Hirschsprung Disease: Cases and Complications

So we're going to talk about complications. It turns out just for your information that personally I have more cases of re-operations than primary procedures, and the reason being that most pediatric surgeons believe that they can operate on his bone disease and therefore, I received many referrals for cloacas or complex malformations, but usually not for primary his bone because all pediatricsurgeons love to do primary his bones. So I have much more experience in re-operation than primaries or simply in patients that come because they suffer from complications. I don't re-operate on them. You are looking at a picture now of a patient that had a pull through for Hirshman disease. Suffers from fecal incontinence and we look into the part of our protocol of studying these patients is through an examination under anesthesia. As you can see here, there is no pectinate line. In other words, the previous surgeons anastomos bowel bowel mucosa directly to the skin. So I, based on my experience, I classify the complications and sequela position into three categories. The first one is what I call the non-preventable camp sequela that is called that is everybody knows about that very unfortunate and mysterious problem called enterocolitis. We look forward for the, as I said before, the new generation of pediatric surgeons to go into serious scientific research to find out what's the problem with that, to explain this and to treat this adequately. The second group of complications of sequelas that I consider partially preventable is constipation. We know that if we leave a very dilated piece of bowel, even being normal ganglionic, the patients most likely will suffer from constipation because As Dr. Luis de la Torre suggested, the very dilated colon suffers for hypomotility, poor motility, and therefore the patients suffer from constipation. So the rule is try to resect not only the ganglionic segment, but as much as possible the dilated portion of the colon. And then comes the group that we call that I call preventable complications that are really very sad, very sad because those are, I believe, 100% preventable, and yet we see them all the time, the hissing and stenosis when the surgeon performs a pull through and the bowel separates and the patient is a catastrophic complication. The strictures. That comes from either ischemia or anastomosis performed under tension or both of them. And then something very sad also perianal and neurogenital fistula. The patients get a pull through for his disease and they develop a fistula into the urinary tract. They pass stool through the urethra. We'll stop now. I will stop and will continue because now I think I, I understand that Risto Rintala is ready and the sound is better. Yep, do you hear me now? Oh, 1000 times better. OK, so now. We are all Jews. Yes, it's perfect. OK, good, because there's something wrong with the other phone. I, this is the 3rd phone out. I, I've, I've, I've tried but hope, hopefully this works. So I was telling you why these studies can be made in Finland. It's long term follow up studies, and the main reason is that we have a social security number. for all Finns. And we can track all our patients back. We know their whereabouts and we also know their sort of medical history from the national records. Let's go on with the talk. We did a study on adults uh with H disease um uh uh we've studied the bowel function and we assessed also the gastrointestinal quality of life. Um, uh. The basis for this study was that actually there are no or very few controlled studies in adults who have been operated on for H disease in their childhood. And There are some full of studies there are many follow up studies but no studies that have that have used control populations of healthy healthy individuals. This was a population-based cross-section study and operated. In our institutions in 1960 and 1986 of the patients have died and had migrated left us 143 patients who were eligible for the study. most of the patients had clinical interview and we used 86 match controls without any previous surgery for our patients. The operations that were performed were mainly Duhamel operations. The story about the life that the figures have changed the other operation used with the founder and also the the operation the operation in about 1% of our patients and 25% of the patients had a OK. But what happened to the flight? Sorry about that. Hold on one second. OK. Um we compared the patients also the patients with the responders who took part in the study with the with the nonresponders and in this study we didn't find any, any difference between the between the responders and no in terms of surgery, the surgery. And the and the and the and the and. We used the function of valid score for the function in health the average score is 1.1. The low score means that that function and the high score means a very good bow function. We also a quality of life score score that is 36 valid score for health related quality of life. This it records Gy physical and social functioning and also emotion station that states of all the all the the patients the average score. In healthy adults it's 125.8. The maximum score is 144. Uh, you, you can see from this slide, uh, slide that, that, uh, that, uh. There were problems in the, in the, in the, uh, bowel function when compared with the, with the, with the controls. Uh, the The Uh, constipation was much more frequent with the, uh, uh, patients, uh, so it was much more frequent. It was, was much more frequent and also the problems were much more frequent in the patients than in the controls. The mean malfunction score of the adult patients with 17.1. And it was 19.1 and this this difference was significant. Uh, however, uh, Uh 25% of all the patients scored the full 20 points uh as opposed to 50% of the con con con to controls. 13% of the patients report frequent soiling. 2% had fatal accidents and 10% had complication that requires treatment. And this figure shows how the how the It's disappeared again, but. Uh, control it? Can we just control it and the size. Right So, I'll be moving your slides. You just tell me when you, when you want the next slide. I'll be moving them, OK, OK. Oh sorry, thanks. So I don't touch that. OK, that's OK. So uh from this uh figure you can see that um. That definitely the bowel functions of the patients with with the with the bowel function is not at the same level than in health in individuals next. And the the gas of life on the other hand, is mostly at the same level than in the in the health population. There are some, some individuals that had the worst outcome in terms of life, but the people who did worse occurred also in the healthy population. Next. Um In the patients, the GP scores, the scores were marginally lower in the patient, but this difference was not statistically significant. However, 22% of the patients had a score lower than 110 that indicates poor get different quality of life. Uh, the patient scored a worse, um, in 11 on the, on the, the 36 items and, and these items are listed on the, on the, on the, on the, on the table left. Um Interestingly, the patients scored better. In the ability to participate in these activities and and and also in the quality of sleep and control. This is very difficult to explain but this kind of questionnaire type studies can give results like this. Next slide please. Uh By doing a sort of multivariate logistic regression analysis, we assess the predictors of poor outcomes, which means the function score lower than 17. And the poor quality of life, which means she score lower than 110. And this where age surgery after the surgery. length and primary diversion and in the in the in the quality of life issues that predicts what age, gender, the body mass index, and bowel function score and what we found out that the increasing age was the only significant predictor of poor functional outcome. And we also found that age was inversely related to the bowel score in the patients but not in controls, and this is sort of a worrying finding, which means that we may expect that our patients that might do quite well when they're young adults might not do so well when they age. And in terms of quality of life, the low bowel function score was the only predictor of poor quality of life. Next slide is. We conclude that the bowel functions continues to be impaired in a significant proportion of adults with operate. That abnormalities in the urge to defecate, fecal soiling, constipation, and social problems in relation to bowel function affect about 1/3 to 15% of the patients. Increasing age increases the risk of poor functional outcome. However, the overall gas in quality of life appears to be comparable its controls, uh, but decreased, um, uh, Gaston's quality of life was found in 1/5 of the patients. And then I continue with the, with the next slide. With some of our more recent studies concerning the outcomes of long segment gangliosisgnosis affecting the entire colon with or without small. In this time, next slide please. We looked at all the concept of patients who were born between 1984 and 2013 and treated. In our institution and these patients suffered from total colonic anosis with a very extension to the small bowel. This is sort of retrospective case note review. And the patient characteristic surgical treatment bowel function dependence on nutrition growth and survival. Next please In total we identified 25 patients, 21 patients, 15 of these patients were male and 6 female. of the patient for me to see this. And syndrome were found in 4 patients. 