From
Colorectal Channel
The Colorectal Quiz Episode 11: Total Colonic Hirschsprung's Part 2
With Dr. Jason Frischer & Dr. Marc Levitt · hosted by Dr. Em Gootee & Dr. Rod Gerardo & Dr. Amanda Jensen
Chapter 1 of 6 · Case-Based Learning
Case recap
Introduction and Case Recap
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No expert statements were drawn from this page.
Host summaries · secondary, not cited in answers
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
Dr. Levitt's previous practice was to wait until the child was potty trained for urine and could sit on a potty before performing pull-through for total colonic Hirschsprung disease.
Dr. Levitt had two patients who waited until older age for pull-through and developed proctalgia (anal sphincter spasm unresponsive to Botox) requiring ileostomy recreation.
Dr. Levitt's current routine is to perform pull-through between 6 and 18 months of age.
Dr. Fisher waits until the child has good growth and more solid stool on table food diet, sometimes adding thickeners to achieve thicker stool consistency.
Dr. Fisher uses a skin training technique where families take stool from the ostomy bag and place it in the diaper for 15-20 minutes to expose virgin buttock skin to stool before pull-through.
For patients with long-standing ileostomies and high output who are not growing well, checking urine sodium (which reflects total body sodium, not just blood sodium) is a key component for growth assessment.
Dr. Fisher checks urine sodium a few weeks after ileostomy creation prior to discharge and again a month or two later.
Low total body sodium can be treated with salt tablets or salt addition.
Oral salt intake improves glucose absorption in the GI tract, leading to better nutrition.
Dr. Levitt instructs families to have pediatricians check urine sodium if the patient is not growing well; it should be greater than 20 mmol/L, and if less, the baby is retaining sodium maximally.
Dr. Fisher typically performs an ileoanal anastomosis (straight pull-through) for total colonic Hirschsprung disease.
Dr. Levitt advocates minimizing the amount of Hirschsprung's bowel left behind because stasis is problematic.
The Duhamel procedure leads to stasis; the Martin procedure (using entire sigmoid and left colon) caused too much stasis and has been abandoned.
The Kamura procedure (right colon connected to ileum as an ileostomy) has been used; Dr. Levitt has had to remove the right colon patch in some cases.
An ileoDuhamel with a very short pouch is a very nice operation for total colonic Hirschsprung disease, and many patients do well with it.
Dr. Levitt's personal preference is straight ileoanal anastomosis, though he acknowledges bias from seeing many failed Duhamels.
The issue with Duhamel failures is not the Duhamel itself but the ganglionic bowel, which is not uniformly functional; ganglionated bowel can decompensate when stool slows in the Duhamel pouch.
Dr. Fisher makes a game-time decision on whether to divert after ileoanal anastomosis based on anastomotic appearance, blood supply, tension, and nutritional optimization; if any concerns exist, he diverts and returns 6-8 weeks later to close the ileostomy.
In theory, an ileoanal anastomosis should not require diversion.
Diverting more proximally may make the patient short on intestine proximal to the ileostomy, leading to inadequate absorption for nutrition and higher ileostomy output.
Family comfort with rectal irrigations is an important consideration when planning surgery.
Dr. Fisher's first-line medication treatment is loperamide.
Dietary recommendations include avoiding sugar (berries are particularly problematic), avoiding fats and oily foods, and bulking the stool.
Levsin (hyoscyamine) is used successfully as an added medicine to slow stool.
Lomotil (diphenoxylate-atropine) is a controlled substance in the United States.
Dr. Fisher gives Botox immediately when intestinal continuity is established.
Dr. Fisher sees the child at 2 weeks post-op for routine check (eating, growth, rash assessment) and does not check the anastomosis until 4 weeks.
At 4 weeks in clinic, Dr. Fisher performs an anastomosis check using Hagar dilators to size to the appropriate size for the child's age.
Dr. Fisher starts with a 7 or 8 Hagar dilator in clinic and gently sizes up to resistance, not to stretch but to check size; for a child who had pull-through at around 10 months, the proper size should be 13 or 14 Hagar dilator.
Studies showed no significant difference in skin excoriation between younger and older patients after pull-through.
Pectin or Imodium can be used to thicken high-output ileostomy stools.
For patients with high ileostomy output and failure to thrive, if urine sodium is less than 20 mmol/L, oral sodium supplementation is needed (GI absorption is better than IV).
Oral sodium supplementation protocol: add 3 mEq/kg/day using a recipe of 1 tablespoon salt plus 40 mL water (yields 2.5 mEq sodium per mL), continue for 1-2 months, then recheck urine sodium.
Sodium is the most abundant electrolyte in extracellular space, acquired through dietary intake, and absorbed actively in the ileum but passively in the jejunum.
Serum sodium is usually normal even when total body sodium is depleted, which can be misleading.
The vast majority of Duhamels do fine.
Patients with total colonic Hirschsprung disease are more susceptible to severe enterocolitis compared to traditional rectosigmoid Hirschsprung patients.
First-line medical management is diet modification, which can be started before pull-through to monitor bowel movements.
Loperamide liquid form contains glucose and sugar which can cause hypermotility; crushing the pill and mixing with applesauce is an alternative.
Both ileoanal and ileoDuhamel are acceptable options for pull-through in total colonic Hirschsprung disease.
A post-op skincare protocol should be in place after pull-through; the Villanova et al. article is a reference.
The role of Botox in total colonic Hirschsprung disease is vital.
Enterocolitis is more common in total colonic Hirschsprung patients; currently there is no good option for preventing it, only directed treatment.
Parents should be extremely proficient with irrigations and have a very low threshold to start them if there is concern for enterocolitis post-op.