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History

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

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History

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

  2. 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.

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

  5. History • 34 yr old female with unbearable neck pain.

  6. Radiologic Findings • Budd Chiari I • Cerebellar and Medulla herniation through the foramen magnum

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

  8. Radiologic Findings • Budd Chiari IV • Cerebellar and Brainstem hypoplasia

  9. 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.

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