we need to know about as a pediatric surgeon and we have a specific session about that topic. Let's start with the management of the newborn. What I'm going to show you now is a series of pictures of brand new babies and we're going to become experts in the next 15 or 20 minutes at the examination of the newborn. And this sounds very basic but we all know that there have been many mismanagement decisions based on an incorrect newborn exam and I think we all have to become experts at this. It's really a key responsibility. So let's talk about that. Well first of all I just wanted to tell you this reference that I came across. This is a 2,000 year old reference to the management of a baby born with an anorectal malformations believe it or not. So what you're supposed to do is take the infant without an anus, put some oil on the perineum, put them in the Sun and then where it's transparent you take a barley grain and slash across the opening and then stool will come out. So I suppose that was the first anoplasty. Well I'm hoping we have evolved since then. Raj and this is not how we do it here right? All right. Brand new baby, no prenatal suspicion of an anorectal malformation, 40 weeks gestation. So Jeff what is this pictures you know like we're at the art museum what is the picture to speak to you? Is this working? So obviously this patient does not have an anal opening. This patient likely has some form of a rectal fistula to the urinary tract, either the urethra or the bladder neck. This patient's bottom is rather flat. He does not have a well-defined gluteal cleft and as a result has a rectal urethral rectal bladder neck fistula. Most likely. It's still early. So we're just born. Is anyone, can I, can we put up an audience response screen? See if that's working. So the baby's just popped out and is now in the in the NICU and it's today Wednesday morning. Is it working? I want to see if we can poll the audience. Oh polling is on. Okay so for those of you here's I'm going to pose the question in the form of an A or B. So A is we operate today B. We wait until tomorrow. Vicki you see the counter right? Is there a counter? So as soon as we get about halfway through the group we'll see the reply. That's the same. All right let's see. Who wants to operate today? Who wants to operate tomorrow? Okay. Does anyone want to speak about operating today? For those of you in the 16% group? The microphone. Anyone want to talk about the choice of operating today? Paola you want to talk about that thought process? Would you operate today? No. Well to operate on the same day you might lose the chance to see if there is some meconium arriving and closer to the perineum. In this case it's unlikely but you don't really need to rush the operation on the day of the birth. You can wait a few hours. But tomorrow I'm leaving for vacation and it's very inconvenient and I can't get OR time but the OR is offering me 2 p.m. today. Let's just do it. Why don't we just do a colostomy today? Have a nice holiday. Ivo? Have a nice holiday. Call a partner, right? Well I think the point that's being made is there's no rush. We shouldn't make a decision to do what we need to do on this patient today. As long as the baby's abdomen is soft and not distended we have an opportunity to do a number of things. Which we'll talk about in a little while. I will tell you though this particular picture was done to be a bit provocative. There is no way there's going to be a fistula in the perineum on this patient. So for those of you who say, who concluded that and Don is nodding his head, there's no perineal fistula happening here. There's most likely a rectal urethral fistula as Jeff has said. I still would probably wait. We have other things we need to do today like make sure there's no esophageal atresia, make sure we know what the kidneys look like, etc. But this is very likely going to be a colostomy and then a distal colostrogram down the road. Would anyone, let's do a poll the audience again. Would anyone do a newborn repair? That would be A. Or colostomy? That would be B. If this kid looks the same tomorrow. Newborn repair today or tomorrow rather, colostomy would be B. Okay. Can we, can we, can we can't see the, the polling, right? It's got to be there somewhere. I just, it's too small. All right, let's see what everyone thought. Anyone doing a newborn repair on this kid? Mark, while we're waiting, Marcella had a comment that she would manage this differently based on if it was a baby with Down syndrome. And I asked her why, and she says because most of them have no fistula. Okay, so if this was a patient with Down syndrome, she would do a colostomy. So Marcella, why don't you ask, answer that. Would you do, is it, I think it was referenced to, let me ask her what she's hitting at. But I think her point was, what is the difference here now in patients? Would it change your management if they had Down syndrome with a less chance of having a fistula? I mean, I think that makes a good point. I mean, I think that makes a good point. A Down syndrome babe with a perineum looking like this is not having, is there's no perineal fistula in this child's future. So I think a diversion probably makes a lot of sense. So I, I, I, that's a good comment. Does anyone know, by the way, of, of patients with Downs, what percentage actually have no fistula? It's, it's not all. It's certainly most. It's about 95%. So you can predict no fistula, but they certainly can have, uh, fistulas. Okay. Ivo de Blau. Thank you. New Netherlands. Newborn baby girl. Yes. Um, what I would do, I'd like to, what, what, what you see, you see, no, no, no, no, no, no, no. And I can't, you can't really tell, uh, uh, much more on this picture, uh, on the type of, uh, malformation, I guess. Uh, you can see the, the bottoms are a bit flat. Um, just as in the, in the other one, you don't see a discoloring of the, where the anal opening, uh, should be, or the, uh, muscle complexes, but you should, uh, take the labia and look inside and see it, whether there's a, uh, vestibular fistula or whether it's a cloaca. Yeah. I think, I think that's wise. Although it has some, it has some cloaca features, you don't really know for sure until you do a careful look at the perineum. So, um, newborn repair tomorrow or colostomy? A or B? I promise you it will get more difficult. Raj, are we making? Raj is really psyched because he's gotten three for three already. Mark, are we making the assumption it's a cloaca or are we making the assumption that's? Oh, yeah. So let's assume this is a cloaca. Thank you. Sorry for that. Let's assume this is a cloaca. Newborn repair tomorrow or colostomy? Who is courageous? That's the question. What? The question really is who is a real courageous surgeon. Who's courageous? No, I think it's who's crazy. Who's crazy. There's a fine line usually. Who wants to eat a mighty crocodile. And many people who are considered crazy are in retrospect considered courageous, right? Yeah. Oh, there's some primary repairers out there. Does anyone want to defend their position? By the way, I find the audience response system is very nice from an anonymity point of view. Usually no one wants to claim their role. And they're usually a bunch of rebels that always pick the opposite answer. But we know who you are. And by the way, the fellows in the room, just so you know, your paths are linked to your program directors. So they are following along today too. Okay. Sir Sutcliffe, representing the great country of the United Kingdom. This is... This baby's already in prone position. You've got to get used to looking at things in prone position. By the way, I tried to turn it around. It doesn't look right. So I've kept it in prone. So this is a brand new baby. What are your impressions? Okay, this is a brand new baby who's in a prone position with a bit of betadine or something on the back. The... There is appearance of an anal dimple with suggestion of muscle. And the scrotum doesn't look particularly biffid. So the issue here is whether or not one can be confident that there is no fistula leading to the urinary tract or not. And I'd like to say how old the baby is. Is this baby... Brand new baby, just popped out. Ignore the fact that we're prepping for surgery. We are... We are... We are... He has a well-veiled circumcision. It was such a good example. It was such a good example of the point that I wanted to make. Yeah, you circumcised the baby? Huh? He's already circumcised. Oh. Yeah, we actually do... It's a fetal intervention. Okay. Okay. It was such a good picture of something I wanted to emphasize, and that is the flatness of the bottom. Does everyone see that? So, I mean, this is a pretty minimal muscular pelvis. So, maybe Jack can say how you would guide this family when they ask you, I hear you do a beautiful analplasty, but will my child's anus work in three years? What would you tell this family? Well, I don't think you can ever be 100% sure, but the flat bottom babies usually have a high fistula, and they usually do not have normal continence. But I'd also want to look at the sacrum and see if it's normal, because that's another predictor of continence. Jack brings up a very good point, and we're going to have a whole section on that, but I agree. I think every anorectal malformation baby needs to be thought of in the context of what is the malformation, what is the quality of the sacrum, and what is the quality of the spine. And we've sort of come up with this idea that we could call that the ARM index. And if you have all three of those factors in mind, I predict, as we run our data, we will be able to predict continence. So for example, a flat bottom, a poor sacrum, and a tethered cord in the newborn period, one could say, Bowel management with enemas in three or four years, don't try to potty train this child, you likely will not succeed. As opposed to perineal fistula, good quality sacrum, good quality spine is a patient that you can be a little bit more confident telling that family, they will have continence. And I think every single ARM patient needs to be always thought about in that way. I can tell you when I review submitted articles about ARM and continence, almost uniformly, the sacrum is not mentioned, the spine is not mentioned. And they're just glommed together as a group of ARM patients. And we all know that that is not true. Bladder neck fistula patient is very different than a perineal fistula patient. And we'll get into some of that in a little while. Okay. Newborn baby. This is rare footage. I actually believe I have a video of this. Let me see if I can find it. Where is that video? Anyone see it? Anyone see