We're gonna go on to our next session. Is everyone excited about colorectal? I know it's the best. OK, we're gonna do updates in colorectal pathology. We have, uh, two experts. We have Caitlin Smith from Seattle Children's and Julia Groski from, uh, Laurie Children's. So here we go. Awesome, thanks for having us. Who has the slide thing? There we are. Oh, cool, um, so I guess today we're gonna talk about some things that, um. Uh, are old but also new and having new life bred into them. Um, we're going to talk about anal dilations following PSARP, um, Botox and Hirschberg disease, and also perineal body sparing PSARP. OK, we have a 3 month old male with a history of rectal, bladder, neck fistula presents 2 weeks following a laparoscopic assisted anal rectoplasty. He has a colostomy and a mucous fistula. What is your next step in management? I think this is Caitlin. Yeah, so, um, I guess I'll just start by asking who in this room would do dilations after a PA for any patient, any age. Any type of repair. 1 person, 2 people, 3. Any age, any surgical approach. All comers get, get dilations mixed bag. OK. OK. Do we have any poll results coming up? Cool. What are the colors? OK, I'm gonna give it a little touch more time. 50/50. Interesting. Yeah, that's kind of where I thought it would end up right there, but we'll give it a little bit more time. Um, So yeah, what do you do? Well, I used to be an A, a protocol driven Hagar dilation sort of gal, um, and over time based on actually this day that we're about to present. Um, I realized that it wasn't always necessary and that it was in for many families, a really big source of stress. The worst thing that they would hear is my baby is born without an anus. You're gonna do this operation, but I saw online that they're gonna have to have these dilations and they would freak out about it. So then, based on this data, I now do it um, not routinely, but, um. Per patient. I decided based on sort of patient presentation and I usually do a dilation only if necessary. Yeah, it's kind of interesting when you talk to the families, I think there's a lot of, um, or maybe historically we didn't, I don't, I don't want to say that people didn't pay attention because that's not the case, but I think that, um, we're listening a bit more to the parent voice, um, in terms of, especially as you're saying like related to these problems and the stress that it causes the family. And the quality of life changes that it can really um create and dilations are really a source of stress for families, um, and, and parents feel like they're hurting their children and, um, you know, they feel, yeah, or doing it wrong. There's a lot of anxiety, um, and so we've sort of moved a little bit away. This, um, actually was from 2021, but, um, it's a smaller series out of nationwide, um, and yeah, you have the clicker. Cool. So, um, single institution, uh, prospective randomized controlled trial, um, and the important thing is that these patients, the decision was made like at the beginning. So you couldn't change stream partway through, which I think is really important. Um, they were all primary, um, patients, so no redos or cloacas were included. They all had a diagnosis of ARM. Um, and they were all, um, relatively young, so younger than 2 years old. Um, the length of follow-up was 12 months, um, and the PSA was, uh, performed at an average of 5 months old. So doing dilation somewhere between like the 5 and 6 months age on average. Um, and a stricture was defined as a Hagar 10 or less, which is kind of what that picture denotes is the, um, the anoplasty. Um, so there are 2 arms, um, 25 patients in each arm, um, and, um. Only a few re-operations and strictures in both groups. Um, strictures were, there were 3 strictures in the dilated group and then, um, 8 in the non-dilated group, but I think what's important is that even though the strictures that overall, um, the number of procedures were relatively the same, um, so a lot of the patients who had, um, strictures in the non-dilated group ended up getting, um, The, uh, they had the, uh, strictureplasty at the time of their colostomy closure. So, they only, only 3 of those patients actually got a separate anesthetic. So I think that's some of the concern with the non-dilation group is that they get, they're getting extra procedures. Doctor Kotigal has a question. Do we have any sense of the impact of doing a strictureplasty or a nanoplasty on long-term continent? Like, I think one of my hesitations around saying, OK, great, we're not going to dilate, and we just move to a place where if they end up with a persistent stricture, they get a nanoplasty. But how does that impact continence? Yeah, really good question. I think. I think that we don't, I, I personally don't have a good sense for that. I mean, you're not technically moving the anoplasty or anything like that. You're breaking. I think it's a strictureplasty. It's a strictureplasty. I think the important thing is to know the the type of stricture that this is appropriate for. So it has to really be like a skin level stricture and not anything deeper. And if you're talking about a longer stricture or Like, uh, anything that's just below or deeper, I guess longer than just the, um, the skin level, you really shouldn't be doing a strictureplasty. So I think that's why we may not, we have kind of like a mixed impression because, um, I'm not sure that all strictureplasties are, are created equal for the same problem. Like patients with longer strictures really should be getting redos, um. So I guess I would ask, I didn't do strictureplasties