All right, uh, appropriately so. Colorectal is our last session before lunch. So, oh, hey, everybody. Yeah, so we're the only thing standing between you and lunch. Don't worry, we have like about an hour and a half of material, so it'll be fine. Uh, hey, let's, next slide. Where's the little magic ring here? All right, backwards, forwards. All right, OK. And, uh, all right, so, hey, Nelson Rosen from Cincinnati Children's with Aaron Garrison and Annie Lewin with me here. We got, uh, Elizabeth Speck, Jamie Harris online, and we're excited to, uh, give you guys an update and our intention is to talk about some things. You know, these are roughly the topics that we're gonna hit. And this was a real challenge. And like I heard earlier, like if you want to go into like the real innovative, you have to look into adult literature too, and we do. However, there isn't a ton of earth shattering stuff that's occurred in the literature in the past couple of years that is really game changing that we could put out there. So, but I did think that it would be a really good exercise. To hit these points because some of these articles are still quite fresh, not all of you may know about where things have gone, and now that things have been out like a year or two and people started doing them back in their home places, you can see like maybe it's not exactly the way they talked about. So hopefully some of that will come out in the conversation and I think with that we'll move into. A clinical scenario and Jamie Harris, you take it away if you're online there. Hi, good morning. Everybody hear me OK. Excellent. So, thanks so much for the opportunity to present the colorectal update. Uh, our first case is one of my favorite calls to get from the NICU, actually. It's a term female infant. She weighs 3 kg and she's got a recto vestibular fistula. Um, they've done the workup in the NICU and besides her interectal malformation, her bacterral workup is completely negative. Uh, so that brings us to our first clinical question that we're going to pose to the, um, to the group here, which is, what is your next step? Um, you can do dilations only, dilations and elective surgery after discharge, a diverting stoma, or a neonatal PAP. Um, and I think we can hopefully do our poll everywhere. Uh, what do we got there? So on the poll, it looks like, uh, dilations and then delayed PSARP is leading by a substantial margin over a neonatal PSARP making a little comeback there. All right. I think as we go through the answers, we can agree that doing dilations alone is probably not the correct management of this for a number of different reasons, but certainly dilations initially will allow for decompression, um, and I would recommend only dilating to a 7 Hagar to decrease the potential scarring along the track for future PAPs. Along those lines if the fistula is decompressing a diverting stoma often not necessary, and those are our two other answers that it seemed like the group, uh, kind of felt similarly with, which is, um. Violations in a later surgery after discharge or a neonatal period uh PARP and a little bit of controversy in these, but there is data to support either of the next steps. Um, so kind of the next question is, you've calibrated the fistula, you're able to get your 7 Hagar dilator, and the baby's decompressing well via the fistula. And so it brings us to the next question. When are you going to do your PSARP? Are you doing it ASAP before the baby leaves the NICU at 1 month of age, 3 months of age? Do you have a weight requirement, or are you going to do it later because you're on vacation? What does our panel think? And what does the group think? Yeah, it start talking. Um, I guess so this is a 3 kg baby, a term baby. I would rather do at 1 month of age just because you do the, if the patient can be decompressed with the rectal dilations, the, uh, the fistula dilations, and then just so the baby can grow a little bit bigger, I would be more comfortable at 1 month of age. I, I think Nelson and I, um, disagree a little bit on this. He likes doing them up front. I hope I'm not putting words in your mouth, but. I like also getting them a little bit, um, older, let the babies go home, um, and then so somewhere between B and C, I think makes, makes sense to me. Yeah, I guess I'm old school on this. Like, I like to, you know, I'm fine maintaining patency. And I think Jamie, you brought up a really important point about that if you're gonna just try to keep it open, you just have to keep it open enough for like little soft, mustardy poop to be able to make it through there. So there's no reason to drive. That dilation like up big but uh I like to do this operation like when I can, it doesn't have to be the next day, the day after, you know, you can uh get it done, but I like to get it done on the neonatal uh admission provided that there's no comorbidities or that the kid's not like, you know, 1.2 kg or