbodies.
!Weldingandflamecuttingworksuggestsultravioletkeratoconjunctivitis.
Theexaminershouldalwaysascertainwhetherthepatienthasade-
quatetetanusimmunization.
Inspection(grossmorphologicexamination):Ocularinjuriesfrequently
causepain,photophobia,andblepharospasm.Afewdropsoftopicalanes-
theticarerecommendedtoallowtheinjuredeyetobeexaminedatrestwith
minimalpaintothepatient.Thecorneaandconjunctivaarethenexamined
forsignsoftraumausingafocusedlight,preferablyonecombinedwitha
magnifyingloupe(seeFig.1.11forexaminationtechnique).Theeyelidsmay
beevertedtoinspectthetarsalsurfaceandconjunctivalfornix.Aforeignbody
canthenberemovedimmediately.
Ophthalmoscopy:Examinationwithafocusedlightorophthalmoscopewill
permitgrossevaluationofdeeperintraocularstructures,suchaswhethera
vitreousorretinalhemorrhageispresent.Avitreoushemorrhagemaybe
identifiedbythelackofredreflexonretroillumination.Careshouldbetaken
toavoidunnecessarymanipulationoftheeyeinanobviouslysevereopen-
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516. 498
globeinjury(characterizedbyasoftglobe,pupildisplacedtowardthepene-
trationsite,prolapsediris,andintraocularbleedingintheanteriorchamber
andvitreousbody).Suchmanipulationmightotherwisecausefurtherdam-
age,suchasextrusionofintraocularcontents.
Toproperlyestimatetheurgencyoftreatingpalpebralandocular
trauma,itisparticularlyimportanttodifferentiatebetweenopen-
globeinjuriesandclosed-globeinjuries.Open-globeinjurieshave
highestpriorityduetotheriskoflosingtheeye.
18.2ClassificationofOcularInjuriesbyMechanismofInjury
!Mechanicalinjuries:
–Eyelidinjuries.
–Injuriestothelacrimalsystem.
–Conjunctivallaceration.
–Foreignbodyinthecorneaandconjunctiva.
–Cornealerosion.
–Nonpenetratinginjury(blunttraumatotheglobe).
–Injurytotheflooroftheorbit(blowoutfracture).
–Penetratinginjury(open-globeinjury).
–Impalementinjurytotheorbit.
!Chemicalinjuries.
!Injuriesduetophysicalagents:
–Burns.
–Radiationinjuries(ionizingradiation).
–Ultravioletkeratoconjunctivitis.
!Indirectoculartrauma:transienttraumaticretinalangiopathy(Purt-
scher’sretinopathy).
18.3MechanicalInjuries
18.3.1EyelidInjury
Etiology:Eyelidinjuriescanoccurinpracticallyeveryfacialinjury.Thefol-
lowingtypeswarrantspecialmention:
!Eyelidlacerationswithinvolvementoftheeyelidmargin.
!Avulsionsoftheeyelidinthemedialcanthuswithavulsionofthelacrimal
canaliculus.
Clinicalpicture:Thehighlyvascularizedandlooselytexturedtissueofthe
eyelidscausesthemtobleedprofuselywheninjured.Hematomaandswell-
ingwillbesevere(Fig.18.1).Abrasionsusuallyinvolveonlythesuperficiallay-
ersoftheskin,whereaspunctures,cuts,andalleyelidavulsionsduetoblunt
18OcularTrauma
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517. 499
trauma(suchasafist)frequentlyinvolvealllayers.Bitewounds(suchasdog
bites)areoftenaccompaniedbyinjuriestothelacrimalsystem.
Treatment:Surgicalrepairofeyelidinjuries,especiallylacerationswith
involvementoftheeyelidmargin,shouldbeperformedwithcare.Thewound
shouldbeclosedinlayersandtheedgesproperlyapproximatedtoensurea
smoothmarginwithouttensiontoavoidlatercomplications,suchascicatri-
cialectropion(Fig.18.2).
18.3.2InjuriestotheLacrimalSystem
Etiology:Lacerationsandtearsinthemedialcanthus(suchasdogbitesor
glasssplinters)candividethelacrimalduct.Obliterationofthepunctumand
lacrimalcanaliculusisusuallytheresultofaburnorchemicalinjury.Injury
tothelacrimalsacorlacrimalglandusuallyoccursinconjunctionwith
severecraniofacialtrauma(suchasakickfromahorseoratrafficaccident).
Dacryocystitisisacommonsequela,whichoftencanonlybetreatedby
surgery(dacryocystorhinostomy).
Clinicalpicture:SeeChapter3fordacryocystitis.SeeFig.18.3foravulsionof
thelowerlacrimalsystem(avulsionsinthemedialcanthus).
Treatment:Lacrimalsysteminjuriesarerepairedunderanoperatingmicro-
scope.Aring-shapedsiliconestentisadvancedintothecanaliculususinga
specialsound(Figs.18.3b–f).Thesiliconestentremainsinsituforthreeto
fourmonthsandisthenremoved.
Surgicalrepairofeyelidandlacrimalsysteminjuriesmustbeperformed
byanophthalmologist.
18.3.3ConjunctivalLaceration
Epidemiology:Duetoitsexposedposition,thinness,andmobility,thecon-
junctivaissusceptibletolacerations,whichareusuallyassociatedwithsub-
conjunctivalhemorrhage.
Etiology:Conjunctivallacerationsmostcommonlyoccurasaresultofpene-
tratingwounds(suchasfrombendingoveraspiked-leafpalmtreeorfroma
branchthatsnapsbackontotheeye).
Symptomsanddiagnosticconsiderations:Thepatientexperiencesafor-
eignbodysensation.Usuallythiswillberathermild.Examinationwillreveal
circumscribedconjunctivalreddeningorsubconjunctivalhemorrhageinthe
injuredarea.Occasionallyonlyapplicationoffluoresceindyetotheinjury
willrevealthesizeoftheconjunctivalgap.
18.3MechanicalInjuries
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518. 500
Lacerationoftheupperandlowereyelidswithavulsionofthe
lacrimalsystem.
Fig.18.1aThe
injuryhasexpos-
edthecornea.
Thepatientisun-
abletoclosethe
eye,andthecor-
neaandconjunc
tivacannolonger
bemoistened.
bPostoperative
findings.
Continued!
18OcularTrauma
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519. 501
Lacerationoftheupperandlowereyelidswithavulsionofthe
lacrimalsystem(continued).
Fig.18.1cFind-
ingstwomonths
postoperatively
aftertreatingthe
woundwith
placementofa
plasticstentin
situ(seealso
Fig.18.3forsur-
gicaltechnique).
Cicatricialectropionintheleftlowereyelidafterimproperrepair.
Fig.18.2Failuretoclosethewoundinlayerswithoutcreatingtensioninthe
woundresultsinascarthat“pulls”thelowereyeliddownward.
18.3MechanicalInjuries
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520. 502
Surgicaltreatmentofavulsionoftheeyelidwithavulsionofthe
lacrimalsystem(bicanicularringintubation).
Fig.18.3a–e
Continued!
b c
d e
18OcularTrauma
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521. 503
Treatment:Minorconjunctivalinjuriesdonotrequiretreatmentasthecon-
junctivahealsquickly.Largerlacerationswithmobileedgesareapproxi-
matedwithabsorbablesutures.
Thepossibilityofaperforatinginjuryshouldalwaysbeconsideredin
conjunctivalinjuries.Whenthewoundistreated,thephysicianshould
inspecttheunderlyingscleraafterapplicationoftopicalanesthetic.
18.3.4CornealandConjunctivalForeignBodies
Epidemiology:Foreignbodiesonthecorneaandconjunctivaarethecom-
monestocularemergencyencounteredbygeneralpractitionersandophthal-
mologists.
Etiology:Airborneforeignbodiesandmetalsplintersfromgrindingorcut-
tingdisksinparticularoftenbecomelodgedintheconjunctivaorcorneaor
burntheirwayintothetissue.
Symptomsanddiagnosticconsiderations:Thepatientexperiencesafor-
eign-bodysensationwitheveryblinkoftheeye.Thisisaccompaniedbyepi-
phora(tearing)andblepharospasm.Dependingonthetimeelapsedsincethe
Surgicaltreatmentofavulsionoftheeyelidwithavulsionofthe
lacrimalsystem(bicanicularringintubation)(continued).
Fig.18.3f
aFindingspriortotreatmentofthewound.bThepigtailsoundisintroduced
throughtheuninjuredsuperiorlacrimalcanaliculus.Nowthesiliconetubecanbein-
troducedatthemedialmarginofthewoundandpulledthrough.cNextthesoundis
advancedintothepunctumoftheinjuredcanaliculustograsptheotherendofthe
siliconetube.dFinally,theendsofthetubearejoinedtoformaring.eandfSurgical
siteaftertherepair.
18.3MechanicalInjuries
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522. 504
injury,i.e.,afterafewhoursorseveraldays,conjunctivalorciliaryinjection
willbepresent(Figs.18.4aandb).Theforeignbodiesontheconjunctivaor
corneaarethemselvesoftensosmallthattheyarevisibleonlyunderloupe
magnification.Theremaybevisibleinfiltrationoraringofrust.Wherethere
isnovisibleforeignbodybutfluoresceindyerevealsverticalcornealstria-
tions,theforeignbodywillbebeneaththetarsus(seeFig.5.11).
Cornealandconjunctivalforeignbodiesandthereamerusedtoremove
them.
Fig.18.4aCon-
junctivalforeign
body(lodged
grainkernel)on
thelimbusofthe
corneawithcon-
junctivalinjec-
tion.
bForeignbody
thathasburned
itswayintothe
cornea.Whilethe
patientwasusing
agrinderwithout
protectiveeye-
weartheprevious
day,asplinter
flewintheeye
(arrow)thatnow
exhibitsaslight
haloofvisiblein-
filtration.Note
theconjunctival
andciliaryinjec-
tionatthesiteoftheforeignbody(seealsoFig.4.6).
Continued!
18OcularTrauma
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523. 505
Cornealandconjunctivalforeignbodiesandthereamerusedtoremove
them(continued).
Fig.18.4c
Reamerusedto
reamoutthede-
fectcreatedby
theforeignbody.
Aforeign-bodysensationwitheveryblinkoftheeyeaccompaniedby
epiphora,blepharospasm,andverticalstriationsonthesurfaceofthe
corneaaretypicalsignsofasubtarsalforeignbody.
Treatment:Cornealandconjunctivalforeignbodies.Theforeignbodyis
priedoutofitsbedwithafineneedleorcannula.Thedefectcreatedbythe
foreignbodywilloftenbecontaminatedwithrustorinfiltratedwithleuko-
cytes.Thisdefectiscarefullyreamedoutwithadrill(Fig.18.4c)andtreated
withanantibioticeyeointmentandbandagedifnecessary.
Subtarsalforeignbodies.Evertingtheupperandlowereyelidswillusually
revealtheforeignbody,whichmaythenberemovedwithamoistcotton
swab.Anantibioticeyebandageisplaceduntilthepatientiscompletelyfree
ofsymptoms.
18.3.5CornealErosion
Etiology:Thisdisorderfollowsinitialtraumatothesurfacecornea,suchas
thefingernailofachildcarriedintheparent’sarms,aspiked-leafpalmtree,
orabranchthatsnapsbackontotheeye.Properlytreated,thisepithelial
defectusuallyhealswithinashorttime,i.e.,24to48hoursdependingonthe
sizeofthedefect.However,occasionallytheepithelialcellsdonotproperly
adheretoBowman’slayersothattheepitheliumrepeatedlyrupturesatthe
siteoftheinitialinjury.Thischaracteristicallyoccursinthemorningwhen
thepatientwakesupandsuddenlyopenshisorhereyes.Thisrecurringero-
sionoftencreatessevereemotionalstressforthepatient.
18.3MechanicalInjuries
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524. 506
Symptomsanddiagnosticconsiderations:Immediatelyaftertheinjury,
thepatientexperiencesasevereforeign-bodysensationassociatedwithtear-
ing.Becausethereisactuallyadefectinthesurfaceofthecornea,thepatient
hasthesubjectivesensationofaforeignbodywithintheeye.Theepithelial
defectcausesseverepain,whichimmediatelyelicitsablepharospasm.Addi-
tionalsymptomsassociatedwithcornealerosionincludeimmediateeyelid
swellingandconjunctivalinjection.Fluoresceinsodiumdyewillreadily
revealthecornealdefectwhentheeyeisexaminedthroughabluelight
(Fig.18.5).
Treatment:Anantibioticointmenteyebandageisused.
Treatmentofrecurrentcornealerosionoftenrequireshospitalization.
Bilateralbandagesareplacedtoensurethattheeyesarecompletely
immobilized.
18.3.6BluntOcularTrauma(OcularContusion)
Epidemiologyandetiology:Ocularcontusionsresultingfromblunttrauma
suchasafist,ball,champagnecork,stone,fallingontheeye,oracow’shorn
areverycommon.Significantdeformationoftheglobecanresultwherethe
diameterofthebluntobjectislessthanthatofthebonystructuresofthe
orbit.
Clinicalpictureanddiagnosticconsiderations:Deformationexertssignifi-
canttractiononintraocularstructuresandcancausethemtotear.Oftenthere
willbebloodintheanteriorchamber,whichwillinitiallypreventthe
examinerfromevaluatingthemoreposteriorintraocularstructures.
Cornealerosion.
Fig.18.5The
epithelialdefect
inthecorneais
readilyvisible
whentheeyeis
examined
throughablue
lightafterad-
ministrationof
fluorescein
sodiumdye.
18OcularTrauma
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525. 507
Donotadministermedicationsthatactonthepupilasthereisariskof
irreversiblemydriasisfromasphinctertear,andpupillarymovements
increasetheriskofsubsequentbleeding.Theposteriorintraocular
structuresshouldonlybethoroughlyexaminedinmydriasistodeter-
minetheextentofinjuryafteraweektotendays.
CommoninjuriesarelistedinTable18.1andFig.18.6.
Latesequelaeofbluntoculartraumainclude:
!Secondaryglaucoma.
!Retinaldetachment.
!Cataract.
Latesequelaeofbluntoculartraumamayoccuryearsaftertheinjury.
Treatment:Thisinvolvesimmobilizingtheeyeinitially,toallowintraocular
bloodtosettle.SeeTable18.1fordetails.
Subsequentbleedingthreeorfourdaysaftertheinjuryiscommon.
18.3.7Blow-outFracture
Etiology(seealsobluntoculartrauma):Blow-outfracturesoftheorbitresult
fromblunttrauma.Bluntobjectsofsmalldiameter,suchasafist,tennisball,
orbaseball,cancompressthecontentsoftheorbitsoseverelythatorbital
wallfractures.Thisfractureusuallyoccurswheretheboneisthinnest,along
thepaper-thinflooroftheorbitoverthemaxillarysinus.Thering-shapedbony
orbitalrimusuallyremainsintact.Thefracturecanresultinprotrusionand
impingementoforbitalfatandtheinferiorrectusanditssheathsinthefrac-
turegap.Wherethemedialethmoidwallfracturesinsteadoftheorbitalfloor,
emphysemaintheeyelidswillresult.
Symptomsanddiagnosticconsiderations:Themoreseverethecontusion,
themoreseveretheintraocularinjuriesandresultingvisualimpairmentwill
be.Impingementoftheinferiorrectuscanresultindiplopia,especiallyin
upwardgaze.Initially,thediplopiamaygounnoticedwhentheeyeisstill
swollenshut.Alargebonedefectmayresultindisplacementoflargerpor-
tionsofthecontentsoftheorbitalcavity.Theeyemayrecedeintotheorbit
(enophthalmos)andthepalpebralfissuremaynarrow.Injurytotheinfraor-
bitalnerve,whichcoursesalongtheflooroftheorbit,mayresult.Thiscan
causehypesthesiaofthefacialskin.
Crepitusuponpalpationduringexaminationoftheeyelidswellingisa
signofemphysemaduetocollapseoftheethmoidalaircells.Thecrepitusis
causedbyairenteringtheorbitfromtheparanasalsinuses.Thepatient
shouldrefrainfromblowinghisorhernoseforthenextfourorfivedaysto
avoidforcingairorgermsintotheorbit.Radiographsshouldbeobtained
andanear,nose,andthroatspecialistconsultedtohelpdeterminetheexact
18.3MechanicalInjuries
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526. 508
Possibleocularinjuriesresultingfromblunttrauma.
Orbital floor
fracture with
impingement
of the inferior
rectus
Sphincter tear Iridodialysis
Hyphema
Retrobulbar
and eyelid
hematoma
T
R
A
U
M
A
Fig.18.6SeetextandTable18.1fordetails.
18OcularTrauma
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527. 509
Traumatic
cataract
(contusion
rosette)
Choroidal rupture
Retinal
contusion
(Berlin's
edema)
Traumatic retinochoroidopathy
Avulsion
of the
optic nerve
Tear in the
ora serrata Subluxation of the lens
Avulsion of the globe
18.3MechanicalInjuries
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528. 510
Table18.1Overviewofpossibleinjuriesresultingfromblunttraumatotheglobe
Description
ofinjury
Definition Sequelae Treatment
IridodialysisAvulsionofthe
rootoftheiris.
!Lossofpupillary
roundness.
!Increasedglare.
!Opticalimpairment
resultsifthereisa
largegapatthe
palpebralfissurelead-
ingtoa‘double
pupil’.
Sutureofthebaseof
theirisisindicatedfor
severeinjuries(patient
hastwopupilsdueto
severeavulsion;see
Fig.18.6).Othercases
donotrequiretreat-
ment.
Traumatic
aniridia
Totalavulsion
oftheiris.
Patientsuffersfrom
increasedglare.
!Sunglasses.
!Whereasimul-
taneouscataractis
present,ablack
prostheticlenswith
anopticalaperture
thesizeofthepupil
isinsertedduring
cataractsurgery.
Recessionof
theangle
Wideningof
theangleof
theanterior
chamber.
Latesequela:secondary
glaucoma.
SeeChapter10.
CyclodialysisAvulsionofthe
ciliarybody
fromthe
sclera.
!Intraocularhypotonia
withchoroidalfolds
andopticdisk
edema.
!Visualimpairment.
Theciliarybodymust
bereattachedwith
suturestoprevent
phthisisbulbi(shrink-
ageoftheeyeball).
Subluxationof
thelens
Avulsionofthe
zonulefibers.
!Dislocationofthe
lensandiridodonesis.
!Decreasedvisualacu-
ity.
Removalofthelens
andimplantationofa
prostheticlens;see
Chapter7.
Vitreous
detachment
Separationof
thebaseofthe
vitreousbody.
Patientseesfloaters
(seeChapter11).
SeeChapter11.
Continued!
18OcularTrauma
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529. 511
Table18.1(Continued)
Description
ofinjury
Definition Sequelae Treatment
Avulsionof
theoraser-
rata
Avulsionofthe
peripheralret-
ina(oraser-
rata).
Retinaldetachment
resultinginflashesof
light,shadows,and
blindness.
Retinalsurgery;see
Chapter12.
SphinctertearTearinthe
sphincter
pupillaewith
elongationof
theiris.
Traumaticmydriasisor
impairedpupillaryfunc-
tionmaybepresent.
Sunglassesareindi-
cated.Otherwiseno
treatmentispossible.
Contusion
rosette
Traumaticlens
opacity(trau-
maticcata-
ract).
!Rosette-shapedsub-
capsularopacityon
theanteriorsurface
ofthelens,which
withtimemigrates
intothedeepercor-
texduetothe
appositionoflens
fibersyetotherwise
remainsunchanged.
!Patientsuffersfrom
graduallyincreasing
lossofvisualacuity.
Opacityintheoptical
centerisroutinelyan
indicationforsurgery
(seeChapter7,for
detailsofsurgery).
Berlin’s
edema
Retinaland
macularedema
attheposterior
poleofthe
globe(contre-
couplocation)
possiblyasso-
ciatedwith
bleeding.
Lossofvisualacuity. Watch-and-wait
approachisadvised
untilswellingrecedes.
Choroidalrup-
tures
Crescenticcon-
centricchoro-
idaltears
aroundthe
pupil.
Tearsthatextend
throughthemaculacan
resultindecreased
visualacuity.
Notreatmentis
possible.Watch-and-
waitapproachis
adviseduntilscarring
develops.
Continued!
18.3MechanicalInjuries
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530. 512
Table18.1(Continued)
Description
ofinjury
Definition Sequelae Treatment
Traumatic
retinochoro-
idopathy
Choroidaland
retinalatrophy
duetoavulsion
orimpinge-
mentofthe
shortposterior
ciliaryarteries.
Lossofvisualacuity. Notreatmentispos-
sible.
Avulsionof
theglobe
Traumaticavul-
sionofthe
globeoutof
theorbit,
frequently
associatedwith
avulsionofthe
opticnerve
(seenextrow).
Immediateblindness.Enucleation.
Avulsionof
theoptic
nerve
Avulsionofthe
entireoptic
nerveatits
pointofentry
intotheglobe.
Immediateblindness.Theseparationofthe
nervefibersisirrevers-
ible.
Injurytothe
opticnerve
Possibleinju-
riesinclude:
!Hematoma
oftheoptic
nervesheath.
!Opticnerve
contusion.
!Fractureof
theoptic
nervecanal.
Atrophyoftheoptic
nervewithlossofvisual
acuityandvisualfield
defects.
Notreatmentispos-
sible.
Retrobulbar
hematoma
Injurytoretro-
bulbarvascular
structures.
!Orbitalbleeding.
!Eyelidhematoma.
!Exophthalmos.
!Waitforbloodtobe
absorbed.
!Surgeryisindicated
onlywhenthecen-
tralretinalarteryis
occludedbypres-
sure.
Continued!
18OcularTrauma
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531. 513
Table18.1(Continued)
Description
ofinjury
Definition Sequelae Treatment
Hyphema Bleedinginthe
anteriorcham-
ber.
Patienthasblurred
vision.
!Patientshould
assumeanupright
posturetoallow
bloodtosettle.This
willrestorevision.
!Hyphemawill
resolvespon-
taneously.
Vitreous
hemorrhage
Bleedinginto
thevitreous
chamber.
!Identifiedbythelack
ofredreflexonretro-
illuminationduring
ophthalmoscopy.
!Lossofvisualacuity.
Waitforspontaneous
recession.
Orbitalfrac-
ture(blow-
outfracture)
Fractureofthe
floorofthe
orbitwithdis-
placementinto
themaxillary
sinus.
!Diplopiainthe
affectedeye.
!Elevationordepres-
siondeficit.
!Patientshould
refrainfromblowing
hisorhernoseif
paranasalsinuses
areinvolved(crepi-
tusuponpalpation).
!Surgicalrepairof
theorbitalfloorand
releaseofimpinged
orbitalcontents.
locationofthefracture.CTstudiesaremorepreciseandmaybeindicated
toevaluatedifficultcases.
Tissuedisplacedintothemaxillarysinuswillresembleahangingdropof
waterintheCTimage.
Treatment:Surgerytorestorenormalanatomyandtheintegrityoftheorbit
shouldbeperformedwithintendays.Thisminimizestheriskofirreversible
damagefromscarringoftheimpingedinferiorrectus.Wheretreatmentis
prompt,theprognosisisgood(seeSection15.8fororbitalsurgery).
Tetanusprophylaxisandtreatmentwithantibioticsarecrucial.
18.3MechanicalInjuries
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532. 514
18.3.8Open-GlobeInjuries
Etiology:Togetherwithseverechemicalinjuries,open-globeinjuriesarethe
mostdevastatingformsofoculartrauma.Theyarecausedbysharpobjects
thatpenetratethecorneaandsclera.Adistinctionismadebetweenpenetra-
tionwithandwithoutanintraocularforeignbody.However,evenblunt
traumacancauseanopen-globeinjuryinaneyeweakenedbyprevious
surgeryorinjurywhereextremelyhigh-energyforcesareinvolved(suchas
fallingonacaneorablowfromacow’shorn).
Clinicalpictureanddiagnosticconsiderations:Penetratinginjuriescover
theentirespectrumofclinicalsyndromes.Symptomscanrangefrommassive
penetrationofthecorneaandsclera(Fig.18.7)withlossoftheanteriorcham-
bertotiny,nearlyinvisibleinjuriesthatclosespontaneously.Thelattermay
includeafinepenetratingwoundortheentrywoundofaforeignbody.
Dependingontheseverityoftheinjury,thepatient’svisualacuitymaybe
severelycompromisedornotinfluencedatall.
Oneofthemostcommonsequelaeisatraumaticcataract.Therupturein
thelenscapsuleallowsaqueoushumortopenetrate,causingthelenstoswell.
Thisresultsinlensopacificationofvaryingseverity.Largedefectswillleadto
totalopacificationofthelenswithinhoursorafewdays.Smallerdefectsthat
closespontaneouslyoftencauseacircumscribedopacity.Typically,penetra-
tionresultsinarosette-shapedanteriororposteriorsubcapsularopacity.
Dependingontheseverityoftheinjury,thefollowingdiagnosticsignswill
bepresentinanopen-globeinjury:
!Theanteriorchamberwillbeshalloworabsent.
!Thepupilwillbedisplacedtowardthepenetrationsite.
Penetratinginjury.
Fig.18.7Open-
globeinjuryfrom
astapleinvolving
thecornea,iris,
lens,sclera,and
retina.
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533. 515
!Swellingofthelenswillbepresent(traumaticcataract).
!Therewillbebleedingintheanteriorchamberandvitreousbody.
!Hypotoniaoftheglobewillbepresent.
Theruptureofthelenscapsuleandvitreoushemorrhageoftenrenderexami-
nationdifficultastheypreventdirectinspection.Thesecases,andanypatient
whosehistorysuggestsanintraocularforeignbody,requireoneorbothofthe
followingdiagnosticimagingstudies:
!Radiographsintwoplanestodeterminewhetherthereisaforeignbodyin
theeye.
!CTstudies,thatpermitpreciselocalizationoftheforeignbodyandcanalso
imageradiolucentforeignbodiessuchasplexiglas.
Aninjurysustainedwhileworkingwithahammerandchiselsuggests
anintraocularforeignbody.Thediagnosismaybeconfirmedbyexa-
miningthefundusinmydriasisandobtainingradiographicstudies.
Treatment:Firstaid.Wherepenetratingtraumaissuspected,asterileband-
ageshouldbeappliedandthepatientreferredtoaneyeclinicfortreatment.
Tetanusimmunizationorprophylaxisandprophylacticantibiotictreatment
areindicatedasamatterofcourse.
Surgery.Surgicaltreatmentofpenetratinginjuriesmustincludesuturingthe
globeandreconstructingtheanteriorchamber.Anyextrudedintraocular
tissue(suchastheiris)mustberemoved.Intraocularforeignbodies(Figs.
18.8aandb)shouldberemovedwhenthewoundisrepaired(i.e.,byvit-
rectomyandextractionoftheforeignbody).
Latesequelae:
!Improperreconstructionoftheanteriorchambermayleadtoadhesions
betweentheirisandtheangleoftheanteriorchamber,resultinginsec-
ondaryangleclosureglaucoma.
!Aretinalinjury(forexampleatthesiteoftheimpactoftheforeignbody)
canleadtoretinaldetachment.
!Failuretoremoveironforeignbodiescanleadtoocularsiderosis,which
causesirreparabledamagetothereceptorsandmaymanifestitselfyears
later.
!Copperforeignbodiescausesevereinflammatoryreactionsintheeye
(ocularchalcosis)withinafewhours.Symptomsrangefromuveitisand
hypopyontophthisisbulbi(shrinkageandhypotoniaoftheeyeball).
!Organicforeignbodies(suchaswood)intheeyeleadtofulminant
endophthalmitis.
18.3.9ImpalementInjuriesoftheOrbit
Etiology:Impalementinjuriesoccurmostfrequentlyinsituationssuchas
these:
18.3MechanicalInjuries
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534. 516
Intraocularforeignbodysustainedwhileworkingwithahammerandchisel.
Fig.18.8aThe
ironsplinteris
lodgedinthe
lens;thecornea
hasclosedspon-
taneouslyimme-
diatelyafterthe
injury(white
arrow).Asphinc-
terinjury
isalsopresent
(blackarrow).
bTheironsplin-
terentered
throughthe
scleraandisnow
lodgedintheret-
inaontheposte-
riorwallofthe
globe,whichit
has“coagulated”
(whitediscolora-
tionofthesur-
roundingretinal
tissue).Focal
burnsareplaced
aroundthefor-
eignbodywith
anargonlasertofixtheretinabeforeavitrectomyisperformedtoremovethe
foreignbody.
!Childrenmayfallonpencilsheldintheirhands(Fig.18.9).
!Injuriesmayresultfromtheactionsofotherpersons(suchasarrowsor
darts).
!Aknifemayslipwhileabutcherisremovingabonefromacutofmeat.
Oftentheimpaling“stake”willglanceofftheroundhardouterlayerofthe
globe(corneaandsclera)andlodgeinthesofttissueoftheorbit.
Symptomsanddiagnosticconsiderations:Thestakecancausedisplace-
mentoftheglobe.Oftentherewillbeminimalbleedinginthesurrounding
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535. 517
Impalementinjuryoftherightorbit.
Fig.18.9Orbitalinjurywithoutin-
jurytotheglobefollowingafallona
pencilheldinthepatient’shand.
(PhotographcourtesyofProf.W.D.
Green,M.D.,Baltimore,Maryland.)
tissue.Diagnosticstudiesusedtoascertainpossibledamagetointraocular
structuresincludeophthalmoscopy,radiographicstudies,andultrasound.
Treatment:Firstaidtreatmentshouldleavethestakeinsitu.Removingthe
stakecouldcauseseverebleedingandorbitalhematoma.Ifnecessary,the
stakeshouldbestabilizedbeforethepatientistransportedtotheeyeclinic.
Oncethepatientisintheclinic,theforeignbodyisremovedfromtheorbit
andtheintegrityoftheglobeisverified,dependingonspecificfindings.Any
bleedingiscontrolled.Prophylacticantibiotictreatmentisindicated
routinelytominimizetheriskoforbitalcellulitis.
18.4ChemicalInjuries
Etiology:Chemicalinjuriescanbecausedbyavarietyofsubstancessuchas
acids,alkalis,detergents,solvents,adhesives,andirritantsliketeargas.
Severitymayrangefromslightirritationoftheeyetototalblindness.
Chemicalinjuriesareamongthemostdangerousocularinjuries.First
aidatthesiteoftheaccidentiscrucialtominimizetheriskofsevere
sequelaesuchasblindness.
18.4ChemicalInjuries
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536. 518
Asageneralrule,acidburnsarelessdangerousthanalkaliburns.Thisis
becausemostacidsdonotactdeeply.Acidsdifferfromalkalisinthatthey
causeimmediatecoagulationnecrosisinthesuperficialtissue.Thishasthe
effectofpreventingtheacidfrompenetratingdeepersothattheburniseffec-
tivelyaself-limitingprocess.However,someacidspenetratedeeplylikealka-
lisandcausesimilarlysevereinjuries.Concentratedsulfuricacid(suchas
fromanexplodingcarbattery)drawswateroutoftissueandsimultaneously
developsintenseheatthataffectseverylayeroftheeye.Hydrofluoricacid
andnitricacidhaveasimilarpenetratingeffect.
Alkalisdifferfrommostacidsinthattheycanpenetratebyhydrolyzing
structuralproteinsanddissolvingcells.Thisisreferredtoasliquefactive
necrosis.Theythencausesevereintraoculardamagebyalkalizingthe
aqueoushumor.
Symptoms:Epiphora,blepharospasm,andseverepainaretheprimary
symptoms.Acidburnsusuallycauseimmediatelossofvisualacuitydueto
thesuperficialnecrosis.Inalkaliinjuries,lossofvisualacuityoftenmanifests
itselfonlyseveraldayslater.
Clinicalpictureanddiagnosticconsiderations:Properdiagnosisofthe
causeandseverityoftheburniscrucialtotreatmentandprognosis.
Alkaliburnsmayappearlesssevereinitiallythanacidburnsbutthey
leadtoblindness.
Morphologicfindingsandtheresultingprognosiscanvarygreatlydepending
ontheseverityanddurationofexposuretothecausticagent.Thisinforma-
tionissummarizedinTable18.2.
Treatment:Firstaidrenderedatthesceneoftheaccidentoftendecidesthe
fateoftheeye.Thefirstfewsecondsandminutesandresoluteactionbyper-
sonsatthescenearecrucial.Immediatecopiousirrigationoftheeyemaybe
performedwithanywaterysolutionofneutralpH,suchastapwater,mineral
water,softdrinks,coffee,tea,orsimilarliquids.Milkshouldbeavoidedasit
theincreasespenetrationoftheburnbyopeningtheepithelialbarrier.Asec-
ondpersonmustrigorouslyrestrainthesevereblepharospasmtoalloweffec-
tiveirrigation.Atopicalanesthetictorelievetheblepharospasmwillrarelybe
availableatthesceneoftheaccident.Coarseparticles(suchaslimeparticles
inalimeinjury)shouldbeflushedandremovedfromtheeye.Onlyafterthese
actionshavebeentakenshouldthepatientbebroughttoanophthalmologist
oreyeclinic.
Chronologyoftreatmentofchemicalinjuries:
!Firstaidatthesceneoftheaccident(coworkersorfamilymembers):
–Restrainblepharospasmbyrigorouslyholdingtheeyelidsopen.
18OcularTrauma
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537. 519
T
able
1
8
.
2
F
indings
in
c
hemical
injuries
of
v
a
rious
degrees
of
se
v
e
rity
Se
v
e
rity
of
the
injur
y
Damage
t
o
t
he
corneal
epithelium
Damage
t
o
t
he
conjunctiv
a
Damage
t
o
t
he
corneal
stroma
Intr
aocular
in
v
olv
e-
ment
Prognosis
Slight
!
Super
ficial
punc-
t
a
t
e
k
e
ratitis.
!
N
o
c
o
r
n
e
a
l
e
r
o
-
sion.
!
Conjunctiv
al
epithelium
largely
int
act.
!
Slight
c
hemosis
(edemat
ous
con-
junctiv
al
sw
elling)
Clear
.
None.
Good:
Healing
without
loss
of
func-
tion.
Moderat
e
t
o
s
e
v
e
r
e
Moderat
e
t
o
t
o
t
al
corneal
erosion.
!
Moderat
e
c
hemo-
sis
!
Segment
al
isc
hemia
of
the
limbal
v
essels.
Slightly
opacified.
Slight
irrit
ation
of
the
ant
erior
c
hamber
(slight
amount
of
cellular
and
pro
t
ein
e
xudat
e
in
t
he
ant
e-
rior
c
hamber).
Def
ect
healing
with
functional
impair-
ment
and
possibly
symblepharon.
S
e
v
e
r
e
T
o
t
a
l
c
o
r
n
e
a
l
e
r
o
s
io
n
including
erosion
of
the
conjunctiv
al
epithelium
at
the
limbus.
!
Se
v
e
re
c
hemosis.
!
T
o
t
a
l
isc
hemia
of
the
limbal
v
essels.
All
la
y
e
r
s
are
opacified
(“cook
ed
fish
e
y
e”;
see
F
ig.
1
8
.
1
1
).
!
S
e
v
e
r
e
ir
r
it
a
t
io
n
of
the
ant
erior
c
hamber
.
!
Damage
t
o
t
he
iris,
lens,
ciliar
y
body
,
and
angle
of
the
ant
erior
c
hamber).
!
P
oor
.
!
Def
ect
healing
with
functional
impairment
that
ma
y
include
loss
of
t
h
e
e
y
e
.
!
Symblepharon.
18.4ChemicalInjuries
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538. 520
–Irrigatetheeyewithinsecondsoftheinjuryusingtapwater,mineral
water,softdrinks,coffee,tea,orsimilarliquids.Carefullyremovecoarse
particlesfromtheconjunctivalsac.
–Notifytherescuesquadatthesametime.
–Transportthepatienttothenearestophthalmologistoreyeclinic.
!Treatmentbytheophthalmologistorattheeyeclinic:
–Administertopicalanesthesiatorelievepainandneutralize
blepharospasm.
–Withtheupperandlowereyelidsfullyeverted,carefullyremovesmall
particlessuchasresiduallimefromthesuperiorandinferiorconjuncti-
valfornicesunderamicroscopeusingamoistcottonswab.
–Flushtheeyewithabuffersolution.Long-termirrigationusinganirri-
gatingcontactlensmaybeindicated(thelensisconnectedtoacannula
toirrigatetheeyewithaconstantstreamofliquid).
–Initiatesystemicpaintherapyifindicated.
!Additionaltreatmentonthewardinaneyeclinic:
Thefollowingtherapeuticmeasuresforseverechemicalinjuriesareusu-
allyperformedontheward:
–Continueirrigation.
–Initiatetopicalcortisonetherapy(dexamethasone0.1%eyedropsand
prednisolone1%eyedrops).
–Administersubconjunctivalsteroids.
–Immobilizethepupilwithatropine1%eyedropsorscopolamine0.25%
eyedropstwicedaily.
–Administeranti-inflammatoryagents(twooraldosesof100mg
indomethacinordiclofenac)or50–200mgsystemicprednisolone.
–AdministeroralandtopicalvitaminCtoneutralizecytotoxicradicals.
–Administer500mgoforalacetazolamide(Diamox)toreduceintraocu-
larpressureasprophylaxisagainstsecondaryglaucoma.
–Administerhyaluronicacidforcornealcaretopromotere-epithelial-
izationandstabilizethephysiologicbarrier.
–Administertopicalantibioticeyedrops.
–Debridenecroticconjunctivalandcornealtissueandmakeradialinci-
sionsintheconjunctiva(Passow’smethod)todrainthesubconjunctival
edema.
!Additionalsurgicaltreatmentinthepresenceofimpairedwoundheal-
ingfollowingextremelyseverechemicalinjuries:
–Aconjunctivalandlimbaltransplantation(stemcelltransfer)can
replaceloststemcellsthatareimportantforcornealhealing.Thiswill
allowre-epithelialization.
–Wherethecorneadoesnotheal,cyanoacrylategluecanbeusedto
attachahardcontactlens(artificialepithelium)topromotehealing.
18OcularTrauma
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539. 521
–ATenon’scapsuloplasty(mobilizationandadvancementofaflapof
subconjunctivaltissueofTenon’scapsuletocoverdefects)canhelpto
eliminateconjunctivalandscleraldefects.
!Latesurgicaltreatmentaftertheeyehasstabilized:
–Lysisofsymblepharon(symblepharonreferstoadhesionsbetweenthe
palpebralandbulbarconjunctiva;seealsoprognosisandcomplica-
tions)toimprovethemotilityoftheglobeandeyelids.
–Plasticsurgeryoftheeyelidstothereleasetheglobe.Thisshouldbe
onlyperformed12to18monthsaftertheinjury).
–Wherethereistotallossofthegobletcells,transplantationofnasal
mucosausuallyrelievespain(thelackofmucusissubstitutedbygoblet
cellsfromthenasalmucosa).
–Penetratingkeratoplasty(seeChapter5)maybeperformedtorestore
vision.Becausethetraumatizedcorneaishighlyvascularized
(Fig.18.10),theseproceduresareplaguedbyahighincidenceofgraft
rejection.Aclearcorneacanrarelybeachievedinaseverelyburned
eyeevenwithaHLA-typedcornealgraftandimmunosuppressivether-
apy.
Prognosisandpossiblecomplications:Thedegreeofischemiaofthecon-
junctivaandthelimbalvesselsisanindicatoroftheseverityoftheinjuryand
theprognosisforhealing(seeTable18.2).Thegreatertheischemiaofthecon-
junctivaandlimbalvessels,themoreseveretheburnwillbe.Themostsevere
formofchemicalinjurypresentsasa“cookedfisheye”(Fig.18.11)forwhich
theprognosisisverypoor,i.e.,blindnessispossible.
Moderatetoseverechemicalinjuriesinvolvingthebulbarandpalpe-
bralconjunctivacanresultinsymblepharon(adhesionsbetweenthepalpe-
Limeinjury.
Fig.18.10
Superficialand
deepcorneal
vascularizationis
present,andthe
eyeisdrydueto
lossofmostof
thegobletcells.
18.4ChemicalInjuries
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540. 522
“Cookedfisheye”followingalkaliinjury.
Fig.18.11The
corneaiswhite
aschalkand
opaque.The
vascularsupplyto
thelimbus(capil-
lariesatitsedge)
hasbeenoblit-
erated.
Symblepharon.
Fig.18.12
Moderateand
severechemical
injuriesmaypro-
duceadhesions
betweenthepal-
pebralandbulbar
conjunctiva.
bralandbulbarconjunctiva;Fig.18.12).Inflammatoryreactionsintheante-
riorchambersecondarytochemicalinjuriescanleadtosecondaryglau-
coma.
18OcularTrauma
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541. 523
18.5InjuriesDuetoPhysicalAgents
18.5.1UltravioletKeratoconjunctivitis
Etiology:Injuryfromultravioletradiationcanoccurfromweldingwithout
propereyeprotection,exposuretohigh-altitudesunlightwiththeeyesopen
withoutpropereyeprotection,orduetosunlightreflectedoffsnowwhen
skiingathighaltitudesonasunnyday.Intenseultravioletlightcanleadto
ultravioletkeratoconjunctivitiswithinashorttime(forexamplejustafew
minutesofweldingwithoutpropereyeprotection).Ultravioletradiation
penetratesonlyslightlyandthereforecausesonlysuperficialnecrosisinthe
cornealepithelium.Theexposedareasofthecorneaandconjunctivainthe
palpebralfissurebecomeedematous,disintegrate,andarefinallycastoff.
Ultravioletkeratoconjunctivitisisoneofthemostcommonocularin-
juries.
Symptomsanddiagnosticconsiderations:Symptomstypicallymanifest
themselvesafteralatencyperiodofsixtoeighthours.Thiscausespatientsto
seektheaidofanophthalmologistoreyeclinicinthemiddleofthenight,
complainingof“acuteblindness”accompaniedbypain,photophobia,epi-
phora,andanintolerableforeign-bodysensation.Oftensevereblepharo-
spasmwillbepresent.Slit-lampexaminationwillrequireadministrationofa
topicalanesthetic.Thisexaminationwillrevealepithelialedemaandsuperfi-
cialpunctatekeratitisorerosioninthepalpebralfissureunderfluorescein
dye(seeFig.18.5).
Thetopicalanestheticwillcompletelyrelievesymptomswithinafew
secondsandallowthepatienttoseeclearlyandopenhisorhereyes
withoutpain.Undernocircumstancesmaythepatientbeallowed
accesstothisanestheticwithoutmedicalsupervision.Uncontrolled
habitualusesuppressesthepainreflex(eyeclosingreflex),whichcould
resultinincalculablecornealdamage.
Treatment:The“blinded”patientshouldbeinstructedthatthesymptoms
willresolvecompletelyundertreatmentwithantibioticointmentwithin24
to48hours.Ointmentisbestbeappliedtobotheyeseverytwoorthreehours
withthepatientatrestindarkenedroom.Thepatientshouldbeinformed
thattheeyeointmentwillnotimmediatelyrelievepainandthateyemove-
mentsshouldbeavoided.
18.5.2Burns
Etiology:Flaringflamessuchasfromacigarettelighter,hotvapors,boiling
water,andsplattersofhotgreaseorhotmetalcausethermalcoagulationof
thecornealandconjunctivalsurface.Becauseoftheeyeclosingreflex,the
eyelidsoftenwillbeaffectedaswell.
18.5InjuriesDuetoPhysicalAgents
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542. 524
Injuriesduetoexplosionorburnsfromastarter’sgunalsoincludeparti-
clesofburnedpowder(powderburns).Injuriesfromagaspistolwillalso
involveachemicalinjury.
Symptomsanddiagnosticconsiderations:Symptomsaresimilartothose
ofchemicalinjuries(epiphora,blepharospasm,andpain).
Atopicalanestheticisadministered,andtheeyeisexaminedasina
chemicalinjury.Immediateopacificationofthecorneawillbereadily
apparent.Thisisduetoscalingoftheepitheliumandtissuenecrosis,whose
depthwillvarywiththeseverityoftheburn.Inburnsfrommetalsplinters,
onewilloftenfindcooledmetalparticlesembeddedinthecornea.
Treatment:Initialtreatmentconsistsofapplyingcoolingantisepticband-
agestorelievepain,afterwhichnecroticareasoftheskin,conjunctiva,and
corneaareremovedunderlocalanesthesia.Foreignparticlessuchas
embeddedashandsmokeparticlesintheeyelidsandfaceareremovedin
cooperationwithadermatologistbybrushingthemoutwithasterile
toothbrushundergeneralanesthesia.Thisisdonetopreventthemfrom
growingintotheskinlikeatattoo.Superficialparticlesinthecorneaand
conjunctivaareremovedunderlocalanesthesiatogetherwiththenecrotic
tissue.Theaffectedareasarethentreatedwithanantibioticointment.
Prognosis:Theclinicalcourseofaburnisusuallylessseverethanthatofa
chemicalinjury.Thisisbecauseburns,likeacidinjuries,causesuperficial
coagulation.Usuallytheyhealwellwhentreatedwithantibioticointment.
18.5.3RadiationInjuries(IonizingRadiation)
Etiology:Ionizingradiation(neutron,orgamma/x-rayradiation)havehigh
energythatcancauseionizationandformationofradicalsincellulartissue.
Penetrationdepthintheeyevarieswiththetypeofradiation,i.e.,the
wavelength,resultingincharacteristictypesoftissuedamage(Fig.18.13).
Thistissuedamagealwaysmanifestsitselfafteralatencyperiod,oftenonly
afteraperiodofyears(seealsoSymptomsandclinicalpicture).Commonsites
includethelens(radiationcataract)andretina(radiationretinopathy).This
tissuedamageisusuallytheresultoftumorirradiationintheeyeor
nasopharynx.Radiationdisordershavebeenobservedinpatientsfrom
HiroshimaandNagasakiand,morerecently,inChernobyl.
Symptomsandclinicalpicture:Lossoftheeyelashesandeyelidpigmenta-
tionaccompaniedbyblepharitisaretypicalsymptoms.Adryeyeisasignof
damagetotheconjunctivalepithelium(lossofthegobletcells).Lossofvisual
acuityduetoaradiationcataractisusuallyobservedwithinoneortwoyears
ofirradiation.Radiationretinopathyintheformofischemicretinopathywith
bleeding,cotton-woolspots,vascularocclusion,andretinalneovasculariza-
tionusuallyoccurswithinmonthsofirradiation.
18OcularTrauma
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543. 525
Possibleradiationdamagetotheeye.
Ultraviolet
keratoconjunctivitis
Radiation
cataract
Radiation
retinopathy
Ultraviolet radiation
Infrared radiation
Gamma radiation
Visible light
Fig.18.13The
penetrationdepth
ofradiationinthe
eyevariesaccord-
ingtowavelength.
Therefore,each
radiationinjury
causescharacteris-
tictissuedamage.
Treatmentandprophylaxis:Careshouldbetakentocovertheeyespriorto
plannedradiationtherapyintheheadandneck.Radiationcataractmaybe
treatedsurgically.Radiationretinopathymaybetreatedwithpanretinalpho-
tocoagulationwithanargonlaser.
18.6IndirectOcularTrauma:Purtscher’sRetinopathy
Etiology:Arterialandvenouscirculatorydisruptionintheretinacharacter-
izedbyasuddenincreaseinintravascularpressuremayoccurfollowing
severechestinjuries(compressiontraumasuchasinaseat-beltinjury)or
fracturesoflongbones(presumablyduetofatembolismsorvascular
spasms).
Symptomsanddiagnosticconsiderations:Acuteretinalischemiawith
impairedvisionandlossofvisualacuitywilloccureitherimmediatelyor
withinthreetofourdaysoftheinjury.Examinationofthefunduswillreveal
cotton-woolspotsandintraretinalbleedingindicativeoffocalretinal
ischemia.Linesofbleedingwillalsobeobserved.
Treatment:Fundussymptomswillusuallydisappearspontaneouslywithin
fourtosixweeks.Reducedvisualacuityandvisualfielddefectsmayocca-
sionallypersist.Occasionallytreatmentwithhighdosesofsystemicsteroids
andprostaglandininhibitorsisattempted.
18.6IndirectOcularTrauma:Purtscher’sRetinopathy
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544. Lang, Ophthalmology © 2000 Thieme
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545. 527
19CardinalSymptoms
GerhardK.Lang
Thislistofcardinalsymptomsisincludedtoprovidethemedicalstudent,
intern,orophthalmologyresidentwithaconciseoverviewoftherangeof
possibleunderlyingclinicalsyndromes.Thiscompilationofcardinalsymp-
tomscannotanddoesnotrepresentacompleteandcomprehensivelisting.
Nonetheless,itwillalsobehelpfulinrecallingthemostimportantclinical
picturesinophthalmologyandprovidingareviewofthematerial.
Lang, Ophthalmology © 2000 Thieme
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546. 528
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
B
u
r
n
in
g
sensation Common
causes:
!
R
eddening
of
the
e
y
elids.
!
A
dhesion
of
the
e
y
elashes.
!
Scales
on
the
e
y
elids
and
bases
of
the
e
y
elashes.
!
Itc
h
y
e
y
elid
margins.
!
Common
in
fair
-haired
patients.
Blepharitis.
Ex
clude
refractiv
e
anomaly
a
s
possible
cause.
!
Sensation
of
pressure,
dr
yness,
and
sand
in
the
e
y
es.
!
Occasionally
e
x
cessiv
ely
t
earing
in
response
t
o
dr
y
e
y
es.
!
Dr
yness
of
o
t
her
mucous
mem-
b
r
a
n
e
s
.
Dr
y
e
y
e
s
(
k
e
rat
oconjunctivitis
sicca).
Ev
aluat
e
t
ear
secre
tion
with
Sc
hirmer
t
ear
t
e
sting
and
t
ear
break
-up
time
(TBUT).
!
R
eddened
conjunctiv
a.
!
Pur
ulent,
mucoid,
or
w
a
t
e
r
y
dis-
c
h
a
r
g
e
.
!
S
tic
ky
e
y
elids
in
the
morning.
Conjunctivitis.
Obt
ain
smear
f
o
r
microbiological
e
x
amination.
R
a
re
causes:
!
Usually
segment
al,
livid
redden-
ing
of
the
conjunctiv
a.
!
Nodular
mobile
sw
elling
that
is
t
ender
t
o
palpation.
Episcleritis.
Unequiv
ocal
diagnosis.
!
Circumscribed
reddening
at
the
pinguecula.
!
Thic
k
ened
conjunctiv
al
v
essels.
Irrit
a
t
e
d
pinguecula.
Unequiv
ocal
diagnosis.
19CardinalSymptoms
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547. 529
Circumscribed
reddening
at
the
p
t
er
y
gium.
Irrit
a
t
e
d
p
t
e
r
y
gium.
Unequiv
ocal
diagnosis.
R
eddening
in
the
upper
circumf
er-
ence
near
the
limbus.
K
e
ratitis
of
the
upper
limbus.
Unequiv
ocal
diagnosis.
T
e
a
r
in
g
(epiphora) In
c
hildren:
!
Buphthalmos.
!
Increased
glare
and
squinting.
!
Unilat
eral
or
bilat
eral.
!
Corneal
opacification.
Congenit
al
glaucoma.
Risk
of
blindness.
!
Measure
intraocular
pressure
immediat
ely
.
!
R
e
d
e
y
e
.
!
Se
v
e
re
f
o
reign-body
sensation.
!
P
ain
causing
blepharospasm.
!
Pho
t
ophobia.
!
Ey
elid
sw
elling.
!
Decreased
visual
acuity
.
!
Subt
ar
sal
or
corneal
f
o
reign
body
.
!
Corneal
erosion.
!
F
ull
e
v
er
sion
of
the
e
y
elids
t
o
localize
subt
ar
sal
f
o
reign
body
.
!
Apply
f
luorescein
dy
e
t
o
e
v
al-
u
a
t
e
c
o
r
n
e
a
w
h
e
r
e
c
o
r
n
e
a
l
e
r
o
-
sion
is
suspect
ed.
!
No
pain.
!
N
e
a
r
ly
c
o
n
s
t
a
n
t
p
u
r
u
le
n
t
w
a
t
e
r
y
d
is
c
h
a
r
g
e
.
!
S
tic
ky
e
y
elids
in
the
morning.
!
No
itc
hing
or
reddening
of
the
e
y
e
and
no
visible
e
y
elid
def
ormity
.
Dacr
y
o
st
enosis
(v
alv
e
of
Hasner).
Irrig
a
t
e
lacrimal
syst
em
t
o
locat
e
the
s
t
enosis.
In
adults
(painless
o
r
n
e
a
r
ly
p
a
in
le
s
s
)
:
!
R
eddening
of
the
conjunctiv
a.
!
Only
minimal
symp
t
oms.
!
Ectropion
de
v
elops
from
con-
st
antly
wiping
a
w
a
y
t
ear
s.
!
Epidermization
of
the
e
xposed
conjunctiv
a.
Ectropion.
Unequiv
ocal
diagnosis.
19CardinalSymptoms
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548. 530
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
T
e
a
r
in
g
In
adults
(painless
o
r
n
e
a
r
ly
p
a
in
le
s
s
)
:
!
Sandy
,
d
r
y
f
eeling.
!
Ey
e
o
f
t
en
free
of
irrit
ation.
!
Oral,
nasal,
and
genit
al
mucous
membranes
of
t
e
n
a
re
also
dr
y
.
!
Dr
y
e
y
es.
!
Dr
y
e
y
e
s
in
k
erat
oconjunctivitis
sicca.
Ev
aluat
e
t
ear
secre
tion
with
Sc
hirmer
t
ear
t
e
sting
and
t
ear
break
-up
time
(TBUT).
!
Slight
pain.
!
Pur
ulent
disc
harge
of
thic
k
ened
t
ear
fluid
and
pus
is
common
(e
xpressed
from
the
punctum
b
y
pressing
on
the
lacrimal
sac).
!
R
ecurrent
dacr
y
ocystitis.
Obstr
uct
ed
drainage
t
hrough
the
lo
w
e
r
lacrimal
syst
em,
possibly
with
inflammation.
Irrig
a
t
e
the
lo
w
er
lacrimal
syst
em
t
o
localize
the
s
t
enosis.
!
Clear
t
ear
fluid.
!
Punctum
is
co
v
e
red
b
y
connec-
tiv
e
tissue
or
projects
from
the
e
y
e
.
Obstr
uction
or
e
v
er
sion
of
the
punctum
lacrimale.
Unequiv
ocal
diagnosis.
P
ainful:
!
Se
v
e
re
f
o
reign-body
sensation.
!
Ey
elid
sw
elling.
!
Blepharospasm
and
pho
t
opho-
bia.
!
R
eddened
e
y
e.
!
Decreased
visual
acuity
.
!
Corneal
erosion.
!
Subt
ar
sal
corneal
f
o
reign
body
.
!
Ev
er
t
e
y
elid
t
o
localize
subt
ar
sal
corneal
f
o
reign
body
.
!
Apply
f
luorescein
dy
e
t
o
e
v
al-
u
a
t
e
c
o
r
n
e
a
w
h
e
r
e
c
o
r
n
e
a
l
e
r
o
-
sion
is
suspect
ed.
!
F
o
reign-body
sensation
(e
y
e
-
lashes
scratc
h
cornea).
!
In
w
a
rd
def
ormity
of
the
e
y
e
-
lashes,
e
y
elid
turned
in
w
a
rd.
T
r
ic
hiasis,
entropion.
Unequiv
ocal
diagnosis.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

