title: PHACE syndrome--clinical features, aetiology and management.
abstract: PHACE syndrome comprises a spectrum of anomalies including posterior fossa malformations, haemangioma, arterial anomalies, cardiac defects and eye anomalies. PHACE should be considered in any patient with a large facial segmental infantile haemangioma (IH), and multidisciplinary management is crucial. Low-dose propranolol is effectively for the treatment of IH associated with PHACE syndrome. Recent evidence suggests IH is comprised of mesoderm-derived haemogenic endothelium.
  
  
  CONCLUSIONS
  The embryonic developmental anomaly nature of IH provides an insight into the origin of PHACE syndrome.
authors.affiliation: Gillies McIndoe Research Institute, Wellington, New Zealand.
authors.forename: Pieta R
authors.initials: PR
authors.lastname: Winter
authors.affiliation: Gillies McIndoe Research Institute, Wellington, New Zealand.
authors.forename: Tinte
authors.initials: T
authors.lastname: Itinteang
authors.affiliation: Gillies McIndoe Research Institute, Wellington, New Zealand.|Centre for the Study & Treatment of Vascular Birthmarks, Wellington Regional Plastic, Maxillofacial & Burns Unit, Wellington, New Zealand.|Department of Paediatrics, Hutt Hospital, Wellington, New Zealand.
authors.forename: Phillip
authors.initials: P
authors.lastname: Leadbitter
authors.affiliation: Gillies McIndoe Research Institute, Wellington, New Zealand.|Centre for the Study & Treatment of Vascular Birthmarks, Wellington Regional Plastic, Maxillofacial & Burns Unit, Wellington, New Zealand.
authors.forename: Swee T
authors.initials: ST
authors.lastname: Tan
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D001017:Aortic Coarctation / Q000188:drug therapy / Q000196:embryology; D005124:Eye Abnormalities / Q000188:drug therapy / Q000196:embryology; D005260:Female; D006801:Humans; D007223:Infant; D020752:Neurocutaneous Syndromes / Q000188:drug therapy / Q000196:embryology
chemical_list: D000319:Adrenergic beta-Antagonists
keywords: Aetiology; Infantile haemangioma; PHACE syndrome; Renin-angiotensin system; β-blockers