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脑-硬脑膜-颞肌-血管融通术治疗儿童烟雾病的远期血管造影和临床转归

Encephalo-duro-myo-synangiosis for the treatment of children w ith moyamoya disease:long-term angiography and clinical outcome

摘要:

目的:探讨脑-硬脑膜-肌肉血管融合术(encephalo-duro-myo-synangiosis, EDMS )治疗儿童烟雾病的远期血管造影和临床转归。方法回顾性分析接受 EDMS 治疗的烟雾病患儿术前、术后临床和影像学资料。结果共纳入21例烟雾病患儿,男性8例,女性13例,年龄4~16岁。首发症状:短暂性脑缺血发作15例,缺血性卒中4例,出血性卒中2例。 Matsushima 临床分型:Ⅰ型8例,Ⅱ型7例,Ⅳ型3例,Ⅴ型1例,Ⅵ型2例。铃木分期:Ⅱ期4例,Ⅲ期11例,Ⅳ期5例,Ⅴ期1例。10例行双侧手术,11例行单侧手术,共计31例侧。随访13~91个月,平均39.8个月。围手术期出现短暂性脑缺血发作3例侧,脑梗死2例侧,颜面水肿7例侧,无死亡病例。术后1年临床显著改善者14例侧(45.2%),良好者13例侧(41.9%),一般者4例侧(12.9%)。术后改良 Rankin 量表(modified Rankin Scale, mRS)评分0~2分的患儿比例显著高于术前[95.2%(20/21)对71.4%(15/21);χ2=4.29,P =0.041]。31例侧复查脑血管造影均可见脑膜中动脉、颞中深动脉不同程度地参与脑皮质供血,25例侧(80.6%)代偿良好,6例侧(19.4%)代偿一般。结论应用 EDMS 治疗烟雾病儿童的远期血管造影和临床转归良好。

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abstracts:

Objective To investigate the long -term angiography and clinical outcome of encephalo -duro-myo-synangiosis (EDMS) for the treatment of children w ith moyamoya disease. Methods The clinical and imaging data of before and after procedure in children w ith moyamoya disease treated w ith EDMS w ere analyzed retrospectively. Results A total of 21 children w ith moyamoya disease w ere enroled, including 13 females and 8 males, aged 4 to 16 years. The initial symptom: transient cerebral ischemic attack in 15 cases, ischemic stroke in 4 cases, and hemorrhagic stroke in 2 cases. Matsushima clinical classification: type Ⅰ in 8 cases, type Ⅱ in 7 cases, type Ⅳ in 3 cases, type Ⅴ in 1 case, and type Ⅵ in 2 cases. Suzuki stage: stageⅡ in 4 cases, stage Ⅲ in 11 cases, stage Ⅳ in 5 cases, and stage Ⅴ in 1 case. Ten children underw ent bilateral operation and 11 underw ent unilateral operation (a total of 31 sides). They w ere folow ed up for 13 to 91 months (mean 39.8 months). Three children had transient ischemic attack, 2 had cerebral infarction, 7 had facial edema, and none of them died during the perioperative period. The clinical symptoms w ere improved significantly in 14 sides (45.2%), good in 13 sides (41.9%), and general in 4 sides (12.9%) 1 year after operation. The proportion of children w ith modified Rankin Scale (mRS) score 0-2 after operation w as significantly higher than that before procedure (95 .2% vs.71.4%; χ2 = 4.29, P = 0.041). The middle meningeal artery and deep temporal artery participated in the blood supply of cerebral cortex in different degrees w ere observed by cerebral angiography again for 31 sides, excelent in 25 (80 .6%) and fair in 6 (19.4%). Conclusions The long-term angiography and clinical outcome in children w ith moyamoya disease treated w ith EDMS is good.

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作者: 张萌 [1] 郝继恒 [1] 张利勇 [1] 张士刚 [1] 王继跃 [1]
栏目名称: 烟雾病
DOI: 10.3760/cma.j.issn.1673-4165.2016.02.006
发布时间: 2016-05-19
基金项目:
国家“十二五”科技支撑计划项目(2011BAI08B04)Fund program National Science & Technology Pilar Program during the Tw elfth Five -Year Plan Period
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