Good morning, uh, Department of Surgery and Anesthesia. Good morning, everyone, uh, for joining us for a combined grand rounds. Uh, we have the pleasure of having Uh, Doctor Rusty Jennings here to present to us. He needs very little introduction in this, uh, sort of home crowd forum. He's been speaking around the world actually about this topic, which, um, he's, uh, as, as we know, um, helped, uh, develop, um, some of these surgical management strategies for what was previously considered, um, a largely non-surgical disease, which is really exciting for those of us who are training with him. Um, and, uh, as you know, he's He did his sort of training at um UCSF, did his, um, uh, fellowship here, um, returned to California and then came back and, uh, helped develop our fetal program and has since developed, um, our esophageal and airway treatment program. And, um, for the groundbreaking work of that team, um, he's been, uh, recently honored with, um, the Department of Surgery Foundation, uh, chair earlier this year. So, um, please join me in welcoming Doctor Jennings. Thank you so much. I apologize. I have a little bit of a cold, so I might sniffle occasionally. I'm just trying to keep my airways clear. I have no disclosures. Um, what we've learned from the esophageal treatment, esophageal and airway treatment center is really interesting. And that is you can't separate the esophagus from the airway from the vascular system, nor can you separate from the spine, the pleura, or the sternum. So, it's actually a giant envelope and everything is interactive within that bony envelope. And so, although we will talk periodically about one organ or another, You have to sort of realize it's part of a much bigger, more complicated, um, decision tree. Uh, these are the teams involved with the esophageal and airway Treatment Center. It's huge. And the reason it's huge is because of all those systems that are involved. And it takes all of these experts actually to have success since he's really, really complicated patients. And you notice, not only the usual, you know, ICU. And anesthesia and surgery people. But there's also cardiac surgery, orthopedics, plastic surgery, nutrition, intervention radiology is key. So, really, I don't think we missed too many major groups in the hospital in order to care for this complicated cohort of patients. This is um sort of a, a non Updated, uh, list of the cases and the types of procedures that we do. We're just gonna focus on what's in purple today. Mostly just focusing on the airway and cutting out because we simply don't have the time to talk about the esophagus and other stuff. Um, now, I love trachoob bronchomalacia. I didn't know anything about it. Um, I was like every other pediatric surgeon. It's sort of a solved problem. You do an aortex and you go home, um, and it's really not that big a deal because all kids will get it, but they're all gonna grow, outgrow it by age 2 years. It's not an adult problem. I never heard of it having an adult problem. Um, but it turns out none of that was true. As we've looked back at our series, started reviewing the literature and got information, about 80% of kids who have esophageal atresia. And or TEF have tracheal bronchomalacia of some form. Many of them are quite serious and life-threatening, and some of them go on to die or have an early death. And probably, I'm guessing with the count cystic fibrosis and you count vascular disease, and you count all the other spontaneous occurrences of trachealia, we probably have in the United States roughly 10,000 patients per year who are affected. It's not just a benign problem. Um, there's no system to understand it. There's no system to diagnose it, and there's no effective treatments out there. They're just passed off to the pulmonologists or the pediatricians, and they do the best they can. In fact, Cochrane did, Cochrane did a review several years ago, long before our work, that said there's no effective treatment strategies, either surgical or medical, including the ortopexy, interestingly enough. I had a great conversation with my father-in-law over this holiday weekend. He's a He's an ethnobiologist, a sort of a social scientist in his mid-90s writing another book. He's very eager to get it out before he dies. And he, he said there's a couple of types of knowledge. There's 3 types of knowledge. There's faith, which is based on no hard data, which is absolutely unshakeable. We can think of religions, or we can think of steroid use, but from pediatricians. There are things that can't be changed. And there is knowledge which is fact-based. You can hold in your hand or calculate some piece of data, and you know that that's unshakable knowledge. But the vast majority of information is all beliefs. We believe something based on a little bit of knowledge and a little bit of intuition and what we're told. And in surgery, that is particularly true. Because really, we are apprentices. We've learned from the people before us and didn't challenge or afraid to challenge what they told us. So, we have a lot of beliefs. And what I'd like to try to do is push away from the beliefs into the knowledge, uh, arenas. We'll see what we can do. So, what are some of the, the therapies for tracheomalacia? Well, tracheomalacia, I mean, what is it? It's just a collapsing airway of some form, um, and we'll talk a little bit about that. But how can you keep a collapsing airway open? Where you can stent it internally with CPAP or PE, but that's a little bit hard on the alveoli if you have high airway pressures to keep the major airways open. Um, it may require a tracheostomy to stent. That part of the trachea that's underneath or around the tracheostomy, and the airways distal to the tracheostomy have to be opened up by airway pressure, so you need a ventilator if that's the problem. Internal stents. Everybody wants to use an internal stent. We use internal stents. We love internal stents. We hate internal stents. Why? Because they're, they're a devil in the details kind of thing. They work to open up the mechanical part of the lumen, but they have their own complications. And we're not gonna talk a lot about stents today cause I sort of wasn't prepared to talk about that. We didn't realize my audience changed. But, um, the stents. There's really two types of stents. There's the open stents and there's the covered stents. The covered stents are great because they open up the lumen, and you can remove them because they don't get ingrown, but they prevent mucus clearance. There's no silly on the inside of those stents. They just get accumulation of mucus and slowly, slowly, um, narrow themselves. And if they're open stents, they tend to get ingrown. They cause inflammatory tissue, granulation tissue, and that tissue tends to be exuberant. Which can grow into the airways until that stent gets embedded in the wall, and then, once it's embedded in the wall, it gets mucosalized, and it's sort of a permanent structure. Now, you've got a problem with growth and stent size. Um, the external support or extraluminal splints, very different than stents. They have, somebody has to put them on the outside of the airway to support it, and we'll talk a little bit about those. Now, the problem when you have a collapsing airway is that it reduces the expiratory airflow. You, when you exhale in a positive intrathoracic pressure, it will compress parts of the airway that are soft. It actually compresses every part of your airway when you think about it, cause all of our airways get smaller to