Chiari Malformations
Chiari malformations are differences in the way the brain and spinal cord connect in the back of the head. Sometimes, these differences cause no symptoms. But a lot of the time, kids with these conditions can have headaches, vision problems, and trouble with balance.
At UVA Health Children’s, our team of pediatric neurosurgeons has extensive experience in caring for children with Chiari malformations. Our experts will help you determine whether your child needs any further studies or tests, and if you should consider any treatment. We can also offer support to help with any developmental concerns caused by the condition.
What Is a Chiari Malformation?
Chiari malformations in children without spina bifida are thought to be a result of the back lower part of the skull not being large enough to accommodate the back lower part of the brain (the cerebellum). With brain growth early in life, the bottom of the cerebellum then grows down into the upper spinal canal.
Chiari malformations are quite common and can be seen in almost 1% of children. Fortunately, only a small portion of these children are affected by the Chiari malformation and require treatment.
How Are Chiari Malformations Diagnosed?
If your child has symptoms or a medical history that make Chiari malformation possible, our radiologists will do an MRI or CT. These scans allow doctors to see details of the brain.
A child with Chiari malformation should be seen by a physician expert experienced in the condition. A medical history and physical exam and possible further imaging studies or tests may reveal problems associated with the Chiari malformation and help determine if your child needs treatment.
What Are the Common Types of Chiari Malformation?
Chiari Malformation Type 1
Type 1 is the most common overall.
Type 1 Symptoms:
- Headaches (often involving the back of the head)
- Neck pain
- Balance trouble
- Scoliosis (abnormally curved spine)
- Numbness or tingling in hands and feet
Chiari Malformation Type 2
Type 2 Chiari malformations occur almost exclusively in children with spina bifida (those children born with myelomeningocele). These malformations develop in the fetal stages because of loss of spinal fluid through the defect (myelomeningocele) in the back.
Type 2 Symptoms:
- Trouble swallowing
- Unusual eye movements (nystagmus)
- Respiratory distress
Our neurosurgeons and neonatal team will talk to you about appropriate treatment options. Surgery for Type 2 Chiari malformation is rarely needed.
Conditions Connected to Type 1 Chiari Malformations
Sometimes, Chiari malformations occur alone. But sometimes they’re related to another issue.
- Craniosynostosis: a condition in which one or more of the fibrous structures in an infant’s skull fuses prematurely, changing the skull’s growth pattern
- Hydrocephalus: a condition that develops when cerebrospinal fluid (CSF) builds up in the brain, causing pressure on brain tissue
- Familial: roughly 5% of Type 1 Chiari malformations are inherited. Our experts can discuss with you whether screening siblings need screening.
Chiari Malformation Treatment
We’ll talk to you about your treatment goals. Most of the time, the goal is to relieve symptoms or prevent them from getting worse.
Watch & Wait
If your child’s symptoms are mild, we may recommend a watch-and-wait approach. During this time, we’ll still continue following up with your family. That way, if problems do emerge, we’ll be able to treat them promptly.
Medical Management
Medicines to control symptoms like headaches can be enough for some children. We’ll talk to you about the risks and benefits of this approach.
Surgery for Chiari Malformation
There are multiple surgical options for Chiari malformation treatment. While not all pediatric neurosurgeons have a lot of experience with this condition, the ones at UVA Health Children’s do.
We’ll talk through the options, risks, and benefits with you.
After surgery, we’ll monitor your child’s recovery and progress. If you live out of town, we offer remote follow-up appointments when appropriate.
Recovery & Development
Before, during, and after treatment, we can offer additional support that may help improve your child’s overall wellbeing. That support includes therapy to improve motor skills, language development, or mobility.
We can also offer counseling for your family. Genetic counseling and testing can help families concerned that genes may be contributing to their child’s condition. And our family stress clinic can help with the emotional and mental strain of having a child who needs medical care. As a children’s hospital, resources like our child life specialists help us make our hospital a welcoming place for children to heal.