Congenital diaphragmatic hernia occurs in approximately every 1-in-5,000 births. Dr. Mark Molitor explores the evolution of how we treat congenital diaphragmatic hernias and why this often includes ECMO.
Phoenix Children's Grand Rounds - October 4, 2022. CME credit provided only for participation during live sessions.
Introduction and I'll go on mute and then that'll solve that problem. Um So again, welcome uh to Serge Grand Rounds. Uh We're really fortunate to have Mark Mulder with us today. Uh And he's gonna talk to us about something that he's uh what I would say, the, the largest content expert uh that we have in our group. Uh a little background on most people may know this about Mark, but he's actually a true Phoenician who grew up in the West Valley, um went to University of Arizona for med school and then uh ultimately to end up in Michigan where he did surgical critical care and develop his interest in eco. And uh and most people know that he's the ECMO director for our organization and has done just an outstanding job through COVID and the growth of our ECMO program which has exploded over the last three or four years uh in that vein. Uh A lot of what we know about congenital fragment, hernia does involve uh some component of ECMO in terms of uh survival and, and taking care of these babies. Uh I've asked him to talk about congenital life for her and not ECMO because unfortunately our previous fellow scooped the ECMO talk, uh, when he left, uh, Juan gave a talk on last fall. So after, after being in University of Michigan, uh, Mark went to, um, uh Salt Lake City and did his, uh, pediatric surgery at primary children's and then, uh, was recruited back here to his hometown, uh, to, to start his pediatric surgery practice, which he has now been pushing almost 10 years. I think eight years. Mark, it will be 10 years, 10 years. Ok. Yeah. So, um and I'm really excited about uh about what Mark gonna talk about today because uh we had, we've got a little stagnant in terms of how we were reporting di for Maura for a period of time. Uh We were part of the, the registry and we've gotten better at that because uh uh Mark really thought it was important and he's driven that and I it's gonna show us some data about outcomes and also talk about uh will learn as a, as an entity itself. So, Mark, thanks for uh thanks for giving this talk. We're really excited to hear it. I know it's gonna be great and we'll take questions afterwards. Perfect. Thank you so much. It is great to be here today. Uh Don't forget to enter your CNE code and do all that good stuff uh through the UK. And so I thought I got to talk to damage and talk about dental dima, hernia and thought of talk a little bit about the evolution of the process here at the children's and mostly over the last 10 years since I've been here. And by no means in any way, am I discuss when I discuss anything today? Um, I, um, giving bad credit to the previous, you know, before I was here, this was just looking at something that was actually good and how we could make it better um as a group. And so, and tell us like, if you build it, will they come and uh look at some data about our numbers and outcomes throughout that time and no disclosures briefly read. Uh You know, this is kind of the outline what we'll talk about today. Uh you know, a bit of time on sort of what C DH is. Um you know, how we treat it both medically and surgically. I will talk a little bit about ECMO uh just because I can't afford not to. Um And yeah, you know, my, one of my second passions, uh then we'll talk about management and then I care here at Phoenix Sultans as it has changed over the years. T DH happens about one in 5000 births. Um And, you know, it's probably a small increase over time, but like all good things in peat surgery, it's about one in 5000. It does seem to be more common in males. The most common defect is that that posterior defect that you see here, the bop to like hernia, uh 10% of the anterior, the Morga hernia that you can uh get some other ones that happen. But the most common one is the Boite hernia, 80% of the time it occurs on the left side, about 19% on the right. And then you can have a total. But the information of the diagram where it doesn't form at all, um along the way, that's fairly rare. Uh And those Children don't tend to do very well overall mortality as we look at this through at um you sort of we consider tertiary care center. So, um the deal with this is about 20 to 30% and that is a decrease over time. We look back probably 15 years ago, the overall mortality for C DH is about 50%. Um And that has now uh a decrease Cathy says they don't see your so OK, give us one moment, didn't miss much. Oh OK. I show here, OK. We need to get back to the and share screen. Um be great if somebody online can tell us whether they can see the slides. Now she says, yeah, perfect. All right. Uh Again, this is like the second slide in the entire thing. So you didn't miss very much. Um As I mentioned, uh you know, um mortality has decreased over time. Um And we look at this completely, you know, it's probably about a 20 or 35% of fetal cases that end in termination or in u fetal fetal demise uh that we don't even know about. So, um there are this population there as well. There are some associate anomalies with uh CBH and they do tend to pretend to have a uh a worse outcome in that. The most common being cardiovascular, about 28% of the time. The most common one being the dasde Coor uh hypoplastic left heart te there are some neurogen uh effects most su or um central system. About a third of the cases do have a genetic abnormality associated with them. Um There are some, there are about seven genetic syndromes that have been described with CD, some of the Trisomy, 18 21 13 spring syndrome, uh the Neighbor Syndrome beam. Um and there does seem to be some auto and link variants that go along with it as well. But the true genetic model of C DH, we do not know what that is yet. Why, why do we care? Um Right. So there's just some guns up in the chest. Well, it turns out the guts up in the chest actually really hurt um lung developments on 22 ways. And the things that I care about once I hear about a diagnosis of cation that we all care about is the pulmonary hypoplasia and the pulmonary hypertension. Um in terms of the hypoplasia is actually a smaller, not only is it a smaller lung but the lung doesn't develop. So, a decreased number in the bronchial and the alveoli that are there when you have no less alveoli, there's less area for gas exchange to occur and it doesn't only happen on the side of the defect. I mean, it's left side or right side, but it's the contralateral lung is affected as well. Then secondarily the pulmonary hypertension as the lung doesn't develop. Well, the blood vessels inside the lung also don't develop. So there's a smaller number of them in the fetal heart, even though in the physiology, even though the placenta is doing most of the work, the heart recognizes that it takes out harder amount of, it's harder to pump blood through the lung that does that is there. And so there's some thought that that part is working a little bit harder even in fetal development, that then leads to an increase in this process, cycling and increased contras in the fetal lung bed that then causes these arterials to be smaller, building up the musculature around the arterial. Such that when they're born, we have these, excuse me, very thin, uh small blood vessels then that are hard to pump blood through once the diagnosis of C DH is made. And really the it's the prenatal counseling. So, you know, we used this used to be like, ok, you have CD A show up, we'll take care of you when you get here. But we recognize now actually the, the treatment of C DH actually starts in the prenatal realm of things. So you have to get the families involved. And so really a multidisciplinary approach to this and I'll kind of briefly talk about it now, but then go into a little bit more as we develop the program here at CD. This involves surgeons, surgery, radiology, neonatology, palliative care, cardiology, um any of the other anomalies as well, genetics, urology, nephrology, um uh neurosurgery, uh cardiovascular surgery, uh can all be involved. And the process really is a lot of prenatal imaging and then discussing with the families, what these pre what all this imaging and testing means and what these predictors are for her. So, ultrasound, uh we use that. So what's going on um ultrasound uh is the main one. So, you know, done between somewhere between 16 and 20 weeks, you know, most women get their sort of high yield ultrasound is done. And that's when most of the diagnoses are made, we then bring the patients back, you know, anywhere between 24 to 28 weeks a week. Um And when we do them in that realm, we can then get some predictors that go along with that, that can tell us sort of the severity of C DH. And when I think of C DH, that's how I think of it. I think of it as a spectrum of