I would like to introduce our radiologist that will be working with us today. We have Dr. Steven Kraus that is going to be sitting very shortly. In my left, and we, and he works at Cincinnati Children's with us. It's very, we are very fortunate to have him with us and also Doctor Rodrigo Ocelami that works in Florianopolis, Brazil. They both have a lot of experience in Hieprung's disease, so I'm gonna pass it to him so we can start the radiology session and again we're gonna have both, so be ready to vote as we go along. Thank you, uh, Dr. Bishop. So I want to thank you also very much for inviting me to be a participant in this Hirsch Bruns event. Um, we're gonna talk about radiology, uh, a little bit and like some of the other issues, there is some controversy, uh, and, uh, that'll be great to talk about it. Uh, Certainly patients who get, uh, enemas, we're going to talk about the neonates, a little bit about the older children, but the neonates in particular. Um you really have to have a really good technique and I just want to say that, you know, years ago in the 70s, 60s, and 70s when radiography and fluoroscopy were pretty much the only modalities that were used, fluoroscopy was done by everybody and it was done very, very, very well. Nowadays, with the advent of MRI and CT and ultrasound. I think there's been a shift to the more extreme and more current modalities, and fluoroscopy is almost a lost art. So in the context of that, let's go ahead and take a look at some things. We're going to use questions certainly to make some key points about Hirschsprung's disease, and let's go ahead and take a look at some of those. So what are the signs of Hirschsprung disease to be seen in plain abdominal radiographs of a newborn, transition zone, distal bowel obstruction. dilated colon. Bowel mucosal irregularities or 23, and 4. So I'll go ahead and let you take a Take a second to Make some choices It's like the majority, it's, it's rapidly changing. We're having a mix of options of all of the options except for 4. It's the only one, but 5 is the most common answer. I guess we'll go ahead and take a look. Let's take a look and see what people thought. So it looks like about 50% of people are saying a combination of these different, different possibilities of distal bowel obstruction, a dilated colon, and bowel mucosal irregularities, and that is true. It's exactly true that there's multiple abnormalities that can be seen. Uh, and I will give a few examples of, of this, um, but just remember again, enemas in children who don't have the clinical signs may be a little bit ambiguous. And so certainly patients who are born with abdominal distension and vomiting or not pooping in the first couple of days of life are the ones that we're really going to benefit from doing these enemas on. So let's take a look. So here's an example of a patient who's a newborn about 24 to 48 hours of age, and the most significant. The sign that you can see is multiple dilated loops of, of, of bowel. Now I want to point out to you that in a newborn, you can't tell really the difference between the colon and the small bowel. So basically all you can say is that there's a patient who has multiple dilated loops and it looks like a distal bowel obstruction. Sometimes you'll see some bubbles of meconium. These bubbles aren't necessarily pneumatosis. We can potentially see pneumatosis in patients, but this is a fairly common appearance for meconium. Uh, that is, uh, distributed throughout, uh, some of the bowel, maybe in colon or maybe in small bowel. And, and also make sure that you understand that the, that there's a different, differential diagnosis for this appearance. It could be a number of different causes of bowel obstruction. The most common ones being, as you see here, Hirschsprung disease is fairly common, and these are, these are separated in the different entities that. Occur in the colon first like Hirschsprung's disease, the small left colon syndrome, which is also called laconian plug syndrome, sometimes called immature colon, and then obviously if a patient on clinical exam has no perineal opening. Then an anorectum malformation would look like this as well as a distal obstruction, but that's a clinical, uh, a clinical, uh, diagnosis. Um, also, remember in the distal part of the small bowel, you can also get appearances that look like this, such as meconium ileus and ilealatregia, and these five things make up about 99% of almost all the bowel obstruction you may see. Um, this is another example of a patient who has distal obstruction. Again, it's just multiple loops of dilated bowel, um, no pneumatosis, and you really can't come up, can't come up with the exact diagnosis, but certainly, Hirsch Crohn's disease should be in your differential. Here's another example. In this example, however, you can see that there are some thickened loops