Decision Making in Colorectal Surgery

Welcome, everybody. Good morning, good afternoon, or even good night to the 3rd webinar, um, Decision Making in Colorectal Surgery. Our expert tonight is, uh, Doctor Mark Levitt, um, who does not need any introduction, maybe the most prominent active colorectal surgeon worldwide. Um, since 2018, he's Chief and professor of the Division of Colorectal Surgery, um, at the Children's National Hospital in Washington. And he's not only an outstanding colorectal surgeon, but a great teacher and a great friend, uh, for a lot of us. Who taught us like many lessons in the past that made a difference for a lot of children with colorectal disorders. So Margaret, it's a great honor for us and for UPSA to have you here tonight with us. But before you have the word, I would like to introduce uh my co-moderator, um, Augusto Zani from the University of Toronto, Gaya Tamara from the UPSA office who set this meeting up again, and our three panelists. Uh, Alejandra Villanova Sanchez, um, a pediatric colorectal surgeon at the University Hospital La Paz in Madrid, Paula Mio, who is the director of pediatric department in Italy, and Stefano Giuliani, who is a pediatric surgeon at Great Ormond Street Hospital in London. So including Gaia and Augusto, it's Italy 4 and um Spain, USA and Germany, 1 each. So I guess we, we can't beat Italy tonight. Uh, so we have to focus maybe on the quality of our answers to make the difference. So as I said, um, Mark, we are all looking forward to this session with you on decision making in colorectal surgery, and now you have the work. All right. Well, thank you again, Martin. I, I love this, uh, format. Um, I love working with Yupa and um we have a lot of wonderful friends on the call. So far, 98 participants. Um, and soon we will all be together in Athens in September. And in the meantime, we will have to work this way, but at least we can all gather together. I, we picked some great cases to discuss that are particularly interesting, controversial, and there's not necessarily one correct answer, and that's what I hope will be provoked in the, in the conversation. So hello to you all from all over the world. And um let's go for the first case. So we need, um, we need the first slide. I hope everyone can see their screen. Yeah, let me just, Mark, let me just, sorry for interrupting. Let me just introduce our format with a few slides we have prepared. So Gaya, next slide, please. So first of all, we are all on Insta now since this week, so you can follow us, next slide. Also on Twitter, we have a new platform there that we are relaunching. So it would be great if you shared um activities there too with us next slide. Also, the YouTube channel where this webinar will go also on from next week. You can, um, we will forward the link and then then you can actually uh listen to or watch the, the session again, next slide. the videos on the YouTube channel, next slide. And these are our next webinars we were, uh, we were having one per month on these topics that you see here. We will have basic science coming up next slide. And uh there's a colorectal course also with Mark Levitt on September the first, uh, in Athens, which is no lectures, but with a panel also, and pure brain on. So, um, it will be, I think, very exciting session. Next slide. And uh some of the um activities nowadays are on that state current app that a lot of you might have. And there's also colorectal quizzes on it. And I guess that's it. So we can start with the first. Patient. And I think Alejandra will introduce, right? Yes. OK, so this is a nine year old healthy female, normal growth curve with constipation for many years, uh, has a bowel movement once a week. The emergency department with a very distended abdomen, soft and non-tender and normal premium. These are the X-rays and contrast studies. So, in the X-rays, um, uh, we can see a very dilated bowel. I would say this is a very dilated colon. And in the contrast enema. I do not clearly, clearly see a transition zone, but I do see abnormal spiculations of the mucosa that remembers me what we usually see in patients with associated hisperous enterocolitis. Uh, can you, uh, advance this slide, please? Gaya. OK. So, she was admitted for evaluation and treatment. After 3 days of enemas, she was taken to the operating room for uh EUA and rectal biopsy. And the rectal biopsy showed no ganglion cells and hypertrophic nerves of 70 mgs. After the cleanout, she felt well and she was discharged home on 5 mg of Bisacorol a day. She, since then, she has had two bowel movements a day, normal bowel control with no soiline and a flat abdomen and had this X-ray. In the x-ray, we can see a small amount of stool in the, in the rectum, but it's pretty clean though, so, uh, no dilation, no, no more dilation. Um, so can you advance the slide, please? Well, let's wait, Alejandro, before, before we advance the slide, um. Let's talk a little bit about the radiology and then we'll discuss the case. So, um, maybe we could, um, just go back. Actually, the same images are here. Um, I, I really like your description of the, um, of the radiology. We have a patient who comes to the emergency room with a very dilated, clearly colon, um, and ultimately gets a contrast study. I agree it looks like an enterocolitis picture. Um, and although