Tag Archives: medical treatment

How Japan handles infant Pyloric Stenosis

Most readers of this blog are more than interested in Pyloric Stenosis (“PS”), either because they or one or more of their children had infant PS as a baby.

PS is the most common form of bowel blockage to affect newborns in their first 4 or so months.  In most developed countries, all but the mildest cases are treated surgically. The surgical technique is a long-standing and firm favourite among surgeons: once mastered it is quick, simple, almost bloodless, and usually immediately effective. Parents who had been traumatised by their baby’s uncontrollable and violent vomiting and then by having to surrender their little one to be anesthetised and then cut open receive their little one back alive and with the promise: All done, you have a new baby, sick no more, home soon, and nothing to worry about in the future!  No wonder surgeons just love the Ramstedt procedure!

But if you look up “pyloric stenosis” on any of the larger social forum websites, you learn more.

  • The parent trauma resulting from PS and infant surgery can be deep and long-lasting,
  • this can affect the child also, and
  • the surgery is not always free of long-term problems, in both the immediate and long terms.

Such problems are probably far from the rule, and most Py babies seem not to look back. However, there are no substantial studies on this subject, and the ongoing effects of infant surgery on parents and sometimes the whole family, let alone the patient, can certainly be significant.

During my 20 years of researching the many issues related to PS, I have been surprised to learn that in some developed countries far from the Anglo-European world, surgery is the exception rather than the rule for PS babies.

The reports relating this are few but persistent.  For many decades now, academics and medical practitioners in countries including Japan, Taiwan, and Turkey have reported on regarding PS  surgery as a last resort.

ranitidine01Their policy is to treat most infant PS medically as the first option. The drugs involved are atropine sulphate and Ranitidine, drugs that reduce gastric acidity production. Very small or fragile babies, and those that do not respond to medical treatment within a stated time, are referred to a surgeon.

The outcomes are comparable by almost every standard: morbidity, mortality, and short-term problems. The hospital stay is longer, but the total cost is lower. Parents are happier and less traumatised.

Now a  2018 Japanese study has reported a small trial of adding a second drug, nitroglycerin or glyceryl trinitrate (GTN), in those cases that do not respond to atropine sulphate.  GTN is widely known as an explosive but is also often used to treat heart conditions.  Using both drugs meant success in treating all the PS infants in the study.

It is well worthwhile clicking on the link in the previous paragraph to read the full article, published by Open Source publisher Science Direct.

This is a story worth recording also! Most medical reports are published by large for-profit companies that first charge researchers for publishing their work and then the readers for access to the reports and discussions.  Open Access publishers believe that new research and discoveries should be published online without cost to the authors and should be freely available to anybody interested.

Posting this great news makes me soooo happy!

I am one of many millions who have gone for years hating our scars from infant surgery. PS survivors know that without this damage most of us might not be alive today!

But what if we knew that our parents had had a choice between a few days or weeks of supervised medical treatment – and disfiguring surgery?  And that they chose surgery without considering how we their child might be affected by that in years to come? We PS babies had no say in our treatment – but our parents were our advocates!  And parents who learn that infant surgery not only saves lives but can come with adhesions, collateral GI damage, and/or long-term psychological effects… who would not give medical treatment a try first?

This free medical article from Japan explains how treating infant PS medically is their first option – and it’s all that’s needed for up to 90% of PS babies to survive. This case reports details 2 cases when old-established medical treatment failed but was successful when combined with skin patches of another drug!

But… can our surgeons wean themselves off their favourite surgery?

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 surgery – “somewhat improved”

Has the treatment of infant pyloric stenosis (“PS”) improved with the years?

Yes!  In a recent post I listed many of the clear and obvious ways it has.

Adults struggling with IBS, adhesions, or PTSD may well doubt that; any and all of these can at times be linked with their infant surgery.

The mother I read about recently must surely also doubt that much has been learnt: she was diagnosed with PTSD a few months after suffering with her newborn through several weeks of slow and shoddy diagnosis followed by “last minute, life-saving” PS surgery.

And the continuing avalanche of parents’ posts on social forum sites like Facebook and BabyCenter show this hapless mother is far from alone.

