PHACE association with intracranial, oropharyngeal hemangiomas, and an atypical patent ductus arteriosus arising from the tortuous left subclavian artery in a premature infant.
- Author:
Do Hyun KIM
1
;
Jang Hwan CHOI
;
Jung Ha LEE
;
Hee Sup KIM
Author Information
- Publication Type:Case Report
- Keywords: PHACE association; Central nervous system cavernous hemangioma; Patent ductus arteriosus; Premature infants
- MeSH: Aorta, Thoracic; Aortic Coarctation; Ductus Arteriosus, Patent; Eye; Eye Abnormalities; Hemangioma; Hemangioma, Cavernous, Central Nervous System; Humans; Infant; Infant, Newborn; Infant, Premature; Neurocutaneous Syndromes; Subclavian Artery
- From:Korean Journal of Pediatrics 2012;55(1):29-33
- CountryRepublic of Korea
- Language:English
- 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.
