Tag Archives: diagnosis

Pyloric stenosis: a mother’s story

In mid-2013 an Australian mother posted a story and some photos of her son for his 8th birthday – with his permission.  She wrote –

I am more than happy for you to use his photo and story to help raise awareness of infant pyloric stenosis (“PS”) in any way you feel will help.  …  I hope it can help others.

What this mother writes is typical of the experience of countless numbers of parents, something this blogsite has often raised and must surely challenge all providers of medical care.  Here is this mother’s story, edited slightly to form one account from her correspondence with me…

SL 2005-13My little fighter: this is my son exactly 8 years since he got out of hospital from his operation.  He was 5 weeks old when he had it done.

Getting a diagnosis

We had had two weeks of hell: my son actually stopped breathing because he was throwing up so much.  I was lucky I reacted quickly when he stopped breathing, perhaps others don’t.  Mind you, the clean up afterwards was awful, he had power chucked across the lounge room, covering everything.  He doesn’t do things by halves, that’s for sure.  That was his worst: most vomits were just 1-2 meters, still awful to deal with.  But once he was breathing we were in the car and on the way to hospital.

We live in a small industrial city of about 22,000 in Australia, and I had taken him to 4 – 5 doctors and to the hospital at home 3 times for his vomiting, and I got told the usual stuff: reflux, etc, even that I was an over-anxious young mum (I was 22, most people here are parents by the time they are 18-19) – and this was after he’d stopped breathing on me.  Unfortunately it seems most doctors either don’t know about PS or don’t want to even suggest it as a possibility.

So we made a call to the hospital in the next town over (45 minutes away) and a night nurse basically diagnosed him over the phone.  It was a huge relief to have a nurse not far away tell us what it probably was.

So it had time to sit and settle in why I was out: I had taken him to 3 private doctors and the hospital 3 times before I called an out-of-town hospital.  I couldn’t believe that so many professionals didn’t know or wouldn’t say anything except that he had reflux.

Three days later he had his operation.

At the hospital

His op was done in 2005 at the children’s hospital of one of Australia’s capital cities, and they were amazing: the nurses were so good and knowing we had little support locally they went out of their way to be even more helpful..  We had the choice of the long slice under his ribs or the key holes: thankfully the surgeon agreed key hole was better.

When we were in the children’s hospital I saw a poster made about PS by one of the nurses or a med student.  I was shocked at the number of cases: 1/200 children are born with it, yet with it being seemingly so common, it was so hard to get it diagnosed.  It affects first born males more than other babies, and yes, it can be fatal.

Since his op

SL 2005-13bThis is what is left of the scars my little man has from his operation when he was 5 weeks old.  Now he’s nearly 9 years old.  He knows he’s lucky to have tiny, tiny scars, and being able to show him what scars others have from the same operation is very helpful.

My son chose these photos himself and is more than happy to let others know what happened.  so other mummies don’t have to be scared like I was.  He is doing really well.  It took a long time for him to put on weight, and even now he’s still very skinny, but he’s hit all his growth markers on time or earlier.  Just glad someone knew what was happening with him.

SL 2005-13aYes, it is so scary.  I had never heard of PS before he was diagnosed, yet after that I found out friends had had it when they were babies.

Time seems to fly once they are over it.  My son has 3 tiny tiny scars, not even 3 mm long: no stitches were required.  One scar is near his bellybutton, the other two are on the right side of his tummy, one up towards his ribs, and one in line with his bellybutton.  If you don’t know they are there you can’t see them.  He’s very lucky in that respect.

If I hadn’t pointed out his scars he wouldn’t know they were there, but I’ve always let him know what happened and how it was handled by the surgeon.  His little brother is upset because he doesn’t have scars too.  It’s quite cute as he knows he’s identical to his big brother in every other way.

My little one now has enough scars of his own, so he’s not too bad now, just cute when he got upset because his big brother had been sick and in hospital and he wasn’t here to look after him and make him feel better.  Not bad since there is a 4 year age gap.

My boys are my world, I know they will learn things on their own, I just need to guide them in the right directions.

Mother’s reflections

I was surprised to find out how common PS is, yet not one of the 3 doctors or dozen nurses we saw mentioned it.

Where I’m from we have lots of young mums, and I would hate to think how many of them get told the same as me, that I was just an over-anxious young mum; I was 22, but what about the younger teenagers who get told it and believe it, not getting their baby the help it needs?  We get given tons of leaflets telling us how to breast feed or which baby product is better, even why we should immunise, but nothing on pyloric stenosis.

Our run was far from easy, I had to fight doctors, nurses and midwives to finally get an answer.  My son has had it easy since his operation, so we are lucky in that aspect.

After what my son went through, a friend of a friend’s son had the same symptoms and she went with her friend to the doctor’s and talked about pyloric stenosis, so he was sent for an ultrasound; otherwise his mum would have been like me, worrying and not knowing.

