Hirschprung's Disease - Daniel von Allmen

It's a pleasure to be back. These are great sessions. Now that we've done all the, all the hard stuff, we can do something easy and Crohn's disease. Uh, I have fortunately a couple of colleagues, uh, Jason Fisher, who's on, um, from the Colorectal Center in Cincinnati, and then we have another expert sitting up here on the two actually, uh, uh, Dr. Langer, who has published, and just as a note, I did the participants see the references? Yes, there is a very complete. Seminars in pediatric surgery all about Hirschberg's disease less than a year ago and Dr. Langer has articles in there and it's a it's a great reference for looking at all these various different topics. So for people that are interested. So let me tell you, so you can get that on the side tabs. You can go to, is it called links or downloads files. If you go to the files tab, you can download all the citations for everyone's talk here. And then Doctor Garrison, who, uh, uh, is, has an interest in this, uh, disease process and uh has uh finished training recently at our center. Whoops, I went back to that so. So what I thought we'd do is just go through some relatively straightforward progressing to less straightforward cases and see how the discussion progresses. The first case is your basic full-term infant, now 72 hours old, transferred with abdominal distention, two episodes of non bilious emesis, and the X-ray that you see there with distended small bowel loops. So the first question really has to do with what's the initial management of this patient in terms of a workup, upper GI and then a contrast enema and a suction rectal biopsy, contrast enema, manometry and an open biopsy, contrast enema, then a suction rectal biopsy, laparotomy, rectal irrigation and observation. OK, so, so I, so I would do, uh, I would do C, um, I, I take a patient by patient basis. In other words, I, I don't routinely get upper GIs in every newborn, uh, obstruction. Um, I look at it clinically if they're distended and have a lot of distal air, I'll get a start with below. So I'll get a contrast enema and a suction rectal biopsy. I agree with the newborn bowel obstruction. I, uh, there tends to be a knee jerk, you know, because we've all been. Uh, trained over and over again that, uh, bilious emesis equals upper GI, but I think this is one of the, one of the exceptions, uh, you know, I mean, unless it's a perfectly normal upper GI and you're, I mean, in a BE and you're still concerned, but, uh, I think there's so many things to rule out non-operative and non-operative besides Hirschberg's disease that a contrast enema really helps. Yeah, I would do C also. Is there anyone that would get an upper GI with a baby with bilio emesis in this situation? And I'm surprised everyone here is shaking their head no, uh, but I'm surprised because, um, again, I keep referring back to the course in Washington DC. Uh, where a significant portion of the fellows, uh, said that they would absolutely get an upper GI first and then move on because it could be, uh, a, a volvulus with a distal type of obstruction, and they would get an upper GI first with, and some people talked about different contrast mediums, people using gastrographin for one and barium for the other. And I felt like I was almost a minority there. So it's interesting that in this audience, uh, this they're fellows, yeah, actually they're not, or they do it for us without telling us. Yeah, so usually by the time I get the first phone call, the upper GI has been done, yeah, very proudly say that the upper GI was normal. So 70%, 84%, um, uh, did say C, Dan. So 7% would do rectal irrigations and observations. Interesting. So, I, I, I'm curious of that 7% where they're located. Uh, we've seen that before, um, uh, where, uh, they may not have, uh, the, now it's 20%, um, the facilities to get a contrast enema at their institution in some of the, uh, countries. Um, I, I guess my own training, I always remember Maury Ziegler's, uh, admonition that the baby's first enema should be a barium enema, and, uh, I think here at least in the United States, our approach is, is certainly to do the BE first. Um, I don't know if you're going to get, you, I don't wanna. There was a when Doctor Pena was here and we did uh element on Hirschprung's we were surprised to see a substantial number of people from different countries that went straight to surgery based on a contrast enema and I don't know if you're gonna get into that or not, but I. No, I mean that's, yeah, sure, it's an interesting discussion. Is there anyone who would go to surgery based on the enema as opposed without a suction rectal biopsy or an open rectal biopsy? It'd be great to ask the, well, we can ask the audience that because I'm sure here everyone would say no, but uh. So I