Airway hemangiomas in PHACE syndrome§

Authors

  • Megan L. Durr MD,

    Corresponding author
    1. Department of Otolaryngology-Head and Neck Surgery, University of California–San Francisco, San Francisco, California, U.S.A.
    • MD, Department of Otolaryngology–Head and Neck Surgery, University of California–San Francisco, 2380 Sutter Street, Box 0342, San Francisco, CA 94115
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  • Anna K. Meyer MD,

    1. Department of Otolaryngology-Head and Neck Surgery, University of California–San Francisco, San Francisco, California, U.S.A.
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  • Kevin C. Huoh MD,

    1. Department of Otolaryngology-Head and Neck Surgery, University of California–San Francisco, San Francisco, California, U.S.A.
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  • Ilona J. Frieden MD,

    1. Department of Dermatology and Department of Pediatrics, University of California–San Francisco, San Francisco, California, U.S.A.
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  • Kristina W. Rosbe MD

    1. Department of Otolaryngology-Head and Neck Surgery, University of California–San Francisco, San Francisco, California, U.S.A.
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  • Presented as a poster at the Triological Society Combined Sections Meeting, Miami, Florida, U.S.A., January 26–28, 2012.

  • Ilona J. Frieden, MD, has consulted for Pierre-Fabre Dermatology and is chair of the Data Safety Monitoring Board for the Hemangiol Clinical Trial.

  • §

    The authors have no other funding, financial relationships, or conflicts of interest to disclose.

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. Laryngoscope, 2012

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