2013

Ocular Immunology & Inflammation, 2013; 21(3): 201–206! Informa Healthcare USA, Inc.

ISSN: 0927-3948 print / 1744-5078 online

DOI: 10.3109/09273948.2013.765015

ORIGINAL ARTICLE

Dark Spot in Fibrinous Central SerousChorioretinopathy Masquerading Choroiditis

Swapnil Parchand, MD1, Vishali Gupta, MD

1, Amod Gupta, MD1, M. R. Dogra, MD

1,Ramandeep Singh, MD

1, and Aman Sharma, MD2

1Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Educationand Research, Chandigarh, India and 2Department of Internal Medicine, Post Graduate Institute

of Medical Education and Research, Chandigarh, India

ABSTRACT

Purpose: The authors observed that eyes with acute fibrinous central serous chorioretinopathy (CSC)masquerading active choroiditis had a ‘‘dark spot’’ within the yellow fibrinous deposit. The present studyaims to describe this sign as a clinical indicator of acute serofibrinous exudative detachment, thus helpingto differentiate it from active choroiditis.

Method: The authors retrospectively reviewed the records of 19 patients of fibrinous CSC masquerading activechoroiditis. Color fundus photographs, fundus fluorescein angiogram (FFA), and optical coherence tomography(OCT) at baseline and follow-up were studied for a dark spot. The systemic steroids were stopped andall patients were followed up.

Results: There were 12 men and 7 women with a mean age of 39.8 years. Fourteen patients had receivedsystemic steroids. Funduscopy revealed creamy yellow subretinal lesion/s simulating active choroiditis lesionin all eyes and exudative retinal detachment in 9 eyes. The dark spot was seen as a round, grayish dark spotwithin the fibrinous lesion in all eyes. On FFA and OCT, this dot corresponded to the site of leakage. All eyesshowed resolution of CSC on follow-up.

Conclusion: Detection of a dark spot within fibrinous CSC is an important clinical sign that, if present, helps toavoid misdiagnosis, unnecessary diagnostic tests, and incorrect treatment.

Keywords: Central serous retinopathy, choroiditis, corticosteroids, dark spot, fibrinous central serouschorioretinopathy

Central serous chorioretinopathy (CSC) is a chorior-etinal disease, characterized by accumulation of fluidbetween the neurosensory retina and retinal pigmentepithelium. In some patients, acute CSC may presentas fibrinous CSC characterized by the presence ofexudative detachment and subretinal fibrin depos-ition.1 Due to similarity in the fundus appearance,these cases with fibrinous CSC maybe misdiagnosedas choroidits, Vogt Koyanagi Harada disease, or focalretinitis and thus be treated with corticosteroids,which may lead to worsening of the disease.2

Subretinal fibrin deposition is actually consideredan important clinical feature of severe/chronic CSC,

which may be induced or exacerbated by inappropri-ate use of systemic corticosteroids. These atypical CSCcases may be misdiagnosed as an inflammatory ocularcondition and differential diagnosis largely relies onan array of other clinical features, along with findingsfrom optical coherence tomography (OCT), fundusfluorescein angiography (FFA), and indocyaninegreen angiography (ICGA).

Funduscopy in patients with fibrinous CSC usuallyshows a large, creamy yellow subretinal lesion/swith/out inferior exudative detachment. On carefulexamination, we observed a round, grayish, translu-cent ‘‘dark spot’’ within this creamy yellow lesion

Correspondence: Vishali Gupta, MD, Additional Professor, Advanced Eye Centre, Department of Ophthalmology, Post Graduate Institute ofMedical Education & Research, Chandigarh, India. E-mail: vishalisara@yahoo.co.in

Received 7 October 2012; revised 3 January 2013; accepted 7 January 2013; published online 6 March 2013

201

Ocu

l Im

mun

ol I

nfla

mm

Dow

nloa

ded

from

info

rmah

ealth

care

.com

by

Yal

e D

erm

atol

ogic

Sur

gery

on

10/1

2/13

For

pers

onal

use

onl

y.

seen in nearly all the patients with fibrinous CSC,which corresponds to the site of leakage on fundusfluorescence angiography (Figure 1A). We found thisclinical sign quite useful in differentiating fibrinousCSC from choroiditis on funduscopy alone. We did aretrospective analysis, including study of fundusphotographs, fluorescein angiograms, and opticalcoherence tomograms of our patients with fibrinousCSC to see whether the dark spot sign couldbe used as a clinical indicator of acute centralserous chorioretinopathy with serofibrinous exuda-tive detachment.

