Chiari & Pregnancy

November 30th, 2009 -Scientists and research physicians are always looking for projects where maximum learning can be rendered from minimal investments. We call these project low hanging fruit. Every year since 2004 usually in the January edition, Conquer Chiari publishes a review article summarizing research progress. While overall progress is slow because of limited research funds, some interesting findings have emerged but for the most part the message sounds like a broken record for those of you old enough to have experienced listening to a broken record. Most of the studies published are individual case studies which aren't particularly helpful for understanding the general condition. The remainder is mostly split between studies reporting on new surgical techniques or comparing surgical techniques along with outcome results and studies looking for new objective measures of diagnosing Chiari primarily using MRI. Surgical and outcome studies have produced results all over the map and have proved difficult to compare because of the lack of standard and valid method for evaluating outcome. And, finding a way to objectively measure the disease in a reliable manner has proved elusive even with the most sophisticated imaging technologies. I remain confident however that these important problems will be resolved in the near future particularly if agencies like the National Institutes of Health increase funding for Chiari research to a fair level.

But, the topic of this column is not so much the need for better treatment outcome and diagnostic measurement research as it is to point out one particular area of needed research that has been on my mind for several years and that is Chiari and child birth. The incidence of Chiari in women may be as much as three-fold higher than it is in men1 as derived from a large landmark prospective study. Also, with increased awareness and better access to MRI, the mean age of symptomatic onset as reported in the same study is about 24 years of age. As a result, many women of child bearing age are finding out on a daily basis that they have Chiari. Many of these women are thinking about starting a family. Some are in a situation where they are already pregnant. In both scenarios, questions abound. Is it safe for me to have a baby? What about my safety? Will labor make my symptoms worse? What are the possible complications? If my Chiari gets worse, how will I manage to care for my baby? These are just a few questions.

It is well known that the strain involved during labor may increase intra-cranial pressure. And this simple fact gives rise to concern when pressure on the hind brain structures is already compromised in patients with Chiari. I have been involved in the Chiari community now for about 12 years. During this time, I have talked with numerous women and have read numerous posts on-line by women who have discussed their problems and fears with child birth. A large number have claimed that labor triggered their symptoms and that prior to becoming pregnant they were asymptomatic. Others claim that child birth significantly worsened their symptoms.

With this in mind, I recently did a search of the medical literature to see what has been reported with regard to Chiari and pregnancy/child birth. In setting out to do this search, I anticipated that I would not find many papers but that within those I did, I would see that Chiari did indeed result in complications during pregnancy and the postpartum period. A quick search revealed five publications2-6, about the number I expected. Four of these papers were case studies. One paper4 was a small study involving seven women. I was shocked after reading the abstracts that not a single complication was reported. In fact, in all cases, the Chiari symptoms of the mothers either resolved or returned to pre-pregnant levels postpartum.

Given the large number of women who have testified otherwise, how can this be? Without getting technical, the likely primary explanation for this apparent discrepancy is that the number of women studied in these publications is small. This deficiency is actually called out in the publication by Mueller and Oro'4. In this paper, the investigators employed a very simple study design where the seven women participating were asked to complete questionnaires pertaining to their symptoms during pregnancy, labor and postpartum. This study really amounts to a pilot study and needs to be repeated using a much larger number of women. In order to do this a study with multiple sites would need to be designed so that a larger number of patients can be recruited to participate in a reasonable period of time. A multi-center study of simple design could be very cost effective and would provide some real insight as to the real incidence of complications associated with child birth as well as the nature of the complications. The study could also be designed to screen women of child bearing age by MRI for possible inclusion in the study who themselves are daughters of a parent with Chiari. This might result in more quickly completing study enrollment as well as provide an idea of the risk of passing along the condition assuming it is genetic.

Sticking my neck out on a limb here a little, let me make an editorial remark which is that the world of Chiari research is dominated by men. As such, they seem to be overly focused with playing with their high tech toys to measure things like intra-cranial flow, pressure, structure dimensions, and compliance or with advancing surgical techniques involving the shape of dural grafts and grafting materials. It's the snakes and snails and puppy dog tails in us that gives us this kind of driven focus and dare I say insensitivity to other needs. We need to listen more to sugar and spice and everything nice a bit more. If we did, I guarantee that there would be more than four case studies and one pilot study in the literature and we could really put a dent in this awful disease. Chiari and complications with child birth looks very much to me like low hanging fruit ripe for picking.

Seasonal Postscript: I would like to clarify for readers that the only proven effective measures for preventing the spread of the common cold and flu, both H1N1 and seasonal, are vaccination, isolation, and respirators (not masks). The spread of these infectious viruses is largely achieved by airborne transmission. Their spread by hand contact is very secondary. There are no clinical data showing that the wearing of dust and surgical masks can prevent the spread of these viruses. Coughing and sneezing into ones elbow has little effect on lessening their dispersion into the air. The washing of hands is effective but only against the relatively small percentage of cases that are spread in this manner. Viruses are extremely small, smaller than most airborne dust particles. If you have ever watched the sunshine beaming through a window, you cannot help but observe all the tiny dust particles that seem to float endlessly in the air. This is exactly what viruses do. When an infected person coughs, sneezes or even breaths they fill the air around them with millions of virus particles. Coughing into ones elbow does little to trap them. The same is true for dust and surgical masks. These tiniest of microbes pass right through them or exit right along with the air around the edges of the mask which are far from air tight. It may be polite to cough in your elbow but it is totally ineffective. The most effective measure to prevent their spread is to dilute their airborne concentration by improving ventilation. If you are in an enclosed room where people are coughing, try to open a window or leave the room. Of course, people with the cold or flu should remain home and work if necessary by remote computer access if possible. I like to provide such information based on data as opposed to medical mythology this time of year because the last thing a Chairi patient needs is the flu or a nasty cold. Stay healthy my friends!

1Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients, Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC, Neurosurgery, 1999 May;44(5):1005-17.

2Neuraxial analgesia during labor in a patient with Arnold-Chiari type I malformation and syringomyelia, López R, Nazar C, Sandoval P, Guerrero I, Mellado P, Lacassie HJ.,
Rev Esp Anestesiol Reanim. 2007 May;54(5):317-21.

3Uneventful epidural labor analgesia and vaginal delivery in a parturient with Arnold-Chiari malformation type I and sickle cell disease, Newhouse BJ, Kuczkowski KM., Arch Gynecol Obstet. 2007 Apr;275(4):311-3.

4Chiari I malformation with or without syringomyelia and pregnancy: case studies and review of the literature, Mueller DM, Oro' J., Am J Perinatol. 2005 Feb;22(2):67-70.

5Anaesthesia for caesarean section in a patient with syringomyelia and Arnold-Chiari type I malformation, Agustí M, Adàlia R, Fernández C, Gomar C., Int J Obstet Anesth. 2004 Apr;13(2):114-6.

6Maternal Arnold-Chiari type I malformation and syringomyelia: a labor management dilemma,
Parker JD, Broberg JC, Napolitano PG., Am J Perinatol. 2002 Nov;19(8):445-50

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.