深部脳静脈の静脈瘤様奇形—自験例および文献の総括的考察

Varicose Malformation of Deep Cerebral Veins:Report of a case and review of literatures

I．はじめに

　深部脳動静脈奇形およびガレン大静脈瘤は表在性のそれらに比べてまれな疾患である。Litvak et al．^23）は正中部の脳動静脈奇形を3型に分類した。1）ガレン大静脈瘤，2）拡張した静脈系還流を伴う蔓状毬球性動静脈奇形（racemose conglomerations of blood vessels），　3）正中部動静脈shuntの移行形（transitional type）。深部脳動静脈奇形の大部分は第二のカテゴリーに属している。

　いわゆるガレン大静脈瘤は先天性のものであり，ガレン大静脈動静脈奇形ともいわれ，この症例はきわめてまれであり，現在私どもの調査した範囲では世界の文献中で62例であつた。これらの症例はいずれも単純撮影，脳血管撮影および剖検によつて確認されたものである。

1) A case of the varicose malformation of the deep cerebral veins in a 20 year old female with an angioma in the left glabella and spastic paresis of left extremities was reported. Curvilinear cal-cification was seen in the trigone of the right lateral ventricle on the plain films. Angiogram revealed varicose dilatation of the deep cerebral veins, super-ior sagittal sinus, confluence sinum and transverse sinus.

2) Arteriovenous aneurysm of the great vein of Galen are rare disorders and only 62 cases could be found in the world literatures. The clinical pictures, diagnosis, therapy and prognosis were disussed.

3) The cardinal signs are manifested by cardiac decompensation in the neonatal, convulsive seizures, and obstructive hydrocephalus associated with aque-ductal stenosis in infancy and subarachnoid hemor-rhage and space occupying lesions in the childhood and adulthood.

4) The diagnosis may be confirmed by enlarged heads in the neonatal period, neuroradiological find-ings produced by hydrocephalus in infancy, curvi-linear calcification in the pineal area on the plain X-ray films and angiographic opacification of the saccular aneurysm of the great cerebral vein in the adulthood.

5) Treatments of choice are ventriculo-atrial shunt for hydrocephalus, obliteration of the fistulae and extirpation of the aneurysms.