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Hi, everyone. Uh Welcome to med education. My name is Ging Jing and I'm usually at the support desk. It's really great to have you today for our talk. We are joined together by doctor Isis who will be chatting to us about pediatric abdominal wall defects for around 45 minutes. During this time. Please put your questions in the chat and we should get round to some of them in the last 15 minutes. At the end of the event, there will be a feedback form emailed to you and once you've completed your certificate will be added to your medo account. Um I'm just introduce our speaker. So uh Doctor Aisha is a pediatric surgeon at the Royal London Hospital. He's honorary senior lecturer at Queen Mary University London and the previous honorary Secretary of the British Association of Pediatric Surgeons. He did his training in the UK and now training in India. Sorry, and he now practices in the UK and I'll just leave it to you. Hello, all. Um Thanks a lot for the introduction. Um So I understand that all of you are practicing in Sudan. Um and I can see that um um the concerns which most of you have is monitoring for potential complications, ensuring adequate nutritional support, providing rehab guidance. Um And there seems to be no concern about communications and coordinating with other health professionals, which is a good thing. So what we'll do is we'll talk about the antenatal diagnosis, the perinatal management, the surgical principles in the management, postnatal care and the longterm prognosis. But when we talk about the abdominal wall defects, we are talking about the umbilical hernia of the cord or an example as minor, the exomphalos major and the gastroschisis. So, there are differences between the exomphalos and the gastroschisis. The exomphalos, which is the absence of the mesoderm, the muscle layer in the abdomen, which is covered with a thin sac of the peritoneal cavity, uh lining as against gastros gastroschisis where all the s are outside the abdominal wall through a defect in the abdominal wall. Um The omy happens because of the failure of mesoderm to replace the body stalk. Um which may be because of embryonic dysplasia, inadequate mesoderm development or very tight junction between the endoderm and ectoderm. What that means is that the body has the endoderm, the inner lining, which is the peritoneum and the ectoderm, which is the skin uh there. And the mesoderm then migrates between the two to form the abdominal wall. So anything which disrupts that traveling of the muscles can uh form the eom for this as against that the gastroschisis is um on the right side of the ab umbilicus and there is a rupture. Uh So the thoughts are that it could either be because of abnormal involution of the right umbilical vein. So when right, um um umbilical vein was uh uh involute, uh it opens up and therefore allows um the defect inside the abdominal wall or there was a small amploule which got ruptures and then uh the sac gets absorbed. But irrespective what the, whether it is an exam for this or um gastroschisis, the basics are the same. What we need to know is how is the baby. We need to look at the ABC that is airway, um and breathing and circulation. So we need to look at the respiratory status. We need to give them the IV excess and the fluid. Um We need to look at the gestation and the size of the baby. We should insert a nasogastric tube because so that we can um uh empty the stomach and uh allow the, make sure that the intestines don't get dilated more than what they are before the closure or the treatment is effective. And analgesia if needed, most of them do not have pain at uh the time of the birth anyway. So let's talk about the three different conditions, exomes major. Um So, exomars which is the lack of abdominal wall, uh covered with the lining can be either omy minor, which is where the defect size is less than five centimeters and it contains only the small bottle. Um The exomars are known to have associated anomalies and therefore, we should check for the associated anomalies which are either genetic or cardiac while um and once the baby is born, the midwives or the health professional who is conducting the delivery should be careful about tying the umbilical cord because if you go too close to the abdominal wall, you are likely to um have uh damage the underlying intestines. But once that is done, example, minor is really very simple. Some people even call that as umbilical hernia of the uh of the sac. So the as you can see, it is an umbilical hernia kind of a thing sitting in the umbilicus and you can just close this as soon as you want. Once the child is stable, what we tend to do here is uh once the baby is born, um the neonatologist will insert a cannula, give some fluids, make sure that the child is hemodynamically stable, the glucose is well maintained and then, and the child can does not need to be intubated. We would um go to the theater, I would reduce all the interstim back inside the abdomen. And as the defect is very small and there is only a small amount of um inter which are outside. There is no concern about it being going inside the abdomen and closing the abdomen. So therefore, you can reduce the hernia, close the abdominal wall and then within the next couple of days, you should be able to start feeding the child. And there should not be a concern from that point of view. And example, is minor, um should not have any longterm consequences unless there is any associated anomalies. So