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K. Fujinami, G. Hanazono, K. Tsunoda, K. Shinoda, Y. Miyake; A Case of Stargardt Disease With Localized Retinal Pigment Epithelium Hypertrophy at Fovea. Invest. Ophthalmol. Vis. Sci. 2009;50(13):5318.
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Stargardt disease is progressive retinal dystrophy characterized by onset during juvenile to young adult years, decreased central vision, diffuse atrophy of the retinal pigment epithelium (RPE) and orange-yellow flecks. Fluorescein angiography (FA) shows dark choroid due to lipofuscin accumulation in the RPE. Fundus autofluorescence (AF) images demonstrate a large oval area of reduced AF in the macula surrounded by small disseminated spots of reduced and increased AF. Optical coherent tomography (OCT) images represent decrease in macular volume. We encountered a case of a Japanese patient presenting ‘typical’ fundus appearance and dark choroid except ‘atypical’ AF findings and maintained function at the fovea.
Report of a case.
A 66-year-old-man was pointed out fundus abnormality in a clinical survey. He complained of photosensitivity and paracentral scotoma which had not progressed since his teens. The best corrected visual acuity was 20/20 OU. The funduscopy identified dark brown, well demarcated RPE hypertrophy at the fovea surrounded by RPE atrophy and orange-yellow flecks in the macula. FA showed blockage at the fovea, ring-shaped mottled hyperfluorescence in the macula and dark choroid. AF at the fovea was not reduced thereby appeared normal but surrounded by disseminated small spots of reduced and increased AF. OCT images represented preserved sensory retina, including inner/outer segment junction and thickened RPE layer at the fovea compared to the extra-macular area. Visual field testing detected bilateral paracentral scotoma within central 10-degree. The scotopic and photopic full-field ERGs were normal. While focal macular ERG elicited by 5-degree stimulus spot was well retained, ERGs by 10-degree and 15-degree spots were reduced.
Normal AF image at the fovea may suggest that RPE hypertrophy may be concerned in maintenance of retinal function even in this relatively old patient.
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