Colorectal Quiz Episode 19: Hirschsprung Disease - The Obstructed Patient Part 1

Hey there listeners, it's Amanda Jensen from Riley Children's. Have you downloaded the new version of the Stay Current app? It is in the Google Play Store and in the Apple App Store. The reason I bring it up is that if you are listening to this episode within the app, you can bring up the pictures and journal articles discussed in real time while we are talking about them. Today we have a very special episode for our colorectal quiz. I'll start off by introducing Doctor Mark Levitt from Children's National. All right, Mark, what are we talking about today? Troublemaker Hirschprung's, the patient with Hirschsprung's disease who is not doing well after their pull-through with obstructive symptoms, cause of course, there's a whole discussion about patients with stools that go too fast. Today, we're gonna be talking about obstructive symptoms. Rebecca, welcome. Hey, thanks so much for having me. Really excited about everything and talking on this podcast. That's Doctor Rebecca Rentia joining us from Children's Mercy in Kansas City. Why don't you start by telling us about your case? Yeah, so we have a seven year old child who presented, uh, for the first time to our emergency room with a history of Hirschsprung's and a, uh, pull-through of unknown type. That was performed at 10 days of life at a referring institution. And basically, they came, the child came in with a very distended abdomen, and that's really all that we knew when the child presented. So, it basically, in summary, you have a 7 year old child with known Hirschprung's, had a pull through, you don't know what type, you can't figure out what type. And they are behaving like they are obstructed, so much so that they're having the ultimate obstruction, and that is uh enterocolitis. Correct. It's an older child, which always presents some of the, the big questions that we'll talk about. Yeah, and so, um, there they are in your ER distended, looking sick, tachycardic, dehydrated, and potentially having some transitive bacteria and uh translocation, behaving septic. It's a problem. Signs and symptoms here that Doctor Levitt is describing are non-specific findings you see in Hirschprung's enterocolitis. First line of attack, first line of attack after an abdominal X-ray and labs and a good physical exam is to try rectal irrigations, which were not tolerated. Oh, so you didn't explain to the seven year old child that rectal irrigations were gonna, gonna make him feel better, and he immediately said, whatever you say, doc, thank you for helping me. Correct. So, basically, we have this child with a really distended abdomen, a trial of irrigations that ultimately to get any of the irrigation done, we had to sedate the child. It's a very important discussion. I, I, let's take it back a notch. If you have an obstructed patient who had a previous history of Hirschsprung's disease, we're sort of alluding to the fact it's awfully nice to know what Hirschprung's pull-through they had in the past. And I really want to emphasize this point, particularly to the gastroenterologist, that the anatomy of the pull-through done originally could very well explain the patient's symptoms. And we need to know what that anatomy is and whether there's a problem with that pull-through that we could ultimately fix and make this kid no longer behave obstructed. And the three main types of pull-throughs that Doctor Levitt is referring to here is the Swensen, the Suave, and the Duel. We'll go into further details later on in this podcast. All right, Rebecca, what was your next course of action? So our plan here for this child, one of the first things that we did was to admit them because we needed to figure out how to make them better. So, we initiated IV antibiotics. We started sedated irrigations. On rectal exam, there was no obvious stricture that could be felt, that could be the cause of this. But other than that, that was the first kind of steps. And so the child was admitted to getting 3 times a day, sedated irrigations, and then our next step was kind of what do you do with this slightly older child who's pretty old. Can we, can we step back a second? That's Doctor Jason Fisher from Cincinnati Children's. So on rectal, on the initial rectal exam. Or attempted, I guess in combination with the attempted irrigation. Did you get an explosive, what came out? What was the result of that first exam? Was there an explosion of stool? Cause I think one key thing to talk about with all our listeners is when you do that exam, make sure you step to the side. A little bit funny, but a little bit, you don't wanna be in the way of what could be coming out. So what came out on this child? What did you find? Clearly, a, a seven year old's hard to do irrigations on any child, especially we could talk about, and this wasn't your patient, did this child ever have symptoms like of enterocolitis before? Did they ever get irrigations before? Were they ever, was the family ever taught about this? So just curious, a little bit more in retrospect, what, what was there? So, um, so on examination, there was a large amount of gas that came out. Um, when the catheter was passed up and on digital exam, a significant but non-explosive amount of foul smelling stool that was liquid was. And then when the catheter was attempted to be passed to do a complete irrigation, you could feel that there was harder pieces of stool down below as well. So it was kind of a mix of all of the things