Chiari malformation can be linked to certain birth defects, so children born with these defects are usually tested to see whether they might also have a Chiari malformation.

Most people with Chiari malformation (usually type I) appear healthy. So the diagnosis can come as a surprise and often follows testing, examination, or treatment for another condition. Chiari malformation also might be hard to diagnose in these cases because the symptoms are vague and can be caused by many other conditions. Often, people with type I Chiari malformation aren’t diagnosed until they’re teens or adults.

If your doctor suspects a Chiari malformation, your child will be examined and tested for things like speech, balance, reflexes, and motor skills. The doctor will probably want your child to undergo one or more of these tests:

• Magnetic resonance imaging (MRI), a safe and painless test that uses magnets, radio waves, and computer technology to produce very good pictures of internal body parts, such as the brain and spinal cord. It can help to determine the severity of a malformation and to monitor it later on. Your child may be given an injection of a dye called a contrast medium to improve the images. The test can take up to an hour to complete.
• Cine MRI is a specific type of MRI that doctors use to see whether the flow of CSF from the brain to the spinal cord is blocked.
• X-rays produce images of bones on film and can detect bone abnormalities caused by Chiari malformations. They are painless, quick, and safe.
• Computed tomography (CT or CAT) scans produce pictures of bones and other body parts using X-rays and a computer. Scanning is quick and painless and can detect obvious blockages or measure how much of the brain is out of place.
• Brainstem auditory evoked response (BAER), a test that measures brain activity in response to sound, helps to determine whether the brain stem is working correctly.
• Somatosensory evoked potential (SSEP) testing helps determine whether messages from the brain can properly travel through the spinal cord.

Currently, no test can determine in advance whether a baby will be born with a Chiari malformation; however, some malformations can be seen on ultrasound images before birth.

Treatment

There is no cure for Chiari malformation. However, most children who don’t have symptoms won’t require treatment. They should, however, continue to see their doctors for regular checkups to make sure that their condition doesn’t change.

When symptoms do occur, treatment will focus on relieving them and stopping further damage to the brain and spinal cord (for example, medications may be used to ease symptoms like headaches and neck pain). And sometimes the doctor will recommend limiting activities that worsen symptoms.

If symptoms become severe, you may be referred to a neurosurgeon to discuss whether surgery is a good idea for your child.

A common procedure for kids with Chiari malformation is decompression. It helps to relieve pressure on the brain and spinal cord, restore the flow of CSF, and prevent additional damage. This surgery usually takes several hours and involves removing part of the skull and a portion of the spine’s top vertebra.

Another surgical option involves implanting a shunt or tube to help drain CSF and relieve pressure on the brain. Shunts are commonly used to help children who have hydrocephalus or syringomyelia.

Surgery isn’t a cure but it can help to greatly improve the problems caused by a Chiari malformation. It does have drawbacks, though. Surgery can be painful and can put a child at risk for infection and other complications. That’s why it’s important to talk to your doctor and weigh the pros and cons when deciding what is best.

Since doctors sometimes disagree on the best way to treat Chiari malformations, it’s also wise to get a second opinion before making a decision.

Outlook

The less severe the condition, the better the prognosis is for kids with Chiari malformations.

Many kids do not experience health problems and those with mild symptoms usually find that medications provide enough relief for them to lead normal lives. Symptoms typically don’t get worse and surgery usually isn’t necessary for kids with these milder forms.

For those who do undergo surgery, a successful long-term outcome depends on the severity of symptoms and how long they existed prior to surgery — kids whose symptoms were present for a brief period before surgery are more likely to have symptoms resolve compared with those who had symptoms for many years.

The Neurology and Neurosurgery programs at Primary Children’s Hospital have some of the nation’s best physicians, providing the latest care to children from across the country with Chiari Malformation.