think we would say that among the faculty here you absolutely have to have a tissue diagnosis of Hirschsprung's disease. We actually published a paper of false positive contrast enemas in newborns. So there was a transition zone that looked like Hirschsprung's, and it was not Hirschprung. So I think it's a dangerous thing to do, OK. So to move to, uh, uh, I would add there is an occasional patient who is really sick from enterocolitis and you may not wanna wait for a tissue diagnosis. It might take a couple of days to get. I mean, there, you, you don't wanna say never. I think there may be a patient that you have to consider operation and diversion because it's a long segment. You can't get it decompressed with rectal irrigation. They always come in on Friday and you're not going to get your pathology back till Wednesday. Um-hum. And so, I think there is an occasional patient you might have to operate on. Yeah, I think it is a good point. We'll get to entercolitis. In a little bit, but I, I do think that's a different patient population than the standard, what's your standard workup for a routine newborn who shows up with a distal bowel obstruction. So the second question then is, so then what if you get this contrast enema and it shows a sigmoid transition zone like the two examples that are up there, what are people's approaches? And again, I think the international response will be interesting for this as well. Is it a leveling colostomy, uh, irrigation followed by a sort of semi-elective, and let's make that a trans anal primary pull through, a laparoscopic assisted pull through, or an open pull through and pick your pick your pull through type. I, I, I like the laparoscopic approach, um, to get your biopsies and while you're waiting, mobilize the, the rectum and, and bowel. I know it's probably, I know Doctor Langer has shown that it's really not necessary to, and it's probably overly invasive, but in some cases, uh, of a low transition. But, um, but anyway, that's generally my, my approach. You, you don't want to be confused by the facts, is that what you exac exactly. You, you hit the nail on the thumb right there. Well, I mean, we, we have trouble getting, uh, operating time, so we irrigate them until we can get them to the operating room and then I would do a, a transanal with an umbilical incision to do the biopsy first because I don't know if you pull up the bowel and do the, yeah, I put a, a Hager, uh, into the through the anus and push the sigmoid up to the to the umbilical incision. And then I do my biopsies and while I'm waiting for the frozen section, I will take down some of the vessels that are going distally. So essentially the same operation you're talking about except instead of laparoscopically, I do it through an umbilical incision. Sure, yeah, I think I agree. I would do the irrigations until we go to the operating room, but I would do a full body prep. But on the contrast enemas here, I think you could have a reasonable likelihood of getting everything out transanally, so I'd probably start prone, um, and go from transanal approach. And I think I've been burned so many times by that, what looks like an obvious low transition zone and you end up just chasing it and chasing it that I like to immobilize the splenic flexor laparoscopically because half the time I'm, I'm trying to, I'm struggling to do that transanally, but uh. Others with more experience may be able to do it more easily. I don't know. Well, it was 8% in Mana Proctor's paper where it looked like a short transition zone and the pathological transition zone was higher, significantly higher, where they have a long transition zone, which is a sort of a new concept. Jason, do you have any comments? I, I, I echoed the feelings of getting burned a few times, and I don't know, do people have A feeling where I know when I was in New York, I think the female patients, we, we seem to get burnt more than the male patients, and we would, um, often at least do a laparoscopic, start with a laparoscopic approach and take our biopsies that way on the females. Um, I don't know if anyone has any male female predilection for that. I don't. I haven't noticed that. Well, the instance of long segment in girls is fifty-fifty, so your odds are much higher. So, um, 83% were lap pulled through. I, I do wanna make a comment about the irrigations about B because a substantial number of people did say B. Um, I personally do see unless there is some size issue, and what I mean by that is if the bowel is very dilated, I might irrigate for a while to let things sort of decompress, um, or if the baby is really tiny, um, which is unusual because we don't see many preemies with Hirstprungs, I might, uh, irrigate, um, until they grow a little bit. So that's unusual. Um, 90% of the time I'll do, see