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Uh, can you see my first sl a month? Sad. Can you see my first light? Yes, I can. Ok. Um, yeah, I think you did just uh um, I just want to make sure. Ok. Right. Um, greetings to everybody. Uh, good afternoon, good evening. Good morning. I see doctor Sabina Siki is joining from Arkansas. Um, so welcome to the Zoom Academic meeting of the Department of Pediatric Surgery in East London, South Africa. And today we have an exciting talk on fetal surgery and exit procedures. And uh the speaker actually in South Africa doesn't need any introduction, but for those who are joining from outside South Africa. Professor Sad Sheikh is the current president of the College of Pediatric Surgeons of South Africa. He did undergraduate training and specialization in general and vascular surgery in the University of Kwazulu, Natal. Subsequently, he did uh what he calls Mini MBA from Manchester Business School and he received Gati International Fellowship which allowed him to gain experience in Tufts University, Harvard University and MIT in Boston in USA. And uh he was exposed to the fetal surgery as it was starting uh in the USA at that time. After he came back, he did specialization in pediatric surgery. And I think he must be the only pediatric surgeon. At least I know who has fellowships in vascular surgery and pediatric surgery. Subsequent to that, he did a fellowship in fetal and neonatal medicine in France and in Belgium and, and sorry. And his current affiliations or, or past and current affiliations are that he has been a former head of division of pediatric surgery and associate professor at Stellenbosch University in Cape Town, South Africa. He still maintains with the department of pediatric and general surgery and fetal medicine at the University of Kwazulu Natal. He is the treasurer of the South African Association of Pediatric Surgeons and he is the current president of the College of Pediatric Surgeons for 2020 to 2023 cycle. And uh he has been instrumental in implementing the radical shift in the way in which we conduct the fellowship exit exams in South Africa. Um and I have been fortunate to be working under his guidance as honorary secretary of our college and we are really pleased the way in which we have maintained quality and uh converted the written exams to a single based answer, multiple choice questions and the orals have been changed to zoom based orals due to COVID. So sat, uh thank you for agreeing to share your experience about fetal surgery and exit procedures. I will stop sharing and then you can start sharing in your talk. Thank you. Uh Thank you, Melinda for, for that kind introduction. I hope my talk will stand up to the sort of expectation that you have uh set the audience up for. Um what I over the past couple of months, I have sat in for a few few postgraduate uh presentations by the registrars and as well as visiting um staff at Red Cross and at some of the other centers in uh South Africa. And uh having listened to these talks, uh what I've realized is that the presenters and a lot of the members of the registrar party are very knowledgeable of the um literature and what the literature has to say about this particular topic. However, the literature in this topic tends to be a little bit sparse in terms of detail in terms of how people can get involved, what they can do. What is the um the actual indications for certain procedures, what we should look for? And the practical aspect seems to be more limited in terms of what uh the knowledge is in general. So what I thought I would do with this topic is try and focus more on some general principles in terms of how people can put into practice. And if they are going to put some of the aspects into practice, and I think the exit procedure is probably the most important um aspect of this talk. If they are going to put this into practice they need to have some sort of a background and what are intent sharing are the actual practical issues in terms of how we implement uh the principles of this uh talk. So, although I will touch on some uh literature based uh support for interfering with the fetus prior to delivery. Most of the talk, I will hope will be try to give you a practical uh scenario in terms of how to approach any involvement that the uh members of the audience wish to have in this particular area. In, in majority of uh situations, there's always a little bit of history that you have to look at. And in terms of the history of fetal surgery, South Africa has a very sort of proud history in terms of development, both from a pathophysiology point of view, as well as an interventional point of view when it came to pediatric interventions. When the fetus many years ago, uh Professor Chris Barnard and Professor Low were doing uh research in the mother city when they were trying to identify what the causes of interstressor, trying to identify where the actual pathophysiology uh started and how it progressed. People across the ocean in the United States were watching carefully and were paying attention more to the actual processes that were involved. And the process that was involved was the operation on a Gravid uh dog delivery of the pups that were present, the fetal pups that were present surgery being performed on these fetal pups. And most importantly, these fetal pups were then returned into the gravid uterus and delivered or rather sacrificed a couple of weeks later to see what had happened with the intervention that was being performed. The aim of this is not just go into the interventions that were being performed at that time, but to just bring to your attention that the people across the ocean in the United States that were watching, this were most impressed by the fact that a gravid uterus could be operated on a fetus, could have some sort of intervention returned and delivered at a later stage. And one of the people watching this was Professor Michael Harrison, who then subsequently used this to uh develop his interventions by performing open hysterotomies and operating on the fetus. Professor Chris Bonner, as we all know, then left uh the University of Cape Town and went across to the United States. And at that time, the knowledge that he had was shared, even though he went across there to develop his interest in transplant surgery. The other factor that has helped to reach the the stage of where we can intervene in fetal pathology has been the massive advances that we've seen in imaging and this has been going on for a long time. Initially, the ultrasound was a very useful method and now we have progressed to using uh magnetic resonance imaging. But even with the imaging modalities that we have, in terms of the um radiology side, there have been a number of other aspects that have developed in terms of genetic testing, chromosomal testing, which can be performed on uh fetuses at a very early stage stages. What has helped mostly uh though is that the ultrasound has helped us identify the natural history of the disease. So