Vitreous fluorophotometry in patients with Best's macular dystrophy.
Ten patients with Best's macular dystrophy were examined with vitreous fluorophotometry and results were compared with a normal population. Seventeen of the 20 affected eyes demonstrated an intact blood-retinal barrier with normal inward permeability of fluorescein dye. Despite diffuse functional impairment of the retinal pigment epithelium (RPE), determined by electro-oculography, as well as accumulation of a lipofuscin or lipofuscin-like substance within virtually all RPE cells, the blood-retinal barrier function of these cells remained intact as determined by clinical fluorophotometry. Similar findings have previously been noted in another hereditary retinal disorder (fundus flavimaculatus) in which a lipofuscin-like substance also accumulates diffusely within RPE cells.
Fishman
G A
GA
Department of Ophthalmology, University of Illinois, College of Medicine, Chicago 60612.
Ward
L M
LM
Rusin
M M
MM
D000293:Adolescent; D000328:Adult; D000368:Aged; D001813:Blood-Retinal Barrier; D002648:Child; D005260:Female; D019793:Fluorescein; D005452:Fluoresceins; D015590:Fluorophotometry; D006801:Humans; D008268:Macular Degeneration / Q000503:physiopathology*; D008297:Male
D005452:Fluoresceins; D019793:Fluorescein