it? Yeah. Mark, this is a lot of different. I'm just saying, oh, I can't see it. Oh, yeah. This is unbelievable. Watch this. Never seen before. You can turn the sound down. Watch this. This is unbelievable. I mean, has anyone ever seen a video of that? I bet not. So that is a patient with a recto urethral fistula. Could you even say recto prostatic? Say it again? Could you even say erectoprostatic? Say it again. If you look at it closely, it's a very vivid scrotum, I think. Did I see it right? No, I don't. Would you suggest it's covered? It's not vivid. The scrotum is covered by meconium. So this is a patient who we know has a rectourethral fistula. Everyone can obviously see that. Okay. Jeff. Newborn baby. So again, just looking at this child, we can see a beta meconium at the perineum. This patient also, again, maybe it's just the way he's positioned with the legs pulled back, but kind of, again, appears to have a rather flat bottom, but again, it's hard to tell. Not that flat. I'd say. Not that flat. It's hard to say. But definitely, I think, here the finding of interest in the beta meconium. What about this is not normal anus? It's eccentric, so it's often a side. It seems to be. So the hole is not in the center of the sphincter? Do you agree with that? It almost seems like there's a scarring at the base of the penis. Yeah. Where is the sphincter? Here? Here? Or here? There. By the way, you guys can look forward if you want. No, I can't see your point. Oh, sorry. It doesn't translate. Good point. We didn't work out that technology, did we? Is the center of the sphincter here, here, or here? Maybe. So you have to look at this and see that there is a pinkish ellipse, right? And that hole needs to be in the center of it, and if it's not, it's not in the right place. But what is your opinion about whether someone described this as an anterior ectopic anus? Ivo, how does that make you feel? In the male? Well, in the male, but there's a lot of discussion on what's an anterior ectopic anus. But if you really want to define it, I think you should define it as being not in a normal position, but has to be circumferential, enclosed by the muscle complex. But this one is certainly outside the muscle complex. But if you have in a female and maybe in a male, it's hardly ever described, because most of them, then you would call it the normal anus. We have some of those. So this is a hole that's too small that's not in the right place. Does everyone agree? Yeah. So there is no doubt that this patient needs some improvement surgically on that opening. So let's poll the audience. It's now, this baby was born today, and we've booked OR for tomorrow. And by the way, the trick that I use is I book the OR for tomorrow, and I say, anoplasty possible colostomy. Find OR time. Tomorrow comes. It's an obvious perineal fistula, and we call the OR. It's going to be an anoplasty. Or it's a rectory urethral fistula. We call the OR and say, actually, we're just going to do a colostomy. But at least we've held the time. So choice A, anoplasty. Choice B, colostomy. Tomorrow. Baby just born today. Okay. Okay. Very good. So Jonathan? I have a question, Mark. Would you say that this child's perineum looks otherwise normal? I don't want to use the word tightly, but almost dysplastic. It looks a little funny at the bottom of the scrotum. I agree. I'm not exactly sure why that is. No scarring. Tell me about your choice. What would you do tomorrow? Well, I was going to ask you something similar. My thoughts are that if there's a degree of anatomical abnormality, it would probably make me tend towards doing a colostomy and doing things a little bit down the line. I probably want to get an ultrasound of the perineum just to make sure. Well, it looks like there's possibly a bit of fullness just on the right-hand side. You might be able to feel whether or not there's a lipoma. It wouldn't necessarily alter things massively, but I'd want to know that before doing a neonatal repair. And that business around the bottom of the scrotum, it does look a little bit abnormal. I just want to know that the renal tract, particularly know that the renal tract was okay and there's no bladder outflow obstruction. Okay. Please. Can you do me a favor, though, and speak in a microphone so that the folks outside can hear you? Sorry to make you get up. Sebastian King has traveled all the way from Australia via Toronto, and he can at least make the 10 feet to the microphone. That's right. What about option C of dilatation and weight, and then do a primary repair? Very good. All right. Let's change the polling. Let's assume this is a patient with everything is fine except for a perineal fistula. Ignore that the scrotum looks a little funny. And perineal fistula, healthy baby. Choices are A, anoplasty tomorrow. B, dilation and anoplasty scheduled electively in the next six months. So this will breed some, this will be a 50-50 split is my prediction. Actually, I predict 30-70. 30-70. Then we can talk about the advantages and disadvantages of both approaches. All right. So we're pretty split. So a lot of people would do an anoplasty tomorrow, and some people will do a dilation and a delayed repair. Jack, you want to give us your thoughts on that choice? Again, this is a straightforward perineal fistula. No other issues. Yeah. Yeah. The first question for me is whether the child is pooping fine through it the way it is. Some perineal fistulas, they actually can poop just fine as a newborn. You don't even need to dilate them. And I don't dilate those. And I wait until they're three to six months. Calibrate. Well, yeah, I would calibrate it right at the beginning, but I wouldn't do that on a regular basis if they're stooling. Now, this one, it's unlikely that he's going to stool normally through this. So what I was taught and what I've generally practiced is to dilate them for three to six months. I just think that you can do a better job with your anoplasty if they're somewhat bigger. It can also be marked that if you are not confident, if you are in a place where you're not confident to do an anoplasty the next day or on the next days, you can still, like, calibrate, not really dilate, because then you ruin the tissue and send the baby somewhere where they are confident to do it as a primary repair. I think that those are the key points. As Jack said, you know, you want to make sure they're stooling properly. These are the patients that I've seen a couple that have actually perforated in the sigmoid because they didn't get the stool out properly. So someone was sort of managing them and waiting. So you have to be very careful, particularly if this is a preemie. But I think the dilation or calibration, gentle dilation, you don't want to injure the tract because that makes the repair much more bloody and more annoying in three to six months, is a very reasonable approach. I mean, tomorrow you might be going on holiday, or there may not be OR time tomorrow, or the baby may have some cardiac issue. There's no rush. However, if you feel confident and it's relaxed time and you have OR time and you want to do it, there's nothing wrong with doing a newborn repair. Also, the tissue is a little different. It's a little more moist. It's a little more fluid. We all know what it's like to operate on a newborn. And sometimes Jack's right. It's nice to wait. My only suggestion would be, well, let's ask this as a question. Please, if you can speak into the... I had a question about... Say where you're from. I'm a fellow from Miami. I'm a pediatric urology fellow, basically. But I'm a pediatric surgeon. Great. Welcome. If you wait for six months, wouldn't you have a dilated colon? Aha. So that's what we're getting at. So let's say we've decided to dilate and delay our repair. The choices are three to five months or eight to 12 months. Eight or longer months. So proximate months after eight months. A, B. And I think you're bringing up a very important technical point. All right. Aha. Very good. So does anyone want to defend the late anoplasty position? I predict that person will remain anonymous. All right. Let's talk about that. Because this is a very confounding problem. Because not infrequently, these patients' diagnosis is delayed. They are sent home from the newborn period. No one noticed that there was a problem. This is particularly relevant in females, which we're about to get to. And six months, they show up at the pediatrician, and the problem is constipation. We've all heard this story. They go see someone else or gastroenterologist and says, wait a minute, something's wrong with this perineum. And they end up in a surgeon's office at about eight or nine months. We've all been there. What is the technical challenge, then, for the surgeon in that situation? Jeff, you want to? So in that situation, you obviously have a dilated rectum. And pulling that down a dilated rectum to the anal canals can be difficult. So that patient will either need a tapering. But better is to try to decompress them and get the rectum to shrink down, probably even pull through. Also in that setting, especially if you're going to do a tapering, you might even want to consider diverting that kid at that point. But it just makes the whole reconstructive process more difficult because of the dilation of the rectum. I also think that the fistulas tract is more inflamed. It's just a much more annoying case than if you do it when they're earlier. They haven't dilated. They haven't gotten as inflamed. And then I would also add another factor. It's not as pleasant to dilate them postoperatively because one-year-olds are stronger than most of us in this room, as you all have seen. Whereas it's really quite pleasant to dilate a four-month-old. It's not nearly as big of a challenge for the family. So I would advocate if you're going to delay the repair, which is perfectly reasonable, try to get it done within the first six months. I think that is going to limit how much frustration you'll have with that case. Of course, if you get to refer the patient at one year of age, you don't have much of a choice. Jack. And I think the key cutoff point is where they go from being breastfed or formula fed to when they start on solid food because there's a very important change in the character of the stool at that point. And that's where they start to get that dilatation of their rectum. It's usually around six months. One other consideration, too, in this population. I mean, there's new data coming out in terms of long-term neurodevelopmental outcomes in kids who get anesthetized earlier. And if this is something that's elective, the question is, well, how long do you wait? And again, I