Dr. Alberto Peña presents the surgical procedures for Hirschsprung Disease. Dr. Andrea Bischoff discusses the surgical approach to total colonic aganglionosis, common complications, irrigation, urinary sodium check, ileostomy retraction, enterocolitis, laparoscopic approach, transanal dissection, and suction rectal biopsy. Discussion is generated by poll questions and case presentations.
Type: video · 128 min · posted 2018-11-13 Canonical: https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736 ## Chapters - [0:00](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=0) Initial approach selection and audience polling - [10:00](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=600) Transanal technique and sphincter preservation - [25:00](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=1500) Historical context and endorectal pull-through - [40:00](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=2400) Indications and contraindications for transanal approach - [60:00](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=3600) Total colonic aganglionosis management - [78:20](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=4700) Laparoscopy role and case discussions - [96:40](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=5800) Case scenarios: diagnostic workup - [110:00](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=6600) Complex cases and rare associations ## Statements - "Between 75 and 80% of Hirschsprung cases can be reached transanally; in the remaining 20%, abdominal approach is added if biopsies remain aganglionotic" — Pena (clinical) [3:42](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=222) - "Concordance between pathology and radiology is 75% in rectosigmoid Hirschsprung patients, meaning 25% discordance" (clinical) [5:30](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=330) - "Concordance is only 25% for long-segment disease" (clinical) [5:39](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=339) - "Leaving 1-2 centimeters of aganglionotic bowel does not explain poor outcomes in most patients" — Pena (opinion) [18:30](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=1110) - "Majority of patients with retention symptoms after pull-through do not have residual aganglionotic bowel" — Pena (clinical) [21:41](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=1301) - "Removing the rectum affects bowel control mechanisms even with perfect technique because the natural reservoir is removed" — Pena (clinical) [22:39](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=1359) - "Fecal incontinence is much more common than believed and not discussed enough in pediatric surgical meetings" — Pena (opinion) [23:44](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=1424) - "The main problems in Hirschsprung disease are related to inexperience and technical incapacity of the surgeon, not the approach used" — Pena (opinion) [11:10](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=670) - "Patients complain about fecal incontinence, not scar size" — Pena (opinion) [12:17](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=737) - "Stretching the anus too much during transanal dissection damages the sphincter mechanism and causes fecal incontinence" — Pena (clinical) [15:06](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=906) - "Rectal irrigations are mandatory before any surgical procedure for Hirschsprung disease" — Luis de la Torre (guideline) [34:05](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=2045) - "Long-segment Hirschsprung patients do not improve with irrigation, unlike rectosigmoid cases" — Luis de la Torre (clinical) [60:03](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=3603) - "More than 80% of Hirschsprung patients have rectosigmoid aganglionosis" — Luis de la Torre (epidemiological) [60:31](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=3631) - "Patients with chronic dilation and massive megacolon are not good candidates for primary transanal pull-through" — Luis de la Torre (clinical) [62:01](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=3721) - "The anal canal consists of three zones: anoderm (squamous epithelium), pectinate line area, and columnar zone" — Luis de la Torre (clinical) [64:05](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=3845) - "Anastomosis below the columnar zone results in fecal incontinence" — Luis de la Torre (clinical) [66:20](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=3980) - "Total colonic aganglionosis ileostomy should only be closed when child is toilet-trained for urine and accepts rectal irrigations" — Andrea (guideline) [72:01](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=4321) - "Closing ileostomy early in total colonic aganglionosis leads to severe unmanageable diaper rash" — Andrea (clinical) [76:14](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=4574) - "Patients with total colonic aganglionosis have high incidence of enterocolitis and will likely need rectal irrigations" — Pena (clinical) [80:29](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=4829) - "There are two types of Hirschsprung disease: benign type without enterocolitis that can go years with huge megacolon, and bad type with newborn manifestations and enterocolitis tendency" — Pena (clinical) [120:20](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=7220) - "PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease" — Andrea (clinical) [121:58](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=7318) - "Anorectal malformation combined with Hirschsprung disease results in guaranteed fecal incontinence because patient has no anal canal and loses natural reservoir" — Andrea (clinical) [126:31](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=7591) - "Post-evacuation films are not reliable for diagnosing Hirschsprung disease because aganglionotic segments can be spastic and expel contrast" — Krauss (clinical) [128:06](https://library.globalcastmd.com/watch/surgical-procedures-for-hirschsprung-disease-736?t=7686) ## Transcript And we are going to discuss the surgical procedures designed to treat disease. Um, I was trained in doing swabve procedures and some dujamel and then, uh, but lately we changed in the last few years to the transanal resection, uh, described by Doctor de la Torre and, and, and, uh, Doctor, um. And take up longer and um because we consider that a great idea and um from the moment I saw that procedure I like it, but I predicted that it has the potential to do damage if you don't do it correctly and what I just want to everybody knows about these techniques we don't have to repeat the basic principles, but I want to mention a few uh important details to avoid. Problems and constipation and complications. The patient who demonstrated the fishbone disease is going to be subjected to a resection and put through. The steps to follow are 1, laparoscopic biopsies followed by resection and put through. 2, transanal dissection with sequential biopsies until finding normal gangbryonic bowel. Number 3, mapping of the of the colon with several biopsies. Laparoscopic or open, followed by put through at a later later time. So everybody can vote, and I think that's the one we've been curious to know what's your answer. Just 1 second and we will have the pulse. So option 1, laparoscopic biopsies. Option 2, trans anal section, and option 3, mapping of the colon. So you can vote 12, or 3. And we have click enter. One It keeps changing, but it says 27% option 1, 52% option two, so the majority saying option two, and 24% option three. Well, let me, let me comment about this. The, the, the surgeons who who like to do laparoscopic biopsies followed by resection and put through is because they argue that they don't want to be surprised in the middle of a transanal to find. That the patient has a long segment or a chronic gangliosis and therefore they want to be sure that that they can reach everything from below. The, um, the number 3, those who propose a mapping of the colon with several biopsies, and laparoscopically open, they want to be absolutely sure of the extent of the uh angliosis and that. Number 3 alternative, I find it reasonable in a place where you don't have a pathologist. In other words, and we used to do something like that in the old time in Mexico. If you feel that you that you don't have a pathologist all the time capable of making an accurate histologic diagnosis, it makes sense to do that and take your time to go back sometime later when you are convinced. Uh, you have the alternative to send the specimen to all the pathologists all around, and once you are sure, you know exactly what you are going to receive. I personally prefer the option number 2, which is the trans anal direction with sequential biopsies, to finding, finding normal ganglion bowel, because between 75 and 80% of the time I reach the normal ganglionic bowel, and in the other 20%, I simply just, if I keep taking biopsies and pulling the bowel down and it's still a ganglionic or I'm not sure, I simply go into the abdomen and continue the resection. So I don't, I don't find it unreasonable to do #1, #2, considering specific circumstances. And, uh, but I personally like to do a #2 option. Anybody else would like to comment? I think this opens up the big question What happens if? Right. And so what happens when You start from the bottom and you, you have a contrast enema that looks like it's rectosigmoid and you're confident you're going to be able to reach from the bottom. It's a girl and you get up and you can't get any further transanally and you convert to your open procedure and you've taken biopsies around the entire colon and you still. A gangliosis. That's the question. That's the million dollar question, right? I think, I think if it's only total colonic ganglionosis, that's not a problem because you do an ileoanal and a protective ileostomy. The problem, the true problem is when it's beyond right, and you're not, but this is extremely, extremely rare. So I think. Uh, doing laparotomy or laparoscopy for everyone to preserve this probably 2% cases that will happen, I think it's overkilling for many patients, except, except if I take, sorry, no, go, um, but Dr. Krauss presented interesting information. He said the concordance between pathology and radiology is 75% in the rectosigmoid patients, so that's 25%. They're all At that point, and it's only 25% in concordance for the lung segment, so I go from the bottom when I think it's a rectosigmoid procedure, but there is a risk, and then I'm just curious how other people, I think it depends on how comfortable you are with the trans. It does get higher, it gets more difficult, and I think if you look at your contrast study and it's in between. There's always that area of that transition. who knows how long that transition is, and that's, I think, where people will get in trouble and you're pulling down transition zones. I, I think most of the, most of the people I speak to start with laparoscopy. I think that's the most common. I don't know what our pulse eventually we can get out. We actually had it more transanal. It was, I think the consequence of doing a transanal and not reading the normal um learning vowels. The consequence of that is that you open the album, which is exactly what you do in the other way. So I don't see, I don't consider that a complication. I don't consider that a, a problem. You see they go and do what, what you was, what you were going to do in the first place. If you go laparoscopic, take biopsies, do you biopsy the entire colon? Or what is a new biopsy laparoscope? So laparoscopically, so you, you almost have to start laparoscopically if you're gonna do laparoscopy, because once you break the peritoneum transangle, you can't really scope very well. So if you're gonna do a laparoscopy, if you think you're gonna be in the belly, that's where I would start, and you could actually look, I mean you can tell when you look at the bowel, sometimes even open what looks normal, what doesn't look normal, and you can see that laparoscopically as well. So I'll look and see where it looks normal, take a biopsy. You there if it comes back as normal, I immobilize my entire splenic flexure, the left colon, and then my transanal becomes like 2 centimeters. It's very quick once you get to the bottom. Laparoscopically, great. That's great. If you're going to do laparoscopically like Jason said, if I know it's back to the point, I'm not going to go in the belly if I don't have to. But it's great. Exactly. So if you go, if you start transanal and you find that you cannot mobilize more, you just go laparoscopically, mobilize the colon and. what's the difference. The issue with that