something. Yeah. And I think that's kind of our sort of Gestalt in the group is, you know, I think both are safe, um, and understanding kind of the surgical principles of the PSAP and this operation can be performed both safely in the neonatal period or after the patient's discharged. Um, there were two kind of important studies that both came out in 2021, sort of looking at this. If at all looked at female perineal or recto vestibular fistulas, this was a Nisquip study. Um, and it defined early repair as less than 7 days delayed 6 to 86 weeks to 8 months. And again, they found no difference in those groups between, um, wound complications, re-operations, and readmissions. And again, there was another study in 2021. This one looked at 30-day outcomes, and it was neonatal versus delayed anoplasty in a multi-institutional retrospective study through the PCPLC. They had 164 patients and their early repair was defined as 14 days versus a late after 14 days, and that on average was about 172 days and the neonatal repair was about 3 days. And again, their primary outcomes we're looking at 30 days. There were more in the delayed group, which is kind of what goes along with what our polls showed. There's about. 81% in that delayed group versus the neonatal group, but what they found was that wound breakdown and dehiscence was the most common complication in both groups, and it was about 5% in delayed 6% in neonatal, but there was no significant difference in any of the incidents of postoperative complications in those two groups, so they concluded kind of it was the same thing. Um, this slide here shows just kind of the discrepancy in terms of delayed repair, um, from a couple of months to multiple months of age, and so there's no kind of right timing of delayed repair, but, um, significant variation in the literature. Um, I would propose a couple of downfalls of a delayed repair. Um, one, like we talked about, that theoretical fibrosis of the fistula tract potentially making your dissection a little bit more difficult on the PSARP, not, um, impossible, and if you're staying at that 7, I think it's probably OK. Um, the other is if you're not completely decompressing it, the rectum can get distended and make it a little bit technically more difficult to perform that PSARP. Um, and then if you do the neonatal repair, they don't require the readmission for their surgery, and with some of the social determinants of health that have been looked at recently, it's not always easy for families to make that trip multiple times for the readmissions, and it's a long distance and can also be pretty expensive. Um, and so, uh, kind of want to open it up to the rest of the group. Any other thoughts on terms of these timing of PSARP and Nelson kind of mentioned size cutoff of these babies. Is it 2.5 2 kg 1.5 kg? Yeah, how many people are doing primary P SAP in the neonatal period in this room, like before discharge from the first NICU stay? Like a very few, like I'm a, I'm a dinosaur with that, which is, which is a little bit bizarre because like, you know, in surgery, we all do it younger, do it smaller, do it quicker, get it done, you know, we always try to do that. So moving to a delayed approach is actually really interesting. And I think in many ways. mature because if you can, if you're more comfortable later in the course, maybe the patient's a little bit bigger, maybe you have less complications from the way you do that operation and talking about vaginal wall injuries and things, the things that people say never happened, except they totally happen. So I think that's important. Yeah, Justin, yeah, I think the data is so mixed. I think it's a decision to share with the family because I just don't think there's strong data one way or the other, um, but if it's a trustworthy family, I think they're fine to send home, and I would agree, don't dilate them to like a 12 like a crazy person because that will make it way harder to do the surgery later, like just enough to let stool go through. Yeah, and it depends on how tight they are. Like we use this term dilation, like we're stretching something that can be stretched up to a certain size, except that's not true. Like when they start out like a pinhole, it's a controlled tear. So if you're doing a controlled tear, get it to be just as big as it needs to be, and then leave it alone until you've actually fix it. Yeah. Well, excellent. All right. Let's see, next slide on that. All right, we're moving on. All right, well, that was a good lead in. So now, uh, how, how is everyone performing their anal recoplasty, the classic PSARP, uh, are you taking an anterior approach? What about the perineal body preserving PSARP, or maybe you guys have some other, uh, way that you're doing it. Let's start the poll in there, Nelson. All right, let's see what the poll says. Interesting. You know, and the one other that like, you know, I was in a presentation that somebody