Move to the case discussion and first I would like to ask our panelists, uh, what's, what type of operation do you do for Hisshprung in your country? So do you do Duhamel? Do you do Suavi? Do you do Swenson? Do you do rebine or any other type? What's your preference? So if I can start with Doctor Alp Numoglu. Um, yeah, obviously it will depend on the level of the disease, but if I can start with the total clinic, uh, for total clinics, we would do the Duhamel, um, initial uh. But for a shorter segment, Hirs Spring's disease, uh, I prefer to use the laparoscopy to do the biopsies and establish the level and then with the laparoscopic assistance dissection of the pelvis as low down as possible and then do a perirectal very much like Professor Penny has described the perirectal dissection uh to join the dissection line to the pelvic dissection that we've done. Very good. uh, Stephanie, do you wanna tell us what type of operation you do for Hisprung? Well, we're doing the transanal approach after de la Torre. We've done the last 40 cases like that, and in some cases we use laparoscopy just to to be sure where the level of the ganglinosis is, but most cases we do a transanal approach. In a resection. Perfect. How about Doctor Al's house? Yes, I do things very similarly to what's already been described. If I can't see the transition zone clearly, I like to use laparoscopy to do the biopsies and locate the transition zone for the total colonic Hirshprung's disease. I totally agree with Alberto about delaying the pull through, but I usually do it similar to other total colectomy patients and do. Ioanal anastomosis, but I think the protective ileostomy is important. And then occasionally for short, shorter segment disease, the pure transanal approach is the case, and I've also used that for some redo pull throughs, uh, where I've have an accurate determination of where the ganglion cells are. Perfect. And I understand Ramon is back on the phone. So what are you using in Spain for his strong disease? Well, he's been there since now we're using de la Torre. In the de la Torre and for uh total chronic anglinosis we're using in our service they are using uh Lester Martin procedure very good and I I see that more or less. And I see that Doctor Souza lost image and sound, but she's saying she can chat. So if she can tell us what do they do for uh Hiprung disease in Brazil, she can just chat and we will all be able to read and Jeffrey, uh, I don't think we have an image or a sound of you also you have we have sound. Perfect. So speak up. What do you do? It's interesting because we have 11 partners and about half of us do a suave and the other half do Swenson's. I, I personally do Swensen's, um, and do a laparoscopic, uh, leveling. Uh, some of our partners will do it for umbilical incisions, uh, to do their leveling, uh, depending on what the contrast enema looks like. Um, some partners that do suavez are switching to what we are now calling the Suaven where they do a very short suave cuff and, uh, transition to the Swenson plain, uh, a couple centimeters above the dentate line. Very good, and I know uh Doctor Ernesto Leva is probably in the phone. If he's in the phone, if he can speak up and somebody's moving our slides, so please don't touch the arrows, otherwise people might get confused. Ernesto. Yes, I am. Yeah, I am. So Ernesto, what do you do for his sprung in Italy? What's the operation? Since a couple of years we used the Sohave approach, but we moved to transinal approach in the last 2 years and we are very happy. We managed with the laparoscopic biopsies and then we moved down to the transal approach. Very good so now we're gonna move to the questions and I'm gonna ask the audience first of all just to look at those pictures and just tell me which ones you have personally met we wanna see who is the person that have met more important people in Hiprung disease. So I can tell I have only met one. So you can tell how many you have met and then let's see if you know who is who. So what's the correct sequence? Which one is Duhamel, which one is Suavi, which one is Swenson, which one is Hisshroom, and which one is de la Torre. And you can go, go ahead and put your answers in the chat box if you think you know. And also tell how many of you of them you have personally met, and if there's one that really met Doctor Histrom that would be fantastic. OK. All right, so, the, the answers are we're getting are three. So, Hirschprung, Swenson, Duhamel, Suave, and de la Torre. That's very good. That's the correct answer. Now I want to know who has personally met more than one people. So if you have met more than one, go ahead and tell us. How many have you met? Yeah, I have met 4. Wow, so we have a winner. I think it's going to be very hard to compete. Any of the faculty? OK. I have not. All right. So there is still time to meet some of them. So we're gonna move to the next question. The next question is a patient with total colonic ganglionosis previously operated suffering from