Arteriovenous shunting as a new feature of PHACES. Case report

J Neurosurg Pediatr. 2009 Jan;3(1):53-6. doi: 10.3171/2008.10.PEDS08169.

Abstract

Patients with the congenital neurocutaneous disorder PHACES are at a markedly increased risk of ischemic infarction during childhood. Although intracranial arterial anomalies have been well described, venous abnormalities have not been documented. The authors report on a unique case of a 3-month-old girl with PHACES and a skull base osteodural arteriovenous fistula. A separate arteriovenous shunt at T-5 may also have been present. Imaging findings and treatment strategies are discussed.

Publication types

  • Case Reports

MeSH terms

  • Central Nervous System Vascular Malformations / diagnosis
  • Central Nervous System Vascular Malformations / therapy*
  • Cerebellum / blood supply
  • Cerebral Angiography
  • Embolization, Therapeutic*
  • Female
  • Follow-Up Studies
  • Humans
  • Hydrocephalus / diagnosis
  • Hydrocephalus / therapy
  • Infant
  • Intracranial Arteriovenous Malformations / diagnosis
  • Intracranial Arteriovenous Malformations / therapy*
  • Neurocutaneous Syndromes / diagnosis
  • Neurocutaneous Syndromes / therapy*
  • Retreatment
  • Skull Base / blood supply*