title: Airway hemangiomas in PHACE syndrome.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of California-San Francisco, San Francisco, California 94115, USA. mdurr@ohns.ucsf.edu
authors.forename: Megan L
authors.initials: ML
authors.lastname: Durr
authors.forename: Anna K
authors.initials: AK
authors.lastname: Meyer
authors.forename: Kevin C
authors.initials: KC
authors.lastname: Huoh
authors.forename: Ilona J
authors.initials: IJ
authors.lastname: Frieden
authors.forename: Kristina W
authors.initials: KW
authors.lastname: Rosbe
mesh_terms: D001017:Aortic Coarctation / Q000175:diagnosis* / Q000628:therapy*; D001999:Bronchoscopy; D018572:Disease-Free Survival; D005124:Eye Abnormalities / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006391:Hemangioma / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007822:Laryngeal Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D007828:Laryngoscopy; D053685:Laser Therapy; D008297:Male; D020752:Neurocutaneous Syndromes / Q000175:diagnosis* / Q000628:therapy*; D011433:Propranolol / Q000627:therapeutic use; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000175:diagnosis / Q000628:therapy; D013256:Steroids / Q000627:therapeutic use; D014140:Tracheotomy; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D013256:Steroids; D014750:Vincristine; D011433:Propranolol