Central Serous Retinopathy

Central Serous Retinopathy is known to be an idiopathic, sporadic, self-limiting collection of fluid at posterior pole which causes mild to moderate visual loss.

1. CSRCSR
Dr.Shah-Noor Hassan FCPS,FRCSDr.Shah-Noor Hassan FCPS,FRCS
Vitreo-Retina ConsultantVitreo-Retina Consultant
Bangladesh Eye Hospital & instituteBangladesh Eye Hospital & institute

2. History
1866: von Graeffe: Relapsing Central Leutic
Retinitis
1955: Bennet : Central Serous
Retinopathy (CSR)
1965: Maumenee: Leak from RPE
1967: Gass : Idiopathic Central Serous
Choroidopathy (ICSC)
Current name : Central Serous
Chorioretionopathy (CSC)

3. Definition
Idiopathic, sporadic, self-
limiting
Collection of fluid at posterior
pole
Acute, localised
neurosensory detachment
Young male
Mild to moderate visual loss
Single or few leaks on FA

4. Asymmetrical bilateral chronic disease with
Acute exacerbation during periods of:
Stress
Steroid intake
High BP spikes
Unknown factors
Recent Concept

5. Demography
Sex: ♂ > ♀ (9:1)
Age: 25-45 yrs
Increased incidence in females & elders
Rarely familial
Bilaterality:
10-25% symptomatic
45% on FA
More in elders

7. Classification
Klein (1953):
Central retinopathy
Central chorioretinopathy
Central choriopathy (juvenile disciform macular
degeneration)
Maumenee (1960) & Wessing (1977):
Type I : Subsensory fld.: 94%
Type II : Sub-RPE fluid: 3%
Intermediate: Both areas: 3%

8. Classification
Nadel (1979):
Unilateral CSR
 Uniocular RPE changes
 Binocular RPE changes
Bilateral CSR
Lodato & Brancato (1988):
Simple (55%)
Multifocal (30%)
Chronic (15%)

9. Classification
Patnaik (1983):
Type I: Unilateral, single leak, benign, idiopathic
Type II: Bilateral, Multifocal PEDs, recurrent,
granulomatous choroiditis (TB/Toxo)
Castro-Correia (1992) & Bujarborua (2001):
Single episode Þ Resolves
Recurrent episodes Þ Resolve
Recurrent episodes Þ Chronicity
Single episode Þ Chronicity

10. Classification
Commonly accepted:
Typical CSR
 Classic CSR
Chronic CSR
 Atypical CSR
 Decompensated RPE
 Diffuse retinal pigment epitheliopathy
 Zweng & Little (1977)

11. Chronic CSR
Atypical CSR, Decompensated RPE,
Diffuse retinal pigment epitheliopathy (@ 10%)
Widespread pigment alteration of RPE
Long-standing SRF (>6mths)
Chronic steroid intake after organ transplantation
Asian descent
6mths
Apr Oct

12. Pathogenesis – Theories
Gass’ hypothesis (1967)
Piccolino’s hypothesis (1981)
Spitznas’ hypothesis (1986)
Yannuzzi’s hypothesis (1986)
Marmor’s hypothesis (1988)
Guyer’s hypothesis (1994)
Ciardella’s hypothesis (2001)

13. Gass’ Hypothesis (1967)
Hyperpermeability of choriocapillaris Þ
serous exudation (sub-RPE or sub-sensory)
Stress Þ Physiologic decompensation at
sites of congenital structural defects

14. Piccolino’s Hypothesis (1981)
RPEpithelitis or choroidal perfusion defect Þ
RPE cell junction damage
Strong adhesion force Þ No detachment
Strong choriocapillaris
hydrostatic pressure Þ
Serous detachment

15. Spitznas’ Hypothesis (1986)
RPE cells secrete ions around rods & cones
Choroidal fluid gets dragged subretinally
Adjacent RPE cells overwork to remove fluid
Seen as scarring
after resolution

16. Behaviour
Yannuzzi’s Hypothesis (1986)
Multifactorial concept Þ Host specificity
Related to balance of:
Genetic endowment
Environment
Behavioural pattern
Adrenergic alteration Þ
Choriocapillaris damage
RPE cell degeneration
BRB breakdown
Environment Genetics

17. Pathogenesis – Theories
Chronic choriocapillaris disturbance
Failure of RPE cell pump
Pooling in sub-RPE space
Sub-retinal fluid accumulation