disease from mild to severe. We get some measurements. The most um one we use, the most out of ultrasound is the lung head ratio and the lung head ratio, we're literally looking at the baby's head, the baby's chest out a four chamber view of the heart. And then they take some measurements in there what goes on and they come up with a ratio. Then that ratio as we look at it really divide into what I think of it. As in the third, we have a less than one, a 1 to 1 point and greater than 1.5. And in that category, basically less than one is a sphere category, uh moderate is the 1 to 1.5 and then greater than 1.5 is really a mild disease. And when we take those numbers then and look at them and follow through the C DH research group, we can actually come up with survival percentages. And within that, you know, again, the worse off you are. So if your L hr is less than one and you're in the severe category, your chances of survival are low, um Your need for ECMO is high. Um And when you're in the mild category of things, your chances for survival are higher and your need for ECMO is less, some of these numbers are a little skewed and I will admit 44% in the um you know, in the data that we had 44% in the mild group is actually probably fairly high. Um As we look at that one, we can also use the ultrasound to protect high drops. Um, you know, again, look at it right versus left. And then what's actually probably more important now is whether the liver is up or not. And when the liver is up, what percentage of the liver is up in the chest and they are giving us some actually probably better prognostic indicators. So how do we measure liver up? Um Now we can get an MRI and I will credit PC H, you're probably one of the leaders in this and taking it to the next level um about uh getting heal MRI S. And the benefit is is that we can actually see spatial resolution that's going on in here. Now, we can actually use the formula in the computer to sit there. Um And doctor Paul and his group will sit and measure the lung volumes that are actually in there and we can get accurate uh readings and then we have regular MRI S as well. So we can compare those what we have to what would be expected. Um You know, with the fetus in that gestational age range and come up with a ratio and then we can have an observed or expected uh total fetal lung volume. And again, we can break that down into categories, we break the less than 25% 25 to 35 and then greater than 35% from that severe to mild uh severity. And then within those come up with outcomes in there as well uh as well. Uh And again, so when you have a little bit of lung, your outcomes and your survival outcome is poor and those are probably less than 25% chance of surviving the cost process. When you're greater than 35% you get into that mild case. And those the survival in that group is up, you know, as as high as 80% in some centers. And I mentioned the liver up, go back to that real quick. So liver up, uh they can actually take and measure, you know, volume with MRI think it's actually, you know, so total fetus, they can see the whole thing, they can actually measure the entire volume of the size of the liver. And then look to see how much again for left C DH how much the left lobe is up in the chest, get a percentage of that and then anything greater than uh 20% is considered very profound. Um And those babies will then fall into the sphere category of things in terms of overall survival, fetal echo, um looking at cardiac position structure. Um And then we can actually get a small predictor of what the pulmonary hypertension will be that using something to help them score. Uh once they get that index to Mm I less than one actually says that there's a high risk for pulmonary hypertension. So we've been beginning counseling the families about what that is again, taking this all into a, into a sort of range and then into a, you know, complex of diet or um findings we can then counsel the families about what to expect once the baby gets here. Is it 100% accurate? No, obviously there's some things that change when they come out. But for the most part, we know um they can give the family some expected management. What's gonna happen the remainder of the pregnancy then and when I said that team and then who I forgot the list on there was the obstetrician, the maternal fetal medicine doctors because they play a big role in this. So once we know these predictors, we can then report back to our um obstetric colleagues. Um and they can manage the remainder of the pregnancy. Uh As it goes through there, we can make some recommendations for that. We don't tell them what to do. We just tell them what's going on with the fetus that they can make the best choices to keep mom healthy during the remainder of her pregnancy. One thing that has happened over the last several years is that there are some, there is some fetal therapy. So if we look at the history of C DH, there was um they would try uh fetal surgery. So you know, trying to do a, a modified exit procedure where they leave the baby on placental circulation, they deliver everything. But the ahead, um at that point in time and do the repair, it didn't actually didn't improve outcomes overall for the C DH. And it actually was, had a bunch of maternal uh comorbidities that would result from that resulted in premature labor. And so that was actually sort of abandoned along the way. But there was some thought about what if you were able to block the trachea and let that amity, the fluid that's in the lungs, sort of in there and don't have the way out, right? Because the baby is breathing in utero. So that fluid moves in and out and that does, that fluid does play a role in lung development. And so the first group in Europe, they did something called vito or fespic trachea fusion where that um you know, well, in utero, they take and they put a fetoscope in transcutaneous translucently. Uh you can see here into the mouth and then they deploy a balloon into the trachea. That balloon stays in place, usually done about 28 weeks, gestation and stays until about 32 weeks and then it has to be removed prior to delivery. And when they did that, they noticed that there was um actually lung growth growth on the lateral side of the defect and on the contralateral side. And when they looked at overall outcome in those babies with the severe category of things, there probably is a slight increase in um increase in uh survival of looking at that group of things compared to babies who were not treated with Beto at that group. That study was actually stopped early uh due to um the positive outcome of that. And so, um in Europe, that's, you know, doing the fetal endoscopic tra lesion is actually pretty common in the severe category of things in the US. We are looking at it as well. There are five centers in the US who are doing feto. Um and we have sent a few of our patients uh to Texas children's, we've sent one to Michigan, uh one to San Francisco along the way and fall in the severe category and who have qualified for fetal surgery. All of those patients have survived so far. Um And we are now seeing them back here at PC H and follow up again, the range of the management is done by obstetricians. Um You know, really we just want the moms to be healthy. There is no benefit to having ac section versus not if the mom is healthy, she can deliver gradually and the baby is taken care of expectantly. Um, you know, we do recommend delivery at a center who knows what to do with C DH though. Um That is the one thing that we do talk with the families about. Um, you know, in terms of making the decision to deliver at a hospital where there there is neat and where people are used to dealing with CH and that has actually shown to be um a better, have better outcomes when that happens. The initial management, once the baby get here is really controlling the pulmonary hypertension um and physiologic stabilization. And so this is kind of a busy slide and no hyper breath. But basically, the idea is we want to control breathing, we want to make sure that the baby is oxygenated, but not at the expense of hype for oxygenation. Um And we don't want the baby to get stressed out. And so that might involve intubating the baby. It'll be involved using sort of low pressures, um high frequencies type ventilation properly, conventional versus the oscillator. But there are some discussions about how that works, controlling the hypertension. Um you know, U A lines and feed her peripheral I DS in order to get fluids in um and keeping the baby as simple as possible and getting them over to the and really just leaving them alone um is sort of like mainstay of therapy and allowing the pulmonary hypertension to sort of um abate and allowing the babies to um you know, to stabilize. What I'm not mentioning here is surgical repair and that actually is the last thing on our minds. And so once the babies are stable, then we'll talk about surgery. Uh but then the main thing is control the