gave not that long ago talking about laparoscopic approach for vestibular fistula, you know, and like, of course my brain exploded and still hasn't recovered, but you know, like if somebody would want to defend that, like knock yourself out, but what, what did that poll show? We missed that I didn't see the final results. It was all right, so, uh, most people, oh, a lot of people doing perineal body preserving PsAP1 out on this one and the classic PSAP, a close second, and there's still a few folks doing the, uh, anterior sagittal approach. Well, there are some data around the perineal body preserving PARP. I think it's our next slide. Um, I think the first publication in 2023 where they demonstrated at the one-year follow-up, no dehiscence, no prolapse, and only 13% of the patients required revision of their anal stream. and 2/3 of the patients went home on postoperative day one, which is, uh, faster than my most recent piece for sure. Nelson, I think you're a big proponent of this. Tell us, tell us your tricks to, to not injuring that anterior or posterior vaginal wall when you're coming around the anterior part of that rectum. Yeah, that's a, that's a great question, uh, because when this came out and, you know, and it was, this was really first described and first done by Belinda Dickey. And, uh, you know, when I like you see an article like this, you're like, oh wait, wow, wait a second, because I, for vestibulars, I was basically doing giant Psarps, you know, from like above the coccyx all the way down uh to the fistula and so started to do this and like really picked up on that this is basically like the same thing one would do in a bulbbar fistula but applied to a vestibular fistula. So I do it and you know it's, it's hard for me to, to think about how this lateralizes. Because when you're a PAP surgeon, that's what you do when you're used to the concepts of working, uh, you know, really religiously about cleaning up the lateral planes before you come around the front. And I think that's the, the, the biggest thing that, but like before that, I think it's perfectly fine when you're starting out doing these to go a little bit into the perineal body on the front and a little bit behind it in the back, you know, just to make sort of like. Your incision that you're gonna work through, not the tightest pinhole incision that you could make. You think a little bit bigger to start with, still keeping a perineal body largely intact, but then like that classic PAP thing of really get to the back wall and mobilize the sides and really mobilize it well, staying on that bowel wall before you decide to come around and then very, very slowly and meticulously making it through that common plane. Um, but this is one of the big things that like the, you know, people who do this, like very frequently have a different comfort level than those who don't. So, like, uh, you know, I'd like to hear different experiences. Why don't, uh, let's go. Yeah, I, I, I like it mainly for the post-op recovery and that you can let them go home earlier. You're not sweating that breakdown in the, in the perineal body, which is like the biggest concern. And remember, these kids, when I was a fellow would stay in the hospital for a week on TPN. So it's, um, it's come a long way. I think it's worth doing just for that alone. But I do think it's harder than opening it all the way anteriorly and you have to. You know, like, I think Doctor Harris on the call said in our planning session, like you can convert this to a regular PA, you can try it and you can always extend your incision if for some reason you're not getting the visualization that you, that you think you need. Yeah, I think that's pretty key is like, if you are lost in the anatomy and you need to make a bigger incision and convert, it's super easy to do that. And if you're not sure where that anterior wall is and where the vagina is, just convert to a standard PAP and go from there, um, which would make you do it safely. I think I agree with both my experience as well. Oops, sorry, go ahead, Annie. I just agree with both Doctor Garrison and Doctor Rosen. I just remember doing one of my first perineal body sparing PARP when I was in fellowship back at Saint Justin, and we just took our time through the small incision and the patient did really good afterwards, but it was a little bit more confusing than doing the classic PARP for these type of cases. I think that point about time is important too. In the second article that came out with that, that was one of the factors they looked at was the time of the operation, and it actually doesn't add any time in that small experience that they had from a standard P art to a perineal body sparing, which I think is important in these neonates to minimize the anesthetic time that they have as well. Yeah, how many folks in the room are doing it? Like a few. I, like I can tell you if this is the kind of thing that you