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Role of Paediatric Surgeon in the management of congenital diaphragmatic hernia” by Dr Sabina Siddiqui, Arkansas, USA



Join this engaging session to listen to Dr. Sabrina Siddiqui, an experienced pediatrics surgeon based in Arkansas, USA, who has significant international experience in countries such as Nicaragua, Iraq, Palestine, Peru, and Kenya. Dr. Siddiqui will be sharing her knowledge and insights on the role of a pediatric surgeon in the management of congenital diaphragmatic hernia, a crucial topic for all involved in pediatric care. She will draw on her expertise in surgical technologies, especially in resource-limited settings, making this session particularly beneficial for medical professionals across the globe. Engage in this exciting opportunity to learn from a thought-leader in pediatric surgery, interact with international colleagues, and enhance your skills and understanding of a key aspect of pediatric care.
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This is the recording of the invited talk on the "Role of Paediatric Surgeon in the management of congenital diaphragmatic hernia" by Dr Sabina Siddiqui, Paediatric Surgeon & Paediatric Critical Care Specialist, Arkansas, USA as a part of Zoom academic meeting of the Department of Paediatric Surgery in East London, South Africa.

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Learning objectives

1. To understand the role of a pediatric surgeon in managing congenital diaphragmatic hernia. 2. To appreciate the challenges and various approaches in diagnosing and treating children with congenital diaphragmatic hernia. 3. To recognize the importance of perioperative stabilization and gentle ventilation in improving patient outcomes. 4. To review the disparities in outcomes depending on the timing and context of diagnosis and treatment. 5. To understand the interplay and impact of pulmonary hypertension, pulmonary hyperplasia, and ventricular dysfunction in children with congenital diaphragmatic hernia.
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The following transcript was generated automatically from the content and has not been checked or corrected manually.

Right? Ok. Um Good afternoon, good evening, good morning. Wherever you are. Uh Thank you very much for joining today's meeting um of the Department of Pediatric Surgery in East London, South Africa. And we are really pleased and honored to have Doctor Sabrina Siddiqui, who is going to talk about a role of pediatric surgeon in the management of congenital diaphragmatic hernia. And uh I'm so glad that we have colleagues who are joining from really all over the world. I can recognize colleagues from the West Indies from India, from um uh Senegal. So we really have uh we are really blessed to have such uh colleagues joining in. Now. I know Doctor Sidiki for last at least three years because we are together board members. And I am a member of the Education Committee of the Global Initiative in Children's Surgery where doctor uh Sidiki is the chair of that committee and she is a pediatric surgeon uh attending as they call in the North America at Arkansas USA. And she has a special interest um in the development of surgical technologies and expansion of surgical technologies in the resource limited settings. And she has had uh experience and has conducted um sort of uh uh outreach programs if I can say that in Nicaragua, Iraq, Palestine Peru, and Kenya. And she says that uh from all these places, she has learned more than what she could give. And uh she is very much interested in teaching and research. So Sabrina, we are really glad to have you. I will stop sharing the screen and you can share and then you can share your experience about congenital diaphragma. Thank you so very much. I am a quite honored to be uh a part of this group and this forum I, you bear with me. We had a call last night and I need to make sure my reflexes are working is my screen share appropriate. Yes, we can see your first light. All right. Excellent. All right. As a doctor Chus was so kind to introduce me. I um I am an Associate professor of surgery at uh the University of Arkansas Medical um school, um which is affiliated with the Arkansas Children's Hospital. We have two campuses in addition to being a professor of surgery, I'm actually a professor of critical care as well. So if our conversation um with diaphragmatic hernias sounds a little bit too geeky. I apologize and please feel free to redirect me to appropriate uh audience. Um I do have some uh interests in medical technology, particularly trying to uh diminish disparities and outcomes by improving technological um uh venues and I also have an interest in, in education as Doctor Chitin was kind enough to point out I don't have any relevant financial disclosures for this group. Um I do have a special thank you and shout out to Doctor Adam Vogel at Texas children's who um from whom I uh heisted quite a few slides as well as from um the not a textbook from uh absa uh resources. So just to launch straight into this, um the interesting and fascinating thing about uh the congenital diaphragmatic hernia is it's, it's not just a hernia. Um As most of the people on this uh platform are aware that uh these hernias come in many forms. The most common of which we see in humans is the bak like variant. So we'll spend a lot of the conversation today kind of focused on those. Um And if we wanna have a discussion on some of the other variants, I'd, I'd be happy to, to have it. Um I just haven't prepared any slides towards that um towards that event. Um So the defect is most of the time left-sided. Um And it's thought to occur through a failure of the fusion of the diaphragm leaves which results in a failure of closing the pleuroperitoneal canal. Um According to the European surveillance of congenital anomalies, the reported incidents um in all pregnancies from 20 weeks on is either 2 to 3 per 10,000 liveborn infants. But as we get deeper into this conversation. We'll, we'll talk about how hard it is to come up with that number in general. Um Through embryological rat models, we find that an altered formation of the posthepatic mesenchymal plate may might be the crucial step that prevents development and proliferation of the peri pleuroperitoneal folds, um which then results into the herniation of intraabdominal contents into the chest cavity and then affects lung development, which we're gonna kinda focus a little bit on how that probably plays more of a role than any kind of surgical intervention we can do in terms of dictating the outcomes of these Children. Um And, and that's we see that in the history of uh of outcomes and mortality for this disease. Early in history of surgeons, we had presumed that the hernia was just a hernia and a anatomic or mechanical defect that compromised the respiratory status of of the Children. And in 1940 when LA and Gross reported their first case series of, of 16 Children um with foramen of bolic defects, they actually stated in their manuscript that a non operative approach was futile and that quote, surgical therapy is the proper treatment for CDH. And that the policy of waiting is responsible for the loss of a great many lives that might might have otherwise been saved. Now, in their series mortality was 44% but these were Children who presented relatively stable to uh doctor Gross over the course of the year since