18. Pathogenesis – Theories
Raised choroidal hydrostatic pressure
ICGA:
 Choroidal vascular hyperpermeability
 Delayed filling
Also in non-leaky areas & in fellow eyes
Infectious Theory:
Tuberculosis
Toxoplasmosis
Viral theory

19. Pathogenesis
Increased stress
Type-A personality
Hypochondriacs & hysterics
Increased cortisol levels
Cushing’s disease
Pregnancy
Steroid intake
Increased catecholamine levels
Hypertension

20. Pathogenesis – Theories
Role of catecholamines
Vasoconstriction
Altered choroidal blow flow
Role of corticosteroids
¯se nitric oxide (vasodilator)
­se capillary fragility
Delayed RPE healing
Reversed RPE polarity
­se catecholamine response

21. Stages
I = RPE inflammation
II = Increase in inter-RPE cell spaces
III = Crenation of RPE
IV = Degeneration & atrophy
V = Recurrance
VI = Neovascularisation

22. Symptoms
Minor blurring of vision (6/6 to 6/60)
Metamorphopsia, Micropsia
Dyschromatopsia (67%)
Blue-yellow defect, red-shift
Poor contrast sensitivity
Hypermetropisation
Central scotoma
Photopsia

23. Signs – Sensory Retina
Well-defined transparent blister
Halo of light reflex
Yellowish foveal
discolouration
Increased
xanthophyll visibility

24. Signs – Sensory Retina
Yellowish-white sub-retinal deposits (@10%)
Proteinaceous deposits
s/o inactive disease
“Bath-tub” effect
SR & sub-RPE fibrin
Intra or SR lipid
(leopard-spot pattern)
Cloudy & grayish SRF
Punctate hemorrhage

25. Signs – RPE
Serous PED:
Single or multiple (<1/4DD)
Bilateral
Pigment migration
RPE atrophy

26. Signs – RD
Peripheral dependent bullous RD with
connecting atrophic RPE tracts
Flask, teardrop, dumbbell, hourglass pattern
Telangiectasia & CNP
Pigment migration & deposits
Gass: “Pseudo-RP-like CSR”

29. Signs – CNVM
Incidence: 4% in chronic RPEpitheliopathy
CNVM in CSR are Type II membranes
Types of CNVM:
Diffuse & irregular leaks
Typical membrane
Post laser therapy
Cause:
Damage to RPE-Bruch’s membrane complex
Ischemia of choriocapillaris
1 mth

30. Signs – Other Complications
Cystoid macular edema
Intracytoplasmic edema of Müller cells Þ Cell
death & degeneration Þ Cystoid spaces
Detachment, SRF & fibrin Þ Toxic to retina Þ
Ischemia Þ Retinal vascular leakage
Ring-like “bull’s eye” RPE window defect
Long-standing CSR
Choriocapillaris atrophy

31. CSR in Women
More common than previously thought
Age: 40-60 yrs
Unilateral in 90%
Pregnancy (3
rd
trimester), SLE
Role of catecholamines, corticosteroids,
estrogen, prostacyclin

32. Systemic Associations
Pregnancy
End-stage renal disease
Organ transplantation
Systemic steroid intake
Choroidal ischemia
SLE, PAN, Wegener’s granulomatosis
DIC, TTP, Toxemia of pregnancy

33. Natural Course
Spontaneous resolution (80%)
within 3 mths with VA > 6/9
Recurrences:
30-50% cases
 50% within 1 year
10% have > 2 episodes
Severe & permanent visual loss (10%):
Persistent detachment
CNVM, CME, RPE atrophy
Day 0
1 mth
3 mths

34. Imaging Techniques
Fundus Fluorescein Angiography (FFA)
Indocyanin green Angiography (ICGA)
Optical Coherence Tomography (OCT)

35. FFA
≥ 1 hyperfluorescent leaks
from RPE
Pattern:
Ink-blot
Smoke-stack
Point (< 1/5 DD)
Combinations
No definite leak

36. FFA – Pattern
Ink-blot (85%):
Even spread in all directions
Smoke-stack (10%):
Shimizu & Tobari (1971)
Rises superiorly Þ Expands laterally
 Mushroom-like
 Umbrella-like
No definite leak (5%):
Hyperfluorescent patches