do, it's a joy. It's like become one of my favorite operations to do. All right, uh, let's keep going. I think it's important, uh, just to mention, you know, kind of to your point, Annie. I've, I tried it one time. It was the fellow's first operation doing a PSA week one of fellowship. We ended up extending our incisions so that we could both feel comfortable with the, the anatomy. If you've never done one, you know, it, it, it does, it does have a little bit of a learning curve. You want to make sure you're comfortable doing it, make sure everyone can see what, what they need to see to do this safe. So I think the end result is, is do what you're most comfortable with, but I, but I like the idea, Nelson, of pushing the envelope. Yeah, like you suggested, and I think if you bring up a really good point, and it reminds me it's sort of like the anti-PAP because the PAP was we did an abdominal perineal sacral incision and that was Doctor Pena saying, well, let me make it bigger. Let me make it bigger until he had the whole thing flayed wide open. And this is the opposite of that. So there's nothing wrong, by the way, with, well, I'll make it a little smaller. I'll make it a little smaller. And as your experience grows, the next thing you know, hey, I did it within the confines of the muscle complex. So I, I think that's a great way to do it. Uh, what about, OK, we got another pole. All right, do you guys do, uh, anal dilations after your PA? Yes, no, maybe a divisive topic, very divisive. This is why we included this. We, we need to know what people are doing. About how many dilators in the room? Show of hands, dilators, routine dilators. Wow. Nelson, keep your hand up. Yeah, my hand is up, up. All right. And that way, that couldn't be more even, that's like kind of 1/3, 1/3, 1/3. It's like the All right, I, I think there are data around this. I think we have to start to question this paradigm, you know, on the next slide we'll show you that that there are data that support, um, parental anxiety, PTSD for both the patients and the caregivers, um, post traumatic stress symptoms in the families. This is a big stressor just like enemas, um, uh, you know, when you have to do something to these patients or the, the. It, it, it, it turns out they're not patients, they're, they're the families, it's their kid. They're at home, they're having to dilate. Preoperative dilations seem to be a little bit more tolerated than the postoperative dilations. I think families are worried that they're going to hurt their babies and they're going to hurt this repair. And I think we have to question this paradigm and, and how we manage postoperatively. The, the next slide shows, uh, single institution review. Uh, if you want to click through that, uh, Nelson, I think the next slide shows, uh, I think this one's out of Spain, and the gist is they followed sort of the historical Pena anal dilation protocol that we've all heard about post-op week two you start dilations, you try to get them to the neo anus size that they need to be, and they just. In the study was that the kids who were smaller than they wanted them to be at that initial um appointment, they got dilations and those who were adequately sized according to the table on the bottom by age, they didn't get dilations and uh on the next slide another single institution review in kids under 2 years of age that shows. Uh, at least the way I interpret this data, half and half dilations versus no dilations, 2 kids in each group had to have a re-operation for that neoanal stricture, and about 15% of the kids required a Heineke Mkowitz, uh, strictoplasty, which, by the way, is a nice backup plan if you, if you're not going to dilate, you at least have a surgical option that's pretty minimally invasive. Uh, pulling the group up there, uh, Aaron and, uh, Nelson, what do you, what are you guys doing? dilations, no dilations, Jamie? Um, personally, I think with, uh, neonate, we dilate, uh, or I dilate if, if it happens to be an older kid, a redo and they're ambulatory or old enough where I think that's traumatic for everybody, then I'll, I will hold off. I also usually dilate um for neonatal cases. One of the things when these studies came out um for the strictoplasty, one of our concerns in Canada is that we don't really have easy access to elective to schedule elective cases and so this is why we prefer to dilate all of these cases up front. Yeah, uh, like, uh, we could go on on the dilation. I'm gonna, we're gonna fly past it because, uh. We got lots of stuff to hit in the last 10 minutes, but, but thankfully there is a big PCPLC study coming out looking at this because a lot of this study is quite flawed, you know, and I flated at the last PCPLC meeting. I don't wanna rehash that, but well, let's, let's keep moving forward. All right, new scenario. OK, so next case you have a term newborn newborn patient with delayed passage of meconium, no other