Orphanet: Arnold Chiari malformation type II

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Arnold-Chiari malformation type II

Disease definition

A rare, central nervous system malformation characterized by caudal displacement of the cerebellum, pons, medulla and fourth ventricle through the foramen magnum into the spinal canal, and is typically associated with myelomeningocele. Variable other central nervous system abnormalities might be present (partial or complete agenesis of the corpus callosum, a small fourth ventricle, obstructive hydrocephalus, falx and tentorium defects, and polygyria). Symptoms include hypotonia, apnea with cyanosis, dysphagia, opisthotonus, nystagmus, spasticity, ataxia, and occipital headache.


Classification level: Disorder
  • Synonym(s):
    • Arnold-Chiari malformation type 2
    • Chiari malformation type 2
    • Chiari malformation type II
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Childhood
  • ICD-10: Q07.0
  • OMIM: 207950
  • UMLS: C0003803  C0555206  C0750930
  • MeSH: -
  • GARD: 9232
  • MedDRA: 10056945

Detailed information


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