PATIENTS AND METHODS

After obtaining institutional ethic committee clear-ance, we retrospectively studied the clinical recordsof patients who presented at the Retina Clinic fromJanuary 2007 to December 2010 and were diagnosedas CSC in our center. We included (1) patients ofCSC who were misdiagnosed as choroiditis else-where, (2) patients in whom there was difficultlyin making diagnosis between choroiditis and CSC,and (3) patients with acute CSC with serofibrinousexudative detachment.

All the enrolled patients had their records ofhistory with ocular and systemic examination avail-able at presentation as well as during follow-up visits.All patients had a detailed history recorded in theretina clinic file, including history of steroid exposure,and underwent a complete ophthalmic examination,including best-corrected visual acuity, intraocularpressure, slit-lamp biomicroscopy, and posteriorsegment examination with slit-lamp biomicroscopy.All the patients had fundus fluorescein angiographyand optical coherence tomography at presentationand on follow-up as and when indicated. A seniorconsultant internist who has special interest in sys-temic inflammatory diseases did systemic evaluationwhenever required. Systemic corticosteroids wereeither stopped or quickly tapered off in all theexposed cases. Focal laser of the leaking points wasdone wherever necessary. Best-corrected visual acuitywas documented in logMAR value. Visual improve-ment was defined as halving of the visual angle, andvisual deterioration as doubling of the visual angle.Visual acuity was said to be stabilized if the finalvisual acuity remained within two lines of thepresenting acuity.

RESULTS

The study included 31 eyes of 19 patients of fibrinousCSC meeting the inclusion criteria. The age at pres-entation ranged between 30 and 50 years (mean¼ 39.8years). Men were more commonly involved than

women, with a men:women ratio of 1.7:1. The detailsof the patients are shown in Table 1.

Nine of the 19 patients were misdiagnosed else-where as having active choroiditis and were alreadyreceiving systemic corticosteroids. Another 5 patientswere receiving systemic corticosteroids for retinalvasculitis, sarcoidosis, panuveitis, postviral facialpalsy, and skin disease, respectively, and developedfibrinous CSC during follow-up. The remaining 5patients were first seen and diagnosed as havingfibrinous CSC at our institute.

Funduscopy in all cases revealed creamy yellowsubretinal lesion/s simulating active choroiditislesion. These lesions varied in size from 1 discdiameter to almost 10 disc diameters. The marginsof these lesions were indistinct because of surround-ing subretinal fluid. Dark spot sign was seen as around dark dot within the fibrinous lesion in all theeyes (Figures 1A and 2A). Inferior exudative detach-ment was seen in 9 eyes. We also noticed a trackoriginating from the dark spot region and ending inlarge, creamy yellow fibrinous exudative deposits in 4eyes. All 4 eyes typically had inferior bullous exuda-tive detachment. Associated PEDs were seen in 24eyes either in the involved eyes or contralateral eyes.

All the patients had fundus fluorescein angiog-raphy (FFA) at presentation. Early-phase FFA showedfocal dye leakage from the choroid into the subretinalspace at the center of the ring-shaped, creamy yellowsubretinal exudate (dark spot sign) and late-phaseshowed staining of the exudates with pooling of dyein subretinal space (Figures 1B and 2B). The subretinalexudation did not block the underlying fluorescence.This characteristic finding was noted in all theinvolved eyes. In 4 patients a linear track of hyper-fluorescence was seen originating from the site ofleakage, leading to massive pooling of dye at itsterminal end. No patient had evidence of activechoroiditis or active vasculitis. The expanding dotsign was the most common pattern of dye leak seen in18 eyes, followed by smokestack appearance in 7 eyesand mushroom pattern in 1 eye.

