BEST1 and Best vitelliform macular dystrophy: [12] Other described phenotypes associated with pathogenic variants of the BEST1 gene include Best vitelliform macular dystrophy (BVMD) [17, 18], adult vitelliform macular dystrophy (AVMD), autosomal dominant vitreoretinochoroidopathy (ADVIRC), [19, 20] autosomal dominant microcornea, rod-cone dystrophy, early-onset cataract, and posterior staphyloma (MRCS) [11], rod-cone dystrophy and retinitis pigmentosa [11, 21].