PHACE association with intracranial, oropharyngeal hemangiomas, and an atypical patent ductus arteriosus arising from the tortuous left subclavian artery in a premature infant

• Main Authors: Do-Hyun Kim, Jang Hwan Choi, Jung Ha Lee, Hee Sup Kim
• Format: Article
• Language: English
• Published: Korean Pediatric Society 2012-01-01
• Series: Korean Journal of Pediatrics
• Subjects: PHACE association; Central nervous system cavernous hemangioma; Patent ductus arteriosus; Premature infants
• Online Access: http://kjp.or.kr/upload/pdf/kjped-55-29.pdf
• ISSN: 1738-1061; 2092-7258

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.