here. Uh, in the left abdomen, some thickening of the, uh, of the bowel wall. You can also see, uh, with these striations mean that there may be some spasm as well. So these are some signs that could lead you to think that there's Hirstprung's disease and you also have to think with bowel thickening colitis, and colitis you can see as an enterocolitis in patients with Hirschsprung's disease. So it's a good example of a plain radiograph that may lead you down that path. Seeing air in the rectum does not tell me that it's not Hirschsprung's disease, but you may or may not see air in the rectum. How about another question? So the radiological diagnosis of enterocolitis is possible with a plain abdominal radiograph, and is that true or is that false? Mixed a split on this, right? I love that. It's actually very helpful, especially in these types of. Where you have a lot of people with different opinions. And it looks like it's pretty split. Although it looks like there is a little bit of a majority. And So I don't have the poll on here, but it looks like it's about 3-quarters say that it is true that you can make the diagnosis of enterocolitis on a radiograph. And I, I think that is true. There are some signs that you can see, especially in the newborn and things, here's an example of a patient who came with, with the diagnosis of distal bowel obstruction clinically. And on the left, you could see some bowel dilation, multiple loops. This is probably either the blood, maybe the bladder up here really dilated bladder coming out the pelvis. Um, uh, but on the left here, you can see multiple dilated loops of bowel. If you get a cross cable or a decubitus view, you can see air fluid levels. And if you see those air fluid levels in the colon, that's another sign that there is probably an inflammatory or some type of process going on in the colon, potentially that would be enterocolitis. Uh, we do that for a little bit, obviously we look for free air, uh, in patients who are newborns, but it's also a good sign and looking for bob often. And here's an older patient, as you know, because you see the femurs, so they're all, she's an older patient, a child, and you can see with the arrows denoting the thickening of the colon in the transverse colon, in the descending colon here on the left, you do see air in the colon, but you see this thickening, especially of the gastrocolic ligament that is. Good sign of thickening of the bowel and colitis in a patient, and I think this patient was, uh, it could have been a patient who has Hirschprug's disease and has had already a pull through, uh, those patients get enterocolitis as well, um, but this also could be a patient who was a delayed diagnosis of Hirschrus. So it's always a potential, uh, sign that you may see, uh, in an alteration in a radiograph. Now if you see an animal like this, In a patient, this is an example of a patient we saw just a couple of years ago. Um, my, uh, impression is, even though that the rectum looks a little bit bigger than the remainder of the colon, enterocolitis in a newborn, in my mind is Hirstprung's disease until proven otherwise. And we saw this enema and the colon looks a little small, um, but, um. In fact, this patient had total intestinal Hirschprung's disease, very, very rare. I've never seen a case, only one case that we have, and Doctor Pena and I actually looked at this together after we did this exam, and I had never seen a case of total intestinal Hirschsprung. Of course we didn't know that at the time of the enema, but I said I am almost positive that this patient is probably gonna have Hirsch. Never really never have had uh uh food to suggest that maybe it's uh allergic colitis because sometimes allergic colitis can look uh uh very bad. Um, I've never seen inflammatory bowel disease like this in a, in a neonate in an infant like this, and it turned out that he did have disease. But when you see that you have to take that. Uh, here's another patient who has, uh, uh, Hirschsprung's disease and has, uh, irregularities of the left colon. Uh, I'm not sure. I, I, I believe this may have been a patient who's already treated, uh, but nevertheless, as you can see, the rectum is, is a good size compared to the rest of the colon, uh, but you see irregularities and, and spasm, uh, then enterocolitis certainly should be in your differential diagnosis of enema. Uh, and here's another patient who had respiratory disease, very irregular, very irregular colon. OK. So you have a patient and the contrast enema, uh, uh, in a newborn baby allows, uh, always allows to make the diagnosis or to rule out Hirschprung's disease. This is a really actually a good question to ask. Um, how good is the enema? And so, is it true that the contrast enema in a newborn baby always allows to make the diagnosis or to rule out Hirschsprung disease, or is it false? And we have