Understanding and treating moyamoya disease in children

Neurosurg Focus. 2009 Apr;26(4):E4. doi: 10.3171/2000.01.FOCUS08306.


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.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Cerebral Arteries / pathology
  • Cerebral Arteries / physiopathology*
  • Cerebral Arteries / surgery*
  • Cerebral Revascularization / methods
  • Cerebral Revascularization / statistics & numerical data
  • Cerebral Revascularization / trends
  • Child
  • Diagnostic Imaging / methods
  • Diagnostic Imaging / trends
  • Genetic Predisposition to Disease / genetics
  • Humans
  • Moyamoya Disease / diagnosis
  • Moyamoya Disease / physiopathology*
  • Moyamoya Disease / surgery*
  • Neurosurgical Procedures / methods*
  • Neurosurgical Procedures / trends
  • Predictive Value of Tests
  • Stroke / physiopathology
  • Stroke / prevention & control
  • Stroke / surgery
  • Vascular Surgical Procedures / methods*
  • Vascular Surgical Procedures / trends