Juvenile Retinoschisis-Case Report

幼年型視網膜分離症－病例報告

周師宇(Shih-Yu Chou) ； 蔡德中(Der-Chong Tsai) ； 李鳳利(Fenq-Lih Lee) ； 許紋銘(Wen-Ming Hsu)

《中華民國眼科醫學會雜誌》 42卷4期 (2003/12) Pp. 426-430

https://doi.org/10.30048/ACTASOS.200312.0006

摘要

本文報導兩例罹患幼年型視網膜分離症的男性個案，病例一為一現年十歲男性，主訴自七歲起便有視力較模糊之現象，眼底檢查發現雙眼黃斑部均有如鈍齒輪般之黃斑部病變，同時並伴隨有下側顳部區域之視網膜分離表現。在視網膜分離的空腔上，可發現許多明顯內層裂孔分佈其中。病人最佳矯正視力右眼為零點二、左眼為零點三。在連續三年的追蹤期間，均沒有視網膜剝離或玻璃體出血的現象產生。病例二則為現年二十一歲男性，首度發現視力模糊時為九歲，可見雙眼黃斑部有典型之車輪輻射狀黃斑部變化，同時可見雙眼顳側上下邊緣有視網膜分離現象。以眼底視網膜斷層掃描(OCT)檢查，可見黃斑部下有明顯水泡狀變化，此病人雙眼最佳矯正視力迄今均維持零點二。雖然這兩位病人均無明顯可追溯之家族病史，但仍需提醒其性聯隱性遺傳的可能性，並持續追蹤。

並列摘要

Purpose: To report clinical features and characteristics of two male cases with juvenile retinoschisis. Method: cases report. Result: Two male cases of juvenile retinoschisis were reported. One was a 10-year-old male with complaints of blurred vision for 3 years. On examination, fundus revealed wheel-like maculopathy associated with schisis involving temporal lower quadrant in both eyes. We could see the presence of multiple inner holes overlying the schisis cavity. The best-corrected visual acuity of both eyes were 6/30 CD and 6/20 OS respectively. During 3 years follow up, no retinal detachment or vitreous hemorrhage developed. The other case showed typical cogwheel appearance in both foveas. Schisis cavities were present at temporal periphery and temporal upper quadrant respectively. The optical coherence tomography demonstrated cystic space in both foveas. The best-corrected visual acuity were 6/30 in both eyes. Conclusion: Juvenile retinoschisis is an uncommon X-linked hereditary vitreoretinal degenerative disease. Foveal schisis is noted in almost all of cases. All the patients need long term follow-up and adequate intervention with surgery or photocoagulation to avoid the developing risk of retinal detachment and vitreous hemorrhage.

並列關鍵字

juvenile retinoschisis ； maculopathy ； inner holes ； retinal detachment ； vitreous hemorrhage