From
Colorectal Channel
Colorectal Quiz: Episode 42 - HD Constipation
With Dr. Chris Gayer · hosted by Dr. Felipe Glu
Chapter 1 of 5 · Case-Based Learning
Case presentation
Case presentation: 20-year-old with post-Hirschsprung constipation
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Host summaries · secondary, not cited in answers
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
Felipe Glu is a colorectal research fellow at Children's National Hospital
Chris Geyer runs a colorectal program at Children's Hospital Los Angeles
Hirschsprung's disease is a very anatomically fixable problem and with a good operation you should get a good result
About one-third of Hirschsprung patients are constipated and need to be proactively and aggressively managed to avoid trouble
Surgeons might leave behind the dilated segment right above the aganglionic segment as an anatomic reason for decompensation
The most relevant reason for decompensation is that the patient never figured out how to successfully empty their sphincters and the pull-through decompensates
Normal calretinin staining is expected because calretinin hangs out with ganglion cells
In younger children age less than 3 or so, hypertrophic nerves are defined as greater than 40 microns in diameter
The definition of hypertrophic nerve is more gray in older patients and in patients who have chronic constipation issues
If there are no ganglion cells and no calretinin staining, that is a retained Hirschsprung's and that patient needs a redo to a higher level
If there are good ganglion cells with abundant ganglion cells and normal calretinin, the nerve hypertrophy could represent transition zone or could represent that the bowel has decompensated over time because it hasn't emptied and gotten dilated
The anorectal manometry is expected to be abnormal in Hirschsprung patients and many patients are going to have an abnormal amen but they're OK
One of the things done with Botox is to try to get patients to learn to overcome non-relaxing sphincters by other maneuvers like pushing on their abdominal wall
There are many patients that are completely asymptomatic doing great with Hirschsprung's that have residual absent RAIR
In Hirschsprung patients with sphincter problems, the colon is not the problem; the problem usually is the sphincters or the pelvic floor
If you get an awake anorectal manometry in a cooperative patient and you get a normal RAIR and can detect the resting pressure, the kid goes home with no anesthesia and no procedure
If the RAIR is absent, you're obligated to do a biopsy and give Botox
Botox is given if the resting pressure of the external sphincter is also high
Pelvic floor dysynergia can be detected on anorectal manometry and is a good indication that pelvic floor physical therapy is going to help that patient
Colonic manometry should not be done in Hirschsprung patients who have obstructive symptoms because it's not the colon but the distal pull-through that's the problem
You must rule out anatomic and pathologic causes before any colonic manometry is considered
You must get rid of the distal obstruction, which is why colonic manometry in a patient with distal obstruction is the wrong test
Once distal obstruction is ruled out or fixed and the colon is still not working, then you have a problem and that would be a case for colonic manometry
The rule of thumb is if there's a segment less than 30 centimeters of inadequate HAPCs, they are not super aggressive about it; over 30 centimeters is definitely more of a red flag
In a PCPLC Consortium study of close to 100 patients with functional constipation and segmental dysmotility of the sigmoid, 97% successfully responded to Malone only and never needed a resection
Five years ago, surgeons were taking sigmoids out of patients with segmental dysmotility, but the new data shows this is not necessary
A Malone procedure is a route for medical treatment that gives antegrade access to the colon for the gastroenterologist to give better medical treatment
An absent rectal anal inhibitory reflex means that the internal anal sphincter doesn't relax when the rectum is distended, which can contribute to constipation
HAPCs (high amplitude propagating contractions) aid in the transfer of colonic contents over long distance and often precede emptying
While surgery can correct the underlying anatomical problem in Hirschsprung disease, many other factors can contribute to constipation including motility disorders, pelvic floor dysfunction, and behavioral issues