儿童烟雾病的临床特征、手术治疗及疗效分析  

Analysis of clinical features and surgical treatment of pediatric moyamoya disease

作　　者：刘阳 郭高超 赵黎明 梁浩[2] 张韶 高涛 孙玉学 栗超跃 Yang Liu;Gaochao Guo;Liming Zhao;Hao Liang;Shao Zhang;Tao Gao;Yuxue Sun;Chaoyue Li(Department of Neurosurgery,Henan Provincial People's Hospital,Zhengzhou University People's Hospital,Henan University People's Hospital,Zhengzhou 450003,China;Department of Neurosurgery,Henan University People's Hospital,Zhengzhou 450003,China)

机构地区：[1]河南省人民医院郑州大学人民医院河南大学人民医院神经外科,郑州450003 [2]河南大学人民医院神经外科,郑州450003

出　　处：《中华脑血管病杂志（电子版）》2021年第4期237-241,共5页Chinese Journal of Cerebrovascular Diseases(Electronic Edition)

摘　　要：目的分析儿童烟雾病(MMD)的临床特征、手术治疗及疗效。方法回顾性分析2017年6月至2020年6月河南省人民医院收治的17例3~14岁的MMD患儿。17例患儿共27侧病变,其中26侧行脑-硬膜-肌肉-血管融通术(EDMS),1侧行保守治疗。术后6个月对患儿进行临床随访。采用磁共振血管造影(MRA)或数字减影血管造影(DSA)和核磁共振灌注成像(PWI)或动脉自旋标记(ASL)评估患儿术后远期脑血运重建;采用改良Rankin量表评分(mRS)评估患儿神经功能恢复情况。结果术后6个月MRA或DSA显示,26侧出现颈外动脉系统向脑皮质代偿供血,PWI或ASL显示脑灌注缺血情况明显改善;24侧(92.3%)术后患儿神经功能缺损症状得到改善,2侧(7.7%)术后患儿症状稳定,未再出现新发的神经功能缺损症状。3侧术后患儿出现一过性癫痫发作,1侧术后出现头皮局部坏死,无脑梗死和脑出血等严重并发症出现。术后6个月17例患儿的mRS评分为(0.2±0.4)分。结论儿童MMD临床特征和影像学表现与成人MMD存在差异,采用EDMS治疗儿童MMD较安全,可以明显改善脑灌注情况,短期随访效果较好。Objective To investigate the clinical features,surgical treatment and curative effect of pediatric moyamoya disease.Methods A group of 17 pediatric patients with moyamoya disease(aged 3-14 years)was retrospectively analyzed from June 2017 to June 2020 in Henan Provincial People's Hospital,in regard to clinical data,imaging features,surgical methods and postoperative treatment.Totally 17 cases(26 sides)underwent brain-dural-temporal muscle-vascular fusion(encepho-dural-myo-synangiosis,EDMS).Postoperative follow-up was conducted,with magnetic resonance angiography(MRA)or digital subtraction angiography(DSA)and perfusion imaging(PWI)or arterial spin labeling(ASL),to evaluate the efficacy of long-term cerebral revascularization after surgery.The improved Rankin scale score(mRs)was used to evaluate the neurological functional recovery.Results Six months after the operation,MRA or DSA showed that external carotid artery system compensated blood supply to the cerebral cortex on 24 sides,and PWI or ASL showed that cerebral perfusion ischemia was significantly improved.Totally 24 side surgeries(92.3%)improved the symptoms of neurological impairment,while in the other 2 side surgeries(7.7%),the symptoms were stable,with no new-onset symptoms of neurological impairment.Transient epileptic seizures occurred after 3 operations;local scalp necrosis occurred after 1 operation;and no serious complications such as cerebral infarction and cerebral hemorrhage occurred.Conclusion EDMS is safe in the treatment of children with moyamoya disease.It can significantly improve cerebral perfusion,and shows a good effect during short-term follow-up.

关 键 词：烟雾病 儿童 脑-硬膜-颞肌-血管融通术 

分 类 号：R743.3[医药卫生—神经病学与精神病学]