Central serous chorioretinopathy

PACHYCHOROID SPECTRUM DISORDERS-
CENTRAL SEROUS CHORIORETINOPATHY
BY
DR ANJALI MAHESHWARI
RESIDENT, OPHTHALMOLOGY

INTRODUCTION
 ‘Pachy’ means ‘thick’ and so ‘pachychoroid’ means thick
choroid.
 Pachychoroid is not a disease but a phenotype characterized
by specific choroidal changes.
 Typical features include:
 Focal or diffuse areas of choriocapillaries
 Sattler’s layer attenuation
 Dilated outer choroidal vessels (Haller’s layer)
 Overlying RPE dysfunction

 The following entities are included in pachychoroid
spectrum:
1. Central serous chorioretinopathy (CSCR)
2. Pachychoroid pigment epitheliopathy (PPE)
3. Pachychoroid neovasculopathy (PNV)
4. Polypoidal choroidal vasculopathy (PCV)
5. Peripapillary pachychoroid syndrome (PPS)
6. Focal choroidal excavation (FCE)

CLINICAL FEATURES
 There are certain common clinical features of the
pachychoroid disorders:
 Fundus appearance –
 Normal fundus tessellations are decreased with or
without additional pigmentary alteration.
 Optical coherence tomography
 Choroidal morphology and the sclero-choroidal junction
are studied either using SD-OCT with EDI or SS-OCT

 Optical coherence tomography
 Choroidal morphology and the sclero-choroidal junction
are studied either using SD-OCT with EDI or SS-OCT
 Choroidal thickening may be focal or diffuse and foveal or
extra-foveal.
 Sub foveal choroidal thickness (SFCT) >300um as
suggestive of a thick choroid.
 In case of extrafoveal choroidal thickening there will be
normal SFCT with area of interest at least greater by
50um as compared to SFCT

 Pathologically dilated outer choroidal vessels in haller’s layer
are seen as hyporeflective lumen with large diameter
 œFocal or diffuse attenuation of choriocapillaries and
intermediate size choroidal vessels.

 ICGA
 Better delineated choroidal vessels
 Presence of choroidal filling defects and hyperfluroscence
 Early phase shows delayed arterial filling
 Mid- late phase shows punctate hyperfluroscence i.e
choroidal vascular hyperpermeability (CVH)
 CVH is mainly due to leakage from dysfunctional outer
choroidal vessels or choriocapillaries

CENTRAL SEROUS CHORIORETINOPATHY

INTRODUCTION
 Idiopathic chorioretinal disorder characterized by serous
detachment of neurosensory retina (NSR) and or retinal
pigment epithelium (RPE) in macular region.
 Young male – 30-40 year affected
 Male : Female – 3:1 to 10:1
 Characterized by accumulation of transparent fluid at the
posterior pole of the fundus.

HISTORY
 In 1866, von Graefe first described disease of the macula
characterized by recurrent serous macular detachment and
named it recurrent central retinitis
 In 1955, Bennet applied the term central serous retinopathy.
 Maumenee observed – In fluorescein angioscopy :
detachment of the macula resulted from a leak at the level
of the retinal pigment epithelium (RPE)
 In 1967, Gass described of the pathogenesis and clinical
features of idiopathic central serous choroidopathy.
 Since the disease involve both the choroid and the retina,
currently accepted name is central serous chorioretinopathy
(CSC).

 There are three types of CSC :
 Typical or classic CSC-
 more common type
 younger patients
 acute localized detachment of the retina with mild to
moderate loss of visual acuity
 associated with one or a few focal leaks seen during
fluorescein angiography

 Red-free photograph of the left eye shows serous neurosensory
macular detachment (white arrows).
 Fluorescein angiography demonstrates pinpoint areas of
hyperfluorescence in the central macula leading to the
characteristic smokestack configuration in the late phase.