don't think we really know what the answer is. And we're not going to definitely wait until the rectum gets distended because they've switched from breast milk to formula. But that's something to kind of think about, too, is, you know, when do we want to anesthetize these children? I agree. Good points. All right. Jonathan. Can I just ask on that question about dilatation? I've got a baby around at the moment who was born at 32 weeks, transferred over from another region, partly with an anorectum inflammation, but also because of an airway problem. And this child in transfer was really crook, lost their airway, CNC went up. Can you translate that? Well, the child became very unstable, having lost the airway, and the pH was 6.7 for a fairly long period of time. So, actually, I didn't think this child would survive and got through the first week really well. It wasn't well enough to go to theatre for a stoma, and I had a look, and there was a membrane that you could see meconium through at the level of the dentate. So, I perforated it, and we started dilatations. And since then, there has been some spontaneous stooling and absolutely no distension. So, my question is, when we get to four months of age, if this child is able to stool without dilatations, I'll obviously do it in any way, would you consider a repair mandatory, or do you think it's possible just to leave it at that? Well, that's a very interesting discussion. So, my response to that is an anus is defined as a properly sized hole in the center of the sphincter that is mucosal lined. If you can confidently state that you have that scenario, I don't think that patient needs surgery. My concern is that the patient has sort of fistulous tissue that will stay constricted or needing of dilation. And in the meantime, the child is growing, and it doesn't grow with the child. But in a male, you don't have the perineal body concern like you do in a female. That scenario has occurred, and then it looks like an anus. But I think if the anus is mucosal-lined, is supple, accepts the dilator easily, and your confidence in the center of the sphincter, it's hard to improve on that scenario. Jack, do you agree? Yeah, I agree. I have often dilated kids with these membranes or with anal stenosis, and there's probably going to be a discussion about that in a little while. But I tend to dilate where some of my colleagues operate. Yeah. You have to ask yourself, what are the goals of treatment? And the goals of treatment is to get an anus that is supposed to do what anuses do. And if you achieve that, you're done. The dilation process is sometimes very gratifying in a sick baby, and then they release the meconium, and their belly decompresses, and then you don't have to rush to do anything. And that happens, and I think that's sort of a trick you need to learn how to do, is how to get the meconium out gently without injuring the urethra. So here's another similar case with a little bit of variation to a theme. So let's poll the audience. Would you consider a newborn or delayed, well, let's put it this way, newborn repair tomorrow or colostomy, A or B? Newborn repair or colostomy, A or B? do you go, I do, I? I? I? I? I? choice? Ivo, you want to give us your opinion? I would consider this, I guess the audience as a trans-scrotal fistula, which are generally maybe in more than 90% low type of malformations, and you will find the rectum very low, and you can do a primary neonatal repair. I've stated this at the Hanover as well, there are some cases reported by one of my colleagues and others as well, in which the trans-scrotal do have a higher rectum, so you should be maybe be a bit prepared that you may find, I mean there is a fistula to the perineum, there's no urethral fistula, but the rectum may be a little bit higher in some cases, but there's only a few that have been reported. But this is generally... Would you do any additional testing in this patient to feel confident of which scenario you're dealing with? I have an... you can do a cross-table x-ray to see, and then you sometimes see the fistula by air, and you can see the rectum at what... if it's one or two centimeters away from the... from the perineum. You can do an ultrasound if you have good radiologists, but this is debatable because this... you need a very good radiologist who doesn't push too much, who doesn't... who is qualified to do it. If you're going to... if you're going to add a radiologic study to this patient's evaluation, who would do A, cross-table lateral film, B, ultrasound at your institution currently? So cross-table D, invertogram? Are you talking about the... Well, I call it a cross-table lateral film. Everyone knows what an invertogram is. You literally hold the baby upside down. They vomit everywhere. It's a big annoyance. But if you just put them up on a bump in prone position, you can get very good data. We have some pictures of that. Yeah, and you should... So A, x-ray, plain x-ray, at the bedside in the NICU. And you should wait at least... not do it neonatal as a newborn, but wait a little bit. 24 hours. Yeah. So A, x-ray, B, perineal ultrasound. I'm just curious to know. I can tell you if I... Yeah. If this slide and that question came up and we were in Europe, the percentage would be heavily favored towards ultrasound. Yeah. But I'm curious to know what this group would say. See, and here we tend to radiate our children more frequently. I think the issue with ultrasound, as Evo has alluded to, is you need a good radiologist. You need to make sure not to push. Maybe Dr. Bates or Dr. Krauss are two token radiologists in the audience surrounded by a bunch of surgeons. Can you make a comment? Good morning. I think with ultrasound, that is the primary criteria you can't push. The other major criteria is the baby has to be quiet. If they are screaming bloody murder and valesalving, you may get a false sense that the position of the rectum is lower than you think. So I think those are the two major criteria. The other thing, too, is that you have an opening in the perineum. And what the cross table or the prone invertegram is supposed to do is supposed to tell you after a long period of time and high pressure in the rectum that you have a distended rectum. And if you have a hole in the rectum, then the air is going to escape there and it's going to give you the false sense that the rectum is higher than it really is. Jack? But I think in this scenario, by far the majority of the kids have a low rectum. By far. So the goal of your radiological testing here is for those very rare cases where it's a higher rectum and then you've got a very thin little tract going down to the perineum. And that scenario is extremely rare. Right. I think if you're approaching these malformations from a posterior incision, your number one responsibility is to know what will be the first structure I encounter. If you can confidently say that the first structure you encounter is rectum, then you're safe. If you don't have that information, you need more data. And the best way to get that data is a colostomy and a distal colostogram. But in a patient like this, and we'll show you some cross-tabellateral films, you can know where the rectum is and where the rectum is of good lumen. Mark? Question from Tripoli, Libya. What is the value of an exam under anesthesia? Could that help change your plan in the operating room? So I think that's a good question. What would an exam under anesthesia look like in a baby like this? Maybe Jeff can say what you would do. They're very comparable cases. So this patient, to me, looks like there ought to be a hole here eventually. And somehow meconium got up this raffae. So I would call this a peroneal fistula. And I would call this a peroneal fistula as well. Isn't it pretty? One's black, one's white. One is meconium, the other is mucus. But it's the same anatomic situation. An examination under anesthesia, I think in this particular patient, with an electrical stimulator, would show contractions here with no opening, except there's got to be a rectum very close by. And an examination under anesthesia here would show this discoloration to be the center of the sphincter and that to be the beginning of the, or the end of the fistula. So these are very similar scenarios. These two patients. Mark, can I ask you, what do you do with the fistula? Did you open it and you scratch it? Like the white baby? Oh, this stuff? Yeah. Did you open it and you scratch? Yeah, I just take that off and it just heals. Unroof it and leave it at home. Yeah. I mean, I wouldn't leave this here. Just unroof the meconium. Was there a question in the middle? Dr. Schaal. Mark, I just wanted to ask you if you were going to talk about the dangers of a newborn repair at some point. For sure. I don't know what your agenda is, but that's something we don't want to. Why don't we tackle that right now? What are some of your, how do you approach a patient like this in the safest possible way? And I want to just make one point about the technical. We're going to show some cases both today and tomorrow. And the goal of showing those cases is not only to show some technical tricks that you might like, but also specifically what to do to avoid trouble. Because in this field there's a lot of trouble. And it's relatively easy if you know how to avoid it to stay out of trouble. And one of my professors like to say it's a lot easier to stay out of trouble than to get out of trouble. So you want to say how you set up such a case? We're on the topic right now about how to avoid the injury that you're alluding to. One of the things I noticed about my junior partners is they're very hesitant to do an anoplasty in some babies even though there may be some meconium on the perineum. And they all want to do a colostomy which surprised me. And so I said, well call me and I'll come do the anoplasty with you. But the truth is that when you're considering doing a newborn anoplasty in a male with a recto perineum fistula, the urinary tract is incredibly close. And so the way I avoid that is I don't do a circumferential mobilization in a newborn male like this. I do what we might call a cutback. So essentially in this baby shown in the previous slide, you can either unroof where all the meconium beading is or you can, if it's a shorter tract, sometimes I'll just take out the tract, leave a little tiny opening in the skin which I then close with chromic sutures, and then do essentially unroofing of the anus. You have to mobilize the posterior rectal wall, but you don't really, you have to be very careful if you start mobilizing the anterior rectal wall because it's amazing how close the urethra