like. So, so you mentioned Doctor Pena before and one of the, uh, one of the other questions or one of the other issues that I had not been familiar with until I came to Cincinnati was the issue of the positioning. Do you position the patient prone or lithotomy? I had always done these in lithotomy. It gave me access to the abdomen. There's A great experience with doing patients prone in Cincinnati and using a total body prep so that if you need to flip the patient you can just flip them over. It does give you, it's much easier on your back and your neck and everything else to do to do them prone, but I'd be interested if anybody else uses that. Louis de la Tor does it that way as well. Um, I prefer them in the thought of me because I just, uh, I don't like flipping them back and forth. You, you know, you just have to get one patient who loses his endotracheal tube while you're doing that and it kind of sours you on that. Um, Scott Bollinger, my previous partner, taught me, showed me this instrument that I has changed the way I do these. So, I, uh, it's a flexible arm that goes across the end of the table and I tie their legs to it. So, when I'm doing my laparoscopic part, it's all sterile. I just lower it down, do my lap part. When I want to do the lower part, just flip it up. You can flip it up and down as much as you want. You can go from below or above very easily. Um, so that's just another thing. I, um, uh, if there's not any more comments, we have a question from the audience actually calling in. Uh, Doctor Thayer, are you there? Yes. Hi. Um, thanks for calling in. Why don't you go ahead with your question. Thank you very much, uh, for taking my call. It's somewhat of a dilemma. I was presented two weeks ago with a 16-year-old, uh, trisomy 21, fairly low functioning, that, uh, defecates once to twice a month and has severe chronic constipation. And my GI guy did a biopsy, suction rectal. And said he didn't see any ganglion cells, but it wasn't quite an adequate specimen. So I took him to the OR and did a biopsy at about 3 centimeters above the dentate line, and lo and behold was told, you have Hirschsprung's. Um, there's no ganglion cells, hypertrophied nerves, and alretin negative. And so I thought, well, that's interesting. Um, I brought him back, did a full bowel prep, and then proceeded to do both repeat transanal biopsies and laparoscopic biopsies. And the next level that I did transanally was at about 5 centimeters, and then kind of marched up laparoscopically to the descending colon. Interestingly enough, the, uh, sigmoid colon and descending colon appeared normal. There was no hypertrophied, um, Uh, muscle and the caliber was normal size. So today I got all my biopsy results back and the 5 centimeter above the dentate line showed normal ganglion cells, non-hypertrophied nerve bundles, and Cretin positive. And I was interested. I'm working with, um, Doctor Reddick, who trained down at Children's in Chicago. And he suggested doing a posterior, um, myotomy strip, but since I have had the opportunity of being on this conference, I was wondering if, uh, my esteemed colleagues could give me some suggestions. Ultra short segment Hirschberg's disease. Yes, this is my first. Yeah, yeah. What are your thoughts? My personal experience with the myectomies has not been, uh, I think it's an extremely difficult technical operation and perhaps it's because I haven't done it right, but, uh, first of all, it's very uncommon to have to do it, and I have not had great success with that. I would ask my other colleagues, Jason, Jason. Um, my, my take on this patient would be, uh, one, I would redo your distal biopsy, your 3 centimeter biopsy, and that's correct. Uh, two, from learning from, uh, Doctor Penny and Doctor Levitt, they do not quote unquote, believe in the ultra short segment Hirschprung's disease. So none of the patients that we've seen here have received. Uh, or undergone myectomies that, uh, that they've seen in the past or we've seen in the last six years I've been here. Um, so that's. Uh, and I was just thinking about the question. I was wondering if you do a Botox injection before that, seeing if you relax your sphincter, what the results would be and, and how that would work as sort of an artificial way of doing your myectomy before actually going through with the definitive procedure. Well, maybe a preliminary study would anybody do myself. I'm glad you, you mentioned that. Would, would anybody do manometry on this patient, 17 year old? I, I probably would have started with manometry because, uh, for older kids, uh, if they have a normal recto anal inhibitory reflux, then you don't need to biopsy them. They don't have Hirstprung's don't right. So, that's my screening test for Hirschsprung's in, in kids who are older. You'll trust it, you'll trust