 Diffuse retinal pigment epitheliopathy or decompensated
RPE or chronic CSC –
 Widespread alteration of pigmentation of the RPE related
to chronic presence of shallow subretinal fluid
 Common in patients with CSC associated with chronic
corticosteroid usage.

 Fluorescein angiogram reveals diffuse decompensation of the
retinal pigment epithelium.
 Mid-phase indocyanine green (ICG) angiography illustrates
multiple patchy areas of hyperfluorescence in the left eye

 CSC which causes bullous retinal detachments usually
located inferiorly:
 associated with shifting fluid
 more frequently observed after organ transplantation
 patients using corticosteroids
 patients of Asian descent

PATHOGENESIS
 Fluorescein angiography demonstrates- one or multiple
focal sites of leakage resulting in detachment of the RPE
and/or neurosensory retina in cases of active CSC
 With the cessation of leaks, detachment regress
 suggesting that fluorescein findings represent fluid
coming from the choroid
 Which escapes into the subretinal space through a defect
in the tight junctions between cells of the RPE

 RPE dysfunction theory :
Areas of RPE leaks on FA where dye has diffused into sub-
retinal space.
 Alteration of RPE polarity
Focus of RPE cells loose polarity
pump fluid in reverse direction and cause NSR detachment

 Choroidal vasculature hyperpermeability
Sympathomimmetics and corticosteroids
( Increase NO ,PGs )
Alter choroidal permeability and increase hydrostatic
pressure in choroid
 PED
 RPE barrier disruption
 Abnormal fluid under NSR

 Choroidal hyperpermeability with congestion of the
choriocapillaris along with exudation of protein and fluid.
 Retinal pigment epithelium (RPE) pump decompensation occurs
over time with the formation of a pigment epithelial detachment

 Eventually, RPE defect develops, leading to leakage into the
subretinal space.
 This leads to elevation of the neurosensory retina and a
neurosensory retinal detachment

RISK FACTORS AND ASSOCIATIONS
 Type A personality
 Emotional stress
 Systemic HTN
 GERD
 Organ transplantation
 Tobacco and alcohol use
 Organ transplantation
 Pregnancy
 Goodpasture syndrome
 Wegner’s granulomatosis
 Drugs
 Corticosteroids
 Psychopharmalogical drugs
 MDMA
 Over the counter
sympathomimmetics

 Photograph of right eye of a
26-year-old female who
developed bilateral bullous
neurosensory detachments
during childbirth
complicated by
preeclampsia.
 After resolution of the
neurosensory detachment
in the right eye there was a
triangular-shaped area of
subretinal fibrinous
exudation consistent with
occlusion of a short
posterior ciliary artery.

SYMPTOMS
 Unilater metamorphsia
 Unilateral blurred vision
 Micropsia
 Impaired dark adaptation
 Colour desaturation
 Delayed retinal recovery time to bright light
 Temporary hyperopic shift

EXAMINATION FINDINGS
 Neurosensory retina
 biomicroscopic examination with a fundus contact lens
reveals :
 Detachment of the neurosensory retina- appears as
well-delineated transparent blister at posterior pole.
 Normal foveal light reflex is absent and replaced by halo
of light reflex delimiting the elevated area.
 Detached neurosensory retina is usually transparent and
of normal thickness.
 Yellowish discoloration of the fovea is visible caused by
increased visibility of retinal xanthophyll.

 Retinal pigment epithelium
 OCT reveals serous PED under the neurosensory elevation
more often than previously recognized
 Two or more PEDs - usually located superior to the
neurosensory detachment since gravity forces the fluid
inferiorly.
 PED has a round or oval shape.
Pink halo surrounding the PED - by shallow separation of
the retina at the edge of the PED.
 Long-standing and recurrent PEDs may present with
pigment migration or atrophy.