My son thinks it’s cool that his scars are on the internet.  I checked with him before I put them up.  I explained to him that once the photos are up everyone can see them, and all the dangers of the internet.  (The little bugger hacked my wifi to download games, so he got the danger talk.)

But he likes that he can show his scars and see what other people’s are like.

Can pyloric stenosis be a mild case?

How long is a piece of string?  The variety of ways infant pyloric stenosis (“PS”) can raise its horrible head is like answering that hoary question.

Almost two years ago I posted an attempt to answer this question,  It is not only a post still well worth reading if you are asking yourself this question.  The post had several responses which underline how frustrating and kaleidoscopic it is to answer several of the questions about PS.  Let me run through the main parts to my answer again here.

Pyloric Stenosis or pylorospasm?

Whether there is a significant difference between Pyloric Stenosis and Pylorospasm has been argued at least since the early years of the 20th century and it is still debated in medical circles.  Those favouring the distinction describe Pylorospasm as a spasming of the pylorus triggered by the nerves and resulting in the delayed emptying of the stomach – but nothing much more .  It is agreed that true PS involves the thickening and hardening of the muscle band of the pyloric ring, and when advanced enough this would not let any food pass into the duodenum, usually with fatal results.

However, others have preferred to see Pylorospasm as the beginning or a milder form of PS.  There is a difference, but it may only be clear from an ultrasound study, as this article explains and illustrates.  Another useful article may be read (although only in summary) here.

From my simple and probably simplistic description it should be clear that Pylorospasm is not serious enough to warrant surgical and perhaps not even medical intervention, and that a full PS will be fatal unless there is some form of intervention.

Baby sick2 (Medium)To add to the confusion, PS is often termed “Pylorospasm”.  I well remember that in my native Netherlands the condition I had was called “pyloruspasmus” even though it was clearly a full-grade PS!  I find that the same confusing interchangeability continues in at least several different languages today.

The bottom line is: some misbehaviour of the pylorus is mild enough to not warrant surgery.  Not all projectile vomiting is deadly, so doctors will sometimes await further developments.  This is not to be confused with the fact that many doctors are accused of belittling parents, not taking time for careful diagnostic questions and tests, and brushing off parents with talk of reflux – all this when the baby has lost significant weight and condition, and even when the parents mention the family’s history of PS.

Many cases of PS do not go to the operating room

Google “I had” together with “pyloric stenosis” and you will find articles, forum comments and posts that support the above statement.

There are many mentions of babies with what was clearly PS and whose condition was responsibly judged not to be severe enough to justify surgery.  But these people in later life continued to battle with PS-like symptoms for many years – or lifelong.  Some eventually sought surgery (usually more major than an infant pyloromyotomy) and some did as best they could by managing their food intake and distressing symptoms.

Pyloric stenosis can continue to “grumble” whether or not there has been surgery

As I have stated many times, many doctors promise parents that a safe, simple and quick operation will solve all their baby’s PS problems and that there are most unlikely to be any after-effects.

Surgery can be very successful in treating PS but it can also lead to short-term or lifelong symptoms which seem to be similar to some extent to mild PS: reflux and IBS, sensitivity to gagging and vomiting, overweight or chronic under-weight, etc.

Mild PS which is not dealt with by surgery is sometimes not treated at all or is treated with medication such as Atropine sulphate or Ranitidine.  Either of these two courses may also lead to lifelong and troublesome gastric misbehaviour.

It seems beyond the ability of medical people to know whether a particular set of borderline or mixed symptoms should be diagnosed as PS, mild PS, pylorospasm, or something else.  It also seems to be impossible to predict the future course of any case of PS, whichever way it has been treated or not treated.

A thorough and substantial research project or two would be so valuable to obtain a clearer picture of the consequences of various levels and treatments of this rather common condition in babies.

Medication can treat many pyloric stenosis cases successfully

In most “Western” countries, the medical treatment of PS with Atropine sulphate or Ranitidine has usually been given no or scant consideration.

These two drugs relax muscles and allow the pylorus to relax, reducing its swelling and allowing it to open and shut without surgical intervention, but because of their effect on all muscles they must be introduced over several days and under careful medical supervision.  This course of treatment has been used for many years by pediatric specialists in many countries, and a recent Japanese project found its use succeeded in avoiding surgery in almost every case in the trial.

Parents who could or should consider medical treatment for their baby must 1) know the symptoms of PS well, 2) have gathered strong evidence of their baby’s condition and perhaps have had previous experience with this malady, 3) have a baby who is not close to death, and 4) are willing to learn how to use the medication.  They may also need a supportive person to stand by their side as they may need to insist on being heard and helped by their doctor.  It must be realised that the safe use of medication requires early diagnosis, a baby who is not frail or newborn, and a baby whose condition allows the several days necessary for the drug to take effect.

