Document Detail


Cervico-cerebrovascular anomalies in children with PHACE syndrome.
MedLine Citation:
PMID:  12923590     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: The acronym PHACE describes a rare neurocutaneous syndrome encompassing the following features: posterior fossa brain malformation, hemangioma, arterial anomalies, coarctation of aorta and cardiac defects, and eye abnormalities. This report describes the cervico-cerebrovascular anomalies (CCVA) in 7 patients with PHACE syndrome. METHODS: The medical records of 7 children (4 girls and 3 boys) with the diagnosis of PHACE syndrome who underwent cranial computed tomography and magnetic resonance imaging as well as complete cerebral angiography were retrospectively studied. RESULTS: All 7 patients had underlying CCVA. Neurologic symptoms were present in 5 of the 7 patients. CCVA included vascular stenosis and/or occlusion (n = 5), aberrant course or origin of arteries (n = 7), persistent embryonic carotid-vertebrobasilar anastomoses (n = 2) and fusiform or berry aneurysm formation (n = 5). Arterial agenesis or absence was found in 2 patients and hypoplastic arteries were present in 3 children. CONCLUSIONS: PHACE syndrome is associated with a high incidence of wide-spectrum CCVA. The most common CCVA is aberrant course or origin of arteries, followed by arterial stenosis and/or occlusion, aneurysm formation, arterial agenesis or hypoplasia and persistent carotid-vertebrobasilar anastomosis. Part of the CCVA may be associated with secondary neurologic sequelae. PHACE syndrome should be considered in any children with extensive facial hemangioma. Neuroimaging such as brain computed tomography, magnetic resonance imaging, and magnetic resonance angiography are recommended in order to search for structural cerebral defects and intracranial vascular alterations.
Authors:
Chao-Bao Luo; P Lasjaunias; Michael M H Teng; Feng Chi Chang; Jiing-Feng Lirng; Cheng-Yen Chang
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of the Formosan Medical Association = Taiwan yi zhi     Volume:  102     ISSN:  0929-6646     ISO Abbreviation:  J. Formos. Med. Assoc.     Publication Date:  2003 Jun 
Date Detail:
Created Date:  2003-08-18     Completed Date:  2003-10-28     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9214933     Medline TA:  J Formos Med Assoc     Country:  China (Republic: 1949- )    
Other Details:
Languages:  eng     Pagination:  379-86     Citation Subset:  IM    
Affiliation:
Department of Radiology, Taipei Veterans General Hospital and National Yang-Ming University School of Medicine, Taipei, Taiwan.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / pathology*
Cerebral Angiography
Cerebral Arteries / abnormalities*
Child, Preschool
Female
Head and Neck Neoplasms / pathology*
Hemangioma / pathology*
Humans
Magnetic Resonance Imaging
Male
Neurocutaneous Syndromes / pathology*
Retrospective Studies
Tomography, X-Ray Computed

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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