Optical coherence tomography of pigment epithelium
detachment (white arrows in the red-free photograph) with
central serous chorioretinopathy confirms presence of localized
elevation consistent with serous pigment epithelial detachment

 Subretinal fluid is usually transparent, allowing clear
visualization of the underlying RPE and choroidal details
 Histopathologic studies shows- presence of subretinal and
subpigment epithelial fibrin.
 With increasing concentrations, fibrin molecules polymerize
to form oval yellow or gray membranes, causing the
subretinal fluid to opacify.

 Conditions predisposing to subretinal fibrin exudation:
 large and multiple PEDs
 chronic and recurrent disease
 pregnancy
 systemic administration of corticosteroids,
 organ transplant,
 Diabete
 male gender
Subretinal lipid may also occur, particularly in patients with
chronic CSC

 Peripheral retinal detachments and RPE atrophic tracts
 subretinal fluid gravitates inferiorly to form a
neurosensory detachment in a flask, teardrop, dumbbell, or
hourglass pattern and is very shallow.
RPE under the chronic retinal detachment develops
atrophic changes that consist of both atrophy and
perivascular pigment deposits or bone spicules
 The retina undergoes changes :
 pigment migration
 capillary dilation (telangiectasia) proximally
 capillary nonperfusion (ischemia) distally in area of
detached retina
.

 Red-free photograph of the right eye reveals pigmentary changes
temporal to the fovea.
 Red-free photograph of the left eye shows a well-circumscribed
neurosensory detachment of the macula with two areas of focal
pigment epithelial detachment (PED) (arrows)

 Mid-phase fluorescein angiography study of the fellow eye shows
window defect hyperfluorescence corresponding to the atrophic
retinal pigment epithelial tract extending inferiorly

 Color photograph shows detachment of the neurosensory retina
at the macula with lipid exudation.
 Color photograph reveals changes in the retinal pigment
epithelium (RPE) at the posterior pole.

 Red-free photograph
confirms neurosensory
detachment, lipid exudation,
and an atrophic RPE tract
(arrows)

 Complications of chronic CSC :
Cystoid retinal changes in the areas of chronic detachment
Cystoid macular edema
 Subretinal lipid deposition
 Choriocapillaris atrophy
 CNV

 Fluorescence angiogram reveals diffuse RPE decompensation
which is typical of CSC.
 Optical coherence tomography shows multiple cystic retinal
spaces in the foveal area consistent with cystoid macular
degeneration

IMAGING TECHNIQUES
 Fluorescein angiography
 Typical angiographic finding - presence of one or several
hyperfluorescent leaks in the level of the
Dye spreads symmetrically to all sides, slowly and evenly
staining the subretinal detachment.
Fluorescein does not stain the retina beyond the edges of
the detachment.

 In some cases dye rises within the neurosensory
detachment in a “smokestack” pattern and expands laterally
in a mushroom-like or umbrella-like fashion at the upper
limit of the detachment.
 This pattern is due to increased concentration of protein
within the subretinal fluid.
 In rare cases, no definite leakage point, but area of
diffuse hyperfluorescence is found.

 Red-free photograph reveals a large neurosensory detachment at
the macula.
 Early fluorescein angiogram shows a small pinpoint leak which
progresses

 Small pinpoint leak which progresses in mid and late phase of
the study into a typical smokestack pattern

 Exudates usually extend into or beyond the fovea
 Most frequently located in a 1-mm-wide ring-like zone
immediately adjacent to the fovea
 Leakage points are most commonly found in the superonasal
quadrant of the posterior pole, followed by inferonasal >
superotemporal > inferotemporal
 Absence of rod cells fovea results in weaker adhesion
between the neurosensory retina and the RPE – cause both
predominance of leakage points and tendency for extension
of the exudate in this area.

INK-BLOT APPEARANCE
 Seen in 93% cases
 Leakage pointwith uniform dye filling is appreciated
 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 suggests late phase of the active disease
process and the active stage itself may be of variable
duration

 Patients with CSC also have window defects in areas
uninvolved by subretinal fluid during fluorescein
angiography.
 These window defects occur over areas of choroidal
vascular hyperpermeability
 In chronic CSC, atrophic RPE tracts appear as mottled
hyperfluorescence.

