Makati Ravie, Shechtman Diana, Besada Eulogio, Pizzimenti Joseph J
*BS †OD, FAAO Nova Southeastern University, Tampa, Florida (all authors).
Optom Vis Sci. 2014 Nov;91(11):e274-7. doi: 10.1097/OPX.0000000000000403.
Best vitelliform macular dystrophy, also known as Best disease, is a macular dystrophy characterized by bilateral yellowish egg yolk-like lesion(s) present within the maculae. It is a slowly progressive disease that usually presents at childhood. Best vitelliform macular dystrophy frequently proceeds through stages, beginning with a classic presentation described as vitelliform. A similar condition, known as adult-onset foveomacular vitelliform dystrophy, has been described among adult patients. Although the two maculopathies may look similar, they are considered two separate entities, because of the age of onset and overall clinical presentation.
A 54-year-old man presented with gradual-onset blurred near vision in each eye. Previous records showed a history of unremarkable dilated fundus examinations for the past 8 years. Best-corrected distance acuities measured 20/20 OD and 20/20 OS. Amsler grid testing revealed a mild metamorphopsia OD and OS. Dilated fundoscopy revealed macular pseudohypopyon in each eye. In vivo imaging of the maculae was obtained with spectral-domain optical coherence tomography findings. Electrooculography findings were consistent with Best vitelliform macular dystrophy of atypical, late onset.
Best vitelliform macular dystrophy may vary in its presentation. Electrooculography and spectral-domain optical coherence tomography can aid in establishing the definitive diagnosis.
最佳卵黄状黄斑营养不良，也称为Best病，是一种黄斑营养不良，其特征为黄斑区出现双侧淡黄色蛋黄样病变。它是一种缓慢进展的疾病，通常在儿童期发病。最佳卵黄状黄斑营养不良常经历多个阶段，始于典型的卵黄样表现。在成年患者中描述了一种类似的情况，称为成人型中心凹黄斑卵黄状营养不良。尽管这两种黄斑病变可能看起来相似，但由于发病年龄和整体临床表现，它们被认为是两个不同的实体。
一名54岁男性，双眼逐渐出现近视力模糊。既往记录显示，在过去8年中，散瞳眼底检查结果均无异常。最佳矫正远视力右眼和左眼均为20/20。Amsler方格表检查显示右眼和左眼均有轻度视物变形。散瞳眼底检查发现双眼黄斑部有假性前房积脓。通过光谱域光学相干断层扫描成像获得了黄斑的体内图像。眼电图检查结果与非典型、迟发性最佳卵黄状黄斑营养不良一致。
最佳卵黄状黄斑营养不良的表现可能有所不同。眼电图和光谱域光学相干断层扫描有助于明确诊断。