it that much. If it's, if, if there's a normal reflex. Uh-huh. Now, if they don't have a normal reflex, then, then you have to biopsy them because the, the, there can be false, false, whatever you want, I don't know, negative, positive, false absence of the of the reflex, um, but for the, the problem I, I, yeah, the child is not functional enough to undergo that. I mean, he is a 16 year old strong 2-year-old men mentally kid. Yeah, so if you can't do it, then that's why I don't do it in the really young kids. So what would you do? So, so in this case, so I just want to make a comment about the term ultra short segment because it bears directly on the, on the discussion. There are, there are two definitions for ultra short segment Hirschmann's disease that people use, and these kinds of discussions get get foiled because one person's using one definition, another person's using another one. Some people call absence of the recto anal inhibitory reflex with normal ganglion cells on the biopsy, they call that ultra short segment Hirschmann's disease, and that to my mind is internal sphincter alagia and not. Ultra short segment Hirschman's ultra short segment Hirschman's, I believe, is a very short segment of a gangliosis where there should be ganglion cells, and it's hard for me to imagine how Doctor Pena could not believe in that. You know, why would there be a disease where there's a minimum of 5 centimeters of a ganglionosis? Why, you know, it's just he's, he's the big spectrum guy, right? There's a spectrum of disease, right? So, so I think what they're talking about when they say they don't believe in it is that they don't believe in ultra short segment Hirschprung's being internal sphincter alagia because it's, that's not Hirschprung's disease. That's it's a different thing. So that being said. Uh, Jason says go ahead and do the trans anal biopsy again. You would say, well, I, I don't disagree with that because sometimes pathology is wrong, but if you repeat the 3 centimeter biopsy, and again it's a ganglionic with no cal retinin, then I would not do a myectomy on this patient. I would do a pull through. Uh-huh. And that would be my response. Because I, I, I've never seen really good results from in a 16 year old, do you, would you divert or would you do a primary pull through with a, without diversion? It depends on the, on the status of the bowel, but in most of the 16. Year olds with newly diagnosed Hirschrun's that I've seen, I've deffunctioned them because the bowel is so huge. Yeah. And I would do it as a laparoscopic dujamel because I, I find that, that rectum gets so thickened trying to do it transanally, uh, you get into the wrong plane, you end up stretching the sphincters way more than you want to. So, I've, I've used a laparoscopic duo. However, I, I, I wonder, because this is effectively an adult sized patient, whether the biopsies were actually of the anal canal, cause the anal canal in an adult is 3 to 4 centimeters long. So you don't really have that problem in an infant because the anal canal is 1 centimeter long, but in adult, it's 4, maybe even 5 centimeters long. But if I recall correctly, there were hypertrophic nerves present on the biopsy, which you should not have. Because, uh, you know, the biopsy of the canal, there are no ganglion cells. Right. But there's a normal dropout of ganglion cells, but it, but you should not have hypertrophic nerves. But you should also not see normal rectum mucosa. You should see Transitional epithelium. If it's really in the end. Right. And there was no transitional epithelium on that, uh, findings on barium enema as well. So, they have, they, they aren't dilated all the way down to the, uh, pelvic floor. Great. All right, uh, Christine, did that answer your question somewhat? Yeah, it did. I mean, it did give me all the considerations, and I, and, um, so, uh, I was considering a Duhamel over doing a primary slave because the, just being in that rectum was a nightmare just doing the biopsies. It was very thick. Um, would you consider doing the Botox first to see if that relieves some of the issues and then, um, The problem is he's so low functioning that doing a diversion on him is going to be a nightmare as well, just managing that. Um, so it, it's not just the technical procedure, it's the patient that it's being done. I think if you're committed to doing a pull through, then doing a Botox injection probably doesn't serve a whole lot of purpose. Um, uh, so, I, I think if, uh, you either have to divert the patient or not based on whether the, on the dilation, you know, the status of the bowel and then do the pull through. So, I'm going to go ahead, um, you know, Christina, I just tell you that, um, one of the new reasons we're, Making a new platform in November is that we want to be able to