Tag Archives: pyloromyotomy

Pyloric stenosis can be for adults too

Infant pyloric stenosis (“PS”) is not well-known in the general community.  Many of those who have been affected by it had never heard of it before.  But today far, far fewer people know anything about the adult form of PS.  As I will explain below, this is (in large part) a measure of the tremendous progress that medical science has been made in this area.

The stories of adult PSers vary even more than those about the infant form of the condition.  Some may find that hard to believe but it’s true!

When did problems start?
What happened?
What did the doctor say and do?
Did it work?
What happened then?
What choices did you face?
How are you doing now?

Think about it:  for adults there are many more variables than a baby might have: diet, wellness, lifestyle, age and stage of life…

However, the basic cause of all PS seems to be the same: high gastric acidity.  And one of the symptoms is usually the same: vomiting that is often severe and possibly (especially in infants) life-threatening.  But unlike babies with PS, adults usually find that with the loss of weight and wellness come pain, bloating, food intolerances, and reflux.  These are usually not a problem for the hungry but (at first) happy infant PS spitter.

In the infant disease, the high gastric acid level over-stimulates the pylorus (the circular muscle valve at the stomach’s exit), causing it to thicken, toughen and choke the muscle’s ability to relax and pass food.

Adult PS is also caused by high acidity, and often also by a virus, Helicobacter pylori, which has the nasty habit of stimulating acid secretion.  The acid erodes the stomach lining and creates conditions which enable the virus to trigger the eruption of gastric ulcers, which then scar as they heal, so thickening the walls of the stomach and pylorus, ultimately narrowing and blocking the stomach outlet.  If left untreated these ulcers can also give rise to stomach cancer.

These differences do not affect the name of the condition: “pyloric stenosis” means a “narrowing” of the pylorus (which means “gate”).  The term “hypertrophic” means “enlargement” and is only used of the infant form.  The effect of PS, whether in its infant or adult form, is also the same: the narrowing of the stomach’s exit ring muscle, causing vomiting and a reduction of food throughput, and thus starvation.

The difference in the development of the two forms of PS explains why the relatively simple “fixes” for infant PS (medication or surgery to relax the pyloric ring) do not relieve the adult form.  The PS baby’s problem is the thickening of the pyloric passage’s muscular outer layers, whereas the adult’s scarring, narrowing and blockage occur inside the pylorus or at the antrum, the tapered (narrowing) part of the stomach closest to the pylorus.

Adult PSers suffer from a range of symptoms including vomiting, reflux, pain, lack of appetite, and inability to maintain body weight.  The adult patients’ general practice doctors and GI specialists will try various treatments, starting with drugs to kill the virus infection and reduce acidity, and then relaxant medication and repeated stretching (“dilation”) to widen the pylorus.  Dilation is often repeated several times but is hardly ever successful as a long-term remedy. Sooner or later the patient usually decides to continue to one or more of a short list of surgical remedies, all of which are more severe and often less effective than the rather simple pyloromyotomy which is the usual surgery of choice for infants.

Pyloromyotomy

The simplest surgical remedy is pyloroplasty, a technique that was modified to become the pyloromyotomy which has usually remedied infant PS since Ramstedt’s accidental discovery in 1912.  Ramstedt discovered that it was not necessary to stitch the cut pylorus after he’d split it to relieve the enlarged muscle. He left the gaping pylorus wound to heal by itself in time.

[Select an image to enlarge it if you wish.]

Pyloroplasty

Pyloroplasty

But very sometimes the infant pylorus will not stay open for various reasons, and then a surgeon may return to the pyloroplasty: here the pylorus is split down to the mucosa or inner lining lengthwise (as in a pyloromyotomy); in a pyloroplasty the split muscle is then stitched closed across the pylorus, thus forcing it to stay open.  But because the adult pylorus is often scarred (thickened and hardened) by ulceration, it is often not in a fit condition to be modified.

