Malrotation Rapid Fire Session: Update Course 2015

It's going to be malrotation and as you can see from this slide, probably the most controversial topic in malrotation is going to be what to do with with heterotaxy syndrome and the rotational defect that goes along with that. There's really no controversy if someone has a volvulus. I mean, you either operate or you get a new job. As far as what to do in this particular situation, uh, I'll be interested to see what all our viewers, uh, would do. So, uh, this is a case that presented to us about 6 months ago. Um, a 6-year-old female, um, uh, with a known history of heteroachy syndrome born outside of this country. Um, you can see that the uh congenital cardiac history includes, uh, single ventricle anatomy, tricuspid atresia. Um, and a variety of other things I won't read to you. Um, she was, uh, our initial operation was at 2 weeks of age. Uh, she essentially, uh, was referred to our cardiac, uh, uh, service, uh, for a completion operation. Um, and, uh, upon pre-op evaluation, uh, was noted to be, uh, very low on her growth curve, uh, was classified as failure to thrive, uh, upon detailed, uh, questioning, really no history of any bilious. Vomiting, no blood in the stool, and no significant abdominal pain per se, just some intermittent spitting up over the years and failure to thrive. So an upper GI was obtained given this history, and what what you can see here is the stomach is on the wrong side and you get an impression that the duodenum and does not make a C loop and So we've got the sitis in versus and a suspicion of malrotation. Uh, they followed this through with the small bowel follow-through, and, and this is pretty interesting. Um, so you can see everything is reversed, it's a, uh, it's a situ in versus, uh, uh, in the abdomen. You can see the cecum is in the right lower quadrant, appendix is down there, uh, and the duodenum makes a straight shot down the left side with all the small bowel on the left side. So, at this point in time, you're really stuck with, um, You know, what are you going to do if you go in and do an operation in the past, most people would just wind up doing an appendectomy, and that's it, because you really cannot widen the root of the mesentery any further than this. So I'd be interested to know how, what people would do at this point. Yeah, let's go ahead, Mark. Does the child have right atrial isomerism or left atrial isomerism? Right. So, in right atrial isomerism, at least in our data, it suggested that there's a higher incidence of malrotation. Going, you know, the other thing to weigh with this, this kid's gone 6 years without a problem. So I think there are some people that would just watch this. On the other hand, if she has any GI symptoms at all, you can always put a laparoscope in and assess how wide the mesentery is. And if it's a wide broad based mesentery, I don't think you can tell from this study, even if the cecum is on one side and the ligament trites is on the other. I think it can be very deceptive with everything floating around in there, but just look and see if it's a narrow base necessary because he's symptomatic now, because he's vomiting. If there if there's a symptom, but I'll tell you, so in our, in our institution based on our data, based on our paper, if there is a child with Right atrial isomerism. We will put a scope in and and look and see because those were the kids that got volvulus. Volvulus, we had only one patient that had any real issues that had left atrial isomerism and it wasn't a true volvulus. But I think you're also dealing with the problem that that that may be confused by the the cardiac issues, but the child is small and is not apparently not thriving. And I think in in you have to be able to sort out that there's not a component of some element of the lads bands causing this as well, and I think that I would put a scope in for no other reason than to, you know, divide in the lad's bands. If anything could be causing that type of issue, take out the appendix. I think you have to do that when the child has some symptoms related to the GI tract, and those, to my mind, include failure to thrive. I, I agree. All right. So very, very interesting, um, all your opinions are, are of course, uh, correct, cause there are no right or wrong answers here. Um, there's been a lot of literature written over the past few years, and I just thought that I would, uh, uh, put up, uh, the hottest one off the press, soon to be published. So Texas Children's, um, presented this at the CAPS meeting about 3 weeks ago. So 95 consecutive patients. With heterotaxy and malrotation, three quarters of them underwent a labs procedure, and a long-term follow-up revealed that none of them volvulized post-op, but they had an 11% small bowel obstruction rate requiring admission and in many cases surgery. 25% of that group got observed with no small bowel obstruction and no volvulus to date, and they're granted, they didn't follow these patients. 80 or 90 years, but we're talking about 10 or 15 year follow-up in many cases. So it's just interesting to note what people are doing out there. I don't think there's a right or wrong thing. Um, uh, I think you do have to be sure of your assessment and, and putting in a scope is, is what I have done in the past. I'm not sure if that's the right or wrong thing. I'll be interested to those of you who put in a scope, do you take the appendix out at that time? I do. So you know, we all know that an appendectomy is not without its obstruction rate in the long term from adhesions. So it's just important to note that you are buying a slight complication with that. I don't take out the appendix. The appendix is in the correct location. If it wasn't the correct, if it's not in the correct location, I think it's Ken's part. The appendix is in the correct location and he's malrotated, so it's a double negative. So his appendix is in the correct location. So, Kenny, that cecum is going to be floppy for this, for this, and I do an inversion appendectomy. OK, so, Kenny, for the sake of time, because we wanted these rapid fires to be 5 minutes each. The high yield point for this is heterotaxs. What I think you're saying, I mean, situs inversus, basically if they're asymptomatic. It sounds like most people here would not do anything if they're symptomatic, put in a laparoscope. You only do that if they're right. Well, I would phrase Todd, Todd, let me just rephrase. I mean, if they're symptomatic, I don't think anybody would just observe and do nothing. And I mean by symptoms, they're having significant pain, vomiting, maybe bilious, bloody stools. I mean that you have to operate on. We're talking about the patient with minimal symptoms, reflux, little spitting up, little reflux, failure to thrive, that, which is the most common patient we see with this actually. Right. OK. All right, ready to move on. Next, next one. We're going to talk about one specific situation with with Maconi Emilius. Kenny, I want to stop you. Sorry, before we go off Malro, before we go off Malro, rapid fire. You have a kid that you're working up for malrotation. They have a C loop that crosses midline, not heterotaxy, normal kid. They have a C loop that crosses midline, but it's a low lying ligament of trites. What do you do? I usually put a scope in and take a look. You put in a scope. What do you do? Scope, scope, scope, scope. I don't do anything, and now I'm going back and forth because we had a big discussion. I may start scoping again, but I did all my scopes have all been negative. Just, just keep in mind one thing on that case you just presented. Yes, it's the low lying ligamented trites that defines malrotation, not something crossing the midline, because something can be floppy when it crosses the midline, and that does not exclude a malrotation. How many patients have you seen that have had a great C loop that crosses midline? I know everyone here is going to say they've seen one. That crossed midline and had a low lying ligaments and you went in and they were mal rotated too. So I know Tony's, I know Tony's in your audience there in fellowship we saw half a dozen of those, so I've probably seen another 3 or 4 since then. Wow, I've seen that. OK. Anyone do anything different than, so everyone would everyone here put in a scope. I wouldn't scope him. OK, I would just watch. OK, we would just watch. Uh, Dan, you would watch. OK. So we're split here. So we had a baby a few years ago who had a low lying ligament trite. It didn't look like anything. We watched and the baby came back with a volvulus. We, we were trying to do a combined study. We wanted to do a multi-center. We talked to Sean about your, we know there was actually 22, yeah, so, um, but, so I may be convinced now. All right, sorry, go ahead, Ken. No problem. Let's, we