Segmental neurovascular syndromes in children

Neuroimaging Clin N Am. 2007 May;17(2):245-58. doi: 10.1016/j.nic.2007.02.006.


The concept of segmental vascular syndromes with different, seemingly unrelated, diseases is based on the embryology of the neural crest and the mesoderm migration of cells that share the same metameric origin. Migrating patterns of these cells link the brain, the cranial bones, and the face on the same side. A somatic mutation developing in the region of the neural crest or the adjacent cephalic mesoderm before migration can, therefore, be postulated to produce arterial or venous metameric syndromes, including PHACES, CAMS, Cobb syndrome, and Sturge-Weber syndrome. Although these diseases may be rare, their relationships among each other and their postulated linkage with the development of the neural crest and the cephalic mesoderm may shed light on the complex pathology and etiology of various cerebral vascular disorders.

Publication types

  • Review

MeSH terms

  • Arteriovenous Malformations*
  • Brain / abnormalities
  • Brain / blood supply*
  • Child
  • Child, Preschool
  • Eye Abnormalities*
  • Face / abnormalities
  • Face / blood supply*
  • Female
  • Heart Defects, Congenital*
  • Humans
  • Intracranial Arteriovenous Malformations
  • Male
  • Neural Crest / abnormalities
  • Neural Crest / blood supply
  • Spinal Cord / abnormalities
  • Spinal Cord / blood supply*
  • Syndrome