549. 531
Increased
glare
!
Gra
y
t
o
whit
e
pupillar
y
r
efle
x.
!
Gradual
progressiv
e
loss
of
visual
acuity
.
Cat
aract.
Slit-lamp
e
xamination.
Diagnosis
is
unequiv
ocal
where
t
he
opacity
is
visible
under
re
troillumination.
!
W
ide
pupil
(m
ydriasis).
!
Little
or
no
pupillar
y
r
esponse
t
o
light.
!
Pupil
width
is
dif
f
e
rent
from
f
el-
lo
w
e
y
e
.
T
r
aumatic
or
dr
ug-induced
paraly-
sis
of
the
sphinct
er
pupillae.
Slit-lamp
e
xamination.
Iris
and
pupillar
y
r
esponse
ma
y
b
e
e
v
al-
uat
ed
under
re
troillumination.
In
c
hildren:
!
Enlargement
of
the
cornea
and
unilat
eral
or
bilat
eral
opacifica-
tion.
!
Increased
glare
with
s
quinting.
Buphthalmos.
Risk
of
blindness.
!
Measure
intraocular
pressure
immediat
ely
.
!
Pigment
ation
deficiency
in
iris.
!
Skin
and
hair
pigment
ation
defi-
ciency
.
Albinism.
Unequiv
ocal
diagnosis.
!
Hist
o
r
y
of
trauma.
!
Pupil
is
no
t
r
ound.
!
Comple
t
e
or
par
tial
aniridia.
Iris
def
ects
(a
vulsion
of
the
r
oo
t
o
f
the
iris
o
r
aniridia).
Unequiv
ocal
diagnosis.
Diplopia Binocular
!
No
e
y
e
pain.
!
Neurologic
symp
t
oms
depend-
ing
on
cause.
!
P
ossible
hist
o
r
y
of
trauma.
Cranial
ner
v
e
palsy
(in
central
isc
hemia
or
apople
xy
,
intracranial
tumor
s,
or
cerebral
trauma).
Neurologic
and
neuroradiologic
diagnostic
studies
are
indicat
ed.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

