Chiari malformation type 2

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Disease Overview

Chiari malformation type 2 (CM type II) is a type of Chiari malformation in which both the cerebellum and brain stem tissue extend into the foramen magnum (the hole at the skull base for passing of the spinal cord).[4050] CM type II is usually accompanied by a myelomeningocele (a form of spina bifida that occurs when the spinal canal and backbone do not close before birth), which can result in partial or complete paralysis of the area below the spinal opening.[4050] While the severity of CM type II can vary greatly, it can potentially cause severe, life-threatening complications during infancy or childhood.[4051] 

The exact cause of CM type II is not known but it appears to be due to defects in the brain and spinal cord that occur during fetal development.[4051] Treatment includes surgery to ease symptoms and/or stop the progression of damage to the nervous system. For most people, surgery improves or stabilizes symptoms. Some people may require more than one surgery.[4050]


Synonyms

  • Arnold-Chiari malformation
  • Chiari type II malformation
  • Chiari malformation type II
  • Arnold Chiari malformation type II

For more information, visit GARD.

National Organization for Rare Disorders