Document Detail


A patient with PHACE syndrome with marked ipsilateral cerebral atrophy.
MedLine Citation:
PMID:  20417465     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The association of posterior fossa malformation, facial cavernous hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects and eye abnormalities (PHACE syndrome) represents a rare congenital anomaly with a broad spectrum of clinical manifestations and female predominance. We herein report on a girl who manifested the typical clinical features of PHACE syndrome, unusually associated with severe ipsilateral cerebral atrophy and hemiplegia. She received surgical aortoplasty, local steroid injection and laser therapy for the hemangioma, and intense physical therapy soon after diagnosis. The etiology of PHACE syndrome remains unclear, and its clinical spectrum is broad. The current case suggests that the spectrum of PHACE syndrome should be further expanded to include other forms of cerebral disorder.
Authors:
Nan-Koong Wang; Kun-Long Hung; Hon-Chai Liao; Yung-Ming Lin
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatrics and neonatology     Volume:  51     ISSN:  1875-9572     ISO Abbreviation:  Pediatr Neonatol     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-04-26     Completed Date:  2010-05-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101484755     Medline TA:  Pediatr Neonatol     Country:  Singapore    
Other Details:
Languages:  eng     Pagination:  130-4     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.
Affiliation:
Department of Pediatrics and Cardiac Surgery, Cathay General Hospital, Taipei, Taiwan. nankoon@ms14.hinet.net
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple*
Aorta / pathology
Aorta, Thoracic / abnormalities
Atrophy
Brain / pathology*
Carotid Artery, Internal / abnormalities
Cerebellum / pathology
Cerebral Ventricles / pathology
Cranial Fossa, Posterior / abnormalities
Dilatation, Pathologic
Female
Hemangioma, Cavernous / congenital*
Humans
Infant
Magnetic Resonance Imaging
Syndrome

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


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