initially, it was used to identify the disease then being relatively noninvasive and with minimal known side effects or complications, it could be used fairly frequently afterwards to follow up the fetus in which an anatomical abnormality was identified and used this to outline the natural history of the of the uh disease process. The classic example in this is the congenital diaphragmatic hernia. The radiologist would make the diagnosis and then as they followed these babies up, they realized there was a significant hidden mortality and at least 60% of these fetuses didn't make it uh to term. They would also be able to, they looked at the number of other other pathologies that were easily identifiable, such as the renal pathologies. And uh they could follow this up. What it allowed us to do once we were able to image these things and understand the natural history to a certain extent was that it gave us the opportunity to counsel patients adequately to give them an idea as to what is going on with the baby and what the likely outcome is going to be, it allowed us to offer the uh parents the different options in terms of how the pregnancy itself can be managed and whether the pregnancy would be allowed to continue or whether there was a good case to be made for interrupting the pregnancy. In some cases, very infrequently. But in some cases, it allowed us to intervene antenatally. And there are a number of different ways to intervene antenatally. Although the focus of this talk is mainly invasive and uh surgical. There are a number of medical techniques that have been used for a prolonged period of time, such as for example, uh the administration of uh thyroid uh hormones to the mom, the management of uh hypothyroidism, which can affect the fetus, the use of steroids, which are given to the uh mother to promote for improvement of the lung development in the premature baby. And a number of other medical interventions that we do have, which are used quite frequently by far. The most important aspect though that uh involves a pediatric surgeon is in terms of planning the delivery and this refers to where the delivery should be performed. And in terms of how the delivery should be performed. Knowing that the options of normal vaginal delivery and cesarean section have to be considered the important aspect of this is that knowing what is going on before the baby is born can be extremely emotionally taxing on the parents and uh the physician as well. And this is a double edged sword because if the parent does not have the option of performing any antenatal intervention, then antenatal screening does not meet the criteria that are required for any form of screening. In this case, we tell the parents that the child has got this particular abnormality and is going to be delivered in nine months time, the parents do not want to intervene and this can cause significant emotional, psychological and often marital strife uh that we see fairly frequently. So one has to think carefully about imaging and antenatally identifying pathologies in terms of how this will be discussed with the parents, taking into account their cultural and social circumstances and whether an intervention will be offered and uh or or not, the important thing about this is that it then brought into focus the approach that we need to look at the fetus as a patient. I mean, currently, this is a huge discussion point in um the United States. Uh as many of you would have seen in the, in the news, there is a number of different factors which are coming up there. And people who are actually following this will have makes it makes for quite an interesting discussion. But once you start seeing the fetus as a patient, what we've got to remember is that we are now giving that fetus a particular status both legally and medically. And this is something that has becomes very important in terms of how you determine what your interventions are going to be. So for many years, people thought the fetus was just there, it did absolutely nothing. It was placid. But what has happened over a period of time is that we have recognized that the fetus actually directs the entire pregnancy and can have a significant influence on maternal health. And this is an area in which we may have to consider the fetus as being a patient. And an intervention that is being performed on the fetus to try and protect uh the mom. When we look at the fetus, we didn't realize as a patient, we realized that there are a number of different doctors involved. Pediatric surgery is in itself a very uh vast general surgical discipline. And we are involved with a number of different uh specialities. But once the fetus is involved, there are additional people that become important in these discussions with the obstetrician, the maternal fetal specialist and the sonographer, you have to have the geneticist and the perinatologist involved. And from our perspective, I think the pediatric surgeon plays an important role in terms of leadership when it comes to the development of pathology. As we as, as we have developed our speciality, we have to realize that sharing the information that we have in terms of the postnatal outcomes in these patients and, and their management. It's important for us to be involved in the antenatal counseling as well rather than leaving this to the uh maternal fetal specialist or the obstetrician as their knowledge in terms of the postnatal processes are usually limited. So, invasive fetal therapy, which differs from the medical type of therapies. For example, the uh intramuscular steroids that are administered to, to mothers at risk of pre time labor or the steroid uh medication that are given invasive fetal therapy refers mainly to interventions that will breach the uh uterus and enter the amniotic cavity. So, there are a number of different indications for it. Uh in general, there are indicators for fetal anomalies that might cause fetal death or significant problems if allowed to persist. But the proviso being that when it is corrected, it will allow for normal development. The conditions must not be uh incompatible with life. Obviously, there mustn't be any other life threating abnormalities and chromosomal and genetic conditions are usually a contraindication to any sort of invasive fetal therapy. There are a variety of different types of uh invasive fetal therapy that are available and uh there are a number of indications and uh for them and the rationale for performing this surgery, the majority of them have remained the same from the time the initial intrauterine transfusions were performed. But as time has progressed, the actual techniques have changed a little and uh the procedures have become progressively less uh invasive, the list on the slide and not something that you need to know. But it's just to give you an idea as to the vast spectrum of diseases. Although extremely infrequent, that may allow intervention on the fetus with an with usually with a reasonable outcome, the evidence for fetal