fecal incontinence and severe diaper rash. On examination, the anal canal was destroyed. What treatment do you propose? Enemas, rectal irrigation, Botox, constipating diet and fiber or a permanent stoma? So everybody can answer. We're getting, uh, we have fours and fives being answered, a mix of some fours and some fives. The, the, um, The the look at the question carefully and you will see that the anal canal is destroyed, meaning that patient is not going to have bowel control. And in addition is total colonic ganglionosis, in other words, liquid stool, so there is no bowel management. And therefore that patient will have a permanent stomach. When the parents hear that it's, it is possible that they refuse. They hope and they go from clinic to clinic hoping to find a better answer for that. So for. Most patients sometimes we can try to give constipating diet and fiber just to convince the parents that there's no other way except the permanent stomach so that's one of the few indications of a permanent stomach that we follow. OK. And Doctor Ernesto Leva is saying is perineal stoma considered a permanent stoma? We hope that it's not because a perineal stoma means that the patient suffers from severe diaper rash in a case like this with a patient with destroyed anal canal. So by permanent stoma we mean that this patient needs an ileostomy in the abdomen, not down there. So we're gonna move to the next question and the next question is a patient previously operated due to Hischsprung disease suffering from enterocolitis rectal biopsy is normal. What do you propose? and I want the faculty to answer first, so Doctor Alp if you wanna give your opinion, what would you do in a case like that? V irrigation. So, um, in the setting of enterocolitis. Very good, Ramon. Rectal irrigation. Stephanie, well, we do rectal irrigation. We have a comparable concept to what you said. We teach the parents the rectal irrigation fairly early, so, um, we would consider rectal irrigation the best choice. Stephanie, let me ask you a question, and then I also want to pose this to Monica about the sending the parents home with rectal irrigations. Um, I've gone back and forth. Uh, I've sent patient parents home with rectal irrigations, but we've seen several children come back that have not been adequately irrigated. How do you know? Do you, do you ever decide, you know what, you're not ready to go home, you're going to stay here? What's your criteria? So, our policy is before the patient goes to the OR for surgery, the parents have to demonstrate rectal irrigations before even going to surgery, and they demonstrate it to the nursing staff on the floor. Yeah. And they're, they're signed off on doing it. Then the patient proceeds to surgery. So we know before that they are able to do it. Do, do you teach them? Do the bedside nurses teach them? The bedside nurses teach them. If it's a patient that we are worried about and we see them immediately in clinic waiting for a surgical date, we teach them in clinic and they demonstrate in clinic, um, being able to do it as we're waiting for the surgical date. OK, um, and, uh, Stephanie, is that about the same thing for you? Yes, we teach the parents on the ward. We have nurses, and I do personally control it that the parents really know how to do a rectal irrigation before they leave the hospital and before, well, they are going to have surgery. And what are the, what are the more common issues that they have if they're not doing it adequately? What have you found is that they're not putting it in far enough. They're not waiting for stuff to come out. What are the problems? Some of both. Some of them are hesitant to advance the catheter far enough, so we have to. Know that they're confident in passing the catheter far enough and then using enough saline that they're getting clear return before they're finished with the irrigation. OK. All right. I think I hear someone's, uh, camera or, uh, microphone's got a lot of background noise. If you could, uh. Either shut off your speakers before we go on, does anyone else have a comment or question to make of the faculty or panelists about that what we were just talking about? I just saw one question asking about what is the contraindication for irrigation, and the only contraindication is a recent operation, not a contraindication. Medication you just have to do it very carefully biopsy. So usually a surgeon should the surgeon that operated should be the one to do an irrigation immediately post-op because we don't want to perforate the anastomosis. A biopsy or a recent biopsy. Skype. Yeah, I see it. Uh, there's a question, uh, go ahead. Uh, it's healthier. Um, before we come to the rectal irrigation teaching the parents, one of the things that at least in this part of the world that we emphasize a lot is this is not a simple gastroenteritis that the child is having in the background of Hirschberg's disease and enterocolitis because very often we find that children are taken to other medical centers and