Update Course 2021: PEDS COLORECTAL CONSORTIUM CONCLUSIONS

we are now going to move on to another session, taking uh quite a turn back to a clinical topic. And by the way, let us know if there are areas that we should talk about next year, um, or if we need something, um, you know, twice a year if you want us to do this. But there were other topics that were brought up to us to talk about that we didn't have time to add to the schedule like physician suicide uh and and things like that. So let us know if there's other topics. So with that, I am going to turn things over to uh Dr. Rebecca Renti and Caitlyn Smith, um, who are going to explain this consortium again, not to surprise anyone. I didn't know something about it. Uh, I did not know anything about this consortium. So explain it and um, what this is Rebecca and Caitlyn and then give us some of the high yield points about what this consortium showed. Yeah, hi everybody. Thank you so much for the invitation and to be among this group of great colleagues today. I'm Rebecca Rentia, I'm at Children's Mercy in Kansas City and I do quite a bit of pediatric colorectal surgery and that's one of my interests and concentrations. and I'm joined by Dr. Caitlyn Smith. Hi there. I'm uh Caitlyn Smith. Um, I'm at Seattle Children's and I'm the program director of the Reconstructive Pelvic Medicine program here. Um, and thanks for having me, um, today along with Rebecca. So, we want today to talk about a few cases and to really hit seven high yield topics that have shifted the needle a little bit in the pediatric colorectal surgery world. And what's important here is that it's changing how we do management, how we view some common paradigms in the pediatric colorectal literature. And the first case that I wanted to talk about before we get into who the consortium is, is to talk about a concept that we all really know about anorectal malformation. And so we're going to start with a case of a three-month-old male with a history of anorectal malformation and a rectobladder neck fistula, who presents two weeks following his laparoscopic repair. Um, he has a colostomy and a mucus fistula, but what's your next step in management? So the first choice is do you begin with protocol driven Hegar anal dilations? Do you perform dilations only if a stricture is present? No dilations are needed or do you begin dilations following a colostomy takedown? So we'll give everybody a moment here to say what they think. And you know, there's a lot of background here, um, on really a history since Dr. Peña did one of the first P sars all the way to now that everybody in their clinic has kind of on the on the walk chalkboard in a handout is this Hegar dilator protocol. So, what do you currently do? And it looks like we have, we have a little bit of a split. Some of the word may have already gotten out on what's going on. And so our first hot topic is anal dilations following posterior sagittal anorectoplasty or the Psar. And this article was published in the Journal of pediatric surgery. It just came out, are routine anal dilations needed following a Psar? And what this study found in a single institution prospective randomized control trial is that they may not be needed. And so we know that there's a lot of psychologic trauma that goes um with families having to perform the dilations on their newborn children or having to perform them on a young child. And there's literature also that there's a component of psychosocial psychologic dissociation of the child on later testing. And this is really this is uh really sensitive literature and this has made various groups and this group is um this study is from based in at nationwide to evaluate are dilations needed. And so the length of follow-up in this study was 12 months. The Psar um had to be performed in a child under 24 months of age. It was a primary surgery, excluded cloaca as a diagnosis. The average Psar was performed at five months and a structure was defined as a Hegar dilator size of less than 10. In this picture courtesy of Dr. Mark Levit is a picture of a dime and this is a Hegar size 14. So when you can kind of um visualize uh what a stricture is based on that. So 10 is two deviations less than what our standard size would be for a newborn, which is about a Hegar size 12. So the study evaluated for primary and secondary outcomes. What was the need for reoperation or any additional surgeries that were required. In each arm dilation and non dilation, there was 25 children. The malformations were equally spread across both gender, gender specific obviously, but also malformation type. So the complexity of the children was also equal. There was strictures that um were non-significant between both groups, three in the dilation arm, eight in the non dilation. and essentially a Heineke Mikulitz anoplasty, which is where a longitudinal incision is made toward the anus and then it's closed transversely to widen the diameter of the circle was able to be performed. Some of the few that had to be done for stricture did have to be done under separate general anesthesia, but