interventions, something that we always look at something that is always um support is always needed for. If we look at two of the major random controlled trials that have been performed for conditions that we are all familiar with. The one do Meningomyelocele trial. This is one of those rare examples of where a trial, a randomized controlled trial was performed within the pediatric surgery arena in such a way that it was considered or sorry, I shouldn't say pediatric surgery but any surgical arena where it was considered to be the best randomized controlled trial for that particular year in the entire world. So these trials are are can be done and they are occasionally performed very effectively. But both the moms trial, which is the Meningomyelocele trial and the uterine interventions for congenital diaphragmatic hernias were both stopped early because a clear advantage was shown for both interventions. The important thing about this is that in terms of surgical interventions, they are not always possible to continue trial for a prolonged period of time, especially intervention show a result fairly early on because the difference between a poor outcome and a good outcome is very obvious as compared to medication related randomized controlled trials where percent, a few percentage points uh are considered important. So there are a number of procedures for which trials have been conducted. Uh This table just gives you a brief idea of some of the trials that are there and I will just briefly mention some of them early. Uh as we go along to give you an idea as to how you get involved in um in these patients. I will just briefly discuss a short case of a patient where a fairly simple percutaneous procedure was required a procedure which the majority of us should be familiar with. And in terms of how the the outcome influenced uh sorry, the outcome of the procedure influenced the pregnancy. I think an important aspect about this is that when you look at the fetus, we have to go back to our basic sciences and understand the pathophysiology of the fetal uh of the fetus itself. It's fetal circulation, it's cardiovascular mechanisms. And in the very same way that you require the this basic anatomical and physiological uh understanding. In order to perform pediatric surgery, you have to understand your uh people or your antenatal history in order to be able to uh intervene. Um Doctor Kumar was uh please mute himself if possible. Uh So we had, we have this uh was faced with a 20 nine-year-old lady who had was generally well, but she had a twin pregnancy a year prior to uh being seen by us. And at that time, she had a, a preterm delivery at 24 weeks both babies, unfortunately, or as expected uh demised. And this was after having tried to fall pregnant for about six years or so and having undergone multiple different interventions to fall pregnant after these babies' demise, they were obviously quite sad. Uh The tattoo actually is off these two little girls. And uh the dad assures me that those were the size of the uh uh the feet of both the girls on his hand, They were actual sizes. So when they couldn't fall pregnant again, and after having lost these, these babies, they decided to take a break and as luck would have it, they fell pregnant, she fell pregnant within a year and she had an uneventful pregnancy and went on to about uh 20 weeks when she had a ultrasound scan done and this was reported as being normal. About a week later, she developed a viral illness that resolved fairly quickly. She thought it was just a flu, but she noticed that over the next week or so, she started rapidly uh gaining weight. And by the time she had reached 24 weeks, they were obviously quite concerned of the rapid weight gain. But at the same time, it reminded them of the loss of their twins. So they requested an ultrasound by the obstetrician. The obstetrician performed an ultrasound, he identified a pleural effusion and referred uh the patient for a level two and a level three assessment to the fetal specialist and the maternal fetal specialist saw the child about, saw the mom about a week later. And when he performed his ultrasound, things had significantly worsened. Mom had severe polyhydramnios. The baby had bilateral pleural effusions, he had soft tissue swelling of the upper body. They didn't identify any cardiac abnormalities. But based on this, your diagnosis was of eye drops. Uh The commonest cause of eye drops is usually due to um hemolytic type of diseases that these Children can have. So they looked at for fetal anemia, they looked at the no the middle cerebral artery and identifying a normal middle cerebral artery flow, excluded uh fetal anemia. So, the diagnosis of severe hydrops, fetalis, most likely postviral given the mother's history was made. And the options were then discussed by the maternal fetal specialist with the with the parents. The one option was to deliver the baby. Uh This was obviously not a good option. The baby already had severe high drops. It was only 24 weeks. It was very likely that if the baby was delivered, he would not survive to allow nature to take its course. This meant that there was a 90% chance of uh fetal mortality. And then we thought about the option of actually relieving the pleural effusions in as we would do if the baby was born postnatally with bilateral pleural effusions. And the reasoning behind this, I will uh discuss on the next slide. So we had a long discussion uh this was when I was consulted and we had a long discussion with all the people that were involved for a couple of which took a few days. And during this time, we had sent off specimens for uh a number of different um studies, vital studies on the amniotic fluids, including genetic uh studies and uh fish analysis. This is for the registrar to look at and this child clearly, this is an ultrasound of the baby, clearly shows all the features that are required to diagnose hydrox fetalis, the soft tissue thickening between the chest wall, the pleural effusions that can be seen with the heart in the center. And uh once we had looked at this picture and made the diagnosis, a repeat scan after about 48 to 72 hours showed progressive eye drops and edema. By this time, we had had the initial analysis back which were both normal and very importantly, if you take into account the enddiastolic volume in the umbilical vessels, these were absent and this indicated urgent or was an indication for emergency delivery because it suggested that the fetus was unlikely to survive uh for any length of time. So given the overall prognosis, now being dismal and uh our options of uh delivering the baby or leaving the pregnancy to progress. Having a poor outcome, we had a discussion about what the likely cause of this would be and we thought that it would be postviral, but the chances of it actually resolving were very slim. However, the there is the possibility that it could worsen and it could have major congenital anomalies and death. And that's typical of a postviral type of uh of uh pleural