Dr. Benjamin Zendejas-Mummert - EAT Clinical Research Updates

you you you you you you you you yourock awesome you you you you you you you you you you you you you you you you you you you you you you you we have Ben zendejas Ben grew up in Mexico my is medical degree in Guadalajara and he obtained his master's degree with a focus on surgical education at the Mayo clinic in Rochester, Minnesota and that is also where he completed his surgical training. the faculty. And since that point, his career has taken off. And he has really made a huge contribution to pediatric surgery within the areas of a soft-and-jewel and airway treatment, as well as vascular ring. And so he currently serves as a surgical director of the EA treatment program, as well as co-director of the vascular ring program. He's an assistant professor of surgery at Harvard Medical School, but most importantly, he plays a directoral in the robust education of the fellow's day in and day out. And Penn has taken us through many of the most complex cases we do, very harrowing operations. And he somehow maintains his calm disposure throughout all the cases and teaches us a lot along the way. So, Ben, we are so grateful to have you. Thank you so much. Thanks, everyone. So, when we ask me to speak about sort of what we've been doing lately, in the soft-and-airway center, I thought, well, gee, we just talked about this, like not that long ago. So, about two years ago, I looked back and literally almost to the day two years ago, I gave a last-generalized surgery ground rounds, and we talked about, as soft-general and asthmonic outcomes and the use of spi-fi and vocal cord assessments and injury rates and nerve monitoring and all the cool stuff we were doing back then. I was like, well, what could we possibly have done differently or new in two years? And I just started looking at what amazing work our team has done in two years. And I had to cut down a lot of the things that I would like to talk to you about. So, I'm mostly going to focus on three main things. There were many more things that our team has done or implemented or innovated in the last two years, but these are three things that I think have a different flavor from last time we talked. I'm going to give a light into some of the future in our team and also some of the very exciting work that they were doing. I'm just here to present. Most of this work has been done by our research team and our nurse practitioners and our other physicians. So, I want to start with saying that that I'm more of an envoy to all of this work that our team's doing. So, we're going to start off about tracheplexi, some of the outcomes that we've looked at lately in some of the future directions. So, off the basics for some of you who don't know what trachamalasia is, it comes from the word soft from the Greek and essentially this is a normal airway. You can see very nice airway clean and then look at particularly pay attention to the shape of the carlogist. This is very, we call it C shape or U shape in a narrow membrane. The membrane has a lot to do. The width of the membrane is very important. Now, contrast that video with, you see that's a normal cloth, you know, about 40-50% of intrusion is expected. Now, contrast that with this other one and see how wide the membrane is and how bow shaped these carlogists are. Very wide, very less structural is just not there. So, every time you coughs, it's like almost 100%, essentially 100%. And even at rest, right there, it's like 80%, 80% post-year intrusion or so. So, very sort of soft floppy airway. Okay. Now, the, what I was talking about, I think is sort of the mechanism as to why the airway, so floppy has to do with a lot of the structural integrity of the carlogist and the wider that membrane is, the more risk of intrusion. And so, why does it intrude? There's one big component to this, which is the esophagus. Right? Most of us are esophagus to the left of our sort of airway. And it's a geotrusion page, it's particularly esophagus ends up being a little more rightward because we've been taught to fix the esophagus from the right chest because the heart's in the wind left chest. The esophagus naturally likes to live right behind the airway. So, if you have a very floppy airway in a very wide bow shaped carlogist and a lot of wide membrane, then naturally this esophagus is what actually intrudes. So, keep that in mind as we talk about other elements. So, how do these kids present? Right? A lot of variation, some that are totally, very severely symptomatic, and others that are not at all. So, the most severe symptoms, and we're going to focus a little bit on these and how we treat these are these called blue spells or death spells or more proper, the proper term is now altes, acute life-threatening events that used to be called, or actually, that way around the other brief resolved and explain events. So, the other ones are the ones who come failed excavations, inability to win respiratory support, CPAP, bipap, etc. Others who were managed to not have some of these or may intermittently have some of these blue spells have recurrent prolonged respiratory infections, pneumonia, bronchitis, ICU stays, ER trips, visits, etc. Or the other ones who maybe just more like chronic cough, the Barkey kid who every time they cough, people turn around and be like, oh, whether something wrong with that kid, are you sick? And they're like, no, that's my cough, and so, they can have feeding difficulties, they can have sleep disturbances, exercise intolerance, etc. Or be truly asymptomatic, so wide variation symptoms. And so, one of the biggest things we hear is that in the natural history is really poorly studied, to be honest. There's a really not a lot of data on this, but the biggest misperception is that your child will grow out of it. This is what, there actually isn't pediatric textbooks and all the pediatricians tell this to their patients. Unfortunately, there's absolutely no data to support this flame, right? Some may but not all do, and we really don't know who will and who won't, and how long does it take. And the biggest question I ask is that what cost and what burden to the patient, to the family, to the healthcare system, are we willing to wait? How many pneumonia hospital missions is just one too many? We don't know, right? And we also know that trigamalasia is present in all age groups, including adults. In fact, I think across the street, the Brigham and the B.I. have a very busy adult trigamalasia practice. So you've sort of heard about how they present and what their symptoms are, and so why do we treat them and who needs treatment? Right? I like this table because it kind of puts you in these categories where symptoms are severe, and your airway shows a very bad airway with a lot of trigamalasia, then these are kids that mostly get a trig apex, right? Our surgical treatment. We'll talk about that in a bit. We have some kids