The Colorectal Quiz Episode 21: The History of Hirschsprung Disease

Harold Hirschsprung figured out that a baby could be sick due to this problem but did not understand the pathology.

The correct name is Hirschsprung disease, not apostrophe S.

Orvar Swenson figured out the pathology by going to the pathology lab and defined the fact that there were no ganglion cells.

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.

Swenson developed the first operation for Hirschsprung's disease, which is a full thickness rectal dissection.

Modern Suave operations are becoming more Swenson-like, making maybe a 1 centimeter cuff, which Dan von Almen describes as basically Swensons with a 1 centimeter cuff.

The operation ought not to be called the Suave because Doctor Yancey was the first surgeon who described a submucosal dissection for Hirschsprung's disease, but he published in a journal that not many people read, while Suave published years later in a more widely-read journal.

The Yancey/Suave technique was developed because people said the Swenson caused fecal and urinary incontinence or voiding dysfunction after the operation.

Doctor Swenson wrote a paper saying the critics were wrong, it's a good operation, and they were doing it wrong by dissecting too wide.

Yancey, Suave, and Duhamel did everything in their power to avoid the full thickness rectal dissection and stay out of that rectal plane to avoid injury to the nerve erigentes.

Doing a proper Swenson right on the bowel wall—if you see fat, you can get closer—keeps you away from the nerves which are in the fatty layer; dissecting too wide will injure them.

Swenson used to write letters to Mark Levitt and Alberto Pena thanking them for promoting the Swenson and asking them to tell everybody it's a good operation; he was 105 when he died.

Duhamel had the idea to leave the original rectum behind and do a pull-through next to it, then mate the two lumens.

At this point, the Duhamel is really only appropriate for an ileoduhamel, although Levitt would still do an ileoanal.

Rabine did a low anterior resection for Hirschsprung's, leaving about 6 centimeters behind; amazingly, some of those patients did perfectly fine with ganglionated bowel pooping through the 6 centimeters of aganglionic bowel.

Doctor Boley was the first one to do the primary coloanal anastomosis of a Suave, eliminating the need to leave the bowel hanging out and come back at day 7.

The proper description is the Suave technique with the Boley modification, i.e., a Suave-Boley.

Hung Bae Kim is the person that figured out the STEP procedure.

Henrys was a pediatric surgeon in the Philippines and was the first surgeon to do a primary pull-through—a transabdominal operation with no preceding stoma.

Henrys did the primary pull-through because patients who were at home with stomas in the Philippines were not cared for due to social stigma, and the babies were basically left to die by their families.

Doctor Martin developed the Martin procedure, which is an expansion of the Duhamel procedure—leaving a longer aganglionic segment of rectum and pulling through ganglionated bowel for long segment Hirschsprung's disease.

Martin's biggest contribution was to ulcerative colitis; in 1977, before the J pouch, he took the endorectal pull-through technique from Hirschsprung's disease and transferred it to the surgical treatment of ulcerative colitis, doing a total proctocolectomy with ileoanal anastomosis.

The transanal dissection, the Suave plane dissection, is the same concept as the mucosectomy in ulcerative colitis.

Helen Noblet is the one who figured out the suction rectal biopsy; she's from Melbourne, Australia.

Keith Jorgeson is the one that did the laparoscopic version of the Suave; in his original description with Tom Hinge, they talked about leaving a 5 centimeter cuff, which nowadays would be way too much.

Jack Langer approached transanally to do a transanal resection of the rectosigmoid with or without laparoscopy or laparotomy; Luis de la Torre did the same around the same time.

Some places around the world are doing transanal only; Levitt does that in certain circumstances.

Dan Teitelbaum did an incredible amount of work in Hirschsprung's disease and particularly a significant amount of research in enterocolitis.