the majority of the people saying it's false, 96%. I think pretty good. That's pretty convincing evidence. So that is false. It, it doesn't always allow the diagnosis. Now, actually. Let's talk a little bit about the accuracy of the transition zone by enema. The false negative rate, if you look at a bunch of studies, is between 20% and 25%. That's a lot. That's a pretty high false negative rate. And I'm not exactly sure why that is. I think in the, in the hands of experienced fluoroscopists, it may not be that high, but we're taking all comers. We're taking all people that do enemas. And so I think that There, there are several reasons for that, and that's a good reason why today we're going to talk about the technique, because I think the technique may have something to do with it, as well as the interpretation. So, uh, that way we can talk about that. And actually I, I have to admit that total colonic Hirschsprung disease is a very difficult diagnosis to make. Which is probably comes into play a little bit for that, and also the diagnosis of short segment disease. And again, part of it I think is technique and how you do the enema. So we'll talk about that later. But the false positive transition zone in up to 43% is also a very interesting concept. And let's talk about that. There was a study that was done that looked at the accuracy of transition zones, and this was done probably in the early 2000s. Um, and they looked at the radiologist, uh, radiologist agreement for the transition zone, and actually, that's fairly high, 90%. That's pretty good that there's a good, you know, when different radiologists, uh, look at the studies, they agree on the transition. And the concordance rate for the radiology and pathology, however, was not very good, uh, almost a coin toss, a little bit over a coin toss. Um, but it turns out that the short segment disease. And. Can you hear us well? If you can just type in the chat saying that you have video, you can see the presentation and you can hear us, then we're gonna proceed with our talk. And I hear one yes, so I'll take a yes for everybody. Thank you, Dr. Crouch. So why don't we go back maybe the last slide and Just reexplain that. Yes, so we were talking a little bit about the contrast enema and the accuracy of the transition zone and talking about how good is the enema. Well, the enema has a high false negative rate. It's about 20 to 25% by a lot of the literature, and there are multiple reasons for that. Part of it may be the technique. Part of it may be interpretation. Part of it may be that it's actually a very difficult enema to interpret. But the false positive transition zone is actually pretty high, and so we'll talk about reasons for that as well. And because of that, there was some literature in the early 2000s about the accuracy of the transition zone. And actually the radiologists' agreement to decide where the transition zone is was fairly good. So interpretive wise, uh, the radiologists are pretty consistent. But if you look at the concordance rate between the radiology and the pathology, and that means that with the, with the radiologist's decision of where the transition zone is and the pathologist's decision about where the transition zone is, their agreement was only at about 62%. And that's not very good, but if you look deeper into it and you look at the disease that results in that number, it turns out that short segment disease, disease that's rectosigmoid or a very low transition zone near the rectum, actually was fairly good. The concordance between the fact that where the radiologists said the transition zone was and the pathologists say the transition zone was, was, it was, it was a concordant in about 75% of the cases. However, if you look at the long segment disease, meaning that somewhere in the, uh, in the descending colon of the splenic flexure or even more proximately, uh, the concordance was only about 25%. What does that mean? Well, it means that if you do an enema and you find a transition zone, which is very high, then doing number one, the actual pathologic transition could be anywhere, and I'll show examples of that. It also means that if you want to do a repeat enema and you want to get a better look at where the transition zone is, you really can't reliably say that that second enema is going to give you a better indication of where it is. So repeat enemas in kids with long segment disease, in my experience, is. Futile. It's not going to give you a better, a better knowledge that it's either more proximal or more distal. And therefore, in a patient with longer segment disease, it's probably better for you to plan your operation thinking that it may be high and not do the, do the correct operation that you think based on the first enema. So let's actually talk a little bit about the technique. So I'll invite Doctor Rodrigo Ocelami from