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In this insightful on-demand teaching session, the speaker explores and discusses the challenges medical professionals face in dealing with complex gastroschisis patients. The speaker comprehensively looks at the factors influencing outcomes including patient-defined factors, clinician controlled factors, and health system issues. They also delve into defining "complex gastroschisis," discuss surgical strategies that could potentially improve survival rates and patient outcomes, and consider what impact congenital problems like congenital atresia, necrosis, and perforation can have. This highly informative session provides the necessary insights needed to better care for patients with this severe congenital anomaly. The speaker also shares their extensive experience and research in dealing with such cases. Medical professionals and student trainees will greatly benefit from attending this informative session.
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This is an invited talk given by Professor Sherif Emil from Montreal, Canada on the topic of "Complex gastroschisis" as a part of a Zoom academic meeting of the Department of Paediatric Surgery in East London, South AfricaTranscript

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Learning objectives

1. Understand the definition and nuances of complex gastroschisis, distinguishing it from simple gastroschisis and other congenital anomalies. 2. Learn about the primary determinants of outcomes in gastroschisis and how these factors are divided into patient-defined, clinician-controlled, and health systems issues. 3. Analyze different surgical strategies that potentially can improve the outcomes of babies with this severe congenital anomaly, with a focus on closure methods and treating intestinal complications. 4. Gain insights from case studies and statistical data on the morbidity and mortality rates associated with gastroschisis, particularly the complex version of the condition. 5. Identify and apply successful practices from global contexts, including care bundles and strategies to bridge resource gaps, to improve the treatment of gastroschisis in various healthcare settings.
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No, thank you so much. It's always really, really wonderful to be with you. Um I think this is the fourth time I joined your teaching and I've learned a ton from your residents and trainees in their own presentations. And I also see people here from different parts of the world. Uh East West, South, North. So just wonderful to have you all with us. And uh I just wanna thank Meland again for making this such a uh a shared experience. Um Yeah, so I'm, I'm not gonna actually have a give a long talk because I thought it would be nice to, to have more interaction, but I would like to share with you some uh some work and some thoughts about a very difficult group of patients that we're all challenged with really uh regardless of where we are. But certainly the less resources you have, the more challenging these patients are. Um And those are the patients with uh what we call complex gastrosis. So, what I would like to do is maybe uh discuss two things, the definition of complex gastroschisis. Um If we don't agree on a definition, it's very hard to agree on how we can measure outcomes of the disease or how we can gauge or compare outcomes between different approaches. So there's a little bit of uh confusion about what this really means. And so just spend a few minutes talking about it and then uh maybe discuss some of the surgical strategies that potentially can improve the outcomes of babies with this um with this very uh severe congenital anomaly. Now, uh I have to tell you that the gastroschisis is one of the reasons I went into pediatric surgery, uh, as a medical student on rotating on the immuno intensive care unit. Uh, here at mill, uh about my second week, a baby was born with gastrosis. And as you all know, it's a very visually impressive anomaly when you see all the bowel loops outside the abdomen in a small baby. Uh, certainly for a medical student, it's sort of a shocking anomaly to look at. And it was quite amazing because by the end of my rotation, 2.5 weeks later, that baby had actually done very, very well. At the time, there was, you know, significant uh, surgery needed to close and silo closure was not really uh an option. But, um, but nevertheless, the baby actually did very, very well. And the fact that a baby who has such an impressive anomaly can leave the hospital a few weeks later, essentially to lead a normal life in many cases, was really quite impressive. Um And I was very fortunate uh about three decades later to edit this um issue of uh seminars that had multiple articles and some of the more controversial uh aspects of uh gastrosis. And maybe I will share and show you all of you have access to it, but I'll share with me and uh the article that addresses what we're talking about today and, and he's welcome to send it out to all on the uh all on the, on the talk today. So, you know, during my lifetime, I've seen gastros cases go from this uh huge abdominal incision, gastrostomy tubes to having babies really look like this with an umbilicus that's almost normal and having no scar uh really to speak of. So if we think about what determines the outcomes and gastroschisis, well, there are really three categories, there are um factors determined by the patient, what I call patient defined factors. There are factors uh controlled by the clinician and those are actually not very many, a lot of the outcomes in gastroschisis are determined by the patient. And then of course, we know that there are health systems uh issues that very much affect outcome. So what are some of the outcomes we've looked at that are patient defined? Well, gestational age, birth weight, uh whether they are associated anomalies. These are not very common for gastroschisis as you know, as opposed to omphalocele. But some patients rarely may still have cardiac anomalies may still have pulmonary hyperplasia and those definitely affect outcomes. But the big one of course, is intestinal complications when suddenly the intestine goes from just exteriorizes intestine with some inflammation matting to um to one with complications. And then if we look at clinician control, these are some of the things that have been looked at. Can we time the delivery to improve outcome? Can we change the mode of delivery to improve outcome? Uh Does closure method matter? Is there any specific nutritional strategy that is beneficial? What about infection control? And then what we'll talk about today are some of the surgical strategies because really, there has not been a whole lot of evidence at all to show that a specific timing