who have very severe symptoms. We'd look at their airway and it's totally normal. So this is the ones you have to really think about alternative explanations, aspiration, immune deficiencies, other things, and we start them on medical treatment. Other kids have maybe moderate symptoms, and they may have moderate severe trigamalasia on bronchoscopy. We always would like to start them on medical management to see if we can optimize them. Sometimes that's how they need, you know, good pulmonary clearance regimen, and they can avoid an operation. If they become refractory or they get worse, they may consider a surgical treatment. There are some kids that have really bad looking airways when we do a bronchoscopy, they have no symptoms or very mild symptoms, and so we just watch them, all right? Then you may put them on medical management only when they get sick to help them clear the infection a little bit better. So now, for the surgical treatment, you've heard our team talk about this a lot, and the short story is that there's not a one size fits all, right? We really like to tailor it to the underlying location severity and character to the collapse, and think about concomitant issues, such as syphagiotrictors or lung pathology. Now, you've seen the or heard about the posterior trachea plexus, essentially the essences. We move the esophagus usually to the right, not always, but usually to the right. And with the series of sutures, we underflocks a bronchoscopy guidance place these pleggeted sutures on the posterior memory of the trachea, which is that pink area right there, and bring it to the anterior lung, so you'll look them into the spine, and nicely open up the airway. All right, and so this works for the vast majority of kids, particularly these kids, we have a really broad-bosche in cartilage. Right? So that has predominantly been now our sort of default sort of strategy. And initially, Hester presented this paper back in 2017, which was an initial experience with 98 patients. These were all comers, right? And in general, most of them improved, significantly, particularly these with severe symptoms over here, the blue spells, the altes, etc. Right? So this was our sort of initial look at this, but it didn't go too very deep into the elements. But so what if we look at, okay, what's happening nationally, right? Are we just seeing the worst or the worst? And are we getting a biased opinion? This is a nice study that came out recently that looked at the FIS database. It's a little data because the data says from 2015 before that. They looked at more than 2500 EATF children from a wide variety of hospitals in the country. And the fact that you can see the burden of airway issues for most of these kids, three out of four had some sort of error digestive procedure after their esophageotriger repair. So that means a prong and then that could be something. And you can see that about 6.7% get a trick yesterday. The vast majority of the trick yesterday, as they happen in this patient population, are due to trigumlation, unfortunately. We'll talk a little bit about that. Only about 2% had an aorta pexy. So we probably weren't necessarily in this in the state of set at that point in time or something, but so a significant burden is the bottom line. And this video sort of showcases why trig yesterday is not the best thing for trigumination because it's only putting the finger trying to cover the sun. It was a trigumlation not going to go away. There's just a different way to deliver positive pressure and the trigumlation is still there. In fact, some of these kids still continue to have blue spells even with a trig gastomy. If you could think about putting a longer trig in, that sometimes works, but it doesn't solve the problem. Now up until now, that was all just all news. This is really cool stuff. It's a little bit of a busy slide, but it's a summary slide of some of the recent work that we've looked at because particularly to address this element of should a kid get a trig gastomy when they have severe symptoms, right? And this is I think a categorically no. As long as there's subplotix space and their vocal core function is adequate or acceptable, I think a trig apex should be the way to go. And I think this is going to be move the field forward to try to change that. For example, these are a recent cohort 80 patients of truly just EA, the majority type C. There's something about the type C configuration that just makes the the trig yet be so much wider from a membrane standpoint compared to the A's and B's. And this is the classic sort of six month old kid who goes home, gets distressed from the hospital and at home is having blue spells or the one who just can't leave the hospital. They're like two, three months intubated multiple phylicesturbations, failure, wiener, bypass, heap app. So these are really the most severe ones. And all these kids have had recommendations to get a trig gastomy. These are ones who were recommended a trig gastomy apparent said, no, thank you. Let me see a second opinion. And most of these kids came to us as a transfer either intubated or or or or positive pressure. Some came from home with these blue spell history. The vast majority had trig apexes performed, some had more than one operation and some had an anterior trig apex. Now the biggest take home point here is everything improved. But the most importantly, 94% avoided a trig gastomy. The few that still needed a trig gastomy, the ones that have very severe subgalatics to noses injury from chronic intubation or they have bilateral vocal cord paralysis from prior operations or from from us. But the vast majority were able to come off the vent, positive pressure and most most went home with room air status. So that this is I think very significant. We're going to be presenting this data to absent and may. Now what are others doing with these children who have these blue spells or altes, right? So this is a nice paper that just came out from Toronto. And their rate of altes is also high. Look at this 22%. Now pay attention, that number we're going to talk about that in a little bit. But out of the 250 not 266 patients that they treated in this time frame, 59 and 22% had a blue spell or alt. All of most of them within the first year of life. And this sort of graph talked to you about people really don't know how to treat these. They get all sorts of interventions, right? They got a fun application, they had an airway pexi, maybe both. But look at the the recurrence of altes, right? They really didn't address the issue. They still have recurrent altes. If you go back to what we showed, none of them had a recurrent alt. Right? So you need to understand, address the reason for it. And with a proper tracheorepexi, I think you can avoid recurrence. I think it's highly uncommon. I would say that if for this to have a recurrence, unless you're not truly treating the issue. So that's recent data