2024-03-20 04 Panel 2 Management of Tracheomalacia from an Aerodigestive Perspective

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Panel 2: Management of Tracheomalacia from an Aerodigestive Perspective. Moderator: Jeremy Prager (ENT), Paul Boesch (Pulm), Jason Smithers (Surgery), Melissa Brooks (Anesthesia), Kimberly Morris (SLP), David Lehenbauer (CTS)

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>> Louder? Louder, okay. We’re going to get started because we’ve got a little bit of time to make up. My name is Jeremy Prager. I’m one of the PZNTs at the University of Colorado and Children’s Hospital Colorado. And I am joined by my colleagues up here to discuss a multidisciplinary approach to the management of tracheomalacia from the aerodigestive perspective, meaning that all of us are in separate programs that are doing this, that are managing this disease. That’s a very much younger version of me. Got to get that updated. I’m also joined here by Paul Besch, who’s the chair of the Division of Pediatric Pulmonology at Mayo Clinic. Kimberly Morris, who’s the lead speech language pathologist at Rady Children’s in the aerodigestive and cardiac feeding programs. Melissa Brooks-Peterson, who is a pediatric anesthesiologist in our own program. Jason Smithers, who’s a pediatric thoracic surgeon and critical care doc at Hopkins. And David Landbauer, who is a congenital heart surgeon at the University of Cincinnati, the Cincinnati Children’s and Kentucky combined programs. So to keep it moving rapidly, I do recall the first patient I ever saw with tracheomalacia, meaning like softness, floppiness, some sort of intrinsic weakness, structural, as well as something built in. But it was a post-op trachea resection of mine, and I am a simple, straightforward surgeon. I put something in to hold it open. I thought I’d done a great job and walked away thinking that was awesome. But it’s way more complicated than that. And I also recall the first patient I saw with a truly like congenital issue who had come to me with a recurrent croup and exercise intolerance, a school-age boy. And through our program, we started to acquire more and more of these patients who had had their initial issues fixed, we thought, but were having significant now Malaysia exercise limitations. And I wanted to get next Dr. Besh’s perspective on how these patients present to the pediatric pulmonologist. All right. Yeah, I’ll try to be too. I mean, everybody sees tracheomalacia patients and they’re noisy. They cough, they cough a lot. Everything that makes them cough makes them cough a lot. It can be barky and brassy. They can have fits of coughing. Anything that makes them breathe harder makes more collapse and provokes coughing. And also noisy breathing. It gets mistaken for asthma quite a bit. The quality is very different because it tends to be loud and raspy and not musical, like monophonic, like one single harsh tone, like a large airway obstruction, but usually more expiratory than expiratory. It does predispose to recurrent respiratory infections, unless you have that reason. In the severest cases, when they will have so much airway compromise that if anything that causes them to strain, like soiling a diaper, will cause them to turn cyanotic or they will, or getting upset or having agitation episodes. How do we in the pulmonary area evaluate these patients? Well, a lot of them are babies, and so we have history, exam, and scoping. Occasionally older kids, because they’re coming for wheezing or exercise limitation, will get spirometry, and we may see this kind of characteristic kind of knee pattern sometimes on the expiratory limb of spirometry. And sometimes we do a methacholian challenge, we actually see improvement of flows because the increased airway tone will lessen that collapse or delay it. CT scan is increasingly being used to evaluate trachomyelasia, I think in part because of the desire to minimize anesthetic exposures, and there is like some usefulness there. There are certainly differences, however. With bronchoscopy, you can, you get other things besides the ability to evaluate the malaysia itself. You see mucosal changes and how much inflammation is there. You can of course identify other lesions that may be mimicking symptoms, and you get an airway sample. So you can evaluate for airway inflammation and/or infection. What you see under bronchoscopy is, has same anesthetic issues as CT scan does, in that the depth of anesthetic and what the child is doing at the time you’re looking or scanning is highly relevant. CT scan offers other advantages in that you can see outside the airway, lumen, what is related to the airway on the outside, such as vascular mediocidal structures, and you also get a lung perin, pulmonary perincomal assessment as well. Some of these scans, however, especially in younger kids, may be done with my passive exhalation, which may be different than doing a true dynamic CT scan where they’re asked to do a more forced expiratory maneuver. So there’s a lot of overlap. There is some good correlation for moderate and severe trachomyelasia. Milder