Síndrome y enfermedad de Moyamoya

moyamoya disease is a cerebrovascular entity of unknown etiology, not frequent on western population, recognized as an important cause of ischemic event at pediatric age for a progressive estenosis of the internal carotide on its intracraneal portion and main branches. provoking a flow reduction at the main vessels, causing the compensatory development of small collateral vessels. the handling could be preservative or with neurosurgical techniques. we do describe 4 pediatric cases with this pathology, diagnosed, treated and followed by the neuropaediatry service. one of the patients has moyamoya syndrome. three patients did receive conservative treatment and one of them required surgical intervention.