continue these conversations even after this live event, so that will be available starting in November. So I apologize, but what I would ask is for you to continue chatting with about this, and we have experts here who can talk to you about the case even if we're not discussing it live on the air. But thank you for calling in. No, thank you for taking my call. I appreciate it. All right, take care. I don't think we ever addressed the issues on the slide here, which is with the routine newborn, what are people's approach gonna be. And did we get the results of the poll from internationally? I didn't see it. Oh, I don't know about international, but this one we got, um, there was an overwhelming, yeah, it was la. OK, so we discussed this. We did. All right, so we're fine. So, um, for the second case, um, it's actually very interesting she left, but Danielle was describing exactly this case. Um, this is case one of my partners, 37 week term infant, uh, presented day 3 of life with abdominal distention. He'd previously passed a small amount of meconium and had this abdominal film, which is difficult to see on the screen, but this clearly showed free air. So the child was taken to the operating room where he underwent exploration, was found to have a fecal perforation, minimal dilation of the small bowel, and no obvious transition zone. So, what, uh, the question is, how do you then manage that patient? Closure, just close the cecum, do an ileostomy 5 centimeters proximal, do biopsies and do a leveling ileostomy or colostomy, or do a primary pull through. Well, I mean, I, I would, I would close the perforation. Most of these cecal perforations from Hirschberg's disease are not total colonic disease. Most of them are shorter segment disease. It's like with a rectal cancer. The cecum gets more distended and pops. So, uh, I would close the cecal perforation, bring out a loop ileostomy, and if the baby was stable enough, I would do some serial biopsies in the colon so that I know where the transition zone is for the next step, which is going to be to do a pull through later on. Would anybody do anything different than that? Well, the question that we, I'm sort of like, I wanna make sure that Jose catches his flight, so we do have to get him on the air, um, but, um, the, you know, the question is the leveling leveling ileostomy. You said you do a loop ileostomy. Do you, do you send off biopsies before doing your loop ileostomy? Um, I, I don't think this is very likely to be total colonic disease. There's no reason why they should perforate their cecum with a transition zone in the ileum, so I probably would not. I would not do a frozen section on the ileum, OK, because it looks like 75% wood, um, in the audience. OK, let's keep going. So in the interest of Jose's flight, we're going to move along here. Um, uh, so, uh, this was the contrast study on the child that was obtained postoperatively and shows, uh, essentially normal caliber colon, uh, without a transition zone. Biopsy showed that there was a transition zone in the ascending colon just above the uh area of the perforation, just distal to the perforation, um. So in terms of timing, how long would you wait to do the operation? Would you do it at the initial hospitalization, send the child home with their diverting ostomy and bring them back? Anybody have any strong feelings? Well, this is long segment disease, so I tend to wait longer in long segment disease because they have terrible perianal excoriation if you, if you pull them through too early. So, I like to wait till the stoma output is thickened up and, uh, and that can be 6 to 12 months sometimes. OK. Other from the peanut gallery, any, uh, a lot of a lot of Agreement. Head shaking, yes. All right. Well, we won't, we won't beat on this since we're moving along. By the way, the, the virtual audience is all over the place. OK. All right. And, uh, uh, then what procedure would you do? And, uh, this is ascending colon, um, for a transition. Uh, do a, Duomel, a swab, a Swenson, a permanent ileostomy or a J pouch of some sort. So, have you done the ostomy at the side, and. It's an ileostomy. It's an ileostomy. Yeah. Well, I'll jump in again. I, I'm not shy. I love to talk. Um, I, my, my experience with, um, with a very, very short pull, pull through like using the cecum essentially have not been very good. So in general, when I'm faced with that anatomy, I just do a, treat it like total colon disease and I do an ileal duhamel. So you would sacrifice that small amount of colon. I would. Interesting. I've done that before. Um, I don't, again, I, I don't know how, I don't remember how I thought it through, but, uh, I just felt that that pulling that cecum down would just be too difficult, and, uh, I guess from a technical standpoint, I thought it'd be easier, so