Category Archives: Associated conditions

Brain damage after Infant Pyloric Stenosis

Patient & doctor03“If my baby has had pyloric stenosis (“PS”) and had surgery to remedy it, will there be any longer term after-effects?”

Most medical professionals will brush this parent concern away: “No, once your baby recovers, you and your little one can put this episode completely behind you!”

If only this were true…

We must all understand this:

Surgeons’ competence and interest are in the immediate surgical “fix”, paediatricians may follow the infant’s story for a few months, but very little has been done to follow these “Py-children’s” stories over 10, 20 or more years.

However, the very few studies that have been done have identified several possible hazards. This blogsite has dealt with most of the most significant ones:

  • continuing gastro-intestinal (“GI”) instability presenting as GERD or reflux, as IBS, or as a tendency to vomit (in over 50% of PS survivors as one study found) – all due to the high gastric acidity levels which helped trigger the PS in infancy and remains high lifelong in many or even most PS survivors;
  • in a substantial minority (estimated 5-15%) of cases adhesions develop as a result of the PS surgery and will cause pain and distress to a significant extent;
  • a minority of PS survivors take many years to come to terms psychologically and emotionally with their surgical scar – acknowledged as a lifesaver but growing more obvious with the years, in which they had no say, and which some parents decline to talk about;
  • a very small number of older PS survivors have gone public about struggling with symptoms of PTSD diagnosed as resulting from the rigours of older style infant surgery, when many surgeons believed there was no need for anesthesia (“babies don’t have a memory”) and when long maternal separation was part of many hospital routines.
  • There is at least one other area of possibly long-term damage that can come with PS and infant surgery.  This is damage to the brain from starvation resulting from delayed diagnosis of PS, and/or repeated anesthesia, especially when the PS came with another hazardous condition of infancy.

This is the subject of this post.

Here follow some of the facts that have been established by medical research and reporting. I have included a link for every point noted but have collected many more reports. Readers are free to ask for my full reference list; contact me by email via the “About me” or “About this blog” tabs at the top of the page.

Fact 1:  Single use anesthesia probably causes no long-term damage

 A 2016 report clearly established that single and simple surgery for hernia repair resulted in no brain damage at 36 and 48 months.  This report has been widely quoted to reassure worried parents.  Lengthy or repeated anesthesia for more complex procedures was found to be damaging.

The anesthesia used in the typical pyloromyotomy (to stop PS) is part of one fairly quick and simple surgical procedure.  However, almost all PS surgery occurs in the first weeks of an infant’s life.  I have not found any study of the effect of a single short anesthetic episode on the brain of a very young infant.

So repeated surgery using general anaesthetic certainly does affect the infant brain.  Prevailing opinion is that single use does not affect a baby, but the studies “establishing” this did not involve newborns but children at 3 and 4 years old!  In matters of life and death general anaesthesia is a known and acceptable hazard.  But contrary to the prevailing opinion of the medical work, most PS babies can quite easily be treated with medication. And it is in many countries. More targeted studies are needed.

Fact 2:  PS-related malnutrition does have long-term consequences

 

A baby’s PS episode can easily result in significant starvation, as prompt and accurate diagnosis is sometimes difficult and sometimes suffers from negligence, lack of the required skills or unnecessary delay.  A baby is developing rapidly and is wired to need regular feeding: this is especially true of the human brain.

Starvation and resulting malnourishment are very real hazards with PS, and several studies including an Australian one published in 2018 have shown that PS infants and children have significantly higher levels of brain damage than control groups.  The cognitive (thinking, reasoning, remembering, imagining, or learning words), receptive language (the ability to understand information), and motor skills (the precise control of muscles in order to perform a specific act, whether in the classroom, in sports, or personal clumsiness) scores are most clearly affected.

I cannot but link these findings with the observation that several of my skills in these areas are clearly below those of my four siblings. This is not to deny that a PS survivor can never be a top athlete, accountant or academic – all I observe here can be questioned… but my lifelong observations remain!

A 1973 Scandinavian study found links between PS-caused starvation and below normal social and communication skills and described them as pervasive developmental disorders (PDD). It found that parents could recognise the symptoms before the age of 3 years.

