Doctors Lounge - Neurology Answers
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Forum Name: Neurology Topics
|my2sons - Wed Jan 14, 2009 11:12 am|
My 12 year old son had a "pull-through" for hirshsprungs disease at 4mths of age. He has since had on-going gut issues but was just diagnosed with Wolfe-Parkinsons-White syndrome as well as facial malformations. All of his issues have been on the left side of his body only. It has been mentioned that it may be a neural crest problem or a vagus nerve issue. I am very worried for my son's health. The holter monitor results have lead the doctor to order a "exercise test" and, perhaps, a trip to genetics.
Any help as to what direction I show go in would be wonderful. As a lay person, I am unable to navigate through the complexeties of neurology.
|John Kenyon, CNA - Wed Jan 21, 2009 11:46 pm|
Hi there -
Your son has Hirschsprung's disease, and has been treated, hopefully effectively, for it. Apparently there is a genetic problem and genetic counseling is always indicated, both in the interest of the health of existing family members and so that as your child grows up he can be informed as to what genetic problems he could possibly have to deal with in having children of his own (I know that seems like an awfully distant prospect, but time sure does fly).
Hirschsprung's can involve lack of vagus nerve cells downstream. While the vagus nerve does control not only stomach and intestinal actions, but also the slowing of the heart rate, it is no doubt involved to some degree. However...
Your son also has Wolfe-Parkinson-White syndrome, which is, like Hirchsprung's, a genetic disorder involving abnormality of nerve tissue, this time in the heart. The two are not directly related, but since they have much in common, are genetically- conferred and involve nerve tissue (extra in WPW, some missing in Hirschsprung's),
along with the facial abnormalities, this may represent a rarified triple familial defect that could have come from far back in the blood line or, as is sometimes the case, could represent a new mutation that can still be passed along.
I'm sure you're now pretty conversant in Hirschsprung's, but WPW may be new to you, and the vagus nerve is not directly implcated in WPW, although it may try to play a role. WPW is the presence of an accessory nerve pathway not normally found in the heart, which bypasses the atrio-ventricular (AV) node, the "hall monitor" of the heart, where all normal impulses arise in the sinus node in the atria, pass through the AV node, and then are distributed equally to both ventricles. With WPW and the accessory bypass tract, whatever goes on in the atria can be passed along to the ventricles, unregulated by the AV node. This is most of the time not a problem, but for some reason people with WPW can also develop episodes of atrial fibrillation (AF). While this is generally rare in young people, it does happen on occasion in those with WPW. This becomes a problem because during AF the atria start to beat so fast as to flutter chaotically at a rate of around 300 beats per minute. This normally would be directed through the AV node, which acts as a sort of "gearbox" and blocks about half the beats, so that AF is conducted to the ventricles at a rate of about 150 beats per minute and very irregularly. This is problem enough, and usually happens in older patients and those who have had heart surgery. However, since with WPW it's likely to happen at some point in the young patient, and since it can be transmitted down that abnormal accessory pathway, the ventricles, which are much larger and less able to adapt to such a rapid, irregular rate and rhythm, can potentially go into ventricular fibrillation, a life-threatening arrhythmia, since they simply cannot keep up with that rate, let alone the irregularity of it.
WPW can be treated medically or, more often and more successfully, with radio frequency ablation via catheter, a one-day in-and-out procedure which destroys the patency of the accessory pathway. Prior to having this done, the patient should be treated with medication to prevent or slow AF if it should occur.
While some doctors seem to be unaware of the potential dangers of WPW, it is responsible for a few deaths in otherwise healthy young people annually, and these are rather unpredictable. For this reason your son should be started on a medication to manage this until such time as the accessory bypass tract can be ablated. (That's a very minimally invasive procedure and when successful, which it almost always is, solves the problem for good with extremely low risk and no long-term problems at all).
I hope this is helpful to you. Please follow up with us as needed, and by all means press your son's doctors to address the WPW issue promptly. Everything else should take a back seat to this for now. Once it's been addressed either by medication or RF ablation, then the other, possibly genetically-related issues, can be managed with relative ease.
Good luck to you both. Please keep us updated.
|my2sons - Thu Jan 22, 2009 8:31 am|
Thank you so much for your help. The cardiologist said it was unusual for someone my son's age to be diagnosed with WPW without showing any symptoms. The reason it was found was because during a blood test, the results showed his triglycerides at 3.6 in the first test and 3.3 in the second. To confuse the issue, his cholesterol was normal both times. Yes, I know, weird. Because the cardiologist feels that what ever is going on with my son, it is not fully expressed as he as not entered puberty fully and is concerned that when the hormones really start flowing, there will be many more issues to be dealt with.
I have learned over the years how to be a "partner" with my sons physicians, and there are many, from GI to Endocrinology to Rheumatoligy, to Cardiology. At this point in time, though, I don't know how to proceed. The cardiologist seems certain that, as long as the heart muscle is strong and he is symptom free, that working out the other concerns, specifically the triglycerides, is by far the most important on the list of things to do.
There are so many issues and I don't know which I should push first. We spend a great deal of time listening to doctors hum and haw as to what is "causing" the issues and what specialist we should see next.
Any thoughts on the triglyceride issue. That one seems to have stumped everyone.
|John Kenyon, CNA - Fri Jan 23, 2009 5:30 pm|
The trigyceride abnormality may well be a pre-diabetic lesion, so probably should have some effort aimed at managing it, either via diet and exercise or possibly with medication. Over time the cause may be discovered, but it may also be just a personal idiosyncracy. There could be a lot of metabolic testing involved in uncovering the underlying cause, but the immediate problem should be treated even so.
The issue of WPW not being normally diagnosed in children as young as your son is something of a misunderstanding among medical people. The main reason so many cases are missed until later is because without symptoms (which are inclined to become more prominent in adolescence and are highy unpredictable anyway and even often missed by the patient), there is usually no reason for a child of that age to be screened for WPW. Among those who are screened due to it being present in family members, or in someone who suddenly develops symptoms, it shows up proportionately much more often.
I think the doctor is correct about the onset of puberty and the possible emergence of more symptoms in general, but this applies to most every area of every child's health when entering that phase of life. Meanwhile, WPW has too often been brushed aside as unimportant because of youth, or because of lack of symptoms, only to have one dangerous incident change the entire outlook and suddenly a whole family is being screened. Having both clinical and personal (family) experience with this particular phenomenon and related issues, I'd be remiss if I didn't strongly urge any parent of a child known to have this abnormality to be aggressive about its being followed, managed and, as early as possible, corrected.
Forgive me if I seem to overemphasize the importance of the finding of WPW, and while I am personally familiar with the case in the following link, I've also had a similar personal family experience and a variant as well, so while it is only one point of view, I feel strongly enough about this to refer you here: http://www.longqt.org/bryan.html
Please follow up with us here and let me know your thoughts also on the subject of WPW as shared in the above-linked site.
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