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Subretinal Fluid in Best’s Vitelliform Macular Dystrophy Associated With Abnormalities of Fundus Autofluorescence

¹ Ophthalmology, NYU Medical Center, New York, NY
² Ophthalmology, The LuEsther T. Mertz Retina Research Center of Manhattan Eye, Ear and Throat Hospital, New York, NY
³ Ophthalmology, The LuEsther T. Mertz Retina Research Center of Manhattan Eye, Ear and Throat Hospital, New York, NY, and Vitreous–Retina–Macula Consultants of New York, New York, NY., New York, NY

Commercial Relationships: A. Morgan, None; C.M. Eandi, None; K. Noble, None; K. Freund, None; R.F. Spaide, None.

Support: None.

Abstract

Purpose:

To examine the fundus autofluorescence optical coherence findings in patients with later stages of Best's disease.

Methods:

We performed a comprehensive examination of five patients with Best’s vitelliform macular dystrophy using fundus photography, fluorescein angiography, optical coherence tomography (OCT), and autofluorescence photography.

Results:

Five patients (3 male, 2 female) were examined. Mean age was 37.4 years (range 25 to 53 years). The OCT scans demonstrated the presence of subretinal fluid in every patient. Fluorescein angiography showed granular areas of transmission defect with focal leaks present in 3 patients. We observed that in later stages of vitelliform macular dystrophy the level of autofluorescence appears more widespread than what the ophthalmoscopic pictures would suggest and this lipofuscin showed a propensity to accumulate at the outer border of the neurosensory detachment.

Conclusions:

Our observations suggest that patients with later stages of Best’s disease have leakage from the level of the retinal pigment epithelium with accumulation of subretinal fluid. This is associated with lipofuscin accumulation extending toward the periphery.

Keywords: retinal pigment epithelium • macula/fovea • retinal degenerations: hereditary

© 2005, The Association for Research in Vision and Ophthalmology, Inc., all rights reserved. For permission to reproduce any part of this abstract, contact the ARVO Office at arvo{at}arvo.org.