anomalies. You do a suction rectal biopsy, which is diagnosis for Hirschpring's disease. First question, when would you perform the pull-through procedure? Before NICU discharge at 1 month of age, at 3 months of age, or would you rather perform a primary ostomy and delayed pull-through in this patient? OK, so. 3 30% would perform it before NICU discharge and 39% at 3 months of age. So our consensus in the literature, there's not really an evidence of the good timing to perform a pull-through procedure. There were some studies that were, uh, published. This was a study from 2021 from PCPLC. It's a retrospective study that compared outcomes of neonatal pull through versus delayed primary pull through. And so for the neonatal group, the median age at surgery was 11 days versus 98 days for the delayed primary pull through, and they found no difference in terms of preoperative enterocolitis, postoperative enterocolitis, and fecal incontinence and full up. Um, I just have a question for the panel or the audience. When do you usually perform the pull-through procedure? I tend to do it a little bit more delayed, and it's because I like the families to demonstrate that they can do irrigations at home. I think that's such an important skill for them to have. And the rate of enterocolitis after pull-through is obviously not zero despite our best efforts. And so I think if a family is really competent with irrigations, they're going to be much more comfortable after a pull-through doing those irrigations, and that's what's going to save the baby's life. So, I delay at least 1 month, sometimes 3 months, depending on when the family can get back, and that's assuming it's a reliable family that is good at irrigation and can come back if they need to. Steve, I completely agree with that. Uh, the family who's really good at irrigations will save a baby's life, and, and I can't tell you that the outcomes are any different with regards to incidence of enterocolitis, but I can tell you that they start treatment before they ever even call me. I'm curious. I'm curious if whether you, um, whether the length of the, uh, a ganglionic segment affects the timing of your pull through. Good, good question. Absolutely. I, I can tell you from experience what I've learned in the kids who, who are, are not able to go home because the family can't ever do really good washouts. They usually have a longer segment, and that pushes my, my hand to do something while they're on the inpatient side, which is important so they don't go home and then they're not adequately decompressed and they end up in the hospital with enterocolitis or a perforation, even worse. I, I would say I used to do them all in the NICU and I've kind of shifted to more delayed for the points you made. I think it's good for the family to do irrigations. The surgery is frankly easier when they're a little bit bigger, and some of the data seems like it suggests maybe better continence in the delayed group, but the studies have all been underpowered. But I think it is still shared decision making with the family. I think the last one, the last primary pull through I did, the mom was in prison, so I didn't think that was a good situation. So I did it before they went home from the NICU. All right, we got to keep moving. Um, and so at the time of your pull through, would you inject intra-sphincteric Botox, um, at the same time? How many in the room do Botox at this, at the primary pull-through? Yeah, when I remember. OK, so from the poll 50% would not inject Botox at the same time of the pull through, and then the rest is either yes or variable. Um, so we as a group would inject Botox, um, at the same time of the surgery. There was a multi-center study that was retrospective published in 2022 that evaluated the use of Botox in patients with Hirschpring disease, and 24% of them received at least one Botox injection. We did a study at Cincinnati Children's, which is a perspective study from 2020 to. 2024 and the results are not published yet but what we found is that within the 31st days after the pull through procedure there's a decreased risk of enterocolitis so I think it was 50% versus uh 30% and the other thing that we found is that patients that received Botox at the time of their pull through had a higher risk of a diaper rash in them. Hm. Higher risk, yeah, 60% of them. Um, and then another question for the audience or panel, what kind of botulinum toxin are you using? What, how many units are you using, the injections frequency, and then do you use ultra ultrasound guidance to do your injections? Yeah, we, we, we start out with Botox and like it took me a while to learn that there was like multiple brands and we only change when there's like some type of, uh, you know, allergy or some issue with one particular one. But the, the number of units is challenging because there's no evidence on it, you know, like I was taking my guidance on like the early