Health Watch: Chiari Malformation: Not Just a Headache

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As most of us know first-hand, headaches are annoying and can often be debilitating. In some cases, they can actually be life-threatening. (KFSN)

As most of us know first-hand, headaches are annoying and can often be debilitating. In some cases, they can actually be life-threatening. Some people are born with a condition that can cause more than just a lot of pain, but the good news is it's completely correctable.

For 10 years, ChonJae Trobridge suffered from chronic, debilitating headaches. Being outside in the sun made it worse.

Trobridge told Ivanhoe, "I was about 10 or 11 and it started as a headache once a day, then it was twice a day."

Sounds like a migraine, but it was much more serious than that- a congenital condition called Chiari malformation.

Allen Kantrowitz, MD, Chief of Neurosurgery at Mount Sinai Medical Center, Miami Beach told Ivanhoe, "It's an unusual cause of headache, but it's very serious, because it's a potentially life- threatening cause of headache and it is completely correctable."

Dr. Kantrowitz was able to fix the cause of ChonJae's pain.

"The area is very constricted and is squeezing a piece of brain tissue against another piece of brain tissue. That's where the danger arises. It can cause respiratory arrest or cardiac arrest," he explained.

Without cutting the brain itself, Dr. Kantrowitz removed a small piece of bone at the base of her skull to create extra space so the brain tissue was no longer squeezed- all done through a small incision.

Trobridge said, "The doctor came in and he was just like, 'well how do you feel?' and that's when I realized that I didn't have a headache anymore. It brought tears to my eyes."

Now ChonJae is able to enjoy the outdoors without getting a headache.

"I can laugh. I can be free. It's wonderful," she said.

The type of headache associated with Chiari malformation is typically in the back of the head and made worse by coughing, sneezing or bearing down. Surgery is the only curative treatment.

BACKGROUND: Chiari malformations, also known as CMs, are defects that develop in the cerebellum of the brain. The cerebellum is the area of the brain that controls a person's balance and sits in the indented space at the base of the skull. The condition occurs when part of the skull is misshapen or abnormally shaped. It is an uncommon disease, often going unnoticed until late childhood or adulthood. However, in Chiari malformation type II, the condition is noticeable at birth since it is congenital. Many patients with Chiari malformations have no signs or symptoms and do not require treatment for the condition. Typical symptoms of the condition are severe headaches, sudden coughing, sneezing or straining, neck pain, dizziness, difficulty swallowing, and poor balance. For patients with Chiari malformation type II, the symptoms tend to resemble those of a type of spina bifida called myelomeningocele, including weakness in arms, changes in breathing pattern and swallowing problems.

TREATMENTS: If a patient is diagnosed with Chiari malformation through testing and no signs or symptoms have presented in the patient, many doctors will recommend no treatment and observation only. The patients can receive additional testing to monitor the progression of the condition as well. Typically, headaches or other types of pain will be treated with pain medication. However, the best treatment for symptomatic Chiari malformation is surgery. Many patients who receive the surgery have a reduction in symptoms or a decrease in symptoms. Some may require multiple surgeries to completely treat the condition. The most common surgery is posterior fossa decompression, a surgery where a small section of bone at the back of the skull is removed to relieve pressure on the brain. Risks included in surgery are infection, fluid in brain, fluid leakage or complications in wound healing.

NEW TECHNOLOGY: Chonjae Trobridge was diagnosed with Chiari malformation after suffering from severe headaches for almost 10 years. Allen Kantrowitz, MD, Chief of Neurosurgery at Mount Sinai Medical Center in Miami treated Trobridge with a surgery localized from the junction of the brain to the cervical spine. Dr. Kantrowitz made a small incision, less than a half an inch, to give her brain relief. Scientists are researching if there are any genetic factors that contribute to developing the malformation. This research can help doctors develop alternate treatment options for patients who want to avoid surgery.

Interview conducted by Ivanhoe Broadcast News in October 2015

So this is an eight-hour operation?

