Central Serous Retinopathy

CENTRAL SEROUS
RETINOPATHY
By Dr. ANKUSH DEEPAK WEGINWAR

INTRODUCTION
Central serous chorioretinopathy (CSC /CSCR) most popularly known as “CSR (Central
Serous Retinopathy)” is a sporadic disease of unknown etiology
Characterized by blister like serous detachment of neurosensory retina and retinal
pigment epithelium (RPE) in the posterior pole of the eye.
The term “central” refers to the form of the disease causing visual symptoms due to the
presence of serous detachments in the macular area
• Usually unilateral
• May be associated with pigment epithelial detachment (PED)
• Relative preservation of visual function despite prolonged separation of neural retina
from the retinal pigment epithelium

This condition was first described by
Von Graefe in 1866 as
“Relapsing Central Luetic Retinitis”
1955 – Bennett – “Central Serous
Retinopathy”
Synonyms:
Central Serous Retinopathy
Central Serous Pigment Epitheliopathy
Central Serous Retinitis

Epidemiology
Incidence 1 in 22,000
o Accounts for about 5% of the cases attending the retina specialist
o It is now considered to be one of the ten most common diseases of the posterior
segment of the eye
Age: 20 – 50 years
o Patients diagnosed at 50 years or older are found to have bilateral disease,
demonstrate a decreased male predominance (2.6:1), and show more diffuse RPE
changes
Gender: Male > female 6:1
Race: Whites and Asians>blacks

Causative Factors
Emotional stress
Type A personality
Physical Strains
Pregnancy
People engaged in visually demanding work
Helicobacter pylori infection has been reported to be associated with CSC
Smoking,
Alcohol consumption and allergic respiratory diseases
Obstructive sleep apnea
Hyperopic eye
Vasoconstrictive agents, endogenous hypercortisolism, Systemic corticosteroids, SLE,
Hypertension

RPE damaged via
immunologic infectious
circulatory and neuronal
mechanism
RPE secretsions in
chorio retinal direction
towards retina
Choroidal fluid gets
attracted into this area
Strong flow disrupts the
diffusion barrier in this
area
PATHOPHYSIOLOGY

Psychogenic,Pre
gnancy,Type A
personality,raise
d cortisols
Adrenergic
reaction causes
damage to the
choricapillaries
Hyperpermeabil
ity of
choriocapillaries
RPE cell
degeneration
Secondary
changes in RPE
causes leaks
Serous retinal
detachment

Mechanism
o The presence of fibrin in the detached space itself indicates that there is sufficient
alteration in the permeability of the choriocapillaries and the RPE
o As the choriocapillaris are fenestrated, the interstitial fluid within the choroid can be
expected to have a large range of molecules
o Normally resorption of fluid and protein molecules within the choroid primarily occurs
by free exchange through these fenestrated vessels and some excess amount is also
drained through the sclera
o In acute CSC, the amount of fluid and solutes are definitely more than what the RPE
cells normally can cope with.

o The increase in interstitial hydrostatic pressure in the choroid drives the fluid towards
the retina and leads to the development of PED
o Microrips at the junction of attached and detached RPE or along the decompensated
RPE cells that cause fluid leak into the subretinal space. The micro rents usually occur in
the parafoveal region
o Once the detachment occurs, it would continue to enlarge until sufficient normal RPE is
exposed to the exuded fluid which would drain it out at a rate equal to the inflow rate
through the leak
o Thus, the detached space in CSC has a dynamic environment into which, and from
which, there is a continuous flux of water, ions, and protein

Role of Corticosteroids and the Catecholamines
It produce CSC by following mechanisms:
o Corticosteroids may cause increased capillary fragility and hyperpermeability
o Affect the production of nitric oxide, prostaglandins, and free radicals, which may affect the
regulation of blood flow in the choroids
o Inhibit formation of collagen, which is the main component of Bruch’s membrane
o Alter ion water transport of epithelia

o May directly damage the RPE cells or their tight junctions;
o May delay any reparative process in damaged RPE cells , by suppressing the synthesis of
extracellular matrix components and inhibiting fibroblastic activity
o Can influence the transcription and expression of adrenergic receptor genes and regulate
adrenergic receptors
o Stimulation of adrenergic receptors within the choroidal circulation results in release of
secondary messengers (e.g. cAMP ) and this may produce the vascular or RPE changes that
result in CSC
o Increase catecholamine mediated vasoconstriction

Role of VEGF
o Vascular endothelial growth factor (VEGF) is produced by damaged retinal and choroidal
cells when abnormal perfusion causes ischemia.
o By uncoupling endothelial cell-to cell junctions, VEGF causes vascular permeability and
edema
o The affected RPE cells start leaking fluid that would move towards the retina as there is
less resistance in this direction

