Otolaryngologic manifestations of PHACE syndrome

Dana S. Smith, Ken K. Lee, Henry A. Milczuk

Research output: Contribution to journalArticle

18 Scopus citations

Abstract

Two case reports of PHACE syndrome (posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), coarctation of the aorta and cardiac defects (C), and eye abnormalities (E)) are presented. Clinical characteristics consisted of cutaneous and airway hemangiomas, aortic coarctation, and left superior vena cava in one child and cutaneous and airway hemangiomas, sternal clefting, and supraumbilical raphe in the other child. Treatment modalities included systemic and intralesional steroids for cutaneous and airway hemangiomas, submucosal resection and laryngotracheal reconstruction for airway hemangiomas, repair of aortic coarctation, and laser treatment of cutaneous hemangiomas. PHACE syndrome poses a significant potential for airway compromise from hemangiomas, which may require multimodality treatment.

Original languageEnglish (US)
Pages (from-to)1445-1450
Number of pages6
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume68
Issue number11
DOIs
StatePublished - Nov 2004

Keywords

  • Airway hemangioma
  • Neurocutaneous syndrome
  • Pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Otorhinolaryngology

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