Pigmented Paravenous Retinochoroidal Atrophy: A Case Report

色素性靜脈旁視網膜脈絡膜變性－病例報告

蘇柏方(Po-Fang Su) ； 羅英源(Andy Y Lo) ； 李毅安(Yi-An Li) ； 蘇姵元(Pei-Yuan Su) ； 林思源(Szu-Yuan Lin) ； 陳慕師(Muh-Shy Chen)

《中華民國眼科醫學會雜誌》 47卷2期 (2008/06) Pp. 162-169

https://doi.org/10.30048/ACTASOS.200806.0010

摘要

目的：報告一個色素性靜脈旁視網膜脈絡膜變性的罕見病例，包括詳細的眼底紀錄及視網膜功能檢查。
方法：病例報告
結果：一位47歲女性，因為意外發現不典型的視網膜色素變性而轉介來門診。我們描述了四年的追蹤期間詳細的眼底影像紀錄、螢光眼底攝影、視網膜電生理、以及其他視網膜功能檢查。除了眼底的色素沉積增加之外，所有視網膜功能檢查在四年內並沒有明顯的變化。此外，在視網膜斷層掃描中發現，視網膜神經纖維層在週邊部有明顯變薄的情形，而且在週邊視網膜的色素表皮層有增生的變化。
結論：色素性靜脈旁視網膜脈絡膜變性是一種罕見的疾病，其特徵為延著視網膜靜脈的色素沉積。在四年的追蹤檢查中，除了眼底色素沉積增加之外，我們報告的病例在視網膜功能上並沒有明顯變化。

並列摘要

Purpose: To present a rare case of pigmented paravenous retinochoroidal atrophy with detail fundus and retinal function studies.
Method: Case report
Result: A 47-year-old woman was referred to our clinic due to an incidental finding of atypical pigmentation of the retina. A detailed photographic record, fluorescein angiography, electrophysiologic exam, and other retina functional studies are described over a course of four years. While retina functional studies remained similar, the patient's fundus showed increased pigment clumps overtime. Retinal analysis with optical coherence tomography showed generalized thinning of the retina and hyperplasia of the retinal pigmented epithelial layer in the mid-peripheral retina.
Conclusion: Pigmented paravenous retinochoroidal atrophy is a rare disorder of unknown cause characterized by bone corpuscle pigmentation along the retinal vein. Over the four-year follow-up period, our experience with this disease is that the patient's fundus showed accumulation of pigment clumps while retina functional studies remained similar overtime.

並列關鍵字

pigmented paravenous retinochoroidal atrophy