 Acute CSCR: self-resolving SRD (sub retinal detachment) within 4 months from the
onset of symptoms
 Non-resolving CSCR (or “persistent” CSCR): acute CSCR with duration of SRD longer
than 4 months after onset of symptoms, often associated with elongated
photoreceptor outer segments on SD-OCT.
 Recurrent CSCR: episode of acute CSCR following a previous episode with complete
SRD resolution
 Chronic CSCR (formerly “diffuse retinal epitheliopathy”): chronic chorioretinopathy
with widespread RPE decompensation with or without SRD, associated or not with
active leakage sites
 Inactive CSCR: patients with history of acute CSCR but without SRD at the time of
evaluation

Blurred vision
Positive Scotoma
Micropsia
Metamorphsia
Imapired Colour vision
Impared Night vision
Entoptics
Light flashes
Photophobia
Impaired depth perception
Headache
Chromatopsia
Cyanopsia
SYMPTOMS

 Presentation:
 Unilateral blurred vision with a relative scotoma in the central visual field
 Unilateral metamorphopsia and/or micropsia
 Patients with extrafoveal involvement may be asymptomatic
 Visual acuity:
 VA ranges from 6/5 to 6/60, usually 6/9 – 6/12
 Acquired hyperopia

SIGNS
 Relative hyperopia
 Positive photostress test
 Fundus
o A round to oval sensory retinal detachment is present at the posterior pole
o In some small PED within the serous detachment may be evident
o Absent foveal reflex
o Small dot like deposits in the posterior of retina which is believed to be the
precipitates of plasma proteins including fibrin

Fundus photograph of CSC with fibrin
deposit
Fundus photograph of CSC with subretinal
precipitates

CSC in Women
o There is a significant difference in the male to female ratio in CSC cases.
o Cortisol responses to psychologic stress was found to be more in male than in female
o CSC in otherwise healthy women tends to occur at an older age than men.

o Conditions commonlyc associated with CSC in women are
 Pregnancy
 Systemic lupus erythematosus.
o Elevation of catecholamine, corticosteroids, estrogen and other hormone levels during
pregnancy may be responsible for it
o Increased levels of prostacyclin during the second and third trimester may be another
important factor.
Prostacyclin is a potent vasodilator and inhibitor of platelet aggregation

Atypical Presentation of CSC
 Bullous or Severe variant of CSC
 Cystoid macular edema
 Ring retinal pigment epithelial window defect
 Leopard spot pattern
 Secondary choroidal vascularization
 Retinal telangiectasia with lipid deposition
 CSC simulating pattern dystrophy
 CSC with retinitis pigmentosa
 Choroidal folds without any evidence of underlying scleritis and with episcleritis

Natural History
CSC is a self-limited disease, but runs a very unpredictable course.
However, the natural course of the disease seems to have 4 distinct patterns
(1) CSC with a single resolving episode
(2) Recurrent resolving CSC –recovery is complete before the recurrence
(3) Recurrent chronic CSC –recurrence before full recovery
(4) Chronic CSC - following a single episode without any appreciable recovery

o The symptoms / sensory retinal detachment (SRD) persist beyond the usual period of
recovery, that is, 6 months.
o In majority (80 to 90%) spontaneous resolution occurs within 1 to 6 months and in 20% it
lingers for more than this period 6-12 months
o But even after recovery, the quality of vision is not the same as before in all the cases.
o The improvement of vision may continue for more than 1 year following resolution.
Poor visual outcome may also be due to development of cystic degeneration of the macula
or cystoid macular edema or due to development of a macular choroidal neovascular
membrane

Bilaterality of CSC
o CSC was once considered as a unilateral disease, but now the literature documents
5-35% incidence of bilateral CSC.
o This tendency increases with age.
According to
 Gass , approximately 10 to 25% develop symptomatic detachment in the opposite eye.
 Bujarborua et al found the overall prevalence of bilateral pathology of CSC to be 44.54%
at the initial visit using FA as the only investigative procedure.
 Yannuzzi et al (1979) reported that 10% of the patients of their series had a concomitant
acute SRD in the fellow eye and additional 12% developed the same during the followup
period ranging from 2 to 8 years.
 They also observed RPE atrophy and pigmentary changes in about 80% of the fellow
eyes by the end of their period of observation.

