| A patient with PHACE syndrome with marked ipsilateral cerebral atrophy. | |
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MedLine Citation:
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PMID: 20417465 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Nan-Koong Wang; Kun-Long Hung; Hon-Chai Liao; Yung-Ming Lin |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Pediatrics and neonatology Volume: 51 ISSN: 1875-9572 ISO Abbreviation: Pediatr Neonatol Publication Date: 2010 Apr |
Date Detail:
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Created Date: 2010-04-26 Completed Date: 2010-05-27 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101484755 Medline TA: Pediatr Neonatol Country: Singapore |
Other Details:
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Languages: eng Pagination: 130-4 Citation Subset: IM |
Copyright Information:
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Copyright 2010 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved. |
Affiliation:
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Department of Pediatrics and Cardiac Surgery, Cathay General Hospital, Taipei, Taiwan. nankoon@ms14.hinet.net |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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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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