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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