Choroidal detachment -Nov 2017

Choroidal detachment & Supra choroidal detachment

1. Choroidal Effusions and Detachment.. Dr.Sameera Vitreo -Retina MMJEI , Hubli

2. contents Definition Anatomy Types Causes Diagnosis Management Prevention

3. Synonyms … First described by von Graefe in 1858 Ciliochoroidal detachment Suprachoroidal effusion Supraciliary effusion the accumulation of fluid in the suprachoroidal space

4. Choroid -functions Vasculature : major supply for the outer retina Choroidal blood flow : cool and warm the retina Contains secretory cells : modulation of vascularization and in growth of the sclera Dramatic changes in choroidal thickness : move the retina forward and back , bringing the photoreceptors into the plane of focus Important role in the drainage of the aqueous humor from the anterior chamber, via the uveoscleral pathway

5. Anatomy Four layers Haller's layer - outermost layer consisting of larger diameter blood vessels Sattler's layer  - layer of medium diameter blood vessels Choriocapillaris  - layer of capillaries Bruch's membrane  (synonyms: Lamina basalis, Complexus basalis, Lamina vitra ) - innermost layer

6. normal eye Choroid lies in close approximation to the sclera Suprachoroidal space  the space between the choroid and the sclera : a potential space Approximately :10 microlitre fluid seen

7. Abnormal … . Fluid originates in the choriocapillaries and seeps across the spongy choroidal tissue into the potential suprachoroidal space Severe cases : fluid escape into the subretinal space  secondary serous retinal detachment

8. TYPES.. Clinically : depending on the characteristics of the fluid contained between the tissue planes  SEROUS  HEMORRHAGIC

9. Pathophysiology Choroidal effusions represent tissue edema Starling’s hypothesis  elucidates the balance of hydrostatic and osmotic gradients between the choroidal capillaries and interstitial space of the eye  intraocular pressure (IOP) :hydrostatic pressure in the interstitial space  process that shifts flow from the choroidal capillaries into the interstitium may lead to an effusion Under normal circumstances : IOP > Pressure in suprachoroidal space > atmospheric pressure

10. pathoPHYsiology Imbalance between fluid production and fluid reabsorption Increased transmural pressure - forces pumping fluid into the extravascular space forces that cause fluid to be reabsorbed Increase in the permeability of the blood vessels : exudation of serum Combination of these mechanisms ( postoperative choroidal detachments) Idiopathic uveal effusion syndrome  uveal effusion with no obvious etiology Accumulation of hemorrhage in the suprachoroidal space  hemorrhagic choroidal detachment, e.g., after trauma or surgery   Globe Hypotony Inflammation

11. pathoPHYsiology  INFLAMMATION – increases colloid leakage into suprachoroidal space Another theory suggests that a tear in the ciliary body allows aqueous humor to flow into the suprachoroidal space* * Fuchs E. Ablosung der Aderhaut nach staaroperation . Albrecht von Graefes Arch Ophthalmol . 1900;51:199-224

12. Epidemiology Incidence following surgery varies between 0.05-6% No racial / sexual predilection Hemorrhagic detachments are seen more often in elderly patients

13. Risk factors-glaucoma Antimetabolites—particularly mitomycin C (MMC)—during trabeculectomy   (5- 33% ) Latanoprost use in eyes with prior cataract extraction Increased risk of subclinical choroidal effusions after laser iridotomy   Increased association of choroidal effusion with nanophthalmos and with Sturge -Weber syndrome Sturge -Weber syndrome : higher risk in the presence of choroidal hemangiomas

14. HISTORY Serous choroidal detachments : painless decrease in vision History  intraocular surgery  trauma  anti-glaucoma medications  panretinal photocoagulation (PRP)/ cryotherapy Hemorrhagic detachments : sudden onset of severe pain / loss of vision  Valsalva maneuver (straining at stools, coughing, sneezing)  Anticoagulants and aspirin may facilitate bleeding

15. Other symptoms Small, peripheral effusions : asymptomatic Large effusions : refractive changes from anterior displacement of the lens-iris diaphragm and resultant myopia Absolute scotoma at the site of effusion

16. ANTERIOR SEGMENT FINDINGS Inflammatory conditions: Congestion /AC cells/ KPs Associated with increased resistance to venous drainage : signs of vascular congestion  dilated episcleral veins /blood in Schlemm’s canal Shallow anterior chamber: Nanophthalmic eyes Massive choroidal effusions Ciliochoroidal effusions

