Update Course 2021: PEDS COLORECTAL CONSORTIUM CONCLUSIONS

We are now gonna 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, or 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'm gonna turn things over to, uh, Doctor Rebecca Rentia and Caitlin Smith. Um, who are gonna explain this consortium again, not to surprise me, 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 Caitlin, 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 Doctor Caitlin Smith. Hi there. I'm, uh, Caitlin 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 wanted today to talk about a few cases and to really hit 7 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 gonna start with the case of a 3 month old male with a history of anorectal malformation and a rectal bladder neck fistula who presents 2 weeks following his laparoscopic repair. Um, he has a colostomy and mucous fistula, but what's your next step in management? So the first choice is, do you begin with protocol-driven Hagar 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 I'll give everybody a moment here. To say what they think. And, you know, there's a lot of background here, um, and really a history since Doctor Pena did one of the first PSARPs all the way to now that everybody in their clinic has kind of on the, on the chalkboard in a handout is this Hagar 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 PSARP. And this article was published in the Journal of Pediatric Surgery. It just came out. Are routine anal dilations needed following a PSARP? And what this study found in a single institution prospective randomized controlled 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 a really sensitive literature, and this has made various groups and this group is, um, this study is from based in at nationwide to evaluate our dilations needed. And so, the length of follow-up in this study was 12 months. The PSARP 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 PSARP was performed at 5 months and a stricture was defined as a Hagar dilator size of less than 10. In this picture, courtesy of Doctor Mark Levitt, is a picture of a dime, and this is a Hagar size 14. So when you can kind of um visualize uh what a stricture is based on that. So 10 is 2 deviations less than what our standard size would be for a newborn, which is about a Hagar size 12. So the study evaluated for primary and secondary outcomes, what was the need for re-operation or any additional surgeries that were required. In each arm dilation and non-dilation, there were 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 were strictures that um were non-significant between both groups, 3. In the dilation arm, 8 in the non-dilation, and essentially a Heineke-Micheletz 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 strictures are the same. The number of needed HM anoplasty is the same. The number of re-operative surgery is the same. It was about 2 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 postoperatively for children with anorectal malformations. I would say that um currently in practice, I do size the anus at 2 weeks and 1 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 strictured anoplasty that's unrecognized. However, since the 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 gonna be, um, large multi-center prospective studies coming out to give even more validation to this. So Doctor Antea just to um uh to uh clarify, what you would do is after the um um after the uh um PSSART, which, um, or the anoplasty, you would not do anything other than sizing at personally for two weeks and 1 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 postoperatively would be in a child that would not need any further anesthesia, such as a primary repair like a rectal perineal fistula or recto vestibular fistula, and the anus is significantly decreasing between the two-week, the 1 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 uh there's a, there's a move, um, that people are not doing colostomies for those, um, relatively simple anorectal malformations and, 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 neonates bedside for a child that could potentially be sent home and have a delayed posterior sagittal anal ectoplasty, that fistula can close up. So this is more posterior sagittal an ectoplasty post-surgery outcomes where we can consider if we can abandon dilations. OK, thank you. I had a question just about Whether or not you see an impact, you know, and it's probably not something that we necessarily have in this data on continents. I think, you know, doing a strictureplasty, right, or an, a redo anoplasty as opposed to dilations and, and what that impact might be over the long term on continence, and I recognize these may be higher malformations, and you expect that your likelihood of continence is lower in folks with a bladder neck, etc. 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 PCC Rebecca, real quick before outlining the PCPLC, I, I just had one question about 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 strictureplasty at that, at that two-month period? Mm, 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%. OK. Well, I'll 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 the different types of institutions, meaning free-standing children's hospitals. Um, everybody basically is welcome at this point with, um, the ability to support, uh, join in, um, 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 gonna let Doctor Smith take over. So, um, moving on to our next case, um, you are called to see a newborn, uh, female infant. Your day of life one was transferred to your hospital. Um, she's 39 weeks, 2.5 kg. Um, her physical exam is normal except for, um, her perineal and external anorectal exam which shows, um, a short perineal body. With the anal opening that's anterior to the muscle complex but still posterior to the labial folds. You size it with Hagar dilators to about a size 6. she has some stool output with that dilation. She has a veal workup 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 a conus 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 is our question. What is your next step um for the pole? Um, discharge home with dilations, divert with a colostomy and a mucous fistula. Um, or perform a primary