Open AccessAngioid streaks in a case of Camurati–Engelmann disease
Author(s) -
Betül Tuğcu,
Taha Sezer,
Ahmet Elbay,
Hakan Özdemir
Publication year - 2017
Publication title -
indian journal of ophthalmology/indian journal of ophthalmology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 51
eISSN - 1998-3689
pISSN - 0301-4738
DOI - 10.4103/ijo.ijo_910_16
Subject(s) - medicine , angioid streaks , hyperostosis , anatomy , pseudoxanthoma elasticum , pathology , disease , ophthalmology , retinal , choroidal neovascularization
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.