From
Colorectal Channel
The Colorectal Quiz Episode 21: The History of Hirschsprung Disease
With Dr. Marc Levitt & Dr. Jason Frischer · hosted by Dr. Amanda Jensen
Chapter 1 of 7 · In-Depth Reviews
Introduction
Introduction and Overview
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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
Harold Hirschsprung identified that a baby could be sick due to this problem but did not understand the pathology.
The correct nomenclature is 'Hirschsprung disease' without an apostrophe S.
Orvar Swenson defined the pathology by discovering the absence of ganglion cells in the pathology lab.
Prior to Swenson's discovery, removal of the dilated colon was the treatment, which was a mistake because the distal narrow colon was the actual problem.
Swenson developed the first operation for Hirschsprung disease, which is a full-thickness rectal dissection.
Doctor Yancey was the first surgeon to describe a submucosal dissection for Hirschsprung disease, but published in a journal that few people read.
Doctor Suave published his article on submucosal dissection years later in a more widely-read journal, which is why the procedure bears his name rather than Yancey's.
The Suave and Duhamel procedures were developed because surgeons believed the Swenson operation caused fecal and urinary incontinence or voiding dysfunction.
A proper Swenson dissection should be performed right on the bowel wall (similar to a PSARP); if fat is visible, the dissection can be closer, as the nerves are in the fatty layer.
Dissecting too wide during a Swenson procedure will injure the nervi erigentes.
Orvar Swenson lived to age 105 and wrote letters to Dr. Levitt and Alberto Pena asking them to promote the Swenson operation.
Duhamel's technique involves leaving the original rectum behind, performing a pull-through next to it, and then mating the two lumens.
The Duhamel procedure is now only appropriate for an ileo-Duhamel, though Dr. Levitt would still perform an ileoanal anastomosis.
Rabine performed a low anterior resection for Hirschsprung disease, leaving about 6 centimeters of aganglionic bowel behind.
Some patients who underwent Rabine's procedure did well, with ganglionated bowel functioning through 6 centimeters of aganglionic bowel, but the operation is no longer performed.
Doctor Boley was the first to perform a primary coloanal anastomosis of a Suave procedure, eliminating the need to leave the bowel hanging out and return at day 7.
The proper description of the modified Suave technique is 'the Suave technique with the Boley modification' or 'Suave-Boley.'
Henry So was the first surgeon to perform a primary pull-through for Hirschsprung disease without a preceding stoma.
Henry So performed primary pull-throughs out of desperation because in the Philippines, babies with stomas faced such severe social stigma that families would leave them to die.
Doctor Martin developed the Martin procedure, an extended Duhamel procedure that leaves a longer aganglionic segment of rectum for long-segment Hirschsprung disease.
In 1977, Doctor Martin was the first to apply the endorectal pull-through technique used in Hirschsprung disease to the surgical treatment of ulcerative colitis, performing total proctocolectomy with ileoanal anastomosis.
Doctor Martin's ulcerative colitis technique predated the J-pouch, which later modified his approach.
The transanal dissection used in the Suave procedure is the same concept as the mucosectomy performed in ulcerative colitis surgery.
Helen Noblett from Melbourne, Australia, developed the suction rectal biopsy technique.
Keith Jorgeson performed the first laparoscopic version of the Suave procedure.
In Jorgeson's original description of the laparoscopic Suave (co-authored with Tom Inge), they described leaving a 5-centimeter cuff, which would now be considered too long.
Jack Langer and Luis de la Torre developed transanal approaches to perform rectosigmoid resection with or without laparoscopy or laparotomy.
Some centers around the world now perform transanal-only approaches for Hirschsprung disease, which Dr. Levitt uses in certain circumstances.
Dan Teitelbaum performed significant research on enterocolitis in Hirschsprung disease before his death from a brain tumor.
Modern Suave procedures are becoming more Swenson-like by making only a 1-centimeter cuff, which Dan von Almen describes as 'basically Swensons with a 1-centimeter cuff.'
Doctor Swenson argued that complications attributed to his operation were due to improper technique—specifically, dissecting too wide—rather than the operation itself.