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TitleAcute Macular Hole and Hemicentral Retinal Vein Occlusion in a girl with Familial Mediterranean Fever and Posterior Uveitis: 5-Year Follow-Up
PurposeFamilial Mediterranean Fever (FMF) is an autoinflammatory disorder predominantly affecting populations of Jewish, Arab, Armenian and Turkish descent. It manifests by sporadic episodes of fever and arthritis, which are self-limiting. Ocular involvement with uveitis is a rare manifestation and the full spectrum and response to treatment is yet to be understood.
MethodsAn 18 year-old Jewish girl, previously diagnosed with FMF, presented with sudden loss of vision in the left eye (LE). Best-corrected visual acuity in LE was finger counting at 1.5 meters and it was 6/6 in the right eye. Retinal vascular sheathing was noted nasally in LE and signs of hemi-central retinal vein occlusion with macular hole were observed. There were no signs of anterior uveitis or vitritis. The examination of the right eye was normal.
ResultsOptical coherence tomography documented left macular hole with subretinal fluid. Fluorescein angiography revealed areas of blocked fluorescence and vascular leakage in the superior retina, optic disc leakage and retinal ischemia around the macula. Extensive investigations ruled-out an underlying hypercoagulable predisposition. Prompt systemic steroids were instituted and the macular hole was sealed 2 weeks later while vision improved to 6/15 (near vision of J2) in 5 months. No recurrence observed over 5 years of follow-up.
ConclusionWe report a rare manifestation of posterior uveitis with concomitant hemi-central retinal vein occlusion and macular hole in a young girl with FMF. Complete macular hole closure was attained and vision recovered after the use of high-dose steroids.
Conflict of interestNo
Authors 1
Last nameVOFO
Initials of first name(s)BN
Authors 2
Last nameAmer
Initials of first name(s)R