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 without 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 because it was the distal narrow colon that was the problem

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

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

The Suave procedure should properly be called the Yancey because Doctor Yancey first described submucosal dissection for Hirschsprung disease but published in a journal not widely read, while Suave published years later in a more widely read journal

The Suave/Yancey 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 complications were due to surgeons doing the operation wrong by dissecting too wide, not from the operation itself

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

A proper Swenson done right on the bowel wall avoids nerve injury because the nerves are in the fatty layer; dissecting too wide will injure them

Orvar Swenson died at age 105 and wrote letters to Mark Levitt and Alberto Pena asking them to tell everybody the Swenson is a good operation

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

The Duhamel is now only appropriate for an ileoduhamel, though the speaker would still do an ileoanal

Rabine did a low anterior resection for Hirschsprung leaving about 6 centimeters of aganglionic bowel behind, and some patients did perfectly fine with ganglionated bowel pooping through the aganglionic segment

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

The proper description is the Suave technique with the Boley modification, abbreviated as Suave-Boley

Hung Bae Kim figured out the STEP procedure

Doctor Henrys in the Philippines was the first surgeon to do a primary pull-through with no preceding stoma

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

Doctor Martin developed the Martin procedure, an expansion of the Duhamel leaving a longer aganglionic segment of rectum and pulling through ganglionated bowel for long-segment Hirschsprung disease

Doctor Martin published in 1977 the first description of applying endorectal pull-through techniques from Hirschsprung surgery to ulcerative colitis treatment, performing total proctocolectomy with ileoanal anastomosis using transanal dissection and Suave plane dissection

Helen Noblet from Melbourne, Australia figured out the suction rectal biopsy

Keith Jorgeson did the laparoscopic version of the Suave

In Jorgeson's original description with Tom Hinge, they talked about leaving a 5 centimeter cuff, which nowadays would be way too much

Jack Langer and Luis de la Torre developed transanal resection of the rectosigmoid with or without laparoscopy or laparotomy

Some places around the world are doing transanal-only approaches for Hirschsprung disease

Dan Teitelbaum did a significant amount of research in enterocolitis related to Hirschsprung disease