37. FFA – Ink-blot

38. FFA – Smoke-stackSmoke-stack

39. FFA – Smoke-stack
Larger CSR
Theories
Jet-like projection of fluid from RPE defect
Diffusion & convection rather than net fluid influx
Increased concentration of proteins in SRF
Low density fluorescein rises by convection
Not all smoke-stacks form umbrellas
Direction changes with head position
Even downward spread has been seen

40. FFA – No Definite Leak
Leaking point has healed
Lies outside macular area
In presence of choroidal
tumour
Associated with ONH pit
07:19

41. FFA
Location of leak:
1mm-wide ring-like zone adjacent to fovea
 No rods in fovea Þ Weaker adhesions
10% lie in the foveal area
30% lie within papillomacular bundle
SNQ > INQ > STQ > ITQ
Window defects in uninvolved areas
Choroidal hyper-permeability
Mottled hyperfluorescence of RPE tracts

42. Parafoveal CSRParafoveal CSR

43. Atrophic Tract

44. FFA
PED:
Detected on FFA if missed clinically
Early or delayed filling
Puncture or blow-out at margin of PED

45. PED

46. ICGA
ICGA & FFA leaks correspond
in 80% cases
Choroidal vascular hyper-
permeability
Unifying feature of all CSR types
Best seen in mid-phase
Localised in inner choroid

47. ICGA

48. ICGA
Late phase:
Centrifugally enlarging hyperfluor. patches
Silhouetting of the larger choroidal vessels
RPE atrophic areas:
Hypofluor. areas with surrounding hyperfluor.
PEDs:
Early diffuse hyperfluor.
Late hypofluor. with hyperfluor. ring
PEDs in ARMD do not stain with ICG

49. FA + ICGA

50. OCT
Role:
Confirms diagnosis
Quantifies detachment
Observes progress or resolution
Reduces need for FFA
Other Findings:
Intra-retinal edema
Cystic changes
No co-relation with colour vision abnormalities

51. Role of OCT
1 month later
6/18
6/9

52. New OCTs
3-D view
Deeper view

54. OCT
B-Scan
C-Scan

55. mf-ERG & f-ERG
Standard ERG is normal
mf-ERG & f-ERG:
Deterioration of oscillatory potential & b-waves
more than a-waves
Functional disturbance seen in all retinal layers
Depressed signals from entire posterior pole in
both eyes

56. Contrast Sensitivity
Acute stage:
Deficient at mid & high spatial frequencies
No co-relation with:
Visual acuity
Duration of disease
Final picture of macula
Similar findings innormal fellow eye also
? Role in early diagnosis

57. Photostress Recovery (PSRT)
Types:
Conventional PSRT (acuity chart) Þ Macular
Pattern PSRT (pattern-VEP) Þ Macular
Pupil PSRT (pupillometer) Þ Central 30
0

Increased recovery time even upto 1 hour
Microperimeter used with SLO:
Reduced initial sensitivity change
Unaffected areas show normal recovery

58. Others
Rod dysfunction test:
Dark adaptation
Static perimetry
Choroidal pulsation measurement:
Laser interferometry
With fundus camera

59. Differental Diagnosis
Infectious & inflammatory diseases
Tumours
Vascular disorders
Optic nerve pit
ARMD
IPCV
CME
Inferior RRD

60. Infectious & Inflammatory D.
POHS:
Peripheral “histo-spots”
Peripapillary atrophy
Arcuate striae in mid-periphery
Idiopathic CNV in young:
CNVM on FA
Sub-retinal hemorrhage
Unilateral
No spontaneous resolution

61. Infectious & Inflammatory D.
Harada’s disease:
Vitritis
Optic disc hyperemia
Systemic associations
Response to anti-
inflammatory Rx
Toxoplasmosis:
Focal retinitis involving
outer half
Serology & skin test

62. Infectious & Inflammatory D.
Sympathetic Ophthalmia:
Intraocular inflammation
Dalen-Fuchs nodules
(cellular RPE detachments)
h/o trauma to fellow eye
Posterior Scleritis:
Scleral thickening
Vitreous cells
Pain on ocular movements
“T-sign” on USG

63. Choroidal Tumours
Melanoma, Hemangioma, Metastasis,
Osteoma, Leukemic infiltrates
May rarely be confused clinically with large PEDs
USG & FFA will diagnose