for a very long time and then I was dilating routinely and I realized that there was this other option. Um, are other people, is this like just us who are doing strictureplasties are other people doing them? Let's do it. We got one, well, yeah, that makes sense. That tracks. Interesting. It is actually a very, very satisfying operation for a skin level stricture. It takes about 20 minutes. Um, those kids can usually go home and it's just sort of cutting that stricture and, you know, making your 10 into a 13, and the babies really tolerate it very well, and then I do not dilate dilate after the strictureplasty. It's been a nice option. Yeah, so usually what I do is I talk to the family about the options and we're talking about doing a pea syrup for babies, I would say generally they tolerate dilations pretty well, so. Um, you know, and they're not as bothered by it. It's not as problematic for the family, but I still talk about all of the options. But anyone over like getting, especially close to 12 months, usually over 6 months, I just, I, I don't dilate, I don't tend to offer dilation to those families. I agree, and I sometimes think when you're getting older than 68 months, sometimes those dilations are actually not that successful, and maybe you're gonna be dealing with the stric anyway, totally. Yes. Publish data, please do. Like I was surprised to see the audience not doing routine dilatations. I was very surprised to see I was kind of the only one raising my hand. If you ask this question in Chile, no, I think that we have read that we have read that that JPS article and it's a good quality, good authors, good methodology, etc. but no one would dare to chan challenge the status quo because of certain author has dominated the scene for so many years and no one. No one would dare to challenge what he has said. And so what I'm saying is please publish that all these children that you're treating are just doing well just like this article said, because it would be great for the world. Yeah, that's really good information. I would, I, I love this feedback. I would say that when I travel, when I've traveled and, and operate as well, I've noted that, um, all those patients are still getting dilated also. Um, uh, I, and there are plans to, there are plans to, there's a going to be a prospective observational trial through the pediatric Colorectal and Pelvic Learning Consortium, um, that will be starting hopefully soon, um, in the next, uh, few months here to sort of prospectively follow all these patients, um, and describe, um, the findings and, and sort of in like en masse in a much larger way. Is your follow-up different because we used to have to come back every week and I don't know about you, but all babies didn't tolerate my dilation so I mean and are the families happier? The families seem happier, um, and, uh, I personally see them anywhere from I see them about 2 to 4 weeks after the operation. Um, whenever I can get them into clinic in that kind of time frame, and then I size the anoplasty in the office, and when I size it, I'm like, OK, like, here's the time where we can talk about what dilations look like. We can do them or not. This is what might happen. You might need to get a strictureplasty. Um, I've talked to the family before the surgery about dilations as an option, so they're not surprised, but if it looks really good, I will skip it. I will skip the dilation. I agree, yes, and I don't usually even really discuss dilations if they have a colostomy because I know they're going back to the operating room at some point for a colostomy closure, which is a perfect time for a strictureplasty. Agree. Is there one? I think there's one more slide there that shows something. Maybe. Yeah, basically, the, the outcomes were equivalent, um, and so just consider, consider, you know, seeing patients closely in follow-up, but not necessarily dilating them, um, knowing that there's like a very easy day surgery, um, backup plan, Doctor Holcomb. Uh, so, uh, this conversation is really new to me or the concept is new to me since I grew up in the era of, you know, making a small, uh, anal opening and then dilating it big. So I'm, I'm interested, uh, in this approach. My question is, do you feel like the stricture. Requiring a strictureplasty develops because you don't, aren't dilating them or because you are dilating them. You talk about strictureplasty at the time of colostomy closure. I'm just trying to. Sort out the cause of the stricture. Well, I mean, the stricture, stricture can develop in either group based on, like, at least in this small group. They did develop in the patients who were undergoing, uh, dilations as well. I mean, I think the same reasons for stricture that we are familiar with are still the cause here. So anything under tension, ischemia, all those sorts of reasons. Um, I think that we're, but we're not, there's this other group of patients that sort of creates just like a band where the suture anastomosis is that I don't know that we're great at predicting which patients will do that and which patients will be fine and not need anything, um. And so I think that's where maybe the, the difference is. There, there certainly is a group that will, you know, if there's ischemia or tension or things like that, that will need redos, um. Uh, still, um, but I guess that's sort of how I reconcile it in my mind. I'm not sure I have a good answer for you, I guess is the bottom line. I can't recall ever having to do a stricture of plasty, and I used to dilate them like, like Alberto taught us to do. So I'm just sort of, sort of