Myelomeningocele, spinal arteriovenous malformations and epidermal nevi syndrome: a possible rare association?

Dev Med Child Neurol. 1993 Aug;35(8):737-41. doi: 10.1111/j.1469-8749.1993.tb11720.x.


A 20-year-old woman with myelomeningocele presented with acute right-ear pain and right hemiplegia which improved, but then progressively deteriorated. Surgery, after MRI, revealed a large arteriovenous malformation (AVM) involving the right side of the upper cervical cord and brainstem. The woman also had two epidermal nevi at the same site as the spinal cord AVMs. There has been no previous report of an association of myelomeningocele, spinal cord AVMs and epidermal nevi syndrome. The same location of the nevus and spinal cord AVMs, with a proposed common pathogenesis, raise the possibility that the association may be more than chance occurrence. Spinal cord AVMs should be considered in patients with myelomeningocele and similar clinical features.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Adult
  • Arnold-Chiari Malformation / diagnosis
  • Arteriovenous Malformations / diagnosis*
  • Brain Diseases / complications
  • Brain Diseases / physiopathology
  • Brain Stem / blood supply
  • Cerebral Angiography
  • Diagnosis, Differential
  • Female
  • Functional Laterality
  • Humans
  • Magnetic Resonance Imaging
  • Meningomyelocele / diagnosis*
  • Nevus, Pigmented / diagnosis*
  • Spinal Cord / blood supply*