Brazil now, and I'm gonna pass your slides, Rodrigo. So just tell me one when you want the other slide. So welcome, and you can start speaking now. Rodrigo, can you hear us? Rodrigo. We can see you, but we are not hearing you. So can you see me? Yes, now we can hear you. Now we have both. Go ahead, OK. Can you, yeah, and mute your computer speakers. OK, so thank you very much for the invitation. And uh it's a great honor and pleasure being here with you. Uh, I just wanted to say that, uh, back in 2002, I was in Cincinnati for three months in the radiology department with Doctor Kraus. So it's actually a great honor for myself to be here and side by side with him. Uh, and yet the technique for contrast enema is actually quite simple, but yet there are some secrets that you should keep in mind, otherwise you should actually lose a list of diagnosis. Rodrigo, Rodrigo, sorry to interrupt you. Can you please mute your computer speakers? Yeah. Did you get that? Yes, thank you. OK, so the technique is quite simple actually, but uh there are some tips that that are very important, so otherwise you're not going to miss the the diagnosis. So basically what we do back here, we just insert a small part of the cat, the rectal tube, like 2 to 3 centimeters in the rectum. And we never used a Foley catheter inside the rectum, and it's surprisingly for me that sometimes they send images for us to take a look, and yet with the Foley within the rectum, so you never use it, you just hold tight. The buttocks or if the child is actually bigger, we just ask for the child to hold the contrast. So if it's in the neonatal period, basically we use the water soluble contrast and we dilate it, dilute it a little bit as 50% of contrast and 50% of saline. And very gently and very slowly we fill the entire colon with a syringe. We then use it with gravity. We actually inject it via a syringe, but again you have to do it very slowly and very gently because otherwise you can miss or distend a ganglionic segment of the colon. Can you give me the next slide, Andrea, please? The big difference for us here is that after the neonatal period, actually we don't feel the entire colon, we just feel up to the transverse colon, and if we feel that the segment that has been studied is normal in distension and the caliber, the mucosa is normal and especially the splenic flexure is normal, not given any suggestion about the total lionic. We just go through the transverse colon. Um, so basically we started on the left lateral and with the contrast inside the colon we take two images in the left lateral, two images in the right lateral decuiss, and two images in the AP position and. Usually take out the tube so you can actually take a good look in the rectum without the tube, and if you find any signs that actually suggest that you do have a positive study, of course we take additional images for best documentation. Can you give me another one, Andrea, please? And of course we've been talking this this morning. What we wanted to find, what we wanted to to see is the transition zone, and as far as soon as you you find it, uh, we actually stopped the the contrast because the diagnosis is made. It's very helpful in our point of view. The inversion of the rectal sigmoid index. We somebody just posted a question just months ago, and we do look for the sign. We we really value the sign. Of course the affected or the ganglionic segment will be spastic, and that's why you should not give contrast with a lot of amount or too too fast because you're going to miss the spasticity of the affected segment and the irregularity of the bowel as Dr. Kraus showed before with or. the enterocolitis is a good sign as well. So I would say that the technique, it's no big deal. I mean, you just go with the contrast, use the contrast that you actually feel better, and then feel the colon as much as you believe and then look for the signs. Can you give me the next man? It's very important for me is to talk to the family after that using barium or using the water soluble. I always ask for good hydration after the study. It helps avoiding, you know, um. Miss hydration or it actually helps for the child that you try to evacuate after the study, but I always show them the contrast that I've been using because I've had a couple of times in the past people calling me after the study saying that white stuff is coming out from the child and I always show the contrast after the study to be sure that they know. What we have been injected in the colon, so whenever the child put it out they know what's going on. So I think that the lead point, the main point in our technique is to never use Foley inside the rectum. It's to inject the contrast very slowly, so gently, and using fluoro all the time. And and then just look for the signs and talk to the family prior and after the study explaining all the things