The Colorectal Quiz Episode 11: Total Colonic Hirschsprung's Part 2

Published: 2021-04-19

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Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Amanda Jensen — host
Rod Gerardo — host
Hira Ahmad — guest
Jason Fisher — guest
Mark Levitt — guest
Speaker 6

Chapters

0:00Introduction and Case Recap — Hosts introduce the episode as part 2 of total colonic Hirschsprung discussion, recapping the 10-month-old patient with delayed meconium passage, severe constipation, and intraoperative findings of aganglionosis extending 15 cm into ileum, who received diverting ileostomy.
2:02Timing of Definitive Pull-Through — Discussion of optimal timing for pull-through surgery, with evolution from waiting until potty training to performing between 6-18 months once child has good growth and thicker stool. Includes management strategies for ileostomy output and skin preparation.
5:07Ileostomy Management and Sodium Supplementation — Detailed discussion of monitoring and managing long-standing ileostomies, emphasizing urine sodium as marker of total body sodium status and oral sodium supplementation protocol for patients with failure to thrive.
7:56Surgical Technique Selection — Comparison of surgical approaches including ileoanal anastomosis versus ileoduhamel, discussion of historical procedures (Martin, Kamura), and considerations for diversion versus primary anastomosis.
10:41Post-Operative Management — Medical management strategies including dietary modifications, medications (loperamide, Levsin, cholestyramine, Lomotil), rectal irrigations, Botox administration, and anastomotic surveillance protocol.
13:36Summary and Closing — Episode summary emphasizing key clinical points and closing remarks.

Key claims

2:23Five 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 — Mark Levitt
2:46Studies showed no significant difference in skin excoriation between younger and older patients undergoing pull-through — Rod Gerardo
2:58Two patients who waited until older age for pull-through developed proctalgia (anal sphincter spasm unresponsive to Botox) requiring ileostomy recreation — Mark Levitt
3:23Current routine is to perform pull-through somewhere between 6 and 18 months of age — Mark Levitt
3:30Timing of pull-through should wait until child has good growth and more solid stool consistency from solid diet — Jason Fisher
3:59High ileostomy output can be managed with pectin or Imodium to thicken stools — Amanda Jensen
4:12Skin 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 — Jason Fisher
4:57These ileostomies can remain in place for 6, 12, or 24 months — Mark Levitt
5:19Urine sodium (total body sodium) is a key component for growth in patients with long-standing ileostomies, not just blood sodium — Jason Fisher
5:34Urine sodium should be checked a few weeks after ileostomy creation, prior to discharge, and a month or two later — Jason Fisher
5:49Low total body sodium can be treated with salt tablets or salt addition — Jason Fisher
5:57For patients with high ileostomy output and failure to thrive, if urine sodium is less than 20, oral sodium supplementation is needed — Hira Ahmad
6:26Oral 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 — Rod Gerardo
6:49Oral salt intake improves glucose absorption in the GI tract, leading to better nutrition — Mark Levitt
7:08Sodium is actively absorbed in the ileum but passively absorbed in the jejunum — Amanda Jensen
7:37Urine sodium should be greater than 20 millimoles per liter; if less, the baby is retaining sodium and likely sodium-depleted despite normal serum sodium — Mark Levitt
8:00For total colonic Hirschsprung, typical approach is ileoanal anastomosis (straight pull-through) — Jason Fisher
8:26Duhamel procedure leads to stasis, which is problematic — Mark Levitt
8:26The Martin procedure (ultimate Duhamel using entire sigmoid and left colon) has been abandoned due to excessive stasis — Mark Levitt
8:50The Kamura procedure involves right colon connected to ileum in form of ileostomy — Mark Levitt
9:01An ileoduhamel with a very short pouch is a very nice operation for total colonic Hirschsprung and many patients do well — Mark Levitt
9:26The issue with Duhamel failures is not the Duhamel itself but the ganglionic bowel, as not all ganglionated bowel is created equally — Mark Levitt
9:38Ganglionated bowel can decompensate when there is slowing of stool in the Duhamel pouch — Rod Gerardo
9:57Decision to redivert after ileoanal pull-through is made intraoperatively based on anastomosis quality, blood supply, tension, and nutritional optimization — Jason Fisher
10:26In theory, an ileoanal anastomosis should not require diversion — Mark Levitt
10:30Diverting more proximally may make the patient short gut with inadequate intestinal length for absorption, resulting in higher output ileostomy — Mark Levitt
10:57Family comfort with rectal irrigations is an important consideration in surgical planning — Jason Fisher
11:04Patients with total colonic Hirschsprung disease are more susceptible to severe enterocolitis compared to traditional rectosigmoid Hirschsprung patients — Rod Gerardo
11:16First-line medical management is diet modification, which can be started before pull-through — Rod Gerardo
11:26First-line medication treatment is loperamide — Jason Fisher
11:30Liquid loperamide contains glucose and sugar which can cause hypermotility; crushing pills in applesauce is preferred — Jason Fisher
11:42Dietary recommendations include avoiding sugar (berries particularly offensive), avoiding fats and oily foods, and bulking the stool — Mark Levitt
11:56Levsin is used successfully as an added medicine to slow down stool — Mark Levitt
12:07Lomotil (atropine-diphenoxylate) is a controlled substance in the United States — Jason Fisher
12:24Botox is given immediately when intestinal continuity is established — Jason Fisher
12:34First post-operative visit is at 2 weeks for routine check of eating, growth, and rash assessment — Jason Fisher
12:38Anastomosis check is performed at 4 weeks post-operatively in clinic using Hagar dilators sized appropriately for child's age — Jason Fisher
13:05Anastomotic sizing starts with 7 or 8 Hagar dilator and gently sizes up to resistance, not to stretch but to check size — Jason Fisher
13:15For a child undergoing pull-through at around 10 months of age, proper anastomotic size should be 13 or 14 Hagar dilator — Jason Fisher