posterior sagittal ectoplasty. We'll give it a second for the poll. Rebecca, I don't know if you wanna 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'm, I'm 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 a 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 well is really beneficial. Yeah, I completely agree with you. I think that um for, um, you know, recive vestibulars and perineal fistulas 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 Nisquip P in the pediatric, uh, Nisquip, 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 Nisquit P, um, for patients with anorectal malformation with perineal and recto vestibular fistulas as their primary diagnosis. Um, early repair was defined as occurring at less than 6 days old and late was defined as anywhere from 6 weeks to 8 months old. There are 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, uh, 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 PSARP also, um, this is out of the PCPLC, um, out this year. Uh, it also looked at thirty-day postoperative 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 recto vestibular 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, uh, I agree with a 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 dilation is probably best for those who have comorbidities. So one would be the first question would be, is there an optimal age to do that with steroid anal rectoplasty? And then the second question is, you just, you just in comment is 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 a 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 stressor 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 3 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, 22 to 3 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 100% on. Cause there's a balance that you get between 3 and 6 months. If you don't, if you don't repair them somewhere around 2 to 3 months. 2 to 3 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 MiraLax 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, And, and I would say 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 uh definitely for like a breastfed infant, um, uh, you know, until 2 to 3 months, that's fine. You really wanna 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 2 to 3 months. I have um, 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, NISWIP, 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, so surgical site infections, whatever, or UTIs or DVTs, but really what I want to make sure of is that this child who has a, 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, at 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, the 30 day, uh, time because that's what we want to know. Excellent point, Doctor Archer. 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 gonna 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 gonna shift disease processes. Um, we're gonna talk about Hirschsprung disease now. We have a 3 month old male who presents to your clinic to establish surgical care. He has a history of total colonic Hirschsprung disease and an endileostomy. 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 5 months to 1 year of age, after potty training for urine, after the age of 4 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 pole here. This is pretty great. So, there's gonna be a couple of concepts that I wanna go through in it for the interest of time, cause my, 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 Hirschsprung's 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 ABSA Outcomes Committee to discuss what is known in the literature about the management outcomes for long, long segment Hirschprung disease. And the, the questions that were asked in this review were, what is the definition and how is long segment Hirschprung's 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 Hirschsprung's defined, it's any disease that's proximal to the rectosigmoid colon for the majority of articles that were reviewed. Um, there's really needs to be an aim towards standardizing nomenclature, and I put this article up. This is a pediatric surgery, Hirschsprung's interest group article from 2019 that really lays out nicely how to communicate with your pathology group, how to discuss and talk about Hirschprung 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 DuML and a Swenson Swave were kind of the top operations. And there's no novel technique that has really occurred in the past several years to, um, uh, you know, change the entire, um, uh, long segment Hirschprung'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 hypermotility 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 Hirschprung's, but it outlines very clearly why an early operation for total colonic Hirschprung's is possible. And by early, I mean around the age of 5 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 continence first or not. That's, that's total colonic Hirschsprung. 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, Imodium 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, Doctor Antea. It's, uh, cause we, I 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. 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 4. And I think if you wait way too long to do a pull through a special of total colonic, they will have 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 4, which is just sort of to set the scene for talking about bowel management, um, and anorectal malformation patients. So, um, case number 4 is a 5 year old male with a history of erectoprostatic, 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 tethered 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 or anesthesia that shows there's no stricture, um, and he actually has a well-centered anoplasty. So, how do we manage these children? I think it's really um Uh, challenging and, uh, 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 patient's 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 bottom, enemas 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 to rely on enemas 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, um. Uh, it overall looked at 624 patients, um, that were in the PCPLC registry with all with, uh, varying diagnosis of severity of anorectal malformation. You can see the breakdown there. Um, and the majority