Document Detail


Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines.
MedLine Citation:
PMID:  22462697     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Moyamoya is a progressive arteriopathy of unknown origin affecting the branches of the internal carotid artery (ICA). The arteriopathy can present as an isolated medical condition, affecting both sides of the brain ("moyamoya disease") or can be unilateral or found in association with systemic disorders ("moyamoya syndrome"). The ischemia resulting from luminal narrowing predisposes children to transient ischemic attacks and stroke-the primary presentations of affected patients. Although it is rare-affecting 1 in 1 million children in the US-moyamoya is implicated in 6% of all childhood strokes. Diagnosis is defined by characteristic findings on arteriograms, including stenosis of the branches of the ICA and a pathognomonic spray of small collateral vessels in this region, descriptively likened to a "puff of smoke" ("moyamoya" in Japanese). Treatment is predicated on restoration of cerebral blood flow by surgical revascularization. The rarity of this disorder has limited research and the development of evidence-based clinical management. While acknowledging these limitations, in this article the authors aim to summarize current studies of pediatric moyamoya, with the objective of providing a framework for construction of evidence-based guidelines for treatment. The compilation of current data in these guidelines should serve as a resource to aid pediatric neurosurgeons in their role as advocates for providing appropriate care to affected children.
Authors:
Edward R Smith; R Michael Scott
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of neurosurgery. Pediatrics     Volume:  9     ISSN:  1933-0715     ISO Abbreviation:  J Neurosurg Pediatr     Publication Date:  2012 Apr 
Date Detail:
Created Date:  2012-04-02     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101463759     Medline TA:  J Neurosurg Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  353-60     Citation Subset:  IM    
Affiliation:
Department of Neurosurgery, Children's Hospital Boston, Harvard Medical Center, Boston, Massachusetts.
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