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Childs Nerv Syst. 2003 Aug;19(7-8):540-50. Epub 2003 Aug 12.

The Chiari II malformation: cause and impact.

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  • 1Division of Pediatric Neurosurgery, The Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614, USA. dmclone@nwu.edu



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


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.

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