Skip to main content
eScholarship
Open Access Publications from the University of California

Airway hemangiomas in PHACE syndrome

  • Author(s): Durr, ML
  • Meyer, AK
  • Huoh, KC
  • Frieden, IJ
  • Rosbe, KW
  • et al.

Published Web Location

https://dx.doi.org/10.1002/lary.23475
No data is associated with this publication.
Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International Public License
Abstract

Objectives/Hypothesis: To describe the clinical presentation and airway characteristics of infants with airway hemangiomas and concomitant PHACE syndrome and to determine the prevalence of airway hemangiomas in PHACE subjects at our institution. Study Design: Case series. Methods: Retrospective review including clinical presentation, airway findings, treatment measures, and outcomes. Results: A total of 23 subjects were diagnosed with definite PHACE at our institution between September 1, 2005 and September 1, 2011. Twelve (52%) of these subjects had documented airway hemangiomas, six of whom were diagnosed and treated at our institution. All six subjects underwent direct laryngoscopy and bronchoscopy by a pediatric otolaryngologist. Five (83%) subjects had subglottic hemangioma. Three subjects (50%) had additional hemangioma within the airway located on the epiglottis, vocal folds, posterior pharyngeal wall, and tracheal wall. Five subjects (83%) were treated with propranolol, five (83%) were treated with systemic steroids, and one subject received vincristine. One subject required laser ablation of subglottic hemangioma and tracheotomy. All subjects were airway symptom free at last follow-up (average, 35 months; range, 13-76 months). Conclusions: Airway hemangiomas can be a life-threatening complication of PHACE syndrome. At our institution, 52% of all PHACE subjects were diagnosed with airway hemangiomas. Early detection of airway involvement is paramount. Given the high rates of airway hemangiomas, we recommend performing direct laryngoscopy and bronchoscopy in all PHACE patients with respiratory symptoms. We recommend having a low threshold for airway evaluation in asymptomatic PHACE patients, especially those who will not be otherwise started on propranolol. Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.

Many UC-authored scholarly publications are freely available on this site because of the UC Academic Senate's Open Access Policy. Let us know how this access is important for you.

Item not freely available? Link broken?
Report a problem accessing this item