Chiari Malformation

Updated Oct. 13, 2020

Chiari malformation is a structural issue in the cerebellum, in which brain tissue extends downward into your spinal canal.

This rare condition occurs when the space at the bottom rear of your skull is smaller than it should be. This can cause the cerebellum and brain stem to be pushed downward, blocking cerebrospinal fluid, resulting in excess pressure on the cerebellum and brain.

The malformation may be present at birth or develop while the brain is growing and not show symptoms until adulthood.

Find out details on Chiari malformation treatment on and learn more about making an appointment.

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Symptoms of Chiari Malformation

Many people never experience symptoms of Chiari malformation and never need treatment. However, the most common symptom is a severe headache that starts in the back of your head and radiates forward, usually after coughing and/or sneezing.

Other symptoms include:

  • Neck pain
  • Loss of muscle strength in hands and arms
  • Dizziness or balance problems
  • Double or blurred vision

“There are symptoms with Chiari malformation that are more typical with the syndrome,” says Robert M. Friedlander, MD, chairman, UPMC Department of Neurological Surgery. “For example, a headache that begins in the back of the head and radiates forward, most often associated with coughing or sneezing or lifting a heavy object. That’s the most typical symptom of a Chiari malformation.

“There are other symptoms that also can present. For example, numbness and tingling of the hands and shoulders, trouble swallowing, ringing in the ears, and a variety of different symptoms.”

Diagnosing Chiari Malformation

Most often, a diagnosis comes by ruling out all other possibilities. Your doctor will review your symptoms and medical history and perform a physical exam. You will also have a range of imaging tests, such as CT and MRI scans.

Because the condition is rare, it’s important to see a doctor with experience. Experts at the UPMC Chiari Center take a comprehensive approach to evaluating, diagnosing, and treating Chiari malformations.

“The most critical part of managing a patient with a Chiari malformation is having the accurate diagnosis,” Dr. Friedlander says.

Chiari Malformation Surgery

The primary treatment for Chiari malformation is surgery.

  • The most common surgery is called posterior fossa decompression. A neurosurgeon removes a small section of bone in the back of the skull to make more space for your brain.
  • The surgeon may also open the covering of your brain (the dura) and sew a patch in place to enlarge the covering. This makes more space and relieves the pressure of blocked cerebrospinal fluid, which is what causes symptoms.

Chiari symptoms can overlap with other neurological conditions, so it’s important to get a thorough evaluation from an experienced neurosurgery team.

“What’s most critical in evaluating a patient with headaches and a lot of other neurologic symptoms is to determine if the patient’s symptoms are related to the Chiari malformation observed on an MRI, for example,” Dr. Friedlander says.

“Here at UPMC, our team has seen many, many patients with these kind of symptoms, and we’re able to make the determination that the symptoms are or are not related to the Chiari malformation. Therefore, the patients that are treated have the best chance of getting better from the procedure.”

About Neurosurgery

The UPMC Department of Neurosurgery is the largest academic neurosurgical provider in the United States. We treat conditions of the brain, skull base, spine, and nerves, including the most complex disorders. We perform more than 11,000 procedures each year, making our team one of the most experienced in the world. Whether your condition requires surgery or not, we strive to provide the most advanced, complete care possible. Our surgeons are developing new techniques and tools, including minimally invasive treatments. Find an expert near you.