However, what I wrote in the above-mentioned post stands: it is beyond doubt that, thank God and thanks to the medical community, infant surgery including the treatment of PS has made huge progress.

Infant surgery03Last year I read the summary of a 2014 report that supports the claim that the actual surgery to remedy PS has also improved – but only marginally.  The survey evaluates the records of 791 little PS patients of a pediatric surgeon over a 35 year period (1969-2003).

Most of the results reported in the Abstract of this article (sadly, all that is publicly available) merely confirm the usual facts about PS, information that will not surprise those who know something about this condition.

  • 82% of the patients were male and 18% were female.
  • The average age (presumably at surgery) was 38 days and ranged from 7 days to 10 months.
  • Only 5% were not Caucasian.
  • 10% had a family history.
  • 15 babies (3.1%) were premature at the time of diagnosis (so in fact, many more).
  • 9% had other conditions or abnormalities.
  • 10 babies (1.2%) developed PS after surgery for another condition.
  • 13 (1.7%) were treated medically and avoided surgery.
  • All the pyloromyotomy operations were done by open surgery: the incisions used were sub-costal, transverse, or upper midline.
  • 14 babies (1.7%) had other surgical work done (presumably including herniation).
  • 87 of the operations (10%) were followed by complications: 1.1% happened during the surgery, and 9% post-operatively.
  • 2 babies died.
  • Other evaluation results showed some areas of improvement.
  • When ultrasound imaging was used, the age at diagnosis was reduced by about 10 days.
  • All the operations were done using general anesthesia and endotracheal intubation (breathing tube).
  • From 1982, precautionary antibiotics were given before surgery and this resulted in wound infections being reduced to 3.9%.

This surgeon was also responsible for correcting the inadequacy of the work of some non-pediatric surgeons, and these statistics make grim reading:

  • There were 13 such little patients, 12 of them transferred from non-pediatric surgeons.
  • These 13 accounted for 16 complications including one death.
  • 5 of the babies needed further surgery: 4 for an incomplete pyloromyotomy and the other for a perforation of the pyloric canal.

The report drew these conclusions:

  • IHPS should be considered in any vomiting infant.
  • Ultrasound examination allows earlier diagnosis.
  • Serious complications are uncommon and avoidable, but recognizable and easily corrected.
  • Surgeons who do more than 14 pyloromyotomies per annum see fewer complications.

This report (as stated above) deals only with the actual surgical treatment of PS, and not the complaints of many about the total management of this condition.  The report featured does not survey the standard of the diagnosis of PS, nor the often uninformed, sweeping, and simplistic reassurances given about the possible short- and long-term after-effects of PS and its surgical treatment, about which so many doctors and parents seem to be quite “in the dark” (or possibly in denial).

RUQ PLM-3This blogsite and the social media posts of countless parents and patients express gratitude for the survival of almost every PS baby, ever since the Ramstedt pyloromyotomy (surgical operation) rapidly became the standard treatment after 1912.

It is often remarked that the Ramstedt pyloromyotomy is one of the few surgical techniques that has continued as the standard and virtually unchanged since it was introduced.  It is relatively quick and simple to perform, and almost always immediately effective (as much as can be expected of any surgical procedure).

What the report implies but fails to acknowledge is that many older surgeons continue to perform Ramstedt’s pyloromyotomy using the old and often disfiguring open incisions.  Other recent statistics show that the new and cosmetically superior laparoscopic surgery is now used in over half of PS operations.  Understandably but sadly, many older surgeons resist mastering current best practice.

What then is clear from the material collected and reviewed in the two posts (this one and the linked post)?

  • The overall management of PS has seen huge progress.
  • The actual surgery for PS has changed little in a century, but continues to be marginally and slowly improved on.
  • There remain several areas of immediate and significant concern to PS patients and their parents which the medical community is loathe to recognise, let alone seriously tackle.

And therefore numerous PS parents and patients will continue to speak up, network – and post!

Networking after pyloric stenosis (3): Diagnosis

Parents of babies afflicted by pyloric stenosis (“PS”) are routinely assured that after surgery their little treasure will be “a different (and hugely improved) baby”.  Often this is immediately true: instead of a baby vomiting him- or herself to death, PS babies often thrive and quickly reach the top of their percentile range.