is, you have the order, right, it's the order, but it's difficult sometimes. We've found that if we start transanally, break through the peritoneum, and then go laparoscopically, it's hard to maintain the pneumo peritoneum sometimes. We sometimes, and I don't know if you have any tricks about it, no, because I wouldn't be down. That's why I have a trick for you. I mean, I, this is so scared me to be disagreeing with, with you guys, but I, I mean, I. It's what I feel comfortable with, and then I look at what's the downside of laparoscopy, um, the dissection is so easy, uh, you really give yourself a head start when you're doing transanal. I get it, I feel comfortable knowing my biopsies. I hear what you're saying. I see both sides. I think when you get into this situation where there's two ways to go about it, um, my question would be for those who say her transanal, what is the downside of laparoscopy? What is it that you don't like about it? Is it that it's, are you considering it more invasive, because I don't think so. I think it's, I think the big thing scopy is being less invasive. So, and you want also to be a nicer abdomen without scars. So if I do a transanal, there's absolutely no scar, and the patients do extremely well postoperative in terms of pain. It's minimal pain. And, and then, and then it's dangerous to do what I'm doing, which is, well, first of all, I just how I do it, but I, but I had been burned. It wasn't me personally, but I saw when I was in training a patient that had total bowel Hirschberg's disease, and they did a transanal, and it was at that point I said I will never, ever, ever do that total bowel. There's nothing to do. You need, yeah, you, right? That's a good point. So because going transanally, it's all about. No, this was transanal, but they're they did go transanal bows gone anywhere. I mean, there's not much, but I, I just, and I hear what you're saying that you could do a straight pull through right there at the time of the operation. Uh, is that what you would do, by the way? Would you do a straight pull through right then, or would you do a stoma? No, no, I would do the put through, and then I decided whether to look open. We'll talk about, we'll talk about, you know, OK. I, I don't think we're gonna, I mean, again, I, I think you were talking about laparotomy versus pure trans anal. But I just feel that it is these 23 millimeter incisions and, and I just feel that it is so minimally invasive as it is and it's such a gorgeous dissection, um, and so easy and so nice la laparoscopically that I think it's a nice approach, but I, I'm here to be convinced. This is why I'm here. So I think it's what you're comfortable with. I mean, you got to do the best operation that you're comfortable with. Andrea, Dr. Pena, myself, Jason, we do a lot of transanals. We're comfortable going high past beyond perineal reflection. If you're not comfortable doing that way, you'll probably cause more harm than if you put the scope in and mobilized everything that way. So we receive this kind of question frequently calls from friends or from patients from other parts of the world. And they say my son or my daughter has Hibone disease and a surgeon is offering me this technique and another surgeon is offering me the laparoscopic and the other the robotic and the rather and and I don't know what to do and if it was my grandson. My, my answer would not be for laparoscopy or non laparoscopy or robotic or non-robotic or open and open because the main problems that we have seen in history from disease are not related with the approach that you got are related with the inexperience and technical incapacity of the surgeon that damaged the baby on a permanent basis both ways laparoscopically or transenally, a bad surgeon will damage the patient. Those are the real problems that we see. So I would go. Whoever has experience and let him do whatever he is accustomed to do well following the basic goal principles of the treatment of his disease, which is I'm going to talk about that not to damage this mechanism, sphincter mechanism that is has been damaged as we are talking now by laparoscopies and non laparoscopies. It's not it's not. It's not because of the laparoscopy or no laparos. It's because of a bad technique that you can be a bad surgeon both ways. So I will go with a surgeon that is well trained and then, and, and I never heard people complaining about the scar or not the scar. I see patients complaining about fecal incontinence remaining that's the real problem, not the size of the scar. So that's what my specific recommendation. There's a lot. There's so many comments in the audience. I don't know if you want to hit those now or we can get to them later, but. Uh, Cynthia Reyes says it was a misleading question because we didn't say the length of the suspected Hirschprungs when we give it that's, I guess your point is it doesn't matter, right? And I love Belinda's point is that, um, I never thought about it. I've always done these laparoscopically. I always have an easy transanal dissection because I've done most of it already. What happens if I get, you know, if I say, OK, I'm switching right now, I probably won't be as good as I was laparoscopically because now your transanal is only like this, whereas our trans anal, we can go like this, so for sure it's, it's very different. Interesting. If I have any question about, and it's putting the whole picture together, it's the biopsy results, the contrast study that you have, the how the patient's behaving, how you're able to irrigate the patient and clean them out and keep them healthy until you're ready to take them to the operating room. If any of those don't line up, I'm more apt to. Enter the abdomen first, laparoscopic, my technique, um, but I think it's like Dr. Pena said, you do what you're comfortable with, but do it right. Right, um, I could, anyways, we could go through and answer some of these, or we could just type them in. So, so the, the, I want to emphasize a few points about the transanal resection. I'm sure Doctor de la Torre. Will illustrate us and will illuminate us with his tricks, but we use, as many of you I'm sure are using the so-called Lone Star retractor retractor, which has the characteristic to immobilize the field every time you are going to do a meticulous, delicate operation, the field should be completely. Immobilized so nobody is pulling in one direction or the other and you can see in this slide the pectinate line here, the pectinate line. So we call this anal canal. So you put these hooks of the retractor at first like this and then we move these hooks into the pectinate line itself. This hook is moved here. This one is moved here. This one is moved here, and by doing that we now have a completely exposed rectal mucosa. In other words, now you are looking at the, you don't see the anal canal because the entire anal canal is folded circumfidentially and protected by definition. At this particular point, I want to emphasize that the surgeons should be careful not to stretch too much because you start the dissection and you forget you want to be comfortable. You put retractors and I walk into the operating room and see sometimes a big hole that what used to be the anus becomes a huge hole and that is damaging by definition the sphincter mechanism. Then the patient may have bowel fecal incontinence. So, so you can see here now that it's only only rectal mucosa and then from here I personally start 2 centimeters from this point deep inside the dissection of the rectum, and every time we do want to do a meticulous dissection we've used multiple silk stitches in order to be able to distribute the tension to avoid damage to the tissues that you are dissecting. Uh, originally, Doctor Luis de la Torres, uh, um, started this operation and still doing it, I think, submucostally and rectally. I personally like to do full thickness like Doctor Swenson used to do, so, um. And here you see now we are advancing the procedure. We don't have time to go over all this, but as we keep pulling the bowel that we are dissecting, we keep taking, we keep taking. We keep taking biopsies every, I personally do it every 5 centimeters until I find normal ganglionic and even go 5 centimeters more higher and at that point we like to do two layer anastomosis, two layers anastomosis. The first layer, as you can see here, these stitches are taking the serum muscular of the bowel and the tissue above the area where I divided the rectum. See, see, is the is the what I call the external layer. And uh we go by the north, south, east, north and west and then resect the entire rectum and make the inner layer which will be mucosa mucosa and the, the key for the operation is number one, to respect the pectinate line through the anal canal, not to stretch too much the, the, the anus, and then mobilize the rectum to be sure that you have a a bowel. With normal ganglionic bowel with good blood supply and anastomosis with no tension, those are the keys if you don't want to have strictures, retractions, so forth. When you are, when we are dissecting this food thickness, keep in mind, and we do it by the way, in prone position. Like in a posterior site that we put the patient prone position, much more comfortable to us than the lithotomy position. Lithotomy position, we are, you try to stay away from that because the surgeon is the only one who is seeing. The others have difficulty seeing. The field is vertical, so you lose instruments. The scrub nurse is difficult to see what you are doing. So there, that's a, that comes from the old times, from the adult surgery, but we don't do that. We prefer the patients in prone position, much more comfortable to do these operations. Doctor Pena, can I ask you a question? Dr. Kapo asked a question about with this technique, are you leaving 2 centimeters of a ganglionic bowel, um, in your dissection? Then a follow up to that. Is your positioning of your sutures and where you start your dissection change if you're operating on a newborn versus a 2 year old? Um, it's not, it's not a big difference between the newborn and the, I don't see the difference between, first of all. Answering Dr. Kapoor. I don't blame on the 1 centimeter or 1.5 centimeters or 2 centimeters of ganglionnosis to explain why a patient doesn't behave well. If you look into your own experience, clinical experience in Houston disease, you'll find patients that all of them you operated with exactly the same technique. And some of them are doing beautiful like normal individuals, and others have symptoms of enterocolitis, and we don't know why. In other words, we have been obsessed with the idea of leaving 1 centimeter or two a ganglionnosis, and that explains symptoms. That simply is not true from my point of view against many patients that are having a normal life with the with the living that piece of piece of power with ganglionnosis. Doctor Kapoor, did you want to make a comment? Uh, yeah, can you hear me? Yes, yeah, so I mean, I don't have a, an ax to grind in, in terms of, uh, because I agree with the fact that there's not a good correlation between the length of neglionic segment and necessarily, uh, how a patient's motility is, and, and we've seen patients with very long segment disease sometimes with surprisingly, uh, you know, taking a long time to get diagnosed. Um, but at the same time, we see patients who continue to have problems and we're gonna talk about this later, I guess with the post pull through and. One question that comes up, was there too much ganglionic bowel left there? And a lot of those patients get worked up, you know, with repeat biopsies and so on. And if I see a ganglionic bowel in a biopsy from a patient like that that's said to be at 2 centimeters and with growth that I could imagine what starts out at 2 centimeters, maybe at 4 centimeters in an older patient. I think that's significant and at least our surgeons often take that information to go back and do a redo procedure and resect more bowel and so I think in a way this argument about, well, people behave differently or patients behave differently cuts both ways because I think there are also probably are patients who have very short segment disease who behave poorly because of their very short segment disease and maybe leaving 2 centimeters in some people isn't. Uh, you know, it, it can be a cause for, uh, continued problems. I don't have great I don't have any data for that. I just throw it out there as a question. So, um, I'll let Doctor comment. I just wanna briefly comment on something. First of all, if a patient comes to us with problems, we always ask for the operative report. So we read the operative report and we wanna know where the surgeon started