550. 532
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Diplopia Binocular
!
Hist
o
r
y
of
trauma
(alw
a
y
s
p
res-
ent
with
ocular
contusion;
when
the
e
y
elid
is
sw
ollen
shut,
diplopia
will
no
t
b
e
apparent
t
o
the
patient).
!
Limit
ed
ocular
mo
tility
in
ele
v
a
-
tion
and
depression.
!
Enophthalmos
(post
e
riorly
dis-
placed
e
y
e).
F
r
acture
o
f
t
he
floor
of
the
orbit.
!
O
b
t
a
in
r
a
d
iog
r
a
p
h
s
.
!
In
dif
ficult
cases,
CT
is
indicat
ed
f
o
r
p
recise
localization
of
the
fracture.
!
P
ain
during
e
y
e
m
o
tion.
!
R
eddening
and
sw
elling
of
the
e
y
elid
and
conjunctiv
a.
Ocular
m
y
ositis.
Ultrasound
scan
of
the
muscles.
!
Se
v
e
re
sw
elling
of
the
e
y
elid
and
conjunctiv
a.
!
Se
v
e
re
malaise.
!
Af
f
ect
ed
e
y
e
is
o
f
t
en
immobile
(“cement
ed”
globe).
!
Ex
ophthalmos
(in
c
hildren,
this
is
a
sign
of
orbit
al
cellulitis).
Orbit
al
cellulitis.
Risk
of
blindness
(op
tic
ner
v
e
atroph
y).
Ca
v
e
rnous
sinus
thrombosis
is
a
lif
e-threat
ening
sequela.
Consult
ENT
specialist:
Orbit
al
cel-
lulitis
originat
es
in
the
paranasal
sinuses
in
60
%
o
f
all
cases,
and
in
8
4
%
o
f
all
cases
in
c
hildren.
!
Associat
ed
Hyper
th
yreosis
(in
60
%
o
f
all
cases)
and
k
e
rat
ocon-
junctivitis
sicca.
Gra
v
es
’
disease.
!
Ultrasound
and/or
CT
is
indi-
cat
ed
t
o
de
t
e
rmine
whe
ther
muscles
are
t
hic
k
ened.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

551. 533
!
Unilat
eral
or
bilat
eral
e
x
ophthal-
mos
ma
y
b
e
p
resent.
!
Charact
eristic
e
y
elid
signs
(see
T
able
1
5
.
3
).
!
Th
yroid
diagnostic
studies
b
y
endocrinologist
a
re
indicat
ed.
!
P
atient
suddenly
e
xperiences
diplopia
vision
(of
t
e
n
a
t
t
he
age
of
tw
o
t
o
six).
!
P
atient
closes
one
e
y
e
t
o
suppress
diplopia.
Lat
e
s
trabismus
with
normal
sensor
y
d
e
v
elopment.
Unequiv
ocal
diagnosis.
!
Scarring
limits
ocular
mo
tility
.
!
Diplopia
in
t
e
mporal
g
aze.
!
Pt
er
y
gium
clearly
visible
with
the
unaided
e
y
e.
Pt
er
y
gium.
Unequiv
ocal
diagnosis.
Monocular
!
Gra
y
t
o
whit
e
pupillar
y
r
efle
x.
!
Gradual
loss
of
visual
acuity
.
!
Increased
glare.
Cat
aract
(multiple
f
ocal
points
in
a
single
lens).
Slit-lamp
e
xamination.
Diagnosis
is
unequiv
ocal
where
t
he
opacity
is
visible
under
re
troillumination.
Alt
ernating
diplopia
(dislocat
ed
lens
c
hanges
its
position
in
the
e
y
e
and
ma
y
f
all
bac
k
int
o
place
in
the
plane
of
the
pupil
when
the
patient
bends
f
o
r
w
ard).
Dislocation
or
subluxation
of
the
lens.
Unequiv
ocal
diagnosis.
Equat
or
of
the
lens
is
visible
in
the
plane
of
the
pupil
under
re
troillumination.
!
Hist
o
r
y
of
trauma
(a
vulsion
of
the
r
oo
t
o
f
t
he
iris).
!
Congenit
al
or
traumatic
aniridia.
“Double”
pupil
due
t
o
an
iris
def
ect
(a
vulsion
of
the
r
oo
t
o
f
t
he
iris
or
aniridia).
Unequiv
ocal
diagnosis.
Conical
or
hemispherical
pro
t
r
u
-
sion
def
ormation
of
the
cornea.
K
e
r
a
t
oc
on
u
s
or
k
e
r
a
t
o
g
lob
u
s
.
Diplopia
results
from
multiple
f
ocal
points
of
the
def
ormed
cor-
nea.
Unequiv
ocal
diagnosis.
Condition
ma
y
b
e
visible
with
t
he
unaided
e
y
e
o
r
v
erified
b
y
st
andard
k
erat
os-
cop
y
o
r
videok
erat
oscop
y
.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

552. 534
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Enophthalmos (e
y
e
recedes
int
o
orbit)
!
Hist
o
r
y
of
trauma
(signs
of
ocu-
lar
contusion).
!
Diplopia.
!
Ey
elid
sw
elling.
!
Limit
ed
ocular
mo
tility
in
ele
v
a
-
tion
and
depression.
F
r
acture
o
f
t
he
floor
of
the
orbit.
!
O
b
t
a
in
r
a
d
iog
r
a
p
h
s
.
!
In
dif
ficult
cases,
CT
is
indicat
ed
f
o
r
p
recise
localization
of
the
fracture.
T
r
iad
of
p
t
osis,
miosis,
enophthal-
mos
(unilat
eral
findings).
Horner
’
s
syndrome.
Neurologic
e
x
amination.
!
Blind
e
y
e.
!
Phthisis
(shrink
age
o
f
t
he
e
y
e-
ball).
!
Pseudoenophthalmos
(se
v
e
re
trauma,
surger
y
,
o
r
r
e
tinal
de
t
a
c
hment)
and
c
h
ronic
in-
flammation
(uv
eitis
or
re
tinitis).
Ocular
atroph
y
with
shrink
age
o
f
the
g
lobe.
Unequiv
ocal
diagnosis.
!
Loss
of
orbit
al
fatty
tissue
in
adv
anced
age
(
e
y
es
recede
int
o
the
orbit).
!
Alw
a
y
s
bilat
eral
Senile
sunk
en
e
y
e.
Unequiv
ocal
diagnosis.
Ex
ophthalmos
(projecting
e
y
e)
!
Associat
ed
h
yper
th
yreosis
(in
60
%
o
f
all
cases).
!
Of
t
e
n
in
association
with
diplopia.
!
Of
t
e
n
in
association
with
k
erat
o-
conjunctivitis
sicca.
Gra
v
es
’
disease.
!
Ultrasound
and/or
CT
is
indi-
cat
ed
t
o
de
t
e
rmine
whe
ther
muscles
are
t
hic
k
ened.
!
Th
yroid
diagnostic
studies
b
y
endocrinologist
a
re
indicat
ed.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

553. 535
!
Me
t
amorphopsia.
!
R
e
tinal
impression
f
olds
are
vis-
ible
under
ophthalmoscop
y
.
R
e
trobulbar
tumor
(e
x
ophthalmos
due
t
o
post
e
rior
pressure
o
n
t
he
g
lob
e
)
.
CT
scan.
!
Hist
o
r
y
of
trauma.
!
Ey
elid
hemat
oma
(blac
k
e
y
e).
!
Ey
elid
sw
elling.
Orbit
al
bleeding.
R
adiographs
t
o
e
x
clude
injur
y
o
f
the
bon
y
s
tr
uctures
of
the
orbit.
!
Pseudoe
x
ophthalmos
due
t
o
long
globe.
!
Occasionally
unilat
eral.
!
Dif
f
e
rence
in
refraction
(ani-
some
tropia).
!
P
oor
dist
ance
vision;
good
near
vision.
Se
v
e
re
m
y
opia.
R
efraction
t
e
sting.
!
P
ain
during
e
y
e
m
o
tion.
!
Diplopia.
!
R
eddening
and
sw
elling
of
the
e
y
elid
and
conjunctiv
a.
Ocular
m
y
ositis.
Ultrasound
scan
of
the
muscles.
!
P
atients
are
o
f
t
en
c
hildren.
!
Se
v
e
re
sw
elling
of
the
e
y
elid
and
conjunctiv
a.
!
Se
v
e
re
malaise.
!
Af
f
ect
ed
e
y
e
is
o
f
t
en
immobile
(“cement
ed”
globe).
Orbit
al
cellulitis.
Risk
of
blindness
(op
tic
ner
v
e
atroph
y).
Ca
v
e
rnous
sinus
thrombosis
is
a
lif
e-threat
ening
sequela.
Consult
ENT
specialist:
Orbit
al
cel-
lulitis
originat
es
in
the
paranasal
sinuses
in
60
%
o
f
all
cases,
and
in
8
4
%
o
f
all
cases
in
c
hildren.
Other
de
v
elopment
al
anomalies
ma
y
accompan
y
e
x
ophthalmos,
whic
h
in
t
hese
cases
is
usually
bilat
eral.
Craniosynost
osis.
Unequiv
ocal
diagnosis.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

554. 536
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Hypop
y
o
n
!
Deep
e
y
e
pain
that
hardly
responds
t
o
analgesics
at
all.
!
R
eddening
and
sw
elling
of
the
e
y
elids
and
conjunctiv
a.
!
A
cut
ely
decreased
visual
acuity
.
!
Prior
intraocular
surger
y
,
pene-
trating
injur
y
,
or
corneal
ulcera-
tion.
A
cut
e
endophthalmitis.
Risk
of
blindness
within
hour
s.
!
Microbiological
diagnostic
stud-
ies.
!
R
eddening
of
the
conjunctiv
a.
!
Corneal
ulcer
.
!
Ey
elid
sw
elling.
!
P
ain.
Serpiginous
corneal
ulcer
.
R
apid
progression
of
the
ulcer
can
threat
en
the
e
y
e
.
!
Microbiological
diagnostic
stud-
ies.
!
No
ocular
pain.
!
Iritis
or
iridocy
clitis.
S
t
erile
h
ypop
y
on.
!
Diagnostic
studies
f
o
r
u
v
eitis.
!
Syst
emic,
immunologic,
and
rheumat
ologic
e
x
aminations
are
required.
Headac
he
!
Unilat
erally
r
ed,
hard
e
y
e
.
!
Pupil
fix
e
d
and
dilat
ed.
!
Corneal
opacification.
!
Se
v
e
re
pain.
!
F
r
equent
v
omiting.
Glaucoma
att
ac
k.
Risk
of
blindness.
!
Measure
intraocular
pressure
immediat
ely
.
!
Sudden
unilat
eral
loss
of
visual
acuity
.
!
P
atients
are
usually
o
v
e
r
60.
!
Headac
he
pain
in
t
e
mples.
!
AION:
ant
erior
isc
hemic
op
tic
neuropath
y
due
t
o
ar
thritis.
!
Giant
cell
ar
thritis
in
t
e
mporal
ar
t
e
ritis.
Risk
of
blindness.
!
Circular
or
segment
al
sw
elling
of
the
o
p
tic
disk
will
be
visible
upon
ophthalmoscop
y
.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

555. 537
!
T
e
mporal
ar
t
e
r
y
t
ender
t
o
palpa-
tion.
!
P
ain
when
c
h
ewing,
w
eight
loss.
!
P
oor
o
v
erall
health.
!
My
algia.
!
S
t
if
f
n
e
c
k
.
!
Ar
t
e
rial
biopsy
and
hist
ologic
e
x
amination
are
indicat
ed.
!
De
t
e
rmine
er
ythrocyt
e
sedi-
ment
ation
rat
e
and
le
v
e
l
o
f
C
-
reactiv
e
p
ro
t
ein
(precipit
ous
drops
occur
in
t
e
mporal
ar
t
e
ritis).
!
P
oor
vision.
!
Ey
eglasses
or
c
hange
o
f
e
y
e
glass
prescrip
tion
needed.
!
R
apid
fatigue
(f
or
e
x
ample
when
reading).
!
Burning
sensation.
Asthenopic
symp
t
oms.
T
e
st
visual
acuity
.
Flashes
of
light
!
Of
t
e
n
in
older
patients.
!
Flashes
of
light
and
shado
ws
seen
when
mo
ving
the
e
y
es,
e
v
e
n
in
t
h
e
d
a
r
k
.
!
Float
er
s.
P
o
st
erior
vitreous
de
t
a
c
hment.
!
Essentially
harmless
age-relat
ed
disorder
.
!
Examine
fundus
t
o
e
x
clude
re
ti-
nal
def
ect.
P
atient
sees
shado
ws
(a
“w
all”
from
belo
w
o
r
a
“cur
t
ain”
from
abo
v
e).
R
e
tinal
de
t
a
c
hment.
Risk
of
blindness.
!
Ophthalmoscop
y
.
Of
t
e
n
without
an
y
o
ther
symp-
t
oms.
R
e
tinal
t
ear
.
Risk
of
re
tinal
de
t
a
c
hment.
!
Ophthalmoscop
y
.
Of
t
e
n
encount
ered
in
patients
with
consump
tiv
e
s
yst
emic
disorder
s
suc
h
a
s
AIDS.
R
e
tinitis.
Consult
int
ernist
f
or
diagnosis
of
cause.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