the 19 forties, we've had a lot of medical innovation that has um impacted the care of these babies and depending on where, what context you're working in and what resources you have. Some of these may still not be available to you or um maybe at the tip of your fingers. But um you know, uh the advent and emergency mechanical ventilation, refinement with a neonatal critical care and N and development of NICU, the development of um of Ecma with Doctor Bartlett in the 19 seventies. And they were all critical advances that were a cornerstone of our current management strategies. However, we really didn't impact mortality until we changed our, our methodology in preoperative stabilization and uh incorporated gentle ventilation into our strategy. So what we did was we kind of turned what Doctor LA and Gross had said about this being a surgical emergency and decided that actually the surgery wasn't the key factor here, but perioperative stabilization as well as being very gentle in type of ventilation, we do so as to not incur any damage on already compromised pulmonary vasculature. Um And in that, and with the induction of those two key features, we were able to impact mortality and reduce it by over 15%. Um And that, that is probably one of the key takeaways from our conversation today. Um The future has some opportunity and there's some exciting things on the horizon but hasn't made it mainstream yet. With uh tracheal occlusion balloons um which we can talk about some more if, if uh people are interested in that. Um So to look at that timeline, you know, we might say, oh, well, you know, that mortality impact isn't so huge. But one of the challenges when you discuss and study CDH is that there's a heterogeneity in the population, the disease presentation and the surgical approach varies depending on how and where the child presents. Um And that then affects your outcomes in the US. Fetal diagnosis occurs in about 50 to 80% of cases and usually is identified in the 20 week screening ultrasound. And when you are looking at neonatal disease, um whether or not you have comorbid conditions affects your survival. Um and whether or not you're born in house in a in a referral center with all, you know, the glitz and the glamour or if you're born, um outbound. Um neonatal presentation often includes respiratory distress, hypoxia hypercarbia. Many of the babies will require ventilatory support and then what their underlying physiology is is really what's gonna dictate your outcomes. The delayed presentation cases, they can occur any time postnatally and the frequency is actually inversely proportional to age and they have very subtle respiratory symptoms, including cough, tachypnea, exertional dyspnea, or maybe just gastrointestinal symptoms such as really bad reflux um in which an incidental x-ray is found and people are able to make the diagnosis but also um these kids have kind of been tested with time and we know that their cardiopulmonary status is stable enough for them to have, you know, made it through life um without medical intervention. And so they tend to have pretty good outcomes and very low mortality if it if identified and treated appropriately. But the other thing that makes conversation around CDH really um difficult is there is this, this what we call this hidden mortality around it. There's a high number of early deaths before patients can ever make it to a referral center. There is a high number of stillborn or early pregnancy losses that we don't account for when we talk about this diagnosis. Um And really we know that uh that when you start trying to add these, these patients into your numbers, your mortality rates go up because these are the Children who who are gonna be the most compromised. So and look, trying to look at this within um the African or uh context itself. I try to review the incidence and outcomes with within that continent. So there is a paucity of literature out there. Um There are a couple of French papers I didn't review given my limitations and language, but we can notice that there's quite a few desperate reports, meaning like they're very different um both in presentation and mortality in this paper out of Cape Town. Um There were 59 patients including newborns. Their average time to surgery was about two days and their survival was about 43%. In a subsequent paper, out of a dis ababa, they had about 12 patients. These patients were all delayed in their presentations to this referral center and they have had an 83% survival rate. So again, speaking to the fact that if you present delayed, you probably have pretty decent cardiopulmonary physiology and have enough um enough reserve to tolerate an operation. But one of the things they commented on here was the fact that, you know, 50% of these patients were misdiagnosed prior to their referral because that diagnosis of a delayed presentation can be quite difficult if you're not looking for it. And then this paper out of Nigeria um had uh a mixture of patients both neonatal and delayed and they found 100% survival within um the the four delayed cases. I apologize. This slide isn't um pulling up the way I thought I had made it. Whereas the three neonatal cases, they had a 0% survival. Again, speaking to the fact that um depending on how you present, um and what kind of cardiopulmonary physiology you have that, that will determine your outcome more than the skill of your surgeon or the operation performed. So the skill of the surgeon is such that we can, you know, fix the mechanical disruption of the diaphragm. But what really is going to be of impact to your patient is the underlying physiology. And so your clinical course really doesn't have a lot to do with the anatomic defect itself. But by these three things that are interplaying in the CDH patient, your pulmonary hypertension, your pulmonary hyperplasia, and your ventricular dysfunction. And most people will be familiar with the conversation around pulmonary hypertension and pulmonary hyperplasia. But over the last 10 years, we've really grown to learn that ventricular dysfunction um plays a huge role in the most severe cases. And the disease doesn't only affect on the organ level. So it's not just what I've always found. So mindblowing is, it's not just the hypoplastic lung on the affected side of the hernia, but the pulmonary vasculature is affected bilaterally. Um all the way down to the tissue and the cellular level. Um on autopsy, reports of babies, we've been able to identify that uh the uh cell proliferation and the impaired vasoresponsiveness of the pulmonary vasculature doesn't just affect the herniated lung or the lung on the herniated side, but will actually affect the good quote unquote, good lung or the lung on the nonherniated side. Again, speaking to the fact that this is a global disease. Um So when you do see that neonatal patient, um and there's, you know, we kinda talked about there's the prenatal diagnosis, the neonatal diagnosis and then the delayed diagnosis. And within that neonatal presentation, there are various phenotypes um to the CDH that we can see and the, the neonatal um literature does a very good job of describing those three phenotypes that you might see. Um based off of echo echocardiogram findings, there is uh the patients who are gonna have what we call a very smooth course. They're gonna have no or very mild pulmonary hypertension. They're gonna have very minimal cardiac dysfunction. Their atrial