Chiari Malformation Specialist

What is Chiari malformation?

Chiari malformation is most often caused by a structural defect at the base of the skull that occurs during fetal development and allows part of the brain (cerebellum) to extend into the spinal canal.

Physicians separate Chiari malformation into different types (I, II, III, and IV) that are identified according to the amount of brain tissue displaced and whether the abnormality is causing developmental problems in the brain or spinal cord.

Type II Chiari malformation, for instance, causes a form of spina bifida, which develops when the spinal canal and backbone do not close completely before birth. Severe complications related to Type II can occur in early infancy and childhood.

The structural abnormality that causes Type I Chiari malformation, the most common type, is present at birth but generally not symptomatic initially because the condition develops as the brain and skull grow.  When symptoms do occur, they’re generally much less severe than those in other types.

Rarely, Chiari malformation Type I may occur later in life as the result of traumatic injury or infection. This is known as secondary or acquired Chiari malformation.

What are the symptoms of Chiari malformation?

Related to pressure on the brainstem and the cerebellum, symptoms of Chiari malformation may include:

• Severe headaches that tend to occur after sneezing, coughing, or exertion
• Varying degrees of neck pain
• Problems with balance or an unsteady gait
• Problems with fine motor skills needed for tasks such as writing or buttoning a shirt
• Numbness and tingling in the hands or feet
• Muscle weakness
• Dizziness
• Difficulty swallowing
• Scoliosis (abnormal curvature of the spine)
• Fluid-filled cyst in the spinal cord (syrinx) that may eventually cause difficulty walking
• Slowed heart rhythm
• Sleep apnea

Notably, Type I Chiari malformation may never cause symptoms and is often discovered during tests performed for another problem.

How do you treat Chiari malformation?

Asymptomatic Chiari malformation generally requires monitoring with periodic imaging studies such as MRI or CT scans to ensure the condition remains stable.

Mild symptoms may respond well to medication to relieve pain and/or balance training and other conservative treatment to help you overcome problems related to Chiari malformation. 

When symptoms are severe or progressing, Dr. Raja may recommend surgery to prevent ongoing damage to the central nervous system.

Surgery for Chiari malformation typically includes posterior fossa decompression. This procedure creates more space for the cerebellum and relieves pressure on the spinal cord. Dr. Raja may also consider laminectomy; he eases pressure in the area by removing a portion of the spinal canal’s bony roof.

For more information about Chiari malformation and treatments available for you or your child, schedule a visit with Dr. Raja today by calling the office or requesting an appointment online