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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. [ABSTRACT FROM AUTHOR]

Purpose: To report the epidemiology, etiology, ocular characteristics, treatment and visual outcome of pediatric uveitis in Israel.Methods: Retrospective study from two tertiary uveitis centers.Results: Included were 107 patients (182 eyes), 55% females. Mean age at diagnosis 8.8 years. Uveitis was predominantly anterior, idiopathic, bilateral, and chronic. Systemic associations were seen in 36% of patients of which the most common disease was juvenile idiopathic arthritis. Infectious uveitis accounted for 37% of posterior uveitis cases of which toxoplasmosis was the most common cause. Anterior segment complications were commonly observed at presentation (41%); the most predominant were posterior synechiae, cataract, and band keratopathy. The most common posterior segment complications were papillitis, epiretinal membrane, and macular atrophy/scar. Ninety-three percent of eyes had visual acuity >20/40 at last follow-up.Conclusion: The pattern of pediatric uveitis in Israel is similar to that in the western world. Visual outcome was good in most eyes. [ABSTRACT FROM AUTHOR]