shunt goes left to right, their PDA shifts left to right. Really our goals um in management of these patients with our neonatologists is to make sure that we do some gentle ventilation, that their respiratory status is well managed. Um And we have on board or available to us a little bit of nitric oxide plus minus some milrinone. And that's usually all these Children will require to uh proceed to the operating room. Um These are the kids that once their honeymoon period is is over, they continue to look good. And these are the ones that we might even consider thoracoscopic repair on. In contrast, you have the Children who present with um with uh cardiac dysfunction and those that dysfunction comes in two forms. You have the Children in which we presume that uh the pulmonary hypoplasia and uh pulmonary vasoconstriction and capillary failure is such that they get right-sided overload and they develop some right ventricular dysfunction. They end up with a right to left atrial shunt, they end up with the right to left PD. These are the Children who are going to require vasodilator support. You're going to want to maintain their ductus. Um I have a list here of some of the pressors you might use in supporting them to maintain their perfusion and to support their right side. Um In contrast, it, something that we've only come to understand in the last few years are the Children who have some significant left ventricular dysfunction. Um These are the kids who their uh their disease process has uh has affected the, the left ventricle, not just in terms of the volume that the left ventricle is seeing, but actually in the function of that ventricle. And they will often have a left to right atrial shunt, but they'll have a right to left PD. Um These kids, you wanna try to maintain their ductus for systemic flow. Um They're gonna need dobutamine in addition to the milrinone for left ventricular support. And these are the kids that's important to identify them because um they're probably the ones that made the nino trial in which uh CDH patients had poor survival with nitric. Um These are probably the patients that, that captured um because nitric oxide will actually worsen your left ventricular dysfunction. Now, this is all very um very critical care and very um geeky to go through, but it doesn't um it does translate into what you're gonna see clinically in the patient and many places um across the globe have very nice um algorithms to help decide um how you're going to medically or surgically manage a patient. So in that first category that we talked about, um these are kids who hang out in what's called the safe zone. This is an algorithm out of Michigan when I was uh when I was working there. Um these are the kids who are gonna require very minimal um ventilatory support, they're gonna require very minimal pressor support. Those pressors will wean with a little bit of volume, they'll meet these particular physiologic goals. And if they maintain these goals for the next couple of days, you can proceed on to CDH repair and often even thoracoscopic CDH repair. If however, in those first few hours, you cannot achieve these goals, you need to accelerate um or become more aggressive in the type of support you provide the patients. And now the details on here, I know this one is difficult to read and the details on here are not really important. It's the, it's the thought process and the management that's important. Um is that if you're not meeting a particular set of goals, in terms of physiology, you need to accelerate and get more aggressive in terms of your support. Well, at that point, you enter into a caution zone and these are the patients that are either going to stabilize over the next two days and, and then revert back into your green zone and be ready for repair or they won't. And depending on what resources you have at your facility these are kids that you could in the next hours to days um increase the amount of support you provide, including ECMO. Um And then there's a whole conversation on when you repair them on or off ECMO. Um or have the really difficult conversation about um futility of care, depending again on the resources that you have available to you. So, through all of that really complicated conversation, the, the the goal um takeaway here is that the key to surgical success is the presentation and the timing of the patient. Um This ha is one of those uh pieces of surgical dogma that has, that has no longer has equipose. And so, um if you look for a Cochrane review on this question, um the Cochrane review will tell you that there are only two very small um studies that take a look at uh the timing of repair for a CDH. And that there actually is no difference between operating on um days, 1 to 2 of life versus days, 3 to 5 of life, which is how we often traditionally divide early versus late repair. Um But most expert opinion is that you're going to wait with the average repair being at about days 3 to 5 of life um to ensure that you understand what the underlying physiology of the patient is. And again, the approach depends on the physiology and the context. Um There are multiple different uh surgical interventions that occur for um CDH patients. Um the most common and the most uh uh well known is the is the open repair. Uh over the last 20 years, we've become more facile in thoracoscopic approaches. And um these often for success depend on proper patient selection. Acma has a key role in um in the care for, for CDH babies, but I do wanna highlight that you don't have to have ECMO to take very excellent care of these babies. And there was a very great epis um set of articles out of um J PS about 15 years ago in which Boston and Toronto compared their outcomes. One being a center that high utilizes ECMO, Boston and another that very rarely to almost never utilizes ECMO, Toronto. And they actually showed similar um survival rates in their CDH population just based off of the way they approach the patient preoperatively. And then in the horizon, we have feto that's um that's emerging as a prenatal um therapy for severe or, or moderate to severe um CDH S. Um I don't have a lot to speak to those two beyond this. Um Just given that my presumption that it's not within the scope of our conversation, but if there's anyone who feels that it is worth talking about. Um I do have a couple of slides prepared on fo and I'm always happy to talk about ECMO with that. I wanted to jump into um what every surgeon is excited about, which is the actual repair. Um We're gonna kind of um book end this conversation with uh conversation on physiology and postoperative management because those tend to be what is key to improving your um outcomes with these kids. So with the open repair timing um is is, is thought to be very key. Um On average, most people are operating on day, day of life 3 to 5 when these Children present um in the neonatal period. The thought process here again is ideally your pulmonary hypertension is, is either resolved or has manifested. Um Your oxygen needs have stabilized. If you are on the ventilator, your peak inspiratory pressures are no more than 25 your um carbon or your PCO two has normalized. Um Again, the timing of repair uh remains an expert opinion. Their Cochrane has been very clear that we don't have good data on it. Um And if anyone's had the opportunity to speak to Doctor Kes out of Florida, um he does make the argument that there is some