Outer Retina Rupture from Subretinal Blood with Spontaneous Sealing and Visual Recovery in Frosted Branch Angiitis from Familial Mediterranean Fever: A Case Report

Abstract

Familial Mediterranean fever (FMF) is a rare autoinflammatory disorder. Ocular involvement is rare. The full spectrum and response to treatment is poorly understood. An 18-year-old girl previously diagnosed with FMF presented with sudden loss of vision in the left eye (LE). Best-corrected visual acuity (BCVA) in the LE was finger counting at 1.5 meters. Angiitis with mild “frosting,” hemi-central retinal vein occlusion (HCRVO), and acute outer retina rupture (ORR) were observed in the LE. Systemic steroids were initiated immediately. The ORR was sealed 2 weeks later while vision improved to 6/15 (near vision: J2) 5 months later. No recurrences were observed over 5 years of follow-up. We report a rare manifestation of frosted branch angiitis with concomitant HCRVO and ORR in a young patient with FMF. Closure of ORR was attained and vision recovered after treatment with high-dose steroids.

Keywords

• frosted branch angiitis
• outer retina rupture
• hemicentral retinal vein occlusion
• familial mediterranean fever
• posterior uveitis
• retinal vascular sheathing
• sudden loss of vision