Gastroenterostomy

The second option is commonly adopted: gastroenterostomy is a bypass of the pylorus by joining the duodenum to the stomach.  This removes the pyloric “gate” between the two which understandably has an effect on digestion.  Dietary changes and smaller, more frequent meals are necessary and often the “dumping syndrome” becomes part of daily living, as the body struggles to maintain a regular and appropriate source of energy.  Gastroparesis is a fairly common problem after gastric surgery: damage and interference cause the nerves and muscles of the region to stop working as they should.

So adult PS is more complex in its causes, symptoms, and available treatments.  And all these several treatment options are far from assured of success, as our gastric passage is easily unsettled and has a mind of its own: it is part of a complex network of different and linked organs and chemical input and processes.  Moreover, like any worker our abdominal organs can protest against being handled with less than good skill by working more slowly or sometimes a complete stop-work.  Medication and especially surgery can be quite successful or can result in unwanted and significant continuing physical side-effects.  Some “survivors” are pleased with the results of their choice, and others find they have to “adjust”, sometimes struggling to do so.

“Major adjustment” is thankfully something that is needed by only a very small minority of infant PS survivors.

Scared_DoctorDespite the dismissive words and comforting promises of pediatric surgeons, infant PSers also run a risk of a short list of abdominal and other complaints, some of them after their early surgery and then possibly also in later life.  Remember that the pyloromyotomy does not deal with the baby’s high gastric acidity.  One of the long-term risks is reflux and developing gastric ulcers – and as a result the adult form of PS.  Deja-vu!

But there’s also good news.  Today, thanks to effective and modern surgery, deaths from infant PS are almost nil.  And adults will find that with antibiotic treatment, H. pylori infection is usually quite easily dealt with, and so gastric ulcers and related surgery peaked in the 1970s and are now far less common.

Some personal observations are indicative despite being anecdotal rather than based on a careful study.  Adult gastric ulcers and PS used to be quite common but the only person I have ever known with it in 70+ years was one of my uncles – possibly a family linkage there?

Contrast this with the 8 or so infant PS patients and survivors I have known of or met.

The reader may discover similar figures!

Another measure is Facebook: following its PS Groups for 10 or so years I have logged 1,020 infant PS survivors but “just” 56 adults struggling with the condition of adulthood (several of tham after having survived infant PS).

The medical journal The Gut published an article in 2011 titled, The scars of time: the disappearance of surgery for pyloric stenosis – referring to the virtual disappearance of the adult form of PS, usually caused by peptic ulcer disease.

If medical science had made similar progress in reducing the incidence of infant PS, many parents and survivors would really party!

For readers who have stories or questions about adult (or infant) PS and its treatment and who use Facebook, I can recommend its “closed” Pyloric Stenosis Support Group which includes more than 50 members who have experienced and have posted (in great variety) about their adult form of PS.  (Any Facebook subscriber can find a “closed” Facebook Group, but it cannot be opened and read only by those who have joined that Group.)

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Can Pyloric Stenosis come with long-term effects?

Ask your General Practice doctor or a pediatric surgeon about the long-term effects of infant pyloric stenosis (“PS”) and you will almost certainly be told there are none.

However, the volume of questions, complaints, and feedback to online social forum sites such as BabyCenter/Centre, Facebook, MedHelp, Patient, and Topix is a clear sign that the answer to this question is not quite as clear and simple!

The issue of possible long-term gastro-intestinal (“GI”) and other complaints after PS is in fact a complex subject.  The social forum interest mentioned above shows that there is a short list of long-term effects that those affected suspect may not be unrelated to having had infant PS and/or the surgery to remedy it.

However, many of these complaints are hard for medical science to study and possibly recognise, and therefore for your doctor to treat.  So unfortunately for us, many busy doctors will brush off patients’ attempt at consultation about them as a waste of their time; others will recognise the problem but can usually offer little more than sympathy!