the dissection and normally if we're gonna re-biopsy. Follow the same principles. We put the Lone Stars. We protect the anal canal if it's preserved, and we take the biopsy 2 centimeters away from the anal canal. So I don't think that situation that you mentioned that you biopsy and you get a ganglionic from a residual bowel would happen. Now I'll let Dr. Pena come in. Let me, let me say the majority of patients that come to us because they had a pull through already or even if we did the pull through. And they have symptoms of retention. In other words, either enterocolitis or constipation. The majority of them do not have a portion of ganglionic bowel left. They simply behave like that, and we do not know why. A few of them have an obvious piece of ganglionic bowel left, but usually it's not 2 centimeters, it's much more than that. And on the other hand, When we talk about going 2 centimeters above detecting a line, when you finish the operation. You'll find that those 2 centimeters that you left are already damaged, so you are very near the pectinate line. So and a real concern about this operation is fecal incontinence. So I'm not so concerned about leaving a piece of gliosis because it never happened to me and I had to re-operate for those 2 centimeters. I'm more concerned about fecal incontinence, which happens. First of all, I want to say. When we remove the rectum of a human being, we are already affecting seriously the mechanisms of bowel control because we are removing the natural reservoir of the patient. Even if we leave an intact anal canal and we do a perfect operation like in the in the ulcerative colitis patients, even those patients with the perfect adult patients have problems sometimes with bowel control, you know, they have accidents at night. it's very common to see that. That's why? Because we are connecting a piece of colon that is constantly moving, having peristalsis, whereas the rectum in natural circumstances is resting all the time, only starts moving when it wants to empty. So when we remove that in a child, the consequence will be passing stool constantly and requires an intact anal canal sensation, intact sphincter. And a lot of cooperation for the patient to have bowel control. So even in patients with a strict, very well preserved anal canal, some children have some from different degrees of fecal incontinence. So and we have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe. And yet the patients that have symptoms of enterocolitis and constipation, most of It is not because of the ganglionnosis, and I hear these discussions about whether the patient had developed more ganglionnosis after the operation because of ischemia or the surgeon left a piece of bowel there, but the fact that at least in my patients, I don't remember having that kind of problem. I'm concerned about fecal incontinence. So you don't divide the posterior cuff. No, there's no cuff. We go full sim. OK, right, right, right, that's true. You go full thickness, but for Suavez, we're gonna show a video now of the technique of the transanal technique. I want to. Dr. Pena will talk through. OK. The key for me is how to avoid fe and incontinence. And I'm very worried about that because of the number of cases that we see suffering from fecal incontinence operating in other places. So I emphasize the importance of the preservation of the pectinate line because if you preserve the infected in that line, by definition you are preserving this crucial part of the bowel. Look at the perineum of a patient that was operated for his bone. The surgeon basically removed the entire anal. Canal and look at what happens in terms of fecal incontinence and this is unacceptable because the patients are born with bowel control. We provoke the fecal incontinence. Look at this patient who has no pectinate line. This is an examination under anesthesia that we do routinely in our patients with disease that come suffering from fecal incontinence. So this is unacceptable and this is and it's still happening, and I'm surprised that it's still happening. So we, we, until the time when we did that video, we had 125 transanal operations, 56 primary he spoons, 42 redo hisrunes, 21 for idiopathic constipation, and we don't think it's a good operation, by the way. So we use the Lone Star retractor and put, put the eight hooks. That you know very well and you just put them at the muco cutaneous line, in other words, at the anal verge, and by doing that we expose the pectinate line you can see there. And I'm showing the effect in a line and then we move the hooks deeper, as I said, to go all the way deep into the rectal mucosa. And by doing that we are protecting the entire anal canal. We are not going to touch it, but I emphasize, do not stretch too much now when you are working in this, keep in mind that the anterior, the dissection of the anterior rectal wall must be conducted in a very meticulous way because the rectum has a common wall with the vagina and the prostatic urethra. So we see cases that come here with fistulas to the vagina, fistula to the urinary tract that are also unacceptable. So you see, now you will start the dissection full thickness and very soon you find the right plane. And keep the traction. And if you see fat around the rectum, that means that you can get closer to the rectum because that means that you are not in the real rectal wall. We learned this full thickness dissection from so many anorectal malformations separated, and that's why we feel confident with the full thickness dissection. Remove all the fat around the rectum to be sure that you are on the bowel wall. As Dr. Swenson emphasized, if you stay right in the bowel wall, you will not provoke the innervation of the urinary tract. Look at the deeper layer there. We are going from the bowel wall. Once we decided what to resect, then we go to the bowel wall to the tissue around, and that's the deeper, the deeper layer or the outer layer, whatever you want to say. We put north and then east. And then in between stitches. That that layer of sutures fix the rectum in the right position and release the tension from the inner layer. That's the south stitch and then the west. And the stitches in between. And then we can resect the bowel, and as we divide the bowel, we take the inner layer that brings mucosa to mucosa. And we are sure that we are dealing with a good blood supply. And that concludes the operation. We try to avoid the use of big retractors pulling in different directions because that's where it stretches too much this sphincter. And that's the finishednoplasty and then we remove the hooks. And I think, I think Dr. de la Torre should be proud of us. So with that, I'm gonna actually introduce Doctor Luis de la Torre. Please welcome Doctor de la Torre from Pittsburgh, so you can start your presentation. Thank you, Andrea. Do you hear me very well? OK, my question is, are you going to pass the slides? Yes. Just one second because I'm receiving instructions from the superior command here. If I'm do you want to share my, my, uh, desk. So you can control your own slides. OK, do you hear me? Yes. OK. Let's start. So, OK. So I agree with All the comments and I hope after this presentation, we can talk. Uh, I think we lost you, Dr. de la Torre. Did you hear me? Yes. OK, that's better. OK. Uh, I can pass the slides, Andrea. We can do it for you, OK, I'm passing. You just tell me next, and then I'll pass. OK, next. No, no, I'm, I'll do it, it's not that. OK. OK, this is a brief uh. Points that we need to remember. Uh The first slide that you can see here. Is the Initial surgical treatment from Trenson that was explained before this operation was done in. Usually in 3 stages, the colostomy. In this time, Doctor Swenson performed multiple biopsies. To determine the exact length of the eranglionic area. Then I can see the presentation. Can you see? OK, Sorry, I, I can't. OK, then the problems became frequent with the Swenson technique because many surgeons provoke uh damage to the pelvic structures, the vagina, the urethra, whatever, so Doctor Duhamel proposed to avoid the anterior dissection of the rectum, so he passed the rectum in a retrorectal. fashion and then Also, many surgeons, uh, found many problems with Dujamel and Suave decide to do the culture, avoiding any dissection in the pelvis, so he decided to do the mucosectomy to leave the muscular cuff and pass the colon through the rectum himself in within, without the mucosectomy next. Then two statues were common for a few times. Well, the colostomy and then they pull through and no more protective colostomies were lived next. In 1980, uh, Philippine surgeons, uh, working in the United States published that He get the primary and the rectal culture for the patient, and this is very important for now today. Andrea commented about the history and uh if you don't read the history you're going to do many, many er problems. So what Henry Saul said is you need to do a very good irrigation before. Start any surgical procedure. So that's the main principle, uh, medical treatment before any surgical procedure, and then he was able to do the primary and the rectal pull through. Uh, using laparotomy and not leaving any colostomy next. Then the abdominal approach that was done by everybody, the laparotomy was uh. Uh, changed with the new, uh, instrument that we have to do laparoscopy, but it's the same thing. It's the same suave, the same sujamel, the same Swenson when you need to use the abdomen, you can use laparoscopy. Even in many countries where laparoscopy is not common, and they. Continue using laparotomies and the patients are well. It doesn't matter if they have laparotomy or laparoscopy and then next please. Uh, we found that some patients, they don't need a laparotomy or laparoscopy, they don't need an abdominal approach. Next. So you can see here this is a huge menu. We can add now the full thickness trans anal approach. This is a complete menu. Maybe we're missing the review procedure, but Any of these are er current er If you can do the Swenson procedure in 3 stages and the patient is, the outcome of the patient is well. It's perfect. You don't need to do a transanal and the rectal pull through or transanal full thickness pull through. If your patient is fine doing colostomy and then a swab or Swenson and then you close your colostomy and the patient is uh has fecal control. I think that's the best operation. Next. So briefly, I want to tell you how we start to do the endorectal transcendental culture next. We follow the same principles that you know very well, the resection of the ganglionic segment, the pull through of normal bowel and anastomosis, not over the anal canal, near the anal canal. Next. So Suave start the mucosectomy from the abdomen, as you can see here in these original drawings from. Franco Suave. You can see here the transitional zone and he started the mucosectomy. Just, uh, just, uh, some centimeters from the peritoneal reflection and then he continued the mucosectomy uh down for what next? Uh, in this beautiful picture from Dr. Suave, you can see here how the cuff is just in the pelvis and he passed the colon through this muscular cuff next. This image is from Doctor So, so we followed the same principles doing the irrigation, you can see here the folate with um syringe doing irrigation, that's very important. Next. And he also remarked about to preserve the anal canal. during the pull through in neonates next. So No, that one, leave it free. Yeah, thank you. So we start to do uh the same procedure that you do in patients. Uh, laparoscopic and transanal approach, you can see here the laparoscopic approach uh we remove and dissect the mesentery of the colon. This model was done in rabbits in 1995. And was published in 1997 and 1996 in two different journals. So we learned that we can remove many um uh very large segments of vowel. Next please. With this uh Experimental model. Next, please. Next, Thank you. OK, regarding the When to do or not to do transcnal or start from above or from below. It's very important. You need to have a very good radiologist. You need to have a very good pathologist and decide because at the end. The decision is not based just only in the pathology or radiology. The decision is taken from yourself. You are the surgeon and you need to take the decision. So when I have patients with this area. Eangonic and the contrast enema shows. This transitional zone, my question is always, is the abdominal approach needed in this patient next? So I don't think so. So we can remove more than that transannually. We can remove the lionic zone. The transitional zone and we can mobilize more than 5 centimeters to achieve a very good column. Next, So