Best's disease (redirected from Vitelliform macular dystrophy)

Best's disease (bsts)

n.

Congenital degeneration of the macula of the retina occurring during the first few years of life.

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Best's disease Best's macular degeneration, vitelliform dystrophy Ophthalmology An AD retinal degeneration of variable penetration and early onset Findings Fundoscopic appearance ranges from mild pigment defects to a vitelliform–egg yolk–appearance in the central macula;
subsequent degeneration may result in subretinal neovascularization
and hemorrhage, and severe macular scarring Molecular pathology Mutation of bestrophin gene, which maps to chromosome 11. See Bestrophin.