Besides this, GI problems in general (apart from PS) are the most common complaint we take to the doctor, so unravelling their cause and getting effective treatment is a painstaking business! In fact, a small but significant percentage of GI complaints have no verifiable cause at all – and even have a name: “Functional Abdominal Pain Syndrome”.

So… what is beyond doubt?

All surgery (even today) comes with short and long-term hazards, which are acceptable if a life is at stake.  These hazards have been minimised and some virtually eliminated in recent decades – but several remain.  The list of possible immediate and short-term hazards includes anesthetic complications, an unsuccessful procedure (so repeat surgery), wound disruption, and infection.

Those that can arise in the long(er) term include surgical adhesions, collateral damage (usually to the duodenum, stomach or vagus nerve), and emotional issues ranging from scar shame and emetophobia to pre-verbal trauma or mild PTSD.

If the baby has been significantly starved for any reason (usually poor and delayed diagnosis) there may be lifelong effects on several areas of brain function.  Emotional damage can also result from (as happened quite often in the past) the baby’s surgery being done with inadequate pain control, accompanied by significant maternal separation, or by the parents’ trauma resulting from the PS and surgery in any way being conveyed to the infant or to the growing child.

Then there is a short list of GI issues, which are common (also) among people without PS and PS surgery in their early history.  However, these problems seem to be experienced more often by survivors, and are acknowledged as possibly linked by some medical professionals and by several (mostly small) studies.  The theory that high gastrin levels (a blood hormone that controls gastric acid release in the stomach) causes PS in babies is one of the most obvious and plausible among the causes / etiology of IHPS, and this theory links strongly to GI problems in later life. It would also explain why GI problems can arise directly from the subject’s history of PS (the condition), and not the surgery.

The list of long-term GI complaints common among PS survivors includes reflux (or GERD or heartburn), several other GI development faults of infancy, high acidity causing IBS, and sometimes gastric dumping, ulcers and cancer.

There have been more than a few small studies reporting all this, and a few social forum reports of medical professionals who recognise the linkage from their own research and experience.

It may come as a surprise that so little is known (or recognised) about the possible long-term medical issues after PS.  It is because PS is so easily and usually successfully dealt with, and I suspect because the long-term problems are not life-threatening, that there have been no large studies of this subject area.  Besides, there are many more pressing medical challenges that need research time and funds.

The only large study that has stood out showed that the risk of PS is very much raised by mother or newborn using any of the macrolide family of antibiotics – which also relates to gastrin levels!

Despite all this, there has been at least one recent attempt to set up a sizable and robust study of the subject – which the PS community awaits with great interest!

In the meantime it must be realised that the medical community continues to submit to the surgeons’ love affair with PS and repeat the med school mantra that there are no long-term issues to keep in mind in relation to PS.

You can find all the above information on the web. If you need or want my evidence of the above, you’re invited to message me with your email address. I have a list of some 1700 reports and other material which can be accessed via the web.

Pyloric Stenosis’ Game-changer

The German Dr Conrad Ramstedt’s surgical remedy for infant pyloric stenosis was announced to the world at a medical conference and publication in 1912.

This event hardly affected the great majority of the human race, but it was of course rather significant for someone who underwent that surgery 33 years later to save his life just 10 days after his mother gave him birth.  And because pyloric stenosis (“PS”) affects between 3 and 5 babies in every thousand born in developed countries, and most of these are treated surgically, there are many people alive today because of Ramstedt’s discovery.

However, one only has to research the history of this condition and its treatment to realise that whilst the German doctor realised he had made a significant discovery, he was also the unwitting cause of trauma in at least some and perhaps many PS babies and their parents.  Let me explain…

In 1912, the medical treatment of PS babies was the rule but very risky, and almost half the infants died despite it.  Several surgical techniques were then being offered as an alternative for PS babies, but these were so drastic and severe on a tiny, malnourished and dehydrated baby that the great majority died of surgical shock, infection, and other related causes.  Most parents took their chances with the available medicines… and prayed.

