Update Course Rewind: 2020 Colorectal Part 1

Hey there listeners. Did you miss our annual pediatric surgery update course? It's a virtual conference that we put on every year to talk about groundbreaking research, practice changing articles, guidelines. Well, don't worry because you can watch it on our website, or you can listen to this podcast where I'm going to break down our favorite sessions from years past. And we've got a lot of content that we can bring to you. So enjoy what we're going to call our update course, rewind. I'm going to start our section on bowel disorders and start with Hirschprung's disease and some of the problems we encounter as surgeons after the surgery's been completed. That's Dr. Megan Durham from Children's Healthcare of Atlanta. And as we all know, it's not as simple as just pulling out the bowel that has no ganglion cells. So the first case that Dr. Durham presents is a 35 week gestational age male who had abdominal distention and failure to pass meconium after 48 hours, which is why we got consulted. They do a suction rectal biopsy on day of life 3 that confirms Hirschberg's disease, so on day of life 9, he undergoes a lap-assisted suave with a mid-sigmoid transition zone. About 5 centimeters of proximal colon with normal ganglion cells in the submucosa. And now they're discharging at day of life 26. So, what would you give this child as a home regimen? I personally will bring you back about 2 weeks or, um, 2 weeks after surgery and I'll calibrate the anus to make sure that anastomosis is OK. And as long as there's, doesn't feel like there's a cicatrix or a narrowing there, um, you, you can decide what to do with the patient at that point. But again, very commonly, very early on, there's not problems. It's usually a little bit later, like we'll see in this patient that problems start occurring in the, in the young child. Um, I'll add in my two cents. I don't, um, do any routine therapies afterwards, um, especially initially just because your anastomosis is healing, so any sort of, um, insertion of, uh, either your finger or, uh, a dilator would have the risk of, um, disrupting your anastomosis. That's Doctor Eunice Huang from Children's Hospital at Vanderbilt. And I, I really think that one of the reasons that there's probably not a standard method is because each patient is a little bit different. Um, I think for all of us who are taking care of these patients, you have the realm of, um, where you feel like you've done the exact same surgery on a patient and some just do beautifully and you, um, think about 3 months later what happened to that patient. They, they obviously have not come back to see me cause they haven't had problems and then the patient that's in your office every couple of weeks because you're not doing very well and so I think it's very important to tailor. Your treatment for these patients to that individual. So what happened with this kid? So this patient, um, he, I did see him early on, and he did show some early signs of constipation, so we eventually did put him on some laxative stimulants on a daily basis. But then he's lost to follow-up. He represents when he's about 2 years old, with the X-ray that you can see if you're in the stay current app. So scroll down under the media player, you're gonna see a plain abdominal X-ray, click on that. And that's how he presents. So they do a DRE which doesn't identify any stricture, and he gets a barium enema that doesn't show any twist or obstruction. So they start treatment with IV fluids, irrigations, and IV antibiotics, but think to yourself, what would you do next? So for me, I would say that I, um, it would depend very much on how that patient has been doing, um, recently or over the course of, of, of the time that he has pulled through. Um, I think if the patient's been doing very well and this is the first episode of enterocolitis, I probably would be more likely to Not intervene very much short of treating the enterocolitis and making sure he recover, he or she recovers appropriately from that. I think if this has become more of a chronic problem or recurrent enterocolitis, I'd be more worried about all the things that we worry about, um, an anatomic problem, um, a, uh, physiologic issue with, um, dysmotility of the, um, the ganglionic segment, um, or something, you know, um. Technically wrong with the, the pull through, such as the twist. So, how does your hospital manage Hirschprung's associated enterocolitis in the acute setting? Well, here's what Doctor Durham said they do at Children's Healthcare of Atlanta. Yeah, so at our hospital, we don't have a fixed NPO time and we don't typically start TPN right away. We do start irrigations, usually about 10 ccs per kilo of normal saline every 8 hours to get at least for the 1st 24 to 40 hours and see how the patient responds. And we typically start IV Flagyl. But they were quick to mention that it's hard to standardize this treatment because as you probably know, patients will show up in Any part of the spectrum, from the patient who comes in with a little bit of a white count and some X-ray that is concerning for some distention, versus the patient who is grossly distended with really obvious signs of shock, those patients are gonna get different treatment, but regardless of where you're practicing, it seems like the tenants are the same. So once that child is resuscitated, and you're probably not gonna do a procedure right then and there. They're going to go home, and what are you going to give them for their home regimen? I would send them home on, on, on, on Flagyl. I would send them home on Some