2 had causes hair ablation, typical Finnish syndrome which occurs also in the Amish population in the US 21 patient had a, had a central uh hyperventilation syndrome, had that syndrome with a neuroblastoma, and one patient had a Sch-Wardenberg syndrome. The median follow-up of these patients were 6.5 years. This gives you gives you an idea what so how how the patient were doing by the level of 5 patients had the gnosis extending very near. To do it, do it in general uh uh uh uh lecture. So they, they have basically sort of total bow egg in their nose. all the patients for us are still alive. Only one has been weaned from parent. The and and and all of these patients have a bow continue one of the patients and underwent successful small bowelation you know and the other patient is waiting for for small about transportation. There were 4 patients who were where the agnosis extended to the mid small bowel level. Uh, I think the phone his phone went out again. Um, Mark, we bring the microphone back up. I, why don't you try to call back in. I think the phone looks like the phone crashed again. So while we are getting Doctor Rintala back, I just, I don't know how many people were able to hear why Finland has such good long-term outcomes, but they have a social security number that is national and they can track medical history, so even if your patient have an operation in another hospital, you can still look at that, because when we try to do our long term follow up, we always Have the risk of the ones responding. Many people would say, are the ones doing well or are the ones not doing well. So we have a kind of a biased view. And in this case they can track all patients from life until death. They can have access to the entire medical history, and I think this is fantastic for learning and to learn what truly happens with a disease as it progresses over years. So hello, hello, yeah. We can hear you. Do you hear me? Yes, perfect. You can go ahead, Dr. Intala. OK, yeah, yeah. Andrea, can you, can you check the previous slide, OK. Yeah, OK, And uh so I was left, I was left to the, to the, uh, in patients who had a sis extending to the mid small bowel, and these patients had a genuine anal pull through um uh uh. But Two of these patients had had a disease that makes small transplantation contraindicated two of these patients died because of of of severe bow motility and the recurrent septic septic infections. None of these patients have been weaned on on nutrition and only one of these patients have a continuity. There were several patients with the was was less than 50% of. 6 of these patients had pouch and 1 had a left mark type operation. All these patients, patients are alive. None of these patients are on the end and all and and all patients have bowel continuity. They were. 5 patients with the purely colony to the call and the notices this patient and they all alive that we and and and they all still have about continue next like. Um This shows the the the the duration of of the of the uh you can see from this slide that it expenses to the memi small bowel or more approximately very few of the patients can can can be we from from not recent and also this affects. The survival, the longer the egg and the segment, the poorer survival like this. And these are the functional outcomes. In patients who had a pouch anal anastomosis, all of the patients have bowel movement. Motions of the 9 which have been toilet trained. The stooling frequency for 24 hours is 4, ranging between 1 and 210. 4 of the 10 patients have uh bowel motions at nighttime. 2 of them have uh have, have, have fecal something, uh, to some degree. None of the patients is suffering from constipation. Um, most of the patients had, had at least one episode of enteritis, um, in these patients, um, uh, the episodes were treated by oral, uh, and, and antibiotics, mainly, uh, uh, metronidazole or cifloxacillin. Obstructed episodes were common also and these were mainly treated by by by the Botox injections some of the patients required more than 2 injections and maximum 6. To conclude, Intended and nois beyond 50 centimeters of of of this island, the achievement of functional continuity or autonomy is exceptional and long-term survival poor without interstellar transportation. If the egg gangnosis is, this is outlook of bowel function is reassuring after a pouch anal anastomosis, although obstructive symptoms and enteritis are frequent but manageable with Botox and metronidazole. We need to continue to follow up these patients to assess the long-term outcomes, outcomes after after uh other part. Moses, thank you very much. Thank you so much, Dr. Rintala. If anybody has any questions to Dr. Rintala, that's the time to type in the chat so he can answer your questions. And if we don't see any question, then we are gonna move on to the complications both through through. But I know some people are typing, so we'll keep looking. And if there are questions, we'll call you again, Doctor Rintala. Sounds good, sounds good. There's a question about genetic testing, rat testing, um, in these patients. I don't know if anyone wants to take that. So in Finland, do you currently test those patients for rat mutation, Doctor Rintala? Yes, we do. OK. So, so I have a comment about what you were pointing out which which we found so fascinating when you were calling back in about the system in Finland. We can't do that in the United States. We have HIPAA. We have problems with maintaining continuity of medical records between hospitals, but what I would actually. task your hospital with is starting a database that anyone around the country or the world that does one of these cases is entered into this database. They're doing this now in the adult world in, for example, hernia repairs, and it's becoming so robust and so helpful that it's changing the way management is happening and I think this is probably the place that should. Initiate such a database, then we're going to get better information. Absolutely we currently are organizing a database for complex malformations. We're not doing yet for the straightforward ones, but through the Hendri Project. We are starting a bowel management for all the complex malformations and we will include complex history and everything so if you have a case, you can see how the patient did. In the future you can see how the surgery was. You can talk to the surgeon that did that operation. No Yes, we have been insisting on doing a registry of cases of anorectal malformations. We already have the database in place because we brought that from New York and we have one person dedicated to our database that has by now over 2500 cases operated and Almost 5000 cases including the non-operative patients. So but we can receive cases from everybody and put them according to the diagnosis and then everybody will have the opportunity to have access to our database and ask as many questions as they want. And Risto, do you, did you find a difference in the quality of life of patients that were operated in the 50s compared to patients that were operated, let's say in the 70s and 80s? In other words, is there some effect of better surgical techniques? Did the techniques improve or the results improve? Could you tell us something about that? Yeah. Well, uh, like I said, the, the, the, the, the, the, the, the main predictor of poor outcome was, uh, was the age. So I think that the, uh, the, the, the type of surgery, uh, plays a role, but we have, we have just finished a, a, a, a, a study in Finland and also sort of Scandinavian study where we have assessed the, the, the, the patients who have um anal movectomy with or without laparoscopic help in and, and this the number of these patients in the study is more than 250, and these are patients from uh from 2000 to 2009 and in and also in these patients, there is a significant amount of uh of, of bowel dysfunction. Let's say about 40% of the patients um uh. Have a so-called normal bowel function at the medium age of like 6 or 7 years. So I don't think that the surgical technique plays a role and probably the patients operated on the 50s may have had an initially worse outcome than they would have now, but still. Even now there are many patients with heart disease who suffer from bowel dysfunction despite optimal surgical techniques. So just regarding the rat mutation, I think uh Doctor Kapoor mentioned the Hisprone collaborative, and I think my dream would be that we would be able to test in all patients so we find the true real incidence and determine that if we should or should not test in every patient. I think currently we don't have the answer for that. So I know there are many things going on in the chat. Do you want to, is there anything we should address because I don't have full access to the chat right now. I, I think that for the sake of time we should probably go ahead because we're behind unless you