History

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History

• 14 yr old female with history of hydrocephalus
  with shunt placement, lower limb paralysis, and
  Arnold-Chiari malformation.

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Arnold Chiari Malformations

• Type I Most common form, generally asymptomatic
  during childhood, but often manifests with
  headaches, blurred vision, and cerebellar
  symptoms.
• Type II is usually accompanied by a
  myelomeningocele leading to partial or complete
  paralysis below the spinal defect. Abnormal
  development of the cerebellar vermis and medulla
  occur, and they both descend into the foramen
  magnum. Hydrocephalus is also nearly always
  present.
• Type III causes severe neurological defects. It
  is associated with an encephalocele. Rare.
• Type IV involves a failure of brain development.
  Rare.

3
History

• 14 yr old female with history of hydrocephalus
  with shunt placement, lower limb paralysis, and
  Arnold-Chiari malformation.

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Radiologic Findings

• Budd Chiari II
• A small posterior fossa, mild tectal beaking and
  cerebellar herniation through an enlarged foramen
  magnum. Hydrocephalus.

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History

• 34 yr old female with unbearable neck pain.

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Radiologic Findings

• Budd Chiari I
• Cerebellar and Medulla herniation through the
  foramen magnum

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History

• 5 month old male with hypotonia, failure to
  thrive, history of ventriculomegaly on fetal
  ultrasound.

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Radiologic Findings

• Budd Chiari IV
• Cerebellar and Brainstem hypoplasia

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Take Home Points

• Four types of Arnold-Chiari Malformation
• Type I most common. Usually presents in
  adulthood headaches, blurred vision, neck pain.
  Treatable with surgery.
• Type II-IV manifest in childhood and are
  associated with neurological deficits.