The cause of Chiari II malformation: a unified theory

Pediatr Neurosci. 1989;15(1):1-12. doi: 10.1159/000120432.


The cause of the Chiari II hindbrain deformity in children born with a myelomeningocele can be explained by the lack of distention of the embryonic ventricular system. Defective occlusion and an open neural tube precludes the accumulation of fluid and pressure within the cranial vesicles. This distention is critical to normal brain development. The small posterior fossa, cerebral disorganization, and lückenschädel are the result.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Arnold-Chiari Malformation / embryology*
  • Arnold-Chiari Malformation / pathology
  • Gestational Age
  • Mice
  • Mice, Mutant Strains
  • Neural Tube Defects / embryology*
  • Neural Tube Defects / pathology