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Swimming upstream: The journey to the source of pyloric stenosis of infancy
55 years later, the memory remains. A killing condition at the onset of a new human life. An extraordinary set of agreed clinical features regularly paraded in a catalogue of books, journals, and magazines. A totally unexplained condition with little evidence of anything more than a passing interest in the cause. I had just had my first introduction to the mystery of pyloric stenosis of infancy (PS).
My career was not to be with babies, but my imagination was already fully engaged. The prevailing surgical interest in Glasgow in 1960 was with the physiology of gastrin induced acid secretion- and feed-back control of acidity. The here and now of an adult surgical career did get in the way somewhat but our early endeavors to explain temporary early acidity in normal neonatal development did show:
(1) that ALL normal babies quickly developed high gastrin levels in the early days after birth and
(2)-that gastric acidity was rising at the same time as gastrin and that the usual negative feed-back between gastrin and acidity appeared to take further time to mature.
The subsequent demonstration by Agunod that acidity peaked in NORMAL development at around 3 weeks of age was entirely consistent with an insensitive negative feed-back which takes around 3 weeks to mature. There goes the mystery of vomiting onset at 4 weeks!
Prof. Dodge had been able to produce PS in puppy dogs with artificial acidity produced by gastrin injections before and after birth. This observation showed-and for the first time-that hyperacidity alone could produce PS as it were from a standing start. No other mechanisms were necessary.
Did our human PS babies have elevated gastrin levels for some reason? The answer was-they did NOT!
Suddenly the possibility dawned that Primary Hyperacidity-in particular an INHERITED hyperacidity-in PS babies would be a neat, simple, and credible explanation. All the following mysterious clinical features were thus well explained.
- PS babies do have more acid and the acidity remains even when gastric outlet obstruction is relieved.
- The male preponderance is explained by the greater acidity in baby males.
- Familial hyperacidity leads to familial PS.
- Babies whose gastric acidity is not neutralized by alkaline amniotic fluid in utero-i.e. esophageal atresia babies-have an increased incidence of PS. They have an acid head start!
- Neutralizing acidity with P.P.I . drugs temporarily facilitate long-term cure and quickly abolishes pre-op. alkalosis.
- Hyperacidity causes the pyloric sphincter to repeatedly contract and inevitably, under the trophic influence of developmental high gastrins, the sphincter becomes hypertrophic.
- Hyperacidity in adults is known to be associated with a relish for food. Hyperacid male babies destined to develop PS classically feed with vigor. Frequent feeds produce high amplitude, frequent sphincter contractions with hypertrophy as the inevitable outcome.
And so it goes on–.
This blog post is called swimming upstream- swimming against the flow. Salmon swim upstream to revisit the scene of their early life and spawn. In some ways exploring the written thoughts and simple truths of those early pioneers who pondered on the cause of PS was for me a similar journey. I was sharing my early student wonder with them undistracted by modern technological discoveries.
- They too had speculated on primary hyperacidity as the cause.
- They too had realized that continued feeding by mouth should be paused or restricted. (Rectal infusions of nutritious fluids instead seemed to do the trick).
- They too had realized that acid entering the duodenum was the most potent way of causing the sphincter to repeatedly contract and cause hypertrophy.
Sadly, simple things, then and now, do not appear to cut any ice and their early intuitions fell on fallow ground.
Modern discoveries have led to alternative modern theories of cause disconnected from a need to explain the classical clinical and time sensitive features.
- The discovery that nitric oxide (NO) causes smooth muscle contraction led naturally to attempts to link NO with PS. All thoughts of other clinical features jettisoned. NO has NOT been found to differ in concentration or quality in PS babies.
- As with our early distraction with gastrin, the simpler, more direct primary hyperacidity has been disregarded.
Others have speculated that an abnormal accumulation of growth factors in the sphincter causes hypertrophy. Indeed, reports have emerged of allegedly increased concentrations even though there have not been, or could never have been, adequate control specimens. These studies also fail to acknowledge that a sphincter repeatedly contracting becomes hypertrophic by attracting growth factors to it. This theory similarly does not stand up to analysis.
As a general surgeon I have indeed been brave to seek to enter the pediatric domain. I have been swimming with difficulty against the flow. My attempt to contribute has not always been welcome.
The modern surgical treatment of the established case is immediately transformative. It is simple and provides almost mortality-free long-term cure and we should honor their contribution.
It is with the ”stuttering” all too common presentations of the early mild case, with symptoms that come and go, that a knowledge of the true cause will come into its own. Reduced feeding and temporary anti-acid treatment similarly has been shown to safely produce long term cures (I can hear the pediatric surgeons grind their teeth!)
The presence of active Pyloric Stenosis Support groups bear witness to the many occasions in which surgical treatment of the emerging case is delayed and treatment is unsatisfactory. Sensitive individual management at this time surely should have a part to play!
Has the disinterest in cause of 55 years ago become sine qua non an established rite of passage for the Pediatricians of today? Have they lost the sense of student wonder? I hope not.
Ready to read this book?
The Cause of Pyloric Stenosis of Infancy chronicles the debate surrounding the cause of pyloric stenosis from its earliest discovery 300 years ago to the present. You can read it now on ScienceDirect or buy your own copy on the Elsevier Bookstore and save 30% + get free shipping when you use promo code STC30.
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