RamstedtConrad operating

Dr Conrad Ramstedt operating

Dr Conrad Ramstedt’s accidental discovery in 1911 was published in October 1912 and represented a major breakthrough: see this post and this one.  The Ramstedt pyloromyotomy was rapidly adopted as the remedy of choice in most developed countries around the world, and in four decades after 1912, deaths from PS fell to almost nil in most countries.  However, even in the 1940s, some countries (including Great Britain) continued to report their PS mortality was still at 25%.  I have been shocked by how many mentions there are on the web of relatives dying of PS, even in the 1950s.  Despite all this, Ramstedt’s technique and better health standards in hospitals have done much to make death from PS most uncommon today.

How did the Ramstedt pyloromyotomy contribute to this?

Pic 13It made treating PS relatively easy and simple for surgeons, hospital staff, and parents. For surgeons the technique requires practice and care, but is essentially one of the simplest surgical procedures on the surgeries list.  The surgery usually ends the violent and deadly vomiting; although some continued vomiting and reflux occur quite often, it is fairly rare that an incomplete myotomy (division of the pyloric muscle) or the muscle’s redevelopment requires a repeat of the surgery.  So: anxious parents are greatly relieved, the surgeon immediately becomes a warrior-hero, and the baby quickly starts to make up for weight lost: I gather that my post-op photo is quite typical!

Apart from the great relief of all concerned, the Ramstedt pyloromyotomy saves pediatric ward staff and the bay’s parents from having to manage (or endure) weeks (and often two months) of medical treatment, with each of the sick baby’s feeds requiring medication be administered beforehand to a rigid schedule, milk having to be given slowly and carefully, daily weighing, and regular consultations with the hospital clinic or paediatrician.  Why put yourself through all that when surrendering your baby for just an hour or so to the gowned and skilled surgical staff produces what often seems like an instant fix?

Some babies are best treated surgically, and of the babies treated medically, up to 20% will not respond sufficiently well to avoid belated surgery.

Superman MD1On the other hand, almost all Ramstedt pyloromyotomies seem to be fully effective, certainly in the immediate sense.  And although the worldwide web includes many thousands of complaints and stories of a long list of troublesome ongoing effects from their PS or its surgery, it is just as clear that the vast majority of survivors and their parents are satisfied enough not to air their troubles.  The possible effects of the surgery are many and real, and sometimes severe, but many PS survivors report little or no gastric or abdominal discomfort, few or no problems with their scar or adhesions, and no trauma.  And this silence of the vast majority of PS patients has enabled most of the medical world to assure anxious and worried parents that “PS and its surgery will have no after-effects on your dear child”.

What I have written so far gives me some cause for concern, however.

Ramstedt’s discovery at once saved my life (and I’ve had more than 70 very good years so far), and it has also been responsible for the trauma I and not a few others have had to deal with (often chiefly in private) for most of our lives.

It was quite unintentional, but it is nevertheless true that Ramstedt’s surgical solution for PS effectively and inexorably moved the medical community’s interest away from perfecting the already (and still today) quite effective treatment of PS with cheap and simple medication and careful maternal nursing.  The Ramstedt pyloromyotomy is (as stated above) in itself what many surgeons call “elegant”: it is easy, quick and usually effective.  But until quite recently the surgery and what came with it could be very severe and traumatic on the baby and also on the parents, and it was associated with more (and more severe) risk factors than the medical alternative.

Thus the Ramstedt pyloromyotomy helped fuel the ascendancy of the power and prestige of the surgeon with which some of us are all too familiar today!  We must remember that specialist and high technology medical science has been very largely responsible for our rising health standards and life expectancies, but in fact PS is one of the maladies that can in most cases be brought under control by medical means and with surgery kept as a last resort.  In some developed countries, PS continues to be managed in this manner, and in many developing countries, medical treatment is far more affordable and widely available, and thus the first option.