DIAGNOSIS
 Distance & Near Visual Acuity testing
 Retinoscopy
 Amsler Grid
 Photostress test/Dazzling time/Re adaptation time
 Ophthalmoscopy
Special investigations
 FFA
 ICG
 OCT
 FFA critical to detect the extent of the retinal abnormalities and to exclude the
presence of other ocular pathology

FFA
Fluorescein angiography in CSC reveals various types of leaks at the level of RPE
depending on the stage of the disease. These leaks are seen in about 95% of all
cases of all types of CSC
Smoke-stack appearance:
• Small hyperfluorescent spot in the early phase due to leakage of dye through the RPE
• Fluorescein passes into the subretinal space and ascends vertically until the upper border, like a
smoke-stack, in the late venous phase
• The dye then spreads laterally, taking on a “mushroom” or “umbrella” configuration until entire
area of detachment is filled

Ink-blot appearance:
• Seen in 93% cases
• Leakage point/s with uniform dye filling is appreciated
• Most common location – upper nasal quadrant
• Least common location – lower temporal quadrant
 In majority of cases (60%), the point leak that appear in the initial phase of the angiography slowly and
symmetrically spread in all sides to about l/4th DD (inkblot type)
 This type of leak probably suggests late phase of the active disease process and the active stage itself may be
of variable duration

Point Leaks:
o Some leaks do not expand much. These are the so called minimally expanding point leak,
whose expansion is always less than l/5th disc diameter in size.
o These types of leaks are usually found in subtle serous detachment of the retina.
o Besides these - diffuse leak, decompensatory leak, window defects of various sizes and
PEDs are visible on FA.

ICG
o ICGA is one of the most important investigations in CSC because it demonstrates the
choroidal vascular abnormalities and can act as a guide to treatments such as
photodynamic therapy.
o Common findings in patients with CSC are multi focal areas of hyper fluorescence in the
early and mid phases of the study, which then fade in the late phase of the study
a) Depicting FA findings and b) Depicting the ICGA findings of
the same fundus showing multiple hyperpermeable areas

OCT
o OCT can detect detachments that remained undetected in FA.
o It can also detect subretinal deposits like fibrin and subretinal precipitates
a) Sub sensory fluid accumulation in CSC b) Detected shallow SRD along with PED

Fundus picture and OCT image showing subretinal fibrin deposit
 Recently introduced Ultra-high resolution OCT provides an axial resolution of approximately 3 μm that
allows improved visualization of the external limiting membrane (ELM), photoreceptor inner and outer
segment junctions (IS / OS) and identification of pathologic changes in the microstructure of the
photoreceptor layer.
 Fourier-domain OCT (FD-OCT) provides a three dimensional view of these structures

Fundus Autofluorescence (FAF) in CSC
o This technique is designed to document the presence of lipofuscin in the RPE
o Lipofuscin is a mixture of proteins, lipids, and small chromatophores generated as
by-products of the retinoid cycle.
o The accumulation of lipofuscin in the RPE is due to impaired or overwhelmed lysosomal
activity, leading to incompletely
digested cellular debris
o The presence of FAF is thought to correspond
to the accumulation or dispersion of lipofuscin
in the subretinal space or RPE

FAF typically shows hypofluorescence at the leakage point and over the area of neurosensory
detachment due to blockage by subretinal fluid
The subretinal yellow dots observed clinically might demonstrate hyperfluorescence
In chronic-recurrent CSC, hyperfluorescence is common in areas of residual neurosensory
detachment.
Other tests
Multifocal ERG has been used to identify focal regions of decreased retinal function, even in
asymptomatic or clinically inactive eyes.
Microperimetry has also shown that, despite clinical resolution of CSR, there is lower retinal
sensitivity in the macula even once visual acuity returned to 20/20

Macular hole
Optic pit
Malignant Hypertension
AMD
Idiopathic uveal effusion syndrome
Ocular histoplasmosis syndrome
Vogt-Koyanagi-Harada’s disease
Pathological myopia
Choroidal tumors
Differential Diagnosis

Treatment
 Observation
 Medical
 Surgical

Management
Non-Drug Treatment
 Reduce stress levels: Biofeedback, meditation, taking a philosophical approach to adversity
 Yoga
 Avoid caffeine containing drinks ( caffeine stimulatesnthe pituitary gland and increases the
cortisol level )
 Avoid excess alcohol consumption.
 Try to avoid “ cortisone treatments ” as far as possible (Monitor cortisol level if possible)
 Avoid unnecessary stress like disease (any form of infection increases cortisol levels),
excessive exercise, crash diets, jet lag, pain, lack of sleep etc
It has been recommended that if symptoms persist for more than 3 months, further active
treatments should be considered

Medical Treatment
Ketoconazole:
 Anti-fungal drug of Imidazole group
 Adrenocorticoid antagonist
 Lowers the endogenous cortisol level in CSC cases after 4 weeks treatment with 600 mg
per day dose of the drug
 The median visual acuity, lesionnheight and the greatest linear dimension remained
unchanged during the month of treatment.
 It seems therapeutic effect would require longer time.

