engleski

Moyamoya in children: Clinical presentation, diagnostic procedure, neurosurgical treatment and outcome

INTRODUCTION: Moyamoya is cronic cerebrovascular disease, characterized by progressive constriction and finally occlusion, of the terminal parts of internal carotid artery and proximal parts of the middle and anterior cerebral arteries, resulting in collateral circulation in the thalamus and basal ganglia. Blood vessels, representing collateral flow in areas of the brain hypoperfusion distal to the stenosis, on the DSA look like "cigarette smoke", which in Japanese is called "moyamoya”. Moyamoya is a common cause of cerebral ischemic stroke in children. Due to high morbidity and mortality rate in untreated patients, revascularization surgery has became the standard therapy in children with Moyamoya. PATIENTS AND METHODS: We present nine patients, five girls and four boys, of different age (2-­‐10 years, peak 5-­‐6 years) and with different clinical picture (transient hemiparesis in 5/9 children, convulsions in 4 / 9 children, headaches in 2/9 and dizziness in 1 child), admitted to our clinic. By processing (Transcranial color Doppler, MRI / MRA and DSA) in all progressive occlusive cerebrovascular arteriopathy was proven (Moyamoya disease in 6/9 and Moyamoya syndrome in 3/9 children). Six of the nine patients underwent neurosurgical revascularization with variable outcome. One patient in young age had extremely progressive course of the disease, developing tetrapareses, epilepsy, blindness, motor aphasia and dysphagia, renal failure and death. Other patients had a better outcome: 3/6 operated have orderly development, and the two have mild cognitive deterioration. CONCLUSION: Unfavorable outcome have small children with bilateral ischemic infarctions and fixed neurological deficit. The clinical presentation and neurological status at the moment of diagnosis are most important prognostic factor and are predictors of long term outcome. Symptomatic patients perform surgical revascularization to reduce the risk of future stroke.

Moyamoya; children

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