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Anrold-chiari malformation
Posted Tue, 30 May 2000
Question
Please may I have some information about the Arnold-Chiari malformation?
Answer
The Arnold-Chiari malformation is a malformation of a particular part of the brain which sometimes occurs in association with hydrocephalus, a so-called type 11 malformation. Hydrocephalus is an abnormal amount of fluid in the ventricles, or spaces, within the brain. Symptoms of type 11 occur in infancy in about 10% of those affected. These are noisy breathing, a weak cry and intermittent stopping of breathing. A more slowly progressing form results in abnormalities in gait, spasticity and increasing lack of coordination during childhood.
A type 1 Arnold-Chiari malformation does not produce symptoms until adolescence or adulthood, and is usually not associated with hydrocephalus. Patients complain of recurrent headaches, neck pain, frequent urination and progressive spasticity of the legs.
These malformations are present at birth, being due to abnormal development of the brain, skull and spine in the womb.
Diagnosis is by CT or MRI scan.
It often occurs with spina bifida.
The accumulation of fluid resulting from hydrocephalus, with or without the Arnold-Chiari malformation, requires what is called a shunt, to remove the accumulated fluid in the brain.
The Arnold-chiari malformation also commonly occurs in association with syringomyelia.
The defect is generally treated by surgically freeing the trapped part of the brain within the upper part of the spinal cord.
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