Update Course Rewind: Omphalocele & Gastroschisis 2020

Hey, this is Rod Gerardo, research resident Cincinnati Children's Hospital. Today, I'm joined by my future replacement. Hey, this is Ellen Antisco, another research resident at Cincinnati Children's Hospital. Ellen, what are we talking about today? Today, we're talking about il and gastrosis. But we're not just going to talk about it. We're going to hear from some experts from the 2020 update course. So we are going to start with an il case and we're going to start by just talking about some of the general physiological principles that that I think are underreported in the literature and then we'll go to management. That's Dr. Sean St. Peter, surgeon in chief at Children's Mercy, Kansas City. And we started out talking about a case of giant il, typically defined as 5 centimeters or greater or liver in the sack. 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. All right, Ellen, so what did they find in that study? They found that patients with giant omphalocele had a greater time to full feeds, required more TPN, had more chromosomal anomalies, and they had a higher incidence of respiratory insufficiency. Wow. Okay. Well, that is interesting. We typically, at least I was taught in in fellowship, think of these as as pulmonary hypoplasias being the main problem, that pulmonary hypertension is a problem of CDH, but pulmonary hypoplasia is the problem when you have uh an omphalocele. 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. But interestingly, we had five patients out of the 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 pulmonary hypertension later. And this was severe pulmonary hypertension where two of the patients ended up dying and one was on a pulmonary vasodilator 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 put these patients at high risk. So Ellen, when you have this giant omphalocele they're talking about, what options do we have for treatment? So the options are, we paint the sack, we remove the sack and place a silo and either passively or actively reduce. We keep the sack and actively reduce, or we just definitively close right now. Okay, let me say this again. So so we either paint it, meaning we just paint it and leave it is why I'm guessing they mean there. You either paint it, remove the sack and the silo for a passive reduction. I guess you just let it fall in on its own. Remove the sack and then active reduction, meaning that we're going to actively push it. Yeah, we're going to push it in. Or you just keep the sack and then you try and reduce it, you try and push it in. And then he added another one, which was closed right now. There's multiple mechanisms that have been described for keep the sack and active reduction 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. And it's a very easy and very smooth technique. As you see, you have to do a a do a hydrocolloid dressing to do a silo without removing the sack. That was Dr. Miguel Gilfren. He's the chief of neonatal surgery at Gonzalez Cortez Children's Hospital in Santiago, Chile. So, Ellen, Dr. Gilfa is out in Chile. His team does something different. What do they do for their giant omphalocele? So they use a hydrocolloid dressing to make a silo and then once that's on, they start reducing from there. And in these 40 patient, we get to close a 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. So do we have to paint the sack before we place this dressing? You don't paint it at all. actually you have to put it over hopefully within the first 24 hours so the sack that doesn't get you know very stiff. And the hydrocolloid it get the the the sack very very smooth, very hydrate. And what about if we have a ruptured omphalocele? What should we do then? We have three three patient that have were born with a rupture omphalocele that we suture the omphalocele and then put the this dressing. Todd was convinced. Mark, you want to try it with me on Monday? We'll faceTime Miguel. So the next part to think about is definitive closure, but Dr. St. Peter had another concern, right? What was that? Yeah, so his concern was that these patients with can have nonrotation. And so the question is, should we do a lad procedure at the time of definitive closure if we are in the abdomen already? Cool. All right, what did they say? And what we found was that uh there was there wasn't increased risk of adhesive valve obstruction, of course, with um gasrosis, but the risk of mid gut volvulus was higher in patients with il. And that supported the claim that if you're exposing the intestines, then it's worthwhile doing a lad procedure at the time because you know these patients have nonrotation or mal rotation and nonrotation does not exclude you from having the possibility of having anatomy that's not favorable with a narrow base of mesentary 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. Well, so what's the what's the whole punch line here? They found that patients with omphalocele are at greater risk of midgut volvulus than patients with gastrosis. And so doing a lad procedure is something to consider. Okay, so I'd say we learned three key points there. First, we learned that patients with giant omphalocele have more respiratory compromise and they can even develop pulmonary hypertension, especially after they have an episode with sepsis. Second, we talked about a novel technique for closing omphaloceles with a hydrocolloid dressing like duoderm as the silo. And last, we learned that patients with are at greater risk for having nonrotation. So if you're already in the abdomen for definitive closure, consider doing a lad procedure. So next we heard about an interesting case from Dr. Salim Islam, who's the chief of pediatric surgery at the University of Florida College of Medicine. It was an infant born with a very large abdominal wall defect. A lot of abdominal contents were viscerated including the stomach, the intestine, and the liver. There was no sack covering everything. Um so they placed a silo. Ellen, I'm looking at this and I have to ask what is this? If you're using the stay current app, take a look at the photo at the bottom of the page. Well, the