Rendu-Osler-Weber病に合併した頭皮動静脈瘻の直接経皮的塞栓術による1治験例

A Case of a Scalp Arteriovenous Fistula Associated with Rendu-Osler-Weber Disease Treated by Direct Percutaneous Embolization

I．はじめに

　Rendu-Osler-Weber病（以下R-O-W病）または遺伝性出血性毛細血管拡張症は全身性に血管形成異常を生じる常染色体優性遺伝性疾患であり，脳神経外科領域においては脳膿瘍や脳動脈瘤，脳動静脈奇形の合併が問題となる．今回われわれは，R-O-W病に合併した頭皮動静脈瘻症例を経験し，経皮的直接穿刺による塞栓術を行い，満足すべき結果を得たので報告する．

We present a case of Rendu-Osler-Weber disease (hereditary hemorrhagic telangiectasia) accompanied by a scalp arteriovenous fistula, which was successfully treated by direct percutaneous embolization.

A 51-year-old man, who had multiple vascular telangiectases and pulmonary arteriovenous fistulae, de-veloped an enlarging pulsatile mass of the scalp anterior to the site of the previous craniotomy for a brain abscess in the right occipital lobe. Angiography demonstrated a high-flow arteriovenous fistula between the right superficial temporal artery and a varix. Percutaneous injection of pure ethyl alcohol was planned but seemed risky because of the major drainage being into the bilateral cavernous sinuses through the su-perior ophthalmic veins. A 24-gauge plastic needle was placed in the right superficial temporal artery just proximal to the fistula, and 0.7 ml of a mixture consisting of n-butyl cyanoacrylate and lipiodol in a ratio of 1: 1 was injected. Then, the varix was directly punctured, and retention of the contrast medium was con-firmed under manual compression by the placement of a circular ring. Embolization of the varix using 1.0 ml of 70% glucose solution and a subsequent 1.0 ml of pure ethyl alcohol was performed with compression, resulting in total occlusion of the fistula. The scalp mass resolved gradually and there was no evidence of recanalization.

We conclude that direct percutaneous embolization is the first therapeutic choice for a scalp arteriove-nous fistula with multiple shuntings associated with Rendu-Osler-Weber disease. Dangerous venous drain-age should be eliminated before performing embolization with ethyl alcohol.