is. And I know of some excellent surgeons who have transected or injured the urethra thinking they were doing something as innocently as a newborn perineal anoplasty. And I think one of the big ways to stay out of trouble is don't put the baby in a prone position. I would do this case with a baby in lithotomy, not in prone. Okay. So let's poll the audience. Who would do this anoplasty? A, prone. B, lithotomy. Okay. A, prone. B, lithotomy. I mean, I don't think it matters whichever you feel is safest. There are some unique advantages, I personally believe, to the prone position because the difficult part of the dissection is always the anterior part, and I like to look down, not up, on the hardest part. And that's also true for Hirschsprung. Here I like to do transanal rectal dissections prone because the hardest part is the anterior, and that's the vagina or the urethra. But there are a couple points to be made here. One is what Don is saying is to simply fill this space with rectal tissue. And I think that's a very nice option, essentially a glorified cutback, provided that this is the beginning of the sphincter. That option is not going to work if the hole is here. Then you really need to transpose that hole down to here. And then you really have to do an anterior dissection. But in this particular case, you do have that option and you can avoid some of the anterior dissection. Jack? Are we allowed to disagree with you, Mark? You were invited here to disagree. So I disagree with your contention that you need to move the front of it back so that it's all within the sphincter. I believe if you just enlarge that opening and bring the back part of it, I think this is what Don is saying also. That's what I said. I said if the fistula were here. No, I'm saying even if the fistula is further forward. Oh, okay. I would just do a cutback. I would do a suture, not like the old cutback where you put a scissors in there and just cut it. But I would just extend the rectum back to the back part of the sphincter. But I would leave the front part in its abnormal position because I think there are several advantages to that. One is that you are avoiding that anterior dangerous dissection. And the second is that it doesn't tend to stricture down afterwards as much. And you don't really need to dilate them on a daily basis because you only have a partial circumference suture line. I think those are good points. In a female, we don't have this luxury because we need to gain a bit of a perineal body. In a male, it's a little bit different. My only concern with that idea is if you're going to leave anal opening outside of the sphincter, what I have seen is patients later in life who've had that done, when they try to close the sphincter, they can't completely close the hole. And they have a little anterior leakage, particularly during athletics or if they have loose stool. So my goal is to try to get the hole completely surrounded by a sphincter that can close the hole. That's my only hesitation. If you had a fistula way up here, when they squeeze this sphincter, they're not going to squeeze the stool in the anterior portion. So I think that I just, I would like to, I want, when I finish, I want to make sure that that principle has been achieved. And we, we know that. Maybe Paola, you can comment, Paola wrote a very nice article about two years ago on the older female that was operated on. Meaning a patient who either did not have a proper repair or never was touched and later in life had some trouble. Yeah, especially when they approached the sexual activity. It's all disturbances in the mechanics. And we had this adult person who became incontinent because of sexual activity. And she basically had a recto vestibular untouched fistula and she didn't know to have it. She was handling the situation, but once she had a partner, she became incontinent. But she could handle for 20 years in her life. And then after we repair her, she said, now I feel the muscles. Before she couldn't really realize where she was constricting the muscles or where or not. I'm going to skip, I just want to show you this one little clinical point. Sometimes they have these bucket handle anomalies. And there's no evidence of a fistula, but you can be very confident that underneath this little bucket handle tag is the fistula. So this is consistent with a perineal fistula. But let's get into the female discussion. So why don't we say this. A, this is a normal female. B, this is a perineal fistula. It's not so simple. I would suggest that probably one of the most common clinic visits is a, is this normal or not question from a pediatrician or guy. From a pediatrician or gastroenterologist. In a female in particular. So A, normal female. B, perineal fistula. All right. So no one thinks it's normal. So what about this picture, Jeff, is abnormal? In what way is this abnormal? So first I think you, as you pointed out, you have to understand what the normal findings are. So the, the, the anus has to be in the center of the anal muscle complex. It has to be appropriately sized. And there has to be adequacy of the perineal body in a female. So in this picture, looking at it, I mean the anal opening obviously looks very small. It's actually smaller than the vaginal introitus. The, it's also just behind the, the, the, the vestibule of the vagina. So it's, and it would be anterior to the expected position. So those two findings, the small anus, the fact that it's anterior. And then you get a sense that the anal muscle complex is actually posterior to it. So given those three criteria, that would suggest that it's a perineal fistula. As opposed to it being normal. And it's outside the vestibule. So that's how you distinguish it from a vestibular fistula. So if we go back to the definition of an anus, that hole is too small. And it's not in the center of the sphincter, which is here. All right. So let's, let's see what the panel would do for this baby. So let's do this. So who does A, newborn analplasty tomorrow. B, dilation with delayed repair in the next six months. Assuming a healthy baby. Healthy baby. A, newborn analplasty tomorrow. B, gentle, elegant dilations and repair in the next six months. Okay. Very good. All right. So what are the questions that we have? Okay. Take a look. I'm going to take a look. I'm going to take a look. Very good. All right. Jonathan. I would concur with the audience. I think that I would also... So one third of the time you would do... On the same page. No. I would do an MR in the time that I'm doing the dilatations. I'd want to know that there's no pre-sacral mass. And I am assuming that we have the capacity to make sure that the child is decompressing. That there's nursing support once the child's gone home. So if they run into problems that we know. If those situations hold them, that's what I'd do. And Jack, a la your discussion in the mail. How is this different? How is this scenario different? No. I would treat this the same. I would dilate it if possible. If the child is evacuating well with the dilatations, then I would come back three to six months. And I would... The anoplasty I do at that time would be the posterior kind that I talked about before. So from a technical point of view, you would not just enlarge this? I would not touch the front of it. I would... Oh, so once again you would just fill this. Okay. So, let's do this. A, you would just fill this space with more anus. B, you would mobilize this structure and put it in the center here and then close where the fistula currently is. So, A, I would call that essentially a formal cutback. Cutback has a negative connotation, but it probably ought not to because there are some advantages to it. As long as it's mobilized and good tissue. Or, B, mobilize this, put it in the center of sphincter and close that space. The big issue is perineal body. How much perineal body do we need? And we will talk about that in the gynecology section. And I can tell you this is a big controversial point of what to do here. Because from a technical point of view, you don't have any anterior dissection. However, from a functional point of view, you want to be absolutely certain. So, most people would do a formal movement of the anal... A transposition, I think most people would call that anal transposition. A transposing of the hole. What I have found as I followed these kids out into teenage years is that what looks like a very small perineal body here actually gets much bigger. It grows. It grows. I think that is a really, really important point. You have to get a sense of which perineal bodies are going to be long enough and which are going to be inadequate. Dr. Jorgensen. In 2005, I sat next to Dr. Stevens at the meeting in Germany. And I had a running, loud commentary about everything. Because he couldn't hear well. And so he also spoke loudly. But anyway, his contention to me, as we sat there with this kind of an issue was, that they all tend to migrate backwards so that a cutback is fine. That that will grow over time. Yeah. I think it's a very important technical point. I think, though, you need some perineal body. I'm not sure if... Does Jerry view it in the audience? Jerry. Our gynecology colleague, maybe you have a comment about the perineal body in the future. Well, I think, you know, it would be important. Our... From a gynecologist standpoint, we would advocate to build as good of a perineal body as you could. Not only to support separation of the reproductive organs from the GI tract, but also in terms of sexual functioning. And also in terms of any possibility of a vaginal delivery. So I know from a gynecologist... Gynecological standpoint, we would advocate building a perineal body. And then the question becomes, how long is long enough for a perineal body? And I don't think there's a great answer for that. But you do bring up a very interesting question. Let's poll the audience. You have a beautiful repair. Either way, everything is healed beautifully. 