that we're going to do and uh and the kind of contrast that you did again thank you very much. I think that's my last slide if I'm not mistaken. That's correct, yeah, and then again just say it's a pleasure being with you and with Doctor Kra at this moment. Thank you very much, Doctor Osalani and Doctor Kraus will continue with his technique now. Thank you very much. Uh, you know, uh, again, excellent technique, uh, Dr. Ozalami. Um, I'll just going to describe a little bit about our technique. Our techniques are very similar. Um, we use an iodinated contrast. It's a water soluble contrast. It is a little bit hyper osmotic, and a lot of the radiologists in particular aren't really aware of all the osmoalities of all the different contrasts, but the one we use is about 400. So it's all, it's very similar to these agents that you use to clean the colon out. So not only does it help to make the diagnosis, but it also will attempt to clean the colon. And in the neonates, it's important to know that because if the contrast does stay there, uh, the little babies. So little neonates can get dehydrated and then they could have run into trouble. And so we make sure that the, the floor, the neonatal ICU knows that. In any event, we use a rectal, a rectal tube in the neonates, um, we tend, and I'll show pictures of them, uh, we use gravity infusion rather than injection. I'm not sure if it really matters a whole lot, but we use a gravity in gravity drip. Uh, not from a bottle, but from a bag that has very large IV, very large tubing, and actually we get actually infuse the fluid at a rate that's not really slow. It's actually a moderate pace to actually show the distal part and the proximal part quickly so that you can actually see a rapid, a rapid picture of the of the transition zone. I feel like that's, you know, fairly important. We do the Lateral rectosigmoid image in the lateral view, as does my, uh, my colleague in Chicago. Early maximal distention is actually the best to see the transition zone because if you wait a long time, you can actually get distention of the distal part, which is, uh, which is the, the, the, uh, the Hirsch involving the Hirschsprung's disease, which is the distal part. And that's because it's not a lead pipe. It is a soft piece of tissue, and you can, uh, distend it. We get an AP image of the rectosigmoid, uh, and again, if the colon and the neonate looks small, we actually fill the entire colon and attempt to reflux into the terminal ileum to make other. Diagnosis, uh, if they are there. Here's the patient in the left side down position, as you can see there. There are the tubes. In a neonate. I tend to use a Foley catheter in a full-term infant, about 12 to 14 French, and in a premature infant, if it's are a little bit premature, a little bit less, um. Here's a normal contrast enema for your, for an example, you can see the nice, well distended rectum. The presacral space is well seen. And why is it well seen? Because the femurs are on top of each other. It's a true lateral image, and you really would like to attempt to get that. Then we get a frontal view, as you can see here, all the way down, making sure that you do see the tube, but you also see a little distal to it. You don't want to cut off the rectum to miss a very distal transition. transition zone, a very distal Hirschbrons, but notice that the proximal colon is much always a little bit smaller here in the toward the splen of Fletcher than the rectum, and that's a normal appearance. Here's a Hirschsprung disease patient, or at least an enema that reflects Hirschsprung's disease. Note that you can see a very short segment. And if you have a Foley catheter that's blown up in here and you put the Foley here to prevent leakage, you'll miss this every single time. And that's how sometimes the diagnosis of Hirschsprung disease is not made. They consider this rest of the colon a normal size, and it's negative. So you have to make sure you use some kind of a tube that doesn't have a balloon on it. Or at least when you have the balloon in, push the balloon in a little further so it doesn't block the end of the colon. Here's the left side. On the right side here, here's a rectosigmoid transition, the typical rectosigmoid Hirschprung's disease. These are usually concordant pathologically and radiologically. Now here's a patient who has long segment disease. Note that the rectum is very small. Here's the Foley catheter blown outside and held up against the recti anus to prevent the leakage. And you can see that there is actually a very, very small, irregular, spastic type of colon to the level of the splenic flexure. And here's total colonic Hirschsprung's disease. The rectum does look pretty small. It's not bigger than the rest of the colon like it should be. And so this, when you see a colon that's one smallish size all the way through, you