is a little less tightly correlated, and they’re at least complementary and one can inform the other. It’s hard for me to say one is superior to the other. From the standpoint of a bronchoscopist, there are certain things that I think are important to keep in mind. Because parents ask all the time, does the malaysia look better, say, on a re-scope? And it doesn’t really matter if it looks much better. It has to behave better because the behavior of it is what is relevant, and the visible appearance is only loosely correlated to that, frankly. So sometimes you see trachomyelasia doesn’t look that bad, but the symptoms are very characteristic, and it probably is the malaysia, even though it doesn’t, quote, “look that bad.” And the interventions have been driven by the symptoms. It is important, especially because some of these children increasingly may go on to having surgical interventions for the malaysia. So the bronchoscopist needs to be very careful about how well– they need to describe the trachomyelasia like a surgeon would describe it, or there needs to be a surgeon in the room at the time to see the shape, the severity, the location. And differentiating compression and collapse alone, which often co-exist, is important. I do mine with an open airway. I don’t like to use a mask or insulation or LMA because you don’t always know how much additional insulation you’re getting, and it’s masking what you can see. I also am going to plug in for PEEP trials, which people really seem to love, that you put a scope in a endotracheal tube, and you figure out how much is the right amount of PEEP for this child. It’s generally not accurate because you don’t know what the PEEP is. You have a scope and a small tube. The ventilator or whatever is measuring some PEEP behind the obstruction. What you’re seeing is much higher PEEP that is being applied by the vent, and it’s rising with every 10, 15 seconds you’re in the airway. So you don’t even really know what you’re looking at. So unless you have a very small scope and a large tube, that’s not really a thing that we pretend that it is. From a medical management, I try to be fairly– have a framework for it. So kids with trachomyelasia cough a lot, so identify their causes for coughing and manage that first. That’s the best thing. You can teach kids to cough against a resistance, like have them hold in the air with their hand to keep an extra pressure open. Some kids actually talk themselves out of this because it hurts less. Same thing with pursed lips breathing with exercise. We try Atrovent, maybe it helps a third. We try systemic steroids, it helps maybe a half, but not all kids respond to that. But these are exacerbation-related interventions. They are at risk for protracted bacterial bronchitis and will treat that with antibiotics. And some of those kids that exacerbate frequently will put them on more chronic azithromycin. Bethenicol can be helpful, but only in kids who have their Malaysia due to a trachostophageal fistula. Airway clearance that focuses on positive extertory pressure, like an acapella aerobica, something like this. And then, of course, surgical interventions for severe cases, which we will go into next. Oh, no, sorry. After speech. Let’s see. Okay. That’s her. So, as he’s pulling these up, we’re going to go into the next slide. We’re going to go into the speech pathologist perspective on or feeding therapists, whether you’re occupational or speech therapy. Obviously, these patients present in so many different ways, and we’re seeing them sometimes from the very beginning, whether it’s the NICU or cardiac unit. And we’re going to just talk a little bit about the different profiles that can help facilitate their feeding and help even determine when surgery is needed and move on to what how we might see those patients present in the outpatient setting in our aero digestive clinics. So, we have our undiagnosed patients that might have trichomellasia or trichobronchomalasia, and then we have our diagnosed patients that we might be finding that out from the very beginning when they’re born, and sometimes we’re part of that differential. Just trying to advance. There we go. These patients present in a lot of different ways, and from the very beginning, it’s important that we become involved. Even with our intubated patients, we’re working on secretion management of just, obviously, we’re suctioning, but you can increase the frequency of them swallowing and their overall experience. We’re facilitating from the very beginning all of the different ways we can establish multi-system alignment so that when we get to sex, swallow, breathe coordination, we have a good understanding of where that patient lies. So what do these patients look like? There’s a lot of variability. We know that, especially with our patients with trichomellasia and trichobronchomalasia, they have increased work of breathing. Their job is to be living and breathing efficiently. So whether we’re just working on secretion management or we’re trying to understand why is this baby not feeding very well, their job is