or specific mode. Certainly in our practice. Now, the overwhelming majority of gastroschisis patients are delivered vaginally, we do not deliver them prenatally. Uh early um closure methods really haven't been shown to make that much of a difference at all, maybe with respect to resources but not, not so much with respect to um uh outcomes. And with regard to nutrition. Again, most of our patients here are on TPN. Uh short of that, there hasn't really been any specific nutritional strategies um that have been shown to affect output. And then finally, of course, there's a health systems, even in our own environment, we talk a lot outside of delivery. Is it better to deliver the mom at a uh center where the baby will immediately be taken care of. Does transfer matter, baby is delivered as what we call an out delivery and then transferred to us. Does that matter in the US? There's been some work on uh showing that insurance status does affect patients with congenital anomalies. But really the two big ones are the human resources and the material resources that are available to take care of the patients. And we've had some webinars before through the project that I know some of you participated at. Looking at um you know, the Wright's work in terms of trying to improve gastros cases in Sub Saharan Africa through care bundles and really ways to try to bridge um the gaps and the deficiencies in human and material resources. So what is our role as surgeons? Well, um one is closure of force, we close the defect to one of many different approaches and then really the other is treating intestinal complications. Those intestinal complications can be congenital like atresia, stenosis, volvulus, necrosis, perforation. Sometimes it can be acquired like a baby who develops necrotizing enterocolitis after gastrosis or a complication of closure as I'll show you in a moment. So if we look at, you know, some of the recent reports uh recent as in the last decade or so, uh last decade or two coming from the west, there is clearly a difference in almost all the reports um between what we would call simple gastroschisis and and complex gastroschisis in terms of survival. So once again, it's really the most important outcome determinant is what the child is born with. We looked at our outcomes here in North America a few years ago and compared outcomes in Canada to outcomes in the US. And you can see the overall survival almost approaches 100% for gastroschisis. It's really high uh for simple, for complex, we're still at the 90%. So we still essentially lose one out of, out of every 10 patients um to this disease. And of those that who survive, um you know, there are several who still go on to succumb because of severe short bowel syndrome and other complications that are not shown in this data. So this is probably a bit more optimistic than it actually is. Now. I've been very interested in getting gastrosis for a long time in different environments. So we did this study uh back when I was in California that, you know, for North America, we were a very busy center almost one a month. Lots of cases. That's uh a lot of centers don't, don't see many gastro cases patients, but our center was very, very busy and we showed that survival to discharge was not different between simple and complex. However, when we continued to follow these patients up to two years, this was where the hidden mortality is survival in complex gastros cases dropped from 95% to 82%. And again, most of these complications were due to severe short bowel syndrome of those who left though most of them did by 12, the ones who survived 82% by two years were for oral feeds. 12% were on a combination of gastrostomy and oral feeds. And really only, uh, one patient was still on TPN at two years of age, but there is most definitely a significant, um, hidden uh mortality. So again, looking at a comparison of the simple and complex ones, the one really stood out, which, you know, has been shown in pretty much all the different uh studies is that of course, ones with complex needed longer. TPN had a almost double the period before they could start feeds, uh were on ventilators longer and their hospital stay was much, much longer. So even in survivors, complex gastroschisis means very significant morbidity. So the question is, what is it, you know, what do you mean by complex gastrostasis? Well, what's not controversial when there's any bowel compromise? I think we would all agree on that. So, if there's atresia, there's necrosis, perforation b short bowel. But what is somewhat controversial is what if there is severe madding? What if the baby develops necrotizing enterocolitis after the fact, what if, uh the baby has such severe gastroesophageal reflux disease? What about associated anomalies? And so, um, it's really important to know what we're talking about. For example, there have been reports coming from some really well-respected centers that, um have, you know, significant numbers of patients undergoing fundoplications, for example, which is very, very bizarre because we don't see that as a problem. Usually gastroschisis patients with reflux and then they call those patients complex gastroschisis and they report their outcomes and obviously those outcomes are pretty good in general. So what I would, um, advance is that what we should think about is that it's really gastrosis that's complicated by a congenital problem by congenital atresia necrosis, perforation or bones. Because that's really where the true challenge lies. A patient who develops necrotizing enterocolitis. After the fact, even if it's fulminant and severe does not really belong in the same category because these are very different interventions that are needed for the patients and for those patients who have these congenital anomalies. So that is the definition I like to use. So for example, if you see this patient who I had a while back and patient was sent home at uh you know, about four weeks of age doing very well and then comes back with a stricture at five months. Well, that patient is not a complex gastroschisis patient, that patient developed a later complication of the kind of global ischemia that we see. But you know, the patients cannot be grouped in the in the uh in under the heading of complex gastroschisis cause their initial outcomes were very similar to those patients who do not have complications. This is really a late complication if you will. So we have here in Canada, um an organization called SNB and II, am sure many of you have seen some publications coming from it. We're really fortunate to have a very finite uh system where all the gastroschisis babies are essentially treated in one of 16 centers. You see here, there are no babies who are uh you know, treated outside the university centers where