Moyamoya disease in children.
OBJECTIVE
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.


CONCLUSIONS
The authors discuss the diagnosis and treatment of moyamoya disease in the pediatric population.
Ibrahimi
David M
DM
Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Tamargo
Rafael J
RJ
Ahn
Edward S
ES
D000328:Adult; D000367:Age Factors; D002545:Brain Ischemia / Q000503:physiopathology*; D002533:Cerebral Angiography; D002548:Cerebral Revascularization / Q000379:methods*; D002648:Child; D006801:Humans; D002546:Ischemic Attack, Transient / Q000503:physiopathology* / Q000517:prevention & control; D009072:Moyamoya Disease / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery*; D020521:Stroke / Q000503:physiopathology*; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods