Angioid streaks in a case of Camurati–Engelmann disease

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...

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Detalles Bibliográficos
Autores principales: Tuğcu, Betül, Sezer, Taha, Elbay, Ahmet, Özdemir, Hakan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2017
Materias:
Brief Communications
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5549422/
https://www.ncbi.nlm.nih.gov/pubmed/28724827
http://dx.doi.org/10.4103/ijo.IJO_910_16
Descripción
Sumario: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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