have to think about total colonic disease. But you could say, well, if this is a little bit of a premature infant, it's just immature, it could be. And actually the enema in a in a in a premature infant does not follow the rules. It could be immature and look small, so you really can't tell the difference. What age would you go down to to do or size would you go down to to do a contrast enema? Well, we can do contrast enemas in even young premature infants. That would be worthwhile, that would actually give you a reasonable result. Um, I would probably say anything greater than 35 to 36 weeks. If you go back further and you get into patients with necrotizing enterocolitis and that when that becomes much more, uh, uh, uh, uh, uh, prevalent, then I think you're not gonna have the same, uh, diagnostic, uh, accuracy. I'm looking at this contrast, Emma, where's the transition zone? Is it proximal 1 or distal 2? So you're going to get a poll to vote. That's. Rectosigmoid, descending column, transverse column, ascending column. Everybody can vote. And we have more now we have a now we changed. So actually while we're putting the pole up, could you go back and show that film again? OK, so here's the enema. It's in a right lateral position, it's not left lateral, but here's the enema and So we have 90% saying rectosigmoid. Well, that's good because that's where it is. That's where the radiographic one is, right? So here you can see that there is a transition at the rectosigmoid. The rectum and sigmoid transition is about S2 or so. So if it's a distal in the rectum, it's going to be distal to S1, S2. If it's more proximal than that, then that's the typical rectosigmoid transition. Um, now, here's a 2 year old male, I'm sorry, a 2 day old male with failure to pass meconium, and here's an enema. So There's the lateral view. And there's the frontal view. So what do we call this one? Now remember too that there are other diagnoses, and one of the other diagnoses in a patient of this age is small left colon. Usually their transition is at the splenic flexure, and usually it is very abrupt, like this case. So what does everybody think? Actually we don't have the the poll for that. So actually, why don't we say, do we think this is, I can make one right now. So what do we do we think this is small left colon or meconian plug syndrome, or do we think this is Hirschprung's disease at the splenic flexure, which is a long segment. So maybe long segment versus small left colon. OK, because this is instructive. And it actually it look, it looks at the principle of is the transition zone accurate in long segment disease. 1, small left colon. 2, meconium plug. Oh, OK. And then long segment disease, is that OK? I can change it. That's good. OK. And we have people voting in all options. Good. Looks like the majority is seeing small colon. Yes, and, and that's, that's what I thought, right? Right. That's the, that's the radiologic, um, dogma is a picture like this. This is a slam dunk, OK. And if you look at the lateral view. The rectum is or isn't bigger than the sigmoid. This is a good point about the rectosigmoid index. Is the rectosigmoid index always good? And I say no. I mean, it's, it's a good principle. If you have a bigger rectum than sigmoid, it's probably normal, but don't stop there. Go all the way up to the splenic flexure. And in this particular case, this patient. Has what has a small rectum on the scout, a small colon to the splenic flexure, and meconium plugs. There's meconium plugs in there, so this is small left colon. Well, is it? No, this was Hirschsprung disease. In fact, can you tell me where the histologic transition should be? So based on that picture that we just saw. This picture, where do you think the transition is? Is it here or is it somewhere else? So a lot of people are saying the transition is at the splenic fletcher. OK. And actually a lot of, well, actually, About half the people are saying proximal to this planet Fletcher. Well, actually, that's pretty good. OK, so here's the picture. The answer was that this was total. Colonic a ganglionosis and in fact the transition was in the terminal ileum. So I just want to bring home the point that if you have a proximal transition zone, you really can't accurately say where that transition is. And I'm not sure how they booked this case, if they did it transantally or if they did it an open procedure or laparoscopically, but I do know that it was a transition zone that was much more proximal, and I think that would make you. Uh, should make you think about doing something that's more invasive rather than just the transianal approach because you really might not know where it is. And actually, as, uh, uh, Dr. Collins was saying, is doing, uh, biopsies and going through the colon, uh, maybe even, you know, the laparoscopic approach would have been good probably for this