treated as simple gastroenteritis by doctors who are not really aware of. Um, perhaps the enterocolitis associated, But that's that's something that we continuously teach our parents that they should come back to our hospital. It's a great point. It's a great point. Thank you for that. Did you want to address? Could you repeat the question? We could, uh, we couldn't hear you perfectly well. He was just, he was explaining that sometimes they'll go to another hospital afterwards who doesn't, who isn't very familiar with this, and they encourage them to make sure they come back to his hospital. I think that's the, the disadvantage with our patients being all over. So that's why we make sure that the family is is very comfortable irrigating before going to the emergency room. Um, there is a lot of places that are uncomfortable with irrigations in general, so we want to make sure that the family is confident doing it. Yeah, I, I, I want to mention you are absolutely right. The, uh, in addition, most pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis. They don't, they don't really know the entity called enterocolitis postsrus. Enterocolitis doesn't mean very much for them. They confuse the terms and they don't understand why do we have to do irrigations and the rationality about this. So when that's that the parents complain because they arrived to a hospital, a general hospital, and they don't, they don't understand what's what the childhood problems is. They don't understand the pro they, they take an X-ray film, they see dilated bowel, they think it's intestinal obstruction. They don't know the, the, the, the entity itself so that's, that's why it's important to train the mates about this. It's a, it's a, uh, we actually, we have, uh, and I, before we move on, I wanna encourage the audience to do exactly what they're doing. We have a lot of questions coming in from the audience and I wanna try to address as many as we can and mix in some of these, these questions that you have as well. Uh, there was a question from Christine White and, and, and all of her friends in Albany, New York. Thank you for joining with a large group. Um, it says, hello, you mentioned two centimeters above the dentate line. This seems like a lot in a newborn. Don't you see problems with enterocolitis and, uh, uh, distention? And then when you rebiopsy, it looks like retained a ganglionosis. Question about that. Yeah, the, the, uh, that's a very good question. Thank you for the opportunity to comment on that question. When you go 2 centimeters above detecting a line, you start the dissection and you have to pull bowel through that. When you finish the operation and you start doing your anastomosis. You see that actually it's no longer 2 centimeters usually the upper part of the mucosa was damaged and actually you end up doing the anastomosis about 1 centimeter about detecting a line. Now, a lot of surgeons believe that the patients have enterocolitis because you left 1 centimeter or 2 centimeters of, of, of, uh, of, of mucosa of the rectum. I simply don't believe that it's that simple. Let me tell you a little bit about an experience that I had in New in New York. In New York there was a a very group of pediatric surgeons that were doing neonatal swabing primary procedure. And when I asked them, do do you see enterocolitis in your patients, they said 0, no enterocolitis in our patients. I was very intrigued by that. That's really 0 enterocolitis in our hospital we recognize at least. 30% chance of enterocolitis. Then when I had the opportunity to follow some of those patients operated by those surgeons, many of them have fecal incontinence. So let me tell you, if you produce fecal incontinence in the patient, the enterocolitis is zero. In other words, um, a patient that you destroyed the anal canal is equivalent to a stoma. So patients with stomach rarely have enterocolitis. So now the question is, what if, if you do a, a good operation and preserve the sphincter and the anal canal, by definition, you, the sphincter close is creating some degree of stasis, and stasis produces enterocolitis. So the question is what do you want? Uh, enterocolitis or fecal incontinence. So, uh, I, I hope I made myself clear about, about this idea. We don't, we don't know why the patients have enterocolitis, but if you produce, if you have very little enterocolitis, chances are that you are damaging the anal canal, and I prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life. And about the question of re-biopsying these patients and then finding finding a uh a ganglionic segment when we have to re-biopsy in case that the patient is presenting symptoms that we suspect that it's a transition zone. Or is the the surgeon pulls through in a ganglionic segment. What we do is we biopsy as high as we can possibly be to be sure that we are biopsying a segment above the anastomosis so we don't have this problem. And there was another question, uh, the use of colonic manometry in these patients, any of our panelists