were enrolled in a bowel management program, so about 418 or 2/3 of the 624 patients. Um, the majority of patients had constipation as their primary complaint, um, and only 40%, uh, were toilet trained, which I think is a really important, uh, note as well. Um, and then about half of them reported a daytime stool accidents. So, obviously, there's a lot of work that um we can do here um but I think this is um really good baseline information and really recognizing and emphasizing that these patients really require a long-term close follow-up through toilet training to make sure that um uh we're able to manage some of these issues. And so, point number 5 that I wanted to talk about is the timing of pull-through for Hirschsprung disease. And so this is a consortium study that looked just at exactly that question. And Doctor Arca, you had mentioned looking at outcomes, and this paper actually does look at outcomes. So what this What this paper did was to look at the timing of endorectal pull-through as early. All infants had to be diagnosed under 1 month of age. These were primary pull-throughs, less than 31 days or greater than 31 days was considered early pull-through or late. And what the study found was that preoperative enterocolitis was the same between both groups. About 2 in each group. Post-operative enterocolitis was the same, between 40 to 50% almost for both groups, at least 1 episode of enterocolitis. Constipation and incontinence tracked all the way to 3.5 years for both groups was the same for both groups. And transition zone really was the marker if a child needed to be treated for constipation. There were too few children that had um a total colonic or any other type of Hirsch. besides rectosigmoid transition zone, which made about 60 to 70% in both groups. So basically, a delayed pull-through with irrigation, sending the family home if there is a support system, and that is my editorialization, if there's a support system, is a safe alternative to an operation in the neonatal period. Moving on so we can um Not take up too much time, but, um, obviously we already spoke about this in the last session, um, but, um, talking about disparities in, um, patients in the colorectal population. Um, obviously, we are well aware that there are non-clinical factors, race, ethnicity, and insurance status that are associated regardless of clinical variables with outcomes. Um, but, and this is actually supposed to say data is limited in pediatric colorectal patient populations. As we talked about, uh, just to emphasize in the last half hour, we know that they are documented in pediatric patient populations. Um, so there is some evidence, um, in patients with spina bifida with Hispanic ethnicity and public insurance that they have lower overall continence rates, um, but this hasn't really been looked at in the anorectal malformation population. Um, and this, uh, data was presented at APSA this year, um, and was recently accepted. This is another consortium study out of the PCPLC that looked at 525 patients, um, and public insurance, uh, was associated with decreased rates of urinary incontinence in ARM, uh, patients, um, and also clinical factors were related, which, um, these are the things we sort of know already that have been documented in the literature, type of ARM, spine and sacrum. We obviously need a lot more further studies to uh investigate this. Um, my commentary on this would be is I wonder, um, since the PCPLC is made up of specialty centers, if we missed some of the race and ethnic disparities that exist since the patients who can afford to travel to one of these centers may be, um, not captured, um, and be able to uh, you know, have care at these specialty centers, so, Improving our outreach, I think, and finding the patients that don't have the means to travel, I think is really important for us as a consortium. And so In the interest of time, we'll move to the last topic, important concepts in cloacal reconstruction surgery, which could be its own discussion. But, have you been thinking about cloacal reconstruction as common channel greater or less than 3 centimeters? And that really is the question to Consider since moving. Oh no, I've lost all control of the monitor. Um, so, overall, this picture here, uh, shows that there are two different types of, um, cloacal anomalies on that high pressure distal cholostogram and the 3D cloacogram. And what we can see is that really, while both of the children have a short common channel in this picture, there are huge Huge differences that really do make a difference to functional outcomes and surgical planning. So the trend in cloacal reconstruction is to actually have a surgical plan and anticipate based on imaging and examination under anesthesia before surgery, what that surgical plan will be and any anticipated anatomic issues that you may come across. And so, In the cloacogram itself, what we're looking for is, does the child need a total urogenital mobilization or a urogenital sinus separation? Does the child, is, are they a candidate for laparoscopy? Or is a laparotomy potentially needed? How long will the operation be? Will their will their vagina actually reach to the perineum? Is the urethra at risk for injury? And so, in the articles that we listed here that will be available, To examine more is that the important points to take away that have really been published in the past couple years are that the urethral length of about 2.5 centimeters has been measured in a lot of VC. of normal females. And so about 1.5 centimeters is needed. If the urethra that is too short is pulled past the bladder neck, there is a risk for incontinence. So understanding that even for a short channel can change your operative management. A short vagina, even in a very, very otherwise shorter common channel operation may require a vaginal replacement. And then finally, rotational fluoroscopy and 3D reconstructions are key to being able to make reliable measurements for anatomy. That's a great review, guys. Thank you for that. And um I think the last 3 slides, particularly that algorithm demonstrates that if you're dealing with a long-channel cloaca, make sure that that patient gets to a complex colorectal center and somebody's that's got the resources and experience to take care of it.