from a high volume center. Now this is another really exciting slide. And I'm going to start for it for it being busy. But this is also new data that some of my and Ali worked on recently and showed very strongly how this concept of well, if all these gets have trigumalasia after the fact, why not just do the tracheorepexi of the time of this object, your treature repair, right? And it sounds crazy. And it probably is. But in our hands, it just adds about five minutes. And it really changes the life of these kids. And particularly in the first year of life, these are the consecutive newborns. Some repair by the EAT, some not repair by the EAT. So this is all covers at our institution in this sort of five year periods. So really contemporary cohort who got a tracheorepexi who didn't was really up to the index surgeon. In fact, there were some of these who were done by a surgeon in the EAT, that didn't have a tracheorepexi up from. Those are particularly kids who have very low birth weights because it's probably a little riskier to do it in the really premature babies. So that was the only significant difference in the sort of demographics. But just look at their hospital state, shorter hospital state. No blue cells in the first year of life. No hospitalizations in the first year of life for a risk of infection. No need for a delayed tracheorepexi. And look at this 24% of the other ones. Yes, probably arthrocial to do an intervention is way lower than others, because we just sort of they're here. Some get coughs and they get referred to us for a bronch and a tracheorepexi. But nonetheless, look at their rate of blue spells, almost 20% very similar to what Toronto found, 22%. So I do think this is a reflection of what actually is happening in the community. Where roughly if you don't do a tracheorepexi, roughly about one and out of four out of five kids will have a significant risk to prevent in the first year of life. We see this all the time. And the other thing very important here, we have, Lindsey, our nutritionist here can attest to it. Their nutritional status at when your life based on z-scores was better. They're not getting sick. So they're eating better. They're not getting sick. They're not coming to the hospital. They're nutritional status better. Okay. So another very important element. Now we're really not the only ones doing it. In fact, we weren't even the first ones to publish on this. The group from Dr. Van der Sien and other lens published this a few years ago, actually in 2021. And they do an ethyl scopically for the newborns. And they saw how the look at this, the incidence of brewery or altteat, 27%. All those numbers match up. We're not making it up. Tracheorepexi only one had a brewery event, 7%. Almost significant, just low numbers. Recurrent, rest retract infections. Also lower, just not enough power because just small numbers overall. But they're doing it. Now even in China, this picked up. This is a paper I came out recently from a Chinese group. And they also start doing primary or prophylactic tracheorepexies. And you can see over here the frequency of respiratory issues is less in the group with the primary tracheorepexi. So they have, for example, there's numbers are also not totally significant, but what they call respiratory dependence, sort of any sort of respiratory issue is less than those in the control group. So again, more tracheastomies in the group without a tracheorepexi, more procedures, etc. So I mean, it's something to consider. Before we switch gears, I want to just do a little summary on the tracheorepexin would anticipate would be the future directions for this particular setup. We could show you how they have significant respiratory morbidity, not just from tracheoremlasia. We didn't even go into orangeical class, vocal cord dysfunction, etc. We will end into that in a little bit. I do think tracheastomies the way to go is opposed to tracheorepexies the way to go is opposed to tracheastomy for these severe life-threatening symptoms. For us, this makes total sense that elsewhere, these kids are getting tracheastomies left and right. So this is something really that we're working on trying to get out there. I do think that if we look down the road in five to 10 years, a significant proportion of kids will be getting primary or perfilectic tracheorepexies at the time of EAT, I prepare. Because it does show that it reduces respiratory morbidity, particularly in that first year of life when it's so critical. So just when you thought that you've heard everything about tracheorepexies that you possibly could or that we have it figured out, we really don't. We do have a set of kids who fail, right? And we need to accept it. We have a set of kids who have more than one operation. And when we start thinking about it, well, why is the case? There are some kids, this particular video is of a patient who had opposed to your tracheorepexie and was initially well and then recurred and had symptoms. You're like, oh my god, it looks like we didn't do anything, like what are we doing? And you're like, well, what happened? Did the sutures come off? Did it just like not hold? Is there something intrinsic with this patient? I mean, this just looks really bad, particularly at this sort of beginning that I showed you. And what tends to happen is these kids who have a really wide bow-shed cartilage or very wide membrane, the front wall is just so wide that just sort of descends on the spine. And so although the posterior membrane is not moving up, it gives you the impression that it is, but the spine acts as a fist and the anterior wall just descends on the spine. So here the problem really is just the shape of the cartilage and the width of it, right? So keep that in mind. Now, historically, we've treated these anterior descent or anterior intrusion with an anterior tracheorepexie, about 10, 15, 20 percent of these kids get an anterior tracheorepexie. Usually it's a year or two afterwards because we get this scenario. There are others that have vascular-related compression, such from a vascular ring that get a vascular-related pexie or relocation of the blood vessel. There are others who just have really floppy cartilage as they've been on steroids all the time or just really mal-shaped and get a splint, an external splint. But really, this idea now became to can we taper or pli-cate or even resect some of this membrane to reshape it, right? And so we have initially thought about, well, how do the adult folks do it? Because the adult tracheorepexie is very similar to this, sort of really bow-shaped, wide-shaped cartilage. And they do application. They do it with mesh, right? So this is what was described in adults and even do it with robots. And they essentially anchor the mesh on one side of the cartilage, anchor it to the other side, and sort of riveted, and pli-cate it. So it's, it kind of brings the edges of the cartilage closer to each