case as well, right, uh, Todd, right. But can I ask the panels, if you get a, and we get this every once in a while, you get a contrast sentiment and it looks like small left colon. Is anyone going to not perform a rectal biopsy? I always do. So other than the game of am I right at getting question, can I make it even more broad? I would do it. I do it in almost any distal, any patient that needed a contrast enema. To rule out a distal obstruction, I'm going to be doing a suction rectal biopsy, whether it's a meconium plug or a small colon. Or whatever it is, I'm going to, I'm going to be doing a suction rectal biopy. What about Maconium milius, even Maconium milius. Yep, definitely. Would you not? I see a blank stare. I think if it's clearly a case of meconium ileus and you reflux into the terminal ileum and you're able to get a response and the patient clinically improves, yeah, I'm not going to do it. I haven't done a rectal biopsy. Dr. Pena, any thoughts? Perhaps I would not do it if I'm sure that it is meconium. I would not. But what about a picture of a small left colon? Would you always do a, I would do a biopsy. I would do it because I, I, I don't know how to distinguish this from. So I would do. OK, let's just talk a little bit about this. What does this look like? Uh, did you want me to preface this in some way? No, just look at the contrast enema and you're going to be asked a question about it. This is a patient that had already received an operation. So look carefully at this contrast enema. So now we're going to pose a question. You can leave that slide up, OK, because the question will show the question so they can look at both at the same time. Awesome. So the question is, the most likely contrast, uh, most likely this contrast enema belongs to a patient who underwent an operation for Hiprung. Following the technique of Soavi #1 Swenson #2 Duhamel 34 I don't know thanks for your grid eyes no, no, I have a computer here I'm not looking from far distance. So we'll just let you just take that excuse me by the polls. People do pretty well. So currently more than 75%, 80% of the people answered SOA. And the answer is. Suave. Very good. And if you look at the picture, what tells you this is, uh, is that look at the distal part. You see, well, not only do you see, not only do you see that there's narrowing and there's a transition. I don't know why it keeps doing that. So not only do you see a transition and narrowing, but you also see very wide presacral space in the region of the rectum and As in this technique, there is only a partial thickness dissection and there's leaving of a cuff of tissue. And that cuff of tissue, uh, if it is prominent, causes you to have a very wide presacral space. Of course that cuff goes all the way around, but I think it's best to see on the lateral view. It's only seen on the lateral view because you can see the presacral space and you can see it's widened. So that's why it's very important in post, uh, in post-surgical patients to get that really, really good true lateral view of, of, of the rectum. Here's another patient with another. Uh, contrast enema contrast enema, who had already a surgical procedure. So what do you think this one is? So we can have the foe again. Is this a Suave, a Swenson, a Duhamel, or I don't know. Everybody can vote and we'll see your answer shortly. And I think we lost the poll, but it's back. I don't know, but I saw that the majority of people were saying Duhamel, so here we can actually see the Duhamel pouch. Exactly. And so when you, when you look for an enema for a Duomel pouch, remember the Duomel is sort of a chimera. It's a chimera of part of the, uh the a ganglionic segment, uh, distally with more proximately the the ganglionic segment, and it's not put side to side or end to end. It's sort of a patchwork of it. Um, and so what you see is you see an extra piece of pouch anteriorly, usually, which contains in a patient who's symptomatic, a whole bunch of stool, and it's the enlargement of this pouch and it's, it's impression on the The ganglionic bowel that actually causes the obstruction that the patients are experiencing, and on the enemas you try to to actually get rid of that, that, that stool and to make the diagnosis by seeing the pouch. Can I make a comment? And just for the audience, this is a lot of people are listening to this and perhaps we'll gain something. I have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended. Even if you go 10 years, 15 years, and yet in these patients we see the rectum, the rectum was opposed to this angryonic, very stretched with fecally impacted. So I wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk disease, and that's why I. Invite everybody in the audience, when you see a patient like this and you perform a resection of that pouch, orient the the the pouch and alert your pathologist