Bloch-Sulzberger症候群（色素失調症）の臨床経過の検討

Clinical course of Bloch-Sulzberger syndrome

　1971年から1991年5月の間に国立小児病院を訪れたBloch-Sulzberger症候群23例（男児1例，女児22例）の臨床経過を検討した。初診時年齢は生後11日から10歳（平均1年2か月）で，12例15眼（32.6％）に血管拡張，蛇行，鈍的分岐，無血管帯などの網膜血管異常を認めた。このうち10例は初診時に異常所見を認めたが，2例は経過観察中に異常が出現し，長期の経過観察が必要と思われた。5例6眼（13.0％）に光凝固を施行し，4眼は予後良好であったが，2眼では網膜全剥離，牽引乳頭，黄斑変性を起こし予後不良であった。予後不良であった2例では，網膜血管異常は広範で後極に及んでおり，術前より血管新生，線維増殖などがみられた。

We reviewed a series of 23 children with Bloch -Sulzberger syndrome seen by us during the fore-going 20 years. The series included 1 male and 22 females. The age at the first visit ranged from 11 days to 10 years, average 14 months. Retinal abnor-malities were present in 15 eyes, 12 patients. They included dilatation and torutuosity of retinal ves-sels, abnormalities in branching and presence ofavascular areas. The abnormalities were detected at the first visit in 10 and later in 2 cases. Photocoa-gulation was performed in 6 eyes, 5 cases. Four eyes responded favorably to the treatment. Two eyes resulted in poor outcome with total retinal detach-ment, dragged disc and maculopathy. These eyes were characterized by retinal vascular abnormality reaching the posterior pole and by marked neovas-cularization and fibrotic tissue proliferation.