Bowel Management for Hirschsprung's Disease
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 anal canal (2 cm above the pectinate line) is the most sensitive tissue in the body, capable of distinguishing gas, liquid, and solid stool.
Damage to the anal canal results in loss of sensation and fecal incontinence.
Damaging the anal canal likely also damages the surrounding sphincter mechanism.
The speaker's institution performs more reoperations for Hirschsprung disease than primary operations.
Transanal full-thickness rectal resection is preferred over submucosal endorectal dissection because staying close to the bowel wall avoids damage to pelvic structures.
Full-thickness rectal dissection close to the rectal wall prevents neurogenic bladder and other pelvic complications.
Intraoperative frozen section biopsies every 5 cm require a pathologist with specific experience in Hirschsprung disease frozen sections, not just board certification.
Traction creates the surgical plane; without traction there is no plane, no good dissection, and complications result.
80% of Hirschsprung cases can reach normal ganglionic bowel through the transanal approach; 20% require laparoscopy or laparotomy.
Transanal resection must divide the rectum 2 cm above the pectinate line to preserve the anal canal and sphincter mechanism.
A technically deficient transanal resection that includes the entire anal canal and anastomoses pull-through bowel to perianal skin results in permanent fecal incontinence.
The speaker's institution has performed 125 transanal resections.
Rectal dissection must remain in intimate contact with the rectal wall, dividing all extrinsic blood supply, to prevent damage to nerves and pelvic structures.
All fat tissue must be dissected away from the rectum during the resection.
Preserving the anal canal sensation area and sphincter mechanism guarantees bowel control.
Postoperative evaluation protocol for incontinent Hirschsprung patients (ages 4-7) includes contrast enema to classify as constipated vs. hypermotility type, and examination under anesthesia to assess anal canal integrity.
Patients with destroyed anal canals (bowel sutured to skin) will have lifelong fecal incontinence requiring lifelong bowel management.
Constipated patients with intact anal canals may have overflow pseudoincontinence treatable with laxatives.
Patients with diarrhea and intact anal canals may achieve continence with constipating diet, loperamide, 3 meals per day, and bulking fiber.
Hirschsprung complications are classified as non-preventable (enterocolitis), partially preventable (constipation), and preventable (dehiscence, stenosis, retraction, fistula, incontinence from anal canal destruction).
The mechanism of enterocolitis in Hirschsprung disease is unknown; it involves bacterial overgrowth and toxin release that can be fatal.
Constipation is partially preventable by resecting not only the aganglionic segment but also the dilated normal ganglionic bowel, which has abnormal peristalsis.
Enterocolitis can develop after technically successful operations and may include C. difficile infection.
Most Hirschsprung pull-throughs at the speaker's institution are performed without colostomy, with patients kept hospitalized for radiologic monitoring and early rectal irrigation if enterocolitis is suspected.
Rectal irrigation is the most valuable life-saving maneuver in Hirschsprung disease; all mothers should learn the technique.
Enemas and irrigations are different procedures; enemas can worsen enterocolitis or cause bowel perforation, while irrigations are therapeutic.
Mothers learn to recognize early enterocolitis signs (poor eating, distention, increased bowel sounds) and perform irrigation before the child becomes severely ill.
Postoperative enterocolitis prophylaxis protocol: discharge with 3 irrigations per day plus metronidazole, taper to 2 per day at 1 month if X-ray and growth are normal, then to 1 per day at 2 months.
Using this proactive enterocolitis protocol, the speaker's institution has not lost a patient to enterocolitis.
Enterocolitis symptoms include fever, abdominal distention, absence of stool, vomiting, explosive diarrhea, and foul-smelling stool or gas.
Catheter size for irrigation: 16 French for children under 1 year, 24 French for children over 1 year.
Saline for irrigation must be warmed and temperature-tested on the wrist before use.
Irrigation technique: advance catheter no further than the wide divider port, inject 20 mL warm saline, allow drainage, advance 1 inch, repeat until return fluid is clear.
If drainage volume is less than instilled volume, move and twist the catheter to drain pockets; gentle suction on the syringe may be used if no resistance is felt.
Parents should perform irrigation at home before seeking emergency care for suspected enterocolitis, and bring supplies to the hospital, because many emergency departments lack irrigation supplies and care may be delayed.
Severe diaper rash in fecally incontinent Hirschsprung patients can develop granulation tissue equivalent to second-degree burns.
In total colonic aganglionosis, resecting the entire colon results in lifelong diarrhea.
Retaining stool in aganglionic bowel (pouch procedures) produces bacterial proliferation, inflammatory changes, and secretory diarrhea.
The speaker does not recommend Martin, Kimura, or any pouch procedures for total colonic aganglionosis based on experience resecting failed pouches.
Preferred approach for total colonic aganglionosis: total colectomy with ileorectal or ileoproctostomy preserving the anal canal, with protective ileostomy maintained until the patient is toilet-trained for urine (typically over 3 years old).
Closing the ileostomy in infancy results in severe diaper rash because the baby passes liquid stool constantly without attempting to hold it.
Ileostomy closure criteria: child is 3+ years old, toilet-trained for urine, communicates need to use toilet, accustomed to clean underwear, and accepts rectal irrigations without distress.
Total colonic aganglionosis patients have a very high incidence of enterocolitis.
When the anal canal is preserved and ileostomy is closed in a toilet-trained 3-year-old with total colonic aganglionosis, the patient achieves fecal continence within 3 days.
Patients without a colon cannot receive enemas because the small bowel absorbs nutrients and cannot be cleaned or stopped from moving between enemas like the colon can.
Doctor Swenson's original Hirschsprung operation, when reproduced by others, resulted in many complications including neurogenic bladder, vaginal injury, and damage to pelvic nerves.
Doctor Franco Suave created the endorectal dissection technique to avoid the pelvic structure injuries that occurred when surgeons attempted to reproduce Swenson's operation.
Doctor Lester Martin pioneered pediatric surgery in Cincinnati after training at Boston Children's Hospital and contributed to total colonic aganglionosis treatment.
Doctor Martin's technique for total colonic aganglionosis preserved part of the aganglionic bowel and created a lateral-lateral anastomosis with normal ganglionic bowel to form a pouch for water absorption and stool formation.
Doctor Kimura used the same pouch principle with the right colon.