Chiari II Malformation

Aka: Chiari II Malformation, Arnold-Chiari Malformation, Arnold Chiari II Malformation

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II. Definition: Chiari II Malformation

  1. Cerebellum and Brain Stem elongate via foramen magnum
  2. Cerebellar Tonsils dip into foramen magnum
    1. Risk of cord injury with even minor Trauma

III. Presentation

  1. Presentation as infant (10% of cases)
    1. Apnea
    2. Weak cry
    3. Stridor
      1. Emergency - may herald Respiratory Failure
  2. Insidious course during childhood
    1. Gait Abnormality
    2. Spasticity
    3. Incoordination

IV. Associated Conditions

  1. Myelomeningocele
  2. Hydrocephalus
  3. Corpus callosum abnormalities

V. Management

  1. Management of Myelomeningocele and Hydrocephalus
  2. Decompression of at foramen magnum may be indicated

VI. Precautions

  1. Acute conditions indicating emergent MRI
    1. Head or neck Trauma (even minor Trauma)
    2. Stridor (may herald Respiratory Failure)

VII. References

  1. Majoewsky (2012) EM:Rap-C3 2(9): 5
  2. Behrman (2000) Nelson Pediatrics, Saunders, p. 1810
  3. Goldman (2000) Cecil Medicine, Saunders, p. 2076

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