儿童单侧出血型烟雾病脑血运重建术的中长期 疗效及预后分析

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【中图分类号】R743；R651 【文献标志码】A 【文章编号】1672-7770（2025）02-0121-05

Abstract： Objective To explore the medium-andlong-term curative effects after cerebrovascular revascularization for children with unilateral hemorrhagic moyamoya disease （MMD）. Methods The clinical data of 13 pediatric patients with unilateral hemorrhagic MMD who underwent treatment at Beijing Tiantan Hospital from October 2010 to February 2025 were analyzed retrospectively. Among them， 1l received combined superficial temporal artery-middle cerebral artery（ STA-MCA） bypass with encephalo-duro-arterio-synangiosis（ EDAS），1 underwent EDAS alone， and 1 was treated conservatively. Follow-up endpoints included rebleeding， cerebral infarction， and death. ResultsAmong 13 children， intraventricular hemorrhage predominated in 4 cases （38.5% ）. During a median follow-up of 93 months（IQR 51 -133）， rebleeding occurred in 1 patient （7.7%） at 29 months post-EDAS surgery. Additionally， 1 patient（ 7.7% ）experienced a transient ischemic attack （ TIA） 2 months after combined revascularization， followed by a new cerebral infarction at 7 months postoperatively. No mortality or contralateral progression was observed on follow-up computed tomography angiography（CTA）. Favorable neurological outcomes （modified Rankin Scale score ？2 ）were achieved in 84.6%（11/13）of cases. Conclusions Unilateral hemorrhagic MMD in pediatric patients shows a low risk of contralateral progression during medium-and long-term follow-up.Combined cerebral revascularization effectively mitigates rebleeding risk and ensures favorable neurological outcomes， highlighting its safety and efficacy in pediatric populations.

Key words： moyamoya disease； cerebrovascular revascularization； child； neurological prognosis； follow-up study

烟雾病（moyamoyadisease，MMD）是一种以颅内动脉末端慢性进行性狭窄/闭塞伴代偿性烟雾状血管网形成为特征的脑血管疾病，其病因尚未完全阐明，可能与遗传（如RNF213基因突变）、免疫异常或环境因素相关[1-3]。（剩余8276字）