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 Vol. 11 No. 5, May 2004
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AAP Grand Rounds 11:55-56 (2004)
© 2004 American Academy of Pediatrics

NEUROSURGERY

Moyamoya Syndrome in Children is Treatable

Source: Scott RM, Smith JL, Robertson RL, et al. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg: Pediatrics. 2004;100:142–149.

The first 20% of the full text of this article appears below.

Moyamoya syndrome is defined by progressive occlusion of the internal carotid artery just beyond its point of entry into the cranial cavity. The insidious development of brain ischemia elicits the development of tiny collateral arterial vessels deriving from the external carotid distribution and the vertebrobasilar system. The cloud-like, indistinct appearance of these collateral vessels on cerebral arteriography gives rise to the esoteric name for this condition, a Japanese term meaning a puff of smoke.1 In children, the syndrome usually presents as cerebral ischemia, while in adults hemorrhage from fragile collateral vessels dominates the clinical picture. Moyamoya syndrome may be idiopathic, but most often arises in the setting of cerebral or systemic disease, such as . . . [Full Text of this Article]

Joseph H. Piatt, Jr, MD, FAAP
Neurological Surgery and Pediatrics, Drexel University College of Medicine, St. Christopher’s Hospital for Children, Philadelphia, PA