ERN eUROGEN Colorectal Webinar Series: Redo surgery for pull-through in Hirschsprung's Disease: When and how

Good evening everyone and welcome to this joint Eurogen Ernica webinar. My name is Olivia Spivak and I am a project manager for Ernica. Before I hand over to our moderator for today, I would like to take this opportunity to tell you a bit about the ear ends. European Reference Networks or ERNs are networks of hospitals and patient organizations from across Europe working together to support patients with rare and complex diseases, their families, and healthcare professionals. This is done by pooling together expertise, knowledge and resources from across Europe. The ERNs were set up in 2017 by the European Commission, and each network is focused on a different rare and or complex disease area. Two ERNs have teamed up to set up this webinar today, and that's Eurogen, the European Reference Network for Rare and Complex Eurogenital Conditions, and ERICA, the European Reference Network for Rare Inherited Congenital Anomalies. The topic for today is redo surgery for pull through in Hirsh Spring's disease, when and how. So without further ado, I'd like to hand over to Professor Yvo de Blau as moderator of this webinar to introduce the speakers. Thank you, uh, uh, Olivia. Um, it's, uh, with great pleasure. I'd like to, uh, introduce the two speakers of today. Uh, on behalf of Eurogen, Anika, and the URSA, these webinars are organized, and today we'll talk about redo surgery for pull-through in Hirschrung's disease, when and how. And today's speakers are Mark and, uh, Julia, Mark Levitt. Um, I guess most of you, uh, know or should know him. He's director of the co-director Program in Washington DC's. I don't think he needs very much introduction. He's known for many lectures, courses, uh, we've been working together, um, a lot on colorectal surgery. Uh, and he has a great knowledge and, uh, a great experience, a lot of experience also in renal surgery. Uh, today's other speaker is Julia Vsigheli from Johannesburg. Uh, and she's running a program, the co-director program in South Africa, Johannesburg, South Africa. Uh, and has been working in the colorectal surgery for many years and working together with Mark as well. Uh, and they together will give the presentation. I hope it to be, uh, interactive, which is sometimes difficult in this type of webinars, but feel free to ask, uh, any questions on the chat. Uh, uh, the, the questions will be forwarded and, uh, we can discuss them, uh, afterwards. So first I'd, I'd like to give the, the word to the floor to Mark. Fantastic. And maybe uh Julia can join at the same time. There you are. Great. Um, well, we're thrilled to be here. Um, Julie and I go, go way, way back. Um, and, uh, this, uh, problem of Hirschberg's disease and is near and dear to our heart. And I know the 300 or more participants on this, uh, webinar also suffer in trying to make these patients better. And, uh, maybe we'll suffer a little bit together. Because I do believe that many patients with Hirschrug's disease who seem to be doing poorly after their pull-through. There is an answer. There is a solution for them. Um, and we wanna try to get to those solutions today. Um, all right, great. I think, um, a better title perhaps is the Problematic Hirschprung's Patient cause not everyone, of course, needs redo, and that's what we're gonna come up with today. So let's, uh, proceed to the next slide, please. All right. I think the first thing to recognize are the different types of operations for Hirschprung's disease. And it's really important to know the anatomy. And interestingly, I think surgeons know this anatomy well, but gastroenterologists who many times will take care of the problematic Hirschrung's patients might not know the details of the anatomy. And it's really in the anatomy where the problems patients have. can be identified and ultimately solved. The operation that is the most pure and leaves behind the least amount of Hirschprung's disease is the Swensen that you see here on the left. The problem with the original Swenson is the surgeons were doing the rectal dissection too wide, and they were injuring the nerve errientes and the patients were left with various morbidities involving the bladder emptying, for example, and the ability To have erections. However, when that operation is done correctly in the proper plane, same plane as you would use for a PSARP, the Swenson is an outstanding operation and leaves behind no Hirschprungs, except, of course, the sphincters. To compensate for some of the problems of the original Swenson came the Duhamel procedure where the original rectum was left behind and the ganglionated bowel was pulled through and anastomos to it. The problem with this operation is that it leaves behind a segment of Hirschprung's, i.e., the original rectum. Although it does not in any way affect the anal canal or the sphincter mechanism, and in that way, it's a good operation. However, it imposes some stasis, and we will get to that. And then the new named procedure, the Yancey Swave procedure, for 60 years was called the Swave procedure. I want everyone please to start changing the name because Doctor Yancey submitted a paper 12 years earlier than Suave with the exact description of the technique. And Suave then wrote his paper 12 years later, uh, working separately. He was in Italy, Yancy was in the United States. Sadly, Yancy's paper was not originally accepted because of the color of his skin, and he only was able to publish in a journal that was not well read. So many of us are working hard to rewrite this history and call this operation the Yancey Suave. And in these circumstances, a muscular cuff was left behind. A submucosal dissection, a la ulcerative colitis operation, and the pull-through is then done. However, the cuff that is left behind can be