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卵黃樣黃斑部變性症-病例報告

Vitelliform Macular Dystrophy Case Report

摘要


卵黃樣黃斑部變性症乃遺傳性黃斑部變性症之一,在早期兩眼之眼底及視力完全正常,然隨病程之進行可出現典型的卵黃樣黃斑部及晚期的色素上皮退化,視力逐漸惡化,且兩眼病程並不一定為一致變化,本報告提出一例眼底左眼出現典型卵黃樣期,而右眼則已是晚期的卵黃樣破裂及色素沈積斑,視力皆在0.1以下無法矯正,螢光眼底照相左眼正常,右眼有色素上皮缺損,兩眼視野皆出現相對性中心暗點,色覺檢查方面兩眼皆有極輕微的藍綠色區域辨色不良,暗適應兩眼閥值上升,網膜電圖檢查兩眼正常,眼電圖檢查兩眼皆不正常,比值小於1.5。本例經過兩年藥物治療,視力無進展。

關鍵字

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並列摘要


The vitelliform macular dystrophy is an autosomal dominant disorder with variable penetrance and expressivity that is characterized by a typical ”vitelliform” or ”egg yolk” lesion. We present a case with a moderatedly advanced macular lesion on right eye and a typical vitelliform lesion on left eye. Fluorescein angiography showed a window defect on the advanced right eye and normal on the left. Color sense seemed to be normal or very mild blue-green confusion. Dark adaptation threshold increased on both eyes, visual field revealed relative central scotoma. ERG was normal, while EOG, L/D ratio below 1.5, was abnormal. There is no treatment available, but asymptomatic carriers can be usually detected by an abnormal EOG, which provides a valuable tool for genetic counseling.

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