出血型烟雾病的临床和影像分析

Clinical and imaging analysis of hemorrhagic moyamoya disease

目的分析出血型烟雾病的临床特点和脑出血类型,探讨脑出血原因。方法回顾性分析2011年8月至2012年5月北京天坛医院收治的30例(37例次)出血型烟雾病患者的临床及影像学资料。结果出血型烟雾病患者占同期收治烟雾病患者的19.6%(30/153)。男7例,女23例,男:女=1:3.3;年龄12～55岁,平均(35.0±11.4)岁。成人烟雾病脑出血发病比例高于儿童及青春期患者(22.6%vs.10.5%)。单纯脑室内出血(15例次)和脑实质出血破入脑室(12例次)是烟雾病脑出血最常见的两种类型(73%)。8例(26.7%)合并动脉瘤。出血侧半球和非出血侧半球Suzuki分期差异无统计学意义(Z=-0.109,P=0.913)。30例60侧半球中,32侧存在脉络膜前动脉合并后交通动脉扩张,发生出血半球20侧(62.5%),非出血半球12侧(37.5%);28侧无脉络膜前动脉合并后交通动脉扩张,发生出血半球10侧(35.7%),非出血半球18侧(64.3%),存在脉络膜前动脉合并后交通动脉扩张的半球出血比例显著升高(掊2=4.286,P=0.038)。结论出血型烟雾病多见于成人和女性患者,单纯脑室内出血和脑实质出血破入脑室是烟雾病脑出血最常见的两种类型,动脉瘤破裂和脉络膜前动脉扩张破裂可能是烟雾病脑出血的原因。

Objective To analyze the clinical and radiological findings of hemorrhagic moyamoya disease （MMD）, as well as the causes of cerebral hemorrhage in these patients. Methods The clinical and imaging data of 30 patients with hemorrhagic moyamoya disease from August 2011 to May 2012 were analyzed retrospectively. Results MMD patients with hemorrhage accounted for 19.6% （30/153） of MMD patients admitted to our hospital over the same period. The male to fe-male ratio was 1：3.3 （7：23）. The age of onset ranged from 12 to 55 years old （mean 35.0±11.4 years old）. The rate of hem-orrhage among adults with MMD was higher than that among children （22.6% vs. 10.5%）. Intra-ventricular hemorrhage （IVH） and intra-cerebral hemorrhage （ICH） combined IVH were the two most common types of hemorrhage in MMD （73%）. Eight patients had aneurysms （26.7%, 8/30）. There was no statistical difference in Suzuki classification between the two groups （Z = -0.109, P = 0.913）. Thrity-two of the 60 hemispheres had dilation of anterior choroidal artery and posterior communicating artery. Among them, the hemorrhagic hemispheres accounted for 62.5% （20/32）, and the non-hemorrhagic hemispheres accounted for 37.5% （12/32） （X^2=4.286, P = 0.038）. Conclusion Hemorrhagic moyamoya disease is preva-lence in adult and female patients. IVH and ICH combined IVH are the two most common types of hemorrhage in MMD. Rupture of aneurysm and the dilated anterior choroidal artery are considered to be two mechanisms that may lead to hem-orrhage in MMD.