pediatric surgeons and heos and multidisciplinary care exist. So it was really an opportunity to capture these patients. And we for the last 1617 years, we've now been capturing the overwhelming majority of these patients that are born across the country in a prospective database. And that has been very, very successful. It has really produced numerous publications looking at all types of things and it's not a, you know, none of these publications of course, are going to be randomized because it's an observational database. But at least it allows us to use relatively clean data that's uh uh collected prospectively by essentially professional data abstractor who know exactly what to look for. Well, one of the uh the the big things that has come out of, of caps that is the GPS or the gastroschisis prognostic score. And this was really one of the first times or perhaps the first time. Um an attempt was made to objectively define what we mean by complex gastroschisis. So four things were looked at mapping uh Atresia, whether it's absent, suspected or present. And I think that's pretty clear. Sometimes you see that the whole bowel is intact and you're pretty clear that the bowel is, has continuity. Sometimes you see significant matting and you're not sure whether there could be an Atresia or not, maybe there's some extended loops and sometimes you clearly see a cut off perforation, absent present and necrosis, absent present. And this is all defined by the surgeon at the first inspection. So it's not something that is defined, you know, at a five days of age, maybe when permanent closure is going to occur or after the patient has been a in a sil or whatever. So this really does reflect the congenital status of the intestine. Now, the one area that's perhaps a bit subjective is mad and there have been studies done through caps net that looked at the rep reproducibility of what a surgeon calls matting. There are examples, if you go to the caps net page, which is open to everyone, there are examples of what no matting looks like or mild matting looks like what severe matting looks like. So the surgeon can actually go back to the site and uh judge for themselves what they believe the matting is based on multiple um examples. And in the first study using the GPS, it was quite significant. So in patients who had a GPS, more equal to four mortality was actually uh quite a bit higher even in our own environment here. And in patients who had a GPS, more or equal to two, essentially all. But the simplest patients, um all of the morbidity factors were higher. So length of stay was higher days to first enteral feed, the number of days on TPN and the incidence of complications. Again. Um We go back to what, what, what is the reliability? What is the inter rater validity of matting? And these are some of the pictures you see on the caps net uh website or what, what looks like no matting, what looks like mild matting and what looks like very severe matting. And there are definitions of what each of these matting uh um uh descriptions uh are and you see those all on the uh on the website. So I've been interesting about this issue in matting because ii sort of challenge the fact that it really is by itself a an independent predictor. So we look back, we did two studies looking back specifically at patients with matting and we actually found that matting by itself is not an independent predictor of outcome. So we recalibrated the GPS looking at a group of patients who what we called the intermediate risk groups. These are patients who had a score of four because of severe matting but no other abnormalities. And we found that these patients actually are somewhere between the very low risk group that is essentially a gastroschisis score. A GPS score of zero versus the higher risk groups where there was atresia and um and uh necrosis or perforation, which really put the patient very much at the extreme end of the spectrum. So now we do not consider matting by itself really high risk anymore. What is really exciting? And this just came out this week is that uh our uh international fellow, Doctor Botello from Brazil, we've been collaborating with his center uh for several years looking at their gastrosis outcomes and um you know, helping them analyze them and so on. And we did a study actually looking at whether the GPS is just a high resource issue or does it actually correlate also in lower resource environments? And of course, Brazil is sort of a middle income country where the gastroschisis outcomes are somewhere between what we see in very low income countries and in high income countries. And it really supports the notion that gastroschisis is in fact, a bell weather condition that can predict um outcomes for an entire healthcare system. And what we found in the study just published in GPS is that the GPS actually functions very well and actually, it is very predictive also in Brazilian babies in a um high volume um hospital. So why didn't you know, this is a patient that had a few years back? And you this is a baby who actually was one of the few patients interestingly enough, not diagnosed prenatally, you see that this is what you see and it's very difficult to tell what is going on. Is this all necrotic bowel that is just scarred? Is there any viable bowel? Certainly, the color is not good. Uh There was this huge uh mass here. You see, we weren't sure if this was a loop of bowel or an obstructed loop and actually ended up being the lesser sac full of uh fluid and this baby was really treated by patients. We had no definitive evidence that there was necrotic bowel. We didn't know also that there was viable bowel. So we waited, we put the baby in a silo, we waited, the matting was quite severe. Um And amazingly, uh this baby actually survived with no additional interventions and took her a few weeks to open up, but she did open up. She started stooling, we started feeding her, no other studies were done. And this is a picture of her when she was a year old. And this is a picture of her when she was about five. She's done very, very well. So when it comes to complex gastros cases, I think we have to be cognizant that, you know, it's very hard to have uh clinical practice guidelines for algorithms that cover the entire spectrum. Every case really is unique. Uh And therefore my my bias is that these are not, their algorithms are wonderful for patients who have simple gastrosis. They've been shown to hasten feeding to hasten discharge. But when it comes to these complex patients, I really think that each of these is unique and it's very difficult to apply universal algorithms to them. So what about when we have an atresia? Well, we treat an Atresia the same way we would treat an atresia essentially without gastroschisis. And that is we think about what is