if there is any genetic defect or associated cardiac defect, then yes, that can cause a problem. The um the other thing which I would normally do when I'm reducing the um example as minor is to make sure that there is no patent V I duct or there is no other connection. Uh so that I can take care of that. At the same time, most of these Children will have associated malrotation or um rather non rotation and you don't need to do anything about that exam is major is a large defect. Uh more than five centimeters in the diameter and it contains liver. So most of these Children would get picked up antenatal, they would be, they would be uh checked for any genetic concerns. So we would do uh chromosomes um antenatal and uh look for the genetic defect. Most common associated genetic problems are trisomy 13, 18 and 21. They can also be Wiman syndrome. Um so that you need to look for uh presence of any hypoglycemia or uh large tongue because they can have macroglossia or cleft palate. They, there are different variants of the examples um which is either can be hypogastric, which means that it is not at the umbilicus but involves the lower segment of the body. And therefore, there could be associated bladder exstrophy or a hind grade duplication, a colonic atresia or a uropathy. And that you might be able to pick it up incu it or it could be epigastric, which always excites all the pediatric surgeons because it involves ectopia corus and canal sinology, which is basically the defect of the sternum pericardium, heart diaphragm in the abdominal wall. So that's the ph um so uh we should ectopia corus is that the defect is open and you can see the heart amps once the example is is uh major is the baby is delivered. You need to, most important thing is to stabilize. They have a very thin um membrane. As you can see on the, on the photograph, very thin layer of peritoneum and then the liver and the other intestines are exposed and therefore they do lose. Uh a lot of there is a lot of opper losses and therefore you need to give them additional fluids. So on day one of life, usually a neonate would get around 60 mils per kilo and day two, they get 90 then they go to 1 20. But in this case, you need to give them additional boluses of cell line to make sure that they are, they don't get um hypovolemic or hypergly uh glycemic. You need to put uh appropriate lines. Um in UK, people do put long lines and uh I'm not aware about the because I believe Sudan is a wide country and you may have different kind of availability of resources. But um so, but the best thing to do is to put as long a line as possible. Uh If you can get into the central vein uh in these small babies, it is most appropriate because these Children can require long term nutrition. Um because of associated genetic problems. If this has not been picked up and uh not been tested. Antenatal, you can do the genetics uh of the baby and also do an echocardiogram to look for associated serv um cardiac issues. Um Following that, there are concerns about whether you should the uh there is a difference whether you can do conservative line of treatment or you do the surgery um because the surgery poses its own challenges. The it has also been shown that exomphalos has a hidden mortality. Um So in this paper published in 2006, there were 445 cases of diagnostic exus in one center, 56% had abnormal karyotype s and those Children which had abnormal karyotype after the parents were appropriately guided, either they agreed for termination of pregnancy or um or uh the child died antenatal, the uh 30% which the babies which were born um were normal and 60% at least 60% had other associated anomalies. Uh 30% as I said, 56% of the Children had abnormal karyotype. But even after that, there were other 3% which died intimately and there were 31 live births, 14% declined any termination or management. And there were only 55 live births. So you can see that out of 4, 45 cases, only 55 babies were born and they had surgical repair. So there are mortalities which we don't know about because all almost 56% more than 250 patients um died before the delivery. So you need to be aware about that. Um The So as I said, the postnatal care starts with preventing inoperative losses. Um If you are reducing the uh contents, then you should watch for the compartment syndrome and you should be providing the supportive care. Um When we talk about the surgical care, the cover, um we cover this with the antiseptic creams. Uh You can either use Betadine gentian violet silver nitrate or mana honey. Um So when I practiced in India, we were quite regularly using silver nitrate or Gentian Violet. Um but it has been showed that um Betadine has uh concern about the iodine toxicity. Gentian violet can also absorb potassium and that can cause problems and silver nitrate can cause silver toxicity. So, in UK nowadays, the trend is to use Manuka honey. Um so you apply that keep it um and allow it to dry. And the one that has happened, you can then uh they allow the child to grow and then you can close the abdominal wall later on. If you need to. The, if you feel that there is a ability to close the sac, um then you can open the abdomen excise the sac, but you would not be able to, it is very difficult to get the two ends of the muscles together. And that can cause the compartment syndrome. The compartment syndrome is the pressure on the abdominal viscera, uh causing um affecting the blood supply to the uh intestines and also the venous return to the heart and the