Distal aortic arch aneurysm in an adult case of PHACE syndrome

Interact Cardiovasc Thorac Surg. 2018 Oct 1;27(4):619-621. doi: 10.1093/icvts/ivy123.

Abstract

A rare case of adult PHACE syndrome with a distal aortic arch aneurysm was treated surgically. The patient had several anomalies including uneven cerebral blood flow, a small left common carotid artery and a narrowed transverse aorta. Under deep hypothermic circulatory arrest, aortic replacement with reconstruction of the left subclavian artery was performed uneventfully. Cystic degeneration of the tunica media was observed in the aneurysm wall.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aorta, Thoracic / diagnostic imaging
  • Aorta, Thoracic / surgery*
  • Aortic Aneurysm, Thoracic / diagnosis*
  • Aortic Aneurysm, Thoracic / etiology
  • Aortic Aneurysm, Thoracic / surgery
  • Aortic Coarctation / complications
  • Aortic Coarctation / diagnosis*
  • Computed Tomography Angiography
  • Eye Abnormalities / complications
  • Eye Abnormalities / diagnosis*
  • Female
  • Humans
  • Neurocutaneous Syndromes / complications
  • Neurocutaneous Syndromes / diagnosis*
  • Rare Diseases
  • Syndrome
  • Vascular Surgical Procedures / methods*

Supplementary concepts

  • PHACE association