want to hit some of them. I mean, it's, we're good. So Dr. Pena will continue with the complications in sequela. I think you mentioned this slide, and we're going to start with this one. Yes. Thank you, Dr. Rintala. You're welcome to keep participating as we go through. I'm sorry, I apologize, Dr. Pena, and if you can stick on the phone, Dr. Rintala, because this question has been asked a few times now, so I guess we should hit this. The rest we can chat with the question of do ganglion cells disappear after a pull through. I mean this question is wonderful. I always a wonderful question because you want to comment on that. The truth is, um, we don't re-biopsy our patients that are doing well because we have no indication to re-biopsy them, so the only true way to answer that is that we have a patient that you did a pull through perfect pull through. Pathology confirmed and the patient is doing well and you re-biopsy this patient, that would be the way to answer what happens to ganglion cells in this distal pull through over time to see if it's actually is it truly a transition zone and it was a mistake at the time of the pull through devascularization of the bowel or if it over time it happens, I think currently nobody knows this answer. Personally, I don't remember a patient. That I operated, came back with symptoms. I rebiopsied and found no ganglion cells after one of my put-throughs. I have found cases like that, but I don't know if it was because the ganglion cells disappeared after the put through or it was an error in the histopathological diagnosis. I don't have the answer. I'm very curious about that. I think there have been some laboratory studies about trying to reproduce that. Can we provoke a ganglionnosis in an experimental animal? That would be the question. I don't know what the other participants have to say. What about Luis de la Torre? What about Roddy Riso? If any of the virtual faculty want to chime in, uh, let us know. Um, while we're waiting for that, I, I just wanna clarify, make sure I understood, you have, you have not had a patient. Get a ganglionosis at your anastomosis. A patient that I operated operated on that comes back, his symptoms. I re-biopsy and now has a ganglionosis in the pull through area and before during the operation it was normal ganglionic. I don't have happened. So here's my conclusion. I think you've done probably 1 or 2 more than me. Maybe that's just 1. And in my, and so I, I've had it happen and, and so what does that mean? The person who's done so many has never had it happen and I've had it happen and I've done a fraction of what you've done. But in your case, did you check back the pathology and they confirmed that was truly good bowel that you? That's the question. And so the The the pathologist looked back at the slides. Now I have not because I no longer work at that hospital, but that pathologist I called said they looked back and they say that it looks good. And so, um, the question is what happened and why is it you? Is it technical? Did I not, you know, did I not go far back enough? Is it a pathologic problem or is it a patient? Is it a patient? You can't be a patient. I think we just ruled that out. By definition, if you've never had it happen, it's not a patient issue. It's got to be technical or pathology. Is that a, is that a too bold of a statement to make? I, I don't know. I, it's a, it's a, I have that question in my mind for many years, and I don't have an answer. Someone just said something. Go ahead, yeah, yeah, yeah. Raj from pathology standpoint, you know, I, I've read as you have, there are papers out there that um. Face value would say, Described the phenomenon of acquired a gangliosis where they claim that in the original pathology, even re-reviewed, there were ganglion cells all the way to the proximal margin, and that the patient with persistent symptoms when rebiopsied or even with a second pulled through, had a ganglionosis, uh. Proximal to the anastomosis, I think there are a lot of variables when you're dealing with an individual patient or reading these reports you have to be careful of that were just alluded to, and it starts with reading the, the fine print in the pathology report. Many places still have clung to this idea that The way to assess a pull through specimen is to do a longitudinal strip of the length of the specimen and not look at the full circumference of the proximal margin, and as you folks all know, the interface between the ganglionic bowel and the a ganglion bowel can be very irregular and can project for 234 centimeters longer along one part of the circumference than the other, so that even if you just look at a longitudinal strip. You could see gangon cells at the proximal margin, but on the opposite side of that margin there may be no gangon cells, and so you may be dealing with a, a low transition zone pull through in that situation. And then when you go back and that patient continues to have symptoms, you see that a ganglion ball that was incorporated into the pull through. The other thing that's really important is that if you're only going on biopsies of this patient with persistent symptoms that you know where you are relative to the anastomotic line, and I don't, I'm not a surgeon, I don't know how easy it is to visualize that years later, but I worry sometimes that maybe the biopsy that what got read as recurrent gangliosis is actually below the line rather than above it. So before I want to, before I send that question over to Doctor Pena or Bischoff, um, I want to make sure I clarify what you're saying. So that to me is summary is that it's a technical error because that means that the surgeon is not going far enough back from the biopsy site and that they're still in the transition zone. So Jack Langer, Jack Langer gave a symposium a few years back, and he polled the Canadian Association of Pediatric Surgeons and asked them, where do you do your resection relative to your serum muscular or full thickness biopsy, and a significant percentage of them, nearly half, said that they went 4 centimeters or or less, and about 10%. In total said that they did uh right at the site of a positive biopsy. So if that's your practice, you, I think, run a high risk of having pull-throughs that are going to be done in this partially a ganglionic zone. How far back do you guys go for me the last biopsy site? We try to go at least. You still have segments sometimes. What if it's not dilated, not dilated, you do. OK. Did you want to remark about how to tell when you go back and do the rebiopsy? Are you able to tell where exactly that is in relation to the previous anastomosis? Yes, you can see the anastomosis very clearly. Yes, and I have not re-biopsied my patients, but I re-biopsied many patients that come to us with problems, right, right. So I think we can move on to complication now. Yeah, are we good? Yeah, so he's never rebiopsied his patient. I didn't get that part. OK. He's never even needed it. OK, all right, so complications. So we were talking about, um, we're talking about complications, and I was saying that part of our protocol to study patients that come to us with suffering from fecal incontinence includes the examination and the anesthesia, and here you can see the patient that has a fortunately a preserved anal canal, but then you can see what we call patulos anus. Even without looking inside the rectum, just by looking externally, you can see that somehow some in some way this anus was damaged, the sphincter was damaged, and the patient comes with a completely open rectum. Some patients come suffering from rectal prolapse after a pull through. That's a very, very bad sign. Rectal prolapse after a pull-through for, that's not a good sign. Something is wrong. So that and we believe that that's surgical damage and and therefore preventable. In this particular case you can see he has a pattuous anus plus no anal canal, so these patients suffer from the worst fecal incontinence, and you can see there is no pectinous line. The bowel is sutured to the skin. In this particular case, you wouldn't believe it, but this particular case is total colonic ganglionnosis, and the surgeon destroyed the anal canal in total colonic ganglionnosis and in addition closed the ileostomy in a baby before 3 years of age. And look at what happened 2 years after the baby, the baby, the parents, the entire family suffered a lot and developed this terrible granulation tissue. The baby could never sit. He was always standing up. It was really dramatic what happened. And of course that baby needs a, an, an, an ileostomy. So because with no anal canal, this is something very important. With no anal canal, there will be no bowel control, and liquid stool with no bowel control is really tragic. And we pediatric surgeons don't talk much about. The diaper problems, you know, the nurses, the mothers are the ones who struggle with this, and we don't discuss much of this, and we should do it because the quality of life of these patients is seriously affected. So this is what we do when a patient comes suffering from fecal incontinence after an operation for this. Here you see in the center a circle that says damage anal canal because this is the key part of the evaluation. Mark, can you make