Speaker: Dr. Mark Wulkan
Thanks, Todd. Uh, great discussion that we're, that we're having here. So I'm gonna go over just some things about technique. I'm gonna try to do it pretty quickly and maybe I can get us caught up a little bit and then I really wanna dive into some of the cases that we're gonna be talking about, uh, you know, so I'm gonna say what's the indication for a minimally invasive, uh, congenital diaphragmatic hernia repair? The statement in and of itself might be a little bit controversial. For me it's, it's uh any patient that can be transported to the OR and that is stable. We generally, you know, this is another part of the discussion that we can get into is when do you fix a congenital diaphragmatic hernia in our institution, uh, we like to wait until at least the right sided pressures are lower than systemic pressures, and I think, I think most people would do that. Is that, is that the case? Everybody wait for that? Getting lots of nods. OK, good. So, uh, you know, what are the contraindications? So in our institution we don't fix patients on ECMO, and there is a recent study, and we'll get to that, that shows that, uh, you know, maybe, uh, if you wait till after they're off ECMO that your survival rates will be better. And then what's not a contraindication liver up, stomach up, uh, you know, oscillator if they're on the oscillator, I guess you have to ask why, but that wouldn't be a contraindication. Necessarily there's some cases that we do deliberately on the oscillator because it makes the MIS easier and then redo is also something that Todd wanted me to talk about because that is also not, it's not studied but it's not a contraindication you can do redos. There, so. You know, some technical pearls that I want to go over because I, I know there is controversy about whether even primary MIS repair is inferior to open repair, and there are the, the first study was the study out of Babies's Hospital, I think it was back when Charlie Stoller was still there, and they, they showed a relatively high recurrence rate for primary repair, and there's been some studies that have shown. A higher recurrence rate for primary repair of congenital diaphragmatic hernia, but I'm going to say that some of this may be related to technique or how we do it. And then even if it's slightly higher, there might be another advantage of the repair, especially if you're going thoracoscopically, and that is reducing the lifelong risk of bowel obstructions and operations for small bowel obstruction throughout the lifetime of the patient. A laparotomy has significant morbidity, and it's something that I think that we don't really think about and is relatively underestimated. We talk about all these advantages of MS. It may be that one of the biggest advantages of MIS is saving these kids from lifelong risk of small bowel obstruction. So some of the technical pearls that we're going to talk about are using pericostal sutures, mesh reinforcement, uh, you know, and then you know the flip side of all this, I'm talking about MIS is if you go in there there's agenesis or near agenesis of the diaphragm, I mean, can you accomplish an MIS repair? Yes, it's an extremely long run for a short slide. In those cases you just convert to open and uh you want to in a way cut your losses. Other considerations again, uh, you'll talk about recurrent hernias, and we're going to skip over more gagney for now. We'll see what. So this is generally where we put the, put the ports. We position the baby so that you're standing over the patient's head, they're sideways on the table and you basically have triangulated ports with your camera, uh, just, you know, just off the tip of the scapula, but maybe a little bit leaning towards the mid axillary line. And you know, here, here's some of the views you see, and I, I think I'm probably at the edge of the distance of this thing. And again, I'm gonna try to go through these quickly. Yeah, keep, keep going. And, and you know, one of the tech one of the things that you'll see here is, you know, a lot of times once you get everything in and you, you push the spleen down, it's a nice cap, and this is a nice primary repair. One of the things that you'll see, I think that we tend to put in a lot of stitches. Uh, you know, and you can't be afraid to tie or feel that one more stitch, oh my gosh, you know, so it's going to take me another 15 minutes to throw a suture. So I think that, that, you know, if, if, if that is an impediment to doing a good repair, then, and you can do a better repair open, I think it is good to do it open. Uh, sometimes you can do a thoracoscopic assisted repair where even you make just a little thoracotomy just above the diaphragm and you can get in there and put some stitches in and open if you're having trouble with them, uh, thoracoscopically. Let's go to the next slide. OK, um, the other thing to consider after the repair is that all these kids have pulmonary agenesis, and, you know, one of the things, you know, I like to keep, we can talk about whether to use chest tubes or not, and I actually didn't make a poll question on that, but I think that would be a good poll question, Mark, is do you leave a chest tube after congenital diaphragmatic hernia repair? I'll do it, um, uh, yeah, is. Yeah, there is