Moyamoya disease in children

David M. Ibrahimi, Rafael J Tamargo, Edward S. Ahn

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1297-1308
Number of pages12
JournalChild's Nervous System
Volume26
Issue number10
DOIs
StatePublished - Oct 2010

Fingerprint

Moyamoya Disease
Pediatrics
Stroke
Cerebrovascular Disorders
Intracranial Hemorrhages
Transient Ischemic Attack
Brain Ischemia
Carotid Arteries
Pathologic Constriction
Therapeutics
Population

Keywords

  • Cerebral revascularization
  • Moyamoya disease
  • Pediatric
  • Pial synangiosis
  • Stroke

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Moyamoya disease in children. / Ibrahimi, David M.; Tamargo, Rafael J; Ahn, Edward S.

In: Child's Nervous System, Vol. 26, No. 10, 10.2010, p. 1297-1308.

Research output: Contribution to journalArticle

Ibrahimi, David M. ; Tamargo, Rafael J ; Ahn, Edward S. / Moyamoya disease in children. In: Child's Nervous System. 2010 ; Vol. 26, No. 10. pp. 1297-1308.
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