decreased blood supply to the kidneys. And so you need to monitor these three aspects when you do the surgical closure and we'll talk about that more than gastros cases because um but if you're doing, because if you do example, as major, that is the biggest risk that you have. Once you, if you're doing that, what um you can use artificial materials, whether it is um prolene mesh or whether it is uh any artificial pigskins or um any other material. But if you use any of those, um then the concern which you have is that if it is exposed outside, it will cause infection. And therefore you need to try and cover that with a skin cover. The way you can achieve that is to put slips on the lateral abdominal wall and then slide the mus uh skin over the uh over the patch. Um Once you do that, very likely that after a few days, you may find that sac is still visible and sac comes out. But if that happens, you don't need to worry about it because by that time, there is a protective layer which is well formed. Um And then you can close it, but it will still be like having used um eua honey or any other coating that. So, um coming to gastroschisis, uh as I said, the def it is the defect on the side of the umbilical cord, usually on the right, more than 80% are on the right side. The it has been shown that there is a causative factors like maternal age, a younger, maternal age can cause gastroschisis. Um This is associated also with higher incidence of smoking, whether it is drugs like cannabis or um marijuana, um or chemical aspects of the smoking like nicotine. We check this out and most of them are picked up in uh on ultrasound because the defect occurs in the 10th week of inter uterine life. The we would normally go and counsel the parents at this point, explain them that the signs are outside. Um The and we talk about how the care would happen to these patients after the surgery because when you look at this, the uh the concern which gastroschisis Children have is the uh size of the defect can be small and the intestines are protruding out. So if those uh get compressed, you may lose that intestine and the child may have short gut syndrome. Um part of the intestine which is outside, floating in the amniotic sac can also twist. And if it is a small twist, you may lose a part of the intestines and cause SIA uh or as I said, if there is a hole of the, the spine which is outside this, then you may be left with a short cut. So, but the mainly the gastroschisis usually does not have genetic defects but has associated G I defects. So people have looked at whether you should deliver them at 34 weeks or whether you should. Um So whether you should wait for them to reach down or you should try to deliver them early. Um And what people have said uh looked at is that the by trying to deliver them early, you're not gaining any benefits. But the risks of having a premature baby being delivered out is much higher. So, um therefore, a planned delivery before 30 gestation is not a useful strategy to improve gut function based on this data. So there is no benefit at all, but the risks associated with the premature babies. Um and the management of those babies is much higher uh UK uh does um or has been doing um the bas cast, what that is is that every center is asked for one year starting say today for another year, we um ask everybody to insert their um uh data and send it back to um sorry, uh send it back to the center registry and they would look at these patients and see what happens, what is their outcome. And the Bats does baca study for gastroschisis in 2010. And what they found is that within the year there were 336 operations done for gastros cases. The primary closure was achieved in 196 patients while the stage closure was done in 140 patients. Let's look at the primary closure. Um The patient was taken straight to the theater in 100 and 70 patients. 25 patients were reduced within the ward. I don't know whether you know that there was um a surgeon called Mr Edwin Bianchi in Manchester and he showed that you can reduce the uh sins on the bedside and then you can just close the abdominal wall um by just pulling the umbilical cord across the defect and then just putting a plaster on that. And in one case, they needed to do a primary closure with a patch. Um So 15 of these patients required a pre formed silo um because they could not be completely closed. Um And 17 had custom silo, sorry, four of them had custom silo out of the 25 who had a ward closure. Again, there was a pre formed silo used in uh four patients um while doing the stage closure, the you do the custom silo in 15. So we I'll talk about the silo in a short time. Um 15 of them had a custom silo and was closed. Um One of them required a patch. There was a pre form silo which was made in was used in 120 patients. Of which one required a patch repair. Two of them uh could not be reduced and had a custom silo made and four were closed. So again, when you look at these patients, what you need to know is how is the bowel? What is the color of the defect? What is the size of the defect on the uh abdominal wall? Does it need widening? And is there a thick peel of the cover on the intestines? So let's look at this. Um So this is a very, very thick beel. You can see that the hole of the inter spine is encased in the um in a sheet and you cannot even make out how long is the length of the gut? I'm sorry that these are uh overlap because I converted this into PDF. But you can see that the gut is compromised here. Um And there is