Cases discussed

0:3510-month-old with total colonic Hirschsprung disease extending 15 cm into ileum, managed with initial diverting ileostomy

Open questions

What is the optimal method for preventing enterocolitis in total colonic Hirschsprung patients post-operatively?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Total Colonic Hirschsprung Disease: Timing, Technique, and Long-Term Ileostomy Management

The episode's main topic retold as a plain-language walkthrough — what it is, why it matters, and what the speakers concluded. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Explainer · AI-written, human-reviewed

Why This Subspecialty Exists

Total colonic Hirschsprung disease — aganglionosis extending through the entire colon and into the terminal ileum — occurs in roughly 5-10% of Hirschsprung cases. These children cannot be managed with standard rectosigmoid pull-through techniques. The problem is not simply absent ganglion cells in a short segment; it is the loss of the colon's entire absorptive and storage capacity, combined with the need to bring small bowel directly to the anus. This creates a distinct set of physiologic challenges that require specialized surgical planning and prolonged medical management.

The Core Clinical Problem

A newborn with total colonic Hirschsprung typically presents with delayed meconium passage and abdominal distension. Intraoperative frozen sections during the initial diverting procedure reveal aganglionosis extending 15 cm or more into the ileum 2:23. The surgeon creates a proximal ileostomy, and the child goes home. The question then becomes: when and how do you restore intestinal continuity, and how do you manage the high-output ileostomy in the interim?

Evolution in Timing

Five to ten years ago, the standard approach was to wait until the child was potty trained for urine and could sit on a toilet before performing definitive pull-through 2:23. The reasoning was straightforward: pulling liquid ileal effluent directly to the anus would cause severe perianal excoriation in an infant still in diapers. Studies comparing younger and older patients, however, showed no significant difference in skin breakdown 2:46. More concerning, two patients who waited until older age developed proctalgia — anal sphincter spasm unresponsive to Botox — requiring ileostomy recreation 2:58.

