先天瞳孔閉鎖による続発緑内障の1例

A case of occluded pupil with secondary glaucoma

要約　背景：「隅角形成異常を伴う先天性瞳孔虹彩水晶体膜」（congenital pupillary-iris-lens membrane with goniodysgenesis：CPILMG）は，1986年にCibisらが報告した片眼性の稀な疾患で，白色膜が隅角，虹彩，水晶体前囊の表面で増殖することが特徴である。目的：本疾患で瞳孔ブロックによる緑内障が生じ，手術を行った症例の報告。症例：生後9日目の男児が，右眼の白濁で紹介受診した。両親は健康で，2名の同胞も正常であるという。妊娠と出産には問題がなく，生下時体重は3,044gであった。所見：右眼に浅前房と白色膜による虹彩後癒着があり，眼圧は右35mmHg，左11mmHgであった。CPILMGによる続発緑内障と診断し，周辺虹彩切除術，隅角癒着解離術，瞳孔形成術を即日実施した。術後は前房深度が正常化し，眼圧は10mmHg台で安定した。6か月後の現在，透光体は透明で，眼底に異常はない。結論：CPILMGによる続発緑内障のある新生児に周辺虹彩切除術，隅角癒着解離術，瞳孔形成術を行い，良好な結果を得た。

Abstract.　Background：Congenital pupillary-iris-lens membrane with goniodysgenesis（CPILMG）is a rare clinical entity reported by Cibis in 1986. It is characterized by proliferative membrane over the iris, anterior to the lens, and in the chamber angle. Purpose：To report an infant of CPILMG with secondary glaucoma treated by surgical means. Case：A 9-day-old infant was referred to us for opaque cornea in the right eye. He was born of healthy parents. His two siblings were reportedly normal. His birthweight was 3,044 g. Findings：The right eye showed shallow anterior chamber and posterior synechia with white pupillary membrane. Intraocular pressure（IOP）was 35 mmHg right and 11 mmHg left. He was diagnosed with CPILMG and secondary glaucoma. The right eye received peripheral iridectomy, goniosynechialysis, and pupilloplasty the same day. Surgery was followed by deep anterior chamber with IOP around 10 mmHg. Six months after surgery, the optical media is clear with normal fundus findings. Conclusion：Surgery was effective for secondary glaucoma in an infant with CPILMG.