But it added that if recognised early and given special remedial support, children with these difficulties will usually overcome the worst of their handicaps.

Fact 3:  There is a possible link between infant PS and Autism Spectrum Disorders (ASD).

A large 2009 study conducted in Western Australia over 15 years found that PS babies did not have a higher incidence of ASD than a control group, whereas babies with many other birth defects did.  But it mentioned that several other studies had found that infant PS also occurred significantly more often in children who were also diagnosed with ASD.

autism01Another large study was reported in 2009: the Autism Spectrum Disorders Report, which is also readily available online.  Like the WA Report, it found links between ASD and many categories of birth disorders, but not with PS.

As with the effects of anesthesia on the fast developing brain, the medical world is not agreed on this subject.  This clearly is one of the areas in which more research (read: more funds) is needed – but of course there are many even more serious conditions that are crying out for more interest and funds for research. It has not been established that there is a common cause of IHPS and ASD (such as a higher incidence of defective genes and/or environmental factors in these children) or that one condition actually triggers the other.

Conclusion:  Greater awareness and more studies are very much needed

My reading list on this subject area reflects a telling lack of clarity and agreement, and this is far from the only subject area of infant PS on which studies have arrived at conflicting and sometimes troubling conclusions.

In 2013, the Journal of Neonatal Surgery published a widely disseminated overview article marking the centenary of German doctor Conrad Ramstedt’s publication of his discovery of and success with the PS surgical technique that quickly became and has remained the standard way of dealing with infant PS.  Dr Raveenthiran Venkatachalam’s remark (as “Athena”) on the subject of this post is worth including here:

Dramatic relief of symptoms following pyloromyotomy has lured many pediatric surgeons to declare a cure after one or two follow-up visits. Long term results are generally presumed to be good rather than proved by evidences. Two recent papers question this com-mon assumption. Using Baylay scales, Walker et al [21] assessed neurological development of 52 infants with HPS and 211 healthy infants at 1 year of age. Cognitive, receptive language and motor scores were significantly lower in HPS infants than in controls. It is unclear whether this adverse outcome is attributable to the dis-ease process or to anesthesia administered during surgery. Although further studies are needed to conclude whether this difference is of any practical significance, the findings of this study are certainly a source of concern. Saps and Bonilla [22], in a case-control study, studied 100 HPS patients and 91 controls in their late childhood. The mean follow-up period was 7.2 years. Nearly 25% of those who underwent pyloromyotomy in infancy developed chronic abdominal pain at a later age; while only 6% of the controls were so. Irritable bowel syndrome, functional dyspepsia and functional abdominal pain were more common in HPS group than in control group. Athena considers these two studies as eye-openers necessitating long-term follow-up of HPS infants.

What most doctors seem to remember about IHPS from their med school classes and textbooks is necessarily basic and general.  If more is said, it’ll be even more easily forgotten.  Medical science is such a huge body of information.  Although PS is quite common, most medical professionals will see very few if any cases.

We can be thankful that in general PS and much infant surgery today have no major ongoing consequences. If there were any life-threatening conditions or obvious disabilities, the medical world would know about this and PS parents would not be silent.

One of the most traumatic events new parents can experience is finding their baby vomitting uncontrollably, forcing them to submit their newborn to life-saving surgery.  We can be thankful that almost all babies and their parents nowadays survive pyloric stenosis (“PS”).

Once that crisis is behind them parents’ next question is inevitably: will this condition or surgery affect our child’s future?  Reliable and informative answers to this question remain challenging and usually unreliable – but few doctors, parents, and survivors seem to be aware of this.

Because of this blogs like this one and social media discussions will continue to advocate for far greater awareness of the facts.

See also my post: Is a sick and starving baby affected for life?

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.)

Pyloric stenosis in its adult forms (2)

My life’s work as a church pastor has made me aware of many of the common medical conditions, as I have supported people with all kinds of cancers, many heart, lung and “lower abdominal” conditions… ah, I don’t want to bore you with the long list!