Current practice has shifted to performing pull-through between 6 and 18 months of age 3:23. The actual trigger is not calendar age but adequate growth and stool consistency 3:30. Families are taught to thicken ileostomy output with pectin or loperamide 3:59, and some centers employ a skin-training technique: placing ostomy output in the diaper for 15-20 minutes to expose virgin buttock skin to stool before definitive surgery 4:12. These ileostomies may remain in place for 6, 12, or even 24 months 4:57.

Managing the Long-Standing Ileostomy

The critical insight for these patients is that serum sodium can be normal while total body sodium is depleted 5:19. Urine sodium — not serum sodium — reflects total body sodium status. It should be checked a few weeks after ileostomy creation, prior to discharge, and again a month or two later 5:34. A urine sodium below 20 mmol/L indicates the kidneys are retaining every available sodium molecule, and the child requires oral supplementation 5:57.

The protocol: add 3 mEq/kg/day using a recipe of 1 tablespoon salt plus 40 mL water, which yields 2.5 mEq sodium per mL. Continue for 1-2 months, then recheck 6:26. Oral sodium is preferred over IV because it enhances glucose absorption in the GI tract via the sodium-glucose cotransporter, improving overall nutrition 6:49. Sodium is actively absorbed in the ileum but only passively in the jejunum 7:08, making oral supplementation particularly effective in these patients. If urine sodium remains below 20 despite normal serum levels, the child is sodium-depleted and will not grow adequately 7:37.

Surgical Technique

The typical approach is an ileoanal anastomosis — a straight pull-through 8:00. The Duhamel procedure, particularly the Martin modification using the entire sigmoid and left colon, has been largely abandoned due to excessive stasis 8:26. An ileoduhamel with a very short pouch is considered a viable alternative by some surgeons and many patients do well 9:01, but the concern is not the Duhamel technique itself but the quality of the ganglionic bowel 9:26. Not all ganglionated bowel functions equally, and some will decompensate when stool slows in a Duhamel pouch 9:38.

The decision to redivert after ileoanal anastomosis is made intraoperatively based on anastomosis quality, blood supply, tension, and nutritional optimization 9:57. In theory, an ileoanal anastomosis should not require diversion 10:26, but diverting too proximally risks creating functional short gut with inadequate absorptive length 10:30. Family comfort with rectal irrigations is also a consideration, as these patients are more susceptible to severe enterocolitis than those with rectosigmoid Hirschsprung 11:04.

Post-Operative Management

First-line medical management is diet modification, which can begin before pull-through 11:16. Avoid sugar — berries are particularly problematic — avoid fats and oily foods, and bulk the stool 11:42. First-line medication is loperamide 11:26; liquid formulations contain glucose and sugar that can worsen hypermotility, so crushing pills in applesauce is preferred 11:30. Levsin is used successfully as an adjunct to slow stool 11:56. Lomotil (atropine-diphenoxylate) is a controlled substance in the United States 12:07.

Botox is administered immediately when intestinal continuity is established 12:24. The first post-operative visit at 2 weeks assesses eating, growth, and perianal rash 12:34. Anastomotic check occurs at 4 weeks in clinic using Hagar dilators sized appropriately for the child's age 12:38. The technique is to start with a 7 or 8 Hagar and gently advance to resistance — the goal is assessment, not dilation 13:05. For a child undergoing pull-through around 10 months, proper anastomotic size should be 13 or 14 Hagar 13:15.

When to Involve This Team

Refer immediately when intraoperative frozen sections during initial diversion reveal aganglionosis extending beyond the splenic flexure. These patients require a center experienced in managing long-standing ileostomies, monitoring total body sodium status, and performing ileoanal anastomoses in infants. If a child with a proximal ileostomy is failing to thrive despite normal serum electrolytes, check urine sodium and consider referral for nutritional optimization before definitive repair.