17. IOP : normal/ low / elevated Low IOPs  S erous choroidal detachments ( reduced aqueous humor production/ retinal detachment) Elevated IOPs  Hemorrhagic CDs  AV malformations  Idiopathic uveal effusion syndrome Angle closure glaucoma may occur with ciliochoroidal detachments of any etiology* *Myelodysplastic syndromes, Ig A nephropathy ANTERIOR SEGMENT FINDINGS

18. POSTERIOR SEGMENT FINDINGS Detachment typically begins in the periphery  an ultrabiomicroscopy (UBM) exam More fluid  t he choroid detaches in a distinct, mound-like fashion Mounds demarcated by the fibrous attachments that correspond to the sites of the vortex veins Very large detachments  the lobes can contact in the visual axis : appositional or ‘kissing choroidals ’ Annular : involving the circumference of the globe 360°

19. POSTERIOR SEGMENT FINDINGS FFA : areas of delayed choroidal perfusion followed by late hyperfluorescence . exudative retinal detachments  shifting subretinal fluid (because of the high protein : more than 26.5 g/ dL ) chronic retinal detachments associated with choroidal effusions, the chronic changes such as a diffuse spotty pigmentation called leopard skin spots choroid is always thickened in choroidal effusions

20. POSTERIOR SEGMENT FINDINGS Inflammatory choroidal effusions : vitreous cells Visualization of the ora serrata without scleral depression :pre equatorial choroidal detachment Ciliochoroidal edema/detachment without evidence of intraocular surgery or trauma ??? Neoplastic/vascular/inflammatory cause

21. POSTERIOR SEGMENT FINDINGS Chronic effusions  blood-ocular barrier break down at the RPE  Exudative RD / Shifting SRF Depigmentation and multifocal hyperplasia of the RPE  leopard spots in the fundus Linear areas of RPE hypertrophy and hyperplasia  Verhoeffs lines

22. INVESTIGATIONS- B scan Most important imaging modality Distincts hemorrhagic versus serous effusions Hemorrhagic choroidal effusions (acoustically empty) vs tumors Delineate the extent of the choroidal detachment Assess the thickness of the choroid in early uveal effusion syndrome Evaluate for posterior scleral thickening / retrobulbar edema around the optic nerve  T-sign in posterior scleritis

23. INVESTIGATIONS- B scan 1) TOPOGRAPHIC • Smooth dome or flat elevation • No disc insertion • Inserts at Ora or ciliary body 2) QUANTITATIVE • Steeply rising ,thick , double peaked spike • 100% amplitude 3)KINETIC • Mild to none after movement

24. INVESTIGATIONS-UBM Assess anterior choroid/ciliary body Any fluid / anterior rotation of the ciliary body associated with angle closure glaucoma Small effusions at ciliary body  Detachment at scleral spur

25. Classification

26. management Conservative Postoperative serous choroidal detachments :resolve on their own within days Cycloplegics and corticosteroids Excessive leakage from a wound or after glaucoma filtering surgery  pressure patching / glue /bandage contact lens

27. management Oral fluids can be given to increase aqueous humor flow Acetazolamide : absorption of suprachoroidal fluid ?? paradoxical

28. prevention During surgery  Good scleral flap ,sutures  judicious use of antimetabolites  meticulous closure of the conjunctiva  High-risk patients    Prophylactic sclerotomies at the time of glaucoma filtration surgery

29. Drugs Topiramate Warfarin Sulfonamides Tetracycline Diuretics Aspirin

30. Uveal effusion syndrome Described for the first time in 1963 by RJ Brockhurst , Schepens Primary underlying cause :congenital anomaly of the sclera / the vortex veins Three types Type 1 : Nanophthalmic eyes  small eyeball (average axial length 16 mm) ,high hypermetropic (average +16 diopters) Type 2 : Non- nanophthalmic eyes with clinically abnormal sclera  normal eyeball size (average axial length 21 mm) , small refractive error Type 3 : Non- nanophthalmic eyes with clinically normal sclera

31. PATHOGENESIS OF UVEAL EFFUSION SYNDROME Suprachoroidal space : no lymphatics Protein escape from the choriocapillaris : osmotic fluid retention Healthy eyes : proteins can diffuse across the sclera   Form of mucopolysaccharidosis -deposition of dermatan sulfates in sclera Egress impaired in Uveal effusion eyes

32. UVEAL EFFUSION SYNDROME U/L or B/L loss of vision  ciliochoroidal and subsequent retinal detachments Metamorphopsia and scotomas caused by shallow macular detachments Bilateral involvement in over 60% of cases

