Postmortem vascular pathology in PHACES syndrome: a case report

Pediatr Dev Pathol. 2012 Nov-Dec;15(6):507-10. doi: 10.2350/12-05-1203-CR.1. Epub 2012 Aug 17.

Abstract

This case describes the clinical course and autopsy findings of a 5-year-old girl with PHACES syndrome. While the etiopathogenesis of this condition is not yet understood, the pathological changes documented support the concept that the primary defect in PHACES syndrome is an arteriopathy.

Publication types

  • Case Reports

MeSH terms

  • Aorta, Thoracic / pathology*
  • Aortic Coarctation / pathology*
  • Aortic Rupture / pathology*
  • Autopsy
  • Child, Preschool
  • Eye Abnormalities / pathology*
  • Fatal Outcome
  • Female
  • Humans
  • Infant, Newborn
  • Neurocutaneous Syndromes / pathology*

Supplementary concepts

  • PHACE association