Figure 1. FAF. Click image to enlarge.

Presentation

A 42-year-old Jamaican male presents with complaints of decreased vision in the right eye for three months. Medical history was unremarkable, and he had not been taking any oral medications recently. Best-corrected visual acuity (BCVA) was 20/200 OD and 20/20 OS. Dilated fundus exam revealed the presence of mild retinal pigment epithelium (RPE) pigmentary changes as well as subretinal fluid. SD-OCT revealed a large area of hyporeflective subretinal fluid encompassing the macula as well as an associated pigment epithelial detachment. Fluorescein angiography (FA) revealed the presence of a “smokestack” pattern of hyperfluorescence within an area of subretinal fluid. Given the three-month duration, a decision was made to start the patient on

spironolactone 25mg PO BID. Prior to starting treatment, potassium levels and kidney function were evaluated. At the one-month follow-up, BCVA improved to 20/40 and OCT showed resolution of most of the subretinal fluid with only the presence of a large serous RPE detachment. By two months, there was almost complete resolution of both subretinal and RPE serous detachments with an overall improvement of about 200μm of retinal thickness. Spironolactone was discontinued by month three with no recurrence documented to date.

ICSC

Idiopathic central serous chorioretinopathy (ICSC) typically affects young (20- to 50-year-old) males. Hence, an older patient presenting with ICSC should be evaluated further for conditions such as wet AMD. Clinical presentation is associated with some degree of visual impairment which may be described as micropsia, blurry vision from a corresponding hyperopic shift, scotomas, metamorphopsia, reduced color vision or contrast sensitivity. Fundus examination reveals the presence of a unilateral blister-like serous macular detachment. This is due to increased permeability of the underlying choroidal vessels and association with retinal pigment dysfunction, leading to a serous detachment of the overlying neurosensory retina.

Common risk factors include stress, type A personality, sleep apnea and use of exogenous corticosteroids or sympathomimetic products. The disease may be recurrent in 10% cases and may even include a bilateral and/or multifocal presentation. Bilateral conditions tend to be seen in older patients with more diffuse RPE changes. Although most CSC are idiopathic, other potential ocular etiologies such as age-related macular degeneration, myopic choroidal neovascularization (CNV), vascular retinopathy or underlying systemic conditions such as pregnancy, Cushion’s disease and Vogt-Koyanagi-Harada may be considered in atypical presentations.

Figure 2. Earlier and late FA photo. Click image to enlarge.

Diagnostic Modalities 

Common diagnostic modalities used in the assessment of ICSC include fundus photography, OCT, fundus autofluorescence (FAF) and FA. Fundus photography shows a dome like blister within the macula. RPE mottling may be seen in more chronic or recurrent cases. Although bullous serofibrinous exudative retinal detachment may be associated with ICSC, the presence of exudates and hemorrhages need further evaluation for a concomitant CNV.

OCT findings include hyporeflective neurosensory retinal detachment with or without the presence of an RPE serous detachment. At times, serous fibrinous hyperreflective material may be seen in the subretinal space. Concomitant RPE disruption is more common for chronic or recurrent cases. The advancement of OCT and the use of enhanced depth imaging have led to the identification of increased choroidal thickness, which may be observed in the uninvolved eye. The term pachychoroid is used to describe such thickness.

Other diagnostic modalities, such as FAF, are particularly useful in revealing areas of guttering. This is described as fluid vertical track marks associated with hyper- and hypofluorescence, signifying decompensated RPE in chronic cases.

As a practicing retinal specialist, Jose Danny Diaz, MD, finds FA to be very useful in the assessment of ICSC. FA findings include classic smokestack or expansile dot patterns of hyperfluorescence and progressive leakage due to focal damage to the RPE. There can also be evidence of pooling into the area of the subretinal fluid. 

Management

Figure 3. OCT. Click image to enlarge.

In most cases, the disease is self-limited and can spontaneously resolve over a period of one to four months. These cases are described as acute and patients can be followed up with recommendations for lifestyle modifications. Yet, five to 10% of cases can be chronic (lasting more than three to six months) and /or recurrent. Such cases may be associated with permanent vision loss due to subretinal fibrosis, RPE atrophy and/or due to the presence of a secondary choroidal neovascular membrane. Management of such cases includes options such as focal laser photocoagulation, photodynamic therapy and potentially the use of oral mineralocorticoid receptor antagonists. Dr. Diaz often chooses one of the laser options, given the extensive research collaborative to the efficacy of the treatment management. Subthreshold micropulse has been implemented in attempts to reduce the complications associated with lasers. A variety of systemic treatments have also been used in the treatment of ICSC. The most common treatment options include mineralocorticoid receptor antagonists (spironolactone or eplerenone), which in some reports have shown to be effective. Anti-VEGF therapy is typically reserved for cases with concomitant CNV.

ICSC is a common ocular condition seen in practice today. While the condition is typically self-limited, atypical cases and chronic and/or recurrent presentations may warrant further evaluation as well as intervention.

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