involvement um of that mass effect in your cardiopulmonary physiology for the unrepaired diaphragm. Um But when you start having these conversations, you're, you're starting to talk about uh some fine hairs in the data that we can get into if the uh you know, if the group would like to discuss it. But again, the optimal timing is once the baby is stabilized and, you know, for a fact that they're not going down a downward spiral. Um in preparation for the or all patients will have a foley, will have an NG tube. You will want your perioperative antibiotics. Um Whatever it is that you guys use for clean contaminated cases. If possible, you want inline pressors including Milone, you want nitric available and you definitely want blood on hand. You want continuous oxygen and CO2 monitoring and then depending on the degree of cardiac dysfunction um and the degree of ventilator failure, you're going to want to have um additional medications available. So this all depends on how sick your patient is and how and what resources you have on hand. Again, to kind of remind you these are the kind of pressors you might want on hand if your child has some dysfunction and if you don't have echocardiogram readily available. Um As I remember in Iraq, we only got echocardiograms on Mondays. Um The physiology of the patient will often help you decide whether or not you need to have um the additional resources available. So once you have all of that set up for yourself, um the positioning on these Children is often um supine. Um I place a small shoulder roll or a bump on the side of the defect that allows the baby to kind of um bend over a little bit and to expose that the backside of that diaphragm a little bit better for me. I always prep the chest in the sterile field. Um Though I've never had to, to open the chest. This is, this is almost always an abdominal approach. Um But you always planned for the worst. Your technique is an abdominal axis with a subcostal incision. Um Usually one ci do about one centimeter below the um the costal margin. You're gonna reduce the herniated abdominal contents. You're gonna assess and classify the size of the defect. Um You will dissect the posterior rim of that diaphragm. And it's always quite surprising how much diaphragm you truly have even in Children that you think don't have any once you unfurl it. And then there's the defect closure which um can happen primarily or with a patch or with tissue repair. And as you close the defect, you'll have to make a decision on whether or not um you're going to want to place a tube thoracostomy and then the abdominal closure can get complicated depending on how much abdominal domain the patient has and whether or not you're gonna do a primary or a patch or a silo closure of the abdominal wall. These are some intraoperative pictures stolen from the not a textbook resource. In the top one, we see the hernia defect with uh bowel in the chest. Um In the second uh image, we see the reduction of the intraabdominal contents. And then in the third, we see how much was actually up in the chest and is now eviscerated into the abdomen and this is gonna play a role because you need to be able to fit this all back into the abdomen without compromising um the the respiratory status of of the child. Um and these Children can be at risk for compartment syndrome. Once you've reduced, uh all of the contents, you assess the size of the defect. And this is uh the algorithm proposed by the CDH study group in terms of how to classify the defect. Um defects. A and B are often defects that can be primarily repaired. Defects. C and D are often defects that will require some kind of mesh or prosthesis. And how you make that decision in terms of how to, how to proceed um is uh gonna depend on what provides you with the least amount of um of, of tension in your repair. Approximately 50% of all the CDH S are gonna be too large for primary repair. So you're gonna need some kind of replacement. Um They're the most common is to use a patch including um nonabsorbable synthetic and absorbable biosynthetic. Um There are people who are very facile at using autologous tissue um by creating muscle flaps. And so we'll talk a little bit about those. Um This is a uh illustration from I believe sc's group out of Utah, um who quite routinely will use a muscle flap to, to repair. Um They do quite a bit of this and have uh have described this technique of separating out the external oblique for the wound closure, but then using um the internal and the transversus for uh closure of the diaphragm by flipping it down and over. Um And in the right hand, this is a very, very good technique. Um It all depends on how well you've trained in it and how, how comfortable you are with it. Um For those, uh what part of the reason they came up with that is initially early on when we used to do patch repairs, they were associated with a very high recurrence rate. And so that group decided that they were going to proceed with, with creating muscle flaps to create a tension free repair. Um Other groups such as the group out of um I believe this one is out of Texas children's um decided that uh they didn't feel as facile with the muscle flap, but they could make a patch work. And so the key principle here um is if you do decide that you're using a patch, you wanna make sure that it is a, it is a quote unquote floppy patch. Um This dome technique that the Texas Children people have described um and have uh diagrammed out allows you to have a redundancy in the patch. So that as the child grows, you do not develop tension on the repair and you reduce the risk of recurrence at least in their series from 28 to 14%. There are a whole bunch of other intra intraoperative decision making opportunities while you are repairing these hernias, um, bleeding is always a huge risk in these cases because it is often a neonatal liver that you're dealing with. You are ma manually reducing the spleen. And then as you're repairing the diaphragm, you're often putting some kind of um retractor on that spleen. Um that posterior rim of the diaphragm can be quite friable as you dissect it free. And so having some blood on, on hand, if possible, um is uh is often recommended. You also have to recognize that once you pull that lung down, there is a change in pulmonary compliance. And when that change happens in pulmonary compliance. And um and uh and your ventilation becomes too aggressive, you can often incur a contralateral pneumothorax. So, if um your anesthesiologist speaks to you of your peak pressures changing, in addition to the possibility that your mediastinum has shifted, you also have to worry about uh high airway pressures on the contralateral lung becoming an issue early in CDH uh literature that contralateral pneumothorax was basically a death sentence and there was a very high mortality um that we saw with that situation. But with the advent of ventilation, we very rarely see that anymore. The other thing to think about in terms of pulmonary compliance is um and we alluded to this earlier is the loss of intraabdominal domain. So once you've reduced all of those herniated contents from the um from the chest into the abdomen. That abdomen, which on presentation in the neonate is often scaphoid won't um accommodate those contents very well. And in that situation, um it is not uncommon to uh to manage the potential compartment syndrome by either leaving the abdomen um open within a