title: Clinical spectrum and risk of PHACE syndrome in cutaneous and airway hemangiomas.
abstract: OBJECTIVE
  To describe the clinical presentation and risk of PHACE syndrome in infants with large facial hemangiomas and concomitant airway hemangiomas.
  
  
  METHODS
  The study involved a case series of infants with cutaneous hemangiomas and airway hemangiomas extracted from a prospective multicenter cohort study. Data regarding clinical features, diagnosis, treatment, and clinical course were obtained from medical charts and physician intake forms. All patients were evaluated for PHACE syndrome using a standardized protocol.
  
  
  METHODS
  Six academic pediatric dermatology clinics.
  
  
  METHODS
  The study included 17 patients younger than 1 year who were diagnosed as having large (>22 cm(2)) facial hemangiomas and airway hemangiomas.
  
  
  RESULTS
  Thirteen patients (76%) had hemangiomas in the bilateral mandibular distribution. Other observed facial patterns included limited involvement of the lip and chin, unilateral reticular frontotemporal and preauricular hemangiomas, and large unilateral hemifacial hemangiomas. Fourteen patients (82%) had symptomatic airway involvement. All symptomatic patients had subglottic airway hemangiomas. The airway hemangioma was circumferential in 10 patients (58%) and more focal in distribution in 7 patients (42%). All patients were treated with oral prednisolone. Eleven patients required additional multimodal therapy. Eight patients (47%) met the criteria for PHACE syndrome.
  
  
  CONCLUSIONS
  Airway hemangiomas represent a potentially fatal complication of infantile hemangiomas. Our data highlight cutaneous presentations in patients with subglottic hemangiomas and large (>22 cm(2)) cutaneous hemangiomas. PHACE syndrome was detected in 8 such patients (47%) in our series.
authors.affiliation: Department of Dermatology, Indiana University, 550 N University Blvd, UH 3240, Indianapolis, IN 46202, USA. ahaggstr@iupui.edu
authors.forename: Anita N
authors.initials: AN
authors.lastname: Haggstrom
authors.forename: Sarah
authors.initials: S
authors.lastname: Skillman
authors.forename: Maria C
authors.initials: MC
authors.lastname: Garzon
authors.forename: Beth A
authors.initials: BA
authors.lastname: Drolet
authors.forename: Kristen
authors.initials: K
authors.lastname: Holland
authors.forename: Bruce
authors.initials: B
authors.lastname: Matt
authors.forename: Catherine
authors.initials: C
authors.lastname: McCuaig
authors.forename: Denise W
authors.initials: DW
authors.lastname: Metry
authors.forename: Kimberly
authors.initials: K
authors.lastname: Morel
authors.forename: Julie
authors.initials: J
authors.lastname: Powell
authors.forename: Ilona J
authors.initials: IJ
authors.lastname: Frieden
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001999:Bronchoscopy; D003937:Diagnosis, Differential; D005124:Eye Abnormalities / Q000175:diagnosis*; D005153:Facial Neoplasms / Q000175:diagnosis*; D005260:Female; D005500:Follow-Up Studies; D006391:Hemangioma / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D018810:Magnetic Resonance Angiography; D008297:Male; D020752:Neurocutaneous Syndromes / Q000175:diagnosis*; D011379:Prognosis; D011446:Prospective Studies; D012142:Respiratory Tract Neoplasms / Q000175:diagnosis*; D012878:Skin Neoplasms / Q000175:diagnosis*; D013577:Syndrome