a blood supply problem, there's a large stomach there as well. And this is the normally what the kind of gut, which you would normally see. So you need to look at the size uh the color of the gut. You need to look at the if uh continuity and see whether there is any Atresia associated with that. And then you decide what, whether you'll be able to do conservative approach or the um primary closure. So as I said, the, the surgical management differs um in that way, depends on the surgeons because there are some surgeons who believe in immediate surgery and some people believe that you should use a silo. Um silo is basically a plastic bag in which you can put the inter signs. The main reason why people are doing silo is because again, because the all the inter have been hanging outside the abdominal cavity, there's not enough space within the abdominal wall, sorry abdominal cavity. So once you put all the inter sts back, again, you can compress the gut and that can cause a problem. So some surgeons believe that you should put that in a bag and gradually push it back inside the abdomen over the next 5 to 7 days. And once you reduce that, then you can uh then you stretch the abdominal cavity uh to accommodate everything. And at that point, you can go and close the abdomen. Um So if you want to use the silo back, the principles of silo management is to always stabilize the patient first. Um It should be put by a responsible surgical team, the person who understands what the silo it does and you can do a gradual reduction. So let's look at that. So that's the intestines, that's a preformed silo. Um So you start putting the silo in the back, gradually, starting with the, the most distal part of the peak. Uh you put some saline in the back and once you have achieved that, uh there is a ring, as you could see in the ring there, this ring which is here can be um inserted inside the abdominal wall. These flanges are to fix them to the abdominal wall by putting a sticking cluster. And then you hang the uh bag to the top of your um incubator. And what you can see is these are the plastic. Um we, you gradually, what I would normally do is every uh twice a day, we would insert, push in around one inch, one inch at a, at a time. And once it has reached the abdominal wall, you can see the abdomen is now compared to this. Um the you can see that the abdomen is now stretched. And once that has happened, you can then take this patient either to the theater and take the stitch on the muscles and close that or if you got a good umbilical cord, you can just remove this bag, pull the umbilical cord across and just put um uh do the ward closure if you cannot achieve the closure. Because the uh all the intestines are still hanging outside. You may require me to use a patch and if there is an atresia, um or if there's already a dead gut there, then you may need to do a stoma. So those are the risk or the concerns which you have. I have not going into the details of the surgical technique, but I'm quite happy to do that. If you do want me to talk about the surgery more, then please put a uh message in the chat. The once salt intestines have been reduced, then we would normally put a long line and start the patient on TPN. Um Once the intestines have been reduced, there is an increased pressure and there is a blood supply to that might be compromised. As I said, we initially for the 1st 48 hours, we are watching to see if this child develops a compartment syndrome. So we are watching for the urine output. We see if the respiratory uh side is affected or not and whether um the abdominal wall feels normal and there's no redness. If any of these things are happening, you see that the urine output is less than 0.25 or 0.5 mils per kilo and does not affect uh does not improve. After doing the boluses, then you uh need to think whether you need to take the child back to the operation, the uh theater and open up the abdomen and look inside. Um and as I said, this is the way I would normally do, I would do a ward closure. So I get the umbilical umbilicus across and then put the, just the sticking plasters um to bring the edges together. And the good thing about it is that when you do that the child doesn't need to go to the theater at all. And therefore the child is um not required any anesthetic uh from the birth, which may be a beneficial thing. Um for the um for the child, uh if there is an atresia or if there is a shortcut, we may need to create a stoma. The concern with the creating the stoma is that especially if the child has uh the associated uh patch repair, then the chances of infection on the patch can become increased. Um If the child is not able to feed because what we have seen is that a lot of these Children have dys motile bowel and, and uh mean uh duration for them to be start feeding is um around 35 days. So some of them can start feeding on day, two day five. and the others may take up to three months. Um So therefore, um and the the movement going across from the dilated intestines, small intestines which are outside and exposed to the derm as to the amniotic fluid and the colon becomes quite difficult. And therefore, uh if the child is not able to start feeding on uh 4 to 6 weeks time, then we consider doing a stoma. Um There is, as I was talking about the closing cases where the defect is closing down and narrowing down can be a big concern. In this case, you can see that the abdominal wall is completely closed and the in outside, hanging in distance is all dead. As against