silo or to uh to uh do a patch repair to give yourself some extra domain there that you will come back and take the patch out later and close the abdomen. Once compliance is better after you reduce the contents. The other thing to inspect for is um pulmonary sequestrations. As illustrated in this image up here. Um There is a known um association of uh pulmonary sequestrations uh with CDH and uh when you see that little um nub in of tissue that uh uh it is uh often recommended to go ahead and take care of that sequestration at that initial operation. There's also very rarely a hernia sac after you reduce the contents that that remains behind. And um it's important to resect that because it will often act as a uh as a hematocele or a uh fluid filled sac within the chest. Um If you leave a hernia sac behind, it's controversial whether or not people leave chest tubes, um post procedure um that's just going to be culturally dependent for you. Um My personal practice is to place a red rubber catheter as I'm um closing uh the hernia defect and I place that catheter on suction as I place my last stitch and pull it and, um, I don't routinely leave chest tubes. However, if you are a person who leaves chest tubes, um, it's very, very important to realize that you should not be placing those chest tubes on suction, but rather leave them on dependent drainage because you run the risk of, um, of displacing the mediastinum. Um, if you put too much pressure on a neonatal chest, and then the final other intraoperative decision that one needs to make is whether or not you're gonna address the nonrotation or the malrotation in this baby. Um There are people who live on both sides of this question and have very strong opinions on it. There was a small series published out of Milwaukee that uh commented on uh almost an 11% incidence of uh later in life. These Children having problems associated with their nonrotation situation. Um But there was a larger uh review database review that looked at Children who had the repairs um of their or or who had their rotation issues addressed in their index operation versus those who didn't and actually saw an higher rate of adhesive bowel disease um later on in life for those kids who had their rotation address. So people debate this often when I'm doing these cases, these Children tend to be relatively sick. And so adding time to their operation has never been a big um a big attraction of my to me. And so I um I often will not address their, their rotation issues in the index operation. All right. So that is the open approach for the Thoracoscopic approach. Um This has become um more and more accepted as AAA way to handle this disease process. Key to a Thoracoscopic approach or the ability to do a thoracoscopic approach is you need to have um an appropriate patient selection. These are the Children who have minimal pre preoperative ventilatory support. Um If you, if you read the paper from doctors Wilson and fishermen, they'll say that these patients have absolutely no pulmonary hypertension. But you and I both know that in the neonatal period as you're transitioning from fetal circulation to, to the real world, that first day echo will always show some degree of pulmonary hypertension. And really the key is is that you don't have pulmonary hypertension that is uh that is limiting you um in terms of your physiology. Um So you uh want time this similar to your open approach most commonly in 3 to 5 days of life for the neonate for those delayed presentation patients. This is a perfect operation. They've already proven through, you know, a trial of life that, that their cardiopulmonary physiology is such that they, they don't need aggressive support. Um So if they're presenting with a pneumonia or something like that, you're gonna want that treated first before you operate on them, the preoperative um oh, the preoperative uh preparation is very similar to what you do with uh with an open approach. There is an argument that some make in particularly the French have made of pla placing near monitoring uh to assess the cerebral perfusion of these patients. Because one of the biggest concerns with thoracoscopic approach has always been um the CO2 insuffi insufflation and the risk for acidosis more on that to follow our positioning. Thoracoscopically is a lateral decubitus. That is a little pish I like that is how I like to describe it. Um Patients are often placed in a slight reverse trendelenburg to help with have gravity, help with the um reduction of the contents. One of the uh recommended uh pos positioning of the or team is, is diagrammed here. Um Though there are variations on this, I think one of the pro tips in this situation is when you're operating and the baby is small, your arm movement is often limited by the by the um presence of the bed and the height of the baby. And as such, if you elevate the child on a couple of blankets, um or towel rolls, you can avoid your instrument limitations when you're sewing that lateral aspect by creating an artificial height. Um so that you can have the leverage you need. Um The trocar placements are there's multiple different diagrams out there if you review the literature but uh you triangulate relative to either anterior or the tip of the scapula. Um My approach is the one that's in the, on the left hand of the screen where it is a five cm report is placed there. I insulate at very low pressures and a very minimal flow. And after the bowel is reduced and I can take a look in there. I stop the insulation. The goal here is to create a safe space to work in without limiting or inhibiting um cardiac and pulmonary function and without hyper expanding that chest cavity. So um I think very early in my career when I was a fellow and we were all learning, we would just flip on the insulation and then we would convert to open because the baby would decompensate. Um And we soon learned that one of the key features to keep that from happening is to start with very low pressures and very minimal flow. The technique is very similar. Otherwise, um you're gonna resect the hernia sac. If it's present, you will reduce the contents. This can be quite tricky because you are using three millimeter instruments in the babies and the the tissue you're handling um wants to live up in the chest, often has adhesions into the chest and can um often be uh be uh difficult to, to reduce without injuring. So you have to be very gentle in this and then uh primary repair is usually the case for these kids. So there are some descriptions on how one might do a patch underlay or a patch overlay. And we can talk a little bit more about that. These kids are do have a high risk for recurrence and then that recurrence. And early in the literature was often on the lateral edge, which is at risk for tearing because it tends to be a the very thinnest part of um the diaphragm. But also you don't really have a good space to suture it. In often a lot of people will use a suture passer technique, one of which is diagrammed here in which they will incorporate that rib um and uh bring that diaphragm in. Um There is and I believe out of the group from emery a argument even with primary um repair to uh lay a thoracoscopic underlying mesh and to incorporate that into the repair, especially on that lateral side to uh minimize the risk of that lateral recurrence. Um And then I found it very interesting and I've never tried this one but a vats assisted approach