Acetazolamide (Diamox)
 Also shown to be effective
 Given in a tapering dose for 6 weeks has been shown to reduce the time for subjective
and objective CSR resolution
 But it can not prevent recurrences and
 Final visual recovery did not differ much from the control group.
 Moreover, this drug is having considerable side affects like paresthesias, nervousness,
and gastric upset and allergies

Bevacizumab (Avastin):
 Antibodyvto VEGF (0.05 ml/1.25 mg intravitreal)
 Reduces the choroidal hyperpermeability
 Reverse the changes.
 Originally it was tried in chronic CSC cases.
 Now it has been tried in acute cases with encouraging result.

Beta-blockers
 Metoprolol, Nadolol, and Trimepranol have been used with controversial results.
 One recent double blind randomized controlled clinical trial from Iran found that
Propanolol (20mg twice daily) cuts short the period of recovery and thereby eliminates
the need for laser therapy.
 But it does not have any effect on visual recovery and its effect on recurrence rate
could not be commented as it had a short term follow-up

Laser Photocoagulation in CSC
Light to moderate intensity applications of all modalities of laser like ruby, argon,
krypton, diode and dye laser photocoagulation help in resolution of the detachment in
CSC
Accelerates the resolution of the detachment
lowers the recurrence rate
MOA:
1. Beam destroys the cluster of diseased pigment epithelium cells, thus stopping the
secretion of fluid beneath the neurosensory retina
2. Resulting scar helps to transport fluid back into the choriocapillaris

Indications:
 Persistence of serous detachment beyond 3-4 months
 Recurrences in eyes with visual deficit from previous episodes
 Presence of permanent visual deficit from previous episodes in the fellow eye
 Development of chronic signs such as cystic changes in the neurosensory retina or
widespread RPE abnormalities
 Occupational or other patient needs that require prompt restoration of vision or
stereopsis

Technique
2 – 3 low to moderate intensity burns applied to the leakage site
spot size of 200µm, for 0.1 seconds
to produce mild graying of the RPE Decreases of duration of detachment.

 Advantages
Decreased duration
Decreased recurrence
 Complications
Choroidal neovascularization
Central scotoma
 Should be avoided if the leak is within 500mm from the centre of the foveal avascular
zone
 Careful follow up required as 2 – 5% of eyes treated with photocoagulation develop
CNV

Photodynamic Therapy (PDT) in CSC
 Use of verteporfin and PDT was first reported in 2003 in the setting of CSC.
 Yannuzziet al89 used ICG angiography to first identify areas of choroidal hyperpermeability, which were
subsequently treated by PDT.
 Most papers describe resolution of subretinal fluid within 1 month of treatment.
Mechanism
o Postulated to be caused by short-term choriocapillaris hypoperfusion and long-term choroidal vascular
Remodeling
The application of conventional PDT in CSC can result
 RPE atrophy
 Choroidal ischemia
 Secondary choroidal neovascularization
To enhance the efficacy of PDT in treating CSC while minimizingits side effects, the dose of verteporfin has
been reduced and the time interval between infusion and laser application has also been

ICG mediated photothrombosis is a technique using a low-intensity laser
combined with ICG dye infusion to treat focal areas of hyperpermeability in the
choroid.

Submacular Surgery in CSC
The current treatment options for the treatment of subfoveal or juxtafoveal choroidal
neovascularization in CSC do not preserve macular function.
 Cooper et al did submacular surgery for removal of choroidal neovascularization in 10
cases.
 It was observed that the eyes, in which the central retinal RPE was preserved had the
best visual outcome and the best cases for surgery appeared to be the eyes in which the
neovascularization lied anterior to the RPE.
 Removal of such membranes could leave the underlying foveal pigment epithelium
intact.
 As the choroidal neovascular membrane develops in CSC from a focal rather than diffuse
abnormality of RPE, it offers the potential for preservation of central retinal pigment
epithelium

Course and Outcome of CSR
 Self-limiting and 90% of the cases will show spontaneous recovery within a few months
 CSC recurs in about 50% of the patients within the first year
 A history of psychiatric illness is associated with a higher rate of recurrence
 Small proportion of patients develop RPE atrophy, CNV development (in up to 6% of
patients), and transformation into PCV (Polypoidal choroidal vasculopathy)
 Patients whose VA recovered might be left with residual symptoms such as
metamorphopsia, scotoma, and reduced contrast sensitivity.

REFERENCES
 Stephen Ryan text book of RETINA 3rd edn
 Retinal and Vitreoretinal diseases and Surgery by
Samuel Boyd
 Kanski 8th edn
 Parsons Diseases of the Eye 22nd edn

Central Serous Retinopathy

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