Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children, London, United Kingdom; Manchester Metropolitan University, Manchester, United Kingdom; UCL-GOSH Institute of Child Health, University College London, London, United Kingdom.
Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children, London, United Kingdom.
J AAPOS. 2020 Dec;24(6):384-386. doi: 10.1016/j.jaapos.2020.08.003. Epub 2020 Oct 22.
Grouped congenital hypertrophy of the retinal pigment epithelium is a conspicuous ocular anomaly wherein highly pigmented, demarcated but flat retinal lesions arise from the retinal pigment epithelium. These lesions ("bear tracks") typically increase in size as they approach the retinal periphery. The discovery of pigmentary lesions in a young infant with a poor red reflex warrants urgent ophthalmological and electrodiagnostic review to exclude serious diagnoses, including an early-onset severe retinal dystrophy. We present the case of a 2-month-old boy with marked bear-tracks over the entirety of each retina, but with normal electrodiagnostic findings, genetics, and visual behavior.
视网膜色素上皮先天性团块性肥大是一种明显的眼部异常，其特征是来自视网膜色素上皮的高度色素沉着、边界清楚但扁平的视网膜病变。这些病变（“熊迹”）通常在接近视网膜周边时增大。在一个红反射差的年轻婴儿中发现色素性病变，需要紧急眼科和电诊断复查以排除严重诊断，包括早期严重视网膜营养不良。我们介绍了一例 2 个月大的男孩，其每只眼睛的视网膜上都有明显的“熊迹”，但电诊断、遗传学和视觉行为均正常。
