先天緑内障を伴ったAxenfeld-Rieger症候群の2症例

Congenital glaucoma in two cases with Axenfeld-Rieger syndrome

　両眼に先天緑内障を伴ったAxenfeld-Rieger症候群の2症例（2歳女児と2歳男児）にトラベクロトミーを行い，術後36か月間観察した。症例1の両眼は術後無治療で眼圧正常となり，視神経乳頭陥凹のC／D比が改善した。症例2は点眼治療を必要とし，右眼は眼圧が正常範囲にあるが，左眼は眼圧コントロールがやや不良であつた。生体顕微鏡検査で虹彩索状物による隅角閉塞が症例1の左眼にみられたが，限局性であった。眼圧上昇機序としてシュレム管および線維柱帯の発育異常が推測された。

We diagnosed bilateral congenital glaucoma in 2 cases, a male and female infant, with Axenfeld-Rieger syndrome. Both were aged 2 years. All the eyes underwent trabeculotomy and were followed up for 36 months. In the first case, control of intraocular pressure (I0P) was achieved without medication. The C/D ratio of the optic disc improved also. The second case necessitated further topical treatment resulting in IOP control in one eye and remained in the low twenties in the other. Ultrasound biomicroscopy (UBM) showed segmental obstruction of the iridocorneal angle in one eye in the first case. It appeared that the congenital glaucoma in our cases was due to dysgenesis of the trabecular meshwork and the canal of Schlemm.