Background: Central serous chorioretinopathy (CSCR) is a disease in which a

serous detachment of the neurosensory retina occurs over an area of leakage

from the choriocapillaris through the retinal pigment epithelium (RPE). Other
causes for RPE leaks, such as choroidal neovascularization, inflammation, or
tumors, should be ruled out to make the diagnosis.
CSCR may be divided into 2 distinct clinical presentations. Classically, CSCR is
caused by one or more discrete isolated leaks at the level of the RPE as seen on
fluorescein angiography (FA). However, it is now recognized that CSCR may
present with diffuse retinal pigment epithelial dysfunction (eg, diffuse retinal
pigment epitheliopathy, chronic CSCR, decompensated RPE) characterized by
neurosensory retinal detachment overlying areas of RPE atrophy and pigment
mottling. During FA, broad areas of granular hyperfluorescence that contain one
or many subtle leaks are seen.
Pathophysiology: Previous hypotheses for the pathophysiology have included
abnormal ion transport across the RPE and focal choroidal vasculopathy. The
advent of indocyanine green (ICG) angiography has highlighted the importance
of the choroidal circulation to the pathogenesis of CSCR. ICG angiography has
demonstrated both multifocal choroidal hyperpermeability and hypofluorescent
areas suggestive of focal choroidal vascular compromise. Some investigators
believe that initial choroidal vascular compromise subsequently leads to
secondary dysfunction of the overlying RPE.
Recent studies employing multifocal electroretinography have demonstrated
bilateral diffuse retinal dysfunction even when CSCR was active only in one eye.
These studies support the belief of diffuse systemic effect on the choroidal
vasculature.
Type A personalities and systemic hypertension may be associated with CSCR.
The pathogenesis here is thought to be elevated circulating cortisol and
epinephrine, which affect the autoregulation of the choroidal circulation.
Mortality/Morbidity: Serous retinal detachments typically resolve spontaneously
in most patients, with the vast majority of patients (80-90%) returning to 20/25 or
better vision. Even with return of good central visual acuity, many of these
patients still notice dyschromatopsia, loss of contrast sensitivity,
metamorphopsia, or, rarely, nyctalopia.

Patients with classic CSCR (characterized by focal leaks) have a 40-50%

risk of recurrence in the same eye.

Risk of choroidal neovascularization from previous CSCR is considered

small (<5%) but has an increasing frequency in older patients diagnosed
with CSCR.

A subset of patients (5-10%) may fail to recover 20/30 or better visual

acuity. These patients often have recurrent or chronic serous retinal
detachments, resulting in progressive RPE atrophy and permanent visual
loss to 20/200 or worse. The final clinical picture represents diffuse retinal
pigment epitheliopathy.

Race: CSCR appears uncommon among African Americans but may be

particularly severe among Hispanics and Asians.
Sex: Classically, CSCR is most common in male patients aged 20-55 years with
type A personality. This condition affects men 6-10 times more often than it
affects women.
Age: Recent reports have described patients with later age of onset (>50 y). For
instance, Spaide et al reviewed 130 consecutive patients with CSCR and found
the age range at first diagnosis to be 22.2-82.9 years, with a mean age of 49.8
years.

Changes in the presentation and demographics of CSCR are observed

with increasing age at first diagnosis. Classically, patients tend to be male
and present with focal, isolated RPE leaks in one eye.

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. Furthermore, these patients are more likely to
have systemic hypertension or a history of corticosteroid use.

History:

Patients typically present with acute symptoms of visual loss and

metamorphopsia (especially micropsia). Other symptoms include
decreased central vision and a positive scotoma.

The decreased vision usually is improved by a small hyperopic correction.

Other clinical signs include a delayed retinal recovery time following

photostress, loss of color saturation, and loss of contrast sensitivity.

Physical:

Clinical exam shows a serous retinal detachment but no subretinal blood.

The neurosensory retinal detachment may be very subtle, requiring
contact lens exam for detection.

Pigment epithelial detachments, RPE mottling and atrophy, subretinal

fibrin, and, rarely, subretinal lipid also may be seen.

Causes: Previous hypotheses for the pathophysiology have included abnormal

ion transport across the RPE and focal choroidal vasculopathy. The advent of
ICG angiography has highlighted the importance of the choroidal circulation to
the pathogenesis of CSCR.

ICG angiography has demonstrated both multifocal choroidal

hyperpermeability and hypofluorescent areas suggestive of focal choroidal
vascular compromise. Some investigators believe that initial choroidal
vascular compromise subsequently leads to secondary dysfunction of the
overlying RPE.

