Bowel Management for Hirschsprung's Disease Patients: Pediatric Bowel...
With Dr. Dr. Todd Ponsky · StayCurrentMD
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, defined as the 2 cm above the pectinate line, is the most sensitive part of the body and can distinguish between gas, liquid, and solid stool—a unique capability no other tissue possesses.
Damage to the anal canal results in poor or absent sensation, leading to fecal incontinence, and likely indicates sphincter damage as well since the sphincter surrounds the anal canal.
The speaker's institution performs more reoperations for Hirschsprung disease than primary operations, reflecting the frequency of technical errors and complications.
The transanal full-thickness resection is preferred over submucosal endorectal dissection because staying close to the bowel wall avoids damage to pelvic structures, a principle learned from anorectal malformation surgery.
Full-thickness rectal dissection close to the rectal wall prevents neurogenic bladder and other pelvic structure damage, a technique routinely used in the speaker's practice.
Intraoperative frozen section biopsies every 5 cm require a pathologist with specific experience in Hirschsprung disease frozen sections; board certification alone does not guarantee accurate interpretation.
The technique of taking biopsies every 5 cm and sending for frozen section can only be implemented if the pathologist has experience with frozen sections in Hirschsprung disease.
Traction creates the surgical plane; without traction there is no plane, without a plane there is no good dissection, and without good dissection there are complications.
In 80% of cases, the transanal approach can reach normal ganglionic bowel; 20% require laparoscopy or laparotomy.
The transanal resection must divide the rectum 2 cm above the pectinate line to preserve the anal canal and sphincter mechanism, which guarantees bowel control.
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 using the described technique.
Remaining in intimate contact with the rectal wall during dissection prevents damage to important nerves and pelvic structures; all fat tissue must be dissected away from the rectum.
The evaluation protocol for fecally incontinent Hirschsprung patients (ages 4-57) includes contrast enema to classify as constipated with overflow incontinence versus hypermotility with non-dilated colon, plus examination under anesthesia to assess anal canal integrity.
When the anal canal is completely destroyed and bowel is sutured to skin, the patient will have lifelong fecal incontinence requiring lifelong bowel management.
Patients with intact anal canal and severe constipation may have overflow pseudo-incontinence treatable with laxatives, potentially achieving continence.
Patients with intact anal canal and diarrhea tendency may achieve control with constipating diet, Imodium, 3 meals per day, and special fiber to bulk stool.
Hirschsprung complications are classified as non-preventable (enterocolitis—cause unknown), partially preventable (constipation—related to resecting dilated ganglionic bowel but some cases unexplained), and preventable (dehiscence, stenosis, retraction, fistulas, and fecal incontinence from anal canal destruction—all technical errors).
Resecting the dilated ganglionic segment of bowel in addition to the aganglionic segment is necessary because dilated bowel lacks normal peristalsis and causes constipation, though some patients develop constipation despite this approach.
Enterocolitis in Hirschsprung disease involves abdominal distention, bacterial proliferation (sometimes C. difficile), toxin release, and can be fatal.
The speaker's institution performs most Hirschsprung pull-throughs without colostomy but keeps patients hospitalized with serial X-rays and starts rectal irrigations at first suspicion of enterocolitis rather than waiting for severe illness.
Rectal irrigation is the most valuable life-saving maneuver in Hirschsprung disease; all mothers should learn the technique.
Many academic institutions confuse enemas and irrigations, using the terms interchangeably, which is wrong; giving enemas to patients with enterocolitis can worsen the condition or cause bowel perforation.
Post-operative protocol: discharge with 3 irrigations per day and Flagyl; at 1 month if X-ray and growth are normal, reduce to 2 irrigations daily and 50% Flagyl; at 2 months if doing well, reduce to 1 irrigation daily and further reduce Flagyl. Using this protocol, the institution has not lost a patient to enterocolitis.
For children with enterocolitis diagnosis, irrigation should be done 3 times daily and more often if needed.
Enterocolitis symptoms requiring immediate irrigation and medical attention include fever, abdominal distention, not stooling, vomiting, explosive diarrhea, and foul-smelling stool or gas.
Catheter sizing for irrigation: 16 French for children under 1 year, 24 French for children over 1 year.
Normal saline for irrigation should be warmed in a sink of warm water and temperature tested on the wrist to ensure it is not too hot.
The catheter should not be advanced further than the wide divider port and should not be forced; it should follow the curve of the colon when gently pushed.
Irrigation technique: inject 20 mL warm saline, disconnect syringe to allow drainage, advance catheter 1 inch, repeat. If drainage is not equal to or greater than input, move and twist catheter to drain pockets. Continue until return fluid is clear.
Parents should perform irrigation at home before seeking emergency care when enterocolitis is suspected, because emergency rooms often lack irrigation supplies and care may be delayed for hours while the child worsens. Parents should take supplies with them.
Severe diaper rash in fecally incontinent Hirschsprung patients, particularly those with diarrhea tendency, can develop chronic granulation tissue equivalent to second-degree burns, causing significant suffering for babies and mothers.
Retaining stool in Hirschsprung disease produces bacterial proliferation and inflammatory changes that cause secretory diarrhea; therefore pouch procedures (Martin, Kimura) are not favored and patients with these pouches do not do well.
For total colonic aganglionosis, the speaker prefers straight ileoproctostomy (ileorectal anastomosis) preserving the anal canal, with protective ileostomy maintained until the patient is toilet-trained for urine (usually over 3 years old).
Closing the ileostomy in a baby with total colonic aganglionosis results in terrible diaper rash (the worst type) because the baby passes liquid stool constantly without making effort to hold it, even with preserved anal canal.
Ileostomy closure criteria for total colonic aganglionosis: patient must be toilet-trained for urine (talks, tells mother, accustomed to clean underwear) and must tolerate rectal irrigations (practiced at home with soft catheter so child understands it doesn't hurt).
Patients with total colonic aganglionosis have a very high incidence of enterocolitis.
When the anal canal is preserved in total colonic aganglionosis and ileostomy is closed after toilet training for urine, the patient becomes totally trained for stool within 3 days.
Patients without a colon cannot have enemas because the small bowel absorbs nutrients and there is no way to clean the small bowel and stop it from moving between enemas as can be done with the colon.
Dr. Franco Soave created the endorectal dissection technique because when surgeons attempted to reproduce Dr. Swenson's operation, many patients suffered damage to pelvic structures including neurogenic bladder and vaginal injury.
Dr. Lester Martin, a pioneer of pediatric surgery in Cincinnati who trained at Boston Children's Hospital, made contributions to the treatment of total colonic aganglionosis.
In total colonic aganglionosis, the entire colon must be resected, resulting in lifelong diarrhea. Dr. Martin's approach preserved part of the aganglionic bowel and created a lateral-lateral anastomosis with normal ganglionic bowel to form a pouch/reservoir for water absorption and solid stool formation. Dr. Kimura used the same principle with the right colon.