that slide for the screen so we can also. Yeah. Perhaps you Thank you. So, so here in the center you see the key part of our evaluation which is what we call the examination under anesthesia. First, we, we book under anesthesia, look into that. If the anal canal has been damaged, we already know that most likely that patient will need enemas for life. In other words, that patient will go straight into bowel management for fecal incontinence. On the other hand, If the patient has a preserved anal canal, there's no guarantee that we have bowel control, but certainly we have more chance to have bowel control. And for that to determine what kind of management are we going to give to that patient, we do a contrast enema. If the contrast enema shows dilatation, in other words, the evidence of constipation that we call hypomotility, by the way, some of the gastroenterologists that do coronic manometry, they say, how can you say that it's hypermotility if you don't have a motility study. And as I say, if the patient doesn't poop only every 3 days and has a dilated colon, that's hypermotility with and without colonic manometry process. So that I don't know how to call it that in other words, for that patient, we give laxatives and if the incontinence persists, then we go for bowel management. If the patient is, uh, is continent, that means that actually the patient was suffering from what we call pseudo incontinence, overflow pseudo incontinence due to constipation. That's good news. On the other hand, if the contra cinema in this patient with a preserved anal canal shows a hypermotility type of colon, a patient that has tendency to diarrhea and Tendency to diarrhea, then we try to slow down the colon. We give them loperamide, constipating diet, and bulking agents, in other words, a type of fiber that makes the stool bulky and trying to, and 3 meals per day and no snacks. If the patient regains continence, that's great. If the patient persists incontinence, the patient needs bowel management. Of course you can, you can say. You already know that it's much difficult to deal with this group of patients with hypermotility and incontinence rather than this, more chance to be successful here, but the, the presence or absence of the anal canal is very important to determine the prognosis of the patient. We have to go and tell the parents that the chances are, are not very good. So we're gonna show a few case scenarios again. So this is an 11 year old male patient born and operated due to Hisstrom disease, suffering from severe constipation. So look carefully at this contrast animal. Again, another image, and we're gonna have a ball. So looking at this contrast enema, what operation do you think he had? Swenson, Suvi, Tranzano, Duhamel, or I don't know. I'm gonna show again while you can vote. So here's the contrast enema. And we have now there was a change. Some people were were saying to us what happens is they see what they looked again and they said this is clearly a dujamel and you can see here you can see here the a ganglionic segment and here's the ganglionic segment. So we performed an examination under anesthesia and we found usually when the patient has a dhael the patient has an intact anal canal, which is a great advantage for having this surgery. So normal anal canal and an adequate septum. The communication between the a ganglionic and the ganglionic bowel was wide open. So what would you recommend in this scenario? A redo Duhamel, a redo transanal with removal of the Duhamel pouch, abdominal reduction of the Duhamel pouch, permanent colostomy, bowel management with laxatives, or bowel management with enemas. So everybody can vote, and I wanna see what's your answer. So, so far we have some people saying redo trans anal with removal of the Duhamel pouch. Some people saying abdominal reduction of the Duhamel pouch. Uh, and bowel management with laxatives. So, very interesting. I would not advise that we do do Hamel. We do do Hamel, sometimes we have to do it because it's our only option, but it's not an easy operation. And if you're gonna re-operate on this patient, you cannot guarantee bowel control. So in this case, since we saw that the septum was not a gigantic dilated pouch, the pouch was not a gigantic dilated pouch, we offered bowel management with laxatives. And the patient had voluntary bowel movements and adequately emptying his colon radiologically demonstrated with 35 mg of senna. So this is a good outcome just managing with laxatives. This is extremely common in patients that underwent a Dujanel procedure. I want to say something about that particular case. We go back to the country. But You see, in these patients, we sometimes you give laxatives and the laxatives have a great effect in this colon that goes behind and the patient gets almost diarrhea and the stool comes here, but some of that, some of that stool goes into the pouch and then the patient is doing well, but every 10 or 15 days comes back fecally impacted because it's not emptying out. So they have to be monitored with X-rays to be sure. But the patient is not accumulating stool sometimes they they are not as easy in managing the patient as in a patient with a simple idiopathic constipation because. Even with enemas, sometimes the enemas are given into the pull through bowel and sometimes the enemas given into the pouch. So it's sometimes it's a challenge to manage these patients. So now we're gonna move to another case. This is a male patient with past medical history of chronic constipation. And I think that's Doctor Luis de la Torre's case, so I'll actually ask Doctor Luis de la Torre to present. Can we have the remote access? Do you want? Can he pass his lights? I'll pass the lights for you, Dr. de la Torres. So just tell me next, and I'll keep passing. We cannot hear you. Can you hear me? Yes, now I can hear you. OK. This is a male patient with past history of chronic constipation. Next please. This is the X-ray that was obtained when he was 1 year and 4 months and you can see here uh poop. In the rectum, and part of the left side. Next. This is another X-ray with um 2 years and 7 months. At this time he was on MiraLax 3 times a day uh and he sometimes used uh suppositories. Next. So one day he went to the emergency department because he had an abdominal pain and they give an enema and after the enema he becomes really sick and this is the X-ray after the enema. Next, After looking at these abdominal X-rays, what would you recommend? Uh, disinfection under anesthesia, emergent colostomy. Medical disinfection. Using Golightly through an energy tube. colonic resection. A biopsy to rule out fish burn or do a contrast enema. OK. So More than 50%. Recommend medical disinfection with enemas or with go lightly. So the patient went to That disaction will go lightly through an NG tube, and this is the X-ray after the. Cleaned out. And then the GIs decide to do a contrast enema. And these are the image from the contrast enema. This is one in the lateral view. Next And these are the image in the front view. To your left is free and then your right is post eva. So For you, is this contrast enema suggestive of his problem? What do you think this patient? With this X-ray could have a huge bone disease. 50/50. 44, 38, 45, 40, I don't know. OK. Max, So what would you do to do? A full thick rectal biopsy. You will try with aggressive managers, with laxatives. Uh, keep him in observation or you don't know what to do in the next step. Remember that this is a patient with almost 7 years. OK. Aggressive management with laxatives. OK, the patient was admitted for a rectal biopsy and the biopsy was informed as a ganglionic. So the patient was taken to the OR. And they performed a colostomy. And it's not here in the slide. But they remove, they resect the sigmoid. And 3 months after the colostomy, the patient had a swab pulled through. And The report of pathology, only 8 centimeters were a ganglionic. After the pull through, the patient suffered, suffered from descent. Next slide. So in a patient with Late diagnosis and Pulled through Without Protective colostomy and a descence of the anastomosis, partial or complete descent. What would you do? colostomy. An ileostomy. You try a redo of the pool tube, or you can place a rectal tube, irrigation, clinical management with antibiotics, NPO, total parenteral nutrition. So the majority is saying ileostomy. OK, the patient was uh treated with a rectal tube. He had a Improvement for some days, 3, and then again he start to have Severe peritonitis. So then, They do an ileostomy. You can see the X-ray with the patient. It was taken to the OR for Ansto next. When you have a patient with Uh, long, how long will you wait for attempting a redo pull through in these patients? What do you think? You need to wait 1 month, 3 months, 6 months? You need to wait 1 year. For 2 years. So the majority is saying 6 months, but we have a few voters for 3 months, 1 year, even 2 years. OK, the patient was admitted to a new culture through. 