Recent studies employing multifocal electroretinography have

demonstrated bilateral diffuse retinal dysfunction even when CSCR was
active only in one eye. This supports the belief of diffuse systemic effect
on the choroidal vasculature.

Systemic associations of CSCR include organ transplantation, exogenous

steroid use, endogenous hypercortisolism (Cushing syndrome), systemic
hypertension, systemic lupus erythematosus, pregnancy, and use of
psychopharmacologic medications.

Finally, type A personalities and systemic hypertension may be associated

with CSCR presumably because of elevated circulating cortisol and
epinephrine, which affect the autoregulation of the choroidal circulation

Lab Studies:

Laboratory tests, in general, are not helpful in the diagnosis, although a

recent case report identified an elevated level of plasminogen activator
inhibitor 1 in the serum of patients with CSCR.

Imaging Studies:

FA of classic CSCR shows one or more focal leaks at the level of the RPE.
The classic "smokestack" appearance of the fluorescein leak is seen only
in 10-15% of cases. FA of diffuse retinal pigment epitheliopathy
demonstrates focal granular hyperfluorescence corresponding to window
defects and blockage caused by RPE atrophy and clumping with one or
more areas of subtle continued leakage.

ICG angiography has shown hypofluorescent areas early in the angiogram

followed by late hyperfluorescence and leakage in choroidal vasculature.
Often, multiple areas of leakage are seen on ICG angiography that are not
evident clinically or on FA. According to some researchers, characteristic
mid phase findings on ICG allow differentiation from occult choroidal
neovascularization in older individuals. Multiple patches of

hyperfluorescence presumably are due to choroidal hyperpermeability,

which, in later phases, results in silhouetting or negative staining of larger
choroidal vessels.
Medical Care: Efficacy of tranquilizers or beta-blockers is unknown.
Furthermore, a recent evaluation of 230 consecutive patients with CSCR found
that use of psychopharmacologic agents (eg, anxiolytics, antidepressants) was a
risk factor for CSCR. Use of corticosteroids in the treatment of CSCR should be
avoided because it may result in exacerbation of serous detachments already
present.
Surgical Care: Laser photocoagulation should be considered under the following
circumstances: (1) persistence of a serous retinal detachment for more than 4
months, (2) recurrence in an eye with visual deficit from previous CSCR, (3)
presence of visual deficits in opposite eye from previous episodes of CSCR, and
(4) occupational or other patient need requiring prompt recovery of vision.

Laser treatment also may be considered in patients with recurrent

episodes of serous detachment with a leak located more than 300 m
from the center of the fovea.

Laser treatment shortens the course of the disease and decreases the risk
of recurrence for CSCR, but it does not appear to improve the final visual
prognosis.

Some evidence suggests that patients with chronic CSCR (diffuse retinal
pigment epitheliopathy) may have better prognosis with laser treatment.

Treatment technique
o

Visualize the macula at the laser slit lamp and compare it with the
projected angiogram image.

Apply light argon green, yellow, or krypton red laser burns to the
area or areas of leakage.

Typical laser settings are burn size of 100-200 m, duration of 0.1

s, and power at 100 mW. Power should be increased until a lightgray burn is achieved.

Treat the area of leakage with one or a few gentle burns. Recurrent
cases with multiple leaks will require more burns.

Activity: Patient participation in stress-reducing activities (eg, exercise,

meditation, yoga) is recommended.
Further Outpatient Care:

Most patients receive follow-up care for 2 months to determine whether

the fluid resolves spontaneously.

Complications:

A small minority of patients develops choroidal neovascularization at the

site of leakage and laser treatments. A retrospective review of cases
shows that one half of these patients may have had signs of occult
choroidal neovascularization at the time of treatment. In the other patients,
the risk of choroidal neovascularization may have been increased by the
laser treatment.

Acute bullous retinal detachment may occur in otherwise healthy patients

with CSCR. This appearance may mimic Vogt-Koyanagi-Harada disease,
rhegmatogenous retinal detachment, or uveal effusion. A case report also
has implicated the use of corticosteroids in CSCR as a factor increasing
the likelihood of subretinal fibrin formation. Reducing the corticosteroid
dose frequently will lead to resolution of the serous retinal detachment.

Prognosis:

Serous retinal detachments typically resolve spontaneously in most

patients, with the vast majority of patients (80-90%) returning to 20/25 or
better vision.

