小儿烟雾病：临床特征、文献综述及一家三级医疗教学机构的16年经验
Pediatric moyamoya disease: clinical profile, literature review and sixteen year experience from a tertiary care teaching institute.
作者信息
Singhi Pratibha, Choudhary Anita, Khandelwal Niranjan
机构信息
Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, 160017, India,
出版信息
Indian J Pediatr. 2013 Dec;80(12):1015-20. doi: 10.1007/s12098-013-1000-4. Epub 2013 Mar 24.
OBJECTIVE
To assess the clinical spectrum and outcome of pediatric moyamoya disease (MMD) in Indian sub-continent.
METHODS
The authors retrospectively analysed data of 23 patients, diagnosed with pediatric moyamoya disease from a single center during the period of 1996-2011.
RESULTS
There were 18 boys and 5 girls. Mean age at onset of symptoms was 3.8 ± 2.2 y. All patients presented with cerebral ischemic events. Recurrent stroke was the presenting feature in 12(52.2 %) patients. Twenty one patients were definitive case of moyamoya disease and two were probable case of moyamoya disease. Posterior circulation was involved in 26.1 % patients. Three patients underwent indirect surgical revascularisation procedure and rest were managed conservatively. On follow up 66.6 % patients had residual neurological deficit.
CONCLUSIONS
Heightened awareness of this entity among pediatric neurologists is required for early diagnosis of pediatric moyamoya disease.
目的
评估印度次大陆小儿烟雾病（MMD）的临床谱及预后。
方法
作者回顾性分析了1996年至2011年期间在单一中心诊断为小儿烟雾病的23例患者的数据。
结果
有18名男孩和5名女孩。症状出现时的平均年龄为3.8±2.2岁。所有患者均表现为脑缺血事件。12例（52.2%）患者以复发性中风为首发特征。21例为烟雾病确诊病例，2例为烟雾病可能病例。26.1%的患者后循环受累。3例患者接受了间接手术血管重建术，其余患者接受保守治疗。随访时，66.6%的患者有残留神经功能缺损。
结论
小儿神经科医生需要提高对该疾病的认识，以便早期诊断小儿烟雾病。
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