if, again, to do this procedure, if you want to do. Pool thickness or in the rectal pool too. Irrigations are mandatory or you need to do the irrigation, not a nurse, not the man, so you can make very clear if you introduce. The catheter 56 or 7 centimeters and the patient starts to pass gas and poop very easy, that's the most probably the area that you can remove. Next. We also use the proposition as you can see in the video from. Uh, Dr. Pena next. Uh It's very important to protect the anal canal. We have patients in our center. Many patients with fecal incontinence, patients with Hisborne disease. Submitted to laparoscopic approach. Uh, very nice abdomens, very nice surgical scarves. But these patients suffer from severe fecal incontinence. I know that the fecal incontinence. Do not come from the laparoscopy. The fecal incontinence becomes because many surgeons do not recognize the anal canal or they not protect the anal canal during the pull too. Next. So when the anal canal is protected, er we start to play the same traction sutures that you can uh you watch in the video from Doctor Pena next. The same thing next. To do a very good endorectal culture, you need to identify a very good plane of dissection. And you need to observe the circular fibers of the rectum that is going to be the rectal cuff. Next. This dissection is very easy using a blonde dissection and if you are in the right line of dissection, this operation is almost uh bloodless. Next. Here is the problem. The problem is the muscular cuff. When you reach the muscular cuff and you can see here a huge, uh, floppy and dilated colon. And you leave this muscular cuff, most probably the patient is going to have chronic obstruction, and this chronic obstruction is going to produce a chronic colitis. To create the muscular cuff, you need to place two sutures and Taking one of the suture is going to take the mucosa and the muscular cough and the other suture is going to take just the muscular cuff and you need to cut in between. And you need to do this around the cost next. And you are with, uh, you are will. Uh, be able to observe the mesentery from the posterior wall and you only need to go in, uh, ligating and cutting this, the vascular vessels and you will reach more, uh, length of color next. And you can see here, the transitional zone. So we can take the biopsy. And if uh Doctor Reyes has said that we have ganglion cells, we are very happy and then we continue the dissection. Uh, next In the meantime, while we wait for the first biopsies. I resect as much as I can the muscular cuff and then I do a myectomy in the posterior wall and I resect uh. 1 or 2 centimeters in, in the length of the muscular coils and I do a short muscularc from below. Next. Then We confirm that we have ganglion cells and We mobilize more than 5 centimeters, maybe 5, maybe 6 and we send this to Dr. Reyes to do the final study next. And then anastomos. Next. This is a video that I can show you. Uh This video was edited to stress the most important aspect of the transannal approach, even if you do transanal in the ectal or transannal full thickness pull. As Doctor Pena mentioned, you need to pay first the Hooks Superficially, you can see here the dented line. Then you need to replace the retractor below the picking line to avoid damage. To the anal canal, so you never have to see the anal canal during a transanal pull through. Again. Deeper OK, and then the same. I use 1 centimeter from. We did, we placed the hooks. And we place many 5 or 6. Silk sutures. For traction. And now I'm going to demonstrate how, where is the anal canal. You can see here the anal canal very clear. And again, this is the anal canal. I'm going to remove the hook. You need to be very gently, so. The hook is protecting the anal canal. Once we're sure that the anal canal was protected. We start the dissection with. Coagulation only we don't use uh cut. We use coagulation and then we develop a very thin uh. Area Uh, to reach just the mucosa. When the mucosa is ready. We start land dissection. If we leave some part of the muscular layer, we are able to dissect. Uh This tissue, it's important if you want to do an endorectal culture. So you need to confirm every time that you are in the right plane. You can see here the mucosa is almost transparent. When you have the right plane of dissection. You can start to do blonde dissection using this. Uh Small bowl of gas. Bolinas in Portuguese. Uh Dissectors in Spanish. And there's more battles that That give supplied to the mucosa, you can. Keep doing the The coagulation. So you can go Until you can feel the muscular cuff is floppy, usually it's 5 to 7 centimeters. And then this is how we open the muscular cuff. We use 2 traction shooters per quadrant. One is the muscular. And the mucosa uh stitch. This is the stitch number one. Then the second stitch is just. The muscular cuff. This is a stitch number 2. And You, you are usually. Place 44 quadrants this uh. Traction shooters. Once we have all the sutures, we need to go up here. You can see here the traction sutures 1, mucosa muscular, and the second one, just musculars. So caught between 1 and #2. And you Get into the peritoneal. Cavity. So you are doing a Trans anal Laparotomy. Some surgeons place uh laparoscopic instruments now 3 or 5 millimeters through. This space and they This is a muscular cough. This is the proximal column. This is a mesentery, so you need to dissect, ligate, and cut these vessels and you can obtain. The land that you need for these patients. Uh, when I have seen some surgeons using laparoscopic instruments introduced by this small space. Look, that we never stretch the sinters. We don't use uh retractors in. In this approach to avoid damage to the sphincters. You need to keep the same traction all the time. And then you need to evaluate the colon length microscopically. So if you have a very good, very good contract enema. Usually, you can have very good correlation with the technique. We always take a full thickness biopsy for. Uh, frozen section. We never send um small biopsies like seromuscular biopsies. Because the pathologist suffers. Uh, when you send a very small biopsies in a frozen section, so you can see here, this is a full thickness biopsy. So Doctor Reyes can observe. Uh, both places. If they say we are good, we have ganglion cells involved. plexus, we're very happy, so we continue doing the Myotomy? And Look How we respect the A muscular cough to do it short. So also we do the myotomy all the way down. Until we reach the anal canal. One of the most common problems in this uh procedure is that you leave a large uh cough and this causes obstruction. This is just for demonstration how it's free the anterior wall. And we are placing Yeah, the muscular cuff just to be sure that it's not folded. It's done. And then We do the same. As you can see, we placed some sutures as two layers anastomosis is the same. Uh Procedure that you observe. In the previous video from The full thickness transcendal approach. OK. It is very important Also, the anastomosis should be performed with. The most perfect technique that you can do using fine sutures, 5 or 6 by drills. To do the anastomosis, to perform the anastomosis, I removed the hooks. Because if you don't remove the hooks to do the anastomosis, you never see the anal canal. So once the I know canal is again er Re-expose it We do the colectomy. And you can see here the mucosa. I A healthy pink. And this is the mucosa. Far from the Anu Canal. So these patients could be. Uh, could have obstruction, I don't know, but no fecal incontinence. That's very important. And then you need to remove the retractor gently. We can continue with the presentation. Next, please. So indications that I found to do a transcendental approach next. First of all, is the patient. Is a candidate for transcendental approach when you do rectal irrigation and this irrigation improves the patients uh immediately. If you do irrigation and the patients er Was on like OK, I think Dr. de la Torre is calling back in. His phone line dropped out, so uh. One minute, he says. Now, the question that we can address one of these questions while we're waiting for him to come back in is. Um, I know this technique is different, but the question of how to not twist the bow. Um, Any suggestions about how to prevent that? So usually when we are progressing in our dissection, we keep a big micro suture so we know the orientation. And also after we are done with the go through, we pass a big foley to be sure that it's going, uh, straightforward without. No. I just use different kind. I use uh micro and and like not undyed micro and some sutures and alternate. positions to make sure I stay 1 or 2 tasks. Yeah, that that person is not uh uh. through the abdomen and right, right, that's when you twist it when you work, so you have you don't twist it, right, right, that's why I've never done the abdomen that's OK. Um Do we have Doctor Dela Torre back. Yeah. Luis, are you there? He's still calling in. Luis, just let us know when you're there, yell out. Um, if not, we can proceed with ours. Did you hear me? Yeah, we can hear you. OK. So the indications uh uh we were saying the patient should improve with the rectal irrigation. Next. Then if the patient improved, this patient most probably suffer a history of disease. So the clinic is very important. Low intestinal obstruction. Place a rectal tube. If with the rectal tube, the patient improves, he can start to have milk, to start feeding, and no more abdominal distention. I Take the patient to the OR and I take a very good biopsy. If the biopsy next. Is negative for the ganglion cells and it has hypertrophic nerve, whatever, confirming his disease. I continued the irrigations and when the patient is in perfect condition, this usually takes 3 to 5 days. I stopped irrigation for 12 hours, 24, and I take the patient to the fluoroscopy department. And we do the uh contrast enema. So you can see here how we place the tube just. Uh, introducing in the anus, we don't, we don't use more than 1 centimeter. 1 centimeter is too much in length to do the contrast enema. And when we found the transitional zone that is very clear in the lateral view. To me, the study is done. We don't want to feel all the colon and the ileum to determine if the patient has or no lung segments. The lung segments are totally different. Lung segments, they don't improve with irrigation. Long segments are, uh, I think is another big problem. Next. So we use the transcendent approach just for patients with rectum or rectosigmoid uh a ganglionosis, that is the most frequent uh history uh type. I can say more than 80% of the patients will have the rectosigmoidal ganglionic next. The question is, if you need to do an abdominal approach when you have a very low segment with a ganglionosis, even if you have the rectum and the sigmoid. Next, For these patients. The abdominal approach is required. So you can do laparoscopy, laparotomy, robotic or whatever next. Obviously patients with Total colonic ganglionosis, they required an ileostomy. Next. A very common problem is uh maybe not in the USA. But in many countries, patients with his problem come late, sometimes at 23456 years. And If this baby, as you can see here, And the father said, my baby has his nest. And the parent requests or anybody requests a primary transal pull through, I think this, this is not a good idea because these patients have chronic dilation of the colon next. So you can see here a very short segment with a huge dilation. These patients are not suitable for a primary transenal vulture. Long and massive megacolons are not a very good candidates for transenal uh primary vultures. Next. Because we need to remove these huge segments of uh chronic uh dilations. Chronic dilations also have poor motility, so we need to resect these segments. Next. Now, I want to remark some anatomical and technical considerations to achieve uh. The most accurate, the most functional and anatomical surgery. Next. One. When you are going to do any culture, laparoscopic, robotic, er, whatever, you need to get a very good bowel. To pull down. The very good bowel means good irrigation, good pathology, and no tension. The second is you need to be very cautious in the blind zone. The blind zone is the pelvis. So if you want to do a dujamel, if you want to do a suave or a swenson, you need to take care of all. The elements that live in the pelvis. Avoid damage. And the third is to do a perfect anastomosis, not too low, not too high, technically well performed and preserve the anal canal. Next. This is uh anal canal just to remember the anal canal. He Composed by three clear songs 1. Is the anoderm that has an squamous epithelium or is not a mucosa. The second is the area. Where the dented line or the pectinate line lives, and the third is the columnar zone. Next. So you can see here the columnar zone. The columnar zone should be preserved for fecal control. Next. Obvious the back in line and then next. The ano there. We have seen patients with uh different techniques. Uh, submitted for hisborne disease, transcanal, uh, laparoscopic, Suarez, Swenson, whatever, and we don't find any of these elements. All these patients are fecal incontinent and it's very interesting. These patients also, they don't have enterocolitis. Next. So this is the rectum. This is where we need to do the anastomosis next. And this is the skin, so we need to identify very clear all these elements when we do a pull through for Hidborne disease. Next. The same, the anoderm. Then you can see the dented line and the columnar zone. Then next. Another aspect of the anal canal, the anoder. The dented line and the columnar zone next. A good anastomosis should be done. If you want to say 5. 