A Chiari (kee-AH-ree) malformation occurs when the back of the brain develops abnormally and pushes into the spinal canal.
The downward movement of the brain tissue puts pressure on the cerebellum. This pressure limits the flow of the protective liquid (cerebrospinal fluid) that surrounds the brain and spinal cord.
The pressure on the cerebellum can prevent parts of the brain and spine from functioning properly. It sometimes causes problems with balance and coordination.
Most children who have Chiari malformation are born with the condition. It often occurs along with conditions such as hydrocephalus, craniosynostosis, Ehlers-Danlos syndrome and Klippel-Feil syndrome.
The most common type of Chiari malformation is caused by genetic changes (mutations) or a lack of certain vitamins and nutrients in a mother’s diet. Secondary Chiari malformation is typically caused by an injury, exposure to toxic substances, or an infection.
Chiari malformation types get categorized based on which parts of the brain push down into the spinal canal.
Some children who have Chiari malformation don’t experience any symptoms. Or, they might not experience symptoms until later in adolescence or even adulthood.
Symptoms of Type I Chiari malformation can include:
Some younger children and infants might have difficulty drinking or swallowing.
Symptoms of Type II Chiari malformation are similar to those found in Type I. However, Type II symptoms are more severe and usually happen at a younger age.
Symptoms of Type II Chiari malformation include:
Some Chiari malformations don’t cause symptoms, and therefore don’t require treatment. However, others cause a wide range of symptoms. The Gillette Children’s Specialty Healthcare team of experts works with specialists to diagnose the condition in your child.
Next, we partner closely with you to find the best treatment plan. Because Chiari malformation affects each child differently, we offer a wide range of tests, treatments and services for affected children, teens and adults. We start with a review of your child’s medical history. Some of the tests we might use to diagnose Chiari malformation include an MRI scan of the brain and/or CT scan.
Pain-relieving or anti-inflammatory medicines can help manage symptoms such as frequent headaches and pain.
Our pediatric neurosurgeons work closely with you to decide whether your child could benefit from surgery.
The Chiari malformation surgery (also known as posterior fossa decompression) increases space for the cerebellum and brain stem. This procedure relieves pressure on the brain and lets cerebrospinal fluid flow normally. If your child also has hydrocephalus—especially in combination with spina bifida and Type II Chiari malformation—shunt surgery might be required before Chiari decompression.
Treatment for the complex symptoms and effects of Chiari malformation require a team approach. At Gillette, your family will work with experts in a wide range of specialties and services that might include:
Our multidisciplinary team of experts is here to support your family, answer questions, and help your child feel their best every step of the way.