色素性靜脈旁脈絡膜網膜變性－病例報告

Pigmented Paravenous Chorioretinal Atrophy-Case Report

袁漢良(Hon-Leung Yuen) ； 周清光(Ching-Kuang Chou) ； 劉榮宏(Jorn-Hon Liu)

《中華民國眼科醫學會會刊》 23卷 (1984/05) Pp. 396-402

https://doi.org/10.30048/ACTASOS.198405.0067

摘要

本篇報告一個單側的色素性靜脈旁脈絡膜網膜變性的女性病例，初診時，在患者右眼眼底發現，除了典型的靜脈旁脈絡膜網膜變性外，還有網膜出血，滲出物等炎症反應，而半年後，出血和滲出物均自動吸收；右眼視野稍為狹窄且有楔狀缺損；眼底螢光血管攝影在視神經盤周圍及靜脈旁有網膜色素上皮缺損及脈絡膜萎縮；暗適應輕度異常；視力與色覺也始終保持良好。患者沒有全身性症狀；實驗室檢查發現除了抗核抗體價達1:256較高外，其他都正常。患者經過半年的門診追踪，一切情況穩定。
色素性靜脈旁脈絡膜網膜變性過去共有36例報告，本例特點為單側性，而且初期有發炎現象，與其他報告稍有不同。

並列摘要

A 48-year-old female with unilateral pigmented paravenous chorioretinal atrophy is presented. In addition to the classical fundus appearance of sharply circumscribed patches of retinochoroidal atrophy and pigmentation along the retinal veins, retinal hemorrhage and exudation were noted at early stage. The other eye was entirely normal. Fluorescein angiography confirmed the presence of peripapillary and paravenous retinal pigment epithelial defect with choroidal atrophy. The visual field of the affected eye showed mild constriction with wedge defect on the nasal side. Dark adaptation showed normal rod threshold but loss of cone/rod break. The patient didn't have any systemic disorders. Laboratory data revealed ANA titer to be 1:256.
This is the first reported case of this entity showing positive inflammatory signs in the fundus and positive ANA titer, thus strongly suggests that this disease is the result of an inflammatory process, or an autoimmue reaction rather than a degenerative nature.

並列關鍵字

retinal hemorrhage ； exudation ； fluorescein angiography ； visual field ； ANA titer