effusion and high drops. However, there was an alternative course to consider in this child and that we thought as a primary chylothorax and the primary chylothorax could have resulted in the number of complications that we were seeing in this uh child. So fetal chylothorax is uncommon. And the reason we thought about this was because all our viral studies and everything else had come back as normal. And uh the we had tapped the pleural fluid which showed quite a high lymphocyte count. So untreated, a fetal child has a high mortality. But if it is treated or if postnatally, if the baby delivers and there is a rapid, fairly rapid insertion of chest drains, the ventilation may improve if the child has good residual lung tissue and there are a variety of different causes uh for this, I'll just skip those causes for now for for the sake of time. But understanding the pathophysiology of how a child develops, uh eye drops is very important because a number of solid and cystic lesions of the lung can contribute towards this. The number of intraabdominal conditions and even some intracranial and other pathological conditions in the fetus which are relevant to us can contribute to the development of eye drops. So if you understand how eye drops develops in the fetus and in terms of the danger that it uh poses for the mother, then it will give us some idea as to why interventions are are necessary in these Children. So the actual, the overall problem if we look purely at uh the child who has a primary portal effusion, the pleural effusion results in cable compression, the compression of the cava results in decreased venous return a decreased cardiac output. You get increased interstitial flow and that worsens the uh pleural effusion like you would in any adult patient who had um with congestive type of cardiac failure. The important aspect effect about this though is that there is always some degree of uh placental compression that results from esophageal compression. So if you get the pleural effusion, besides the cava being compressed, the esophagus is compressed, the baby is unable to swallow. He develops polyhydramnios and that results in placental compression. Placental compression results in the release of a number of vasoactive substances which act both on the baby causing a decreased cardiac output. And at the same time acts on the mother by entering a systemic circulation and giving rise to what is called the maternal mirror syndrome where which now endangers the mother. So fetal eye drops and a hydropic placenta can result in a maternal mirror syndrome which puts the mother at risk. This is usually seen by the rapid weight gain that these patients have and later on as the disease progresses, they develop peripheral edema and they develop cardiac compromise, which becomes clinically relevant with a very poor outcome if not managed early. So, what we opted to do after discussion with these parents uh was that we would, we would do what we would have done in any patient with a pleural effusion postnatally. And that was to actually drain the fluid out of the uh pleural effusions. And uh there are a number of indications for inserting a shunt antenatally and all of these are related to pleural effusions. But the pleural effusion can result either from thoracic cystic lesions from congenital uh adenomatoid malformations. Although this is becoming less of an issue. And in some cases, pulmonary lymphangiectasia and pulmonary sequestrations can also result in a pleural effusion and the instruments and equipment that you need are fairly similar to what we are used to which is a trka, a needle, uh trer and a needle and a shunt. These shunts are specifically made for babies, very soft material. They are about 1.9 millimeters in diameter to two millimeters in diameter. They are two different types that I am aware of that are available and they are a double pigtail type of the shunt which can be inserted so that it remains outside the uh fetal body cavity. The actual procedure itself is often performed under local anesthetic as this was the first procedure we had performed. And due to maternal anxiety, we did this under general anesthetic, the needle itself is about a three millimeter in diameter needle with the what 2.5 uh diameter central channel and a 0.4 millimeter injection site. Alongside this under ultrasound guidance. Local anesthetic is uh given the needle is inserted through the abdominal wall into the uterus. And you will see in this particular picture that what we once we've entered the pleural cavity, then the uh stent is introduced under ultrasound guidance through the actual trka, there are markings on the TRCA and the needle lens are different. And the double J which is the distal part is inserted into the cavity. And the distance between the two varies between 1.5 and 3.5 centimeters between the two pigtails. Uh The distance is variable and then once this is deployed, the needle is removed from the pleural cavity and the needle is removed from the pleural cavity. The second part is deployed outside the chest wall due to the diameter of this and the size of the actual holes in the shunt, the pleural cavity slowly decompresses. So you don't have a rapid decompression of the pleural cavity. It slowly decompresses over about 24 hours or so to allow the baby to compensate for this decompression and the lung slowly uh expands. So this was performed in this child. Uh These are just the pictures showing you the placement of the shunt, which is fairly easy to see on the uh ultrasound. It took us a very long time. Uh But it was an uneventful procedure. We combined it with an amnio reduction. Obviously, we have a maternal fetal specialist present with us so they can help us from the obstetric side as well. Uh In this particular mom, there were some contractions. We used um medication called Actos ban. It's important to understand and to know what the different types of tocolytic agents are if you are going to be involved. And uh we had quite a good outcome as can be seen in this uh particular patient event came time to deliver this baby. And what we've got to realize now is that delivering this baby is a bit of a tricky situation mainly because we have a tube sitting in its pleural cavity which is open to the environment. So if we deliver this baby and allow the baby to cry and take a deep breath in, and then look, uh by the time we get the baby across the recess trolley and things like that, it will suck in a whole lot of air into the pleural cavity. And then we will develop a tension pneumothorax which will result in a compromised baby post uh delivery. So it's very important to clip the actual shunt before the baby is allowed to take the breath in and then to continue with your procedure. So overall, once you now identify the fetus as a patient. We've got to look at the different aspects in which we the different ways in which we can intervene in these Children. Very broadly. There are categories that uh we can use. The very broad categories that we