other and makes more of a C-shaped cartilage. And it makes a lot of sense, but that's a lot of morbidity, particularly having mesh for 70, 80 years in a child's body. In particular, most of the time, we're also doing a soft-gear procedure, so having mesh with a soft-gear procedure may not be the smartest thing to do. So we wanted to try to find a way to do this without mesh. And we came up with a really sort of neat approach. And so this is another example of just, you know, bad malaysia. This is a child who, who with, you know, cough has their complete collapse. If you look at that, we usually sometimes have to tickle them. That's what the catheter is they're doing for them to get them to cough. We're really bow-shaped cartilage, just wide membrane. You say, well, what if we do a bit of us, or like a tummy tuck approach and just take out a strip of the membrane, right? And could we reshape the cartilage without mesh? And we did. And this is some entropid picture of that. This is a right thorax, and this is the back wall of the trapezoid. This is the right main stem here. I know. Yeah. Right. It means stem, left main stem. We're sort of looking at the back. And so this, we said that this is the vagus nerve on this vessel loop. The spines over here. Okay. And so if you think about it, it's almost like the patient's facing down and you're looking at the back of it. So then we place these marking sutures to mark the midline or the membrane S. And then we cut first them, the very few people actually know that there is a muscle layer of the posterior membrane. Most people think it's just because of it. There's actually a muscle layer and then mucosa. So we make an island of sort of muscle cutting the muscle both sides, leaving them mucosa intact to begin with. And later we cut out that entire strip and then sew it up. So this is sort of that additional taper on the broncus. And this is how it looks. And you can see how if you go back to how it looked before, it was much wider. And now it looks like it almost has like a corset to it, right? It's just like a tummy tuck. And this is sort of how it looks pre-operately. It has these bow-shaped carolages and post-operately a bunch of paid attention to to the shape of it. It just looks so much more normal. Same patient, C-shaped carolage, you see the taper line right down the middle. And it totally just looks like a normal airway. If you remember that first video, I showed you what an airway looks like. It's almost really hard to believe. First view we did with this, we're like, oh my goodness, it doesn't even need a trick. A good back seat looks so good. And in fact, we have done some, a few without a trick a back seat and just for second in the memory. Now, let me show you that that is still not clear. Sean did a really nice video on sort of this approach that we're going to present at ACS, hopefully. So this is that video to kind of describe that in detail. Tracumalasia is characterized by excessive dynamic collapse of the posterior membrane of the trachea. In severe cases, the posterior membrane of the trachea is very wide. And a posterior trachea pexy is often inadequate given the redundant membrane. In adults, some have used mesh to plicate the wide posterior membrane, but mesh erosion are a concern, particularly due to the life expectancy of the pediatric patient. We highlight the tapering membrane reduction tracheobronco plastic, which is an innovative procedure to resolve this redundant membrane. A five year old boy with history of type C esophageal atregia and tracheosophageal fistula repaired at birth presents with ongoing issues of trachea amalasia with recurrent respiratory infections that have been worsening, necessitating operative correction. Caughting elicit an almost complete posterior intrusion. Preoperative broncoscopy demonstrates a wide, bow shaped cartilage with wide membrane trachea. Through a posterior thoracotomy, the chest is explored, and the esophagus is fully mobilized, exposing the back wall of the trachea while making sure to take care of the vagus nerve. With the esophagus mobilized, the trachea can be properly visualized. Full thickness marking sutures are placed in the midline of the posterior tracheal membrane from the thoracic inlet to the carina. Broncoscopic guidance is utilized to ensure proper suture positioning. Starting cephalad and working codily, the muscle layer of the posterior membrane is incised, exposing the mucusa. Following this, the mucusa is incised, and the full thickness strip of membrane is resected one centimeter at a time, with concurrent longitudinal closure. This is performed with a first layer of running horizontal mattress and a second layer of simple running. This process is repeated over an affected broncus, if applicable, such as in our patient. Post-serior tracheopexy stitches are then applied to further stabilize the membrane onto the spine. Post-operative broncoscopy is performed to view and confirm the tapered posterior membrane. A negative section test is performed at this time to ensure stability of the posterior membrane. A before and after photo demonstrates our results. This is so far. We've done about 15 of these patients between Florida and our team. And we don't have a lot of data yet to show the outcomes. As we started doing this in September this so far, we've been very pleased with it, so we'll be looking at the outcomes, particularly noticing whether any of these kids that we can decrease in the rate of additional interventions, particularly decreased need of anterior tracheopexy, which just shapes the tracheo so much better. I do think that that's the case. Now, so looking forward, I think we can tailor this helps us. It's one more tool in our toolbox, the tailor, the type of tracheopexy that the patient or airway intervention the patient is going to need, particularly if it's a wide membrane. It's really a game changer for these kids. We used to do two rows of tracheopexies, never really would line up perfectly in this really and make it look much, much better. So moving on. And I don't know if we're going to get to all these topics, but I definitely want to talk on this second one, which I think is a huge collaboration we had with our radiology team. I would Dr. Cara Barnowell and with our oral team Dr. Sukit Choi and Sean and Jay before him spearheaded this. And it was a really exciting topic for me because it's rare to get surgeons excited about vocal cord dysfunction. It's kind of like maybe not something that we will have as most surgeons. However, we deal with it so much that it really, it's incumbent upon us to to address it and understand it and measure it. And that's sort of why we're sort of spearheading this this effort. So we previously published, we actually came out recently this paper that described how our screening rates before we started doing this, meaning in terms of how many kids had a vocal vocal vocal movement assessment