Diffuse retinal pigment epithelial disease in an adult with cystic fibrosis

Abstract

An adult with cystic fibrosis presented for decreased vision in the right eye of several months' duration. Biomicroscopy and fluorescein angiography demonstrated bilateral large subretinal drusenoid deposits concentrated temporal to the fovea, as well as a small subretinal hemorrhage associated with focal leakage of fluid within the macula in the right eye. Optical coherence tomography demonstrated subretinal fluid consistent with choroidal neovascular membrane in the right eye with the absence of fluid in the left eye. The retinal manifestations of bilateral diffuse drusen associated with choroidal neovascular membrane may be consistent with autosomal dominant drusen, age-related macular degeneration, and type II glomerulonephritis, or alternatively may be a unique finding associated with cystic fibrosis. To the authors' knowledge, these findings have not previously been described in association with cystic fibrosis.