556. 538
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Ey
elid
sw
elling
Inflammat
or
y:
!
Clear
v
esicles
on
the
e
y
elids.
!
Ey
elid
sw
elling.
!
Inflammat
or
y
p
t
osis.
Herpes
simple
x
vir
us
inf
ection.
Unequiv
ocal
diagnosis.
!
P
ainful
pressure
point
on
the
e
y
elid.
!
Circumscribed
sw
elling
and
red-
dening
of
the
e
y
elid.
!
Of
t
e
n
s
e
v
ere
pulsating
pain.
!
Spo
t
o
f
y
ello
w
pus.
!
Pseudop
t
osis.
Hordeolum.
Unequiv
ocal
diagnosis.
!
S
ting
is
of
t
e
n
visible.
!
Clear
sw
elling.
!
Unilat
eral.
!
Itc
hing.
Insect
sting.
Unequiv
ocal
diagnosis.
!
R
e
d
e
y
e
.
!
O
f
t
e
n
f
e
w
s
y
m
p
t
o
m
s
.
!
S
tic
ky
e
y
elids
in
the
morning.
!
Pur
ulent
or
w
a
t
e
r
y
disc
harge.
Conjunctivitis.
Microbiological
diagnostic
studies.
!
Large,
hard
s
w
elling
and
redden-
ing
with
edema
are
o
f
t
en
pres-
ent.
!
P
ain.
!
Pt
osis.
Ey
elid
abscess.
Unequiv
ocal
diagnosis.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

557. 539
!
Se
v
e
re
pain.
!
Bleeding
v
esicles.
!
P
att
ern
o
f
lesions
f
ollo
ws
trigeminal
ner
v
e
.
Herpes
zost
e
r
ophthalmicus.
R
e
f
e
r
patient
t
o
dermat
ologist.
Noninflammat
or
y
!
P
ainless,
circumscribed
sw
elling
of
the
e
y
elid.
!
No
reddening.
!
Hard
palpable
nodules
on
the
e
y
elid.
!
Pseudop
t
osis.
Chalazion.
Unequiv
ocal
diagnosis.
!
Occur
s
in
older
patients
(elderly
skin).
!
Limp,
drooping
e
y
elid.
!
Drooping
e
y
ebro
w
s.
!
Cutis
laxa
senilis.
!
Blepharoc
halasis.
Unequiv
ocal
diagnosis.
!
S-shaped
upper
e
y
elid.
!
No
reddening.
!
P
alpable
mass.
!
Ey
elid
tumor
.
!
Lacrimal
gland
tumor
.
Biopsy
.
No
o
t
her
ocular
symp
t
oms.
Syst
emic
cause
(hear
t,
kidne
y
,
or
th
yroid
disorder).
R
e
f
e
r
patient
t
o
int
ernist.
Y
ello
wish
mobile
prolapsed
fat
under
the
e
y
elids.
Orbit
al
fat
hernia.
Unequiv
ocal
diagnosis.
!
Enophthalmos.
!
Hist
o
r
y
of
trauma
(ocular
con-
tusion).
!
Diplopia
ma
y
b
e
p
resent.
F
r
acture
o
f
t
he
floor
of
the
orbit.
!
O
b
t
a
in
r
a
d
iog
r
a
p
h
s
.
!
In
dif
ficult
cases,
CT
is
indicat
ed
f
o
r
p
recise
localization
of
the
fracture.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
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558. 540
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Pseudop
t
osis
!
In
older
patients.
!
Limp
e
y
elid
skin.
!
Drooping
e
y
elids.
!
Cutis
laxa
senilis.
!
Blepharoc
halasis.
Unequiv
ocal
diagnosis.
!
Hist
o
r
y
of
trauma
(signs
of
ocu-
lar
contusion).
!
Diplopia
ma
y
b
e
p
resent.
!
Ey
elid
sw
elling.
!
Enophthalmos.
F
r
acture
o
f
t
he
floor
of
the
orbit.
!
O
b
t
a
in
r
a
d
iog
r
a
p
h
s
.
!
In
dif
ficult
cases,
CT
is
indicat
ed
f
o
r
p
recise
localization
of
the
fracture.
!
Pseudoenophthalmos.
!
Of
t
e
n
secondar
y
t
o
s
e
v
ere
trauma,
surger
y
,
o
r
c
hronic
inflammation
(uv
eitis
or
re
tinitis).
!
Blind
e
y
e.
Phthisis
(shrink
age
o
f
t
he
e
y
eball).
Unequiv
ocal
diagnosis.
P
alpable,
immobile
sw
elling.
Ey
elid
tumor
s.
Biopsy
.
Pt
osis
Common:
Hist
o
r
y
of
trauma
or
older
patient.
T
ear
in
the
le
v
at
or
palpebrae.
Unequiv
ocal
diagnosis.
Secondar
y
t
o
intraocular
surger
y
.
Elong
ation
of
the
le
v
at
or
palpe-
b
r
a
e
.
Unequiv
ocal
diagnosis.
Usually
bilat
eral;
present
at
bir
th.
Congenit
al
p
t
osis.
Unequiv
ocal
diagnosis.
R
a
re:
P
aralysis
of
one
of
all
e
xtraocular
muscles.
Chronic
progressiv
e
e
xt
ernal
oph-
thalmoplegia.
R
e
f
e
r
patient
t
o
neurologist.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

559. 541
!
Ey
elid
sw
elling.
!
P
ain.
!
F
o
reign-body
sensation.
!
Blepharospasm.
!
Corneal
erosion.
!
Corneal
f
o
reign
body
.
!
Subt
ar
sal
corneal
f
o
reign
body
.
!
Examine
cornea.
!
F
ully
e
v
e
r
t
the
e
y
elids
where
subt
ar
sal
f
o
reign
body
is
sus-
pect
ed.
!
Apply
f
luorescein
dy
e
t
o
e
v
al-
u
a
t
e
c
o
r
n
e
a
w
h
e
r
e
c
o
r
n
e
a
l
e
r
o
-
sion
is
suspect
ed.
Secondar
y
t
o
application
of
anti-
glaucoma
medications
cont
aining
guane
thidine.
F
o
r
d
r
u
g
side
ef
f
ects.
Unequiv
ocal
diagnosis.
T
r
iad
of
p
t
osis,
miosis,
and
enoph-
thalmos.
Horner
’
s
syndrome.
R
e
f
e
r
patient
t
o
neurologist.
Se
v
e
rity
of
p
t
osis
can
v
a
r
y
from
da
y
t
o
d
a
y
.
My
asthenia
gra
vis.
R
e
f
e
r
patient
t
o
neurologist.
A
ccompanied
b
y
dilat
ed
pupil
and
diplopia.
Oculomo
t
o
r
ner
v
e
palsy
.
R
ef
er
patient
t
o
neurologist.
Pupillar
y
dysfunction Miosis:
Secondar
y
t
o
application
of
pilo-
carpine.
Dr
ug-induced
miosis.
Unequiv
ocal
diagnosis.
Secondar
y
t
o
use
of
morphine.
T
o
xic
miosis.
Unequiv
ocal
diagnosis.
A
ccompanied
b
y
p
t
osis
and
enoph-
thalmos.
Horner
’
s
syndrome.
R
e
f
e
r
patient
t
o
neurologist.
!
A
ccompanied
b
y
iritis
or
irido-
cy
clitis.
!
R
e
d
e
y
e
.
!
P
ain.
R
eactiv
e
miosis.
Unequiv
ocal
diagnosis.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
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560. 542
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Pupillar
y
dysfunction Mydriasis:
Secondar
y
t
o
administration
of
atropine
or
m
ydriatics.
Dr
ug-induced
m
ydriasis.
Unequiv
ocal
diagnosis.
!
Isc
hemic.
!
T
umor
.
!
Hist
o
r
y
of
trauma.
Lesion
of
the
o
p
tic
ner
v
e
or
op
tic
t
r
a
c
t
.
R
e
f
e
r
patient
t
o
neurologist.
Pupil
does
no
t
r
espond
t
o
light.
F
ollo
wing
sudden
blindness.
Unequiv
ocal
diagnosis.
Rings
around
light
sources
!
Gradual
progressiv
e
loss
of
visual
acuity
.
!
Increased
glare.
!
Gra
yish
whit
e
pupillar
y
r
efle
x.
Cat
aract.
Slit-lamp
e
xamination.
Diagnosis
is
unequiv
ocal
where
t
he
opacity
is
visible
under
re
troillumination.
Corneal
edema.
Increased
intraocular
pressure.
Measure
intraocular
pressure.
R
e
d
e
y
e
!
Conjunctiv
al
injection.
!
F
ull
visual
acuity
.
!
Pur
ulent
or
w
a
t
e
r
y
disc
harge.
!
Sw
elling
of
the
e
y
elid
and
con-
junctiv
a.
!
S
tic
ky
e
y
elids
in
the
morning.
Conjunctivitis.
Obt
ain
smear
f
o
r
microbiological
e
x
amination.
!
Combined
injection.
!
R
educed
visual
acuity
.
!
Intraocular
str
uctures
obscured.
!
P
ain.
Scleritis
and/or
episcleritis.
Unequiv
ocal
diagnosis.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

561. 543
!
Ey
e
hard
t
o
palpation.
!
Pupil
fix
e
d
and
dilat
ed.
!
Head
and
e
y
e
pain.
!
Loss
of
visual
acuity
.
!
N
ausea,
possibly
with
v
omiting.
Glaucoma
att
ac
k.
Risk
of
blindness.
!
Measure
intraocular
pressure
immediat
ely
.
!
Spont
aneous
(normal
hist
o
r
y).
!
Secondar
y
t
o
e
x
ercise
(suc
h
a
s
lif
ting
hea
vy
objects,
pressing,
def
ecation
of
hard
s
t
ool)
and
coughing
or
sneezing.
!
Secondar
y
t
o
t
rauma
or
surger
y
.
!
Due
t
o
ar
t
e
riosclerosis
(ma
y
b
e
recurrent
in
older
patients).
!
W
ith
impaired
coagulation
(hemophilia
or
medication
suc
h
as
coumarin
deriv
at
es).
Subconjunctiv
al
hemorrhage.
Diagnosis
is
unequiv
ocal
where
confirmed
b
y
patient’
s
hist
o
r
y
.
Blac
k
spo
ts
bef
ore
t
he
e
y
es
(float
er
s)
!
Usually
n
o
o
ther
ocular
symp-
t
oms.
!
Decreased
visual
acuity
only
in
se
v
e
re
cases.
V
itreous
opacification.
Unequiv
ocal
diagnosis.
!
P
atients
are
o
f
t
en
older
.
!
P
atient
perceiv
es
v
eils
and
cur-
t
ains
in
the
e
y
e
,
e
v
e
n
in
t
he
d
a
r
k
.
!
Float
er
s
m
o
v
e
with
t
he
e
y
e.
!
Flashes
of
light.
P
o
st
erior
vitreous
de
t
a
c
hment.
!
Isolat
ed
findings
are
harmless.
!
Examine
fundus
t
o
e
x
clude
re
ti-
nal
def
ects.
Inflammat
or
y
debris
in
the
vitreous
body
.
P
o
st
erior
uv
eitis.
Examine
fundus.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

562. 544
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Decreased
visual
acuity
F
undus
e
x
ami-
nation
is
indi-
cat
ed
where
visual
acuity
is
decreased.
T
r
ansient
(visual
acuity
impro
v
es
with
2
4
hour
s,
usu-
ally
within
one
hour)
!
L
a
s
t
s
a
f
e
w
s
e
c
on
d
s
.
!
Dark
ening
that
ma
y
include
amaurosis.
Amaurosis
fug
ax
(suc
h
a
s
in
ipsi-
lat
eral
st
enosis
in
the
int
ernal
caro
tid
ar
t
e
r
y).
No
abnormal
ocular
findings.
!
P
oor
general
health.
!
V
isual
acuity
impro
v
es
with
impro
v
ement
in
general
health.
Circulat
or
y
f
ailure.
No
abnormal
ocular
findings.
!
V
isual
field
def
ects.
!
Scintillating
sco
t
oma
f
o
r
1
0
–
2
0
minut
es.
!
V
e
r
tigo
and
v
omiting.
Ocular
migraine.
Unequiv
ocal
diagnosis.
T
r
ansient
!
Blurred
vision.
!
General
f
eeling
of
fatigue.
Hypogly
cemia.
Risk
of
blindness.
!
A
dminist
e
r
g
lucose.
Diagnosis
is
unequiv
ocal
where
visual
acuity
re
turns
t
o
normal
as
the
le
v
el
of
blood
glucose
increases.
P
e
r
sisting
longer
than
2
4
hour
s,
sud-
den
onse
t,
painless.
!
Unilat
eral
loss
of
visual
acuity
.
!
Headac
he
is
possible.
!
AION:
ant
erior
isc
hemic
op
tic
neuropath
y
.
!
De
t
e
rmine
er
ythrocyt
e
sedi-
ment
ation
rat
e
and
le
v
e
l
o
f
C
-
reactiv
e
p
ro
t
ein
(precipit
ous
drops
occur
in
t
e
mporal
ar
t
e
ritis).
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

563. 545
!
Mobile
shado
ws
bef
ore
t
he
e
y
es.
!
Allo
w
t
o
clear
b
y
immobilizing
the
e
y
e
s
s
o
t
hat
blood
will
se
ttle.
V
itreous
hemorrhage.
Examine
fundus.
Diagnosis
is
unequiv
ocal
where
fundus
is
obscured.
!
Se
v
e
re
loss
of
visual
acuity
.
!
Flashes
of
light.
R
e
tinal
de
t
a
c
hment.
Risk
of
blindness.
!
Ophthalmoscop
y
(clearly
visible
re
tinal
de
t
a
c
hment).
!
P
ain
from
post
e
rior
sw
elling
and
with
e
y
e
mo
tion.
!
Increasing
loss
of
visual
acuity
f
ollo
wing
e
x
ercise.
!
Central
sco
t
oma.
!
Normal
findings
upon
ophthal-
moscop
y
(patient
sees
no
thing;
e
x
aminer
sees
no
thing).
R
e
trobulbar
op
tic
neuritis.
Neurologic
e
x
amination.
Intrare
tinal
linear
hemorrhages:
!
I
n
on
e
q
u
a
d
r
a
n
t
!
In
tw
o
q
uadrants
!
I
n
f
ou
r
q
u
a
d
r
a
n
t
s
!
Branc
h
r
e
tinal
v
ein
occlusion.
!
Hemispherical
occlusion.
!
Central
re
tinal
v
ein
occlusion.
!
Ophthalmoscop
y
(linear
hemor-
rhages).
!
Fluorescein
angiograph
y
t
o
dif
f
e
rentiat
e
isc
hemic
from
non-
isc
hemic
type.
!
Segment
al
or
t
o
t
a
l
visual
field
def
ects.
!
Sudden
unilat
eral
blindness.
Central
re
tinal
ar
t
e
r
y
occlusion.
Ophthalmoscop
y:
whitish
re
tinal
edema,
visible
“c
herr
y
red
spo
t”
(macula).
19CardinalSymptoms
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All rights reserved. Usage subject to terms and conditions of license.

564. 546
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Decreased
visual
acuity P
e
r
sisting
longer
than
2
4
hour
s,
sud-
den
onse
t,
painless.
!
P
atient
is
usually
o
v
e
r
60.
!
Unilat
eral
decrease
in
visual
acu-
ity
.
!
Headac
hes.
!
T
e
mporal
ar
t
e
r
y
is
t
ender
t
o
pal-
pation.
!
Cer
vical
m
y
algia.
!
P
ain
when
c
h
ewing.
!
W
eight
loss.
!
AION:
ant
erior
isc
hemic
op
tic
neuropath
y
due
t
o
ar
thritis
in
giant
cell
ar
thritis
or
t
e
mporal
ar
t
e
ritis.
Risk
of
blindness.
!
Ar
t
e
rial
biopsy
and
hist
ologic
e
x
amination
are
indicat
ed.
!
Circular
or
segment
al
sw
elling
of
the
o
p
tic
disk
will
be
visible
upon
ophthalmoscop
y
.
!
De
t
e
rmine
er
ythrocyt
e
sedi-
ment
ation
rat
e
and
le
v
e
l
o
f
C
-
reactiv
e
p
ro
t
ein
(height
ened
le
v
els
in
t
e
mporal
ar
t
e
ritis).
!
Unequiv
ocal
diagnosis.
Slo
wly
increasing
o
v
er
a
period
of
w
eek
s,
months,
or
y
ear
s;
painless:
!
Gra
y
t
o
whit
e
pupillar
y
r
efle
x.
!
Loss
of
contrast.
!
Increased
glare.
Cat
aract.
Slit-lamp
e
xamination.
Diagnosis
is
unequiv
ocal
where
t
he
opacity
is
visible
under
re
troillumination.
!
Corneal
opacification.
!
C
o
r
n
e
a
l
s
c
a
r
r
in
g
.
Chronic
corneal
degeneration,
k
e
r
a
t
o
p
a
t
h
y
.
!
Unequiv
ocal
diagnosis.
!
Slit-lamp
e
xamination
will
re
v
eal
corneal
degeneration
and
scar-
r
in
g
.
Slo
w
,
painless:
!
Central
visual
field
def
ect.
!
P
atient
is
usually
o
v
e
r
65.
!
Blurred
vision,
micropsia,
and
macropsia
ma
y
b
e
p
resent.
A
g
e-relat
ed
macular
degenera-
tion.
Fluorescein
angiograph
y
.
!
Increased
intraocular
pressure.
!
V
isual
field
def
ects
Primar
y
c
hronic
open
angle
glau-
coma.
Risk
of
blindness.
!
Measure
intraocular
pressure.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

565. 547
Decreased
visual
acuity
is
typically
more
s
e
v
ere
in
t
he
morning
than
in
the
e
v
ening.
F
u
c
h
s
’
endo
thelial
dystroph
y
.
!
Slit-lamp
e
xamination.
Specifically
decreased
visual
acuity
in
near
or
dist
ance
vision.
!
My
opia.
!
Hyperopia.
T
e
st
visual
acuity
.
P
ainful,
acut
e:
Whitish
corneal
opacification.
!
R
e
d
e
y
e
,
hard
t
o
palpation.
!
Pupil
fix
e
d
and
dilat
ed.
!
N
ausea,
possible
with
v
omiting.
!
Central
sco
t
oma.
!
Increasing
loss
of
visual
acuity
f
ollo
wing
e
x
ercise;
pain
from
post
e
rior
sw
elling
and
with
e
y
e
mo
tion.
!
Normal
findings
upon
ophthal-
moscop
y
(patient
sees
no
thing;
e
x
aminer
sees
no
thing).
A
cut
e
k
erat
oconus.
Glaucoma
att
ac
k.
R
e
trobulbar
op
tic
neuritis.
T
ypical
conical
projection
of
the
cornea
is
visible
under
slit-lamp
e
x
amination.
Risk
of
blindness.
!
Measure
intraocular
pressure
immediat
ely
.
Neurologic
e
x
amination.
!
Combined
injection.
!
Ey
e
pain.
!
F
ibrin
and
cells
in
the
ant
erior
c
hamber
.
!
V
itreous
infiltration.
!
Ant
erior
and
post
e
rior
syne-
c
hiae.
Uv
eitis.
Slit-lamp
e
xamination.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

566. 548
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
Whit
e
pupillar
y
refle
x
(leuk
o-
coria)
in
c
hildren
Of
t
e
n
f
ir
st
no
ticed
in
pho
t
o
s
R
e
tinoblas-
t
oma
(whitish
vitreous,
re
ti-
nal,
or
subre
ti-
nal
tumor)
should
be
e
x
cluded
in
leuk
ocoria.
Unilat
eral
or
bilat
eral.
Cat
aract.
Slit-lamp
e
xamination.
Diagnosis
is
unequiv
ocal
where
lens
opacity
is
visible
under
re
troillumination.
!
Up
t
o
90
%
o
f
patients
are
male
among
c
hildren
and
t
eenager
s.
!
Unilat
eral
leuk
ocoria
(occa-
sionally
combined
with
s
tra-
bismus).
!
Exudativ
e
r
e
tinal
de
t
a
c
hment
visible
upon
ophthalmoscop
y
.
Coats
’
disease.
Unequiv
ocal
diagnosis.
R
e
tinal
de
t
a
c
hment
visible
upon
ophthalmoscop
y
.
R
e
tinal
de
t
a
c
hment,
f
o
r
e
xample
in
re
tinopath
y
of
prematurity
.
Unequiv
ocal
diagnosis.
!
Usually
unilat
eral.
!
Congenit
al
(leuk
ocoria
manif
ests
itself
at
bir
th).
!
Microphthalmos
is
usually
p
res-
ent.
PHPV
(per
sist
ent
h
yperplastic
pri-
mar
y
vitreous).
Ultrasound
scan.
!
Usually
unilat
eral
(tw
o-thirds
of
all
cases).
!
Ma
y
b
e
accompanied
b
y
red
e
y
e
.
!
Child
is
usually
belo
w
t
he
age
o
f
three.
!
Globe
is
normal
size.
R
e
tinoblast
oma
(whitish
vitreous,
re
tinal,
or
subre
tinal
tumor).
!
Ophthalmoscop
y
also
in
f
ello
w
e
y
e
t
o
e
x
clude
a
bilat
eral
re
tino-
blast
oma.
!
CT
scan.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

567. 549
B
lu
r
r
e
d
o
r
d
is
-
t
o
r
t
ed
vision
Especially
when
fixating
close
or
remo
t
e
objects.
R
efraction
anomaly
(
m
y
opia
or
h
yperopia).
R
efraction
t
e
sting.
!
Older
patients
(65
and
older).
!
Gradual
progressiv
e
loss
of
visual
acuity
.
A
g
e-relat
ed
macular
degenera-
tion.
Ophthalmoscop
y
.
!
P
atient
under
emo
tional
of
ph
ysical
stress.
!
Men
in
their
thir
ties
and
f
o
r
ties
are
most
commonly
a
f
f
ect
ed.
!
Objects
appear
enlarged
o
r
reduced
in
size.
!
Central
relativ
e
visual
field
def
ects
(patients
see
a
dark
spo
t).
Central
serous
c
horiore
tinopath
y
.
Ophthalmoscop
y
.
Headac
hes,
possibly
with
nausea.Scintillating
sco
t
oma.
Ocular
migraine.
Unequiv
ocal
diagnosis.
!
P
e
rmanent
or
w
o
r
sening.
!
P
ossibly
with
diplopia.
!
Increased
glare.
!
Gra
y
t
o
whit
e
pupillar
y
r
efle
x.
Cat
aract.
Slit-lamp
e
xamination
will
re
v
eal
ob
vious
lens
opacity
where
a
cat
a-
ract
is
present.
N
a
rro
w
ed
or
dilat
ed
pupil.
F
ollo
wing
administration
of
e
y
e-
drops
(mio
tics
or
m
ydriatics).
Unequiv
ocal
diagnosis.
!
F
undus
refle
x
absent
or
w
eak.
!
P
atient
sees
shado
ws
(a
“w
all”
from
belo
w
o
r
a
“cur
t
ain”
from
abo
v
e).
R
e
tinal
de
t
a
c
hment.
!
Neurologic
e
x
amination.
!
CT
scan.
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

568. 550
Cardinal s
y
m
pt
o
m
s
P
ossible
associated
sympt
oms
and
f
indings
T
ent
ativ
e
diagnosis
(probable
underlying
clinical
picture)
F
u
r
t
her
diagnostic
w
o
rk
-up
B
lu
r
r
e
d
o
r
d
is
-
t
o
r
t
ed
vision
!
Headac
he.
!
V
isual
field
def
ect.
!
Diplopia.
!
Ophthalmoplegia.
!
Prominent,
edemat
ous
op
tic
d
is
k
.
Cerebral
cause
(tumor
or
increas-
ed
intracranial
pressure).
19CardinalSymptoms
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

