Thanks everybody. This is uh great to be here today. Um, I'm going to start our section on pers um on bowel disorders and start with Hirschsprung's disease and some of the problems we encounter as surgeons after the surgery's been completed. And um, as we all know, um, it's not as simple as just pulling out the bowel that has no ganglion cells. Um, it there are problems that persist in certain patients and um it's often times hard to predict which one, which patients are going to have trouble. So we will um go through a process that's um trying to evaluate how to um take care of these patients. So the first patient that I'm going to present is an ex 35 weeker who did not pass stool in the first 48 hours. He had a suction rectal biopsy that confirmed Hirschsprung's and day of life three, underwent an uncomplicated Suave type laparoscopic assisted pull through on the day of life nine. had a normal expected mid sigmoid transition zone and had five centimeters of proximal colon that was resected with normal ganglion cells and no hypertrophic nerve trunks during that procedure. He was discharged on day of life 26 and um was doing okay before discharge. So the first question, I and I'm not sure any of my questions have one right answer. Um I think there's a lot of opportunities that you know, we as a group can come come to uh to help try to re make these regimens more standardized. But the first question is are there routine regimens that Hirschsprung's patients always get sent home on? Are there routine postop dilatations, routine postop irrigations, stimulant or osmotic laxatives or you just send a patient home with no uh routine therapies and then treat them as problems arise or is there another plan uh to treat them? I know I typically, I mean, I'll tell you what I do and I again, I'm not sure if there's any correct answer. um and we'll see what what the audience thinks. But I typically I'll send patients home but see them back pretty quickly. Um typically as babies are start stooling, um there are no problems with stooling, but as they as the healing process goes on and there can have a narrowing of that anastomotic area, they sometimes can come back with um structure or an outlet obstruction type problem that just might need some dilatations. The baby's stool at this age is often very liquidy. And so it doesn't help maintain that that umsis that may be trying to narrow down in the normal healing process. It looks like the poll results are coming in and a overwhelming amount of folks are saying no home regimen. That's the most popular answer is uh is nothing. And then uh shortly after that, it looks like there are some people who are saying saying rectal irrigations uh and then uh daily anal dilatations is the third most popular there. And I think it may change. I mean I I personally I'll bring it back about two weeks or um two weeks after surgery and I'll calibrate the anus to make sure that anastomosis is okay. And as long as there's doesn't feel like there's a cicatrics 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 I tend to also probably along with the based on the the survey results, um with the uh majority that I don't um do any routine therapies afterwards. Um especially initially just because your anastosis 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 anastosis. Um and I I really think that while 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 in three months later what happened to that patient. They they obviously have not come back to see me because 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 um uh patient and their results. And that's a great point. 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. Um, at one point he was lost to follow for about a year and he presented back with this X-ray. So at this point he's about two years old. Um, he comes in with fever, an X-ray that looks like the the one shown. Um so immediately in the ER we did a digital rectal exam. He didn't have a stricture. We then did a barium enema which didn't show a twist or an obstructing cuff. So we admitted him, placed him on IV fluids, we irrigated him and we placed them on IV antibiotics to help treat the apparent Hirschsprung's enterocolitis. So you've got this patient in your hospital now and uh what are the next steps because this is obviously an obstructed postop Hirschsprung's patient. And so we're going to work through how to work handle each handle this patient. So the next question is, do you just do a simple anal botox injection? You know you've shown that you don't have a mechanical obstruction from a surgery that maybe was done not perfectly. Do you divert him? He's got a very dilated colon on that left hand side, is it just time to give him an ostomy? Um, do you do a four quadrant rectal biopsy and anal botox, a strip rectal biopsy and anal botox? Do you just redo his pull through at that time or do you treat his Hirschsprung's enterocolitis, let the inflammation get down and come back and do some surgical options in the future. Um And again, I'm not sure there's any right answer. I think some action needs to be taken. Um but what would be the initial thought from the the audience? 