So Ramstedt’s operation short-circuited interest in understanding and managing the causes, pathogenesis (biochemical development such as proposed by Dr Ian M Rogers) and even prevention of PS.

Whilst I am deeply grateful for the operation that saved my life and realise that in my case surgery may well have been the only responsible remedy, I have often wished that I could have been treated more gently, without a disfiguring scar, and without inflicting life-long trauma on my parents and me.

And in this wish I am not alone.

Pyloric stenosis: its infant and adult forms

This blogsite has mentioned several times that pyloric stenosis (“PS”) afflicts adults as well as infants.

Pain01Recently I devoted a post to the adult form, passing on the stories of several people who had related these on one of the Facebook PS Groups’ pages.  By using the “Categories” box at the top right of this site, interested readers can locate several posts that deal with adult PS.

The stories of adult PSers vary even more than those about the infant form of the condition.  However, the basic cause is the same: high gastric acidity.

In the infant disease, the high gastric acid level over-stimulates the pylorus (the circular muscle valve at the stomach’s exit, causing it to thicken, toughen and choke the muscle’s ability to relax and pass food. Peptic ulcer3

Adult PS is also caused by high acidity and often a virus, Helicobacter pylori. The acid erodes the stomach lining and creates conditions which enable the virus to trigger the eruption of gastric ulcers, which then scar the stomach and pylorus, ultimately narrowing and blocking the stomach outlet.  These ulcers can also give rise to cancer.

These differences do not affect the name of the condition: “pyloric stenosis” means “narrowing” of the pylorus (which means “gate”).  The term “hypertrophic” means “enlargement” and is only used of the infant form.  The effect of PS is also the same: the blockage of the narrow exit valve, starvation and vomiting.

The difference in the development of the two forms of PS explains why the relatively simple “fixes” for infant PS (medication or surgery to relax the pyloric ring) do not relieve the adult form of the condition.  The PS baby’s problem is the thickening of the pyloric passage’s muscular outer, whereas the adult’s blockage occurs inside the pylorus or at the antrum, the tapered part of the stomach closest to the pylorus.

Scared_DoctorThe main post mentioned above reflects the range of symptoms that adult PSers may suffer and the various treatments which are offered by GI specialists: unhappily adult PS is more complex in its causes, symptoms and available treatment.  All treatment options are far from assured of success and they tend to have unwanted and significant physical side-effects, far more often so than the great majority of infant PS survivors report.

Infant PSers have a much higher risk of a list of abdominal complaints after their early surgery and in later life.  One of the risks is of developing gastric ulcers – and the other form of PS.  Deja-vu!

But today, thanks to effective antibiotic treatment, H. pylori infection is usually quite easily dealt with, and so gastric ulcers are now much less common than they were in my younger years.

The next post will continue the stories of adults with PS.

100 years of Pyloric Stenosis surgery – an academic’s review

In October 2012 I posted a tribute and three reflective articles on the centenary of the publication of the German Dr Conrad Ramstedt’s surgical technique to relieve pyloric stenosis (“PS”).

This operation saved my life less than two weeks after my birth in 1945, and it has become the most used treatment of this condition and the most common non-elective infant surgery.  However, debate continues on issues such as –

  • Can PS be more promptly diagnosed more often so that more treatment options are available?
  • Should medical (rather than surgical) treatment be tried on more PS babies, with surgery as a later option?
  • How important is it to choose a surgical technique that reduces scarring in later life?
  • Can the symptoms reported by PS surgery survivors (PTSD and a long list of immediately or later evident abdominal complaints) be linked with PS or its treatment, and perhaps avoided?
  • Do the medical journal articles on PS and pyloromyotomy (PLM) deal with great repetition on just a small number of the related issues and avoid other significant matters?

Last year I found only one or two medical journal articles prompted by the centenary of Ramstedt’s pyloromyotomy: these were available only upon what would be for many a costly subscription, and the Abstract (supplied by only one publisher) gave nothing significant away.