curious about why you feel like the stricture develops. Yeah, I think it's, I, I, I, it's a good question. I'm not sure that I have a, uh, I mean, I, I just think sometimes it's that reaction between like the epidermis and the mucosa. It just sort of like creates this band. Yeah, I think there were some other questions. Yeah, I can, I can, you know, Tom and Mark know this, but Keith Jorgeson used to tell us if you don't dilate him at 2 weeks in the office, you're going to be hosed for a stricture. You got to do it at 2 weeks. So that's all I know. I don't know much about it. But I know in, in DC now, no one gets dilations and then they all come back in a month to get a strictureplasty or something. So, something is different. I'm not sure what's the right answer though. Yeah, I'm just gonna swing this the other way. The data you were looking at, we're talking to an international audience, I could interpret that data differently and be like, actually, I'll recommend dilations for places where access will be an issue and just coming back to surgery a second time, most people cannot afford that, which means it's the more reason why you should encourage dilation in that scenario, so. Yeah, no, I think that's a really good point is that um the care definitely, especially for this patient population, highly varies depending on your setting, the resources of the patients you're taking care of, and, and that's a really good point. I think that um there are definitely patients who could, you know, um, especially, I mean, in the United States or anywhere that cannot afford to come back to the hospital and so I do think, yeah, I agree that should be taken account into the decision making of which sort of arm. You're gonna fall into, I'd say some of this data suggests that maybe we could change that protocol that we've been all doing for years and years and years and maybe the twice a day dilations for however many weeks and months might be able to be teased down a little bit so that the family stress, which does seem to be a stress, but that we can sort of mitigate that a little bit by just modifying the um the dilation plan, but that's a great point. All right, let's move on. OK. Our second clinical scenario, you have a 5 month old male with diagnosis of Hirschsprung disease status post, uh, Swenson pulled through an infancy at your institution. Uh, patient now has had, uh, 2 episodes of Hirschprung associated enterocolitis responding to home irrigations. There's been no evidence of a stricture on rectal examination and contrast enema, uh, was done without any abnormality. Pathology reviewed and no evidence of a transition zone on pull-through. What is your next step? OK, so, um, your options, there are a gazillion options that you can do in this patient plethora, one of them being do nothing, um, but there's some big ones if you're thinking 22 episodes of enterocolitis, do a laparotomy, um, something's awry, gonna do, we do this, pull, pull through, maybe we'll try some Botox, who knows if that's gonna work, or a myotomy or myectomy, some sort of sphincter cutting procedure. Um, so while we were waiting for the poll, I can tell you. How do we know that it's, that it's pending? Is it going? Is it out there in the, in the universe? Oh, OK, OK. So when we are thinking about this patient, I tried to like set it up a little bit so they're not 2 years old. Their pull through wasn't 2 years ago. It was done at your institution by yourself or someone that you trust. You have pathologists that you trust so that you don't have to think about sort of there's other things that are causing enterocolitis. So you have to always do your due diligence with a patient who has had their pull through. Why are they getting recurrent enterocolitis? And one of the answers is you did a great operation, and they have enterocolitis because they have a poorly functioning sphincter with, with sphincter, which every baby with with Hirschsprung disease has by nature of the disease. So you wanna first make sure that it's not a mechanical obstruction. So let's see what are people doing. Oh yeah, good. My, my work is done here. Um, I would say a laparotomy with colostomy creation is not. I would say that would be a little bit bold for this patient based on the fact that they're responding to irrigations and home antibiotics, so that might be a little bit aggressive. I'm a redo pull through with no evidence of a transition zone pull through and you like your path that seems like that would be maybe not needed right at this moment. Trial of Botox. We'll talk about that. Some sort of sphincter cutting procedure used to be the answer. Maybe not after 2 episodes of enterocolitis, but maybe after 6 episodes or not responding to home irrigations. That was an option that we talked about a lot, a little bit more historically that people are doing less and less and less because of um how we're seeing how well Botox works. So that will be the next slide. OK, so here, may I ask, how do you, just because I'm curious, how do you do your Botox? Oh, thank you for asking. Um, I am 100 units because there's a wide variety actually. Oh, there is, yes, um, in reading every paper that was ever written on Botox in the anus in the last couple of months, there people are doing any amount of Botox in any aloquats in any number of locations, yes. So I am 100 units in 1 mL. I used to do 8 like semicircle. I never go anterior. I'm afraid of the genital urinary tract, um, but now I'm usually more like 34 in the dentate line. What about you? I do, yeah, I do 100 units, same 1 cc of saline. Often our pharmacy, which I