in which they actually introduced an entire patch um through a minimal um costal incision um with laparoscopic securement or thoracoscopic securement. Thoracoscopy has its own set of complications. The most common being conversion to open due to decompensation of the patient. Um hypercarbia is probably the most treacherous one we worry about from a physiology perspective. Um Mostly because remember, hypercarbia is going to exacerbate um your pulmonary hypertension by pushing the baby into acidosis and then that will then decompensate um heart function and make the baby unstable subsequently leading to an open procedure. So if you do find that you are struggling and it's taking some time, you might consider again, turning off the insulation um and or taking breaks from the insulation while you're trying to get the hernia contents reduced intestinal injury, splenic injury have both been described. Um and then we talked a little bit about the high recurrence rate. So, in a patient that otherwise is equivalent, what is the better approach? Open birth sy thoracoscopic? This discussion is just a very, very nuanced for a number of reasons. Um like all surgeries, uh open repair versus thoracoscopic, you're gonna want to do what the surgeon is most comfortable with and is good at. You don't wanna throw the surgeon into a situation where he or she is doing a surgery that they are uncomfortable with. So that's my first rule of thumb for this. Also, when you take a look at the literature of open versus Thoracoscopic approach, it may seem that the Thoracoscopic approach hasn't improved mortality as seen by these this plot diagram here. However, as we've talked about earlier, there is a patient selection bias when you're looking at thoracoscopy, as these Children tend to either be more delayed in their presentation or much more stable in their presentation compared to our open patients. When you do do the repair, that being said most pieces of literature describing the thoracoscopic approach will tell you that the patients have shorter hospital stays, shorter lymph ventilation days. Um and uh improved cosmesis that being said, um again, there is that patient selection that we that, that these Children undergo. Um And so with that caveat, both sets of the patients are going to have some degree of perioperative complications you need to look out for. So, one of the things that shifted um surgical practice from early repair within the 1st 48 hours to a delayed repair was that a large part of the mortality we were seeing was due to an exacerbation of the pulmonary hypertension. Once you've reduced the hernia sac, um and the chest wall compliance and the pulmonary compliance changes. Um Shunting is promoted and your RV afterload often increases. And this creates that vicious cycle that we see in pulmonary hypertension of hypoxia acidosis and hypotension. So even if you take the patient at the best possible time, just the stress of the surgery might exacerbate or push them into a pulmonary hypertensive crisis. What the the key thing to note is that same algorithm charts that we created pre-op can still be applied to manage these kids POSTOP. And again, those algorithms should be created depending on what your local resources are. Um And what your team is comfortable managing. Um There's also uh hypertension that may be secondary to the mechanical effects of of your insulation if you're doing thoracoscopy that you have to think about. So again, if you do high flows um and high pressures, um this is much more an issue. Um The contralateral pneumothorax is always something to be aware of, especially if you have a patient who's been looking really great. And then on a turn of a dime, all of a sudden looks ill. Um bleeding is a very common complication. Much more common if you're using ECMO as a perioperative management strategy, but still common even in um non ECMO patients. And of course, hypothermia as all of the internal organs are eviscerated for a majority of this case in the open repair, not so much a complication in thoracoscopic repairs in the postoperative setting. You want to make sure that you're um using very gentle ventilation. Um If you do decide that you're gonna place a chest tube, you wanna make sure that there is no suction or very low section as babies have a very mobile mediastinum. Um And often nutrition management plays a very key role in terms of your, your nicu length of stay and your ability to go home. As a lot of these Children, not only have prolonged illes but poor feeding is very common and we'll discuss that a little bit more as we talk about um long term outcomes for these kids. We know that if you do a long term follow up these Children um with repair of their hernia all of their woes are not uh alleviated. Uh Very many of them are going to have um prolonged pulmonary issues up until at least five or six years of age, which is when um lung development uh continues on until um they often have uh needs for return to the hospital for pneumonia and other respiratory illnesses. Quite a number of these are gonna have neurodevelopmental issues either because of a um comorbid congenital conditions or because of the hypoxia during their uh perioperative care. Um and or as a complication of echo intervention if that's what uh we use for our perioperative management as, as surgeons. Um The thing that we deal most with is reflux, which can happen up to 100% of these patients. Um with the literature saying, um at least 20 to 30% of them are gonna require um a fund application um at some point in their life and at least 50% of them are gonna require surgical feeding access. Mhm. Um In this uh vein, it is important to have a, a organized methodology to, to how you're gonna follow these patients long term. And the CDH study group has, has uh published dividing the patients into high risk and low risk um as to how often you should be seeing them POSTOP and what we should be looking for based off of all those different systems that we've just kind of talked about being affected. I told you weren't gonna talk about FTO unless there's an interest. But that is a summary of um how we deal with CDH. Um Sabina, I think we, we will just pause here um because uh we have quite a few questions in the chat box and I want to get comments from various experts who are attending. So, yes. No, thank you very much. I think this uh critical care perspective uh in addition to being a pediatric surgeon has been really very good for me. I mean, I have been studying this for uh I know about this for about 35 years, but I have still learned a lot from this talk. So thank you for that. And can you see the questions in the chat box? Uh You can sort of scroll up and then Doctor Parter had two questions and then uh there are other questions. So if you can just go from the top, um I think the first question is about percentage of CDH in your series associated with congenital anomalies. So that's a very good question. Um And a lot of what speaks to whether or not you address the CDH has to do with the the survival statistics, right? Um Patients who go on to repair have a 80 to 90% survival Children who don't have repair um have about a 40% survival and that's in, in, in all series that you can look at roughly. Um So what happens with the associated congenital anomalies is, um, a, what are the anomalies? Is it the bronchopulmonary sequestration? Um, and the patient is stable? Well, that's gonna be a