pulmonary hypertension, how we control that in the, you know, over time and, you know, varies. And that is what has changed over the years at that management, you know, in terms of general ventilation and with permissive hypercapnia and the things that we all use, we don't have to have oxygen saturations, the 90% though you can get them 80 85 as long as their ph is OK. And their CO2 isn't, you know, through the roof with that, we can control these babies and allow this permissive hypercapnia. And then we actually do better for them in this realm. And the babies had a higher survival when we can allow that to happen. Sometimes they do need some vasopressors and tropes. And so we um you know, those will be started as needed. Um and then really control the pine component hypertension. And um if you know, just plain on oxygen doesn't do it, then we can start things like called nitric oxide depending on the echo results. And whether we have RV or LV function, we can start some of the uh either PGE to keep the ductus open and then the remo of the process cycles um in order to help with the, to control that and the lax, the vascular bed following the ois. Um And as I'll show you a little bit and talks about, you know, maybe o I can give us some clues about when to repair the baby as well. And then in long term, if we're unable to control the pulmonary hypertension, then we will look at some of the oral or IV medications that still benefit across the, that go on. And then if in all that, we can't control the pulmonary hypertension, uh then we'll talk about e yeah, and this is just a slide sort of about from Doctor Menon, uh who shows us, you know, sort of what the pathway is that we look at the, the medical therapy for pulmonary hypertension. Um and how it affects the various uh cells uh within the musculature of the pulmonary vascular bed indications for ECMO really is you can't oxygen kid or you've got cardiac failure with, you know, poor oxygenation. And then we're gonna go on um you know, having to use really high dense settings with that's greater than 28 or so uh maps on the oscillator, you know, greater than 15 to 18. Um You know, then those are all reasons to think about ECMO from an, from an oxy standpoint, you know, unable to maintenance sats in the 85%. A ph less than seven point even with those things. So a ph of less than 7.2 saying that the baby isn't able to, to compensate uh physiologically speaking heart failure. And they, some of these do have some heart defects as they go along. And, and there's some controversy about whether cardiovascular defects should allow you to be on ECMO or not. Um, but, you know, because of overall survival outcomes. Uh but, you know, we'll look at the cardiovascular failure and then just a cli acute clinical deterioration. Sometimes these babies come out and they just go down fast and we don't know really what's going on. And so decisions may be made to go on echo um for ECMO to the decision making um, and therapy type of EOVD versus VA. Um Well, really it, in the end, it probably doesn't matter what matters is really what their center is good at. Um, you know, for, uh there are centers who do VB um and they have great outcomes and equivalent outcomes and centers that do va there was a child from the C DH study group that looked at that and what they did is they took all, they also data from 2000 to 2000 to 2016. Um And with about 3000 infants who with C DH, they were on that there were 2400 in the B A group and 838 BP group with that. And they were propensity matched or tried to, you know, to try to control for nonrandom organization and all that because this is all this clinical adjustment about whether they went on B A or BB, the primary outcomes were mortality and then severe neurologic injury. Um and then, and there's some sub group analysis of the timing of, of repair on ECMO. And basically, in the end, there was no difference. So if you just look at overall survival that there's and neurologic injury, there is no difference between the two groups there. If you needed ECMO prior to C DH repair, then B A had a slight increase. The survival of that. If you um did not have premo C DH repair, then VV would have um lower rates of the sort of neurologic injury. And that, so that kind of makes sense, right? If you're really sick before you had the repair done, you probably need B A. If you need it afterwards, you might not be as sick. And so VB probably works and we know in BBO in general that there is a less rate, there's a smaller rate of neurologic injury that goes along with that. But the clinical judgment and experience uh really should drive, you know, which method you use type of pump has been a discussion in the ECMO world. And so in 2005, so 2009, 7, early 2008, 2009, the advent of the cental pump came out. So we went from the old roller head, um you know, pump into the tropical pump that happened with there. And in the adult world, the centrifugal pump has been a wonder. But in the neonatal world, there's been this question about whether the cental pump actually causes increased bleeding complications. They go along with that. And so there was a, the same group ce H group and took that data and tried to look at in the same subs analysis of things and looked at the type of pump that was going on with that uh VA RCG roller versus uh centrifugal pump. Um sorry went backwards there uh in the whole process and, and really in the end, there was no difference. Uh there was no survival damage, there was no di survival advantage, excuse me, uh no difference in severe neurologic injury. The centrifugal pump probably in the early group did because we didn't know what we were doing and how to use it. I think the experience in that is that there was some more red cell lys in that group of things. So the hemolysis and the complications that go along with hemolysis. Um But in the end, and you look at it nowadays, um in our last meeting a few weeks ago, you know, kind of raise your hands, we use this if you use this roller. Um And there are a lot of cases that do that are used as and our center included in that. So, you know, once we get the baby stabilized, whether that's medically or amber with, we have to talk about repair. And so, you know, multiple approaches to that and, and timing is definitely a discussion about, you know, sort of when we should repair it. And this is sort of one slide, you know, it's sort of looking at things with a group, the group in Utah, um my hometown or, you know, my uh training town that most recently looked at whether O I can be a predictor of, you know, timing for repair. And so in their group, they took, again, they took their uh C DH to single center experience, uh C DH from, you know, 2010 through current, uh, to look at how, um, you know, can o I be a predictor of timing and what they found is that the lowest O I within the 1st 24 hours is a predictor of, of, you know, what your o I was sort of going to be during the entire course of therapy in there. And then if your O I was less than 10 within the 1st 24 hours, there was no benefit of waiting for surgery in that sort of magical, you know, 2 24 or 48 to 72 hour period that a lot of us, um, think about now, sorry. Um, that a lot of us think about now or when they're sick, you're waiting longer than 36 hours when your hypertension to stabilize. If your O I was greater than 25 in that 1st 24 hour period of things, then those are the needs who mean it's waiting longer. And so I kind of coming out with that. And so be interesting to see how this plays out in terms of timing the rest of us probably follow, which are similar to this. Um You know, we're looking at things where if you have very little pulmonary hypertension, you're gonna get fixed early within that 1st 48 to 72 hours. And if you have that pulmonary hypertension, we're gonna wait until pulmonary hypertension is controlled, which usually is more than seven, more of that sort of 3 to 5 day range. Um in terms of fixing it, you know, open C DH repair uh via laparotomy or the bar uh in order to, so here's a picture of an open repair, uh left side, you can see the liver pulling it down, spleens out there, but you can see the defect up here in this, the thor scopic repair uh in there. And I wish I was this fast. But uh it's just kind of an idea of showing you uh what happens and you're looking from the chest. So this is uh you know, where the camera is looking up completely, the axilla down towards the uh the diaphragm here. And this is the the defect of diaphragm. And this uh patient, particular patient has enough diaphragm here that we can actually just do a primary repair. And so this is just showing um again, examples of a primary repair from diaphragm dia or when the hole is big enough, sometimes you have to put a patch in uh for attack of some other synthetic patch in order to cover the defect. Um And then this is a particular baby just to show um in this one, we actually had to do an operation in the abdomen. So you may not a