3 months after the ileostomy. And, but are you looking is the Contrast study from the ileostomy after the 2nd pull through. Next slide. So this patient came to To the colorectal center with us suffering from abdominal pain. Fecal and urinary incontinence uh using diapers. Next. So we obtained an X-ray and you can see here. Uh Gas, a lot of gas in the right side. Down from, from, to the rectum. And also you can see. A gas in the right side of the abdomen next. So what would you do? With this patient A new redo pulled through. An examination under anesthesia to determine the integrity of the anal canal and rule out the structures or a rectal biopsy. So we have more than 90% of the audience saying exam under anesthesia to determine the integrity of the anal canal and rule out. Perfect. So that's the, the, that's the best plan for this patient. So we found a damaged anal canal. And the question now is what will you do? I really pulled through. A rectal biopsy. Or public management with enemas followed by a Malone procedure if a successful program with enemas. So we're going to have our phone. Mark, we got covered by the screen. Can you uncover it? Thank you. So we do Rectal biopsy or bowel management with enemas followed by Malone procedure. And we have the majority of the people were saying bowel management with enemas followed by Malone procedure is successful, now 100%. OK, that's perfect. Next. Um So this is another patient. This is a, this is a patient, a newborn patient with 12 hours of life. He presented, uh, bilous vomiting, abdominal distention, normal anus, and explosive bowel movement after rectal stimulation. Next. Uh, what study do you recommend for this patient? An upper GI, an abdominal X-ray, a bar enema, manometry, or rectal biopsy? Everybody can vote and we have many people saying abdominal x-ray, and. Hm? OK, so this is the x-ray for the patient. You can see uh loops of probably colon. Uh, they lighted and no gas in the rectum. Next. So based on the abdominal X-ray, which plan do you recommend for these patients? A Irrigation, barium enema. And rectal biopsy. Irrigations, rectal biopsy, and then barium enema. And upper GI, um, barium enema, and manometry. Barium enema, rectal biopsy, and irrigations, rectal biopsy, barium enema, and irrigation. What is your preference and D. Now this is getting more most of the, yeah, most of the people want to do irrigation first, which is very good then. It's 40% with very minimum. OK, next, uh, um, which one do you use the word barium minimum? Do they use barium in your hospital? No, no, no, we don't use barium contrast enema. OK, so that's, I feel better, yeah, me too. Contrast enema, no. So the patient was uh submitted for a contrast cinema and this is the initial X-ray. This is the first er image from the contrascinema in the lateral view. So you can see here the small rectum. And then you can see. A portion that is becoming dilated. Next. This is the next image from the contrast enema. And you can see here another image next. And this is the front view from the contrast cinema. Then the patient was submitted to biopsies. So The biopsy from the rectum was ganglionic with ancillary methods positive for his disease. Next. So based on the barium enema and rectal biopsy, what do you recommend? laparoscopic pull through. With transoperative biopsies, transanal and rectal culture, transchannel full thickness culture, colostomy and biopsies of the colon. Uh umbilical incision to do biopsies and then a transanal culture. So we have all answers, but number 4, so nobody wants to do a colostomy, but we have answers everywhere. The majority saying trans anal and the rectal. OK, I'm 60% laparoscopic culture. Doctor is saying that's because Doctor de la Torre is talking. Then people are voting to try to be polite with you. OK, very good. So this patient was submitted a laparoscopic in the rectal culture. Of the proximal sigmoid. The biopsies from the distal sigmoid, proximal and uh sigmoid and splenic flexure were normal with ganglion cells. So they did a laparoscopic culture next. This is a The story. The patient, when the patient was in the post-anesthesive care unit in the recovery area. The pathologist reviewed the doughnut, the famous doughnut. Of the resected semoid colon and ganglion cells were present, however, They found suspicion of a transitional zone. The patient was in the recovery area. Next. So imagine this scenario. This is the, this is the real life. You are uh talking with the parents that the patient was very well, the pulse was perfect and Then you come back to the OR and the pathologist inform to you that maybe you move a transitional song. So based on this transoperative report of the doughnut, what do you recommend? 1, you create a colostomy and wait for definitive report. 2, reopen and perform a new laparoscopic pull-through with more trans-operative biopsies. Leave the patient to recover and wait for definitive reports. Perform a rectal myectomy 2 weeks after the pull-through, or you can use Botox if the patient develops constipation. So we have the majority of the people saying leave the patient to recover and wait for definitive report. OK, Next slide. I would do nothing. That's what the majority of people. OK, so the surgeon decided to take the patient again to the OR because they were afraid of the transitional zone. And they do a new transreal indirectal uh pull through. It was described as transanal and ectal proctectomy and indirectal pull through of proximal transverse colon swab type, uh, proximal transverse colo anal anastomosis. Max After this 2nd pull through. Uh, for the following 18 months, the patient suffered from chronic diarrhea with more than 20 bowel movements per day, leakage stools with residual food. Uh, he had 6 months with difficult and erectile dilatations and was admitted to treatment for diarrhea and allergy to different formulas. They also use Imodium and constipated diet. With this history, the patient was was sent to the colorectal center with us. Next, OK, so you have the patient in clinic with this history and the question is what to do. Irrigations, loramide, bioenema, enemas, Imodium, and bioenema. Upper GI contrast enema and manometry, and a rectal examination under anesthesia, rectal biopsy and contrast enema, rectal biopsy and contrast enema and senna. Fair enough. OK, perfect. So everybody Agree. So this is the X-ray that we obtained. The patient came for the first time with us. And you can see here gas now on the right side and no gas in the pelvis next. This is the contrast enema so you can see. The sitcom. Probably Near from the liver and they pulled down part of the right transverse column. So this is a short. Coler Next. This is a lateral view. And you can see here. A white pre-circle mass, uh, area space. Next. The same after uh we removed part of the contrast with that tube because the patient was not able to. To add nothing next. gained a lateral view. Next So this is the This is a picture of the OR. In the OR from the anal canal, so you can see here part of the anal canal was lost and, and the structure and the stenosis and we were not able to pass ahead at number 9. Next, We introduced the, the endoscope and this is a retroversion of The segment and you can see just the lumen of the The stenosis was obstructed by the endoscope and Uh, a lot of residual foods next. We take a rectal biopsy that was normal. Next. OK. So for you, the next step with this patient, what do you recommend for this baby? He's 2 years old with a stenosis, short colon. The anal canal is partially damaged. You want to do a colostomy, try dilations with a steroid injection. Try a new laparoscopic pull through with more trans operative biopsies. Uh, transanal resection of the stenosis or an ileostomy and resection of the stenosis. So we have answers everywhere and the most, uh, frequent one is ileostomy and resection of the. OK. What we do next, please. We prepared the patient. With a very good cleanout with irrigation. Until clear we Lipi patient NPO central line. And this is the image from the operation and you can see here part of the anal canal damage and the stenosis. Then we place. Uh Multiple 50 traction sutures around the stenosis. And we did the resection next. Um, very interesting, we found the stenosis that is, uh, pointed out with a blue arrow. You can see the rectal cuff from the previous swab procedure. And the colon that was pulled through. So we resect only the stenosis and we resect part of the rectal cuff and did a new anastomosis next. This is the end of the surgery and you can see how the anastomosis is really low. Next, This is a segment that was resected, the stenosis. And this is the X-ray 5 weeks after the resection. Next, you can see poop below. What we learned from this case is it was a newborn. With A ganglionic area of affecting only the rectum. So to me the laparoscopy er was not indicated but It was on. The anastomosis was too low, so the patient. It's going to be fecal incontinent. He had a stenosis, most probably due to ischemia. Re-operation for uh discord and pathologic report on frozen section. Uh, the speed of the evidence of a transitional zone on the sigmoid and previous biopsies of the sigmoid and splenic fracture with ganglion cells, uh, also was a big mistake. And it was a large unnecessary colon resection. The patient, the current outcome of this patient is having. Soft stools, starting potty training, but we are not optimistic regarding the fecal control. This is the last one. Other complications. Can