to breathe. And we know that feeding is the hardest thing that they’re doing in a day, even for the patients with more mild trichomellasia. And so if we think about that and we think about their job being needing to maintain minute ventilation, if we start feeding them, we then are taking consideration right from the beginning, how slow are we giving them their bottle, are we positioning them appropriately, how are they feeding at the breast, and what are the, most importantly, what are the things that we’re doing that change what we see? It’s easy to say, oh, this patient doesn’t want to eat or this patient doesn’t coordinate very well. But what do we do that actually helps facilitate that? So that might be their work of breathing, they might not be managing their secretions well, they might be refusing a pacifier, but why are they refusing the pacifier? Is it just because they can’t swallow and they can’t pass something through? Is we’re in that differential? Are they able to swallow but they’re so used to just spending time in breathing so that they are holding their secretions and then they don’t want the pacifier? What can we do to help them move along? The entire time from the very beginning when we’re with these patients in the inpatient setting, we are helping them be able to coordinate multi-system alignment. And that’s from the very, we’re not going through all these, but thinking in our mind, what is that patient’s sense of stability? And then how do we facilitate that through very early experiences at the breast or just more comfort measures that they’re going to trust what their body is able to do and challenge them in the right ways? One of the biggest things that I see in the inpatient setting, especially with our patients who were trying to figure out whether surgery is needed or not, and they’re beginning to feed or just at that very beginning stage when they’re just engaging in non-nutritive sec, we’ll hear they can’t handle their secretions or I try to suction them but there’s nothing in, there’s nothing in their mouth. One of the strategies that I’ll do, and we’ll talk a little about this a little bit later, is getting them to suck on my, on a finger and putting a suction right at the, at next to my finger parallel. And instead of just going in and suctioning what’s in the oral cavity, that then pulls from the pharynx. And so a lot of kids that are pooling in their molecular space, they will then pull there, it will pull from that area and then they will engage in non-nutritive suck and we can get patients to even engage in feeding right away. So when we get into determining whether if a patient did need, did need surgery, where if we worked with them preoperatively, then we, and helping facilitate that differential, we’re then able to understand what was the change postoperatively. We know that these patients are not straightforward and so even if there’s a TF repair and we know that that was one of the issues, they have a different profile as well and is that, was that really the only issue? So what do we see clinically afterwards and then what can we do? So a lot of times we’re doing it instrumental if needed, especially when surgery is needed to understand how the entire air digestive system is working. So that could be a thes, a lot of times we’ll do a combined upper GI and esophagram with the swallow study and then at times manometry when needed. So clinically what do they look like? If a patient is holding, if you look at this video and the patient is pulling off the nipple and swallowing multiple times, is that just because they’re having difficulty breathing and maintaining that ventilation or is it they’re having trouble moving that through their esophagus or are they aspirating and they’re pulling off because it doesn’t feel good? And so all the things that we’re doing clinically, that’s why it’s important to say, okay, this was what I see, let me try these things, how do I improve their stability to guide them? And we know that it’s not just about, if you have a, if you have a kid with tracheomal alysia, yes we know there’s a high incidence where you could have esophageal atresia and fistulas but they also have other things going on. We have, we can have vascular rings. We could have some stenosis with just tracheomal alysia. So it’s just important to be understand what you might be seeing clinically and what’s underneath the surface and how we’re shaping from preoperatively to postoperatively or even if they don’t need surgery as they’re getting older, how do we facilitate their success with their swallowing and trusting their system? This is an example of how we’ll do a combined swallow study and an esophagram and if you look at the one on the right, as they’re swallowing, that was, there was no issue immediately but that was immediate reflex. If you watch it again, I’ll play it one more time. That baby had been eating and even though if they had been repaired, that’s, they’re having reflex. So we all blame things on reflex but sometimes it is and so it’s just understanding how that plays a role. We know that