Troublesome and also serious medical conditions often occur without warning and they can totally surprise the sufferer.  When I needed two kinds of eye surgery some years ago, I already knew a lot about the cause, the procedure, and the likely outcome.  My father had already charted my path, as had several people I pastored. 112

But most non-medical people have not had not worked with as many sick people as I have, and many health problems don’t have a clear family / genetic factor.  For these folk especially it can be very frustrating to be dealing with something life-affecting they know nothing about; quite likely they’d never heard of their condition before.

Adult pyloric stenosis (“PS”) is often like that: it is far rarer than the infant form (which itself is not exactly everyday), and it’s not always obviously linked with the family’s genes or the patient’s lifestyle.

A 1998 medical report deals with such a case.  For the details, please use the link to find and read the full article.  All three medical journal reports mentioned in this post are commendably available free and in full on the web! Points of general interest in this first article include –

  • It concerns a (then) 47 year old Caucasian woman, a rare thing as PS in both its infant and adult forms affects about 4 times as many males as females.
  • It seems this PS case was regarded as “primary” – without an obvious cause.  Several symptoms started only some 2 – 3 months before her surgery, and although she had suffered from dyspepsia and occasional vomitting she had none of the common precursors to adult PS: infant PS, adhesions, a gastric ulcer or cancer (which would have made this a “secondary PS”).
  • Examinations and imaging before her surgery showed little abnormality apart from reduced stomach emptying, but post-op pathology showed abnormal cell and muscle structure in the pyloric region.
  • The distal gastrectomy surgery involved removing the lower part of the stomach and the pylorus and attaching the stomach to the duodenum.
  • The report tells us there were no continuing symptoms, although it is not hard to find less concise and straightforward comments from other patients elsewhere!  This patient had severe surgery that should be expected to change digestive functioning.

Several other matters are also worth mentioning –

  • It is mentioned at several points that the cause of primary adult PS is little understood, and it is claimed that there are no statistics on how frequently it occurs.
  • Several surgical techniques are used to manage adult PS, all of which are more radical and affect digestion far more than Ramstedt’s operation which is the usual procedure used to remedy infant PS.

In light of this, I was somewhat intrigued to discover that a 1965 article (also freely available in full) contradicted several of the most significant assertions made by the previous report!  Specifically, Dr Milo W Keynes claimed that –

  • “Primary”, “uncomplicated” or “simple” PS in adults seems to have a genetic base.
  • It is far more common than is often claimed – but adult PS patients and those with a “simple” PS are less likely to seek treatment than the parents of a very sick baby.
  • “Secondary” PS occurs more often than the “primary” form, although reports of the incidence vary greatly from 10:1 to 3:1.
  • Many of the patients had struggled with symptoms for many years (e.g., 12 years), and sometimes from childhood.
  • There is a long list of symptoms, and these vary greatly.
  • Most patients were older, but the documented cases ranged from age 14 to 85.
  • Adult PS occurs in about 2 males to every female – different from the infant form.
  • It is claimed that “simple” PS can usually be traced to the patient’s infancy.
  • It is also claimed that it can be managed without major surgery: by the same muscle-splitting Ramstedt operation as done with babies.
  • However, at the end of the article it is stated that the preferred management of adult PS is by one of the much more severe surgical techniques, so that underlying disease such as a cancer or an ulcer can be dealt with if necessary and because the Ramstedt procedure does not always relieve the obstruction.  This leaves the patient and surgeon with a choice between gastro-duodenostomy, gastroenterostomy, gastro-jejunostomy, partial gastrectomy and pyloroplasty.

Another article in the same medical journal issue as the previous one reported on a study of 100 adult PS patients, all of whom had their diagnosis confirmed at surgery.  All but 3 of these were found to have a cancer or peptic ulcer(s): of these 3, one had Hodgkin’s disease, leaving the other two with a “simple” adult hypertrophic (enlarged) PS. Details in this report which are likely to be of interest to the reader of this blog include –

  • Although the symptoms of adult PS vary greatly, the core symptoms are common and quite unmistakable: vomiting containing food eaten a day or two earlier, loss of appetite, weight loss, and gastric splash (an audible sloshing sound in the stomach when rocked more than 3 hours after a meal).
  • A duodenal ulcer can cause obstruction without vomiting.

These two reviews of informative articles conclude a series of three on adult PS.

