| PHACES association: a vasculocutaneous syndrome. | |
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MedLine Citation:
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PMID: 18427881 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Rohit P Rao; Beth A Drolet; Kristen E Holland; Peter C Frommelt |
Publication Detail:
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Type: Journal Article Date: 2008-04-22 |
Journal Detail:
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Title: Pediatric cardiology Volume: 29 ISSN: 0172-0643 ISO Abbreviation: Pediatr Cardiol Publication Date: 2008 Jul |
Date Detail:
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Created Date: 2008-07-07 Completed Date: 2008-12-04 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8003849 Medline TA: Pediatr Cardiol Country: United States |
Other Details:
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Languages: eng Pagination: 793-9 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Divisions of Cardiology and Critical Care, Medical College of Wisconsin, Milwaukee, WI, 53226, USA. rrao@chw.org |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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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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