The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique

You can always get out of the OR without doing something permanent

On frozen section, you can rule out Hirschsprung disease but cannot rule it in

Hypertrophic nerves are mainly a sacral nerve plexus entity, found in sigmoid and rectum, not in more proximal colon

If you are going to do a primary pull-through 4 days later waiting for permanent sections, you must ensure the child is being irrigated and doing well with irrigation, not having smoldering or low-grade enterocolitis features

An ileostomy almost definitely is going to successfully divert the patient, whereas with a diverting colostomy based on frozen section there is concern it may not be at a good level

In parts of the world without pathology support, diverting in the dilated segment is a very reasonable strategy, and if that bowel works, that is where the pull-through will go

Biopsies should be marked with permanent suture using different numbers of tails for each biopsy site to enable identification at subsequent operation

If the colon is incredibly backed up with chalky stool after several months, irrigations will not do anything, but if you don't have that issue the colon can stay without needing a way to irrigate it

If you don't have ganglion cells and you're at the splenic flexure, you should not do a pull-through that day and need to wait for permanent section

There are functional outcome differences if you start pulling through transverse colon versus pulling through left-sided colon

If you have even a little bit of colon, if you have your right colon, you can make one formed stool per day

For mid-transverse pull-through, the blood supply is based on right colic artery

When bringing down mid-transverse colon, you need to de-rotate the bowel, otherwise you will bring the mesentery right across the duodenum and create an obstruction

For mid-transverse pull-through, you need to ligate the middle colic and very likely ligate the right colic, with blood supply dependent on ileocolic and the marginal artery paralleling the right colon

For proximal pull-through, you de-rotate by putting the cecum at the liver bed and bringing the pull-through down the right side, with all small bowel on the left side, which is essentially a rotation opposite to a Ladd's procedure

When doing a proximal pull-through, there is a slight twist in the mesentery when pulling it down, so ensuring adequate blood supply without kinking is very important

Personal preference is to do proximal pull-through open, probably through the incision for ileostomy closure, though some have done it laparoscopically