But of course this is so only if the operation was –

  • without complications (like infection or a hernia), and
  • successful in opening up the pyloric passage (which it usually but certainly not always is), and
  • if the baby is not left with years of GERD (reflux) related problems, a rather common outcome.

Many with a personal experience of PS, whether as parents or survivors, do have ongoing problems –

  • during the immediate recovery period,
  • during the child’s first years and sometimes continuing lifelong, and/or
  • in adulthood.
  • These people often resent that the medical advice they received
  • denied these possibilities,
  • left them with false expectations that” all would be well”, or
  • skirted over the future  possibilities embedded in a list of “to be mentioned” items the parents had to acknowledge and sign before surgery.

And when problems did arise (sometimes within days, sometimes after some weeks or years), the typically rather obvious link with abdominal surgery was denied despite several research reports to the contrary.

Diagn palp.jpgAmong those who report having had a bad time with PS however, no group is as numerous or angry as those who experienced a diagnosis debacle, and in this post we listen to just a few of these parents, again from the several PS Groups on Facebook.

A L was born about 1985 and had a son in 2009
I was born with PS and my second child, a son, William was diagnosed with it at 4 weeks.  Luckily, having had it myself, I was well aware of the odds of my child having it, especially a son.  The pediatrician as well as the ER wasn’t extremely willing to diagnose PS until I advised them that I had also had it.  Prior to that, I got quite a bit of attitude and snubs…
It is very disturbing to me reading others’ posts that something that is so easily rectified is so easily dismissed.

E D, born 1984
It feels so nice to know I’m not the only person to have had this condition!  Even now doctors, midwives etc don’t know what PS is: awareness needs to be raised!  I was one of the unlucky ones and had my operation in 1984.  My mum had to fight with the doctors as they wouldn’t diagnose me with PS as I was a girl!  I had my operation at 3 months when my weight was lower than my birth weight – thankfully I’m here to tell the story!  Have had two little boys and thankfully neither of them have had it!

arrogant doc5L G – son born 2014
My son had PS at 5 weeks.  The doc said it was reflux, even though the health visitor said it was PS – because he was born at 36 weeks he said he couldn’t have it that young.

M G – son born 2010
My son was diagnosed at 5½ weeks old with PS in 2010.  It took doctors 5 days and numerous times of me arguing till I was sent to a new hospital to find out what he had.  My son almost died but had his surgery and soon bounced back.  I am so thankful he is better.  But I wish hospitals and doctors would check babies closer for this kind of condition instead of it being fobbed off as reflux all the time.

C L – son born 2014
My son had PS and had his operation at 5½ weeks old after me refusing to leave the doctors as they just kept prescribing gaviscon for reflux.  He had key-hole surgery and was instantly better!
Our surgeon told us our son had been left that long his body had used all its fats and was about to start using up its muscle.  Thank God he was seen when he was.

M K – daughter born 2013
self-harmMy daughter, now 10 months old, had her surgery at 5 weeks old, was misdiagnosed by 5 different doctors over a 5 day period and had 2 negative upper GIs… she weighed 6 lbs at birth and at 5 weeks old was 4lbs 10oz after no doctor would test her for PS.  We almost lost her from dehydration before I could find one that would… all because she was my second born daughter and they said [that the] odds were PS was not the cause for her projectile vomiting!
She is now experiencing bad reactions to milk.  Took her to the doctor and they had no explanation other than possible allergy symptoms from sensitive stomach MAYBE related to her having pyloric stenosis as a newborn.  But they have no clue.  I thought it was all behind us, as those few weeks back in May were the worst I’ve ever experienced.

D M – son born 2014
My son is 16 weeks old and had surgery for PS at 4 weeks after 3 days in a row of me going to the doctor, 3 days in hospital and then still saying it was a virus!!!  Long story short he had his op but still has some ongoing issues.  I have joined other groups and it is clear to see a lot more research needs to be done for PS.  The whole “normally happens in first born sons and everything returns to normal after 48 hours” is just not true in all cases!!!

