Clinical Features, Investigations, Management, and Prognosis of Serpiginous Choroiditis

Abstract

Serpiginous choroiditis is an inflammatory form of posterior uveitis that affects the choroid, retinal pigment epithelium, and choriocapillaries. Known to be recurrent and progressive in nature, this bilateral disease can result in severe visual loss both during the active and healed stages. The age at onset is 40 to 50 years, but it can affect younger Indian population. It can be further classified into 3 types based on its clinical presentation, and they are, namely, peripapillary, macular, and ampiginous. Histological features further qualify the site of inflammation as evidenced by lymphocytic infiltration within the choroid and atrophy of choriocapillaries and retinal pigment epithelium. Despite a multitude of etiologies such as autoimmunity, infection, and degeneration, autoimmune causes remain the mainstay. Laboratory investigations can help rule out infections, whereas angiography can provide further insights into the progression of the disease. Newer investigations such as optical coherence tomography and fundus autofluorescence can help locate the lesion and identify the phase of the disease. Corticosteroids with immunosuppressives are the major management modality. Treatment of complications may require intravitreal injections, laser photocoagulation, or even vitreoretinal surgery.

Acute Vision Loss and Bilateral Macular Lesions

“…The choroidal inflammation presents as 1 of 3 patterns-classic, macular, or ampiginous (sometimes referred to as relentless placoid chorioretinitis). 2,4 The classic pattern is present in approximately 80% of cases and exhibits grayish or yellowish, serpentine subretinal infiltrates that begin in the juxtapapillary region and extend centrifugally. There may be overlying serous retinal detachments or retinal edema present.…”

“…1 The complications of serpiginous choroiditis include decreased central vision secondary to macular involvement, chorioretinal atrophy, choroidal neovascularization, subretinal fibrosis, retinal ischemia, retinal vascular occlusions, retinal neovascularization, and serous or pigment epithelial detachments. 1,2,4 Fluorescein and indocyanine green angiography may be helpful in diagnosing serpiginous choroiditis. Characteristic features in fluorescein angiography include early hypoflourescence in areas of atrophic choriocapillaris and late hyperfluorescence at the borders of the lesions.…”

“…Characteristic features in fluorescein angiography include early hypoflourescence in areas of atrophic choriocapillaris and late hyperfluorescence at the borders of the lesions. 2,4,5 Indocyanine green angiography exhibits hypofluorescence in areas of choriocapillaris nonperfusion throughout the early and late stages. 2,4,5 Optical coherence tomography may demonstrate outer retinal, choriocapillaris, and choroidal hyperreflectivity along with the disruption of the outer retinal layers, including the ellipsoid zone.…”

“…2,4,5 Indocyanine green angiography exhibits hypofluorescence in areas of choriocapillaris nonperfusion throughout the early and late stages. 2,4,5 Optical coherence tomography may demonstrate outer retinal, choriocapillaris, and choroidal hyperreflectivity along with the disruption of the outer retinal layers, including the ellipsoid zone. 5 Fundus autofluorescence is also helpful in monitoring the lesions, and the pattern of hyper-or hypofluorescence varies with the stages of lesion activity.…”

“…The anterior slitlamp examination is typically unremarkable and the eye frequently appears quiet with absent or minimal cell and flare in the anterior chamber or vitreous. The choroidal inflammation presents as 1 of 3 patterns—classic, macular, or ampiginous (sometimes referred to as relentless placoid chorioretinitis) . The classic pattern is present in approximately 80% of cases and exhibits grayish or yellowish, serpentine subretinal infiltrates that begin in the juxtapapillary region and extend centrifugally.…”