中文摘要：

目的：探讨儿童烟雾病临床特征及实施脑硬膜颞浅动脉融通术（ EDAS ）的预后。方法根据患者第1次手术时的年龄,将2004年1月-2010年12月317例接受EDAS的儿童烟雾病患者分为3组：幼儿组（年龄〈3岁,16例）,学龄前组（年龄36岁,42例）,青少年组（〉617岁,259例）。回顾性分析患者的临床资料并对手术疗效进行评估。结果（1）3组患者中,手术干预前脑梗死发生率幼儿组（81．2%,13/16）和学龄前组（69．0%,29/42）显著高于青少年组（48．3%,125/259）,差异有统计学意义（χ2=11．741,P〈0．01）；（2）手术干预前原脑梗死体积增大或在不同部位脑梗死再发率幼儿组（62．5%,10/16）高于学龄前组（31．0%,13/42）和青少年组（3．9%,10/259）,差异有统计学意义（χ2=77．437,P〈0．01）；（3）总体预后优良率为86．4%（274/317）,3组间比较,差异有统计学意义（χ2=9．026,P〈0．02）。结论烟雾病幼儿患者病情进展迅速,临床预后差；对儿童烟雾病患者及早实施EDAS安全有效。

英文摘要：

Objective To investigate the clinical features of moyamoya disease in children and the prognosis of encephaloduroarteriosynangiosis （ EDAS） . Methods According to the age of first operated patients,317 children with moyamoya disease who received EDAS from January 2004 to December 2010 were divided into 3 groups：infant group （n=16,〈3 years of age）,preschool group （n=42,3 to 6 years of age）,and adolescent group （n=259,6 to 17 years of age）. The clinical data and the efficacy of operation of the patients were analyzed retrospectively. Results （1） Among the 3 groups of patients,the incidences of cerebral infarction in the infant group （81. 2%,13/16） or the preschool group （69. 0%,29/42） before procedure were significantly higher than the adolescent group （48. 3%,125/259）. There were significant differences （χ2 =11. 741,P〈0. 01）. （2） Before surgical intervention,the infarct volume enlargement or the recurrence of infarction rate at different parts of brain in the infant group （62. 5%,10/16） was higher than that of the preschool group （31. 0%,13/42） and adolescent group （3. 9%,10/259）. There was significant difference （χ2 =77. 437,P 〈0. 01）. （3） The overall rate of favourable prognosis was 86. 4% （274/317）. There were significant differences between the 3 groups （χ2 =9. 026,P〈0.02）. Conclusion The conditions of children with moyamoya disease progresses rapidly and their clinical prognosis is poor. It is safe and effective to perform EDAS early moyamoya disease in children.