Case #40 – Best Vitelliform Macular Dystrophy

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Case 2: Autofluorescent Fundus Image OS In Best vitelliform macular dystrophy, the lesion is normally hyperautofluorescent in the early stages due to the accumulation of lipofuscin between the RPE and neurosensory retina.⁶ As the disease progresses and atrophy of the RPE occurs, the lesion becomes hypoautofluorescent often with surrounding areas of hyperautofluorescence.⁶

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Case 2: Autofluorescent Fundus Image OD Fundus autofluorescence revealed an area of hypoautofluorescence in the central macula and surrounding areas of hyperautofluorescence. The areas of hyperautofluorescence are due to elevated amounts of lipofuscin. The central zone of hypoautofluorescence is caused by the absence of lipofuscin, secondary to RPE atrophy.

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Case 2: Topcon 3D OCT Color Fundus Image OD Case 2: A 45-year-old male diagnosed with Best nine years prior presented for follow-up. He reported that his vision had worsened in both eyes since his last eye exam (in 2004) and now used a magnifier to read. He also described the vision

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Histological Section as Compared to the OCT Image  Although the Photoreceptor Integrity Line, or the PIL (defined as the junction between the inner and outer segments) is barely visible in most histological sections, it is highly prominent in normal SD OCTs. The PIL, as shown above, should be continuous throughout the entire scan in

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