Vitelliform Macular Dystrophy

**Definition:** 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.

**Tree numbers:** - C11.768.585.439.433 - C16.320.290.763

**Synonyms:** - Best Macular Dystrophy - Best Vitelliform Macular Dystrophy - Best's Disease - Best Disease - Macular Dystrophy, Vitelliform - Vitelliform Dystrophy - Macular Degeneration, Polymorphic Vitelline