Chiari malformation

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Brain MRI showing herniation of cerebellar tonsils into the foramen magnum in a patient with Arnold-Chiari malformation of the first type

Chiari malformation (Arnold-Chiari malformation) is a congenital CNS anomaly. It is a dystopia of the cerebellum and medulla oblongata into the spinal canal, which is clinically manifested by hydrocephalus. RWe distinguish four types of rhombencephalon abnormalities (cerebellum, pons, oblongata):

  • Type 1 - herniation of the cerebellar tonsil under the foramen magnum, IV. the chamber is stored normally
  • Type 2 - usually the co-presence of myelomeningocele
  • Type 3 - severe dislocation of structures in the posterior pit, often associated with suboccipital encephalomeningocele; usually incompatible with life
  • Type 4 - cerebellar hypoplasia without herniation.
Chiari malformation of the second type

Clinical picture[edit | edit source]

Clinically, the defect is manifested mainly by headache, weakened grip and spasticity of DK.

Diagnosis[edit | edit source]

Native X-ray, MRI.

Therapy[edit | edit source]

The main problem is hydrocephalus – decompression of the craniospinal junction, short-circuit drainage operations.


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