Hey there listeners, it's Amanda Jensen from Riley Children's. Today we bring to you a very special episode of our colorectal quiz. It is episode 22 and we are gonna talk about Hirschsprung's disease again and specifically the soiling patient. Doctor Fisher, why don't you start us off? Welcome back, everyone. Here we are with the colorectal quizzes. Wow. We have been doing this for a long time, Mark. I feel like we might be on like season 2 almost. Fantastic. That was Doctor Jason Fisher from Cincinnati Children's. Yes, and, and the good news about that is you can, you know, skip ahead to the next, the next one, you can watch them in sequence, you can watch them out of order. It's not like the old days where you'd have to wait until the next week. That was Doctor Mark Levitt from Children's National. Although I'm sure there's some people out there that are uh listening to this in sequence live. I mean, literally live within the next week of it being created, but. What are we, uh, what are we talking about today? Last week was a good session on, on Hirschprung's disease, the obstructed patient. Seems like it needs a follow-up. Uh, you know, that's amazing. What a lead-in. I think that's why we've gotten out of the pilot series, and they actually rehired us for a second season. Because go figure, but the next topic is Hirschrung's disease, post pull through, not doing well due to soiling, not obstruction, but the opposite, too much coming out. How do we deal with it? Such problems. So, yes, so, uh, I actually like to talk about the problematic post- pull through Hirschprung's patient in two versions. The obstructed patient, they are not emptying, they're having distention, they're having enterocolitis episodes, multiple, they're having failure to thrive. That's what we talked about last podcast. Then there's a group of patients. That don't have any of those obstructive symptoms, or if they do, they're fairly minimal, they're basically some constipation. But they are soiling, they're pooping all the time, never distended. That's the patient that we're gonna talk about today. So remember, there's the soilers, and then there's those that are obstructed, and today we're gonna talk about soiling. Hey, Mark, let me ask you a question. We've done a few podcasts on Hirschsprung's disease, but I don't think we touched on this. And I think it's important because uh both of us have seen many patients with Hirschsprung's disease, and, When you meet a family, day of life 123, when the diagnosis is being constructed, and we finally get to a diagnosis of Hirschwrung's disease, what do you tell the family, the parents, what to expect long term? Because I think this is what frustrates some families, is that they're told. We're gonna do an operation. It's a somewhat complex operation, but after that, everything's gonna be fine. But yet, we're dedicating podcasts to things not being fine. So, what do you tell them? Yeah, this is a, you know, I think if you ask most surgeons, they would say Hirschrun's is a great operation because You know what to do, you get the pathology right, you do a nice surgery, pull through works, everything's great, you're done. And the reality is that's just simply not true for Hirschrun's disease, but I will tell you, it is definitely different than an anorectal malformation scenario, where, That perhaps is more frustrating, cause in that case, you can do the most perfect anatomic reconstruction, and because the patient has a problematic spine or sacrum, sphincter muscles, etc. you don't have a good result, you still have a soiling patient that you need to deal with. I believe that in Hirschprung's disease, if you get the pull through right, with no anatomic problems, and a preservation of the sphincter mechanism. Most of those patients do extremely well. Some of them need a little medical treatment, usually to tinker with constipation. Yeah. So that's what I tell them. If I am doing that operation, I'm very confident I can achieve that. Of course, you need nursing help, you need tinkering, you need to make sure that they're pooping once or twice a day. You need to help them get to the right consistency of stool, but there is no reason why any Hirschprung patient should be obstructed, and there's no reason why any Hirschprung patients should be soiling, and if they are, We need to investigate why, and we can fix them, and that's what we're gonna talk about today. I think that's the key is, I, I, I tell the families that there is the potential of issues. I mean, some of it's out of our hands, pathology, let's say, right? Uh, we all have good pathologists and, and we have to trust your pathology team, but sometimes, We have pathology that changes. I mean, we've had cases, Mark, I remember when we were working together, where there's a case where we reviewed the pathology, and at the time of pull-through, ganglion cells were present, and at the time of problems, 7 years later, we do a biopsy, and there's no ganglion cells and hypertrophic nerves. Oh, you know, well, that's a whole another, that's a whole another discussion, which I think we touched on a little bit, uh, last time that something about that pull through deteriorated. Or the original pathology was off. Right. And I'm not sure which it is, but I definitely concede that the pathology might have been perfect, and then that pulter decompensated, and why did it decompensate? Probably because the patient did not have adequate medical management or sphincter management. Which we're gonna get to, but basically, they need to flow well after their pull-through, and if they don't, and they don't get treatment for that, their pull-through can decompensate, and I think it can even go to the point of hypertrophying the nerves. I think that there are no ganglion cells seven years later, there probably weren't ganglion cells originally, but there is this concept of the vanishing