PHACE syndrome

PHACE syndrome, also known as cutaneous hemangioma–vascular complex syndrome or Pascual-Castroviejo type II syndrome, is a phakomatosis that comprises of:

When sternal clefting and/or supraumbilical raphe are also present it is termed PHACES syndrome.

Clinical presentation

Clinical diagnosis of PHACE syndrome requires the presence of a characteristic segmental hemangioma or hemangioma >5 cm on the head (face or scalp) plus 1 major criterion or 2 minor criteria 5

Major criteria:

Minor criteria:

Historic and etymology

Initially, this syndrome was described as an association of large cutaneous hemangiomas of the head and anomalies of the cerebral vasculature by Pascual-Castroviejo in 1978 7,8. Subsequently, the name PHACE syndrome was coined by Ilona Frieden et al 2.

Article information

rID: 1810
Section: Syndromes
Synonyms or Alternate Spellings:
  • PHACES syndrome
  • PHACE association
  • Cutaneous haemangioma–vascular complex syndrome
  • Pascual-Castroviejo type II syndrome

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Cases and figures

  • Case 1: posterior fossa anomaly
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  • Case 1: absent right ICA
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  • Case 2: left periorbital hemangioma, posterior fossa anomaly, hypoplasia of left ICA
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