going to be an obligate pneumothorax in most of these patients, and if somebody's sucking on that, does that put undue pressure on your repair and aside from shifting the mediastinum and probably causing some some barotrauma to the lung. Let's go to the next slide. So You know, another piece is, uh, you know, this is the right-sided, uh, congenital diaphragmatic hernia repair. Uh, you know, you, you, you want to reduce the bowel, push it in front of the liver, get the liver down, and I'm going to show you the see-saw pericostal sutures that you can use. So let's go to the next slide. So here's actually another left side. Let's just keep going through these pretty quickly. But this was an interesting case where the diaphragm was actually really folded up and there was very little anterior diaphragm. And very little to sew to on the edge. So we use multiple little pericostal stitches around the patient's, uh, uh, around the pericostal margin. And let's go to the next slide. Um, just one thing to avoid is you don't wanna, you don't want a flat diaphragm there, uh, but here, let me show you these pericostal stitches. Let's go ahead and run that video. So you basically come in. This is a different case. Uh, and this is a piece of mesh. I'll, I'll, I'll mention this in a second. This is a biologic mesh. If you use a biologic mesh like I used it here, you will have a higher recurrence rate. And when we saw that, we changed, and I'm going to show you what we changed to. But you bring the suture in through a little stab wound, tiny little 11 blade stab wound, and you come around the mesh or you can come around the diaphragm. It can be that corner stitch in a primary repair. Let's go to the next slide and see how we manage it on the outside. It's just showing it again. Let's go to the next one. So you use the seesaw method where you push the stitch out. It doesn't, you don't have to push it out right exactly where you put it in. You can bring the tip of the needle out without bringing the heel of the needle and then back the needle back through the hole that you made. Uh, and actually I, I stole this from, uh, not the video but this technique, uh, I stole from one of my, our former fellows in Atlanta, and that's Han Min Lee. Who's I think most of you know in San Francisco. You basically bring it out through that little hole and you tie it down. And what was interesting is in the patient where we unfurled the diaphragm across all of this, there were about, I think we had maybe 4 of these little holes on the side and I'm thinking that the kid looked like he was, you know, shot with a BB gun or something. When I saw him at 1 year and then down the line from that, uh, it actually all faded away. Let's go to the next slide. So again, here's what you're doing is with that case coming down through the rib and out. Let's go on next slide. Yeah Uh, the other thing that we do a lot of, and, uh, you know, is the underlay mesh and a lot of our primary repairs, if there's a couple things. One is some things we maybe didn't do before. I do think it's important to probably rough up the edges a little bit. We use silk or something that's going to cause a little bit of inflammation. So that it sticks, you don't want to just put two nice peritoneal surfaces together. And the other thing is that we use very liberal any tension whatsoever, uh, use of an underlay biologic mesh. We're gonna go over that. I'll think, uh, actually several of these slides are from, uh, Matt Clifton that you heard from before. So Matt, I hope you don't mind. I stole your slides. Uh, one of the things, this is just a picture from a redo repair, is, uh, one of the, there's some tricks for pulling the mesh into the chest, and one of the, instead of trying to push it in with your, uh, with your trope, you know, with your instrument is to pull it in. So you run the instrument out and you can see here in this video if you want to go ahead and click that. And you run it out the other trocar you grab it, you just sort of roll it up and you grab it and pull it in and that is tremendously easier than doing this, but then trying to push it in and you can use that same technique in the abdomen. I like the pull on the mesh. Let's go to the next thing. So here again is a is a patch repair and we can probably skip this video, but one of the things that that I'm showing here is that. You know, if you want to measure your patch, so this is controversial, so I guess poll question, who would do a patch repair thoracoscopically? Um, you know, you need to measure how big your patch is, and if you know how wide your jaws are of your instrument, then you can just sort of use that to gauge, uh, to measure the size of the, uh, defect. So let's just, uh, let's just, we'll skip the patch repair video, but this is one of the things that we've done. Remember I talked about biologics. We went through this phase where. You know, gosh, biologics, next best thing, you know, the PTFE would always pull off the chest wall. We saw recurrences. Why not put something there that's going to remodel into diaphragm? Uh, we actually, you know, we actually studied this in a, in a pig model. Just so happens it was pig biologics. You know that's one of those, you know, problems with my study that we didn't think of up front and afterwards