the best option to establish bowel continuity or conditions for early initiation of feedings, which is what I call the immediate morbidity. How can we preserve bowel lengths and absorb the capacity, try to abort your bowel syndrome if possible, which is really the long term morbidity. And then if there's grossly dilated bowel, what to do with it. And that is really the very latest morbidity that can haunt patients sometimes years later. So one of the things I've had an interested in is timing of intestinal operations because we have seem to have developed a surgical dic that says, you know, if you feel like you're going to operate on a patient with gastrosis, then you, you really have to wait several weeks, you know, four or 56 weeks or even longer, some people advocate. So I've, you know, found in my experience that that really doesn't sort of pan out like usually a couple of weeks afterwards, the bowel is very different, not in all cases, but in most cases. So through caps that we did a study looking at patients with intestinal obstruction essentially that had early surgery within the first few weeks, versus those that had it more than 21 days. And their numbers were not large. Despite using this large prospective database, we had about 26 patients or so in each arm. But there was really no, uh, disadvantage to operating early. And in fact, the patients who operated early had a much earlier start of enteral feeds and TPN duration was significantly lower. So at least from a resource perspective, operating early was really beneficial. And I went back and looked at the literature and it's really quite fascinating if you look at this paper from what, 40 years ago now? And this is like, you know, at the early, you know, the gastroschisis literature really didn't start getting published until the 19 seventies. If you look back at the, the history of publications on gastroschisis, I mean, the disease was essentially defined in the sixties. Um So uh surgeons back then were actually advocated to operate early. And this is a quote from one of these very early papers, their experience concurs with the finding of others that the intestine appears nearly normal within 2 to 3 weeks of primary closure. So at some point, the, you know, the surgical victim changed and instead of operating at 2 to 3 weeks, it was this emphasis to wait and you know, park the bowel for 4 to 6 weeks, hoping that mapping will improve and so on. And I think that's really not supported by evidence. So one of the things we can do, uh maybe one of the few things we can do when we are suspicious that there is an obstruction is to not leave the patient for so long to actually, especially if we're having in an environment where continuation of TPN and support becomes uh very challenging. So, um this is the review article I'm talking about in seminars and some of the issues I looked at uh in terms of surgical strategies are prenatal diagnosis, customized closure. Um In fact, immediate bowel resection and anastomosis may be appropriate in selective patients. Of course, not all patients, but we have to make a judgment in terms again of um are you able to actually run the bowel and define its continuity or not? Uh But the principle in general should be early established with valve continuity. If there's chronically dilated bowel replicated and then early exploration for a persistent obstruction, sometimes again, especially if we don't do not know for sure that there's ania we tend to sit on patients who are not progressing for quite a long time. So how do we predict? Uh yes, are there any patients, you know, we can say before birth if we are in an environment where most of the mothers are getting prenatal ultrasound? Well, people have looked at all kinds of things, you know, I looked at uh extraabdominal bowel dilatation, intraabdominal bowel dilatation. Uh gastric uh dilatation, even the size of the defect, et cetera. And really the one that emerges as the most consistent is the intraabdominal bowel. We always think of what's happening to the bowel. We see that is extraabdominal, but that bowel almost always will have significant inflammation, matting, et cetera. And sometimes it will dilate and many times we will see dilated bowel. As I'm sure all of you have seen, we're really looking at a sigmoid colon that is full of meconium and has just not emptied. And then we, that becomes the dilated bowel that's being seen on ultrasound. So what's more important is the intraabdominal bowel, the bowel that is still within the abdomen and that, um, you know, multiple series looking and analyzing predictors is really the most significant, the most specific sign of a potential complication, a potential obstruction, but more important is the trajectory. So at one point in time finding is far less important, then what we see happening as the ultrasound progresses during the prenatal period. So it's really that trajectory that's far more important than a single finding. And this is a case just to show you what we call the sour grapes because we had seen this bowel here. And you can see that the bowel, this is the exteriorized bowel. You can see that the bowel is sort of thickened and it's really, uh kind of, you can see it sort of hanging on its mesentery and then what we found over time is that bowel disappeared, basically shrunk. And instead we had turbid fluid, turbid amniotic fluid. So this was uh really a predictor that we, we had a significant abnormality. And when we went and delivered this mom right away after we saw this, we found evolvulus with re you see here, a significant amount of the bowel patient still had about 60 to 70 centimeters. So, so at birth, we did the resection and endosy in this case. And then at six weeks, we we did an ileocolic anastomosis and a gastrostomy. And in retrospect, you could have probably done an anastomosis actually much earlier than that. And that baby would have fed quite a bit earlier. Another problem we're trying to predict prenatally is closing gastroschisis. Now, this, you know, ii saw my first one a couple of years into my practice, but it seems to becoming more common. Um And in fact, in our series from California, it accounted for one third of all patients with complex gastroschisis. And I know more and more surgeons are, are seeing this problem and, and what it is, it's really a clinical triad. It's usually a very narrow defect, like a strangulated hernia if you will. So a proximal and distal atresia tend to develop. And if the defect is very, very narrow, the extraintestinal con the extra abdominal contents will, will die, will necro just like any strangulated hernias. Now, our colleagues um in England have done some work trying to predict this and what we look for now are, is really three things. They don't all have to be there. But