中文摘要：

目的探讨出血型烟雾病合并后循环病变患者的临床及影像学特征。方法回顾性分析2002年12月至2011年12月出血型烟雾病患者224例的临床及影像资料,根据后循环受累与否分为后循环受累组57例,非受累组167例。结果 （1）后循环受累组铃木分期集中在Ⅴ期和Ⅵ期,分别占42.1%（24/57）、40.4%（23/57）;非受累组铃木分期多集中在Ⅲ期和Ⅳ期,分别占31.1%（52/167）、41.9%（70/167）。两组患者的铃木分期分布差异有统计学意义（P〈0.01）。（2）出血类型：后循环受累组中,丘脑出血占43.9%（25/57）,脑室出血占38.6%（22/57）,基底节出血占3.5%（2/57）,脑叶出血0例,蛛网膜下腔出血占14.0%（8/57）。非受累组中,丘脑出血0例,脑室出血占37.7%（63/167）,基底节出血占25.1%（42/167）,脑叶出血占21.6%（36/167）,蛛网膜下腔出血占15.6%（26/167）。两组患者出血类型分布差异有统计学意义（P〈0.01）。（3）出血侧异常血管：后循环受累组中,脉络膜前动脉、脉络膜后动脉扩张率及后交通动脉、后胼周动脉开放率分别为19.3%（11/57）、82.5%（47/57）、36.8%（21/57）、78.9%（45/57）;非受累组中,脉络膜前动脉、脉络膜后动脉扩张率及后交通动脉、后胼周动脉开放率分别为36.5%（61/167）、3.0%（5/167）、68.9%（115/167）、80.2%（134/167）。两组患者出血侧异常血管发生率差异有统计学意义（P〈0.01）。结论出血型烟雾病合并后循环病变不少见,铃木分期多处于烟雾病血管分期的晚期,以丘脑出血为主,脉络膜后动脉破裂可能是出血的主要原因。

英文摘要：

Objective To investigate the clinical and angiographic features in hemorrhagic moyamoya disease patients with the posterior circulation involvement（ PCI）. Methods The clinical and imaging data of 224 patients with hemorrhagic moyamoya disease from December 2002 to December 2011 were analyzed retrospectively. The patients were divided into either a PCI group（ n = 57） or a non-PCI group（ n = 167）according to whether they had PCI or not. Results（ 1） Suzuki staging concentrated in stage Ⅴ and Ⅵ in the PCI group,accounting for 42. 1 %（ 24 /57） and 40. 4 %（ 23 /57） respectively; Suzuki staging concentrated in stage Ⅲ and Ⅳ in the non-PCI group,accounting for 31. 1 %（ 52 /167） and 41. 9 %（ 70 /167） respectively. There was significant difference in the distribution of Suzuki staging between the patients of both groups（ P〈0. 01）.（ 2） In the PCI group,the thalamic hemorrhage,intraventricular hemorrhage,ganglia hemorrhage,subarachnoid hemorrhage accounted for 43. 9%（ 26 /57）,38. 6%（ 22 /57）,3. 5%（ 2 /57）,14. 0%（ 8 /57）,respectively; There was no cerebral lobe hemorrhage. In the non-PCI group,the thalamic hemorrhage,intraventricular hemorrhage,basal ganglia hemorrhage,subarachnoid hemorrhage and cerebral lobe hemorrhage accounted for 0,37. 7%（ 63 /167）,25. 1%（ 42 /167）,15. 6%（ 26 /167）,21. 6（ 36/167）,respectively. The proportion of hemorrhage types between the PCI group and non-PCI group was significantly different（ P〈0. 01）.（ 3） The dilation of anterior,posterior choroidal artery,and patency of posterior communicating artery,posterior pericallosal artery in bleeding side accounting for 19. 3%（ 11 /57）,82. 5%（ 47 /57）,36. 8%（ 21 /57）,78. 9%（ 45 /57） in PCI group and 36. 5%（ 61 /167）,3. 0%（ 5 /167）,68. 9%（ 115 /167）,80. 2%（ 134 /167） in non-PCI group. The ratio of of abnormal vessels between PCI group and non-PCI group had significantly statistical difference（ P〈0. 01）. Conclusion Hemorrhagic moyam