for children who had a colostomy, that stricture was able to be taken care of at the time of colostomy takedown. So what we see in this study is that the number of structures are the same. The number of needed HM anoplasty is the same. The number of reoperative surgery is the same. It was about two in each group and the number of children with prolapse is consistent with the previous literature. So there is a really good case here to abandon doing dilations post-operatively for children with anorectal malformations. I would say that um, currently in practice, I do size the anus at two weeks and one month and I believe a lot of surgeons really still need to understand what the diameter of the anoplasty is so that stooling can um not be obstructed by a structured anoplasty that's unrecognized. However, since the HM anoplasty is an option and we know that for slightly older children, dilations really are even more traumatic than for neonates. Um, this should be something that we consider and I'm sure there's going to be um large multicenter prospective studies coming out to give even more validation to this. So Dr. Rea, just to um uh to uh clarify. What you would do is after the um um after the uh um a Psar uh which um or the anoplasty what you would not do uh anything other than sizing at personally for uh two weeks and one month. And then um at the time of the the colostomy, that's when you would do the um strictureplasty? Correct. That's exactly right. And so the only time when I would consider initiating dilations post-operatively would be in a child that would not need any further anesthesia such as a primary repair like a rectal fistula or rectovestibular fistula and the anus is significantly decreasing between the two week the one month follow up and you're concerned that you might need to put the child under a general anesthesia. That would be the only time that I would consider now initiating full dilations. Because there's um, there's a move um that people are not doing colostomies for those um relatively simple anorectal malformations. Um, uh putting them NPO and giving them some parental nutrition for a few days. Right. I think there's still I think there's still a question about children that are just sent home with dilations through their fistula. And that is something that really is difficult to get away from completely because even not doing a dilation for a day at the neonate's bedside for a child that could potentially be sent home and have a delayed posterior sagittal anorectoplasty, that fistula can close up. So this is more posterior sagittal anorectoplasty post surgery outcomes where we can consider we can abandon dilations. Okay. Thank you. I had a question just about whether or not you see an impact, you know, I know then this is probably not something that we necessarily have in this data on uh continent. I think, you know, doing a a strictureplasty right or a redu anoplasty as opposed to dilations and and what that impact might be over the long term on continent. And I recognize these may be higher malformations and you expect that your likely sort of continent is lower in folks with the bladder neck etcetera. But doing another surgical procedure whether or not they are under anesthesia for a different reason, um may may have downsides to it as well. Yeah, I agree. Um, I would say though that the HM anoplasty, there's um a really big stress in that paper that long strictures that go through the sphincter complex are likely not to be done well through an HM anoplasty, which should really take place at the skin level. Um, but you are right, kind of stretching that anal opening with dilations or doing additional surgical procedures. We really don't have those functional outcomes yet. And so I'm going to go now and chat briefly about what the PCPLC is. So the PC Rebecca real quick before outlining the PCPLC. I I just had one question about um what you're currently seeing independent of the trial and I know this is kind of a moving target because you're probably doing the anoplasty a little bit different knowing that you're not going to be able to use dilations as a crutch later. What would you say the current percentage of patients who require a stricture plasti at that at that two month period. Probably about 5 to 8%. Um, and a lot of them and there's also in there the question always if there's prolapse, will it go away? Um, but yeah, I would say about 5 to 8%. Okay, well, I will say that uh this is the only thing in the world that actually is free, um to everybody who's here and we're going to hear about the PCPLC, which is not free, uh to those of us who have to support Rebecca and her colleagues. Right. So the PCPLC, which is one of the more difficult acronyms to uh get right every time, is the pediatric colorectal and pelvic learning consortium. This is a multi-institutional consortium that is across the United States, currently comprised of 17 different institutions. Um, all different types of institutions, meaning freestanding children's hospitals. Um, everybody basically is welcome at this point