Interested parents should use the “Categories” search box to the right of this page to find and read posts like this one on “the hushed up alternative to PS surgery.

Pyloric stenosis and reflux (GERD)

Parents of a pyloric stenosis (“PS”) baby often face one or both of two questions –

1                    Does this sick baby have PS or reflux ?

2                    My baby’s had surgery for PS but is still sicking up – is this still PS or is it reflux ?

This blogsite’s main post on telling the difference between PS and reflux (or GERD or GORD) was written 30 months ago and is one of the most often visited.  That’s two good reasons to look at the main facts again.

First, some necessary information about GERD.

GERD stands for gastro-esophageal reflux disease (the American spelling), and GORD is the English equivalent.  What is it ?

Reflux or GERD in babied ((c) Mayo Clinic)

Reflux (GERD) in babies – (c) Mayo Foundation

If the muscle ring at the lower end of the esophagus (the tube that takes our nourishment down from the throat to the stomach) is unusually relaxed, weakened, does not close when it should, or becomes herniated, the gastric (or stomach) contents are forced back up the esophagus.  But this fluid material will now have become acidified, and so it will burn and damage  the lining (the mucosa) of the esophagus.  What we feel as a result is usually described as heartburn, chest pain, regurgitation, and nausea.

Reflux can be minor or very, very unpleasant but in itself it doesn’t kill.

It is believed that in the “Western” world between 10 and 20% of people will suffer with GERD at some time or chronically.  Serious and continued GERD can cause other problems, ranging from coughs and esophagitis to ulcers, scarring and even cancer.

Almost everyone will sometimes experience passing reflux.  Depending on its seriousness, GERD can be managed with diet, antacids and other medications.  Some of GERD’s causes may require surgery.

Babies have an immature system, including their gastric tract, and for this reason most babies will “sick up” – some occasionally and some very much.  Anything that upsets a baby’s stomach is likely to affect its working, whether it’s mother’s lovely spicy dinner flavouring her breast milk, or abdominal surgery during which some strange tools and gloved fingers fiddled with its insides.

So it’s not hard to realize that almost all babies will continue to vomit and have reflux after abdominal surgery such as a pyloromyotomy.  This may happen just a few times, or during the days of post-operative recovery, or for some weeks or even months and years, or baby may (now) have a chronic problem.  Unless the baby’s immediate family are all troubled by GERD or have never known a sign of it, it may be hard to get a sense that a PS baby’s post-op GERD is either a family trait or was probably triggered by the surgery.

My reading and listening have shown me several things –

            1. Little substantial medical research has been done and published about this common problem.
            2. The very few small samples that have been reported have all found (1) that PS people have a higher rate of gastro-intestinal “conditions” – but (2) hasten to add that the number is not significant.  My logic and reading make me disagree!
            3. Pediatricians and peds ward workers will usually warn parents to expect some post-op vomiting and GERD.
            4. When GERD continues, most doctors dismiss it as resulting from the surgery.
            5. Those affected by PS, surgery and GERD should be aware of the fact that (like every part of our broken society) the medical world engages in what might be termed “power play”.  Doctors like their many kinds of authority, hate it being questioned, protect each other, work and talk together, etc.  They also dislike spending time that will affect their day and the patients they must see: issues (and thus people) will not always be adequately dealt with.

Now about pyloric stenosis…

First some major differences between GERD and PS.

          1. GERD in itself is not deadly but untreated PS usually is – and quite quickly so.
          2. Linked with this, GERD will slow down weight gain and growth but PS will usually reverse it.
          3. Although both result in vomiting, GERD is caused by the stomach’s entrance muscle ring and PS by the exit muscle.
          4. In GERD the muscle ring is not 100% effective but will usually develop; in PS the muscle ring is stimulated to over-develop, a process that can usually be stopped only by medication or surgery.
Pyloric stenosis in babies

Pyloric stenosis in babies

Many of our medical advisors (GPs and paediatricians) will not diagnose a baby with PS (or even consider this) until all other possibilities have been eliminated.  This is correct: we don’t rush to infant surgery unless it is necessary.  But this sound principle sometimes masks diagnostic ignorance or incompetence.  Countless hundreds of outraged parents have written up their traumatised stories on the internet to warn others that their baby could have died before their doctor might have diagnosed PS.  They usually tell us they went to another doctor, or (quite often, it seems) created a scene at the local hospital’s Emergency Dept.

Soon I want to revisit the different degrees of infant vomiting caused by the pylorus.

As stated above, true PS will usually kill.

The baby loses first weight and then condition.  Bowel motions and then urination will slow and stop.  The stomach and esophagus will be damaged by the acid and violence of its vomit – which may show traces of fresh or old blood.  The baby will not only be ravenously hungry and starve, but it will also visibly dehydrate, and (unseen to the eye) its blood chemistry (make-up) will become so messed up that it will move into sleepiness – this being the sleep of death.

