Coats病様眼底変化を生じた網膜色素変性症の1例

Fundus manifestations simulating Coats disease in a case of retinitis pigmentosa

要約　目的：Coats病に似た眼底を呈し，硝子体出血が生じた網膜色素変性症の症例の報告。症例：61歳男性が左眼視力障害で受診した。13歳で夜盲を自覚し，17歳のときに網膜色素変性症と診断された。兄2人と従兄弟1人に網膜色素変性症がある。4年前に白内障手術を受け，左右とも0.04の視力であった。所見：矯正視力は右0.03，左指数弁で，右眼に網膜色素変性症の所見，左眼に硝子体出血と眼底上方に増殖膜があった。左眼に硝子体手術を行い，器質化した増殖膜と新生血管，毛細血管の拡張蛇行があった。一年後の現在，左右眼とも0.03の矯正視力を維持している。結論：本症例でのCoats病様の網膜血管拡張，新生血管，増殖膜形成は，網膜色素変性症に併発したと解釈され，硝子体手術が有効であった。

Abstract.　Purpose：To report a case of retinitis pigmentosa who showed fundus changes simulating Coats disease with vitreous hemorrhage. Case：A 61-yaer-old male presented with impaired visual acuity in his left eye. He had had night blindness at the age of 13 and been diagnosed with retinitis pigmentosa at the age of 17 years. Retinitis pigmentosa was present in his two brothers and one cousin. He had received cataract surgery in both eyes 4 years before and had visual acuity of 0.04 in either eye. Findings：His corrected visual acuity was 0.03 right and counting fingers left. The right eye showed findings compatible with retinitis pigmentosa. The left eye showed vitreous hemorrhage and proliferative membrane in the superior hemisphere. During vitrectomy,the left eye showed organized proliferative membrane,newly formed vessels,and tortuous retinal vessels. Visual acuity improved to 0.03 in either eye one year after surgery. Conclusion：This case illustrates that retinitis pigmentosa may manifest neovascularization,vasodilatation and proliferative membrane in the retina simulating Coats disease. Vitreous surgery was effective for the fundus lesions.