ganglion cells related to tension or ischemia or whatever, which is quite controversial. Right. All right, I just want to dial in specifically on this Hirschprung's disease patient that is soiling. What is the most important question to ask? I'm gonna really emphasize this, to always ask the question, and by the way, this is true for any soiling patient. What is the patient's potential for bowel control? This is a question that is often neglected. There are patients that are soiling, that are getting treated. Without asking that question. And when it comes to Hirschprung's disease, as I said earlier, all patients have the best possible potential for bowel control. Why? Cause they were born with normal sphincters. And let me put a footnote there, sometimes too strong, where they don't relax, but they certainly aren't lax sphincters, and an intact dentate line anal canal sensation should be intact. So, when you take care of a Hirschrung's patient, and their dentate line is intact, and their sphincters are intact, they have all the potential to have voluntary bowel movements and to have bowel control. If their dentate line is lost, because the dissection was started too low, or their sphincters were overstretched. And don't swing back well and don't contract well, now they may have lost their potential for bowel control. So that is the number one question, and we're gonna go through a series of pictures. Again, I encourage you to get the app, because then you'll be able to see the pictures, and I'm gonna show you guys photos. I don't know if you've seen these before, and my question will be, how are the sphincters, or how is the dentate line? All right, so let's do the first picture. Yeah, again, very important, I think, to really grasp this part of the, the, this podcast is to get the stay current app and see these pictures because it really helps with the whole understanding of what's going on. So, getting the app is key here. All right, so the first picture. Is what I consider a very normal set of sphincters. If you're on the stay current app, you can click on image number one. It is, the anus is closed. These are all pictures with the patient awake, by the way. So that's what you want. Now, to formally check, you can do an anorectal manometry. I'm not sure what you guys are doing there, Jason in Cincinnati, but we are now doing 3D. Anorectal manometry, which is really, really cool, and you can see whether the sphincter is, uh, whether the patient has a good squeeze and whether that squeeze is, is concentric. Recently, we saw a patient that had a good squeeze, but there was no squeeze on the anterior side. Um, I, I think these are all adjuncts that are great, whether it be 3D, anorectal manometry, endorectal ultrasound, all looking at where is the muscle and using it on patients with Hirschpring's disease, post pull through or anorectal malformations, both, I think, great adjuncts to looking. But you made a subtle hint that these are all patients awake. Yes. And I think that's key to understanding versus a patient that you might do an exam under anesthesia, a little bit harder to assess the sphincters per se. Yeah, so, um, the sphincters, I, I, I like to get a look at how they look. Do they look patches, or do they look closed? And anorectal manometry is obviously an objective way of seeing whether they're intact or not. You can actually get the patient to squeeze for you. Alright, now go ahead and look at image #2 on the stay current app. And this is a picture, and if you look closely here, you see that you can actually see in to the anus. There's like a slit, and it's not closed. So, to me, this is a set of sphincters that may have been overstretched. And how do they get overstretched? Transiental approach, deep dissection, wrong plane, retractors in the anus. Very bad, the sphincters won't come back to normal. All right, now turn your attention to image number 3 on the state current app. Kira, what do you think? What say you on, on that photo? So this is the patient that's still awake. Uh, we can see that the anal opening is not completely closed. It's pattuous. The squeeze is not that great. I don't, um, it doesn't look like they're even trying to squeeze in this picture, but either way, the anus is not completely closed in this picture. So, as Doctor Levitt mentioned before. Either the dissection was started too low, there's some potential damage to the sphincters, and patients not able to um squeeze them. That was Doctor Hira Ahmad from Seattle Children's. So, our first question that we posed is what is the patient's potential for achieving bowel control with overstretched sphincters that cannot close? What would be your answer to that question in this patient? In this patient, I would suspect it would be unfortunately poor because they're not able to even have the anus closed in the resting position. Right, and for other patient groups that don't have good potential for bowel control, like ARM with a poor spine and a poor sacrum, like spina bifida. What do we do for the soiling patient with poor potential for bowel control? Yeah, so they generally need to be in a dedicated bowel management program, either on enemas, could potentially considered an anti-grade option. So Doctor Levitt, what's the bottom line here? They need a mechanical emptying if they have lost their potential for bowel control, and of course, there are patients that are borderline, but I think a mechanical program can get them to a point where they are clean. And psychologically want to be clean, and then more likely to successfully potty train, but in a patient with injured sphincters or lost dentate line or both, they need a mechanical program. Before starting a mechanical program, would you do an anorectal manometry in these patients? Yeah, so my routine now is to do a 3D anorectal manometry in all of these patients, and if they have a good squeeze, Then, you