Total Colonic Hirschsprung Disease with Malrotation: Difficult Cases

Yeah, good afternoon, Todd and other members of the faculty. I am going to present a very rare case of multiple GI anomaly. Next month. I put that on baby. Present with delayed passage of meconium of more than 48 hours, but after doing this examination, he passed meconium. Um-hum. And we keep the baby in the hospital for observation. At day 7 of his age, he presents with signs and symptoms of, uh, Hirsch disease, including abdominal distension, tight rectum with passage of explosive stool after removing the examining finger. For, uh, full thickness sector biopsy confirmed the absence of ganglion cell. Next slide please. We performed parrotomy in order to create a transverse colostomy, but on exploration we observed the following finding. We found mal rotation with multiple bands. Really it was there were 3 bands, one band between the loop of bone and the other band between the bowel and the liver. And the third one was between the bowel and abdominal wall. There were collapsed colon, anterior ileum with typical corn segment. Looks like this. During the surgery you perform that procedure, we release all bands and we create dystony just as a conciment. We take biopsy from the appendix 1al ileum, which proved to be touch a lung disease. Postparatively we give IV fluid antibiotic with a total nutrition and after, uh, bowel mission become positive, we start on a special formula from Newsure which is a high calorie formula with the addition of vitamin B12. Next slide please. Firstly, the infant developed multiple attacks of dehydration and, uh, required hospital admission of IV replacement. The infant on the day of, uh, submission of my presentation, he was 70 days, but now he is, uh, 4 months. Uh, his weight was 3.5 kg. Next slide. My consultation to Global. What is the best feeding regime for such infant because he is gaining weight slowly and what is the best definitive procedure? It is best to perform Martin procedure which prepared by some surgeon or to perform red wine or bully or to perform mosis with creation of ileal pouch. And what is the risk of incontinence? And what's the best time to perform the definitive procedure? It's related to the weight or the age? All right. Next slide. OK. Let's, let's open that up to the, uh. Yeah, regarding, uh. Go, go, go ahead, Doctor Jafar, sorry. Yeah. Regarding review of article for, uh, such similar, uh, conditions, only a few cases reported to have total chronic assciated with maltation. Philone has had 4 patients and uh have 1 patient and Zbra reported 3 patients by me, but no, no cases reported to have all 3 anomalies. Next slide please. Regarding the congenital bonds which present in this case it's rare and only a few cases reported to have congenital bond is unknown could be attributed to antenatal perforation of the bowel or finding. Uh, as far as I know, it's the first reported cases. Next slide please. This picture show bands which I removed between the loop of bowel, small bowel. The next, next slide, please. And this picture shows a band between the abdominal wall and the wrong one next slide. And this picture shows spread duodenum between two forces for trade in the absence of fixation. Next slide and uh just show configment in the terminal ileum of collapsed terminal ileum and colon until we create ostomy. As you see in this picture, we created this to me at the left upper quadrant because uh yeah the same area was in this place. All right. And thank you. Thank you very much. So, uh, tough case. Uh, so mal rotation and Hirschprung's, uh, how would you approach it? Well, I've, I've seen this a few times. Um, the usual scenario though is a child with bilious vomiting who gets an initial, uh, contrast study that shows the malrotation. So you go, you do a lab procedure and congratulate yourself. You saved the baby's life, and then the baby at that point doesn't open up and you have to go back and rethink, you know, what, what, why is the baby not opening up, and then it turns out to be total colonics. We've had a, a few kids like that, um, and that's one teaching point is that, um, once you've done a lab procedure, if they don't open up, you got to think about other potential causes for the, for the bilious vomiting. It's a great point. Um, the, uh, they're usually short segments. They're not usually total colonic. No, you're correct. Just I think it's probably in the same, the same distribution as Hirschsprung's in general, but, um, you know, we have seen a few kids with total colonic, and it's, I mean, it's the same teaching point, right? If they don't open up after the lab procedure, then you have to think, you know, what else could be causing this, uh, persistent obstruction. Um, I, I just wanted to also address the question of what operation to do for total clonic Hirschprung's and when to do it. Um, I'm sure there are lots of different opinions about that, but my, my operation is that I prefer is uh Duhamel, just a straightforward Duhamel. Um, it's simple. It's safe. Um, it does provide a bit of a reservoir at the bottom, which, uh, suave does not. Um, and I don't base the timing on either age or weight. I base it on the consistency of the ileostomy output. Um, I think they don't do very well if you do it too early when the ileostomy output is still very liquidy. I like to wait until it firms up, which usually happens when they get onto solid food. Um, and that's, that's the determining factor for me. Yeah, I, I would ask you too though, I, the Duomel is. What I've been taught and used for the long segment or total colonic, um. There was obviously having a longer