Outer retina rupture from subretinal blood with spontaneous sealing and visual recovery in frosted branch angiitis from Familial Mediterranean Fever: A case report

Familial Mediterranean Fever (FMF) is a rare autoinflammatory disorder. Ocular involvement is rare and 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 LE was finger counting at 1.5 meters. “Mild frosting” with angiitis, hemi-central retinal vein occlusion (HCRVO) and acute outer retina rupture (ORR) were observed in the LE. Prompt systemic steroids were instituted and the ORR was sealed 2 weeks later while vision improved to 6/15 (near vision: J2) five 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.