Takeaways from this story

Urine sodium below 20 mmol/L indicates total body sodium depletion despite normal serum levels; oral supplementation improves growth.
Pull-through timing shifted from potty-training age to 6-18 months after studies showed no difference in skin excoriation.
Ileoanal anastomosis is standard; Duhamel with short pouch is viable, but long pouches cause stasis and are abandoned.
Liquid loperamide contains glucose that worsens hypermotility; crush pills in applesauce instead.
Anastomotic check at 4 weeks uses Hagar dilators to assess size, not stretch; proper size at 10 months is 13-14 Hagar.

Total Colonic Hirschsprung: When Waiting Too Long Creates New Problems

The patient case from this episode, retold from presentation to outcome with the decisions made along the way. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Case narrative · AI-written, human-reviewed

The Case

A 10-month-old presented to the emergency department with abdominal distention and severe constipation, without fever or signs of sepsis 2:23. The history was revealing: delayed passage of meconium at birth, with a contrast enema at that time showing an amorphous round colon. Current imaging demonstrated severe constipation. Rectal biopsy showed no ganglion cells. Intraoperative frozen section biopsies traced the aganglionosis through the entire colon and nearly 15 cm into the ileum — total colonic Hirschsprung disease extending into small bowel. The team performed a diverting ileostomy and deferred definitive repair pending final pathology.

The Decision Point

The question was not whether to perform a pull-through, but when. Five to ten years ago, the standard recommendation was to wait until the child was potty trained for urine and could sit on a potty before performing pull-through 2:23. The reasoning seemed sound: an ileoanal anastomosis would produce loose stool, and a child in diapers would develop severe perianal excoriation. Better to wait until the child could sit on the toilet and manage their own hygiene.

Studies challenged that assumption. When outcomes were compared, there was no significant difference in skin excoriation between younger and older patients undergoing pull-through 2:46. The perceived advantage of waiting evaporated. Worse, two patients who waited until older age for pull-through developed proctalgia — anal sphincter spasm unresponsive to Botox — severe enough to require ileostomy recreation 2:58. The children had learned to hold stool so efficiently that their sphincters would not relax.

That experience changed the approach. Current routine is to perform pull-through somewhere between 6 and 18 months of age 3:23. The team waits for good growth and more solid stool consistency from a solid diet 3:30, not for toilet training. High ileostomy output can be managed with pectin or Imodium to thicken stools 3:59. One technique involves taking stool from the ostomy bag and placing it in the diaper for 15-20 minutes before pull-through — exposing virgin buttock skin to stool in controlled doses 4:12.

What the Team Did

These ileostomies can remain in place for 6, 12, or 24 months 4:57. During that interval, growth monitoring is critical. Urine sodium — reflecting total body sodium, not just serum sodium — is a key component for growth in patients with long-standing ileostomies 5:19. Urine sodium should be checked a few weeks after ileostomy creation, prior to discharge, and a month or two later 5:34. If urine sodium is less than 20 millimoles per liter, the baby is retaining sodium and likely sodium-depleted despite normal serum sodium 7:37. Treatment is oral sodium supplementation: 3 mEq/kg/day using a recipe of 1 tablespoon salt plus 40 mL water, which gives 2.5 mEq sodium per mL, continued 1-2 months then rechecked 6:26. Oral salt intake improves glucose absorption in the GI tract, leading to better nutrition 6:49.

For the definitive repair, the typical approach for total colonic Hirschsprung is ileoanal anastomosis — a straight pull-through 8:00. The Duhamel procedure leads to stasis, which is problematic 8:26. The Martin procedure, using the entire sigmoid and left colon, has been abandoned due to excessive stasis 8:26. An ileoduhamel with a very short pouch is a very nice operation for total colonic Hirschsprung and many patients do well 9:01, but the issue with Duhamel failures is not the Duhamel itself but the ganglionic bowel — not all ganglionated bowel is created equally 9:26. Ganglionated bowel can decompensate when there is slowing of stool in the Duhamel pouch 9:38.