Optical coherence tomography line scan was doneon the horizontal and vertical planes through the darkspot area at baseline and at the follow-up. OCTscans of16 patients could be retrieved. In the area of theexudates, OCT showed moderate or highly reflectivemasses bridging the neurosensory retina and theRPE in all eyes. In the area of the dark spot, OCTshowed an optically clear space beneath the neurosen-sory retina (Figures 1C and 2C). Systemic corticoster-oids were either stopped or quickly tapered off in allthe 14 patients receiving it. Resolution of CSC was seenin 18 of 19 cases, while 1 patient was lost to follow-up(Figures 3 and 4). Laser photocoagulation of the site ofleakage was done in 10 eyes. At the last follow-up, thebest-corrected visual acuity (logMAR value) hadimproved in 20 eyes, stabilized in 8 eyes, and

202 S. Parchand et al.

Ocular Immunology & Inflammation

Ocu

l Im

mun

ol I

nfla

mm

Dow

nloa

ded

from

info

rmah

ealth

care

.com

by

Yal

e D

erm

atol

ogic

Sur

gery

on

10/1

2/13

For

pers

onal

use

onl

y.

TA

BL

E1.

Cli

nic

ald

etai

lso

fp

atie

nts

wit

hfi

bri

no

us

CS

C.

Sr.

no

.A

ge/

sex

Fo

rmo

fst

ero

ids

exp

osu

reIn

itia

ld

iag

no

sis

Ey

eP

atte

rno

nF

FA

Init

ial

vis

ual

acu

ity

(lo

gM

AR

)

Fin

alv

isu

alac

uit

y(l

og

MA

R)

Co

mm

ents

150

/F

Ora

lT

ub

ercu

lou

sch

oro

idit

isR

Ex

pan

din

gd

ot

sig

n0.

50

AT

Tan

do

ral

ster

oid

sst

op

ped

LE

xp

and

ing

do

tsi

gn

0.6

02

45/

MP

ost

erio

rsu

bte

no

nT

riam

cin

olo

ne

(1in

ject

ion

)

Tu

ber

culo

us

vas

culi

tis

LE

xp

and

ing

Do

tsi

gn

00

Rec

eiv

edA

TT

trea

tmen

tw

ith

po

ster

ior

sub

-T

eno

ntr

iam

cin

olo

ne

for

tub

ercu

lou

sv

ascu

-li

tis

bu

to

nfo

llo

wu

pd

evel

op

edC

SC

330

/M

Ora

lT

ub

ercu

lou

sch

oro

idit

isR

Sm

ok

est

ack

app

eara

nce

0.6

1A

TT

and

ora

lst

ero

ids

sto

pp

edL

Ex

pan

din

gd

ot

sig

n0

04

36/

MO

ral

Ch

oro

idit

isR

Sm

ok

est

ack

app

eara

nce

10.

8S

top

ped

ora

lst

ero

ids

wit

hla

ser

of

leak

ing

po

ints

inb

oth

eyes

LS

mo

ke

stac

kap

pea

ran

ce0.

21

539

/M

Ora

lC

SC

RP

ED

s0.

20

Sto

pp

edo

ral

ster

oid

sL

Sm

ok

est

ack

app

eara

nce

0.8

0.2

637

/F

Ora

lC

ho

roid

itis

RE

xp

and

ing

do

tsi

gn

0.3

0S

top

ped

ster

oid

sL

Ex

pan

din

gd

ot

sig

n0.

40.

