不同临床分型、影像学分期烟雾病的治疗策略与疗效

Treatment strategy and efficacy for moyamoya disease of different clinical types and radiographic stages

目的探讨不同临床分型、不同影像学分期烟雾病患者的治疗策略。方法回顾性分析2010~2013年收治的39例烟雾病患者的临床分型、影像学分期与治疗方法;术后采用CT血管成像（CTA）、CT灌注（CT perfusion,CTP）和数字减影血管造影（DSA）评价疗效,平均随访（21.3±3.1）个月（7~44个月）。结果Ⅰ~Ⅴ型患者34例,其中6例接受脑-硬膜-肌肉血管融合术,28例接受综合手术治疗（颞浅动脉-大脑中动脉吻合＋脑-硬膜-肌肉血管融合术）,4例首次手术半年后行对侧综合手术治疗;Ⅵ型患者5例,急诊行颅内血肿清除＋去骨瓣减压＋脑-硬膜-肌肉血管融合术。Ⅰ~Ⅱ期患者一般选择随访观察及保守治疗;Ⅲ期以上患者接受颅内外血管重建手术治疗,Ⅴ期及Ⅵ期患者采用个体化设计皮瓣、骨窗、硬膜翻转贴敷,以最大限度保留术前已自发形成的颅内外血管吻合。随访期间患者临床症状明显改善,脑血流灌注较术前改善,脑血流量（CBF）、脑血容量（CBV）较术前增加,达峰时间（TTP）较术前缩短,差异有统计学意义（P〈0.05）,这些变化随着随访时间的延长而更加明显。结论根据烟雾病患者不同的临床分型、影像学分期,采取不同的治疗方法,可以使患者获得良好的临床效果。

Objective To assess the therapeutic strategies and efficacy of moyamoya disease of different clinical types and radiographic stages.Methods The clinical types,radiographic stages and treatment strategies of 39 patients with moyamoya disease,who were treated in our medical centers from April 2010 to December 2013,were retrospectively analyzed.The treatment efficacies were analyzed by CT angiography,CT perfusion and DSA.The average follow-up time was（21.3±3.1）months（range：7-44months）.Results Thirty-four patients had typeⅠ-Ⅴ moyamoya disease,including 6treated by encephoduro-myo-synangiosis（EDMS）and 28 by STA-MCA anastomosis combined with EDMS,with 4receiving a second surgery on the other hemisphere 6months after first surgery.Five patients with typeⅥ moyamoya disease underwent emergency surgery：cerebral hematoma evacuation＋decompressive craniectomy＋EDMS.Stage Ⅰ and Ⅱ patients were treated by conservative therapies.StageⅢ-Ⅵ patients were treated by EC-IC cerebrovascular reconstruction.The skin incision,bone window,and dura incision were designed individually in order to protect anastomoses formed pre-op in stageⅤ ＆ Ⅵ patients.All patients had improved clinical condition during follow-up.CTP imaging showed significantly increased relative cerebral blood flow and relative cerebral blood volume,and decreased time-to-peak after the operation（P〈0.05）,and these changes were intensified with the prolong of follow-up.Conclusion Selection of appropriate treatment strategy according to different clinical types and radiographic stages can achieve better therapeutic efficacy for moyamoya disease patients.