with um the ability to support uh joining to perform collaborative research for pediatric colorectal patients. And so the slide is here for more information, but it's really allowed us to track long-term data and this uh consortium was started a few years ago and at this time is now when we're starting to see the fruits of the data collection that has been going on. It's quite intensive in terms of the amount of data that is actually collected by the consortium. So, the next case, I'm going to let Dr. Smith take over. So, um, moving on to our next case, um, you are called to see a newborn uh female infant who day of life one was transferred to your hospital. Um, she's 39 weeks, two and a half kilos. Um, her physical exam is normal except for um her perineal and external anorectal exam which shows um a short perineal body with a anal opening that's anterior to the muscle complex but still posterior to the labial folds. You size it with Hegar dilators to about a size six. Um, she has some stool output with that dilation. She has a vectral work up with the echo that shows a PDA with left to right flow but no other congenital structural anomalies and um mild renal uh issue with some hydronephrosis on the right and aus that ends at L1 or terminates at L1. So if this is the infant that you are um going to see in the NICU, here's our question, what is your next step um for the poll. Um, discharge home with dilations, divert with a colostomy and a mucus fistula, um, or perform a primary posterior sagittal interrectoplasty. We'll give it a second for the poll. Rebecca, I don't know if you want to chime in what would your um while the poll is uh filling in, but what would your primary um modality be to take care of this patient. So, I am, I am a fan of doing dilations in the neonatal period and having the family um be discharged as soon as possible to home. I I think making sure that there's no other really big risk factors cardiac, kind of doing that full workup, but having the family bond. The diagnosis is often such a surprise to families that um having them be able to leave uh and understand what the problem is and know is really beneficial. Yeah, I completely agree with you. I think that um for um, you know, rectovestibulars and perineal fistula, um for our um female infants, I think especially and and male infants, it's nice to be able to send the patients home with dilations for a period of time and and have that um that happen. Um, so going into some literature. So, um, this is a paper that um uh looked at the 30-day outcomes between early and delayed repair of anorectal malformations, um, looking at P in the pediatric uh ACS database. And what this ends up, um, what the data ends up showing, if I'm advancing the slide to there we go. Um, so it looked at all uh 30-day outcomes um in P um for patients with anorectal malformation with perineal and rectovestibular fistula as their primary diagnosis. Um, early repair was defined as occurring at less than six days old and late was defined as anywhere from six weeks to eight months old. They're about 291 patients. Um, 66 patients were repaired early and uh 231 were repaired late. Um, and what uh was the outcome, the the the ultimate conclusion was that 30-day complications are not statistically different between these two groups. Um, this uh paper out of the um and looking at timing of Psar also, um this is out of the PCPLC um out this year. Uh, it also looked at 30-day post operative outcomes of neonatal versus um delayed anoplasty of perineal and vestibular fistulas. Um, and uh has uh similar outcomes. Uh basically also looking at 30-day outcomes in the PCPLC registry, um, which is our multi-institution consortium. Um, also looking at perineal and rectovestibular fistulas. Early was defined slightly differently as 14 days uh or younger, um and then late was defined, late repair was defined as anytime after 14 days old. 164 patients were looked at, 31 early and 133 late repairs and also um the conclusion of this paper was that there was no um uh no difference in um uh 30-day outcomes in terms of complications. Ask the question? Yes. So, so agree with great great studies and great review. Um so the question would be if you do dilations and you send them home, what is there an optimal age? Assuming it's a healthy baby, no comorbidities like you're saying, we understand that dilations probably best for those who have comorbidities. So one would be the first question be is there an optimal age to do that posterior sagittal anorectoplasty. And then the second question is, you just you just and commented as you just presented in your previous paper not to do dilations. Don't need to and now you're saying send this kid home with with dilations when you have the same outcomes of early repair versus delayed repair. So just kind of Yeah. Because we've had this discussion before and I don't know the right answer. So. No, I I completely agree. I know it sounds, it does sound conflicting. I would think um, I think for the neonates, for the infants, the dilations are really well tolerated. Um, for