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Acta Paediatr ; 105(2): 145-53, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26469095

ABSTRACT

UNLABELLED: 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. CONCLUSION: The embryonic developmental anomaly nature of IH provides an insight into the origin of PHACE syndrome.


Subject(s)
Aortic Coarctation , Eye Abnormalities , Neurocutaneous Syndromes , Adrenergic beta-Antagonists/therapeutic use , Aortic Coarctation/drug therapy , Aortic Coarctation/embryology , Eye Abnormalities/drug therapy , Eye Abnormalities/embryology , Female , Humans , Infant , Neurocutaneous Syndromes/drug therapy , Neurocutaneous Syndromes/embryology
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