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Chiari malformation (Arnold-Chiari malformation)

Classification: Chiari type I - cerebellar herniation restricted to tonsils which may be atrophic or sclerosed; associated with syringomyelia, small posterior fossa, occipital dysplasia; often asymptomatic, may cause late onset hydrocephalus. Chiari type II - elongation of inferior vermis & brainstem with displacement into the cervical spinal canal; most commonly found in infants & associated with myelomeningocele & hydrocephalus Chiari type III - occipitocervical or high cervical bony defect with herniation of cerebellum, may also include beaking of tectum, brainstem elongation & lumbar spina bifida; occurrence is very rare.

Specific

Chiari malformation type I Chiari malformation type II

General

cephalic malformation spinal cord malformation; myelatelia

References

  1. Greenfield's Neuropathology, 5th ed., 1992 p.543-548
  2. National Institute of Neurological Disorders and Stroke (NINDS) - NINDS Chiari Malformation Fact Sheet https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Chiari-Malformation-Fact-Sheet - NINDS Chiari Malformation Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Chiari-Malformation-Information-Page