there’s a high incidence of laryngeal clefts and so in the, after you’re repaired or even if it’s just tracheomal alysia and you didn’t, it was more benign, you may have a laryngeal cleft that we need to pick up later. So why is that patient not feeding well? Do they have a delayed initiation of their swallow that hits the piriform sinuses and that’s the reason if they have the laryngeal cleft, you’re going to pick up and have more symptoms of that or do they have a more timely swallow with a laryngeal cleft and then they might not be as symptomatic. So we just have to piece it apart. This is what I was talking about before. What was their feeding experience from the very beginning to when we’re seeing them in the outpatient aerodigestive clinic? Was that patient successfully suctioned when they were a baby where it helped them facilitate their secretion management and they trust that or was someone aggressively suctioning them and not able to help them get to the point where they trust their system more? How were they being fed? We know how, whether the patient was needing to be on continuous feeds, whether they were able to be fed at all, tolerating bolus feeds, it all matters, it all integrates with how they’re coordinating their systems and what they trust. And what do we do after we actually are helping these babies have more stable systems, whether it’s an EA kid or a kid that had a trichostophageal fistula, we fix them, what if they’re refusing? What do we do? Is there really a problem still? How do we push through that? One of the things that we talk a lot about on our team is trusting the baby. So we can trust the baby the best that we can, but what if they continue to refuse? What’s the balance of being able to push through that and help them understand that they can trust their system? And sometimes that can be done even just in a session. You might have a patient who pulls away when you go to bring their hand to their mouth, like here, and they grimace, but then you show them as they swallow those secretions that they’re pulling in their pharynx, or even in their esophagus where you help them move that through, they start to trust things a lot more, and then they begin to feed even within a session. And so the idea is don’t blame it on one specific impairment and be looking at the whole patient, but from the beginning, really be trying to facilitate what you see to talk as a team and understand where do we move that patient along and what really is making a difference. And hopefully getting to the point where they can all be eating cake on their birthdays. And now they’re going to go into a little bit more — is it anesthesia next? Okay, there you go. >> Thank you. >> More? Of course. [ Applause ] >> Thank you, sir. >> Yeah. >> Thank you. >> You should be good. >> Thank you so much. >> Almost. >> I can do it off the computer. >> Almost. >> I can do it off the computer. >> It’s not showing. >> He has to come to magic stuff. >> Hi, everyone. I have a secret hope that I’m going to make public now. Is there any other anesthesiologist in this room? Please somebody raise your hand. Okay. Well, I’m the lone ranger. This is a very comfortable place for me in the Aero Society. And I’m happy to be the only anesthesiologist. Please bring me your questions and let’s recruit some more to be here next year. I’m going to share with you guys just a brief snapshot of the way I look at Aero and hopefully the way your friends and the champions that our prior panel spoke about also look at Aero Digestive. So we’re lucky in Colorado that we have time for us on Wednesdays to attend in clinic to be the fourth physician. And when we see kids in the clinic, we get really into the nitty gritty about what to expect when they fall asleep and how to best optimize their trip to the operating room or trips to the operating room. We always are interested in prior airway instrumentation and what our colleagues have documented really to figure out what we predict to happen under general anesthesia physiology and then how we can adjust that general anesthetic to tinker with the child’s physiology. I always ask also about respiratory symptoms when they’re well and when they’re ill. I think that the Aero Digestive triple scope and Aero Digestive care under anesthesia actually mimics quite well what happens when a child gets sick. We stuff stuff into their airway similar to viruses stuffing stuff into their airway. We give them a lot of atelectasis and a lot more secretions than they had when they were awake before they fall asleep, just like when they get sick and they start to produce a lot of secretions. I also always ask about OSA and severity, and I always consider transitioning them to whatever support they sometimes or always need at home under conditions of general anesthesia. So if I think that they’re not going to make it to the PACU to wake up crisply and cleanly, I will transition them to whatever they use for sleep. And I always also assess their oxygen requirements similar to their need for any BiPAP or CPAP so that we can plan for that in their transition from