前部円錐水晶体を合併したAlport症候群

Anterior lenticonus in a case of Alport syndrome

要約　目的：前部円錐水晶体を伴ったAlport症候群に対して水晶体再建術を行った報告。症例：46歳，男性。8か月前に両眼の視力低下を自覚して受診した。慢性腎不全と難聴の既往があり，長女がAlport症候群で治療中であった。母親に血尿が認められていた。初診時の視力は左右とも0.07（0.3）で，両眼に前部円錐水晶体を認めたため，水晶体再建術を施行した。術中は，前囊は薄く伸展性に富み，continuous curvicular capsulotomyが困難であった。切除した前囊の組織像では，全層にわたり微細な亀裂が認められた。術後の矯正視力は右（1.0），左（0.9）で，その後3年間視力を維持している。結論：Alport症候群に合併した前部円錐水晶体による視力低下に対し，水晶体再建術を施行した結果，視機能の改善を得た。

Abstract.　Purpose：To report a case of Alport syndrome who developed anterior lenticonus. Case：A 46-year-old male presented with decreased vision in both eyes since 8 months before. He had had hearing difficulty and chronic renal failure. Reportedly, his mother had hematuria. One of his sisters had been treated for renal failure. blurring in the left eye. Findings：Visual acuity was 0.07 in either eye. When corrected by about-8 diopters, it improved to 0.3 in either eye. Both eyes showed anterior lenticonus. As intraoperative finding, the anterior lens capsule was thin and elastic, making continuous curvicular capsulotomy difficult. The excised anterior lens capsule showed, when observed by electron microscopy, dehiscences involving the whole layer from the basement membrane to the surface of lens capsule. Visual acuity has been maintained at 1.0 right and 0.9 left for 3 years until present. Conclusion：Lens reconstruction was effective for anterior lenticonus in the present case of Alport syndrome.