before or after each operation, less than 20% before we started this. So only pretty much only those who were symptomatic or high risk or had you know, suspicion of some problems. And we started really pushing our oral team to hey, they all need to get an assessment, they all need to get an assessment. We started seeing really that we were only looking at the tip of the iceberg. And so consequently, our rate of injury would be considered higher because now we're truly looking at the entire iceberg and not just the tip. Right. So then roughly between 2019 and 2020, we started to implement the nerve monitoring technology. And I think that's kind of how we are starting to see a decrease and I'm hopeful that soon we can show you 2022 and 2023 even lower. But this sort of shows you how as you increase your screening, you're going to find more vocal fold dysfunction now. Is it relevant? Is it important? Yes, because a lot of these because they're asymptomatic and have symptoms. So I have an aspiration, chronic infections, and there's the ones that say, okay, you just have infectious tissue, you have to take a Malaysia or no. Yeah, you may have to take a Malaysia, but you also have a vocal fold dysfunction and you ask for it and you need to be on a thickener. So it actually changes the way we feed them, right. So this shows how only really half of them are truly symptomatic. There were other ones are asymptomatic or supposedly symptomatic. So that's what it's important to screen everybody at risk before and after. In fact, we found several that work congenital in nature. So that's actually something that can happen. So kids can be born with a congenital vocal fold dysfunction. So it's important to screen that before. Now, this is a state that was recently presented at the Harvard Research Surgical Day and I'm going to try to do a justice. Sean presented it much nicely than I will, but I think it's such an important data to show the team to highlight this collaboration that we have. Now, this is a video that showcases the gold standard, right. This is a vocal fold movement assessment with the flexible nasal iron gauze, which is a camera that's put down to the child. You often need more than one individual holding out the child because it's just sort of what happens. You can see it's torture for the child, right. And you're like, whoa, how can they even tell by looking at that video where there one moves at a half of the time. It's mucus half of the time the view is not perfect and they have to make a judgment call based on that information. So sometimes it's imperfect information, harassing the child, air-isolate, generally some procedure. So all the downsides, right. But it's the gold standard. Keep that in mind. So we talked about why it's poorly tolerated. And really ultrasound becoming so prevalent and so widely available that we said, well, why can't we use ultrasound to look at the vocal cords, particularly the pediatric airway as the advantage of not yet being calcified as much as the adult airway. And so you can have a great window to look at the larynx. They have been done at the fore, but only small studies are not really unoperative patients like our patient population, particularly not in like young babies, like we looked at that. So we really had this was a diagnostic accuracy study to compare the accuracy of one modality over the other to see if how does it compare with the gold standard, looking specifically at vocal fold movement impairment, which is the proper term for this. Right. And so this was a prospective single center study that we ran over the last two years. And we had pretty much any child in the parioperative setting that had both an awake flexible laryngeoscopy by one of our RLT members in a laryngeal ultrasound. Right. So the laryngeal ultrasound were scored as quality of the assessment, be an optimal or suboptimal or non-diagnostic, and whether there was any movement on one side or the other or bilateral abnormality. Right. So fairly similar. I want to make you aware of the vast majority of kids where we're young kids less than a year old, which is consistent with our practice and a little greater proportion in males there. There were very few who didn't complete the study. Mostly it was hard. We tried with a few retreat gastin, we made a difficult to see a larynx, so that probably is an exclusion criteria to look at our vocal fluid and paired prevalence across the board. Now these are all a lot of them who come to us already with an injury. There were definitely some injuries that we made, but you can see it's such a prevalent element that one in three, one in four has this issue, so we really need to have a good screening modality. So when we looked at only the optimal studies, we saw that 58 of them and the accuracy and the agreement was excellent. You know 98.3 percent, the specificity of 100 percent, you know, it just can't get any better than this to be honest. Very excellent studies. There was only one patient that was incorrectly classified by ultrasound where it was felt to be a normal assessment, but the flexible nasal larynxoscopy saw that there was maybe some weakness in the movement. Right. So here you have to sort of wonder was it really how weak was it? So it was one of those soft costs that we still classified it as incorrect assessment. When we gathered all assessments, both the optimal and suboptimal, it just got better because there were no disagreements with the suboptimal assessment. So the overall agreement, the accuracy, everything went up. Right. So the only one, this is almost 100 kids. So I'm not going to bore you to death to this, but essentially we looked at all the subanalysis, whether the age, weight, the diagnosis, the procedure, the time between the studies that any of that matter and it really didn't. Okay. And so this is going to show you why ultrasound is so awesome for this. Now this is an example of how you look at the attention of these white lines. Those are the vocal folds, right. And the space in between them is the glottis. And so in the normal exam, you're going to see how they open and close. And this is a normal exam of a flexible nasal larynxoscopy. First of all, you're also like upside down. So there have been a few times when they say left and it was right and they say right and it was left. So that can also be misleading when you're doing the flexible nasal larynxoscopy. This is an example of an abnormal left exam. Is he how one clearly moves and the other one doesn't? So in conclusion, we saw that larynginal ultrasound is excellent in accurate screening tool for this vocal fold movement impairment. There really no places like clinical decomposition. We really think it can be anticipated. We'll be our routine modality for screening. And we know that vocal fold movement impairment is bad. It contributes to significant rest of the morbidity. It really does change the way we treat kids. It changes