If in any doubt, see your doctor or the hospital, and if still in doubt about the outcome, insist on doing a test feed, and having blood tests and imaging tests done (xray, ultrasound).

If your baby is not showing these terrible symptoms, before or after PS surgery, its problem is unlikely to be PS.

gerd-or-ihps-table1I recently came across the story of a 10 month old boy whose distressed mother suspected that an incomplete pyloromyotomy was responsible for his continued projectile vomiting and his being only half the weight normal for his age.  Was this a case of continuing PS and doctors denying an obviously incomplete pyloromyotomy?  Or was it severe GERD that was responsible?  A truly independent doctor was very much needed to decide.

Parents and patients will sometimes find such people-of-integrity and compassion hard to find.

When pyloric stenosis is hard to diagnose

Scared_DoctorA 1975 article on the difficulty of diagnosing some cases of infant pyloric stenosis (“PS”) underlined the sympathy I have for medical doctors: these important professionals have to be across thousands of symptoms and medical conditions, on good days and less easy ones, and also across the latest findings and journal reports… and up to dealing with upset or just plain difficult people, and with their receptionists and colleagues.  Is it any wonder that we, the general public, tell and retell many GP and MD stories that are not all that flash?

Mum w baby01This blogsite includes several posts about this, warning parents of sick children to –
1) do their own homework,
2) be your child’s best and well-informed advocate,
3) take somebody supportive if needed to what may be a difficult consultation,
4) stand your ground if it is solid, and
5) if all else fails, get a second opinion, go to the hospital Emergency Department and find another doctor.

Far too often I have quoted or commented on the stories of some parents of PS babies are outrageously out of order.  See the “Topic Categories” box (upper right of this page) to find some of the stories of parents and PS patients (at the list’s bottom).

Having mentioned the sad and bad stories again, I have also written about the complexity of diagnosing some cases of PS (see “Pyloric Stenosis – Diagnosis” in the Topic Categories).  The 1975 report I mentioned above deals with four unusual but not abnormal PS cases, and this report has told me more than it actually says.  Click the link above to read the entire and (to me at least) interesting 1975 report which I will overview and comment on here.

It is stated of 3 of the 4 babies that their PS showed itself quite clearly within two weeks of their birth.  All 4 were eventually operated on – but not until well after the normal “window” of “3 days to 3 months” for the majority diagnosis and treatment of PS.  As I understood the report, the youngest was almost 4 months and the oldest 9½ months at surgery.  What happened?

Q mark1All four babies showed inconsistent and unusual symptoms of PS –

  • their vomiting was unusual, sometimes subsiding or intermittent,
  • although all failed to thrive and gain weight normally, they never fell below their birth weight;
  • gastric peristalsis (muscle movement across the upper belly after feeding) was often weak or absent and didn’t cause the usual cramping, pain or distress;
  • the swollen pylorus could not always be felt (“palpated”) and did not always show clearly on x-rays.

ponderOn the other hand –

  • their vomit was bile free, showing their stomachs were blocked,
  • changing or thickening their milk or formula and keeping them upright after feeding did nothing to remedy their condition, and
  • medication with antispasmodics to rule out pylorospasm (in which the pylorus spasms but does not thicken and block the passage *) and confirm gastric obstruction brought no relief.

Yes, my complaint about doctors’ poor attitude and diagnoses has been often and clearly stated on this blog.  But in the light of this report it should be clear that –

1                    When there is a family history of PS, when parents are experienced, when they show they have done their homework, and when several of the symptoms of PS are clearly present, a wise doctor will agree to immediate further tests, with good reason (see below) although this is something that is commonly resisted.

2                    When there are some symptoms suggesting PS but not enough evidence for a diagnosis of PS or another condition it would also seem sensible to run further tests.

3                    When no clear diagnosis of PS is possible it is necessary to eliminate other abdominal conditions including pylorospasm from the doctor’s considerations.  This may be done by changing the feeding regime (as mentioned above), trying an anti-spasmodic medication, conducting further tests, and/or by waiting for a week or so and then if necessary repeating some of the tests.

4                    Waiting will be difficult for anxious parents of a small baby who is losing weight and condition.  A good doctor will understand this and convey empathy as well as working through what such parents may need to watch out for and do in the interim if necessary.

Father compassion01e5                    Again, if the doctor is not supportive and seems negligent and especially when the baby is clearly losing condition (losing significant weight, not soiling diapers / nappies and not urinating, appears drowsy and is sleeping excessively) it is high time to bypass the family doctor or paediatrician and insist on the local hospital taking responsibility – and urgently.

What eventually did help the four babies in the report was the comparison of their earlier with more recent x-rays: there are several typical radiological (x-ray) signs of PS, all of which are not always evident or clear from one series of images, but do become so by comparisons.