was painfully obvious, but it did, you know, those nice rowth. There wasn't a lot of scar tissue and you know you even saw some muscle, muscle fibers under the microscope. But in real life in humans, when you use the biologics, a lot of times the biologics will actually dissolve in the middle because you don't get ingrowth the way you need to. The other thing is that biologics work really well. When you have tissue on either side of them, so you remember that obligate pneumothorax. There's not good tissue apposition on the chest side. You have good tissue position on the abdominal side where you can have mesothelial cells populate that, but not on the other side. So it doesn't populate as fast as you need, and that thing's gone in a few months and you end up with a little hole in the middle because it appears that most of the ingrowth occurs from the sides towards the center. Well, the problem with biologics is we're seeing recurrences in the dome of the diaphragm, if you will, and then with PTFE or solid mesh, we were seeing recurrences where it pulls out laterally. So we came up with the uh with the sandwich, uh, with uh PTFE on the lung side and the biologic on the abdominal side. And you want a biologic underlay and then we sew the PTFE on top. Let's go to the next slide. And uh here is uh so let's go just skip over this one we can go we can go through this quickly just skip over that. And, and here's basically what it looks like. So that the first slide was so we put the biologic underlay, we pull it through. You have that there. There's still a little defect laterally, and we put a nice little piece of PTFE there. And so that's how we've been doing it, uh, when we. Looked at and I have some slides at the end of this which we probably will not get to looking at our experience over the last, it's probably about 1213 years now and when we look at a multivariate analysis of our experiences, one of the one of the risk factors, one of the biggest risk factors for recurrence was use of just a biologic mesh. When we've gone to this, we've seen a recurrence rate for patch repairs. While it's it's still there just like everybody else's, it's significantly lower. Just, uh, technically, so just to make sure I understand, you first stitch in the biologic, yeah, so go back to that, uh, other slide there, uh, go back again. I'm sorry. So what, what we did, so that biologic is, is, is actually it was pulled underneath the incision. Uh, underneath the diaphragm repair so that it's actually out around beyond you're not getting the edges of it. No, it's actually laid out beyond even the primary repair and when you come to sewing it to the sides. I'm actually not sewing the side, the edge of the biologic to the edge of the diaphragm. I'm actually coming over it. I'm taking a bite of the diaphragm and then taking a bite of the mesh, actually 1 centimeter or 2 away from, you know, away from the edge so that I have a centimeter or 2 of over underlay on the incision. And then on the next slide you sew the patch to the edge of the diaphragm. Any, any other questions about that? And, and I think it, it works nicely. Again, I don't think there's any technique with some of these patients, and we're gonna get to one at the end, uh, that works 100% of the time. But, so let's go on. So I can keep going, um. ECMO, I just wanted to show, so another controversial point, do you do, so who would do an MIS repair? Another poll question, who would do an MIS repair after a patient has been on ECMO, not on ECMO, but has been on ECMO. And, and we do. I think it's worth putting a scope in everybody, and the reason for that again, I think that if you just say you're going to do an open repair, then you're going to end up, and a lot of us do it through the belly, you're going to end up with downstream bowel obstructions that you could otherwise prevent. So let's go to the next slide. Uh, this is just an example of, uh, an agenesis of the diaphragm. There's a little bit of a, a sac there. You take that sack off, but it's like if you go in with a scope and you see this, don't spend 4 hours trying to figure this out or anything, then you just go ahead and convert to an open repair. So next slide, uh, this is a nice Gore-Tex SIS domed patch in, in that patient that was done open, it's on the right side. So again, technical points open if a large defect. Don't use biologic mesh alone to bridge a gap. Don't put too much tension on your repair. I think one of the other technical pearls when we're doing an MIS diaphragmatic hernia repair is that we tend to underestimate the amount of tension we're putting on the diaphragm. You want to see a domed diaphragm, so you do want to have liberal use of a patch if you need it. And then the other piece is to put enough sutures. So, let's go on to the next slide. So first case. So you can see here this is a basically a term baby uh moderate respiratory distress, very minimal support. echo shows that the pulmonary pressures are mildly elevated but not systemic, so. So I'm gonna poll the audience locally and so we should be pulling these folks, uh, everywhere, but I just wanna see a raise of hands. So who would do this as soon as there's, you know, next time you can schedule it next open OR. Nobody. OK. So you don't have my choices up there