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DS Boyer, EL Thomas, RP Gallemore, TG Chu, RL Novack; Ocular Coherence Tomography (OCT) In Best's Disease: Demonstration of a Sub-Retinal Pigment Epithelial (RPE) Translucent Space . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1188.
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© ARVO (1962-2015); The Authors (2016-present)
Purpose: To demonstrate the presence of an optically translucent sub-RPE space in Best's disease eyes which hyperfluoresces on fluorescein angiography (FA) and confirms previous histopathologic findings. Methods: Six eyes of three patients with Best's disease with mild to moderate visual loss ranging from 20/40 to 20/200 were studied with FA and OCT (Zeiss-Humphrey). Results: All eyes were noted to have localized, well circumscribed hyperfluorescence in the macular area on FA. The pattern was not typical of cystoid macular edema (CME) and there was no evidence of choroidal neovascularization (CNV). OCT showed an optically clear space more typical of RPE detachment than vascular leakage seen with either CNV or CME. These spaces appeared to fill on FA and could be measured with OCT in a range of 150-200 ums in depth. Cystoid spaces and intraretinal exudates did not develop. Typical FA and OCT studies from these eyes will be presented. Conclusion: By using OCT in Best's vitelliform dystrophy, an optically translucent space beneath the RPE has been identified. O'Gorman, et. al.,* have described post-mortem eyes with the sub-RPE deposition of an amorphous material made up of degeneration photoreceptors, lipofuchsin granules, outersegment debris and phagocytic cells. The OCT studies presented confirm the presence of a sub-RPE space consistent with previous histopathologic findings. *O'Gorman, S., W. A. Flaherty, et al. (1988). "Histopathologic findings in Best's vitelliform macular dystrophy." Arch Ophthalmol 106(9): 1261-8.
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