What did I learn?

1                    The report shows clearly that when it is not possible to diagnose PS with confidence (and what parent, patient or doctor wants unnecessary surgery or medication?) a careful process of tests, management and observation will clarify the real problem.

2                    PS manifests itself and can develop in a variety of ways.  Although most cases arise along textbook lines, there are occasionally exceptions to the “rules”.  Case 3 (a 7 month old girl) is the clearest example of this: her case should be read in full by interested readers.  Her PS was evident at 10 days but seemed insufficiently developed at her first pyloromyotomy at the age 7 months and she needed a second operation at 9½ months when a fully developed PS was found.

3                    It will be clear that these four cases were different from other groups of PS sufferers, including the “late onset” infants who first show PS symptoms after age 3 months, and the mild PS cases who are not considered as needing surgery, perhaps treated with medication, and sometimes continue to live with the signs of mild PS, to the extent that some of them seek a pyloromyotomy later in their childhood, teenage or adult years.  There are numerous stories from and about such PS sufferers on the web.

4                    Once again there was a loud silence in this 1975 report about the medical treatment alternative for PS babies.  This blog has often urged that medical management of PS be tried before surgery is even considered.  The “Topic Categories” box (top right of page, “Pyloric stenosis – Medical treatment”) will give the links.  Medical treatment of PS is of course slower to take effect than surgery, but it controls the condition in 70-90% of cases (depending on which report you read), makes good use of the time lag that is necessary to eliminate the possible diagnosis of pylorospasm, can always be set aside for surgery when necessary, and is non-life-threatening and non-traumatic for both baby and parents.

M820/00925                    Parents can learn from this 1975 report and (I trust) my post about it.  It underlines the need for parents to be as well-informed as they can be: to be their baby’s best possible advocate, they will know about and understand the symptoms of PS, will ensure that their medical consultants are exercising their duty of care, and will be willing to “change horses” if necessary.
This may sound presumptuous and “a big ask” of distressed parents, but as I have explained, it is far from impossible, especially with the help of a suitable supportive family member or friend.
Realize that the life of your little one may be at stake.
Today, medication, anesthesia and infant surgery for PS are almost always safe.  But from what appears on the web, the negligent delay, diagnosis and treatment of PS are the most frequent killers of PS infants.

*  What is the difference between pyloric stenosis and pylorospasm?
Narrowing of the pyloric canal is a common finding in infants with chronic vomiting, and it is most often due to pylorospasm.  In such cases, there is no thickening and hardening (hypertrophy) of the pyloric passage, causing it to narrow (“stenose”), and the spasms (contractions) of the pyloric muscle can be treated by modifying the feeding technique and/or with an anti-spasmodic drug.
When the vomiting persists, a question arises as to whether it is due to pyloric stenosis.  Distinguishing between the two conditions is difficult but important, for while spasm can be treated medically, true stenosis (physical narrowing) may require a carefully managed course of medication or surgical intervention.
Some believe that surgery is the only remedy for true pyloric stenosis and that all cases that respond to medication only had pylorospasm; this has been rejected in the light of studies of diagnosis and treatment.

 

Improvements in infant surgery (4) – Good diagnosis

Poor diagnosis and arrogant manners are by far the two most frequently heard complaints from the parents of pyloric stenosis babies.  As I write this post I am following a TV program about parents taking their sick children to (cop this) chiropractors!  One Australian chiro on the show told us that 1/3 of his patients are children.

And why?

Chiropractor_smallBecause many parents find doctors take no time for their patients, and especially not for children and their complaints.  So (in Australia at least) growing numbers of parents are asking their friendly chiropractor about asthma, autism, colic and reflux.  And chiropractors are setting up specialist pediatric clinics.  Wow!

I have often posted about the large of number of parents who air on the web their deeply felt grievances about the pediatrican they consulted about a very sick baby, and about the simple things both doctors and parents should remember and do better.

I recently read a “good news” post on this subject, from a doctor (“Casey”) in Broome, a town of 15,000 located on the coast of NW Australia.  He wrote about the challenge of recognizing a pyloric stenosis baby when so many sick babies are brought to his clinic.  What he says about the diagnosis is far from rocket science: I have often mentioned the short list of easily understood and quite recognizable symptoms.

Remind yourself of these basics . . .

Picking the baby with pyloric stenosis from all the refluxing, positing and normal kids can be tricky.  In GP land this is a really common presentation: a new mum with a kid who “spews” after feeding.  We know that the vast majority of these kids will have no pathology, but you really don’t want to  miss an important diagnosis like hypertrophic pyloric stenosis (HPS) or a more sinister GI obstruction, sepsis or other badness.  So I thought I would tell you all about my all time favourite PS diagnosis and have a look at the recent evidence for the diagnosis and management of HPS.