33. UVEAL EFFUSION SYNDROME A/S : episcleral vascular dilation/ blood in Schlemm’s canal No inflammatory signs IOP : usually normal Few vitreous cells +/- CD, Exudative RD Angiography : leopard-skin appearance of hyperfluorescence and hypofluorescence OCT : focal thickening of the RPE corresponding to the areas of leopard spots

34. NANOPHTHALMOS Small eye (less than 21 mm in axial length) but with an otherwise normal structure Bilateral; both males and females Syndromic /Non -syndromic Thickened abnormal sclera  disruption of the normal arrangement of scleral collagen bundles and a ‘fraying’ of the collagen fibrils Abnormal formation and packing of the collagen bundles Hamper the escape of aqueous by the uveo -scleral route Outflow obstruction of the vortex veins

35. NANOPHTHALMOS High hypermetropes Shallow AC Angle closure glaucoma Fundus: Foveal hypoplasia/Rudimentary foveal avascular zone/Foveal schisis Choroidal effusions/Exudative RD

36. Differential diagnosis Choroidal melanoma Multifocal, atypical central serous retinopathy Vogt- Koyanagi -Harada disease Posterior scleritis Hypertension Metastasis

37. Management Small peripheral uveal effusions :Observe Fluid involves or threatens the fovea :  vortex vein decompression    full-thickness scleral decompression ( Gass et al) Sclerectomy / Sclerotomy Uveal effusion with out Nanophthalmos : ? Vitrectomy – Quick retinal reattachment

38. Management Pre-operative B-scan: identify the area of maximal effusion 360° peritomy / dissection of quadrants Isolation of extraocular muscles: care not to avulse vortex veins Creation of a lamellar sclerectomy : approximately ~2 2 mm 50% scleral thickness, with or without vortex vein decompression May or may not perform full thickness sclerotomies in sclerectomy bed to drain choroidal fluid Perform in all four quadrants Drain subretinal fluid in severe cases at a separate sclerotomy site Restore IOP with saline or gas/air injection Consider sub-Tenon’s steroids at the end of surgery  

39. ViDEO

40. Other Effusions HUNTER’S SYNDROME systemic mucopolysaccharidosis : short stature, joint abnormalities and mental retardation Circular peripheral choroidal detachments /exudative RDs ARTERIOVENOUS FISTULAS proptosis (often pulsatile), episcleral venous dilation palsy of the cranial nerves in the cavernous sinus and the orbit

41. INflammations Scleritis : generalized inflammation of the choroid  leakage from the choriocapillaris localized or an annular choroidal detachment Females Severe pain, Ant scleritis +/-, Raised IOP Disc oedema,Macular oedema , T-sign Mx : Steroids

42. Suprachoroidal hemorrhage

43. Suprachoroidal hemorrhage (SCH) One of the most dreaded complications Could result in total visual loss and phthisis

44. Suprachoroidal hemorrhage (SCH) Limited hemorrhage Massive hemorrhage Appositional (“kissing”). Expulsive

45. Mechanism Impeding vortex vein outflow - RBH,Retrobulbar anaesthesia,Scleral buckle,Pressure during surgery Fluctuation in intra ocular fluid dynamics and pressure  Hypotony – serous effusion – tension on ciliary vessels* – rupture -sudden compression and decompression events Rarely – spontaneous *long posterior cilairy arteries

46. Risk factors Systemic : Advanced age, Arteriosclerosis,DM,HT , Anticoagulation,IHD Ocular : Glaucoma, Increased axial length, Previous surgery (Vitrectomy),Previous laser photocoagulation, Aphakia , Uveitis,Recent trauma Intra operative : High IOP,High Myopia, Open sky procedures, Intra operative tachycardia, Sudden decrease of IOP, Vitreous loss, Buckling

47. Intra operative symptoms and signs Sudden onset of severe intra operative pain Shallowing of anterior chamber/excessive iris movement or prolapse Forward movement of lens and vitreous Darkening/loss of red reflex Excessive bleeding of conjunctiva/ episclera Vitreous hemorrhage/RD Expulsion of intra ocular contents Tachycardia

48. Delayed non expulsive suprachoroidal hemorrhage Decreased vision/Pain Shallow anterior chamber with mild cells and flare Smooth orange- brown elevation of the retina and choroid B scan: Smooth dome shaped highly reflective solid appearing mass