some degree. It's pretty normal to have 20 or 30% narrowing of your airway. It's sort of God's design to get the airflow, increase the acceleration of the airflow, so you can carry the mucus faster. It's pretty clever design. But if it, in adult studies on CT scans with adults coughing and goes up to 50%, there are even some studies at 70% narrowing from posterior intrusion. That's all sort of within the range of normal for adults. But if it gets coaptation, if it starts touching, all airflow stops, and that's when you get that vibratory sound. You get a barky cough, they sound like a seal, sometimes called a brassy cough. There's no real perfect thing. Um, in England, they call it the tough cough cause esophagus is spelled with an O back there. Um, but really prevents, the biggest problem with tracheomalacia isn't airflow, it's actually reduced mucus clearance. If you can't clear the mucus, you can't get the bacteria out, so you, the infections accelerate and get worse, and, and causes lung damage and prolonged recovery from upper respiratory infections. So it has serious complicate uh consequences, including if you just can't move enough airflow because your airways collapsed, you can't exercise very well. If you want to be a couch potato, that's fine, but if you wanna get and move, and you can't because you can't move enough air, that's a problem. It can be as bad as like people who just can't walk very far. You have blue spells, hypoxic episodes, what they call alties or bruits, and, and because of prolong um. Recurrent infections, you can have damage to the lungs, including bronchiectasis, which is a destruction of the distal airways, so then they become chronically infected. It's very hard to clear, very much like a cystic fibrosis patient, and they can lead to death. So, the tracheomalacia is a word fails us. We've failed just to define the problem because I've designed all of the, I've described all these problems, but we really haven't defined tracheomalacia because there's no standard understanding. It's very frustrating. So, we need some precision to understand it. But the word tracheomalacia encompasses a bunch of different problems. It includes the airway that dynamically collapses. In adults, they call it excessive dynamic airway collapse, which is the posterior membrane. Has posterior intrusion. It also includes tracheal compression. So, if you see an airway that's triangular shaped or compressed from the front, that's called tracheomalacia. Um, if we see an airway that's malformed, is an A-frame or it's got some weird shape that's narrower than around it, the airway above and below it, that's called tracheomalacia. Or if it's got some intrinsic cartilaginous issues, which we'll talk a little bit about that, that's called tracheumulation. So, We don't know what we're talking about. If somebody calls me up and says, I have a kid who has bad trachoacia, I have no idea what they're talking except they have some narrow airway. And so we need to understand this problem much better, and I think we're just starting on that. I'm not gonna say by any means that we understand it. So normal tracheal anatomy is this. This is a kid with a normal trachea. I'm proud to say this is a normal trachea. After repair of an esophageal resia with distal tracheoesophageal fistula, you can see the cartilaginous rings are sort of normal shaped, the membranes in normal position, and the airway doesn't collapse very much during breathing. This is a normal trachea in a 2 year old who's never had any surgery, and it's really important to take a look at these. We don't see a lot of normal tracheas, but here's a normal larynx, what we call T1 and T2 looks completely normal in this child. We can see a little bit of posterior intrusion when he coughs. That's the carina, the right main stem, going to the bronchus intermedius in the right upper lobe, nice and clean, not much in the way of secretions like an airway is supposed to be. We look at the left main stem, we see there's a little bit of posterior intrusion. That's normal because of the descending aorta going behind the middle portion of the left main stem. So this is actually what we want to see. We love that. This is not what we want to see. These cartilages aren't C-shaped, they're more like a bow and arrow. They're bow shaped like a bow and arrow, and even though this kid's barely breathing, you can see he already has significant compression of the airway. We can see that when that same child coughs, it's a different airway even more. All the way, it's pretty narrow now, now it collapsed. The posterior membrane completely closes off the airway. So, once that tissue from the back membrane contacts the front membrane, all airflow stops. Mucous clearance stops, and everything's become static. Here's another kid with tracheomalacia, has no other anomalies. So this is the part, these kids are often missed and diagnosed with asthma or recurrent croup, or chronic cough or something like that. And you can see that at rest, the child has some posterior intrusion in his upper airway, as we work our way down through the bronchoscope, there's secretions, and that's not normal to have secretions in the airway, not that much. And as we work our way down, It's just not gonna play much, but we can see there's a lot of posterior intrusion that's narrowing this airway. So there's no standard definition, nor is there a standard classification system, system for tracheumulation. We're working hard to push that forward. So here we have cartilagian shapes supposed to be C-shaped, supposed to be, they're not supposed to be shaped like Us, which has a longer posterior membrane, they're not supposed to be bow-shaped, which has a very long posterior membrane, and it's more likely to cause posterior uh intrusion with collapse. We also have different types of collapse. We have anterior collapse like in B. We have I can show like this is anterior collapse right here where it's instead of being bowed forward, we have posterior intrusion and very often we'll see a combination of the two. So the posterior intrusion tends to be very dynamic with breathing and with coughing. Anterior collapse tends to be more of a stenosis. It doesn't really change much, although it may be pulsatile, and the combination of the two tends to amplify the effects of each. And then there's the severity of the collapse. We have severity of the anterior collapse, which is a stenosis problem, and the severity of the posterior intrusion changes, but the combination can narrow the area, the area of the airway. So, normal is less than 50% posterior intrusion, and we wanna get that with coughing, not with shallow breathing. And we wanna do that coughing under anesthesia, which is I'm so thrilled you guys are here, and I know we have anesthesia here. We need to change this talk a little bit to include more of what what you do. Um, then we, different parts of the airway collapse differently. And so we've divided the airway into all these different segments. Labia looks so complicated, T1, T2, T3, carina. Left main stem's got 3 segments, the right main stem only 2. Who thought of this? It's not that easy. Well, each lump has different structures around it. That's why we designed it. If you look at it, T1 has a thyroid and then nothing. T2 has the innominate vein and the innominate artery. T3 has the aorta in front and on the side, and left main stem has this aortic crossing behind it in L2, the middle portion, seldom in L1. And the pulmonary arteries in front of the carina L1 and R1. So