The Colorectal Quiz Episode 22: Hirschsprung Disease - the Soiling Patient Part 1

There are two types of problematic post-pull-through Hirschsprung's patients: obstructed patients (not emptying, distention, enterocolitis, failure to thrive) and soiling patients (pooping constantly, never distended, minimal constipation).

If the pull-through is done correctly with no anatomic problems and preservation of the sphincter mechanism, most Hirschsprung's patients do extremely well, though some need medical treatment to manage constipation.

There is no reason why any Hirschsprung patient should be obstructed or soiling if properly managed; if they are, investigation is needed to identify and fix the problem.

Pathology can change over time; cases exist where ganglion cells were present at pull-through but absent with hypertrophic nerves 7 years later when problems developed.

A pull-through can decompensate if the patient does not have adequate medical management or sphincter management, potentially leading to nerve hypertrophy.

The most important question for any soiling patient is: what is the patient's potential for bowel control? This question is often neglected.

All Hirschsprung's patients have the best possible potential for bowel control because they were born with normal sphincters (sometimes too strong and non-relaxing, but not lax) and intact dentate line with preserved anal canal sensation.

When a Hirschsprung's patient has an intact dentate line and intact sphincters, they have full potential for voluntary bowel movements and bowel control.

If the dentate line is lost (because dissection was started too low) or sphincters were overstretched and don't contract well, the patient may have lost their potential for bowel control.

3D anorectal manometry can objectively assess whether sphincters are intact, whether the patient has a good squeeze, and whether that squeeze is concentric.

Sphincters should be assessed with the patient awake; examination under anesthesia makes it harder to assess sphincter function.

Sphincters become overstretched from transanal approach with deep dissection, wrong plane, or retractors placed in the anus; overstretched sphincters will not return to normal.

Patients with poor potential for bowel control (like anorectal malformation with poor spine/sacrum or spina bifida) need to be in a dedicated bowel management program with enemas or antegrade options.

Patients with lost potential for bowel control (injured sphincters or lost dentate line) need a mechanical emptying program.

A mechanical program can get borderline patients clean and psychologically motivated to be clean, making them more likely to successfully potty train.

Mark Levitt's current routine is to perform 3D anorectal manometry in all soiling Hirschsprung's patients to assess squeeze quality and dentate line presence, then decide whether to attempt potty training with laxatives or start mechanical cleaning.

A new sphincter tightening technique has been developed and published in JPS with great results in multiple patients with disrupted or patulous sphincters.

Rectal prolapse after Hirschsprung's pull-through is iatrogenic and should never occur if sphincters are preserved; it results from overstretched sphincters becoming patulous to the point of laxity.

During normal bowel movements, sphincters relax and the anus opens with some mucosal descent, but as soon as sphincters tighten, the mucosa retracts back in; prolapse at rest indicates damaged muscles and/or mucosa brought too low at anastomosis.

For patients with patulous sphincters, Mark Levitt would offer sphincter reconstruction and perform a Malone at the same time, as the Malone can serve as a bridge to continence by allowing patients to practice holding and releasing flushes on command.