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History of Hirschsprung Disease
With Dr. Alberto Pena · hosted by Dr. Todd Ponsky & Dr. Andrea Bischoff
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
The most serious challenge in Hirschsprung disease is the basic science approach to solve the problem of enterocolitis and many other problems affecting children with the disease.
Hirschsprung disease is not only about ganglion cells or no ganglion cells; it is a much more complex condition.
Electric enemas were described around 1908, involving 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.
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.
Harald Hirschsprung was a pediatrician who developed the hydrostatic reduction of ileocolonic intussusception.
Early theories attempting to explain Hirschsprung disease etiology were all wrong because everyone was obsessed that the dilated portion was the diseased one, trying to explain why the dilated portion was the cause rather than the consequence of the disease.
William Osler, one of the four founding professors of Johns Hopkins and creator of the residency, proposed colostomy or rectal tube and irrigation as possible treatments for Hirschsprung disease.
In 1946, Orvar Swenson finally recognized the finding of no ganglion cells in the narrow rectal sigmoid as the cause of Hirschsprung disease, whereas others before him had noted absent ganglion cells but thought it was an acquired condition.
Resection of the distal non-dilated portion was not used as a treatment for Hirschsprung disease prior to 1946.
Barium enema technique became the standard diagnostic test for Hirschsprung disease in 1948, with Dr. Swenson involved in this publication.
Barry Shandling, who worked in Canada, proposed punch biopsies for newborns that required no closure nor anesthesia.
Dr. Syndergaard from Sweden performed the first successful operation for total colonic aganglionosis in 1953, doing a colon resection with an ileo-anal anastomosis.
Dr. Swenson observed that when patients had a colostomy the obstruction was relieved, but after closing the colostomy the disease returned.
Dr. Swenson scoped from the rectum when the patient had a colostomy and saw there was no true obstruction.
Dr. Swenson used a probe on the proximal stoma and saw normal peristalsis, but when he put a probe in the distal stoma he saw there was no peristalsis.
Dr. Swenson performed a contrast study and observed a non-dilated portion followed by a dilated portion, leading him to conclude that the distal portion was the diseased one.