can use are the fetal image guided surgery. This is effectively percutaneous ultrasound, guided surgery. We could then use a camera and a telescope inserted into the uterus. So we can visualize what we are doing. And these are the fetal endoscopic procedures. Very rarely. There are some patients who would require open surgery and uh hysterotomy. And most importantly is that we could perform procedures at the time of delivery before the uh fetus is disconnected from the placental circulation. And these are the exit uh type of procedures I shown the slide earlier and uh showed you the evidence for different forms of fetal therapy. What's important though, before we actually get involved is to try and decide now how, how necessary are these fetal interventions going to be? I mean, is it really something that we are going to be able to intervene in how, what is the caseload? Does one country need 25 different uh centers to be able to do this or is one center enough in the western world? We, they probably have access to a lot of this information. We don't have access to the type of information we require to estimate the burden of disease. Uh congenital anomalies are not all notifiable. So we don't really know what we are seeing in terms of uh the entire country. So what I did was look briefly at one of these articles, we looked at late termination of pregnancy reported from one of our centers and the uh provisa here was that by looking at late terminations, the understanding was that these patients who probably had major anatomical abnormalities, uh that required termination rather than chromosomal abnormalities or major fetal anomalies, which would result in early terminations or early spontaneous abortions. So, we used this and uh looked at their figures and worked out that there was a very small number of cases, although this was just from purely one province in that province, we estimated that there would be about 50 to 60 patients per year who may benefit from uh Trump, some sort of intervention, looking at the different maternofetal clinics and looking at the total number of terminations of pregnancy per year that is performed in South Africa. And that's, that's quite a large number over 90,000 per year. And that was in 2013. And using an estimate of about 4% of livebirths having major congenital anomalies, we sort of estimated that the total number of patients countrywide that would may benefit from some sort of intervention would be uh close to about 250 or so. Uh patients, once you've decided that you are going to offer some sort of fetal uh intervention type of procedure. And as I mentioned earlier, I believe this is the uh role of the pediatric surgeon. And this is something that we need to take a leadership role in where possible you have to develop some kind of a strategy in terms of how to get involved. And I think the most important a relationship that you can develop here is with the maternal fetal specialist. You've got to make sure that you get to know them well. You've got to be involved in these patients in terms of the antenatal diagnosis. This is the only way to ensure adequate quality of care. And then once you've done this, we should then aim to become the actual fetal maternal specialist because the maternal fetal specialists are involved in looking after the moms and they do know a bit about the child. But I think we know a lot more about the fetus. The important thing about this is that we should be able to offer an alternative intervention. And then once we have done this, start off using established interventions and then slowly see if there is any need to formulate your own type of interventions. And whilst you are doing this to audit and review uh what you have done. So just briefly to go over some of the different types of procedures that we have and the different areas that we can be involved in the fetal image guided surgery. I've mentioned earlier on, this is the least invasive with the lowest risk of amniotic injury and labor. And they can either be diagnostic as is often done or they can be uh therapeutic. From the therapeutic point of view. The uh areas that the pediatric surgeons should be in can be involved in uh in terms of the lower urinary tract obstructions. The important thing to remember about any lower urinary tract interventions is that the aim of the intervention is to improve pulmonary growth and to prevent pulmonary hypoplasia. The people often tend to focus on the actual relief uh of the urinary tract obstruction and the drainage of the urine and in terms of preserving renal function. But the most important uh factors here are actually extrarenal and related to part to ensuring that the lungs do grow and that uh limb abnormalities uh are prevented. They have shown that there has been overall improvement in survival, but the renal improvement has been variable and there are a number of different techniques and the technique that has been shown in the video clip, it's very similar to the insertion of the uh pleuroamniotic shunt and it is under ultrasound guidance that the bladder has been cannulated. In important aspect here though is that the bladder uh the is the sorry, it is the problem of the oligohydramnios and you may have to do an amnio infusion to allow access and allow space for the uh shunts to be replaced with, I very rarely do these anymore. And if we do get a child for intervention with lower urinary out of the obstruction, I tend to do a cystoscopy and a laser ablation of the valve simply to try and improve the uh bladder outcome later on fetal cardiac interventions, very limited. But it is certainly a role. It's certainly a condition where there is a role to play. The most important being in the patient with the aortic stenosis and hyperplastic left ventricle. And in the this little clip, you can see what, what is being done is that the needle is passed through the rib cage and then it penetrates into the um heart itself into the left ventricle. And from there, we pass a little guidewire in through the aortic valve and through that, we pass a little balloon and uh basically use uh inflate the balloon and then pull this across the valve, effectively doing a uncontrolled valvuloplasty, which then converts, you know, ventricular cardiac function into biventricular cardiac function. These patients still need intervention in the postnatal period. But overall, there is some improvement in the postnatal care because they now have biventricular function during the intrauterine uh period. Uh We also use a 1.5 millimeter needles and the hole in the uh myocardium actually seems to close up uh spontaneously without a problem. Once we introduce an endoscope into the procedure, it allows us to visualize the placenta and it allows us to visualize the fetus when it comes to the fetus, we can uh do things such as am uh release amniotic bands. But the main use of uh endoscopic procedure are for the management of 2020 transfusion syndrome where in a set of twins, there are anastomosis between the blood vessels