want to answer this? Do you use manometry to evaluate these patients either preoperative or postoperatively, Michael. Yes, one of the problems that I see is that our GI doctors have recently started doing a lot more anorectal manometry, and it's like when you're a hammer, everything looks like a nail approach. So they're doing a lot of these on babies and kids with Hirstprung disease referred to them, and I think it's rare even for a post-op patient to have a normal anorectal manometry. So we're getting a lot of misinformation. And I also have seen a spate of people recently who've come to me from their GI doctors with with Hirschprung's disease whose parents have been told that they have chronic bacterial overgrowth syndrome, and they're started on a variety of antibiotics when I think what they really have is a type of of enterocolitis and the antibiotics alone is not the total solution, so. Uh, one of the things to back up a little bit that I also felt was important was to, to stress how crucial it is to the parents because You know, a lot of young parents don't want to hurt their babies, and they feel the irrigations as a, as a kind of a chore and something that's tough. And so it's what Monica said is so true. You really have to instruct them, stress the importance, and teach them excellent technique. There was another question about the Flagyl, the metronidazole. We try to give it oral because it has better effect, and at the end when we are tapering, sometimes we give with the with the irrigation through the rectum. There was also a question about if using regular water is appropriate, and we always use saline and importantly, to, to warm the saline, especially on like your neonatal babies to keep the body temperature normal. Um-hum. Um, question from McMaster, uh, uh, for Doctor Pena. It says, what is the incidence of enterocolitis in your pull through patients? About 30%. OK. About 30%. 30%. Does, I, I, I'm just curious from the audience, have you, uh, Doctor Sosa, I see that you're back on the phone line. Can you hear us? I see that you're back on the phone line. Can you hear us? Uh, so, uh, someone's got their speakers on. If you could shut off your speakers. Doctor Sosa, can you hear us? Yeah, I can hear you. What is your, what do you think is your incidence of enterocolitis for? We have, we have a low incidence. We use the Jhamal technique and actually we have a low incidence of, of enterocolitis in our patients, but I have no explanation to this. All right. Again, if I could ask the, uh, faculty, one of the faculty members must have their computer speakers on. We're hearing an echo. If you could shut off your computer speakers and just listen to the phone. Uh, we also have a question about when is, when is irrigation contraindicated? Um so, that we answered. OK. Oh. He was not paying attention. I wasn't paying. Well, let me ask you a question. After you do a biopsy, Uh, do you have to wait a certain period of time before, so usually we wait 48 hours after a biopsy to start rectal irrigation. So usually we have our patients, our planning of when they come is very organized. So what we do, we have them in clinic first, we teach irrigation, and then after that we go and do the biopsy. So they learn how to do it and it's not a fresh. Uh, incision or a biopsy, right? And I just wondered, do you think really 48 hours it's going to close by then? I I have no idea. OK. I mean, uh, I mean, a week, I get, cause it might be closed, but 48 hours is still going to be open. So, my guess is either you do it right away or you wait several, I mean, I just, I haven't, have you heard of, have you actually, have you seen someone perforate from a, a, an irrigation after a rectal biopsy? No. After a biopsy, no. We have never seen it. OK. I have had a little blood tinge, but not perforation. I'm sure. OK. Um, So, all right, did you wanna go to, we have a couple more questions, but did you wanna use this time to go into the next case? I saw you had a question so we can just ask the audience, uh, in which operations fecal incontinence is more frequently seen, option one, Swenson and Soavi, or option two, Dujamel and rebine just vote. And most people are saying option one, option one. OK, and the answer is correct is option one. So now we're gonna move to the next question. And it's not moving? No. Is that the last slide? That's the last slide. OK. OK, good. Did you want to address these other two questions here? Um, Oh, there was one question about uh prediction from the stooling pattern postoperative in patients with anorectal malformation, and the answer if we the question was can we predict from the stool pattern of the child postoperatively if this child is gonna be fecally incontinent or not usually yes and Doctor Pena always says that what we are looking. For from the operation until 3 years of age, that's the age when most kids are gonna be potty trained for urine and stool, we want to establish regularity, so we want if the child starts to having the bowel