remember, this is an older patient. So, the older patients with Hirschprung's tend to not have such serious illness like the very bad enterocolitis we often see, uh, in babies. So that's why this case is a bit confounding. Um, and what did they do? They admitted her and basically cleaned her out as if she was an impacted patient. And they successfully got her cleaned out with enemas, and then they went to the operating room for an exam and found a normal exam, and the rectal biopsy shows no ganglion cells and hypertrophic nerves. So my question to you, the panel, is this Hirschprung's disease? Let's hear from each of you. Um, I would say yes, this patient probably has a very short segment of Hytrin's disease and she might be overcoming the functional obstruction of the internal sphincter because the, the lung of the ganglionic bowel is very short, but of course, this is Histrin's disease with hypertrophic nerves and no ganglion cells. OK. Does, does everyone agree? Paula, is this Hirschprung's disease? Well, if, if the, hello everybody, first of all, um, if the, the biopsy were taken correctly because she's an old patient, so that cannot be done by suction biopsy. And so I assume that done correctly surgically at least 3 centimeters from the anal verge because she's already 9 years old. So yes, no ganglia is equal to um Hestrung, but because she has come to this old age without great discomfort, I would be very, very conservative in the treatment. Oh, we'll, we'll, we'll, we, we will definitely get to treatment and um I appreciate you mentioning the technical details of the biopsy. That's very important. Obviously, we have to make sure the biopsy was done correctly. Uh, maybe we can talk briefly. Are there some clues on the pathology that the biopsy, a biopsy, not this one, but a biopsy is not done correctly? Um, who, who, um, who can answer that? Maybe, maybe Augusto, how, what do you want to absolutely see in a biopsy before you conclude that it's a good biopsy? So you wanna see the ganglion cells, uh, uh, but also, I mean, what we do in my hospital is one of the questions on the chat is also, uh, the caloridin staining, but if it's positive, it's also confirming the presence of the, of the cells. The hypertrophic nerves are associated with, uh, a typical, a typical feature of, uh. I sprungs uh with the on uh the acetylcholinesterase, but I don't, I think for me it's the absence of the cells. All right. So I, and I agree, and I think we have to be careful not to fall into any traps. One trap is if they do not talk about the nerves. And I've definitely seen patients that had no ganglion cells, but no comment of the nerves, and that is not Hirschprung's until someone tells me about those nerves. The other thing that I want to know is that there is no squamous epithelium on the biopsy, because if there is, then the biopsy is too low. Another interesting thing to look for is, are there many eosinophils in the presence of ganglion cells? And in a baby, does anyone know what that could mean? I, I think is that what they call the FPS, the food protein allergy, milk, milk protein allergy. I'll tell you, once I had a patient that looked exactly like Hirschprung's disease, had a classic X-ray. I took a picture of the X-ray and added it to my Hirschprung's carousel of slides because it was so perfect. And the biopsy then came back with ganglion cells and eosinophils, meaning it was milk protein allergy, and I removed the slide because the patient obviously did not have Hirschprung's disease. They had an allergy to the milk. But it looks very much like Hirschprung's disease. All right. Well, anyway, I think this biopsy was done well. And by the way, we know it was done well because Yaron Armon from Jerusalem sent us this case, um, and, um, he did a beautiful biopsy, I am certain, and it's clearly Hirschprung. So now, let's go to the next slide and discuss whether this patient um needs surgery. So what do you think, um, Stefano? Yes, hello, everyone. Um, I think she's, uh, she's quite healthy. She has coped with, uh, this ultra short segment of Ip disease for 9 years. Uh, I understand, uh, in, uh, without having enterocolitis, uh, and the suffering of constipation, uh, which is also common in the general population, and, uh, uh, I mean, if she's well now and she's able to pass stools as we can. Uh, as we have assessed after the cleanout, um, really, I would, uh, I would observe her and see how she behaves. Um, I mean, there is quite a lot of dilatation, especially on the first, uh, abdominal X-ray, and, uh, there is a component, uh, of, uh, uh, mega mega rectum and megasigmoid possibly, and that will take a while to shrink. But with, uh, a good bowel management, she may, you know, cope well, you know. Without the surgery. Yeah, I, I think she may need surgery if she, if she gets back on enterocolitis and uh And further troubles. I think that's very wise. I think the lesson here is that we treat the patient, not the biopsy. Um, the patient has Hirschprung's. This is not the first time I've seen a relatively well patient with Hirschprung's that we did not operate on. Um, and I think you just have to watch this patient very closely. And if they misbehave and start having obstructive symptoms, then you have to do, you have to do an operation. But at the way, the way right now that she was managed