幼儿烟雾病的诊断及外科治疗分析  

Analysis of diagnosis and surgical treatment of childhood moyamoya disease

作　　者：袁宏耀 许新科 陈程 林锦荣 王方宇 李方成 李军亮 Yuan Hongyao;Xu Xinke;Chen Cheng;Lin Jinrong;Wang Fangyu;Li Fangcheng;Li Junliang(Guangzhou Women & Children's Medical Center,Guangzhou 510000,China)

机构地区：[1]广州市妇女儿童医疗中心神经外科

出　　处：《临床小儿外科杂志》2019年第9期739-743,共5页Journal of Clinical Pediatric Surgery

基　　金：国家自然科学基金(编号:81873739)

摘　　要：目的探讨幼儿烟雾病的诊断、外科治疗方案及其预后状况。方法回顾性分析2015年3月至2018年3月广州市妇女儿童医疗中心神经外科收治的15例烟雾病患儿(年龄均≤6岁)的临床资料,分析患儿的首发症状、Suzuki分期、影像学特征、手术方式及手术疗效,总结幼儿烟雾病的临床特征及手术疗效。结果幼儿烟雾病的首发症状包括:短暂性脑缺血发作(Transient ischemic attack,TIA)5例,脑梗死8例,头痛、头晕2例;Suzuki分期显示:Ⅱ期1例,Ⅲ期6例,Ⅳ期5例,Ⅴ期3例。12例(17侧)行手术治疗,其中左侧2例,右侧5例,双侧5例。均行脑-硬脑膜-颞肌-血管融合(Encepho-dural-myo-synangiosis,EDMS)术,其中3侧联合行额骨多点钻孔(Multiple buur holes,MBH)术。患儿均获12~30个月随访,中位随访时间16.3个月。术后3个月磁共振血管成像(Magnetic resonance angiography,MRA)或血管造影(Digital subtraction angiography,DSA)显示,17侧均出现颈外动脉向脑皮层代偿供血现象,表示脑血流灌注均得到改善;16侧手术(94.1%)后患儿神经功能缺损症状得到改善,1侧手术(5.9%)后患儿进展性症状稳定。1例患儿出现硬膜下出血,余14例均未出现手术侧脑梗死、出血、颞肌占位及头皮坏死等并发症。结论EDMS治疗幼儿烟雾病安全有效,联合EDMS和额骨钻孔术可达到更大范围的间接血管重建,有利于进一步改善脑灌注状况。Objective To explore the diagnosis,surgical treatment and prognosis of moyamoya disease in young children. Methods From March 2015 to March 2018,clinical data retrospectively reviewed for 15 hospitalized children with moyamoya disease (age ≤ 6 years) were analyzed retrospectively.Their clinical features and surgical efficacies were summarized.And initial symptoms,Suzuki stages,imaging features,surgical approaches and surgical efficacy were analyzed. Results The initial symptoms were TIA ( n =5),cerebral infarction ( n =8) and headache & dizziness ( n =2).And Suzuki stage was Ⅱ( n =1),Ⅲ( n =6),Ⅳ( n =5) and Ⅴ( n =3).Twelve children underwent a total of 17 side operations,including left ( n =2),right ( n =5) and bilateral ( n =5).All children underwent encepho-dural-myo-synangiosis (EDMS) and three sides underwent concurrent multiple buur holes (MBH).The average follow-up period was 16.3 (12-30) months.MRA/DSA indicated that external carotid artery was compensated for blood supply to had better perfusion.Ischemic symptoms were relieved in 16 hemispheres (94.1%) and stabilized in 1 hemisphere (5.9%).There was one case of subdural hemorrhage and the remainder had no complications such as cerebral infarction,hemorrhage,diaphragmatic space-occupying or scalp necrosis. Conclusion EDMS is both safe and effective for MMD in young children.And combining EDMS and MBH of frontal bone for achieving a greater range of indirect revascularization may further improve cerebral perfusion.

关 键 词：烟雾病 幼儿 脑-硬脑膜-颞肌-血管融合术 多点钻孔术 

分 类 号：R726[医药卫生—儿科] R742[医药卫生—临床医学]