20 years from now, the patient asks you... Tells you that they're pregnant and they want to have a delivery. You as the surgeon would advise, A, normal vaginal delivery. B, mandatory cesarean section. And I'll tell you there's no right answer to this. But I'm curious to know what everyone's opinion is. Vaginal delivery will be no problem, don't worry. B, cesarean section. And I will tell you, there's no right answer to this, but I'm curious to know what everyone's opinion is. The responses are getting slower. Either people are getting tired or... Are they increasing? Okay, good. All right, we've got to watch our time. There's a lot more. Oh, look at that. It's quite mixed. Anyone on the panel want to... I would definitely say C-section. I would definitely say C-section. C-section from the Netherlands, C-section in Italy, Seattle. I've seen women become incontinent without these fistulas because of the work, and I think the risk is too high. It's not worth the risk afterwards. That's what I think. Although, some cases can have a vaginal delivery, I'm sure. But I wouldn't take the risk. That's my point. Surprisingly, I disagree. I would say vaginal delivery, but with a lateral episiotomy, if they need an episiotomy, if it's not going well. And that's an education issue. The patient has to understand that issue and has to talk to their obstetrician about it. I agree, and I actually think that it's an important connection, and we're working very hard on making that connection between Jerry and the folks that might actually deliver these babies and making sure they understand what is an anorectal malformation and what is a reconstructed perineal body. Because I can tell you most of them probably don't have that in their mind when they're at 3 o'clock in the morning trying to deliver a baby. That's a delivery that needs to be properly planned and discussed with a pediatric gynecologist who knows about these kinds of repairs. Mark, can I point a last aspect on this? If we do, like as Jack is suggesting, like cutback, particularly well done cutback, another, I am against cutback, but one positive aspect is that you don't throw away any rectal tissue or minimum rectal tissue because someone says that you still have some sensory fibers there. So one potential advantage is you save more of a sort of a dented plane, line, or whatever it is there. We will have more opportunity to talk about that today because this is a comparable case will happen today. We'll discuss some of these issues. Okay. Anyone have a comment on this newborn female? Nate, what is the diagnosis? So I can tell you that this case was described as imperfect anus with urogenital sinus. It's true. So that's a lot of words. And I got a six-letter word that says the same thing. Right? So this is a cloaca, single peroneal arpore. But we need to make a very important distinction. And I am being a little bit provocative with this slide because you don't really see the anus. So for those of you who are bothered by that, you are right. Because if you had the exact same appearance with a normal anus, that's a different situation. That's urogenital sinus. That happens with or without androgenization. That's a word. Okay? However, if there's no anus at all, that's a cloaca. By definition, they do not need an endocrine evaluation. Their electrolytes will be normal. However, if there's a normal anus, that's a whole different story. Shamal, do you want to make a quick comment about that distinction? We need a microphone. Otherwise, the outside folks can't hear you. I mean, just, you know, how you, when you get called to the neonatal unit about a, quote, urogenital sinus, and the anus is, in fact, normal, what's different about that than this? Well, I mean, that would be consistent with disorder of sexual development and the concern of the congenital adrenal hyperplasia. So that's a relatively emergent workup. But if you look at all the experience that you've had, and we've done this before, the incidence of congenital adrenal hyperplasia in the anorectal malformation subgroup is almost zero. Yeah. This is an anatomic problem. This is not an endocrinologic problem. These children all have normal ovaries, and they do not need a workup. But many times, these patients are evaluated for ambiguous genitalia. And in the female section, I have some more pictures of that. Okay. Let's move a little quicker now, because we have more to cover. Diagnosis, now that you're experts. What's A? Shout it out. What's A and B? Oh, you want to do A or B? Okay. A, normal. So is B. Well, you should say a normal one. Does everyone agree this is a, what would you call this? Fouchef. Fouchef. Yeah. Vestibular. No. Fouchef. Perineal. Perineal. Perineal. Perineal. Oh, okay. So it is a little more controversial. So there's a hole here. It's too small. It's too anteriorly located. Jack, would you do a formal cutback in this case? For me, the difference between a perineal fistula and a vestibular fistula is whether there's a perineal body. Correct. In this case, there is no perineal body. So this is a vestibular fistula. And I would do a minimal PSAR, but I would normally do this without a colostomy. And that's a controversy. Most of my colleagues would do it with a colostomy. So does everyone understand that distinction? A neonatal PSARP you do. All right. So let's ask that question. Who would do a newborn PSARP? Let's put it this way. Who would do a newborn PSARP or a delayed PSARP with no diversion? A. B. Colostomy with a repair sometime later. And everyone recognizes the difference here. There's basically no perineal body here. We've got to do something about that. What do you think about that being? Let's get the answer first. A. The primary repair, either newborn or delayed after a time of dilations. B. Colostomy and then delayed repair. All right. So and by the way, both answers are correct. I think it's based on the clinical experience of the surgeon. I personally would feel comfortable doing an undiverted repair. But I can assure you, you will have less perineal complications if all your vestibulars are diverted. But then you get the exchange of having all the colostomy complications and the colostomy closure. So I think if you get comfortable with this procedure, it's definitely one that's doable in a primary way without a diversion. And perhaps an intervening step is to do a primary repair with diversion at the same time. Yeah. And then do a colostomy closure down the road. But I think the days of colostomy repair, colostomy closure in three stages, are probably not needed. You can probably do a definitive repair with diversion, then colostomy closure, or definitive repair. But Jonathan, you had a question. Yeah. I just wonder if it's possible that this child has got a degree of one of those congenital perineal grooves. So that in which case, an EUA, you'd see whether or not actually there was a bit of a perineal body. So what Jonathan is saying is maybe this tissue here is actually perineal body covered by mucosa. And he has termed that a perineal groove. And I certainly think that is reasonable. There are much deeper holes that are clearly vestibular. This is clearly on the borderline. Mother Nature makes fun of our categorizations. Because every time we try to categorize, she puts something in the middle. Okay. There's a good one. Yeah. This is the bigger case. Normal. A normal. B abnormal. A normal. B abnormal. And then the perineal body. Normal. A normal. B abnormal. And there is some still coming up. She's not a neonate. It's not a neonate. Okay. Oh, my gosh. See? Look at this. So in the group that said abnormal, which is 69% of you, you would be booking OR for this patient. Is that correct? You can ask that. Well, there is a category. Normal but doesn't need an operation. A normal. A normal. Right. Great. A normal. Thank you. No surgery. A abnormal needs surgery. B abnormal. Leave the child alone. Vicki, are you keeping up? Vicki Lane, are you keeping up? No, are you keeping up with the questions? Okay. So what did I say? A abnormal needs surgery. B abnormal but don't touch the child. This is one of those times where you have to have the conversations. I'm really sorry to disappoint you, but you will not need surgery. They start crying. They were hoping for surgery, right? We've all been there. All right. So most of you say that you think something about this is not quite right. But most of you say leave the child alone. Okay. Jeff. So, I mean, we'll see these kinds of kids in clinic all the time. And so it becomes this dilemma of, do they need an operation or not? Because these are the ones that are always deemed the anterior anuses. So, again, it gets back to what's a normal anus. If you look at this anus, it looks like it's surrounded by muscle, as best we can tell. So, again, the important thing, and you can determine this in clinic, is put a Hagar in the bottom and size it and see if it's appropriately sized. And you can also even test the muscle by using a Q-tip or just looking for the anal link. And if it looks like it's in the center of the muscle complex and it sizes appropriately, I think you can leave these kids alone. I think the perineal body with time will get bigger. Sometimes I'll see these kids back in six months, a year, just to confirm that, reassure the parents, watch for the constipation issues. But a lot of times these kids will present with constipation. And even so, it's usually during that transition time. But fixing it, it's not going to necessarily make the constipation go away. So, unless it's inappropriately sized. But outside that, I would leave this alone personally. Is the hole the right size? Yes. Is the hole surrounded by sphincter? Essentially, yes. Is there a perineal body that's adequate and will certainly get bigger? Yes. So, this is a patient I would not touch. I will tell you, this is out of a coming out article by Risto Rentalha about this issue of the, quote, anterior ectopic anus. And this is what I would call an anterior ectopic anus because that is truly an anus. There's a dentate line. It's surrounded by sphincter. It looks like an anus. That's an untouched, a patient that does not need to be touched. And with his permission, I took that picture from his article, which has not been published yet, to show because I think it's very, very instructive. Now, if you're not sure, you can always do an examination under anesthesia. Stimulate. Check with a Hagar. But I can assure you, this patient has a good size, properly surrounded by sphincter. Yes, the perineal body is a little bit short, but I think it's going to be a lot longer down the road, and the patient does not need to be touched. Mark. And Mark. China, they want to know about the use of MRI. MRI? MRI. To identify the muscle complex. Perineal MRI, I guess. I'm going to defer to my radiology colleagues. MRI to define the perineum? Is that the question? Yeah, the muscle complex. The muscle complex. The muscle complex. Ah. How good is MRI, the very, very distal aspect of the, wherever the rectum exits? Yes. So, um. Is this on? No. No, this is. So, we use MRI usually in the patients who are coming back after analplasty and having problems. And in that scenario, it's pretty good in showing that the muscle complex is surrounding the rectum or not. In a patient who has not been operated on, you certainly can