Diffuse subretinal fibrosis syndrome: A rare entity

Abstract

Background/ purpose:

Diffuse subretinal fibrosis and uveitis syndrome is an inflammatory disease rarely reported. The purpose of this article is to describe a rare case of diffuse subretinal fibrosis syndrome in an 8-year-old child.

Methods and results:

The patient initially presented with a loss of vision in his right eye, with a visual acuity of 20/200. The visual acuity of the left eye was normal. Clinical examination showed bilateral anterior and posterior inflammation while the fundus revealed on the right eye an inter maculopapillar fibrosed lesion with a discrete retinal hemorrhage and a similar but smaller lesion on the left eye. The optical coherence tomography showed hyper-reflective material compatible with pre-retinal and subretinal pigment epithelial fibrosis and associated subretinal fluid. The angiography facilitated the diagnosis of neovascularization that was associated with the fibrotic lesion. The rest of the clinical pediatric examination remained negative and a diagnosis of diffuse subretinal fibrosis syndrome complicated with neovascularization was made. Treatment with systemic corticosteroids at the dose of 1 mg/kg/day for 1 month with a progressive decrease of 5 mg per month allowed for both anatomical and functional improvement. The visual acuity of the right eye improved from 20/200 to 20/63 at 1 month, 20/32 at 2 months, and 20/20 at 4 months.

Conclusion:

To our knowledge, this is the first reported case of bilateral diffuse subretinal fibrosis complicated by neovascularization. General corticosteroid therapy proved to be effective in this case and appears to be a viable option in first-line treatment.