4 or 6 millimeters or, or 2 centimeters, whatever. It's on your own risk. But it's not uh good. It's prohibited to do the anastomosis below this line. Next, if you do the anastomosis below this line, next please. The patient will be fecal incontinent. Using any of this. If you use all this shadow area, the patient is going to be full fecal incontinence. If you use 50%, you're going to have partial fecal incontinence. So take care of this area next. Next, please, I think this is the last one. So thank you so much Dr. de la Torre. We're gonna continue with uh our presentation, so I just wanna make sure that now everybody is seeing a diagram. I was actually trained during my fellowship to do Duhamel procedure. It's an easy procedure and the concept is that you're going to amputate here in the rectum, then you're going to remove. A ganglionic bowel and you're going to pull through this normal ganglionic bowel behind the rectum in front of the sacrum and then you're going to anastoize the posterior wall of this a ganglionic rectum with the posterior wall of the ganglionic rectum anterior, sorry, then you're going to, it's actually the posterior, then you're To use the stapler to make a common wall, so you will have the famous Duhamel pouch that at the end you have anterior a ganglionic and posterior ganglionic segment. Here we don't do the Dujamel, but we, we are aware that many of them work without any problems. But we, we receive the ones that are not working so well. So now we're going to talk about total colonic ganglionosis. And I want to start with a question. Everybody can vote in the poll. What is your surgical approach for total colonic ganglionosis? Option 1, Lester Martin procedure. Option 2, rebind procedure. Option 3, straight ileoanal anastomosis. Option 4, J pouch. Option 5, Duhamel. Option 6, Kimura. So everybody can vote. And currently I'm seeing Duhamel, straight Ioano, Rabin, and Lester Martin, so I don't see anyone with J pouch. And I don't see anyone with chimura. Very good. So we perform straight ileoanal anastomosis, and the technique is the same as Dr. Pena described. So here is a diagram of Lester Martin procedure, and the idea was to use the water absorption capacity of the a ganglionic column and anastomose it to the small bowel that was the normal ganglionic, and the same with Dr. Kimura, but in his case he thought that the ascending column had more water absorption capacity, so in his technique he was using the right column. And again, these ideas are very good. Because it takes advantage of the normal peristalsis and the water absorption of the a ganglionic bowel in order to try to form solid stool and decrease the number of bowel movements. Those were great ideas, but unfortunately sometimes good ideas don't work, and cheating on Mother Nature is not easy. So we believe that total colonic ganglionosis is still a challenge for pediatric surgeons. We have suboptimal long term results and a high incidence of complications. The most common complications that we see is ileostomy prolapse, and I'm going to discuss on how to try to avoid all of them. The whenever you open a stomach in a mobile portion of the colon or the intestine, you are at risk of having prolapse. So the way we avoid ileostomy prolapse is we tack it the bowel proximal to the stomach to the abdominal wall. Now obstructive symptoms following pouch pull through, we do not recommend to perform pouch pull through in patients with Hiprung. Wrong pathological diagnosis. This is one that it's difficult for us to solve. You actually have to get another pathologist, and sometimes it's not in our control. Anastomotic stricture or acquired atrigia, those can be avoided by following good surgical technique. Severe diaper rash. It can happen if you have a destroyed anal canal and the patient suffers from fecal incontinence, but in cases of total colonic ganglionosis, it can also happen if you perform the pull through too early. That's why we're going to explain what's our routine, and enterocolitis, as you have heard this panel saying many times, we think it's something that we don't know everything about and it's not totally preventable. So our approach, if the baby was born here, we diagnose total colonic ganglionosis as a healthy looking baby, good weight, we would perform a colectomy with a straight ileoanal anastomosis and ileostomy at presentation. And then we would only close the colostomy when the child is toilet trained for urine and when the child is willing to accept rectal irrigation. The mother actually has to demonstrate to us in clinic that the patient accepts rectal irrigation because we know that those are the patients with a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation. So now I'm going to present a case where the patient is not born here and it's a scenario that you can face. So this is a 2 month old male patient with a history of delayed elimination of meconium and diagnosis of total colonic ganglionosis shortly after birth. Ganglion cells were only present 45 centimeters proximal to the ileocecal valve. Ileostomy and mucous fistula were done. Patient came to us for a second opinion. Currently, the patient is thriving and growing well. So here is the contrast enema in this patient with total colonic ganglionosis. So what would you do in this case? Confirm the diagnosis by reviewing pathologist slides. Proceed with total colectomy and ileoanal anastomosis, observation only until time of pull through, irrigation, teach the irrigation technique, check the urinary sodium, or options 14, and 5, so everybody can vote, and I'm curious about how you would approach this patient. So we have Some people saying confirm the diagnosis by reviewing pathologist slides, others saying observation only until time of the put through, and the majority of people, more than 65, 75% now saying options 14, and 5. So that's the one we agree you want to confirm that the patient actually has Hiprung's, so you want to make sure your pathologist agrees with this diagnosis and that all the biopsies were well taken. You want to teach the mother irrigation technique because remember this patient at the time of the pull through will, the mother has to be a master on how to do irrigation. And also patients that have the colon, they are at least at risk of having enterocolitis in this unused colon, and you want to check the urinary sodium. So we check if the patient is less than 20 millimoles per liter, then we start oral replacement with sodium. So options 14, and 5 would be the right one. So we confirmed that the patient had Hirschsprung. The patient was doing extremely well until 10 months old when he presented with ileostomy retraction and episodes of enterocolitis. So now what would you do proceed with total colectomy and ileoanal anastomosis, proceed with ileostomy revision only, proceed with subtotal colectomy and ileostomy revision so everybody can vote. And we have answers all over the place, so. 30% saying proceed with total colectomy and ileoanal anastomosis. And 20% saying proceed with ileostomy revision only. And 35% proceed with subtotal colectomy and ileostomy revision. So I just want to remember that this patient had a ganglionosis 45 centimeters proximal to the ileocecal valve, and this patient is only 10 months old. So our philosophy is that you should not do an ileo. Anal until the patient is toilet trained for urine because the patient needs to know how to go to the bathroom and evacuate in the toilet, otherwise you will have the worst unmanageable diaper rash that you've ever seen. So that's why we tell the patients to wait, be patient, and once they are toilet trained, Then we will do the ileoanal. In this case, doing a total colectomy, an ileoanal, and a more proximal ileostomy. Remember you're already very far in your ileum, so I think you would put this kid in danger to decompensate, a situation that he was compensated. Ileostomy revision only you would leave this unused column that it's now having enterocolitis and you cannot irrigate well, so we actually proceeded with a subtotal colectomy and ileostomy revision and we're going to follow our protocol of only doing ileoanal anastomosis once the patient is toilet trained for urine and accepts rectal irrigation. Do you want to comment, Dr. Pen, in our Management of total colonic. Perhaps in that case I would have done a total colectomy, ileoileoanal anastomosis, and ileostomy. Yeah, the reason why I didn't do that is because this was 45 centimeters proximal to the ileocecal valve, so that that child was doing very well, and it would be if you do an ileo anal and another ileostomy, I would lose more ileum. So that's the reason why I didn't do it. I agree. I would, I would avoid, I would preserve that ileum as long as possible. So any questions about total colonic ganglionosis before we move to our next session? Let me say a few words about the philosophy of um closing the ileostomy, but only by the time that the patient is toilet trained for urine and accepts irrigations. It's kind of strange for many pediatric surgeons. We are in a competition. Usually pediatric surgeons compete with each other, trying to close the stoma as early as possible, trying not to leave the stomach as early as possible. If somebody is proposing an operation at 6 months of age, another one will do it 3 months, another 11 month, another one neonatal. But actually it came up into my mind the idea of waiting because I watched the babies in which the ileostomy was closed early in life. And remember that in patients with Hip disease with or without total colonic ganglionosis, we resect the natural reservoir of the patient and we connect a piece of bowel that has high motility like small bowel to the anal canal. And in an adult that receives that operation for ulcerative colitis, you can tell him to try to hold it. And that adult will live the rest of his life trying to avoid accidents like having terrible diarrhea all his life and pouches and patches and my experiences are not good in his disease because the bowel, when when you produce intentionally stasis in the bowel, you have bacteria proliferation and colitis and inflammatory changes that produce secretory diarrhea and the patient actually gets worse. Whereas if you, if you wait until the patient is totally trained for urine, it usually takes about 3 years. The parents usually don't like the idea originally, and many surgeons will offer the family to close the ileostomy earlier, but actually the baby is happy with the ileostomy. The only unhappy people are others, but not the baby. And when you, when your baby is really totally trained for urine, usually around 3 years of age. You close the ileostomy and you will be surprised how soon the baby becomes totally trained for a stool, provided you did a correct operation and preserved the anal canal. And the idea of accepting erectile irrigations cannot be overemphasized. If you take a child that has been traumatized with rectal maneuvers and you try to do rectal irrigations, it's going to be a lot of problems, whereas if a patient has an ileostomy and the mother does rectal irrigations once in a while with a soft catheter, the baby will accept that. So these patients with total cochronic ganglinosis have a high incidence of enterocolitis and therefore most likely the patient will need rectal irrigation and it's not going to be easy to do rectal irrigations in a 3 year old that has severe diaper rash and doesn't want anything near the anus, and that's the reason for that philosophy. I strongly suggest that. So now we're gonna invite, if there are no further questions, we're gonna invite Doctor Belinda Dickey to proceed with laparoscopy and the surgical management