found interesting when we started using more and more Botox, was, um, they wouldn't, um. Uh, one, they wouldn't mix it for me. Um, yeah, and then the other thing to know is that often they'll tell you the units, especially for the kids, are too much, but 100 units has been shown to be safe. So, but many pharmacies will tell you that it's above their, uh, weight-based whatever, and you shouldn't give 100 units, but I think most places give, give 100 units, and that's sort of what's been. Um, commonly practiced, and I think, I don't know if it matters if it's in one unit. Sometimes GI does it for different things in the pylorus, they'll use it a little bit less dilute or a little bit more diluted. I think the 1 mL is a nice amount of fluid to be injecting into the, um, anus, but, and then ultrasound plus or minus per your preference. actually Oh cool. Oh, look at you. Every time somebody would order it, you'd get a different thing or you'd get a different pharmacist or somebody. So we now actually have a Botox for Hirschprung's order set so that we just can order it and pharmacy knows how to draw it. And what is your order set? How much do you use? We use 100, uh, in 1 mL divided in 4. So 25 aquats per 4 set it. Got it, got it. Um, I do. I know that some sometimes the pharmacy will mix it. I generally mix it myself, but I learned this from Doctor Levitt that many times you like think you're taking it all up and, but you really, it's like sort of challenging to mix it and to make sure that you're not losing any, um. Yes, hi, get a little bit to the science of Hershey's colitis. Wanted to know your thoughts on, you know, the, the etiology. What, what do you think caused it? I know, you know, no matter what operation we do for Hirschprung's, we always leave a ganglionic bowel that, that bowel is at least the internal sphincter because we can't take that out. So I was wondering, do you think that that is creating the fertile grounds for intercolitis, or is it a mucosal disease? Is it immunologic? What, what are your thoughts? Well, I think that it is you in order to maintain continence, you want to preserve the dentate line and you have to leave a tiny bit of a ganglionic internal sphincter. And I think that, and I, this is because I think babies outgrow it because their external sphincter is able to overcome their internal sphincter, and as they grow and as they're potty trained, they generally, as long as there's no functional and uh like surgical problem and as long as you've taken out all of the a gangliononic, it's not transition zone, I think that they outgrow it. And I think that that takes time for their body to mature. I don't know necessarily, and correct me if you think something differently, that we have really found much in the way of mucosal abnormalities in the, in this, and we know that the, the proximal bowel hasn't worked before they're pulled through, and so there's something wrong with that motility, but I don't know that we have pinpointed the the actual pathophysiology. I think it's, um, like if you think, I, I think the all of the Hirschberg's physiology sorts of sets up the colon to act like a pond, right? Like, I mean, there's poor emptying, um, and motility issues and, uh, ultimately with, if you're not very diligent and about clearing the colon. Uh, it just has the ability for the bacteria to overgrow and, and create, uh, you know, illness in that setting. Um, and I think everything about the physiology of Hirschring's is set up for that. Um, I like to think of my Hirschru's patients as having a colon like a river rather than a pond. Um, so the pool, as long as the poop is flowing, they're irrigating, whatever, it's gonna go well, um, and we can hopefully avoid entercolitis. Obviously that's completely anecdotal and just like my sort of the way I imagine it, but all of the things are. All of the things that prevent enterocolitis are increasing and improving clearance of stool from the colon and not allowing for it to sit there stagnant. So that being said, something that's very interesting is we know that there's a significantly higher rate of enterocolitis in total colon or Hirschprung disease after pull-through. So when you take out the entire colon, they have a higher rate of enterocolitis than babies who have shorter segment and have all that colon to have bacteria. So that has always been a little counterintuitive to me. And also much higher rate of enterocolitis in babies with trisomy 21 or children with trisomy 21. We don't really have a good reason to explain that. So they're obviously in many different levels, and you can still get enterocolitis even if you are doing rectal irrigations at home. There are times when it's just not enough to empty that colon or the bowel. Do you, uh, do you guys believe you can get enterocolitis in a diverted colon? Never seen it, but I, I've never seen, I've never seen it. Mark tells me it happens, but I, I have never seen it. Maybe a tight. I have a very, that's the case Henri gave me in my oral board. And did you? No, I got it. I was like, what? Yeah, I, I think it's good to be aware that that colon is not clearing well, especially if you're in a diverted patient with Hirschprung's who hasn't had their pull through yet. If that's the scenario that you're talking about, I think that, um. Uh, I haven't seen it in practice. Um, we often have our patients who are diverted. They'll irrigate like once a week just to make sure, um, and ensure for that very reason though, just to make sure that they're clearing the colon of the mucus and the bacteria that's just naturally in there. I think