This baby ticked all the boxes :

  • he was a boy (~80 % of HPS in males),
  • His symptoms started around 4 weeks age, and presented at 5 weeks (classic age)
  • he was a few weeks premature (commoner in premmy babies – and they present relatively younger),
  • his mother described a progressive history of increasingly voluminous and forceful vomiting over the past week, this was non-bilious.
  • He was constantly hungry and wanting to feed immediately after spewing.
  • He had lost weight i.e. was not thriving as is often the case in overfed babies who get “overflow” vomits

My practice with the neonate presenting with vomiting is to observe a test feed in the ED or the rooms and watch to see what happens – usually you see a palmful of milky posit come up and can be reasonably reassured that this is likely normal “mum badge” production.

  • This little boy had a test feed and within 3 minutes he erupted like a volcano with a full feed being thrown about a foot in the air over mum, me and the bed – convincing.

The classical triad of HPS is:

  • a palpable “olive” or “tumour” in the RUQ, 48% of cases
  • visible peristalsis ~ 25 % of cases
  • Hypochloremic (hypokalemic) metabolic alkalosis
    • Interestingly hemetemesis was present in 16% at diagnosis – not a traditional symptom of HPS
    • Seeing one component of the triad is common, seeing all 3 is pretty rare – so do not rule out if your patient hasn’t a full deck of signs.

This triad is becoming a rare beast.  Taylor et al (from Westmead in Sydney) published this paper in Journal of Paediatric Child Health in Jan 2013.  They showed that clinical diagnosis is now uncommon – less than 10%, with more reliance on imaging such as Ultrasound and Barium studies.  Only about half had a “tumour” – which might be due to deskilling in us, or just earlier diagnosis?  Ultrasound seems like a good initial investigation as it is painless, non-invasive and has high sensitivity and specificity…   This remains a technically tricky modality – so not one I would do myself in the Emergency Dept and hang my hat upon.

The story continues:

So our little guy – what did examination show?  Well, I could not feel a mass, the vomiting was very convincing….. but technology sealed the diagnosis!  The little guy’s grandfather was an anaesthetist [in a hospital on the other side of the country], who just happened to be doing a list for a paediatric surgeon that morning.  Mum had videoed a clip of “visible peristalsis” after his morning feed and emailed it from her iPhone to her father, who showed it to the surgeon, who then called me just as I was observing the impressively projectile vomiting.  Slam dunk diagnosis!

So I placed an IV and took some blood for VBG and electrolytes.  Resuscitation and electrolyte correction are the cornerstones of initial management…

So now – here is the conundrum.  This boy needs to get to a surgeon to have his pyloromyotomy soonish, yet he is at risk for apnoeas.  So when is it safe to transfer him?

Can we predict who might have apnoeas? …  I think in the really small and premature babies the risk would be higher – so you might need a super-specialist advice on that one.

Alright – that is all I know about pyloric stenosis.  It can be tough to pick the true PS out of the crowd of spewing kids.  Our classical triad is not the norm, however I would advocate the use of Ultrasound early as it is pretty good and there is no real downside.

There was one Comment on Casey’s Post, and I found it significant as it added to Casey’s theme –

Great case Case!  Saw a suspected case with a GP registrar when I was an intern, he only had the olive and not impressive vomiting.

Since then, plenty of garden variety vomiting kids.  Have stopped asking about the nature of vomiting though.  While working as a paeds reg for a few months in town, every child was described as having ‘projectile’ vomiting.  The classic “how far across the room does it go?” might be more differentiating?

I like the use of video technology too.

Dr Casey’s Post gives no indication of his attitude to his patients, but he makes clear that unlike many of his colleagues he is alert to the obvious signs of pyloric stenosis.  This (sadly) seems remarkable.  If only more doctors were as alert, aware, sensible and “grounded” as this one!

Chronic or recurrent abdominal pain

Many of the questions discussed on web forum sites* by people who have had surgery for infant pyloric stenosis (“PS”) are about abdominal pain.  Any of us, whether we were “Py babies” or not, may have (or have had) a problem here: it affects babies, children, teenagers and adults alike.  Empathize with this typical cry for help:

kid stomach pain3My daughter had the surgery [for PS] at 4 weeks.  First female in my area.  She has just turned 11 years.  Easter break she started hurting bad.  She missed her own birthday party.  Now it’s 6 weeks later and our lives are on hold.  She is an A student but has missed 4 weeks now.  She hurts in her scar area.  We have had blood work, x-rays, cat scan, ultrasound.  And no one can tell us why she is hurting and losing weight, dehydration.  Please someone help me!!!

Questions:
On the basis of what this worried parent has asked, what would you advise?
Are these symptoms related to the young girl’s infant surgery?

In this post I want to overview this taxing area of personal health issues, so that those affected will be better able to –

  1. understand the possible causes of their problem,
  2. appreciate the complexity of the doctor’s task in helping them, and
  3. help their doctors to arrive at a good diagnosis and management as soon as possible.

