The Colorectal Quiz Episode 21: The History of Hirschsprung Disease
Harold Hirschsprung identified that a baby could be sick due to this problem but did not understand the pathology.
clinicalMarc Levitt0:26 ↗
The disease is named Hirschsprung disease, not apostrophe S.
guidelineMarc Levitt0:26 ↗
Orvar Swenson figured out the pathology by going to the pathology lab and defined the fact that there were no ganglion cells.
clinicalMarc Levitt0:26 ↗
Prior to Swenson's work, removal of the dilated colon was the treatment, which was a mistake; it was the distal narrow colon that was the problem.
clinicalMarc Levitt0:26 ↗
Swenson developed the first operation for Hirschsprung disease, a full-thickness rectal dissection.
clinicalMarc Levitt0:26 ↗
Some surgeons still do Suave procedures, but they are becoming more Swenson-like with maybe a one-centimeter cuff; Dan von Allman calls those 'Suavesons with a one-centimeter cuff.'
opinionMarc Levitt0:26 ↗
Dr. Yancey was the first surgeon to describe a submucosal dissection for Hirschsprung disease, but published in a journal not widely read; Dr. Suave published later in a more widely read journal, so the technique is called Suave rather than Yancey.
clinicalMarc Levitt0:26 ↗
The Suave technique was developed because people said the Swenson caused fecal and urinary incontinence or voiding dysfunction; Swenson himself wrote that the operation was good but surgeons were doing it wrong by dissecting too wide.
clinicalMarc Levitt0:26 ↗
Doing a proper Swenson right on the bowel wall, like a PSARP right on the bowel wall, avoids nerve injury; if you see fat you can get closer, as the nerves are in the fatty layer.
clinicalMarc Levitt0:26 ↗
Swenson was 105 when he died and used to write letters to Levitt and Alberto Pena asking them to promote the Swenson operation.
clinicalMarc Levitt0:26 ↗
Duhamel had the idea to leave the original rectum behind and do a pull-through next to it, then mate the two lumens.
clinicalMarc Levitt0:26 ↗
The Duhamel is now really only appropriate for an ilio-Duhamel, although Levitt would still do an ilioanal.
opinionMarc Levitt0:26 ↗
Rabine did a low anterior resection for Hirschsprung disease, leaving about six centimeters behind; some patients did fine as ganglionated bowel pooped through the six centimeters of aganglionated bowel, but that operation has gone to the wayside.
clinicalMarc Levitt0:26 ↗
Dr. Boley was the first to do the primary coloanal anastomosis of a Suave, eliminating the need to leave the bowel hanging out and come back at day seven; the proper description is the Suave technique with the Boley modification, i.e., a Suave-Boley.
clinicalMarc Levitt5:57 ↗
Henry So was a pediatric surgeon in the Philippines and the first to do a primary pull-through (transabdominal) with no preceding stoma, because patients with stomas at home in the Philippines faced such social stigma that babies were basically left to die by their families.
clinicalMarc Levitt5:57 ↗
Dr. Martin was the first surgeon-in-chief and pediatric surgeon at Cincinnati Children's Hospital.
clinicalJason Frischer9:40 ↗
Dr. Martin developed the Martin procedure, an expansion of the Duhamel for long-segment Hirschsprung disease, leaving a longer aganglionated segment of rectum and pulling through ganglionated bowel.
clinicalJason Frischer9:40 ↗
Dr. Martin's biggest contribution was in ulcerative colitis; in 1977, before the J-pouch, he took the endorectal pull-through technique used in Hirschsprung disease and applied it to ulcerative colitis, doing a total proctocolectomy with ilioanal anastomosis.
clinicalMarc Levitt10:34 ↗
The transanal dissection and Suave plane dissection is the same concept as the mucosectomy in ulcerative colitis.
clinicalMarc Levitt10:34 ↗
Helen Noblet figured out the suction rectal biopsy; she is from Melbourne, Australia.
clinicalMarc Levitt11:41 ↗
Keith Jorgensen did the laparoscopic version of the Suave; in his original description (with Tom Inge on the paper), they talked about leaving a five-centimeter cuff, which nowadays would be way too much.
clinicalMarc Levitt11:41 ↗
Jack Langer approached Hirschsprung disease transanally, doing a transanal resection of the rectosigmoid with or without laparoscopy or laparotomy.
clinicalMarc Levitt11:41 ↗
Luis de la Torre also did transanal resection around the same time as Jack Langer; some places around the world are doing transanal only, and Levitt does that in certain circumstances.
clinicalMarc Levitt11:41 ↗
Dan Teitelbaum did an incredible amount of work in Hirschsprung disease, particularly significant research in enterocolitis; he passed away from a brain tumor.
clinicalMarc Levitt11:41 ↗
Isotonic maintenance fluid: Practice Gap discussion at Update Course 2018
Iatrogenic hyponatremia is one of the biggest problems in hospitals, related to ADH physiology and the use of hypotonic fluids rather than normal saline.
clinical1:23 ↗
Children become hyponatremic because they are given half normal saline, and the data on this are clear.
clinical1:23 ↗
The correct maintenance fluid for a 15-year-old post-colectomy patient is D5 normal saline with 20 milliequivalents of potassium.
guideline2:05 ↗
The American Academy of Pediatrics is coming out with a consensus statement on isotonic maintenance fluids (anticipated as of March).
guideline2:31 ↗
Hospitalized children develop hyponatremia because they receive less than isotonic (hypotonic) solutions, leading to cerebral edema and other complications.
clinical2:31 ↗
The traditional teaching was to resuscitate with isotonic fluid and then convert to hypotonic maintenance fluids; the new practice is to keep patients on isotonic fluid to prevent hyponatremia.
guideline2:58 ↗