Open AccessDiffuse Outer Layer Opacification: A Novel Finding in Patients With Autosomal Recessive Bestrophinopathy
Author(s) -
Emily Witsberger,
Alan D. Marmorstein,
José S. Pulido
Publication year - 2019
Publication title -
asia-pacific journal of ophthalmology
Language(s) - English
Resource type - Journals
ISSN - 2162-0989
DOI - 10.1097/apo.0000000000000261
Subject(s) - medicine , retinal pigment epithelium , fundus photography , ophthalmology , retinal , fluorescein angiography , fundus (uterus) , posterior pole
Autosomal recessive bestrophinopathy (ARB) is a rare inherited retinal dystrophy resulted from mutations in bestrophin-1 (BEST1) which affect functioning of the retinal pigment epithelium (RPE). Descriptions of disease findings in patients with ARB to date have focused only on macular changes. In this case series, we report previously undescribed mid-peripheral retinal changes occurring in 4 patients with ARB.