with a cleanout and a low dose of laxative, I think it's safe to observe. Martin, do you agree? Yeah, I agree. Um, however, I would still be concerned that a severe anticolitiss at some time would make the patient really severely sick. Um, but it's, um, it's a judgment call, you have to, um, you have to do with the patient. So Mark, there's an interesting comment from the chat. So Dr. Kumar is saying, is that ultra short Hirshbones? Could you comment how you define ultra-short Hirschbongs and maybe why this is not a case of that type? Yeah, you know, I prefer to not use that term. I think we should talk about Hirschsprung's anatomically. We should say rectal, sigmoid, left colon, splenic flexure, transverse or right colon, or total colon. Hirschprung's. Ultra short Hirschsprung's is just confusing. If there are ganglion cells. 1 centimeter proximal to the dentate line. It is not Hirschprung's, period. Period. Now, you may find hypertrophic nerves, but I think those hypertrophic nerves are a secondary phenomenon to the constipation and the dilation. It doesn't mean the patient does not need an intervention and doesn't need help from a surgeon, but it's not Hirschrung's. Hirschrung's means A gangliosis, hypertrophic nerves, and the patient would benefit from a resection, in most cases, rectosigmoid resection. The patients who have ganglion cells within 1 centimeter of the anal canal can have severe symptoms, constipation. I've even seen patients like that with enterocolitis, but it is not Hirschrung's disease. Remember, that patient might benefit from Botox, because that patient may, their biggest problem could be just the sphincters. And it's the same sphincter dysfunction, and I'm describing internal sphincter alagia, the same sphincter dysfunction that Hirschprung's patients have. The only difference is that patient does not need any transanal rectal resection. So I would say Hirschprung's or severe functional constipation in two different categories, and Mother Nature makes fun of us when we make categories, so she finds patients that are Hirschprung's-like, but in fact have functional constipation, and there are functional constipation patients that behave like they have Hirschprung's. And what is this case? This case behaves mostly like a constipated patient, yet we have a biopsy that shows it's Hirschprung. So this patient is on the border zone between the two. And someone wrote in, someone wrote in the chat, let me just respond to that, can benefit from myectomy. I'm not a fan of myectomy. I would rather do Hirschsprung's, I'm sorry, I would rather do Botox treatment. And the patient eventually will improve. The problem with myectomy is it can give permanent damage to the sphincters. Augusto, you had a question. Yeah, about the biopsy. So when you have a patient that is older, it is not the typical infant, uh, how, how, it very much depends where you take your biopsy, especially if you're doing an open biopsy. Do you go above that 1 centimeter, uh, proximal to the, to the dental line, 2 centimeters? What, what, what do you do? Yeah, I mean, Paula mentioned 3 centimeters, and for me, that's a little bit far. I, if you can see the dentate line well, and you're at least 1 centimeter above the dentate line, clearly in columnar epithelium, I think you will get a good result. You obviously don't want to be too close to the dentate line. Because that normally has a zone of a ganglionosis, but I think 1 centimeter proximal to the crypts, you're gonna be safe. I want to make, I want to recognize that we have a few cases to go through. So, um, if it's OK, Martin, shall we go to the second case? I think it's a great idea. Yeah. OK. OK. This is the 2nd case. This is a neonate, male neonate uh born with an ARM who underwent a colostomy, uh, the, the next day from birth and, um, quite in a strange manner, several days after he was born, they noticed that a dot of meconium appeared at the base of the scrotum. So they took a, a contrast enema, uh, from, um, to, to define the anatomy uh of the distal column and this is what is shown in the picture. So the column is filled with contrast and the dot, uh, which is at the base of the, um, Of, of the X-ray is where you want to put the uh marker uh at the uh annal dimple. So, uh, next slide, please, Gaya. OK. So with this kind of uh uh X-ray and um information, uh, what do you think is this anorectum malformation? Meaning, uh, he has a colostomy, uh, colosstogram, and apparently a big piece of uh, uh, rectum is missing, uh, up to the, uh, the, the dot line, the dot point. But he has meconium at the base of the scrotum, so what do you think this is? Yeah, so let's discuss this because obviously, this is not the typical sequence. Usually, you, uh, you go to the operating room for a colostomy when you're very confident that there's no perineal fistula, but sometimes the perineal fistula is not obvious, and the safe thing to do, of course, is a colostomy. And then, a couple of days after the colostomy, there is meconium on the perineum. So, very strange. What is, what do, what do people think? Alejandra, is, do you have a, a name for this malformation? I don't think I can comment on this malformation because the contrast enema is not, is not done under enough pressure. So it might be Yeah, a longer piece of bowel reaching the perineum, but we