The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique

Hey there listeners. How do you catch up on our podcast? Are you an Apple podcast? Are you in Spotify or Stitcher? Well, I want you to use whatever's easiest for you, but you should know that if you're listening to this podcast on anything other than the Stay Current in Pediatric Surgery app, you're missing out. Especially for these colorectal quizzes, because we have images that Dr. Levitt and Dr. Frischer are referring to in the app. So you can click on it, expand it, and you can look at exactly what they're talking about while they're talking about it to you. We also have links to journal articles when they reference them. There's a lot of content on there that you're missing out on. So if you haven't already, download the Stay Current in Pediatric Surgery app in the Apple app store or your Google play store. But until then, enjoy the episode. Last week, Dr. Levitt and Dr. Frischer walked us through the surgical technique for your straightforward rectosigmoid Hirschsprung's disease. It brought along a couple friends: Dr. Aaron Garrison from Cincinnati Children's and Dr. Andrea Beddio from Children's National Hospital in Washington DC. It's such a good episode. If you missed it, then I would recommend jumping out of this one and going to last week's episode so you can catch it. But if you've been following along and you're ready for a more challenging case, well, here it is. Now, to refresh everyone's memory, this is a patient who has more proximal disease. And I say that because I know the phrasing can get confusing. So instead of saying short segment, long segment, we're going to try to stick with anatomic descriptions, transverse colon, hepatic flexure versus last week, which is more of a sigmoid disease. This time, they're going to walk you through the steps of surgical considerations for more proximal, as in transverse colon, Hirschsprung's disease. I'm Rod Gerardo from Cincinnati Children's and this is the colorectal quiz. Now let's talk about the conduct of the operation when we have a more difficult transition zone. If you're in the Stay Current app, scroll down a little bit under the media player, you'll see some images. Pull it up. We have an x-ray and a contrast enema, and that's what Dr. Levitt's going to be talking about. Let's say we have this kid, you see the contrast study, mid transverse looks dilated, the rest distal looks decompressed, and you do your biopsy and you find ganglion cells in the mid transverse colon only. And the biopsy that you did in the sigmoid and in the left colon were aganglionic. Now, what do we do? We're in the newborn period. It's a baby that had done well with irrigations and we started off by doing our biopsies and here's where here's where we find ourselves. Um, well, I think like you we mentioned in the last podcast, sometimes people take too many biopsies. So I think just, you know, confirming sort of that left colon um, is still aganglionic. We've done that. And then you said transverse colon, we biopsied and that um has ganglion cells, but you didn't say whether they're hypertrophic nerves. So have they commented on that? Yeah. Yes, so sorry, the um the the sigmoid and the left colon are aganglionic and hypertrophic nerves. The mid transverse biopsy is ganglionic and they they can't find any nerves. There's no comment. So I think these are the cases that are a little uh stressful, especially early in your career and you're trying to decide uh what to do. And I think kind of keeping in mind that you can always get out of the OR without doing something permanent is is kind of the goal. I think that's an awesome point. A really take home point. This is all based on frozen sections. That's right. We're still in the OR. So you don't have to make a decision just now. In fact, in the back of Dr. Garrison's head, he's thinking in the back of my mind I would be thinking about whether we need to uh divert the patient either with a colostomy or or more likely a an ileostomy. You do not need to do this pull through today. Instead, you could spend your time getting better biopsies all over the place, sigmoid, left colon, splenic flexure, transverse. You could do hepatic flexure or right colon, and then you got a choice. Divert at the dilated portion of the mid transverse colon or divert at the ileum or I'll even give you a third option. Close all your biopsy sites and stop, wait 4 days for the pathology to come back and then do your pull through. So you're waiting for your permanent section, because when it comes to frozen, you can rule out Hirschsprung's disease, but you can't rule it in. And what's the nightmare situation? And I've seen cases like this where they are not finding gangling cells and particularly makes me nervous when they're not finding nerves. And in fact, there are ganglion cells there. You don't want to throw out a piece of code that might be good. And then the other thing to remember is the hypertrophic nerve concept is mainly a sacral nerve plexus entity. So hypertrophic nerves are sigmoid and rectum. So anything more proximal, don't worry about the nerves. Really you just want to know, are there ganglion cells, yes or no. I do think if you're going to take that third approach which you mentioned, which I'm not suggesting we do of doing a primary pull through 4 days later, you have to make sure you're in a situation where that child is is getting irrigated and doing well with the irrigations. Because you don't want a baby who's going to still smoldering and having low grade or enterocolitis like features, um, and delaying the diversion which will make that baby better. See that's that I think that's a very vital point and that's the reason why I personally would do colonic mapping and an ileostomy because I really want to hit a home run today and get that baby well. And I'm always a little nervous with frozen section and a diverting colostomy because what if it's not in a good level, whereas an ileostomy almost definitely is going to divert successfully that patient. Of course, you need to send a biopsy from that ileum. But if you're going to do this empirically, remember, there are there are many places in the world that do not have pathology to help them. They need to divert in the dilated