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¹Selçuk Üniversitesi, Tıp Fakültesi, Nöroşirürji Anabilim Dalı, Konya, Türkiye
²Kocaeli Üniversitesi, Tıp Fakültesi, Nöroşirürji Anabilim Dalı, Kocaeli, Türkiye Moyamoya disease is a chronic and progressive cerebrovascular disease characterized by stenosis or occlusion of the bilateral terminal internal carotid arteries at the base of the brain forming Willis’s polygon, and results in the characteristic development of an abnormal collateral vascular network. The disease has been named “moyamoya” coming from the Japanese word which means “something hazy just like a puff of cigarette smoke drifting in the air”. The cause of Moyamoya disease is unknown. Although the coexistence of Moyamoya disease and tumoral diseases has been shown, mostly genetic and congenital factors are suggested. It is more frequent in East Asian race and females are slightly more predisposed. The disease is mostly presented with ischemic findings as; strokes, transient ischemic attacks, and seizures in pediatric age group while adults more often present with intracranial haemorrhage. MRI and magnetic resonance angiography is generally used for diagnosis however cerebral digital substraction angiography is still the gold standard. The aim of treatment is to prevent ischemia and avoid haemorrhage. In early stages, medical treatment can be useful however surgical anastomosis of intracranial and extracranial vascular supply to improve the cortical blood flow directly or in an indirect way is well accepted. The ideal cases for surgical treatment are the ones with repeated or progressive transient ischemic attacks and with reduced cerebral perfusion reserves. Anahtar Kelimeler : Cerebrovascular, Moyamoya disease, Pediatric