certainly, um when, when we see this prenatally, we are very concerned about the possibility that there's an impending closing gastro schesis. So those three things are intraabdominal bowel dilatation. And then often because of the proximal atresia at the inlet site, you will see extraabdominal bowel shrinkage or stability. In other words, the intraabdominal bowel will dilate, but the extraabdominal bowel will not dilate but sometimes it will actually decrease in caliber. And here in caps that now we are actually measuring the defect size and this has not been standard, but we're trying to see if the defect size in fact, can correlate. And this is a patient that really taught me a lot. It was the patient with closing gastrosis who had the best outcome I've seen. And that's because the baby was born prematurely at 31 weeks. It was a causing gastroschisis but the baby went, the mom went into premature labor and you can see what was going on. This was the extraabdominal bowel now exteriorized. And you can see what this is actually the bowel at the level of the defect. You can imagine that if this went on, this entire bowel may have been lost. And this patient actually is one of the few patients with closing gastrosis do not have short bowel. And we were able to do an immediate uh anastomosis in this case because the bowel was completely patent and there was no need to park the bowel. We established continuity right away. This is another patient we had recently, which we predicted with ultrasound. And in fact, at birth, again, we induced the mom. We, we uh we delivered her around 34 weeks and at birth, in fact, the the the defect was very small and completely adherent to the mesentery and the bowel. And again, you can see here after this patient was treated operatively, there was no atresia yet, but you can see the noose around the bowel that existed here. So I do believe that with, with more experience in ultrasound, we may be able to salvage some of these patients by delivering them early, maybe one of the few indications for actually early delivery. And this is another closing gastroschisis again with necrotic bowel. And again, the idea here is when you already had this, the bowel intraabdominal and the bowel that is on the both sides of this is actually pretty well preserved because this bowel is all intraabdominal. So there's no reason in this case to leave an athe behind or you know, do a stoma, et cetera. You can actually do uh an immediate anastomosis to establish uh continuity. Now, uh this is, these are patients uh here in the next couple of slides who do not have a complex gastrosis, but, but just to for all of us to be attentive to um what can potentially happen that can mimic that. So this is a baby who was actually placed in a silo by one of my colleagues. And about 36 hours later, part of the bowel became necrotic. And again, all gastroschisis bowel is high risk bowel. I mean, we're already starting with bowel whose vascularity is not optimal. So a second hit doesn't have to be significant. And you can see here that this bowel went on to become gangrenous. Thankfully, the rest of the valve was still uh pretty safe. And in this case, I actually did a resection with a primary anastomosis. Put another side note, let the valve reduce and then close the abdomen. This is a very tragic case that I myself had. That was really humbling. This was a um simple gastroschisis. Uh the bowel, the, the defect was a bit tight, but I felt it was reasonable. I did not enlarge it. We put the baby in a silo and unfortunately, within a few hours, the bowel went on um to Necrose and we lost this baby who who survival should have really approached 100%. So there is a a youtube video and the links here where we talk about kind of the nuances of how to use a silo safely. If you are using silo in your practice. There really are some significant safety issues that are important to be aware of when you put a silo in and you observe uh the battle in a silo. And you know, ii have to say I learned this the hard way. This is another patient with valgus and atresia. And of course, here you cannot just anastomosis to ends. I mean, you can see that valve is very matted. You need to get more information. So here you know, we resect the valves, we leave the valve closed, we study the patient allows us to know what exists distal, what exists proximal. And then we can take the patient and do a primary anastomosis. So another question that we all deal with is when should you explore the patient who has no atresia or maybe suspected atresia who's not tolerating feeds? And here you have to be careful because often we do contrast studies to see if there's a complete obstruction or not, but actually they're not very helpful. I find, I mean, most of us do not want to use barium. So we use water. So a soluble contrast, these babies all have poor transit, whether they have a complete obstruction or not. So often you don't get good definition, you dilute and a severe ileus, severe dismotility can still look very much like a uh like an actual mechanical obstruction. So I really tend to go by what the bowel gas pattern looks like. And if there is an atresia that's suspected at the time of closure, then I will typically explore that maybe at 2 to 3 weeks. If there's no signs of bowel function quite early. If Atresia was not suspected, then we all know that some times ileus will last for quite some time. And I will usually explore that baby at four weeks. But I don't rely on studies as much as really what the abdominal gas pattern looks like. And, um, and what the trajectory of the baby has looked like. Have they been able to pass in stool? Have they been able to tolerate in the feeds, et cetera? So we just summarize some of these points. I think there, there are cases where we may be able to preidentify patients and really offer prophylactic care so that we don't end up with a complex gastroschisis. Cytos have to be used carefully. We should not uh consider immediate or early bowel surgery to be contraindicated. In fact, in, in well selected patients, this can be life saving and, and you could establish about continuity early and then to think about exploring patients early if there is a persistent obstruction. So thank you very much, I'll stop there. Uh Milan gave me half an hour, I think we're right on time and I'll be very, very happy to uh hear your thoughts, experiences and of course, any questions I'm happy to address, um Sheriff. Uh Thank you very much. I will uh share our experience in short bit later. Uh But thank you that was, that was excellent. There are two or three questions and comments. Uh Are you able to see them and answer one after the other doctor? Yes, absolutely. And doctor ac has uh two comments. Then Doctor Lin has a question and I think that doctor