569. 551
Appendix
1
T
opical
ophthalmic
preparations
Pharmaceutical
Indications
Ocular
ef
fects
and
side
ef
fects
Syst
emic
side
ef
fects
A
c
y
clo
vir
!
Herpes
simple
x
k
eratitis.
!
Herpes
zost
e
r
ophthalmi-
cus.
Local
irrit
ation,
k
e
ratitis,
allergic
reac-
tion
in
e
y
elids
and
conjunctiv
a.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
A
t
r
o
p
in
e
!
Cy
cloplegia.
!
Uv
eitis.
Mydriasis,
angle
closure
g
laucoma,
cy
cloplegia,
decreased
visual
acuity
,
increased
intraocular
pressure.
Confusion,
t
a
c
h
y
cardia,
dr
y
mouth.
Be
t
a
bloc
k
e
r
s
(timolol)
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
decreased
visual
acuity
,
d
r
y
e
y
e.
Bronc
hoconstriction,
brady
car-
dia.
Carbac
hol
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
miosis,
accommodation
spasm,
decreased
visual
acuity
.
F
e
v
e
r
,
syncope,
nausea.
Clonidine
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
decreased
blood
supply
t
o
t
he
head
of
the
o
p
tic
ner
v
e
.
Decreased
blood
pressure.
Cy
clopent
olat
e
!
Mydriatic.
!
Cy
cloplegic.
Mydriasis,
angle
closure
g
laucoma,
decreased
visual
acuity
,
increased
intraocular
pressure.
Central
ner
v
ous
syst
em
dys-
function,
t
a
c
h
y
cardia,
dr
y
mouth,
nausea.
Chloramphenicol
Se
v
e
re
ocular
bact
erial
inf
ec-
tions.
Local
irrit
ation,
k
e
ratitis,
allergic
reac-
tion
in
e
y
elids
and
conjunctiv
a,
k
er-
atitis.
Aplastic
anemia
(rare).
Continued
!
Appendix1
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

570. 552
Appendix
1
(Continued)
Pharmaceutical
Indications
Ocular
ef
fects
and
side
ef
fects
Systemic
side
ef
fects
Dor
zolamide
(local
carbonic
anh
ydrase
inhibit
or)
!
Glaucoma
therap
y
.
!
Proph
ylaxis
ag
ainst
increased
intraocular
pres-
sure
f
ollo
wing
laser
s
u
r
g
e
r
y
.
Local
allergic
reaction
in
e
y
elids
and
conjunctiv
a.
Malaise,
depression,
me
t
allic
t
a
s
t
e
.
Dipiv
efrin
!
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
local
irrit
ation
and
allergic
reaction
in
e
y
elids
and
conjunctiv
a,
m
ydriasis,
angle
clo-
sure
g
laucoma.
T
a
c
h
y
cardia,
cardiac
arrh
yth-
mia,
increased
blood
pressure,
headac
hes.
Epinephrine
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
cyst
oid
macular
edema.
Headac
hes,
per
spiration,
syn-
cope.
Ec
ho
thiophat
e
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
miosis,
decreased
visual
acuity
,
accommoda-
tion
spasm.
N
ausea,
dyspnea,
brady
cardia.
Gent
amicin
Ocular
bact
erial
inf
ections,
especially
Pseudomonas
aer-
uginosa
,
Esc
heric
hia
coli
,
Pr
o-
t
eus
species,
Klebsiella
pneu-
moniae
.
Local
irrit
ation
and
allergic
reaction
in
e
y
elids
and
conjunctiv
a,
k
e
ratitis;
intra
vitreous
administration
ma
y
cause
re
tinal
damage
and
atroph
y
o
f
t
he
op
tic
ner
v
e
.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
Glucocor
ticoids
Anti-inflammat
or
y
t
herap
y
.
Increased
intraocular
pressure,
post
e
-
rior
subcapsular
cat
aract.
Decreased
plasma
cor
tisol
le
v
els.
Appendix1
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

571. 553
Guane
thidine
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
local
irrit
ation,
miosis,
p
t
osis,
decreased
visual
acuity
.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
Ido
xuridine,
trifluridine,
vidarabine
Herpes
simple
x
k
eratitis
Local
irrit
ation,
corneal
damage,
p
t
osis,
and
obstr
uction
of
the
punctum
lacri-
male.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
N
aphazoline
Symp
t
omatic
treatment
of
allergic
or
inflammat
or
y
r
eac-
tions.
Conjunctiv
al
v
asoconstriction,
local
irri-
t
ation,
m
ydriasis,
angle
closure
g
lau-
coma,
k
e
ratitis.
R
a
re:
headac
hes,
increased
blood
pressure,
nausea,
car-
d
ia
c
a
r
r
h
y
t
h
m
ia
.
Neostigmine
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
local
irrit
ation,
miosis,
accommodation
spasm,
decreased
visual
acuity
.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
P
enicillin
Ocular
bact
erial
inf
ections.
Local
irrit
ations,
allergic
reactions
in
e
y
elids
and
conjunctiv
a.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
P
h
e
n
y
le
p
h
r
in
e
!
Mydriatic.
!
V
asoconstrict
or
.
Mydriasis,
angle
closure
g
laucoma,
v
asoconstriction.
Increased
blood
pressure,
m
y
ocardial
inf
arction,
t
a
c
h
y-
cardia.
Pilocarpine
Glaucoma
therap
y
.
Decreased
intraocular
pressure,
miosis,
accommodation
spasm,
decreased
visual
acuity
,
r
e
tinal
t
ear
s
(
rare).
Headac
hes,
nausea,
brady
car-
dia,
decreased
blood
pressure,
bronc
hospasm.
Rifampicin
Ocular
inf
ections
with
Chlam
y-
dia
.
Conjunctiv
al
h
yperemia,
pain,
t
earing.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
Continued
!
Appendix1
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

572. 554
Appendix
1
(Continued)
Pharmaceutical
Indications
Ocular
ef
fects
and
side
ef
fects
Systemic
side
ef
fects
Scopolamine
!
Therapeutic
m
ydriasis.
!
Uv
eitis.
Decreased
visual
acuity
,
m
ydriasis,
angle
closure
g
laucoma,
cy
cloplegia,
increased
intraocular
pressure.
Confusion,
hallucinations.
Sulf
onamide
Ocular
bact
erial
inf
ections.
Local
irrit
ation,
allergic
reaction,
k
er-
atitis.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
T
e
tracy
cline
Ocular
bact
erial
inf
ections
(including
My
coplasma
strains).
Unspecific
conjunctivitis,
allergic
reac-
tions.
No
kno
wn
syst
emic
ef
f
ects
from
t
opical
use.
Appendix1
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

573. 555
Appendix
2
Non-ophthalmic
preparations
with
ocular
side
ef
f
ects
S
y
s
te
m
i
c
c
a
r
di
o
v
a
s
c
u
-
lar
prepar
ations
Indications
Ocular
ef
fects
and
side
ef
fects
Systemic
side
ef
fects
A
t
r
o
p
in
e
!
Brady
cardic
arrh
ythmia.
!
Gastroint
estinal
spasms.
Decreased
visual
acuity
,
m
ydriasis,
angle
closure
g
laucoma,
visual
halluci-
nations.
T
a
c
h
y
cardia,
agit
ation,
confu-
sion.
Amiodarone
!
V
e
n
t
r
ic
u
la
r
a
r
r
h
y
t
h
m
ia
s
that
do
no
t
r
espond
t
o
treatment.
Y
ello
wish
bro
w
n
deposits
in
the
cornea,
conjunctiv
a,
and
lens.
Th
yroid
dysfunction,
pulmo-
nar
y
f
ibrosis,
pho
t
osensitivity
.
Be
t
a
bloc
k
e
r
s
!
Ar
t
e
rial
h
yper
t
ension.
!
Coronar
y
hear
t
disease.
!
Cardiac
insuf
ficiency
(in
lo
w
doses).
Decreased
visual
acuity
,
visual
halluci-
nations,
decreased
intraocular
pres-
sure,
dr
y
e
y
e
.
Decreased
blood
pressure,
brady
cardia,
dyspnea,
stupor
.
Clonidine
Ar
t
e
rial
h
yper
t
ension.
Decreased
intraocular
pressure,
decreased
visual
acuity
,
allergic
reac-
tion
in
e
y
elids
and
conjunctiv
a.
Sedation,
brady
cardia,
dr
y
mouth,
depressiv
e
moods.
Digit
alis
gly
cosides
(digo
xin,
digit
o
xin,
ace
tyldigo
xin)
!
Cardiac
insuf
ficiency
.
!
Cardiac
arrh
ythmia.
Color
vision
def
ects
(xanthopsia).
N
ausea,
brady
cardia.
Guane
thidine
Ar
t
e
rial
h
yper
t
ension.
Decreased
visual
acuity
,
irrit
ation,
mio-
sis,
p
t
osis,
diplopia,
decreased
intraocular
pressure.
Or
thost
atic
circulat
or
y
symp-
t
o
m
s
,
d
ia
r
r
h
e
a
.
Continued
!
Appendix2
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

574. 556
Appendix
2
(Continued)
S
y
s
te
m
i
c
CN
S
pr
e
pa
r
a
-
tions
Indications
Ocular
ef
fects
and
side
ef
fects
Systemic
side
ef
fects
Amphe
t
amines
!
N
arcolepsy
.
!
Appe
tit
e
suppression.
!
Hyperkine
tic
c
hild
syn-
drome
(pediatrics).
Decreased
visual
acuity
,
m
ydriasis,
angle
closure
g
laucoma,
enlarged
palpebral
fissures,
visual
hallucinations.
A
git
at
ed
and
restless
st
at
es,
t
a
c
h
y
cardia,
insomnia.
Barbiturat
es
!
Epilepsy
.
!
Anesthesia.
!
T
r
anquilizer
s
and
sedativ
es.
Ocular
mo
tility
disturbances
(depressed
con
v
e
rgence
response,
ophthalmoplegia,
n
y
st
agmus),
p
t
osis,
and
blepharoclonus
from
c
h
ronic
use.
Decreased
blood
pressure,
suppression
of
REM
sleep
phases,
respirat
or
y
depression,
h
yperalgesia.
Benzodiazepines
(alpra-
zolam,
diazepam,
clonazepam,
mida-
zolam)
!
Anxie
ty
and
agit
at
ed
st
at
es.
!
Epilepsy
.
!
Insomnia.
Suppression
of
corneal
refle
x
,
depressed
accommodation
and
dep
th
percep
tion,
ocular
mo
tility
distur-
bances,
allergic
conjunctivitis.
R
espirat
or
y
depression,
fatigue,
de
v
elopment
of
t
oler-
ance.
Chloral
h
ydrat
e
Sedativ
e.
Miosis,
p
t
osis,
depressed
con
v
e
rgence
response.
Irrit
ation
of
mucous
mem-
branes,
liv
er
t
o
xicity
.
Chlorpromazine, thioridazine,
per-
phenazine
(group
of
pheno
thiazine
neu-
rolep
tics)
!
Sc
hizophrenia.
!
Psy
c
homo
t
o
r
agit
ation.
!
Manias.
!
Chronic
pain
syndromes.
Decreased
visual
acuity
,
pigment
deposits
on
the
sur
face
of
the
lens
and
cornea,
c
hanges
in
the
r
e
tinal
pigment
epithelium
(especially
with
thioridazine).
P
a
rkinson
’
s
disease,
early
dys-
kinesia
and
t
a
rdiv
e
d
yskinesia,
liv
er
damage.
Appendix2
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

575. 557
Carbamazepine
!
Epilepsy
.
!
Neuralgia
(trigeminal
neuralgia).
Diplopia,
blurred
vision,
sensation
of
hea
viness
in
the
e
y
elids.
F
atigue,
at
axia,
blood
count
c
hanges.
L
-dopa
P
a
rkinson
’
s
disease.
Mydriasis
(angle
closure
g
laucoma),
e
y
elid
re
traction,
p
t
osis.
Or
thost
atic
circulat
or
y
symp-
t
oms,
nausea,
dyskinesia,
psy-
c
hosis.
Haloperidol
(group
of
butyrophenone
neu-
rolep
tics)
!
Sc
hizophrenia.
!
Psy
c
homo
t
o
r
agit
ation.
!
Manias.
!
Chronic
pain
syndromes.
Mydriasis,
decreased
visual
acuity
.
P
arkinson
’
s
disease,
early
dys-
kinesia
and
t
a
rdiv
e
d
yskinesia,
liv
er
damage.
Lithium
!
Manic
phases.
!
Proph
ylaxis
ag
ainst
endog-
enous
depression.
Decreased
visual
acuity
,
n
yst
agmus,
e
x
ophthalmos
(due
t
o
th
yroid
dysfunc-
tion).
Goit
er
,
a
t
axia,
diarrhea,
tremor
.
Morphine
Se
v
e
re
pain.
Miosis,
decreased
visual
acuity
,
decreased
accommodation
and
con
v
er-
gence
reaction.
During
withdra
w
al:
m
ydriasis,
t
earing,
and
diplopia.
R
espirat
or
y
depression,
bron-
c
hoconstriction,
constipation,
euphoria
(addictiv
e).
Phen
yt
oin
Epilepsy
.
N
yst
agmus,
decreased
visual
acuity
,
m
ydriasis.
Hyper
tric
hosis,
gingiv
al
h
yper-
plasia,
cerebral
at
axia,
ost
eopath
y
.
T
r
icy
clic
antidepressants
(amitrip
tyline,
desi-
pramine,
imipramine)
Depression.
Mydriasis,
angle
closure
g
laucoma,
cy
cloplegia,
dr
y
e
y
es,
diplopia.
T
a
c
h
y
cardia,
constipation,
mic-
turition
dif
ficulties.
Continued
!
Appendix2
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

576. 558
Appendix
2
(Continued)
S
y
s
t
e
m
i
c
m
e
d
i
c
a
t
i
o
n
s
for
t
reating
infection
Indications
Indications
Ocular
ef
fects
and
side
ef
fects
Systemic
side
ef
fects
Chloramphenicol
Se
v
e
re
bact
erial
inf
ections
suc
h
a
s
abdominal
typhus,
Haemophilus
influenzae
meningitis.
Decreased
visual
acuity
,
visual
field
c
hanges
(sco
t
omas
or
limit
ation),
op
tic
neuritis
or
re
trobulbar
op
tic
neuritis,
local
allergic
reactions.
Aplastic
anemia,
g
a
stroint
esti-
n
a
l
d
y
s
f
u
n
c
t
io
n
,
f
e
v
e
r
,
›
g
r
a
y
syndrome
’.
Chloroquine
and
h
ydro
x
y
c
hloroquine
!
Malaria.
!
Amebiasis.
Deposits
on
the
cornea,
c
hanges
in
the
re
tinal
pigment
epithelium
(bull’
s
e
y
e
maculopath
y),
visual
field
c
hanges.
N
ausea,
headac
he,
bleac
hing
of
the
hair
,
blood
count
c
hanges.
Quinine
Malaria
inf
ection.
Decreased
visual
acuity
including
t
o
xic
ambly
opia,
m
ydriasis,
re
tinal
damage
(edema
or
v
ascular
constriction),
op
tic
disk
edema,
sco
t
omas.
Hemolytic
anemia,
allergic
reactions,
hearing
loss.
Ethambut
ol
T
uberculosis.
Op
tic
neuritis,
visual
field
c
hanges,
color
vision
def
ects.
Hyper
uricemia,
nausea.
Isoniazid
T
uberculosis.
Op
tic
neuritis,
atroph
y
o
f
t
he
op
tic
ner
v
e
,
visual
field
c
hanges,
op
tic
disk
edema,
color
vision
def
ects.
P
o
lyneuropath
y
(vit
amin
B6
me
t
abolic
dysfunction),
aller-
gic
reactions,
liv
er
damage.
P
enicillin
Bact
erial
inf
ections.
Mydriasis,
depressed
accommodation,
diplopia,
op
tic
disk
edema
with
cere-
bral
pseudo
tumor
(secondar
y).
N
ausea,
allergic
reactions.
Appendix2
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

577. 559
Rifampicin
T
uberculosis.
Conjunctiv
al
h
yperemia,
blepharocon-
junctivitis,
color
c
hange
(orange)
of
fluid
is
possible.
Liv
er
dysfunction,
nausea,
allergic
reactions,
hepatic
enzyme
induction.
S
t
rep
t
om
y
cin
T
uberculosis.
Nyst
agmus,
decreased
visual
acuity
,
t
o
xic
ambly
opia,
color
vision
def
ect,
atroph
y
o
f
t
he
op
tic
ner
v
e
.
Ot
o
t
o
xicity
,
nephro
t
o
xicity
,
a
lle
r
g
y
.
Sulf
onamides
Bact
erial
inf
ections.
My
opia,
unspecific
irrit
ation.
Allergic
reactions,
nausea,
pho
t
osensitivity
.
T
e
tracy
cline
Bact
erial
inf
ections.
My
opia,
op
tic
disk
edema
with
cerebral
pseudo
tumor
,
decreased
visual
acuity
,
diplopia.
N
ausea,
allergic
reactions,
liv
er
damage.
S
y
s
t
e
m
i
c
m
e
d
i
c
a
t
i
o
n
s
for
t
reating
rheumatic
disorder
s
Indications
Ocular
ef
fects
and
side
ef
fects
Systemic
side
ef
fects
Chloroquine
and
h
ydro
x
y
c
hloroquine
Base
medication
in
rheuma-
t
oid
ar
thritis.
Deposits
on
the
cornea,
c
hanges
in
the
re
tinal
pigment
epithelium
(bull’
s
e
y
e
maculopath
y),
visual
field
c
hanges.
N
ausea,
headac
he,
bleac
hing
of
the
hair
,
blood
count
c
hanges.
Gold
salts
Base
medication
in
rheuma-
t
oid
ar
thritis.
Deposits
on
the
e
y
elids,
conjunctiv
a,
cornea
(c
hr
ysiasis),
and
lens
(rare).
Pt
o-
sis,
n
y
st
agmus,
and
diplopia
are
r
are.
Blood
count
c
hanges,
nephro
t
o
xicity
,
mucous
mem-
brane
damage.
Continued
!
Appendix2
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

578. 560
Appendix
2
(Continued)
S
y
s
t
e
m
i
c
m
e
d
i
c
a
t
i
o
n
s
for
t
reating
rheumatic
disorder
s
Indications
Ocular
ef
fects
and
side
ef
fects
Systemic
side
ef
fects
Ibuprof
en
!
Rheumat
oid
ar
thritis.
!
Inflammation
in
degenera-
tiv
e
joint
disease.
Blurred
vision,
diplopia,
color
vision
def
ects,
dr
y
e
y
es,
op
tic
neuritis
(rare).
Damage
t
o
g
astroint
estinal
mucous
membranes.
Indome
thacin
!
Rheumat
oid
ar
thritis.
!
Inflammation
in
degenera-
tiv
e
joint
disease.
Decreased
visual
acuity
,
diplopia,
color
vision
def
ects,
corneal
deposits.
Damage
t
o
g
astroint
estinal
mucous
membranes,
head-
ac
hes.
A
c
e
t
ylsalicylic
acid
and
salicylic
acid
!
F
e
v
e
r
,
pain.
!
Rheumat
oid
ar
thritis.
!
Thrombocyt
e
aggreg
ation
inhibit
or
.
Allergies,
conjunctivitis,
decreased
visual
acuity
,
t
ransient
blindness.
Microscopic
g
a
stroint
estinal
bleeding,
allergies,
bron-
c
hospasm,
o
t
o
t
o
xic
side
ef
f
ects.
Hormone
prepar
ations
Indications
Ocular
ef
fects
and
s
i
d
e
e
f
f
e
c
t
s
S
y
s
t
e
m
i
c
s
i
d
e
e
f
f
e
c
t
s
Glucocor
ticoids
!
Anaph
ylactic
shoc
k,
immu-
nosuppressiv
e
t
herap
y
(suc
h
a
s
in
ulcerous
colitis
or
immunohemolytic
anemia).
!
Bronc
hial
asthma.
!
A
cut
e
r
heumatic
f
e
v
e
r
.
Decreased
visual
acuity
,
increased
intraocular
pressure,
post
e
rior
sub-
capsular
cat
aract.
Increased
blood
glucose
le
v
els,
Cushing’
s
syndrome,
ost
eo-
porosis,
increased
risk
of
thrombosis,
increased
suscep-
tibility
t
o
inf
ection.
Appendix2
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

579. 561
Oral
contracep
tiv
es
!
Contracep
tion.
!
R
egulation
of
menstr
ual
cy
cle.
Decreased
visual
acuity
,
r
e
tinal
v
ascular
c
hanges
(occlusion,
bleeding,
spasm),
re
tinal
edema,
visual
field
c
hanges,
op
tic
neuritis.
V
a
ricosis,
migraine,
edemas.
Other
impor
t
ant
medi-
cations
with
ocular
side
ef
fects
Coumarin
deriv
ativ
es
(phenprocoumon,
w
ar-
farin)
Thinning
of
blood
as
proph
y-
laxis
ag
ainst
and
treatment
of
v
enous
thrombosis.
Subconjunctiv
al
or
re
tinal
bleeding,
h
yphema.
Loss
of
hair
,
nausea,
cerebral
bleeding,
spont
aneous
hema-
t
omas.
V
it
amin
A
!
V
it
amin
A
deficiency
.
!
A
cne
vulg
aris.
Loss
of
e
y
elashes,
increased
intracranial
pressure
(cerebral
pseudo
tumor),
diplopia,
strabismus.
Se
v
e
re
headac
hes,
loss
of
hair
,
nausea,
pr
uritus,
rhag
ades,
bone
and
joint
pain.
V
it
amin
D
!
V
it
amin
D
deficiency
.
!
Hypoparath
y
roidism.
S
t
rabismus,
calcium
deposits
in
the
conjunctiv
a
and
cornea
(calcific
band
k
e
rat
opath
y),
atroph
y
o
f
t
he
op
tic
ner
v
e
due
t
o
calcium
occlusion
of
the
op
tic
canal.
Calcification
of
parenc
h
ymal
org
ans
suc
h
a
s
t
he
kidne
ys.
Nico
tinic
acid
F
a
t
m
e
t
abolism
disorder
s.
Cyst
oid
maculopath
y
,
decreased
visual
acuity
,
local
allergic
reactions.
Flush
symp
t
oms,
restlessness,
nausea,
v
omiting,
diarrhea.
Appendix2
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

580. 562
Appendix
3
Ocular
symp
t
oms
of
poisoning
T
o
xic
subst
ance
Ocular
ef
fects
and
side
ef
fects
Systemic
side
ef
fects
A
t
ropine
Mydriasis,
decreased
visual
acuity
,
angle
closure
glaucoma,
cy
cloplegia,
increased
intraocular
pres-
sure.
Dr
y
mouth,
dr
y
skin,
confusion,
t
a
c
h
y
cardia,
h
yper-
thermia.
Lead
Increased
intraocular
pressure.
F
atigue,
headac
he
and
pain
in
the
e
xtremities,
pale-
ness,
colic,
paralysis,
lead
halo
on
the
gums.
Quinine
Decreased
visual
acuity
,
r
e
tinal
v
ascular
spasms,
atroph
y
o
f
t
he
op
tic
ner
v
e
including
blindness.
Allergic
reactions,
hemolytic
anemia,
v
e
r
tigo,
tinni-
tus,
cy
anosis,
cardiac
death.
Digit
alis
Scintillation,
patient
sees
clouds,
color
vision
def
ects.
Cardiac
arrh
ythmia,
(A
V
conduction
bloc
k
s
,
bigemin
y),
nausea,
v
omiting,
headac
hes,
confusion.
Ethanol
T
r
ansient
ambly
opia,
decreased
intraocular
pres-
sure,
n
y
st
agmus,
diplopia,
conjunctiv
al
h
yperemia.
Disturbed
g
ait,
disorient
ation
including
impaired
consciousness,
cramps,
t
a
c
h
y
cardia.
Me
th
yl
alcohol
A
t
roph
y
o
f
t
he
op
tic
ner
v
e
including
blindness.
N
ausea,
colic,
acidosis,
oliguria.
Appendix3
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