awake to asleep to somewhat awake in the recovery room to all the way awake to go home. I try to figure out, and this will be a panel later this afternoon about real reflux, meaning regurgitation, and actual secretion or food coming up from the esophagus versus like the fake baby GERD. I think from my very limited perspective, we kind of have a pendulum happening of reflux or GERD in everyone, reflux or GERD in nobody, and we’re kind of coming back like to the middle from the nobody has real reflux or reflux diagnoses. One important thing for anesthesiologists, we’ll get to this a little bit in the panel this afternoon, is kind of a rudimentary gastric emptying assessment, and then a Nissen or Retsch history. I put Nissen and Retsch in the same column because I think they go hand in hand, I suspect, but I don’t know that that’s a neurologic phenomenon from tying up the bottom of the esophagus. I also think that’s a pendulum thing over the last decade or so of what we’re up to. From my perspective, I’m kind of like a pilot. I’m always worried about the really, really, really dangerous stuff like crashing the plane on landing of the plane or crashing the plane or not getting off the ground during taking off of the plane. So an aspiration event is really, really, really rare, but it is very dangerous. I’m talking about aspiration under anesthesia, not the aspiration all of our aerodigestive patients do during the daytime and sleep time. There are a lot of things we can do to mitigate the risk of aspiration, either before induction of anesthesia during the case or after the case and in the recovery room, and that falls on the shoulders of your anesthesiologist. So I always try to talk with patients about what I think their gastric emptying assessment is and how full I think their oropharynx, hypopharynx, esophagus, and stomach might be at any given time, even when they’re NPO. I think a really critical part of my involvement with patients with severe tracheal bronchomalacia, like these are frequent flyer families. These parents know the system. They’re very well versed in what’s going to happen to them when they come to the hospital, when they’re well, when they’re ill. I think for anesthesia, I talk to them very directly about managing their expectations, and usually parents appreciate that. I describe to them the risks of a full stomach every time. Frequently that garners me different information than my surgical colleagues in clinic, and when we meet then as a multidisciplinary team, I sometimes get more detail out of the family about what’s really going on with their full or not full stomachs, because when I explain to them the risks, it scares them, and then they’re truthful. We have an interesting relationship with our PACU in Colorado, and I think expectation management for our PACU team and for parents and for the PICU, if kids go to the ICU, is that I always tell families they’re going to sound like one of those old school washing machines on the low agitate cycle. The top loaders, you put everything in, and it goes err, err, err, err to get all the stains out. I tell patients and parents and the PICU and the PACU, this is normal for aero. This is what we expect, because invariably if it’s a kid who aspirates and they work really hard to thicken to avoid aspiration, keep their lungs healthy, they get to the PACU and they look awful for about an hour or two, until the atelectasis goes away, until the lavage fluid clears, and I tell parents directly, you will wonder if we broke your baby, but after about an hour, I promise they will look better, and they do look better. I always explain to parents that our anesthetic planning is like a light switch, like on/off, a very clean, short-acting anesthetic. I always describe that we want your child awake, awake, awake in the recovery room, meaning they might be cranky and they might be a little pissed that we just drove a bunch of cameras around their body, but that’s by design. We want them to wake all the way up awake. The medications I usually rely on, and this is like a whole talk in and of itself, Civo Fluorine, because it’s the shortest acting and most comfortable to breathe while awake, fluorinated and inhaled anesthetic fentanyl, because its onset is about 30 seconds to 45 seconds, and you see peak effect at about two and a half to three minutes, which means all the bad stuff happens on my watch. Propofol, because it’s also short acting, although its tail is quite profound in getting kids to wake up after an IV anesthetic, so I tend to like to lean on Civo Fluorine more, because we can get rid of it faster. Always dexamethasone for airway swelling, although that’s really the only medication left in the patient when they leave our care. And then IV lidocaine is one of our favorite, or my favorite tricks for reducing the small airway cough reflex. I always ask parents to bring their BiPAP and CPAP machine and whatever oxygen they use for travel or when the kid is sick, so that we don’t have to deal with DME stuff on the back end in the PACU, because that invariably also then falls on my watch