movements around the same time and 2 times a day or 3 times a day, that's a good indication that this patient will most likely potty train for stool. Yeah, yeah, I, I want to say the remember that I was, I was talking about the big, uh, physiopathological change that we introduce in a human being when we resect the rectosigmoid. So that makes the patients, uh, to have some difficulty becoming toilet trained if, um, children with a, with a perfect put through preserving the anal canal sometimes have problems toilet training. We don't have an explanation for those and um if the child in addition is hyperactive, has deficit uh attention, the disorder, those patients are going to have more problem in toilet training because they, they have a, remember they have a piece of colon. That doesn't act like a reservoir connected to the rectum to to the rectum and is moving constantly, so it takes a significant amount of cooperation from the child to be toilet trained. So even if you do a perfect operations, patients may have certain problems in toilet training. But if you destroy the, the anal canal, then for sure they are going to be totally incontinent. Some patients come with the anal canal partially destroyed. And they have more problems, of course. I think Dr. Alshaus wants to say something. Yeah, I think that point Alberto made is extremely important because if you study the manometry of the colon, those migrating complexes or the high amplitude contractions stop in the sigmoid colon in most people. They don't go to the rectum, and now we've moved that down to the anus and When you're toilet training these kids, they don't always get the same type of, uh, warning of an impending bowel movement. They don't have as much time and you have to factor that in the decision when you're trying to teach them that to make sure you take, um, use of things like their gastrocolic reflex. Oh, and by the way, we don't use, uh, I personally don't find useful rectal manometry in his bone disease. We don't find useful in anything, and that's my personal experience after many years of, of having rectal manometries. I don't see. I don't feel the need of a rectal manometry to make this clinical decisions in patients with constipation or anorectal malformations or disease. OK. And if anyone disagrees, speak up. Um, I, I'm so excited here because Doctor Osorio wrote Tiene Experiencia con rifaximina, and I just clicked enable translation and that says, uh, do you have any experience with rifaximin? Well, what is that I don't know actually, rifaximin. We don't, I, I have never heard of it, so we don't have experience with that. Any of the faculty, has anyone heard of that? Uh, Doctor Osorio, maybe tell us what that is, but, uh, maybe it has a different name, and I don't think anyone here has heard of that. OK. Um, there's a question here from, uh, Doctor Leal. He says if you have a preserved anal canal and the patient has constant events of enterocolitis, after how many, how much time would you consider a different approach to evaluate maybe an ileostomy? Yeah, when the patients, we have patients that. As I said, we give the, we, we discharge them with three irrigations per day and, and Flagyl, metronidazole. And then every month we decrease the amount, but sometimes you decrease the number of irrigations and the patient again is, Eocolitis and every time you try to decrease the patient doesn't tolerate the lack of irrigation. So by the time that the baby's 6 months post-op and still on irrigation, the parents are getting very nervous and uh at that point we start talking about other options and other options from my point of view is another resection of colon, normal ganglionic colon, but to remove more. Because there is some the see we, we learn about Hirp disease and we believe that everything is about ganglion cells and no ganglion cells. The evidence shows that Hippo disease is something much more than ganglion cells and no ganglion cells. We don't know why some patients, first of all with Hirp disease, we operate on them. They never had an episode of enterocolitis. They become totally trained very early, and they behave like normal children. And there are other patients that have severe enterocolitis from day one, and they, they are real, a problem. You, you have seen patients that we that we, that they have what we call benign Hip disease. These are patients that come to you, to your clinic when they are 8 years old, 10 years old with Higbu disease with a spectacular dramatic image of the classic image of Hip disease with the abdomen distended but never had an episode of enterocolitis and they are grow they've grown and developed normal and you operate on them and. They do very well, whereas in at least in the United States we're making more earlier and earlier diagnosis of patients that have what we call bad Hiprune's. They have enterocolitis from day one, very sick, and you operate on them and they have high incidence of enterocolitis. So there is a lot of things that we don't know