Colorectal Quiz: Episode 42 - HD Constipation
With Dr. Jason Fisher & Dr. Mark Levitt & Dr. Chris Geyer · hosted by Dr. Felipe Glu · Colorectal Channel
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
Hirschsprung disease is a very anatomically fixable problem and with a good operation you should get a good result
Approximately one-third of Hirschsprung patients are constipated post-operatively and need proactive, aggressive management 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
Calretinin hangs out with ganglion cells, so normal calretinin staining provides double evidence of good 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 and the patient needs a redo to a higher level
Nerve hypertrophy with good ganglion cells could represent transition zone or could represent that the bowel has decompensated over time because it hasn't emptied and gotten dilated
An absent rectal anal inhibitory reflex means the internal anal sphincter doesn't relax when the rectum is distended, which can contribute to constipation
Many patients are going to have an abnormal anorectal manometry but they're OK
Botox helps patients learn to overcome non-relaxing sphincters by other maneuvers like pushing on their abdominal wall
Many patients are completely asymptomatic doing great with Hirschsprung's that have residual absent rectal anal inhibitory reflex
In Hirschsprung patients with constipation, 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 with a normal rectal anal inhibitory reflex and can detect the resting pressure, the evaluation is complete without anesthesia or procedure
If the rectal anal inhibitory reflex 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 will help the patient
Colonic manometry should not be done in Hirschsprung patients with obstructive symptoms because it's not the colon but the distal pull-through that's the problem
Anatomic and pathologic causes must be ruled out before any colonic manometry is considered
Colonic manometry in a patient with a 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 warranting colonic manometry
If there's a segment less than 30 centimeters of inadequate high amplitude propagating contractions, the approach is not super aggressive; over 30 centimeters is definitely more of a red flag
High amplitude propagating contractions aid in the transfer of colonic contents over long distance and often precede emptying
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 without needing resection
Five years ago, surgeons were taking sigmoids out of patients with segmental dysmotility, but this practice has changed based on new data
A Malone procedure is a route for medical treatment, providing antegrade access to the colon for gastroenterologists to give better medical treatment
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