Gastroschisis - Clinical Practice Updates

So we are going to start with an um oncase and we're going to start by just talking about some of the general physiologic principles that that I think are underreported in literature and then we'll go to management. And I'm going to get some input from um Miguel Gilfoyd on that one. So, babies born with something that looks like that. And we're going to call it a 5.5 centimeter on with liver in the sack and compared to any other baby with on, compared to the routine on that doesn't have these dimensions, the parents should be consulted that this has a comparable risk of respiratory compromise. There is a greater chance of having a chromosomal anomaly. Time to full feeds will be the same as any other baby withal, time to closure will likely be a year or more and if the echo shows no pulmonary hypertension on day of life three, there is no risk of subsequent respiratory compromise. And so to talk about that a little bit, we put together a two-center um retrospective study that encompassed 20 years and we had 97 survivors. And patients who were who were found to have a giantal, typically or typically defined as 5 centimeters or greater or liver in the sack. Um those patients with a giantal had a greater time to full feeds, required more TPN. They also had a greater risk of respiratory insufficiency and they did have a higher incidence of chromosomal anomalies. But interestingly, we typically, at least I was taught in in fellowship, think of these as as pulmonary hypoplasia is being the main problem that pulmonary hypertension is a problem with CDH but pulmonary hypoplasia is the problem when you have uh an onal. But what we found was that 56 patients of those 97 were identified as having pulmonary hypertension. Now most of those were diagnosed within the first week of life. Um, but interestingly, we had five patients out of out of those 56 that had no signs of pulmonary hypertension in their first echo within the first seven days of life and then they subsequently uh went on to have um pulmonary hypertension later and this was severe pulmonary hypertension. Where two of the patients ended up dying and one was on a pulmonary vasodiator for more than a year. And all of these were associated with an episode of sepsis. So what that what that tells us is that even in the patient who doesn't have signs of respiratory compromise early, sepsis later, um and one of them was 52 days, 52 days after their initial echo showed no pulmonary hypertension that a single episode of sepsis put these patients at high risk. So now that we've got um, I guess I'll ask Todd or Rod if we see anything in the chat feed on how people answered that question. Uh, nothing in the chat feed yet. They're still hanging on, still have some some questions about um contrast and things like that. But I will say the poll results are coming in overwhelmingly people are saying all of the above. 65% of the people who are responding are saying all the above. And then the next most common is people are saying that you got to talk to these patients these parents about sepsis. Yep. So, now what? That's those that's the background of an onal, but how are we going to manage this? And we could paint the sack and we'll we'll we'll take this exact example. How would you manage that patient right there? Paint the sack, remove the sack and passive reduction, remove the sack, active reduction, keep the sack and active reduction and the choice I didn't really give would just be definitive closure right now. Um, this is where I I'm going to turn to Miguel Gilfoit because there's there's multiple mechanisms that have been described for keep the sack and active reduction. So that was the answer that that we're looking for there um because the the time to definitive closure does not have to be more than a year and with that risk of sepsis, there's the the theoretical advantage of the quicker you can get to closure then the better off you are. So Miguel, do you want to chime in on on your technique here that I've got a few pictures of and this is keeping the sack in place and then doing active reduction. Yeah, the thing that that the initial picture is a a bill in this case was a little bigger than the initial that you showed but this is this is a um a technique developed by Dr. Abello from Colombia that we uh start doing that about three years ago and together we have treated almost 40 patients already and it's a very easy and very smooth technique. As you see, you have to do a a do a hydrocoloid uh dressing uh to do a silo without removing the sag. And in these 40 patient, we get to close uh 97% of them uh within 30 days and 92% within 15 days. So, actually we have done very well. All these patient keep in in ICU ventilated and completely paralyzed but we can get a a very high um closure in time uh almost in 95% of them. Yeah, I think Miguel, you presented this at APSA and I and I moderated that uh discussion. That was quite amazing. I don't know Sean, what do you think? Uh, I have not been able to close every single one of these big ones. No, likewise, um I was impressed that they didn't have more troubles with um with pulmonary compromise with the active reduction. Um, I think that's the difference. We give up on active reduction when the patients don't tolerate it from a respiratory standpoint and then go to painting. And we've had some recently that are just incredibly that even being in a completely supp position they have respiratory compromise. They're stronger in Chile, I guess. So can I'm sorry. can you just explain again? So so when you paint first and then put this on or how so how long after the baby's born do you apply this? You asking me? Yeah, I'll ask you. No, we you you just put this, I