 Indocyanine green angiography
 Hyperpermeability of the choroid during ICG angiography.
 Best seen in the midphase of the angiogram and appear
localized in the inner
As the liver removes ICG from circulation, dye that has
leaked into the choroid appears to disperse, into deeper
layers of the choroid
giving characterstic hyperfluorescent patches in the choroid
with negative staining or silhouetting of the larger choroidal
vessels in the later phases of the ICGA

 Hyperpermeability of the choroid - better observed during the
mid-phase of the study when compared to the late phase

 Areas of hyperfluorescence appear to enlarge centrifugally
during later stages of ICGA radiating from loci of
hyperpermeability
 Multifocal hyperpermeable areas noted on wide-angle ICG
angiography are presumed to be occult PEDs
 They extend far beyond the posterior pole

 Early indocyanine green angiogram shows hyperfluorescent areas
at the posterior pole which appear to enlarge during the late
phase

 Wide-angle indocyanine
green angiogram with
central serous
chorioretinopathy
 Shows multiple
hyperfluorescent areas
including retina far beyond
the posterior pole

OCT
 IMPORTANCE
1.In diagonosis
2.Following the progress
3.Quantifying serous detachment
 OCT reveals many aspects of pathophysiology of CSC,ranging
from SRF, PED,& retinal atrophy following chronic disease
 OCT is especially helpful in identifying subtle, even
subclinical, neurosensory & macular detachments.
 OCT is also helpful in identifying the dreaded complication of
Choroidal NeoVascular membrane
 Increased choroidal thickening is a hallmark of CSC

COURSE AND OUTCOME
 Neurosensory detachment in CSC resolves spontaneously
within 3 months, with most recovering baseline visual acuity.
 After healing, a pigment epithelial scar can be observed in
area considerably larger than the original leakage point and
is characterized by the accumulation of dot-like areas of
hyperfluorescence and hypofluorescence with no signs of
dye leakage.

 Recurrences develop in about one-third to one-half of all
cases after the first episode and 10% have three or more
recurrences.
 Clinical course is variable in patients with larger
 Larger PEDs
 Multiple recurrences
 Subretinal fibrin deposits
 Multifocal leaks
 Dependent neurosensory detachments
 Atrophic RPE tracts
These are at greater risk for visual impairment.

DIFFRENTIAL DIAGNOSIS
 CNVM
 Polypoidal choroidal vasculopathy
 Optic disc pits
 VKH
 Macular holes
 Choroidal hemangioma
 Choroidal melanoma
 Choroidal metastasis
 Coat’s disease
 AMD
 Ocular hisoplasmosis

TREATMENT
 Most resolves spontaneously within a few months and visual
acuity returns to 20/25 or better
 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

PHOTOCOAGULATION
 Photocoagulation shortens course of the disease,
accelerating the resorption of fluid
 But has no effect on final visual acuity.

 Red-free photograph reveals retinal pigment epithelium (RPE)
changes with acute neurosensory detachment of the retina
inferior to the temporal superior arcade.
 Vertical optical coherence tomography (OCT) confirms subretinal
fluid and in addition shows a small RPE detachment

POST PHOTOCOAGULATION
 Two weeks after focal laser treatment there is complete
resolution of the subretinal fluid but vertical OCT reveals the
remaining RPE detachment

 As a general rule:
 Observe new-onset acute serous macular detachment for
the first 3 months.
 unless the patient has special occupational considerations
that require rapid improvement of visual acuity or in the
case of monocular patients.
 If the macular detachment has not resolved after the first
3 months and the leakage point is remote from the center of
the fovea, treat a symptomatic patient.
 If the leakage point is within 500 μm from the center of
the fovea, observe for 6 months before treatment.