anymore, Mark, so I can't see my, uh, see my thing. OK, but you can show that. I can go, I can go to my cheat sheet here, make sure I say them in the right order. So who would wait two days? You can show the hands now. I got it. So who would wait 2 days? Who would wait 4 days? Who would wait a week? Is nobody fixing this kid? Nobody's raising their hand. So what would you do? What do you do, Matt? What do you do? Yeah, well, I mean, I think it depends. I'd, I'd give the kid, if that's the newborn film when they're first born, yeah, I'd give them 48 hours to make sure that they didn't develop severe pulmonary hypertension in that interim. But if after 2 days they're still looking fine, I think it's OK to go. If they've developed a little mild pulmonary hypertension in the interim, then I'd wait it out until they're. They were not super systemic. So Matt Harmon, are you back there? We haven't heard from you yet today, have we? OK. Anybody gonna wait longer? So yeah, assuming everything else is OK in South America, you do the same thing a couple of days. Yeah, OK. How long? 7. 47 days. Jorge. OK, Mark, this is Mark, this is Mac. I had a, a faculty member at CHOP when I was in training who said that he, he liked to fix these the day before discharge. Uh, so he was doing MIS repairs then? So, so, uh, for in Santiago, if they're stable, you still wait 4 to 7 days, 2 or 3 days. OK, so Mark, not, not to badge you about the question, but the days really aren't really as important as like Matt said, it's like maybe change in pressure over change in time. We're, we're looking at the physiology of the pulmonary hypertension, so we don't end up having to act more someone for hypertension. So I, I guess what everybody's getting at, I think it sounds like everybody would wait some period of time to make sure that the kid doesn't develop pulmonary hypertension, and that's, yeah, I, I think that period of time is getting shorter and shorter, but I think those of us with more gray hair I've always seen the kid who came in, looks great, sort of has the typical honeymoon period that for some reason we don't seem to see quite as often now as we used to, but that they look good and then, you know, I've seen kids that look great and then. You know, in a day or two they're on ECMO, but this patient was somebody who just came in, stayed stable that whole time, uh, and if you did an echo every day you'd find that the pulmonary pressures were mildly elevated. Um, is there anyone that would go less than 2 days that would just say, you know what, this is a stable patient, let's just fix it. Nobody, OK, so is there an advantage of repairing them before they get to that unstable period? Well, you know, it's funny because that's what that, that was, that was what people used to say, but I don't, but you throw them, you may be throwing them over the edge, you know, the physiologic defect, you know, we say in the South it's not the chits in the chest, right? So it's not the, it's not the intestines in the chest. For those of you who don't know, chits or chitlins, which are intestines. Some of us say that try to translate, not all of us say that translate for the Yankees, um. But if you, uh, you know, that's not the physiologic problem. The physiologic problems all pulmonary and so that's not, you know, so we can argue about whether, not that it would make them better, but the safest time to do the repair, which is an elective repair if you're saying I'm just trying to be devil's advocate since no one answered this, uh, could you, could you argue that that fixing them. The a safe time to fix them is before they've gotten any severe pulmonary hypertension. You could, or you could argue that the safe time to fix them is after their pulmonary hypertension is over. But this is, yeah, OK. So let's go, let's go to the, uh, the next slide. It's just gonna say, how do you repair this. And thoracotomy, laparotomy, thoracoscopy, laparoscopy. So by the way, do you have the, do you have those things on your, oh, we're waiting for all these people to answer. Let's look at some of the results of the things you've asked. We've asked a lot of questions. Yeah. So first of all, almost completely split on do you leave a chest tube? Um, I, uh, do leave a chest tube. Um, would you do a patch repair thoracoscopically? 60%, this, see, I wonder if this is a skewed audience, a skewed, uh, group, but 63% say they would do a thoracoscopic patch repair. I do not think that's representative of the country or the world. Uh, would you do a minimally invasive repair after the patient is taken off of ECMO? 60% said yes. Um. And then when, when do you elect to repair 62%, especially after Clifton said 48 hours agreed with him, 62%. And then now we're getting the results of how would you do the repair and the majority of the audience, 67% say thoracoscopically. So you, you think we have a, you, you think we have a skewed audience here. OK, OK, fair enough, fair enough. So let's go to the, the next case. So this is a, uh, 35 week gestational age baby, so. Little bit, little bit early, not too bad, but has a high ventilator settings. Uh, OI of 45 is on dopamine of 14, brought to the NICU, and has super systemic pulmonary pressure. So let's ask some questions about this patient, see what people will do with this