Chiari Malformation

Chiari malformation occurs when part of the brain—the cerebellar tonsils—extends downward into the spinal canal. It’s often present from birth and may be discovered on imaging performed for another reason. This “crowding” at the base of the skull can affect how spinal fluid flows and may lead to symptoms in some children.

Chiari malformation is diagnosed in about 1–3% of children who receive an MRI of the brain or spine.

Many children with Chiari malformation have no symptoms. When symptoms are present, they can vary depending on the pressure and location of the brain tissue involved. Symptoms may include:

• Headaches at the back of the head, especially after coughing or sneezing
• Dizziness or balance problems
• Numbness, tingling or weakness in the arms or legs
• Trouble walking
• Difficulty swallowing or breathing (snoring, choking)
• Curved spine (scoliosis)
• Sleep apnea or disrupted breathing during sleep

Chiari malformation is typically diagnosed using MRI or CT scans of the brain and spine. Imaging helps your child’s neurosurgeon evaluate how far the cerebellar tonsils extend and whether other findings, like syringomyelia (a fluid-filled cavity in the spinal cord), are present.

Sometimes, imaging reports may be unclear or vary based on how measurements are taken. Our neurosurgery team will carefully review your child’s imaging and symptoms to make a clear diagnosis.

Not every child with Chiari malformation needs treatment. In fact, most children do not require surgery. Our neurosurgery team tailors the treatment plan to your child’s specific condition and needs.

Treatment options may include:

• Monitoring only, especially for children with no symptoms
• Surgery to reduce pressure or improve spinal fluid flow, typically only when symptoms are significant
• Additional testing such as a full spine MRI, scoliosis X-rays or referrals to other specialties (neurology, sleep medicine, aerodigestive, pain management)

We’ll guide you through every step and help you understand what’s best for your child.

Surgery is considered when symptoms are causing problems with daily life or when other issues, like syringomyelia, are present. At Connecticut Children’s, our pediatric neurosurgeons participate in the Park Reeves Syringomyelia Research Consortium, which allows us to offer advanced, research-informed surgical techniques when needed.

Most children with Chiari malformation are followed for two years after diagnosis, or longer if they are under age five. New symptoms are rare—fewer than 3% of children develop new issues after diagnosis.

If your child’s condition is stable and they remain symptom-free, they may not need ongoing follow-up or repeat imaging. Your neurosurgery team will guide you based on your child’s age, symptoms and test results.

Some children can continue all normal activities, while others may need to avoid contact sports or gymnastics. Your care team will review your child’s MRI and symptoms to determine what’s safe.