the way we feed them particularly. And really having this available will be more accessible for broader screening. Also be more comfortable for the patients. So I still think we're in the phase of needing to confirm an abnormal assessment with flexible nasal larynxoscopy. It's still technically considered a gold standard. So we are trying to minimize that, but I think it's still a roll for both. So that sort of goes off topic now for this next section. But I just want to take a minute to say how practice change in that will become for our practice. And my hope will be for other practices as well. I think it's a very underappreciated, under recognized sort of morbidity for these patients. So switching gears a little bit, because this is also a topic that's fairly popular in our group. We are pretty good at putting these suffocates together. And for the most part, our leak rate is very low. But we still struggling with strictures. And strictures is an issue that we tackle. Both strictures that we created and strictures that get sent to us. And really the definition of strictures on the eye, they beholder would. There's really no established age, luminal diameter. What is the structure? And we know that symptoms can be misleading. Some kids can drink a whole bottle through a one millimeter opening. And you're surprised when you do the endoscopy and there's a structure. So symptoms aren't always the way to diagnose the structure. It's very common. In fact, some recent studies have showcased that up to 50% of EATF children may have a a structure that's symptomatic that requires dilation within the first year or show you some data that we talked about the symptoms of varied. We do endoscopy, esophage grams, etc. Now, why do strictures even happen? Well, let's go back to sort of healing, right? Any healing wound, you first do your inflammation, granulation tissue, and then contraction. So if you have, particularly kids who have a leak, other than estimates, there's a lot of inflammation with a leak. So it could be a lot of scar tissue with a leak and there's going to be a lot of contraction. So leak is a single most predictive risk factor for a for a restriction. And that's why. Now, we really can't predict who will, who won't just basically purely on the appearance of the the anastomosis. When you do the anastomosis, sometimes they all look a little bit ischemic, a little bit of demotus, and you're like, geez, there's no way that's going to heal. But some do and some don't, and you're like, well, why did the one on the top structure and whether top of the one, I'm do not, or very mildly so, right? So really don't understand that. And some would say, well, it's acid. It has to be acid. Well, not really. Now, it is vast majority of kids are on acid suppression medications. This is a recent study from the Midwest pediatric surgery consortium. 90% of the kids were on acid suppression. And look at that, 51% stricture rate, requiring dilation within the first year of life. A quarter had a leak. And I think that's really, really high. Arleicrate is not that high. And this is only 11% long gap kids. So you could see how that is an issue. And although it was not significant here, there are an asthmatic leak rate was almost very strong predictors I talked about. Look at that thoraxcopic approach. Everybody's really excited about thoracoscopy for E.A. repairs. You can see how it's at districts that was one of the criteria for or predictors of stricture rate. I think you just sometimes you can't make a great and estimate the thoraxcopic as you can open sometimes. So this is a study, a data from our study from our center that looked at, well, why does, why does stricture seem matter? Why do we worry about them when they're young, if they can drink a whole bottle, why do you need to treat it, if they're drinking and they're growing and doing everything well? Well, one of the reasons is one of the predictors and modifiable features that we can do to change their dependence on two feeds. Some of these kids have a G2, some of them have an NG2 and they're depending on two because it can't progress to age-appropriate diet. And look at the other feature too. The other, the strongest odds ratio in that table is vocal fold movement impairment, just to kind of piggyback on what we were just talking about. So this is an affair in a 30 children that we cared for in this three-year period. So that's what's important. How do we treat them? Mostly with dilations, all sorts of dilations. There's really no one approach that has been superior to the other. We do think that underfoot life, fluoroscopy with contrast study before and after is the safest, particularly if you can detect microleaks and treat them appropriately. But some of these are done blindly, some are done at home, some are done by the radiology team. So there's all sorts of variation here. And there are studies ongoing to try to compare these head to head a little bit better. So I went to treat them. There's there's different strategies and approaches. And the guidelines, if you look at the NASPigan and NASPigan guidelines, which are sort of like the GI societies and look at this, they really is not a great sort of sense of doing it proactively as we do in terms of symptom-based versus routine schedule. So what we do know is that not all NASPigans have the same risk for restriction. We just talked about leak being one of the biggest issues. So we have a little bit of a different strategy. We're all are high-risk in NASPigans, which are most of our cases. Any long app, EA, any leak or any stricture sentient, all those are considered high-risk for stricture, then get an NASPigan a month. And the reason why we do it at the NASPigan a month isn't necessarily to start treating the stricture that early, but also to get a sense of the prognostic ability of that size. And we'll talk about a little bit about them. But that it does work to a point. This is another great slide from our team that showed that yes, dilations work, but the further out you get, the less chance to success the risk. And it also shows how leak is such a strong feature of refractory of that stricture to respond to dilation. So we have to get the come nose with 100 dilations, 200 dilation, a dilation every other week. So at some point, you need to draw the line in the sand and say enough is enough that needs an operation, right? So I think this shows you how pretty much up to like maybe 10 dilations. You're still pretty good chance of success. But unless you start getting beyond 10, I think you need to start thinking about a stricture section or other alternatives, right? One of the other alternatives that we employ fairly early is steroids. Folks have also used might have nice and see another anti-fibrosis agents. Steroids, at least in this study from our group, showed that although it's not a game changer in terms of oh my goodness, it changes the outcome significant. Some