don't, we don't know because there is not enough pressure. The rectum is flat. It means we are missing some rectum there. So it is probably a perineal fistula. Uh, that we, we, we don't know from, from that image. Yes, so, um, I really want you to burn this image into your brain. The distal rectum is flat, and where is it flat? It's flat at the PC line, the pubococcygeal line. If you drew a line from the pubic bone to the coccyx, that would be the line. And that is the normal compression of the rectum by the pelvic musculature. So you should see this image and you should immediately say this is an inadequate study. Also, say to yourself, how could it possibly be that there is a no fistula, yet there's perineal meconium. Something is wrong here. And what's wrong here is that the radiology is trying to fool you and you will not be fooled. So, the adequate pressure would show a very deep And very reachable rectum in almost every case of a perineal fistula. Someone wrote in the chat that maybe it's a high rectum with a long fistula. That's a concept that comes from probably inadequate pressure. I have seen that very rarely, maybe once or 2 times. Hundreds of patients, the rectum is very, very low, within 1 centimeter or 2. Stefano, do you have anything to add? Uh, not really. I, I, I agree that this cholostogram is, is not well executed, and, uh, we will need, uh, basically more pressure, um, you know, I, I, I also, you know, I've read, I, I don't have direct experience, but I read a few reports saying that, uh, you know, it is possible to have, uh, you know, a high and rectum malformation with a very tiny fistula running in parallel with the urethra, and, uh, you know, it is very important to define that. Uh, again, with more pressure because, uh, the strategy for the surgery, it's, uh, it's, it's, it's, it's essential to avoid damages to the urinary tract. OK. Well, to follow up on that, what would be your surgical approach for this patient? I'm, I'm assuming all of us would do a repeat distal cholostogram, and we can anticipate that the rectum will be much lower than what we see here, and probably we can delineate the fistula. And then, so what would be your surgical approach, uh, laparoscopy or posterior sagittal approach? Maybe Paula can take that question. Um, definitely posterior surgittal approach, especially if the cholostogram demonstrate what you just said, that the rectum is expected to be really very, very low. And you can try to cannulate from where the meconium dot comes out. Try to gently see if there is some. Some fistula that um leads you to the rectum but without forcing or chasing anything, you go straight PSA and the first structure you will enter is, uh, it will be rectum. Now, to follow up on Stefano's comment, which I think is very clever, very commonly, there is a long fistula paralleling the urethra. So what do you do in that circumstance? When you open posterior sagitli and you find your reachable rectum, what do you do with that fistula? Martin, do you have a comment about that? Well, there are two options. You can either leave it, just lay it open, it will epithelialize. I, I, I used to cut it out, but that's not really necessary. But my point here, I wouldn't even do a PA. I would follow the fistula, lay it open, and then it's more or less a perineal fistula where you can do almost a cutback. What do you think of that? Well, I think, I think that the, I think it's a very wise point. The problem here is the fistula is in the scrotum. So this is not a perineal fistula type of malformation that's amenable to a cutback. But many males, the fistula is at the anterior point within the ellipse of the sphincter. And then I think a cutback, a minimal posterior mobilization is very reasonable. But in this case, we mentioned that the base of the scrotum is where the fistula is. So be very, very careful because the fistula is paralleling the urethra. You don't need to go anywhere near there. It will just disappear. You just do your standard mobilization of the rectum, make a nice anoplasty, and don't mess with the urethra because you will get into spongiosum bleeding, which is really, really uh unpleasant. All right. I want to, uh, I wanna make sure we get through our great cases today. So let's go to the 3rd case. Yeah, sorry, if you, if you had seen the meconium pearl within the 1st 24 hours with a completely covered the anus, so there was like, you know, even a dimple, would have gone, so the concept here, I think the take home message is, uh, if you have these meconium pearls, for sure there, it's a low and rectal malformation. Maybe there's not a need for a colostomy. You go for a PSAP. You would have done a primary PsARP. Yes, yes, I, I would have done a cross table lateral film to see how low is the rectum, and based on how low was the rectum on that image, I would go for a primary repair knowing I was dealing with a perineal fistula. So yes, you're, you're quite correct, and And someone noted that um they would have liked to see a photograph of the perineum, and yes, we agree, we're just trying to limit some of those kind of photos because the last time we did a presentation like this, we were, we were shut down because of those images. Now the Next slide, we're gonna leave up very briefly because it has some of those images, but I, trust me, it was a perineal, tiny