| Risk for PHACE syndrome in infants with large facial hemangiomas. | |
| | |
MedLine Citation:
|
PMID: 20643720 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
OBJECTIVES: This study was conducted to determine the prevalence of posterior fossae of the brain, arterial anomalies, cardiac anomalies, and eye anomalies (PHACE) in infants with large facial hemangiomas. The extracutaneous manifestations of PHACE may be associated with significant morbidity, and the prevalence of PHACE in patients with facial hemangiomas has not previously been reported. METHODS: A multicenter prospective study was conducted with 108 infants who had large facial hemangiomas and were systematically evaluated for manifestations of PHACE. The prevalence of PHACE and its extracutaneous manifestations in this cohort was calculated. The relationship between hemangioma distribution and the manifestations of PHACE was analyzed. RESULTS: Thirty-three (31%) of 108 had PHACE. Thirty of the 33 patients with PHACE had >1 extracutaneous finding. The risk for PHACE syndrome was higher in infants with larger hemangiomas and in those with hemangiomas that encompassed >1 facial segment. The most common extracutaneous anomalies observed in infants with PHACE were of the arteries of the cerebrovasculature (91%) and cardiac anomalies (67%). Upper face (frontotemporal and frontonasal) hemangiomas were commonly observed in infants with PHACE; isolated maxillary hemangiomas were rarely associated with PHACE. CONCLUSIONS: In infants with large facial hemangiomas, one-third have extracutaneous manifestations consistent with the diagnosis of PHACE syndrome, most commonly cerebrovascular and cardiovascular anomalies. The high prevalence of arterial anomalies in this cohort has implications for clinical management and future research regarding the pathophysiology of PHACE. |
| | |
Authors:
|
Anita N Haggstrom; Maria C Garzon; Eulalia Baselga; Sarah L Chamlin; Ilona J Frieden; Kristen Holland; Sheilagh Maguiness; Anthony J Mancini; Catherine McCuaig; Denise W Metry; Kimberly Morel; Julie Powell; Susan M Perkins; Dawn Siegel; Beth A Drolet |
Publication Detail:
|
Type: Journal Article Date: 2010-07-19 |
Journal Detail:
|
Title: Pediatrics Volume: 126 ISSN: 1098-4275 ISO Abbreviation: Pediatrics Publication Date: 2010 Aug |
Date Detail:
|
Created Date: 2010-08-03 Completed Date: 2010-09-02 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 0376422 Medline TA: Pediatrics Country: United States |
Other Details:
|
Languages: eng Pagination: e418-26 Citation Subset: AIM; IM |
Affiliation:
|
Department of Dermatology and Pediatrics, Division of Biostatistics, Indiana University, Indianapolis, Indiana, USA. ahaggstr@iupui.edu |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Abnormalities, Multiple
/
pathology Airway Obstruction / complications Cerebrovascular Disorders / complications*, pathology* Ear Diseases / complications* Eye Diseases / complications* Facial Neoplasms / complications*, pathology* Female Gestational Age Heart Defects, Congenital / complications* Hemangioma / complications*, pathology* Humans Infant Infant, Newborn Male Neurocutaneous Syndromes / complications*, pathology* Prospective Studies Syndrome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Electronic health record decision support and quality of care for children with ADHD.
Next Document: Changes and Variability in High Levels of Low-Density Lipoprotein Cholesterol Among Children.