マルファン症候群に先天緑内障を合併した1例

Congenital glaucoma in a case of Marfan syndrome

　斜視の疑いで20か月の女児を紹介された。初診時に両眼に角膜拡大，角膜のHaab線，隅角の虹彩高位付着があり，眼圧は右24mmHg，左26mmHgであった。緑内障手術の際に両眼の水晶体偏位を発見した。身長と体重は標準で，循環器系に異常がなく，血漿と尿のアミノ酸分画は正常であった。母に水晶体偏位，高身長，細長い上下肢，くも指があった。これらの所見から，本症例をマルファン症候群と診断し，先天緑内障を合併した非定型例と考えた。

A 20-month female infant was referred to us for suspected strabismus. She manifested ker-atectasia, high insertion of the chamber angle, Haab's striae and elevated intraocular pressure in both eyes. Bilateral lens dislocation was detected during surgery for glaucoma. Her systemic status was normal. There was no abnormal amino acids in the serum or urine. Abnormal signs of cardiovascular system were absent. Her mother showed lens dislocation, excessive height and arachnodatyly. These features led us to diagnose the infant as atypical case of Marfan syndrome with congenital glaucoma.