Understandably, those with a history of PS or other abdominal surgery will regard that as a prime suspect, and justifiably so.  If more cases of PS were treated medically and by regarding surgery as a second option, there would be fewer RAP cases among “Py survivors”.  Several studies have shown that there is indeed a higher rate of gastro-intestinal problems after abdominal surgery including for PS.  This is also suggested by reading the web-based experiences of PS people.  Abdominal operations inevitably result in the formation of a surface and internal scarring (adhesions) which sometimes cause great grief.  My several posts about adhesions may be found via the Categories box on the upper right of this page.  And adhesions are only one of the possible long-term effects of infant surgery, as this and its sister blogsites bear out.

Sometimes the complaint or question about RAP will relate to the surgical scar or to pulling, tearing or stabbing pain under it.  But not often.  There is a very long list of organic (body organ) and functional abdominal disorders that can cause discomfort or pain, as many of the websites on chronic or recurrent abdominal pain will make clear.

Diagnosis01Chronic or recurrent abdominal pain has its own catchy acronym: “RAP”.

Doctors try to distinguish between organic gastrointestinal (“GI”), organic non-GI, and functional GI disorders.  This can be difficult, but specific criteria are used, as will be explained below.

RAP is significant because –

  • it is one of the most common symptoms in children and adults worldwide, estimated to trouble 13 – 15% of children.  These children with RAP account for 2% to 4% of visits to primary care doctors and 50% to pediatric gastroenterologists.  And again, RAP is certainly not limited to little people!
  • in children it is responsible for considerable distress, reduced school days and academic performance, disturbed peer interaction, family stress, and a high use of health resources.

RAP may be caused by functional disorders (those which cannot be explained by structural or biochemical disorders) and organic disorders.

Among the many organic GI conditions that can lead to RAP are inflammatory bowel diseases, esophagitis, chronic pancreatitis, and gallbladder disease.  One study found that only 8% of patients with RAP had, after extensive investigation, any organic disorder.

Diagnosis02Functional abdominal pain is not a specific condition but rather a description for a variety of symptoms.  By definition, children who have abdominal pain but lack blood, mucosal, radiographic, and structural evidence of disease are regarded as having a functional disorder.  Functional GI disorders have their own acronym (FGID) and are also divided into categories – four in fact.

The possible causes of each of these different kinds of RAP may be complex and only very partly understood.  They cannot even be outlined here, but the informative websites listed below are well worth reading by those interested.

The frequent uncertainty about diagnosis, RAP’s chronic nature, and growing parent/patient anxiety often follow the unrelenting and disruptive path of the RAP.  This can make management by GPs and pediatricians very difficult, time-consuming and expensive.

To answer my earlier questions…

On the basis of what this worried parent has asked, what would you advise?
My answer:  Read what the websites I have recommended** say about RAP and so help your doctor get the problem in clearer focus.

Are these symptoms related to the young girl’s infant surgery?
Probably not but possibly so.  If your doctor dismisses this possibility (as doctors are inclined to do) make sure you understand why.

* There are many forum discussion sites that share personal experiences in dealing with discomfort and pain after PS surgery, including BabyCenter (USA), BabyCentre (UK), Experience Project, Facebook, MedHelp, PatientUK, and Topix, and Yahoo! Answers.

** Websites that give more detailed information about RAP may be found here and here and here.

Some more oddities – with a dash of humour

A baby with pyloric stenosis is far from funny for the anxious parents.  Pregnancy, giving birth and learning the ways of the little stranger who has moved in with you bring more than enough stress.  Who wants their new baby to vomit with increasing violence no matter what paediatricians and baby health sisters suggest?

But some of us who have had surgery for pyloric stenosis (“PS”) grow up to discover we have not only a scar and perhaps other long-term effects, but also a sense of humour.  However, even some of the humorous anecdotes show a trace of the annoyance (to put it diplomatically) about matters that this blogsite has often mentioned.

Here is a selection of the delightful stories and cryptic comments I have discovered …

Story 1

Baby stunned1My scar is from my first drinking story from when I was three months old.  I had Pyloric Stenosis, and was projectile vomiting because my lower stomach muscle closed.  The Doctors had to operate and cut out a section of my stomach.  When the Doctor was finished, he handed me to my mother, which was when she smelled scotch, and believed that he had been drinking.  I guess he saw the worried expression on her face, and he said, “No no, I gave it to the baby.  A small child can die from anesthesia, so I gave Nathan the scotch.”  So that’s the first time I drank.

When I was 20, I saw someone with this same scar, and it was exciting to see that.