Interested readers might use the “Categories” box (top right of the “page”) to find other posts on this subject. DiscussionForum

Facebook members might join one or more of the largest PS Groups on this social web forum, as some 12 adult PS sufferers have shared something of their varied and common experience of this condition.  Several of these Groups are “closed”: all can be found via the Facebook Search box, but to encourage candid sharing only those who join can see their contents.

Pyloric stenosis in its adult forms (1)

Back in the 1950s I once overheard my parents talking about my oldest (and long deceased) uncle Fred having a stomach ulcer and an operation to deal with this.  I well remember being transfixed and “all ears”, but having been stonewalled so often, I dared not ask my parents the question on my mind.  At this time I would have been about 10 years old, and I had worked out that my prominent and hated belly scar was from an operation I’d had as a baby as I’d developed a blocked stomach (pyloric stenosis, “PS”).  I was keen to know whether my uncle Fred would be sporting a scar just like his young namesake.

When I got to see my uncle in swim-mode some 20 years later I was disappointed: his scar was small and almost invisible, far less disfiguring than mine.  Compared with infant surgery in the 1940s, abdominal surgery on adults and 20 years later was quite tidy.

Gastric ulcer01What I’ve discovered only recently is that infant PS and adult gastric ulcers and PS are often related.  All three conditions and several others have been linked with a high output of gastric acid and this is often a family trait.  So my connection with my namesake uncle (and hero) likely went further than our family realised!

The previous post sets out some of the important similarities and differences between the infant and adult forms of PS.  This post will outline two medical articles on adult PS.

The first report was published in 2010 and deals with the case of a 71 year old male who was diagnosed with PS.  The interested reader is encouraged to use the link to read the full article which has been kindly made available to the public.

In brief, it notes that adult PS is found in two forms.  It is often caused by other conditions: a long history of a mild form of the early version, an earlier stomach problem such as a gastric ulcer, or cancer.  Sometimes, as in this case, no underlying disease was found.  This 71 year old was unaware of any such factors, yet tests and surgery proved this man did indeed have a PS.  His symptoms of “abdominal distension, nausea, and vomiting” had been noticed for (just) 3 months.

It is noted that both forms of PS occur far more often in males than in females, and that they often recur in certain families.

The aim of this report is to be applauded: to raise the awareness of both forms of PS within the medical community, and especially the various ways it can appear in adults.

For those like myself and (I expect) most of our readers, the article’s value is also in its acknowledgement that the infant and adult forms of PS are related, and that the infant form does not necessarily end with a simple surgery.  Far from it, as many of us have well come to know!

The second report can thankfully also be read in full by those interested in more detail.  It outlines the very large decline in the number of adult PS sufferers needing surgery.  I have already posted about this.

downward_graph_smallThis study was undertaken by a York (UK) body gathering data on peptic ulcer disease, and based on the statistics from the 3 kinds of stomach surgery done on 4,178 adults and how often adult PS was found during these procedures.  The study ran from 1929 to 1997 and included people born between 1889 and 1959.  It found that the age at surgery fell during these years, that PS was found in 17% of the earliest patients, and just 3% in the last group, and that gastric surgery rates fell markedly (although not uniformly) during the almost 70 years under the lens.

These results show very clearly the effectiveness of modern medical treatment for stomach conditions and introduced in 1977; this includes acid-suppressing medication which reduces the formation of gastric ulcers and resultant scarring of the stomach wall and pylorus.  However, the reduction of PS numbers was even greater than the falling numbers of stomach surgery, suggesting that a number of factors are at work, including diet, medication, and the management of upper bowel disease.

Once again, the great majority of readers will be less interested in the medical messages of the report than in what it might mean for interested lay people.  What do these figures mean for you and me whose history includes PS?

  • yay1People who have had infant PS are less likely to have serious long-term effects than they might have expected some 40 years ago or earlier, thanks to better diet options and medication.
    The fact that today we can read much more about the long-term problems experienced after infant PS is a reflection of our access to the media, not of rising numbers.
  • People who have had infant PS are ever less likely to need further surgery for ongoing problems including a renewed stomach blockage. We need to be aware that for some of us these problems are significant, testing, and often interminable.
    The report states what many have found (as I have read), that surgical responses to such problems are not only severe but also likely to be ineffective in the longer term.
  • Peptic ulcer disease continues today but it now very rarely results in PS.

