Pyloric stenosis: unearthing my case history (1)

As we grow older, exploring our past becomes increasingly important to many people.  In general terms, youth enjoys today and dreams about tomorrow, middle age is busy with work, personal and family commitments, and in our senior years we try to make more sense of our journey.

“Pyloric stenosis” was the title of one of the first chapters of my life, but these pages were closed to me until recent years.  Only recently, thanks to the internet, have I been able to open most of the pages of this chapter.  My parents avoided reading them to me, and the local libraries’ resources did not extend to minority medical matters.

Because of this, imagine my excitement when I recently found an old medical report on how pyloric stenosis (“PS”) was dealt with and written by the medical faculty of the provincial capital nearest to where I had my surgery!  And it was written in late 1939 – less than 6 years before I was born!  The Dutch language was no problem for me: here was the most accurate and fullest description I’m ever likely to read of the condition that almost meant the end of me before I was a month old – and how it was treated “in my day”!

Because many of the readers of these posts are older folk who had PS and its surgery in the 1940s, ‘50s and ‘60s, I have translated the article to share it around – please bear with me!

Peristalsis from pyloric stenosis at work

When an otherwise healthy infant vomits soon after a feed during its first weeks, this vomit is without bile, becomes regular, increases in force, is expelled explosively with an arch, and when one can see peristalsis [bulging muscle movements] over the stomach – then a diagnosis of hypertrophic pyloric stenosis is very likely.  Usually the baby loses weight rapidly.  The “tumour” cannot always be felt.

A relatively small operation, as indicated by Ramstedt, brings about rapid healing.  The results of larger series of surgically treated cases of PS not yet having been published in the Netherlands, we decided to research the lot of the tiny patients operated on at the Surgical Clinic in Groningen [in the northern Netherlands].

Large series of similarly treated patients have been published in Germany (Heile 67 cases, Kirschner 47 cases, Kehl 50 cases, and Max Page 150 cases).

PS seems to be rare in the Dutch East Indies: 3 doctors have reported dealing with just 15 cases in the past 4 years, of which only one concerned a Javanese child from a well-to-do family.  One of these doctors reported privately that he had not seen a single case among the Javanese people during 10 years of practising in the tropics.  The other 14 cases belonged to the European population.

A Ramstedt-Weber pyloromyotomy was performed on 130 infants at the Surgical Clinic in Groningen in the 13 years since 1926: 102 on boys and 28 on girls.  In 7 cases the clinical diagnosis of PS proved to be incorrect when the abdomen was opened; in 5 there were no abnormalities, and in two there was a congenital abnormality of the duodenum.

This misdiagnosis is mentioned in other literature: Harrenstein found nothing wrong upon surgery in 2 cases out of about 50.  It would be possible to avoid such cases by x-raying before surgery, as recommended by Meuwissen and Sloof.

The decision on whether to operate is always made in close consultation with the paediatrician.

During the early years of the period under review, cases were often managed conservatively before resorting to surgery.  In the later years Van Lookeren-Campagne recommended the following guideline:  If after 4 – 6 days of conservative therapy (consisting of very frequent feeds of small quantities of breast milk administered with a teaspoon, together with atropine drops and subcutaneous hydration) the baby’s condition has improved but its weight is not steadily increasing, in order to keep the risk of surgery to a minimum it is advisable to operate unless the infant is approaching 3 months of age.

Heile believes the possibility of successful surgery has passed if there is a significant enlargement of the stomach.

The development of the condition before the infant received surgery varied greatly.

It has been the practice of the Groningen clinic to conduct both the conservative therapy and the entire preparation for surgery at the Children’s Hospital.

We never refused to perform surgery due to the baby’s poor condition: several had very poor blood chemistry, one weighed 1880 g, one 1900 g and 11 others less than 1500 grams.

Several reports have mentioned x-ray investigation of the stomach’s appearance associated with PS in order to discover better indications for surgery.  The most significant symptoms were peristalsis, a lengthened and narrowed pyloric channel, stomach swelling and delayed stomach emptying.

The value of x-ray investigation is debated.  The relatively small number of patients whose clinical diagnosis proved incorrect shows that one’s goal is generally reached even without such testing. [sic]  Sometimes one makes an interesting discovery: Nauta for example discovered a diaphragmatic hernia in two infants clinically diagnosed with PS: he x-rayed them before the PS surgery and again several years later.

Only about 1/3rd of our sick infants were x-rayed before it was decided to operate… In both the enlarged duodenum cases mentioned above an x-ray before the operation found an enlarged stomach as it did in 5 other cases – although at operation no abnormalities were found.

An hour before surgery the infant is transferred to the Surgical Clinic.  Daily before the surgery and also several hours after it the children are given electrolytes (blood salts in solution form) with 5% glucose intravenously.  If gastric retention has been severe the stomach is sometimes emptied and washed out before the surgery, but Von Haberer and Hundsdörfer regard this as too harsh.  During the surgery one must be watchful about undue cooling and it is the Clinic’s practice to place the infant on an electrically warmed cushion and wrap it in cotton-wool during the procedure.