of the two of the twins and effectively blood is uh stolen from one twin and used up by the other twin resulting in one twin who is very small and now the other twin who is very large. So the larger twin develops higher uh fluid overload and cardiac failure on that basis. And the smaller twin is effectively robbed of his circulation. What is done is the endoscope is passed and as you can see on the picture, the umbilical cord is followed up to the vessels looking for the anastomotic area and this runs in a longitudinal section across the placenta. And then the laser is used to coagulate the vessels alongside this plateau. You can differentiate the arteries from the veins by looking at the uh coloring of the blood vessels. The laser works by coagulating these vessels just on the upper surface of the placenta and not dividing the placenta physically, but the circulation is then separated between the twins and uh the twins usually improve uh over a period of time. The other use of the fetal endoscopic procedure is in terms of tracheal occlusion for congenital diaphragmatic hernias. This was another trial that was uh stopped early. Uh mainly because of the, it was shown to be useful in Children with uh in fetuses with severe left-sided diaphragmatic hernia using a number of different scoring system. And uh what the uh total trials showed, which is the trial that looks at tracheal occlusion for congenital diaphragmatic hernias showed that uh survival to hospital discharge was improved if an antenatal intervention was performed. But there was an increased risk of preterm, pre labor rupture of membranes and preterm birth. As is our nemesis with all the other fetal conditions that we are involved in the procedure is performed. If you look at this little video clip by uh under ultrasound guidance, uh Trka is passed through the skin into the um uterus. We were very fortunate in this particular child that she was look, it was, the position was perfectly correct. Once the Trka is passed in uh a 1.9 to 2 millimeter Fosco or even up to a three millimeter Fosco is then passed through, it enters the mouth of the child. The tongue is displaced anteriorly as this, the tongue being moved out of the way and then you enter. Now, the trachea we're getting into the trachea. And once you are in the trachea, we deploy an embolectomy balloon, which is used by the radiologist. It's quite a clever little idea. The uh catheter is passed in the balloon then passes into the trachea, it is inflated with a syringe and a little electrical current is passed through that little orange bit that you can see. And there is a little wire which connects the uh the balloon to the rest of the cannula. And then once that electric current goes through that melts and then the balloon is uh deployed. The idea behind this is that the occlusion of the trachea results in an increase in the pressure in the lungs from the fluid that is produced by the um lungs. And then that results in uh expansion and growth. It has to be removed at about 34 weeks and then the baby is uh then delivered. Looking at exit procedures. These procedures are interventions that are performed at the time of delivery. It is very similar to a routine cesarean section in some ways, but it's very different in other ways and it is usually performed under general anesthesia. The most important areas that this particular procedure is performed is for establishment of an airway and management of an airway where the airway will be obstructed for a variety of reasons. It was initially used when the trachea was ligated in the very early days in the management of diaphragmatic hernias when the trachea was ligated or clipped during an open procedure and then when the baby was born, obviously, these clips had to be removed and this um trachea digate to allow respiration to occur. And the exit procedure was performed at that time for the diaphragmatic hernias originally. So if we look at the two procedures, there are slight differences between the Cesarean section and the exit. The Cesarean section is usually performed under regional anesthesia. The idea is to look at that baby as quickly as possible and to allow that uterus to contract down quickly for the exit procedures, it's usually performed under general anesthesia. And most importantly, we want to maintain uterine it for as long as possible to allow us to uh to allow us to intervene in the baby. We have about 1 to 3 hours of time to do uh whatever may be necessary. And if you have a still baby, because the anesthetic is changed to allow anesthetic uh agents to pass to the baby and keep the baby um asleep or still during the time that you are performing this procedure. The most important aspect during the exit procedure is to maintain maternal BP, mainly because you want to ensure placental flow. This is a brief description of the procedure and it looks at v at all the different aspects that are involved. It looks at the personnel that are required. It gives you an idea about the maternal anesthesia, which is very important. So you need a team for this how to access the uterine cavity. And uh if we're looking at the exit procedure is obviously through a lower transverse incision because the lower segment is well developed and then tells you a little bit about the fetal exposure and the fetal airway uh management. The way we do it is we have uh two theaters. Uh We duplicate everything because this was for early days and we make sure that we have double of everything. We, the theater is warmed and we are prepared for the baby rather than for mom. We have a very spacious theater because you can see there are two anesthetic machines. We have two ultrasound machines, a cardi, a fetal cardiac echo machine, uh an incubator and we have two sets of stars. Our drugs are all drawn up in a sterile fashion. The most important drugs that we require atropine and fentaNYL because any interference with the umbilical cord often results in the bradycardia and we usually have an adjacent theater prepared if the intention is to try and perform uh uh surgery which is called oops or surgery under placental bypass. We use general anesthetics. So there's transvaal fetal anesthesia. The agents that we use a combination of propofol, remifentanil and desflurane. Once the patient has been induced, uh we ensure that we maintain our BP at a preinduction mean using usually ephedrine or phenylepherine. And then the hysterotomy is performed using a previously a staples, hysterotomy using absorbable staples. This is an important part of the operation because that's where you get bleeding from. And unfortunately, it has become very difficult to get absorbable staplers anymore. The company that was making them has reduced its production dramatically. And during the COVID period, uh we were unable to get them. So, a variety of different techniques have been used. You can use the harmonic scalpel, you can use a metal stapler as long as you excise the area where the metal staples have been applied before. And this should