中文摘要：

目的探讨蛛网膜下腔出血型烟雾病的临床特征和脑硬膜颞浅动脉血管融通术（EDAS）的治疗效果。方法回顾性分析2002年1月至2013年4月解放军第三○七医院神经外科收治的38例蛛网膜下腔出血型烟雾病患者的临床和影像学资料。对35例患者行单侧或双侧EDAS,共行64侧EDAS,4例合并动脉瘤的患者先期行血管内栓塞术。另3例患者未行手术治疗。结果 （1）蛛网膜下腔出血型烟雾病患者占同期收治的出血型烟雾病患者的10.8%（38/353）,其中成人37例,儿童1例,男∶女=1∶3.22（9/29）,发病年龄为12~59岁,平均（39±11）岁。4例合并动脉瘤,其余34例未合并动脉瘤患者的铃木分期分布、脉络膜前动脉扩张情况及后交通动脉扩张情况,在出血侧与非出血侧差异均无统计学意义（均P〉0.05）。（2）术后随访13~125个月,平均（51±27）个月,有2例发生再出血,1例为脑室出血,1例为脑实质出血,35例接受EDAS治疗患者的术后改良Rankin评分较术前明显降低,差异有统计学意义（P〈0.05）。16例患者术后3~19个月复查正电子发射计算机体层显像显示,23侧手术半球中,17侧半球代谢较术前改善,6侧较术前无明显变化。13例患者于术后5~30个月复查全脑DSA,示23侧手术半球中,19侧血管重建有效。结论蛛网膜下腔出血型烟雾病好发于成人,女性多见。EDAS可获得良好的血管重建效果及改善患者的脑代谢,从而缓解脑缺血症状。

英文摘要：

Objective To investigate the clinical features of subarachnoid hemorrhagic moyamoya disease and the therapeutic effect of encephalo-duro-arterio-synangiosis（ EDAS）. Methods The clinical and imaging data of 38 patients with subarachnoid hemorrhagic moyamoya disease admitted to the Department of Neurosurgery,the 307 th Hospital of PLA from January 2002 to April 2013 were analyzed retrospectively.Thirty-five patients underwent unilateral or bilateral EDAS（ 64-sides underwent EDAS,4 patients with aneurysm s underwent endovascular em bolization first）,and 3 patients did not undergo any surgery.Results（ 1） Subarachnoid hemorrhagic moyamoya disease accounted for 10. 8%（ 38 /353） of all the hemorrhagic moyamoya disease admitted in hospital over the same period,including 37 adults and 1 child. The male to female ratio was 1 ∶ 3. 22（ 9 /29）,and the age of onset was 12 to 59 years. The mean age of patients was 39 ± 11 years. Four patients were combined with aneurysms. There were no significant differences in the distribution of Suzuki stage,anterior choroidal artery dilatation and posterior communicating artery dilatation in the remaining 34 patients without aneurysms between the bleeding sides and non-bleeding sides（ P 0. 05）.（ 2） The patients were followed up for 13- 125 months（ mean 51 ± 27 months）,two patients had rebleeding,one of them was intraventricular hemorrhage,the other was parenchymal hemorrhage. The postoperative modified Rankin score（ mRS） was significantly lower in 35 patients whom were treated with EDAS. Compared with before surgery,there was significant difference（ P〉0. 05）. The re-examination of positron emission tomography（ PET） for 16 patients at 3 to 19 months after surgery showed that among the23 surgically treated hemispheres,the cerebral metabolisms of 17 hemispheres were improved after surgery,and 6 did not have any change after surgery. The re-examination of whole brain digital subtraction angiography（ DSA） at 5 to 30 months after surgery in 13