The Colorectal Quiz Episode 20: Hirschsprung Disease Obstruction Part 2
With Dr. Jason Fisher & Dr. Hira Ahmad & Dr. Mark Levitt & Dr. Rebecca Rentia · hosted by Dr. Amanda Jensen · Colorectal Channel
Cued at 2:23 · stops at 3:08 · press play
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
For exam under anesthesia in Hirschsprung's patients, digital exam should assess for circumferential stricture at the anastomosis
Hagar dilator can be used to size the anastomotic opening to ensure adequate caliber
Foley catheter passage can determine if there is a twist in the pull-through segment
Swabe cuff presents as a 1-2 centimeter circumferential narrowing that is not the anastomosis itself
For patients with previous Duhamel procedure, full-thickness rectal biopsy should be performed posteriorly because that segment should be ganglionic
In Duhamel procedure, rectal exam should assess for two lumens and a spur between them, as stool can flow into the Duhamel pouch, fill it, and compress the ganglionated pull-through
Treatment for problematic Duhamel spur is to take out the common wall; occasionally the Duhamel pouch itself needs excision
Before anesthesia induction, it is important to examine the anus for sphincteric contraction vs. patulous appearance
A patulous anus will not develop enterocolitis
During exam and biopsy, close examination is needed to ensure the dentate line was preserved at the original pull-through
If the dentate line has been lost at original pull-through or sphincters have been overstretched, the patient will not have enterocolitis but will have fecal incontinence
Botox is being studied to prevent enterocolitis with the theory of chemo-denervating nerves in the area instead of permanent stretch or anatomic destruction from surgery
Lone Star retractor should be used to obtain a circumferential view of the anastomotic area during exam under anesthesia
If repeat biopsy after pull-through shows no ganglion cells, it is very likely an aganglionotic or transition zone pull-through, though sampling error must be considered
Ganglion cells with hypertrophic nerves on repeat biopsy is more controversial; some surgeons offer redo for significantly obstructive patients with this finding
Hypertrophic nerves in the presence of ganglion cells can occur in functional constipation patients, and may develop in Hirschsprung's patients not emptying well due to sphincter dysfunction
Until comparing original and repeat pathology, it is unclear whether hypertrophic nerves represent original pathology error or secondary changes that evolved over time
Many Swabe surgeons are now approaching a Swenson technique or have transitioned to Swenson, using a mini-cuff (approximately 1 centimeter)
In the original laparoscopic Swabe described by Keith Jorgeson, a 5 centimeter cuff was recommended, which is too long
The Swenson procedure was the first Hirschsprung's operation and is the purest, leaving the least amount of Hirschsprung's tissue behind
The Swabe and Duhamel procedures were developed because surgeons were doing the Swenson in too wide a plane and injuring the nervi erigentes in the mesorectum
If you stay right on the bowel wall during Swenson dissection, you avoid nerve injury
Sometimes the Swabe cuff fuses back together or is not cut all the way, creating an aganglionotic obstructive ring around the pull-through
To palpate for Swabe cuff, place finger against the sacrum and pull down to feel rubbery tissue outside the pull-through
For redo pull-through in aganglionotic cases, the approach is total body prep, then transanal dissection in prone position first, going as far as possible; if healthy bowel cannot reach, be prepared for laparoscopy or laparotomy
Redo pull-throughs are easier to perform in prone position
Initial treatment for obstructed Hirschsprung's patient must include rectal irrigation, done early
Every institution should have a protocol for obstructed Hirschsprung's patients to ensure quick and efficient response
Systematic workup for obstructed Hirschsprung's includes: resuscitation, imaging studies, and comprehensive rectal exam under anesthesia
Gastroenterologists and surgeons must know the anatomy of the original pull-through procedure (Swabe, Swenson, or Duhamel) when evaluating obstructed patients