Autosomal dominant hereditary maculopathy with childhood-onset accumulation of LIPOFUSION in RETINAL PIGMENT EPITHELIUM. Affected individuals develop progressive central acuity loss, and distorted vision (METAMORPHOPSIA). It is associated with mutations in bestrophin, a chloride channel.
Also Known As:
Adult-Onset Vitelliform Macular Dystrophy; Best Disease; Best Macular Dystrophy; Best Vitelliform Macular Dystrophy; Best's Disease; Juvenile-Onset Vitelliform Macular Dystrophy; Vitelliform Macular Dystrophy, Adult-Onset; Vitelliform Macular Dystrophy, Juvenile-Onset; Adult Onset Vitelliform Macular Dystrophy; Bests Disease; Disease, Best; Disease, Best's; Dystrophies, Vitelliform Macular; Dystrophy, Best Macular; Dystrophy, Vitelliform Macular; Juvenile Onset Vitelliform Macular Dystrophy; Macular Dystrophies, Vitelliform; Macular Dystrophy, Best; Macular Dystrophy, Vitelliform; Vitelliform Macular Dystrophies; Vitelliform Macular Dystrophy, Adult Onset; Vitelliform Macular Dystrophy, Juvenile Onset