use it. I think that if you stimulate that and looking at that, it looks like the rectum is within the complex. So, doing an MRI is sort of a moot point. And I think if you want to know how much muscle there is, you know, you can be at comparisons to patients who are normal and who are not. But I'm not sure it's necessary to do that. You can. But I don't know what advantage you're going to have to show that. My response to that is a pretty fancy test for something you can determine clinically. Right. Exactly. And here are the two pictures side by side. Please burn this into your, into your memory. Because I think this is a very confounding office visit for many, many of us. That can really be clarified by a good clinical exam. Mark, last ask for it. These girls tend to have the family members like them. Like the mother and the sisters or the cousins. I've seen a cluster of these females being untouched. So, grandma has to come to clinics as well. I'm a transitional care worker. Mark, can I ask you a question? Would you recommend that both of these patients would have a full bacterial screen or not? No, that's a very good question. So, A, full ARM radiologic evaluation. B, nothing. Including echo, right? Including echo. The patient on the right we're talking about. The patient on the left, I think we'd all agree is an anorectal malformation. The patient on the right, full ARM eval. B, nothing needs to be done other than a physical exam. Okay. Okay. I personally would just do a physical exam and leave the child alone. Does anyone have a difference of opinion? If you don't operate, you don't do anything else. That would be the cutoff. If you decide to operate, it's because there's not a situation like on the panel on the right. And otherwise, you have to discuss the length of the prionineum to decide whether you do the screening. Because if half a centimeter is going to be the decision, or one centimeter or two centimeters, what is then the size you would have to choose? It's very difficult. And so if you decide not to name it, not to call it an anorectal malformation, but a normal anus inside a schrinkter complex, I don't think you should do it. Jack? I would. And the reason I would is because I don't know of any data that tell me whether this is associated with other bacterial things. And the bacterial workup is non-invasive, and it's relatively inexpensive. The other thing I think that's important, though, is to do an ultrasound, or if it's an older child, an MRI, to look for a presacral mass. Because this is the kind of situation you might get in a occur, you know, triad. Patient on the right. Patient on the right. The same thing was said from... We have some pictures of that. Would you echo that? The international audience was saying the same thing. They would do a workup. For the presacral mass concern. Yeah, very good. We have a couple pictures of that coming up. Well, I was just going to ask, though, for like a standard perennial fistula. Who would get an MRI versus not get an MRI looking for a presacral mass? Yes. Okay. I actually want the... Maybe you guys are radiologists. By the way, I've been blessed with two phenomenal radiologists, one in Cincinnati visiting us, Steve, and now here at Nationwide. And I hope all of you go find yourself a radiologist to bond with, because they take very, very good care of you, and they keep you out of trouble. So I'm curious to know your thoughts on that. Well, let's talk about it this way. Should we poll first? Well, I want... What would be your question? Well, for a perennial fistula, A, who would get an MRI, and B, who would not get an MRI? Are we getting a spinal ultrasound, though? Because my question... I mean, you're going to... Yeah, I guess it's... My question to you is, how good is a spinal ultrasound at determining whether there's a presacral mass? Because I can assure you, both of you are clued in to this presacral mass worry. But most radiologists do a spinal ultrasound, look at the distal spine, and quit. They wouldn't necessarily look carefully. We have added that to our protocol in doing the spinal ultrasounds, and all of your patients, particularly, is to look for a presacral mass. In an ARM patient. In an ARM patient. Yeah. Particularly, look for a presacral mass. I think you have to sometimes increase the depth of the ultrasound examination to make sure you're looking in the presacral space. Obviously, it's going to depend on size of the presacral mass. If it's extraordinarily small, you potentially may not see it. But most presacral masses will be seen, particularly if cystic or solid. But you have to look for it, because in a routine spine ultrasound, you will not, when you're looking particularly just at the conus position and seeing if there's a tethering lipoma or a cutaneous tract. I agree with Jack. I think a spinal ultrasound is pretty easy to do. Kidney ultrasound is very easy to do. But your radiologists need to be clued in to the fact that this is an anorectal malformation, and they need to look at the presacral space. Because there's nothing worse than missing a presacral mass. And we're going to talk about that in a little while. But I know of two patients in whom a presacral mass was missed, and the teratoma became malignant. And they had a full evaluation as a newborn. And that's something absolutely avoidable. Mark? The spine ultrasound is good. We also use the images from the pelvic ultrasound when looking at the kidneys and the bladder, because you can oftentimes see the anterior sacral mass through the bladder. And so... All right. Here's a... Picture? I think this is your picture, Paola. Yeah. A, normal. B, abnormal. Seems a bit out of line. And everyone knows the next question. So let's get that answer first. A, normal. B, abnormal. By the way, the urethra and the vagina are normal. Okay? It's hard to see, but the urethra and the vagina are normal. A, normal. B, abnormal. What's the answer there? Okay. Need surgery? A. Leave the child alone. B. Okay. A-ha. Who would like to operate on this child? 29% of you want to operate on this child. Yes. She's 95 years old. And how many of you are willing to defend that position? I'm not sure how to make this better, actually. Paola, you have an opinion? Well, this is a child of mine, so I have the follow-up on her. I didn't touch her. She's 95 years old. She's 95 years old, and she's passing feces normally. Yeah. It's hard to improve on it. I decided not to touch it, although at the beginning I was really impressed, because it seems to be offline, and it seems to have like a little groove. But it was definitely in the center of the muscle complex, adequate size. I have seen, unfortunately, patients like this operated on, and the anus has been moved out of the sphincter mechanism. That's the risk. Because someone in their mind said, I need to make more of a perineal body. And that hole will work, and that hole is centered by sphincter, and that perineal body will get bigger. One of my professors like to say, it's hard to improve on an asymptomatic patient. It's hard to make this patient better. So I personally would agree with Paola, and now we know the answer was correct, because she has, what, 10 years of follow-up? Yeah. So don't touch it. Did you stimulate the patient? I did, because, yeah, that was back years ago. And we decided to stimulate the patients to see if the visual impression corresponds to the anatomical impression. And after we have done, like, 10, 15 stimulation under EUA, we decided to believe on our visual impression. Do you want this slide or the previous? Previous. So, Mark, I think, again, in a female with a perineal fistula, or if you're unsure, I think, again, that's an important factor. You know, I think that's a good case to dilate that patient and wait or calibrate and wait to see how much that perineal body does grow in length, especially if you're unsure. I think that's a good suggestion. There's no rush. This child, I predict, would calibrate very normally. Yeah. All right. Let's go through some of the rest of these quickly so we can get to some of the newborn evaluation. Diagnosis. Just shout it out. Vestibular fistula. Okay. Stenosis. So, how's the size of the hole? Too small. It's properly centered. Right? That's small. What would you call this? So, this is anal stenosis. And this is the patient that you have to make sure you rule out a presacral mass, take a plain x-ray of the sacrum, and make sure your ultrasound is looking for a presacral mass. And then, treatment? Diagnosis. Dilate only. And I think if you can get a supple anal canal that's not feeling like it's stricturing, you're pretty much done. However, if it's narrow, and sometimes the stricture is a good length, you actually need to do something. And I'm going to show you a couple slides on a nice technique to do that without violating this beautiful dentate line. All right. All right. We sort of showed that one. We showed that one. That's a diagnosis? Peroneal body in a male. Okay. What do you think of that? So, we tried to put a dilator in this, and it didn't go anywhere. What's this diagnosis? It's terminal. What would you call this? It's terminal. Rectal atresia. Right. So, I would call this rectal atresia. It's a hole. It's in the right place, but it doesn't go anywhere. So, this is on the same line of anal stenosis. One will pass stool. One will not. This, obviously, will need surgery. And we have a nice video of this case, which we're going to show you when we start to go to set up for the OR. So, just remind Vicky to get to show that video at that time. Okay? Okay? What's this? Many holes. It's like... Too many holes. How many? A double vagina? What do you say? Too many? Too many holes. So, Shamile and I and Richard were in South Africa. And we went to an ostrich farm, which I strongly recommend because it was fascinating. And they were talking to the three of us about what is a one-holer. They call ostriches one-holers. And they explained what a cloaca was. And they were going over this whole thing. And we were sort of giggling that we had a little bit of knowledge of what that was. But it was quite fascinating. So, this is a two-holer. Right? So, what's in there? What are the holes? It's getting tricky now, right? So, I can tell you this is a urethra. Which is a urethra? This is a urethra. Okay. And it's only a urethra. You put a cystoscope in there. You see a urethra and a bladder. This is an anus. And it's the right size. And we can argue whether that perineal body is short. But there's a perineal body there. So, what's this malformation? Any other two holes on the side? Yeah. It seems that are... Nothing. Oh. Okay. It looks like