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 oftis, I probably would be more likely to not intervene very much short of treating thetis and making sure he recover, he or she recovers appropriately from that. I think if this has become more of a chronic problem or recurrenttis, I'd be more worried about all the things that we worry about, um an anatomic problem, um a uh logic issue with um ty of the um the gangonic segment um or something, you know, um technically wrong with the the pull through such as a twist. Ands and Megan, can you elaborate on what your uh management oftis is at your hospital? Do you have a fixed amount of NPO time? Do you start TPN? Do irrigations, how often? Yeah, so at our hospital, we uh 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 eight hours to get at least for the first 24 to 48 hours and see how the patient responds. And we typically would start IV flagil for the patients when they first come in. Ours is similar and um, you know, I think one of the goals for us is to have a more um structured protocol for treatment oftis and that, you know, Asco's has been working a lot on that in terms of appropriately definingtis and then um coming up with a standard uh management for that. Um, but part of it is because it it is a bit of a spectrum because sometimes patients come in, they have a normal count, they look distended but they don't have any other signs of sepsis. And in those patients we're more inclined to just start IV flagil. For patients who have other signs of sepsis, we're more inclined to do broad spectrum antibiotic um add on top of gram negative coverage. Um, we tend to leave them NPO initially because they usually present with vomiting as a symptom. Um and then we're fairly aggressive about initial um wash out two or three times a day until they clinically um uh improve and then really kind of um oral start um just is a is a clinical decision based on how the patient's looking. And then the question is do you send them home on uh standard flagil therapy for a few months or is it a single course? And these are all questions that are currently not not answered, I don't think. so. And do you send them home on irrigations if they've been admitted fortis? We I typically do. I don't think as a standard our hospitals even we're not even standardized here but I typically do for at least at least a few weeks to try to make sure that they continue to have a good uh stooling process and they're not not getting backed up again. But these are great opportunities. Yeah, I'm looking at the poll results and it's interesting. So the vast majority don't really do much. Um, it's about 46.7%. Now, if you take the two uh biopsy uh to biopsy options together, they're both about 15% a piece, so they're a close second. I think that it was it was split pretty evenly until you started your discussion and then it started to more people uh shied away from the biopsy. So one of the questions in the uh in the polling that or in the chat room is about sending them home with probiotics. Do either of you use probiotics? And then the other question was, is there any role in this setting formetry? Those are great questions. Um in terms of probiotics, um I think our again, there's there's no standard way and I probably there's eight of us here. We probably all give you eight different answers. But I think the general trend is to use probiotics as a chronic management and less so as acute management of um somebody with um Hirschsprung's. In terms of analrectometry, we do have that availability here. Um, but we do it in the outpatient clinic setting and so I find it a challenging um uh uh diagnostic study to use on the very small child who may not be able to um sort of uh um sit still for the appropriate uh measurements. And for me, I think we may have eight different ideas about how to use probiotics. If you just look at the probiotics in the market, there are none of them are standardized. So I think that adds in another layer of non standardization to probiotic therapy. So I think um even if we are we are using them, we may not be using the same thing. So I think that that's kind of an unknown how beneficial it is, but I do agree it's more of a chronic thing. Um and and I think in this patient, you know, with that dilated colon, I thinkmetry is very useful and I think it it does it is part of the paradigm, but in the in the situation where you have a very dilated colon anal analmetry and even uh colonicmetry, the results may be varied because you have an actively dilated colon and you haven't it's not decompressed well enough to get good results. So, after you've treated him now fortis, I guess we've kind of gone into this, what do you do for him then? We've talked about I would send him home on on on on flagil. I would send him home on some laxatives and probably some rectal irrigations just to keep them moving for um some period of time. Is there anybody out there who who wouldn't send him home on anything or is that is that a choice from uh either a one time Hirschsprung's presentation or this child who maybe has a more chronic problem because his his colon is obviously very dilated um on the left hand side. My bias for this kid is he's got he's for some reason one of those kids that dilates their colons or some kids who don't. This kid obviously does so there's 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. I agree with Megan. I think that um so one of the one of the components for me as I think about patients I care for with Hirschsprung's is how to equip the parents appropriately to be to handle problems at home. And so I think irrigations is one of those things that um it's a tool that you have to teach them. If you feel like the this patient's going to be somebody who um is likely to have some level recurrence of of this problem. And so um I I think one of the challenges for a lot of families are just um when do they show up to the emergency room? When when is the um is their constipation bad enough to show up to the emergency room. And if you um if you appropriately teach the parents how to do a wash out and give them the tools to do so. It just allows them have to have a little bit more freedom that they could do that initial wash out if they