The decision to redivert after ileoanal pull-through is made intraoperatively based on anastomosis quality, blood supply, tension, and nutritional optimization 9:57. In theory, an ileoanal anastomosis should not require diversion 10:26, but diverting more proximally may make the patient short gut with inadequate intestinal length for absorption, resulting in higher output ileostomy 10:30.

What the Case Changes

Patients with total colonic Hirschsprung disease are more susceptible to severe enterocolitis compared to traditional rectosigmoid Hirschsprung patients 11:04. First-line medical management is diet modification, which can be started before pull-through 11:16. First-line medication treatment is loperamide 11:26; liquid loperamide contains glucose and sugar which can cause hypermotility, so crushing pills in applesauce is preferred 11:30. Dietary recommendations include avoiding sugar (berries particularly offensive), avoiding fats and oily foods, and bulking the stool 11:42.

Botox is given immediately when intestinal continuity is established 12:24. First post-operative visit is at 2 weeks for routine check of eating, growth, and rash assessment 12:34. Anastomosis check is performed at 4 weeks post-operatively in clinic using Hagar dilators sized appropriately for child's age 12:38. For a child undergoing pull-through at around 10 months of age, proper anastomotic size should be 13 or 14 Hagar dilator 13:15.

The transferable judgment: waiting for toilet training in total colonic Hirschsprung creates sphincter dysfunction that is harder to manage than perianal excoriation. Operate when the child is growing well and the stool is formed, not when they can sit on a potty. Monitor urine sodium, not just serum sodium, in patients with long-standing ileostomies — total body sodium depletion will limit growth even when blood work looks normal.

Takeaways from this story

Waiting for toilet training in total colonic Hirschsprung creates proctalgia requiring ileostomy recreation — operate at 6-18 months instead
Check urine sodium (not just serum) in long-standing ileostomies — if <20 mmol/L, supplement 3 mEq/kg/day PO salt to restore growth
Ileoanal anastomosis is preferred over Duhamel for total colonic disease — stasis in Duhamel pouch can decompensate ganglionic bowel
Liquid loperamide contains glucose causing hypermotility — crush pills in applesauce instead for post-pull-through stool management

Topic overview

This episode discusses the management of total colonic Hirschsprung disease, focusing on timing of definitive pull-through surgery after initial diverting ileostomy. Key clinical points include: optimal timing is 6-18 months when the child has good growth and thicker stool consistency (rather than waiting for potty training); urine sodium monitoring is critical for patients with long-standing ileostomies to detect total body sodium depletion despite normal serum sodium; both ileoanal and ileoduhamel approaches are viable, though straight ileoanal is preferred by the discussants to minimize stasis; and post-operative management requires aggressive skin care protocols, Botox injection, and parental proficiency with rectal irrigations given the higher enterocolitis risk in total colonic disease.

Key takeaways

Current practice favors pull-through at 6-18 months rather than waiting for potty training—delayed surgery risks proctalgia from stool retention.
Pre-operative stool thickening (diet modification, pectin, Imodium) and skin training reduce post-pull-through perianal excoriation.
Long-standing ileostomies require urine sodium monitoring—levels <20 indicate total body sodium depletion requiring oral supplementation.
Intraoperative frozen sections guide extent of resection—total colonic aganglionosis may extend 15+ cm into ileum.
Growth optimization before definitive pull-through is critical—manage high ostomy output to achieve solid stool consistency first.

Keywords

total colonic Hirschsprung disease aganglionosis constipation enterocolitis proctalgia anal sphincter spasm failure to thrive dehydration electrolyte abnormalities sodium depletion skin excoriation ileostomy pull-through ileoanal anastomosis ileoduhamel

Hashtags

#HirschsprungDisease #PediatricSurgery #ColorectalSurgery #IleostomyCare

Transcript

Click "Show Transcript" to view the full text (14790 characters)