 Other indications for laser treatment:
 Primary detachment with visual decline in a patient who
has experienced permanent visual loss from an untreated
macular detachment in the fellow eye
 Recurrent macular detachment in the eye of a patient
who has experienced permanent visual loss from the initial
episode.

Severe forms of CSC with poor prognosis if left untreated:
 Multiple serous detachments of the RPE
 bullous sensory retinal detachment
 dependent neurosensory detachment
 epithelial tracts
 diffuse RPE decompensation
 subretinal deposits of fibrin and lipids
 CSC associated with secondary CNV

ICG ANGIOGRAPHIC-GUIDED PHOTODYNAMIC
THERAPY
 For treatment of chronic CSC especially in cases with diffuse
decompensation of RPE
 Long-standing macular detachments of years’ duration
shows complete resolution of fluid in majority of patients
 Rationale behind such a therapeutic approach is to cause a
reduction of the blood flow in the hyperpermeable
choriocapillaris

 Safety-enhanced PDT was performed using half the normal
dose of verteporfin at 3 mg/m2 or either using half the
fluence i.e 25 mJ.
 Infusion of verteporfin was performed over 8 minutes
 Delivery of laser at 692 nm 10 minutes afterwards

 Mid-phase indocyanine green (ICG) angiogram shows a large area
of hyperfluorescence in the macula.
 Optical coherence tomography (OCT) reveals a shallow
detachment of the neurosensory retina.

POST ICGS GUIDED PDT
 PDT with verteporfin and slight increase in vision observed .
There was a circular hypofluorescence at the macula in the ICG
angiogram 3 weeks later.
 OCT confirms the complete resolution of fluid with some
hyperreflective changes in the retinal pigment epithelial layer due
to a long-standing history of subretinal fluid.

 Composite of the fluorescein angiogram with chronic CSC reveals
diffuse retinal pigment epithelium decompensation also outside
the arcades.
 Composite of the ICGA shows multiple hyperfluorescent areas
which do not always correspond to hyperfluorescent regions on
fluorescein angiography.

 FA reveals reduction in hyperfluorescence in the treated areas.
Note two temporal hyperfluorescent areas which are still leaking.
 PDT was performed on two different areas resulting in two
circular hypofluorescent regions at macula and superior to the
optic disc, as noted on the ICG angiogram 4 weeks after
treatment

 Complications of laser treatment of CSC :
 inadvertent photocoagulation of the fovea.
 persistent scotoma at site of laser photocoagulation, may
occur after the treatment.
 Secondary CNV may be induced by laser treatment when
excessive intensity is used

 Slow but progressive enlargement of the area of RPE
atrophy caused by the laser treatment.
 When the treatment site is close to center of the fovea -
enlargement of the RPE scar eventually involve fovea and
cause delayed irreversible visual loss.
Laser treatment of a leaking point in CSC should be avoided
within the foveal avascular zone.

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, lesion height and the greatest
linear dimension remained unchanged during the month of
treatment.
 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
 Have considerable side affects like paresthesias,
nervousness, and gastric upset and allergies

 Bevacizumab (Avastin):
 Anti VEGF (0.05 ml/1.25 mg intravitreal)
 Reduces the choroidal hyperpermeability
 Reverse the changes.
 Originally it was tried in chronic CSC cases

 Beta-blockers
 Metoprolol, Nadolol, and Trimepranol have been used
with controversial results
 It does not have any effect on visual recovery and its
effect on recurrence rate could not be commented as it had
short term follow-up

 Rifampin
 For chronic severe CSC recalcitrant to normal therapy

SUMMARY
 Key features of CSCR:
1 or more areas of SRF in macula
 1 or more areas of RPE detachment
 1 or more areas of IRF
 1 or more focal leakes at level of RPE on FA
 Choroidal hyperpermeability on ICGA
 Choroidal thickening on OCT
 Mottling of RPE
 Yellowish white subretinal deposit

Central serous chorioretinopathy

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Notes de l'éditeur