49. Fundus findings Anterior to the equator Extends in an annular fashion Postequatorial unilobulated or multilobulated.

50. Fundus findings Localised choroidal hemorrhage  choroidal melanoma (DDs) pigmented choroidal nevus subretinal pigment epithelial hamartoma Acoustic hollowness

51. Serous CD Vs Hemorrhagic CD Low IOP Transiiluminates No pain Pre equatorial Resolves with in 3 weeks Good visual acuity High IOP No transillumination Painful More voluminous posteriorly Resolves 6-25 days; resorbs ~ 4 weeks to months Poor visual acuity

52. Serous CD Vs Hemorrhagic CD

53. Management: delayed hemorrhage Limited hemorrhage  Conservative: Cycloplegics and topical steroids Usually resolves with in 1-2 months Delayed massive hemorrhage  Systemic corticosteroids with serial B scans ( liquefaction of hemorrhage - low reflective mobile echoes )  Surgery :7-14 days post hemorrhage

54. When to operate on a choroidal hemorrhage … . Massive hemorrhage with severe pain Kissing choroidals Corneo lenticular touch Persistently high IOP Persistently flat AC Massive hemorrhage under macula/ subretinal /vitreous cavity Retinal/Vitreous incarceration Preferably after liquefaction

55. Intra operative management Direct digital pressure on open wounds Rapid wound closure Post PK: Immediate cardinal sutures to be put Reform AC with viscoelastic Posterior sclerotomy done only if wound apposition not possible Post operatively : Control IOP/Inflammation/Pain

56. Secondary management Associted RD/VH VR traction Dislocated lens/fragments :treated accordingly PFCL injected to flatten the retina and drain the hemorrhage Silicon oil/Long acting gas for a long term tamponade

57. Choroidal hemorrhage in trauma Intraocular structural damage + High likelihood of RD and associated PVR B-scan : hemorrhage tend to be more diffuse and less elevated

58. Supra choroidal drainage Optimal drainage site : highest point of choroidal elevation, as determined by echography or ophthalmoscopy Sclerotomies : radial and created with a cut – down incision Isolation of the rectus muscle using silk ties/ traction sutures : good exposure of the sclera

59. drainage Conjunctival peritomy  2 to 3 mm radial incision made in the sclera : 3 to 4 mm posterior to the limbus Incision deepened until the suprachoroidal space is entered  fluid is released Fluid : clear and yellowish in serous CDs dark red with blood clots in hemorrhagic CDs Incision’s edges may be pulled apart by forceps or cautery to facilitate fluid egress Sclerotomy site left open with closure of overlying conjunctiva Eye should be kept pressurized with injection of BSS / viscoelastic in AC / ACmaintainer

60. VIDEO

61. Surgical technique Trocar in tangential angle inserted into the suprachoroidal space Place the anterior chamber infusion, the choroidal fluid/hemorrhage drains spontaneously through the cannulas Pars plana vitrectomy +/-

62. Word to the wise … . Non- valved cannulas ; preferable to drain the infero -temporal quadrant first Serous CD : one drainage site Hemorrhagic CD : more than one drainage site may be needed “Dry tap” Hemorrhagic CD  clot blocking the cannula Turn the cannula side to side /gentle scleral depression with a cotton swab Serous CD  intra-scleral cannula position / cannula is too anterior and is blocked by choroid, retina or vitreous (if so, sclerotomy placement at 7 mm from limbus) Avoid 3 and 9 o’clock meridians : Traumatise ciliary nerve

63. Outcome Good prognosis Delayed/limited hemorrhage seen after a week Hemorrhage after cataract surgery Good visual acuity Poor prognosis RD Hemorrhage all quadrants Retinal incarceration Extention to posterior post pole

64. The suprachoroidal space: from potential space to a space with potential Suprachoroidal buckling  illuminated catheter inserted into the SCS and navigated to any desired location  long-lasting hyaluronic acid filler can be injected to create internal choroidal indentation  Vitrectomy +/-  myopic tractional maculopathies, RD

65. SCS as a route for drug delivery bypasses the internal limiting membrane barrier and outer blood-retina-barrier preferred route for drug delivery targeting RPE Triamcinolone (Uveitis) “SAPPHIRE” : SCS steroid vs Aflibercept

66. Interesting facts … . Choroid supply the inner retina (Guinea pigs) as well..!! In humans, the choroid is approximately 200 μm thick at birth and decreases to about 80 μm by age 90  Choroids are thick at night … .. Thin at day.. ! Teleosts   : choroidal gland  supplemental oxygen carrier

67. References

68. Thank you …

69. Drainage of choroidal hemorrhage