little bit after the diagram repair was done. And this is what it looks like when you look from the diaphragm looking up at the chest. And uh in terms of doing the operation, what it looks like in a big stand and primary repair um open versus million invasive approach, you know, is there a difference? Um And so, you know, again, this the C DH study group, you know, has taken their own data, what we submit to them in terms of looking at another 3000 patients looking at open versus mis and only invasive repair um within there. And really even in these patients, the only thing they can show the difference in between was that there was a decreased length of stay with the mis version of things, there was probably a decrease, there was a decreased incidence of small boru. Um But there were slightly higher recurrence rates in this group. Uh when they looked at things, um I think a lot of this data, we have to take a little bit carefully in that, you know, when we look at the early repair with patch, we didn't know how to sew a patch in I think, um, you know, overlap, uh you know, doing the tension free, leaving it redundant, all these things that probably weren't done in the beginning. And I know that even in my own experience when I first trained and looking at this, we just sewed the edges of the patch to the edge of the diaphragm. And I think those are higher rates, but as we look in more of our modern era when we are leading overlap and, you know, bringing a bigger patch in the recurrence rates are much smaller and actually, actually probably equal what the recurrence rates are for the um the uh standard repair of the diagram diaphragm, primary repair uh patch versus primary again, you know, this is one group and again, this is a little bit of, yeah, that totally makes sense. Um that if you have to put a patch, primary repair is preferred, preferred and if you have to put a patch and you're gonna get slightly, you know, uh worse outcomes and, and higher rates, those are bigger defects, they have other things that are going along with them, the bigger defects tend to have more congenital anomalies that go along with it as well. And so your outcomes are a little bit higher but the or a little bit more, excuse me. Um And you think about it, I'm putting a piece of synthetic in there that attracts attention and makes scars. So having a little bit of obstruction that goes along with that is just part of the process that goes along in them. And so, um, you know, just kind of uh proving or agreeing with what we sort of know would happen, like putting a page. There are some other repairs as well besides just the primary diaphragm diaphragm or, or patch in and you can use some muscle flaps. Um And so in Salt Lake City, we trained with the slit abdominal wall, uh flap and, and the problem with the, the flaps or the slit abdominal wall is you actually have to think about that before you even make your incision. Because if you make a standard and subcostal incision, which is usually about one finger bread below the uh causal margin on the left side or right side. Um You don't have enough muscle there. And so you can't do a muscle flex. So you actually have to think about it because your incision has to go down close to your uh so a Chevron subcostal type incision, but it has to be down to the level of the um like because you have to have enough muscle to be able to rotate it down very similar to like a or um and take the post to your muscle, which is the internal to the um internal and transversus ominous. Um And you've taken it off the rectus, but then take those two muscle layers down and rotate them into the position of the diaphragm. And, and so if you don't plan that beforehand, then, then you can't do that. And if you make your, if you try to do it and you have to make your incision low enough, uh then you run out of muscle and you end up having to put a patch in there anyway. Or you have a lot of tension, you have a higher recurrence rate that goes along with that flat versus patch. Um It depends who you ask really in the end. Um And so six children's and Cincinnati children's each had about 100 and 80 patients. Um And when they looked at their uh differences and that there were, or when they looked at their outcomes, there were really no difference uh in terms of recurrence rates, small value. Actually, you think that you asked the group in, then we think that the muscle flap is the way to go. Um And so they had slightly, they had very high recurrence rates in their patch group uh compared to their uh muscle group. Um And so I will say that I trained doing muscle flaps for two years uh in Salt Lake City. Um And doing that and again, I think this, what I think this really goes to is you do what you're good at, right? And so if you're really good at doing muscle flaps, then you do a muscle flap and if you're not good at doing muscle flaps and put a patch in and you will get the same outcomes in the end. You just do what you're good at in terms of postoperative management. Really, it's control of pulmonary hypertension uh that still goes along with that and making sure that, you know, we don't do anything to upset the babies. We need the event, uh initiate feeds, you know, minimizing any other comorbidities that igen comorbidities that can happen. Some people leave the chest in place. Some people don't. Um The main thing is just sort of want to take that out. Some people leave it in place because there's a higher instance of Kyle leaks, especially the THOS group. Um So leaving that in place while you feed in order to make sure that you don't have a Kyle leak in there. Um And then really, it's a lot of discharge planning and management that goes along with that when we look at sort of long term outcomes that go along with that really. Um You know, it would be hard to see, but in this side up here, we, um the study group, um CD study group has listed sort of what the defect looks like. So an ABC or D sort of category of things with D beings are the largest defects are really missing, that sort of heavy diaphragm on the side, the D group is the highest E for a patch, they have the highest recurrence rate as it turns out when you have a little the diaphragm there. You also have a higher rates of neurologic gastro and 10 or pulmonology com comorbidities that go along with it as well. And so we can look at that as when you have a smaller defect that pretends to itself the primary repair, you have fewer anomalies or comorbidities that go along with it. And that's shown in this sort of bar graph down here like a pulmonary neurologic gastrointestinal or overall morbidities. Um They go along with our, our defects, we can then take that data and look at it and we can come up with sort of what it is. And so this is everything from, you know, how long you're gonna be on the ventilator to, you know, what your risk of dying are uh depending upon whether you have C DH. Um it needs ECMO or doesn't need ECMO um back in there and you may have put this backward of the echo slide. But, you know, kind of overall we look at this and, you know, if you don't need ECMO, chances are you again, you have lesser comorbidities and so your needs for things are smaller when you need ECMO, you tend to be sicker, um and your needs, uh you know, post uh repair um tend to be higher. So, can we look at this and come up with a unifying answer as to how we should do this? So the absa Outcomes Committee tried so back in 2015, um the group, a group of pediatric surgeons, uh they involved other people as well trying to look at all the data that was out there and I get it, it's seven years old now. Um But, you know, they made the first attempt at trying to um unify and come up with AAA standard set of guidelines for how we should manage CB. Um And, you know, in the end, unfortunately, it proved that centers should do what they're good at. OK. Because there's no ran, there are no randomized control trials out there looking at management of C DH to tell us that one treatment method is better than another. So we have tons of case reports, case series, you know, analysis to look at these things. Um But what they can tell us, you know, is that, you know, what has proven out there or what we can see is that that um high frequency low pressure sort of ventilation is the standard to go. So minimizing barotrauma that if we can keep the pips less than 25 you know, and your peak 3 to 5 and keep your post to your predal stats and then sort of 85% range while minimizing acidosis, um whatever strategy gets you there and stabilizes the baby is the best strategy, high frequency ventilation. Uh Whether a general the oscillator didn't prove to be better than the other, it just proved as an adjunct. And again, if your center is good at using it, then you had better outcomes in those centers who use it only as rescue or intermittently a nitric oxide. Um we all use it right. So we have, it doesn't matter. We have pul hypertension in the patient. We put nitric oxide on