Hey there listeners, this is Rod Gerardo and it's Amanda Jensen from Cincinnati Children's. If you haven't downloaded the new version of the Stay Current pediatric surgery app, it's in the Apple App Store and in the Google Play Store. If you're listening to this podcast on anything other than the Stay Current app, that's OK. Just know that we're making these podcasts specifically to be played in the app. You can bring up journal articles, radiographic images, research articles, links to videos in the app. You can get all of that if you listen to the podcast through the state Current pediatric surgery app, so download it today. Until then, enjoy the episode. In the last episode, we left you on a little bit of a cliffhanger, we walked you through a patient who has total colonic Hirschprung disease, but we kind of stopped halfway through the case. So to recap, I brought some friends. Hey, it's Hiraman from Nationwide Children's Hospital, and it's Amanda Jensen from Cincinnati Children's. All right, can you two please help me summarize last week's case? We talked about a patient who's 10 months old and presented to the ER with abdominal distention, had severe constipation. No fevers, they're not septic or anything, but Remember, this patient had a history of delayed passage of meconium at birth, and that was diagnosed with the contrast enema at the time, which demonstrated a very amorphous round colon. So now this patient is in the ER. He's 10 months old and X-ray and contrast enemas shows severe constipation. So what did we do? Given the history, a rectal biopsy was performed which showed no ganglion cells. So we went to the OR. Intra-op frozen section biopsies demonstrated total colonic ganglionosis. Wait, no ganglion cells? No ganglion cells until nearly 15 centimeters on ileum. So they didn't do the pull through that day. Instead they did a diverting ileostomy and closed to wait for the final pathology. What a doozy. So, here is our part two of total colonic Hirschprungs. Without further ado, this is the colorectal quiz. So I think this is a really interesting topic. That's Dr. Jason Fisher from Cincinnati Children's Hospital. When I started practicing. I had one approach to these patients, and currently I have a little bit of a different approach. It's 180 degrees. That's Dr. Mark Levitt from Children's National in DC. 5, even 5, 10 years ago, my recommendation in these cases was to wait until the child was potty trained for urine and could sit on a potty. Because think about it. Then when you go do that pull through, you're gonna pull through to the anus and then loose stool will cause excoriation of the skin around that area. They actually studied this, and it turns out there's not that much of a difference between excoriated skin in younger patients versus older patients if you wait. So there was really no advantage to waiting. I've had two patients with, that I waited until they were older, and then we did their pull through, and they held in the stool so efficiently, that they got proctalgia. Which is an anal sphincter spasm, unresponsive to Botox that I needed to recreate the ileostomy. And that's the moment that Doctor Levitt changed his own practice. So what does he do now? So now my routine is to do this case somewhere between 6 and 18 months. So what is Doctor Levitt actually waiting for? He's waiting until the child has good growth. That seems to be what. Fisher does as well. So I'm in the exact same boat. So how do you get them to the point where they're having more solid stool, you know, when they're on more of a solid diet and table food diet, and maybe we even add some thickeners to their diet so that their stool becomes more thick. If the ileostomy output is really runny, you want to wait until you can get that stool to a more thick consistency, and If diet alone isn't working. Other ways to manage this high output is with use of pectin or Imodium to thicken the stools as well. Another cool trick by Doctor Frischer is to actually expose the native or virgin skin to stool. Some skin training, if you may. So it's not surprised and gets excoriated by the time the patient is stooling. He does that by having the family take the stool from the ostomy bag and put it in their diaper for a few minutes. The skin, the buttock skin, has not really seen or been exposed to stool. And so I have the families put some stool in the diaper and expose that skin to stool for a little. While, you know, 1520 minutes in the diaper, to sort of strengthen that skin. On that note, keep in mind that this patient got their ileostomy at 10 months old. I mean, usually these patients get their ileostomies in the newborn period, or think of the patient with necrotizing enterocolitis, they'll have that for maybe a couple of months. But these ileostomies can be there for 6, 1224 months. What do we worry about with long standing ileostomies? Because we're all fearful of