perineal pearl pearl of meconium in the base of the scrotum. But Margaret, the last time we were on Facebook, so this concern is not uh a problem tonight. OK. Well, I'm happy to hear that, but just, uh, uh, your question is correct. We would love to show you a perineal photo and just trust me, it would have shown a very normal-looking perineum, obviously, with no anus, and a little bead of meconium at the base of the scrotum. All right, let's go to the next case. Yes, so next case is a 5 year old boy who was born with uh rectal malformation, underwent the 3-stage repair with a colostomy, a PSAP, and the stoma closure within the first year of life, and then presented, uh, back years later with uh a fecal incontinence. Um, now below you can see some inoperative images of the assessment. Um, I think you know in, in this case, the most important thing is how we approach a child with fecal incontinence or with problems a few years down the line after the repair. And uh you know, I, I would uh advocate for a very thorough assessment, uh, not only of the rectum but also of the spine and of the urinary tract, uh, to understand uh what is the best continence that we can achieve in this child and if there are any other associated anomalies that can cause fecal incontinence. And uh of course, at the end, we need to also understand if the anus is in the right position so it has been pulled through in the middle of the muscle complex or not. Um, from what we can see here, um, the exam under anesthesia, anesthesia showed uh um that the pain stimulate, you know, the, the, the stimulation of the, um. Of the muscle complex is basically off uh where the opening where the neo anus was created. And uh this will poses probably the next questions and uh. Discussions. Yeah, so let's, let's leave this image up. This is, um, a common scenario where in your clinic comes a patient who's had a previous PSARP and is presenting to you with fecal incontinence at the age of 5. And as you have nicely stated, Stefano, we have to figure out what is the anatomy of that PSAP and what are the associated malformations that could affect bowel control. So we want to know the quality of the sacrum and the quality of the spine, and we'd like to know what was the original malformation. And based on those three criteria, we can make an assessment of whether this patient with well-done anatomy should achieve bowel control or not. In this particular case, the surgery has two major problems with it. One is the anus has a, the anoplasty appears to have been placed lateral to the sphincter mechanism, and you can see that in the middle panel. The sphincter mechanism is in the middle, where the anal dimple is, and the anoplasty is to the side. My question to you, Paula, how do you make sure to not do a mislocation when you do one of your PSAs? What are your maneuvers? During a regular PSA, you, that's, that's correct, because obviously this patient had a PSARP and the surgeon put the anoplasty in the wrong place, and the question is, how do you avoid this mistake? You, you absolutely need to check it with the stimulation, and there are many devices on the market to stimulate the muscle complex. And you want to stay in, in the midline, uh, within, uh, you, you open, uh, along the midline, and by the time you go, uh, layer by layer more deep, you see the parasagittal fibers and the muscle complex coming, uh, from both sides, and it, yep. I was gonna say the one thing I like to do is I like to mark the sphincters before I start. I think, I think one of the problems is that a surgeon is excited, ready to do their PSAP. They open posterior sagitally, and then they get a little bit lost in where all the muscles are jumping around, and then they put it in the wrong place. But I think if you mark the anoplasty where your eye believes it should be and where the sphincter stimulation tells you where it should be. And then you match that location once you are open, you will very likely not make this mistake. Martin, Martin, can you, oh yeah, go ahead, Paula, um. I, I, I did, I, I always marked the place before and I am not using the marking pen anymore because by the time you arrive at the endoplasty, it's, uh, disappear. So I put two stitches, uh, like one corresponding to the anterior margin and one to the posterior on both sides, so they will stay. And with the, by the way, I, I wanna, I wanna just comment on what my friend Alan Mortel just wrote, um, which is one of the hard parts of colorectal care. Unfortunately, even with a perfectly placed anoplasty, fecal incontinence can be the final outcome. Um, Alan, that is very, um, philosophical. And when I first met you, you were not such a sage, but I guess you've gotten older. Um. Here's the thing, you can do an absolutely perfect anatomic operation and not get a good result. And that is the most frustrating part of colorectal care because usually in surgery, if you do a really nice technical operation, the patient does well. Well, that's not true in colorectal care because they might have a bad sacrum, poor sphincters, a bad spine. But one thing we must guarantee as surgeons is we must give them the best anatomic repair that is possible, and that is our obligation. And then, of course, some will not achieve success still. This patient actually, I believe, has good potential for bowel