Chiari Malformation

Chiari malformation is a disorder in which part of the brain extends into an opening in the base of the skull. Normally, the brain's cerebellum and brain stem sit in a cavity at the lower back of the skull, and only the spinal cord goes through the opening.

If the cavity is too small, usually because of a birth defect, part of the brain can be pushed down through the opening. Chiari malformation can also develop in adulthood if an injury or infection depletes too much of the fluid that cushions the brain and spinal cord.

Symptoms and treatment vary depending on severity. Sometimes symptoms don't emerge until late childhood or adulthood.

Symptoms

Common symptoms:

  • Headaches, sometimes severe
  • Neck and shoulder pain
  • Trouble walking steadily 
  • Balance problems
  • Poor hand coordination
  • Numbness and tingling in hands and feet
  • Dizziness
  • Swallowing problems, gagging, choking and vomiting
  • Blurry vision or double vision
  • Trouble speaking clearly  

Less common symptoms:

  • Ringing or buzzing in ears
  • Poor bladder control
  • Chest pain
  • Curvature of the spine, or scoliosis
  • Stops in breathing during sleep, or sleep apnea

Nonsurgical treatments

  • Monitoring: If you have no symptoms, your doctor will monitor your condition with regular examinations, and MRI and CT scans.
  • Medication: If you have headaches or other pain, your doctor may recommend anti-inflammatory medications or painkillers such as indomethacin (Indocin).

Surgical treatment

  • Posterior fossa craniectomy: If you have severe symptoms or your pain doesn’t get better with treatment, you may need surgery to relieve the pressure on your brain and spinal cord. In posterior fossa craniectomy, the most common surgery for chiari malformation, a surgeon removes a small section of bone at the back of the skull to give the brain more room.