that you might see, but definitely the foul smelling stool. And This child, after discussing more with the family, their past history, they've never been on irrigations, but prior to coming to our hospital about 3 to 4 weeks before that, they'd had 2 to 3 other significant similar episodes, one of which landed them in the PICU at, at that other hospital. Wow, yeah, so pretty significant past history. Let's talk about the possibilities here of what could be wrong with. This pull-through. So why don't you give me a list of the anatomic reasons why a pull-through is not working well for a patient. We also have with us today, Doctor Hira Hammad from Seattle Children's. Right, so every time we have a post- pull-through patient who's presenting with obstructive problems, you know, any type of pull-through can have a stricture or a twist. That's regardless of the initial type of pull-through they had. And then specific to the type of pull-through, um, you know, if they have a previous Suawe, we have to think about swaawe cuff. If they have a previous Duomel, then we have to think about a Duomel spur or just this large distended segment that's just not functioning. And then there's other types like with Swenson, you could potentially have a stricture, and as I mentioned, a twist as well. The other thing to think about is depending on when they have their pull-through, there's other types like Raine procedure, which could just present with this non-functional segment that just does not empty. So all of those are mechanical. Problems with the, with the pull through segment. Yeah, that's great. Those are, uh, that's a very complete list, and I think if you can get the original uh operative note, great. But if you can't, a contrast study is really helpful, and sometimes you can infer the original surgery based on the findings of the contrast study. So I think you really need to be an expert. At looking at these contrast studies and getting your radiologist totally in tune with how to look at contrast studies post pull-through. So, I suspect, Rebecca, after you settled this kid out with some sedation, some irrigation, got him in control, got him some IV fluids, Your next step was probably a contrast study. Am I right? Yup, correct. So we got nothing concerning on the X-ray. I think another important point is that a contrast study, while extremely valuable, um, in a, in a very, very ill child would not be the best option. But since They did improve significantly from our initial maneuvers and there was no large amount of speculation or some imprinting on the X-ray. We went ahead with the contrast study. Let, let's go back one second because I think we all just touched a little bit, but a really important. Subject in this situation is how sick these children can get. And we have, uh probably, I assume everyone on this call has seen a child really sick from Hirschprung's disease. You mentioned just before, a couple of weeks earlier, this child was in a PICU at a different institution. So these children can get really ill, and IV hydration, the rectal irrigation, starting systemic antibiotics, all important steps. I think we all agree the irrigation is extremely important, and then these children can be very behind. In their fluid resuscitation and making sure their resuscitation is good. In fact, we had a child get so sick a few years ago, we developed a protocol. We'll link it to, to our podcast, going over all these things cause you know, these things happen at 2 in the morning, they don't happen at 2 in the afternoon. If you're listening to the podcast in the stay current app, you can click on the link below to see the protocol. You know, I'm, I'm glad you mentioned that because the gastroenterologists and pediatricians on the call. I think it's important for them to understand why the patients get so sick. Now, of course, they get, um, they're used to patients with very bad diarrhea getting very dehydrated. But I think the same concept is happening here. They're getting diarrhea, but the poop is not coming out because they're obstructed. But their colon is filling with liquid stool with a severe amount of bacterial overgrowth, lots of loss of fluid into the lumen of the bowel, hypovolemia, and then, of course, translocation and bacteremia from that. All of that is happening with no passage of stool. However, of course, you see that the patient is distended. So I think when a pediatrician has someone in their practice that had Hirschsprung's disease, A little bit of diarrhea or certainly problematic distention, irritable child, fever with distention is enterocolitis until proven otherwise. And as you mentioned, you gotta break the cycle, you gotta stop the stasis, and irrigation is the, is the best way to go because they're not passing stool because of distal obstruction. And it's important to remember this can happen before surgery. It can happen after surgery, and in a baby, it can happen after successful surgery. And we all know that, we talked about that on another podcast. The patients don't relax their sphincters, they're not coordinating their sphincters, they can hold their stool in so efficiently that they also can develop enterocolitis. But this case at age 7 to me, sounds like we're gonna find something anatomically. Or pathologically, we didn't mention that wrong with this pull through. So, Rebecca, let's go over, let's take a look at the contrast study and see if we can learn anything from it. So before we move on, I know I've, I've always had this