not be a great difficult problem. The reason we can't leave metal staples behind is because it usually results in uh the patient becoming infertile. Once this has been done, it's important that uh the anesthetic gasses are increased and maintains a soft uterus. So the uterus doesn't want to remove the placenta and disconnect the baby. Then we deliver the baby's head and shoulders, we can clamp uh if we have a shunt, as I showed in the picture, we clamp that insert an ivy line, we usually give the drugs preemptively giving um atropine and fentaNYL in the doses that I've uh written up there. And then the baby is intubated. Once that's done, we then just can proceed with our surgical procedure or deliver the baby. And once that's done, then the cord is clamped, the anesthetic drugs are reversed and Oxytocin or any of the other agents are prescribed to monitor the fetus. We use an oximeter. These are actually all come in sterile. We use the neonatal probe and we aim for a saturation of about 60 to 70%. We continuously perform an echo of the baby to ensure that I think that's the most important indicator is the heart rate and the actual contractility. And we have about 2.5 hours. The longest we've been is just about two hours to perform a procedure on the baby. If uh necessary, there are a variety of different things you can do the most important thing to establish a fetal airway. And if you have a chaos or a high uh congenital type of fetal obstruction, you may have to resort to things like direct laryngoscopy. Occasionally, you may have to resect the mass and sometimes uh tracheostomy, we tend to give uh surfactant uh to our patients because we usually, they are usually uh slightly preterm. And it's very important not to give any ventilation and just maintain the airway until the cord is clamped because you don't want to risk uh getting the baby to take a breath in too early. So, if we look at uh one of the fetus that we used, I'll try and go through these things quickly now because I think we've been getting short of time. But in this particular baby, this was the one very similar to the other baby, except that this baby had uh a lesion of the lung, uh specifying lesion of the lung. It was a pulmonary sequestration and he had a major shunt into this actual sequestration which had resulting in cardiac dysfunction in the baby. So, whilst the baby was still connected to the placenta. We throw out the thos clamped uh feeding vessel, managed to dissect and uh break down the actual sequestration and remove that. Um with the skull, it's a fairly big uh plaster around there. But the lesion was actually a fairly small lesion. And these are the uh pictures after the baby had healed, open surgical hysterectomy once again under general anesthetic, very similar to the way the uh procedure, the exit procedure is performed except that there is no lower segment. So it's usually a classical type of Cesarean section or classical hysterotomy that is performed. We only expose the part of the fetus that is going to be operated on. And we, we've got to make sure that you are replacing the amniotic fluid. So there are a small number of conditions for which this is now indicated, mainly the Meningomyeloceles and uh the sacral coccygeal teratomas uh less often for the cam S and I haven't had to use it for obstructive hydronephrosis for a very long time. This is the uh child. Just to give you an idea. The uterus has been opened longitudinally. That's the baby with a Meningomyelocele, baby has been given the uh muscle relaxant and medication. And the only area that like I said is exposed is the Meningomyelocele and that area is operated on. I'm not sure how many of our trainees now go through uh neurosurgery and learn how to do these techniques. But previously, it was a pediatric surgical intervention and it is closed in the standard fashion. Although it is possible for us to now do this brachys cop and uh sorry, uh laparoscopically or minimally invasively and place a sheath over or a mesh over the actual defect to prevent the amniotic fluid from causing uh neural damage. Sacro coccygeal teratoma, a very similar process. But the uh procedure is merely merely to debulk that tumor and a staple is applied across the base of that to limit the blood flow into the tumor and allow the baby to develop. Normally. The number of challenges that uh we face in this particular area is the ethical dilemma that we are faced with where we have to look at female autonomy because it's important that if we consider the fetus as a patient, then the fetus will have some uh rights where if we refuse to uh if they refuse a request for fetal treatment from the mom, because we think the mom's uh not going to benefit in any way. Then this is a form of paternalism uh that we are involved in. Uh the ethics is a huge issue in this. And there are a variety of different support areas that you have to look at in terms of trying to make decisions correctly. There is the risk of maternal and fetal an anesthesia. This is quite difficult, you've got to take this into account and the techniques that you develop in your center will depend on your anesthetist and how you work with them. And there are risks both to the fetus and the mom. And very importantly is the postintervention tocolysis. So things like Ibuprofen, uh which can be administered, it's very safe. It's quite useful and very reasonably priced may not work at times. In which case, more expensive therapies such as the toan may be required and this may not be available at all centers. The risks of the fetus, both immediate and long term. The most important long term are considered to be the neurological a anomalies. And these have been followed up for a long time and a few people who have got long term experience with this, don't think that there is any relationship to the neurological anomalies that are found in these Children and the surgical procedure, but rather to the primary underlying procedure that is um uh sorry, the underlying pathology that's present. And uh the initial thought was that it was the vagal tone and the bradycardic response that these babies had that was resulting in the neurological abnormalities. But uh some of the work shows that this is probably not the case, but it is an important aspect to remember. And the other aspects such as prematurity, p, time rupture of membranes, infections, all important areas to uh be concerned about these fetuses may be superheroes, but they certainly do feel pain. We've got to understand when pain pathways develop. And normally after 24 weeks or so, the perception of pain is very possible. The actual um tracks the spinothalamic tracts which carry pain pathways are there from about 17 to 19 weeks onwards. And uh but in terms of stimulating a stress response, this only usually becomes evident in the fetus after about 24 to 26 weeks. So, pain itself is a subjective uh feeling. But uh we've got to remember this in the fetus by using the stress response. So after 24 weeks, any intervention will require