F M son 2014
My poor boy has not been able to feed for over a week due to “reflux”, the hospital said.  I had doubts and have had him here 3 times (currently back in as we speak).  Finally after him losing 9 oz in 3 days, they have listened to me which I tried to get them to do the first time I brought him in – which was a week today.  I have said all along I thought he has pyloric stenosis but they brushed it off as reflux.  Brought him back today as no improvement with gaviscon and ranitidine.  They did an ultra sound and what does he have?  Pyloric stenosis like I said 1 million times.  My poor boy has been ill and sick after every feed, massive amounts and then made constipated with gaviscon and it’s not even f***ing reflux.
I am so angry it took 5 minutes to diagnose today and should have been done last week but they fobbed me off.  Now having to go to another hospital so he can have surgery a s a p to have the issue fixed.  Can’t believe they let it go on so long without listening to me and checking for this earlier, my poor boy has been through hell because they messed up and he has been made worse because they didn’t believe me.  Upset, angry, and relieved we finally have it confirmed.
Ladies, if you think something is wrong don’t give up, and keep pushing them… if I hadn’t we would have been sent away [for another week] and he would then have been treated for cow’s milk allergy, and God knows what could have happened because he hasn’t been getting anything from his feeds.
Can’t believe they wouldn’t listen to me and I knew what was wrong.

J S
arrogant doc4I had pylorics, so did my son and 16 other family members; unfortunately one did pass away but that was back in the 50’s.  And doctors are still saying it’s not hereditary.  lol  I think our family has proved them somewhat wrong. lol
Total fools!  The thing is, it was on my side and my partner’s side, as I said, 16 members, but [my son was] still diagnosed with gastro reflux for 3 months even with this history.  Total joke!
16 members across the family had PS, and we saw the symptoms straight away.  This stems over 55 years: my son is the most recent case and I was the 10th member but female, so not diagnosed as soon as usual.

L S – son born in 2007
My little boy had pyloric stenosis.  He was diagnosed at five weeks after a hideous time of not being taken seriously by the doctors.  I went back for the third time and refused to move unless they saw us again and self-diagnosed.  Finally they took me seriously.  Literally moments after his op he was a different baby.
He’s seven and a half.  Very pleased, yes.  It’s taken this long to talk about it though.

E T – son born 2014
My son is 2½ month old and 2 weeks ago we noticed small changes in his behavior.  Not as many poopy diapers, acting colicky, constantly hungry like [he was in] one really big growth spurt.  He was never a spit up baby, but a week ago he started projectile vomiting.  We went to his pediatrician twice, only to be told he had a very nasty virus.  We were told to give him clear fluids for 24 hours and the virus should work itself out of his system.  He then started vomiting blood… lots of blood-filled vomit.  We went to an after-hours clinic and they sent us to the hospital because he was dehydrated.  We went to the hospital, they did a blood panel, x-ray, administered fluids via IV, and sent us home with zofran.  The next morning he was still vomiting blood so we went back to the hospital and they took an MRI and discovered he had PS and referred us to a children’s hospital for the operation to fix it.  During the operation I cried, but I cried more after the operation since he had to wait 8 hours before he could eat anything and could only have ½ ounce and was crying uncontrollably because of hunger pains.  My heart hurt for him.  He is doing much better, being 4 days after his surgery!

Doctor woman makes a warningS Y – self born in 1985 (& father 1953)
I was born in Dec 1985 and by the second day I was projectile vomiting every time I was fed, and sometimes after my parents thought I was done I would go again for another round.  My parents both knew that something was wrong with me, my dad remembered his parents telling him what happened with him (they thought he had PS but it turned out to be a tumor bouncing up and down in his stomach)… My parents went to my pediatrician and he said that there was nothing to worry about – all babies throw up.  Then they went through 3 other doctors and they got the same story and a few even told them that the likelihood that I had PS was slim to none, this went on until I was 15 days old and my mom and dad decided to take me back to the hospital I was born at, went into the ER and found the doctor that saved my life.  He was a pediatric emergency surgeon.  I was in surgery within a few hours of being admitted so that they could get all the tests and get me prepped for surgery.  By this time I had lost 1 lb, which was huge considering I was only 6 lb 5 oz.  I was released just in time for my very first Christmas.
After my parents recuperated from my ordeal they talked to that surgeon and found out that if they were to choose to have another child that child most likely would have the same thing I had.  They talked and couldn’t go through it again.
My dad had his surgery in 1953…

The awful stories above would be understandable if PS were a rare condition.  But it is not.  The incidence varies a bit, but in developed countries ranges between 2 and 5 in every 1000.  This means most of us would know several people who carry a PS story.