is also a question. Yeah. Ok. I'll go and order. Um, so in terms of our prenatal ultrasound, yes, it's all done by the uh what we call maternal fetal medicine specialist or obstetricians who all, uh, a group of obstetricians that only treats high risk pregnancies. So they do have treatment this experience and that we're very fortunate and really sometimes picking up things that are very, very subtle that you would not think would be picked up. So, uh our team for prenatal ultrasound is exclusively our uh maternal fetal medicine, high risk obstetricians. Um Are there any risk factors to complex gastroschisis as opposed to typical? So this is sort of what I alluded to. And that's the problem is that many things have been looked at and there are usually retrospective case series that talk about bowel dilatation and talk about gastric dilatation. Even sometimes, uh people have tried to look at flow in the mesentery. These are all very difficult, but really the one thing that alarms us is when we see progressive intraabdominal bowel dilatation. Uh And especially if the patient starts to develop polyhydramnios, and we really see a trajectory that is driving us towards obstruction and then there are other findings like the turbidity of the amniotic fluid, like the consistency of the extraabdominal bowel. But the one consistent uh sign we look for is intraabdominal bowel dilatation. Um No, we do not induce patients at all. Uh We actually allow patients to go into spontaneous labor unless we are delivering the mother early for one of these indications that uh I mentioned exactly. So for suspecting closing gastrosis or, you know, an atresia with a high grade obstruction, then we will induce the mother and deliver early. But we do not. Uh we no longer, uh we no longer this used to be a practice, but we no longer uh have patients deliver any earlier than we should. Um Well, vanishing gastroschisis, I think this was kind of an earlier name if you will, because it was essentially what I referred to is that the bowel would be dilated and significant and then the bowel will start to shrink and vanish because it would, as you saw in some of these pictures, it would actually lose its volume. So I don't think there's a difference in the two. I think it was just descriptions of what we were seeing and I think that's all the questions I see in the chat. Um K kly, you can ask you a question, just introduce yourself and ask you a question. Um Good evening. Um Doctor Kirsty Pederson, I'm a medical officer at East London for a hospital. For pediatric surgery. We've had two experiences with allograft and closure of gastrosis babies that we've struggled um, to get primary closure for. I was just wondering if you've had any experience and any take home messages or ways that we can improve our outcomes for those babies. Yeah, I think once you need to use that, um that becomes a different kind of patient and really the challenges are, are significant. Now, the only time I've used this, to be perfectly honest are in two babies who also had liver herniation, uh liver herniation is not spoken about much in gastrosis and it occurs in a very, very small number, maybe 5% or less. But when you have liver herniation, these patients really have, um, you know, sometimes it's called um like just a uh absent abdominal wall. It's not just a small defect. Um And you would have to use something like that. So we've used um Alloderm which is a biological uh matrix. It's very, very expensive, but it does not adhere to the bowel, et cetera. But both of those patients actually succumbed. They did not do well, not so much because of the abdominal closure issue. They developed significant liver uh problems. And if you look at the literature, it seems to be a predefined risk. If you have a patient with gastroschisis and liver herniation, they already seem to be predestined to significant um complications, both of the liver itself, uh early liver failure, et cetera, but also significant dysmotility and dysfunction. So that's the only experience I have, uh, have had in using them. But otherwise even the most complex patients, I find that once you deal with the bowel obstruction, um, we've, we were typically able to close them. Um, Kirie, does that answer your question? Yes, thank you. Yeah. Uh, Sheriff, uh, just, uh, uh, briefly sort of summarizing our experience of almost last 30 years is we here in South Africa, uh probably somewhere where Brazil is, it's a middle-income country. So we do not have shortage of resources as bad as, as John Seira in Uganda. Uh But obviously, um we do not uh have in the public prenatal diagnosis. So unfortunately, uh most of our babies are delivered unannounced and many of them um outside our hospital. And uh obviously, there is delayed transfer to our hospital and we still have an unacceptably high mortality and morbidity of our gastroschisis babies. Unfortunately, those who are delivered in our hospital also, we battle with sepsis and our survival is really is problematic. Um The thing, what we sort of I can say happy about is uh my colleagues have developed uh a sort of uh preformed silo into inverted commas, uh which is an impro version because we cannot afford the preform silos. And uh so they have used plastic rings and sutured uh intravenous infusion bags uh of different sizes and we try and use those uh but uh uh I think we still have a long way to go to make our patients uh survive. At, at the first instance. Uh I just wanted to ask you, we have anecdotally observed increased incidence of gastroschisis during COVID pandemic. And we were wondering whether you have any experience or you have heard or about a study, uh whether uh the incidence of gastroschisis has increased during COVID uh era? Your comments? She, that's a fascinating uh question. No, we, we have not seen that in our practice. Our numbers are been pretty consistent over the last three years. Uh And I'm not, I'm not aware if somebody has actually uh even reported this anecdotally. That would be really fascinating. So, no, I'm not. Um one thing I did want to mention about um the comments you just made is I totally agree that the uh silos that are available from the, you know, main manufacturers are really um horribly expensive and unnecessarily. So, but um one thing that is now available that a lot of people are using are the wound protectors. So the wound protectors um that are now available are on the order of about I think 10 to $15. They're, they're pretty, they're pretty inexpensive. The other thing is um to address this Naomi, you know, doing work in um several countries in South Africa, including Uganda has been able to introduce a, a silo that is, that is made in China. Works very, very well, also extremely inexpensive. I think also on the order of 10 to $15. So there are