儿童烟雾综合征的临床特征及外科手术疗效  

Clinical features and surgical efficacy of moyamoya syndrome in children

作　　者：叶挺 韩聪 段炼 

机构地区：[1]解放军第三○七医院神经外科,北京100071

出　　处：《中国脑血管病杂志》2015年第1期12-16,共5页Chinese Journal of Cerebrovascular Diseases

基　　金：国家自然科学基金面上项目(81171083)

摘　　要：目的探讨烟雾综合征患儿的临床特征及外科治疗预后。方法回顾性分析2002年12月至2013年10月解放军第三○七医院神经外科收治的12例烟雾综合征患儿的临床资料,其中11例患儿接受外科脑-硬脑膜-血管融通术(EDAS)治疗。以同期收治的550例烟雾病患儿为对照组。通过对比分析两组患儿的性别、发病年龄、首发症状、进展症状、铃木分期、影像学特征及手术疗效等临床资料,分析儿童烟雾综合征的临床特征及手术疗效。结果儿童烟雾综合征的男女性别比为1∶2,平均发病年龄为(12±5)岁。烟雾综合征患儿在首发症状(脑梗死及脑出血)和病情进展方面与对照组比较差异有统计学意义[分别为5/12比14.5%(80/550),3/12比61.8%(340/550),5/12比8.7%(48/550);均P<0.05]。随访期内,11例接受EDAS治疗的患儿中,7例未再发病,4例症状明显改善;手术后改良Rankin量表(mRS)评分与术前比较,差异有统计学意义[0(0,1)分比2(1,2)分,P<0.05]。结论儿童烟雾综合征在临床特征方面与烟雾病有一定差异。及时行EDAS治疗可以阻止病情进展,改善患儿预后。Objective Toinvestigatetheclinicalfeaturesandsurgicalprognosisofmoyamoya syndromeinchildren.Methods Theclinicaldataof12childrenwithmoyamoyasyndromeadmittedto the 307th Hospital of People′s Liberation Army from December 2002 to October 2013 were analyzed retrospectively. Eleven of them underwent encephalo-duro-arterio-synangiosis （EDAS）. A total of 550 children with moyamoya disease in the same period were used as a control group. The clinical characteristics and surgical efficacy of the children with moyamoya syndrome were summarized and concluded by comparing the clinical data of the two groups,including sex,age of onset,initial symptom,progress symptoms, Suzukiinstallments,imagingfeatures,andsurgicalefficacy.Results Themaleandfemaleratioof the children with moyamoya syndrome was 1∶2. Their mean age of onset was 12 ＆#177; 5 years old. There were significant differences in the initial symptom （cerebral infarction and cerebral hemorrhage ）and disease progress between the children with moyamoya syndrome group and the control group （5/12 vs. 14. 5%[80/550], 3/12 vs. 61. 8%[340/550],and 5/12 vs. 8.7%[48/550],respectively;all P〈0. 05）. Within the follow-up period,of the 11 children underwent EDAS,7 cases had no further attack,and 4 cases were improved significantly. There was significant difference in the modified Rankin scale （mRS）between the beforeandaftersurgery（0[0,1]vs.2[1,2];P〈0.05）.Conclusions Theclinicalfeaturesofthe children with moyamoya syndrome have some differences with those with moyamoya disease. Timely and effective EDAS treatment may effectively prevent disease progression and improve the prognosis of patients.

关 键 词：烟雾综合征 儿童 烟雾病 脑-硬脑膜-血管融通术 

分 类 号：R726.5[医药卫生—儿科]