It must also be recognised that PS quite often (but far from always) takes a week or more to become “full blown” and able to be clearly diagnosed by touch, x-ray and ultrasound scan.  And the health system as well as parents would not take kindly to an unnecessary surgical operation on a baby.

Yet the message is clear from the above stories and hundreds like it on the web.  It is also a simple message that should not be impossible to learn and remember, especially by the highest IQ endowed people in society.

  • Arrogant shirt1Too many parents get “attitude” from their GP and pediatrician: patronising condescension and dismissiveness, even when the parents have done their homework, have PS in their family or personal genes, and find their baby’s weight loss has become serious.
  • One would hope it is true that today’s trainee doctors are being taught more about people skills.
  • Many doctors seem to wait far too long before ordering tests, resulting in too many PS babies being near death and possibly damaged for life by hunger and dehydration.
  • From the countless available stories it seems few doctors advise seeking a second opinion or refer a baby to somebody more knowledgeable.
  • One would hope that those who are humble and self-aware do one of the above – with the result of no traumatised and angry parents writing to a forum site!

This blog has several posts on the treatment of PS by medication with atropine or Ranitidine, a non-invasive option that is 100% safe for PS babies born full-term and older than 2 weeks and is standard practice in several developed (but non-English speaking) countries.  Find these posts using the “Categories” box at the top right.

Parents who strongly suspect their newborn has PS have very good reason to insist on their doctor giving them respect, time, and clear explanations of their advice.  It may be helpful for them to take a supportive person along to the consultation.

Understanding infant pyloric stenosis (Conclusions)

In the previous few posts I have overviewed and “translated” in some detail the major and professional medical section of a small book recently published by Dr Ian M Rogers and myself, with the descriptive title, The consequence and cause of pyloric stenosis of infancy: Two personal stories.

The reason for this series of posts?

At least some of the survivors of infant pyloric stenosis (“PS”) and their parents will be interested to know and understand this condition better, as we keep being told that “we still don’t know what causes it”.

Let’s face it, “knowledge is power”, and how many parents don’t wish they knew more about PS when they are suddenly and horribly faced with it?  What causes this?  What can and should I do?  How do I look after my sick baby when I feel the doctor fobs me off? Can my beautiful baby avoid surgery for this? What are the possible short- and long-term side effects?

Although I have never had a child with PS, I had it as a baby and wish I had known what I know now much, much earlier.  In the past this was virtually impossible, but the power of the web has changed this completely.

True, there are still some areas that are not fully understood, but it’s also true that drawing on a lifetime’s work with PS, the retired professor Ian Rogers is able to give us a clear and (it seems to me) compelling explanation of what does in fact cause PS.  The fact that he has written his part of the book using a lot of medical language must not keep his knowledgeable and compelling explanations and rich insights from those of us most affected by PS!

In this the last post of this series, I look at the last three of 6 questions which are often regarded as summing up the mysteries of PS, but which can in fact be answered in the light of Ian Rogers’ discussion of the discoveries that together explain PS.

4   Why is PS more frequent in the first born?

first-time-mom2First-born babies are cared for by cared for by first-time mothers.  PS babies are hungry and vigorous, ravenously hungry (at first), and unusually, the condition does not leave them feeling nauseous but immediately voraciously hungry for more.  Understandably but tragically, this gives the stomach and the acid-caused work-enlarged pyloric muscle no rest; an experienced mother will be more inclined to give feeding a break.