laxatives and probably some rectal irrigation just to keep them moving for um some period of time. My bias for this kid is he, he, he's, he's, for some reason, one of those kids that dilates their colon. There are some kids who don't. This kid obviously does, so they're sending him home on nothing would just be expecting him to come back in in the same situation a few months later. Something needs to be done more for this child. Yeah. But you know what could be standardized. Um, if you appropriately teach the parents how to do a washout and give them the tools to do so, it just allows them to have to have a little bit more freedom that they could do that initial washout if they see their child getting sick, and, um, it's, I think it, it improves their quality of life at home. That's a good point. I think that's, that's very important, but we've also, so we here have recently come up with a very standardized teaching regimen for our different campuses. Um, we also have found that there was a lot of education that needs to be done on the floor while they are inpatients, and there's, because this is not an everyday common occurrence, the nursing staff very commonly will have a different level of, of education based on. Rectal irrigations, rectal enemas, and so there is, we, we, we do have a regular education process that we do to the floor nurses to make sure they understand. And we also have come up with a standardized order form now so that it's much more clear for the nurses about types of tubes, how it's supposed to be done with a, with links to, um, policy. So it's been, it's an education for parental needs, but also education for in-hospital to make sure the care is, the care is delivered as you expect in the hospital as well. So at this point, if you're feeling a little bit overwhelmed or confused by the complexity of these patients when they come back, Check out the next image we have for you. It's a guideline. So here's, this is one guideline that's come up. Doctor Langer published this, um, with APSA Hirschprung's Disease Research Interest Group. So, if you're in the app right now, you can open up this guideline, look at it, read through it. If you want to read the article in its entirety, I've put a link in the description. So scroll down, click on that link, it'll take you to the article. But the long and short of it, this is an algorithm for the diagnosis and management of a child with obstructive symptoms after they've had their pull through for Hirschsprung disease. So, let's look at the main decision points here. Obviously, starting from the rectal exam and the contrast enema, you may have to move to a rectal biopsy. What does the rectal biopsy show? That could take you to a botulinum toxin injection, and then you see how the child responds to that. Ultimately, if you get to the bottom of this, you can see that they may need a motility workup. The results of that workup will either take you to, does this child need further colonic resection, or do they need bowel management, a stoma, or an ACE. I'll give you a minute to look at it. And when we come back, case number 2. I have one other patient that we'll talk about, and this is probably a more difficult. The babies that are obstructed show up when they're a few months old or a couple of years old, because if they're not passing stool, it's a very easy sign for parents to see. This child had the opposite. He's um hypermodal. He's having fecal incontinence. So, if you're in the stay current app, open up the next image. That's for this case. And you'll see a contrast enema. Now, this child is a 9-year-old male. He had a pull through on Day of Life 3 because he had a transition zone in his descending colon, but then when he was 7, he needed an ACE procedure. Unfortunately, this poor child was soiling pretty frequently, needing to change clothes maybe 2 or 3 times a day, so had to be homeschooled, and currently getting flushings with 100 cc's of normal saline and soap. So, if he's in clinic with you, what do you do for this kid with that story you have? Well, the first step is to try and slow the patient down. Now, during this conference, most of the respondents wanted to slow them down with anti-diarrheal meds or a constipating diet, but that's a conversation to have with the family. The next step, the part that you're probably waiting for is what happens when we go to the OR. Um, for this child himself, he, he underwent, we went exam under anesthesia, intact sphincters. He had an intact dentate line, went on to manometry studies, but he had HAPC, so he had these high amplitude contractions that were up pressures upwards of 400 millimeters of mercury all the way down to his anus, and I don't think anybody can control. That type of pressure coming down to your anus. So this patient was found to have pseudo incontinence with hypermotility. For him, they were able to adjust his fiber intake, his daily Imodium, and added cholestyramine, and they were able to get him back in school with other kids within about a year. So, he ended up getting a good result out of it. So there you have it, a couple of interesting. Hirschbrung cases brought to you by some experts from across the country. Did you enjoy it? Or maybe it was a refresher because you were at our update course last year, or maybe you want more of these. Well, don't worry, we got plenty more of these update course rewinds coming your way, as we lead up to the 2021 update course. Mark your calendars. It's August 20th. There'll be more information to come. But until then, I'm Rod Gerardo from Cincinnati Children's, and remember, knowledge should be free.