スタージ・ウェーバー病—顔面に血管腫をもつ153例についての臨床的研究

STURGE-WEBER DISEASE : A CLINICAL STUDY OF 41 CASES OF 153 FACIAL HEMANGIOMAS

I．はじめに

　Sturge-Weber病（SWD）ではほぼ全例にてんかん発作を合併し^2,6,9），かつてんかん発作につづく半身麻痺あるいは知能発達遅延が多くの例（50％以上^6）に認められるといわれる。SWDに随伴する難治性てんかんに対して手術療法がCushing以来多くの人によつて試みられてきた^4,5,7,9）。最近では手術療法はてんかん発作が頻発する以前，かつ知能発達遅延が生ずる以前，すなわち生後1歳未満，できれば数カ月以内に行われるべきという意見も多い。さらに今日CT scansが小児に対してもさかんに行われるようになり，ことに頭蓋内の石灰沈着や血管異常を早期に発見することが可能になつてきた^11,12）。

　しかしSWDの多数例について長期に亘つて経過を観察した報告はMayo clinic^9）からの35例についての報告をみるのみである。顔面血管腫をもつ患者全体についてはその神経症状随伴の頻度などについて報告したものは見当らない。トロント小児病院はオンタリオ地方で最大かつ最古の小児病院であることもあり，形成外科において20年前から顔面の血管性母斑に対して積極的に手術療法が試みられてきており，神経外科においても1957年の1obectomyを第1例としてSWDに対して積極的な手術療法がなされてきた。

This report was based on 153 patients with facial hemangiomas who were admitted to the Division of Plastic Surgery and Neurosurgery, Department of Surgery, Hospital for Sick Children in these 20 years. One hundred fifty three patients were aged between 4 weeks and 18 years. Among them, 46 were male, and 107 were female. The results were summerized as follows:

1) Fourty-one cases (26.8%) of 153 patients who had facial hemangiomas had been suffered from epilepsy and/or neurological impairements. In 39 of 41 patients that should be called Sturge-Weber disease the first branch of trigeminal nerve was affected by hemangiomas.

2) Eight patients who showed only seizure dis-orders had experienced only one to four epileptic attacks which had appeared after one year of agein 6 of 8 cases. All of those attacks were general seizures. Electroencephalograms were normal in 6 cases.

3) Twelve patients were impaired neurologically in slight or moderate grade with epilepsy. In only three of them were started epileptic seizures within a year of age, which were well controlled with drugs. Seven patients showed mild hemi-paresis in the opposite side of facial hemangiomas. Nine patients were mentally retarded but showed more than 50 in IQ (intelligence quantity).

4) Twelve patients had been severely impaired neurologically. In all cases were started epileptic seizures within a year of age and were partial seizures which were sometimes generalized. Ten patients had physical disabilities, and four of them were incapacitated. Other four cases had severe mental retardation with moderate hemiparesis.

5) Operative intervetion was performed in 9 cases. Six cases of 8 patients who were done hemi-spherectomy were in left side and two cases in right side. Seven cases were operated within a year of age. The first case which was done right temporal lobectomy in 1957 had been failed to follow up after 3 years of age. One patient was dead at the age of 8 years. Four cases are doing well in normal school life in 6 to 14 years of age with mild hemiparesis and slight mental retardation. One ten-year-old boy who was performed hemi-spherectomy in the age of six is moderately retarded mentally. Two patients who were operated in recent two years are too early to judge mental prognosis.