Raveenthiran Prof Dr V 2012.bmpHowever, I am very thankful that during the past week, the Indian Prof. Venkatachalam Raveenthiran published an online review of the past 100 years of PS surgery which he generously made available to anyone interested.  There is also a ‘pdf version available on the web.

So once again I’d like to report on a medical journal report:  to summarise it, highlight the main points, and where necessary “translate” it and sometimes comment on it for general reading.

I have found that some of Prof. Raveenthiran’s observations are of real interest to one who has learnt to be thankful for the discovery of this surgery – despite the many years of my struggle with some of its after-effects.

  • The discovery of a surgical technique that brought to an end the very high mortality rate of PS babies has not reduced the interest in the condition.  In fact, in recent years the number of research reports about PS and PLM published annually has increased, and several of the formerly accepted data have been contradicted or rejected.
    However, I (Fred) am not alone in having observed that much of this output is very repetitive and sometimes even trite (stating what is already established).
  • US measures PLM01-1Dr Raveenthiran observes and discusses the growing reliance on diagnosing PS by ultrasound (U/S) technology rather than by simply palpating “the olive” (feeling for the swollen pylorus).  Others and I have reported this also.  The Professor reviews some of the current rather rigid diagnostic criteria, their failings, and their nature which show the need for more research if U/S is to be a more reliable and effective tool.
  • Another current “discovery” is questioned and analysed by the doctor: the widely quoted Danish study that claimed that bottle-fed babies had 4.6 times as much risk of developing PS as breast-fed infants.  This research report also contradicts the “established belief” of earlier years, and Dr Raveenthiran asks some pertinent questions.
  • Plm Lap single port01During the last 20 years circumbilical (around the umbilicus or navel) and Minimal Access Surgery (MAS, also laparoscopic) techniques and equipment have been increasingly applied to infant surgery, including PLM.  In recent years, as this website page shows, MAS using miniaturised equipment (still leaving three but smaller scars) and single-incision laparoscopy (SILS) through the umbilicus have come into use, for both adult and infant patients, and most reports state that after a learning process is completed, the results with the new techniques show no major disadvantages plus the benefit of an improved cosmetic outcome.
    Prof. Raveenthiran expresses bemusement at this string of claimed improvements, questions the practicality of SILS, and mentions yet another new technique, endoscopic PLM (removing the blockage from inside the gastric canal) which has not yet been used on human subjects.
    At this point I (Fred again) recalled reading a recent report that found most pediatric surgeons who had graduated more than 20 years ago were still using open incisions for their PLMs.  As they say,  It’s hard to teach an ol’ dog a new trick.
    As one of the countless PS survivors whose main after-effect has been our struggle to come to terms with an eye-catching and ugly scar, I say (as strongly as I can):  Hey! Please! Yes!  All other considerations being equal, bring on more cosmetically-considered infant surgery!
    Note: See also Dr Raveenthiran’s comment below.
  • In his final paragraph, Prof. Raveenthiran castigates the common attitude that “PS doesn’t have any after effects”.  My comment on this: Hear! Hear!
    He mentions something else I and others have often pointed out: the almost total lack of long-term follow-up of PLM patients, and of research into the long-term effects of PS and PLM.  He touches on some of the problems that have been discovered (but largely ignored and unknown): significantly lower cognitive, receptive language and motor scores, irritable bowel syndrome, and chronic functional dyspepsia and functional abdominal pain at a later age.  He calls for much more long-term study of these problems.

On a personal level, I was once again gratified and reassured as I found that almost everything Prof. Raveenthiran chose to mention and highlight in his essay was a matter that I have also raised, discussed and advocated for in these blogs – albeit without the benefit of medical training and without supposed academic status.  Isn’t it wonderful that the web allows any and all of us to dig out information for our own and others’ help and healing?

Another new year has dawned… Here’s to the victory of truth!