Colorectal Quiz: Episode 44 - HD Frozen Section

Late-onset Hirschsprung disease patients tend to present with more subtle symptoms, more constipation, and less enterocolitis compared to neonatal presentations

Frozen section pathology for Hirschsprung disease can be misleading; marking biopsy sites with prolene stitches allows for reliable permanent pathology before pull-through

If biopsy sites are not marked, it is very hard to find the biopsies in the future

Laparoscopic approach allows preservation of the colonic arcade without overstretching the anus and provides a well-perfused, tension-free pull-through

The laparoscopic portion of Hirschsprung pull-through minimizes the amount of anal stretch required and is very precise and elegant

In older children with Hirschsprung disease where the transition zone level is confirmed, transanal-only approach may be considered because you know exactly where the transition zone is and can reach it

Taking biopsies at the time of ileostomy can result in adhesions of former biopsy sites with the lateral abdominal wall, which is a disadvantage

The anastomotic donut should be cut into 4 quadrants to ensure ganglion cells are present in all quadrants, because the transition zone is sometimes like a lip and not circumferential

Sending the entire donut for pathology analysis is a lot of work for pathologists and is used more as final pathology confirmation rather than intraoperative decision-making

The common teaching to go 5 centimeters above the transition zone is not actually true; there is a longer transition zone in many cases

There are advantages to having the ultimate pull-through nice and straight (usually upper sigmoid) if you ever need to irrigate

It is very important to send a cube-shaped full thickness specimen, not a diamond shape; the seromuscular layer square needs to be the same size square as the mucosa to prevent errors from tangential cutting

Ganglionosis should be visualized like a paint can where ganglion cells are dripping down the sides, not as concentric circles; you could theoretically biopsy in a transition zone where there is only a drip of paint

Pathologists will refuse to do analysis unless you give them submucosa; checking seromuscular layer only is a cardinal sin because it could have ganglion cells while the submucosal layer has hypertrophic nerves

Pathologists doing it right need to check all levels and confirm that not only is the seromuscular layer good, but the submucosal layer is good

After a couple of weeks post-operatively, there is no chance to go back in again for re-operation, and results of reoperative transanal pull-throughs are not great with high rates of anastomotic dehiscence

One reason for high dehiscence rates in delayed reoperative surgery is that you can never re-enter the anal canal in the same plane

If you go back early, you can release the stitches and arrive in the same plane again to do re-anastomosis to the anal canal exactly in the same plane where you went in initially, which is why this case did not face any dehiscence

Reoperative Hirschsprung transanal surgery is not as complicated if you go back in early

The surgical lesson is that it is hard to stop and say time out, I need to back up and solve the problem now and not let it cascade