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The Colorectal Quiz Episode 23: Hirschsprung Disease - The Soiling Patient...
With Dr. Marc Levitt & Dr. Hira Ahmad & Dr. Jason Frischer · hosted by Dr. Amanda Jensen
Chapter 1 of 7 · Fundamentals
Continence components
Introduction and Review of Continence Components
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
The three components of continence are quality of sphincters, quality of dentate line, and motility.
In Hirschsprung disease, two sphincters are of concern: the external sphincter (under voluntary control) and the internal sphincter (which tends not to relax due to absent rectoanal inhibitory reflex).
If both internal and external sphincters have been overstretched, both become problematic.
A patient who has voluntary bowel movements during the day but soils at night indicates working external sphincters but non-functioning internal sphincters; when sleeping, they relax the external sphincter and lose control.
Loss of dentate line can occur with overstretching and some preservation of external sphincter, resulting in daytime control but nighttime soiling.
The dentate (or pectinate) line is the transition from squamous epithelium to columnar epithelium, located about 2/3 of the way up the anal canal.
Blood supply changes occur at the dentate line (splanchnic versus systemic), and there is also innervation in that area.
The nerves located at the dentate line region tell you gas, liquid, solid; how hard, how long, and how tight to squeeze.
Preserving the dentate line region is key because injury to that region affects a patient's ability to be continent.
The rectum (not the anal canal) has proprioception capacity to detect stretch, which is the signal that stool is accumulating and it's time to hold stool and find a bathroom.
In anorectal malformation patients, the rectum should be preserved because rectal stretch provides proprioception.
When the internal sphincter is functioning properly, it relaxes at the time of rectal stretch.
Giving anorectal malformation patients stool softeners is problematic because they never feel the stretch; loose stool just flows and they will never have control of that.
ARM patients are much better off with bulk (kicked out by a laxative) than a stool softener that slowly oozes out.
In children with anorectal malformation, Hirschsprung disease, spinal conditions, or combinations thereof, the ability to sense stool in the rectum or neorectum region is critical to success; the right consistency and bulk of stool is very important.
If clinicians make stool too soft or too loose with medications, they put a child on the edge of having control or not and throw them over that edge, preventing success.
Loose stool is the enemy if you have borderline continence or even completely normal continence, because you don't know for sure that it's there.
We are very dependent on the stretch and bulk of stool in the rectum; that's when the external sphincter goes into motion and the internal sphincter relaxes.
Hirschsprung patients with absolutely intact sphincters are dependent on that stretch; they don't have a rectum (it's been removed) and their sigmoid has taken over that job.
Hirschsprung patients with injured sphincters are particularly in trouble regarding dependence on stool bulk and stretch.
A patient with missing dentate line can develop bowel control provided their sphincters are working, but they will be very sensitive to loose stool.
Patients with missing dentate line won't be good at detecting that something is there unless they have bulk, but with the right diet, right stool consistency, and intact sphincters, they can achieve control.
A Hirschsprung patient with missing dentate line and intact muscle should be able to achieve continence, similar to an anorectal malformation anastomosis (rectum or colon mucosa to skin).
Patients with no dentate line and patulous sphincters who are soiling develop severe skin irritation from sitting in pull-ups or diapers.
Patients with horrific diaper rash and perineal excoriation related to no dentate line and no intact sphincters need temporary stomas; some may need permanent stomas.
In a soiling Hirschsprung patient with no obstruction, no distention, no enterocolitis, and 3 stools a day, that's a patient who needs management of a slow-moving colon.
In both scenarios (intact or deficient sphincters/dentate line), patients with slow-moving colon need a bowel management program; if sphincters are poor and dentate line deficient, mechanical emptying with enema program is needed; if intact, can start mechanical and potentially switch to laxative program.
For hypermotile patients (stooling 7-8 times a day), the approach is to constipate them first, then figure out how to empty them in a time-controlled fashion.
Whether hypermotile patients can empty on their own versus needing mechanical emptying to maintain social continence depends on their sphincter function.
Hirschsprung disease is an obstruction problem; once that's solved, the tough part is getting patients clean—two separate and independent challenges.
The vast majority of Hirschsprung patients get clean on their own and have great success stories.
Clinicians need to determine if Hirschsprung patients are too slow or too fast, how to manipulate their motility, and understand if they have the mechanisms needed for continence (sphincters and dentate line).
For hypermotile patients, skin care is vitally important; a cyanoacrylate-based barrier is very helpful.
Wound care improvements for perineums in Hirschsprung or any hypermotile patient have dramatically improved over the last 4-5 years.
Proton pump inhibitors are helpful to reduce the acidity of stool in hypermotile patients.
Some antacid products can be taken orally as liquid and put on the skin to reduce acidity and help excoriation.
Small volume enemas are a very helpful maneuver for hypermotile patients.
Water-soluble fiber (not water-insoluble) produces bulky stool and is helpful for hypermotile patients.
Loperamide is very helpful medicine for hypermotile patients; the maximum dose is 0.5 to 0.8 mg per kilogram divided daily based on patient weight.
Cholestyramine is the next level of treatment after loperamide for hypermotile patients.
Hyoscyamine (Levsin) 0.125 mg tablet every six hours has been used for hypermotile patients.
Diphenoxylate-atropine (Lomotil) is almost never used because it has cardiac side effects.
Tincture of opium is useful for slowing stool but is a controlled substance and difficult to get prescribed.
If everything checks out with the pull-through and patients are still not emptying, Botox may be needed to help patients train and control their non-relaxing sphincters rather than being withholders.
Some Hirschsprung patients who have a good operation have super strong sphincters that just need a little relaxation to allow passage of stool until they learn proper sphincter coordination for evacuation.
Botox helps train sphincters in Hirschsprung patients; anorectal manometry can provide objective data showing resting pressures are usually on the higher end of normal, and some patients need extra relaxation to allow them to go.
Nutrition in Hirschsprung patients is very important; some patients are very sensitive to certain foods, particularly lactose.
Paying attention to diet (whether breastfed infants or older children) is important because lactose is particularly problematic in some Hirschsprung patients.
Every Hirschsprung patient should be able to do well; they might need help and might need mechanical evacuations or Botox, but all should be able to get on the right track.
Of all soiling patients (anorectal malformation, Hirschsprung, functional constipation, spinal), the hardest group is definitely Hirschsprung without question, because the sphincters are so troublesome.
Of Hirschsprung patients, the hypermotile are much harder to manage than the hypomotile.
With systematic strategies—knowing if patients have potential for bowel control and manipulating motility accordingly—many Hirschsprung patients who were told they could never be clean can achieve cleanliness.