problematic. Next slide. All right. And here are some pictures for your reference of the original Suave procedure in 1964. Next slide. And there's some pictures from the swabbe procedure where the bowel was left hanging out of the abdomen. Quite different technique today. Next slide. And Yancy in 1952, wrote the same procedure. So he really deserves the credit for this very cool idea, which was a modification of the Swenson. Today, many of us, uh, the two speakers today and our moderator, do a Swenson. We went back to the original Swenson because I believe it's the best of the operations, but we can talk details about that as we get there. Next slide. All right. I'm gonna start off with this statement and that all patients with Hirschsprung's disease should stool spontaneously and be cleaned for stool. If a patient with Hirschprung's disease is not stooling spontaneously after their pull-through, something must be wrong with the pull-through, or the sphincters have not been managed satisfactorily. These patients should be stooling, and all patients with Hirschsprung's are born with the capacity to be clean for stool. If they are not clean for stool, something is missing in their medical management, or they may have an iatrogenically caused problem with an injury to the either the sphincters or to the dentate line. And this is what we are gonna talk about today. Next slide. All right, we divide the problem into either soiling. Loose stool, uncontrolled stool, or obstruction. Next slide. OK, let's discuss this patient. So Julia, you have a patient in your clinic who had previous surgery by another surgeon with Hirschprung's disease, and they have been lost to follow-up and they come to you around the age of 6, and they are soiling. The good news is that you find that their anal canal and their sphincters are good. It seems the original surgery was done well. What is your interpretation of this, but don't give the answer yet, because the audience has an opportunity to, uh, to give their answer. Am I right, Luz? The audience should be able to, we should be able to pull the audience. Now launch the poll, so you can all answer it. OK, here we go. So Julia, before you give your answer, I wanna know what everyone's answer is. This patient's sphincters are intact. The dentate line is intact. And you see the contrast study. Was this a colon that moved too slow, moved too fast, has a residual transition zone, or has a stricture? Before I start giving my answer, can I, uh, can I say that, um, it, it is absolutely very important to have a proper history. So if the records for the patient are not available, which happens in most cases, for example, in this setting where I work, most of the times, you absolutely don't know what happened to the patient. But having a proper history, meaning asking the mother, um, is there abdominal distention, what is the frequency of stools? Is the patient soiling, already gives you a lot of information. And then in most cases, you also should be able to perform an EUA um either in clinic or in theater and in most cases, by the time you have a contrast enema, you also have a full thickness rectal biopsy. Um, but, uh, so these are the information that, um, I usually needed for these patients that are not doing well. Um, but, um, yeah, I can see that. I was intentionally a little bit cryptic with this case because I wanted to see what would happen. Everyone here is quite, quite correct. We have to be sure there's no stricture. We must do an exam, to Julia's point. We have to be sure there is not a transition zone. We have to repeat the biopsy. And having that data in advance and those results being normal, we conclude that the patient's colon is moving slowly. All right, let's see the next slide. And now, to compare this situation, let's say this patient has that evaluation that Julia just described, has no, has good anatomy. So let's forget about C and D for the poll, because the transition zone is. is OK. There is no transition zone. It's a good pull through. There is no stricture. What is your interpretation of this image? Is this the colon that's moving too slowly or too quickly? And the point that I'm making here is that if the anatomy is OK. There are 2 kinds of patients with Hirschprung's, those that move too fast and those that move too slow. And I'm hoping the audience is gonna say that this particular colon is moving too quickly. So let's see what the results are of the poll. Some people thought that was moving too slowly, and it, it is a non-ilated colon. Julia, Julia, do you have any comments? Can we go to the next slide and just show that image again? There we go. Let Julia talk about this. Go back one, I'm sorry. No, I, I completely agree. And I think it completely highlights the importance of a properly done contrast enema in a patient like this. And I also think that the contrast enema is also a very dynamic, um, dynamic. Study to do. So, for example, um, it is very important also to specify is this immediately during the contrast enema or is it during the evacuation time for the contrast enema? Because that also tells you what you're looking at. So, um, yeah, I, I, I totally agree with what you're showing, but I'm just saying that sometimes when you're showing, when you're At your X-ray meeting in your hospital and you're shown a static image like this without knowing anything about the patient, you panic a little bit. But then it, it all fits together with the patient that you, that you have in front of you. And yeah, it's all very important to know also to plan the bowel management later. Very good. So let me just summarize this section of the talk, and that is The patient needs a good history. You need to try to figure out what the original surgery that was done. They need an examination under anesthesia to look for things like a stricture, to palpate whether or not there's a cuff, to determine whether there's a twist, and to repeat the biopsy to be certain that you have not have a patient with a transition zone pull-through. Then you want to determine If all of that checks out, and all of that is well, there is no anatomic problem, there is no pathologic problem, then is the patient moving too quickly or too slowly. And the history is important, 5 stools a day without any distention. Is moving too quickly. 