question, you know, how do you diagnose enterocolitis, and there's several criteria that are out there. Is there one that you guys prefer over the other, or as Doctor Levitt just mentioned, you know, clinically, if you have then you just start the treatment. Well, I'll start by saying, if you have a patient that comes in with a diagnosis of Hirschprung's in the past and comes with, comes in sick, you have to assume it's Hirschprung's associated enterocolitis. None of this treatment will injure or deflect or cause harm. And, if the diagnosis is different, let's say it turns out to be Crohn's disease or um just a E. coli, enterocolitis, or whatever it might be. If you give rectal irrigations, IV fluid resuscitation, and systemic antibiotics, you're not burning a bridge. Or causing any harm from a different diagnosis. And Jason, I'm gonna underscore that point. I think there's a lot of new literature that just came out talking about various protocols of treating enterocolitis at home or maybe having a um lesser length stay in the hospital, but I would say the underlying point is that there's either a really, really good System whereby children are known and evaluated and so they can have a treatment course at home versus they're in the hospital and there's a protocol to be able to address their needs so that it's basically it's better to overtreat than it is to miss this diagnosis. I think that's a fantastic point. You're dead. I, I also, you know, you have to be careful when you read the literature and they quote percentages of enterocolitis. What do they mean? Do they mean admission? Do they mean needed irrigations? Did they mean needed antibiotics? Um, there is a very nice score. Um, I know the PCPLC, the consortium is working on validating the Langer score of, um, HAEC so that we can really apply it uniformly. When you read articles about Hirschspring disease, just be aware. I will tell you, we did study, Jason, I'm sure you remember, post- pull-through enterocolitis in Cincinnati and in Columbus. Within the 1st 3 months was about 20%, which is a significant number. We, we put our, pulled our group together in all those cases, and we're trying very hard to reduce the rate, and it doesn't seem like we're succeeding in figuring out how to do that. In my personal practice, I make sure the families know how to do an irrigation. They have the supplies in order to do it. Obviously, you need to wait a week or two after the surgery and maybe the first irrigation should be performed by someone who actually feels confident, knows where the anastomosis is, etc. but families need to intervene. There's nothing worse, there's nothing worse than having a patient in an ER with mild symptoms that could have gotten the patient better very quickly. With an irrigation, I, I think, I think we're hitting a huge point that obviously is not this case that Rebecca is presenting because the patient was treated elsewhere and, and new to your, your institution and, and your program, but I think everyone on this podcast would say you do not do an operation on a Hirschprung's patient until the family can demonstrate that they are able to do irrigations. Um, beforehand. I know I don't, and, and it's a must, and we make sure they have the supplies, and on discharge, there's a whole coordinated effort to make sure they go home with a goodie bag of supplies to take care of doing an irrigation, cause, uh, let's be honest, it could, a simple thing of a Foley catheter, some saline, and a syringe, and a, and a bucket could be life-saving. So I think it's extremely important. Irrigation is CPR of the colon is how I compare it to family. I love it. Love it. All right, back to the case. Let's talk a little bit more about the contrast enema. The contrast study, for those of you who have the app. You can take a look at the contrast study, and I think it's very, very interesting, very instructive. So let me describe it to you for those of you who can't see the picture. Mark, just to reiterate, and I know we touched on this, it is not vital to get this contrast study on the initial presentation for this patient, right? We resuscitated the patient. Patient responded like Rebecca said to the resuscitation. And now we're digging for the diagnosis. Yeah, absolutely, and I would also say, just so you have all of the items you need in your armamentarium, if you have a patient that's so ill and sedation in the ER doesn't work, I would go to the OR, general anesthesia. Irrigate, irrigate, irrigate until they feel better. And there are some circumstances where you need to do an ileostomy, just to get the kid out of trouble and then work up what's wrong with the pull-through down the road. That's quite rare, but be prepared. So would you do a biopsy or consider doing a biopsy at the same time, or would you work up this patient later? Well, I would certainly, I, I mean, it's a good question. I would certainly do an exam under anesthesia. And let's talk about that. Wait, let's go back. Hold on. Are you asking at the time you go do the ileostomy or at the time of the workup of this patient? Yeah, no, at the time of the workup. So this patient is really sick. We're taking to the OR under sedation, and we do a ton of rectal irrigation, and then the fluid is clear. Jason, I, I knew what she was saying. I already speak here, um, after 3 years. I'm very fluent in Hira, um, but, um, the, uh, I mean, it's sort of a, we have to answer this in two ways. If we end up going to the operating room emergently. We're obviously gonna