自然破囊を生じたAlport症候群の1例

A case of Alport syndrome with spontaneous rupture of lens capsule

要約　目的：水晶体前囊が自然破囊したAlport症候群の症例の報告。症例：31歳男性が左眼の視力低下で受診した。幼少時に血尿があり，Alport症候群と診断され，15歳の時から腎不全に対し人工透析を受けている。所見：矯正視力は右0.5，左0.2で，両眼に前部円錐水晶体があり，左眼の水晶体中央部に前囊の破裂があった。左眼に白内障手術を行った。採取した前囊の厚みは不均一で亀裂があった。水晶体上皮細胞の変性と空胞化した細胞があった。眼内レンズの囊内固定は問題なくできた。結論：Alport症候群に伴う前部円錐水晶体に，前囊の脆弱性を示す組織学的所見があった。

Abstract.　Purpose：To present a case of Alport syndrome with spontaneous rupture of anterior lens capsule. Case：A 31-year-old male presented with impaired visual acuity in the left eye. He had had hematuria during childhood leading to the diagnosis of Alport syndrome. He had been receiving artificial hemodialysis for renal insufficiency since 15 years of age. Findings：Corrected visual acuity was 0.5 right and 0.2 left. Both eyes showed anterior lenticonus. The left eye showed rupture of central part of anterior lens capsule. The left eye was treated by cataract surgery. The anterior lens capsule showed irregular thickness and dehiscences. The epithelial cells showed degeneration interspersed with vacuolized cells. The intraocular lens could be firmly fixated in the capsule. Conclusion：Microscopic findings were present suggesting fragility of anterior lens capsule in a case of Alport syndrome.