1 stool every 3rd day with distention is moving too slowly. And once you know that the anatomy is OK and the pathology is OK, you can treat that patient medically. If the patient is moving too slowly, you give laxatives. If the patient is moving too quickly, you can slow them down with fiber or loperamide. And then the one confounding factor which we will get to at the end, is how to manage non-relaxing sphincters, because that can cause trouble to a very good pull-through, if they're not relaxing their sphincters appropriately. All right, now we're gonna go through some pictures of our EUA of our exam. So, we're gonna go to the pole for this. This is an examination under anesthesia in an awake patient. We're about to put them to sleep, perhaps to do our biopsies. I think they need to see the picture a little bit longer. Can we? We don't see it. OK. So, what I saw there, well, well, Julia, what did, what did you see in that, in that image that flashed by quickly? I saw like A good perineum, although just around the anus, there was some excoriation. But if this child is asleep, he has a good tone. So I do think that the sphincters are overall good sphincters, but I do think that this child is experiencing issues with, um, with soiling probably, um, or uh enterocolitis, and he has acidic stools, and that's why he has such a nappy rash. That's my, sorry. The point that I wanted to make, if you can just show the picture now. Sorry, I thought, I thought it was a, so no, the tone is not that good. I retract what I'm saying, but it's not too bad. It's not completely pattuless, but it's also not completely closed, and there is some nappy rash around it. So I, I agree. This is a problematic sphincter. It may have been a result of overstretching at the original pull-through, and this is an important thing to notice on your exam. All right, now let's look at the next one. All right. Let's study this for a second before we go to the poll. Think about what your answer will be, but once we go to the pole, we lose the picture. So let's just look at this picture for a second. Examination under anesthesia, previous pull-through. And the question is, how did the surgeon do in preservation of the dentate line? Is the dentate line intact, missing, or partially intact? All right. Let's see what people think, and then we'll get Julia's opinion. Now, look at that. Isn't that crazy? All right, let's show the picture. Boy, we really, I really successfully fooled you because I wanna say this is a patient who has never had any surgery before. This is a rule out Hirschprung's patient that we're about to biopsy. This patient's never had any surgery of any kind. This is a completely normal and fully intact dentate line. And you can see though, you can see the little rugae and the little crypts. This is a very normal appearance. I'm gonna show you in a couple other slides, the loss of the dentate line, but I really want you to become good at knowing. What is a dentate line? What does it look like? And you must teach for the surgeons on the call, you must teach your gastroenterologist what they are looking at to determine whether the dentate line is intact. Let's see if you get the next one right, uh, audience. All right, here we go. Stare at that for a little bit before we go to the pole. This is a post Hirschprung's operation. You see a little stitch there. We've done a biopsy. Is the dentate line intact, missing, or partially intact? OK, let's see what we, what we people think. Yes, this is a completely lost dentate line. Very sad. Let's show the picture again so we can talk about it. Um, this dentate line is gone. There are no, um, there's no dentate line here at all. There are no crypts. The surgeon started the dissection too low, and what's missing now? Why is this a problem, Julia? Why do you need your dentate line? So because the dented line is um the area of the body that can give you the exquisite sensation of your rectal, if your rectum and your anal canal is full, and it can also tell your brain if what's inside is solid liquid or gas and therefore they control the motor system um and contract the sphincters. Uh, by definition, Hirspring's patients lose their reservoir with the pull-through. And if you destroy the dented line, they don't have a reservoir and they also don't have sensation, so they will be incontinent for life. Yes, and let me, let me show you a very extreme example. The next slide shows a very sad and extreme example of the loss of the dentate line, overstretched sphincters. You can go to the next slide, Loz, please. And in a patient who has very little colon. So this patient is in very deep trouble and this patient most likely might need a diversion because the anal canal and the sphincters have been so uh lost during the original uh dissection. All right, I'm gonna show you the next slide, which is a very dramatic slide as well. This is a post pull through patient. What are your thoughts here? Julia. Like, my initial thought is, did this patient have an associated inectal malformation? Because the thought is that it was a pull-through that was done at the skin level, um, which is, like, there's no dented line left behind. Like, the pull-through segment wasn't astomo straight to the skin. So I wonder if behind here, there was also an associated in erectal malformation or it was some caustic enema, because if it was a pull-through for Hash burns, then it was a