Ray D’Alonzo, Ph.D., is a visiting professor in the Chemistry Department of the University of Massachusetts Amherst and a retired R&D Manager of Procter & Gamble where he worked for over 31 years.  He has led research programs in bone metabolism, infectious disease, respiratory disease, arthritis, and nutrition and has published scientific papers on a wide variety of topics from the chemical composition of fats and oils to the pharmacoeconomics of osteoporosis.  Dr. D’Alonzo is the recipient of the Chancellor’s Medal from the University of Massachusetts, Amherst, in part, for his contributions to the development of new pharmaceutical agents.  As both a patient and scientist, he has made a personal effort to increase the awareness of Chiari in the health care sector and to assist others afflicted with the syndrome.  He has published the story of his personal struggle with Chiari in a book, Contents Under Pressure, with 100% of royalties going towards Chiari education, awareness, and research programs.

March 31st, 2009 --     

Recently, a reader sent me an email asking me to recommend a doctor. He told me that both neurologists and neurosurgeons had warned him about “over eager doctors" with respect to Chiari. This was not the first time I had heard of patients being told this. Over the last 10 years, I have heard it dozens of times. While I have never heard it myself, I have heard it so many times from others that I am convinced it is said frequently.

Unfortunately, there are doctors who believe that some of their colleagues perform decompression surgery unnecessarily. These allegations are both serious and disturbing and only serve to confuse Chiari patients attempting to sort out their needed treatment options. I thought I would offer some possible explanations as to why this happens.

When an individual becomes a physician he or she takes the Hippocratic Oath to first do no harm. I believe most doctors do their very best to live up to this oath. I submit that most neurosurgeons are in fact conservative in their decision to perform Chiari decompressions. So why the disconnect?

There are several reasons. First, some doctors have never diagnosed a Chiari case and most have had little formal training about Chiari. What many of them know is second hand and/or outdated information. Our knowledge about Chiari is relatively new. It wasn't possible to study it in any depth prior to the advent of MRI. MRI didn't begin to go into wide spread use until the mid 1980's. MRI studies and subsequent clinical studies on Chiari didn't begin to find their way into the medical literature until the 1990's. Even after studies make their way into the literature it takes several years for the information to make its way into text books and the class room. As a result, many doctors believe herniated cerebellar tonsils are a variant of normal anatomy even when the extent of herniation is quite significant. These doctors do not believe herniated tonsils cause symptoms and often believe the complaining patient to be simply suffering from clinical depression. Doctors oriented in this way, can't imagine other doctors justifying the need for surgery. They believe antidepressants are the best course of treatment. Careful not to slander colleagues, they use the term “over eager” rather than a term like reckless.

Second, Chiari is complex. No two patients with Chiari present the same way. It is hard for doctors to see a clinical pattern they easily recognize as Chiari. They don't begin to more readily recognize it until they have seen dozens of cases. Chiari also resembles other diseases which like Chiari do not present with objective signs and symptoms. These other diseases include depression, chronic fatigue syndrome, and fibromyalgia. Ruling out these other diseases can be extremely difficult. For one, Chiari often coexists with depression. It can also coexist with chronic fatigue syndrome and fibromyalgia but to a much lesser extent. (When a doctor gives a diagnosis of Chiari and chronic fatigue syndrome/fibromyalgia, the patient should be highly skeptical of the latter.) Chronic fatigue and fibromyalgia are also controversial in the minds of many doctors. Obtaining an objective handle on Chiari even with MRI can prove to be difficult in many cases. Many doctors need to see clear objective balance problems, central sleep apnea or involuntary eye movements before being convinced the patient is suffering from Chiari. Unfortunately, according to the only prospective study1 in the medical literature on the symptoms of Chiari, less than half (46%) of the patients studied presented with dysequilibrium, and only 5.7%, 5.6%, and 5% present with apnea, vertigo, and nystagmus (involuntary eye movement) respectively. And, while coexistence of syringomyelia (spinal cord cyst) is sometimes considered important or confirmatory in the diagnosis of symptomatic Chiari, few doctors order MRI scans of the spine for a wide variety of reasons ranging from cost to not observing clinical signs and symptoms of syringomyelia. MRI of the entire spine should be performed in the absence of classic clinical symptoms of syringomyelia because syringomyelia can be asymptomatic and can also occur at any location along the spinal cord.

Third, adding to the complex situation described above was an article that was published in the Wall Street Journal about ten years ago. The article was about a very small handful of surgeons performing hindbrain decompressions not on Chiari patients but on patients with fibromyalgia. It implied that the surgeons were performing the procedure for the purpose of collecting lucrative surgery fees. It caused an uproar in the neurosurgery community resulting in labeling the surgeons as over eager.

Fourth, Chiari is thought of by most doctors as non-fatal with the risk of death from general surgery higher than that for untreated Chiari. However, this may not be true. The medical literature contains many case studies of death assigned to Chiari induced cardiac arrest or pulmonary failure. The true incidence of these deaths is likely to be falsely low due to the low awareness of Chiari in general. Further, there is evidence that the suicide rate in Chiari patients may be as much as ten-fold higher than in the general population. This estimate stems from the finding1 that 47% of Chiari patients present with depression, many of which find little relief with antidepressants and many of which have a history of suicide ideation and/or suicide attempts.

Fifth, unfortunately, the failure rate for decompression surgery is about 20% with failure defined as no improvement or a continuation of worsening. The reason for this relatively high failure rate is not well understood but it probably is only minimally influenced by over eager surgeons. It is more likely due to the complex nature of the syndrome. It may also be attributed in part to the manner in which surgical outcomes is measured. There is no validated standard outcome measure used for Chiari decompression surgery. Surgical outcomes are also measured at different time points by different surgeons. The relevance here is that a significant number of patients continue to feel poorly and complain about it following surgery and given the orientation of certain critical doctors, it serves as confirmatory evidence that too many decompressions are being performed out of aggressive eagerness. It in essence falsely validates their beliefs.

So where am I going with this and what is the point I am trying to make? My point is that alternative treatments to surgery are for the most part ineffective. Further, using drugs to treat the symptoms of Chiari can mask symptoms and signs of progressive neurological damage such as syringomyelia. Decompression surgery is the best hope for recovery for many Chiari patients and even though the failure rate is significant, it must be seriously considered without the insertion of fear. Patients must not let warnings of over eager surgeons frighten them from considering surgery. What is extremely important is for the patients who are considering surgery to keep their ears open as opposed to hearing what they want to hear. There is about a one in five chance that decompression surgery will provide a significant benefit but here is also about a one in five chance that it will not provide any benefit. In the end, the procedure is elective and only the patient can decide to have the surgery. The decision must be made with the best available information. Doctors are entitled to their opinions. Patients should consider the inevitable warnings about over eager surgeons but place them in perspective at the same time.

1 Mueller & Oro, J. Am. Acad. Nurs. Prac., Vol. 16, Issue 3, pp. 134-138, March 2004.

-- Ray D'Alonzo

 ** If you would like to share your comments, thoughts, or ideas with Ray,  please send them to dalonzo.rp@gmail.com.  Due to the volume and nature of email received, individual responses are not possible. **

[Ed. Note:  The opinions expressed above are solely those of the author.  They do not represent the opinions of the editor, publisher, or this publication.  Mr. D'Alonzo is not a medical doctor and does not give medical advice.  Anyone with a medical problem is strongly encouraged to seek professional medical care.]    
 

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