there's actually a reason for it. It just makes it simpler to talk about. And this is what a chart would look like with a graph what we did. And actually, this article just got published in JAMA, uh, showing exactly this picture. Um, and it just shows sort of the lay of everything and how we'd record the information we get. Here's posterior intrusion. You can see that's pretty significant, closing that area, not quite closing, but almost closing. Let's go. And here's anterior collapse. This is pretty significant anterior collapse. This airway is fairly flat back here, and you can see that airway is pretty narrow, and there's no reason it shows this dynamically because the anterior collapse seldom changes except maybe pulsatile. Here's a child who had both severe anterior collapse, tracheostomy dependent, no exercise tolerance, recurrent infections. She had an esophageal resia that was repaired. That's what she breathed through, and they can't get a tracheostomy through it because if they put the tube through this narrow area, Then it impedes, it gets stuck in this little pouch from where she had her esophageal resia repaired, um, which I'll show you right here as we force our way through, it gets the tracheostomy would sit in here. So she was a complicated airway because then she couldn't have a trachostomy. This is her carina. The entire airway was flattened. Um, she was, uh, totally reconstructed and doing great in, in college now. Um. Uh, so, how do you do an airway exam? as I was traveling around the world telling people how to fix tracheomalacia, I realized nobody knows how to diagnose it. So, now we, we, we teach as I travel around, teach you about the three-phase dynamic bronchoscopy, which we're very familiar with here. We all do it under anesthesia here, but when we, everybody who does it the first time is kinda scared because the first one is the normal one that everybody, every ENT doctor does and is taught by Cincinnati, therefore, it's sort of the de facto around the world exam. And that is No ventilating bronchoscope. You just slip the telescope in while they're shallow breathing under deep anesthesia, and what you see is what you get. Um, you can suck out some of the secretions, um, but it reveals the compression, it really reveals the static component, but it doesn't really reveal anything else, and you may miss a lot of anatomy. Uh, and it's not good for cord motion. What we want to do is put in the ventilating bronchoscope so we can deliver oxygen, have the child actively cough while under anesthesia, which is The part that makes everybody nervous. Um, and it will then reveal the maximum collapse of that airway, and also the regions it collapses, and it reveals all the secretions that come out of the lower airways, because remember, they can't clear their airways. So, this shows us our tracheal bronchoomalacia. And also, if we need to assess the small airways, we get them to cough. Sometimes we need a flexible uh bronchoscope. And the third part, if we put them totally to sleep or paralyzed, it's up to the anesthesiologist, then we distend their airways maximally. The reason we do this is to assess the anatomy. We haven't looked at good anatomy at the first two exams. Now we can find things like diverticula and fistulas and aberrant bronchi and all these other crazy things that we couldn't see when the airway was not fully distended. And this is why we pick up so many fistulas that other people don't, is because we actually look for them. And if we find anything we don't understand, the 4th part of our examination is, is a video bronchography, where we'll fill the airways and inject everything we need to with contrast to see where everything goes. And this is a great examination that very few people do. We've been able to find all kinds of interesting problems, uh, by just injecting all these little diverticula. So, this now allows us to precisely define the anatomy and some of the function of the airway, and really allows us to talk over the phone now. I found in segment T2 that there is a tracheal diverticulum with no TEF. And now everybody on our team and those who understand what we're talking about, understand what we talked about. And we want to be very precise, say there's 40% anterior collapse, and there's 20% posterior intrusion. And it's done. So finally, we can communicate. Then, now we need to know how can we fix it. If it's bad enough to fix, we get a dynamic airway CT scan. We don't need to have the, we really don't care if they're intubated anymore. First half of the, the program, we wanted them with an LMA spontaneously breathing, and we want them 20 centimeters positive and then 0, but it turns out that wasn't that great an examination, um, but it's still sort of shows a lack of information that is now rolling out. People are starting to do that exam as we've moved past it and gone on to the new one. because it really Unfortunately, it was hijacked, and by the radiologists and the pulmonologists and ENT said, we'll rule out tracheomalacia by getting a dynamic airway CT scan, but it doesn't rule out anything. It's, it's such a poor examination for tracheomalacia. All it does is it shows regional areas where things are a little bit compressed. Um, but this is some of the, the examination that we actually published this shows the trachea collapsing with um expiration at zero airway pressure, that was bad tracheomalacia. And this shows what we can show the parents, it's a beautiful little lack of air column when the airway gets compressed at zero airway pressure. So, how would we go back once we've defined these problems, fixing? Well, the standard operation developed by Fuller, uh, uh, in the 70s and before that, by, actually, I just found it by Gross in 1945, um was to move the innominate artery forward, take out the thymus and move the artery forward. And it pulls the uh areolar tissue in the pretracheal fascia forward, which supports the trachea. The posterior fascia will pull the back membrane back, distend the esophagus a little bit, which is tacked to the spine. So, we have this sort of accordion or sandwich of things we're pulling apart to get it to work. It works OK initially and then over time tends to fail because all these areas that are real tissue tend to keep growing under stress and everything sort of relaxes and you go back to the same problem. Um, here we can see this is out of the JAMA article. You can see that the aorta is compressing the trachea right here, the indominal artery is compressing it right here. But we have space. If we take this thymus out, we can move things forward and unload that. And that was the insight from Gross and then later Filler, who said, we have a space here we can use to decompress this trachea, and there's an esophagus behind it. We don't need to worry about the backside of the trachea cause we have space on the front. So the procedure developed was pretty simple. Just pull the innominal artery in the aorta forward. It unloads the front of the trachea, and you can see it helps round out or take the pressure off that anterior collapse of the trachea. We've later discovered there's 2 problems with this theory. One is that everything relaxes and, and fails. The second one, the cartilage is malformed. It doesn't actually pop open. It doesn't actually open up because it was shaped in that collapsed uh position developmentally, and it's not gonna just spontaneously pop open, um, like a car dent. It just stays in the same exact shape. It just moves forward a little bit. But it does have