them. Again, there's no randomized control trial that tells us that nitric oxide uh is better than no treatment at all. In fact, there are some studies that might say nitric oxide is actually worse. Um in C DH, the studies which say it works as a transition, meaning that if you have a baby whose pulmonary hypertension is under control, it works as a bridge to either get them onto ECMO or another um type of treatment in order to control the pulmonary hypertension, but we use it right. And so there's, but there's no evidence for it. Um you know, the Dennis or all of our treatment pul hypertension. Again, it's not that it doesn't work, it's just that there's not um randomized control trial that tells us that there's a benefit of any other treatment along the way. Ok. Uh Cortico steroids showed no benefit. Uh in the end, you know, we use steroids for lung development method, but there was no benefit shown from that nor in the postnatal period except in those patients who are proven to be adrenal insufficient. Um In which case, you can use steroids for, to help with their um their blood pressure control mode of ECMO, again, looking at the data present at the time. And the other studies I showed you looking at the B A versus EB actually came after the 2015. And so even back as early as 2015, there was no survival benefit to B A versus EV in the studies that were available. Um in terms of length on CEO, um you know, there used to be this old adage that you were on eco for more than 21 days, days uh with the C DH that you weren't gonna survive, that's probably not true. Um But there is uh the survival probably does drop off after about a month. Um So when we look at patients who are on patients who are on acma, more than 30 days, actually, survival does dramatically start to decrease at that point in time. And so, while we would never probably pull a baby off, um I think that there are a lot of discussions that begin happening once we get to that one month time frame um in terms of overall care of the baby length on optimal technique, you know, open versus invasive. Again, it's probably comparable. Um There was some data that looks at the um patch repair, you know, because of the Laros Anu use CO2 installation that they had higher, slightly higher PAC O twos interoperate results in lower ph. Um But the data really was you know, not conclusive on that. Um, but what they did find is, and again, kind of goes along with what I talked about in terms of the initially sewing the patch in, you know, tight versus making it domed redundant. But overlap, uh, was better than it was the better method, uh, later repair, you know, in terms of timing, honestly, again, it's what your center is good at. And so when we look at, you know, timing of repair, you know, going back to that chart, you know, 24 76 you know, in there on E mode, you repair as soon as you go on E mode, do you repair when you're ready to come off E mode, do you repair off um on again, what your center is good at is what you should do because there's no data that suggests that one method is better than the other. So we should hear a little bit. And so that's the treatment of C DH and kind of how we think about it and talk a little bit now about what's happened here at uh Phoenix Children's, you know, in the last 10 years with things. And so really what we've gone to is is this, we've developed this multidisciplinary team of specialists that provides care in every area. So time in pregnancy to delivery to the inpatient's day to the outpatient follow up. Um and there's this whole multimodal effect that, you know, takes place really early and, you know, and now we're following these kids, you know, I'm seeing some patients in the C DH clinic that he's in the room. So I'll pick on Doctor Chia that he fixed back when he got here. You know, and they're teenagers now and some of them are even ready to start having their own meetings. So I'm sorry, eight year old, um, you know, in the process of things. But I think that, but that's the clinic we built um and where we are and things, but how did that start? So in 2013, when we got here, we looked at this um you know, within even our own surgical group, they were doing about 10 C DH S per year, which is a high volume center. Anything more than five is considered a high volume center uh in the C DH world. Um And the survival when we look at it was about 50%. Um This was based on the cohort of patients that we can find, you know, with in the data uh that were done here at Phoenix Children's done at Saint Joseph's. And then at the time, we were covering the banner um for good slam IC U to look at that. So obviously, this is, I will admit incomplete data. Uh We don't have it all. But when we look at it, this is what we found and we thought and at this point in time, survival had switched. So the guidelines and I'll show you in 2010, um you know, had recommended sort of a treatment algorithm that we weren't necessarily following here and we were getting survival in the 70 to 80 that's when the survival started going up in that 70%. Um And so we recognize that we probably weren't doing as good of a job and we needed to do a better job. So, what did we do? So, um I will say that there was a group of us and it was Doctor Egan myself and Dr Rama Sharaa, one of our former neonatologists who sort of took, who spearheaded this and said, all right, we have the same surgeons who are operating here at PC H and then now only in Saint Joe's and we're taking care of these really sick kids, the only difference is the neonatologists. And while the neonatologists were the same group, meaning that they belong to the same um entity, they were different people, right? And so neonatologists that worked here at PC H were different than neonatologists who worked over at Saint Joe's. So if we could come up with a way to standardize it so that our neonatologists here and there were doing the same thing at the same time, you know, in the same timeframe for caring for these kids, um then maybe we can make a difference. And as I mentioned that in 2010, the European CD, this European equivalent of the CPH group came up with their consensus guidelines um and believe it or not. So, Europe is really good at getting all their, the countries country intercontinental to um really uh come together and come up with the guideline and that they all followed and did randomized control trials and looked at it. And these were basically the things that they said. So delivery at 39 weeks, the 40 neuromuscular blockade during delivery, the reducto satin at 80 95% range of targeted CO2 with permissive hypercapnia, um conventional mechanical ventilations initial strategy. And then they actually did say use I I and when they did that and switched their practice, their outcomes markedly improved. And so we said, let's do the same thing. Um And so we came up with our first version of the neonatal Management Protocol, um which is not novel, other people have had strategies before. But we fit this for what we had here at our group and looking at things and employing those strategies. And then we deployed this to both our um got buying from Neato here and we got buying from the neonatologist over at Saint Joe's. And what was unique probably to us was that this was the first time that our group also looked at it and said, ok, but if you're a really severe C DH, maybe we should look at other things and instead of doing things to the baby we should, maybe we should look at how we're doing things for the baby and not only that, how we're doing things for the family. And so we came up with a set of guidelines um that actually looked at the severe category of things and if we couldn't ventilate a baby, meaning get their co2 under 100 if we couldn't get the ph up things like that and those are babies who are in our group right now, weren't going to survive, at least with, with in our current means of technology and our current means of care. Um And then got really involved in palliative care team with those babies. It wasn't a lot, it's maybe one in the entire group, but it was an important step in our process in terms of looking at this is how are we taking care of the babies instead of, you know, and doing things for the baby in the family versus doing things to the baby um in that process. And then I will say in this whole time frame as well. And many of us know the fetal care program here really took off. And so doctor Van Leen and the group, you know, sort of had this idea. Um you know, we weren't taking care of the babies over at Good Sam anymore. Um And we just saw a little drop off in our numbers in general. So how could we go out to the community and really tout what Phoenix children's was doing at that point in time and sort of building our care process. Um And so, you know, we got the fetal recently the fetal care program, as we know it today, we started in infancy, you know, around 2015 or so, you know, hiring a couple of coordinators and really pulling a few people from all