mean, hours after the baby is born. Yeah. Uh you don't paint it at all. Uh actually you have to put it over hopefully within the first 24 hours so the sack doesn't get, you know, very stiff. Uh and the hydrocoloid get the the the sack very very smooth, very hydrate. So Miguel, don't you get scared that that it's not going to when you put so much pressure on a very thin wall, it's going to burst? No. And actually, we have that we have three patients that had uh were born with a raptureal that we suture theal and then put the this dressing. For not as bother as Miguel. We sometimes wait a few days, but it still seems to work Miguel if you, you know, if you're afraid like Todd's saying that the sack might tear um from all the pressure in the first couple of days, like we've certainly waited a couple days, had some other dressing on and then once the sack is no longer like paper thin and see through, then start the derm and seems like it still works. I know I I I I I didn't say doesn't work, but it gets stiffer. So, the beauty of this that you I mean, the baby has to be stable to start doing the compressions, but usually you can you can start within 48 hours. About all of these patients are intubated and completely paralyzed during this procedure. Okay. Um, I'm going to try. We just had a kid born. Wolkan and I disagreed. I think he's crazy. Uh, he wanted to push it in. I thought it was too big. Uh, so maybe we'll compromise and and do this uh on Monday. Mark, you want to try it with me on Monday? We'll FaceTime Miguel. Absolutely. Okay. Okay, so now we're going to move on to the definitive closure, you get it all reduced and now it's time to close the belly and um the question is should we do a lad procedure at that time since we are in the abdomen? If your closure involves being in the abdomen. And data that uh we joined with um with Milwaukee. This was Amy Wegner's project. Uh she wanted to look at the risk of somebody having aus um versus an adhesive ball obstruction after they've had a definitive closure for um for al or gastroschisis. And what we found was that uh there was there wasn't increased risk of adhesive ball obstruction of course with um gastroschisis, but the risk of midgut us was higher in patients with onal. And that supported the claim that if you're if you're exposing the intestines, then it's worthwhile doing a lad procedure at the time because you know these patients have non-rotation or mal rotation and they're not going to have the same adhesions that you have with gastroschisis. So Sean, I I I I don't ever I've never done, I mean, I would now I guess, but I've never done ads in any of these patients. But I always with this slide you're showing, I always have a problem with that slide. Uh, the one it's okay, you don't have to pull it up, but the comparing bowel obstruction to to midgut voluus. Yeah, so it's not point is lethal and one is. No, sorry, they're they're both just on the same on the same chart, but they're not comparing the two. It's just showing that if you have gastrosis, you have a much higher risk of ball obstruction. We all know that. But the risk of midgut valus is higher in patients who have onal than with gastrosis. And so we just don't see a lot of valus with gastrosis, but we do see it with onal. Just simply making the point that I guess take it another way, if if you have to operate on somebody with gastrosis, you typically end up making sure you line them up like a lads, you make sure that the colon's on the left, small belt's on the right, the anterior surface of the mesentery is exposed. Um, as opposed to just tucking it in and so it's sort of the same boat that if you have access to the intestine, and that was the soft part about that manuscript was that we're not saying it's worthwhile to go through the sack if that's not your technique. So the sack is maintained in Miguel's technique. So it wouldn't be worth going after it inside the sack. Um, but if your operation involves exposing the intestines, then it may be worth considering a lad procedure. So Sean, when you stuff, do you do a bedside gastrosis tape closure? Oh, yeah, we do we do sutureless closure now. Right. So we never we never see it. lay anything any kind of way you want. Exactly, you never you never get to see it in that circumstance and then you're hoping that the adhesions take care of it from there. Well another option I think what you're coming to Todd is is that this is similar to diaphragmatic hernias. So when you're doing a diaphragmatic hernia repair, you expect them to kind of have a mal rotation because the gut's not in the right place. And so how many people would do a lad procedure concomitant to a diaphragmatic hernia repair? Sean, do you do those? Yes and no. So I'm not going to take out the appendix, but I'm going to make darn sure that the small bowels to the right, the colon to the left and the anterior surface of the mesentery is exposed and it's it's not rolled in. I mean the the risk, the thing that you're looking for is those patients where the secum and the proximal bowel are just kind of rolled in together and you can just un unroll them like a scroll and that's that's the key move that's going to theoretically decrease the distance between the two ends or increase the distance between the two ends of the jump rope so that you don't have that risk of doing the full twist. Right. So it's it's a judgment thing. So you you'll judge look at it and you'll see it. So Mac was shaking his head, no. Mac, you never do it? Well I can't do a lad procedure through a thoracoscopy approach. Well, if you're doing thoracoscopy, that's Right. That's going to be really challenge. That goes without saying. Uh, Joe Icono really wants us to pay attention to the terminology