dehydration and electrolyte abnormalities, a standard distal ileostomy, usually we're able to keep up, and the child is able to keep up from a fluid and electrolyte standpoint. But the one thing that I learned checking a urine sodium, the total body sodium and not just the blood sodium, is a key component for these patients, which is obviously important in growth. And so I often check a urine sodium. Near a few weeks after the, the, the um creation of an ileostomy, prior to discharge, um, a month or two later, and making sure that the patient has the right total body sodium, not just blood sodium, and it's easy to treat. You just treat with some salt tabs or some salt. Addition and you could correct that abnormality. Hira, can you summarize that for a patient with a long standing ileostomy, especially the ones who have high ileostomy output, they have failure to thrive, they're not growing well, check for a urine sodium, which is a calculation of your total body sodium. If it is less than 20, you need to supplement with oral sodium because the GI absorption is much better than IV. All right, well, get your cookbooks out. Here's our recipe for the PO sodium supplementation. You add 3 extra mil equivalents per kg per day. The recipe is 1 tablespoon of salt plus 40 mL of water. That's gonna give you 2.5 milli equivalents of sodium per mL. You do that for about 1 to 2 months, recheck the urine sodium. Plus there's another reason that PO salt might be better for these patients in particular. If you have salt in your GI tract that you've taken in orally. You're much better at absorbing glucose, so you have a better absorption of glucose and better nutrition. Wow, because you're really going there, biochem, 2nd year of college. Sodium is the most abundant electrolyte in the extracellular space. It's acquired through dietary intake, and most of it gets absorbed actively in the ileum, but passively in the djunum. I did not think the colorectal quizzes would start talking about the sodium glucose transporter. You've gone there. Well, listen, uh, you know, we have to keep, we have to keep this audience excited and interested, otherwise they won't, they won't come back. So, if the patient is not growing well, Doctor Levitt will usually tell the family, please have your pediatrician check a urine sodium. It should be greater than 20 millimoles per liter, and if it's less than that, that means the baby is holding on to every morsel of sodium they can. They're not letting any out in their urine. And then their serum sodium is probably gonna be normal, so it can kind of trip you up. All right, let's move on to the type of repair. Doctor Frischer has some thoughts on preference. For this case, I typically do an ileoanal anastomosis, a straight pull through, but there are a few other options. Being here in Cincinnati, I think we'd be remiss not to mention Doctor Lester Martin, uh, and his contributions in this field. Do you remember the, uh, The education policy No Child Left Behind. Well, I am a big fan of No Hirschprung's Left Behind. Anything that you can do to minimize the amount of Hirschprung's that you're leaving behind, the better off, because stasis is not our friend, and a Duhamel leads to stasis, and the ultimate Duhamel was the Martin, which has been abandoned, where essentially you use the entire sigmoid and left colon, too much stasis. And then, there was another procedure, the Camura procedure, and that's the right colon connected to the ileum in the form of an ileostomy. I've actually had to go in and remove the right colon patch. Now, I will tell you that an ileoduhamel with a very short pouch is a very nice operation for total colonic, and I, And I've seen many, many patients do quite well with that. It's not my personal preference, I do straight ileoanal, just like you do. But Doctor Levitt admits that he's a little bit biased because he sees a lot of patients who have failed Duhamel's. And I think the issue is not the Duhamel itself, I think the issue is the ganglionic bowel, and not all ganglionated bowel is created equally. The vast majority of DuMLs do fine. It's just that sometimes the ganglionated bowel will decompensate when there's slowing down of stool in the Duomel pouch. And a good ileoduhael with a very short pouch is a very good operation. And I'm very happy with the ileoanal, but if you do the ileoanal, do you also redivert? I talk to the families and I, I say it's a game time decision. So if at the end of the case, the anastomosis looks fantastic, blood supply is great, there's no tension, and you know that from a nutritional standpoint, the patient is really well optimized. I'm happy to not leave. It diverted. But if there's any concern with any of those factors, I, I also am not shy about diverting and allowing things to heal and coming back 68 weeks later to um close that ileostomy. In theory though, an ileoanal should not require diversion. The