摘要目的 探讨成年型烟雾病(moyamoya disease,MMD)侧支循环分布模式.方法 从南京卒中注册系统中提取2008年8月至2011年1月经血管造影明确诊断的119例成年卒中型MMD住院患者.以卒中半球同侧的颅内和(或)颅外的侧支循环为研究对象,且将其分为3个等级:1级侧支循环(大脑前动脉→软脑膜动脉→大脑中动脉)、2级侧支循环[扩张和(或)延长的脉络膜前动脉、开放的后交通动脉→大脑后动脉→软脑膜动脉→大脑前动脉和(或)大脑中动脉、大脑后动脉→软脑膜动脉→大脑前动脉和(或)大脑中动脉、脉络膜后动脉→胼周后动脉→大脑前动脉]、3级侧支循环(颈外动脉向颅内代偿通路).分析这些侧支循环的分布模式与Suzuki分级的关系.结果 在参与评估的与卒中半球同侧的117个血管单位中,侧支循环共有200个(分为3个等级).1级、2级和3级侧支循环所占比例依次为11.5％( 23/200)、52.0％( 104/200)和36.5％ (73/200).随着Suzuki 级别的升高,1级侧支循环所占比例逐渐降低,主要集中在MMD的早期(Suzuki Ⅰ～Ⅱ级),占91.3％(21/23;Z=-7.270,P＜0.01).3级侧支循环所占比例随着Suzuki级别的升高而升高,全部出现在MMD中期(SuzukiⅢ～Ⅳ级)和晚期(Suzuki Ⅴ～Ⅵ级),分别占37.0％( 27/73)和63.0％(46/73；Z=-7.270,P＜0.01).与1级和3级侧支循环总体分布比较,随着Suzuki Ⅰ～Ⅵ级变化,2级侧支循环分布比例依次为6.7％ (7/104)、7.7％ (8/104)、15.4％(16/104)、40.4％ (42/104)、14.4％ (15/104)和15.4％(16/104),差异虽无统计学意义(Z=-1.074,P=0.282),但主要分布在MMD的中期.结论 MMD侧支循环模式是多样的,随着病程的进展而变化；尤其在MMD的中期,2级侧支循环在整个侧支循环中占有较高的比例.此数据提示这些侧支通路可能在MMD整个病程中发挥重要的侧支代偿作用.

abstractsObjective To study the patterns of collateral circulation in adults moyamoya disease (MMD). Methods One hundred and nineteen consecutive adult patients with MMD (ischemic or hemorrhagic type) were identified by digital cerebral angiography in Nanjing Stroke Registry Program of Jinling Hospital between August 2004 and January 2010.The extracranial and (or) intracranial collateral circulations ipsilateral to stroke hemisphere were regarded as the research objects,and furthermore,these collateral circulations were divided into three different grades:Grade 1 collateral (anterior cerebral artery (ACA) → meningeal arteries (MLA) → middle cerebral artery (MCA) ),Grade 2 collateral ( dilating and extensing anterior choroidal artery beyond choroid fissure,patent posterior communicating artery → posterior cerebral artery→MLA→ ACA and(or) MCA,posterior cerebral artery→MLA→ACA and (or) MCA and posterior choroidal artery → posterior pericallosal arteries → ACA ) and Grade 3 collateral (collateral originating from the external carotid artery supplying to cerebral blood flow). The relationship between collateral distribution patterns in adult MMD and Suzuki' s classification was analyzed.Results In 117 assessed vessel units of the collateral circulation ipsilateral to stroke hemisphere,there were a total of 200 collateral circulations.The percentage of numbers in Grade 1,Grade 2 and Grade 3 collateral was 11.5％(23/200),52.0％ (104/200) and 36.5％ (73/200),respectively.The distribution percentage of Grade 1 was gradually decreased from Suzuki's Ⅰ to Ⅵ,mainly distributed in the early stage of MMD ( Suzuki's Ⅰ -Ⅱ ) and accounted for 91.3％ (21/23; Z =- 7.270,P ＜ 0.01 ).The distribution percentage of Grade 3 was gradually increased from Suzuki' s Ⅰ to Ⅵ,especially in the late stage of MMD ( Suzuki' s Ⅴ-Ⅵ) and accounted respectively for 37.0％ (27/73) and 63.0％ (46/73; Z =-7.270,P ＜0.01 ).Compared with the total distribution of Grade 1 and 3 collateral circulation,the distribution percentage of Grade 2 was 6.7％ (7/104),7.7％ (8/104),15.4％ ( 16/104),40.4％ (42/104),14.4％ (15/104)and 15.4％ (16/104) from Suzuki' s Ⅰ to Ⅶ.Although there was not significant difference,Grade 2 mainly distributed in the medium stage of MMD ( Suzuki' s Ⅲ-Ⅳ ).Conclusions The patterns of collateral distribution is various,changing with the progression of MMD. Grade 2 collateral circulation accounts for a higher proportion,especially in the medium stage of the disease,which suggests that these collaterals play an important compensatory role of blood flow.