I did an ileo DuML. So can I ask Jack, does it make a difference whether it's in the ascending colon or the hepatic flexure, uh, if you throw that. Colon away? No, it, it does make a difference to me. If it's hepatic flexure, then I, then I would bring the colon down. I still would do it as a duhamel, I think. But, uh, but I would preserve the colon. But when it's just the cecum, it's, it's this big bag of stuff, and, and they end up with a lot of stasis and enterocolitis. I've just been unhappy with it the, the few times I did it, and then, then I switched. I think my tendency, having seen, I don't think anybody's seen a whole lot of these, but is to try to preserve the bit of colon that you have as long as it's enough to do a reasonable amount and you're not just pulling the cecum down because I agree it just gives you a big patchless reservoir that collects stool. I chime in. I agree also in trying to keep the uh colon, if we can keep a segment of colon to just um help with the forming of stool. But I guess the technical question of keeping the cecum is, do you rotate it clockwise or counterclockwise or quote unquote doing a de-rotation to bring it down in the technical aspects of that? And does anyone have any inside or comments on which way they prefer to turn their right colons? If you bring them down Jack, for, for if I'm bringing the whole, it's a right hepatic flexure and I'm bringing the right colon down, I, I, I flip it over so I don't try and turn it, I just flip it. So the front of it becomes the, yeah, and, and it's hard because the blood supply is, uh, it is you have to, yeah, you have to try and preserve the marginal artery and there may be some anatomic variations that make you do one over the other, but whatever, whichever, I think whichever way it lays properly is the way it goes. Jason, were you gonna comment? I, I just, I agree. I just think you have to pay attention to that ileocholic blood supply and make sure you're not, you're, you're probably either way you turn it, you're going to put a little tension or kink on it, but you just have to be careful, that's all. OK, uh, so, uh, there was the pull through, um, which was laparoscopic combined with the transanal approach, so there's a lot of colon. So question 4 was if there was no perforation but presented with abdominal distention and the suction rectal biopsy shows Hirschberg's disease with an enema that's suspicious for a long segment, how would that change your approach? I mean, obviously this child that was presented presented with acute abdomen and required immediate surgery. If you suspect long segment Hirschprung's, how might that change your approach? Or do you, I would still do the same thing and I, because I start all of these laparoscopically and do biopsies to, uh, so determine a level, but I would counsel the family differently, obviously, that would probably be the biggest difference. So I guess the question that really should be posed to Erin or people that do prone, would you still do that in this patient? No, not, not if you have suspicion that it's long segment. OK. So, management wouldn't be much different, but I would leave a mucous fistula too for irrigations and decompression if you can. For the, so you would do an initial ostomy, decompress with a divided ostomy mucus operate at a year or even older. OK. Any other. No, that's worked well for me. A lot of these, I think you can't irrigate clean, so I've done stomas, limited stoma with a laparoscope, and then, Uh, do a fan and steel kind of pull through and, and just the only scar I have on the abdomen then is, uh, uh, where the stoma was at a later time. And if the stoma's in the umbilicus, then you don't see it. That's perfect. Yeah, there you go. Um and I do a Swenson pull through, I just have to say that. Nobody else does them, so. Yeah. Rafensberg. I like to do what one of my partners in Chapel Hill calls a swabsson. Yeah. It's kind of some of both. Yeah. Um, so the next case we've already discussed a little bit, a 3 year old with issues of constipation switching from breast milk to formula, has been on multiple bowel regimens without improvement. Recent admissions for impactions requiring cleanout. So basically the older child with a with a question of to rule out Hirschprung. So what do people use for their standard workup for this? Is your question, what do you do first? What would you do first? How when this kid shows up in your clinic, because these are outpatients who show up in your clinic and What's the approach? Is it, uh, uh, manometry as, as you alluded to? And I don't know in a three year old where, where that falls in your, uh, your algorithm. It's probably a little young. Our, our people won't really do manometry till about age 5 or 6. So, so, I would say start with a contrast study, I