27

43/

MO

ral

Ch

oro

idit

isL

Sm

ok

est

ack

app

eara

nce

11

845

/F

Ora

lT

ub

ercu

lou

sp

anu

vet

itis

RE

xp

and

ing

do

tsi

gn

0.2

0R

ecei

ved

AT

Tþ

ora

lst

ero

ids

for

pan

uv

eiti

sb

ut

ov

erfo

llo

w-u

pd

evel

op

edC

SR

;st

op

ped

ora

lst

ero

id9

44/

FO

ral

Tu

ber

culo

us

cho

roid

itis

LE

xp

and

ing

do

tsi

gn

0.5

–S

top

ped

ster

oid

sþ

AT

T10

33/

MO

ral

Tu

ber

culo

us

cho

roid

itis

RS

mo

ke

stac

kap

pea

ran

ce0.

50

Sto

pp

edst

ero

idsþ

AT

TL

Mo

ttle

d0

011

40/

FN

ost

ero

ids

Fib

rin

ou

sC

SC

RP

ED

s0

–L

ost

tofo

llo

w-u

pL

Ex

pan

din

gd

ot

sig

n0.

6–

1242

/M

No

ster

oid

sF

ibri

no

us

CS

CR

Mo

ttle

d0.

20

LE

XP

AN

DIN

GD

ot

sig

n0.

50

1339

/M

Ora

lS

arco

ido

sis

RE

xp

and

ing

do

tsi

gn

0.2

0S

top

ped

ora

lst

ero

ids

1444

/M

IVan

do

ral

Ch

oro

idit

isR

Sm

ok

est

ack

app

eara

nce

10.

3S

top

ped

ster

oid

sL

Mo

ttle

d0

015

34/

MO

ral

Ch

oro

idit

isR

Ex

pan

din

gd

ot

sig

n0.

50

Sto

pp

edo

ral

ster

oid

sL

Mu

shro

om

shap

ed0.

50

1640

/F

No

ster

oid

sF

ibri

no

us

CS

CR

Ex

pan

din

gd

ot

sig

n1

1L

Ex

pan

din

gd

ot

sig

n0.

40

1743

/F

No

ster

oid

sF

ibri

no

us

CS

CL

Ex

pan

din

gD

ot

sig

n0.

80.

318

37/

MN

ost

ero

ids

Fib

rin

ou

sC

SC

RE

xp

and

ing

do

tsi

gn

0.8

0.5

LE

xp

and

ing

do

tsi

gn

0.2

0.6

1937

/M

Ora

lF

ibri

no

us

CS

CL

Ex

pan

din

gd

ot

sig

n2

2S

top

ped

ora

lst

ero

ids

M,

mal

e;F,

fem

ale;

CS

C,

cen

tral

sero

us

cho

rio

reti

no

pat

hy

;P

ED

,p

igm

ent

epit

hel

ial

det

ach

men

t;A

TT

,an

ti-t

ub

ercu

lar

ther

apy.

Fibrinous Central Serous Chorioretinopathy 203

! 2013 Informa Healthcare USA, Inc.

Ocu

l Im

mun

ol I

nfla

mm

Dow

nloa

ded

from

info

rmah

ealth

care

.com

by

Yal

e D

erm

atol

ogic

Sur

gery

on

10/1

2/13

For

pers

onal

use

onl

y.

deteriorated in 3 eyes. Complications included scar (10eyes), atrophy (16 eyes), RIP (1 eye), and CNVM (1 eye).

DISCUSSION

Serous detachment with the presence of exudativedeposits and subretinal fibrin is one of the atypicalforms of central serous chorioretinopathy (CSC)observed in approximately 10–15% of cases.3 It isusually seen in 50–90% of CSC cases seen duringpregnancy.1,3 Quillen et al. reported that subretinalfibrin was more common in patients exposed tosystemic glucocorticoids.4 Many of these patients areinitially misdiagnosed as having choroiditis andreceive systemic steroids that worsen the condition.2

