水晶体嚢に特徴的な電顕所見を認めたアルポート症候群の1例

Ultrastructural characteristics of the lens capsule in a case of Alport's syndrome

　43歳男性で，前円錐水晶体を合併したアルポート症候群の症例において，超音波乳化吸引術および眼内レンズ挿入術を施行した。その際，得られた前嚢について電子顕微鏡にて観察したところ，幅約0.5μmの裂隙がところどころにみられた。裂隙は水晶体上皮直下から始まり，前嚢内を縦横に走行し，その辺縁においては基底膜構造が崩れて空胞を呈する部位もみられた。基底膜の先天的構造異常に化学的要因，物理的要因が関与し，これらの裂隙が徐々に拡大し，水晶体嚢の緊張を低下させ，前円錐水晶体の発生を惹起させたものと推測した。

A 43-year-old male presented with bilateral lenticonus with anterior polar cataract. Chronic glomerulonephritis had set in at the age of 15 years. Hypoacusia had set in 4 years before. He was diagnosed as Alport's syndrome. He underwent phacoemulsification with intraocular lens implantation in both eyes. The anterior lens capsule in the right eye was examined by electron microscopy. There were numerous dehiscences of about 0.5 j.c m in width, beginning at the inner surface and extending straight out or curving around in the capsule. Some of the dehiscences had fibrillar or vacuolar contents. These findings were interpreted as congenital abnormalities of the basement membrane, causing anterior lenticonus with forward protusion of the anterior lens capsule.