The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique

Surgeons can always leave the operating room without doing something permanent when faced with uncertain intraoperative findings.

The hypertrophic nerve concept is mainly a sacral nerve plexus entity, occurring in sigmoid and rectum, so hypertrophic nerves are not a concern for more proximal disease.

If choosing to wait for permanent sections before pull-through (rather than diverting), the child must be doing well with irrigations, as delaying diversion in a child with smoldering or low-grade enterocolitis features is inappropriate.

For proximal disease with uncertain frozen sections, ileostomy diversion is preferred over colostomy because it will almost definitely divert successfully, whereas colostomy based on frozen section may not be at the correct level.

When performing ileostomy for proximal disease, a biopsy from the ileum should be sent.

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

When performing colonic mapping, biopsy sites should be marked with permanent suture using different numbers of tails for each biopsy to enable identification months later.

If a child with ileostomy and defunctionalized colon presents months later with severely backed-up, chalky stool that cannot be cleared with irrigations, the colon may need to be addressed, but this is a small population.

For proximal Hirschsprung disease with transition zone at splenic flexure or beyond, surgeons should not perform pull-through on the day of initial operation but should wait for permanent sections.

There are functional outcome differences between pulling through transverse colon versus left-sided colon.

If a patient has even a little bit of colon—specifically if they have their right colon—they can make one formed stool per day.

For mid-transverse colon pull-through, the blood supply is based on right colic artery, but to bring the colon down without creating duodenal obstruction, the bowel must be de-rotated.

For mid-transverse pull-through, the middle colic and very likely the right colic arteries must be ligated, making blood supply dependent on the ileocolic artery and the marginal artery paralleling the right colon.

The de-rotation for proximal pull-through involves placing the cecum at the liver bed, bringing the pull-through down the right side, and putting all small bowel on the left side—essentially a rotation opposite to a Ladd's procedure.

When performing de-rotation for proximal pull-through, there is a slight twist in the mesentery, so ensuring adequate blood supply without kinking is very important.

For proximal Hirschsprung pull-through, the preferred approach is open surgery, likely through the ileostomy closure incision, though some surgeons have performed it laparoscopically.