Abstract |
PHACE association is a rare neurocutaneous condition in which facial hemangiomas associate with a spectrum of posterior fossa malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, and eye anomalies. We reported a case of PHACE association in a premature infant showing facial, intracranial, and oropharyngeal hemangiomas with evidence of the Dandy-Walker variant and complicated cardiovascular anomalies, including a right-sided aortic arch and an atypical patent ductus arteriosus arising from a tortuous left subclavian artery. To our knowledge, intracranial hemangiomas are rare in PHACE association, and a concomitant oropharyngeal hemangioma has not been previously reported in the PHACE association literature. In infants presenting with large, plaque-like facial hemangiomas, it is important to conduct active cardiovascular and neurological evaluations. Special attention should be given to the laryngoscopic examination to search for additional hemangiomas in the airway.
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Authors | Do-Hyun Kim, Jang Hwan Choi, Jung Ha Lee, Hee Sup Kim |
Journal | Korean journal of pediatrics
(Korean J Pediatr)
Vol. 55
Issue 1
Pg. 29-33
(Jan 2012)
ISSN: 2092-7258 [Electronic] Korea (South) |
PMID | 22359528
(Publication Type: Journal Article)
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