Central Serous Chorioretinopathy

Definition

• Central serous chorioretinopathy (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. CSCR is a diagnosis of exclusion. Care must also be taken to ensure that corticosteroid administration is not exacerbating the CSCR.

Etiology

• 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.

• Corticosteroids have long been described as an exacerbating or precipitating factor in CSCR.² The ophthalmologist should carefully question a patient with CSCR to determine any recent corticosteroid use. Affected individuals may have forgotten previous intraarticular corticosteroid injections or may not realize that their inhaler, nose spray or skin cream contains corticosteroids. The ophthalmologist may need to communicate with the other physicians coman-aging the patient’s care to ensure that only corticosteroid-sparing medications are being used. This is particularly crucial in chronic or recurrent cases because the lack of resolution may be due to unrecognized corticosteroid use.

Risk factor

• Demographically, CSCR is believed to be predominantly a disease of men 20 to 45 years of age; however, in women and older patients (> 50 years) CSCR may be more common than initially reported. CSCR diagnosed after age 50 should raise a high suspicion of neovascular age-related macular degeneration instead.

• CSCR is more common in Cauca-sians, Hispanics and Asians, and less common in those of African descent. Persons with CSCR have a reported higher prevalence of migrainelike headaches or psychiatric conditions, including hypochondria, hysteria and conversion disorder. High-stress occupations or lifestyles also have been associated with CSCR.

• 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.

Symptoms

• 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.

• Other clinical signs include a delayed retinal recovery time following photostress, loss of color saturation, and loss of contrast sensitivity.

Pathophisiology • 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.

• Studies using 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.

• Corticosteroids have a direct influence on the expression of adrenergic receptor genes and, thus, contribute to the overall effect of catecholamines on the pathogenesis of CSCR. Consequently, multiple studies have conclusively implicated the effect of corticosteroids in the development of CSCR. Carvalho-Recchia et al showed in a series that 52% of patients with CSCR had used exogenous steroids within 1 month of presentation as compared with 18% of control subjects.

Diagnosis examination

• Clinical examination shows a serous retinal detachment but no subretinal blood. The neurosensory retinal detachment may be very subtle, requiring contact lens examination for detection.

• Pigment epithelial detachments, RPE mottling and atrophy, subretinal fibrin, and rarely subretinal lipid or lipofuscinoid flecks also may be seen.

Imaging

• Optical coherence tomography (OCT) reveals many aspects of the pathophysiology of central serous chorioretinopathy (CSCR), ranging from subretinal fluid, pigment epithelial detachments, and retinal atrophy following chronic disease. OCT is especially helpful in identifying subtle, even subclinical, neurosensory macular detachments. Spaide correlated lipofuscinoid deposition of material in CSCR that might mimic vitelliform lesions in pattern dystrophies. OCT showed accumulation of this material on the outer surface of the retina in neurosensory detachments.

• FA (Fluorescein angiography) 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

Hallo Macula

Treatment

• 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.

Prognosis • Serous retinal detachments typically resolve spontaneously in most

patients, with most 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.

• 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.

• http://www.aao.org/eyenet/article/diagnosing-managing-central-serous-chorioretinopat?February-2006

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