theoretically if you had a tight, tight stoma that wasn't emptying well, then you could get enterocolitis. I've never seen that. You mean like a diverted colon though, that's like. Yeah, yeah, yeah, like an ileostomy, like a diverted colon, that's like, yeah, like in the colon it's diverted, yeah, yeah, I haven't seen it, but I have not seen I've heard the same thing, and it's, you know, frightening. You don't want to. There is discussion of whether or not when you, when you make the diagnosis of total colon Hirschprung at that time, should you do the colectomy because of the risk of enterocolitis, and I don't have a strong opinion on that. If we historically have left the colon in. We have, we have as well, and taken it out at the time of pull through a patient who was seen in an outside hospital. Who was eventually diagnosed with total colonic, and the surgeon did the colectomy at the time and it came back as not her. So I think that is a good cautionary tale. Thank you very much to not taking out the colon and waiting for the final pathology. Yeah, just to be clear, we would never advocate for anyone to do a colon resection based on frozen section that is, uh, extended. OK, so now I'm gonna go very quickly through these slides. There are really, really, really a lot of studies, and most of them are single institution. Most of them are retrospective. The thing about Hirschsprung disease, I think we all realize is that there's so much phenotypic variance. Like there are some babies with Hirschprung disease that are super duper duper sick when you first meet them, and then there's some that are just sort of like not pooping that well. So in studying the use of Botox in, um, post for post pull through enterocolitis, um, the data is not. Perfect. Um, even prospective data is not going to be perfect because people are very different. Different types of operations that you do, you have different levels of disease. You have different other comorbidities, syndromes, etc. um, so some of the data suggest that, um, prophylactic Botox is, does not, has not been shown to decrease the risk of enterocolitis. That being said, it has been shown to decrease the length of stay in patients who have been admitted for enterocolitis and also has been shown to potentially decrease hospitalizations in patients who present with recurrent episodes of obstruction or enterocolitis, so. I take this data. I mean, there's so much of it, but I take this data to, to suggest that maybe not everyone needs it at the time of pull through. We are studying that prospectively, but maybe that's not a necessary, but maybe there is a subset of patients who really do have some pre predisposition to enterocolitis, and using Botox as part of that treatment strategy would be beneficial. Should we move on to the 3rd question? Yeah, OK, yeah, very quickly. Oh my goodness, 1 minute. OK, here we are. We talked a lot of parents. So, we'll read this quick and then you might want to just make it, yeah, I'll just make the point. OK. 1 month old female in senior office with a rectal vestibular fistula. Back will, uh, workup performed. No other abnormalities. It's been schooling well with dilations. Oh, probably that would help. And uh so you could read it and what would you do as your uh repair? OK, so this is a very, uh, relatively new thing. There's a couple of papers that have come out this year, so I just wanted to bring, can you just flip to the pictures though, so that we can actually like, um, yeah, see them. Uh, so there's two variations of something called a perineal body sparing PsAP that have come out in the last 6 months, really, even one from Boston, one from DC. Um, there are slightly different techniques. This is the Boston, um, technique. It shows us slightly, um, I don't know if you can see well on the screen the silks that mark the sphincter, but there's a slight posterior sagittal, um, extension of the incision, um, and so really these are all, um, these techniques that are being described, um, are, um, all about sparing the perineal body and preserving long-term gynecologic function for our female patients. Um, the perineal body is really important for sexual function and. Obstetric, um, outcomes in the future and, uh, so preserving that is sort of like, um, come to the front of our minds, especially as we see more of these patients as they age and are telling us how their experiences are as adults. Um, so two papers have come out, um, like I said, I think one is 6 and one is 4 patients, um, similar techniques, but just something that's out there, um, obviously if you're not comfortable, uh. Uh, mending your technique, we wouldn't recommend that. We would only do this if you're, if you're, this is, this one out of DC is just an incision straight through the, uh, sphincter, just through the sphincter alone, preserving the perineal body skin in its entirety. So. Um, obviously you do which technique you're comfortable with, but it's just something that's out there and it's important to think about, uh, for our patients as they age. I think one thing that's nice is for the patient who is undergoing dilations and isn't diverted, that perineal body seeing stool right away is such a setup for a post-op infection that is like the bane of our existence. So I think this is a really nice way to avoid that. Awesome. All right, this was great. Um, I think if there's, I think what we'll do is just because we're out of time, so we'll just go ahead to the next thing. Um, Ryan, actually, why don't you just roll, roll the, the video and then we'll get set up here. So we've got ICG coming up.