some, some work. You can see here, we can have a totally collapsed airway, and if, after you pull the trachea forward, it often would work. The problem is over time, it tends to relax, if it works at all initially. And this is how I actually saw this during a case. We did an anterior tracheopexy, you know, went in to explore him. Again, why it isn't it working? And it turns out the lung was coming in from the back and compressing the back wall of the trachea and pushing that membrane inside. So, we forgot when we did this anterior work, that we're dealing in a system with lungs and esophagus, and all these structures around it. And when the child causes positive uh intrathoracic pressure, This esophagus and these lungs are gonna push into this hollow structure called the trachea. So we had to come up with a different idea, and this is maybe 10 years ago, 7 years ago, we tried it. And we said this, if this is our problem, and this is occurring, why don't we just support the back wall of the membrane? Well, the problem is everybody had told us beforehand, you can't do that. There's an esophagus back here. Well, when you fix an esophageal resia, very often you end up with the esophagus to the side of the trachea anyway. So we said, well, just move it. We're surgeons, we can move things around. So we just moved the thoracic duct, we move the um. Uh, esophagus off to the side. The orthopedic surgeons taught me how to get comfortable with the anterior spine, and then this is what we decide. If we can support that back membrane by moving the esophagus off to the side, we can then open up that back membrane, which is really the problem in the majority of the kids anyway. And if we need to, we can still do the work on the front as sort of a separate procedure. And that turns out to be true. We need about 10 or 15% of the kids, after posterior work, we'll still need some anterior work, but only 10 or 15%. That's what it looks like when a real artist draws it, you can see the posterior support right here, the esophagus is moved off to the side. And then if we need to, we can do an anterior tracheopexy. Remember we talked about the cartilages are malformed because of that vascular compression. They're not shaped correctly. So now we can pull those dents out with the anterior tracheopexy sutures, and we can pull the vessels off with the aortopexy sutures and have a much better result. And this is sort of, you know, diagrammatically what it looks like. And here it is, you can see preoperatively, we have this terrible, terrible tracheomalacia. Here's the operation. This is actually I had why I hear Tom guiding us. He's the spotter for this case. That looks good. We're putting the stitches in the posterior membrane. This is the anesthesiologist providing us a view during the entire case. They're telling us where to put the stitches, how deep to go, because position medial on the left we're seeing what it's doing over here and we're going back and forth, back and forth, every single stitch. And, um, many of the anesthesiologists are quite good at this and can be our spotters. They'll say, no, no, go left, go to the right, too deep, um, and that, that's good. So that's, it's, it's a, it's a skill not only surgeons can have, but it's, it takes a team to place every one of these stitches. It's very hard to look here while we're operating and look here up at the screen without pulling the needle out while we're trying to see what's going on. Oh. And this is what it looks like from um RA you uh apparently that media got lost. So, when we pull on these sutures, you can see the posterior membrane coming back, and it ends up when exactly like this, this picture looks sort of the same, this posterior membrane coming back. It's working so slowly. And so here it is afterwards. Hm, here it was before. And here it is afterwards. Or playing a little slow. Well, you would see that it certainly stays like that, it doesn't really collapse when the child's under anesthesia. Uh, we looked at our patients and, and Uh, analyze these patients first, we tried to do 100, we had 98 at the point at the time of this publication. Surgically, we had, uh, significant improvement in every one of these airways to pretty significant, um, P value, and, uh, more importantly, clinically, they all did much better by cough, barking cough, noisy breathing, and prolonged infections. They all had decreased infections, and our exercise tolerance wasn't that significant cause a lot of them were babies, it's hard to assess. Um, but most importantly, we had no more respiratory problems, off oxygen, off the ventilator for many of the kids, and we stopped Bluebells and Alttes. So, it's clinically and structurally pretty significant. Um, success. So the system works, with the classification, our scores correlate with the symptoms, and our posterior tracheopexy effectively treated the majority of patients with TBM. So Turns out you can also do it while you fix the esophageal resia. So, now, when we fix an esophageal resia, as a baby, we'll go divide that fistula, take off the diverticulum, and fix the tracheomalacia all at the same time. Um, we were not the first to publish it, we published it as, as part of a, a paper. Uh, Doctor Van der Zee in the Netherlands just published a paper. Uh, doing it thoracoscopically. So when he does a thoracoscopic esophageal res repair, he published a paper of primary repair of eso of tracheomalacia at the same time. It's quite good results, so we're quite excited about that sort of move. It is a little bit of risk to the thoracic duct cause it's on the anterior spine, and we do have to be care careful of recurrent laryngeal nerve injuries, but no more careful than we already are doing the esophageal resia. Um, a couple of other things we see, there's aberrant right upper lobe bronchus with the tracheal diverticulum, aberrant subclavian artery and tracheumation. So, sometimes we get very complicated airways. You can see this chart has a very complicated airway, all of these anomalies. You can see on the CT scan, there's this aberrant bronchus, which is going to this little abscess cavity out here. There's a right upper lobe, there's a, there's a bronchus intermedius, and there's this. Um, bronchus, which had previously been diagnosed as a pig bronchus or broncho suus. They're actually quite different. Here's the esophageal tresia repaired diverticulum, and here you can see the broncho suus. The problem is it's coming off the back of the trachea, not off the side where a bronchosuus would, and that is malasic, and it's chronically collecting mucus. So this child had multiple problems, all of which need to be addressed. If you fix one of them, you haven't really helped the child all that much. So, um, it's very important to understand the difference between a bronchus suus and an aberrant bronchus, um, and we just try to, we try to teach people that as we travel. Uh, one of our other problems, very interesting, this is an unsolved problem, is the left main stem bronchus. It's not the same as the right. The left main stem, it tends to be captured on three sides by vascular structures, posteriorly by the descending aorta, superiorly by the arch of the aorta, anteriorly by the pulmonary artery, and so, it's trapped on three sides and tends to get quite compressed. You can see here it is quite compressed here. Um, in this child and can lead here, you can see it totally compressed. This anteriority, this anterior displaced descending aorta is compressing that trachea completely flat here. The other