patients who are up to even several months old, I think outside of that for the older, the patients who have older repair, I think um, at least in my own experience, it uh in terms of psychological stresser for the parents, um, and the patient and I avoid them in those age groups, but I do think that the neonates and those that are under about three months age tolerate them really well. Personally, I repair, uh I don't think there's any data that I know of to really guide the exact timing, but generally around um, you know, two, two to three months, somewhere in there is usually where I fix these, Rebecca, I don't know. I mean, I think that's probably what most of us do on. 100%. Because there's a balance that you get between three and six months. If you don't, if you don't repair them somewhere around two to three months, two to three months is nice because you're just keeping the fistula open. You're not trying to increase the size, you're not trying to quote unquote go anywhere to like get to a 12. You're just wanting the stool to be able to come out. And if you manage their constipation with a little bit of mirror lax or something to keep the stool soft, you can get that repair done without readmissions before the child even gets repaired massive constipation. And so I agree. And I would also for um this would be, you know, things that might push you to do it earlier would be a little earlier would be like if it's a formula fed infant who needs like caloric concentration and their stools are thicker, like you can delay for definitely for like a breastfed infant. Um, uh you know, until two to three months, that's fine. You really want to get it done before they start solids also because that can make the dilation strategy at home, um, you know, much more difficult. So those are the things in terms of timing that I think of, but um, obviously there's no specific data to say exactly when to do it. Um, you know, but I think most of us probably do it around two to three months. I have a a question for uh both the speakers and thank you for um what you've got. But um all of us who uh look at uh Niquip uh P and I haven't uh looked at, you know, the outcomes that you look at with the uh the consortium. But none of those look at functionality. I mean, soul surgical site infections, whatever or UTIs or DVTs, but really what I want to make sure of is that this child who has a low anorectal malformation, um, does not need further operations, is not more constipated, is not, you know, having uh worse outcomes because perhaps the sphincter is not easily seen uh this particular age, which is actually what we were taught. Um, and so I would actually welcome data with regards to functionality and prolapse and, you know, all those things moving forward, not just within the the 30 day um Yes. 30 day uh time because that's what we want to know. Um, excellent point, Dr. yeah, I think long-term data is definitely needed to see if this is a safe strategy from a functional outcome for this patient population and I think that that study is in the works if I or I'm pretty sure. Um, I was going to say I think that definitely it's in the works. So we should hopefully have some long-term data looking at this um and have some more functional outcomes hopefully in the near future. Thank you. So, we're going to shift disease processes. Um we're going to talk about Hirschsprung disease now. We have a three-month-old male who presents to your clinic to establish surgical care. He has a history of total colonic Hirschsprung disease and an end Ileostomy. When would you recommend pull through? I left it intentionally a little bit vague. So only if the family is ready at or around the age of five months to one year of age, after potty training for urine, after the age of four years old or whenever the schedulers can add the case on. All right, I'm seeing I'm seeing quite a bit of oh, everything is shifting. I like that I can see the poll here. This is pretty great. So there's going to be a couple concepts that I want to go through and for the interest of time, because my time my I'm always watching the clock to make sure we stay on target for these high yield points here. Um is in the interest of time as I go through we'll kind of spend some time at the end discussing um some of the nuances, but really total colonic Her Springs has changed a bit. And the in the poll here it says after potty training for urine. But what what the literature really shows is that there's a shift that's happening to a slightly earlier pull through time and I will chat about why. And so the next topic is the timing of pull through for total colonic Hirschsprung disease. And uh first I wanted to talk a little bit about work that has been done by the A outcomes committee to discuss what is known in the literature about the management outcomes for long long segment Hirschsprung disease. And the the questions that were asked in this review were what is the definition and how is long segment Her Springs best determined, what is the preferred method of surgical repair, what are the long-term