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History of Pediatric Surgery
hosted by Dr. Em Gootee & Dr. Todd Ponsky
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Pyloric Stenosis 9 items
Chapter 1 of 15 · Fundamentals
Introduction
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What the experts said
Pierre Fredet in Paris was the first to describe projectile vomiting and visible peristalsis in pyloric stenosis.
Pierre Fredet's innovation was the extramucosal pyloroplasty—staying outside the mucosa with a longitudinal incision, though he attempted transverse closure and often added gastroenterostomy.
In Hirschsprung disease, the visible lesion (megacolon) is not the cause; the true pathology is aganglionosis of the distal colon and anorectum.
Aganglionosis in Hirschsprung disease was first described in 1901, but its clinical significance was not fully realized until the 1940s.
Helen Taussig, a diagnostician, first approached Robert Gross about performing a Blalock-Taussig shunt for tetralogy of Fallot.
Esophageal atresia with distal tracheoesophageal fistula has been known since the late 17th century, along with associated cardiac, renal, and intestinal anomalies.
The first attempt to repair esophageal atresia was through upper midline laparotomy in 1888, which was unsuccessful.
Joseph Brennemann in Chicago tried gastrostomy for esophageal atresia, but it flooded the lungs via the fistula, causing aspiration and death; jejunostomy had the same result.
Harry Richter, working with Brennemann in Chicago, addressed the fistula itself in 1913 via right chest through a vertical paravertebral incision, sixth interspace, resecting posterior ribs, using open-drop ether and a homemade pump for positive-pressure ventilation.
Richter's two patients died at 2 and 22 hours postoperatively; he admitted he did not know the anatomy or what he was looking at.
William Ladd in Boston and Logan Leven in Minneapolis began dividing the tracheoesophageal fistula, performing cutaneous esophagostomy, feeding via gastrostomy, and constructing skin-lined tubes over the anterior chest through multiple staged operations.
Thomas Lanman's 1940 review of 32 esophageal atresia cases from Boston Children's Hospital—all fatalities—established that surgeons must operate early (within days of birth), diagnose without oral contrast (inability to pass NG tube plus air in GI tract confirms diagnosis), and avoid fluid overload.
Lanman's team knew aspiration was always a threat and that delay caused malnourishment, dehydration, and pneumonia, necessitating early operation before the child deteriorated.
Many early esophageal atresia deaths were attributed to 'fluid overload' or 'injudicious fluid administration' because surgeons had no concept of maintenance fluids or resuscitation volumes (the 4-2-1 and 150/20 formulas were unknown).
Cameron Haight at University of Michigan achieved the first long-term survivor in 1941—case 10 of 15 attempts.
Haight's survivor was a 12-day-old girl in good condition with IV hydration; she had aspirated barium but did not develop pneumonia, suggesting she was 'indestructible.'
Haight performed the operation under local anesthesia supplemented with open-drop ether, with the baby prone, via left thoracotomy with vertical paravertebral incision removing posterior segments of ribs 2–6.
Haight's left-sided approach required working around the aorta and subclavian artery; the field was not fully visible, requiring retraction shifts throughout the operation.
Postoperatively, Haight gave rectal sulfathiazole (an antibiotic), which was considered inadequate by modern standards (antibiotics should be given before incision, not after).
Saliva appeared in the drain a few days postoperatively; Haight placed a gastrostomy, then feeding began leaking from the gastrostomy into the drain.
Haight photographed his first survivor as a young teenager for his 1957 AATS presidential address; legend holds she was the last patient he saw before his death in 1970.
Haight probed the anastomosis by passing a catheter through the baby's mouth, which exited the wound, confirming a leak.
The Halifax explosion on December 6, 1917, was the most powerful man-made blast until the Trinity atomic bomb test in 1945.
William Ladd organized a convoy of 40 physicians and nurses to Halifax, arriving 2 days after the blast and staying for a month during December 1917–January 1918.
The first smallpox vaccination in 1796 was administered to an 8-year-old by Edward Jenner, a surgeon trained by John Hunter.
One of Crawford Long's first patients to receive ether anesthesia was a teenager undergoing finger amputation.
Joseph Lister's first patient to receive carbolic acid in 1865 was a 7-year-old with a compound fracture; 4 of his first 11 patients (reported 1867) were children.
The first splenectomy for blunt trauma was performed on a 14-year-old laborer who fell from a scaffold and ruptured his spleen.
Pyloric stenosis was first described by Hezekiah Beardsley in 1788 and later by Harald Hirschsprung in Denmark in 1888.
Hirschsprung's first two reported cases of pyloric stenosis were female infants, despite the disease predominantly affecting males.
Early treatment for pyloric stenosis included belladonna (for presumed pyloric spasm), bicarbonate gastric lavage (for acid secretions), and refeeding the child with vomited material.