problem what we 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 called this a ruptured omphalocele. And the rationale for calling this a ruptured omphalocele 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 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 so both those to the fascia ledges. Or you can do and take a long vacation that you always wanted to and so you're faced this situation where there simply is nothing to sew. There's no amnion at all. What do you do in that situation? And so here we heard from a few different people. Uh first from Dr. Gilfren. In in this huge whatever gastro or that I don't have a place to put the hydrocolloid uh we have using the same technique for the last 15 years 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 prolene mesh because it stays forever until we can close the the case and we protect that within a plastic bag within the bowel. Then we heard from Todd. I'm just going to call it abdominal wall defect. I don't need to know the. But when I have a giant abdominal wall defect and I put a Bentex 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. So Dr. Islam and his team used biological mesh 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 wanted 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. Wait a second. Did he just say they use the four skin as a graft? Yeah, you heard that right? Okay, that just making sure, carry 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 serieally 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, you managed to get all that skin coverage, he survived and now what do you do? Now you got just a gigantic ventral hernia. It looks like he's he's about the guts are about to fall out of his belly every time he walks. Wow, okay. So what do we do with that? So there are a couple of different options we can consider for closing such a big abdominal wall defect. Um the first is using tissue expanders either in the abdomen or in the subcutaneous space to help increase the abdominal domain. A second option is using component separation. This is separating the tissue at the external oblique and and just beyond about a centimeter beyond the rectus sheath and then 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 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 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, they reported the use of this in nine kids and they used it in every age from seven days to about 10 years of age. A majority of them were ompaloceals 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. 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. Okay, so to summarize, uh we learned about an infant with a very large abdominal wall defect. We're not really sure if it was an omphalocele or gastrosis initially. Uh and in infancy, we can use something like either biological or prolene mesh to scaffold and and help us close the defect. Then a few years later when they're older, they'll have a very large ventral hernia. And to close this, we can use again different techniques like tissue expanders and abdominal component separation. All right, so at the end of this discussion, they went over some key points, the first one being from Dr. Islam about complex gastrosis. Complex gasis is is really a different disease almost to the simple variety of this uh condition. Um everything is worse. They're they're the hospital length of stay, the requirement for further operations, they're um sepsis rates, everything is just worse. Another point important about gasis is how to manage it. First, suture versus sutureless closure. For those who don't practice it, what a sutureless closure means is you put the silo on or you just tuck the bell in and then you put an occlusive dressing on there. Usually you change it at five days and then at the next change, it's the it's mostly closed at that point. Um and you can go to simple dressings. Um but what we found was that there was no difference in time to full feeds, TPN use or duration of hospital stay, but there were fewer anesthetics and the patients who were being managed with with the sutureless closure got antibiotics less frequently and they ended up with less infections also. So they had they had fewer infection and septic events despite having less antibiotics and that's probably provider specific, but just the same what we took away from it is that we probably shouldn't be so aggressive with antibiotics after uh babies are born with gastrosis regardless of how we manage them. And if we're going to do sutureless closure, um try to avoid an intubation and an anesthetic and then that's becomes the advantage that they have. And then what about silo placement versus immediate closure? We had done a randomized trial. We ended up randomizing over 50 patients and found that there was um no difference between immediate closure and silo. And the conclusion of that was that this pave the way for sutureless closure because if we went from silo to sutureless and we never answered the question, does just tucking it all in and closing the defect initially do better, then we wouldn't go down the sutureless path. But once we saw that there was no difference between silo and immediate closure, then we could study sutured versus sutureless closure. Okay, so that's gastrosis. First, we reviewed that complex gasis is overall a different beast. Second, we reviewed the management of gasis. We reviewed that when we studied sutured versus sutureless closure, we learned that getting patients fewer antibiotics is overall a good thing. And we learned that with sutureless closure, we need less anesthesia. And last, we reviewed using a silo versus just closing immediately and found that the outcomes are overall similar. Thanks for listening to this rewind podcast on and gastrosis. Don't forget to mark your calendars for the update course coming up on August 27th. It's going to be virtual and very interactive. In the meantime, keep listening to our podcast and look for update courses posted on our website. Or wait for the real deal. Our update course for 2021 is August 27th, mark your calendar. Have a great rest of your week and remember, knowledge should be free.