control. But the anoplasty will never allow this patient to achieve bowel control where it is currently located. The other thing I want you all to to notice, why did we do an MRI? And I think a pelvic MRI is mandatory in these evaluations because What do you see on this MRI? What was the other thing done wrong at this original surgery? Anyone want to put in the chat what they think is the answer before Paul Paula gives the answer? I would like to make a comment going back to the uh how we mark the sphincters. It's very important to tell the anesthesiologist to avoid muscular relaxation before we mark the sphincters because we can underestimate the strength of the of the muscular complex and we can be confused whether uh we should put the, the, the endoplasty. So this is, this is very important. So Mark, may I ask, ask a question? I, I realized that, that people put in like the wheatlander retractor too early. From you, I learned to put on these lone Star pins and really go slowly. Also looking at the paraceular fibers, re-stimulate, and people rush into that, then spread with the wheatlander, destroyed their midline, and then go deeper, and then that happens. Do you, do you agree that's part of the problem, or? Yes, I agree with that. I agree with that. You go, you skate perfectly in the midline. And gently go through and define all of your layers, um, and don't injure, anywhere the, uh, the muscles. I think the opening is very important for the ultimate closure. Remember, the PAP is to open. Then you find the rectum, and then you close. And the closing is just around the rectum that wasn't there at the beginning. That's the, that's the job. So, a number of you got the answer correctly on the MRI. You see an out pouching from the urethra. I used to call this a posterior urethral diverticulum, but our urologist said, Mark, it's not urethral, and it's not a diverticulum. Could you come up with a new name? So Vicky Lane, our fellow from the UK at the time, said, how about this, remnant of the original fistula, roof, which I think is a much better term, because that is the old rectum. The surgeon went in posterior sagittally. Found the rectum was too far away from the urethra and left behind the old distal rectum, which is why we have must do an MRI because we don't want to fix this rectum and not solve the problem of the roof, remnant of the original fistula. And by the way, I always do a pelvic MRI and these redo evaluations, and I also do a cystoscopy, because the MRI could miss it if there's no urine in the, in the roof at the time of the MRI and the cystoscopy can also miss it if the urothelium has healed over, there still could be a mass that is the original rectum. So I always do. A um a cystoscopy and an MRI and someone wrote, does the roof cause symptoms? In this case, not yet. But I think if we're going to do a redo to relocate the anus, that is the time to remove this remnant because that remnant can cause symptoms down the road. Most typically dribbling of urine after a complete void. Also, the the mucus can leak into the urethra and They can void mucus. I've seen patients with stone formation in that, uh, distal rectum, and I have seen one case of an adenocarcinoma in a 30-year-old from a remnant of the original fistula. So just take a look for those. In this particular case, looks like it was a low prostatic rectum. And I think that a redo, removing that, putting the anus in the correct location can really do wonders for this patient. So I want to, in the interest of time, I want to make sure that we can go to the next two cases, so we finish on time. Let's present case number 4. OK. I, I can start. This is 11 of my favorite topics. So this is an interesting series of uh pictures of female patients. And the question is, which, which one needs surgery and which 1 may not? So the first one on the left, a panel A, clearly there is no anus or maybe Paula, let, uh, let's have them vote. Let's have them vote. OK, so let's let's talk a patient A surgery, surgery or no surgery. Put, put them in the chat. And by the way, in the chat also put what country you are from. Because I want everyone to realize how incredibly international YUSA is and how we are all fighting the same battles every day, and we suffer together and we enjoy being able to suffer together. So, a, most people, it seems, Paula, are saying, oh, we have Greece, we have Slovakia, we have Germany, we have Iraq, Bahrain. Indonesia. Someone put Jakarta and they put Indonesia in parentheses so that I made sure to know, but I promise you I already knew the answer. Bolivia, Netherlands. Hi, Sanna, how are you? Good to see you on this call. Zimbabwe. Cape Town, which is, uh, by the way, I think in South Africa, India, Singapore, Vietnam, unbelievable. Pakistan. Do you like how I pronounced that correctly, right? Pakistan. OK, surgery for A. Everyone agree on the panel. Stefano, you agree. Alejandro, you agree. Martin, Augusto, absolutely. Thumbs up. OK, cause that's a perineal fistula that is clearly superior anterior to the sphincter ellipse. OK, Paula, continue. So panel B. We see a hole in the perineum, which is really close to the posterior rochet. And not really look at normal looking anus because there are no wrinkles and there are questions on the diameter. So surgery for this. Other checking besides just