condition that you will, you will proceed with. Is it a, a heart defect that is, um, gonna require palliation? Well, in that situation, it's gonna kinda be something you decide with your, with your, um, cardiothoracic surgeons and your cardiologist in terms of how aggressive you're going to be. Um, I have had about, uh, 15 patients with, uh, severe congenital anomalies that, uh, that the family decided to make comfort care. And, um, and, uh, those have been the ones that we flatly told the family that ecma was not a very, um, viable option for them and the children's pulmonary hypertension and cardiopulmonary status, uh, dictated, uh, their life course. I hope that understands, uh, that helps you. Now, I think that answers the next question I think is again from him, uh, any misdiagnosis you have had. Um, yeah, so, so interestingly enough, uh, II think Doctor Patan, you actually a asked about series as well. One of the, uh, last papers I did out of Milwaukee was, uh, in conjunction with our radiologist. I think it was a series of about six patients he who had been diagnosed with pneumonia, um, had been diagnosed with reflux. And, um, and, uh, one who came in with a bowel obstruction of unknown etiology and a combination of, of chest x-rays, upper gi s and placement of the NG tube um in these patients uh is what helped us make the, make the diagnosis. It's actually really fascinating because when we went back and looked at uh at the Children who as neonates presented with other ailments. Um and not in an unknown diagnosis of CDH, we found that actually all of those Children had their prenatal ultrasounds cause they all had prenatal care, um had their prenatal ultrasounds in community hospitals um by nonradiologic um or non pediatric radiologists. Um And uh and so the question we had that we weren't able to discern is whether or not there were subtle findings that people without a high volume were unable to differentiate. Um or um whether it was that um if you look at the embryology of the disease process is that um at the point in time that the ultrasound was performed um was uh were the hernia contents uh not intrathoracic per se. Um because the other thing about these delayed cases, almost 90% of them were type a deformities. So there weren't very big deformities at all. Yeah. Uh Just a very um interesting story uh when uh I was still in India, one of my bamate who was an anesthetist, she had a daughter and uh her daughter was diagnosed by one of her colleagues uh friend, pediatrician only at one year of age when she presented with a pneumonia into inverted comas. And the little girl had repair and now she's an adult pulmonologist herself. So she was lucky she had delayed presentation and she was, she's, well, now I think the next question is uh from Doctor George KN or is, does side any field have any place in the management of uh diag hernia? So, um Sildenafil does have a role to play. It depends on whether or not you have IV or PO formulations. Um traditionally Sildenafil, um, was something that we used once the patient was stabilized, came off of their nitric oxide and how we transition them off of nitric oxide onto an oral medication, um, as we stabilize them. Um And so that's usually the role that, that we see them. The other place that we use. Sildenafil is as we're transitioning off of ECMO. Um, because of, uh, the, the difficulty in obtaining IV formulations and running that IV. Um, it's very rare for us to do it preoperatively. It's often most, most often used in, in my practice as a postoperative um, medications once we've kind of tried to wean off of all the other, um, vasodilatory medications or the pulmonary vasodilatory medications. Ok. Thank you. I think the next question is doc from doctor fare. Is, are there any tricks to close the lateral defect? You know, so there are many tricks as there are probably surgeons in this room right now. Yeah. And everybody and, and everybody has, um, has a, a different uh belief. I think the thing that complicates the answer to this is not all diaphragmatic defects, especially as you know, we all start, most of us will start immediately and work our way lateral. Um is how that lateral corner presents to you in the end. Sometimes the defect is kind of displayed and kinda wants a kind of at repair and sometimes it comes together nicely and you just have a little hump of tissue. So I think all those things kinda dictate what you do. Um For me, I think there's uh there, there's two key uh things here. One is if, if that lateral edge is very thin um recognizing that no matter what you place, um it's at high risk of tearing through. Um And I will, I will often use pledgets at this, at this juncture even if it looks relatively robust to me, but also having a very um very low uh threshold for, for using some kind of um mesh or patch. Um whatever it is you you are, are good at to, to um buttress and provide attention free repair. Um As I think the key principles there. Yes, II agree with you. Thank you. The next question is from Doctor Nebe Moyo. Uh What do you consider to be the best prognostic indicator for how a baby with CDH will do after birth? So that's gonna, that's gonna depend on what your resources are, right? So I think what we have, um that has very good validation are, are um expected to observe LHR ratios. Um It's very well documented, it's very reproducible and all you really need is an ultrasound to be able to, to make those diagnoses. Um And um it'll, it'll tell you a lot about what to anticipate for the baby. Um What might be a little bit more specific but hasn't, I think made real time is um preoperative echocardiography with um a look at what your pulmonary artery pressures look like and your pulmonary artery index. Um I'm a huge fan of pulmonary artery indexes. However, in moving from institution to institution and traveling country to country, that's not something that's easily accessible. I think you will agree that liver and chest or a prenatal ultrasound is also a poor prognostic factor. Absolutely. Yes. Uh The next question is again from Doctor Fare. Uh is the timing to operate, decided by surgeon neonatologist or both? Ideally both. I'm not gonna, I'm not gonna be pollyanna and say that we all get along wonderfully each time and that we all agree. And I know that in my travels, a lot of my surgical colleagues tell me that they get pushed by their neonatologists for earlier repair. Um uh which I can understand because especially if the child's struggling or they're struggling on the ventilator. Um You sometimes grab at anything you can to try to improve your situation. Um But in an ideal situation, you have a collaborative approach. And I think what is actually very key to developing that relationship is, you know, that algorithm that I displayed uh that kind of goes through, you know, the clinical parameters you wanna see before proceeding to the operating room and what interventions you can do. Um That algorithm was a combined effort between the neonatology group and the surgery group and we came up with those parameters together. And so it's always nice to be able to say, hey, listen, when we weren't stressed and we didn't have this blue baby in front of us, we all decided together that this is how we were gonna approach this. Um And so it helps a lot to have their buy in um into the algorithm, their contribution to the algorithm. So that when things are very stressful, you, we all remember