儿童烟雾病的临床及影像学特点

Clinical and imaging characteristics of moyamoya disease in children

目的分析儿童烟雾病临床及影像学特点。方法对2002年1月至2011年6月在首都医科大学附属北京天坛医院经数字减影血管造影（DSA）或磁共振血管成像确诊的141例烟雾病患儿病历资料进行回顾性分析。结果141例患儿中，男77例，女64例。发病年龄2—18岁[（9．50±4．06）岁]，5—10岁为发病高峰，共71例（50．4％）。以缺血为首发症状者136例（96．45％），出血为首发症状者5例（3．55％）。常见的首发症状包括肢体无力[80例（56．7％）]、头痛[41例（29．1％）]、失语或构音障碍E24例（17．0％）]，其他表现包括抽搐发作、面瘫、眩晕、眼部症状、感觉症状等。113例行DSA检查的患儿中，44例为颈内动脉末端病变，69例为颈内动脉末端合并大脑中动脉或大脑前动脉或大脑后动脉病变。56例（52．8％）患儿行间接血运重建术，39例（36．8％）行直接血运重建术，7例（6．6％）行直接＋间接血运重建术，4例（3．7％）行血管栓塞术或病变血管切除术。未经手术治疗的患儿予保守治疗。结论儿童烟雾病多以缺血性卒中为首发症状，颈内动脉末端、大脑中动脉或大脑前动脉起始部最常累及，DSA是确诊的首选检查。

Objective To summarize the clinical and imaging characteristics of moyamoya disease in children. Methods The clinical data of 141 children with moyamoya disease who were hospitalized and diagnosed by digital subtraction angiography （ DSA ） or magnetic resonance angiography in Beijing Tiantan Hospital Affiliated to the Capital Medical University from Jan. 2002 to Jun. 2011 were retrospectively analyzed. Results There were 77 boys and 64 girls,the onset age ranged from 2 to 18 years oldl median onset age was （9.50±4.06） years old] ,of which patients of 5 to 10 years old accounted for 50.4%. Among the 141 patients,cerebral ischemia occurred in 136 cases （96.45%） and was the commonest initial manifestation. Haemorrhage occurred in 5 patients （3.55%）. The clinical manifestations were different,including common symptoms such as paresis or hemiplegia 80 cases （56. 7% ） , headache 41 cases （29.1% ）, dysphasia 24 cases （ 17.0% ） and other symptoms such as epileptic seizures, facial palsy, dizziness, sensory disturbance and so on. One hundred and thirteen patients received DSA examination which showed that internal carotid artery was involved in 44 patients, internal carotid artery and middle cerebral artery or anterior cerebral artery or poste- rior cerebral artery were involved in 69 patients, 56 cases （52. 8% ） received indirect revascularization, 39 cases （36.8%） received direct revascularization, 7 cases （ 6.6% ） received direct and indirect revascularization, 4 cases （3.7%） received embolization or lesion resection. Patients without surgery received conservative treatment. Conclu- sions Most children with moyamoya has ischemic symptom at first. DSA is the optimal selection to give definite diag- nosis. The terminal of internal carotid artery and the beginning of middle cerebral artery or anterior cerebral artery are the most involved artery.