HistoryHistory

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

Arnold Chiari Arnold Chiari MalformationsMalformations

• Type I: Most common form, generally Type I: Most common form, generally asymptomatic during childhood, but often asymptomatic during childhood, but often manifests with headaches, blurred vision, and manifests with headaches, blurred vision, and cerebellar symptoms. cerebellar symptoms.

• Type II is usually accompanied by a Type II is usually accompanied by a myelomeningocele leading to partial or complete myelomeningocele leading to partial or complete paralysis below the spinal defect. Abnormal paralysis below the spinal defect. Abnormal development of the cerebellar vermis and medulla development of the cerebellar vermis and medulla occur, and they both descend into the foramen occur, and they both descend into the foramen magnum. Hydrocephalus is also nearly always magnum. Hydrocephalus is also nearly always present. present.

• Type III causes severe neurological defects. It is Type III causes severe neurological defects. It is associated with an encephalocele. Rare.associated with an encephalocele. Rare.

• Type IV involves a failure of brain development. Type IV involves a failure of brain development. Rare.Rare.

HistoryHistory

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

Radiologic FindingsRadiologic Findings

• Budd Chiari IIBudd Chiari II

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

HistoryHistory

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

Radiologic FindingsRadiologic Findings

• Budd Chiari IBudd Chiari I

• Cerebellar and Medulla herniation Cerebellar and Medulla herniation through the foramen magnumthrough the foramen magnum

HistoryHistory

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

Radiologic FindingsRadiologic Findings

• Budd Chiari IVBudd Chiari IV

• Cerebellar and Brainstem hypoplasiaCerebellar and Brainstem hypoplasia

Take Home PointsTake Home Points

• Four types of Arnold-Chiari MalformationFour types of Arnold-Chiari Malformation

• Type I most common. Usually presents in Type I most common. Usually presents in adulthood – headaches, blurred vision, adulthood – headaches, blurred vision, neck pain. Treatable with surgery.neck pain. Treatable with surgery.

• Type II-IV manifest in childhood and are Type II-IV manifest in childhood and are associated with neurological deficits.associated with neurological deficits.