581. 563
Index
A
Apatterndeviation469,471
A-modescan409
abducentnerve
lesions482
palsy486,490–491
retractionsyndrome
490–491
aberrations456–458
astigmaticaberration457,
458
chromaticaberration456
curvatureoffield456,458
sphericalaberration456–
457
abscess
eyelid36–37,64,538
lacrimalsac57,58
vitreousbody291
absoluteglaucoma236
absorptionlenses450
Acanthamoebakeratitis136–
137,455
accommodation425–428
impairments445–447
palsy446–447
spasm445–446
rangeof427–428
accommodativeconver-
gence/accommoda-
tion
ratio470–471
accommodativeesotropia
470
accommodometer445
aceclidine
effectsonpupil224
glaucomatreatment256
acetazolamide,glaucoma
treatment260
acetylcholine,effectson
pupil224
acetyldigoxin555
acetylsalicylicacid560
aciclovir551
acidburns518
acidicmucopolysaccharide
deposits148,150
acquired
nuclearcataract173,174,
176
ptosis22–24
strabismus469
acquiredimmunedeficiency
syndrome(AIDS)113
relatedretinaldisorders
349–350
acute
conjunctivitis74,98,100
dacryoadenitis64
dacryocystitis57–58
glaucoma265–270
iridocyclitis208–212
iritis208–212
retinalnecrosissyndrome
133
adaptation303,428
sensoryadaptation466
adenocarcinoma
eyelids47
lacrimalgland66
adenoma
lacrimalgland66
pituitarygland396,397
Adie'stonicpupilsyndrome
228
adrenochromedeposits111,
114
against-the-ruleastig-
matism441
age-relatedmacular
degeneration337–
338,546,549
aging
accommodationchanges
428
conjunctiva69–74
fundus310
lens168
opticdisk310
retinaldegenerativedis-
ordersseeretina
vitreousbody282–284
AIDSseeacquiredimmune
deficiencysyndrome
albinism201,206–207,531
irisprintlensesand454
alexia401
alkaliburns518
alkaptonuriaseeochronosis
allergicconjunctivitis
76–78,82,98–99,
101–102
allograftrejection152–153,
154
Alport'ssyndrome169
alprazolam556
alternating
esotropia468
strabismus73
amaurosisfugax544
amauroticcat'seye354
amblyopia
anisometropic444
congenitalcataractand
193,195
congenitalptosisand23,
24
formsof467
strabismusand469
A
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

582. 564
amblyopia
strabismusand
amblyopiasecondaryto
suppression466–467
infantilestrabismic
amblyopia472–473,
477–479
ametropia424,451
amiodarone555
amitriptyline557
amphetamines556
amyloiddeposits148
vitreousbody287
anesthesia,incataract
surgery187
aneurysm,internalcarotid
artery398,399
angiography201,202,409
fundus307–308
angiomatosisretinae356
angleclosureglaucomasee
glaucoma
angleofdeviation
incomitant481,488–490
measurementof474–
476,492–493
primary490
secondary490
angularartery404
angularvein405
aniridia202–203,531,533
irisprintlensesand454
traumatic510
aniseikonia187,444,465–
466
anisocoria226
simple229
withconstrictedpupilin
theaffectedeye229–
230
withdilatedpupilinthe
affectedeye228–229
completeoculomotor
palsy228
irisdefects229
tonicpupil228
anisometropia444–445,
451,465
contactlenscorrection
444,445
ankyloblepharon22
anteriorchamber
chronicinflammation146
depthof11–12,238
examinationof11–12
gonioscopy238–240,275
obliqueillumination
238
slit-lampexamination
238
anteriorischemicopticneu-
ropathy(AION)374–
379,536,544,546
arteriosclerotic374–378
arteritic378–379
aperture428
aphakia438,440
postoperative187,438
unilateral445
applanationtonometry240,
242,275,435
apraclonidine,glaucoma
treatment258
aqueoushumor167
circulation233–235
arcofWilbrand389,398
arcussenilis145
Ardenratio313
argonlasertrabeculoplasty
261–262
Argyll-Robertsonpupil230–
231
argyrosis114,146
Arlt'striangle208
arterialpulse13
arterioscleroticchanges
323–325
anteriorischemicoptic
neuropathy(AION)
374–378,536,544,
546
arteriovenousfistula409
arteriticanteriorischemic
optic
neuropathy(AION)378–
379
artery
angular404
ciliary157,199,361
hyaloid,persistent285,
366
internalcarotid396,404
aneurysm398,399
stenosis404
ophthalmic199,404
retinal302
central302,361
occlusion320–323,545
supraorbital404
supratrochlear404
aspergillomycosis417
asteroidhyalosis287
asthenopicsymptoms438,
537
astigmaticaberration457,
458
astigmatism5,121,433,
440–444,457
against-the-rule441
correction442–444
contactlenses453
photorefractivekera-
tectomy156
external441,444
internal441,444
irregular441–442,444
oblique441
regular441,442
total440
with-the-rule441
astrocytoma
opticnerve385–386
retina355–356
atrophy534
infantilerecessiveoptic
atrophy383
Leber's382–383
opticnerve380–383,
399,407
waxypalloropticatrophy
383
atropine551,555,562
effectsonpupil225
automatedrefractometry
431
avulsion
globe512
opticnerve512
axial
hyperopia438
myopia434
B
B-modescan408
bacterial
conjunctivitis60,76–78,
81–87
Index
B
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

583. 565
keratitis130–132
Bagolinitest476–477
barbiturates556
Barkan'smembrane273
barreldistortion457
basalcellcarcinoma45–47
Behr'sdisease383
Bell'sphenomenon17
benzodiazepines556
Bergmeister'spapilla366
Berlin'sedema511
Best'svitelliformdystrophy
313,341–342
betablockers551,555
glaucomatreatment259
betaxolol,glaucomatreat-
ment259
bicanicularringintubation
502–503
Bielschowskyheadtilttest
488,489,492
bifocallenses
contactlenses454
eyeglasses447
accommodative
esotropiacorrection
470
bilateralafferentpupillary
defect227
binocular
alignment6
loupes456
testing451
vision461–465,466,469
fusion464
simultaneousvision
461–464
stereoscopicvision465
testingof476–477
birthmarks,conjunctival
108
Bitot'sspots73
blackcataract173
bleedingseehemorrhage
blepharitis528
inherpeszosterophthal-
micus35
seborrheic33–34
blepharochalasis19,539,
540
blepharophimosis21,23
blepharospasm10,30
entropionand26,27
withconjunctivitis74
blindspot360
blindersphenomenon399
blinking17
blow-outfracture507–513,
532,534,539,540
blurredvision549–550
bonysocket403
blow-outfracture507–
513,532,534,539,
540
botulism,accommodation
palsyin446
Bourneville'sdisease355,
386
Bowman'slayerofthecor-
nea70,117
calcificdeposits146–147
brachytherapy217
branchretinalveinocclu-
sion319,545
bridgecoloboma203
brimonidine,glaucoma
treatment258
Brodmann'sarea17:389
Bruch'smembrane201,339
Brückner'stest168–169
brunescentnuclearcataract
173
Budge'scenter220
bulbarconjunctiva67,68
bullouskeratopathy143–
144,148
buphthalmos124,127,273,
285,531
Burkitt'slymphoma421
burns523–524
chemicalburns518
C
calcareousinfiltration72
calcificbandkeratophathy
146–147,212
canalofSchlemm157,234
canalicularstenosis53
canaliculitis61
conjunctivitisin82
Candidaalbicanskeratitis
134–135
capsulorrhexis189
carbachol551
effectsonpupil224
glaucomatreatment256
carbamazepine557
carbonicanhydraseinhibi-
tors552
glaucomatreatment255,
260,268
carcinoma
basalcell45–47
conjunctival107
lacrimalgland66
lacrimalsac61
squamouscell47,107
carteolol,glaucomatreat-
ment259
caruncle,lacrimal67
cataract12,150,167,171–
195,434
classification171–172,
173
complicatedcataracts
180,181
congenital182–185,192–
195
anteriorpyramidalcat-
aract183
ceruleancataract183
coronarycataract183
fromtransplacental
infection185
lamellar/zonularcata-
ract183,184
nuclearcataract183,
184
polarcataract183
definition170
examinationof168–169
insystemicdisease179–
180
cataractwithmyotonic
dystrophy179–180
dermatogeniccataract
180,181
diabeticcataract179
dialysiscataract179
galactosemiccataract
179,185
snowflakecataract179
tetanycataract180
medicaltreatment185
membranouscataract285
secondarycataract192,
193,194,212–213
Index
C
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

584. 566
cataract
secondarycataract
afterintraocular
surgery180
senile173–179
corticalcataract173–
176
cuneiformcataract176
hypermaturecataract
174,177–179
maturecataract174,
177,186
Morgagni'scataract178
nuclearcataract173,
174,176
posteriorsubcapsular
cataract174,176–177
surgicaltreatment185–
195
anesthesia187
durationofhospitaliza-
tion186
inchildren192–195
indications185–186
optionsforachieving
refractive
correction187–188
prognosis186,193
surgicaltechniques
189–192
vitreousbodyand294
symptoms170–171,531,
533,542,546,548,549
inchildren192
toxiccataract182
traumatic180–182,514
contusioncataract180,
182
electricalinjury182
infraredradiationcata-
ract182
visualacuitytesting431
cataracteyeglasses187,188
inchildren194
cavernoussinus
fistula418
thrombosis58,415–416
cellulitis,orbital404,414–
415,416,532,535
differentialdiagnosis58,
64,415
treatment415
cementedglobe414,415
central
fixation476
Horner'ssyndrome230
retinalartery302,361
occlusion321,545
retinalvein361
occlusion319,545
scotoma466
serouschorioretinopathy
335–336,549
ceruleancataract183
chalazion38,39–40,47,
539
checkligaments460
chemicalinjuries517–522
treatment518–521
chemosis
inconjunctivitis76,78,79
inorbitalcellulitis414
inorbitalpseudotumor
416
chiasmsyndrome399
children
cataracts192–195
conjunctivitis93
contactlensuse194,195
glaucoma236,273–277,
529
infantilerecessiveoptic
atrophy383
leukocoria548
opticnerveglioma422
orbitalcellulitis414–415
retinoblastoma353–355
rhabdomyosarcoma421
strabismus459,465–471,
477–479
infantilestrabismic
amblyopia472–473,
477–479
seealsoneonate
chlamydialconjunctivitis
seeconjunctivitis
chloralhydrate556
chloramphenicol551,558
chloroquine558,559
toxicity345,346
chlorpromazine556
cholinergicagents,glau-
comatreatment256
cholinesteraseinhibitors,
glaucoma
treatment257
choristoma420
choroid201
blunttraumaand512
rupture511
examinationof202
tumors202
benign217
melanoma216,217
vessels308
seealsouvealtract
choroiditis208,209,213–
214
chromaticaberration456
chronic
anteriorchamberinflam-
mation146
dacryoadenitis65
dacryocystitis60
iridocyclitis212–213
iritis212–213
progressiveexternaloph-
thalmoplegia(CPEO)
486
chrysiasis146
cicatricial
ectropion28,29,501
entropion27
ciliary
arteries157,199,361
body(muscle)201,438
accommodationmech-
anism426–427
accommodationspasm
445–446
examinationof202
melanoma217
seealsouvealtract
injection75,78,157
nerves157,199
processes201
cilioretinalvessels361
circleofleastconfusion440
clonazepam556
clonidine551,555
glaucomatreatment258
Cloquet'scanal279
closed-circuitTVmagnifier
456
coatedlenses450
Coats'disease325–326,548
cobblestonedegenerations
334
cocaine,effectsonpupil226
Index
C
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

585. 567
coloboma20–21,203–205
bridgecoloboma203
opticdisk384–385
rudimentary364,384
surgical203
traumatic203
coloragnosia401
colorvisionevaluation311–
312,362
computedtomography(CT),
orbitalcavity409
computerizedcorneal
topography121,122–
123,442
computerizedstaticper-
imetry247
concavelenses5,435
seealsoeyeglasslenses
concomitantstrabismus
459,465–479
acquired469
causesof465–466
diagnosis471–477
alternatingstrabismus
473
angleofdeviation
measurement474–
476
differentialdiagnosis
493
infantilestrabismic
amblyopia472–473
ocularalignmenteval-
uation471–472
typeoffixation476
unilateralstrabismus
473
formsof467
microstrabismus469
therapy477–479
eyeglassprescription
477
strabismicamblyopia
477–479
surgery479
verticaldeviations471
seealsoesotropia;
exotropia;strabismus
cone-roddystrophy343,
344
cones303
spacing423
confocalcornealmicroscopy
125,136
confrontationfieldtesting
14
congenital
cataractseecataract
ectropion28,29
entropion24,26,27
esotropia468–469
melanosis108,110,112–
113
nystagmus495
ocularmotilitydistur-
bances482
ptosis22–24,540
toxoplasmosis348–349
seealsochildren;neonate
conjunctiva67–115
bulbarconjunctiva67,68
degenerationandaging
changes69–74
calcareousinfiltration
72
conjunctivalxerosis
72–74,148
pinguecula69,70
pseudopterygium71
pterygium69–71
subconjunctivalhemor-
rhage72
deposits114–115
examinationof7–10,
68–69,80–82
foreignbodies503–505
fornix67
function67
laceration499–503
palpebralconjunctiva17,
20,67
structureof67,68
traumaseeoculartrauma
tumors104–114
carcinoma107
congenitalocularmela-
nosis110,112–113
cysts105,106
epibulbardermoid104,
105
hemangioma104,106
Kaposi'ssarcoma113–
114
lymphoma113
melanosis108–112
nevus108,109
papilloma106–107
seealsoconjunctivitis;
gobletcells
conjunctival
fluoresceindyetest53,
63,69
injection75–78,157
smear80
conjunctivitis74–104,160,
528,538,542
acute74,98,100
allergic76–78,82,
98–99,101–102
bacterial60,76–78,
81–87
chlamydial76–78,
80–81,83,88,92–93
neonatal95,98
contactlenscomplica-
tions455
examinationmethods
80–82
conjunctivalsmear80
epithelialsmear80–82
eyelideversion80
irrigation82
giantpapillarycon-
junctivitis98,102
contactlenscomplica-
tions455
granulomatous104
hordeolumand38
inclusionconjunctivitis
60,88,92–93
mycotic82,91,98
neonatal95–98
differentialdiagnosis
96–97
nodose91
parasitic90–91,98
seborrheicblepharitisand
33
symptomsandfindings
74–79
toxic76–78,97
vernal98,101
viral76–79,81,89,
93–95
seealsokeratoconjuncti-
vitis
consecutiveexotropia471
consensuallightreflex220
Index
C
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

586. 568
consensuallightreflex
testingof221–223
contacteczema,eyelids
30–31
contactlenses451–455
advantagesof451
anisometropiacorrection
444,445
characteristicsof451–
452
complications454–455
disadvantagesof454
followingcataractsurgery
187,188
ininfants194,195
myopiacorrection436
problemswith141–142
rigidcontactlenses452–
453
softcontactlenses453
speciallenses453–454
bifocalcontactlenses
454
cornealshields453
irisprintlenses454
therapeuticcontact
lenses453
sphericallenses453
toriclenses453
contusion506–507
cataract180,182
rosette511
convergentstrabismussee
esotropia
convexlenses5,439,440
seealsoeyeglasslenses
cookedfisheye521,522
copperforeignbodies515
cornea117–156
anatomy117–118
Bowman'slayer70,117
calcificdeposits146–
147
bullouskeratopathy143–
144
contactlensproblems
141–142,454–455
cornealvascularization
455
mechanicalinfluences
454
metabolicinfluences
454
degeneration146–148,
546
calcificbandkera-
topathy146–147
peripheralfurrowker-
atitis147–148
deposits145–146
arcussenilis145
argyrosis146
chrysiasis146
cornealverticillata145–
146
ironlines146
Kayser-Fleischerring
146
Descemet'smembrane
118,128
tearsof126,275
developmentalanomalies
125–127
keratoconus125–126
keratoglobus127
sizeanomalies127
dystrophies148–150
edema208,210
embryology117
endothelium118
epithelium117
densityof121–124
erosion141,453,505–
506,529,530,540
recurring505
examinationof10–11,
120–125
confocalcorneal
microscopy125
densityofepithelium
121–124
diameter124
dyeexamination120–
121
inchildren275
pachymetry125
sensitivity121
slitlamp120
topography121
foreignbodies503–505,
529,530,540
importanceforeye117
mechanicalindentationof
268
nervesupply119
protectivemechanisms
127–128
pterygium70
regeneration117
sensitivity10–11,121,134
lossof141
stroma117
tearfilmsignificance119
transparency119,143
opacification275
traumaseeoculartrauma
seealsokeratitis
corneaplana127
cornealshields453
cornealsurgery150–156
excimerlaserinsitu
keratomileusis
(LASIK)156
holmiumlasercorrection
ofhyperopia156
lamellarkeratoplasty153–
154
penetratingkeratoplasty
(corneal
transplant)117–118,
143,149,152–153
photorefractivekera-
tectomy155
astigmatismcorrection
156
phototherapeutickera-
tectomy154
radialkeratotomy155–
156
coronarycataract183
cortical
blindness401
cataract173–176
cortisone
cataract183
glaucoma271
couching189
coumarinderivatives561
covertest6
crablice37
cranialnervepalsies490,
531
combinedpalsies491–
492
seealsospecificnerves
craniofacialdysplasia409–
410
Index
C
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

587. 569
craniofacialdysostosis
410
oxycephaly409
craniopharyngioma396–
397
craniostenosis409
craniosynostosis535
Credé'sprophylaxis60,95,
97,98
crepitus507
cuneiformcataract176
curvatureoffield456,458
cutaneoushorn42,43
cutislaxasenilis19,539,
540
cyclitis208
Fuchs'heterochromic
cyclitis206
seealsoiridocyclitis
cycloablation264
cyclocryotherapy216,264
cyclodialysis262–264
blunttraumaand510
cyclodiathermy264
cyclopentolate551
effectsonpupil225
cycloplegics426–427
cylindricallenses5,442,
443
cysts
conjunctival105,106
dermoid420
ductal40
epidermoid420
pseudocysts108
round40,41
seealsodacryocystitis
cytomegalovirusretinitis
350,351
D
dacryoadenitis50,64–65
acute64
chronic65
dacryocystitis57–60
acute57–58
chronic60
conjunctivitisin82
neonatal60,97
traumaand499
dacryocystography53
dacryocystorhinostomy
58–59,60
dacryostenosis529
Dalrymple'ssign412
darkadaptation303,304
degenerative
myopia339–340
retinaldisordersseeret-
ina
retinoschisis330,333–
334
seealsoaging
dendritickeratitis132–133
deposits
acidicmucopolysac-
charide148,150
adrenochrome111,114
amyloid148
vitreousbody287
conjunctiva114–115
cornea145–146
Bowman'slayer146–
147
hyaline148–149
iron111,114
depthperception465
dermatochalasis19
dermatogeniccataract180,
181
dermoidcyst420
descemetocele128,129
Descemet'smembrane118,
128
Kayser-Fleischerring146
tearsof126,275
desipramine557
Desmarreseyelidretractor
2,3,9
diabetic
cataract179
papillopathy374
retinopathy272,314–318,
323
nonproliferative315
proliferative215,216,
315–316,317
dialysiscataract179
diaphragmasellae396
diazepam556
dichlorphenamide,glau-
comatreatment260
digitalsubtractiondacry-
ocystography53
digitalis555,562
digitoxin555
digoxin555
dilatorpupillaemuscle200,
220
diphthericconjunctivitis85
dipivefrin552
glaucomatreatment257
diplopia(doublevision)
464,465,486–488,
531–533
binocular531–533
cataractand434
compensatoryheadpos-
ture487–489
crosseddoubleimages
488
inorbitalpseudotumor
416
management493
monocular533
physiologic464
strabismusand466
uncrosseddoubleimages
488,490
vertical491
withblow-outfracture
507
discharge,inconjunctivitis
76,78
diskiformkeratitis133
dislocationofthelens195–
197,533
dispersion456
distortedvision549–550
distortion457
divergentstrabismussee
exotropia
L-dopa557
Dopplerscans409
dorzolamide552
glaucomatreatment260
doublevisionseediplopia
Draeger'sapplanation
tonometry240
Drägeresthesiometer121
drusen310,337,366
opticdisk366–367,372
dryeyesseekeratocon-
junctivitissicca
ductalcysts40
dysostosismultiplex411
dystrophy
Index
D
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

588. 570
dystrophy
Best'svitelliform313,
341–342
cataractwithmyotonic
dystrophy179–180
corneal148–150
Fuchs'endothelial148,
149–150,547
macular148,150,340–342
retinal340–345
vitreoretinal293
E
eccentricfixation476
echothiophate552
ectasia158
ectopialentis196
ectropion28–29,529
cicatricial28,29,501
congenital28,29
paralytic28,29
senile28,29
withductalcysts40,41
eczema,eyelids30–31
edema
Berlin's511
cornea208,210
differentialdiagnosis
32–33
eyelids31–33
macular317,319
opticdisk367–368
infiltrative379
papilledema368–370,
371,372
pseudopapilledema
364–365,372
Edinger-Westphalnuclei
220,229
edrophoniumchloridetest
485
electricalinjury,cataract
and182
electro-oculogram(EOG)
312–313,314
electroretinogram(ERG)
312,313
elephantiasisoftheeyelids
45
emboli,retinalarteryocclu-
sionand320–321
embryology
cornea117
lens165–167,279
retina299
vitreousbody279,280
emergencykeratoplasty
136,152
emmetropia423,424,425,
428
refractionin429
endophthalmitis290–292,
536
endoscopy,lacrimal53
endothelialdystrophy148
endotheliitis133
enophthalmos405,534
causesof406
blow-outfracture507
Horner'ssyndrome230
senile27
entopicphenomenon307
entropion24–27,530
cicatricial27
congenital24,26,27
spastic26–27
eosinophilicgranuloma421
epibulbardermoid104,105
epicanthalfolds21,23
epidermoidcyst420
epikeratophakia151,156
epinephrine552
effectsonpupil226
epipapillarymembrane366
epiphora7,30,64,529–530
inconjunctivitis74,76,
78,98
episcleritis158,159–160,
528,542
epithelialcornealdystrophy
148
epithelialsmear80–82
erythemamultiforme99,
102
esotropia438,459,467–
471
accommodative470
acquiredstrabismus469
alternating468
congenital/infantile468
hyperopiaand465
microstrabismus469
surgicalcorrection479
ethambutol558
ethanol562
ethmoidalaircells403
eversionofeyelids80
lowereyelid7–8,67
uppereyelid8–9,20,67
examination1–16
anteriorchamber11–12
binocularalignment6
choroid202
ciliarybody202
confrontationfieldtesting
14
conjunctiva7–10,68–69,
80–82
lowereyelideversion
7–9,20,69,80
cornea10–11,120–125
equipment1–3
eyelids7,19–20
glaucomaseeglaucoma
history3–4,497
intraocularpressure
measurementsee
intraocularpressure
iris201–202
lacrimalsystem52–57
nasolacrimalduct7
lens12,168–169
ocularmotility5
ophthalmoscopy13
opticnerve223,244–
246,252,362–363
orbitalcavity405–409
pupil221–224
refractiontesting429–
431
retina304–313
fundus304–308,407
sclera157
strabismus471–477
trauma497–498
visualacuity4–5
visualfieldtestingsee
visualfield
visualpathway391–394
vitreousbody281
excimerlaserinsitukerat-
omileusis
(LASIK)151,156
exophthalmometry407–
408
exophthalmos405,534–
535
Index
E
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