looking or no surgery? By the way, I put in my answer, Paula, and I put um surgery, United States, and I am an honorary European. If, if you'll have me, about a year ago, there was some question whether the Americans were going to be kicked out completely of Yupsa, but hopefully you'll still have me. Well, because you behave, basically. All right, it looks like, it looks like everyone wants surgery. How about the panel, Martin, Augusto, Stefano, you agree. By, by the way, I will tell you this is an interesting case back to the cutback discussion. The question is, do you need to mobilize the anterior part of this rectal wall? And what do you think, Paula? Does this require an anterior rectal wall dissection or only a posterior wall dissection? Well, likely it, it needs to be fully mobilized, but you need to see where the muscle complex is, and from the picture. The pinky area where the muscle complex is looks completely posterior. So, yes, I think upon stimulation, uh, he needs a full mobilization. Yeah, so you're quite correct. Take the patient to the operating room, stimulate the anus. If if the hole is too small, but it is inside the ellipse, just happens to be anterior, you can do a posterior wall mobilization only, essentially a glorified cutback. However, if the hole is not in the sphincter, you do not want to leave the patient with an anal opening with no anterior sphincter, cause that can lead to incontinence. All right, how about C, Paula? Mario, sorry to interrupt. Can I ask a question for B? So if you just did a Heineken-Mikolizkowski and wait. Until the child becomes continent or not. I think in these cases you have to balance relocation the anus and losing the dentate line and the canal versus uh. Martin and I, Martin, Martin, I agree with you. I think this patient, what I would do for this patient is I would mobilize the posterior wall only and do a, a larger anoplasty than what you see here, and I would leave the anterior wall alone, provided when I stimulate to to confirm Paula's point. There is some muscle anterior. You don't need to do very much at all. The only goal is to make the hole adequately sized. The, the perineal body is long enough here and it will grow. All right, C, I think is very similar to B. Let's talk a little bit about D. Who says, and now we're gonna wait for everyone to respond. The surgery or no surgery. So D looks much different from the others because the don't answer yet. Don't answer yet. I'm not answered just one says the D dilate, dilate, OK, dilate, no surgery. Also, don't, we want to know where you're from. Anal rectal angle. The angle is gonna be normal when the rectum is so low. Someone wrote, Paula is an influencer. I like that. I like that. Yeah, by the way, Paula, what you have like 100,000 followers on Instagram, right? Wow, still below my average. Is the question is, do you have more followers than your age? Ah, OK. So that, that's, I'm fine. I'm fine, thank you. Anyone, is anyone operating on D? To check first. All right, very good. Now, Paula, tell them what you were seeing. Why is D so different? Because D, uh, looks a normal anus only really, um. Uh, close to the Fouchette. Uh, normal anus because the wrinkles are 360 degrees surrounding the anus, therefore, the muscle complex is there and very likely when the, the muscle is all around, this is a stretchable anus. So, um, For me, it, there is no um need to take extra examination under anesthesia, provided that the uh the anus is stretchable and in a term baby, this is a long discussion still going on, in a term baby, um, the anus is around 10 millimeters. Someone goes for 12, someone for 8. We as ArnetT consortium are going for 10. So, um, in, in a 6 month old, I don't know, this baby looks older than a newborn, uh, it should accommodate at 1314 millimeters without, um, excessive, excessive stretching and screaming. I, I, I agree. It's, you cannot improve. That one is as good as it can be. All right, let's do the final, let's do the final case, so we end on time. The final case. Who's presenting the fifth case? Um, I could do the first. Go ahead, Alejandra. OK. One year old with total colonic history disease, ileostomy and colonic biopsies in the newborn period. He presents with massive abdominal distention. OK, so that's all you're gonna get. We're gonna talk about this patient. So, a total colonic Hirschprung's confirmed by biopsy of the colon and has an ileostomy that has done well until this recent, very impressive uh visit to the emergency room. OK. So, August Augusto, what do, what do you think here? What's happening here? So I see that there's uh small bowel loops that are dilated, so there's uh an issue with, uh, with the ileostomy. I would, I would assume that whoever made did the ostomy actually confirmed that it is ganglionic, that that's important. I wanna know whether um the, the, I would examine the, the patient and look at uh the, the stomach itself and make sure that it's uh it's working OK because it could be that there's, uh, if it's all of a sudden, uh any stoma can twist or, or, or be obstructed. OK, so that's the obvious answer, and in fact, Likely the answer here, but I do want you to think about another very uncommon possibility. Was, was the colon left in place? Yes, the patient, so, so first of all, let me just reaffirm, Augusto, 100% correct. This is