next steps. Next, next slide. So who would, who would sort of ride, you know, so they just arrived in your NICU. Um, and, uh, so who, who would, who would just say this patient needs to go on ECMO, put them on now, or whose neonatologist will, you know, maybe futz around with them. So we'll see what, what we say on this one. So repeat echo in 6 hours to see how they're doing. Uh, put them on VV ECMO, VA ECMO, a trial of inhaled nitric oxide before you go to ECMO, see if that, see if that works. And so see what people are saying that, who, who is involved in this decision making at their hospital and who is. So that's a whole another thing. Is it the pediatric surgeon's decision? Is the neonatologist's decision? Is it a joint decision? So that's, that'd be something else we can, we can talk about if we have time. So in, in this room, uh, who, who would, uh, who would just say, OK, this baby needs to crash on ECMO? OI 45 on, you know, maybe on the oscillator. I think the original criteria is we're supposed to separate the OIs by a couple of hours to have repeat events and so the, the question was set up a little bit. I know you're not being deceptive, but you can only put so much in the question, but we're, we're supposed to have repeated evidence that, that the OI is really that high. Correct. So I mean, I think most of us, uh, I, I think most places, you know, we probably wait and, you know, first of all, the patient just came from, you know, from an ambulance unless they're inborn. I think if it's inborn baby, it may be a little bit different because you've managed them from the start. Uh, most of our kids come from other hospitals. We have, we don't have a maternity ward. So if it's a patient that's coming off an ambulance, you, you know, we'd futz with the baby. Our neonatologist would fuss with the baby, uh, but then if, if those, if those things persisted, we would go on ECMO. So let's go to the next slide. So let's just say that we went on ECMO. Are we gonna go? Are we working now? Yeah, well, the receivers are here now. Ah, OK. So I'll bring it closer. That's good. So VA or VV ECMO for a congenital, congenital diaphragmatic hernia. Who here would put a CDH on VV ECMO? Who would put them on VA ECMO? It's because it's the diaphragm. Let's see what Jason, were you, was your hand up there? I didn't our institution puts them on VAM. That's right. You're, you're the, uh, you're, you're not the, yeah, you're not the right congenital diaphragmatic hernia doctor. You're the. OK. Um, so let's go, let's go to the next slide. Uh, in our institution we would, we would do VV ACMO and um. You know, it's interesting. So there, there is some data and there's some evidence around this. So you can do, everybody worries about the shift of the mediastinum this that and another. We've been doing VV ECMO on diaphragms at least since I got to Atlanta when I was in Birmingham as a fellow. We were doing it there as well. And I'm pretty sure that uh they do the Michigan group also does a lot of VV ECMO, but this has been uh written up and published and and I know this slide has a lot of words on it so you so Kurt, if you wanna criticize my slides too, that's, that's OK uh. But you know, basically overall outcomes may favor VVECMO actually over VA ECMO, and it shows that you can do it. Uh, VA ECMO, as you would expect, is associated with more neurologic events. and VV ECMO in this study from the mid mid early 2000s, I guess 2006-20207, uh, actually showed you may have some more renal complications with VVECMO, but overall. VV ACM appears to be a little bit safer. And uh you know this last propensity analysis, uh, you know, basically showed that uh survival of VV ECMO is 60% versus 46%. So I think there are advantages to VV ECMO. Sean, do you, I mean, I think you're, you're the expert on this stuff. You probably understand propensity analysis better than any of the rest of us, but I think that was a pretty decent study. Yeah, and the, the, the problem with that is that you've got people who are doing VVECMO who are really good at it and their system's good at it, and then that's how they get into the ESO registry with VV ECMO and what Witt was alluding to is that, uh, our center doesn't know how to do VVECM and not alluding to that's exactly what he said. And he's, he's pretty much right because if we put in VVECO, it's usually that, that next evening that they call and say that they need to go on VECO because they're not doing well enough and I it's, it's a system thing. It's just a matter of getting comfortable with it. We had the same problem in, in the PICU where we weren't doing a lot of ECHO in the PICU. Once we started doing that, then we were getting called, uh, several times a day that we were having misplacement of the catheters and we had to move the catheters and, and it, it took some experience to get to realize when you're not doing well, you can't just immediately blame it on the catheters and, and think about something else. And the same thing with VV ECMO, you know, you gotta be comfortable with giving it a full run and dealing with the, with the problems that you have and. As soon as they have any, any instability in our center, they ask us to convert them to VA. And so after getting bit a handful of times, um, with a, with a painful conversion