kids for some kids it does, but there is a delta diameter meaning the change from one endoscopy to the other at the beginning of that assessment, at least of a millimeter. Does a millimeter matter? Maybe over the course of several treatments it does because for some of these kids that can be the difference between a stricture section or not. So large data set, fairly strong result. So we use steroids regularly early on. Stents have a bad reputation and rightfully so are really not that great. Sometimes you can salvage a stricture. In the best cans, I would say this is our team and we use them very selectively, about a 40% treatment success. And so there's not a great success from stents. It may still be worthwhile trying it. However, sometimes you end up with more tissue damage from the stent than the help. So that can turn an easy stricture section to a really hard one, or sometimes even into a jaduna interposition. The biggest feature of success that we found was the change in from once you remove the stent, it always looks great. But when you look again in a week or two, if it didn't narrow by more than four millimeters, then that one's going to work. It's going to be good. But if it narrows back down to one, two millimeters, it's just not going to work. Also a very important thing for stents is short duration of stent therapy. Two to three weeks and sometimes even just a week. There are folks out there who put a stent in there, leave it in for a month, two months. That's really dangerous. Those are the ones that we've seen a road into structures and become problematic. So another really cool element of our sticture management strategy is this incisional therapy where we can, essentially it's a coterie, incise the scar band. Because what's happening with the dilation is you're like uncontrolled tearing of a wall. This way you can control which wall you want to tear. And then you can actually sometimes it's a little too deep or part of the healing mechanism when you want. You can put an evax sponge right into that cavity and get it to granulate into heal. So this data from our group as well showed that after incisional therapy, we have significant improvement. So let's get onto some of the surgical outcomes just to wrap up and see how do these kids do when they actually get an operation. Because historically, one of the reasons why some folks get a hundred dilations because they're a little too afraid to do an operation to fix the strictures because they believe the outcomes are terrible or really high chance of leak, high chance of problems. And so we wanted to see what's our outcomes for this. So we looked at refractory structures that failed management within Doscopy. So the four options, stricteroplastia, segmentorasexion, a few of them are really long strictures that need a reception along with the focal process that get them together and some get into software replacement. A few things to think about when you're thinking about this is the length of the structure. Is it in the neck? Is it in the chest? Is it by the G junction? Is there a high or hernia or not? What's the quality of the esophics in both ends and is there existing trichamilasia that we address at the same time? There's often more than one thing, right? Not going to get into the weeds here. We have a very sort of complex way of thinking about it that we're going to get into. So roughly this 10-year period at our center and a few of the patients adjacent treated it before it has since then, we've done roughly about 150 stricture sections. A quarter of those are long-gampy A kids. Most of them happen about a year of age. So we fairly soon after that, South-June Estimosis within the first few months know whether that child's going to be successful or not. So we don't delay it. We have kids coming to us three, four, five years of age with the refractory structure that been dilating all their life. So this is what we're talking about drawing a line in the sand. You have to act early so they can move on with oral independence. 65% of them were segmental stricture sections, a third were stricture plastics, and about 6% were these folk or assisted recessions. Now, what do we mean with non-refractory? We did operate in a few that were non-refractory, particularly in the setting of severe trichamilasia. They had a mild to moderate stricture that otherwise probably would have been okay to manage them this quickly, but since we were already operating on the patient in these illicit fixin, we would often address that at the time of the trichamilasia, either with the stricture plastic more often or a resection. Okay, so we have a fairly reasonable follow up for this cohort and the vast majority were able to preserve the racophagus. I think that's a huge take home point in the 92%. These are all cut numbers were able to preserve the racophagus. Eight out of eight out of 10 were free of a poor outcome. I think that's a really good take home point that eight out of 10 don't have a leak, don't have a refractory structure, and don't need a repeat operation. They may need some dilations, but not that many. The medium number of dilations after that was about three. Some of them needed advance in the scapula maneuvers. Most of these went on to a repeat stricture section. So this is sort of the depiction of what went wrong, and when they're non-refractory, very rarely do they leak or they have a problem, right? Only 2%. So we're fairly encouraged about them, and we fairly liberal about it based on this data. Now 10% leak rate, if you think about the leak rate that they showed for first time EATF of 24%, I still think this is fairly good for a stricture section, but it's still I think too high, we're doing things to kind of get it lower. But the median time of a leak presenting is roughly about two weeks. Now that's a little bit later than the standard EATF repair because most of we sometimes keep them paralyzed for the first few days. So that may be why it's a little later that it presents. 