卵黄样黄斑营养不良又称 Best病 ,为常染色体显性遗传性疾病 ,发病年龄多在 3～ 15岁 (平均 6岁 )。本病为双眼患病 ,两侧对称 ,少数病人可两眼先后发病。在病情进展过程中 ,形态变化很大 ,视力受损程度也不同。现将我们发现的同胞姐弟两例的临床表现、荧光素眼底血管造影 (fun Yolk-like macular dystrophy, also known as Best disease, is an autosomal dominant genetic disease, the onset of age more than 3 to 15 years (mean 6 years). The disease is binocular disease, bilateral symmetry, a small number of patients may have two eyes disease. In the process of disease progression, the morphological changes are great, the degree of visual impairment is also different. Now we found the siblings two cases of clinical manifestations, fluorescein fundus angiography (fun