Dr. Kantrowitz: There's a lot of preparation and timing, the actually surgery is considerably shorter than that. But the way we do this surgery now is the patient is very carefully positioned, actually prone. The navigation systems that we use to very precisely target the incision and the bone removal do take a little bit of time to set up and to allow us to make extremely precise measurements of where in the patients head we are. And then, of course, the work is done very, very carefully and delicately under microscope.

In a nutshell, this malformation, is it something you're born with?

Dr. Kantrowitz: Yes. You're born with a shape which predisposes you to entrapment of the cerebella tonsil, of the interior pole of the cerebella tonsil and then, over the decades, typically by the 20s, or the 30s people get into trouble from it. It will present as a headache, typically in the posterior region. Rarely, it will be a more diffused headache but the headache pattern that we are on the alert for is a headache that is experienced in the back of the head. Particularly if it is exacerbated by a cough or a sneeze or bearing down. Even more alarming would be any kind of syncope or fainting spell that's associated with a cough or a sneeze. Occasionally, patients will feel cardiac palpitations, which are due to the wedge compression of the brain stem by the cerebella tonsil as we had talked about before.

Is it dangerous?

Dr. Kantrowitz: Yes, it can cause cardiac arrest. It can cause respiratory arrest or cardiac arrest.

But, in many cases, people walk around with this and don't even know they have it?

Dr. Kantrowitz: Correct, correct.

What percentage of people?

Dr. Kantrowitz: It's an unusual cause of headache but it's a very serious cause, it's a potentially life-threatening cause of headache and is completely correctable. The treatment consists of a one shot deal with the surgery and that is the beginning and end of the problem in most cases.

She said she was feeling some numbness, weakness or something?

Dr. Kantrowitz: As the cerebella tonsil is jammed further down in to the top of the cervical spine, it creates more and more compression of the brain stem and of the highest segment of the cervical spine. That can produce any syndrome in the arms or the legs. Walking difficulty or it can produce any symptom in the arms and legs, motor or sensory problems or leg motor sensory problems.

This tonsil that you keep referring to, does it serve a specific purpose of that part of the brain?

Dr. Kantrowitz: It's involved with coordination and control of the muscles for walking and this kind of area.

So it's not as much the entrapment of that tissue as what happens with the fluid?

Dr. Kantrowitz: There are a number of consequences to the wedge compression. The wedge compression of the tonsil can create brain stem and upper cervical cord dysfunction. So that can be a life and death issue. That can cause cardiac arrhythmias, it can cause irregularities in breathing and that is the most dangerous of all the complications of Chiari. However, there are other elaborations of it. The trapping of the spinal fluid can cause a distortion in the structure of the spinal cord itself, a condition we call hydromyelia, which can interfere with the arms and the legs. There is an even rarer complication in which the hydromyelia cavity created in the spinal cord dissects upward and starts causing problems with control over the throat and perhaps, creating double vision. So there are rare cases where the Chiari becomes Chiari plus and plus. Plus, where there are more elaborate issues. What's extraordinary is the simple operation here, very frequently can correct even those elaborate complications. So we're very eager to do that. There are some patients who have an association with their Chiari and have problems with spinal fluid that we call hydrocephalus. Some of those patients are more appropriately treated with a distinct operation called ventriculoperitoneal shunting. This is an operation that is performed for hydrocephalus of any cause, Chiari is just one in a long list of potential causes of hydrocephalus. So we're looking for all of these issues in a patient who we have identified with Chiari and they all need to be addressed and thought about.

You said it's not really very prevalent, why is this important to get out there?

Dr. Kantrowitz: The reason it's important is for physicians to have a high index of suspicion. Because a patient who is previously well, who has a completely benign conditions, it can still be life-threatening and can change lives. And it is completely correctable and curable so the reason is Chiari can ruin people's lives and yet it can be fixed.

So how are their headaches related to this particular condition?

Dr. Kantrowitz: It's important to understand that there are no pain nerve endings in the brain tissue itself. There are, however, pain fibers in the lining of the skull and patients develop the headache back in this localized area because the brain tissue is directly mechanically irritating the lining in the back of the skull.

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