Neurological Surgery 脳神経外科37巻1号

2009年01月発行

症例

遺伝性出血性毛細血管拡張症に合併したhigh-flow AVFの検討

著者： 笹森徹¹ 飛騨一利¹ 浅野剛² 中山若樹¹ 黒田敏¹ 岩㟢喜信¹

所属機関： ¹北海道大学大学院医学研究科神経外科 ²北海道大学大学院医学研究科放射線科

ページ範囲：P.57 - P.63

Ⅰ．はじめに

　遺伝性出血性毛細血管拡張症（hereditary haemorrhagic telangiectasia：HHT）は，皮膚・粘膜の毛細血管拡張を特徴とする常染色体優性の遺伝性疾患である³⁾．全身の血管系に異常が出現し，中枢神経系にも動静脈奇形（arteriovenous malformation：AVM）を合併することが知られている⁸⁾．

　今回，われわれはHHTに合併した中枢神経動静脈瘻（arteriovenous fistula：AVF）の3例を経験し，いずれもhigh-flowな病変であった．1例は巨大なvarixを伴ったcerebral pial AVFであり，2例は脊髄perimedullary AVFである．Cerebral AVFの症例は，subarachnoid hemorrhage（SAH）で発症した脊髄AVF患児の実母であり，無症候であったがHHTを疑って施行したスクリーニング検査で偶然異常を指摘された．

　HHTの病態について触れ，特に中枢神経AVFとの関連について文献的考察を加え報告する．

参考文献

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