thing is it's fairly mobile. You can see it goes down here on inspiration, and it goes over here on expiration. So, it, more than any other airway is very mobile, and we have to take that into account as we're trying to repair these airways. This makes Um, fixation of this airway kind of difficult when you realize it's, it's moving so much. And this shows the, the, the effect of an anteriorly displaced descending aorta compressing L2. This is L1, which is open, this is L3, which is open, L2 is compressed, and when you take a deep breath, it tends to open and you exhale and it closes. And this is why, because that descending aorta is compressing against the pulmonary artery, and as everything moves, and as you exhale, everything gets smaller, it just compresses that airway which does not have enough cartilaginous support. What I, what do I mean by an anteriorly displaced descending aorta? These are two completely normal CT scans. This is the spine, this is the spine. This is the descending aorta in a normal position. This is the descending aorta in normal position. The difference is that this one's almost level with the anterior longitudinal ligament of the spine. This one's in front of it, and that is what's causing the problem in that job. So, what do we do about left main stem compression? We're still trying to figure this out. We can move the descending aorta. That's what we can do. We can also help open it up. We can put splints, we can put on stents on the inside. Excuse me. We can move the pulmonary artery with lots of options. We need to know which one is gonna be best. It's, so we've developed a technique to move the descending aorta, which is on the left side of the chest in most kids, from the right chest. Um, which is quite a trick, um, but now it's absolutely routine. Uh, and the, the, the concept is pretty simple. If this is the right main stem bronchus, this is the left main stem bronchus, this is the esophagus, and the aorta, and thoracic duct in front of the spine, this is all we wanna do. So easy conceptually, technically a little bit more challenging. So, we've done about 500 poster tracheopexies. Um, about 1000 of them have undergone descending aortopexies and not including vascular rings and aberrant subclavian arteries and things like that. Um, and so we have some information that it's a fairly successful procedure. We do have to be careful if removing the artery, moving the aorta that we're gonna damage an artery that feeds the spine called the artery of Adamkowitz. So, now we know where it is in every child, and thank you for radiology and Doctor um Sanjay Prabhu who figured out the technique so we can find the art adamkowitz in almost every child, even infants, so we know where it is before we start the operation. And a couple of times, the artery has been right where we wanna operate and we've changed the operation in order to protect the spine. So, we have much work to be done. We've developed lots of anterior work and posterior work, um, but I would like Ali Kamron to talk a little bit about his work. Ali. He's our research fellow who's here from Canada, and he's done an amazing job. He's been working a little Bauska off trying to get us some information. OK, thank you. OK. OK. Uh, this is a slide is a summary of our initial, uh, series of posterior tri uh tarchopexya, uh, trachoscopic approach. Uh, this, uh, study presented and published before, I don't want to, uh, move to the deep of the details, but Uh, this series include, uh, included 10 patients ranging in age from 8 months to 19 years old and in weight from 5.6 to more than 100 kg. All were symptomatic and except one patient with a history of EA repair, none of the other patients had undergone prior uh uh thoracic operation. Uh, one patient had uh trachostomy ventilation dependency from chronic lung disease of, uh, prematurity, and preoperatively, our patients underwent rigid, uh, dynamic bronchoscopy to uh correlate their symptoms with their presence of uh severe tarcho bronchoalacia. And, uh, patients were hospitalized for 3 to 7 days with 1 day for ICU observation. All patients had improved respiratory symptoms following the operation, and except for, for the patients with pre-existing trachostomy and ventilator dependency, and remained hospitalized for over, uh, for over 8 months. This patient was found to have improved. Airway patency for the trachea and proximal bronch but had persistent severe distal bronchoalacia. And 3 patients require subsequent anterior orthopexy and trachopexy to prevent symptomatic dynamic uh anterior, anterior airway collapse, and 4 patients had dysphagia after operation, and 2 of them required dilation. And what we, uh, conclude from this series is each operation needs to, uh, be customized based on the, uh, preoperative uh diagnostic evaluation and, uh, desire external anatomic and relation to the tracho bronchoalacia system, and minor degrees of imprecision can lead to a distorted airway shape that must be prevented. And connect uh corrected during the procedure. The continuous flexible bronchoscopic visualization in, uh, is required to confirm, uh, to confirm the precision of suture placement and avoid full thick, uh, thickness sutures. And, uh, the trachoscopic surgery with robotic uh assistance can eliminate some technical elimination of the, uh, standard, standard video assisted approach by provi providing a more uh accessible platform for uh complicated suturing angles. Um, and, OK, let's go to the next topic is bio-resolvable, uh, external spleen for airway, and some of our patients have a moles, uh, molesic airway segment with a lack of cartilage support or cartilage alteration from experiencing compression. In this patient, a therapeutic option can be the uh placement of a customized external bioabsorbable splint, which can provide adequate rigidity and radial support to maintain airway patency and prevent external compression, but also allows internal expansion to accommodate airway growth. Uh, bio-resolvable external a splint have been described in several animal models and patient casesies. In 2013, Doctor Green's group from the University of Michigan published the index case of a 3D printed bio resolvable Er splint for a 3 month old infant with a severe truch bronchoalacia affecting the left main stem bronchus under emergency use exemption from the FDA. And these patients have been treated with, uh, have been treated with trachostomy and mechanical ventilation, transposition of the right pulmonary artery, and failed orthopexy. After placing the splint, ventilator support was weaned off by 2 21st day, and at one-year follow-up imaging and endoscopy demonstrated a patent left meum bronchus, uh bronchus. After that, this group uh demonstrated a survival benefit in a preclinical pine model of trachomalacia. And then, uh, Doctor Morrison et al. described successful implantation of 3D printed by a resolvable external airway splint in 3 infants with severe tracho bronchoalacia. At our center, the bio-resolvable splints were uh size and shape based on the location and length of the molesci uh segment and then secured externally on the collapsing area with simultaneous bronchoscopic visualization. Our initial studies include 7 patients ranging in age from 2 months to 15 years. All patients had symptomatic tracho bronchoalacia and were found to have a maleic airway segment with scabbard deformality on preoperative bronchoscopic evaluation. After placing the splint, our patient demonstrated significant bronchoscopic improvement in the airway