64. Vascular disorders
Collagen vascular disorders (SLE, PAN)
Fibrinoid necrosis of choroidal vessels
Chronic intake of systemic steroids
Malignant HT, Toxemia of pregnancy, DIC
Acute multifocal occlusion of choroidal vessels
Necrosis of overlying RPE (Elschnig’s spots)

65. Optic nerve pit
Schisis-like separation
of macular layers
Outer-layer detachment:
No obvious connection with optic nerve pit
Relatively opaque
Inner-layer detachment:
Communicates with optic nerve pit
Transparent

66. Optic nerve pit
FFA: no leak, no filling
OCT: identifies schisis

67. ARMD
CSR may be seen > 50 yrs
CSR may show secondary CNV
FFA: diffuse hyperfluorescence
Ill-defined CNV or
Diffuse ‘ooze’ of CSR
ICGA:
CSR: multifocal early hyperfluorescence that
fades in late phase
ARMD: shows late hyperfluorescence

68. IPCV
Isolated macular variant
Polypoidal lesions resemble
PEDs
ICG Angiography:
Small-caliber vascular network
Multiple polypoidal lesions

69. Others
Inferior Rhegmatogenous RD
Presence of break
Dome shaped configuration
Non-shifting fluid
Photo-toxicity
CME
Role of steroids

70. Treatment
Aim:
To speed up recovery
Improve vision quality
Prevent recurrences
Methods:
Conservative approach
Medical management
Laser treatment

71. Conservative approach
Reduce stress
Avoid caffeine
Less alcohol
Avoid steroids

72. Medical management
St John's Wort
Acycloguanosine
Procaine HCl (AntiCort)
Picogenol
Beta blockers
Diazepam based tranquillizers
Imipramine
Indomethacin
Alpha helical CRF
Acetyl-L-carnitine
RU-486
Bilberry
Eyebright
Bayberry bark
Capsicum leaves
Anti-VEGFs
Long list of
medications tried
Finally, response is
to:“TIME”!

73. Medical management
Ketoconazole & mifepristone
Role of AKT
IV Anti-VEGFs

74. 1 month: 6/9 (P)
Pre-Injection: 6/18
?PEDs!

75. Laser Treatment
Advantages:
Shortens the course of disease
Reduces morbidity
May reduce the recurrence rate
Disadvantages:
No effect on final visual acuity
Possible complications

76. Gass Recomendations
Wait 4 mths: Primary episode
Wait 6 mths: Leak <1/4
th
DD from fovea
Wait 1 mth: Recurrence, with good prior
recovery
Prompt Rx:
Primary episode > 4 mths old
Recurrence, with poor prior visual recovery
Occupational demands
 Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment. 3rd ed.
St. Louis: CV Mosby; 1987. p 46-59.

77. Early Treatment
Affects people in prime of life
Anxiety & Depression
Reduced BCVA if resolution takes > 4 mths
Impending foveal involvement
Monocular patients
Severe & Complicated CSR

78. Laser Treatment – Types
Direct: Over the RPE leak
Locally debrides RPE cells
Replaced by healthy cells
Indirect: Rim of detachment
RPE breakdown allows fluid to pass into choroid
Inflammatory material blocks leakage point
Was advised in past for leaks within the FAZ or
on papillomacular bundle
May be still used when no leak is seen
“Sham” or “Photofomentation”

79. Laser Treatment – Method
Generally avoided within FAZ
Diode laser may be used within FAZ
Spot size = 100-200μ
Duration = 0.1-0.2 sec
Power = 100-400 mW (diode > argon)
Light grey burn achieved
Re-treatment >1mth if leak persists

80. Laser Treatment – Prognosis
Anatomic resolution:
2 wks in typical CSR
6 wks if turbid SRF
Visual Recovery:
4-12 weeks
Recurrence:
Generally adjacent to the original site of leakage
Reactivation (inadequate laser)
New leak

81. Laser Treatment
Complications
Secondary CNV (2-5%)
Inadvertent damage to fovea
Scotoma
Slow enlargement of area of RPE atrophy
Long-term outcome:
> 85% show extremely good prognosis
<12% show marked visual impairment

82. ICGA-guided PDT
Has shown anatomic & functional
improvement
Mechanism:
Damage to choriocapillaris endothelium
Causes reduction in hyper-permeability of
choriocapillaris
Reperfusion within 2-3 weeks
Indication:
Diffuse decompensation of RPE