do a rectal exam. Um, we're gonna do irrigations, etc. Let's talk about that rectal exam in Rebecca's patient. She's gotten the patient better. Irrigations have worked, the kid's properly hydrated. Now it's time to figure out what's wrong with this pull-through. Contrast study, we're gonna talk about, let me describe that, and then from there, the next step here, uh, maybe you'll go over what exactly you're trying to feel on that rectal exam. What, what data are you trying to understand to make an explanation for why this pull-through isn't working. For those in a, in a logical workup, I would, if, if you're not forced to go to the OR emergently. I think we all agree we get the contrast study before going to the operating room for a rectal exam, which we know we're doing also because he might unravel something or, or give you some hints into what to do next. But let's talk about this contrast study. So I'm gonna, I'm gonna describe the contrast study, but I'm not gonna give you my impression of the contrast study. I'm just gonna say what I see for those of you to try to, while you're in your car or you can't open the, you don't have the app open. Though there's a very dilated colon here. And it narrows down for the distal, maybe 6 centimeters, um, and it stays narrow in all images. Um, the other thing I noticed, and this is something very, very important to look for, is there seems to be space between the hollow of the sacrum and the pull-through. The presacral space is a little wider than is normal. So, those are my observations, and now I'd like to know maybe um Jason or Rebecca's opinion of what those observations mean. No, I agree with your description. I think it's important, and, and whoever did this study did a great job of getting a lateral of that rectum, cause it really gives you a lot of information. Um, like Mark said, I think we're trained to look at that space between the sacrum. And the rectum, and then, this looks to be increased uh uniformly throughout this narrowed portion of the most distal part of what I'd call the neorectum for this patient. But Rebecca, why don't you tell us exactly what, uh, what happened there? Yeah. So, um, the, uh, this contrast study, interestingly, because the child required so much sedation to do irrigations, this was actually a contrast study that was performed in the operating room prior to another irrigation, um, with the examination under anesthesia. And so, um, You can see in this study also that um there's a good seal, so all the contrast is not leaking out. The catheter also has not been inserted too high or the balloon is, it's not put under too much pressure because some of the contrast studies if there's um uh basically the low pathology cannot be ascertained easily on a contrast study. And so I think they did a good job on this study. Um, radiology joined us and we're able to get a lot of information here, um, that we could then kind of confirm on exam. So Rebecca, I, I agree with you 100%. What was your working diagnosis theory going into the OR with these images that you already had available to you now? Um, that there was either, uh, so that there's an anatomic issue and it's or a pathologic issue, um, in the lower kind of 6 centimeters of the bowel. Of the pull through. Right, I mean, to me, it looks one of two things. Either you have retained a ganglionosis, you have a pathology problem, or, and again, we don't know if this was a suave, and I'm, with these images, I think it was a suave, seeing that there's so much space between the sacrum and rectum like Mark had mentioned earlier. Is this a pretty long suave cuff that's constricting that most distal portion of neorrectum. Yeah. We pulled through Bal. All right, and unfortunately, we are out of time for this episode. In summary, we discussed a 7-year-old who presented to the emergency room with non-specific findings of Hirschprung's enterocolitis with a history of a previous pull through at 10 days of life at an outside institution. He came to our facility and we did general management with, uh, first starting with rectal irrigation and antibiotics and IV resuscitation. With the most important of these steps really being the rectal irrigation, they are key to the child getting better. And remember you can see uh Hirschsprung's associated enterocolitis protocol that is attached to this podcast within the Stay Current app, so click on the link. And lastly, the contrast enema findings demonstrated a dilated colon with a narrowing at the distal 6 centimeters with a presacral space between the pull through and the sacrum. Remembering the importance on these images to have not only the anterior posterior, but also lateral. With these images, we thought that there was most likely an anatomical or pathological issue with the pull-through in the distal 6 centimeters and that this could be either retained ganglionosis or that this is a swabve that is constricting the most distal portion of the pull-through bowel. All right, and now to our colorectal joke of the day. Did you know that Google Has a platform now for recording your bowel movements. You know its name. Wow, are you, are you serious? There's an app or, or you can do it on, on Google. It's called Google Sheets. That's a good one. All right, that wraps up our colorectal quiz. This was episode 19, Hirschsprung's Disease and the Obstructed Patient, Part 1. next week we'll dive deeper into the rectal exam under anesthesia. Remember, knowledge should be free. This is Amanda Jensen from Riley Children's.