in the middle of the night, uh, we started going back to VA ACMO. Yeah, so I mean you bring up a great point is that a lot of these things are institutionally dependent and you know, it even comes back, you know, we're talking about the ERCP and management of common bile duct stone. is that you, you have to be able to do what you, you know, work with the resources you have in your institution and the expertise you have in your institution. A couple of questions about nitric thoughts on nitric oxide and, and whether or not pressers affects your decision to go on VV versus VA. So, for, for us, uh, pressors does not. I mean, you know, as somebody once taught me a long time ago, uh, oxygen is an incredible isotrope. Uh, and when you put the, once you start giving them oxygenated blood, uh, and if you want to make an argument, you know, VV Acumo actually puts oxygenated blood through the lungs, which is the organ that you're trying to help. Then usually you can come off a lot of those pressors just the same. So in our institution, whether you're on pressors, what does affect us is if the patient has heart failure or pump failure, and even then if the primary disease is not cardiac, we will still do a trial of. But to Sean's point, we do lots and lots of EV ECMO, and it's one of those things that we're, we're used to, and it's pretty rare for us to have to convert to VA ECMO, but you know, in a diaphragm, I, I don't remember, you know, the, the last time I put somebody on VA ECMO. Matt and Kurt may, may have better memory than I do. David. In a baby, so we, so I've done Semi Selinger and I've tried percutaneous way back when when Ron Herschel first reported it and then Ron told, you know that there have been some untoward events that have happened with that, so we do open. We do a cut down and in our institution with VV ECM we do a cephalide cannula. I've been trying to eliminate it for the last 18 years since I got there but have not been successful. But the flip side is that a cephalide cannula, when you put a cannula up the jugular, actually smooths out that 1st 24 hours of your VV run so that the profusionists and the intensivists aren't bugging you quite as much, so you seem to get a little bit better flows. It helps with that initial point where you, where the flows are up and down. So, let's go to the next slide. Right, so repair on VV ECMO. So, so you decide to, so this kid had their VV ECMO run. And so are you gonna fix the kid on ECMO? Uh, are you gonna fix them on ECMO just before they come off versus early in the run off ECMO open or fix them. Off ECMO MIS after they come off. So who would fix a patient like this on ECMO? Anybody anymore that would still fix these patients on ECMO? You would, Sean, Kansas City, somebody else who's, who's that in the back there? Cincinnati, Santiago, you'd fix them on ECM. Yeah, so you can't do an MIS repair on neck. You, you do it, but Jorge, you might be able to. Can I, can I clarify that question because this is something that Avi and I talk a lot about. This is Avi's interest too. Um, so when you say that, when you, uh, what's that? When you, when you ask that question, are you saying that, uh, they're not getting better and so you will agree, they, they won't get better, so you'll agree to do it. While they're on ECMO or they're getting better, but you want to repair them before you take them off ECMO. Yeah, so there's, there's a school of thought that if you fix them while they're on ECMO before you take them off, that's actually pretty safe because If you take them off and then you fix them and they crash from the operation, then you'd have to go back on ECMO. So some people would actually fix this patient on ECMO. This is not, we can ask the question again about the last ditch effort. Those are rare patients that we're talking about someone get off who's who's getting ready to come off ECMO. They're doing well on ECMO. They're getting ready to decanulate. Would you fix them, Jorge, uh, and I don't know if Michele is here, uh, but would you, uh, Carlos, or would you fix them? Just before you decanulate them. Or do you wait till after they're decanangulated? Give me the microphone. Do you have any other? No. Gluten. You, you, you, do you take them off ECMO and then fix them or fix them? You with. So, so, so, yeah, Santiago, we fixed it on Emo. Kurt, you had a question. In the microphone for the consequences of red cells in the chest scope just to see, but I, I think we end up with a spleen all the red cells and platelets so that it may be very hard to do repair a scope you can't do it. Uh, I mean, off, off ECMO. Oh, you're saying, right, I mean the, the physiology is we destroy a lot of these cells and the spleen can become quite large afterwards, which might make it, if it's in the chest, could make it a, a challenging repair. Yeah, what's, well, what's interesting is, so I mean, as, as you know in our data that we, we are successful in repairing it now. The biggest impediment is really the size of the defect. So kids that go on ECMO, so whatever damaged the lungs was a bigger hit because they have this ECMO run, so the diaphragmatic defect is bigger. But for the ones that we can accomplish primary repair, I, we didn't have any issues with the spleen in, in that series. Um, so who would wait