Patients with classic CSCR (characterized by focal leaks) have a 40-50%

risk of recurrence in the same eye.

Even with return of good central visual acuity, many of these patients still
notice dyschromatopsia, loss of contrast sensitivity, metamorphopsia, or
nyctalopia.

A subset of patients (5-10%) may fail to recover 20/30 or better visual

acuity. These patients often have recurrent or chronic serous retinal
detachments, resulting in progressive RPE atrophy and permanent visual
loss to 20/200 or worse. The final clinical picture represents diffuse retinal
pigment epitheliopathy.

Risk of choroidal neovascularization from previous CSCR is considered

small (<5%) but has an increasing frequency in older patients diagnosed
with CSCR.

Patient Education:

If possible, patients should avoid stressful situations. Patient participation

in stress-reducing activities (eg, exercise, meditation, yoga) is
recommended.

Recent evidence associates systemic hypertension with CSCR, but it is

unknown as to whether careful control of systemic hypertension will
reduce the incidence of CSCR.

Caption: Picture 1. Fluorescein angiography in the early recirculation phase of a

patient with a localized neurosensory detachment in the macula from central serous
chorioretinopathy. Note the focal hyperfluorescence.
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Caption: Picture 2. Fluorescein angiography in the late recirculation phase of the
same patient. Note the distribution of leakage of fluorescein dye within the
neurosensory detachment.

pertengahan dan mungkin berkaitan dengan kejadian-kejadian stres kehidupan. Sebagian besar pasien
datang dengan penglihatan kabur, mikropsia, metamorfosia, dan skotoma sentralis yang timbul
mendadak. Ketajaman penglihatan sering hanya berkurang secara sedang dan dapat diperbaiki
mendekati normal dengan koreksi hi-peropik kecil.
Diagnosis ditegakkan dengan pemeriksaan fundus dengan slitlamp\ adanya pelepasan serosa retina
sensorik tanpa peradangan mata, neovaskularisasi retina, suatu lubang kecil optik, atau tumor koroid
bersifat diagnostik. Lesi epitel pigmen retina tampak sebagai bercak abu-abu kekuningan, bundar atau
oval, kecil yang ukurannya bervariasi dan mungkin sulit dideteksi tanpa bantuan angiografi
fluoresens. Zat warna fluoresens yang bocor dari koriokapilaris dapat tertimbun di bawah epitel pig-

men atau retina sensorik, sehingga menimbulkan berma-cam-macam pola termasuk konfigurasi
cerobong asap yang terkenal itu.
Sekitar 80% mata dengan korioretinopati serosa sentralis mengalami resorpsi spontan cairan subretina
dan pemulihan ketajaman penglihatan normal dalam 6 bulan setelah awitan gejala. Namun, walaupun
ketajaman penglihatan normal, banyak pasien mengalami defek penglihatan permanen, misalnya
penurunan ketajaman kepekaan terhadap warna, mikropsia, atau skotoma rela-tif. Duapuluh sampai 30
persen akan mengalami sekali atau lebih kekambuhan penyakit, dan pepiah dilaporkan adanya
penyulittermasuk neovaskularisasi subretina dan edema makula sistoid kronikpada pasien yang
sering dan berkepanjangan mengalami pelepasan serosa.
Gambar 10-3. Angiogram fluoresens korioretinopati sentralis memperlihatkan penyakit aktif dengan epitel pigmen
retina (tanda panah kecil) dan pelepasan i sensorik (tanda panah besar). Juga terdapat dua fokus penya*.] kit
inaktif (tanda panah terbuka).

Penyebab korioretinopati serosa sentralis tidak i ketahui; tidak terdapat bukti yang meyakinkan bahv*
penyakit bersifat infeksiosa atau disebabkan oleh distrg| j epitel pigmen retina. Fotokoagulasi laser
argon yawl diarahkan ke bagian yang bocor akan secara bermakn$3 mempersingkat durasi pelepasan
retina sensorik da$'| mempercepat pemulihan penglihatan sentral, tetapi tidak terdapat bukti bahwa
fotokoagulasi yang segeja dilakukan akan menurunkan kemungkinan gangguan penglihatan
permanen. Walaupun penyulit fotokoagulasi laser retina sedikit, terapi fotokoagulasi laser segera
sebaiknya tidak dianjurkan untuk semua pasien konV retinopati serosa sentralis. Lama dan letak
penyakit* keadaan mata yang lain, dan kebutuhan visual oku-pasional merupakan faktor-faktor yang
perlu dipertim-bangkan dalam memutuskan pengobatan.