The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique

The nightmare scenario is not finding ganglion cells on frozen section (especially when not finding nerves) when in fact ganglion cells are present, leading to unnecessary resection of good colon.

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

If doing primary pull-through several days after mapping (waiting for permanent sections), ensure the child is doing well with irrigations and not having smoldering enterocolitis, which would warrant immediate diversion.

Ileostomy is preferred over colostomy when uncertain about transition zone level because ileostomy will almost definitely divert successfully.

When performing ileostomy for uncertain proximal disease, send a biopsy from the ileum to confirm it is ganglionic.

In settings without pathology support, empiric diversion in the dilated segment is a reasonable strategy; if that bowel works, that is where the pull-through will go.

Mark biopsy sites with permanent suture using different numbers of tails for each site and document in operative report.

If planning to wait several months before pull-through and not diverting the colon, consider whether the colon needs to be beaten (decompressed), though the answer is uncertain.

Most defunctionalized colons can stay without needing irrigation access; only a small population will need the colon addressed if it becomes severely backed up with chalky stool.

If frozen sections show no ganglion cells at the splenic flexure, do not proceed with pull-through that day; wait for permanent sections.

There are functional outcome differences between pulling through transverse colon versus 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 colon transition zone, the pull-through blood supply is based on right colic vessels, and the middle colic must be ligated.

To bring mid-transverse colon down for pull-through, the bowel must be de-rotated to avoid bringing the mesentery across the duodenum and creating obstruction.

For transverse colon pull-through, ligate middle colic and very likely right colic; blood supply depends on ileocolic and the marginal artery paralleling the right colon.

De-rotation for transverse colon pull-through places the cecum at the liver bed, brings the pull-through down the right side, and puts small bowel on the left side — opposite rotation from Ladd's procedure.

When pulling transverse colon down, there is a slight twist in the mesentery, so ensuring adequate blood supply without kinking is critical.

Preference is to perform proximal Hirschsprung pull-through open, possibly through the ileostomy closure incision, though some do it laparoscopically.