589. 571
causesof406
incraniostenosis409
inGraves'disease411–
412
inorbitalcellulitis414
inorbitalpseudotumor
416
intermittent419
pulsating418–419
withscleritis162
seealsoorbitalcavity
exotropia409,459,471
consecutive471
intermittent471,472
secondary471
surgicalcorrection479
exposurekeratitis140
inGraves'disease413
external
astigmatism441,444
oculomotornervepalsy
491
extracapsularcataract
extraction189–192
extraocularmuscles459–
460,462–463
directionofpull461
nervesupply461
paralysis486,491
exudativeretinaldetach-
ment328,329,330
eyebandage16
watchglassbandage29
eyecharts1–2
eyeclosingreflex14,119
eyeointment15
eyepatching478
eyebrows19
eyedrops15,16
eyeglasslenses
aberrations456–458
astigmaticaberration
457,458
chromaticaberration
456
curvatureoffield457,
458
sphericalaberration
456–457
absorptionlenses450
coatedlenses450
cylindricallenses5,442,
443
minus(concave)lenses5,
435–436
monofocallenses447
multifocallenses447–
449
bifocals447
progressiveaddition
lenses449
trifocals449
occlusion478
photochromiclenses450
plasticlenses450
plus(convex)lenses5,
439,440
prescription448,451
sphericallenses447
strabismustreatment477,
480
subjectiverefractiontest-
ing450–451
toriclenses447
eyelashes19
trichiasis30
eyelids17–47
deformities2230
blepharospasm30
ectropion28–29
entropion24–27
ptosis22–24
trichiasis30
developmentalanomalies
20–22
ankyloblepharon22
blepharophimosis21
coloboma20–21
epicanthalfolds21
examinationof7,19–20
eversion7–9,20,67,80
glandulardisorders
38–39
chalazion39
hordeolum38–39
injuriesto498–499,500–
503
protectivefunction17
signs,inGraves'disease
411–412
skin/margindisorders
30–37
abscess36–37,64,538
contacteczema30–31
edema31–33
herpessimplex34
herpeszosterophthal-
micus35–36
louseinfestation37
seborrheicblepharitis
33–34
tickinfestation37
structureof17–19
surgicalretractionof
uppereyelid24,25
swelling538–539
tumors40–47,539,540
adenocarcinoma47
basalcellcarcinoma
45–47
cutaneoushorn42,43
ductalcysts40
hemangioma43–44
keratoacanthoma
42–43
molluscumcontagio-
sum42
neurofibromatosis
(Recklinghausen's
disease)44–45
squamouscellcarci-
noma47
xanthelasma40–42
F
Fabry'sdisease146
facialnervepalsy140
familyhistory3
farpoint427–428
Farnsworth-Munselltests
311,312
farsightednessseehyper-
opia
Fasanella-Servatprocedure
25
Ferry'sline146
filamentarykeratitis63
filtrationsurgery262,263
fistula
arteriovenous409
cavernoussinus418
lacrimalsac57
fixationtype476
flashesoflight537
Fleischerring146
flickerERG312
floaters282,330,543
Index
F
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

590. 572
fluoresceinangiography
307–308,309
fluoresceindyetest53
conjunctivalexamination
63,69
cornealexamination120
focalERG312
follicles,inconjunctivitis76,
78,79
foreignbody503–505,516,
529
conjunctivitisand98
copperforeignbodies515
ironforeignbodies515
metallicforeignbodiesin
theconjunctiva111,
115
organicforeignbodies515
removal2,3,505
subtarsal505,529,530,
540
fortificationspectra402
FosterKennedysyndrome
368
foveacentralis300–302,
321
fovealreflex308
Franceschetti'ssyndrome
20,28,410
Fuchs'
blackspot339
endothelialdystrophy
148,149–150,547
heterochromiccyclitis
206
fundus308–309
abnormalchanges310
arteriosclerosis323–
325
age-relatedchanges310
examinationof304–308,
407
contactlensexamina-
tion306–307
fluorescenceangiogra-
phy307–308,309
ophthalmoscopy304–
306
photography307
ultrasonography307,
308
fundusflavimaculatus340
funnelofMartegiani280,
283
fusion464
G
galactosemiccataract179,
185
gazecenters463
lesions482,486
gazepalsy481,486
horizontal483
nystagmus495
vertical484,486
gentamicin552
geometrichoropter464
giantcellarteritis378,536,
546
giantpapillaryconjunctivi-
tis98,102
contactlenscomplica-
tions455
glandsofKrause50,51
glandsofMoll17
hordeolum38
roundcysts40,41
glandsofWolfring50,51
glandsofZeis17
adenocarcinoma47
hordeolum38
glare531
sensitivityto303–304
absorptionlensesand
450
glassblower'scataract182
glassesseeeyeglasslenses
glaucoma15,233–277,536,
543,547
absoluteglaucoma236
acuteglaucoma265–270
angleclosureglaucoma
234–235,236,265–
270
maturecataractand177
primary265–270
rubeosisiridisand215–
216,271–272
secondary212–213,
215–216,271–272
traumaand272
bullouskeratopathyand
143
cataractand177,178
childhood236,273–277,
529
classification235–237
cortisoneglaucoma271
definition233
differentialdiagnosis210–
211,215,267,275–
276
epidemiology233,251–
252,265
examinationmethods
238–250
gonioscopy238–240,
252,275
intraocularpressure
measurement15,
240–244,252,275
obliqueilluminationof
theanterior
chamber238
opticdiskophthalmos-
copy244–246,252,
275
retinalnervefiberlayer
examination250–
251
slit-lampexamination
238
visualfieldtesting246–
250
inflammatoryglaucoma
271
juvenileglaucoma236,
273–277
low-tensionglaucoma
253,254
myopiaand435
neovascularglaucoma
271,272
openangleglaucoma235,
236,251–264
iritisand211,212
primary251–264,546
secondary211,212,271
phacolyticglaucoma178,
179,271
pigmentaryglaucoma271
pseudoexfoliativeglau-
coma271
pupillarydilationand11
Index
G
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

591. 573
secondaryglaucomas133,
211–216,233,270–
273
followingchemical
injury522
treatment253–264,267–
270,273,276
indicationsfor253–
254
mechanicalindentation
ofthecornea268
medicaltherapy254–
255,256–261,267–
268
surgicaltreatment256–
264,268–270
Glifford'ssign412
glioma387
opticnerve422
glucocorticoids552,560
glycerine,glaucomatreat-
ment261
glycolipidlipidosis146
gobletcells51
degenerationinconjunc-
tivalxerosis73
densityof53
goldsalts559
Goldenhar'ssyndrome20,
104
Goldmannhemispheric
perimeter391,392–
393
Goldmann'sapplanation
tonometry240,242
gonioscopy202,238–240,
252
inchildren275
goniotomy276
gonococcalconjunctivitis
85
neonatal95,96–98
granulardystrophy148,149
granulomas80,104
eosinophilic421
Graves'disease411–413,
484,532–533,534
guanethidine553,555
glaucomatreatment259
H
Haab'sstriae275
Haemophilusaegyptiuscon-
junctivitis87
Haemophilusinfluenzaecon-
junctivitis86
haloperidol557
Hand-Schüller-Christian
disease421
Harmstangenttable492–
493
hayfever98,101
headache536–537
Helmholtzophthalmometer
442
hemangioma
choroid202
conjunctival104,106
eyelid43–44
opticnerve386
orbitalcavity420
retina356–357
hematoma
orbital419
retrobulbar512
hemorrhage535
subconjunctival72,499,
543
vitreous287–290,294,
497,513,545
herpessimplex34,538
conjunctivitis89,98
keratitis132–134
retinalinfection350,351
herpeszosterophthalmicus
35–36,539
conjunctivitis89
keratitis134
retinalinfection350,351
Hertelmirrorexoph-
thalmometer407–
408
heterochromia206
heterophoria455,466,480
highbloodpressuresee
hypertension
Hirschberg'smethod471–
472
histiocytosisX421
history3–4,497
holmiumlasercorrectionof
hyperopia151,156
homatropine,effectson
pupil225
homocystinuria,lensdis-
placementand195,
196
hordeolum38–39,538
differentialdiagnosis58,
64
horizontalgazepalsy483
Horner'ssyndrome23,24,
229–230,534,541
central230
peripheral230
Horton'sarteritis320–321
Hruby-Irvine-Gasssyn-
drome294
Hudson-Stähliline146
humanpapillomavirus106
Humphreyfieldanalyzer
391,394–395
Hurler'ssyndrome411
Hutchinson'ssign134
hyaline
degeneration69
deposits148,149
hyaloid
artery,persistent285,366
canal279,284
fossa165
membrane280
hydrophthalmia273
hydrophthalmos273,285
hydroxychloroquine558,
559
hyperemia75,76
hyperlysinemia195
hypermaturecataract177–
179
hypermetropiaseehyper-
opia
hyperopia5,433,436–440,
547,549
axial438
correction439–440
converginglenses439–
440
epikeratophakia156
holmiumlaser156
photorefractivekera-
tectomy155
latent438–439,445
newborn437
refractive438
Index
H
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

592. 574
hyperopia
strabismusand465
hyperostoses420
hypertension323
ocular253
hypertensiveretinopathy
323–325
hypertropia471
hyphema11,208,210,513
hypoglycemia544
hypoplasia,orbital410
hypopyon11,128,129,536
inuvealtractinflamma-
tion208,210
sterile536
withbacterialkeratitis
131
withmycotickeratitis135
hypotensionpapilledema
369
hypothalamus396
hypotropia471
I
ibuprofen560
idoxuridine553
illacrimation64
withconjunctivitis74,76,
78
imipramine557
impalementinjuriesofthe
orbit515–517
impressioncytology53,63
inclusionconjunctivitis60,
80,92–93
incomitant
angleofdeviation481,
488–490
strabismus459
indentationtonometry240,
241
indomethacin560
infantile
esotropia468–469
recessiveopticatrophy383
strabismicamblyopia
472–473
treatmentand
avoidance477–479
infantsseechildren;
neonate
inferiorconcha51
inflammation
anteriorchamber146
lacrimalsacseedacry-
ocystitis
orbitalseeorbitalcavity
phacogenic208
sclera158–159
uvealtract208–215
inflammatoryglaucoma271
infranuclearocularmotility
disturbance482,483
infraredradiationcataract
182
injuriesseeoculartrauma
intermittent
exophthalmos419
exotropia471,472
internal
astigmatism441,444
oculomotornervepalsy
491
internalcarotidartery396,
404
aneurysm398,399
stenosis404
internuclearocularmotility
disturbance(INO)
482,484,485
intracapsularcataract
extraction189
intraocularlens(IOL)187,
188
anisometropiacorrection
444,445
implantation191
forcongenitalcataract
194
myopiacorrection436
intraocularpressure233,
542
measurementof15,240–
244,252,451
applanationtonometry
240,242,275,435
inchildren275
palpation15,240
pneumaticnon-contact
tonometry240–241
Schiøtzindentation
tonometry240,241
tonometricselfexami-
nation243–244
twenty-fourhourpres-
surecurve243,252
seealsoglaucoma
ionizingradiationinjuries
524–525
iridectomy,peripheral268
iridocyclitis208,209
acute208–212
chronic212–213
complicatedcataractin
180,181
recurrent206
iridodialysis,blunttrauma
and510
iridodonesis169,196
iridotomy268
iris199–201,428
aniridia202–203
collarette200
color201
pigmentationanoma-
lies206
defects229,531,533
examinationmethods
201–202
melanoma216
neovascularization215–
216
prolapse129
seealsouvealtract
irisprintlenses454
iritis208
acute208–212
chronic212–213
irondeposits
conjunctiva111,114
cornea146
ironforeignbodies515
irregularastigmatism441–
442,444
irrigation53,54,55
followingchemicalinjury
518–520
inconjunctivitis82
Ishiharaplates311
isocoria220,226
withconstrictedpupils
230–231
withdilatedpupils231–
232
withnormalpupilsize
227
isoniazid558
Index
I
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

593. 575
J
jaundice115,158
Javalophthalmometer442
juvenile
glaucoma236,273–277
retinoschisis293
K
Kaposi'ssarcoma113–114
Kayser-Fleischerring146
keratectomy
photorefractive151,155
astigmatismcorrection
156
phototherapeutic151,154
keratitis127–141,529
Acanthamoeba136–137,
455
bacterial130–132
contactlenscomplica-
tions454–455
dendritic132–133
diagnosis130
diskiform133
exposure140
inGraves'disease413
mycotic134–136
neuroparalytic141
pathogenesis128–130
peripheralfurrow147–
148
predisposingfactors128
stromal133
superficialpunctate138–
139
viral132–134
herpessimplex132–
134
herpeszoster134
keratoacanthoma42,43
keratoconjunctivitis74,89,
93–95
contactlensincompati-
bilityand142
phlyctenular98,102
sicca62–63,138,528,
530
rosebengaltest52,63
ultravioletradiation-
induced523
keratoconus125–126,533,
547
keratoglobus127,533
keratomalacia148
keratopathy546
bullous143–144,148
calcificband146–147,
212
keratoplasty136
allograftrejection152–
153,154
emergency136,152
epikeratophakic151,156
lamellar151,153–154
penetrating(corneal
transplant)117–118,
143,149,151–153,
521
tectonic152
keratoscope121,442
keratotomy,radial151,155–
156
Kestenbaum'soperation494
kineticperimetry391,392–
393
Kocher'ssign412
L
lacrimal
canaliculi51
caruncle67
endoscopy53
nerve50
lacrimalgland50,51
accessorylacrimalglands
50,51
disorders64–66
dacryoadenitis64–65
tumors66
injuriesto499
nervesupply50
structureof50
lacrimalsac51
abscess57
fistula57
inflammationofsee
dacryocystitis
injuriesto499
stenosis57
tumors61
lacrimalsystem49–66
disordersoflowerlacri-
malsystem57–61,
530
canaliculitis61
dacryocystitis57–60
dysfunction62–64,138
illacrimation64
karatoconjunctivitis
sicca62–63
examinationmethods
52–57
teardrainageevalua-
tion53–57
tearformationevalua-
tion52–53
injuriesto499,500–503
teardrainage51
tearfilm50–51
seealsolacrimalgland;
nasolacrimalduct
lacrimationseeillacrimation
lagophthalmos140
lamellar
cataract183,184
keratoplasty151,153–
154
allograftrejection154
laminacribrosa157,361
Langerhans'cellprolifera-
tion421
Lang'stest477
laserinterferencevisual
acuitytesting431
lasertherapy
diabeticretinopathy317,
318
excimerlaserinsitu
keratomileusis
(LASIK)151,156
glaucoma
argonlasertrabeculo-
plasty261–262
lasercycloablation264
Nd:YAGlaseriridotomy
268,269
holmiumlasercorrec-
tionofhyperopia156
latanoprost,glaucomatreat-
ment260
latent
hyperopia438–439,445
nystagmus468
Index
L
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

594. 576
latent
strabismusseehetero-
phoria
lateralgeniculatebody359,
389
lesions400
lattice
degeneration334–335
dystrophy148
lead562
Leber'satrophy382–383
lens165–197
accommodationmecha-
nism426
aging168
developmentalanomalies
169
dislocation195–197,533
embryology165–167,279
examinationof12,168–
169
retroillumination
(Brückner'stest)168–
169
function165
metabolism167–168
positionineye165,166
shape165,166
subluxation195,197,510,
533
surgicalremovalof436
watercontent168
seealsocataract;contact
lenses;eyeglass
lenses;intraocular
lens(IOL)
lenticonus169,170
lentiglobus169
Letterer-Siwedisease421
leukemicinfiltrations421
leukocoria169,185
differentialdiagnosis286
inchildren548
inCoats'disease325,548
inretinoblastoma354,548
withpersistenthyper-
plasticprimaryvit-
reous285,286
levatorpalpebraemuscle23
elongationof540
tearsin540
levobunolol,glaucoma
treatment259
lidlag23
ligament
ofLockwood460
ofWieger280
light
adaptation303
reflex219–220
consensual220
testingof221–223
limeinjury518,521
lipodermoid,differential
diagnosis65
lipomatouspseudophakia
285
liquefactivenecrosis518
lithium557
loaloa90
loupes456
louseinfestation,eyelids37
low-tensionglaucoma253,
254
lowereyelidseeeyelids
luxationofthelens195
Lyell'ssyndrome99,103
Lymedisease351–352
lymphnodes,swollen,in
conjunctivitis76,79
lymphoma421
conjunctival113
M
macropsia335,337
maculalutea300–302,389
macular
degeneration,age-related
337–338,546,549
dystrophies148,150,340–
342
Best'svitelliformdys-
trophy313,341–342
Stargardt'sdisease311,
340–341
edema317,319
Maddox'scross475
magneticresonanceimag-
ing,orbitalcavity409
magnifyingvisionaids455–
456
mandibulofacial
dysostosis20,410
dysplasia410
mannitol,glaucomatreat-
ment261
marble-bonediseaseof
Albers-Schönberg411
MarcusGunnpupil227
Marfan'ssyndrome,lens
displacementand
195,196,197
maturecataract174,177,
186
maxillarysinus403
mechanicalinjuriesseeocu-
lartrauma
medicalhistory3
megalocornea118,124,127
meibomianglands18,50
adenocarcinoma47
hordeolum38
melanin201
deficiency206
melanocytoma385
melanoma110,112–113
choroid216,217
ciliarybody217
iris216
melanosis
congenital108,110,112–
113
conjunctival108–112
oculodermal112
oftheiris206
membranes,inconjunctivi-
tis76,78,81
membranouscataract285
meningioma387,398,407,
420–421
meningoencephalocele410
metallicforeignbodiesin
theconjunctiva111,
115
metamorphopsia335,337
metastases
orbitalcavity421
uveal217
methylalcohol562
metipranolol,glaucoma
treatment259
microcornea118,124,127
microphakia169
microphthalmos124,285,
438
micropsia335,337
microstrabismus469
Index
M
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

595. 577
midazolam556
migraine,ocular402,544,
549
Mikulicz'ssyndrome65
minimum
thresholdresolution423,
424
visualangle423
minuslenses5,435–436
seealsocontactlenses;
eyeglasslenses
miosis220,427,541
Horner'ssyndrome230
Mittendorf'sdot183,284
molluscumcontagiosum42
monocular
loupes456
testing450–451
monofocallenses447
Moraxella
conjunctivitis87
keratitis130
Morgagni'scataract178
morningglorydisk385
morphine557
mucocele417
mucormycosis417
multifocallensesseeeye-
glasslenses
Munson'ssign126
muscle
ciliaryseeciliarybody
(muscle)
dilatorpupillae200,220
extraocularmuscles459–
463
paralysis486,491
levatorpalpebrae23
elongationof540
tearsin540
oblique459–463
primaryobliquemuscle
dysfunction471,479
orbicularisoculi17,51
rectus459–462
sphincter200,220
blunttraumaand511
tarsal17
myastheniagravis23–24,
484–485,541
mycoses417
mycotic
conjunctivitis82,91,98
endophthalmitis291,292
keratitis134–136
mydriasis220,427,542
myelinatednervefibers
365–366
myogenic
ophthalmoplegia484–
486
ptosis23–24
myopia5,432–436,535,
547,549
axial434
correction435–436
contactlenses436
diverginglenses435–
436
epikeratophakia156
excimerlaserinsitu
keratomileusis
(LASIK)156
photorefractivekera-
tectomy155
radialkeratotomy155–
156
degenerative339–340
pathologic434
refractionin432
refractive434
simple434
staphyloma158,159
myopiasyndrome434–435
myopic
crescent364
sclerosis434
myositis416–417,486,532,
535
myotonicdystrophy,cata-
ractwith179–180
N
Nagelanomaloscope311
naphazoline553
nasolacrimalduct51
examinationof7
Nd:YAGlaseriridotomy268,
269
nearpoint427
testing451
nearreflex220
evaluationof223–224
nearsightednessseemyopia
necrosis
acuteretinalnecrosissyn-
drome133
liquefactive518
neonate
conjunctivitis95–98
differentialdiagnosis
96–97
dacryocystitis60,97
newbornhyperopia437
seealsochildren
neostigmine553
glaucomatreatment257
neovascularglaucoma271,
272
neovascularizationinthe
irisseerubeosisiridis
nerve
abducent,lesions482
palsy486,490–491
ciliary157,199
cranial,palsies490,531
facial,palsy140
lacrimal50
oculomotorseeoculomo-
tornerve
opticseeopticnerve
trigeminal134,141
trochlear,lesions482
palsy486,487–488,
489,491
nervusintermedius50
neurinoma420
neuritisseeopticneuritis
neurofibroma45,420
neurofibromatosis44–45,
386,420,421,422
neuroparalytickeratitis141
neuroretinalrim361
nevus
choroidal217
conjunctival108,109
flammeus44
Ota's112
newbornhyperopia437
seealsoneonate
nicotinicacid561
nodoseconjunctivitis91
nuclearcataract
acquired173,174,176
congenital183,184
nuclearocularmotilitydis-
turbance482,483
Index
N
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

596. 578
nystagmus494–495
congenital495
fixation495
formsof495
gazepalsy495
latent468,495
ocular495
optokinetic494
O
oblique
astigmatism441
entryofopticnerve363–
364
obliquemuscles459–460,
462–463
directionofpull461
nervesupply461
primaryobliquemuscle
dysfunction471
surgicalcorrection479
occlusiontherapy477–479
ochronosis112,114,158
ocular
albinism206,207
alignmentevaluation471–
472
hypertelorism409
hypertension253
migraine402,544,549
motilityseeocularmotil-
ity
myositis416–417,486,
532,535
nystagmus495
pemphigoid99,103
torticollis488
traumaseeoculartrauma
ocularmotility5,407,459–
461
acquireddisturbances
482
congenitaldisturbances
482
incavernoussinusthrom-
bosis415–416
inorbitalcellulitis414,
415
inorbitalpseudotumor
416
mechanicaldisturbances
486
myogenicdisturbances
484–486
neurogenicdisturbances
482–484
infranucleardistur-
bance482,483
internucleardistur-
bance482,484,485
nucleardisturbance
482,483
supranucleardistur-
bance482,483–484
seealsonystagmus;stra-
bismus
oculartrauma497–525
chemicalinjuries517–
522
treatment518–521
classificationofinjuries
498
colobomasand203,205
examinationmethods
497–498
indirecttrauma525
injuriesduetophysical
agents523–525
burns523–524
radiationinjuries524–
525
ultravioletkeratocon-
junctivitis523
lensdisplacementand
195,196
mechanicalinjuries498–
517
blow-outfracture507–
513,532,534,539,
540
blunttrauma(ocular
contusion)506–507,
508–513
conjunctivallaceration
499–503
eyelidinjury498–499,
500–503
foreignbodies503–505
impalementinjuriesof
theorbit515–517
open-globeinjuries
514–515
tolacrimalsystem499,
500–503
sclera158
secondaryangleclosure
glaucomaand272
traumaticcataractseecat-
aract
ocular-mucocutaneoussyn-
dromes99,102
oculoauriculovertebraldys-
plasia20,410
oculocutaneousalbinism
206
oculodermalmelanosis112
oculomandibulardysostosis
410
oculomotornerve461
aplasia23
lesions482
palsy23,228,491,541
complete228,491
external491
internal491
Onchocercavolvulus352
onchocerciasis90,352
openangleglaucomasee
glaucoma
open-globeinjuries514–
515
ophthalmia
neonatorum(neonatal
conjunctivitis)95–98
sympathetic214–215,
446
ophthalmic
artery199,404
veins199,405
ophthalmicexaminationsee
examination
ophthalmometer442,443
ophthalmoplegia481–494,
540
chronicprogressiveexter-
nal
ophthalmoplegia
(CPEO)486
diagnosis492
doublevision486–487
myogenic484–486
neurogenic482–484
total415
seealsoocularmotility
Index
O
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

