The Colorectal Quiz Episode 11: Total Colonic Hirschsprung's Part 2
With Dr. Hira Ahmad & Dr. Jason Fisher & Dr. Mark Levitt · hosted by Dr. Amanda Jensen & Dr. Rod Gerardo · Colorectal Channel
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
Five to ten years ago, the recommendation for total colonic Hirschsprung was to wait until the child was potty trained for urine and could sit on a potty before performing pull-through
Studies showed no significant difference in skin excoriation between younger and older patients undergoing pull-through
Two patients who waited until older age for pull-through developed proctalgia (anal sphincter spasm unresponsive to Botox) requiring ileostomy recreation
Current routine is to perform pull-through somewhere between 6 and 18 months of age
Timing of pull-through should wait until child has good growth and more solid stool consistency from solid diet
High ileostomy output can be managed with pectin or Imodium to thicken stools
Skin training technique involves taking stool from ostomy bag and placing in diaper for 15-20 minutes to expose virgin buttock skin to stool before pull-through
These ileostomies can remain in place for 6, 12, or 24 months
Urine sodium (total body sodium) is a key component for growth in patients with long-standing ileostomies, not just blood sodium
Urine sodium should be checked a few weeks after ileostomy creation, prior to discharge, and a month or two later
Low total body sodium can be treated with salt tablets or salt addition
For patients with high ileostomy output and failure to thrive, if urine sodium is less than 20, oral sodium supplementation is needed
Oral sodium supplementation protocol: add 3 mEq/kg/day using recipe of 1 tablespoon salt plus 40 mL water (gives 2.5 mEq sodium per mL), continue 1-2 months then recheck urine sodium
Oral salt intake improves glucose absorption in the GI tract, leading to better nutrition
Sodium is actively absorbed in the ileum but passively absorbed in the jejunum
Urine sodium should be greater than 20 millimoles per liter; if less, the baby is retaining sodium and likely sodium-depleted despite normal serum sodium
For total colonic Hirschsprung, typical approach is ileoanal anastomosis (straight pull-through)
Duhamel procedure leads to stasis, which is problematic
The Martin procedure (ultimate Duhamel using entire sigmoid and left colon) has been abandoned due to excessive stasis
The Kamura procedure involves right colon connected to ileum in form of ileostomy
An ileoduhamel with a very short pouch is a very nice operation for total colonic Hirschsprung and many patients do well
The issue with Duhamel failures is not the Duhamel itself but the ganglionic bowel, as not all ganglionated bowel is created equally
Ganglionated bowel can decompensate when there is slowing of stool in the Duhamel pouch
Decision to redivert after ileoanal pull-through is made intraoperatively based on anastomosis quality, blood supply, tension, and nutritional optimization
In theory, an ileoanal anastomosis should not require diversion
Diverting more proximally may make the patient short gut with inadequate intestinal length for absorption, resulting in higher output ileostomy
Family comfort with rectal irrigations is an important consideration in surgical planning
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
First-line medication treatment is loperamide
Liquid loperamide contains glucose and sugar which can cause hypermotility; crushing pills in applesauce is preferred
Dietary recommendations include avoiding sugar (berries particularly offensive), avoiding fats and oily foods, and bulking the stool
Levsin is used successfully as an added medicine to slow down stool
Lomotil (atropine-diphenoxylate) is a controlled substance in the United States
Botox is given immediately when intestinal continuity is established
First post-operative visit is at 2 weeks for routine check of eating, growth, and rash assessment
Anastomosis check is performed at 4 weeks post-operatively in clinic using Hagar dilators sized appropriately for child's age
Anastomotic sizing starts with 7 or 8 Hagar dilator and gently sizes up to resistance, not to stretch but to check size
For a child undergoing pull-through at around 10 months of age, proper anastomotic size should be 13 or 14 Hagar dilator