Best Disease


File number: 130604


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    • By Marcelo Zas, MD PhD
      Hospital de Clinicas-University of Buenos Aires
      Co-author(s): Luciano Scorsetti MD, Pablo Chiaradia MD PhD, Juan Cortalezzi MD, Alejandro Tau MD, Marcos Mendaro MD, Mariano Cotic MD, Virginia Vittar MD, Natalia Gerschovsky MD, Enzo Dilascio MD
    • Uploaded on Apr 24, 2024.
    • Last modified by Joshua Friedman on Apr 24, 2024.
    Appears in
    Macular Dystrophy
    Luciano Scorsetti MD
    Best vitelliform macular dystrophy (BVMD) or Best disease. Is the most common autosomal dominant macular dystrophy. It involves the retinal pigment epithelium (RPE), and leads to a characteristic bilateral yellow “egg-yolk” appearance of the macula as you can see in this image. Essentially, BVMD is considered to have 6 clinical stages: Previtelliform, Vitelliform, Pseudohypopyon, Vitelleruptive, Atrophic and Choroidal neovascularization. As the disease progresses, patients may experience a slow, bilateral decrease in visual acuity, central scotoma, or metamorphopsia. With secondary CNV, visual decline can be rapid, however.

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