collapse. However, in one patient, the fractured bron bronchial splint require replacing later with a thicker one. Other procedures were also performed in the patients with the congenital tracheal stenosis, severe posterior air, airway intrusion, or major cardiovascular problems to achieve optimal airway patency. One patient remained hospitalized with ongoing respiratory symptoms from severe bilateral segmental bronchalacia along with right lung hypoplasia. OK. The next topic that, that I want to talk about is the circumflex aortic arch. And the circumflex aortic arch is a rare vascular anomaly where the descending aorta crosses the midline behind the tra uh behind the trachea or carina and frequently compresses the uh trachea or bronchi. And the aortic uncrossing procedure is indicated, indicated for patients with a cir circumflex aortic arch causing airway compression. In this approach, the aortic arch is brought lateral to the trachea and anastomos at the same, same side with descending aorta. The this procedure relieves the lateral and posterior tracho compression. However, these patients often have persistent trachomalacia after the uncrossing procedure. Our initial series included 7 patients ranging in age from 4 months to 15 years. They underwent the aortic uncrossing procedure with a concurrent trachopexy and or slight trachoplasty between September 2017 and December 2018, and 12 of these patients had prior operation including double aortic arch and uh ductal ligament diversion without improvement. Uh, the aortic uncrossing procedure was performed through a median sternotomy with a cardiopulmonary bypass, hypothermia, and regional perfusion without circulatory arrest. The mean cardiopulmonary bypass time was 49 minutes. An intra intraoperative bronchoscopy demonstrated no patients had residual trocho bronchoalacia and all arrays were open, greater than 90%. There were no post-operative mortalities, uh, neurological complications, chylothosis, and correctations. 2 patients require trachostomy for bilateral recurrent laryngeal nerve paris. And, uh, all patients, uh, sorry, let's go back, uh, and all patients underwent post-operative computed uh CT scan demonstrating an obstructed arch, and one patient with bronchial stenosis require stenting. At the median follow-up of 15 months range, 1 month to 2 years, all patients were alive without evidence of significant respiratory symptoms. And the aortic uncrossing procedure can be performed safely in uh in patients of all ages without circulatory arrest, and concurrent trachopexy addresses commonly associated traomalacia and significant improve, improved respiratory symptoms. OK. Then back to Doctor Jennings for the rest of the. Thanks, Alex. So we've knocked off a couple of the simple ones, ascending aorta and nominal artery, vascular, um, and this, now we talk, talked a little bit about the circumflex aorta. So, what's this innominate artery, so many of us have heard about the innominate artery compression syndrome. That's when this picture sucks, by the way. But basically, it's a late takeoff of the, of the inominate artery, so it's bowstringed across the front of the trachea, causing anterior compression. You can see this compression here. And those of us who work in our room see this all the time. It can be as little as nothing in, in normal people to 90% or more in severe indominate artery compression. And these cartilages are mouth-shaped. Now they're, they're very happy in that shape. That's where they developed, and they don't fight back against that artery very much. So, there's also a thing called an aberrant subclavian artery. This is very different than an um inominate artery compression. And that's when the, if you have a left arch, the right subclavian artery comes off on the back of the arter instead of on the front where it's supposed to cross in front. In that case, you can see here, um, that it is crossing, this is a back view. It's crossing behind the trachea. It can either behind the trachea or behind the trachea and the esophagus, causing a posterior pulsatile mass. Um, and here you can see its effect on the trachea and on the esophagus. And it's interesting because it causes such, it traps the esophagus often and it's like a little narrow notch, and it causes a thing called dysphagia lossoria, which pediatric gastroenterologists don't think exists. And that's where the Esophagus gets compressed by this and trapped in that little wedge right here, and becomes quite narrow, and so they have solid dysphagia typically can drink liquids just fine, but they don't eat solids. And when you, and you can see why that would be the problem. This is an esophagram. You can see the posterior compression. Here's another esophagram of a child who's having solid dysphagia. You can see the posterior compression from that artery, and what's called a diverticular camara, which is the part where it takes off of the aorta, causing significant esophageal compression. And the, the recognition that this is an etiology of dysphagia lussori, which is really solid dysphagia because of an artery compression in the esophagus, um. is what we really wanna figure out and, and repair. And in addition, because of its location behind the esophagus and the esophagus behind the trachea, you can see this stripe right here. You can see how it's compressing the trachea as well. So not only can it cause dysphagia lossoria, it can also cause tracheal compression from the backside. So, um, we've been pretty aggressive now about identifying the kids who are symptomatic, if they're symptomatic, repairing it. And it's not uncommon to have the Uh, artery either divided or divided and reimplanted, and the symptoms persist, and they persist because of the diverticulo chimera. So that, what we've, our current strategy is to do the right thoracotomy and the left arch, through the third innerspace, place an arterial line in the arm that's gonna have the artery divided and divide it. If there's a low blood pressure in the right arm, we lose all pulsatility, then we'll have to reimplant it, and we use, um, we, we typically call one of the cardiac guys like Uh, Chris Barrett or Dave Hoganson come and help us reimplant it. Why? Because we want the experts to do it. And then we make sure to resect that diverticulum of camora, which is this little thumb, the stump of where the um, Uh, artery inserted into the aorta, and then we'll do a descending posterior aortopexy. Move that aorta out of the way and do a posterior tracheopexy to open that part of the trachea up and rotate the esophagus off to the side. And this is what Uh, and, and that has been almost 100% successful. Uh, uh, I think Ollie's looking up our aberrant subclavian artery literature right now. Uh, the right arch with the circumflex aorta is what Ollie was just talking about. You can see the effects on the airway. The right arch is compressing the trachea, and as the circumflex aorta comes across, it compresses the chine and both main stem bronchi, as seen here from the back. It really causes a big problem. Here it is, the aorta coming across, compressing against the um pulmonary artery. And in that case, it is the aortic uncrossing procedure, which uh Ollie has nicely described. Um, but we not only do the aortic uncrossing, we then move the descending aorta out of the way so it's not compressing the trachea. And because the trachea has been compressed and malformed, we'll then do a posterior trachyopexy and often combine that with an anterior tracheopexy to help keep everything