Brad Weprin, M.D. Answers Questions On: Chiari Malformation

We don’t clearly know what causes this structural defect in the cerebellum. It’s primarily a congenital problem – the formation occurs during fetal development – but sometimes they don’t become symptomatic until late childhood or early adulthood, and sometimes never at all.

We believe they are present in 1 in 1,000 births or 1 in 1,200 births, so they are fairly common, but the vast majority of people never have problems. That’s a significant aspect of this diagnosis that interests me – trying to figure out the natural history. What’s different about the people who develop symptoms versus those who don’t? I’m fascinated by the research going on in this area.

Sometimes the Chiari can affect quality of life quite significantly due to pain or some of the neurological deficits that occur as a result of it.

Surgery is most beneficial for individuals who have clearly defined problems related to Chiari. There are a lot of people around the country being operated on for Chiari who do not need to be, and they are now experiencing complications that could have been avoided. 

I have very clear-cut indications for who needs surgery. These include a specific type of headache in the back of the head that affects daily activities and doesn’t respond well to medical management. I can perform a safe operation that cures these headaches, and they never come back. 

Another indication for surgery is when the Chiari malformation is associated with a spinal cord problem called syringohydromyelia that can cause scoliosis over many years and will eventually result in loss of function in the arms and legs. Surgery on the Chiari malformation eliminates this problem.

And finally, if people have certain forms of sleep apnea or swallowing problems related to the neurologic effects of Chiari on the brain stem or upper cervical spinal cord, they would benefit from Chiari surgery, but these situations are quite rare.