Sasamori Toru, Hida Kazutoshi, Asano Takeshi, Nakayama Naoki, Kuroda Satoshi, Iwasaki Yoshinobu
Department of Neurosurgery, Hokkaido University Graduate School of Medicine, North-15 West-7, Kita-ku, Sapporo 060-8638, Japan.
No Shinkei Geka. 2009 Jan;37(1):57-63.
The authors reported three cases of high flow arteriovenous fistula (AVF) associated with hereditary haemorrhagic telangiectasia (HHT). The first case was a 9-month-old boy who presented with subarachnoid hemorrhage (SAH). Digital subtraction angiography (DSA) revealed a perimedullary AVF at the cervical spine and the fistula was successfully interrupted by transarterial embolization with no additional neurological deficits. The second case was his mother, a 29-year-old women. She also had history of epistaxis, so imaging screens of her central nervous system (CNS) were undertaken. MRI showed a giant intracerebral varix associated with a high-flow pial AVF. This patient was treated by surgery and postoperative CT angiography showed extinction of AVF completely. The third case was an 8-year-old girl with a family history of HHT. She presented with gradual progression of paraparesis, sensory disturbance in the bilateral lower extremities and bladder dysfunction. MRI disclosed a perimedullary AVF with a large varix at the L3/4 level. The AVF was successfully interrupted by transarterial embolization with platinum coils. Post-embolization DSA showed complete disappearance of the AVF. The existence of HHT must always be considered a possibility when AVF of the CNS is diagnosed.
作者报告了3例与遗传性出血性毛细血管扩张症（HHT）相关的高流量动静脉瘘（AVF）病例。第一例是一名9个月大的男孩，表现为蛛网膜下腔出血（SAH）。数字减影血管造影（DSA）显示颈椎髓周AVF，经动脉栓塞成功阻断瘘管，未出现额外的神经功能缺损。第二例是他的母亲，一名29岁的女性。她也有鼻出血史，因此对其中枢神经系统（CNS）进行了影像学筛查。MRI显示一个巨大的脑内静脉曲张合并高流量软脑膜AVF。该患者接受了手术治疗，术后CT血管造影显示AVF完全消失。第三例是一名8岁女孩，有HHT家族史。她表现为双下肢轻瘫逐渐加重、感觉障碍和膀胱功能障碍。MRI显示L3/4水平有一个髓周AVF和一个大的静脉曲张。经动脉用铂圈栓塞成功阻断了AVF。栓塞后DSA显示AVF完全消失。当诊断出中枢神经系统AVF时，必须始终考虑HHT的存在。