until after ECMO? And fix it after they're off ECMO. OK, so let's go to the next. I, I keep forgetting I have this. So Mark, I just clicked it. Did I get it, Mark? Is it there? You put the slides up, Mark. This is Dan von Alman. I can't see you just from Cincinnati. I, I think it's important to recognize that there's a different patient population that's on ECM, as you just alluded to, and Kurt did. It's different population that's on ECMO and off ECMO and. Uh, in our institution, given the evidence to suggest that a muscle flap repair has a dramatically lower recurrence rate than a patch repair, if we think the patient's going to need a patch, they would get a laparotomy and a muscle repair as opposed to a patch, and that's largely going to be the patients who end up on ECM. Yeah, you know, um, that, that's, that's actually a great point, Dan, and that's something that we don't talk about and it's not, not published. Uh, it's published, but there's actually old data. About, about the muscle flap and uh Dale Johnson from Utah, yeah, and, uh, you know, in, in Atlanta, Ted Brand at uh at on one of our campuses, he's, he's now retired, but, uh, those of you know Ted, he was in private practice for a long time, he used to always do patch repairs and he used to always like to tell me he never had any recurrences. And, and honestly I never saw any of his patients come back to us, so I don't know, um, but that is something that I do think that is a skill that we probably should put back into our armamentarium if we don't already have it. So let's go to the next slide, um, you know, so this was, uh, an Elsa registry study, uh, and you know, basically looking at, and again, uh. Supposedly matched patients, but for centers that repaired on ECMO versus after ECMO actually showed after ECMO having higher survival. Now where the hidden mortality is, I can tell you in our center. We don't, we don't do a last ditch effort to take the guts out of the chest. We firmly believe that that's not the physiologic defect unless there's something that looks like it's, you know, tension, guts in the chest or something like that, or we're having problems with that. I think that that I can think of, there's been a handful of cases that we've even tried this on, but we will actually, if we can't get the baby off of ECMO, we, we'll basically go to comfort measures and let a baby die unrepaired. Um, and I, I don't know what other institutions' experiences with that, but this data, so that would skew this data a little bit, but it's not a high-end, and if you look at this data, it's, it's a pretty good study, and, uh, it's uh. It's in the Journal of Pediatric Surgery, but it it's. It would strongly suggest that you have an, uh, an advantage to waiting until you're off ECMO. Come off ECMO, repairing the diaphragm is not an emergency. Jason, I agree with everything you're saying. I think there are different strategies, and I ask this question every time a CDH session comes up at one of our national meetings. I, I, this question really interests me. But I think the strategies change and so I hear a number of institutions now in the really sick CDH patients repairing on ECMO within the 1st 24 or 48 hours and there's a few papers out saying the risk of bleeding is decreased, although I could tell you I've dealt with a lot of bleeding repairing a patient on ECMO in, uh, repairing a CDH patient on ECMO. That being said, so some institutions now with the really sick. Patients are repairing early and that might skew the data that you just presented and our strategy strategy is to if we can repair off ECMO we do. Especially in the patients that sort of linger for a couple days and then have an acute hypertensive crisis, go on ECMO and we think we could get them off in 3 or 5 days. We'll ride the storm, come off, and then repair, usually typically with a muscle flap, but the patients that we think might be long riders, if we could get them in a state in the 1st 24, 48 hours, we'll try the repair early and go on, and I think that might skew some of the data of survival on or off ECMO repairs. So it's a great question. I, I, I, I agree. I love the answer. Yeah, I, I, yeah, I, I'd love to see the answer to that as well. But, uh, yeah, and one of the options was repair early in the run. How many people on the poll said repair early in the run, um. Uh, uh, actually, nobody, so nobody said they'd repair early in the run on, on the sick, but I, I didn't, I, I may not, but you're saying only in the very, very sick, and yeah, if we, if we do it, we're already 10 minutes over, so maybe wrap up in the next like 5 minutes. Sure, OK, cool, sure. OK, so let's, uh, let's go back to the slides real quick. OK, so because of that I'm not gonna ask that question again. I'm gonna say what kind of patch we can answer. You can't put those poll questions out there, um, but that's, that's fine. Uh, so here's, uh, here's the last case we'll go through pretty quickly, um. Although this kid dragged along for me for a while, uh, so this is a term kid, right sided diaphragm with a sac, uh, repaired primarily, and this was, this was quite a while ago, and I did not excise the sac. So who here, we'll just do a quick poll of the audience that's here, who would excise the sac? I actually would now, but. So who