um there are substitutes for these very expensive silos that are made by essentially two companies um in the West. I think the main thing is whether TPN is available or not. I think that really still becomes a huge divider between centers that still can have reasonable morbidity, at least for the patients who are, you know, the simple patients and those who still lose most of them because the, the nutrition is still an issue, especially if you're in a place where you don't have prenatal diagnosis. And um and you're getting patients who already ar arrive at your center in poor nutritional status. So I'm curious, are you, are you using TPN? Is that now routine in your practice for your patients? Uh Yes, sheriff, we have been very fortunate for last 30 years. We have had easy access to TPN. Uh So that's not a problem for us at all. But uh but sepsis is the biggest killer and we just really don't seem to get over it. And initially, we thought that the Children who were born outside our hospital were prone for sepsis. But recently we have observed that even Children who were born in our hospital also, we are battling with uh sepsis. Um So we are trying to address that. Uh but uh TBN, we don't have a problem. We are lucky in that way. Um I see. Doctor Nira Patel is a, is a consultant at Baragwanath Hospital in Johannesburg. So, Nira, any comments, uh any sharing of experience in Johannesburg, you know, are you still here? Um maybe need of has to live. Um Anybody else who wants to ask a question, either you can type or just unmute yourself and ask a question? No, I don't think uh uh ok. Doctor please unmute a second. Good evening. Yes. Um So regarding the risk factors, I was more wanting to know if they have the typical demographics of a pre Gravida young mom or what is, what is the population of these mothers that are giving birth to this complex gastroschisis? Yeah, I think that's a fantastic question because I don't think it's static. You know, I think it's dynamic. So as you know, the, the epidemiology was sort of uh repeatedly described to be a young moms, uh even sometimes before age 20 like you said, premi gravida, uh poor prenatal care. That was sort of the uh you know, the epidemiology of gastroschisis. I think it is shifting. I really think it's shifting. We're seeing more and more uh older mothers. Uh We're seeing mothers who have had three or four completely normal pregnancies. And then that gastrosis baby, the one thing that has been shown uh through caps neck is um smoking and drug use during pregnancy definitely has been shown through caps net to significantly increase the risk. So these are two, you know, modifiable behavioral issues that can affect the risk in our population in Canada. This is very, very fascinating. The indigenous populations are Inuit in three patients from, you know, the the north of Canada, very uh much higher incidence. And of course, there hasn't really been a genetics of gastroschisis looked at as you, as you're aware, but that actually there are groups here that are starting to look at this. Are there actually genetic predispositions in certain populations that might predispose them? So this is work that's again, very dynamic. But uh like in our practice, because we get in Montreal, we get all our northern population. Uh at least 30% of our patients with gastroschisis, maybe one out of every three will be a patient from our uh from our indigenous population. So, again, very dynamic. But I think the the epidemiology is also changing. Um I think what uh our majority of our practice, I mean, my practice 90% is in the state hospital where we service uh poor patients from rural background. And uh we have a a massive incidence of gastroschisis. And if I compare that to my colleagues in private setup, obviously they only uh service about 10% of uh the population, but it's much less. So. So I wonder whether poverty and socioeconomic status has anything to do with these issues uh related to gastroschisis. Yeah, I mean, again, that that has been such a classic um finding. Um uh me and the issue often also was is that like a surrogate for just having poor health care, you know, poor preventive care, poor prenatal care or is it in itself a, a risk factor? And that's why in Canada it's really a, a great place to investigate that we have done it for other diseases like appendicitis, for example. Um because patients all have the same insurance, you know, uh despite having different socioeconomic status, still the insurance, the coverage is essentially the same. So the access to the system at least hypothetically should be the same. So I don't, I don't believe we have actually shown that to be the case in Canada, we have not shown that socioeconomic status actually um is a, is kind of an independent predictor again to be a surrogate for other uh issues. But at least I think it's more related to access to the system and the quality of the prenatal care rather than um you know, belonging to a specific strata. Mm I agree. But Doctor Roen has a question, Doctor Roen, you can unmute yourself and ask the question. All right, I'm moving from Uganda. I work with Doctor Seka. So in our setting, we do not have access to TPN for our Children. And most times we come across Children with complex gastrosis with Atresia, you're able to completely reduce them. And most literature says you go, you actually explore them at the time of closure. They put it at six weeks now in our setting where we don't have TP and these Children cannot survive up to six weeks. What would be the optimal time or the most appropriate time for you to go in and try and fix them? Sorry. II didn't, I didn't quite understand the question. You were saying you don't have TPN. No, we do not have TPN. So we cannot have the chance to wait for that three weeks as the literature puts it, right? That that's when you can go in and operate on the child. But, but so what, yeah, but that's assuming that you know that there's an obstruction. Is that what I'm Yes. Yes, yes. Yes. Cause sometimes you actually observe the atresia the time of putting the cow back. Yes. OK. So here is, and I've, I've had this scenario, you know, I've, I've dealt with this scenario on some of my travels. Here is my feeling is that, you know, you know that the chances of that child will survive are very, very low if you do not, if you're not able to have bowel continuity. So one thing I have done uh is that you're kind of operating blindly in the sense that you don't have contrast studies, you haven't had the luxury of being able to do contrast studies to really know what you're dealing with and so on. But if you know there's an atresia, I would operate very early, uh, meaning within the first two weeks. Now, if you, at the time of the bowel, there are sometimes Atresias with