Problematic Anorectal Malformation Cases: Pediatric Colorectal Controversies...

Analysts what their standard protocol is for follow up. Dilations, um, and examination after an ARM repair in a female, which is, which is where these slides are gonna take us. So Jeff, would you mind getting started on that? What is, how do you follow these kids routinely? Discharged from the hospital? So they get discharged. I'll see them back usually at 2 weeks' time, uh, to start dilations, and then, you know, we, we cover, you know, our hospitals, uh, located in an area that covers 25% of the US land mass. So some of our patients live in Alaska and Montana, so follow up becomes very challenging. If I can, I'll see them back. If they live locally, I'll see them back in 1 month, then 3 months, and then 6 months, and then usually like in a year. And then follow them at least yearly thereafter, um, if they live like in Montana or Alaska, we'll make sure that there's a local follow up, um, just to keep an eye on the perineum, but I'll make an effort to have them come back in at least 3 months and, and 6 months if possible. I see them back at 2 weeks, start the dilatation process. I dilate them a week later and teach the parents how to dilate them and then send them home with the Hagar dilators, dilate them up to 12 or 13 depending on the size of the patient. Um, then I examine them and if it's supple, which it usually is by then, then, uh, I see them back about 2 months later to make sure it's not, uh, stricturing. And after that I follow them the first year every 3 months. Um, we have a pretty strict protocol in terms of some of these patients have a tendency toward constipation. I warn the parents about that. Tell them not to let the child go more than a day and a half or 2 days without a stool, and um. Most parents are pretty religious about it. If I suspect that the patient's not going to follow through, then I see the patient more frequently. So I take a minority opinion on this one. I, I don't, um, I don't think that daily dilatation by the parents is necessary most of the time, so I see them. Between 1 week and 2 weeks after, and this is true for all of the ARMs and my Hirschmann's patients, I calibrate, uh, the anastomosis, and if it's open, then I see them on a weekly basis, usually for about 6 weeks, and I do the calibration in the clinic. I don't have the parents dilating at home, um, when we, uh, when we looked at our experience because I'm on, I'm the only one in our group who does that, so we had a control group that had parental dilatations and. When we compared the outcomes, there was no difference in stricture rate, perforation rate, uh, or or enterocolitis rate in the Hirschmann's patients, um, but the parents, uh, most of them did not have to dilate their children, which is psychologically a very difficult thing for them to do. Uh, about 10% of them ended up narrowing down during this process, and those ones I taught them how to dilate, and they dilated at home. But I, I continue to believe that this daily dilatation by the parents is a psychological hardship for them, and I don't think it's necessary most of the time. So can I ask you a question? What you do about your kids who are coming from far away, or, I mean, and obviously Canada as well, but there's a lot of, you know, a lot of people have trouble getting to hospital appointments and time off work and all those lost opportunities. I mean, uh, well, so I, I do make, uh, allowances for that, so. You know, we, I do like to keep them around Toronto for at least 3 or 4 weeks afterwards. I mean, that's, I, I ask them to make that commitment to their child, um, the ones that absolutely can't, then I teach those parents how to do it and I, I try and find somebody in their community who can, uh, keep an eye on them. I, I gotta, uh, I, I do dilate, but I gotta say sometimes you have a 3 or 4 year old that missed a window of, of getting their annoplasty early, and you can't dilate those children. The parents certainly can't at home, and I don't see a difference in how they do. And you know, you will every week or two they'll come to clinic and I'll do a dilatation, but um, they do fine. It's interesting. That's a topic worth studying randomized. I'd be very cool. You know, I, I wouldn't, I wouldn't have, uh, equipoise on that. Uh, I would not be willing to randomize my children to daily dilatation. Some might feel the opposite way. Ivo, so sorry, uh, what about the European representative on the panel? Sorry, the program is a little bit delayed. We have, we have the discussion on the calibration of dinotations in Europe as well, particularly with the, the, the parent organizations about the post-op dilatations we were talking about. Of what structure? The anastomosis after anus anus, OK, because we had just been talking about vaginal, no, not, not the anus. They particularly want to reduce it, so we're thinking of making a kind of a first step protocol to try some kind of trial to start off with at least 6 weeks of dilatations, see what the effect is, and then maybe reduce it, but most of us are still a bit scared because in a way we don't want to. Um, yeah, we don't want to rule out, uh, uh, the things we do right at this moment which are kind of successful, but Like you say, I've, I've read your manuscript as well, and it seems to be OK. Not mine. So I, we have a whole very lovely session on uh problems post ARM pull-through that I was excited about, and I'm sorry we delayed the program. There was a case going on, I guess, but, um, but uh there's gonna be a bus at 5. I may invade that a little bit, be about 5, 10 minutes if that's OK. Just so we can show a couple of these