segment. And that is a very reasonable strategy to dilate and to divert in the dilated segment and if that bowel then works, then that's where your pull through is going to go. And then, don't forget, mark your biopsies with a permanent suture. That I think is the key that we haven't mentioned. I use prolines, permanent suture and I use different number of tails for each biopsy. It's not fun. It's like you're going to biopsy everybody. And then in my operative report, I label those biopsies. I think that's important because going back in a few months later, then Dr. Levitt mentions if you're in a part of the world where you really can't do an ileostomy because you're worried about dehydrating the child, then you have to consider leveling at the colon. You know, if you're not going to pull this kid through for six months is feeding that colon important? Not sure we have the answer. Would you I think some people would probably ask if you're going to do an ileostomy and leave that big colon in, would you do a long mucus fistula or like a sectomy to be able to irrigate or do you just leave that dysfunctionized colon behind and um curious what you guys think I typically would not. I think there're sort of two varieties that this comes in. Um, that's Dr. Andrea Beddio from Children's National Hospital in DC, and she brought up a good point. Hypothetically, let's say this patient comes in after several months and the colon is incredibly backed up. The stool can be really chalky and irrigations aren't going to do anything. But if you don't have that issue, I think the colon can stay without really needing a way to irrigate it and that's a smaller population of kids that are going to need that colon addressed. Mark, can I ask one question? Sorry, but I think there's a question I get asked a lot. Where is your transition from primary pull through to hey, damage control, let's not do any harm. Is there a location? Where do you say, hold your horses, time to change the the direction here? So our our hard and fast rule here is if you don't have ganglion cells and you're at the splenic flexure, you're not doing a pull through today. You need to wait for permanent section because I agree with that. I mean, I think there's functional outcome differences if you start pulling through transverse colon versus pulling through left sided colon and it would be upsetting if you if pathology just wasn't, you know, right at that time for whatever reason and waiting I think to get that final pathology uh could make the difference for that child. So if the frozen sections make it seem like you're going to need to do an anal, pump the brakes and reassess because you don't want to resect more colon than you have to. You need colon. If you have even a little bit of colon, if you have your right colon, you can make one one stool one form stool per day. So I think that brings up a like the real pearl. And we said this at and what Aaron and Andrea really highlighted right at the beginning. And that is do no harm, especially for these complex patients. All right, getting back on track. Sorry, I keep diverting us. Mark, get a little slow there. Okay, okay. So let's say we did all of this. We made our ileostomy and we have our permanent section which shows what we thought previously. We have a transition zone in the transverse colon. It's pretty proximal. So now we have to do a pull through, but look at this distance. So I think it's a nice uh exercise to think about what blood supply you're basing the pull through on. And so at mid transverse, you're going to be sort of right colic, right? And and in order to bring that down, you're going to need to derotate the bowel, otherwise you're going to be bringing that mesentery right across the duodenum and creating an obstruction. So you need to be able to bring that down in the right pelvis. Yeah, I I agree with that and you need to ligate the middle colic and very likely ligate the right colic and your blood supply is dependent on your ilio colic and the marginal that parallels the right colon and then you derotate, so you put the cecum at the liver bed and you bring the pull through down the right side and you put all the small bowel over on the left side, which is essentially a a rotation opposite what you would do for a Lad's procedure. And really what happens when you think about this is you have a slight twist in your mesentery when you're pulling it down. So obviously making sure that the blood supply to your pull through is adequate and not kinked in any way is very important. My personal preference is to do this kind of thing open and I would probably do it through the incision for the ileostomy closure in this scenario. But I know um some folks have done this but it makes me a little bit nervous. Well, that's a tough case, huh? Yeah, discussed a lot of good points. Um, so you want to, you want um to sum up a little bit? So this was a baby with bilious vomiting and then contrast enema concerning for a proximal transition zone on the transverse colon or more proximal, and you don't have to do a pull through today. Instead, think about potentially doing an ileostomy or a leveling colostomy depending on the situation. And then when the baby is better and you're ready to do the pull through, you have the permanent sections, you know exactly where you're going to go. Look at the blood supply. You might have to do derotation of the right colon to get the colon to reach the pelvis and to not kink off or have that mesentery of the right colon really making an accordion string and that might cause a duodenal obstruction. Yikes. So, a lot more complex than last week's case, a lot of great learning points and don't worry. I got not just one, but two jokes for you. Want to hear a poop joke? Nah, they always stink. That's terrible. Terrible. I've gone way down. And here's here's a backup to that joke, okay? What do you call a snobby criminal going down the stairs? A condescending con descending. Oh boy. I think we both digress. You guys might have killed this feature. Well, I can't always guarantee we're going to have quality humor, but we're always going to give you quality surgical technique. I hope you enjoyed the episode. I'm Rod Gerardo from Cincinnati Children's and remember, knowledge should be free.