for H-prung's disease. So I think we've already discussed this topic a little bit, um, but this is to, to further our discussion on when is it appropriate and when is it not appropriate. Oops. So, you can break down laparoscopy and Hirschmann's disease, I think, into three sort of categories where you would use it during the diagnosis, during your definitive surgery, and postoperatively, um, as the child gets older, whether you would use it for management of this fecal incontinence and creating them alone. So initially in diagnosis, so people would use it potentially and, and, um please feel free to comment for the rest of the panel if there is any concerns that this is more than just rectosigmoid and you want to do leveling biopsies and in the, in if, if an ostomy is required in the ostomy creation. So this is just an example of a contrast study on a one month old who had presented with inability to stool well. She had abdominal distention and was not growing well. A rectal biopsy came back as no ganglion cells but also no hypertrophic nerves. Um, she was actually presented at an outside institution and they proceeded with a contrast enema and this is what came back. Um, so at this point in time, I, there's no poll questions, but maybe the panel can comment on what they would do at this point. When I, when I see a patient like this, this is where you put together the clinical picture, the radiographic picture and pathologic picture, and it's a little confusing and it's not the straightforward rectosigmoid, and this is where I get concerned and will not go for a transanal approach for this type of procedure. How would you read this contrast study, Dr. Krauss? So, uh, We have a single frontal view of the whole colon. I don't have a lateral view. I didn't see what it was initially. That's OK. Oh, there it is. OK. So this is what I would like to say about this. If you see the rectosigmoid and you didn't do the whole thing, you might say, Oh, this looks pretty normal because the rectum is bigger than the than the sigmoid. But I don't think that's the case here. I think this is abnormal. The rectum is fairly small, and see how there's a small colon, and then there is a little bit of a transition, but even the proximal colon is, I think, smaller than it should be. And so I would be worried about proximal disease, and I don't know where the transition's going to be here. I would say it's not rectosigmoid. It's some kind of a longer transition, and I don't think a repeat enema is, is going to help you. I just think that this is one of those difficult cases, and when you get a biopsy and it's Hirschprung's, it's hard to tell where it is here. So this is one that we actually took to the operating room and did leveling biopsies with a laparoscope, and she actually turned out to have no ganglion cells up to 20 centimeters into proximal to her ileocecal valve, so she ended up with an ileostomy, and we actually left a mucous fistulas for her. Can I ask a question on minutia for a second before we move on. When you do your laparoscopic biopsies, full thickness and a stitch or just a serumuscular? Full thickness and a stitch, OK, yeah. This is one where I'm sorry, where you could either do it laparoscopically with your 3 millimeter instruments and an endosheer, or if you have the proper positioning, you could just take a piece of the colon out, the umbilicus or one of your port sites and do it with a, you know, a, a open technique to do your full thickness biopsy. Uh, um-hum. There's one trick, uh, laparoscopically, you could, That you could stitch, you could stitch it actually and tie it extra corporeally, and the colon always just pretty much comes up to the abdominal wall. You just tie it down and they go, so that's one trick if that if laparoscopic suturing isn't, you know, or you're watching your fellow try to do that, who's helping you, right? And that question going way back, but I just want to make sure we asked it, the suction rectal biopsies, how many do you do and and what's your, how far up do you go when you do those? For anybody here, so I usually do 3 because that's the 3 capsules that it comes with the kit. Oh, you use that, yeah, OK, right. So I usually get 3 and I try to, to insert this much I would say. We have to get the whole bullet in until there's there's lines on there, so I put it up to at least the second. Yeah, I go to the second line if you have that kit, which is about 2 centimeters plus. So you don't go the first one this level, the 2nd +11 centimeter higher or anything like that. I sort of rotator, yeah, I go to. I do, I do like posterior. I do like 4:30, 6, maybe 7:30. All at the same proximal distal level. Well, you take it out and you insert again, so I cannot guarantee that you're, but you don't march up when you do that, OK. And I think that you, you would be concerned if you're marching up in a newborn because peritoneum is not very far. So I mean, the, the suction rectal biopsy is nice because if say you did go full thickness, it's low enough that the kid's not going to get intraabdominal sepsis. So confronted with this, with this kind of case, the X-ray, the contra cinema for me shows that either a long segment history or total coronal ganglionosis, and with a rectal biopsy showing no ganglion cells, I would do a laparotomy and then and end up with an ileostomy. That's what I would do if the baby was in very good condition, an excellent condition. I may even do a total colectomy in an ileum, put through an ileostomy again, provided we have enough ileum. We don't want to take too much ileum. The reason is that if you leave the babies, the colon in these babies, some babies do very well, but some babies come back with symptoms from the from the piece of colon that accumulates mucus and becomes infected, and sometimes they have a kind of spectacular symptoms from that, and it's very difficult to irrigate. That's important. So I always warn my the the parents whenever we do this that um we were just doing a diverting ileostomy, but if the patient or the baby gets recurrent enterocolitis, becomes distended, doesn't do well, we may have to do the colectomy sooner than later. Can I make just one more comment about this, the colon, and I just want to reemphasize that if you look at the proximal colon that is present here, that the caliber of it would be expected to be bigger if the transition really was somewhere in the colon. And the reason why I think it's not is because the caliber of the remaining colon doesn't look all that big. It looks like a normal caliber, and it should be bigger if it's really Hirschberg's disease that's more distal. That's my, that's my feeling. Well, you were right. So looking at um actually the, the definitive pull through laparoscopy, as we've discussed already has been applicable to all um the techniques of Swenson, Suave, and Duamel. And I've just got some contrast studies sort of to point out, uh, Todd's point earlier on what level would people consider doing laparoscopy when they see their contrast, um, study. So I don't know if we can put up a poll Todd or not. So on this contrast study, I think I only have the lateral on this one. who would do laparoscopy who or who would do just a straightforward trans anal rectosigmoid. Give me 1 2nd. So we can just pull the table here, Todd, right, so I would do laparoscopy on everybody, so I'm not a good one to ask. So even, even at this level you would do that. Dr. Pena and Andrea, I know your answer. I will do this. I would do. I would. So this is what this is, of course, I'm in Cincinnati. This is why it's good to bring an outsider. I'm just curious, you know, well, look, the, the audience is split. I mean, not divided, but definitely more in favor of transanal, but you definitely have a, a, a fair number of people that still prefer laparoscopy, and I think it goes to your point that you said before. All right, how about on this case? This is the same. How fast can you type? Well, I can just clearly, it's going to be the same choices, right? Same choices. Who would do laparoscopy on this one or laparotomy, I guess if you want, if people aren't using laparoscopy versus just trans anal. Looks like 75% laparoscopy. So I think as you get beyond sort of just your straightforward rectosigmoid, left colon splenic flexure transverse, I think la laparoscopy is very helpful. But, but it's not really correct to say laparoscopy or transenal is laparoscopy plus transanal, right? When you say laparoscopy, you mean you go all the way down laparoscopy. I'll add the word pure, like, like, like that's a combination, sorry. Uh That's my fault. I should have written it better. So Doctor Pena, for this case, would you start in the abdomen or would you still start transan? For me it's not a difference. I, I understand the problem of leakage of gas when you do a laparoscopy, but you can pack from below, and that's, uh, but most likely I would prepare the family to say that the patient may, may need a laparoscopy, and I will invite Belinda to do it, or I will open the abdomen. Here's another one. Where do you think the transition is here? So. I, I think that the, I, I don't think it's at the splenic flexure, so I don't think this is a small left colon. I do think it's above the rectosigmoid, uh, in the typical rectosigmoid, which is lower, you know, at the lumbosacral junction. I think it's higher than that. It could be descending colon sigmoid region. I would, that's what I would be my guess. How are people voting on this one? Well, no one pure trans anal. The divisions between laparoscopy and laparotomy, and my question would be for those, if they can answer in the, or even Doctor Pena, for those who, who would answer laparotomy, is it because you don't feel comfortable with laparoscopy or because you think laparotomy is a better approach? There is, there are some people saying pure transenal here. The, the, I think the answer is. How confident would you feel with doing laparoscopic procedure if you have well trained and you feel very confident, go ahead and do it. Provided, provided you don't say that that that's the only way to do it, that's, that's, that's the only. So this is just a schematic of where we could potentially put our port sites for laparoscopy. It really depends on the patient how it is, um, the size of the patient, what age the patient is. Start usually umbilicus, and then you will put 2 or 3 ports otherwise in. Pretty similar, I'm assuming what you would do as well. I, you just, um, have one extra port from me. I'm just wondering what you there's just if you need to for mobilization, and I think it depends what a transition zone is. If it's a lower transition zone. Um, it depends if you're going all the way to hepatic flexure or something, then you may need a left sided, so that's, this is the sort of one that you would put extra if you want to put it in there. Yeah, my ports are exactly the same without that one, yeah. So the definitive pull through, if you're using laparoscopy together with your transanal. Um, people, um, would use laparoscopy for your leveling biopsies, um, aid in mobilization of the colon above the peritoneal reflection, takedown of the splenic flexure and sigmoid disease, mobilization of colon in long segment, and then it is helpful when you're pulling the, uh, somebody had asked about twisting before and how you make sure you're not twisted, uh, when you're pulling through, you can watch the pull through go through and make sure there's no twists or kinks. So I think, um, sorry, whenever you're ready, Dr. de la Torre wanted to make a comment, so I won't be able to hear because my earpiece isn't working, but if you could bring him in. No. That's it. OK, let's keep going, sorry. So the potential pitfalls of laparoscopy, one is a if you're not comfortable, 2, I think it, it becomes difficult when you have to pull the transverse of the right colon and you actually need to de-rotate the colon to make it straight to come through and not kink the terminal ileum. If that's the case, I will usually end up making a smaller laparotomy incision than what you would need to to do the cold total clottic mobilization, but in this way you can actually de-rotate the right colon to bring it down. And if you have a very distended colon and your visualization is poor, of course your laparoscopy becomes a little bit more tenuous. Benefits of laparoscopy versus doing pure trans anals, and we had once again talked about this briefly, is I think you really decrease the stretch of your anal sphincters on on ones where you're going high and you have to put your retractors in in order to see um there's a lot of stretch on the anal sphincters which could in turn. Affect the fecal continence afterwards