Mention is made of a 1962 article by Dr N M Jacoby, who compared two groups of 100 babies treated medically and surgically for PS, with one baby (only) of each group dying.  Of the medically treated children, Jacoby stresses the importance of (1) relative under-feeding, (2) the drug involved (atropine) being carefully used in relation to body weight, and (3) regular stomach washouts.  Two of these cautions can be linked to the hyper-acidity theory.

It is often mentioned that PS occurs far less frequently in under-developed countries: this also fits the theory, although more factors may be involved.

5   Why does pyloromyotomy, and not gastro-enterostomy, cause the tumour to disappear?

There are reports about 52 year old patients whose pylorus was found to be still enlarged after a gastro-enterostomy surgery in infancy to overcome PS; after pyloromyotomy the pylorus returns to its normal condition after several weeks.

Unlike gastro-enterostomy, pyloromyotomy disables the muscle ring and widens the opening temporarily, which breaks the cycle of its becoming enlarged by overwork.  Gastro-enterostomy was used before pyloromyotomy was discovered and is still used occasionally: it by-passes the pylorus which removes the immediate feeding problem but the pylorus continues to be over-stimulated by an over-acidic stomach.

6   Why does PS present at around 3-4 weeks of age?

Dr Rogers mentions two possible reasons.

It could take some weeks for higher than usual acidity to enlarge the pylorus sufficiently to make a clear diagnosis of PS possible.  But if this were so, we could expect other problems caused by hyper-acidity to arise at this time – and they don’t.

More likely is that the switching mechanism between alkalinity and acidity is more immature than usual during the first weeks.

In evolutionary terms, the benefits of the normal high acidity in a baby during the first weeks (incl. guarding against microbial attack as mentioned earlier) outweigh the negative of a few babies with hyper-acidity having problems!

Other lines of enquiry

1   Genetics

genetics1Genetic studies have shown that more than one gene is involved in PS, and that the condition is multifactorial: it can be caused by any one or more of several factors.  This is confirmed by studies of identical twins who are more likely to share PS than non-identical twins or siblings but far from always.

It has been found that PS infants tend to have higher birth weight, and (anecdotally) they may also become more athletic!

2   Chemistry of the pylorus

Prof. Rogers gives three reasons why the reports of chemical abnormalities of the pyloric muscle (the presence of growth factors and the absence of other chemicals) are theoretically attractive but do not stand up to scrutiny.

3   Infection

The analysis of swabs taken from the nose and throat of babies have shown no abnormality in PS babies.

A previous post has mentioned links between hyperacidity in PS babies and in adults infected with a stomach bug, Helicobacter pylorus, which can trigger gastro-duodenal ulcers.  But several studies of PS infants have not discovered any H. pylorus infection.

Dr Rogers remarks how strange it is that none of these studies links the hyperacidity triggered by a H pylorus infection with the hyperacidity that is part of PS.  Strange indeed!

Conclusion

Dr Rogers sums up:

Constitutional hyperacidity coupled with developmental hyperacidity begets pyloric contractions which begets work hypertrophy which begets IHPS.  IHPS begets further hyperacidity, and so on.
Maternal anxiety in the novice mother means that the hungry but vomiting baby is frequently fed with more pyloric contractions and more work hypertrophy – and a bigger tumour.

Wryly, he adds that it seems there is nothing new under the sun, and that “we are almost back where we started”.  In 1921 Dr J Thompson already proposed that the pylorus spasming and overdeveloping through over-work were the cause of PS, and still earlier, in 1903 Dr W Freund had suggested that excess hydrochloric acidity was a key factor.

The future

Dr Rogers urges that his theory is “perfectly testable”.

  • It is well known that PS babies’ serious loss of acid through their vomiting must be remedied before surgery, as their alkalosis (excess alkaline in the body) causes dangerous hazards during and after the operation.
  • ranitidine01Adults similarly affected by vomiting and acid loss are quickly and effectively treated with drugs to reduce the excessive release of acid.
  • Successful medical treatment of babies with PS is regularly reported: intravenous atropine is used to rapidly reduce the size of the pyloric tumour while the malnutrition and chemical imbalance are corrected.
  • Surgical treatment is now more prompt with a drug (cimetidine) that rapidly corrects alkalosis.  The author mentions an as yet unpublished report that this drug when given to mild cases made surgery unnecessary in 16 of 17 cases.
  • Another powerful acid blocking drug, Ranitidine, has also proved to be very effective in avoiding surgery when PS is recognized early; it should also be useful when surgery is not safe or accessible.  Dr Rogers then briefly outlines several other avenues of treating PS safely and medically.

