Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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PHACE association with intracranial, oropharyngeal hemangiomas, and an atypical patent ductus arteriosus arising from the tortuous left subclavian artery in a premature infant

  • Kim, Do-Hyun (Department of Pediatrics, Dongguk University Ilsan Hospital) ;
  • Choi, Jang-Hwan (Department of Pediatrics, Dongguk University Ilsan Hospital) ;
  • Lee, Jung-Ha (Department of Pediatrics, Dongguk University Ilsan Hospital) ;
  • Kim, Hee-Sup (Department of Pediatrics, Dongguk University Ilsan Hospital)
  • 투고 : 2011.02.22
  • 심사 : 2011.07.21
  • 발행 : 2012.01.15
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초록

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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참고문헌

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피인용 문헌

  1. Enlargement of the Internal Auditory Canal and Associated Posterior Fossa Anomalies in PHACES Association vol.36, pp.11, 2012, https://doi.org/10.3174/ajnr.a4395
  2. The Most Common Comorbidities in Dandy-Walker Syndrome Patients: A Systematic Review of Case Reports vol.32, pp.10, 2017, https://doi.org/10.1177/0883073817712589
  3. Truncus arteriosus as presentation of PHACE syndrome vol.14, pp.9, 2012, https://doi.org/10.1136/bcr-2021-244620