ERN eUROGEN Colorectal Webinar Series: Redo surgery for pull-through in Hirschsprung's Disease: When and how
With Dr. Mark Levitt & Dr. Julia Vsigheli · hosted by Dr. Olivia Spivak & Dr. Yvo de Blau
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What the experts said
The Swenson procedure leaves behind the least amount of Hirschsprung's disease (only the sphincters) when done in the proper plane (same as PSARP plane).
The original Swenson caused injury to nerve erigentes when rectal dissection was done too wide, resulting in bladder emptying problems and erectile dysfunction.
The Duhamel procedure leaves behind a segment of Hirschsprung's (the original rectum), which can impose stasis.
The Yancey-Suave procedure should be renamed from 'Suave' because Dr. Yancey published the technique 12 years earlier (1952 vs. 1964), but his paper was not well-read due to racial discrimination.
The muscular cuff left behind in the Yancey-Suave procedure can be problematic and cause obstruction.
All patients with Hirschsprung's disease should stool spontaneously and be clean for stool. If not stooling spontaneously, something must be wrong with the pull-through or sphincter management.
When anatomy and pathology are normal after pull-through, patients can be divided into two groups: those whose colon moves too fast (multiple stools daily without distention) and those whose colon moves too slowly (infrequent stools with distention).
Patients with slow colonic motility should receive laxatives; patients with fast motility can be slowed with fiber or loperamide.
The dentate line provides exquisite sensation to distinguish whether the rectum contains solid, liquid, or gas, and controls the motor system to contract sphincters appropriately.
Hirschsprung's patients lose their reservoir with pull-through; if the dentate line is also destroyed, they will be incontinent for life.
To avoid overstretching sphincters during pull-through, use laparoscopy for most of the deep pelvic rectal dissection so the transanal portion is relatively short, and avoid aggressive placement of pins.
For overstretched sphincters, a new technique involves tacking the sphincter muscles circumferentially to the pull-through to bring them closer to the bowel, with approximately 10 cases showing very nice results.
After ileostomy closure following pull-through, if the patient vomits on post-op day 2 with colonic dilatation on X-ray, the problem is typically that the pull-through has never worked before and is obstructed; irrigation from below is more appropriate than nasogastric decompression.
A retained transition zone on contrast enema appears as a dilated segment with no haustral markings and no peristalsis, resembling a 'summer squash' according to Dr. Andrew Badillo.
An obstructing Yancey-Suave cuff appears on lateral contrast enema as increased presacral space between the pull-through and sacrum, and can be felt on digital exam as rubbery tissue obstructing the pull-through.
The Swenson is preferred over Yancey-Suave because it avoids leaving behind a cuff that can obstruct.
In Duhamel procedures, if the common wall between the two lumens is not adequately divided, the original rectum fills with stool and compresses the pull-through, preventing it from emptying.
The solution for an obstructing Duhamel spur is to remove the common wall using an endovascular stapler to complete separation of the two lumens.
Even healthy ganglionated bowel can decompensate if any stasis is imposed by strictures, cuffs, twists, retained transition zone, or a large non-functional Duhamel pouch.
All Hirschsprung's patients have an absent recto-anal inhibitory reflex and fail to relax their internal sphincter upon rectal distention.
Botox technique for non-relaxing sphincter: 100 units mixed with 1cc saline, injected into the dentate line hitting both internal and external sphincter.
After starting a protocol of giving all total colonic aganglionosis patients Botox one month after surgery (but not short-segment patients), huge improvements were seen.
If Botox helps a patient with retained Suave cuff but symptoms return immediately when it wears off, suspect the cuff is the problem and consider removal.
A properly done Hirschsprung's surgery should have no urologic implications. Urinary retention and erectile dysfunction only occur if surgery is done improperly with injury to nerve erigentes from too-wide rectal dissection.
Botox is administered as a day procedure using a lone star retractor with 4 hooks, insulin syringe, injecting 25 units per quadrant at 2, 5, 7, and 10 o'clock positions at the dentate line.
Botox effects begin at 48 hours, reach full effect by 7 days, and last up to 3 months.
Usually 1-2 Botox injections are sufficient (3 months apart), maximum 4 injections. If more are needed, suspect a surgical problem and work up with contrast enema, EUA, stimulation, and full-thickness rectal biopsy.
Myectomy should never be performed because it is permanent and could leave the patient with permanent fecal incontinence, whereas Botox provides temporary disruption that will wear off.
If no ganglion cells are found on frozen section in the left colon or sigmoid, do not do a pull-through that day. Perform mapping, wait for permanent sections, and consider ileostomy.
Frozen section can only rule out Hirschsprung's disease, not rule it in. You need to evaluate 100 levels of a specimen to know for sure there are no ganglion cells.
For a mid-transverse colon transition zone, the patient needs a de-rotation (Deloyers procedure) with colon brought down the right side. If brought down the left side, the main mesenteric vessel will cross the duodenum and cause duodenal obstruction.
Patients without a dentate line (whether Hirschsprung's or anorectal malformation) cannot distinguish between solid, liquid, and gas. However, if sphincters are good and they can feel distal rectal distention, they can squeeze the external sphincter in time to hold stool.
It is a mistake to give anorectal malformation patients stool softeners because without dentate line sensation they will just have soiling. Give laxatives that provoke a bowel movement and aim for formed stool.
Anorectal malformation patients have a harder time with continence than Hirschsprung's patients without dentate line because ARM patients lack an internal sphincter, whereas Hirschsprung's patients have an internal sphincter that sometimes works too well.
Three requirements for continence: sensation (ability to feel rectal stretch), functional sphincters to close the anal canal, and reliable consistent motility.