see their child getting sick and um it's I think it it improves their quality of life at home. And that's a good point. I think that's that's very important. Well we've all 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 in patients and there's because this is not a everyday common occurrence, the nursing staff very commonly will um have a different level of of education based on rectal irrigations, rectal enemas. And so there is we do have a regular education process that we do to the floor nurses to make sure they understand what are and and what those is very important 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 all with links to um policies. So it's been it's an education for parental needs but also education for in hospital to make sure the cares the care is delivered as you expect in the hospital as well. Hey Megan ors, um me ask you a question. Um, as uh I was interested as y'all were talking, is there any evidence for anything that that y'all have mentioned? I mean, it seems like this is a right topic to try to get some better evidence for that. but I'm just interested to know, is there evidence for what y'all are for any of these um suggested treatments. No, that's why I don't think there's any right answer in these and I I'm hoping that we can come, I mean we we can come to some some better plans and recommendations for for us. Um but I I think we're at the beginning stages of kind of coordinating what is just likes mentioned, what is and come up up a scores to make sure we diagnose it correctly. And then as I'll show some um some pathways of how do you work these patients up. If we're not working them up the same way or treating them the same way, it's really hard to draw conclusions. So I I think you're right, there's no there's there's not a lot of data. Um but there's a lot of opportunity to try to come up with that in the future. And I think that's something, you know, for those who are interested, the Apsa Hirschsprung's um interest group is working on that. Um, like Megan said, I think I think definingtis if we could do it appropriately allows us to actually have better data on outcomes and we don't define it well. So in in all the retrospective studies, the um appropriate management and outcomes are all all over the place because, you know, somebody coming in with constipation, you may categorize astis and put them on flagil, but that's really not the same patient that comes in hypotensive, cardic uh with an elevated white count. And so to be able to appropriately uh risk stratify patients initially is useful. And so yeah, I mean the the idea of what you should send these patients home with in terms of length of therapy, both on um wash outs and on antibiotics is um is is relatively undefined. So here's this is one guideline that's come up. Dr. Langer published this um with Apsa Hirschsprung's disease research interest group. And it just looks like for the kid the child who has Hirschsprung's who is who's obstructed post operative, how do you work them through? And so the first thing is what we've got outlined, you examine them. You um you make sure there's not a mechanical obstruction. You then proceed on to rectal biopsy if there's no mechanical obstruction but but the signs of obstruction still persist. You either going to find that you had a good procedure done before that has no significant pathology. Um and then you move on to trying to treat their obstructive symptoms with their we all know that with an absent rare, they're going to have somewhat of an outlet obstruction at their anus. And so the Botox has been very helpful to try to overcome some of that um intense uh contraction of the of the anal sphincter. The problem with the kids that are the ones who don't improve very well and those you do move on to motility work up whether it's sits markers for a transit or you do formal motility studies with either with both anmetry to see if they're withholding um or if they have a functional area they're calling that maybe isn't working well that needs to be resected because it's acting as a common cavity and it's just not letting the normal progression of stool passage occur. Um, and then there's also the children who then are still having some difficulty and then you move on to a bowel management with either astoma, which is um usually not the family's first choice or an antigrade continence catheter or procedure that allows you to wash um antigrade enemas um in a antigrade fashion. I do have to say, you know, the the Hirschsprung's kids are a little more difficult than the rectalformation kids because they do have an intact sphincter and that outlet obstruction at their anus sometimes is very difficult to manage even with an ace. Um, um, but it can be done and this is the next step for those kids who are acting obstructed. So I think this kind of thing is the first step to trying to to study kids long term to make sure we're all doing the same thing at the beginning. Now we have one other patient that we'll talk about and this is probably more difficult. The babies that are obstructed show up when they're a few months old to a couple years old when 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 with Hirschsprung's disease. and those kids often present a little bit later, not because anything's occurred, but early on in life if patients are all um in diapers and they're stooling in diapers, then stooling too much may be missed. It's not until they get into kindergarten or school age when the fecal soling becomes more of a an identified problem. Um so this child had Hirschsprung's disease diagnosed as a newborn. He was treated at an outside facility. He had a pull through on day of life three, normal