we're using and how they're different mal rotation and non-rotation. Yeah, that's very important. Fair enough. Huge difference. And these patients are all non-rotated. Yeah. For the most part. And also several other people, Ben and a lot of other people are are posting other protocols of reducing. So, um, that's worth looking at. I mean, I guess the point is non-rotation shouldn't have much of a risk of voluus. So, you know, I think you're you're uh data she says otherwise, Sean. I mean, you have non-rotated babies getting midgut voluus. Yeah, that's that's true and and I've I've found that non-rotation does not exclude you from having the possibility of having anatomy that's not favorable with a narrow base of mesentery and the two ends being fairly close together. It's not universal, but that's that's what we found is that it does happen. Okay. That was awesome. Liam's sharing the screen now. Is that right? Yeah, and I started them at the end. So, what you have is and I'll show the images in a second. You have a 36 week gestational age male who so full term, born with a prenatal diagnosis of was an abdominal wall defect that the MFMs had a lot of trouble trying to characterize. Is it onal? Is it a gastroschisis? Unclear. After delivery, he's noted to have a very large defect, almost no anterior abdomen at all with the viscera that includes almost entire liver, a large amount of intestine, the stomach. There's no visible amnion or any covering of the and basically what you have to do is um put on a gigantic silo, which we used a 10 cm silo here to just cover the viscera and try to figure out what's going on. So there's another image of it. You can see the entire liver is on to the back, the stomach, the colon and everything is there right now. So, the question is, how would people in the group I classify this defect? Are you dealing with a gastroschisis or are you dealing with anal? Sean, what do you think in this situation? I would take a look at the abdomen. Well, here it is. I mean you saw it. Where is the defect relative to the chord? It's right smack dab in the middle. There is there's a chord just to the uh upper part of it or it's unclear. Seems to be on the upper part. Then I guess the question would be, does it matter and uh it would depend on the physiology of the patient at that point and I'd be more concerned about what the lungs are doing because your belly status is is what it is. Yeah. And so in this case that's anybody else? So Todd, what would you call it? Are you going to call it again? Yeah, I I would just say just what Sean said, now you just have bowel outside of the belly, uh other than looking for congenital anomalies which is rare in a large seal, uh there shouldn't be really any difference between the two babies. Agree. Well, the problem what we've had so is is basically in in terms of the next management. Yeah, we got the silo on. We have that all done. So we call this a ruptured onal and the rationale for calling this a ruptured onal was that the defect was very large and the entire liver was out. In a gastrosis you don't expect the liver to be out. And when you have almost no abdominal domain, it becomes really challenging to try to do anything in place. So, here are your choices. So I think do you guys um, Ray, do you have that in the J, you have that in the um Globalcast part two? Yep, we got it. Okay. So here are the choices. You either leave the silo in place and start squeezing down on that. You can create a separate silastic silo that you sew to either the fascia or the skin. You can create a silo with PTFE mesh or with biologic mesh, which is the next choice and sew both those to the facial edges or you can do and take a long vacation that you always wanted to and and leave it to somebody else. So let uh how many seconds you want to give that uh Todd? like 10? Uh it takes them it we're it's a 20 second delay between when you talk and they get it so uh let me at least. So let's ask let's ask guys here, Miguel. So you're you're faced this situation where there simply is nothing to sell. There's no amnion at all. What do you do in that situation? In in this huge whatever gastros or that I I don't have a place to put the hydrocoloid uh we have using the same technique for the last 15 years uh because I know it's going to be a long, long round to reduce all things and the only thing have been okay with us is put a proline mesh because it stays forever until we can close the the case and we protect that within a plastic bag within the bowel. So um the answers are uh the most common answer is leave the silo in place. Um it's changing every second. Um some a lot of people are saying create a biological mesh silo, uh which I don't like to be honest it it it adheres to the bowel but that's um I will tell you one thing I wonder if any other people have had a problem when I have a giant I'm just going to call it abdominal wall defect. I don't need to know the. when I have a giant abdominal wall defect and I put a tech silo, a spring loaded silo and I push down, the forces go out. And I find that it actually makes your defect much bigger over time. And I used to tease people that would do the old fashioned sewing but I actually think in these cases it may pretend a better outcome. Well, that's good. It's interesting that you brought that up because, you know, when you try to squeeze on that mesh, when people try to squeeze on it, it just popped right out because the defect is so big that you can't nothing holds it in place. So what we did was, so we eventually and there's no right answer in that poll, but it's interesting that most people were choosing the biologic mesh. We used that because we use it as a scaffold. So Todd, you mentioned that it sticks to the