Chiari Malformation

Chiari Malformation

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If your child was born with a Chiari malformation, he or she may need surgery immediately after birth. Or doctors may recommend simply monitoring for any symptoms that develop as your child grows. Duke pediatric neurosurgeons identify Chiari malformations at the earliest possible stage, evaluate your child's condition, and recommend a treatment plan to prevent or limit neurological damage. We work with specialists throughout Duke to manage your child's care, especially if your child has another medical condition associated with Chiari malformation.

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About Chiari Malformations

Chiari malformations are structural defects that cause symptoms ranging from headaches and muscle weakness to developmental delays and breathing problems. Typically, they occur in the area where the brain and spinal cord meet. In most cases, parts of the brain push down into the spinal canal and cause pressure in the brain and on the spinal cord. This often blocks the flow of cerebrospinal fluid (CSF), which can cause fluid-filled cysts on the spinal cord and hydrocephalus (a buildup of fluid in the brain). There are four types of Chiari malformations. Types III and IV are rarely seen.

Type I is the most common and can go undiagnosed until symptoms develop during childhood, adolescence, or even into adulthood. This type can cause fluid-filled cysts (syringomyelia) on the spinal cord. Scoliosis (spine curvature) often is associated with type I.

Type II typically affects children born with spina bifida, specifically infants with myelomeningoceles -- spinal cord tissue that protrudes through an opening in the spine.

Type III is a rare and very serious form of Chiari malformation. Some parts of the brain may stick out, or herniate, through an abnormal opening in the back of the skull. 

Type IV is very rare. In this type, part of the brain is underdeveloped or missing.

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Duke Health offers locations throughout the Triangle. Find one near you.

Diagnosing Chiari Malformations

Duke Children’s pediatric neurosurgeons combine clinical evaluation with advanced testing to fully understand your child’s condition and recommend the right level of care for type I and type II Chiari malformations. 

Type I Chiari Malformations
For type I Chiari malformations, we conduct a thorough medical history and physical examination, including a review of family history to look for a possible genetic cause. We also perform a detailed neurological assessment of your child. A sudden headache when coughing, sneezing, or laughing -- anything that causes pressure in the head -- is one of the most common symptoms.

Type II Chiari Malformations
For type II malformations occurring with spina bifida, we collaborate with your child’s neurologist and otolaryngologist (to assess for evidence of brainstem dysfunction) for diagnosis and treatment.

Imaging Tests
We may recommend imaging tests, such as MRI or CT scans, to confirm the diagnosis. A special type of MRI -- a cine MRI scan or CSF flow study -- will show the flow of cerebrospinal fluid and help determine whether it’s building up in the brain or spine.

If your child’s symptoms are minor, we may recommend monitoring the Chiari malformation with periodic imaging as your child grows. In younger children, a Chiari malformation may actually improve on its own, and surgery may not be needed.

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Our pediatric neurosurgeons collaborate with specialists who care for children with conditions associated with Chiari malformations. Your child's care team may include an orthopedic surgeon if scoliosis is involved, or a neurologist if your child was born with spina bifida or recently developed symptoms from a type I Chiari malformation. These specialists will continue to monitor your child's progress to ensure effective treatment.

Headache Medication

If a Chiari malformation is causing headaches as the only symptom, pain medication may be all that is needed.

Shunt Repair Surgery

Often, symptoms of a type II Chiari malformation can be relieved by repairing a malfunctioning shunt, which drains accumulated spinal fluid from the brain to treat hydrocephalus. If your child has a shunt that is no longer working, it can make Chiari malformation symptoms worse. Shunt repair may be all that’s needed.

Decompression Surgery

When symptoms become severe and affect your child’s ability to function, surgery can help. Decompression surgery creates more space around the lower brain and upper spine area by removing bone, which relieves pressure on the spinal cord and halts further damage. Depending on your child’s condition and circumstances, the surgeon may consider opening the dura and inserting a patch to further expand the space. Decompression surgery also restores the normal flow of spinal fluid.

Syringomyelia Surgery

When a fluid-filled cyst develops on the spine, our pediatric neurosurgeons open the spinal cord and insert a shunt to drain the cyst and relieve pressure. This may improve scoliosis if caused by the cyst and eliminate or postpone more invasive scoliosis surgery.

Duke Children's Hospital is nationally ranked in 9 specialties
Best Children's Hospital in NC
Duke Children's Hospital & Health Center is proud to be nationally ranked in nine pediatric specialties.

Care of Chiari Malformations from Recognized Experts

Whether surgery or monitoring, your child receives care from specialists who are contributing to the national understanding of Chiari malformations with the goal of improving diagnosis and treatment.

Network of Expert Surgeons
Duke Children’s is part of a national group of neurosurgeons and orthopaedic surgeons who share their knowledge and work to improve treatment for Chiari malformation.

Latest Research Studies
We’re part of a study funded by the National Institutes of Health that’s investigating hereditary factors of type I Chiari malformations. This will lead to improved diagnosis and treatments.

Regionally Respected
We are regionally known for our expertise in treating children with Chiari malformations. Families come to Duke Children’s for second opinions and, after meeting our experienced team, often choose us to care for their children.

Recognized Children's Surgery Center
Duke Children’s is one of a small number of U.S. hospitals verified as a Level I children’s surgery center by the American College of Surgeons. This Level I designation recognizes our commitment to providing the safest and highest-quality surgical care to our young patients.