open. Um, double aortic arches turn out to be, although it's a not uncommon, uh, problem, uh, and the, the esophagram is often diagnostic. You'll see this sort of double, double sort of, uh, intrusion into the, uh, lumen of the esophagus. It turns out that they're often associated with circumflex aorta. So, the standard operation, which I was taught as a young pediatric surgeon, I think most people are taught, is to divide this, the non-dominant arch. Divide the ligament, and then just spread and let everything pop open. But as we've discussed, the trachea doesn't pop open. The cartilages have been formed in that position, and when you spread and get the aorta off, that's really nice, but nothing changes. And then the scar happens, and that ligament reforms, and you're back to the same exact spot you started with many double aortic arches we've had to re-operate on. The post-operative vascular ring still has airway compression in many children. Because of this recurrence of scar tissue, the cartilages were never reshaped. They're still maintained their sort of compressed ring shape. Uh, and that fibrosis that occurred between the two ends of the divided ligament then sort of contracts and reforms that. Um, it's even been published a few times that there's still persistent, uh, occurrence after vascular ring division, but I think it's much more than that, because in every child, we've had a vascular ring in and we've repaired. Every single one so far has had distal tracheal bronchoalacia. And in some of the kids, the biggest problem is the tracheomalacia, not the vascular ring compression. So, if you're treating the main problem of vascular ring, and you're treating it ineffectively, and you're not treating at all the tracheomalacia, it's not surprising that you're not having fabulous results. So, the new strategy, which we've sort of been developing, and it's still in evolution, is to divide the arch opposite the descending aorta, even if it's the non-dominant side, and then do an uncrossing to to revascularize or open up the um non-dominant side. Divide the ductal ligament, resect the diverticulum of camoel, um, and then do the descending aortopexy to move the aorta out of the way, posterior tracheal bronchopexy, and if we need to do anterior work, including anterior opexy or uh possibly anterior tracheopexy. And that's have, have much better results. Um, this is a trachea. It's already had the, the vascular ring has been divided, and you can see that there's this thumb sticking out of the way, out of the, off of the descending yard, and that's the diverticular camara causing posterior tracheal compression, and that's the problem. We need to get rid of that. Either that's And that's what has to be divided, flush with the aorta and then rotated out of the way. And this is what it looks like preoperatively. You can see this divertic diverticular camarel compressing the trachea posteriorly, and here's this aorta, which has been circumflexed right in front of the spine causing posterior tracheal compression. And these concepts are really easy to understand. If postoperatively, we resected this diverticulocael, you can see now we've opened up that airway, we've done a posterior tracheopexy, opened that airway from that size to that size. And we moved the aorta from being behind the trachea, we moved it off to the side. So now this airway is wide open. And by pxing it or supporting it anterior posterior, we have a much better airway. Here's a child who was even more interesting, repaired esophageal resia, had a right art circumflex aorta, and the child was vent dependent, terribly compressed airway. Here you can see T1 has got significant compression, but not life threatening. T2 is completely compressed, T3 is completely compressed, 50% anterior, the rest is posterior, has this large tracheal diverticulum just in front of the carina. So that is a child who's complicated. And takes really multidisciplinary care, and that child took an aortic gun crossing, as well as a tracheal uh resection with a slide uh tracheoplasty to get rid of that diverticulum cause everything was so malasic. You can see here this procedure. During this whole thing, we've come up with some innovations in tra slide tracheoplasties. It's very interesting, we've talked about this in the past. The standard way of doing a slide tracheoplasty is just, you know, cut out the bad segment and slide them together, which is not that hard, and he ends up with this sort of figure of eight result, which often is not much bigger aluminum, and it's still fairly collapsible because you're losing some structural integrity. Um, we've, um, oh, that changed. What we, um, came up with is what we call the straightening stitch, and that is a stitch which placed in these figure of eights, will then bring it out. So they're now half moon shaped. So when we sew it together, it's just like one continuous circular lumen, and that's been quite successfully doing that many times. It's just sort of part of the case, we're actually now using in the bronchus. I just wanted to very quickly give you some sort of a flavor for the complexity of tracheobronchoomalacia as I see it. Um, these are the types of tracheobronchoalacias that we've encountered and that I can, that I can recall. Um, this is sort of flattened. This is a scabbard shape. This is the compressed ring, often seen around double aortic arches. This is often seen around double aortic arches. We have this sort of, uh, A-frame shape or this is an A-frame with a foot. This is, we, we see this not uncommonly with, with uh tetragia flow with absent pulmonary valve. Um, and you have this gigantic compression of the airway. What if you have no rings at all? We have this sort of square tracheas, we have these lean-tos, and this is a complete rings. All these different, these are bow-shaped. This is a normal one, by the way. And here's a, um, a bow-shaped trachea with an aberrant bronchus. Here's a persistent diverticulum, and here's a big abscess or cyst in the back of the trachea after somebody doubly ligated the um uh uh TEF. And so, how do we approach these? And these are some of the thoughts. If we have the staple deformity, we've tried the anterior posterior, it opens up a little bit. Posterior didn't work that well. Sometimes they need a splint to open them all the way open. Scabbard deformities, doesn't we make a little bit of headway by doing posterior. Anterior doesn't help that much. We've had much better success by opening up the leg with a straightening stitch. This is the compressed ring that uh common in, in, um, Um, vascular and double aortic arches. And we found we can really, by opening up that posterior membrane in a, in a manner that brings that ring open, have a pretty good airway at this level. These rings are strong, they're just malformed, and then treat the tracheomalacia that's distal to that. The A-frame, we've had to figure out how to open that A frame up, and, and, um, in the A frame which has a little leg on here, we've, or the foot, we've figured out how to open that up with a straightening stitch. So, we're making some progress in this pup tent deformity. We've had to do a, um, a splint, and it's been successful. With no cartilage deformity, we don't have this one solved. I think that's gonna end up being either internal stents or resections because placing a splint around it has not actually been successful. The box deformity, similarly, you place a splint around it, you'd think you would open it up, but somehow that airway still collapses. And