bowel. Yeah, it does and it does create a scaffold and that's what you want it to do to allow the skin to epithelialize, which it did. We then did a circumcision, used that skin to kind of also create a graft and put that on and then the plastic surgeons came and put tissue expanders in, we got flaps and we managed to finally get coverage. And then we created some domain by by before the the mesh got incorporated is we just serially resected it and then just reduced it and get the upper parts of the mesh and stretch the fascia a bit. So now the question becomes, he's four years old, we managed to get all that skin coverage. He survived and now what do you do? Now you've got just a gigantic ventral hernia. It looks like he's he's about the the guts are about to fall out of his belly every time he walks. So, the choices are, you can just do nothing, leave him with a gigantic ventral hernia. You can replace it with a permanent mesh, either PTFE or nylon. You can do a separation of components closure of the fascia with some mesh if needed or vacations always an option. And I wanted to see while we wait for the responses, I just wanted to uh address some of the people in the comment section here. A lot of people posting like Todd was saying earlier articles, but now even uh technique videos so you can upload a YouTube link here for everyone else to see how other people are closing these giant defects. Um, and then a lot of really great questions here. Uh, some people asking about, hey, what about tissue expanders in this scenario? I know you talked about we're trying to get domain. Is that an option? Some people talking about timing and things like that. And uh with all of this adding up a lot of people are still choosing to take that long vacation. Vacations are never a bad idea, never. Salim? Yeah, yes Miguel. I I I think to get a a huge ventral should be the last decision. I think I think we our experience have been completely different from that. We have able to manage this huge gastrosis and and the less of of them get a ventral and even though it's a smaller one that just leave them to size. We we need to we have to be treated them with very good results. Mm. So you would you would have closed them in the neonatal period. No, no, no, well I will I will try to do the the the thing I said this proline mesh that state I I have these for months without taking out that same mesh and I I can say 80% of them we can close it within you know two or three months. M. But if you get you know the one thing you don't have on there Saleem we've done in Atlanta is uh it was the tissue expanders. I think Kurt had may have mentioned that in the chat and have the plastic surgeons come in and put some tissue expanders in before you try to close it and it gives you some more it'll stretch the fascia and the whole wall and you can get it closed. Mark, you put the tissue expanders inside the belly. So somewhere that inside. Yeah. Saleem, it's Eric. We we've had a different experience using it primarily subcutaneously. So I think one of the key things particularly if you anticipate having to do serial procedures is you need really healthy skin and particularly the ones that are treated with paint and weight initially don't have very good skin. So we would expand the subcutaneous layer get really good redundant skin and then if it's the example that you showed, I think you're going to need to do serial procedures where you're imbricating or excising uh segments of of a biological mesh or something like that so that you'll gradually get those rectus margins upwards. We the other option I think would be a component separation and I think in an older child that's a possibility. Yeah, and so um and again remember the vacation. Everybody should remember the vacation. So component separation is what we ended up doing and um, you know, so so Mark, we we talk thought thought about putting a a um expander in, but our plastic folks felt that if you put it in the belly without any, you know, domain really or without anything, you would basically just push all the contents up and out. And and that's what their concern was. But I think tissue expanders remain a great idea. We did use tissue expanders in the subcutaneous tissue to create space. But the component separation technique basically involves the use of of separating the tissue at the external oblique and and just beyond about a centimeter beyond the rectus sheath, which is right here. and then uh that creates a lot of space and you do this on both sides and then of course you have to dissect beneath between the external oblique and the transversus and the and internal oblique and then that creates a lot of room. You can also make an incision on top of the rectus sheath, the anterior rectus sheath to give you another centimeter or so. and remember this this is on both sides. So you can create a lot of room to to get space. And in a in a report from um Texas, uh UT Houston, the group there, uh Cogen and uh Rich and Recy in that group, they reported the use of this in nine kids and they used it in every age you can see from seven days to about 10 years of age. majority of them were onal and giant defects. And they were able to close almost every single one of them. They did use some mesh in some cases to help them bridge defects, but they were again able to get facial closure in a vast majority of them, which was pretty good. So, um, again, separation of components technique which our adult colleagues use all the time is something to always remember and and they again felt that they could use it even in babies uh where we could get good good results. So that was just a little twist on theal to kind of end that and um discussion. see if I can come up. All right, um, so