Arnold-Chiari malformation

Arnold-Chiari malformation. This is a condition where portions of the base of the brain project into the upper part of the spinal canal. Structure within the brain and upper part of the spinal cord can become compressed leading to pain in the neck, hoarseness, frequent upper respiratory tract infections, and, in advanced cases, progressive weakness in the arms and legs. Also, the normal outlets for the brain's fluid spaces (the ventricles) can become obstructed leading to hydrocephalus and cause increase pressure on the brain.

In some cases, symptoms begin during infancy, however onset of symptoms may be delayed until adolescence or adulthood. Symptoms usually include vomiting, muscle weakness in the head and face, difficulty swallowing, and varying degrees of mental impairment. Paralysis of the arms and legs may also occur.

Adults and adolescents with Chiari malformation who previously were asymptomatic may show signs of progressive brain impairment as they grow older, such as involuntary, rapid, downward eye movements. Other symptoms include dizziness, headache, double vision, deafness, an impaired ability to coordinate movement, and episodes of acute pain in and around the eyes.

ADULT TREATMENT

Hydrocephalus (if present) may be treated with surgical implantation of a shunt to relieve increased pressure on the brain. Patients with Arnold Chiari malformation may benefit from surgery to enlarge the opening in the back of the skull to relieve intracranial pressure. This procedure is done also to release pressure on the base of the brain and upper spine.  Dr. Maroon and his associates have often preformed this operation and are consider experts in this area.

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© 2008 Tri-State Neurosurgical Associates - UPMC

Last Updated: January 1, 2008