Freddy: You know, I'm a rather brilliant surgeon. Perhaps I can help you with that hump.
Igor: What hump?
This is Chapter 2 of the continuing saga of FrankenKristin’s brain. If you wish, you may read Chapter 1 and Chapter 1.5.
Arnold Chiari Malformation (ACM) is an uncommon, congenital anomaly in which the lower part of the cerebellum protrudes down into the spinal canal. This causes tissue compression, which hinders the normal flow of the cerebral spinal fluid (CSF). It was first identified by Austrian pathologist Hans Chiari in 1891.
Although ACM is congenital, many people are asymptomatic. In many adults with ACM symptoms are often brought on by trauma such as whiplash and can have wide-ranging severity with only subtle neurological signs, or cause complete incapacitation due to extreme headache and cervical pain, vertigo, ataxia, visual disturbances and diminished sensation. Studies are also beginning to indicate an overlap between ACM symptoms and those attributed to Fibromyalgia and Chronic Fatigue Syndrome. The difference between them being the presence of tonsillar herniation and interruption of CSF flow. Unfortunately there is no cure for ACM, and the only treatment is surgery. The most common procedure is Posterior Fossa Decompression whereby a small area of the bone at the base of the skull is removed to enlarge the posterior fossa. In some cases, the cerebellar tonsils are shrunk using an electrocautery to restore normal CSF flow.
The question many people (including family, friends and our medical insurance company) asked was why we needed to go all the way New York for evaluation. After all, there are plenty of other neurosurgeons in MN, plus the world renowned Mayo Clinic is just down the road. We asked ourselves the same question.
Our decision to go to The Chiari Institute was based on the fact that we couldn’t afford to waste our time and money going from one neurosurgeon to another (local or otherwise) with only marginal experience in this area. We wanted to know for sure whether or not the ACM was the source of the problem, due to Kristin's desire to avoid surgery unless it was truly necessary. We were clearly looking for the best possible treatment options and we wanted to avoid the possibility of a less experienced doctor recommending a treatment protocol that would not address the problem.
Our research also showed that The Chiari Institute is the world's first comprehensive, multidisciplinary center for the management of patients suffering from Chiari Malformation. The surgeons at the Institute specialize in Chiari and have more experience with this condition than any other surgeons in the country. Beucause there is no standardized surgical method for ACM, most surgeons tend to develop a method that works for them and use it consistently for all their Chiari surgeries, regardless of the presenting physiology. The doctors at TCI have developed a technique using Intraoperative Color Doppler Ultrasonography, a method used in the past to monitor blood flow during aneurysm surgeries. The technology was adapted to measure the flow of cerebral spinal fluid during decompression surgeries thus allowing the surgeon to tailor each surgery according to patient-specific variables.
The procedures used by TCI reduces the additional costs that are often associated with complications, corrections, and/or multiple surgeries. In fact over half of the surgeries performed at TCI are revisions of failed surgeries performed elsewhere. Dozens of which were initially performed at some of the most prominent medical institutions in the United States.
So off we went. Neither of us had been to New York, and since I was finished with school for the year, we decided to make a vacation out of it. We spent a weekend in Manhattan with my cousin and her husband, who’ve lived in NY for years, acting as our tour guides. We visited the Brooklyn Bridge, The Statue of Liberty, Ground Zero, St. Partick’s Cathedral, and St. Paul's Chapel. We took in a Yankees game, had dinner at a fancy restaurant, pizza at the oldest pizza parlor in the US, and cannoli at a NY institution. In addition to riding the subway all over town we walked about 8 miles that the weekend.
On Monday we made our way to Great Neck and The Chiari Institute. Although we brought along an armload of films and test results Kristin still spent the better part of a morning undergoing additional X-Rays, CT Scans and a cineMRI designed not only to depict the inside of her head but to also measure the flow of cerebrospinal fluid.
The next day we found ourselves in the lobby at TCI waiting, and waiting, for our evaluation. While they pride themselves on quality of care, by giving each patient as much time as they need, the wait time can often be excruciating. We finally met with Dr. Mora who completed a patient history, and a detailed neurological exam. Although he was thorough, and helpful, he was simply setting the stage for Dr. Bolognese, one of two surgeons at TCI. Dr. Bolognese, along with Dr. Milhorat, perform nearly 300 surgeries a year. In the afternoon Dr. B. is available for consultation and evaluation. Being one of the foremost experts in Chiari, we eagerly awaited Dr. B’s opinion, hoping he would give us a definitive answer.
When the door to our exam room finally opened, he strode in freshly scrubbed, chart in hand. Without even introducing himself, he slammed the chart on the table and in a thick Italian accent declared, “There is no way in hell your symptoms are not caused by this Chiari.” Over the next hour he dissected the test results, explained the reason for the symptoms, and outlined the prognosis. When we asked how the surgeon we saw in MN could have looked at the very same films and determined that the Chiari was asymptomatic he responded, “When you’re an expert in one thing, it means you suck at everything else. She probably sees one or two Chiaris per month, I do 4 surgeries a week.”
Ultimately, he determined that although Kristin’s symptoms were significant, she was not an emergency case. While she was a candidate for surgery, her need was not immediate and thus he left the decision up to her. However, he did say that if she decided against surgery, her current state would be her new baseline and that over time she would most likely get progressively worse. While the prospect of surgery was scary, it was a relief to know that there might actually be hope for improvement.
So we came home with what we believed was the definitive answer we were looking for. Unfortunately our auto and medical insurance companies were not as easily convinced. Not only did we have a major decision to make regarding the surgery but we were also facing an uphill battle in our attempt to get coverage. Nonetheless, Kristin called a few days later and scheduled her procedure for September, the soonest available date, and we began to plead our case with both insurance companies.