the various specialties in to say, what can we do to take better care of the BES? And so along this time in 2017, we sort of updated our process, but really updated the whole management process. So again, going back to my earlier slide that we took this whole prenatal evaluation and put it down into an algorithm that said, look when the an obstetrician in the community gets a baby and they do their high, their high level ultrasound, they need a diagnosis of C DH, what do they do? And so we pass this around and again, it's hard to read, but you can see there's a number and it says if you have a baby with AC DH and you're concerned, refer them to us and not take the baby away from you, we can, you know how you deliver and manage and take care of the mom that's really up to you, but we can then give the mom at least or the family some information about what C DH is and what's gonna happen with their baby. Um Look at that and then have this whole process for Holly would refer to various subspecialties, um you know, depending upon what was found and that pathway became even more uh you know, uh owner and complicated as you start looking at, you know, ultrasounds and adding MRI into that. And then, you know, when they fit into our mild, severe or, you know, uh category of things, you know, who do they get referred to in terms of looking at this. But this whole process that, you know, subsequently developed to come up with this plan of how to take, you know, this multispecialty uh care for the families. Um You know, in 2018, we updated our protocol. There's some new data out there. We had the uh outcomes from the new study groups, you know, from the C DH study group with things. And so, you know, mild adjustments that were made to this um around this time also is when, you know CD or PC H begins to think about how, how we're going to uh run our N US and what's happening there. And there's a big transition in our nu care in our, in our current group, uh with Doctor Martin and doctor and the team sort of come in and begin taking over our nu here and then subsequently in 2021 began managing the NU over at Saint Joe's as well. So I'll tell you when you have a cohesive group you have a group that then has a symbol like mind about how we're gonna take care of patients. It's amazing what you can do at that point in time. Uh because you can all come together and then you can form multispecialty teams that do things all at the same time. And so in 2018, we initiate the CBH rounding team. Um we get buy in from the ecology, we get pedic surgery. Obviously, we're invested cardiology. We have a dedicated pharmacist, a N pharmacist, but obviously she's a, a dedicated in this whole process of things. And then we get b from pulmonology and Neth, which are our two most frequent consults um in the neonatal period. Um to say, all right, we get a baby with C DH. We're all notified, doesn't matter whether it's one o'clock in the morning or not. We all get notified of the process that there's a baby there. Then we all plan to meet at 8 30 in the morning at the bedside when that baby gets there. And if there are problems before, then they can call any one of us that's on call for those specialties. And we will come to together at two o'clock in the morning if we need to in order to manage the patients. And so we start these daily multidisciplinary rounds. They occur every day at 8 30 while once the baby is born until honestly, the baby reaches the point where we feel that they are no different than the standard Nikki baby. Uh So after repair and they're at the quote Peter Grower stage and then we'll all say, OK, now it's time we don't have to meet anymore on this particular baby. And we can take a step back and let the n you manage them the way they normally would. We refine again, refine the protocol again. And did you notice every, with every iteration, it becomes a little less uh sort of complicated. And there are the things that we felt are really important to stand up now on the protocol and this is what this was actually, we just revised this a few months ago. Uh Now, looking at that in terms of our initial management guidelines for that and I will tell you this has gone out to almost every hospital uh in the greater Phoenix area and around um because there's a problem in there for even if you don't know. Right. So a mom walks in, delivers a baby and they find that the baby has C DH now, even our local obstetricians again and neonatologists, you know, nurse practitioners, whoever is pediatricians can look at this protocol and say, OK, if I do these things and manage it upfront, I can buy the baby some time until I can get them transferred to that tertiary care center, which is either here at Children's or um at the one of the other local hospitals ok. Um And we do this and as it turns out, we actually, and I will pick on that again because in the room, we actually found a flaw in our guidelines, uh right after we wrote them and published them out there again, because what happens when you have a baby who we know has a DH but has all their predictors that are really, really good. Right. So we're planning for the worst babies in this case, we forgot to plan for the babies who come out and don't know that they had a Diro hernia. Um And so we'll have to make some uh vision to the guidelines here as well as we go through. But in this time process now we gotta, we've been thinking about how do we take care of these kids once they're ready to go home. And so we created this new C DH transition to home team. Um And really, it's a group of RN and MA coordinators who meet the patients basically as soon as the families get here, uh they start, they meet the mom and dad uh in the family. Um and they work with them throughout the entire hospitalization. Uh just even the point of contact or as a sounding board to set up what's gonna happen when their baby goes home, coming to the C DH clinic, coming to the Neuro Developmental Clinic, um and whatever the other appointments are and they all get this and hopefully it turns out a little bit here, but they get this sheet that says, you know, what they're expected to do and, and kind of give them an idea that their baby with C DH is gonna need follow up and pretty extensive follow up and when that follow up needs to happen. And then down here on the bottom are the numbers that call again, the three most common use are pediatric surgery, cardiology, and pulmonology. And so those are the numbers to their clinics. So their call service so that they have that readily available to them and can call 24 7. And the long term remains if you go again, that slide that looked at all of those things that can go on in the baby with C DH, you know, and in there. And so how do we follow all that and, and um and keep track of that. So honestly, what we did is we developed C DH clinic. Um and so myself, Doctor Menon from cardiology and Doctor Williams from Pulmonology. Um you know, agreed that, you know, again, it's the top three specialties involved with that, that you can come together and we have a multi, it's a once a month multispecialty clinic. Um I did put Glenda Gilbert, she's our RN coordinator for the clinic down here as well because quite honestly, we couldn't do this clinic without her because she arranges all the patients to be in there. Um but we see them and you know, we started this process in about 27 or so in there with the C DH Clinic. And honestly, it was pretty boring. I got to know Doctor Menon and Doctor Williams really well because we hang out together to see one or two patients. Um and you know, slowly, but now we have over 100 patients involved in the CV H clinic. We're meeting once a month, there's on average 10 to 12 patients per clinic that we see um that need all of these things. And then we can not only follow their, you know, the out the local outs from cardiology, pulmonology and then the recurrence rates, but then get them referred to the other sub specialties as well. The Nest clinic has really taken off as well as the neurodevelopmental clinic. So all of our babies that we know about at least born here are automatically referred to the Nest clinic and follow their their neurodevelopmental outcomes. But not only is the patients born here at tch but other patients born at other hospitals who are taking care of, you know, at our local competitor, um end up getting referred to us. And so we see them as well and I put, we put them in the nest clinic right away. And so just this whole collaborative group that now has built this whole process in order to take care of our C DH patients. So what has that done. So if we look again, go back to our overall survivals and mortality. Um You know, back in that 20 kind of 2012 13 round when we got here, we had about 50% mortality in here. Um We started our kind of first program, our first iteration of those guidelines back in 2013, 2014. And we saw, you know, some decrease in mortality at that point in time, 2015 was a weird year. We had about 10 