The Chiari II malformation: cause and impact

Childs Nerv Syst. 2003 Aug;19(7-8):540-50. doi: 10.1007/s00381-003-0792-3. Epub 2003 Aug 12.

Abstract

Introduction: It is the Chiari II malformation and its effects that determine the quality of life of the individual born with spina bifida.

Discussion: The cause of this malformation has been a source of debate for many years. Understanding the cause enables strategies for the management of problems created by this malformation to be developed. An open neural tube defect allows fluid to escape from the cranial vesicles, altering the intracranial environment and leads to all of the brain changes seen in the Chiari II malformation. Decompression of the intracranial vesicles causes overcrowding, decrease in the size of the third ventricle, and changes in the fetal skull. It also permanently links the intracranial ventricular system to the spinal cord central canal.

Publication types

  • Comparative Study

MeSH terms

  • Animals
  • Arnold-Chiari Malformation / complications*
  • Arnold-Chiari Malformation / embryology
  • Arnold-Chiari Malformation / ultrastructure
  • Brain / anatomy & histology*
  • Brain / embryology
  • Calcinosis / pathology
  • Central Nervous System / abnormalities*
  • Central Nervous System / ultrastructure
  • Chick Embryo
  • Contrast Media / metabolism
  • Disease Models, Animal
  • Embryonic and Fetal Development*
  • Female
  • Fetus
  • Humans
  • Hydrocephalus / complications
  • Hydrocephalus / pathology
  • Magnetic Resonance Imaging
  • Meningomyelocele / complications
  • Meningomyelocele / pathology
  • Mice
  • Microscopy, Electron, Scanning
  • Neural Tube Defects / complications*
  • Neural Tube Defects / embryology
  • Neural Tube Defects / ultrastructure
  • Pregnancy
  • Spinal Cord / anatomy & histology
  • Spinal Cord / embryology
  • Spinal Dysraphism / complications
  • Spinal Dysraphism / embryology
  • Spinal Dysraphism / ultrastructure

Substances

  • Contrast Media