other thing, of course, you need to worry about is if you do divert more proximal, you may make them a little bit short. They do not have enough intestine proximal to that ileostomy to have adequate absorption for nutrition and ultimately have a high output ileostomy, more so than the one they had previously. So, another consideration when you're talking to the family. I make sure. Whether or not the family is comfortable with rectal irrigations. Because we know that these patients with total colonic Hirschprung disease are more susceptible to a severe enterocolitis, compared to the traditional rectosigmoid Hirschprung patients. And then you've got to think about the medical management. First line is diet, and you can start that before you even do the pull through, just to monitor how the bowel movements are. And then if you need medication. My first line treatment is usually with loperamide. That comes in a pill form or a liquid form. Just keep in mind the liquid form has glucose and the sugar can cause hypermotility. That's not going to help. Otherwise you could take the pill and crush it and put it in some apple sauce. Let's break down the food part really quick. From a diet point of view, you want to avoid sugar. Berries are particularly offensive. Try to avoid fats and oily things like that, and you want to really want to try to bulk, bulk the stool. There are other modalities. Um, we used something called, um, Levsin very successfully. As an added uh medicine to slow down the stool. We've talked about cholestyramine previously on this podcast. There's Lomotil, which is anatropine, diphenoxyate, atropine. Uh, in the United States, that's a, what is it called, um, controlled controlled substance. Thank you. Now we got some last minute questions here. When, when do you see this kid back in clinic, and how do you check? I give the Botox immediately, um, when we have intestinal continuity, and I see the child at 2 weeks post-op just for a routine check. That's just making sure that the kid is eating well, growing well, making sure the rash is. isn't too bad. I don't check the anastomosis until 4 weeks. At 4 weeks in the clinic, I do an anastomosis check, usually using Hagar dilators to size, to the appropriate size for the child's age. So you, you have them in clinic, and you sort of gently start putting in the dilators. First of all, I know what size it is when I leave the operating room, so I have a, a knowledge, a background knowledge of What size the anastomosis was when I leave. But in the OR in the clinic, I'll start with like a 7 or 8 Hagar dilator and gently size up to any resistance. One, I'm not here to stretch it out. I'm just here to check the size, and if, in this child, a, uh, to pull through at around 10 months of age or so would probably be a 13 or 14 Hagar dilator should be the proper size. What a case. We took you from last episode through the workthrough, and then this episode, we talked you through the perioperative considerations and some different approaches. I think we're ready for a summary here. Amanda, help me out. So to summarize, um, total clonic Hirschberg disease is very challenging. The optimal timing of pull through is debated, but should be performed once the ileostomy output is put in consistency and the patients are growing well. Both ileoanal and ileoduomel are great options for pull through. We should have a post-op skincare protocol in place after the. Through there's a great article by Villanova et al. that we can refer to. The role of Botox in total clonic Hirschsprung's disease is vital. Enterocolitis is more common in these patients. Currently there's no good option for preventing it, but only directed at treatment of enterocolitis. Parents should be extremely proficient with irrigations, and they should have a very low threshold to start them if there's ever a concern for enterocolitis post-op. Amazing work. Honestly couldn't have done it better myself. And listeners, don't worry, we're not gonna leave you without your weekly joke. Hey Mark. Did you hear about the guy who invented the knock knock joke? I did not. He won the Nobel Prize. On that note, I think we all have to leave. Say goodnight, and we look forward to chatting with you next time. I hope you guys enjoyed the episode. If you missed last week's episode and you wanna hear it, the best way to do it is in the stay current pediatric surgery app. You could just search colorectal, you could search colorectal quiz, you'll get the list of all of them. You can't really do that in other podcast players, but you could do that in the app. So download it today, it's in the Apple App Store, it's in the Google Play Store. But until next time, I'm Rod Gerardo. Again, I'm Amanda Jensen from Cincinnati Children's, and Perra Ahmad from Nationwide Children's Hospital. And just remember, knowledge should be free.

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