that. In this case, there is still some viability, there is patchy necrosis in some areas, but the defect is small. And in that case, you need to, first thing which you need to do on the baby is born is to widen this part and make sure that the um pressure on the blood vessels is uh taken away. And it may be, these are the cases where we do consider uh delivering the baby early. So what happens with the gastro's cases in the UK is that they would have an ultrasound every two weeks, we would look at the um we are looking for intraabdominal dilatation. So if there is a dilatation of the gut inside the abdomen, um then we can, we think that there is a possible SIA happening because there's an um the intraabdominal loops will get dilated when the uh blood supply is affected and nothing is able to go through. Um And if you think that there is a compromised blood supply, then you can consider doing an early delivery. Um So as I said, the time to feed has been around 21 days because we are concerned about gastroschisis associated with intestinal dysmotility. Everybody can create such names. Uh Gait was the name given by this authors. They found that the time average time to feed is three weeks, hospital stay was around two months. Um intestinal failure where the child required hos um enteral uh sorry TN or partial nutrition. Um for more than three weeks was in 81% of the patients and unplanned to operation was found in 42% of the patients. Um We also looked at the cost of the managing the gastro cases in the UK. And we found that the average cost per patient is around 30 38.5 1000 lbs in one patient who stayed in the hospital for more than a year for a short death syndrome. The cost was around a million, a million pounds. Well, the hospital usually gets paid only 10,000 lbs per patient. Um but um that's where we go. Um I'm quite happy to elaborate on any side of the things, topics which you want to. But you please put a question in the in the chart um if there are no questions. Um and you want me to uh so you wanted to know about the potential complications of um the abdominal wall defects. As I mentioned, I'm just going through the only pole which we have. Um sorry. And the first question was when encountering a Petric abdominal wall defect, which diagnostic method do you primarily allow on for initial identification and differentiation. Um So, clinical examination is the most important thing. Um You usually uh when you say ultrasound imaging, I believe you're talking about antenatal ultrasounds uh to pick up the defect and you're absolutely right. We would not do MRI um but it is usually a good thing to do is to talk to these parents before birth. If you have a good link with your gy gynecologist, because it is important for the parents to understand what is gonna happen to this child. Um because I'm sure the cost is a factor. Also, I'm not sure how the Sudan uh geography is, but it has been showed by um um the Global Health side of our British Association Pediatric Surgeons where in the subs Saharan Africa, we found that uh teaching the primary health care workers how to use a Xylo is very important because it helps them in identifying the child with gastroschisis. Um And then transferring him across to where the pediatric surgical expertise lies. So the other thing which I did not mention is that if the baby is born outside the hospital, uh where the pediatric surgeons are there, all you need to do is to make sure that dent the spine is not twisted. So you teach your primary care physicians or uh healthcare workers in India, we have um the Awadi workers. So these are the ladies who go to remote villages and do the delivery. And if they come across any of these Children with excuses, uh then all they need to do is to put the s on the abdominal wall so that it doesn't twist and then just use uh a cling film if it is available or just a um wart wet cloth on top of it, which is clean so that the child can be then immediately transferred off to the nearest hospital. So that is what we would normally do. Um The uh As far as the complications are concerned as I already spoke about that, the complications you need to monitor is the lack of um the blood supply to the interstim, either within the silo or within the um either in the silo or once it is reduced and you're done a primary closure then to look for any compartment syndrome developing um the nutritional part. Um um as I said, the average age at which these Children can start feeding is around 21 days and therefore you would need to have some sort of a uh total parental nutrition. Um And the in gastros cases, once the baby is fully fed and goes home, there is not much of a concern because they have mainly the G I associated problems and therefore they should not have any major issues. Um But you need to warn the parents that this is going to be a long stay. Um, the Children with the omy would need um uh genetic testing as well as the cardiac monitoring and their longterm uh effect will be decided by their uh support. So that's where we are. If there are no more questions, um then we'll call you today. Thank you very much doctor. Thank you very much for everyone who's joined us. Um Our feedback form will be emailed to you and once completed your attendance certificate will be on your Meadow account and I'm just going to pop in a little link to the next event this evening, which is on knee trauma, if any of you would like to join us there. Thank you very much, doctor. Thank you very much to the attendees. I end the