白内障手術を試みた角膜内皮異常のあるAxenfeld-Rieger症候群の1例

Cataract surgery in a case of Axenfeld-Rieger syndrome with abnormal corneal endothelium

要約　目的：角膜内皮に異常があるAxenfeld-Rieger症候群の症例に白内障手術を行った報告をする。症例：42歳の女性が両眼の視力低下で受診した。右眼に－17D，左眼に－15Dの近視があり，矯正視力は右0.09，左0.05であった。両眼に虹彩萎縮，白内障，角膜内皮の滴状反射があり，左眼に瞳孔偏位と偽多瞳孔があった。眼軸長は右27mm，左26mmであった。歯牙欠損，低身長，上顎低形成，眼角隔離症があり，Axenfeld-Rieger症候群と診断した。白内障手術の強い希望があり，角膜内皮を保護するため，分散・凝集型粘弾性物質を使うソフトシェル法で水晶体乳化吸引術と眼内レンズ挿入術を行った。術後8か月の現在，矯正視力は右0.9，左0.3で，角膜は透明である。結論：角膜内皮に異常があるAxenfeld-Rieger症候群の症例に，角膜内皮を保護しながら白内障手術を行い，良好な結果が得られた。

Abstract.　Purpose：To report a case of Axenfeld-Rieger syndrome who showed abnormal corneal endothelium and who received uneventful cataract surgery. Case：A 42-year-old female presented with impaired visual acuity in both eyes as chief complaint. She had myopia of －17 diopters right and －15 diopters left. Her corrected visual acuity was 0.09 right and 0.05 left. Both eyes showed iris atrophy, cataract, and droplet-like reflexes in the corneal endothelium. The left eye showed ectopic pupil with pseudopolycoria. The axial length was 27mm right and 26mm left. She showed defective teeth, telecanthus, maxillary hypoplasia, and short stature. She was diagnosed with Axenfeld-Rieger syndrome. Both eyes underwent phacoemulsification-aspiration with intraocular lens implantation with adjunct use of viscoelastic material in order to protect the corneal endothelium. Eight months later, her visual acuity improved to 0.9 right and 0.3 left with transparent cornea. Conclusion：Cataract surgery resulted in improved vision in a case of Axenfeld-Rieger syndrome with the use of viscoelastic material to protect the corneal endothelium.