The brain and spinal cord are surrounded by a clear fluid called cerebrospinal fluid (CSF). This fluid is produced and stored in cavities in the brain called ventricles. It circulates around the brain, moving from ventricle to ventricle. The purposes of the fluid are to cushion and protect the brain and spinal cord, to supply them with nutrients, and to remove some of their waste products. Any excess fluid drains away from the brain and is absorbed by veins at the top of the brain.
Chiari malformations affect females more often than males.
Scientists once believed that Chiari malformations occurred in only 1 in every 1,000 births. But the increased use of diagnostic imaging techniques such as CT scans and MRIs suggests that the condition may be much more common.
Accurate estimates are difficult to make. That's because some children who are born with the condition either never develop symptoms or don't develop symptoms until they reach adolescence or adulthood.
Causes of Chiari Malformations
Chiari malformations are usually caused by structural defects in the brain and spinal cord. These defects develop during fetal development.
Due to genetic mutations or a maternal diet that lacked certain nutrients, the indented bony space at the base of the skull is abnormally small. As a result, pressure is placed on the cerebellum. This blocks the flow of the cerebrospinal fluid. That's the fluid that surrounds and protects the brain and spinal cord.
Most Chiari malformations occur during fetal development. Much less commonly, Chiari malformations can occur later in life. This can happen when an excessive amount of cerebrospinal fluid is drained away because of:
exposure to toxic substances
Types of Chiari Malformations
There are four types of Chiari malformations:
Type I. This is by far the most commonly observed type in children. In this type, the lower part of the cerebellum -- but not the brain stem -- extends into an opening at the base of the skull. The opening is called the foramen magnum. Normally, only the spinal cord passes through this opening.