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Chiari Malformation Center

Chiari malformations occur in the back of the head where the brain and spinal cord connect. When this intersection does not form correctly, structures on the underside of the brain can push through a normal opening (foramen magnum) in the bottom of the skull where it joins the spinal canal. Problems associated with Chiari malformation include headache, sleep apnea, hydrocephalus and neuromuscular issues.

The Chiari Malformation Center's team of surgeons brings together years of expertise in addressing the entire spectrum of Chiari malformation conditions, in infants and children and in adult patients. Our multidisciplinary approach allows us to confer with colleagues in all areas affected by these disorders, including orthopaedists, radiologists, rehabilitation medicine specialists, rheumatologists, urologists, and other experts.

Chiari Malformation: What You Need to Know

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  • Chiari malformation (also known as an Arnold-Chiari malformation) is a congenital defect, meaning it is present at birth. The exact cause of Chiari malformations is not known.
  • There are different forms of Chiari malformation. Type 1 is likely to be diagnosed in teens and adults. Type 2 is evident in newborns and infants and may be associated with spina bifida.
  • A Chiari malformation can result in syringomyelia, in which a cyst (pocket of fluid) forms in the spinal column. As the cyst expands, it can put pressure on the spinal cord, resulting in neurological problems. Syringomyelia can progress to paralysis if its underlying cause is untreated.
  • Surgery may relieve symptoms.

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Chiari Malformation in Adults

  • Although Chiari malformation type 1 is present at birth, it may not cause symptoms until years later when the affected person is a teenager or an adult.
  • Chiari malformation type 1 symptoms include headaches that are usually located at the back of the head. These headaches often get worse with exertion. 

Patient Resources

Chiari Malformation in Children

  • Chiari malformation type 2 is typically seen in infants born with spina bifida, a neurological condition that causes a portion of the spinal cord and the surrounding structures to develop outside, instead of inside, the body.
  • Type 2 Chiari malformations can also be associated with hydrocephalus, an overproduction or lack of absorption of the cerebral spinal fluid (CSF) inside of the ventricles (fluid-filled areas) of the brain.
  • Excess fluid in the ventricles causes increased pressure inside of the head. The baby’s skull bones can expand to a larger-than-normal appearance.

Meet Our Physicians

Why choose Johns Hopkins for treatment of Chiari malformation?

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Our Treatments

Our team puts together a treatment strategy for each individual based on factors such as the type of Chiari malformation, the patient's age, and new and ongoing symptoms.

Decompression surgery may be recommended to make more room for the part of the brain that is pushing out (herniating) through the opening in the bottom of the skull. Surgical procedures at Johns Hopkins include unique means of lifting the skull bone to allow the brain to return to a more natural position, and cranioplasty for adult patients to restore more normal skull contours following decompression.

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Our Patient Care

Our combined years of experience in diagnosing and treating patients of all ages with Chiari malformations and syringomyelia enable us to plan seamless treatment and follow-up for patients from infancy into adulthood.

Johns Hopkins’ experts are passionately dedicated to ensuring that all patients and families affected by Chiari malformation and syringomyelia have access to the most effective therapies, compassionate treatment, information, support and empowerment.

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Maryland Patients

Adult Neurology: 410-955-9441
Pediatric Neurology: 410-955-4259

Adult Neurosurgery: 410-955-6406
Pediatric Neurosurgery: 410-955-7337


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