I, can I, can I, can I interrupt you a little, uh, the, the, um. Show me the previous film. These patients suffered from a stricture back. From a stricture and yet he was having many bowel movements per day like diarrhea, right? Ma That. Yes, was having, you, you can see here that the patient was having liquid stool. This is an air fluid level. And something very important about patients with Hip disease is that a patient may have stricture and yet suffer from terrible diarrhea. And yet when we go for the to see the film that you, that you take, that you take that you took after you put through shows. Show solid stored in the column, see. Yeah. And a lot of people don't know that stricture and stasis of stool in the colon in fishbone disease, even if you have a normal ionic bowel, produces secretory diarrhea. And and because the patient was having diarrhea before your operation and after your operation, I'm assuming that the diarrhea is getting much better. Of course it's never going to be normal because it's a short column and because most likely it's going to be incontinent, as you mentioned. I agree Go ahead proceed with the other. So, uh, before we, is it OK if we could field a couple of questions? And so, um, there were two questions, uh, for you. One is about, do you, do you ask for intraoperative frozen section measurement of the nerves in order to avoid the transition zone. So they want and so that here I can tell that whenever we send a frozen section they immediately give us the size of the nose. And you, you, you, and you use that to say oh we might still be in the transition zone. Has that ever, has that happened? And they, they even tell us, I think we are still in a transition zone. So that, that, uh, I'm embarrassed to say it, but that's my job is to make myself look stupid here. I, I, uh, I don't, I've never done that. I've never utilized the measurement of the nerves to decide if I need to go further back, so that's something I definitely learned today. Yeah, me too, I never, I never considered the size of the nerves in a frozen section. Interesting. It's, it's a difference of opinion. So I'm learning this is why this is good. The other, I wanna, there was a comment about for those that don't have laparoscopy, um, who can, can you do, what can you do for an umbilical incision, and Belinda touched on that earlier, um, using the umbilical incision. I, um, I'll answer first and then I'll turn it over, um. One thing that I did before I did laparoscopy was a trainee and put a Hagar, yeah, and bring that up to the umbilicus and do the biopsy as far as the dissection, which is the second half of the question, um. I think you could do almost like an omega incision of the umbilicus where instead of just infram vocal you almost extend it out like an omega shape and you can actually do quite a bit through that, but it's not going to be so easy. You're you're probably going to be doing a mini laparotomy. I don't know if any of you have had experience with doing that. So I would, if you're going to do a laparotomy, I would take advantage of doing a laparotomy and seeing everything. Don't limit your exposure. If you think about the long term and the true real problems that the patient can face, I think a beautiful scar because your suture is very beautiful. It's nothing compared to all the tragic things that can happen if you don't have an adequate exposure. So I'm all in favor. Do your midline laparotomy, do a good surgery, make sure you have good bowel, good blood supply, and the patient will be happy with a perfect midline scar and a neat, midline scar, midline in color. All about midline laparotomy. You want to leave your quadrant screen for install, and I know everybody was trained in doing transverse in babies, but actually you have excellent view in midline. I love that you said that the other reason why we do midline incisions. Also non-rectal malformations and engagement because you don't sometimes you think that you know how much mobilization you need of the colon but frequently you are grown and you have to go and mobilize the splenic flexure and sometimes the hepatic flexure so. I was trained in the area of the maximum maximum invasiveness, OK, because most of the mistakes that we saw in the old time were consecutive to lack of exposure. I'm afraid that that's still valid many times, not always, you see, of course, now it's fashionable to go for minimally invasive, but it's, it's perfectly right to be minimally invasive. But you, the priority is to do an operation that you finish happy. The happiness of the surgeon at the end of the procedure is very important, and you look at the patients and say, you know something, I'm happy and the family feels happy and you sleep well. So this, this, so I want to can I. 2 points. Number 1. Laparoscopy is minimally invasive, but gives you actually maybe even better exposure, so you're not compromising exposure with laparoscopy. A small umbilical incision, now you are compromising exposure, and my general rule is I'll do umbilical incisions if I'm doing small bowel on a baby. Like it's a generalresia, I might make a small incision, but colonic surgery, I think is very tough to do unless it's maybe a biopsy, very tough to do an umbilical incision. Yeah. And the other thing with the midline, if you need to. You can always extend if you have a transverse, there's no way you can extend to the side, but that will not necessarily help you in a bigger picture. So is it heresy to say that we always do these, uh, transverse incisions, not just for this, but should we be doing benign incisions more often for almost everything for colony, for colony colon surgery for necrotizing in our colitis. That's, uh, why not the reason we always have to do transverse. I train in transverse incisions all my life. I was doing transverses, but now I'm more director midline is uh very fascinating. OK, Doctor de la Torre, you can go ahead with your, not your with complications following. OK, next, because this in jail, it was done. OK. The most common or a common complication or Not good outcome is the colitis. Colitis is always associated with obstructive symptoms. So, to me, uh, the future of the research in this problem should be focused on obstructive etiologies. So, this is a patient with Uh, ostic colitis, you can. See the, in the left lower quadrant uh dilated loop of colon next. This is another patient with a more uh severe form of obstructive colitis next. And this is uh more severe colitis. Next. And this is an extremely severe colitis, so you can see different uh Types of uh severities of colitis is the spectrum of the obstructive colitis. You can see here how the left colon in the contrast enema is narrow. This colon is no ganglionic for sure. And you can see in the lateral view how the rectum also is narrow uh with the shape of the The rectum with inflammation. Next. When we have patients with obstruction like this one, this is another patient. That he came to the Uh, with us because he had probably after the Pultro next. We do a study that we are calling now um mucosogram. I don't know if that is correct in English, but when we do the er mucosograms for the esophagus when the patient had a portal hypertension many years ago, we can see the mucosa of the esophagus uh looking for viruses. So what we are doing is er Given a small amount of contrast to see the Uh, shape of the rectum and you can see here how the rectum is narrow in the digital segment. Next. Next slide. These green lines. Right. OK, I think we've lost Doctor Luis de la Torres, so I'm gonna advance his slides a little bit. And here is the removal of a squatting cuff. And those were the last slides. So Doctor de la Torre, I'm sorry, we're gonna move on because we're a little late. So the next patient is a newborn male patient with abdominal distension and no meconian elimination after 24 hours of life. Suction rectal biopsy positive for his strong. So here is the abdominal X-ray. Here, contrast enema. Look, look carefully in this contrast enema because you will be asked if you think this is hisrung or not. So look carefully. Sound. Do we have sound? I think we do we have sound? Everybody can hear us. OK, good, good. So this patient underwent a diverting colostomy, colostomy takedown, and a transanal salave. The patient also underwent 9 rectal dilations under anesthesia and came to us for evaluation of possible stricture at 2 years of age. So what would you do in this case, contrast enema and rectal examination in clinic, contrast enema and rectal examination under anesthesia? Wait until potty training age, or I don't know. Everybody can vote, and I'm curious to know what's your answer. We have a poll and most people are saying contrast enema and rectal examination under anesthesia that's what we do so here is the contrast enema. And we examined this patient under anesthesia and we found a severe stricture. Here you can see it was a ring-like stricture that could only accommodate a 8 Heger dilator. So the anal canal was preserved, sometimes the pictures doesn't show perfectly, but it was, so the patient underwent a redo transanal pull through and we removed this stricture and we anatomized health bowel. So here's at the end of the operation. And our routine. When