CD patients and none of them died uh that year. Uh And so, you know, it's a little bit odd but that if you look at our trend line, you know, come back up. Um What really happened in there is that between 2014 and 2016 as we put our guidelines into effect, we get buy in from both institutions. Again, that's both PC H or PPP Children's and Saint Joe's, uh the different neonatology groups um that are there different neonatologists are there? We get buy in from that they follow the protocol to a certain extent. Um But our mortality immediately drops. Now, whether that drops because of the protocol or that drops because we're paying attention to it. And we actually have a multidisciplinary team who's going to take care of these patients, you know, hard to say, but the protocol is there, right? And so we see this effect and then in 2019, again, as we get our second sort of group needed here when we take over the, when PC P shoulders takes over these nick and we have a cohesive group. It's the same group. Neato that are both at, uh, Ph children's and Saint Joe's. Uh, following this protocol. We get our second drop in mortality. So it's that we're hanging out probably around the 10% to the 10 to 20% realm at this point in time. If we take it as in, in a sort of uh E Boxx, meaning that if we look at 2010 to 2015, our overall um survival was about 60%. Ok? When we look at that, when we look at those numbers, uh and survival not mortality. So 40% mortality uh within that group of things. If we then take that second group and go 2017 to 2020 and looking at it, our overall um our survival goes up into the 85% of things and that's all comers not, I'm not um stratifying that be based upon mildest severe uh conditions. But our overall survival in this group goes and uh you know, very high with the 80 to 85% of things now. And I'm looking at some of my partners in the room here and they're thinking we have some really sick kids and I will tell you they're in that time frame as well. And we're working on the data right now to make sure it's clean. I don't have the exact ones to give you, but our L hr S they're in that time were very low. We're in the less than the majority of our patients are 1.3 and lower. So, meaning that we're in the more sort of moderate and severe category of things. And those babies are surviving in pic children's hospital when they used to not. And, and we can compare those to some of the published data out there from a couple of centers who say that they are the best in the world at taking care of these C DH, you know, and having these survivals in 80 to 90%. And we, we are as good as they are C DH and echo. So if we look at the numbers and in this graph, I don't share on the other one. So this is the white is looking at sort of the total number of C DH taken care of by year. Um And already in 2022 we have taken care of 10 CD patients um within our system. OK? And then sort of the red shows you the number of patients that were put on ECMO at that point in time. Um So, ECMO, you know, we don't use a lot of it in CD anymore. And then the number repaired on EO and then what I can compare the next year is sort of what our mortality is uh based upon our overall mortality and in 2019, we didn't have a great year. Uh, we lost all of our patients that were done on eo uh that particular year and same thing in 2014 and 2015. Um That again, 2020 2021 2022. Um, you know, we've had some patients that have gone on ECMO, they survive. Um What I was telling you that happened in 2020 in terms of changes is we changed how we do our bleeding protocol on. Um And so we made a bunch of changes to that. And so our bleeding more, our bleeding morbidities, uh decrease uh significantly which I think has led to some of our, uh which has led to our uh decrease in mortality as well. Uh While on a, so we've taken this sense system. Uh We've built it along and so the title of my talk was if you build it, will they come. And so the answer to that is yes. And so what I'm showing you here is the number of referrals for C DH patients that have gone into our fetal care center uh since 2014. So we look at, you know, it's not that we weren't seeing patients back before the fetal care center was developed. We were right. So they get a diagnosis, they would call, um, you know, our, at the time, the, the private practice office and we can get in and we'd see these um old consults for C DH. And then we get our feet honestly, the field again, around 2015 in there as we start developing the fetal care center. Um and then we can just watch the number of referrals go up interestingly in 2019 as average else, there was a decrease in the number of referrals that came in during that year. But then right back up in 2020 20 little small decrease in 2021. But again, the trend line, that sort of looks here um is that we are continuing to go up, there have already been 22 referrals for C DH in the first half of 2022. Um that we've seen as a process of growth of our. So where are we going with C DH care? I really think you have to divide that into 22 processes, right? So I think that there are gonna be, there's the future of treatment for C DH and then there's gonna be the advances in our own local program and sort of what we can do if I, so looking out there, looking at the literature, what are the advances in C DH care or where are we gonna go? And I think that actually comes in two rounds. I think it's PTO or the P fetoscopy end Traa infusion that's been going on. So, proven some efficacy in the severe group right now, the trials are actually looking at that moderate group to see. Um and they're, they are randomizing patients uh and that to receiving feto if you fall into the moderate group. And right now, the preliminary data would show that in the moderate group if you fall into an L hr of less than 1.3. So in the more severe end of the moderate group, that there probably is some benefits tracheal inclusion in terms of lung development and decreasing pulmonary hypertension stem cell therapy. So, the group in Texas right now is looking at stem cells. So if they can take cod blood, um they can um filter out the stem cells that go, that go along as a special specialized work form of those stem cells. They can actually inject those into the lung um that will simulate lung growth in the process of things. And so preliminary work all in mice so far. But that's, I think that's the translational research that will be coming along over the next couple of years. Uh Sean Kisa, John Kisa at uh Duke is looking at taking stem cell research also, but he's looking at growing a diaphragm. So you have a baby who you know has a chromatic hernia. Um they come out, you take this, you take the cord blood, you filter out the stem cells, he then takes those uh with a little piece of muscle tissue and then he can stimulate that to grow into a diaphragm so that you take and you give the patient basically their own muscle into as a patch. I mean, it's a patch right now, right? It's not a functional diaphragm, it's a patch, but it's a muscle patch that theoretically will develop a blood supply uh that can grow uh while the patient with the patient. And so hopefully decrease um the recurrence rate within our own program. I think the area is that we're gonna see growth. So, ECMO transport, right? So we know that a lot of, well, even though we recommend that these babies are born in a tertiary care center and, and for us, that means Saint Joe's within, you know, within our system. Um but they're not all born there. Um And sometimes patients don't know, they have AC PH and they're born on the outside and then those patients can get sick. So can we go get them? Not only can we go get them, but if they're really sick and we put them on ECMO there and then bring them back uh to PC H. And I will tell you the answer to that question is yes, because I've done it um in the last, in the last year, in fact. Um And so, you know, the program is there and you know, buy in from administration and just grow, you know, need a and, and all those other specialists that we need to grow our program and we need, we need to be the leaders in terms of taking care of these babies within our own state and then just an out outreach, right? So, so what, so we're taking care of these babies, but what happens to them in the end? And so doctor is looking at a group at a study now where we look at um access to health care. And so which patients are actually using our system? Right? I know. So I have 100 and some patients that are following me in C DH Clinic. But are we, I can tell you we're not following every patient that we've ever seen and delivered here or taken care of. And I know that because I, I got gathered data from, you know, looking at our numbers in C DH, I saw a lot of names and thought I've never seen that patient in C DH Clinic. Um And so I think there's a lot of outreach and follow up that we can do and we'll look at that and the health disparities that go along with that. Um In terms of