with laparoscopy, as Todd and I had spoke about, you can dissect way down to the pelvic floor, so your actual transanal dissection is very short and there's very limited stretch on your sphincters. Linda, before, before, when you talked about your port placement, can I also ask you how you position. When you do laparoscopy and then transanal, so we do a total body prep, um, and then I just flip the legs up so they're in lithotomy. If you're uncomfortable doing that, sometimes we also flip the patient and put them prone to do the initial transanal dissection. So when they're total body prep because their feet are prepped in, then you have the luxury of flipping them like a rotisserie. OK, so. On the bigger patients, it becomes a little bit more difficult and sometimes we will have to put them in lithotomy, re-prep them, flip them prone, re-prep them, and just go back and forth that way, which makes it a little bit more difficult. You know, the one trick that someone showed me that I love is I have this bar that I put up over. And it's flexible, so I go down when I'm doing the laparoscopy, then I just flip it up. Everything's sterile and prepped so I can go up and down at will if I'm doing lap or trans anal, but that's if the baby is in, yeah. So that I mean that's essentially what we do with the legs. So if it's total body prep, we take them, we actually clip them. They have stockings on, and you clip them up to the ether screen of your anesthesia person. So that same sort of concept. Um, compared to laparotomy, I think your laparotomy, laparoscopy does decrease your incision and reduce, uh, discomfort. Um, there is the potential earlier initiation of bowel functions, you know, it, it's sort of 11 or 2 days at the most, I would think compared to if you were to do a laparotomy. If you're doing transi alone, I don't think it offers that much, um, decrease in your time of your return of bowel function. And then that's the same thing with your postoperative hospitalization. So here's just a case study. This is a 3 year old boy who had chronic constipation, and I'll show you his contrast cinema, which showed a very dilated rectum. Um, the rectal biopsy then showed no ganglion cells and hypertrophic nerves, so this is the contrast study that was obtained. And if you want to comment on that, Dr. Krauss, so, uh, again, a frontal view, this is, this is the, so when we do patients for chronic constipation, we do a little bit different enema actually we do the same lateral view. And we do the same frontal view, but we actually fill the entire colon because it's kind of important to see what is the relationship of the rectosigmoid to the remainder of the colon. Are they all dilated, or is one segment dilated out of proportion to the others? Usually the rectosigmoid is the one that's more dilated in a patient with chronic constipation, such as in this patient. It looks like they can take and put a watermelon in there. It is huge. It's a huge rectosigmoid, but if you look at the remainder of the colon, it really doesn't look all that, all that dilated. And so this is characteristic of something we would see in a patient with chronic constipation. We, this patient ended up having biopsies, um, but, and it did show no ganglion cells and hypertrophic nerves, so we felt that this was sort of consistent with Hirschprung's disease. The contrast study that we're showing there is a very, very, very bad study. I will send the patient back. I would, I would say that most likely it was done by a technician who put the tube there, open the, the, the, the contrast, and fill up the entire colon, and some patients, they feel even the small bowel. Some patients even vomit, you know, they're so, so I would ask them to, to be done repeated by a, by a good radiologist. And, and because that study doesn't say anything to me, I will need many more films to. I want to see the lateral film. I want to see if the dilatation goes all the way down to the pubiccoy line and if indeed the colon is dilated all the way down, that is not history, even if the biopsy says something different. I don't have all the pictures, but there was a transition. There was a um non-dilated portion then going into this very large one. This was just the all filled up version. Although this is kind of atypical again because the rectum is so much bigger than the remainder of the colon. The remainder of the colon doesn't look all that dilated, and I agree with Dr. Pena that if I saw a transition, if we indeed saw a transition, which we can't, can't see here because the lateral view is not showing it, if we did, I wouldn't go very much further. I wouldn't fill up the whole colon like they did here. We ended up um treating the patient as Hirschprung's disease and we did laparoscopy first and as we had talked about with this very dilated rectum as you're trying to pull it through in a transanal that thing's giant and and I think you would have damaged the sphincters and caused a lot of stretch so we did most of the mobilization laparoscopically and had a very short transanal and we were able to do the rest of it transanally, um, and then the patient did great two days was returned to bowel function and and discharged home and has been home now with very. So. So any questions for Doctor Dickey about laparoscopy in the management of Hichsprung's disease? If you have any questions, that's the time to ask. Did you do a bowel prep on that patient? Yeah, so our bowel preps, so we do go lightly bowel preps, but um, we also do rectal irrigations, um, so the nurses on the floor know they, they give the go lightly until clear, but simultaneously they'll do rectal irrigations to help, um, evacuate. So I see that Dr. Rintala is already on. Dr. Rintala, we had some technical issues, so we are delayed 30 minutes, but stay on, we'll be with you shortly. We're going to present a few case scenarios just to entertain everyone, and those are all real cases, so it's what happens when you're dealing with your patient. So the first one is a 1 month old female patient comes to your clinic with suspected his bruh. Past medical history delayed meconian elimination upon physical examination, digital rectal exam with explosive stool. So what is the next, next step? suction rectal biopsy, full thickness rectal biopsy, contrast enema, rectal irrigation, so everybody can vote, we're gonna have our poll. Mm. Sorry. Just 1 2nd. You can think about Dr. Fonsky is working on it and you're gonna get your full. So everybody can vote now. So I saw suction rectal biopsy, contrast enema, and rectal irrigation, and I think everybody would agree that if you are suspicious of His prong, you have to do those three. Exactly how it's gonna be the order of those three, I think it has to do with logistics in your hospital. Normally if I can, I want the contrast enema first because based on the contrast enema, then I'll see if the patient truly needs a suction biopsy or not. So and I also take advantage and do the rectal irrigation before my biopsy so the mom learns how to do it, but I think the order as long as you do them very. With a short amount of time it doesn't matter, it depends on your logistics, but we truly believe the rectal irrigation is a maneuver that every patient with Hirschsprung's disease, the parents have to be completely comfortable doing it, so we have a video and you can even in our website you can access this video on how to do rectal irrigations and we're going to show it for you. We have audio in this video, so I just want to make sure it's working. If your child has been diagnosed with entercolitis, we recommend that irrigation should be done 3 times a day and more often if needed. If your child has entercolitis symptoms of fever, belly distention, not stooling, vomiting, explosive diarrhea, foul smelling stool or gas, you should irrigate first, then seek medical attention immediately. In order to perform a colonic irrigation, you will need to gather some supplies. You will need normal saline. A few bath towels are. Two basins or small tubs. A 60 mL syringe with a catheter tip. Lubrication that is water soluble. A silicone catheter, you will need a 16 French catheter if your child is under 1 year of age, or a 24 French catheter if your child is over 1 year of age. Start by warming the normal saline. It is suggested to sit the bottle of saline in a sink of warm water. It is very important to test the temperature of the saline on your wrist to ensure that it is not too hot. Pour the warm saline into one of your basins, lubricate the appropriate size catheter and gently insert it into the rectum. Pause and allow for any stool or gas to run out the end of the catheter into the basin. As you advance the catheter, allow for any other pockets of stool or gas to empty. If gently pushed, the catheter should follow the curve of the colon. It is important not to force or advance the catheter further than the wide divider port of the catheter. Note that the best position is to have your child laying on their back with their knees bent to their chest. Draw up 20 mLs of warm saline into your catheter tip syringe. Connect the syringe into the end of the catheter and inject the saline into the catheter. Disconnect the syringe from the end of the catheter and allow for the saline solution to drip into an empty basin. You will repeat this process by advancing the catheter about 1 inch each time before injecting 20 mLs of warm saline. It is important that between each installation of saline to allow for the solution to drain from the catheter into the basin. If the amount that is draining out is not equal to or more than the amount of saline that you put in, continue to move the catheter in and out while twisting to drain the pockets of saline, gas, and stool. If you feel this has not allowed the colon to drain well, you can attach the syringe to the catheter and pull back very gently on the syringe. It is important not to pull on the syringe if you feel resistance. Repeat this process until the fluid that is draining back out from the catheter is clear. Once the return fluid is clear, remove the catheter from the rectum. Wash your supplies with soap and water and allow to dry. These supplies can be reused. of the patient look at very carefully. Another view. So with this history and contrast enema, what would be your approach? Start transanal, start laparoscopically with multiple biopsies. Start through laparotomy with multiple biopsies, or a leveling colostomy? Everybody can vote. So I'm gonna go back so you can see the contrast enema again. And we have 57% now we have 50/50. Now transano is getting more, so it's about split between transanal and laparoscopy with multiple biopsies. So I think it's what we mentioned already, whatever you are comfortable, I think with this good contrast enema and this beautiful transition zone. I would start transanally. So in this case, we did a trans-anal operation at 2 months of age, trans-anal only, and the patient did very well postoperative. So now let Doctor Frischer present another case. Can I say something about that. The um, Dr. Luis de la Torre already mentioned something about the preoperative preparation in this particular case that you presented. If it was a newborn baby that comes distended with that kind of symptoms, without study, I will give the baby nothing by mouth NPO. I would put a nasogastric tube. I would put a central line, give parenteral nutrition, and do irrigations and would not feed the baby anymore. That I will keep him with the irrigations until the abdomen is completely flat and we obtain basically bile through the irrigations. The baby will be ready for the operating room. In other words, if I, if it's so evident like in that case, I simply, I will not feed the baby or keep him in the hospital. And there are, there are, we were talking about cases that you could do laparoscopy or you could not do it, but in this particular case, I think laparoscopy will be overused. In other words, if that baby, I'm fairly, fairly comfortable to know that we can pull the rectum without touching the abdomen in either way. So that I don't see the need for that. OK, moving to a case presentation, a 36 week gestational baby who had not passed meconium for 3 days undergoes a suction rectal biopsy which was consistent with Hirschprung disease. Rectal irrigations were initiated with good results, and a contrast study was obtained, as demonstrated here. Is your AP version. And the post vac films. So with this history and contrast enema, what approach would you take? A trans anal swave or Swenson-like procedure, uh, start laparoscopically with multiple biopsies, start through laparotomy with multiple biopsies, or a leveling colostomy. And Dr. Krauss, while we're