fetal anesthesia and analgesia, there are a variety of maternal risks that have to be taken into account. Overall, it's a fairly safe procedure in the entire trial. I think about 400 mothers or so in the United States who had procedures performed, they did not have any uh maternal mortality. Uh This is a better outcome than in, in liver transplants, for example. But uh overall, it is something that we have to be fairly concerned about because the risks are high to the uh mom. Uh I'm not gonna go through all of these slides, but just to give you an idea as to where this focus of this talk should be. Is that over a period of time in the patients that I have seen very early on the number of patients that were referred to me. Uh Initially, it was about 228 but now more recently, about a couple of months ago, it had gone up to about 605 patients that I had seen because we have a reasonably captive environment here in K ZN in terms of antenatal diagnosis and referrals. But the important thing about this and it's very important for me is that if you look at how it has progressed, once the antenatal diagnosis has been made, the majority of the patients now require postnatal treatment and just antenatal counseling. As compared to fetal therapy, the fetal therapy has not increased in number proportionally to the number of patients that are being referred for antenatal uh counseling. So overall, it is the uh there are a number of different ethical and legal dilemmas that we face. The legal factors are important uh to take into account. Litigation is progressive. We are responsible for these kids until they're about 21 years of age. But now you also face litigation from the maternal side. And uh the expectations need to be managed for these uh type of procedures because they remain still at the sort of forefront of uh medical development and expectations can be uh way greater than uh the the truth is an important thing that has happened with COVID over the past two years is that the number of uh referrals for antenatal counseling decreased dramatically. But at the same time, the number of deliveries in I can talk for K ZN. But there is certainly literature available for the rest of the world as well, that showed that the overall delivery rates also decreased significantly uh throughout the world. This this can be for a variety of reasons, premature deliveries had also decreased. But for whatever reason, there has been a significant decrease in the number since the beginning of this year. Though, the number of referrals to me for antenatal counseling and major congenital anomalies has increased. But even with that worldwide, the number of major congenital anomalies is a lot less than previously seen. And uh conversely, there has been a steady increase in the number of pregnancy interruptions that have been that are being performed. And there are a variety of reasons for this litigation may be a reason. But one has to understand that in terms of global economy and certainly our local economy, young parents being faced with the prospect of 68 weeks, 12 weeks in hospital and then a child requiring long term uh supportive care for some of the major congenital anomalies is an important factor in terms of helping them make a decision as to whether to continue with the pregnancy or not. There are a number of different novel technologies that are now becoming available. There's stem cell therapy, which we can use. For example, the fetuses with thalassemia major where stem cell transplants can be performed and have had some success. Similarly, in some of the hemolytic anemia such as sickle cell disease, there is a possibility. There is a small trial going on in terms of gene modification and uh attempts at correcting this disease prior to delivery. So certainly, stem cell therapy, gene modification are different forms of therapeutic modalities which will in uh which will become more important uh in the future. So there is still large scope. It's an area that is changing rapidly. It is converting surgeons to a little bit of being physicians as well because these are the type of uh procedures they may need to still be involved in. But it is something that keeps pediatric surgery uh exciting. So overall, this is an area that's uh still developing many years as it's progressed, it changes all the time mainly because of the relative infrequency with which you will see Children who require major fetal interventions. But as we expand our knowledge in terms of being able to care for these fetuses and realize that we are partly responsible from the for them way before they are being born, we will probably to be able to understand and deliver better care as uh we uh proceed. Thanks Moland. Uh these are the people who have sort of helped me out with with procedures. I've been doing the majority of our my referrals still come from the rest of the country in terms of word of mouth rather than uh physician directed referrals. So the number of people that know about these procedures is certainly increasing in the country. Uh Thanks Melinda. That's where I'll, I'll stop for now. Uh Thank you very much, sad. Uh This was really a comprehensive review about um fetal surgery, uh uh e exit procedures. And uh I'm so happy and so proud that uh we are able to, you and your team are able to do these procedures uh in our country. So that's excellent. I'm going to invite some comments from uh senior colleagues who are here. Just one question from my side. Why do we maintain fetal oxygen saturation between 60 70%? I'm sorry, that's uh you must remember this is still placental circulation. So it's uh the fetal circulation is only going to be about 60 or 70%. Uh That's the normal saturation in the fetus because it's post placental blood. So we are still relying on maternal um oxygenation via the umbilical uh vein. So it's venous blood that is going into the baby and the venous blood of the mom will contain about 60 to 70%. So that's normal requirements for the patient if we try to increase the saturation by increasing the mom's saturation too much. Because remember that will be one of the driving forces for closure of the doctor's arteriosis and a change in the fetal um circulation initially and then later on hypoxia will result in it being in staying open. So 60 to 70% because the fetus is still reliant on maternal uh supply the baby is not independent yet. Ok, thank you. Um uh I'll uh invite Professor Headley uh to make any comments, ask any questions to prof prof hardly. Uh uh Thanks Melander and Sam, thanks. II, apologize to those of you thought that this was my wife uh watching this uh later. Um II very distinctly, remember when I was interviewed for the post of um head of Department in Durban, way back in 1989 Ronnie Green Thompson asked me what I was going to do about uh fetal surgery. And I said at the time that as soon as we could consistently keep otherwise healthy babies with esophageal resia alive, then I would start looking to increase our patient load by looking at fetuses. And I'm