disaster. Yes, I, um, it was a pull-through for Hirschprung's. It has, um, a significantly overstretched sphincters. Now, I wanna bring you some new information about what to do in this circumstance. First of all, the most important thing is to avoid this. You need to not overstretch your transanal dissection. I think one way to accomplish that is to use laparoscopy for most of your rectal, deep pelvic rectal dissection, so that the transanal portion of the operation is relatively short. Don't overstretch by, by putting those pins in in too aggressive a manner. And of course, preserving the dentate line. When you begin your transanal dissection. Well, there is some hope for some of these patients. And I'm gonna show you what I did here, the next slide. This patient had very impressive prolapse, and we removed a fair bit of this rectum. And then upon upon closure of the pull-through, what I did was I tacked the muscle. To the pull through, circumferentially. So, the overstretched muscles are now brought more into the midline and hugging the uh pull through now because of these bites of tissue. And let me illustrate that with the next picture. Imagine, next slide, Imagine you have a car driving through a tunnel. And the tunnel are the sphincters, and they are far away from the car. We want them to squeeze the pull through. We want those muscles to squeeze the pull through. So what has been lost here is not the muscles themselves. They are still there. They are just widely stretched, and the pull through is now too loose within that tunnel. So what I've What I've done in these circumstances, and we've done in about 10 of these cases now with very nice results. Again, these are the patients in whom the sphincters were overstretched. So this is new information, new article that I recommend you check out. We pull the muscles down onto the pull-through. And the next slide shows you a very cool illustration of the three dimensional. Anorectal manometry before and after tacking the muscles to the pull-through, and you can see preoperatively, it was a wide channel, no ability by that patient to squeeze their sphincters down. and successfully close the pull-through. But after the surgery, and this is several months later, now the manometry shows the success of closing the anal canal because the sphincters are now working. All right, let's go to the next slide, and now let's talk about obstruction. So the other half of the problem, soiling being one half, obstruction being the other half. Usually results from either an anatomic or pathologic problem. So what we're gonna show you now are a series of problematic pull-throughs, and I want you to tell us what is the cause of the patient not emptying and what are they doing by not emptying. They are having enterocolitis or chronic obstruction and distention or failure to thrive. So let's see the next slide. All right. Previous pull through. The the pre previous pull-through after a preceding ileostomy, the ileostomy is closed, and now the pull-through is allowed to function. And the patient starts to vomit. What is your conclusion? Is this a small bowel obstruction? Post-operative ileus, a distended colon, sigmoid valvulus, or a perforation. Think about that. Liz, I think we need to give them a little bit more time with the X-ray. Can you just go back to the X-ray for one second? I'm so sorry to do that. There. All right, take a look at that. This is a very typical scenario. It's post-op day 2. You've closed the ileostomy after doing the pull-through. Your patient starts to throw up, the nasogastric tube is placed. What is the cause of this problem? OK. So now let's go to the poll. And then Julia is gonna tell us. Look at that. So, all right, go to the next slide which will show the picture again I think. Cool, so, um, next slide, let's see the picture. There we go. OK, I think you can go back, go back, go back. Sorry, hold on there. All right, what do you see? OK. You can see a little bit of backgrounds, small bowel dilatation, but there is one big loop of bowel there, which is in keeping with colon. Um, and I do think that if with this history and with this specific patient, I just think that there is a bit of um Contracted sphincter, and I don't think this patient is working yet. Like the, the, the stool is not coming and the gas is not coming out. So what I would probably do in this patient also to have an answer after seeing an X-ray like this is A PR or a gentle irrigation, even just placing a Foley catheter inside of the anus to see how much that bowel decompresses. Because this patient has not had ever had any stool going through the colon after the pull-through. So I just think he's experiencing some um constipation and, um, like withholding gas because the sphincters are not used to work anymore. 100% correct. Obviously, 2 days ago when the ileostomy was closed, you made sure to check that there was no anastomotic stricture. This is because this pull-through has never been asked to do any work and it's obstructed. So actually, in this patient, a nasogastric tube was really not necessary. The patient, what they really needed was an irrigation from below. No, that's perfectly safe. This patient has a perfectly healed anastomosis already. And an irrigation would have made this patient much better. And I think it's good to learn the diff distinction between small bowel dilatation and colonic dilatation in managing some of these patients. All right, let's try another case. Let's go to the next slide. All right. What's wrong with this pull-through? This is a contrast study and an examination. Don't go to the poll quite yet. I'll tell you when, lose. Um, this is a problematic patient. They're having enterocolitis episodes. We've done our contrast study. We've done our examination under anesthesia. And we need to find the diagnostic cause of the obstructive symptoms. Anastomotic