9. Needed a repeat stricture section. Not all leaks ended up in stricture section. So you hear this like the overlapping circles. So not all of them went onto a stricture section. Some did, but usually within a few months we can tell whether it's going to work or not. About 13% underwent a self-replacement. This is not patients just overall procedures. So there are some that undergo a self-replacement. It's just not enough esophagus to salvage. Now this is important. Six patients that required a repeat stricture section were still able to salvage their softening. So sometimes even a repeat stricture section may be worth it. They're difficult and they're morbid, but sometimes that may be important. Now in terms of the risk factor for a poor outcome, this sort of shows that the stricture length, the history of the leak, and history of a focal process are associated with poor outcome. But also very importantly, which we haven't touched about right now, which is sort of like this last take home point, is the G.E. junction location. Meaning do they have a high ador hernia at the time of the stricture section or not? And that's a very important element, we believe. And so this makes sort of the case even harder when you have a coexisting high ador hernia in our refractory stricture. Which do you fix first? Do you fix the high ador hernia first? Do you fix the stricture first? If you do the high ador hernia first, you may facilitate the proper placement of the G.E. junction, but then you may make your stricture section harder, particularly because you divest the lower soft because of the mobilization of the high adors. If you do the stricture section first, you may make the high ador hernia larger and then maybe harder to just do from the abdomen. You may need a chest and belly approach. So we've been back and forth with this. Sometimes we fix it before, sometimes we fix it after for the high ador hernia. Based on our data, it doesn't appear that there's one way that's better than the other. But we do know that if we space them too short to each other, they do worse. So we like to space them out at least two to three months so that there's time for that as often as to heal. Now, so in general, we're preferring to do the high ador hernia pair first, if possible. Sometimes you can't because there's a coexisting trick-to-soft geofacial or a really complete obstruction. And so those are cases where you may do the stricture section first. So this is an area of future research. But to wrap up, to leave some time for questions, I know that this were sort of like three very disparate topics. They have some relationship to one another. I just sort of showcase is the amazing work our team has been doing over the last couple of years. And there's still many more projects that I didn't get a chance to talk about. I've been essentially work-sided and there's plenty to come. Thanks. So they're most important slides, sorry. Then absolutely wonderful talk and congratulations to you and your team. I think the data that you showed with the ultrasound for vocal cord movement is phenomenal. But I like to address one of the more controversial things that you said. And that's that perhaps all kids with the sopidure latrisia should get a tracheoepaxi. And if we take a look at your data, it would indicate that maybe 20% have severe airway issues. Is there any way a priori that you could predict which of these patients will end up with the severe airway issues? Yes and no. I think that first of all, that takes to another controversial topic. Still to this day, not every child is getting an original bronch up front before they repair. So that's, you know, before we get to the other question, whether they need a tracheoepaxi. I mean, I think that from our, based on our sort of assessment, unless the child's like super unstable and there's less than a kilo, I still think most of these kids should get a bronch to understand that question, right? So yes, I think there are some kids that innately have a much broader bow-shaped carlidge from the get go. But even from that very first time you look, you're like, oh my goodness. There have been several kids that we've looked and appear to have a normal shaped carlidge that have Malaysian afterwards. So it's not a guarantee that if you have a good looking area to begin with, they won't sort of manifest as Malaysia. Particularly, I think the type C patients. I think for the type A and the type B, there's their trachea structurally better. There's something about the fissure limb and the location of the fissure limb. Particularly the fissure that goes to like the mid-porsche of the trachea. When it goes to the carina, it's a little less disturbing to the membrane. But if it's in anywhere on the membrane of the body of the trachea, it's just going to make it very wide. And then the biggest issue is then the esophagus sits right behind it. And if you get any little bit of esophageal stricture, you have upstream dilation of the esophagus that just makes it worse. And so I think the trachea pexia addresses sort of three elements. One, that prevents a recurrent trick of the esophageal, which is one of the main things we didn't even talk about. The reason why the trachea pexia began was to treat recurrent trick of the fissure. So this way, you guarantee that you're not going to get a recurrent trick of the fissure, almost guarantee. Which is one of the main reasons why I do it. Because otherwise the sutralisers are just touching each other. Sometimes they're not, but they're very close to each other. And then the other reason is you prevent the esophagus from sneaking behind there and causing one of these issues. And you provide stability to the trick too. So I think for all those reasons, it just makes sense. Yes, it's how do you implement that widespread to the masses, to the pediatric surgeons who are doing one or two of these a year? I think that's going to be unlikely. And that's sort of a shame it's going to be hard for it to be implemented widely. But I think in places who have high volume and do these sort of cases on a routine basis, it's an over-interfer us. Thank you. Other questions? Dr. Schamberg. So I've had the pleasure of reading some of your reports. And I think the approach that you've taken where you've really looked at the results of your efforts and intervention are to be commended. I think there's always some concern that you might find something wrong, but you haven't taken that approach and looked at everything. I will confess I worried when I read the title one of your things about having every child that has a TEF repair has a tracheopathic. But I think in your proposed publication, you took a very conservative approach and stressed that this isn't the best for every center because a lot of places don't have the experience that you've had. Now I will share with Tom that results on the vocal chord function by the ultrasound that was presented at Harvard Research and Posting. My found was really impressive appearing it with the video of what to get to go through if they have it done awake. Really highlights why that assessment needs to be more widely used. Clearly there's going to need to be learning curve, the ultras and auto-fers that are doing this at other centers, but that's not to say that that shouldn't become a widely accepted means. That's already happening. We've had some Texas children, this was doing it. In fact, in our CICU, the cardiologist learned how to do it and they're doing it themselves. So the cardiac ICU attendings are some of them are trained and they're doing it themselves. It's also very prevalent in the cardiac population. A great job of looking at assessing the outcome of all of your efforts. Thanks everyone. Yeah, I'll see you, Chad. Let's see. Oh, never mind. Yeah, any further questions? Okay. Thank you, man. Great job. Thanks, guys.