to be to do a study and try to determine whether or not there are ganglion cells in that rectum, because I suspect that this patient never had Hodgkin disease. Doctor Collins, did you hear me? I do. And I, uh, I have not by any means seen all of the pouches that have been resected, um, here, but the ones that I've seen have had ganglionic and a ganglionic parts to them, but I don't know what their contrast, um, looks like. So here's another patient, a little bit different than the other ones. It's a little bit older patient. This is a young, a young child, and There's a lateral view which shows a very nice presacral space, which is normal and a normal looking rectum, but the whole colon is enlarged. It is redundant and it is filled with a bunch of stool, especially proximately, not so much as distally, but proximately. Um, and so let's, what do you think about this? With this image, what is your diagnosis? Is it short segment Hirschsprung disease, ultrash Hirschprung disease, long segment Hirschprung disease, idiopathic constipation, or alagia of the internal sphincter? Everybody can vote. It's like the majority are saying idiopathic constipation. So in this same patient, the surgeon did a biopsy and found a ganglionosis. What is the next step? Option 1, colostomy. Option 2, colostomy, followed by resection and put-through. Option 3, rectal myectomy. Option 4, primary put-through. Option 5, medical management. So everybody can vote. Option 6. And I think it's everywhere. I think there are 12% saying medical management, primary go through 5%, 31% saying rectal myectomy, uh, 35% colostomy, and about 99% colostomy, so. You go back to Dr. Collins has the pathologist has another operation she would like to perform. You can re-biopsy. So Dr. Collins is saying a rebiopsy. OK, well, let me say, if I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view. And, and I'm, if I take a biopsy, I will run into the risk of having. An angryonic result which for me doesn't mean anything because this patient has no Hirshman disease and uh and, and then the other diagnosis put the other slide and the short segment Hirp disease this patient doesn't have such thing. The ultra short. I don't know what is that because there is no way to differentiate the so-called ultra short with idiopathic constipation and the um and then there is a normal have another. And if you look at this particular diagram that I made years ago, supposedly what we have, what we heard through the years is that the rectum, the rectum has ganglion cells that you can see with the pluses here, normal ganglionic bowel, and then there is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being. There is mention in the literature about a normal bionic segment and normal above depicting a line, but we don't know how much is that in a preemie, in a full term baby, in a 6 month old baby, and there is no such an accurate study. That's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge. So conceivably somebody could take a biopsy in that area and come up with the result of no ganglion cells. It doesn't mean anything for us. And then concerning the other diagnosis such as alacia of the internal sphincter, to begin with, the internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personally, and I have opened these normal rectums in different, different ages. I have been looking at that. I don't have a microscope, but I have never seen that thickening. And if that exists, nobody ever determined the exact limit of that thickening at different ages, so it becomes in the kind of magic witchery type of diagnosis when we talk about alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept, and we went through that with the so-called lower esophageal sphincter and other sphincters that have been defined, but we had never seen that. So I don't pay attention to that and I don't perform myectomies and I don't recommend those myectomies and myotomies and buttock and those concepts because we don't know where where you inject. What area of the muscle, nobody knows how they do it, they are doing, but the fact is that it paralyzes whatever muscle is there and of course facilitates the passing of stool, but it's not curing a condition of unknown origin. So I think with that slide we're going to end our radiology session and we're going to take a 10 minute break. We're going to start sharply back in 10 minutes. So we'll see you soon and we're going to have our surgical session and case scenarios next. And I want to make one comment. We apologize for the problem with the server, but this recording will all be available in 48 hours in high definition. Very good quality, so you'll be able to look at it by chapter and look things up and it will be great audio, great high definition. So with that, we'll take a 10 minute break and come right back. Good, we'll see you soon. We have here.
