Understanding and treating moyamoya disease in children

L. Smith Jodi

Research output: Contribution to journalReview article

8 Scopus citations

Abstract

Moyamoya disease, a known cause of pediatric stroke, is an unremitting cerebrovascular occlusive disorder of unknown etiology that can lead to devastating, permanent neurological disability if left untreated. It is characterized by progressive stenosis of the intracranial internal carotid arteries and their distal branches and the nearly simultaneous appearance of basal arterial collateral vessels that vascularize hypoperfused brain distal to the occluded vessels. Moyamoya disease may be idiopathic or may occur in association with other syndromes. Most children with moyamoya disease present with recurrent transient ischemic attacks or strokes. Although there is no definitive medical treatment, numerous direct and indirect revascularization procedures have been used to improve the compromised cerebral circulation, with outcomes varying according to procedure type. Such techniques improve the long-term outcome of patients with both idiopathic and syndrome-associated moyamoya disease. This review provides a comprehensive discussion of moyamoya disease in children, with an emphasis on the most effective surgical treatment options.

Original languageEnglish (US)
Pages (from-to)1-11
Number of pages11
JournalNeurosurgical focus
Volume26
Issue number4
DOIs
StatePublished - Oct 13 2009

Keywords

  • Cerebral revascularization
  • Children
  • Ischemia
  • Moyamoya disease
  • Pial synangiosis
  • Stroke

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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