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Clinical spectrum and risk of PHACE syndrome in cutaneous and airway hemangiomas. Arch Otolaryngol Head Neck Surg 2011 Jul;137(7):680-7 PMID: 21768412

Pubmed ID





OBJECTIVE: To describe the clinical presentation and risk of PHACE syndrome in infants with large facial hemangiomas and concomitant airway hemangiomas.

DESIGN: 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.

SETTING: Six academic pediatric dermatology clinics.

PATIENTS: 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.

Author List

Haggstrom AN, Skillman S, Garzon MC, Drolet BA, Holland K, Matt B, McCuaig C, Metry DW, Morel K, Powell J, Frieden IJ


2-s2.0-79960490804   16 Citations

MESH terms used to index this publication - Major topics in bold

Abnormalities, Multiple
Diagnosis, Differential
Eye Abnormalities
Facial Neoplasms
Follow-Up Studies
Infant, Newborn
Magnetic Resonance Angiography
Neurocutaneous Syndromes
Prospective Studies
Respiratory Tract Neoplasms
Skin Neoplasms
jenkins-FCD Prod-321 98992d628744e349846c2f62ac68f241d7e1ea70