would not excise the sack? Anybody? So a few, a few would not. So I didn't. Let's go back to the slides, Mark, thanks. Um, so this kid came back and recurred at 7 months. So, who would, uh, who would do a, uh, approaches? The original approach was thoracoscopically, uh, through the chest. Uh, so who would approach this again thoracoscopically or thoracotomy, laparotomy, or laparoscopy? And so we can throw that pole up and see what we get. And then Todd, when I, when I get to the next one, you can tell me what it is. So, thoracoscopy converted to open, uh, so we, yeah, this kid, the, the lung was just. Socked down to this thing and we actually converted to open through the chest we excised the sac and again it was strange it wasn't under much tension so I went and did just a primary closure. So is that what everybody else would have done or would anyone have done it any differently? Who who would have done something completely different? So, uh, on, on a recurrence I might consider just going laparoscopically at that point because, you know, uh, you can see it from below and if you can pull the contents out and then you can put an underlay patch, I, I think that's, that's a, that's a great thing. So, so I, I, I, so on the next recurrence I thought the same thing. So let's go to the next slide. So, yeah, the slides back up there, Mark. Thanks. OK, so then two months later, so I mean, it's like I, I, I suck at this. Um, so how do I approach this one? So I actually did, uh, laparoscopically and I did a big underlay biologic patch on this thing. And then, you know, and then the kid like this is the gift that kept on giving, came back 10 months later. So you're right. So I, I, and I, and I don't have that, you know, I know about it. I, I, I, at this point here, I didn't have it necessarily in my armamentarium, and I, and I am interested in, in, in this now. But, uh, the kid presented with some vomiting and actually had an incarceration. Of some intestines in a small recurrence. Again, it was just a small recurrence again repaired laparoscopically, and there was incarcerated stomach and put another large biologic patch. You can argue that I did the same thing twice, but this was under no tension. I do not like I think some of these kids, there are kids. I don't know whether there's a confusion. There's a transition from patch to diaphragm, and I'm just, or from sac to diaphragm, and maybe there was just some, it was just scar tissue I was sewing to. Finally, after this, that third recurrence, it's like the kid's now 10 years old, so I'm keeping my fingers crossed, and I did check. I, I actually checked the medical record to make sure that, you know, I did care everywhere in Epic just to make sure that the kid didn't show up somewhere else. So 10 years out, no recurrences so far. But uh I'm curious to hear what you guys think about sort of these recurrent, recurrent, recurrent. I mean, I think there's some kids that just do that or is it because we're bad surgeons? Any thoughts on number one? Yeah, go ahead, please. I, I just think sometimes there's that you have to sew to good tissue, and if you don't have good tissue to sew to. Stuff's gonna happen. Um, we're big proponents of muscle flaps. So when we get to the recurrent recurrent recurrence, we're doing latissimus flaps or really big chest wall muscle flaps and, you know, older teenage patients who are in this type of situation and we're getting, you know, second opinions and, and doing these large reconstructions. We first go for a transversus abdominus flap. They work beautifully. I could tell you I've been. I never did it in my training and when I was in New York and when I went to Cincinnati I've watched it for 9 years I could tell you there are no recurrences that are coming back using the muscle flap. So how, how, how do you do that? Can you give us a 32nd, maybe 15 2nd? It's a large incision and it's a very. Um, there is some deformity to it, so there comes at a risk. So picking the right patients who need a real muscle flap versus not, I think, is to be determined, but it's an abdominal transverse abdominal incision about at the level of the umbilicus going up the midline and then. Developing the transversus plane in the muscle, which is quite easy to do and very quick, trying to maintain your blood supply to some of the muscles so that you have blood supply and you just flap it down and in fact, half of the flap you don't even have to sew in because it's already attached to the lateral wall. So you're even putting in less suture. Some of the flap it maintains its natural connection and so it's actually it works beautifully. I hope that was the 2nd version. David Everybody else incisional hernia that I had to repair. Disheartening. Somebody mentioned it you can go minimally in if, if you're going to go MIS, you can go back and forth, and this kid did ultimately didn't need a patch. It was just an onlay patch, but you can go from the chest to the belly to the chest to the belly and back and forth, uh, as you do that and as you you depending on what's going on. But thanks. Awesome. That was a great review of things that we don't normally go in such depth on. So that's why it's not the we need that we need.
Click "Show Transcript" to view the full transcription (42598 characters)
Comments