would not do a suction rectal biopsy in a three year old, and I would do an open rectal biopsy. So, you would take him to the OR for an exam under anesthesia and a rectal biopsy. I would start with a contrast study and then do it. Yeah, after the, and then under general anesthesia. Yes. And in fact. Regardless of the results of the contrast. Yes. Yeah. And, uh, in fact, um, I'm curious what age people would stop doing suction rectal biopsies. Uh, this is a three year old. What about a two year old? What about a 15 month old? Uh, so, Remember you gotta, even though you might, the, the specimen may come out, the logistics of taking a 2 year old and, uh, holding them they can fight back. They're too old. All right, to keep moving along. It's an abdominal film. Um. So, uhtipation. Yeah. So, if the biopsies, uh, are positive, uh, then, uh, what would the approach be? Would you divert that child? Would you do a primary pull through? Um, would you do any type of colectomy during the pull through or just, uh, do a pull through? I don't think I would do a primary pull through, but you would do I don't know. I don't think I would, is it, how old is that patient? 3, yeah, so a 3 year old with that, that degree of dilation and chronicity, I think I'd probably, as much as I hate stomas in general, um, probably would, would do a leveling colostomy and probably resect that dilated segment. It's gonna have to go eventually and it's gonna have to go. But. So, what was that? Wasn't that one of the choices there, colectomy? Yeah. Yeah. So, I'll just see. What, anything different you would do? No, I think it all depends on the size of the bowel. If you, if you think it needs to be, uh, reduced in size, then you do a stone and let it collapse down. Yeah, I think you try and irrigate them. I mean, my approach would be to divert them, try to irrigate them out and see if you could get that to collapse down. And if it doesn't, then. OK, so I agree, but that thing that you showed, um, is so mammothly dilated, I don't think that would close. That's why I was initially going to say, Divert, but I think that that's not going to come down no matter how long you wait. So, you do your swab A Swensen there? Is that what you do as a pull through. Yes, swabs, the swabs. This patient's on the schedule for Monday, and our, our plan is to, um, do a primary pull through with the potential for doing a diverting, uh, stoma if we're uncomfortable with our anastomosis, which we've, uh, on these older children, and I think the next few slides is a 16 year old with the same situation. We've been really leaning towards doing diversion after just to protect our anastomosis. So Jason, when you do a primary pull though, you're just going to pull out all of that dilated stuff too, or are you going to stop at the transitions where it's where it's, you have normal? We'll pull out all that. I mean, it looks like from the contrast study, you know, the descending colon looks to be normal caliber. So the plan was to do this almost entirely laparoscopically. Um, we definitely found that if we do this, take the, try to resect these colons transanally. The stretch that we put on the sphincter is enormous, and their, uh, continence postoperatively is definitely, definitely suffers. So we go all the way down with our laparoscopic dissection, um, and then do very little of this resection transanally, do our, do our anastomosis, which will probably be somewhere in the descending colon, so we'd have to mobilize the splenic flexure. And then probably do a diverting ileostomy. That's the game plan anyway. We'll let you know on Monday. Yeah, I, I agree with all of that, except that I have had success in, uh, getting that distal bowel to, uh, shrink in size. Um-hum. With the stoma for 6 or 8 months. So, I would do, I would do that. I'd do the stoma first and then I think the pull through is a lot easier. If the, if the bowel has shrunk down in size. And, uh, the older the kid, the less likely that it's going to shrink down in size. But, uh, at this age OK. Uh, I think it will. Do you do anything to promote, do you wash them out from below to try to get, keep that empty or I wash them out at the time of the operation, but then, uh, after that, I don't. Cause it's C function, so there's not that much that's going to collect in. OK. OK, so the next, uh, case is actually, uh, very similar to the one that we discussed with the caller, so I think we'll just skip that in the interest of time. But, uh, you know, this is the kind of problem where you have what you, what you were all describing with this big dilated segment of bowel, and at age 17, that's unlikely to ever get better. The only thing I want to comment on that case is that patient was at a well-known institution in Ohio. And had, was seen by GI and had multiple biopsies