most likely a stoma problem, either stomal stenosis or a pathology problem of the stoma. Remember, if you have a stoma in Hirschprung's disease, you are stooling through a low pressure system. You should never get distended. You should never get enterocolitis. If you get enterocolitis in a stoma, you have to blame the stoma because there's no way you should be having obstruction in a stoma unless there's something mechanically or pathologically wrong with that stoma. So I'm sure everyone thought of that. We did an exam, the stoma was somewhat narrow. And we needed to do a revision of the stoma, but. I want you to think about something very uncommon, and that is the deffunctionalized colon. We always seem to forget about the colon, if a patient is already with a stoma. I have seen patients where the colon can get develop a toxic megacolon, even though it is distal. And why does that happen? Because the bacteria overgrow. And I remember a vivid case that I was convinced it was the stoma, and finally I operated and the transverse colon was the size of Switzerland. And it was so thin and almost ready to perforate. So just keep that in mind. In this case, the patient's stoma was the problem. The biopsy was OK. It was a stenotic uh fascia, but the dysfunctionalized colon can misbehave, and there are patients occasionally who are not ready for their pull-through. But misbehave. They're not gaining weight, they're always distended, they behave like they have a low-grade enterocolitis, and for those patients on occasion, you need to go in and remove the colon and leave a Hartmann's, and keep them with their ileostomy until they grow and are very happy, and are ready for their pull-through. And just to complete the discussion, once this patient is doing well, Alejandra, and thank you for your contribution in the literature on this very important point. When would you do their pull-through for total colonic Hirschrung's disease? I think this patient is 1 year old. He's ready to perform the total, the, the pull-through. So we don't have to wait until the patient is continent for stools uh for urine as we classically thought. So we have good results during the straight pull-throughs in babies around 6 to 8 months old. So I think this patient is ready. And I also want to comment on this um uh topic about enterocolitis in the remaining colon. So in patients with Hiss, total colonic Hispern's disease, I always tell the families to start to do irrigations once a week or once every 2 weeks even with the ileostomy in place because we can avoid and Enterocolitis and we can train family to do irrigation because most likely in the post-op period they will need to do irrigation. So, um, I would recommend to start doing irrigations even with the ileostomy in place for patients with total colonic histone disease. Yes, I, I agree and I agree very much, and Alejandra, I'm very thankful for that article that you recently wrote. That, uh, the patient, if they're growing well and the, and the ileostomy fluid is thick, they can have surgery somewhere usually between 6 and 18 months. I used to wait. I think that was a mistake. I think it should be done earlier. And then what surgery is up to you. My personal preference is an ileoanal, and many people would do an ileoduhamel, which is fine, but just make sure it's a very short pouch. Because the Duhamel can cause too much stasis and give, give some trouble. All right. In the interest of time, we are at exactly 11 a.m. in Washington DC and who knows where it is in the rest of the world, um, but I think it's uh 7 p.m. in most of Europe. Uh, 5 p.m. 5 p.m. Sorry. Uh, listen, I really hope we can all be together at YOSA. We need each other. We are owed a bunch of handshakes and hugs, etc. from all of our good friends. We have a special session on September 1st, Wednesday, a pre-congress in Colorectal. And it's a beautiful program, and I hope everyone makes the effort to come. Um, and Gaya, do you have any final thoughts? And I also want to recognize, and we can't thank Gaya enough for her incredible organization of YOSA. She is the glue that keeps this very complicated puzzle. Uh, together, and it's basically like a puzzle, and you open the box and all the pieces fall on the floor, and then Gaia comes and puts all the pieces together, uh, and now it's, and now it's a beautiful puzzle. So Gaia, any final thoughts, uh, welcoming everyone to Athens? Uh, no, uh, thank you very much for your nice words. Uh, we just suggest you to book in advance your flights and your Hotel. So hotel reservations are still possible without any cancellation fee. So book your hotels and book your flight with a chance of canceling just in case, but we really hope to meet you all in Athens. We will be there and wait for you. All right, Martin, you wanna, uh, close the, close the session? Yeah. Also, on behalf of, of Augusto and the panel, that was great fun and thanks for joining. And we just realized we have to extend our, um, Zoom subscription because we were limited to 100, um, participants. So next time we, we have to be bigger, can go bigger, and we will make sure we can have more people join. But those who could not make it today, can watch on YouTube from next week on. And thank you again Mark and Gaya. Thank you all. My pleasure. Thank you all. See you soon. Bye bye. Ciao. Thank you.