Chiari Malformation

Chiari malformation, or Arnold-Chiari syndrome, is a series of nervous system symptoms.

This condition is often present at birth, but can also develop later in life. Some people with very mild forms of this syndrome may never know they have the condition. For others it can be more severe and require treatment.

What is Chiari Malformation?

Chiari malformation is caused by a problem in the back of the skull. The skull should have an indented space in the back of the head. The rear lower part of the brain and the brainstem are in this space. In some people, this indented skull space does not develop well. This is called Chiari malformation. The space inside the skull is too small for the brain. As a result, the brain and brain stem are pushed downward. It blocks the flow of fluid from the brain to the spinal column.

The problem with the skull develops before birth. It is not clear why it happens. In some cases, it is accompanied by a myelomeningocele, which is a form of spina bifida.

Symptoms in adolescents are usually milder and may include:

  • Lightheadedness
  • Fainting
  • Weakness in the legs
  • Headaches
  • Deafness or ringing in the ears
  • Eye problems such as difficulty seeing, eye pain and rapid eye movement
  • Poor coordination
  • Uncontrolled shaking or trembling
  • Difficulty walking
  • Numbness or tingling in the arms or legs

Along with symptoms, your doctor may need detailed images of the brain and skull using one of these:

  • MRI
  • CT scan

Your doctor may do special studies to evaluate the flow of fluid around your brain and spinal cord.

Treating Chiari Malformation

Depending on your symptoms, you may need:

  • Braces, a wheelchair, physical or occupational therapy to improve muscular coordination and trembling
  • Speech therapy for speech or swallowing problems
  • Medication to help manage headaches and pain

The malformation may also block the flow of fluid in the brain and spine. Surgery to install a shunt or stent can correct the flow of fluid and to release compression of the brain.

Talk with your doctor about the best treatment plan for you.

Genetic Counseling

While we know of no way to prevent Chiari malformation, genetic counseling may help parents of a child with this condition to determine the risk in future children.


Content was created using EBSCO’s Health Library. Edits to original content made by Rector and Visitors of the University of Virginia. This information is not a substitute for professional medical advice.