about Higp disease. Some bowel has no the story is much more than absent ganglion cells and, and taking. Bowel with normal ganglion cells down doesn't mean that that bowel is 100% normal. Some people believe that perhaps that bowel has neuronal intestinal dysplasia. We like that diagnosis because we thought was the explanation, but actually neuronintestinal dysplasia is a very, very controversial histopathological diagnosis. So we don't know what's wrong. We have to, we have to learn much more about these conditions. So we have just a few minutes left and there's a lot of questions and so I'm, I'll try to pick a couple of them, but we will try to answer these in the chat if we can't get to everything. What's interesting about doing these is you have people from all over the world who happen to have different uh knowledge about different things. A lot of people have been writing about this, this drug that we were just, that was asked about. A lot of people seem to know about it. It's an absorbable antibiotic. Uh, and I, I guess if someone could call in who's had use with this and explain to us how you would use it and why you would use it. And does it have higher efficacy? It just seems like most of us don't really, uh, know about that. So maybe in this country, it's not a common thing used. Um, there was a question about, um, if you receive an operated patient with a wave technique, a normal biopsy. But, uh, but, uh, normal biopsy now, but a long muscular cuff, which technique do you prefer in those patients? First, I want to know if the patient has any symptoms or not. If the patient has no symptoms and doing, is doing fine and you just see a, a long cuff, I wouldn't do anything. I would just let the patient grow and live the way. Sometimes some patients might present with obstructive symptoms and especially in the contrast enema you'll find the image of a narrow bowel and then a very dilated bowel, meaning that there is an obstructive cuff in those cases we would do a Swenson type resecting the cuff full thickness and removal of the cuff. Any, uh, any comments from the faculty who disagrees with what we've been saying or want to make a comment? Yeah, what about, uh, trying to, uh, split the cuff going in from the laparotomy or laparoscopy and, uh, without actually resecting, just trying to split the cuff as should have been, uh, happened at the. Uh, Uh, yeah, I guess, Al, for some reason when you talk at the very end, it's sort of the, the sound fades away, so we didn't quite catch the end of what you said. I will shout a little bit more. Um. Another option is to do a laparotomy or laparoscopy and try to split the cuff, uh, in front of the sacrum, uh, in that setting without, uh, doing a resection. Just would like to, uh, hear the views of around that laparoscopy laparotomy to split the cough from inside. Uh, uh, the, the, I'm a little skeptical about the idea of the cough producing an obstruction. I'm, um, I think there's a possibility that that happens, but for me to believe that, I would like to see the cough producing a real obstruction manifested by a very dilated colon above the cuff, and, uh, and, and it's very unusual to see something like that. The cough is there, but it's not, we like to blame the cough, but, uh, we have to, we must have evidence that it is really producing, uh, obstruction. So, but how, how to deal with that in the hospital, Doctor Mark Levitt, my partner is, uh, has experience with that, and he does that transanally. I, I never heard doing it laparoscopically, but it's not a bad idea. It's interesting. Yeah. Yeah. Uh, what about Botox? Um, So Botox, we don't use Botox first of all, Botox is a temporary effect and what it produces some incontinence for a period of time so we don't think that that's the treatment for enterocolitis for example because it's temporary. Have to come to a solution that it's final and we don't think that, uh, Botox is a final solution, so we do not recommend. What about the fact, uh, go ahead. Remember, remember, patients with total fecal incontinence have no enterocolitis. So all those operations, myectomies, myotomies, Botox, and massive dilatation, some surgeons put 3 fingers in the rectum. Everything actually are procedures that are moving toward fecal incontinence temporary or permanently. And we, in fact, we have seen patients subjected to myotomies and myectomies or repeated Botox injections of Botox that eventually developed more degree, more severe fecal incontinence, so. I, I don't believe in those procedures. We don't use Botox. And we are, we have to remember that we are dealing with chronic diseases. The same thing with constipation. Sometimes they offer Botox for constipation, but it's a chronic disease. So I think it's very simplistic to think that you're going to do Botox and that the patient will learn or will overcome the problem and then suddenly starts defecating normally. So we don't recommend Botox. So, um, we, uh, we have a couple of other questions that we'll try to answer in the.