The first operations for pyloric stenosis were gastroenterostomy and Heineke-Mikulicz pyloroplasty, adapted from adult operations for obstructing gastric cancer and peptic strictures.
Early pyloric stenosis operations had 50–65% mortality due to aspiration (full stomach, open-drop ether), gastric contents flooding the abdomen, and lack of resuscitation.
Fredet achieved 17% mortality through preoperative gastric decompression with a catheter, intraoperative warming, and postoperative fluid resuscitation by clysis (subcutaneous fluids).
Conrad Ramstedt performed the first modern pyloromyotomy in 1911 in Münster, Germany, on a nobleman's son. He attempted transverse closure but found the pylorus too stiff; when the channel gaped open, 'the thought shot through my head' that leaving it open was the correct approach.
Ramstedt's pyloromyotomy has been described as 'the most consistently successful operation ever described.'
Ramstedt's operation was slow to be adopted in England, only gaining acceptance in 1918 after World War I.
Early operations for Hirschsprung disease included colectomy, sigmoid straightening, and sympathectomy—all based on incorrect understanding of pathophysiology.
The first edition of Ladd and Gross's 'Abdominal Surgery of Infancy and Childhood' advocated sympathectomy for Hirschsprung disease, with impressive before-and-after photos showing distended-to-scaphoid abdomen.
Mary Elizabeth Tiffin, a pathologist at Stanford, rediscovered aganglionosis in the myenteric plexus in 1940 in a patient with short-segment Hirschsprung disease.
Orvar Swenson was hired by William Ladd to set up a research lab at Boston Children's Hospital and became interested in megacolon patients who were dying without effective treatment.
Swenson borrowed equipment from Sidney Farber to measure intestinal peristalsis and found that colon above a diverting colostomy had active peristalsis, while colon below the colostomy did not contract.
Swenson concluded that Hirschsprung obstruction was functional, not mechanical, demonstrating the physiological consequences of aganglionosis that Tiffin had found pathologically.
Swenson's pull-through operation was essentially a very low anterior resection, everting the rectum through the anus to complete the anastomosis outside the body.
Swenson asked radiologist Edwin Neuhauser to perform lateral (not AP) barium enema X-rays to visualize the transition zone, enabling preoperative diagnosis.
After Swenson's sixth patient remained obstructed post-operatively, he said 'Well, hell, let's do a biopsy,' discovering residual aganglionosis and establishing rectal biopsy for diagnosis and verification of adequate resection.
Robert Gross opposed Swenson's pull-through operation, fearing urinary incontinence and sexual dysfunction from extensive pelvic dissection.
Gross prohibited Swenson from performing rectal biopsies, fearing pelvic infections.
When Swenson proceeded with his operations despite Gross's opposition, he was forced out of Boston Children's Hospital within months; subsequent reports came from the Floating Hospital for Children and Tufts University.
The next edition of 'Surgery of Infancy and Childhood,' written solely by Gross after Ladd's retirement, credited Swenson's outstanding work and included diagrams of the Swenson procedure but attributed the lateral barium enema to Neuhauser and omitted mention of rectal biopsy.
The Soave endorectal pull-through procedure was first described by David Sabiston and Mark Ravitch at Hopkins in 1947 (for ulcerative colitis/familial polyposis in dogs), then performed in an adult with Hirschsprung disease by Asa Yancey in Tuskegee, Alabama, in 1952—11 years before Franco Soave's 1963 infant operation.
The Duhamel procedure was described in 1956, Keith Jorgenson performed the first laparoscopic pull-through in 1995, and de la Torre in Mexico City performed the first completely transanal pull-through in 1998.
Evarts Graham, Chair of Surgery at Washington University, was offered a 50-year-old man in cardiac failure as a PDA candidate by the pediatrics department, illustrating how little pediatricians valued surgery at the time.
Robert Gross and pediatric cardiologist John Hubbard, who trained as fellows together, agreed that PDA ligation was a solution for patent ductus arteriosus.
William Ladd absolutely forbade Gross from performing PDA ligation.
Gross waited until Ladd's August vacation, obtained permission from acting chief Thomas Lanman, and performed the first successful PDA ligation in 1938 on a 7-year-old under open-drop ether by anesthetist Betty Lank.
The PDA ligation took one hour in complete silence; the murmur was so loud it could almost be heard in the room, and it stopped after ligation was confirmed with a sterilized stethoscope.
When Ladd asked Gross 'How are things at the hospital?' at a club, Gross replied 'Nothing special,' concealing that he had just invented cardiac surgery.
Ladd fired Gross for insubordination and lying; Gross was reinstated only after hospital board intervention, but their relationship never recovered.
Helen Taussig heard Gross speak about PDA ligation and proposed creating a systemic-to-pulmonary fistula to palliate tetralogy of Fallot ('blue babies').