that we're approaching this collaboratively. Yeah, I think there are two questions. One is from our registrar. It's a long question. What's your experience of type C and D in the developed world? Uh Those no, without severe anomalies. Yeah. So a lot of them, you're right. A lot of them are gonna be uh the patients that I do on ECMO. One of hence, one of the reasons that I'm not um I'm not uh too keen on addressing rotation issues because the patient is often very, very sick. Um There are also kids that are often on um off and on um high frequency oscillation if they're not on ECMO when we're doing the repair. Um because otherwise, uh my personal approach is to at least attempt thoracoscopy before um before proceeding with uh with an open approach. Um So my experience, these, these Children are often quite marginal um even though they're stabilized from their birth presentation. Mm OK. There is another question from another registrar. Uh What's your view about I iatrogenic injury to the phrenic nerve while dissecting and excising the sac? Oh, that is a really good question. I do think it's a little academic. Um often, you know, often these, these Children, I if you can identify a chronic nerve and you can preserve it, you know, very good on you. Uh I cannot tell you but for a handful of times that I was able to see exactly the course of a phrenic nerve on whatever diaphragm leaflet there is there. Um It is, it is quite difficult to uh to see and identify there. Yeah. OK. Uh I think we will stop with the questions. I just need uh I would like to have comments from experts who are present and due to lack of time, I think please short comments only maximum up to a minute or so. So Doctor Feli Kisa is with us and uh she uh is an important active part of gigs and education committee, Feis your comments. No, thank you me. Uh this was a very good talk and um certainly very challenging in uh places like ours uh to deal with. And uh I just wanted to mention to uh the trainees that uh in addition to what uh doctors have been said about having like um pulmonary sequestration we've recently seen at our center, a child that presented with a hepatopleural fusion. And they're extremely difficult even when the Children present late, like it's not always easy to deal with and you have to be careful approaching that. Thank you. Uh uh uh Th thank you Phyllis. Um I see. Uh I hope doctor Rasi Shah is still here. Doctor Rasi Shah is a very senior pediatric surgeon from Mumbai India and he has been a pioneer of endoscopic uh surgery in India. So, Rasi any comments, uh I think it was a very nice talk. Uh and I enjoyed thoroughly uh no, no particular uh comments as such. Uh that, that is what I would say. You are doing a good work by organizing this uh monthly CME and it is useful to everybody. Thank you. Thank you. Uh Thank you. I thank you very much. Um Another uh good personal friend, a senior pediatric surgeon from India. Doctor Raju S Raju, are you still around? Maybe he has left uh Doctor Marion Arnold from Cape Town was here but she has also left is uh Doctor Shaman Harilal is still here. Shama Harilal is a pediatric surgeon uh in private practice in Cape Town. So Shama any different uh experience in private practice? Um uh Hi Pro. Uh Thank you so much for allowing me to come in. Um Yeah, in, in private, since I've started for the last eight months or so, we've had two CDH uh kid with CDH. Unfortunately, both of them had four antenatal uh prognostic factors. Um One's currently slow on oscillator. Um What we're trying to and hoping for in the future is we get some access to ECMO, which we still don't have available even in the, the private sector. Um But hopefully, when we're starting to develop that we can get some input from uh our experienced uh speaker and uh it would be nice to keep in contact with that. Um Thank you so much. It was excellent talk. Um I really did enjoy it. Thank you and thanks for organizing this. Thank you, Shama. Um I'll ask uh our consultant, junior consultant, pediatric surgeon, Doctor Nla Mosha, who trained in Johannesburg and is now working with us for just about uh a year. Knox. Any different experience at bar? Not any questions, comments. Um Thank you. Thank you, Sabina for the insightful talk. Um No, not really much difference um in, yeah, in experience. Um the, the one thing um that we practice differently here um which was controversial that um Sabina did discuss was the, the use of um IC drains, um which in joburg, they don't routinely insert um intercostal drains while this side. Um We actually do. So. Um, for me there, it was a bit a contentious point when I started off but, um, I just do it as the Romans do. Um, because I did understand what the pros and the cons were for each. Um, I just wanted to find out what the, the incidence of bowel obstruction with mesh repair is. And, um, is it also related to, um, also the approach that you take sly or open? Um That was my only question. Thank you. That's a very good question. So the best data we have on that is that population study that um that looked at that specifically was trying to look at whether or not a uh a rotational anomaly should be addressed at the index operation. And those patients um uh were um presented with the majority of bowel obstruction. Um The hard part about that in my own personal experience with, with taking a child back with bowel obstruction is your adhesions are kind of diffuse and everywhere they're not just at the mesh, but you often have to address the adhesions at the mesh to be able to um understand the anatomy you're working with. Um So whether or not the mesh is playing a role in your adhesive bowel disease, um I think is, is, is unknown but your overall percentage of bowel obstruction and requiring some form of intervention for that is in the 10% range. Uh Thank you. Sabina, I think I would just like you to give a take home message and then we can end the talk if, if there's any take home message. This is the one place where surgeons shine when they understand the underlying physiology and can make the argument to their, their neonatal um colleagues as to um finding the optimal timing and the optimal perioperative management with channel ventilation and stabilization. Um We've shown that to be what has impacted mortality the most um even without the use of ECMO. So just with those two techniques, um you can make a huge impact in your patient population. Thank you, Sabina. Is it right to say that it's a medical disease and occasionally it's, it needs surgery at the right time. I think that's a, that's a very good point. OK, Sabrina. Thank you very much. Uh I'm going to uh uh sh sh like uh we uh we have been recording this meeting. So II will upload it on youtube and share the link on various platforms. Doctor S has kindly allowed us to do that and uh the next meeting will be on the third Tuesday of January. And uh when uh Professor John Lazarus, who is the pediatric urologist from Red Cross Children's Hospital and University of Cape Town, he will be talking about um incontinent and continent urinary diversions. So I will share the meeting uh invitation a week in advance. So, thank you Sabina again. Thank you. Everybody. We have had 50 participants all throughout the meeting, which is really great and all of us have enjoyed uh your sharing of your knowledge and, and everybody should have a good holiday. A ha a Happy Christmas and New Year. Thank you, Sabina. Thank you.