pictures. Is that, would that be all right? Maybe 5, 10 minutes of this. Um, we talked a little bit about this problematic female. This is a patient who had a previous pull-through, and what do you see is the problem? What did the original surgeon not do? Does everyone see what is looking, what is this? Yeah, so that's the original vestibular fistula that was never removed. Now, the reality is if this anus is OK, this is really of no consequence, but sometimes this is quite large and actually it goes behind the perineal body and then the perineal body has no substance to it at all. So I have removed these, particularly when the anus needs to be redone. And this is a, um, this is an interesting colorectal problem, not related to ARM. This is an adult who had a, a episiotomy that didn't heal. But it looks exactly like a mislocated anus situation after a dehisted perineal body after a PSAP. Looks the same. Essentially, there's no perineal body in the. The goal for the surgical repair is to get healthy rectum down, healthy vagina up, and make a muscular structure of the perineal body in between. You live in Austin. You see all, all sorts of things with, with this. Yes. Well, I think you can choose. You can either do a colostomy to divert or keep the patient NPO. Those are always your choices. Um, uh, uh, on, on, uh, D10, 7 days, etc. like we talked about before. Here is, here is the vestibular fistula that is actually still connected to the perineum. So, obviously, this needs a redo with a full remobilization of the rectum. Um, this is a video, but actually, I don't need to show you the video. Can everyone see where this anus ought to be? Oh, look, oh, the video's working. See that? Anuses should have been there, right? It's too a little bit too posterior. So in this patient who has good potential for bowel control, good sacrum, good spine, I'm going to offer this child a redo and move the anus to here, where it will be concentrically closed by the sphincters. Saw that. This is the most common um problem in, in, uh, uh cloacas, where the surgeon only dealt with the rectum and didn't do anything to the urogenital sinus, needs a redo. All right. So mislocation, posterior mislocation. Nice. And that's what this looks like. This is actually one of those cases. This is a, the rectum was done quite nicely, but the patient was left with a fistula. They missed it. Bulbar fistula not touched. All right, what do you think this is? Shamael just fluttered his eyes like. This is unbelievable, really, but I can tell you this uh surgeon was moving along and said, there's a beautiful, nice midline whitish structure, let's go get that. And it's the bladder neck. They pull through the bladder neck, and there's the urethra. This is not hard to do. Unless, if you don't know the anatomy before going in, you can do this. Brent still here? No, so this is a, uh, you may have shown some of these mislocated, uh, pull through on MRI. I've never prolapse. Prolapse. I, I caught this fish. I caught this fish. There are 4 fishermen in the boat. I brought this fish in. Can you believe it? So I did a quick anoplasty and got him back in the water. All right, so that's a, a nice repair. Uh, I can tell you I've, I, I have started to do this. I'm sure, I'm curious to know what you all think. I'll do half only. And then do the other half 3 months later. And it heals very quickly. I do that as an ambulatory procedure and then no dilations are necessary. Anyone have an opinion about how they manage prolapse? This is something we all are confounded by, Jack. Well, I'm actually surprised at how often the only half is prolapsing in many of them. Well, when it's just half, then it's nice. You can just do the other. But I do it as an ambulatory procedure. I, I do, and I don't, and I don't dilate them. I just calibrate them the way I suggested. So me too. I think if you do a circumferential dissection, you've got to watch for a stricture. Where if you only do half, there's no way it's going to stricture. So I've started to do half as an ambulatory and half as an ambulatory 3 months later, and when that's offered to the family, they would love that rather than, you know, a circumferential worry about dilations, etc. and have to stay in the hospital. All right, so the same. There's, there's your half. So which is the faculty half and which is the fellow half? All right. So now, this is a post laparoscopic pull through for a bulbar. Does anyone know what that is? And the answer is not two bladders. So that you would call a posterior urethral diverticulum, which is the old original rectum. And Keith, how do you avoid that? I think I mentioned, I think I mentioned that this morning. First of all, I think you should not overestimate your skill set and make sure that you're comfortable doing this is a much more complex operation than, uh, the Hirschprung's operation. But um and then the other thing is if you don't know where you are, open the colon and find the fistula from inside. Yeah, so this is a complication that is happening a lot more because people are not dissecting distally enough or just don't doing it properly. And here is a patient who's this has been left behind. And this is a patient with a similar problem, but this developed into an adenocarcinoma. This is the original distal rectum and an adenocarcinoma of the pelvis in a 30-year-old, so that posterior urethral diverticulum, which is clonic mucosa bathed by urine for 30 years, is a, is a cancer risk at least in one, in one case. Clearly though, I don't think that was done laparoscopically. It, it was not. It was not. And here's a pretty impressive had to defend my turf. Urethral stricture, and I, I think that's.