A Chiari malformation is a problem with how the brain sits in the skull. The brain normally sits fully inside the skull. With a Chiari malformation, the lower part of the brain (cerebellum) dips down through a normal opening (foramen magnum) at the bottom of the skull. In some cases, more brain tissue also dips down through this opening. The normal opening becomes crowded because too much tissue is there, and that can affect the tissues and surrounding fluid. This can cause head pain and/or trouble with balance or movement. In most cases, the problem is present at birth (congenital).
Why choose WVU Medicine Children’s?
Patients with Chiari malformation travel from all over the region to receive care from our experienced neurosurgery team. Most of our patients return to a normal life without restrictions, thanks in large part to our treatment approach, the expertise and experience of our physicians and careful, long-term follow-up care.
WVU Medicine Children’s is home to some of the most experienced neurosurgeons in the country. Since 2018, more than 75 children have undergone decompression surgery for Chiari malformation type I at WVU Medicine Children’s.
Personalized treatment approach
WVU Medicine Children’s works with families to evaluate the severity of a child’s condition and develop a personalized treatment plan. This involves performing diagnostic tests to determine the severity of the Chiari malformation, addressing health concerns associated with Chiari malformation, and monitoring your child’s symptoms to determine whether they improve or worsen over time.
Our experienced pediatric neurosurgeons use sophisticated techniques to reduce the risks of neurological surgery. They can treat patients whose conditions previously were considered inoperable.
Collaboration is an important factor to successful treatment. Our specialists include a pediatric neurosurgeon, neurologist, neuroradiologist, among others. They share their insights and expertise, establish a comprehensive diagnosis, create a detailed care plan and monitor each child’s progress closely to ensure that treatment is as effective as possible.
A second opinion from our team can reassure families their child is receiving the best possible care – or offer insights about other treatment options available at WVU Medicine Children’s.
What causes a Chiari malformation type I in a child?
Health experts don’t know the exact cause of a congenital Chiari malformation type I. A problem during fetal growth may cause the defect. It may be caused by contact with harmful substances during pregnancy, or it may be linked with genetic problems that run in families.
What are the symptoms of a Chiari malformation type I in a child?
Your child may not have any symptoms. Or symptoms may develop slowly over time. Most children don’t have symptoms until they are teens or young adults. The most common symptoms are headaches or pain in the back of the head or neck. The headaches and pain are brought on by coughing, laughing, or sneezing. Your child may also have other symptoms of a Chiari malformation type I. These include:
- Hoarseness or trouble speaking
- Trouble swallowing
- Rapid, back and forth eye movements (nystagmus)
- Periods of not breathing during sleep (sleep apnea)
- Weakness or abnormal movements
- Trouble with balance
- Abnormal reflexes
- Abnormal shape of the spine (scoliosis)
Your child may also have a pocket of fluid in the spinal cord or brain stem. This is called a syrinx. A syrinx can cause trouble with the function of the spinal cord, such as movement or sensations of the arms and legs.
How is a Chiari malformation type I diagnosed in a child?
In a child with no symptoms, the problem may be found when imaging tests are done for other reasons. For a child with symptoms, the healthcare provider will ask about your child’s health history and give your child a physical exam. They may refer your child to a specialist.
Imaging tests are done to discover a Chiari malformation type I. Your child may have one or more of these tests:
• MRI - This test uses large magnets and a computer to make detailed pictures of the inside of the body. In some cases, a special dye is injected into a vein for the test but is not usually needed when getting images for a Chiari malformation. This lets us clearly see the tissue of the brain and spinal cord, and the fluid surrounding those tissues.
• CT scan - This test uses a series of X-rays and a computer to create detailed pictures of the inside of the body. A CT scan is more detailed than a regular X-ray, and ultimately focuses on the bones of the skull and spine.
How is a Chiari malformation type I treated in a child?
Your child may be treated by neurologists and neurosurgeons. These are experts in brain and spinal cord problems. Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.
