Moyamoya Disease: A Cerebral Disorder of Children

Abstract

Moyamoya disease is an increasingly recognized arteriopathy associated with cerebral ischemia and has been associated with approximately 6% of childhood strokes. It is characterized by chronic progressive stenosis at the apices of the intracranial internal carotid arteries (ICA), including the proximal anterior cerebral arteries and middle cerebral arteries. Occurring in tandem with reduction in flow in the major vessels of the anterior circulation of the brain, there is compensatory development of collateral vasculature by small vessels near the carotid apices, on the cortical surface, leptomeninges, and branches of the external carotid artery supplying the dura and skull base. Moyamoya is an increasingly recognized entity associated with cerebral ischemia. Diagnosis is made from clinical and radiographic findings. Surgical revascularization is recommended for definitive treatment of children with moyamoya syndrome. Moyamoya is an increasingly recognized cause of stroke in both children and adults. Patients with certain conditions such as Down’s syndrome19 and sickle cell disease may be particularly at risk for moyamoya. Characteristic radiographic findings confirm the diagnosis, and recognition of the disease early in its course, with prompt institution of therapy, is critical in order to achieve the best outcome in patients. Revascularization surgery appears to be effective in preventing stroke in patients with moyamoya.