StayCurrentMD · Hirschsprung Disease: History
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Video14 min·Published Feb 2015Older

Hirschsprung Disease: History

With CCHMC Pediatric Surgery · hosted by Dr. Todd Ponsky

Chapter 1 of 5 · Fundamentals

Early theories

Early History and Initial Theories of Hirschsprung Disease

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What the experts said3 expert statements · 15 host summaries
Electric enemas were described around 1908 and involved passing a tube through the rectum with an electrode inside and another electrode on the abdomen, delivering about 40 milliamps of interrupted cycling current to facilitate expulsion of saline solution from the colon.
Clinical
Barry Shandling worked in Canada.
Clinical
Dr. Lester Martin was the chief of pediatric surgery at Cincinnati Children's Hospital.
Clinical
Harald Hirschsprung presented a paper on constipation in newborns due to dilation and hypertrophy of the colon in 1886 at the Society of Pediatrics in Berlin.
Host summaryThe host summarizing the discussion · not cited in answers
Dr. Hirschsprung was a pediatrician who developed the hydrostatic reduction of ileocolonic intussusception, a contribution unrelated to Hirschsprung's disease.
Host summaryThe host summarizing the discussion · not cited in answers
Early theories to explain Hirschsprung disease etiology included: obstruction (mechanical blockage from redundant colon or rectal valves), malformation (hypertrophied colon as primary congenital defect), and spastic distal colon causing functional obstruction.
Host summaryThe host summarizing the discussion · not cited in answers
The malformation theory (hypertrophied colon as primary defect) came first among early theories, but all theories were wrong because they focused on the dilated portion as diseased rather than as a consequence of the disease.
Host summaryThe host summarizing the discussion · not cited in answers
William Osler, one of the four founding professors of Johns Hopkins and creator of the residency system, proposed colostomy or rectal tube and irrigation as possible treatments for Hirschsprung's disease.
Host summaryThe host summarizing the discussion · not cited in answers
The finding of no ganglion cells in the narrow rectal sigmoid was finally recognized as the cause of Hirschsprung disease in 1946 by Orvar Swenson.
Host summaryThe host summarizing the discussion · not cited in answers
Others before Swenson, including Dr. Dalalé in 1920, recognized absent ganglion cells in the distal portion but thought it was an acquired condition rather than the cause of the disease.
Host summaryThe host summarizing the discussion · not cited in answers
Treatments used for Hirschsprung disease prior to 1946 included anal sphincter dilation, rectosigmoid myotomy, spinal anesthesia, lumbar sympathectomy, and electric enemas, but not resection of the distal non-dilated portion.
Host summaryThe host summarizing the discussion · not cited in answers
Barium enema technique became the standard diagnostic test for Hirschsprung's disease in 1948, with Dr. Swenson involved in this publication.
Host summaryThe host summarizing the discussion · not cited in answers
Barry Shandling proposed punch biopsies for newborns that required no closure nor anesthesia.
Host summaryThe host summarizing the discussion · not cited in answers
Dr. Sydegaard from Sweden performed the first successful operation for total colonic aganglionosis in 1953, doing a colon resection with an ileoanal anastomosis.
Host summaryThe host summarizing the discussion · not cited in answers
Swenson observed that when patients had a colostomy, the obstruction was relieved, but when the colostomy was closed, the disease returned.
Host summaryThe host summarizing the discussion · not cited in answers
Swenson scoped from the rectum in patients with colostomy and saw there was no true mechanical obstruction.
Host summaryThe host summarizing the discussion · not cited in answers
Swenson used a probe on the proximal stoma and observed normal peristalsis, but when he put a probe in the distal stoma, there was no peristalsis.
Host summaryThe host summarizing the discussion · not cited in answers
Swenson performed contrast studies and observed a non-dilated portion followed by a dilated portion, leading him to conclude that the distal portion was the diseased one.
Host summaryThe host summarizing the discussion · not cited in answers