work, like an early paper from Sam Nerko's group in Boston where they took, uh, they did like a, a per kilo up to a maximum of 100. But then tapped out there, uh, and then, but when I came to Cincinnati, everybody was doing like 100 regardless of the size of the child and that didn't make much sense to me. But then again, I've always been underwhelmed by my weight-based dosing, so, uh, maybe we need a little bit better data on this. I definitely use an ultrasound, but that's just my practice. I use the ultrasound for everything. Do you find it more practical to use the ultrasound so you know exactly where you go? Yes, ma'am. I can see every layer. I can see where the needle is. I can see how much I put where and where it diffuses to. It's really nice. But you have to be comfortable using the ultrasound, otherwise, uh, it's kind of useless if you don't know how to use it. You have to, you have to have it available, yeah. Spec, do you, when you inject, do you go in through the anal canal, or do you go in the outside through the on the outside, yep, on the outside, and then I can follow the needle in line and you can see the internal and external anal sphincter. You can see the superficial external anal sphincter. You can tell, uh, you can see the pubil rectalis sometimes. Great. Um, last case, and I don't think it'll be long because we just wanted to, we've been sorting through a couple of challenging patients with ARMs and, uh, Trisomy 21 at our center. So I thought it would be a good review kind of of, um, which patients to screen for this and, and maybe a discussion about looking at fistula tissue. So, um, I guess let's start with the poll. What is the rate of Hirschsprung's and ARM? Together That's right. So it looks like most people, 2%, 2 to 5%, and I think A is probably most correct based on a few of the papers, but there are some that show as high as 3 or 4%. Um, Doctor Pena's paper looking at his series was about 2%. Um, and then, um, in a recent paper that was less than 1%. So it's still, uh, it's very rare, but it is something to be aware of as you're working through some of these challenging patients. Um, next slide, this is, uh, two learning points that we wanted to take away from this paper, um, also from the PC PLC. Um, they, at a single center, looked through all of their rectal fistula, um, specimens that they took down, uh, that they did during the PAPs, and just to kind of establish what is a baseline for ganglion cells when you, when you send that tissue, because that's not physiologic tissue, right? It's fistula tissue. So, um, they found ganglion cells in 90%. And, um, hypoganglionosis or absent ganglion cells in the rest. And I think it's important to know that just because you don't see ganglion cells in fistula tissue does not necessarily mean it's Hirschsprung disease, um, although it may warrant further workup. So, to, um, add to power for this, uh, study, they looked at the PCPLC database and there were about 1700, um, patients in this, where they looked to see, um, who had both. And, um, the main thing to take away for, for trainees or for those who need a reminder is that the patients that tended to have both diagnoses tend to have chromosomal anomalies. So trisomy 21, palierchian, um, and some others. So, uh, on the, looking at this the other way, if you have a complex patient who's really not responding to laxatives, to enemas. And they have those, um, those diagnoses, then that's someone that we think is worth, uh, working up. Any comments, questions, or? About this, hey, do you think, Aaron, like as we move in minimally invasive PAP and, and smaller incisions, and one of the things that comes with smaller incisions, by the way, is the concept of how much do I actually need to mobilize, you know, and as an early PARP surgeon, I was taught to mobilize. To like completely like all the way up from like bottom to mouth practically so that you could move this thing anywhere and there was as minimal tension as possible. But like the the older I get, every little flick of the bovie, like that's a blood vessel, that's a nerve, and that's another week in the bowel management clinic and, and I, I think like that. And, and that's gotten me to say like, hey, maybe I don't need so much mobilization. Maybe I can stop right there and maybe I can resect even less just like the pure fistula. I knew you're gonna use that line and I was gonna say it if you didn't. So, um, I, I think you have to mobilize what you feel comfortable mobilizing, but I think more importantly in regards to this topic is talking to your pathologist about where they're looking at the specimen. So, are they actually in the deep portion, full thickness bowel and not in that distal fistula tissue and that may be worth having a conversation with them as you send the specimen. Good point. 2 seconds left. And we hit that one out of the park. Thanks, everybody. All right. Um, we are gonna take a break.
