遺伝性末梢血管拡張症患者におけるpial arteriovenous fistulaeの1例

A Case of Pial Arteriovenous Fistulae Associated with Hereditary Hemorrhagic Telangiectasia

Ⅰ．はじめに

　脳動静脈瘻（pial arteriovenous fistulae：pial AVF）は，脳動脈と静脈がnidusを介さずに高血流での直接短絡をもつ稀な疾患であり，全脳動静脈奇形の1.6％と言われている¹⁾．また，遺伝性出血性毛細血管拡張症（hereditary hemorrhagic telangiectasia：HHT）は，pial AVFなどの脳動静脈奇形を5〜11％に合併する．Pial AVFの治療は血管内治療と直達手術などが報告されているが，症例ごとに選択されているのが現状と思われる．Pial AVFはhigh flow shuntを有するため血管内治療に難渋することがあるが，今回，balloon guiding catheter（BGC）でflow controlを行い，良好に塞栓できたpial AVFの1例を経験したため報告する．

　Pial arteriovenous fistulae（pial AVF）are rare vascular lesions. Pial AVF is a complication of hereditary hemorrhagic telangiectasia（HHT）and is associated with a high mortality rate. Here, we report a case of a 14-year-old boy with pial AVF associated with HHT who presented with a seizure. CT and MRI showed enlarged vessels with venous varices in the Sylvian fissure. Digital subtraction angiography（DSA）revealed a pial AVF with a single feeder originating from the middle cerebral artery with large varicose venous drainage, which drained into the transverse sinus, and superior sagittal sinus. A transarterial coil embolization was performed. During the procedure, flow control by a balloon guiding catheter was used. The patient had no neurological deficits after treatment. However, while microsurgery or endovascular treatment are the primary treatment options for pial AVF, there is no consensus regarding their efficacy. Using a balloon guiding catheter to control flow is useful for the treatment of pial AVF with a high-flow shunt.