minimal, uh, matting. Uh, amazingly, you can still have a complete obstruction, but the matting may not be severe. And in those cases, what I usually do is just check the bowel at the time of the surgery. In other words, you know, instill saline in the bowel distally follow it through and make sure that it's things are patent all the way down. Sometimes they also put a small catheter in the rectum and connect it to a syringe with 60 CCS of Saline and ask the nurse to go under the, uh, drapes and actually inject so I can distend the colon and make sure the colon is also patent. So those are some intraoperative ways you can make sure you're not operating missing a distal obstruction that of course, will end up causing you a significant leak if the bowel still looks very matted and you really don't feel you can tell what's, what, what's proximal, what's distal. Is there something, um, is there something else going on? Then I still wouldn't wait more than two weeks. You'll be amazed after two weeks, uh, may not still be an easy dissection, but you will actually find that the bowel is much easier to delineate and much easier to understand because especially in your situation, if you wait much longer then I think you're, you're pretty much guaranteeing 100% mortality, the mortality may still be very high with these interventions, but at least it gives a child a chance. All right. Thank you. Uh Thank you for sharing your experience. Uh And we know the great work uh you uh under guidance of Doctor John Se uh Nera. Uh Are you able to unmute yourself and ask the question? Yes, yes, II can now thank you, apologies for earlier. So, so my question is, what are the rates of termination with antenatally diagnosed gastroschisis in your environment? I feel like uh uh like uh prof said in our practice in public practice, we can see that the incidence and the prevalence of gastroschisis is actually increasing. And uh part of that is because in our public health care, we don't have routine antenatal antenatal screening. Well, um I mean, in Canada, the law of the land is that termination of pregnancy can occur at any point up to viability even sometimes uh after 30 weeks. Uh and it, it the law of the land is you don't, you actually don't have to show even a Gena Anoma. I mean, it's uh it's very, very open. Um Now, having said that, you know, our, our role is to counsel patients because we often see patients who we know have very simple uh straightforward gastroschisis, nothing on ultrasound that predicts any complications. And maybe even a couple of ultrasounds have been done, the baby has grown and we're now, you know, well into the end of the second trimester and the parents uh decide that they simply do not want, uh, you know, the burden of having a child stay in the hospital for a few weeks, the risk of something else might develop the risk that the child might need a care. And if they decide that that is their decision, that's, you know, legal and it's done. Um, I, in our experience having said that I think with appropriate counseling of risks and benefits, I would say that and that actually has been looked at, I don't know the number off the top of my head, but it actually is available through the caps net reports which again on the website. So you'll be able to see that. But II believe the termination rate in general in Canada for gastroschisis is, is, is below 10%. I may be wrong on that. Actually, your, your question has now prompted me to go back and look at it, but I believe it's below 10%. Yes. Uh Thank you. Um I think Sheriff, uh I just mention, need to mention to you is it's not all doom and gloom for us in South Africa. Uh We also have some, some, uh, stories which we cherish like in 1996 we delivered a baby in one operation theater and the second theater we could immediately take the baby to theater because in those days, uh if, if we could close, we would close without inserting a silo. The girls uh in the hospital developed necrotizing enterocolitis but did not need any surgery. And how I know about her when she was about 18 or 19. Somehow she got hold of me and she messaged me that uh they said this is how it happened and you were part of my journey. So I think, uh we, we still trying our best and we uh trying to give individualized care. Uh But uh but I think there's a lot of uh things which we can improve in South Africa. I think we are coming to the end of the meeting. It was, it was very nice. Uh And uh you have a lot of research on it and people now will be aware what has been published where to look for it, but just maybe just a couple of sentences, your take home message and then we will say goodbye. II think my take home message in general. This is one of the things I've learned that you try to impress on our trainees is be uh careful of surgical victims, you know, like when something gets hold in our field and we're all not criticizing anybody in particular because we're all part of the process. But once something takes hold, um it can be very, very hard to undo. And so our role is really to question uh often the surgical victims. One of the surgical victims we discussed here is this victim that's very prevalent, by the way in North America of, of operating late waiting many, many weeks, 68 weeks before you operate. And it's a victim that has no, not on, not only is it unsupported by evidence. But if you actually look at at how the literature evolved, our forefathers, our, our our predecessors were telling us not to do that. And then, you know, the pendulum swung. So especially for the young people here when, when you see these uh things taking hold, you know, spend a few hours looking at, is there really evidence behind them or is this just a practice? And I think for me, uh the, the way we deal with these complex patients is one of those examples where it really is about challenging a surgical victim. Yes. No, that's, that's a great uh message. Sherry. No. Thank you very much again for finding time to, to participate in this teaching activity, uh your very busy schedule. And uh and I will, I will share the presentation on various platforms for those who couldn't attend and uh all the best until we communicate with each other. Uh Again, Sheriff, thanks a lot. Thank you so much. Always a pleasure and so nice to see people from all over the world. Yes. No, we are, we are really very blessed and these meetings are are uh yeah, really. We are very proud about it and we learned a lot from the meetings. Ok. We'll walk you in sometime next year again. Absolutely. Have a great day. Thank you, bye-bye. Um In a month's time, uh we will have Doctor Derek Harrison who is a senior pediatric surgeon at Baraga Hospital in Johannesburg. He will be talking about an oncology topic very likely about will's tumor. So see you in a month. Thank you buh-bye.