know that they have potential for bowel control. I note whether they have a dentate line or not, which we're about to discuss in the next little segment here. And then you can make a judgment call, whether you wanna try to potty train them with medicines like laxatives, or give them a break, and get them mechanically clean, and then try the laxatives another time. And whether you do rectal enemas or a Malone is another philosophical discussion that we can have on another podcast. Depends on how willing they are to tolerate rectal enemas, it depends on their age, but the bottom line is they need, I think they need a mechanical program to start, and then some of them need a mechanical program forever, and then some of them can transition over to normal bowel control. Nowadays, I do a 3D anorectal manometry. Because I've started to do a new technique, which we just wrote up in JPS of tightening of the sphincters, um, which has actually had great results. We've done it in a number of patients, and, and for a patient who had disrupted sphincters or pattula sphincters, tightening them up will, will be, um, I think will be ultimately a helpful, a helpful maneuver. All right, now let's look at image number 4. If you're on the stay current app, go ahead and click on that picture. And what do you see here? Um, so in this patient has a circumferential about 1 centimeter, um, 0.5 centimeter to 1 centimeter of circumferential rectal prolapse. Rectal prolapse after a Hirschrung's operation? Kira, what are you talking about? It's probably secondary to uh sphincters being damaged and then um them not being able to, or them, well, I guess they're not really squeezing that hard though, right? So no, no, no, this is, this is iatrogenic. There is no, there is no patient, you have never seen. And all those wonderful colorectal people that you have gotten the opportunity to train with, none of those people have ever had a rectal prolapse after Hirschsprung's pull-through, ever. Jason Fisher, never. Me, never. Why? Because the sphincters are preserved. If you overstretch the sphincter, you, this is one of the worst pictures I've ever seen. If you overstretch the sphincter, it will become pattuous. It almost looks like a spina bifida anus at this point. And then the additional problem here, in this particular picture, there's also no dentate line. So the sphincters have been overstretched to the point of laxity, to the point of prolapse, and the dentate line is also lost because the surgeon started the operation too low, and sewed the colon to the, to the anus, which we're gonna get to in the next series of of, of the photos. Harry, you were, you were dead on, I mean. The muscles help control prolapse, right? When everyone has a bowel movement, your, your sphincters relax, the anus opens up, some mucosa comes down, but as soon as you tighten back up, that is retracted back in. This is a patient at rest with prolapsed mucosa. The two things went awry. One, the, the muscles were damaged from stretch, from iatrogenic injury, a combination of both, and also, it looks like the mucosa was brought too low on their anastomosis, and so, That's an unfortunate picture. So in all, in all of these patients, they, I believe they need a mechanical program. Uh, we can visually see that these sphincters are problematic. I would like to objectively confirm that. I think the gold standard for that is 3D manometry. It's not available to everybody. But just looking at it, patula sphincters is a patient that needs a mechanical program, either enemas or uh from below, or, or enemas from above with a malone. Nowadays, if I found a patula sphincter, I would offer the patient a sphincter reconstruction, and I, um, I hope my colleagues around the world will learn how to do this because it's not not hard at all, it's uh technically good. Uh, to a reproducible operation, and Jason, I can show you a nice video of this, um, and as I said, it is written up in the technique section of JPS a couple of months ago. And if you're in the stay current app, go ahead and click on the link and it will bring you to the article that Doctor Levitt is referring to. That's what I would offer them, and I would do them alone at the same time. Because I don't know for sure that the sphincter reconstruction is gonna be the ticket, but I'm hoping that they will then be clean with the Malone, and actually the malone, as we talked about in another podcast, can be a bridge to continents, they can practice holding in the flush, releasing the flush on command, and maybe improve their sphincters, and actually get to the point of bowel control. All right, and that wraps up part one of a two-part series in the colorectal quiz, episode 22, Hirschrung's Disease and the soiling Patient. So to summarize for this episode, In a patient with Hirschsprung's disease that's not doing well, and specifically having issues with soiling, the most important first question is what is the patient's potential for achieving bowel control? And really to assess this, you want to assess both the dentate line and the sphincter. Regarding the sphincters, we want to know what is their squeeze and is it concentric regarding the dentate line. We want to know if it is intact. It's important to evaluate these things with the patient awake. In addition, a great way to analyze this is also through 3D anorectal manometry. Overall, if you think that either #1, the sphincter is poor, or #2, the dentate line is inadequate and this patient will have poor bowel control, really the answer and solution to this problem is mechanical emptying, and this can be in the way of A uh Malone or retrograde enemas. And with the next episode, we will be talking about Hirschman's disease and the soiling patient and specifically talking about the dentate line. Until next time, this is Amanda Jensen at Riley Children's. And remember, knowledge should be free.
Comments