Hirschsprung Disease: Cases and Complications
With Dr. Risto Rintala & Dr. Luis de la Torre · hosted by Dr. Todd Ponsky & Dr. Andrea Bischoff · StayCurrentMD
Cued at 13:29 · stops at 14:14 · 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
The speaker personally has more re-operation cases than primary Hirschsprung procedures because most pediatric surgeons believe they can operate on Hirschsprung disease, so referrals are typically for complex cases or complications rather than primary procedures.
Hirschsprung complications are classified into three categories: non-preventable (enterocolitis), partially preventable (constipation from retained dilated bowel), and preventable (dehiscence, strictures, perianal/urogenital fistulas).
The surgical rule is to resect not only the aganglionic segment but as much as possible of the dilated portion of the colon to prevent constipation.
Finland can conduct long-term follow-up studies because all Finns have a social security number that allows tracking of patients and access to their complete medical history from national records throughout their lifetime.
In the Finnish adult Hirschsprung study (patients operated 1960-1986), the mean bowel function score was 17.1 in patients versus 19.1 in controls (p<0.05), with lower scores indicating worse function.
In the Finnish study, 25% of adult Hirschsprung patients scored the full 20 points on bowel function versus 50% of controls; 13% reported frequent soiling, 2% had fecal accidents, and 10% had complications requiring treatment.
Overall gastrointestinal quality of life (GIQLI score) in adult Hirschsprung patients was mostly at the same level as healthy controls, though 22% of patients had scores below 110 indicating poor quality of life.
Increasing age was the only significant predictor of poor functional outcome in adult Hirschsprung patients, and age was inversely related to bowel function score in patients but not controls—a worrying finding suggesting patients may deteriorate as they age.
In patients with total colonic aganglionosis, if aganglionosis extends beyond 50 cm of terminal ileum, achievement of functional bowel continuity or autonomy from parenteral nutrition is exceptional and long-term survival is poor without intestinal transplantation.
When aganglionosis is limited to the colon, the outlook for bowel function after pouch-anal anastomosis is reassuring, although obstructive symptoms and enterocolitis are frequent but manageable with Botox injections and metronidazole.
In Finland, RET mutation testing is performed in Hirschsprung patients.
Rectal prolapse after a Hirschsprung pull-through is a very bad sign indicating something is wrong, likely surgical damage.
The pathologist at the speaker's institution immediately reports the size of nerves on frozen section, and will state if they believe the specimen is still in a transition zone.
With no anal canal, there will be no bowel control, and liquid stool with no bowel control is tragic for quality of life.
If a patient has a preserved anal canal after Hirschsprung surgery, there is more chance for bowel control, though it is not guaranteed.
A patient with hypomotility (dilated colon, infrequent bowel movements) has hypomotility with or without colonic manometry—the clinical picture is diagnostic.
Patients with Duhamel procedures can be challenging to manage because laxatives may cause stool to enter the pouch, leading to fecal impaction every 10-15 days even when the patient appears to be doing well.
Stricture and stasis of stool in the colon in Hirschsprung disease produces secretory diarrhea, even with normal ganglionic bowel—a fact many clinicians do not know.
Midline laparotomy incisions are preferred over transverse incisions for Hirschsprung surgery because they provide excellent exposure, can be extended if needed, and allow mobilization of splenic and hepatic flexures when necessary.
The priority in surgery is to finish the operation happy—surgeon happiness at the end of the procedure is very important, and the family feels happy when the surgeon is confident, allowing the surgeon to sleep well.
Laparoscopy is minimally invasive but provides maybe even better exposure than open surgery, so exposure is not compromised with laparoscopy.
Obstructive colitis in Hirschsprung disease exists on a spectrum of severity, and future research should focus on obstructive etiologies to understand this complication.
The speaker's institution starts anal dilations in Hirschsprung patients one month after surgery, only if a ring-like anastomosis is palpable on digital rectal exam; if the anastomosis is soft, no dilations are performed.
The speaker's protocol for post-Hirschsprung pull-through is to keep patients on oral Flagyl (metronidazole) and rectal irrigations, tapering both down over time.
Enemas are contraindicated in Hirschsprung disease; irrigations are used prophylactically instead. Enemas contain irritant ingredients that provoke bowel contraction, which is not helpful when normal peristalsis is absent. Irrigations involve passing a tube and allowing liquid stool to drain passively with small amounts of saline to clear the tube.
The speaker's irrigation protocol: mothers learn to do 3 irrigations per day with Flagyl starting immediately post-op; at 1 month decrease to twice daily and reduce Flagyl by 50%; continue tapering monthly; stop everything by 3 months if patient is thriving.
Enterocolitis is such a severe complication that babies sometimes die between home and hospital, which is why prophylactic measures are critical.
Some Hirschsprung patients develop enterocolitis every time irrigation frequency is decreased, making weaning very difficult—the reason for this variability is unknown.
In anorectal malformations, anal dilations start with a very small Hegar and increase weekly; in Hirschsprung, dilations can start near the goal size (e.g., size 12 if goal is 13) because the anastomosis is deeper and usually less tight.
Anal dilations are performed twice daily in both anorectal malformations and Hirschsprung disease, typically with diaper changes.
The association between Hirschsprung disease and anorectal malformations is extremely uncommon. The speaker has seen only two demonstrated cases in their career, despite traditional literature suggesting frequent association.
Many patients with anorectal malformations suffer from constipation and are incorrectly diagnosed with Hirschsprung when biopsies show no ganglion cells (which can occur in normal children for various technical reasons), leading to unnecessary pull-throughs and incontinence.
Before making a diagnosis of Hirschsprung in a patient with anorectal malformation, surgeons should think carefully—most likely it is not Hirschsprung, as many patients have constipation but very few have true Hirschsprung.
For hypermotile (diarrhea-prone) Hirschsprung patients with fecal incontinence, the correct bowel management is small-volume saline enemas only (250 mL), not laxatives, fiber, or enemas with additives.
Laxatives do not produce immediate, predictable bowel movements—they cause multiple unpredictable bowel movements, which worsens incontinence in fecally incontinent patients.
Giving laxatives to force bowel movements while simultaneously giving Imodium to decrease motility is counterproductive because the drugs have opposite effects.
Enterocolitis does not occur in pure anorectal malformations without Hirschsprung disease.
The speaker has seen patients with severe idiopathic constipation overlapping with intestinal pseudo-obstruction who develop enterocolitis-like symptoms (clostridium infections, colonic necrosis) and nearly die, but this is a different disease entity.
Published experience with sacral nerve stimulation (InterStim) in anorectal malformations is limited to only 10 patients in the literature, without specification of malformation type or sacral quality, making results impossible to evaluate.
The mechanism of sacral nerve stimulation assumes electrical stimulation reaches sacral nerves, which frequently do not exist in anorectal malformation patients with abnormal sacrums, making the speaker skeptical about its utility in this population.
According to Dr. Luis de la Torre's suggestion, very dilated colon suffers from hypomotility/poor motility, causing constipation even when the bowel is ganglionic.
A survey of the Canadian Association of Pediatric Surgeons by Jack Langer found that nearly half of surgeons resect 4 cm or less from the biopsy site, and about 10% resect right at the site of positive biopsy, creating high risk for pull-throughs in the transition zone.
A Salt Lake City group (Solake Utah) published a paper showing that prophylactic proactive irrigations starting in the first 5 days after pull-through can prevent enterocolitis.