Central Serous Chorioretinopathy

Central serous chorioretinopathy (CSCR) is a problem that affects the macula (central portion of the retina). The exact cause is not understood.

CSCR occurs when a small break forms in the pigment layer of the retina. Fluid from the layer of blood vessels that lie underneath the retina seeps up through the break, causing a small detachment to form under the retina.

CSCR is often a straightforward clinical diagnosis when it presents as a typical serous neurosensory retinal detachment in a middle-aged male. However, atypical presentations or chronic cases are more of a challenge.

Despite observations using indocyanine green angiography (ICGA) that suggest that CSCR is primarily a choroidopathy, debate continues over whether the primary underlying pathology is at the level of the choroid, the retinal pigment epithelium (RPE) or both.

The retinal pigment epithelium

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.
Demographically, CSCR is believed to be predominantly a disease of men 20 to 45 years of age. CSCR diagnosed after the age of 50, should raise a high suspicion of neovascular age-related macular degeneration.
CSCR is more common in Caucasians, Hispanics and Asians, and less common in those of African descent. Persons with CSCR have a reported higher prevalence of migraine like headaches or psychiatric conditions, including hypochondria and hysteria. High-stress occupations or lifestyles also have been associated with CSCR.
Patients with CSCR may complain of decreased visual acuity, micropsia, metamorphopsia, abnormal color vision and scotomas. Patients may be asymptomatic if the fovea is uninvolved.
Corticosteroids have long been described as an exacerbating or precipitating factor in CSCR. Affected individuals may have forgotten previous intraarticular corticosteroid injections or may not realize that their inhaler, nose spray or skin cream contains corticosteroids.
A patient with CSCR usually presents with visual acuity in the range of 20/20 to 20/200, with an average presenting visual acuity of 20/30. Visual acuity can often be improved with a small hyperopic correction given the associated shallow neurosensory detachment.
Amsler grid testing may demonstrate metamorphopsia in eyes with near-normal visual acuity.

The anterior segment and vitreous are normal with no evidence of inflammation.
A dilated fundus examination with biomicroscopy and indirect ophthalmoscopy is essential to appreciate the characteristic findings of CSCR.
RPE atrophy may be present in one or both eyes as evidence of an active or previous CSCR episode.
Inferior, peripheral, atrophic RPE tracts (linear pigment lines) characterize a less common type of CSCR associated with a poorer prognosis. Excessive or prolonged subretinal leakage is responsible for this type of CSCR. Gravity causes the subretinal fluid to collect inferiorly, forming a “teardrop” or “hourglass” shape. Additional manifestations of chronic retinal detachment in these eyes may include cystoid macular edema, extrafoveal cystoid changes in the detached retina, lipid deposits, retinal telangiectasia, CNV and perivascular or bone spicule pigmentation patterns. This more severe type of CSCR is more common in persons of Hispanic or Asian descent.
Another atypical variant of CSCR usually is found in healthy middle-aged men and presents with multiple PEDs and multiple bullous serous neurosensory detachments that demonstrate shifting fluid. This form of CSCR is characterized by a more fibrinous subretinal exudate that imparts an opaque appearance to the subretinal fluid. Late sequelae of this fibrinous exudate include subretinal fibrosis, CNV or RPE rips.

Ancillary Testing
Fluorescein angiography is often used to establish the diagnosis of CSCR. Frequently, FA demonstrates single or multiple discrete leakage points that evenly distribute dye throughout the subretinal fluid or that may reveal evidence of a PED. The classic “smokestack” appearance occurs in less than 20 percent of cases.
Ocular coherence tomography demonstrates the neurosensory detachment in addition to any PED associated with the CSCR. Also, OCT is an objective way to follow changes in the amount of subretinal fluid.

In some cases, ICGA may be required to differentiate between CSCR and AMD or polypoidal choroidal vasculopathy (PCV). Multiple areas of choroidal hyperpermeability in the mid-to-late phases of ICGA are indicative of CSCR and not AMD or PCV.³

Natural History
Generally, CSCR has a good prognosis for spontaneous visual recovery, with the vast majority of eyes stabilizing with 20/30 to 20/40 visual acuity. The neurosensory detachments usually resolve within three to four months, but vision may continue to improve up to a year later. One-third to one-half of CSCR cases will recur, and 10 percent of recurrences will have at least three episodes. Of the eyes that have more than one episode, nearly 50 percent recur within one year of the initial incident. Even after anatomical resolution, visual symptoms may include abnormal color vision, metamorphopsia, micropsia and relative scotomas.
Certain features and coexistent conditions are associated with lower final visual acuity. Patients who develop persistent or recurrent foveal detachments, CNV, subretinal fibrosis, subfoveal RPE atrophy, multiple sites of leakage, larger PEDs, cystoid macular edema, dependent detachments and atrophic RPE tracts have a poorer prognosis. Also associated with a poorer prognosis are concomitant end-stage renal failure, organ transplantation, pregnancy, corticosteroid use or elevated endogenous cortisol levels (type A personalities, systemic hypertension, and obstructive sleep apnea may be associated with elevated circulating cortisol and epinephrine, which affect the autoregulation of the choroidal circulation). Other risk factors included antibiotic use, alcohol use, untreated hypertension, and allergic respiratory disorders.

No medical treatment has proven effective for CSCR; however, acetazolamide has been suggested to hasten the resolution of subretinal fluid. Corticosteroids are contraindicated and have no therapeutic role in CSCR.²
Direct focal laser photocoagulation, with low-intensity laser burns to the leakage site, abbreviates the disease course but has no effect on final visual acuity or recurrence rate. Because of the generally good prognosis of CSCR and the tangible risks associated with laser photocoagulation to the macula, including scarring and secondary CNV formation, laser treatment is seldom indicated for CSCR.