a hole. Those don't go anywhere. Those are little crypts. Okay. Okay? Diagnosis. Very good. So, this is an absent vagina situation. And the interesting thing about this is the anus is normal. So, I think it's appropriate to call this Rokitansky. But you could have this hole here for urethra. And the other hole of the rectum being right there. And then I would call that vestibular fistula with absent vagina. That's a whole unique discussion. Which is out of the context of this particular. We're going to talk a little bit about that. We're going to discuss female. Okay? How about this one? And then I really want to go to the workup part. So, this is a male who has been dilated. So, Jack, would you operate on this patient? He's been dilated? He's been dilated. And when you stimulate, there's the sphincter. Right here with his little raised edges. But that looks pretty good, doesn't it? Yeah. Good size. Mucosal lined. So, A, operate. B, leave the child alone. That is a good size, normal 12. Hagar goes in here. Center of the sphincter is right here. A, operate. B, don't touch him. On the posterior part, which has some pressure. I know, it's like a Jew. That's kind of like that. So, Jack, take your cup back. Okay? You can do a cup. What do we got? All right. Anyone disagree with the decision to operate? Would anyone on the panel not operate? All right. I agree. I think this is very pretty, but it's not going to function. It needs to be here. Okay. Okay. Okay? H-type fistula. Normal urethra, normal vagina, normal anus. Except there seems to be a problem. And this is a patient that started stooling out their vagina. So, what this is, this is an H-type rectovaginal fistula. And in this particular case, to me, it looked very congenital. Perfectly midline. Maybe we can ask the audience in Asia who see this a lot more than we do. This is a relatively common malformation that they see. And we don't see it too often over here. But I can tell you, this is the real deal. This is normal urethra, normal vagina, normal anus. And there's a fistula's communication between the two structures. Mark, I've heard two case presentations about that last one. And both seem to present with the people saying that they present with a left-sided labial abscess. Yeah. So, there are two types of H-type fistulas. I think they're the ones that are congenital and the ones that are acquired. And the ones that are acquired are the ones that had somehow had a perineal abscess. And then either the fistula came first or the abscess came first. But now there's a communication between the two structures. But I've seen this in a brand new baby also. So, it is a real congenital entity that one can consider. All right. Let's move into the newborn workup. All right. I want to show, this is a prenatal evaluation. This is a 20-week fetus who had an ultrasound that showed a cystic mass and followed by this fetal MRI. Dr. Bates, do you have any thoughts about this? What does this picture say to you? The family should talk to you. It's a T2-weighted sagittal sequence. As you can see, not having a pointer here, there is a bright oval structure along the anterior inferior abdominal wall, which will presume to be bladder. And there is a fluid-filled structure extending down toward the perineum, back up into the abdomen. Cannot clearly define meconium, but you said... Yeah, so this is... That's exactly what we would want you to evaluate as a radiologist. There's a bladder, and then there's this large thing in the middle. It's a female. Right. So, we would assume that that is a hydrocorpus. So, this is a cloaca. 20-week fetus with a cloaca. Pretty neat image, right? So, there's a hydrocorpus there. All right. Everyone knows what this is. I can tell you, you don't want to be the surgeon that does a beautiful colostomy on an anorectal malformation and then start feeding the baby on day three and discover... I know some of you have been there because this has happened. Yeah. You must rule out an esophageal atresia when you have an ARM. And this is a particularly confounding situation in an ARM is when you have a duodenal atresia and an ARM. Because a lot of the cues that we are looking for, and they're fond of putting this on the boards, guys. So, a lot of the abdominal distension cues are missing here. So, many times these patients need duodenal repair with colostomy because you really can't figure out what the anorectal anatomy is because you don't have distension. Can I just make a comment about that? Because it's fresh in my mind, having just done the colostomy about five days ago on a kid like this. The colostomy is difficult because the colon is... You expect a dilated colon with an anorectal malformation. This is an unused, tiny colon. And bringing that out as a colostomy was very challenging. That's a neat point. Yeah. I was saying, how would it... You're speaking to the... Otherwise, the international analyst can't hear you. If he has a microcolon, I would suggest that there are multiple atresias. And there's a more distal atresia in this patient other than just the isolated duodenal atresia. Well, we put fluid all the way through the intestines and did not find a distal atresia. It wasn't a microcolon, but it was a smallish colon. But still very difficult to bring out as a good functioning colostomy. Who does in every ARM... A would be the answer, an echocardiogram. And B... Not always. Selective. A, every ARM gets an echo. B, we get selective echoes at our institution. Newborn. This is newborn evaluation. Newborn evaluation. A, everyone gets an echo. B, we do it when we... On a selected basis. All right. So most people do an echo. And that... I think that's... Can I just comment on that? We published a paper a few years ago that suggested that if you have a normal physical exam, normal x-ray, and normal ECG, that you never... We never found a significant cardiac problem that would interfere with the anesthetic or that would be relevant. So we don't get routine echoes. I am sure we get way too many echoes. But our routine is to get an echo here. I don't think they all need an echo, but they all do it. Anesthesia wants it most of the time. Anesthesia would insist on an echo before taking an ARM to the OR? Is that what you're saying? Yeah. It would be true. Thanks for publishing the paper. Our echo guys quote it all the time. I won't easily come and do the echoes. And some of it... A lot of it is considered... I think if they've had an antenatal echo that was normal, that's fine. But the thing for me is this issue of a postnatally normal exam. Because I think if you're relying on one of our very junior doctors to say normal heart sounds and good pulses and good perfusion, I think there's a chance of missing things myself. So I'm happy for them to come and do that. You recently had a case of a kid with a perineal fistula who had an L-kappa, so an anomalous left coronary artery insertion. Oh, yeah. And, you know, normal physical exam findings went to sleep and coded on the table. So, I mean, it can't happen. It's rare. But I know Mike had... Rollins, you published a paper a couple of years ago looking at specifically the incidence of cardiac anomalies and perineal fistulas. One second. Right. One second. You want to say? Sorry to put you on the spot. Well, we looked at... The question of our paper was, did all perineal fistula patients need a complete bacterial evaluation? Because at least our practice in the past had been to selectively evaluate them. And we found a large percentage of perineal fistula patients did have abnormalities, whether it's the GU system, cardiac system. The ones that present outside of the newborn period, I don't think need an echo, because they've demonstrated that they don't have a significant cardiac lesion. And we usually just have our cardiologists evaluate them. But in the newborn period, I agree that an echo is needed, even in a perineal fistula patient. Because you guys had an incidence of almost like 30%. 30% of associated anomalies across the board for perineal fistula patients. Let's talk a little bit about the sacrum. We mentioned that there are three factors that can predict continence in a newborn. The type of malformation, which you'll either figure out on physical exam or at the time of distal colostogram. The quality of the sacrum and the quality of the spine. And I think we all can become experts at evaluating sacrums. So this is a lateral image of the sacrum. So we'll poll the audience. A, good sacrum. B, hypodeveloped, poor sacrum. A, good sacrum. B, poor sacrum. And, of course, A means good potential for bowel control. Poor sacrum means poor potential for bowel control. We have a lot of power in the newborn period if we look at these things because we can make some good predictions for the family. So let's see what the audience thinks. So that you don't... It's tilted, so the bottom part is hidden. So we like to look at the lateral. We look at both, but the lateral is more reliable. It's tilted, so it's more reliable. All right. We're going to talk a little bit about cloaca in the female part. So let's skip. Here, let's do some cross-table lateral films. A, rectum is reachable, newborn repair. B, rectum is not reachable, needs a colostomy. Again, this is a patient who at about 20 or so hours of life, there's no evidence of a perineal fistula. Or it's one of those perineal fistulas, but we don't see... We want to know how close is the rectum. So A, let's do a primary repair. B, colostomy, and come fight another day. What do we got? Does this patient have downs? No downs. No downs. So people feel that that is a reachable rectum. Okay? A, primary repair. B, colostomy. Does everyone notice how radiologists name things after, you know, like the basketball sign or the football sign? Right? So I have a new one for you guys. Based on this. Ready? The Twitter sign. New sign. Brand new. Invented today. Twitter sign. Consistent with a high rectum with a probable recto urethral fistula. So what was the answer most people would do with colostomy? Ah, 3% are attacking this patient in the newborn period. Let me guess. You don't want to defend your position. Anyone want to speak? Who's the 3%? Ah! You like this one, Greg? It's good. Yes, I do. It's a good. It's a good with a squam, you can start using it. You can start using it. Just talk like it's been there forever, and people will think, wow, that's cool. That's neat. By looking at the anus and physical exam, do we need – I'm just curious. I don't know the answer. Mostly, I look at the anus, and if I see the fistula, I'm completely – not completely, mostly sure that it is a low imperfection. Do we need to get these pictures? If I