whenever we have anorectal malformations, usually 14 days after surgery, we start with our protocol of anal dilation, and the reason why we do sooner after the operation is because we have an anastomosis at the skin level. In Hirschsprung's disease we have a deeper anastomosis, so we usually only start one month after surgery. And what we do is in clinic. We do a digital rectal exam. If we feel the ring line anastomos anastomosis, then we start the anal dilations. If we don't feel, if it's very soft, then we don't do anal dilation. We treat like just any bowel anastomosis that doesn't need dilation. So that's our routine. I don't stop me if you're going to address this later. What if you feel it and there is no ring? When do you see them back again? Do you, what's your protocol for the patient that does well after surgery? So that's very interesting. If it's a primary patient. So if you just did the pull through, we keep the patients on Flagyl, metronidazole, and we keep the patients with rectal irrigations, and we taper down the irrigation and the metronidazole. Now if it's a redo pull through. The patient never had enterocolitis. Like in this case, the patient came just because of a stricture. I make sure the patient is emptying well, having regular bowel movements with abdominal X-rays. So we ask for an abdominal X-ray 1 month, 3 months, 6 months, and the family always contact us in case of any problems. But in a patient that is a standard pull through, not never had enterocolitis. Your protocol after surgery, you don't do enemas with Flagyl in those patients. No, we do rectal irrigation. Yes, you do. No enemas. Big difference. This is very important to verify. We'll talk about that because most institutions and doctors don't know the difference between enemas and irrigation. Enemas are contraindicated in disease irrigation. We do it on prophylactic. Let me tell you a common experience. A patient is subjected to put through successful. The surgeon is very happy. He's competing in the ABSA meeting, saying that he discharges the patients within 2 hours after the procedure, discharges the patient. Patient goes home, and then the baby starts getting distended with enterocolitis at home. So I remember one paper that was presented years ago by the group of Solake Utah in which they, they, they started doing prophylactic proactive irrigations in the patients. So we showed the mother how to do irrigations during the 1st 5 days after they pull through. And it's very, it's very safe because it's a straight shot. The mother learns to do it. Wow. So even immediately after, yes, so she goes home doing 3 irrigations per day and with Flagyl, and then a month, every month they come back. The first month we do a rectal exam. If there is nothing palpable there, there's no dilatation, but they continue on irrigations after 1 month if the baby's thriving, doing very well. Decrease the number of irrigation twice per day and decrease to 50% the Flagyl the following month, the same, and the following month this stops everything. We prefer that rather than having the patient calling you in the middle of the night at two weeks after your operation, you think that you did a great procedure. The patient comes back extended with enterocolitis. So the enterocolitis is such a bad complication, such a bad problem. The baby sometimes died from home to the hospital and that's why we don't want to run into that questions for you. You were about to, so enemas and irrigations, so enemas is the difference is how much the enema consists in giving a certain amount of fluid with the with the with the ingredients that are irritant to the bowels that provoke a bowel contraction, but the, the disease consists in. Not having the normal peristalsis therefore are not indicated. Irrigation means to take a tube like you saw in the video, pass it through the rectum, and through the lumen of the tube, liquid stool will come out. We just pass a small amount of saline to clear the lumen of the tube. Big difference in in the concept. Great point, um, and the, the, the, this idea of using prophylactic irrigations postoperatively, I've never done that. Um, and I'm wondering, I want to pull the audience to see who, who does, uh, irrigations afterwards, but, um, has that, has there been a trial comparing those who do or don't, no, except for that paper in Salt Lake, Utah. But, but the, if you don't do, if you decide not to go for irrigations, my suggestion is to be very careful and, and follow the patient. First of all, it's very important how reliable is the mother for you. There are mothers that are very reliable. They will be calling you every 3 days, and there are mothers that just disappear, and then you hear that they went to the emergency room in a general hospital where they don't know what enterocolitis is all about. So, so if you want to play safe, you take it. There are also people that don't do irrigations prophylactics, but they do inject Botox immediately after surgery. And that would be the same as doing irrigation because the patient will not have enterocolitis while the Botox is. So Mark, I didn't see the results. Was it mostly, do you know if the numbers, mostly no 66%. So interestingly, I almost want to ask Nicole again now that we've heard. This practice of prophylactic in everybody getting prophylactic irrigations for 6 months, 3 months if the patient is doing well, 3 months and then, OK, but some patients you decrease the frequency of irrigation. The patient has enterocolitis and every time you try to decrease the. Venter and it's very difficult. That's amazing. OK, I'm curious if people would now change what we can. We don't know why some patients develop ventero colitis and some others don't. That's the problem. OK. There's something there that we don't know that we must learn and the dilations, if they felt the ring, how, I'm sorry, I missed if you said how. How do you ramp up? You go start with an 8 or whatever you say. So usually in anorectal malformation we start with a very small Hager and we increase every week, every week. So that's for anorectal malformation. In his room you don't have to. You can start with the almost with the number that you already. So if the goal size is the number 13, I would start 12 this week and next week the mom can be in the 13. So each week you, you up, whether in rectal or Hirsch one's, you go up by the week. Yeah, but the difference in his broom, you would start already with the one, got it, because the anastomosis is usually and twice a day, 3 times a day. How often do you tell them to dilate? Twice a day, twice a day with the diaper changes. OK, got it. And the dilatation also provokes the bowel movements in babies. So now we have another case, it's actually a true and very sad case, so it's a 20 month old male patient at 6 weeks old had abdominal distention, constipation, and poor feeding. Contrast enema and suction rectal biopsy showed contrast enema was done and the suction rectal biopsy showed no ganglion cells. So here's the contrast enema, look carefully. So I'm passing all images. Do you think this patient has Hiprung's disease? You can vote yes or no, and I'm gonna go back so you can see again the image. Doctor Pen is saying that I passed too fast, too fast. So we're gonna go back so you can look carefully and say if you think this is his prune, yes or no. So we have the majority of the pat of the patients, of the people saying yes. Well, maybe very good. So we have the radiologist saying maybe it's always safer to say maybe. So suction rectal biopsy was performed. The radiologist is consistent with by saying that they don't take responsibility. You see it's a good point though. I mean, if you look at this animal, let's comment on it. The animal shows that there's a spasm of the rectum. OK, so why is there spasm? You know, it's not the most florid case. There's not a huge transition zone. The size of the rectum is kind of equivocal. It's not huge. It should be big. I mean, it should be bigger than most of the other bowel. Granted, sometimes the transverse colon, if it's filled with air and they're very gassy, you know, babies are supine, so all the air is up, and so the transverse colon can be bigger. But the remainder of the colon is pretty big compared to the rectum, and it's spasming, and that's one of the criteria of Hirschprung's is spasm, even if you don't see a really florid transition zone. But um, it may be that the patient has milk colitis or you see eosinophilic colitis, um, which you could also see spasm and irregularity. Um, so I would say maybe, I would say this is the kind of case that's equivocal and the patient needs further workup. It's it's not yes or no. It's, it's maybe. So it is an honest opinion, but if it is, it's going to be a long segment. If it's going to be a long segment. The word spasm even comes into the possibilities. Doesn't that warrant a potential second contrast? No. The spasm theoretically wouldn't be there the next time you try it. Well, it may or may not, but does it mean it's Hirschsprung's or not? I don't think it does. That's my opinion. And sorry, since there's a silence, uh, Andrea, you, I, I saw you go like this, so it's oral flagyl. They want to know oral flag, not in your arrogant oral flage OK. So