Carotid rete mirabile and pseudoxanthoma elasticum: an accidental association?

Eur J Vasc Endovasc Surg. 2011 Sep;42(3):292-4. doi: 10.1016/j.ejvs.2011.05.007. Epub 2011 Jul 1.


We report the case of a young female patient with a transient amaurosis due to a carotid rete mirabile (CRM), a rare congenital carotid malformation, and pseudoxanthoma elasticum (PXE), an inherited autosomal recessive systemic metabolic disorder characterised by fragmentation and mineralisation of elastic fibres in connective tissues (skin, eyes) and the vascular system. CRM is a rare form of intracranial carotid malformation whose association with PXE (6 cases at present) would appear not to be accidental. This observation suggests a new link between congenital arterial remodelling and the PXE.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Blindness / etiology*
  • Carotid Arteries / abnormalities*
  • Carotid Artery Diseases / complications
  • Carotid Artery Diseases / congenital
  • Carotid Artery Diseases / diagnosis*
  • Female
  • Humans
  • Pseudoxanthoma Elasticum / complications*