オスラー病に脳硬膜動静脈瘻を合併した難治性鼻出血の1例

A case of Rendu-Osler-Weber disease associated with dural arteriovenous fistula

I．はじめに

　オスラー病（Rendu-Osler-Weber病）は，1896年にRenduが初めて報告した常染色体優性遺伝性疾患であり^1），遺伝性出血性毛細血管拡張症（hereditary hemorrhagic telangiectasia：以下，HHT）とも呼ばれている。鼻出血，毛細血管拡張，内臓血管異常，遺伝性を主徴とする疾患であり，初発症状が鼻出血であることが多い。今回われわれは，脳硬膜動静脈瘻を伴い，鼻出血のコントロールに難渋したオスラー病の1例を経験したので，若干の文献的考察を加えて報告する。

A 49-year-old man presented with massive and recurrent epistaxis. He showed marked telangiectasia in his tongue and nasal mucosa,and these findings were compatible with Rendu-Osler-Weber disease. Carotid angiography revealed a dural arteriovenous fistula in the anterior cranial fossa. MRI depicted the dilated tortuous ethmoidal vein,a draining vein of the fistula,which was suspected to be the major source of hemorrhage. The epistaxis was successfully treated by ligation and embolization of the feeding arteries of the arteriovenous fistula combined with ultrasonic cautery of the nasal mucosa using a harmonic scalpel. Complications and management of Rendu-Osler-Weber disease were bibliographically reviewed.