BASICS

DESCRIPTION

• Central serous chorioretinopathy, also called central serous retinopathy or idiopathic central serous retinopathy, is an ocular condition characterized by:

– Serous retinal detachment, typically seen in the macular region

– One or more retinal pigment epithelial detachments, which are generally less than 300 microns across, are responsible for the leakage of plasma into the subretinal space.

– Signs and symptoms are limited to the eye.

– Rarely, bullous central serous chorioretinopathy can be associated with a large serous retinal detachment with shifting subretinal fluid. Retinal pigment epithelial leaks in this variant are typically greater than 500 microns across.

EPIDEMIOLOGY

Incidence

• Men outnumber women by a factor of 6:1.

• The mean annual age-adjusted incidence per 100,000 for men is 9.9 (95% confidence interval [CI], 7.4–12.4).

• The mean annual age-adjusted incidences per 100,000 for women is 1.7 (95% CI, 0.7–2.7).

• Approximately two-third of fellow eyes followed for 10 years demonstrate some evidence, (although often asymptomatic) of central serous chorioretinopathy.

• The entity is most typically encountered in patients aged 25–50 years.

• Recurrence:

– Approximately 30% of cases will experience a recurrence

– The median time of recurrence is 1.3 years

– Recurrences have been seen from 3 months to 18 years

RISK FACTORS

• Use of corticosteroids

• Type “A” personality profile

• Cushing’s disease

Genetics

No genetic or hereditary association has routinely been described.

GENERAL PREVENTION

• Since central serous chorioretinopathy appears to be associated with the classic type A personality profile, patients should at least be informed of the association.

• Patients should be cautioned about the association of glucocorticosteroid use and central serous chorioretinopathy.

PATHOPHYSIOLOGY

A retinal pigment epithelial detachment, or other leak at the retinal pigment epithelium level, facilitates the leakage of plasma into the subretinal space.

ETIOLOGY

• Plasma from the choroid travels through damaged retinal pigment epithelium and into the subretinal space.

• The cause of the leak is uncertain, although glucocorticosteroids have been implicated in select cases.

• An association with Helicobacter pylori was noted in one paper, but this association needs confirmation.

COMMONLY ASSOCIATED CONDITIONS

• Central serous chorioretinopathy is often associated with a “type A” personality profile

• Recent use of glucocorticosteroids

DIAGNOSIS

• A serous detachment of the sensory retina is seen in the macular region with ophthalmoscopy.

• Subretinal precipitates can be seen on the underside of the retina in select cases.

• The presence of subretinal and/or intraretinal blood rules out the diagnosis of central serous chorioretinopathy.

• The anterior segment is uninvolved.

• Intravenous fluorescein angiography discloses one or more leaks at the level of the pigment epithelium. The leaks are often difficult to see ophthalmoscopically.

• Leaks can occur superiorly to the posterior pole view. Thus, the fluorescein angiographic photographer should take at least 1 frame in this region.

HISTORY

• Patients most often complain about a gray or black spot centrally

• Micropsia is also often present

• Metamorphopsia is present in two-third of affected eyes

• A history of severe stress (losing a loved one, a house, or a job) may be present

• The use of some form of corticosteroid (oral, injected, or topical) may be present

PHYSICAL EXAM

• Ophthalmoscopic examination demonstrates a serous detachment of the sensory retina, most often involving the central macular retina.

• Subretinal precipitates may be seen on the underside of the detached sensory retina.

• Retinal or subretinal blood is not seen with this entity. If blood is present, consider neovascular macular degeneration.

DIAGNOSTIC TESTS & INTERPRETATION

Diagnostic Procedures/Other

• Intravenous fluorescein angiography typically demonstrates a focal area of hyperfluorescence corresponding to a retinal pigment epithelial detachment leaking plasma into the subretinal space.

– A “smokestack” pattern of leakage can be seen as the fluorescein dye rises within the subretinal space in 20% of cases.

– Fluorescein leakage may be sufficient to produce hyperfluorescence throughout the entire retinal detachment.

– In more chronic cases, one or more linear streaks of “guttering”, characterized by retinal pigment changes can be seen. This occurs secondary to chronic subretinal fluid tracking inferiorly.

• Optical coherence tomography generally reveals a retinal pigment epithelial detachment associated with a larger area of overlying serous retinal detachment.

Pathological Findings

A serous, sensory retinal detachment associated with a smaller retinal pigment epithelial detachment.

DIFFERENTIAL DIAGNOSIS

• Entities in the differential diagnosis for central serous chorioretinopathy include those which can produce serous detachment of the sensory retina in the posterior pole.