see meconium coming out of fistula, I don't need this image. There was some discussion earlier about confirming – there's nothing wrong with getting this picture, but as I think Steve said, that it may have decompressed, and then the rectum is actually – appears higher, but in fact, it's very reachable. It is exceedingly rare to have perineal meconium and a long fistula. Almost uniformly, those patients have a very reachable rectum, and I would approach them primarily because I feel confident that the perineal fistula, just within a centimeter or so, will be healthy rectum that is mobilizable. And so if you don't see a fistula, you get it? If you don't see any evidence of a perineal fistula, and it's about 20 to 24 hours of life, now's the time to get this image, and then you help – that helps guide you whether you do a – go for a primary repair or do a colostomy. Mark, I think the – that those lateral images are most useful when they show a very short distance because the ones that show a long distance, sometimes there's meconium sitting there that prevents the air from getting all the way to the perineal. So you may – yeah, so this one I think is very useful because there's no other real good explanation for this other than a very short distance between the skin and the rectum. But with this one, it's possible that there's meconium there. This could still be a short one. For sure. But at least you are not going to make the mistake of going in too early if you have this picture. But without a doubt, it's always safe as to just do a colostomy. Mark? Well, to drive in that point, we just had a patient six months ago who I did a distal colostogram on who I actually know that when they did this cross-table lateral, it looked like that. And in retrospect, the distal colostogram showed a perineal fistula. And I think what happened was is that there was no meconium after two days. And sometimes you don't see it. And you'll get a couple of patients. Nothing is 100% except death and taxes. And needing a good radiologist to collaborate with. So this is a picture of that scenario. This patient had a colostomy in the newborn period and then got this distal colostogram. And in fact, had a very reachable rectum, actually. I can tell you it was perfectly safe to do that colostomy. But this potentially could have been a reachable rectum in the newborn period with good evidence, as Jack has said, of a very close rectum. Now, this was read as high rectum, long fistula. But the truth is that's not the case because this is simply a non-distended distal rectum because all the contrast leaked out the perineum. But that is the scenario that Steve is describing. All right. We go to the operating room for our colostomy. We can talk about a little bit of the technical details of what type of incision and whether to use laparoscopy, et cetera. But some of the basic principles. Can everyone suggest, maybe from the panel, where would you open your colostomy? So this is a left lower cologen oblique incision. So why don't we poll the audience? Why don't we say A is here. A is here. By the way, the head is here somewhere. A, open here. B, open somewhere over here. So A is the very proximal sigmoid. And B is the mid to lower sigmoid. In this case. All right. What do we got? All right. Most people would agree that they open in the proximal sigmoid. And let me just show you a nice anatomic cue for that. So here is the left retroperitoneal attachments of the left colon and where the sigmoid begins. So we like to open here, right at the very proximal sigmoid so then that part of the colon won't prolapse. Clean out all this meconium, which is a very important job. Don't leave that inside. And then make a mucous fistula. So let me ask the group. Who is doing A, an incision colostomy, or B, a laparoscopic colostomy? A, laparotomy, mini laparotomy, left lower quadrant or transverse incision. B, laparoscopic colostomy. Male. Male. Male. Yeah, we'll talk about some of the nuances of cloaca colostomies, but we'll just basically. Male. Next. So who do we got? What do we got? So most people are doing an incision. Does anyone want to comment on your laparoscopic approach? Okay. Anyone? Dr. Teitelbaum snuck in. You're hiding back there. We're going to get you up here soon. How's Michigan football these days? I can tell you. I will just tell you, full disclosure. So we both have freshmen at Michigan, even though I'm an OSU faculty. But my son just texted me last night, and he said, basketball season cannot come soon enough. Okay. Yeah, my daughter was shell-shocked when she said that, let me tell you. I'll do both, to be honest with you. But there are times when actually laparoscopy's not so bad in actually making sure. This is nice. I mean, you've got this well-oriented. Sometimes I've seen surgeons get disoriented, and you can. If you don't have a large enough incision, you're pulling up a portion of bowel that you think is good. But actually, laparoscopically, you can orient yourself a little bit better to know exactly where you want to grab a proximal enough piece of colon. And sometimes people who are going in with an incision, I think, can go too distal, and they think they're in the right spot. I agree, and I think it's a nice, the other nice advantage of laparoscopy for colostomy is you can grab exactly where you want to grab it, pull that loop out, divide, clean out the distal meconium, and then take the mucous fistula and pass it, and then you have no skin bridge. So that's a very nice technique. So you have no bridge of skin that's sutured closed between the two stomas. So you don't have that wound complication. And some of our stoma therapists in the room, I'm sure, would be happy to lament to you all the problems that we as surgeons create for them in our colostomy creation. And one of the biggest problems is the space between the two stomas. Of course, then there's the option of a loop stoma, which has no skin bridge. Any opinion about a loop stoma, Paola? In an ARM? In an ARM, it should be avoided because then you might have some spillage of pieces in the distal part. Although we have learned from around the world that they still do it in many places. Apparently with low incidence of complications. Probably because now there is a tendency to repair the children earlier than in the past. So you give less time to have complications. Michael, do you want to make a... Well, let me just say, Michael and I just... Maybe Michael can comment. We just reviewed this. I still personally believe that loop stomas are theoretically problematic because they prolapse and they theoretically allow for distal spillage. But? But there's a paper from Toronto looking at loop stomas. And we recently reviewed ours as well. And there is no difference in complication rates regarding urinary tract infections. What's interesting is no matter what stoma type you choose, there's a high complication rate such as prolapse. But it doesn't seem to affect the urinary tract infection rate. What does affect that is if they have vesco-uretoreflux or neurogenic bladder. Yeah. Shemal, you want to add anything to that? As we go about reviewing things, sometimes what we look at is biased. If you look at the later series that we have in terms of the patients from the late 80s and 90s to now, many of the patients who have no failure have right upper quadrant incisions. And so the question is, as you point out, is it a comorbidity from neurogenic bladder? Is it reflux from metabolic acidosis and that long distal limb? I think the answer is that there are probably cases where a transverse or a loop stoma is not going to affect the patient when the fistula is very small or when the bladder is normal. There's no other type of problem. But I think to say that it's safe is probably not the best thing. I personally will be continuing to divide my stomas. But there are people arguing about loop stomas. And it's hard to say there's a lot of evidence that's against that idea. Theoretically, they allow spillage, but maybe it's okay. And I think Paola's point is probably very important. Just do the repair early. I think a lot of the data was patients were being kept until 6, 12 months of life. And if you do it within 3 months, there's less time for not only urinary complications but also stoma complications because as soon as you do the PSARP, you can get rid of the stoma. So here are some landmarks for you. Thank you. Maybe Brenda could give a comment. Would you mind giving us a comment about where you'd like to see the stoma created based on this picture? And then we may end with this point because we need to take a break. So looking at this abdomen and the landmarks here, obviously you want to not stay too – place the stoma too low. How about here? Mark, can you use the mouse to point the mouse on your – Oh, sure. Sorry. That way the – yeah. Thank you. How about a stoma here, Brenda? You like it? I would not really prefer that to happen. How about here? Not that either. So – Here. That would be a little better placement in my opinion. Staying away – a little bit away from the umbilicus so you could come down a little bit if you needed to. But giving some room for an appliance would be great. I always mark these landmarks and try to find the flattest point. Yeah, Jack. So the flattest point, the part that's going to have the least trouble with the umbilicus is the umbilicus. So what I usually do is a lower transverse incision, and then I bring an end stoma out right through the umbilicus. And the other advantage of that is that when you close the stoma, you end up with a scar in the umbilicus, and it avoids one extra scar on the abdomen. So the international audience agrees with the gentleman with the mustache. They love his article. It's Movember. All right. I'm just going to flip through and make sure we don't miss anything before we take our break. Here are some pictures. There's the mucous fistula made small. There's a lovely loop prolapse. There's a transverse colostomy loop in the epigastrium. Here the surgeon flipped where they wanted to put the stoma and caused a lot of perineal trouble, which is not hard to do. This is a colostomy opened to distal. This is really something you need to avoid. You really need to make sure you have lots of distal bowel for the pull through. And there's a loop that allowed for distal accumulation of mucus and stool. Brenda doesn't like this one in the crease. And we'll talk about this with cloaca. So let's take a break and reconvene at about... I just can't be a man. I can't be a man. I can go. I can be a man. I can be a man. I can be a man. I can be a man. I can be a man. I can be a man. I can be a man.
