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先天性視網膜分裂－八病例的臨床觀察
Congenital Retinoschisis Clinical Observations of 8 Cases
摘要
本文收集本院自民國六十九年至七十七年間，八個先天性視網膜裂的病人。追踪期間從2個月到八年不等。八例全為男性，且都兩眼發病。視力由僅辨光覺到0.4。所有病例皆有視網膜中心凹及玻璃體變化。其中有75%有典型車輪狀視網膜中心凹病變。有40%有家族史可循。追踪期間並從事各項檢查，暗適應檢查，所有病人都呈桿狀細胞閥值升高，視野檢查在相對於視網膜分來處，呈現相對或絕對性的視野缺損。螢光眼底攝影皆呈黃斑部超透螢光，有些病人則有樹枝狀血管及螢光滲出和血浪不灌流區。E.R.G.檢查所有病人b波皆顯著下降，E.O.G.明暗比也下降。色覺呈現一般性不正常，並無特定色覺異常。綜合各項檢查，配合臨床觀察，提出報告。
關鍵字
無資料並列摘要
Congenital retinoschisis is inherited by male as an X-linked mendelian recessive trait. Changes are mainly confined to the fovea, retinal periphery and vitreous. Histopathological studies demonstrated nerve fiber layer splitting resulted in schisis. Vision is impaired early in the life, and remains stationary for years. Eight cases have been collected during 1980-1988. All are males and bilaterally affected. Typical stellate foveal lesions were noted in 75% of affected eyes. Peripheral retinoschisis was found in 62.5% of patients and temporal lower retina was the most common site iovolved. All cases have vitreous changes. Inner layer holes were noted in 31% of affected eyes, but only one has an outer layer hole with localized retinal detatchment. F.A.G. revealed macula hyperfluorescence (62.5%) and dendritic vessels (31.0%) Decreased b/a ratio was noted in E.R.G.. Abnormal color sense, visual field defects and elevated rod threshold in dark adaptation was also noted.