outcomes and what novel techniques exist. And in terms of how is the long-term uh long segment Hirschsprungs defined, it's any disease that's proximal to the rectosigmoid colon for the majority of articles that were reviewed. Um, there is really needs to be an aim toward standardizing nomenclature and I put this article up. This is a pediatric surgery Her Springs interest group article from 2019 that really lays out nicely how to communicate with your pathology group, how to discuss and talk about Her Springs disease in the operating room and on studies. Um, and then a contrast study itself is very inaccurate um and colonic mapping needs to be performed to determine the level of the transition zone. And so then for long-term outcomes, um, patients should be followed long-term. There was no superior operation or more common operation that was performed, although Duamel and a Swenson Suave were kind of the top operations and there's no novel technique that has really occurred in the past several years to um you know, change the entire um uh long segment Hirschsprung's uh landscape. There's a potential for stem cell therapy, but that's still in its infancy. So the article that I wanted to talk about is um this hyper motility and skin rash protocol um in the European Journal of pediatric surgery. And why this article is important is it has a mixed population of children with total colonic Her Springs, but it outlines very clearly why an early operation for total colonic Her Springs is possible. And by early I mean around the age of five months old. And why an operation at a slightly earlier age than toilet training for urine is possible is because if a child with an Ileostomy is adequately prepared and the family is able to participate in learning to thicken up stool, then they are able to actually have a pull through that does not result in complete perineal skin breakdown and ultimately learn techniques that will help this child because this is one of the hardest groups to um toilet train overall regardless if they have urinary continents first or not. That's that's total colonic. So basically, I think that there is a role with all the techniques that we have now in terms of using water soluble fiber to thicken stool, iodium to slow stool down to be able to titrate the Ileostomy effluent before the child has a pull through. That wasn't one of your um options uh Dr. It's uh because we actually look at what's coming out of the Ileostomy um, you know, to to figure it out because you don't want an Ileostomy in the bottom. Um, it's easier to deal with it. So uh less volume um um something that is more formed. Um that was a trick. It was. I learned something. Because the the question otherwise really becomes um, I just always do it when the child is toilet trained for urine. I only do it at the age of four. And I think if you wait way too long to do a pull through especially with total colonic, they will have a horrible anal sphincter spasm and potentially horrible pelvic disease that makes keeping a pull through really challenging. Yeah. Well, unless there are any other comments for the sake of time, we'll move on to review this topic case number four, which is just sort of to set the scene for talking about bowel management um in anorectal malformation patients. So, um case number four is a five-year-old male with a history of rectoprostatic um anorectal malformation. He's status post repair. hasn't really been followed since his surgery. He has a lateral sacral ratio of 0.5. He also had tether cord repaired uh in infancy and he presents to your clinic with multiple soiling episodes a day, um still in diapers, um and going to kindergarten this fall. He doesn't ever feel the sensation or verbalize the need to stool. Um, and he uh you perform a rectal exam anesthesia that shows there's no structure um and he had actually has a well-centered anoplasty. So, how do we manage these children? I think it's really um uh challenging and especially as they often have had surgery and not necessarily been followed long term. Um, so this uh study is out of the PCPLC looking at bowel management strategies in children with anorectal malformations. Um, it came out in uh just last year. Um, and what this uh paper talks about, I'll go through this graph uh schematic here um in detail, but it shows um the patients divided by age, type of anorectal malformation in terms of mild, uh moderate or complex severity on the um, I guess screen left. Um, and then on the right it talks it has the information about the types or quality of sacrum and spine. Um, and then um, hopefully you can see on the bottomemas is noted in red in terms of the strategy of bowel management that the patient is on. Um, and I think this is a really important graph because it shows what percentage of um, you know, even mild uh and moderate anorectal malformation patients in this 5 to 12 year old group really need rely onemas and lots of other bowel management strategies to stay clean when they're um heading into school age. So I think that was really important information um that we gained.