transition zone in the descending colon. He uh went to kindergarten and then was quickly withdrawn uh because he was having so much fecal soling. He has two older siblings and they both were in in school, but he needed to be homeschooled due to his fecal soling. So he had an ace procedure performed at seven years of age. He then relocated to Atlanta, came to our outpatient clinic and he was soling. He um was at that time getting 100 ccs of normal saline and some castile soap flushed through his his ace tube. But he was soling five to six times a day, not just smears but full bowel movements. Very normal kid, very active, very intelligent, but just needed to change his clothes a couple times a day and that just wasn't allowing him to function in society uh where he should have been at his age. So, if he's in clinic with you, what do you do for this kid? with that story you have. there's not much you can do while you're action clinic. But so what do you what do you tell the family on that first clinic visit? what do you send him home with? What is the next step? And we'll go on to surgical stuff as well, but what do you do for them while you're there? Do you increase his flush volume because 100 ccs for a nine-year old's pretty small. Do you add glycerin to try to evacuate a little bit better? Do you just try to stop him up now with antidiarrheal? Do you give them laxatives, do you give fiber? Do you cons do you add in cospating diet? Um, again, very many options, I'm not sure if there's any right answer, but any thoughts about what the next step would be? I would say, um we're running five minutes behind. So why don't we just skip through like just get a quick answer and we'll do rapid fire for these. So obviously we need to slow them down. so we slowed them down a little bit and then we went on to the operating room to try to figure out what the problem is. And this child, his issues potentially was the opposite of the other kid. Did somebody injure his sphincters and his outlet is not working? Can he not sense things or does he have a a motility problem where he's um just not able to control what's coming down. And for um for this child himself, he he underwent we went exam under anesthesia, intact sphincter. He had an intact dentate line, went on tometry 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. You know as a as a newborn with Hirschsprung's capacitance organ, his rectum was removed. So now he had just had these high flow pressures coming down to his anus and he just wasn't able to physically control them. So in this chart that was um published by PiAM, um he would be considered to have pseudo incontinence hypermotility and we did all the things listed here and we constipated him with um diet, ummine anddium and um we still then use his his uh J tube and we had to move to twice a day irrigations in addition to 24 milligrams ofdium a day, 27 grams of fiber andmine. And he's we finally got him back at school after about a year, but it took about a year to try to figure out his system because everybody's system is a little bit different and but and they and not one thing works for every single child. That's great Megan. Uh there's a lot of great questions being asked in the chat room. I know we're running behind like uh Todd was saying, we're going to try to get through these cases really quickly. Um, so hopefully uh you, Beth and will have time to answer some of the questions in the chat room as well. Great. Thanks. Now I thinks has a case. Or are we going to Beth? Yeah. Uh I'm sharing a case. Um so I'm going to talk a little bit about rectal prolapse. This is a relatively unusual problem, but we recently had two systematic reviews that came out and so I thought it was worthwhile kind of discussing this. Um, so um I'm going to just start with a case presentation. Three and a half year old male presents with episodic rectal prolapse. has had several episodes of circumferential prolapse, um that spontaneously reduced, but then recently the prolapse required manual reduction in the emergency room, which was what prompted the mother to seek further evaluation. So how would you manage this patient? So I'm going to allow people to so we'll talk a little bit about this. So probably the most most common time we see prolapse are when kids are in their um potty training phase. Um, so I think um it's not common for surgeons to really specifically be involved in these patients. Are you seeing responses and any comments on that? I'm actually I'm not seeing responses right now. I think we're having some people with some technical difficulties. Go ahead and type in the chat if you guys have thought Oh, it's popping up now. Here we go. So go ahead and answer the poll. Uh but before we get to those answers, I mean how about some of the uh the moderators here. What do you guys think? What are you guys what's on your mind here? I think this is like a nightmare in clinic. honestly. This is the patient that no one wants to see, right? I mean rectal prolapse is it's very distressing to the patient and the family and we don't really have a lot of of great options. So if it's a patient that I've never met before, I typically will address, you know, concerns about the constipation first and sort of prove or disprove whether or not this patient is significantly constipated. Um, I think if they are constipated, that's like the easier patient to deal with, but every once in a while you get a kid who is well managed on whatever their regimen is and they're still prolapsing. And then I think the question is, what do you do? And if someone has the answer, I would love love to learn it. So, that's that's great point.
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