that were normal. Those were done by colonoscopy, um, and then, so the chart read no Hirschprung's disease, uh, throughout the chart, um, and then came to see us and I did a rectal biopsy, and lo and behold, no ganglion cells and hypertrophic nerves and our usual findings. So, uh, just caution to watch out what type of biopsy was performed and how Hirschprung's was ruled out. It's, it's an argument for the manometry uh diagnosis because biopsies in an older child can be unreliable. OK, let's move on. Um, 6 month old whose status post primary pull through at birth presents with abdominal distention, fever, diarrhea, temperature of 38.5. The abdomen is distended and tympanitic, moderately tender. It has a white count of 16.5. Abdominal film does not have free air but has some distended loops of bowel. This is getting to the patient that you were describing earlier. So what would people's management The flagyl, saline enema, rectal dilation, discharge, intravenous flat metronidazole, IV fluids, saline irrigation, saline irrigations and broad spectrum antibiotics as well as oral and broad spectrum antibiotics and diverting ileostomy. Uh, 6 months after a transam, about 6 or 7. 6 or 7 months old, right? What's that? Yeah, 6 months old, I'll write that. It's pretty sick, so I think I would do IV, more, more, more broad spectrum antibiotics and, uh, and irrigation. I mean, obviously if the kid's got peritonitis or is otherwise septic or, uh, SERS, then you'd, you'd have to. Uh, consider it a diversion, but, uh, it sounds like based on the presentation that you have that you'd probably get by with irrigations, I think, and antibiotics. Would anybody do anything different? Pretty straightforward. The audience agrees. Yeah. Um, The child has trisomy 21 and 3 episodes of medically treated Hirschberg's associated with enterocolitis in the 1st 18 months of life, so your management would include. Anal dilations, Botox, chronic treatment, your, uh, prophylactic treatment with oral metronidazole, revision of the pull through permanent ileostomy. The incidence of enterocolitis is significantly higher in children with trisomy 21, as much as double the incidence in genetically normal kids. So I'd just be interested in how people would approach recurrent episodes in this highly susceptible population. Now you didn't mention rebiopsy. Yeah, I mean you could make this list about 100 things wrong, and I think you're right, that's. Probably the first thing to do is make sure they don't have, uh, persistent, uh, a ganglionosis. So based on this and then the last slide, anal dilations, the majority of the audience did not do those, but I think it depends on the rectal exam. If you do a rectal after your anastomosis and it's so tight, then they'll need that plus all the treatment for the. Yeah, I mean, you have to rule out distal obstruction depending on what your pull through was. And then you, and you have to make sure that there are normal gangban cells. But uh and then you go on to the to the treatment of the actual enterocolitis. So I think our typical management would be an exam under anesthesia with biopsies and and to rule out exactly what you said, additional obstruction and to prove that there's not a transition zone pulled through or that it's a ganglion. We tend to use oral Flagyl very liberally in these kids and we keep them on it for a long time. I, I think that was part of what the discussion I wanted to engender is what's the utility and, and what are people's thresholds for using that and using it more than just as an acute therapy. Yeah, we have, we have a lot of kids on chronic, uh, metronidazole. And when they, you know, they can be on it for three months and then you try taking them off and they start getting symptoms again. So, some of them need it for a long, long time. Uh, I'm more, more of a fan of Botox in the, at least in the younger kids. So, I don't know what's your experience. Well, we use Botox as well, as, as you know, and, um, we actually published a, uh, that Botox decreased the number of hospitalizations for enterocolitis in, in those children. It doesn't always work, though. Uh, but. Yeah. Sometimes it works and sometimes it doesn't. Yeah. But if it works, it's a great way of doing it. So, I've just been informed that both of you, your car is coming in an hour to pick you both up. So, we're going to, like I said, I'm gonna start moving along. Yeah, let's, let's, uh, should we wrap? Yeah, let's wrap. OK, um, and, uh, and then what we're gonna do is take a like a 2 minute break and we're gonna bring Jose and, uh, Tim up and then we'll keep going with necrotizing enterocolitis and GERD and then we'll finish up the program. So thank you. Sorry for cutting off your last slide, I think so no problem. All right, thanks for listening so much. So who