– Neovascular macular degeneration, age-related or from other causes

– Polypoidal choroidal vasculopathy

– Subretinal or intraretinal fluid associated with a congenital pit of the optic nerve head

– Shallow rhegmatogenous retinal detachment

– Severe hypertensive retinopathy with serous retinal detachment due to hypertensive choroidopathy

– Posterior retinal detachment with macular hole

– Posterior retinal detachment with high myopia and posterior retinal break

– Harada’s disease

– Nanophthalmos with serous retinal detachment

– Serous retinal detachment associated with choroidal metastasis

– Serous retinal detachment associated with choroidal melanoma

• Among patients with a diagnosis of Cushing’s disease, 5% of patients have one or more episodes of documented central serous chorioretinopathy. The cases seem to occur during episodes of hypercortisolism.

TREATMENT

ADDITIONAL TREATMENT

General Measures

• Approximately 85% of cases demonstrate spontaneous reabsorption of the subretinal fluid within 4 months.

• Laser photocoagulation can be considered in those cases in which the subretinal fluid has not reabsorbed within 3–4 months.

– Laser photocoagulation results in reabsorption of the subretinal fluid 2 months earlier than no therapy, although the visual results are similar with treatment and without treatment in a Level A randomized, clinical trial by Robertson and Ilstrup.

– Light laser photocoagulation using 100–200 micron spot sizes can be applied to the leaking retinal pigment epithelial detachment(s).

– Follow-up by Yap and Robertson at 11–15 years disclosed that none of 6 eyes treated with laser photocoagulation had a recurrence, while 17/32 (53%) eyes that did not undergo laser therapy had at least 1 recurrence.

– Remember that the laser scars can enlarge considerably over a decade or more, resulting in late vision loss.

• In instances in which the leak is closer than 750 microns from the center of the foveal avascular zone, half fluence photodynamic therapy can be utilized.

• Intravitreal bevacizumab has also been shown to be of therapeutic benefit (Level B non-randomized clinical trial, Artunay et al.).

SURGERY/OTHER PROCEDURES

Surgery has not been shown to be of benefit.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Ophthalmologist

• Low vision if vision is decreased in both eyes.

• Annual visits if stable.

PROGNOSIS

• Wang et al. noted that over 90% of eyes regain vision of 20/30 or better by 6 months.

• Although most retina specialists treat leaks at the level of the retinal pigment epithelium if the serous retinal detachment is still present at 3–4 months, a long-term prospective clinical trial with Level I evidence (type 1 error <0.05, and type 2 error <0.20) does not exist.

• At 10 years, persistence of a retinal pigment epithelial detachment, persistent subretinal fluid, and recurrences resulted in a greater chance of having vision less than 20/40.

COMPLICATIONS

It is questionable whether laser therapy results in a higher incidence of subsequent choroidal neovascularization.

ADDITIONAL READING

• Kitzmann AS, Pulido JS, Diehl NN, et al. The incidence of central serous chorioretinopathy in Olmsted County, Minnesota, 1980–2002. Ophthalmology 2008 Jan;115(1):169–173.

• Bouzas EA, Scott MH, Mastorakos G, et al. Central serous chorioretinopathy in endogenous hypercortisolism. Arch Ophthalmol. 1993;111:1229–1233.

• Robertson DM, Ilstrup D. Direct, indirect, and sham laser photocoagulation in the management of central serous chorioretinopathy. Am J Ophthalmol 1983;95:457–466.

• Yap EY, Robertson DM. The long-term outcome of central serous chorioretinopathy. Arch Ophthalmol 1996;114(6):689–692.

• Lim JW, Kim MU. The efficacy of intravitreal bevacizumab for idiopathic central serous chorioretinopathy. Graefes Arch Clin Exp Ophthalmol. 2011;249(7):969–974.

• Gemenetzi M, De Salvo G, Lotery AJ. Central serous chorioretinopathy: An update on pathogenesis and treatment. Eye (Lond). 2010;24(12):1743–1756.

• Artunay O, Yuzbasioglu E, Rasier R, et al. Intravitreal bevacizumab in treatment of idiopathic persistent central serous chorioretinopathy: A prospective, controlled clinical study. Curr Eye Res. 2010;35:91–98.

• Loo RH, Scott IU, Flynn HW Jr, et al. Factors associated with reduced visual acuity during long-term follow-up of patients with idiopathic central serous chorioretinopathy. Retina 2002;22(1):19–24.

• Wang M, Munch IC, Hasler PW, et al. Central serous chorioretinopathy. Acta Ophthalmologica 2008;86:126–145.

CODES

ICD9

362.41 Central serous retinopathy

CLINICAL PEARLS

• Be certain to look at the optic nerve with slit-lamp biomicroscopy to rule out the possibility of a serous, macular retinal detachment occurring secondary to a congenital pit of the optic disc. Pits of the optic disc can be missed with indirect ophthalmoscopy.

• If any retinal blood and/or subretinal blood is present, the most probable diagnosis is choroidal neovascularization.

• Chronic central serous retinopathy may eventually lead to neovascular age-related macular degeneration years later.

• Consider early, aggressive laser therapy to the larger retinal pigment epithelial leaks seen in association with bullous central serous chorioretinopathy.

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