• With no symptoms - Your child’s health may be watched closely. Your child’s healthcare provider may advise surgery to prevent problems.
• With symptoms - Your child’s symptoms will be discussed to determine if they are related to the Chiari malformation. Decompression surgery may be recommended if the symptoms are felt to be related to the Chiari and likely to improve with surgery. This is done to give the tissues more space and restore flow of spinal fluid around the base of the brain. If your child’s symptoms are not likely related to the Chiari malformation, you may be referred to a neurologist for medical management.
• With few or no symptoms, but a syrinx - When fluid builds up in the spine, surgery may be recommended. This is done to prevent symptoms, by restoring flow of spinal fluid around the base of the brain and reducing damage to the spinal cord. If the fluid on the spinal cord is quite small and your child has no symptoms, close monitoring with follow up imaging may be considered.
• With signs of sleep apnea - Your child may need a sleep study. In this test, your child will be watched during sleep to look for problems. A sleep study can also help the healthcare provider decide on additional treatment.
What are the possible complications of Chiari Type I in a child?
It is possible your child may not have any symptoms in the future. However, some children develop complications. These include:
- Long-term pain
- Development of syrinx
- Permanent damage to muscles or nerves
Carefully watching for changes in your child’s health can help prevent complications. This helps to make sure that treatment is done early.
How can I help my child live with Chiari malformation type I?
It is hard for healthcare providers to predict how a Chiari malformation type I will affect a child’s long-term health. Your child may not have any changes caused by the malformation. Or they may have nervous system problems that get worse. Your child’s health will be closely watched. This may include frequent physical exams and imaging tests, such as MRI. There is ongoing research as how to best manage Chiari malformations.
When should I call my child’s healthcare provider?
Call your child’s healthcare provider if you notice any changes in your child. Be sure to call if you notice problems with:
- Walking or moving
Key points about Chiari malformation type I in children
- With a Chiari malformation, the lower part of the brain dips down through a normal opening at the bottom of the skull. In some cases, more brain tissue also dips down through this opening. In most cases, the problem is present at birth (congenital).
- The most common symptoms are headaches or pain in the back of the head or neck. Prior to a headache, a normal sneeze or cough causes a headache.
- Treatments can include careful watching, surgery, and exams and tests.
- Carefully watching for changes in your child’s health can help prevent complications. This helps to make sure that treatment is done early.
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
- Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
- Know how you can contact your child’s healthcare provider after office hours, on weekends, and on holidays. This is important if your child becomes ill and you have questions or need advice.
There are 4 types of Chiari malformations (CM); however, it’s important to note that while all these types are called “Chiari,” they are completely separate. In other words, they are not a spectrum of disease severity or a progression (someone doesn’t progress from Chiari Type I to Chiari Type II and so on).
- Type I (CM-I): This is the most common type. Part of the cerebellum dips down through the bottom of the skull. This type is most often congenital (also called “primary CM”) but is often not found until a child is a teen or young adult. In rare cases, this type may also develop later in life. This is known as “acquired or secondary CM.” It occurs from a loss of spinal fluid. This can happen because of an injury, contact with harmful substances, or an infection.
- Type II (CM-II or Arnold-Chiari malformation): Part of the cerebellum and the brain stem dip down through the bottom of the skull. This is most often seen in babies born with spinal myelomeningoceles or spina bifida. A myelomeningocele is when a part of the spinal cord and backbone (spine) develop outside the body. A common problem with Type II CM is too much fluid on the brain (hydrocephalus). The extra fluid causes the pressure in the brain to increase and the skull bones to expand beyond normal size.
- Type III (CM-III): The cerebellum, brain stem, and possibly other parts of the brain dip down through the bottom of the skull. In rare cases, the brain and brain covering may poke out through the back of the head or neck. A baby with Type III CM may not live long. Children who do will have severe nervous system problems, such as thinking problems, seizures, and muscle problems.
- Type IV: This is a very rare condition where the brain doesn’t develop fully. Most babies with this type don’t survive.
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