How is PHACE syndrome in infantile hemangioma characterized?

Updated: Feb 06, 2019
  • Author: Richard J Antaya, MD; Chief Editor: William D James, MD  more...
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Answer

Patients with PHACE syndrome present with hemangiomas and one or more extracutaneous congenital anomalies. Reports have also described intracranial invasion of associated infantile hemangiomas. [56] PHACE syndrome is an acronym denoting posterior fossa abnormalities (characteristically Dandy-Walker malformation and other forms of brain hypoplasia), hemangiomas (cervicofacial, segmental/>5 cm in diameter), arterial anomalies (especially carotid, cerebral, and vertebral), cardiac anomalies (especially coarctation of the aorta), eye abnormalities, and, rarely, midline ventral abnormalities (sternal clefting or supraumbilical raphe). See Mortality/Morbidity.


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