Moyamoya disease in children.

PURPOSE: Moyamoya disease, a rare cause of pediatric stroke, is a cerebrovascular occlusive disorder resulting from progressive stenosis of the distal intracranial carotid arteries and their proximal branches. In response to brain ischemia, there is the development of basal collateral vessels, which give rise to the characteristic angiographic appearance of moyamoya. If left untreated, the disease can result in overwhelming permanent neurological and cognitive deficits.

METHODS

Whereas moyamoya disease refers to the idiopathic form, moyamoya syndrome refers to the condition in which children with moyamoya also have a recognized clinical disorder. As opposed to adults who typically present in the setting of intracranial hemorrhage, the classic pediatric presentation is recurrent transient ischemic attacks and/or completed ischemic strokes.

RESULTS

Surgical revascularization, including direct and indirect techniques, remains the mainstay of treatment, and has been shown to improve long-term outcome in children with moyamoya.

CONCLUSION

The authors discuss the diagnosis and treatment of moyamoya disease in the pediatric population.