So – generally good news for the PS-aware community!  My next post will review several other medical articles on adult PS.

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.

Understanding infant pyloric stenosis (2)

This post is the 4th in a straight series in which I have overviewed the section of a book in which the retired Scottish professor of paediatrics, Dr Ian Rogers, recounts what he has learnt from a lifetime of the observation and study of infant pyloric stenosis (“IPS”).

How digestion works

Dr Rogers describes the process of digestion that occurs in the stomach.  He describes the stomach as having two parts, each with a distinct structure and function, and the stomach’s three stage role in digestion.  During these stages the pylorus contracts and relaxes, early to allow fine and easily processed food to pass, after which the stomach continues grinding and breaking down the coarser food to chyme (or pulp) before the pylorus allows it to pass.  He concludes that the contraction of the pyloric ring muscle is associated with feeding.

This raises the question: how would this process work in a baby?  Dr Rogers points out that –

  • Milk in the stomach raises alkalinity which triggers the secretion of gastrin and acid.  This in turn activates the pylorus, and if acid secretion is higher or feeding is in greater quantity and/or more frequent than usual, then the pyloric muscle is overworked and over-develops.
    IHPS causation - I Rogers
  • Artificially narrowing the pylorus in rats stimulates the development of the stomach’s acid secreting lining, thereby increasing even more the stimulation of the pylorus.
  • Studies have shown that gastrin-induced hyper-acidity is indeed a symptom of IPS.
  • In 1976 Dr J A Dodge reported that he had generated PS in 28% of 84 puppies after injecting 20 bitches with a synthetic form of gastrin; it is known that gastrin crosses a dog’s placenta and stimulates acid secretion.  Still more puppies developed PS when they were injected after birth.

Dr Rogers concludes from this that the pyloric muscle is clearly the culprit.

  • Gastroenterostomy03Disable the pylorus by splitting the muscle and its enlargement soon disappears; bypass it (which the gastroenterostomy operation does) and the enlargement remains.
  • This also shows that although the gastric hyperacidity may be hereditary, the cause of IPS is not a hereditary tendency for the pylorus to be enlarged.
  • The only cause of pylorus enlargement that has been found is repeated contraction.
  • Pathological study has found no abnormality in the tumour tissue.
  • The erythromycin phenomenon also confirms this conclusion.  Erythromycin is a macrolide antibiotic widely used to treat bacterial infections, but when taken during pregnancy or by a newborn it has been found to cause a 7-fold increase in the incidence of IPS.  This group of antibiotics works like the hormone motilin which increases stomach activity and contractions of the pylorus when the stomach should be empty after it has done its work: an empty duodenum (as with IPS) releases motilin.  Some details of the function of motilin are not yet fully understood.

Other indicators – clinical aspects

  • Diagn palp.jpgIPS is normally recognized by a doctor using the standard test meal for a baby who is vomiting profusely without bile in the vomitus:  gentle palpation (feeling while stroking) will reveal the swollen pylorus (the “pyloric olive”) in some 80% of cases, and peristaltic waves can be seen moving from left to right.
  • Adults with a duodenal ulcer caused by hyper-acidity can be treated by ranitidine (or in the past by atropine sulphate), a drug that blocks the release of acid, thus raising the alkalinity of the stomach contents.  This suggests the possibility of also managing IPS by using a drug that reduces the secretion of acid in the digestion process.
  • As mentioned, a baby who inherits a high gastric acidity added to the raised acidity levels normal in early infancy can develop uncontrolled hyperacidity which will over-work and over-develop the pylorus.  A first-time and understandably anxious mother who continually re-feeds an obviously hungry baby will amplify what happens.
  • A decline in both IPS and sudden infant death syndrome (SIDS) in babies who sleep on their back has recently been reported in Sweden.  The back sleeping position results in feeds moving with gravity, making gastric emptying easier and faster: this would also seem to confirm hyperacidity as the cause of IPS.

In the next post, we follow Dr Rogers as he applies the information in these four posts to the classic characteristics of PS in babies.

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.