Moyamoya disease and moyamoya syndrome in children : case reports and a literature review

Background: Moyamoya disease is a rare angiopathy (vascular disorder) that leads to a narrowing and occlusion of the distal internal carotid artery and proximal anterior and middle cerebral arteries. The presence of a rich network of collateral blood vessels at the base of the brain is called "moyamoya syndrome". Moyamoya disease is diagnosed in patients with unidentified risk factors. Moyamoya disease should rather be excluded and moyamoya syndrome diagnosed in patients with conditions and risk factors which may promote these specific vascular lesions. Case Reports: Three cases of the disease in pediatric patients have been described in this article. The first one concerns a nine-year-old girl who experienced ischemic stroke and had cerebral vascular abnormalities typical of moyamoya disease. The second case is a seventeen-year-old female patient with a history of subarachnoid hemorrhage history. Also in this patient, clinical features suggested moyamoya disease. The third case report concerns a thirteen-year-old girl with type I neurofibromatosis and a history of transient ischemic attack (TIA), who was diagnosed with moyamoya syndrome. Conclusions: Moyamoya disease occurs both in pediatric and adult patients. In pediatric patients, this medical condition should be considered if cerebral ischemia of unknown origin or acute neurological deficits develop.