stricture are your choices, retain transition zone, Duhamel pouch, or an obstructing Yancey suave cuff. OK, let's go to the questions. Let's see what the audience thinks. All right. Are we getting some good responses? Very good. So this is clearly an anastomotic stricture. I think uh um the possibility that it could be transition zone is certainly there, but it's a very obvious stricture, a stricture. On exam. Um, the contrast study doesn't really show a Duhamel, um, but it could be a cuff also. I think the most likely is what you see right before you, which is a stricture. All right, let's go to the next one. All right. So this is the same story. This is a patient who has a pull-through, having recurrent episodes of enterocolitis, and what's most likely wrong with this pull-through is it retained transition zone, a Duhamel pouch, a cuff, a twist, or a stricture. I think the only one thing I I would like to say is that sometimes um when you see an AP contrast study, it's not always easy to understand um like um What you're actually dealing with. Sometimes that the lateral reveals a lot, I think, especially when it comes to the duhanal pouch, um, or I still have a cuff. Just because you really want to look at that presacral space and see if it is increased and you also want to see how the bowel goes into the rectum because if you have a big Um, a big dilated loop and then something insert in Poiri, you already know that you're dealing with the duomo pouch. So sometimes also the AP is not enough to have answers, um, although it usually hints, uh, what's the issue. I 100% agree. Let's look at this image together. Again, All right. So, first of all, the top panel. It looks like the patient still has Hirschprung's disease. The distal segment is narrow. So that's very obvious on the first image. The second image is a different patient and has this very dilated structure that I would consider missinghaustral markings. Remember, the patient has had a pull-through. So this should be sigmoid with no Haustral. And to give credit to my wonderful partner here in Washington DC, Andrew Badillo, she says, it looks like a summer squash. And this is a very typical picture of a retained transition zone where the piece of bowel that's at the bottom is smoothly contoured with no haustral markings, no peristalsis happening in that bowel. This is very typical of a transition zone. Two different images. One, the top one, looks like Hirschprung's disease where it's narrow distally. The bottom one. Looks like a summer squash, meaning the dilated segment is there with no action, no, no activity in that segment of bowel and that patient needed a redo. I completely agree with you that we need a lateral, and I think that will be very nicely described, uh, two cases from now. Let's see the next one and see what everybody thinks. All right. Similar scenario, we have a patient who's having recurrent episodes of enterocolitis. And has this contrast study. Would this be, again, wait for the poll, let everyone study this for a second, anastomotic stricture, retained transition zone, Duhamel pouch, twisted pull through, or a cuff, obstructing. And let's go to the poll. So everyone commits to a vote. And then shortly we'll have uh Julia. To her analysis. All right, very good. So, um, let's show the picture and as Julia talks about what she saw on this image. So, again, um, the importance of a dynamic study, but these two pictures I think are fairly striking clear. So you do have a quite a dilated colon, um, proximally, then you have some sort of cut off and then you have a narrow. Um, pull-through segment and uh you can almost see it, um, in, in the images itself, like, um, it resolves and then it comes back there. So, I think it's very much in keeping with a twisted pull-through. Um, the anastomotic stricture, you would see more distal, um, and, um, so it's more proximal to where an anastomosis should be. The transition zone could look similar, but you have a, like a swirl, swirl of contrast here. So, um, I, I, I wouldn't say B. The Duhamel pouch, it doesn't look like this because you have a very dilated, um, uh, pull-through segment and an error segment that comes through. Um, and then an obstructing calf, yes, it could look like that, but that cutoff here is not in keeping with that. So I do think that the twisted pull through is the most correct, um, diagnosis like the audience was saying. I would agree. All right, let's do another one. All right. Multiple episodes of enterocolitis. Can you tell from this? Was the original surgery a Yancey Suave, a Swenson, a Duhamel, or I can't tell from this image. So look at this image closely. Something is obstructing this pull through. To me, it looks like this patient had a Nissan fundoplication of their pull-through. All right, so let's see what everyone thinks. And I'm gonna show you some very cool images. Yes, that's correct. So, let me just show you the next slide. Actually, let me show you this slide first. You make a note before you go on uh to the next slide. Look at the presacral space here. There's too much space between the pull-through and the sacrum. That is the cuff. And on exam, you can feel that cuff. You do a digital exam, you feel your finger along the sacrum. And you can feel this rubbery tissue that's sort of obstructing your pull-through. And look how it looks like the pull-through is being strangulated. The next slide I'm gonna show you are 4 cases during which we remove this cuff. There's like a band of tissue around the pull-through. I believe either the surgeon failed to split the cuff, or the cuff fused back, or it rolled up. Unclear what happens, but I believe it obstructs. This is one of the main reasons why I like to do a Swenson. And to complete the picture, let's go to the next slide. One of the problems we haven't talked about. So, can you tell if this patient had, A