this patient underwent a salave endorectal pull through and interesting enough frozen section said no ganglion cells, but the final pathology rectum said ganglion cells identified rectal sigmoid ganglion cells identified, sigmoid colon ganglion cells identified. Two weeks postoperative, the patient continued to have abdominal distention and difficulty feeding. The diagnosis of an ectopic anus was considered, and the patient underwent a posterior sagittal anorectoplasty. So how often does anorectal malformation and Hisprung disease happen together? Frequently, extremely uncommon, or I don't know. Everybody can vote, and I think it's very important that we all agree upon this association. And I'm very happy to see that 100% that's extremely uncommon. It is extremely uncommon. Let me comment there. The literature, traditional literature talks about frequent association between Human and rectum malformations. Personally, I have seen in my life two cases, demonstrated cases of Himan with an rectum malformation, which is a very bad association. But on the other hand, I have seen many cases with anorectal malformations suffering from constipation where the surgeons accustomed to suspect Hurunes in cases of constipation, took a biopsy that showed no ganglion cells because many normal children may have no ganglion cells in a biopsy for different reasons. There are many factors of error, and they did a push through and left the patient incontinent, a patient that never had history. In other words, before you make that diagnosis and before you embark yourself in a pushthrough for history, a patient with anorectal malformation, think about it. Most likely it's not Hines. So there are many, many patients suffering from constipation and very few suffering from heburn in patients with anorectal malism. So just think about the techniques that the patient underwent, the patient had an endorectal pull-through with the removal of the rectal sigmoid, followed by a posterior sagittal anorectoplasty, where the most distal part, the fistula, is removed. So now what's the prognosis for bowel control in this patient? Excellent, provided the patient has a normal sacrum, not that record and meticulous surgical technique was observed or poor, the patient will be fecally incontinent now because it has no anal canal and no rectal sigmoid, so everybody can vote. And I'm seeing 100% saying poor, the patient will be fecally incontinent, and that's absolutely true and absolutely sad. So here you can see that the sacrum of the patient is normal. And here is the contrast enema just to prove that the patient truly underwent a pull through for Hipro, so there's no, we don't see the sigmoid curve anymore. And they also did an MRI, so the patient does not have that report. So in conclusion, we do not know if the patient originally had an anorectal malformation or not, but we do know based on the pathology report that the patient did not have Hiro. If he had an anorectal malformation, he was initially in the good side of the spectrum with normal sacrum and no tetracord, and now this is a patient that is fecally incontinent and will need bowel management in order to be clean in the underwear. So now looking at. Looking at this contrast enema and with this history, which group do you believe this patient belongs to? Does this patient have a tendency to diarrhea, hypermodal, or a tendency to constipation, hypomodal? Everybody can vote, and it's important to differentiate those two groups because they have different treatment. The enema will be differently depending on where this patient belongs to. And everybody got it right. That means that they learn from the bowel management global cast that we did, and it's truly hyper modal with tendency to diarrhea. So knowing that, what is your bowel management? What's the bowel management that you will recommend? Laxatives at night and Imodium during the day, laxatives and fiber, enema of 250 mLs of saline only. Enema of 500 mLs of saline, 30 mLs of glycerin, 27 mLs of castile soap, and pediatric fleet, and everybody can vote, and I'm seeing everywhere that means that they actually didn't learn from the bowel Management Globalcast, so maybe you should watch it again. It's on the recording. No, now we have better, better answers. It's getting better, so I'm still positive. Maybe some people didn't sign up for the bowel management globalcast. So we actually have the majority, almost 80%, saying 250 mL of saline only. That's an important point for all of those here. Um, that was a fantastic event. You can go and watch it any time. Uh, it was a great event about the whole management of of constipation. Bowel management was just fantastic, and if you go on the site, you can watch that recording. It's great. And I just want to comment in this very attractive answer the laxatives at night and Imodium during the day. That is a dream drug that we don't have. If we knew that you would take the laxative and you would have the immediate effect, meaning I take the pill and I will immediately go to the to the toilet and have a bowel movement, that would be perfect. But unfortunately we don't have this drug available in the market. If you take laxative, you will have many bowel movements at unpredictable. Time, so if you're giving laxatives to a patient that is fecally incontinent, you're just making this patient worse. Now if you're giving laxatives to force the patient to have bowel movement and at the same time you're giving a drug to try to decrease the bowel, the motility of the bowel, you're just giving drugs that have opposite effect, so unfortunately they don't work. Any other questions about this case or any question that we have um in the post or in the chat? No, there was a comment that that there's, you know, has anyone really ever seen a patient of case of enterocolitis with an innerectal malformation? Have you ever seen that? So I presented one case here that was a true Hirprung with an ectal malformation, but that's an extremely rare case with the mutation. I'm saying no Hirschprung's. Pure analectal malformation with enterocolitis. Oh no, I have doesn't exist. We have, we have seen, I have seen patients with severe, severe idiopathic constipation. Overlapping with intestinal pseudo obstruction. With symptoms of enterocolitis that almost die from that from clostridium and things like that or even necrosis of the colon, but that's a, that's a different disease. Um, have you, uh, when we were talking more about fecal incontinence, have you ever in any of your patients used the InterStim, the, the stimulation? Have you heard of that or used it at all sacral nerve stimulsacral nerve stimulation. So not yet, not yet. Here, not yet. And I think we just recently reviewed the literature about the experience in patients with anorectal malformation. Published reports we could only find 10 patients, and they don't specify the type of malformation. Then they don't specify the quality of the sacrum, so we don't, we cannot evaluate the results. So I think the experience is not enough. The literature of that mainly in the use of electrical stimulation in adult patients, mainly ladies who had a delivery of a baby had some sort of fecal incontinence, and they use that electrical stimulation. But the problem in non rectum malformation is that they frequently have abnormal sacrum. So when you ask them about the, what's the mechanism of action, they assume that the electrical stimulation goes to the nerves, sacral nerves, which in many anorectal malformation patients don't exist. So it's a, I'm rather skeptical about that, but we, we had a, we are discussing a protocol to try it because we want to demonstrate it to see what's happening actually, but I'm not very optimistic about that. So this was a case that actually Doctor Fisher was gonna present, but he was called to the operating room. That's why he's not here anymore. So present is just the last case and it's a very short case. This is a 2 year old male patient with a history of no meconium elimination after 3 days of life. Patient underwent a suction rectal biopsy that confirmed hisrune. Underwent leveling colostomy followed by a suave colostomy put through came to us after recurrent episodes of enterocolitis. So here you can see in a simple abdominal X-ray film you can see a lot of gas in the column just by looking at this abdominal X-ray, we will tell our fellow immediately do a rectal irrigation. And here is the contrast enema of the patient. And here in an AP view the contrast enema of the patient. So Doctor Fisher did an examination under anesthesia, found an intact anal canal and no stricture, did a biopsy, and the biopsy came as a ganglionosis, so the patient underwent a redo trans anal rectal sigmoid resection and a diverting ileostomy followed by ileostomy closure. How much ganglionics the patient that I don't know because Dr. Fisher is not here just presenting his case. I'm assuming that it was just this portion up to here, but I cannot confirm that he would be the one to say that. So before we end this event, I just want to mention that every month we have an international Colorectal web meeting together with Doctor Alp Numanoglu from Cape Town. I hope he's joining the show, at least he said he would be joining the show, so.