Story 2

I was born premature and jaundiced due to mother’s Rh- blood type, and nicknamed “Dormouse” by the nurses (yellowy and curled up!): Sick, very sick.  I had a pyloric stenosis which causes projectile vomiting but wasn’t diagnosed till I was at death’s door.  My parents were reassured by the paediatrician daughter of one of Grannie’s bridge friends that Pyloric babies were as tough as old boots once they’d had their surgery – so I acquired two nicknames before I got my official ones – when I was about 3 or 4 I refused to answer to “Tuffy”.

Story 3

Baby worried1I want to let all parents know about a sickness in infants that most doctors don’t even know about.  It is when the muscle in the stomach works more than it should and then it squeezes the intestine off and won’t allow food through, which in turn starves the child…  Watch for vomiting, no bowel movement, weight loss, and painful crying.

I have had two boys with it and both times I had to fight to get the doctors to listen to me.  We came within 8 hours of losing the first child because the doctors had never heard of it.  My second son had a better chance because I knew the signs, but the doctors told me there was 1 in 2 million chance he had the same thing as my first son, so we again nearly lost him.  I don’t want this to happen to anyone else so I hope this post gets to a lot of people.  Oh, and it is mostly found it white baby boys although I don’t tend to trust what the doctors say anymore.

My boys are doing wonderful now.  Thank God. when I brought the youngest home from his surgery his big brother saw his cut and showed us his and told us that brother must have had his doctor because he cut him too.  Now he is always showing everyone his scar and letting them see his brother’s.  It’s cute.  My husband told him when we brought his brother home he couldn’t touch the cut and that got it all started.  Then when he started asking why my husband told him that it was so everyone could tell they were brothers and just alike in some ways.

Story 4

Baby knowing1Perhaps this story, which Joe says may be a myth, best captures his life.

“I was born with pyloric stenosis and born at a time when it was a fatal disease.  Although Dr. Flood, the greatest infant surgeon at Mt Zion Hospital, would perform the surgery when I was 6 days old, the hospital staff told my parents to prepare for the worst and buy a casket.

When I didn’t die, my Uncle Vinny threw a party…  They threw the tiny casket in the fireplace, things got out of control, and the house burnt down.”

Story 5

Baby surprised1I had PS as a baby back in 1974.  It is far more common in male children.  Girls have a 1/1000 chance of having it.  Ha ha, forget the lottery, but I was the lucky one to have the blockage. It was quite serious in my case because the doctors failed to diagnose it right away.  They thought my mother was a nervous first-time mom and ignored her concerns that I wouldn’t burp, but had projectile vomiting etc… Finally at 5 weeks of age, my esophagus ruptured from vomiting and I was rushed in for emergency exploratory surgery.  By that point I had a 50/50 chance of surviving the surgery.  The problem was corrected, but to this day I have a lovely scar from stem to stern.

Story 6 – from an older doctor after cancer surgery –

It’s a rush but life is full of risk.  A doctor friend enjoys riding his motor cycle at high speed in the mountains, too.  In the past, I’ve spent two and a half hours in the water, in bad seas, in the Pacific, without so much as a life jacket, after a sailing accident and been rescued by a fishing boat, against all odds – so the forty-nine years since, have been a bonus.  …… maybe when I nearly vomited to death from pyloric stenosis in 1937 at the age of five weeks was my first crisis… 

baby quizzical1This morning I did a pre-operative clinic, which, like all my work I thoroughly enjoyed.  It was actually quite amusing when I interviewed a lady in her forties who had undergone complicated surgery.  She showed her now healed but very widely scarred midline abdominal incision to me.  I mentioned that I also had one just four weeks old in 1937.  She indicated that she would be interested in viewing my nicely healed narrow scar so I showed her the upper part of it for a second.  I don’t suppose that constitutes unethical conduct and it is a really good scar!

Story 7 – takes my prize (just!)

Baby disgusted1When I was an infant of seven weeks’ age I had abdominal surgery.  They sewed me up with tiny little stitches but as I got bigger, so did the scars the stitches left.  I have a line of four strange little puckered scars on my abdomen that look like old stab wounds.  Of course most people never see them, but on those occasions when they do – at poolside, for instance – my scars are a source of great evil fun.  When someone asks about the scars, I tell them:

“When I was seven years old, I spent the summer on my grandpa’s farm.  My cousins and I were in the barn taking turns jumping out of the loft into a pile of hay – but we didn’t know that someone had left a pitchfork in the hay, and its tines were pointing straight up – “

I’ve never been able to finish this creative tale because the listener always BEGS me to stop here.  I love doing this! 

Remark 1

Baby laughing1I had pylorics, so did my son and 16 other family members; unfortunately one did pass away but it 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.

Remark 2

I had PS in ’71.  Fixed.  Fixed too good.  Now I have a gastric bypass, so it turns out the surgery was unnecessary, but it served me well for 40 years.

Note: I’m grateful to the people whose posts and forum comments I have quoted in this and the previous posts.  Links to the references are available on request.