segment of. Colon left and I think most of us have retreated to a relatively short piece. You're almost making a small reservoir, and I think that's uh, to me is an important piece. They no longer the long kind of element, uh, the, the, the Martin modification element. Really, it's a short piece of colon that should be there. But we should remember when you look at the data, you know, that good control in long segment Hirschprung's disease is really about 50% of the population of the people that get it done. It's the data really isn't that great. So you gotta make sure you paint the right picture to people that, that, you know, having good control going forward with the long segment. There's been a lot of discussion with the concept of long segment. Is it just, you know, total colonic or if you involve small bowel? And many people feel as you start to get, say, more than 50 or so from the, uh, ileocecal valve, it's a much more progressive disease. It makes sense. It's a more global disease. They all have a bigger dysmotility, uh, element to them. And again, you can't look for the, the simple fix of our classic operations that are gonna do the trick for them. Yeah, any comments, um, from the faculty? I was, Yeah, I, uh, I think so. Sharif, why don't you go first. Um, yeah, I, I've had a chance to look at this a few times every, every time one of these comes up, and there's been a lot of meta-analysis and reviews trying to figure out what's the best, and I think I'm convinced that the best is still what you do best. In other words, I don't think it's because long segment that Dujamel is better than a suave, although, yes, intrinsically it would sort of make sense to have a reservoir, but there also may be higher episodes of enterocolitis and other things, so. I, I don't think there's any evidence in the literature, and if there is, I'd love to see it, but I just don't think there's any evidence that any particular procedure really should be done just because the patient has long segments. So I think whatever you have had the best results with is what you should still stick with, and, and certainly my experience is very much emphasize that. So that's, in terms of that, um, that's how I would make the decision, but I, I think an important point here that we should, we should, um, Just, uh, discussed very briefly before ending is why this baby is not gaining weight, even though the, the, the, the, uh, uh, ileostomy seems quite distal, and the rest of the bowel is normal. And I think this is something that we all sometimes struggle with in a baby who's getting all the adequate calories, a blood, uh, test seem normal. And many times it has to do with sodium loss. And unless you check the sodium levels and the effluent, you will not catch that because the serum sodium will be normal for many months before it starts to decrease. But if you're getting more than 5 to 7 mL equivalents per liter of sodium out of the ileostomy, that baby will not grow. It just will not gain weight. And I think whatever procedure you're going to do, you've got to get the baby gaining weight and growing before you even get there. I think actually measuring the urinary sodium is uh is the best way to um to guide how much sodium replacement you're giving, but I mean some people say, and I think it's probably a good idea that every baby with an ileostomy should get sodium supplementation. Hmm. The other thing too is the iron deficiency in these kids though going forward is uh something that gets lost and translate not immediate at this time, you know, they're really young, but long time it's a big issue with any repairs that you end up doing that, that's forgotten. So, uh, yeah, I just a few comments um regarding uh the operation. I don't do Duhamel because I do most of my cases Suave, and I'm having a good result with that, but I think in the total colonic fishprung disease, if you decide to do this, you have to. Wait until the baby grows, until the ileostomy is a bit thicker. And then if you decide to do a wave or other procedure, you can just continue with some um uh bulking agents or um uh something like anti um Uh, diarrheal agents that can help the patients. Uh, we had a few patients that went abroad and did Duamel and actually came back with a lot of problems, enterocolitis and obstruction and distension of this Duamel. So I don't know how much Duhamel you have to do so that in the future that doesn't have any problems. Um, so anyway, um, I mean the segment or the length of the Duhamel. Uh, so I think that the most important thing is that the baby should monitor, as you said, the ileostomy output before deciding to do any procedure because we've seen patients also that had the ileoanal anastomosis and then came up with a very, very, um, uh, you know, the bottom like was very, very bad, uh, erosive, and it was um. And not not healthy, so we had to do an ileostomy to protect his anus and treat it before we do any other procedures. So I think it's better to manage these patients in the long term rather than just at this point. Very good. Uh, OK, but maybe, yeah, but maybe rebind procedure or, uh, may be better, uh, for the continence because we know it's lower, uh, anastomosis and there may be at more risk of incontinence. We do a swab for the regular Hebung disease patients, not the total colonic, and we don't see an incontinence with these patients. I think it's just you follow the the the the the the procedure very well. You should not have a damaged sphincters. I don't know. You are right, but I mean for total economic against the officially we have, uh, already, uh, again the term so we will excite the junction.