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中华脑血管病杂志(电子版)

中华脑血管病杂志(电子版) ›› 2021, Vol. 15 ›› Issue (04) : 237 -241. doi: 10.11817/j.issn.1673-9248.2021.04.008

论著

儿童烟雾病的临床特征、手术治疗及疗效分析
刘阳1, 郭高超1, 赵黎明1, 梁浩2, 张韶2, 高涛1, 孙玉学1, 栗超跃1,†()   
  1. 1. 450003　郑州，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
    2. 450003　郑州，河南大学人民医院神经外科
  • 收稿日期:2020-12-30 出版日期:2021-08-09
  • 通信作者: 栗超跃

Analysis of clinical features and surgical treatment of pediatric moyamoya disease

Yang Liu1, Gaochao Guo1, Liming Zhao1, Hao Liang2, Shao Zhang2, Tao Gao1, Yuxue Sun1, Chaoyue Li1,†()   

  1. 1. Department of Neurosurgery, Henan Provincial People's Hospital, Zhengzhou University People's Hospital, Henan University People's Hospital, Zhengzhou 450003, China
    2. Department of Neurosurgery, Henan University People's Hospital, Zhengzhou 450003, China
  • Received:2020-12-30 Published:2021-08-09
  • Corresponding author: Chaoyue Li
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刘阳, 郭高超, 赵黎明, 梁浩, 张韶, 高涛, 孙玉学, 栗超跃. 儿童烟雾病的临床特征、手术治疗及疗效分析[J]. 中华脑血管病杂志(电子版), 2021, 15(04): 237-241.

Yang Liu, Gaochao Guo, Liming Zhao, Hao Liang, Shao Zhang, Tao Gao, Yuxue Sun, Chaoyue Li. Analysis of clinical features and surgical treatment of pediatric moyamoya disease[J]. Chinese Journal of Cerebrovascular Diseases(Electronic Edition), 2021, 15(04): 237-241.

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。

关键词: 烟雾病, 儿童, 脑-硬膜-颞肌-血管融通术
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.

Key words: Moyamoya disease, Children, Encepho-duro-myo-synangiosis
表1 17例MMD患儿的临床资料
编号 性别 年龄（岁） 首发症状 Suzuki分期 手术方式 术后并发症 mRs评分（分）b
术前 术后6个月
1 女性 8 TIA 左侧Ⅱ期，右侧Ⅲ期 右侧EDMSa 无 1 0
2 女性 12 脑梗死 右侧Ⅲ期 右侧EDMS 无 2 0
3 女性 8 头痛、头晕 左侧Ⅲ期，右侧Ⅲ期 双侧EDMS 癫痫 1 0
4 男性 3 TIA 右侧Ⅱ期 右侧EDMS 无 1 0
5 女性 9 头痛、头晕 左侧Ⅲ期，右侧Ⅲ期 双侧EDMS 无 1 0
6 女性 7 TIA 左侧Ⅲ期，右侧Ⅲ期 双侧EDMS 无 1 0
7 女性 14 脑梗死 左侧Ⅳ期，右侧Ⅳ期 双侧EDMS 癫痫 2 1
8 女性 6 脑梗死 左侧Ⅲ期，右侧Ⅲ期 双侧EDMS 无 2 0
9 男性 7 TIA 左侧Ⅲ期，右侧Ⅲ期 双侧EDMS 头皮坏死 1 0
10 男性 11 头痛、头晕 右侧Ⅲ期 右侧EDMS 无 1 0
11 女性 14 TIA 左侧Ⅲ期，右侧Ⅲ期 双侧EDMS 无 1 0
12 男性 6 脑梗死 右侧Ⅳ期 右侧EDMS 癫痫 2 1
13 女性 6 TIA 右侧Ⅲ期 右侧EDMS 无 1 0
14 男性 3 TIA 左侧Ⅲ期，右侧Ⅲ期 双侧EDMS 无 1 0
15 男性 7 脑梗死 右侧Ⅲ期 右侧EDMS 无 2 1
16 男性 8 TIA 左侧Ⅲ期，右侧Ⅲ期 双侧EDMS 无 1 0
17 女性 11 TIA 左侧Ⅲ期 左侧EDMS 无 1 0
图1 烟雾病患儿手术前后影像学表现。图a、b为术前正、侧位脑血管造影；图c、d为术后6个月正、侧位脑血管造影；图e为术前灌注成像；图f为术后6个月灌注成像
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