We always operated with the help of local anesthesia of the abdominal wall.  If the baby strains a lot during the operation, a sister will give it a sugar cube to suck, which usually calms the infant immediately.  Kirschner and others give a general anesthetic, claiming that local anesthesia has an unfavourable effect on the calm and haste of the operation.  We have not experienced these objections.

The location of the skin incision varied somewhat.  During the last few years we used a right sub-costal incision or a paramedian trans-rectal incision; Prof Eerland prefers the latter.  Both approaches have the advantage of the liver lying under the wound, avoiding a prolapse of the intestines.

Splitting the pyloric “tumor”

The surgeon brings the “pyloric tumour” outside the wound, taking care not to expose more of the stomach and duodenum than is absolutely necessary.  The wall of the pyloric muscle is partly split with a scalpel, and the muscle fibres are spread further apart using a dissector until the mucosa [or mucous membrane] bulges out somewhat for the entire length of the “tumour”.  The front of the muscle is incised, having the least blood vessels.  When the usually minimal bleeding has been managed with a cotton wad soaked in adrenaline and perhaps several interrupted sutures, the pylorus is returned to the abdominal cavity and the abdominal wall is closed in layers using catgut and silk sutures.  After this we generally applied a pressure bandage.

Kirschner advises using only the scalpel to divide the muscle but our method seems safer.  Occasionally a slip in dividing the muscle had to be sutured.

In 32 of the infants the division of the muscle resulted in a perforation of the duodenal mucosal membrane.  These perforations were closed using fine silk knotted sutures.  Despite this, however, 3 of these children died from peritonitis.

Thus even when the mucosa is damaged the prognosis is usually good, provided the breach is closed with care.  It is recommended that at the end of every operation, even when the mucosa seems to be intact, to check that it is indeed undamaged.  This is best done by stroking the air in the stomach towards the pyloric end and to look for bubbles emerging.

Dehiscence [rupturing] of the wound’s edges occurred in 7 of the infants, twice with a prolapse of the intestines.  Three times it was possible to restore the abdominal wall with secondary sutures; in all these cases a transverse skin incision had been used.

One operation was followed by internal bleeding, necessitating a transfusion; the baby recovered.  Sometimes mild suture infections delayed healing.  Siefert has published a case in which the baby continued to vomit after the surgery and died.  Examination found circular folds of the mucosal membrane inside the pylorus, which prevented the stomach from emptying.  Mandl also remarked that the surgery could fail due to the development of severe mucosal folding narrowing the lumen [opening].

Continued next post:  post-operative care, morbidity and mortality.

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3 thoughts on “Pyloric stenosis: unearthing my case history (1)

  1. Mark

    Very interesting, Thanks Fred for all you work translating. Well done!

    While reading, I started to wonder if you had ever run into any specific medical records of PS patients? I know ours are long beyond reach but always wondered how they would read.

    Looking forward to next week’s instalment.

    Reply
  2. Wendy

    Local anesthetic and a sugar cube! Yikes! And no mention of the baby’s pain or discomfort. The general anesthesia was used because “local anesthesia has an unfavourable effect on the calm and haste of the operation.” That photo of the cutting of the pylorus was graphic. Good to see but difficult to swallow (pun intended). So Fred, the jury’s still out as to whether you had local or general anesthesia for your PS surgery? Thank you so much for the care you put into bringing us these details.

    Reply
  3. Fred Vanderbom Post author

    Thank you for both your responses. You well understand that translating and publishing this material meant a lot to me.
    Mark, in all my years of searching I have never found anything near a medical or journal record of a PS patient’s sickness and treatment. The nearest thing to it was my own weight chart which my mother kept and showed me once; she kept it in her bedroom drawer when I was young, so I’d sometimes take a sneak-peek, but I never saw it again after those early years. In the 1950s sensitivity to children’s interests (let alone “rights”) were still well over the horizon. And even now hospitals regard patient records as their property and destroy them after a time. Parents’ stories of their baby having PS are typically quite detailed until soon after the surgery. I imagine they have little interest in keeping a record to pass on to their child in later years.
    Wendy, I think the weight of evidence says that I was operated on much as described in this article and many contemporary ones. However, as I recently reread some of the UK and US articles from the 20 years after the late 1940s, I was again surprised by how many surgeons were using ether. But one of these reports gave a poignant reason for using local anesthesia plus sugar or alcohol to keep the baby happy: this surgeon lost his first PS baby to an overdose of ether and wrote that since then he would only use local pain control. The contemporary literature shows a very deep divide on the pros and cons of each practice – and the alleged benefits and disadvantages don’t always match! Like parents, teachers and most of us, “we usually teach out of the way we were taught”.

    Reply

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