a Yancey Suave, a Swenson, a Duhamel, or you can't tell. And I, um, I do have a lateral for this one, but I just want to know what people think. Seeing, um, what I, what I hope you all see is impaction. In the rectal area. All right. So what do people think? What was the original surgery here? Just OK. OK. Yes, so that is correct. And let me show you the next picture. Is the contrast that, there we go. So here are two cases. Of Duhamel scenarios. And I want you to study these. The top 2 are colo, I'm sorry, the top 2 are Io Duhamels, and the bottom 2 are Colo Duhamels. Do you see the problem? Do you see what's happening to these Duhamels? These are all improperly done Dujamels. Julia, do you want to, uh, explain what you see here? What is the problem with these Dujamels? So, you have The anastomosis, which is, so in the Duhamel procedure itself, obviously, the rectum is left behind as a reservoir, and then the anastomosis is done on the posterior aspect of the rectum with either the ilium or the column coming through. So sometimes what happens if the window between the two is not open enough, you create a spur. And then, um, that rectum fills up with stool and then pushes back on the more proximal bowel and creates constipation in the more proximal bowel. So that is what you're seeing here in these two contrast studies. Um, I'm very surprised, I must say I've never seen it, the top one on the left, because it almost looks like the spur is inferior to, um, it, it's usually superior to the spur. It's not inferior. It almost looks like the li is floppy and pushing. The rectum at the bottom like this. So, the rectum is not that dilated. It is the ilium rubber that is now very dilated and pushing on the rectum anteriorly. Yeah, so I'm not exactly sure what happened in the original surgery at the top left, but it's clearly a problem. The two, the two lumens have not been successfully united. And what I believe happens is the original rectum fills with stool and compresses the pull through. And therefore the pull-through cannot empty. And if you were to unite the walls of the two lumens, then the good bowel could empty out the anus. But because this, the pouch fills with stool preferentially, it ultimately will squeeze closed and compress the pull through, and the pull through will be unable to empty. And the solution for this is actually very simple and showed on the next slide. You have to remove the common wall. So here is a nice picture of the two lumens. Nowadays, I would use an endovascular stapler, which is a little bit smaller, and you want to make the two lumens and complete the separation of the two lumens. Again, I'm not a Duhamelist, but I know that some. Surgeons are, and many Duhamels do extremely well. But you need to make a small pouch, and you need to make sure that the two lumens are mated successfully. And if you have a patient with a Duhamel who's not emptying well, you need to suspect this problem. If removal of this spur does not solve the problem, then the Duhamel pouch itself needs to be removed. And I want to illustrate that point with the next slide. And the next slide shows, I was once having a conversation with a family trying to explain to them why their child's pull-through was not working, and, and I asked the two parents, what kinds of cars did they have. And one of them had the car on the left, and the father had the car on the right. And then I said to them, both of these cars work quite well on the highway. About the same. However, if you go off-road, obviously, you would prefer the more, uh, the more, uh, the Jeep-like car, right? And I think this is relevant for Hirschberg's disease. Some ganglionated bowel does not work well. Even though it is ganglionated. So if you impose any stasis on healthy ganglionated bowel, that bowel can decompensate. Similar to the, the car on the left would decompensate off-road, but does perfectly fine on the highway. And what are the things that obstruct pull-throughs? Well, strictures, cuffs, twists. Retained uh transition zone, and a large Duhamel pouch that is not functional and not allowing the ganglion bowel to work. So, we have to respond to this. Now, having said that, if we know that there is no anatomy problem, we have ruled out all anatomy problems. We have ruled out all Pathologic problems, the biopsy is good. We are still confounded by the fact that all Hirschrung's patients have an absent, rare recto anal inhibitory reflux. They fail to relax their internal sphincter upon rectal distention. And the next slide. it gives us an opportunity to talk a little bit about when to administer Botox. And this is one of my favorite slides of the two dogs before and after the administration of Botox. And here is an anal canal. And just to remind you, a technique that I use is to give 100 units of Botox mixed with 1 cc of saline injected into the dentate line, hitting both the internal and external sphincter. Some people use ultrasound to help guide them. I don't. The purpose of this is to solve the problem of the non-relaxing sphincter. Because the non-relaxing sphincter is going to impose stasis and give patients a lot of trouble. Julia, do you wanna, um, And any commentary about Botox? Um, no, just, uh, I just wanted to say that we, uh, I was I, I wasn't used to Botox, um, before working with you. Um, and after our last meeting, I started using Botox as basically as a protocol. So all um alloanals do get Botox one month after the surgery, and if it is like a shorter, um, segment Hirschman's disease, they don't. And we actually have seen huge improvements. Um, so that has been really interesting from our side. Unfortunately, now, the hospital ran out of Botox, so I can't, uh, really speak anymore. Uh, but it has been, um, I, I strongly recommend it. It, it makes a huge difference in patients. Yes, it really does. All right, the next slide is