waiting, any comments on the images that you see? Anything striking? So is this the first one I don't think so. OK, go with the second. So, um, So this is interesting. So there's the rectosigmoid is a pretty good caliber, um, but it looks like the left colon is either spastic. Or it truly is much, much more narrow to the level of the splenic flexure, um, and there's a lot of meconium in there. And so, um, some people would say, well, maybe this is a small left colon, which is sort of in its natural history, potentially. Um. This is one of the more difficult cases, especially when the transition is at the sple of Fletcher. So this one could go either way, and I'm very careful about when, when I see these, it would be, it would have been nice to see, let's see the lateral again. Um, OK, so the lateral. It's one of these equivocal ones. I mean, you look at the rectum and the sigmoid, they're about equal, and then you see a small, I mean this could be a small left colon. Let's see your post vac. Your postt vac looks like it spontaneously evacuated. Um, which, you know, would go along with potentially more of a functional small left colon type or Maconian plug syndrome type case. So this is one of those ones which you probably should biopsy and see if it's Hirschsprung's or not. And we did, which came back consistent with Hirschprung's disease. And so what did the audience choose? Oh, we closed. Mark, can you put that last poll back up? thickness biopsy, rectal biopsy, suction, rectal biopsy suction. Well, this for us, I, I read this as a rectosigmoid. Transition zone with the possible I book all my cases if I'm doing a transanal approach with possible laparoscopy, but we were able to reach this transanally um and do a standard pull-through procedure and the patient did fine, right? So any questions? If we don't have any questions, we're going to move to another case scenario. This is a 3 weeks old female patient with a history of delayed meconium elimination after birth, 50 hours with a suppository. After discharge from the hospital, patient has 1 bowel movement per week, and she was referred by the pediatrician to our colorectal center. Upon physical examination, it was a healthy looking baby, normal anus. The patient is eating and growing well. So here is the cult film prior to the contrast enema, so just look very carefully. And here we're starting the contrast enema. And AP view now. Both Yvette. So do you think this contrast enema is suspicious for hisprung? Option 1, yes. Option 2, no, and everybody can vote. Very good. So we have 90% saying no. That's what I also agreed that it was not suspicious for for hitch prone. With this history and contrast enema, what would you do? Observation, rectal irrigation, suction rectal biopsy, full thickness rectal biopsy, or primary put through? So you look at the contrast enema, it doesn't look like his broom. Baby is doing well otherwise, but it's not having frequent bowel movements, so what would you do? and everybody can vote observation, rectal irrigation, suction rectal biopsy, full thickness rectal biopsy, or a primary put through and I think we have the majority of people saying observation and suction rectal biopsy. So we always, I think there is no downside in teaching the mother how to do rectal irrigation, even if it's not his brung, it just helps the patient in case you end up being hissh prune. So we taught the mother how to do rectal irrigation. And we told the mother to come back and she had all our contact information until you didn't completely rule out his broom, you want to have the patient very close to you, so she was instructed to come back if the patient didn't start stooling between irrigations, meaning that the symptoms persisted. So the patient did come back with the symptoms and then we did a suction rectal biopsy and we found that it was completely normal, not Hiprung. So those are the cases that you have to look at the mother and say, I don't know, but I know it's not Hiprung. Our belief is that we think this is an early manifestation of a child that will suffer from severe constipation. So in that case we just start managing constipation, small doses of laxatives or some digital stimulation, and we observe the patient over time. Do you have any comment? Any comments? So we're gonna move to, if you don't have any questions, we're gonna move to another case now. Doctor Pena will present the next case, yes. This is an 11 year old male patient with severe constipation and no history of enterocolitis. And this is the father was a surgeon, by the way. And he presented to me with this contrast study. And um We have been discussing about the technique of the contrast study in patients to rule out history disease, and I wanted to present this image because it's a A dramatic example of the importance of doing a good study. When they presented this to me, I said, let me, I want to see the entire study and I want to see the beginning and I want to see different positions because this looks like the dilatation is very low. But actually when you go for the for another film, you find this. I'm going back here. You see, it looks, it looks like the dilatation in this patient goes very low all the way down to this point. But actually what happens is there is an overimposed image. Doctor Krasa, you're looking at this image. Please don't get distracted. Come to the. Sure, sure, sure, so, so the, the, it's an overimposed because sometimes the, the colon is so largely redundant that it's in front that when you go lateral as Doctor Kraus suggested, then you find the the reality. This patient is one of those patients that is a Hirp disease, but I happen to believe that there are two types, two big types of Hip disease a benign type. Patients never have an episode of enterocolitis, and those are the patients that can go for for years and years with a colon like this with clinic classic image of a transition zone huge prunes and with no enterocolitis, and yet it's huge prunes. And the patients that have manifestations in during the newborn period, but for me, by definition belong to the bad group of patients that have a great tendency to suffer enterocolitis and even with a good operation, they sometimes have enterocolitis. And indeed in this patient I operated on him and the patient become completely asymptomatic, bowel controlled, very benign, and never had enterocolitis. So any questions about this case? If not, we're going to move to our last case. This is a very interesting and unusual case. That's why we're presenting. So this is a 2 year old female patient born with a rectal perineal fistula. She also had FOX2B mutation. Patent foramen ovale, patent ductus arteriosis, tricuspid, and pulmonary regurgitation. I'm sure everybody knows what is a Fox-2B mutation, but just in case there's a medical student in your room and He asks about it. The FOX2B gene provides instruction for making a protein that acts early in development to help promote the formation of nerve cells and regulate the process by which neurons mature to carry out specific functions. The protein is active in the neuro crest. Neuro crest cells migrate to form parts of the autonomic nervous system which controls body functions such as breathing. Pay attention because it comes the next question blood pressure, heart rate, and digestion. Now knowing. That that everybody already knew what diseases do you think are associated with Fox 2B mutation? Option 1 hemangioma, pulmonary hypertension, and Wilms tumor. Option 2, congenital central hypoventilation syndrome. Neuroblastoma and Hichbrun option 3, anorectal malformation, pre-sacral mass, and sudden infant death, or I don't know, so everybody can vote and we look forward to your answer. So we have a few people saying I don't know, we think it's a very good thing to say, I don't know if you truly don't know. And the majority of people paid attention in the definition of the FOX 2 gene, so they know that the answer is congenital central hypoventilation syndrome, neuroblastoma, and Hispru. The congenital central hypoventilation syndrome is associated with a myth, so I don't know if anyone knows about it. If you don't know, Dr. Pena will comment on that. Everybody knows that. It is the how do you say dine or y y din din and the dine, they call it the dine syndrome, and a lot of people repeat that, but if you go to to study to see what why it's called like that, it turns out that the ine myth is a water nymph who had an unfaithful mortal lover. He swore to her that his every waking breath would be a testimony of his love, and upon witnessing his adultery, she cursed that if he should fall asleep, he would forget to breathe. Eventually he fell asleep from sheer exhaustion, and his breathing stopped. So these are patients that stop breathing when they fall asleep sometimes. So those patients, they need tracheostomy and they need assisted ventilation during sleep. So the parents contacted our center due to the rare association of anorectal malformation and Hirschsprung's disease, and here is the abdominal X-ray film during the newborn period. And here is the contrast enema. So you can look carefully. Because I'm going to ask, where do you think is the transition zone? So by looking at this country's enema, do you consider it suspicious for hisroom? Yes or no? Everybody can vote. So I'm gonna show again while you can vote, do you consider this suspicious for Hisprung's disease? Yes or no. And we have 100% yes, very good. It is suspicious. Based on the contrast enema, where do you think is the transition zone located rectosigmoid, splenic flexure, hepatic flexure, ascending colon, or I don't know. Everybody can vote. And we have the majority of people saying split currently splenic flexure and rectal sigmoid, very good. So at 20 days of life this patient underwent an exploratory laparotomy with multiple colonic biopsies and a left transverse colostomy and mucous fistula. So we asked to review all the slides and here is a map of what what it was found. So the patient had, uh, here it was like a transition zone and where the mucous fistula was made there was ganglion cells, so. Hise-Brung was confirmed and here once you confirm that this patient has Hieprung's the surgery that we would need to do is put through the proximal stoma. Now the association of HR and anorectal malformation is a very bad association because every patient will be fecally incontinent. If the patient has anorectal malformation, this patient by definition has no anal canal. And we are going to resect due through the due to the presence of Hirprung disease, we are for sure going to resect the natural reservoir, the rectal sigmoid. So those patients will be fecally incontinent, and it's very important to discuss to the parents this prior to the operation. So here is the operation. We start with a posterior sagittal approach. We took biopsies to confirm what we already knew. So the proximal colostomy was pulled down. All the distal segment, mucous fistula down was a ganglionic. We also performed the Malone procedure because we knew this patient was going to be fecally incontinent, and the patient is currently clean on bowel management. So this is an extremely rare scenario, but it's just an interesting patient, those rare associations. That's why we decided to present. I think now we're going to take a 20 minute break for lunch and we'll be back with Dr. Rintala discussing the long term outcomes in hisprung and total colonic ganglionosis, and I think that Dr. Kraus wants to comment. One thing, there was a there was a question that I wanted to comment on because it's kind of too long to type, and the question was by Dr. Ortiz, and he asked, in the post evacuation colon, what percent is normal? And I wanted to make a comment. I don't for Hirschsprung patients, I don't rely on the post evacuation film to tell me whether it is Hirschsprung's or not. However, I can say that in patients who do have Hirschsprung's, there is delayed evacuation. In patients that have equivocal findings, I also don't use it because sometimes the Hirschprung segment is spastic and it just expels the contrast. And sometimes they say you may even get a huge expulsion, even clinically in some patients, especially when you do a rectal exam. So I don't use that in patients with chronic constipation. That's a different story, and we do look for evacuation and usually about half or so, if you can tell of the contrast coming out, it's usually. You know, not a percentage that's based on a measurement. It's a gestalt. So if that answers your question. I, I appreciate that. So we're gonna take a break and we're sharply. We'll see you soon. --- Not medical advice · citation policy: https://library.globalcastmd.com/ai