My previous post translated the first half of a 1939 Dutch medical journal article which has given me the clearest information yet on how my life-threatening illness would have been managed at the time. The article is based on 130 PS cases in the 13 years after 1926 in a northern Netherlands city clinic. This post reflects the second half of the article – which answers some of my questions with statistics of the dangers, the follow-up, and the long-term after-effects of PS as these applied to me.
Regarding after-care, it is the Groningen Surgical Clinic’s practice not to discharge the baby until its wound has healed. Kirschner and others transfer the infant to the pediatric clinic immediately after surgery. There can be no objections to our practice, provided the feeding regime is arranged in close cooperation with the pediatrician. The child is given its first feed two hours after the operation, using a teaspoon with a small amount of breast milk with a third part of water. If during the surgery there was a breach of the duodenum or stomach, nothing is given orally for 8 hours. If the illness was of short duration and the infant is in a good condition, intake can be increased to normal breast or bottle feeds over a few days.
The longer the duration of the PS and the worse the baby’s condition was at the time of surgery, the more care is needed in increasing the amount of nourishment given. Many of the infants vomit once or more often during the 24 hours after the surgery; they may also vomit after this, sometimes for several days. It diminishes slowly, and healing usually follows. Too great quantities given too suddenly or too soon several times resulted in acute dyspepsia.
The mortality rate associated with Ramstedt’s operation is significant in assessing the procedure. We have been relatively fortunate, especially considering that we operated on all the infants referred to us regardless of how slim their prospects sometimes seemed.
Seven babies died soon after the surgery. In three of them the pathologist found no clear cause of death other than severe malnutrition; we must conclude therefore that for these babies the procedure was too severe.
Three children died of a perforation resulting in peritonitis. In two of these cases no perforation was evident despite careful inspection during the surgery. In relation to this we refer to Stich and Makkas who demonstrated that remaining thickened muscle tissue at the duodenum end of the pylorus, through pressure from stomach peristalsis [muscle movement], can cause a secondary breach of the mucosa and peritonitis. It is therefore essential to ensure that the mucosa is thoroughly freed. This will result in a percentage of mucosal perforations for which the prognosis need not be bad, provided they are properly closed. The trick is to find the right middle course! One infant died of a strangulated ileus 25 days after the surgery. The mortality rate was thus 5.4%.
For a long time Kirschner had a mortality rate of 0%, but later he had several deaths. Heile lost 3 infants of 67 cases. Griep had one death from 28 cases. Ramstedt recorded mortality at 14.4% whilst Ibrahim calculated 10.6% for a collection of statistics.
Three babies continued to vomit as they had before the surgery. All three healed after several months of conservative therapy. The reason for the continued vomiting never became clear.
After their operation the babies stayed at the Clinic for an average of 15 days, after which they returned to the care of their family doctor.
Unlike what Meuwissen and Sloof, Lanz, Noetzel and others have reported, it was never necessary for us to operate a second time due to a recurrence of the condition.
In one case cited by Meuwissen and Sloof, minimal hypertrophy [enlargement] of the pyloric passage was found at the first operation, but at the second surgery five weeks later the hypertrophy was very marked. The five infants in our series in whom we found no PS recovered later without any special treatment, and no hypertrophy developed. Two months after surgery that was performed elsewhere, Lanz found a hard tumour with a neat straight scar. Noetzel also found a scar at the second operation: it consisted of mucosa and serosa [the membrane that lines the abdominal cavity] but was strong enough to stop the mucosa bulging [as it should when the swollen pylorus is split and opened].
We saw several family-related cases, as did Brendl, Werner Schmidt, and Halbertsma: twice we saw two brothers and once a brother and sister.
Functional and anatomical healing are not always coordinated in hypertrophic PS. Many researchers have observed that PS infants treated conservatively [with medication rather than surgery] who had been clinically healed for some time continued to show the symptoms of the condition when x-rayed.
Paas and Kirschner found no noteworthy delay in gastric emptying at later consultations, nor did Heile – provided surgery had been prompt. However, Werner Schmidt, using repeated x-rays, found significant differences in healed as well as sick children.
We were able to follow up the lives of 80 of the patients we operated on, partly through later examinations by the children’s hospital where a professor kindly provided us with data, and partly by information obtained from family doctors.
Not one infant suffered from wound rupture, and we found only one with a somewhat widened scar. 38 of the children are healthy and have developed completely normally, whilst three died of an unrelated illness. 24 had clear signs of neuropathy [damage to the nervous system caused by the surgery that may affect involuntary body functions, including bowel function, heart rate and blood pressure], often with exudative diathesis [damage to cells and tissue].
One neuropathic child suffered a recurrence soon after being discharged from the hospital, and after extended conservative treatment at the children’s hospital it was discharged as healed. One infant was admitted to hospital with dyspepsia several times during the first year.
Four children complained about a bloated feeling in their stomachs after meals and had a poor appetite, and another five vomited occasionally for several months after their discharge, although no longer in the typically projectile way. Several of the children were clearly backward and debilitated and one of these suffered from epilepsy. Finally, one of the patients was diagnosed with muscular dystrophy at age six.
Our follow-up was restricted to those children who had their surgery at least one year previously; the oldest child is now almost 14 years old.
The second half of this article makes several things very clear.
- Babies with PS or other conditions of infancy used to run high risks of death, “collateral damage” and complications – especially if they had their surgery and all that went with it before the later 20th century. Of 130 tiny patients, 7 died and some 45 suffered various kinds and degrees of after-effects: I am grateful for the honesty and thoroughness of this Dutch study. It helps me to unearth important parts of my early life story – things my parents probably never knew but which affected them and me.
- No wonder many parents of those times were scared, upset and traumatised, and small wonder that this has affected at least some of their children. If my surgeon ever told them what he would have known, no wonder my parents (as children of their time) chose not to tell me anything.
- So many of us with a PS story have had minor or major problems as a consequence of the condition and/or the surgery. Not all of them were even recognised in 1939. PTSD has affected millions but its damaging effects are only now being mapped and addressed.
- We are (or should be) grateful that so much has been learnt since this honest and scary report was written: about PS and other illnesses and malformations of infancy, about the most fitting treatments, about minimising the trauma and damage done by surgery on little people, on good hospital care and support for the parents of pediatric patients… the work goes on.