He concludes his comments on considering non-surgical treatment by adding:

Such a pre-operative strategy with babies with IHPS is long overdue. It should not come as a surprise if we find that such temporary treatment promotes a lasting cure. 

Understanding infant pyloric stenosis (3)

Infant pyloric stenosis (“PS”) is a condition that may affect babies during their first months, and it is regarded as the most common life-threatening affliction of early infancy, affecting between 3 and 5 babies in every 1,000 in “Western” countries.  It will usually starve the infant to death unless treated, either by surgery or medically.

ponderStrangely, we are usually told that the cause of this malady is unknown.  But is it really?

In the previous 4 posts I have summarised the main points made by Dr Ian M Rogers in a recently published small book, The consequence and cause of pyloric stenosis of infancy: Two personal stories.  Ian is a retired surgeon and professor of pediatric surgery, and his book enlarges on his argument in several medical articles which are based on a lifetime of observation, research and experience with PS.

Dr Rogers believes that the cause of PS is not a mystery, and that surgery is often not needed to remedy it.

This post continues a series and overviews the first three of Ian Rogers’ six conclusions, all based on the discoveries and facts which he has presented.  I have outlined Dr Rogers’ book in lay terms to make it accessible and understandable for non-medically trained parents, PS survivors and others interested in this subject.

Having set out the information which I have covered (selectively) in the previous posts, Ian Rogers comments that in 1970 his career put a halt to his work on PS, but that he returned to it in the 1990s, when several basic questions called for answers he believed could be given.

1   What makes some babies develop IHPS when normal babies do not?

Without entering into the more complex areas of the medical science that Dr Ian visits and must be considered to answer this question more fully, “the bottom line” is that inherited hyperacidity would be an answer to this question that fits what we know, although some details remain to be discovered.

Dr Rogers mentions several other known facts that add support to this answer, even explaining why some babies develop PS well before the usual 3 weeks to 3 months “window”, and the well-known link between PS and “O” blood.  Of course heredity as a key factor also explains the frequency of PS in certain families.

Babies with normal gastric acid secretion do not overwork the pylorus despite the raised levels of the hormone gastrin during the first post-natal weeks.

2   Why do male babies predominate?

Male dominance1The 4-5:1 male dominance of PS runs parallel to the incidence of gastric ulcers, well known to be linked with hyperacidity, and males having a more active parietal cell mass (PCM), the acid secreting part of the stomach.

Male babies have also been shown to secrete almost twice as much gastric acid as girls during their first 10 days.

3   Why self-cure with the passage of time?

Release of the hormone gastrin is at very high levels after birth, normally peaks at between 10 and 17 days, and then falls to the equivalent of adult levels at 3 – 4 months.  In normal babies raised acid levels continue for about 4 weeks, thus protecting the baby from microbe attack.  But in PS infants, while gastrin release starts to fall, acidity continues to rise and the sensing that normally allows the stomach to switch between acidity and alkalinity is overwhelmed.

Pyloromyotomy (the operation to remedy PS) disables the pyloric ring, immediately blocking its overwork and allowing normal peristalsis and acidity and alkalinity to return.

The traditional medical treatment of PS in babies and adults was based on the belief that the muscle was not hypertrophied (enlarged through overwork) but spasming, and cutting the vagus nerve and administering the anti-spasmodic drug atropine, combined with regular stomach washouts were used to reduce acidity and spasms.

Effectively, these measures (together with the relief of dehydration and the restoration of healthy blood chemistry) allow babies to survive until they are old enough to outlive the natural raised acidity levels of the first post-natal weeks.

Next post –
Dr Ian Rogers answers three other “obvious” questions about the character and “unknown” cause of PS.

