成人发病的单侧性Best黄斑营养不良（VMD2）。
Adult onset Best's macular dystrophy (VMD2) with unilateral presentation.
作者信息
Kamat Pradnya, Doshi Pratik, Prabhudesai Manasi, Prabhudesai Srishti
出版信息
Nepal J Ophthalmol. 2016 Jul;8(16):171-173. doi: 10.3126/nepjoph.v8i2.17014.
BACKGROUND
Vitelliform macular dystrophy is an autosomal-dominant disease and has two clinical variants: Best's (VMD2) and adult onset vitelliform macular dystrophy (AOVMD). We report an atypical presentation of VMD2.
CASE
A 50-year-old male presented with history of blurring of vision in left eye since two year. On fundus examination, left eye revealed a single, circular, yellow-opaque egg yolk-like macular lesion with no other abnormality. Fundus examination of right eye was unremarkable. Fundus fluoresceine angiography showed blocked fluorescence in the area of lesion. EOG-Arden ratios were found to be severely reduced bilaterally. OCT left eye showed sub-retinal hyperreflective echo.
CONCLUSION
The age of onset and presentation can be highly variable in VMD2 and the vitelliform phase of VMD2 in elderly can be confused for adult onset vitelliform macular dystrophy(AOVMD). However, Arden ratio less than 1.5 is diagnostic of VMD2.
背景
卵黄样黄斑营养不良是一种常染色体显性疾病，有两种临床变体：Best病（VMD2）和成人型卵黄样黄斑营养不良（AOVMD）。我们报告一例VMD2的非典型表现。
病例
一名50岁男性，自述两年以来左眼视力模糊。眼底检查发现，左眼有一个单一的、圆形的、黄色不透明的蛋黄样黄斑病变，无其他异常。右眼眼底检查无明显异常。眼底荧光血管造影显示病变区域荧光遮挡。双眼EOG-Arden比值均严重降低。左眼光学相干断层扫描显示视网膜下高反射回声。
结论
VMD2的发病年龄和表现可能有很大差异，老年人VMD2的卵黄样期可能与成人型卵黄样黄斑营养不良（AOVMD）相混淆。然而，Arden比值小于1.5可诊断为VMD2。
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