Nine patients in the present series were initiallymisdiagnosed as active choroiditis because of thesimilarity in clinical appearance of subretinal exud-ation of fibrin deposition mimicking choroiditisand were started on corticosteroids elsewhere, leadingto worsening of this condition. However, biomicro-scopic examination of these patients did not reveal anycellular reaction, healed chorioretinal scars, or anysigns of vasculitis. On funduscopy, we noticed darkspot/s within the subretinal fibrin exudation that was

seen in all of the involved eyes. This dark spotcorresponds to the point of leakage in CSC.We believe that the leakage site appears as a darkspot because as the fluid is leaking, it displacesthe surrounding yellowish-white fibrin away fromthe point of leakage and thus this is seen as a clear spotthat appears dark because of surrounding yellowcoloration of the fibrin. On FFA, this sign actuallycorresponded with the site of leakage from the choroidin subretinal space in all eyes, and the OCT scanspassing through the dark spot showed an opticallyclear space (indicating the presence of clear fluid)beneath the neurosensory retina in contrast to moder-ate to highly reflective fibrin seen surrounding this spot(Figures 1 and 2).

The exact pathophysiology of fibrinous CSCremains unclear but it is thought that disturbance ofthe posterior blood–retinal barrier, the choriocapil-laris, Bruch’s membrane, or the RPE may be the siteof the primary damage. Gass5 suggested that thepresence of one or several focal areas of increasedcapillary permeability in the choriocapillaries mightbe responsible for the serous exudation beneaththe RPE. Yoshioka et al.6 experimentally produceda CSC-like condition in monkeys after repeatedintravenous injections of epinephrine. The adrenergic

FIGURE 1. (A) Funduscopy of right eye of patient (diagnosed elsewhere as choroiditis and started on oral steroids) showing large,creamy subretinal lesion with indistinct margins involving the whole of the posterior pole. Two dark spots can be seen within thislarge, creamy lesion (black arrow). Linear track (red arrow) can be seen superotemporal to the fovea originating from this dark spotand ending up in large, creamy, yellow fibrinous deposits. (B) Early-phase FFA showing leakage of dye from the choroid into thesubretinal space at the center of the ring-shaped, creamy subretinal exudates (dark spot sign) and extending upward in smoke stackpattern (red arrow). (C) On OCT, horizontal scan passing through the center of this point of leakage showed an optically clear space(red arrow) beneath the neurosensory retina surrounded by moderate or highly reflective masses (fibrin) bridging the neurosensoryretina and the RPE.

204 S. Parchand et al.

Ocular Immunology & Inflammation

Ocu

l Im

mun

ol I

nfla

mm

Dow

nloa

ded

from

info

rmah

ealth

care

.com

by

Yal

e D

erm

atol

ogic

Sur

gery

on

10/1

2/13

For

pers

onal

use

onl

y.

effect of epinephrine may cause damage to thechoriocapillaries and increase choriocapillarypermeability.

Cortisol permits normal responsiveness of arteri-oles to the constructive actions of catecholamineand could potentiate the process. Also, inhibitionof collagen synthesis by cortisol may produce thin-ning of capillary walls, resulting in increased capillaryfragility. Normally proteins do not leak through

choriocapillaries, but due to the damaging effect ofcorticosteroids leading to increased capillary perme-ability large molecules are able to diffuse out. It hasbeen shown that blowouts of PED occur at the areaof maximal stress, causing access of this exudationto subretinal space. Thus, this proteinaceous exud-ation gains excess to subretinal space through theseblowout areas and gets deposited in this space aroundthis point of leakage.

FIGURE 2. (A) Funduscopy of the left eye of the same patient showing creamy subretinal lesion with indistinct margins along theinferotemporal arcade. Two dark spots can be seen within this large, creamy lesion (red arrow). (B) Early-phase FFA showing leakageof dye from the choroid into the subretinal space at the center of the ring-shaped, creamy subretinal exudates (dark spot sign). (C) OnOCT, a horizontal scan passing through center of this point of leakage showed an optically clear space (red arrow) beneath theneurosensory retina surrounded by moderate or highly reflective masses (fibrin) bridging the neurosensory retina and the RPE. PEDcan also be seen.

FIGURE 3. (A) Funduscopy of right eye at presentation. (B) Funduscopy of right eye at 6-month follow-up after oral steroids werestopped and focal laser was used at leaking points twice, showing resolution of creamy lesion with deposition of subretinal fibrousstrand (red arrow) and pigmentary changes in fovea.