a little bit of history. And then we're gonna open up for questions, and then we'll engage our moderator, uh, Ivo. So here are some of the dignitaries that have changed the world for many, many thousands and thousands of children with their contributions in Hirschprung's disease. Um, I actually was very lucky and I got to know Doctor Boli, who modified the original suave. I knew Doctor So, who was the first surgeon to do a primary pull-through for Hirschprung's in the Philippines without a diverting stoma. I knew Doctor Martin in Cincinnati, who did a lot of work with Hirschsprung's disease. Uh, Helen Noblette invented the suction rectal biopsy. I'm dear friends with Keith, uh, Jorgeson, who added laparoscopy to Hirschprung's disease. And Jack and Luis, uh, contributed the transanal technique. And of course, uh, sadly, my dear friend Dan Teitelbaum passed away several years ago, way too young, who made major contributions to the understanding of Hirschprung's disease, particularly enterocolitis. So let's do some questions. Evo, let's get you engaged, maybe open your camera and uh spend the rest of the time uh fielding questions from the audience or Evo discussing anything that you think we covered uh too quickly. No All right. Do we have, uh, do we have, uh, uh, audience, uh, questions? Evo, I can't hear you. What I'm having trouble hearing you. Ivo is, uh, is muted. Liz, do we have, uh, other questions from the audience to field? Uh, yes, I'm sending them through to you. I don't know if you can see it. Do you have any questions? Mm Where, where would I look? Um, under the gray box questions, otherwise, I'll read them out aloud. They're not here. Julia, do you see? I don't see. I don't see them on the questions and I don't see them on the chat, so I, I don't see them, sorry. Uh, OK, then I'll start with the first one. have you seen success with Botox in a retained swath curve? Yeah, that's a good question. I think it helps a little bit, but the symptoms come back right away. Um, you need to know if there's a cuff, and I would usually remove that cuff. But yes, sometimes those patients are getting Botox over and over and over again, and every time the Botox wears off, they immediately get their symptoms again. Those are ones I'd be worried about that it's the cuff that's the problem, and I would look for a cuff. Uh, a question from Professor Weitz from Niemeer. What is the risk on neurological problems and fertility? Well, that's a really good question. You know, if the surgery is done properly, There should be no neurologic implication. If the surgery is done improperly, like we talked about with the original Swenson where the rectal dissection is done too widely, the nerve erigentes can be injured and those patients can have urinary retention, and they can um uh lose the capacity to have erections. But, but a properly done surgery should have no ururologic implications. Another um question from Vicky Wong, how would you do the Botox injection? Julie, you wanna explain your technique? Sure. So, um, we do it as a daily procedure. We, um, just, um, position the patient in, um, with the legs up, um, and we insert a lone star. Um, usually, it is enough to use just 4 hooks. Uh, you don't need more than that. And then, uh, we usually use an insulin syringe just because we can dilute it better and you take 100 units and you dilute it in 1 mL of normal saline. Um, and then, uh, you use the smallest needle you have and you would inject at, um, 2 o'clock, 5 o'clock, 7 o'clock, and 10 o'clock. And you would inject 25 units in each quadrant and it's just at the dented line and you're aiming for the space between the internal sphincter and the external sphincter. Um, and then you just remove your lone stalk, and that's it. Um, we advise the mothers that, um, after they should be doing an irrigation for at least 2 more days, but then after 48 hours, they will start seeing the benefits, and usually by 7 days, it's full on, and it usually lasts up to 3 months. Thank you. And uh Kylas Bodnar, you asked us, how often do you give Botox? So, in my experience, um, if everything went fine with the pull-through, and it's just an internal anal sphincter akalasia now, um, usually 1 or 2 injections of Botox is enough, 3 months apart. And usually, by the time they are 6 months to a year after they pull-through, then they just grow out of it. And you don't need to do anything else. In patients that actually do need more than that, then I would start working the patient up on why the pull-through is not successful. So I would usually do the, do a contrast enema, I would do an EUA, probably a stimulation, and I would also do a full thickness rectal biopsy to exclude that the transition zone was pulled through. So the answer is usually 2 Botoxes are enough, maximum 4. After that, I would consider that something went wrong with the surgery. Uh, and, uh, would you consider other modalities as well, um, Julia or Mark? I mean, uh, in the past, people did some myectomies. Um, do you still consider this as a possible treatment? Or do you think it's it's not, it's more or less banned because of the Botox? Yes, I, I would never advocate uh to do a myectomy because it's permanent and it could leave the patient with permanent fecal incontinence. I would much rather provide temporary disruption of the sphincters, which I know will wear off from the Botox. OK, uh, Luz, are there any more questions from the audience? Yes, a lot. Um, I'll start at the top. Uh, a question from, uh, Mahmoud Marais. Can you please tell us about how long segment for the Heidi pull through do you recommend for laparoscopy? What about if you need to do the lawyer's procedure? Yeah, it's a very sophisticated question. Thank you for that. So my routine would be to do laparoscopy and biopsy. Um, you know, the proximal sigmoid, in most cases, that shows ganglion cells. If