Clinical and imaging characteristics of moyamoya disease in children

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 old[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. Conclusions Most children with moyamoya has ischemic symptom at first.DSA is the optimal selection to give definite diagnosis.The terminal of internal carotid artery and the beginning of middle cerebral artery or anterior cerebral artery are the most involved artery. Key words: Moyamoya disease; Digital subtraction arteriography; Digital subtraction angiography; Magnetic resonance angiography; Child