this child required a tracheostomy. Um, when we have this hinge deformity where we see this cartilaginous failure on one side, if we can bring that hinge open, it seems to be effective to open up that trachea. And the ring deformities we've talked about, the straightening stitch, really helps when we do the slide tracheoplasty. Um, for a U-shaped cartilages, we've gone away from doing a single posterior tracheopexy like we used to do, and we now do double rows, which tend to help open it up more and keep the posterior intrusion laterally. Same thing when we have the bow-shaped cartilages. Do two rows to keep everything opened up more, and if we need to, we can always do anterior work. And with a lean-to with a hinge or without a hinge, we found that we often will do the, get pretty good work with two rows posteriorly, we can always do anterior work if we need it later. If we have an aberrant bronchus with all this other stuff we've talked about it, we have to get that aberrant bronchus out, and then because of that localized malaysia, then we do the two rows of posterior membrane support. We've had very good success with that, with no recurrences of fistulas, with uh uh diverticulum. We just resect the diverticulum. There's an adage amongst pulmonologists and pediatric surgeons, ENT, the diverticulum is not a problem, but it acts as a, um, sort of retainer of mucus in a constant source of infection. So, we just resect it, do a nice flush suture line, which our anesthesiologists help us with all the time. Takes a special anesthesiologist to deal with the wide-open airway. And then, um, you do a posteriorexy and the same thing when you have these cysts and abscesses. So, we're making progress. Many, many innovations in the EEA program. Um, over the years, and we just keep coming up with new ideas as we run into new problems. We'll be talking about these problems in May 1718, 2019 in this very hall. We hope we can see some of you there. Um, and I was like Mark Twain, who said, it's not what you know that gets you into trouble, it's what you know for sure that just ain't so. And so, when you think about our beliefs, we have to be able to challenge our beliefs. In this case, challenge our beliefs of tracheal bronchoalalacia and vascular compression, so that we can move from beliefs into knowledge, which is often a very different picture. So, thank you very much for attending. Any questions? Rutty, uh, thank you for a remarkably, uh, uh, interesting and, uh, uh, creative, uh, talk. Uh, it's always amazing to hear you, uh, speak and, uh, uh, uh, also to garner insight in how you think, which is, uh, uh, fantastic. Um, one thing that you mentioned, uh, was the use of, uh, tracheopexy at the time of esophageal atresia repair. And uh that brings up the question as to who should get this repair. And I think we're hampered in several respects there. First, the first repair of an esophageal atresia was in 1948, so the oldest person possible is 71, and none of us as pediatric surgeons have been particularly good about following the natural history of the disease, so we really don't know uh uh what uh uh these people are like. And secondly, I'm not sure that the uh uh uh anatomy is that predictable. So, the short question is who should get a, if you will, a prophylactic uh um uh tracheopexy. That's um, that's a great question. So, I think you can do a lot with a live baby, and I think not having a, uh, not contributing a child's illness by avoiding the by avoiding addressing the problem of tracheomalacia. We leave a lot of sick kids out there. So, if you look at a baby and you look at an adult, our cine is essentially the same place in our mediastinum. It doesn't move a lot relative, so I'm, I don't feel this is gonna cause a great deal of growth discordance by fixing the potion membrane to the anterior spine, but I don't know that. So, all our patients have to be very aware, this is innovative care, and we don't know the long term outcomes. However, that being said, If you can breathe, you can get to a long-term outcome, we'll deal with the problems if there are any in the future. I don't think our sutures hold for very much, very long. The truth is, you go back in 6 weeks later, most sutures aren't doing much. We're just allowing spot welds, and we're allowing scar tissue to sort of form to hold things open. And so that has a certain laxity to it, and we've had a couple of failures, maybe 3 or 4 kids with the posterexy just didn't work, we had to go redo them. So, um, so to circle back to the question, who should get it? I think every kid. With primary oesophageal resia repair, who has a collapsing ear which should be repaired, and that's 80% of them. Um, Doctor Vanderzee just published a paper doing it thoracoscopically. He only had a follow up on a couple of the kids, but there's such a dramatic improvement in their breathing compared to the kids who didn't have it, that he sold, and he was my greatest European critic for 5 years. He couldn't say enough bad things about me, um, and now he's a fan. So, I think it's, it's gonna happen. If people are gonna start fixing this. If there's a better way, stents, splints. Tell the membrane to get shorter. Maybe there's a better way to fix it so we can make it more firm. I'm 100% for it. I'm totally technique agnostic, but I do want to recognize the problem that we have collapsing airway causes serious consequences to these kids. Rusty, I just wanna echo, uh, uh, Tom's comment. Uh, you really, you're an explorer and you're really exploring these new, and sometimes when the, when it, it, that, uh, channel doesn't pan out, then you move to another direction. So, uh, we're, we're all, uh, excited about your work. Uh, uh, to circle back to that same question, you, you know, where sutures are fixing things and on the one hand, you, you commented that aortopexy. That was one of the problems with your anteriorortopexy that that loosens up and so I wonder, I'm intrigued, is it different with posterior aortopexy cause you don't have that pulsatile against it or, or do you see some of the same, uh same concerns there? No, it's a great question. So, Many of our patients have had prior anterior aortopexsies, and it failed. And when you get a CT scan, you'll see the aorta back in its normal position. So, the sutures pulled through, just like a cheese wire, they just pull through. So, we try to fight against that by putting pledgets on our sutures, so they can't pull through. Uh, and we'll find out how well that works over time, but so far, it seems to be working by having pledgeted, um, horizontal mattress sutures, particularly if we can use the, um, Uh, polyester or Teflon pledgets that really grow in, and we know from cardiac surgeons that those pledgets last forever, um, because they really do get grown in like the cuffs on roviacs. We're, we've gone away from using as an innovation, gone away from using any foreign material in these cases because If we use an aortopexy stitch, which is polyester, we've seen them inside the trachea and the esophagus later, which is a little scary when you think about the esophagus and forming little abscesses around the aorta. So, we've gone away. Now, we only use autologous tissue, pericardium or pleura or scar tissue. But the same technique is using a big pledget that I don't think is gonna pull through. Um, but we're still analyzing our data and we're still following these kids up, and the only time will tell. Thank you very much A trustee, fantastic.
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