Document Detail


PHACES association: a vasculocutaneous syndrome.
MedLine Citation:
PMID:  18427881     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PHACES association is a spectrum of anomalies that might occur in infants with large facial hemangiomas. Most infants with PHACES association have segmental hemangiomas of the head or neck. Cardiac and cerebrovascular anomalies might be the most important association, as they carry a significant risk of complications. This article summarizes the dermatologic, cardiac, and cerebral vascular findings in a cohort of infants diagnosed with PHACES association. All had large segmental facial hemangiomas and aortic arch abnormalities. Four of the five were not suspected of having arch obstruction prior to imaging studies because of the aberrant origin of both subclavian arteries, and 4/5 required either interventional or surgical repair for arch obstruction. In contrast to classic aortic coarctation, the aortic anomalies found in the cohort had unusually complex and unpredictable anatomic involvement. Cerebral vascular anomalies were identified in 5/5, and 2/5 had neurologic complications secondary to abnormal cerebral vascular supply. It is important for care providers to recognize this association that presents with a cutaneous stigma, as it is associated with potentially lethal and often unrecognized vascular anomalies. Earlier recognition of the associated vascular pathologies might enable preemptive treatments before potentially devastating and irreversible sequelae.
Authors:
Rohit P Rao; Beth A Drolet; Kristen E Holland; Peter C Frommelt
Publication Detail:
Type:  Journal Article     Date:  2008-04-22
Journal Detail:
Title:  Pediatric cardiology     Volume:  29     ISSN:  0172-0643     ISO Abbreviation:  Pediatr Cardiol     Publication Date:  2008 Jul 
Date Detail:
Created Date:  2008-07-07     Completed Date:  2008-12-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8003849     Medline TA:  Pediatr Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  793-9     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Divisions of Cardiology and Critical Care, Medical College of Wisconsin, Milwaukee, WI, 53226, USA. rrao@chw.org
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple*
Aorta, Thoracic / abnormalities*
Face
Female
Hemangioma / complications*,  therapy
Humans
Infant
Infant, Newborn
Male
Skin Neoplasms / complications*,  therapy
Syndrome
Vascular Malformations / complications*,  surgery*

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


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