The Colorectal Quiz Episode 23: Hirschsprung Disease - The Soiling Patient...
With Dr. Doctor Levitt & Dr. Doctor Hira Ahmad & Dr. Jason Fisher · hosted by Dr. Amanda Jensen · StayCurrentMD
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)
Patients who have voluntary bowel movements during the day but accidents at night indicate working external sphincters but non-functioning internal sphincters
The dentate line represents the transition from squamous epithelium to columnar epithelium, occurring about 2/3 of the way up the anal canal
The dentate line region contains nerves that provide sensation to distinguish gas, liquid, and solid stool and determine how hard and how long to squeeze
Proprioception from rectal stretch is the signal that stool is accumulating and triggers external sphincter control
In anorectal malformation patients, giving stool softeners is problematic because patients never feel the stretch and loose stool just flows out
ARM and Hirschsprung patients are better off with bulk stool kicked out by a laxative than stool softener that slowly oozes out
The ability to sense stool in the rectum or neorectum region is critical for success in children with anorectal malformation, Hirschsprung disease, or spinal conditions
Making stool too soft or loose puts patients on the edge of control over the edge into incontinence
Loose stool is hard to control even with completely normal continence because you cannot detect it reliably without bulk
Patients with Hirschsprung disease are dependent on stretch sensation because their rectum has been removed and the sigmoid has taken over that job
Patients with missing dentate line can develop bowel control if their sphincters are working, but they will be very sensitive to loose stool
A missing dentate line scenario is similar to an anorectal malformation anastomosis (rectum or colon mucosa to skin)
Hirschsprung is an obstruction problem, but getting patients clean afterward is a separate and independent challenge
The vast majority of Hirschsprung patients get clean on their own and have great success stories
Cyanoacrylate-based barriers are very helpful for skin care in hypermotile patients
Wound care improvements for perineums in Hirschsprung disease and hypermotile patients have dramatically improved over the last 4-5 years
Proton pump inhibitors help reduce the acidity of stool in hypermotile patients
Water-soluble fiber produces bulk stool, which is preferred over water-insoluble fiber
Loperamide dosing is 0.5 to 0.8 mg per kilogram divided daily
Hyoscyamine (Levsin) is dosed at 0.125 mg tablet every six hours
Diphenoxylate atropine (Lomotil) is almost never used because it has cardiac side effects
Tincture of opium is useful but difficult to prescribe because it is a controlled substance
Some Hirschsprung patients with good operations have super strong sphincters that need relaxation to allow stool passage until they learn proper coordination
Anorectal manometry can provide objective data showing resting pressures are usually on the higher end of normal in these patients
Patients with Hirschsprung disease are very sensitive to some foods, particularly lactose
Of all soiling patient groups (anorectal malformation, Hirschsprung, functional constipation, and spinal), Hirschsprung is the hardest group because the sphincters are so troublesome
Within Hirschsprung patients, hypermotile patients are much harder to manage than hypomotile patients
With systematic strategies, many Hirschsprung patients who were told they could never be clean can achieve continence