not, I would biopsy the left colon. If there are no ganglion cells on frozen section in the left colon, I will not do a pull-through today. I will do mapping and wait for permanent section. And then you can choose whether you want to do an ileostomy. I would confirm their ganglion cells at the ileum if you did that, or wait on your biopsies and come back 4 days later. My preference is to make the baby better and let the family go home and do an ileostomy. The reason why I say that is because if you keep biopsying the colon and not finding ganglion cells, you can't know for sure that That it's an accurate assessment. Remember, frozen section can only rule out Hirschsprung's disease. It cannot rule in Hirschprung's disease. You need to evaluate 100 levels of a specimen to know for sure that there are no ganglion cells, and there have been some very sad cases of patients in whom the entire colon was removed. An ileoanal pull-through done and the final pathology showed that the colon had lots of ganglion cells. So, I will never do a pull-through primarily if I do not see ganglion cells on frozen section in the left colon or the sigmoid colon. If I'm proximal to the splenic flexure, I will, I will do a mapping and wait and do the pull-through on another day. Now, having mapped such a patient and found that the right colon is good. I would even go so far as to say that the mid-transverse colon is good and everything distal is bad. That patient needs a de-rotation. And the questionnaire used the term Delos, which means the colon is de-rotated and brought down the right side. Very, very important maneuver for Hirschrun's. If you bring a mid-transverse transition zone down the patient's left side, the main mesenteric vessel will cross the duodenum and cause duodenal obstruction. You must do a de-rotation. It's essentially the opposite maneuver of a lad's procedure, where the colon goes on the right and the small bowel goes on the left and it Delos, as opposed to a lad's procedure, which you're used to. So, very good question. Now, I realize that we are running out of time. I do wanna throw out there my email, which is M Levitt, M L E V as in Victor I T T at children'snational.org. You are welcome to email me any questions that are persisting after this, and I would strongly recommend everyone get the Stay Current app. It's a great app, lots of good content. There is a colorectal channel there with all of the videos, and the one really fun thing there is the weekly colorectal quiz. So you can engage in a new quiz that I put out every single week. It comes out on a Monday. Any final questions, but I do believe we want to be respectful of the time. I agree, um. Lose Yeah, we have a lot of questions, but I think I'll do one last question and the rest I will send to you by email. OK. We have a question, um, in RM reconstruction, almost in all cases, the distant colonel is trimmed, just removing the dentate line. Yet almost 80% of the low types are constipated. How can this be understood? I don't know if I understand the question, but on a malformations or a from. I think at the same time it's to say how come that in Hirspring's disease, um, like they become incontinent without a dented line and anorectal malformations, especially the low, they don't have a dented line and yet they're not incontinent. Oh, that's a great question. By the way, I just, I don't think you're necessarily incontinent just because you don't have a dentate line. I think you can remain continent provided your sphincters are good. The one thing you're missing. And this is where ARM and Hirschsprung's without a dentate line are the same. What you're missing is the sensation, the diff the difficulty in discerning the difference between solid liquid and gas. ARM patients cannot do that. They cannot do that. Therefore, it's a mistake to give them stool softeners. You want to give them laxatives that provoke a bowel movement, you want formed stool, because if they do not have formed stool in ARM, they just have soiling. Similarly, a Hirschrung's patient with a lost dentate line will also not have the exquisite sensation of knowing solid liquid or gas. However, if the sphincters are good, in either case, ARM or Hirschprung's, and they can feel distention of the distal rectum, or the neorrectum and Hirschprung's, they can squeeze their external sphincter in time to hold in the stool. The ARM patients have a much harder time with that, because they don't have an internal sphincter. The Hirschrung's patients do. In fact, they have an internal sphincter that is working too well sometimes. So it's a fascinating difference, but the basic principles of continence are, you need sensation, you need to be able to feel the stretch of the rectum or detect what's happening at the anal canal. You need sphincters to close the anal canal when you know you need to hold in the stool, and then you need reliable, consistent motility. And on that, I think is a good way to end. Thank you very much, Mark. Thank you very much, Julia. Luis, do you want to say something more to the audience? Yes, please. Of course, Mark and Julia, thank you for this wonderful presentation and for you at home, thank you all for joining us tonight. The webinar has been recorded and will be available on both our go to webinar platform and the YouTube channel, and I will send the links for you, uh, tomorrow. All links for preview sessions are on our website, as are the details of the forthcoming webinars. And finally, um, all of you who have attended today will also receive a survey immediately after the session finished, and we would be very grateful if you could spend a few minutes filling it in to help us continuously improve further sessions. So for now, I would like to thank you all and wish you a very good night. Great, nice to be with you. Evo, Julia? Thank you. Thank you. Bye. Great talks.