597. 579
ophthalmoplegicmigraine
402
ophthalmoscope1
ophthalmoscopy13
choroid202
fundus304–306
opticdisk244–246,252
inchildren275
trauma497
vitreousbody281
opticchiasm359,389
lesions396–400
opticcup244–245,275,
308,361
myopiaand435
opticdisk13,308,360
age-relatedchanges310
bloodsupply361
coloboma385
disordersthatobscurethe
margin363–379
anteriorischemicoptic
neuropathy(AION)
374–379,536,544,
546
Bergmeister'spapilla
366
drusen366–367,372
infiltrativeedema379
myelinatednervefibers
365–366
obliqueentryofthe
opticnerve363–364
opticneuritis370–374
papilledema368–370,
371,372
pseudopapilledema
364–365,372
tilteddisk364
inhyperopia438
ophthalmoscopy244–
246,252
inchildren275
shuntvessels407
tomography246
seealsoopticnerve
opticnerve359–387,389,
396
atrophy380–383,399,
407
primary380–381,382
secondary381–382
avulsion512
bloodsupply361,362
examinationmethods
244–246,252,362–
363
swingingflashlighttest
223
glaucomaand244–246,
247
injuryto512
intracranialportion361
intraocularportion360–
361
intraorbitalportion361
myelinatednervefibers
365–366
obliqueentryof363–364
pits383–384
tumors385–387
astrocytoma385–386
glioma422
hemangioma386
intraocular385–386
melanocytoma385
retrobulbar387
seealsoopticdisk
opticneuritis370–374,415
papillitis370,372–374,
375
retrobulbar363,370,372–
373,545,547
opticneuropathyseeante-
riorischemicoptic
neuropathy(AION)
opticradiations389
lesions400
optictract359,389
lesions400,542
optokineticnystagmus494
oralcontraceptives561
orbicularisoculimuscle17,
51
orbital
apexsyndrome415
cavityseeorbitalcavity
cellulitis404,414–415,
416,532,535
differentialdiagnosis
58,64,415
treatment415
hematoma419
hypoplasia410
periostitis417
pseudotumor416
orbitalcavity403–422
bloodsupply404–405
developmentalanomalies
409–411
craniofacialdysplasia
409–410
mandibulofacialdys-
plasia410
meningoencephalocele
410
osteopathies411
examinationmethods
405–409
fathernia539
Graves'diseaseand411–
413
impalementinjuries515–
517
importanceforeye403
inflammation413–417
cavernoussinusthrom-
bosis415–416
mucocele417
mycoses417
myositis416–417
orbitalperiostitis417
orbitalpseudotumor
416
seealsoorbitalcellulitis
surgery422
tumors420–422
dermoidcyst420
epidermoidcyst420
glioma422
hemangioma420
histiocytosisX421
leukemicinfiltrations
421
lymphoma421
meningioma420–421
metastases421
neurinoma420
neurofibroma420
rhabdomyosarcoma421
vasculardisorders418–
419
hematoma419
intermittentexophthal-
mos419
pulsatingexophthalmos
418–419
organicforeignbodies515
orthopticexercises480
Index
O
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

598. 580
osmoticagents,glaucoma
treatment255,261,
267
osteopathies411
Ota'snevus112
oxycephaly409
P
pachymetry,corneal125
Paget'sdisease411
palpation,intraocularpres-
suremeasurement
15,240
palpebral
conjunctiva17,20
fissure
shorteningof21,22
widthof19,507
palsy490,531
abducentnerve486,490–
491
retractionsyndrome
490–491
accommodationimpair-
ments446–447
combinedpalsies491–
492
facialnerve140
oculomotornerve23,228,
491,541
complete228,491
trigeminalnerve,oph-
thalmicdivision141
trochlearnerve486,487–
488,489,491
seealsogazepalsy
panelD15test362
pannusformation,incon-
junctivitis76,79
panoramavision471
Panum'sarea465
panuveitis208
papillae
Bergmeister's366
contactlensincompati-
bilityand142
inconjunctivitis76,78,80
papilledema368–370,371,
372
FosterKennedysyndrome
368
hypotensionpapilledema
369
incraniostenosis409
papillitis370,372–374,375
papillomas
conjunctival106–107
lacrimalsac61
paralytic
ectropion28,29
keratitis141
ptosis23
strabismus459,481–494
parasitic
conjunctivitis90–91,98
retinaldisorders352–353
seealsospecificparasites
parastriatearea389
parasympatholyticagents
426–427
parasympathomimetic
agents427
effectsonpupil224–225
glaucomatreatment255,
256–257
paresis481,486
Parinaud'ssyndrome80,
104,231,484,486
parsplana201
examination202
vitrectomy(PPV)294,295
parsplicata201
pathologicmyopia434
patterndeviations469,471
patternERG312
penetratingkeratoplasty
117–118,143,149,
151,152–153
allograftrejection152–
153,154
penicillin553,558
perforatedcornealulcer128
pericornealinjection75
perimetryseevisualfield
testing
periostitis,orbital417
differentialdiagnosis65
peripheral
furrowkeratitis147–148
Horner'ssyndrome230
iridectomy268
retinaldegenerations
334–335
peristriatearea389
Perlia'snucleus220
perphenazine556
persistent
fetalvasculature284–287
hyaloidartery285,366
hyperplasticprimaryvit-
reous(PHPV)285–
287,548
phacodonesis169,196
phacoemulsification190,
191
phacogenicinflammation
208
phacolyticglaucoma178,
179,271
phacomatosis44
phenprocoumon561
phenylephrine553
effectsonpupil226
phenytoin557
phlyctenularkeratocon-
junctivitis98,102
Phoropter5,450
photochromiclenses450
photophobia74
inalbinism206
photopsia330
photorefractivekeratectomy
151,155
astigmatismcorrection
156
photosensitivity30
phototherapeutickera-
tectomy151,154
phthisis540
physiologicdiplopia464
physostigmine,effectson
pupil224
pigmentaryglaucoma271
pilocarpine553
effectsonpupil224
glaucomatreatment256,
268
pincushiondistortion457
pinguecula69,70,528
pituitarygland396
adenomas396,397
Placido'sdisk121,442
plasticlenses450
plicasemilunaris67
pluslenses5,439,440
seealsocontactlenses;
eyeglasslenses
Index
P
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

599. 581
pneumaticnon-contact
tonometry240–241
pneumococcalconjunctivi-
tis84
Pneumocystiscariniiinfec-
tion350
polarcataracts183
polymethylmethacrylate
(PMMA)452
post-diphtheriaaccommo-
dationpalsy446
posteriorsubcapsularcata-
ract174,176–177
postoperativeaphakia187,
438
powderburns524
preferentiallookingtest
472–473
presbyopia428
bifocalcontactlensesand
454
progressiveaddition
lensesand449
preseptalcellulitis415
primary
angleclosureglaucoma
265–270
angleofdeviation490
choroidalprocess214
obliquemuscledysfunc-
tion471
surgicalcorrection479
openangleglaucoma251–
264,546
opticnerveatrophy380–
381,382
visualarea389
vitreous279
prismloupes456
prisms455
prismbars475
prismdiopters476
strabismuscorrection474,
480,493
progressiveadditionlenses
449
proliferative
diabeticretinopathysee
diabeticretinopathy
vitreoretinopathy(PVR)
293
proptosis,withscleritis162
prostigmin,effectsonpupil
224
pseudocysts108
pseudoenophthalmos405
pseudoexfoliation
glaucomaand271
lensdisplacementand
195,196
pseudoexophthalmos405
pseudoisochromaticplates
311
pseudomembranes,incon-
junctivitis76,78
Pseudomonas
conjunctivitis86
keratitis130
pseudopapilledema364–
365,372
pseudophakia187
lipomatous285
pseudopterygium71
pseudoptosis74,540
pseudoretinitispigmentosa
344–345
pseudostrabismus481
pseudotumor,orbital416
pterygium69–71,529,533
ptosis22–24,540–541
acquired22–24
congenital22–24,540
myogenic23–24
paralytic23
sympathetic23,24
pulsatingexophthalmos
418–419
pump-leaksystem167
punctalacrimales51,530
punctalplugs63
pupil219–232
distancebetweenpupils
451
examinationmethods
221–224
swingingflashlighttest
223
testingthelightreflex
221–223
testingthenearreflex
223–224
lightreflex219–220
motordysfunction226–
232,541–542
seealsoanisocoria;iso-
coria
pharmacologicagent
effects224–226,231
size220
pupillaryblock212–213,
234,265,266,269
seealsoglaucoma
Purtscher'sretinopathy525
pyramidalcataract183
Q
quinine558,562
R
radialkeratotomy151,155–
156
radiationinjuries524–525
infraredradiationcataract
182
ultravioletkeratocon-
junctivitis523
radiography
lacrimalsystem53,57
orbitalcavity409
Recklinghausen'sdisease
44–45,386,420,421,
422
reclination189
rectusmuscles459–460,
462
directionofpull461
nervesupply461
redeye542–543
reflex
eyeclosing14,119
foveal308
light219–220
testingof221–223
near220
evaluationof223–224
wall308,310
refraction423,426
anomalies432–445
seealsoanisometropia;
astigmatism;hyper-
opia;myopia
Index
R
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

600. 582
refraction
correctionoferrorssee
contactlenses;eye-
glasslenses;prisms
testing429–431
subjectiverefraction
testingforeyeglasses
450–451
refractive
hyperopia438
myopia434
power425,426
ofeyeglasslenses447
refractometry431
regularastigmatism441,
442
relativeafferentpupillary
defect223,227
resolution423,424
seealsovisualacuity
retina299–357
correspondingpointson
464
degenerativedisorders
328–340
age-relatedmacular
degeneration337–
338
centralserouschori-
oretinopathy335–
336,549
degenerativemyopia
339–340
degenerativeretinos-
chisis333–334
peripheralretinal
degenerations334–
335
seealsoretinaldetach-
ment
dystrophies340–345
retinitispigmentosa
343–345
seealsomaculardystro-
phies
embryology299
examinationmethods
304–313
colorvisionevaluation
311–312
electrophysiologic
examinationmethods
312–314
fundusexamination
304–308
swingingflashlighttest
223
inhyperopia438
inflammatorydisease
346–353
AIDS-relateddisorders
349–350
parasiticdisorders352–
353
posterioruveitisdueto
toxoplasmosis348–
349
retinalvasculitis346–
348
retinitisinLymedisease
351–352
viralretinitis351
injuryto515
layersof299–300,301
parscaecaretinae299
parsopticaretinae299
resolvingpowerseevisual
acuity
sensitivity
toglare303–304
tolightintensity303,
428
thicknessof300
tumors353–357
astrocytoma355–356
hemangiomas356–357
retinoblastoma286,
353–355
vasculardisorders314–
327
arterioscleroticchanges
323–325
Coats'disease325–
326,548
diabeticretinopathy
314–318
hypertensivereti-
nopathy323–325
retinalarteryocclusion
320–322
retinalveinocclusion
318–320
vascularsupply302
seealsofundus;reti-
nopathy
retinal
arteries302
central302,361
occlusion320–323,
545
detachmentseeretinal
detachment
dysplasia285
nervefiberlayerexami-
nation250–251
periphlebitis215
tears537
vitreousdetachment
and282–283
vascularproliferation
293–294
vasculitis346–348
veins302
central302,361
occlusion215,272,318–
320,545
retinaldetachment328–
333,537,545,548,
549
choroidalmelanomaand
217
exudative328,329,330
inCoats'disease325–326
myopiaand435
opticnervepitsand384
persistenthyperplastic
primaryvitreousand
285
rhegmatogenous293,
328–329,330,331–
332,334
tractional328,329,330
treatment295–298
tumor-related328,329,
330
vitreousdetachmentand
282,293,328–329
retinitis537
inLymedisease351–352
pigmentosa312,343–345
viral351
cytomegalovirus350
retinoblastoma286,353–
355,548
retinochoroiditis214,292
retinohypothalamictract
389–391
Index
R
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

601. 583
retinopathy
blunttraumaand512
diabetic272,314–318,
323
nonproliferative315
proliferative215,216,
315–316,317
hypertensive323–325
ofprematurity326–327,
548
Purtscher'sretinopathy
525
toxic345–346
retinoschisis
degenerative330,333–
334
juvenile293
retinoscopy430
retractionsyndrome490–
491
retrobulbar
hematoma512
opticnervetumors387
opticneuritis363,370,
372–373,545,547
retroilluminationofthelens
(Brückner'stest)168–
169,281
rhabdomyosarcoma421
rhegmatogenousretinal
detachment293,
328–329,330,331–
332,334
rifampicin553,559
rigidcontactlenses452–
563
seealsocontactlenses
ringofZinn361
ringsaroundlightsources
542
riverblindness90
rod-conedystrophy343,
344
Rodenstockperimeter391
rods303
rosebengaltest52,69
cornealexamination120
inkeratoconjunctivitis
sicca52,63
Roth'sspots291–292
rubella
congenitalcataractand
185
retinalinfection351
rubeosisiridis215–216,
271–272,
317
Rubinstein–Taybisyndrome
410
rudimentarycoloboma364,
384
S
salicylicacid560
sarcoma
Kaposi's113–114
lacrimalsac61
Schiøtzindentation
tonometry240,241
Schirmerteartesting52
inkeratoconjunctivitis
sicca62
sclera157–163
colorchanges157–158
ectasia158
examinationmethods157
inflammations158–159
staphyloma158,159
trauma158
scleritis158–159,161–163,
542
anterior
necrotizing162,163
non-necrotizing161,
163
posterior162,163
staphylomaand158
scleromalaciaperforans161,
162
scopolamine554
effectsonpupil225
scotoma
central466
fixationpoint466
incentralserouschoriore-
tinopathy335
inglaucoma246,249,
250,253
inocularmigraine402
withopticneuritis372–
373
sebaceousglands17,18,50
seborrheicblepharitis
33–34
secondary
angleofdeviation490
cataract192,193,212–
213
inchildren194
exotropia471
glaucoma133,211–216,
233,270–273,522
opticnerveatrophy381–
382
vitreous279
sellaturcica396,399–400
senile
cataractseecataract
ectropion28,29
enophthalmos27
sunkeneye534
sensoryadaptation466
serpiginouscornealulcer
129–130,131,134–
135,536
shadowtesting430
shortsightednessseemyopia
silvercatarrh60
simple
anisocoria229
myopia434
Simpsontest485
simultaneousvision461–
464
slitlamp
anteriorchamberexami-
nation238
cornealexamination120,
121,125,135
irisexamination201–202
lensexamination12,168–
169
snailtrackdegeneration334
Snell'slaw425
snowflakecataract179
softcontactlenses453
seealsocontactlenses
spasticentropion26–27
spectaclesseeeyeglass
lenses
specularmicroscopy121–
124
spherical
aberration456–457
lenses
contactlenses453
eyeglasses447
Index
S
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

602. 584
spherophakia434
sphinctermuscle200,220
blunttraumaand511
spokephenomenon293
squamouscellcarcinoma47
staphylococcal
conjunctivitis84
hordeolum38
keratitis130
staphyloma158,159
Stargardt'sdisease311,340–
341
staticperimetry391,394–
395
Stellwag'ssign412
stenosis
craniostenosis409
dacryostenosis529
internalcarotidartery
404
lacrimalsystem53,55,
56,57
stereoscopicvision465
steroidcataract182,183
Stevens–Johnsonsyndrome
99,102
Stilling-Velhagenplates311
Stocker-Busaccaline146
strabismus459,533
latentseeheterophoria
paralytic459,481–494
prismsand455
pseudostrabismus481
seealsoconcomitantstra-
bismus;esotropia;
exotropia;ocular
motility
streptococcalconjunctivitis
84
streptomycin559
striatecortex389
stromal
cornealdystrophy148–
150
keratitis133
subcapsularcataract174
posterior174,176–177
subconjunctivalhemor-
rhage72,543
traumaand499
subluxationofthelens195,
197,510,533
sulfiteoxidasedeficiency
195
sulfonamide554,559
superficialpunctateker-
atitis138–139
suppression466
amblyopiasecondaryto
466–467
supranuclearocularmotility
disturbance482,483–
484
supraorbitalartery404
supratrochlearartery404
sweatglands17,50
swingingflashlighttest223
symblepharon521,522
sympathetic
heterochromia206
ophthalmia214–215,446
ptosis23,24
sympatholyticagents,glau-
comatreatment255,
259
sympathomimeticagents
effectsonpupil225–226
glaucomatreatment255,
257–258
synchysis282
scintillans287
synechiae211–212
T
Taeniasolium353
tarsal
glands18
muscle17
plate17
tarsorrhaphy,lateral140
tearbreak-uptime(TBUT)
52
inkeratoconjunctivitis
sicca62
teardrainage51
evaluationof53–57
tearfilm50–51
evaluationof52–53
inkeratoconjunctivitis
sicca62,63
significanceforcornea
119
tearingseeepiphora
tectonickeratoplasty152
telangiectasia325,326
telescopes456
Telleracuitycard473
temporalarteritis320,378–
379,536,546
tenonitis416
Tenon'scapsule160,416,
460
Tensilontest485
tertiaryvitreous279
tetanycataract180
tetracycline554,559
thioridazine556
thyroiddysfunction411–
413
tickinfestation,eyelids37
tilted-disksyndrome364
timolol551
glaucomatreatment259
Tolosa-Huntsyndrome416
tonicpupil228,446,447
tonometry240–244
applanationtonometry
240,242,275,435
pneumaticnon-contact
tonometry240–241
Schiøtzindentation
tonometry240,241
tonometricself-examina-
tion243–244
toriclenses
contactlenses453
eyeglasses447
total
astigmatism440
ophthalmoplegia415
toxic
cataract182
conjunctivitis76–78,97
retinopathy345–346
Toxocaracanis352,353,355
Toxocaracati352,353
toxoplasmosis348–349
AIDS-relatedretinalinfec-
tion350
trabecularnetwork157,
234–235,273
argonlasertrabeculo-
plasty261–262
trabeculotomy276,277
trachoma88,93
Index
T
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

603. 585
tractionalretinaldetach-
ment328,329,330
traumaseeoculartrauma
traumaticcataractseecata-
ract
TreacherCollins'syndrome
410
trichiasis27,30,530
tricyclicantidepressants
557
trifluridine553
trifocallenses449
trigeminalnerve134,141
palsyofophthalmicdivi-
sion141
trigeminalneuralgia,
blepharospasmand
30
trilateralretinoblastoma
354
trochlearnervelesions482
palsy486,487–488,489,
491
tropia6
tropicamide,effectson
pupil225
tuberoussclerosis386
tumor-relatedretinal
detachment328,329,
330
tumors
choroid202
conjunctivalseeconjunc-
tiva
eyelidsseeeyelids
lacrimalgland66
lacrimalsac61
opticnerve385–387
orbitalcavity420–422
retina353–357
uvealtract216–217
visualpathway396–398
tunicavasculosalentis279,
280
Tyndalleffect202,208,210
U
Uhthoffsymptom372
ulcers,corneal
perforated128
serpiginous129–130,131,
134–135,536
seealsokeratitis
ulcus
rodens46
terebans46
ultrasonography
A-modescan409
B-modescan408
fundusexamination307,
308
orbitalcavityexamination
408–409
vitreousbody281
ultravioletkeratocon-
junctivitis523
uncovertest480
unilateral
aphakia445
strabismus473
unilateralcovertest473–
474
uppereyelidseeeyelids
uvealtract199–217
developmentalanomalies
202–205
aniridia202–203
coloboma203–205
examinationmethods
201–202
inflammation208–215
neurovascularsupply199,
200
pigmentationanomalies
206–207
tumors216–217
metastases217
seealsochoroid;ciliary
body;iris
uveitis208–215,547
causesof209
posterior543
duetotoxoplasmosis
348–349
withscleritis162
V
Vpatterndeviation469,471
varicellazostervirussee
herpeszosteroph-
thalmicus
vascularpigmentedlayer
seeuvealtract
vasculitis,retinal346–348
vein
angular405
ophthalmic199,405
retinal302
central302,361
occlusion215,272,318–
320,545
vortex157,199
venouspulse13
vernalconjunctivitis98,101
vertexdistance451
vertical
deviations471
dissociated471
diplopia491
gazepalsy484,486
verticillata,corneal145–
146
vidarabine553
videokeratoscopy121,122–
123,442
viral
conjunctivitis76–79,81,
89,93–95
keratitis132–134
retinitis351
visual
agnosia401
areas461
primaryvisualarea389
cortex389
lesions400
visualacuity
corrected423
decreased544–547
testing4–5
inthepresenceof
opacifications431
uncorrected423
visualevokedpotential
(VEP)313,363
visualfield391
testing246–250,253,
391–394
confrontationfieldtest-
ing14
inretinalarteryocclu-
sion321
kineticperimetry391,
392–393
Index
V
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.

604. 586
visualfield
testing
orbitalcavitydisorders
408
staticperimetry391,
394–395
visualpathway389–402
disorders394–402
chiasmallesions396–
400
prechiasmallesions394
retrochiasmallesions
400–402
examinationmethods
391–394
seealsoopticnerve
vitaminA561
deficiency
conjunctivalxerosis
72–74,148
tearfilmand62
vitaminD561
vitrectomy294–298,331
vitreoretinaldystrophies293
vitreous
amyloidosis287
hemorrhage287–290,
294,497,513,545
vitreousbody279–298
abnormalchanges284–293
abnormalopacities287
endophthalmitis290–
292
persistentfetalvascula-
ture284–287
vitreoretinaldystro-
phies293
vitreoushemorrhage
287–290
vitritis290–292
abscess291
agingchanges282–284
synchysis282
seealsovitreous
detachment
attachmentsof280–281
cataractsurgeryand294
composition279
embryology279,280
examinationmethods281
importanceforeye279
neurovascularsupply281
retinaldetachmentand
293
retinalvascularprolifera-
tionand293–294
retinalvasculitisand346–
347
surgicaltreatment294–
298
vitreousdetachment282–
284,537,543
blunttraumaand510
retinaldetachmentand
282,293,328–329
vitritis290–292
Vogt-Koyanagi-Haradasyn-
drome330
vonGraefe'ssign412
vonHippeldisease386
vonHippel-Lindaudisease
356,386
vortexveins157,199
W
Waardenburgsyndrome23
Wagner'sdisease293
wallreflex308,310
warfarin561
watchglassbandage29
waxypalloropticatrophy
383
Weill-Marchesanisyndrome
169
lensdisplacementand
195,196
Weiss'ring283,284
Wesselykeratometer124
Wilson'sdisease146
with-the-ruleastigmatism
441
X
xanthelasma40–42
xerosis,conjunctival72–74,
148
Z
zonularcataract183
zonule201
zonulefibers165,195,279
accommodationmecha-
nism426
Index
Z
Lang, Ophthalmology © 2000 Thieme
All rights reserved. Usage subject to terms and conditions of license.