蝶形花纹营养不良:视网膜成像结果

Houda Bezza, Amine Mounsif, Zineb Algouti, Y. Bennouk, Houssaine Ait Lhaj, Mohamed Kriet, Fouad Elasri
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摘要

蝶形花斑营养不良(BPD)是一种遗传性黄斑疾病,其特征是色素/脂褐素在视网膜色素上皮细胞内堆积,可能会被误诊为老年性黄斑变性(AMD)。视网膜成像是鉴别诊断模式营养不良症的有用工具。在本报告中,我们描述了一名 64 岁男性的双眼变性和视力下降。眼底检查发现一个类似蝴蝶的色素脱失区。光学视网膜扫描(OCT)显示视网膜色素上皮(RPE)上方有眼底高反射沉积,而眼底自动荧光(FAF)显示低自荧光区被脂褐素沉积勾勒为高自荧光区。最后,荧光素血管造影(FFA)显示早期黄斑高荧光。
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Butterfly-shaped Pattern Dystrophy : Findings of Retinal Imaging
Butterfly shaped pattern dystophy (BPD) is a inherited macular disease which is characterized by the accumulation of pigment/lipofuscin in the retinal pigment epithelium, it might be misdiagnosed as age-related macular degeneration (AMD). Retinal imaging is a useful tool for the differential diagnosis of pattern dystrophy. In this report, we describe a 64 year old man presented metamorphopsia and reduced visual acuity in both eyes. Fundus examination showed an area of depigmentation delimited resembling a butterfly. The OCT revealed a subfoveal hyperreflective deposit above the retinal pigment epithelium (RPE) while fundus autofluorescence (FAF) shows hypoautofluorescent areas outlined by a lipofuscin deposits as hyperautofluorescent Finally, fluoroscein angiography (FFA) revealed early macular hyperfluorescence. 
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