developing our program, I would be remiss if I didn't if I said I did this alone, right? So I think I got the picture out here. I got to put doctor, you get in there. Um and then also a blank space because I need a G I doctor. So G I needs to go in here as well. You see um really community doctor doctor and doctor, Doctor Williams from Pulmonology, Louis has been called this and his team from, uh, radiology looking at things Karen Pez and neurology, uh, Shred Men. And, um, and then RM Cur is our Nicky, uh pharmacist that really works with us and honestly, we couldn't have done any of this without sort of buying and, and helping each of these people. Um, and then our, uh, sort of fetal care coordinators and so Abby and Noel are, are two, they, they're our enemy ma coordinator who go to the NICU and meet these families and follow them and can really stay involved the entire time. Um Tiffany is, is actually on the cardiology side, but as I've mentioned, you know, most of these THBPS actually have a cardiac defect as well. And so Tiffy is really working with us, Jessica, our original fetal care coordinator. And so now it's the manager in the system, but really it helped take it off and then I will say that I'm a lucky guy because I got to work with Alicia twice. Um And I could never have done this without ali to be quite honest. Um, you know, not only from the side first got here and developed the program, but now that she transitioned and, and is now one of our fetal care coordinators. I mean, she honestly is the face and voice of the fetal care program. So she sees pretty much every C DH that comes in and she's, has been responsible for over these last couple of years. And so, um, again, I give the kudos in the team building approach to, you know, to these five women, uh, who really taken it over and manage it. And with that, I will say, are there any questions? Uh, so when, when they're doing the veto procedure, what's, how risky is that for the, for the mom and the baby? The question was uh in the veto procedure, what are the risks of the mom? And so the biggest risk so that they are sedated. So it's an, it's an procedure. So mom is an for it. Um and the baby and almost enough to make the baby Anestis it, the baby is as well. So the biggest risk to mom is actually premature rupture of membranes. Um And so there is a higher incidence of premature delivery. I mean, the vito group compared to the non feto group. Obviously, there are some things that they've done over the years to change the anesthesia to try to minimize that, but it is an irritation to the process and vito can't be done with a really an interior um placenta and so they can't go through it. So there are some limitations to how that can be done as well. Yeah. So, um the, you, you briefly went over the results of patients on va and, you know, for the neurological and for years like what we've been saying, oh, you can like the product but some way that that kind of, so the question is on va eo um that there, there's a slight increase in mortality in that group. Um and neuro neurologic injury, I should say uh with that particular group and uh because you like in the car artery and is there any evidence to suggest that we shouldn't be doing that? And the real answer is we don't know. Right. Um So the most recent studies out there say that there is no difference in neurologic outcome uh when lighting the carted not. And that's just, you know, particular studies looking at that. It'll be very interesting because the group in the UK. Um and they, we try to do that here, which is really hard. Um And maybe we still can, but the group in the UK will probably publish this data first. So they are looking at the CD, a consortium for all of Europe. Um And they have been able to get their data, look at uh ECMO non eo uh carted ligation carted repair. And they are now following these patients and I believe they are going to publish their five year follow up of the kindergarten data. Um Probably next year is what the doctor has told me when I was talking to her also a couple of weeks ago. Um And so, but it'll be so that's great. We'll so we'll have that. And so I think we'll get our first glimpse at that answer. But it'll be very interesting is because they also have patients who are now in high school in the process of this as well to actually give us our probably first look at what the true developmental outcome is of lighting. The A, the group. Um There's a group in Atlanta, Texas, children's obviously with the CD, a study group, we're trying to get that data here. But as you can imagine in the US, it's a lot harder to do um and follow up and to get people to participate, it's hard to get people in the US to participate in multi institutional studies and give up their data. Is it the best point? Uh I can think about see you um if there would be any role for a month to try and continue to keep in contact with these in addition to the numbers that are on. Uh So the question was uh is there any thought in terms of our outreach and development uh in, in terms of using telemedicine and actually some, that's something I forgot to mention in the whole process. So we actually do. So most of our fetal uh so I'll put the whole thing. So most of uh most of our fetal um counseling groups that go on are actually by telemedicine. So make it easier for families to join. It also allows us to be able to have these conferences seven o'clock at night if we need to because that's what's convenient for the family and again, buy in from the whole group. So, um you know, in terms of being able to do this, and I will say that the my best one yet so far from prenatal counseling was that I was meeting with the family recently who were here in Phoenix, obviously for the mom and dad, but grandma was from India. And so she was uh zooming in from India to join the conference uh while we were um doing the prenatal counseling, but we do use um Zoom, you know, as, as you've adopted Zoom here. So we use Zoom and as well. So even for ce clinic, we will use Zoom, the coordinators, the MA R and coordinators uh keep in contact with these families either way, phone Zoom in person, whatever needs to be done. So we are utilizing that. But I think there are other ways to utilize that in the future in terms of keeping in contact with the family. So definitely something we are looking into that dream, right? Thanks. Wonderful review of what seems like a lot of major development or relative for a period of time um this program. And um when we get into the uh where we were mainly for a couple years after repair, um what is the difference in how we are talking about the occurrences like a lot of other surgeries that we do and uh the occurrence is like the four letter report and the most of the surgeries that we do. Um the CD. So I think the, the question is how do we counseled and families, um, you know, both prenatally and post and post repair. Um And for the risk of recurrence. And I would, I would say, is that like, you know, most of us, I think in the, I do all the, the prenatal counseling, obviously that's distributed within our group. I think all of us go over that, right? We just sort of say the occurrence is happening, but I will say that we talk about in the clinic or at least the one that I see um like the comments on is I don't use it as a four letter word. Um But that I just tell them that it's part of the process. So it's a, it's, it's development, right? So they didn't develop a diaphragm. Um And so we gave them one or we gave them a separation between, you know, their abdomen and their, their chest cavities. Um And sometimes it grows and sometimes it doesn't. Um And so we just have to follow that it's part of the process and that at some point in time, we likely will have to do another operation. So setting the expectation that they may need another operation without the fear of like, you know, oh my gosh, I gotta, you know, he sniffled. So I gotta take him in and, you know, look at this from a recurrent standpoint of things. And so I think it's all about setting expectations. Um And I know that that's what I've done. And from what I can gather is that by the time they come see me in CD, a clinic, I try to send um all of the patients at least a few times to see the primary surgeon before we, you know, sort of get them into the C DH clinic. And I will say that when I have that conversation with them, um, all of them have said like, oh, yeah, Doctor Egan told me that before. Oh, yeah, or doctor somebody, they've all said like, oh, that's what they told us before. And so I think even within our own group looking at that, we're already doing that counseling to prepare them for it. Ok. We're over time, but we got one last question and this one is short. Excellent talk. Are we recruiting for a fetal surgeon for a fetal? Um, that is beyond my. Mhm. So, all right. Thank you so much. Everybody. Have a great day. You end up here. Yeah. Yeah.
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