Fibrinous Central Serous Chorioretinopathy 205

! 2013 Informa Healthcare USA, Inc.

Ocu

l Im

mun

ol I

nfla

mm

Dow

nloa

ded

from

info

rmah

ealth

care

.com

by

Yal

e D

erm

atol

ogic

Sur

gery

on

10/1

2/13

For

pers

onal

use

onl

y.

Histophathological analysis has revealed the pres-ence of high protein content within the subretinalfluid,7 which may be the cause of smokestack leaksseen in these patients.8 These studies as well as theclinical appearance of the exudation suggest that thereis proteinaceous material, including the possibilityof fibrin, present in subretinal space in central serouschorioretinopathy, which might contribute to theformation of a subretinal exudative deposits.Presence of subretinal fibrin is also confirmed onOCT, which shows highly reflective masses bridgingthe neurosensory retina and the RPE in the area ofa creamy yellowish lesion. Also, this type of CSC withfibrin deposition typically heals with developmentof fibrous strands (Figures 3 and 4). The dark spotseen at the point of leakage seems to be the result offluid leaking with force (fountain-like effect) thatdisplaces the fibrin to periphery and the dark spot isseen as a clear area within the fibrinous deposits. It isimportant to recognize this sign clinically as it is astrong clinical indicator that helps differentiate CSCfrom choroiditis and thus initiation of systemic cor-ticosteroids could be avoided. The dark spot signwould help in differentiating fibrinous CSC not onlyfrom choroidal lesions, but also from subretinalfibrosis associated with choroiditis.

Apart from being a retrospective study, the absenceof a control group was a limitation in this study.In short, the detection of a dark spot within fibrinousCSC is an important sign that, if present, helps toavoid misdiagnosis, unnecessary diagnostic tests,and incorrect treatment. FFA confirms the diagnosis,

but in conditions where it is not feasible, this sign canbe used to diagnose this condition.

DECLARATION OF INTEREST

The authors report no conflicts of interest. The authorsalone are responsible for the content and writing ofthe paper.

REFERENCES

1. Bouzas EA, Karadimas P, Pournaras CJ. Central serouschorioretinopathy and glucocorticoids. Surv Ophthlmol.2002;47:431–448.

2. Khairallah M, Kahloun R, Tugal-Tutkun I. Central serouschorioretinopathy, corticosteroids, and uveitis. OculImmunol Inflamm. 2012;20:76–85.

3. Gass JDM. Central serous chorioretinopathy and whitesubretinal exudation during pregnancy. Arch Ophthalmol.1991;109:677–681.

4. Quillen DA, Gass DM, Brod RD. Central serous chorior-etinopathy in women. Ophthalmology. 1996;103:72–79.

5. Gass JDM. Pathogenesis of disciform detachment of theneuroepithelium, II: idiopathic central serous choroido-pathy. Am J Ophthalmol. 1967;63:587–615.

6. Yoshioka H, Katsume Y, Akune H. Experimental cen-tral serous chorioretinopathy, I: clinical findings. Jpn JOphthalmol. 1981;25:112–118.

7. Ikui H. Histologic examination of central serous retinop-athy. Nippon Ganka Kiyo. 1969;20:1035–1041.

8. Shimizu K, Tobari I. Central serous retinopathydynamics of subretinal fluid. Mod Prob Ophthalmol.1971;9:152.

FIGURE 4. (A) Funduscopy of left eye at presentation. (B) Funduscopy of left eye at 6-month follow-up after oral steroidswere stopped and focal laser was used at leaking points twice showing resolution of creamy lesion with deposition of subretinalfibrous strand (red arrow).

206 S. Parchand et al.

Ocular Immunology & Inflammation

Ocu

l Im

mun

ol I

nfla

mm

Dow

nloa

ded

from

info

rmah

ealth

care

.com

by

Yal

e D

erm

atol

ogic

Sur

gery

on

10/1

2/13

For

pers

onal

use

onl

y.