Understanding infant pyloric stenosis (1)

PS book coverIn the previous two posts I have “translated” several parts of the larger share of the small book I have co-written with the emeritus Prof. Dr Ian Rogers, titled  The consequence and cause of pyloric stenosis of infancy: Two personal stories.  My co-author has addressed his section to the medical profession but gives much information that is of no small interest to many of us who have been touched by infant pyloric stenosis (“PS”).  It has certainly helped me to understand much more of the what, how and why of this rather enigmatic condition, something I have longed for ever since I became aware of the strange markings on my belly!

In this post I want to revisit (in more detail than my initial post) the first pages of Dr Rogers’ section, in which he writes about the function of the hormone gastrin in digestion.  He mentions that the Glasgow academics under whom he trained specialised in the physiology (working) of the stomach and intestines, clearly because duodenal ulcers were a major cause of illness at the time.

When Ian Rogers finished his surgical training in the early 1970s, it was known that –

  • Stomach01hyper- (or over-) acidity could be constitutional or acquired, and was the major cause of duodenal ulcers;
  • hydrochloric acid release is triggered by the vagus nerve and/or the hormone gastrin;
  • the upper (proximal or first) part of the stomach secretes the hydrochloric acid;
  • gastrin occurs in the mucosa (lining) of the distal (or end) part of the stomach;
  • food in the stomach causes chemical change which releases gastrin into the bloodstream and this as well as stomach activity triggers the release of acid into the stomach contents;
  • acid is essential to digestion as well as sterilising food;
  • normally, gastrin controls acidity, not allowing its level to become too high or low;
  • duodenal ulcers were thought to be caused by hyper-acidity, most common in anxious men; often this could be controlled by cutting the vagus nerve, and otherwise by a gastro-enterostomy (which involves more radical surgery).

It has only become known in more recent decades that Helicobacter pylori bacteria are also involved in hyper-acidity and cause some 80% of duodenal ulcers, and also that antibiotic treatment and/or suppression of vagus nerve activity with atropine could control acidity, almost eliminating the need for surgery to relieve gastric ulcers.

Although both men and women have rates of H. pylori infection that rise during their lives and are almost similar at age 70, the acid-secreting stomach cells are more numerous in adult men than women.

Thus there are some interesting parallels between patients with PS of infancy and those with gastric ulcers in adulthood –

  • a 5:1 male preponderance;
  • high acidity;
  • a good appetite;
  • other family members affected.

The above facts represent what was commonly known in the early 1970s when Ian Rogers completed his surgical training in London and returned to Glasgow.

In later pages Dr Rogers continues by describing the “clinical (observable) signs” of PS – as they give many clues to its cause.

  1. Peptic ulcer3it arises very early in a baby’s life;
  2. it affects many more boys than girls;
  3. it cures itself in time if the patient can be treated medically;
  4. it can have a strong family link;
  5. if the pyloric tumour is split surgically the swelling soon disappears;
  6. the swelling does not disappear if the blockage is surgically by-passed;
  7. the high acidity in the stomach cannot be explained as accumulation due to the pylorus being closed;
  8. survivors continue to secrete higher than usual acid;
  9. PS babies have a ravenous appetite;
  10. they are often first-born babies;
  11. some PS babies are found to have a superficial duodenal ulcer.

Despite these clear pointers, the medical community still finds the cause of PS a mystery more than 120 years after it was first fully described.

Dr Rogers then refers to the significant 1921 paper of the Scottish Dr John Thompson who observed that –

  • the “pyloric tumour” is in fact over-developed muscle;
  • PS is self-limiting (see #3 above);
  • PS can be managed with small feeds, daily stomach wash-outs, and if feeding has been unsuitable, with IV feeding;
  • there are 3 kinds of PS: the acute (with sudden and violent symptoms), ordinary, and very mild.

Thompson accepted the theory that PS is caused by a functional abnormality, referring to an 18th century anatomist who found that all muscles develop with use, but involuntary muscles (ones like the pylorus over which we have no control) more than others.  However, this theory was not further explored, even though PS occurs also in other mammals.

Dr Rogers has now laid the foundations for his conclusions about the cause of PS, and in the next post we’ll see how what he has been mentioned in these three posts applies to gastrin, hyper-acidity and PS in the newborn.