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Angioid streaks in a case of Camurati-Engelmann disease
Bet L Tugcu1, Taha Sezer1, Ahmet Elbay1
1Department of Ophthalmology, Faculty of Medicine, Bezmialem Vakif University, Istanbul, Turkey.
Abstract:
Camurati-Engelmann disease (CED) is a rare autosomal dominant disease with various phenotypic expressions. The hallmark of the disease is bilateral symmetric diaphyseal hyperostosis of the long bones with progressive involvement of the metaphysis. Ocular manifestations occur rarely and mainly result from bony overgrowth of the orbit and optic canal stenosis. We report a case of CED showing angioid streaks (ASs) in both fundi with no macular involvement and discuss the possible theories of the pathogenesis of AS in this disease.
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