if the kid spends a long time in the PACU. That chart gets flagged, and then I get an inquiry as to why we didn’t meet our discharge parameters. If DME is an issue, that always delays. I also warn parents and all of my friends, including PACU nurses, our surgical team, about real risks of waxing and waning O2 sats in the operating room. I describe to parents that I expect to see very low saturations and that it’s safe to do that because we make sure we can control that patient’s physiology before we let them desat to 68. I might let them desat to 68 and tell my pulmonary colleague, keep going, keep lavaging, get the rest of the fluid that you can get out. I know that I can bring the sats back up within seconds of making a change in the airway or a sharing that airway in a maneuver. And I describe that to parents because the last thing I want to have happen is their sats to drop to 68, them to get a little bit bradycardic. We do chest compressions for 15 seconds, get the oxygen back up, everything goes back to normal. My heart rate stays normal, but then if I have to go out to the recovery room and explain that to the family, and that comes as a surprise to them, they will think that oxygen saturation of 68 was a complication. An arrow, it’s a little funny to say that’s not a complication, that’s normal, that’s what we expect. And 68 is a random number I picked. But I explain that to the parents ahead of time so I’m not coming out and surprising them with deranged physiology that they then think is an anesthetic complication. I also have a lot of practice in spending time with parents describing to me what has happened in prior anesthetics as a complication. And when I hear the story and I’m able to piece all that together, I can have an almost reparative conversation with the family about what goes on in the operating room and helping them understand that in some ways for their particular child, those things are expected and maybe aren’t a complication, it’s just the reality for that child. So we’ve had lots of kind of repair and recover conversations in that way. So to the meat of what we do, anesthesia tricks for trachobronchomalacia triple scopes. Induction is actually a gold mine of information and frequently for kids who we suspect really look bad or their tracheas are horrible, I’ll make sure that my friends are in the room for induction or I will even sometimes whip my phone out if something freaky is happening and I’ll video it so if my friends aren’t in the room, they’re in the room next door doing the prior scope, they can see what happens because in induction of anesthesia, stage one is the beginning so kid comes in the room until they kind of start to get a little wacky and lose consciousness and their eyes start to roll. That’s not really an informative, that’s a behaviorally informative time period but not necessarily a physiologically informative time period. Stage two is where things can get kind of interesting and I like my friends to see what’s happening or I report to them what went on. So it’s an excitable phase of anesthesia when the ceboflurane starts to affect the brain. There is loss of consciousness. It’s the time period between loss of consciousness and then onset of atomaticity of breath. In that time frame, there’s a lot of irregular breathing, a lot of very active kind of discoordinated muscular breathing. They do lose their eyelash reflexes so one thing you’ll see is like we touch the eyelash to make sure we can poke them to put their IV in but that doesn’t mean that their other reflexes are gone. Those other reflexes are intact but they’re very uncoordinated but very active. So when we talk about what happens when a kid gets really upset, what happens to a kid’s trachea when they’re having this coughing fit that they can’t control, it looks a lot like what goes on in stage two. So we have this little window of time where we get to see what’s going on during that dynamic tracheal motion. Sometimes we can diagnose tracheobronchomalacia in an otherwise seemingly normal kid who just goes completely haywire during stage two of anesthesia and an experienced anesthesiologist will be able to describe to their ENT or pulmonary or surgical friends what was going on with that trachea even though all we really have is a mask on the outside. So look at stage two and the induction of anesthesia for all the nonanesthesiologists here as an opportunity. Oh, what did I just do? I just touched the cord and the slides went away. Hold please. Come on, Carlos. Sorry. Yeah. Don’t worry. Sorry, buddy. Thank you for your help. All right. Thank you for helping. I’ll do it in one minute. There it is. All right. There it is. Okay. So stage two, opportunity. Stage three, four, boring. You don’t need to know about those. Much deeper planes of anesthesia, but they do let our surgeon friends cut. So we like those. Importantly, so we love what we do in aero, we’re all like tricky people, right? We can do fancy stuff. The reality is our kids don’t live in the fancy world. They live out where like they’re going to get into a car accident in rural Wyoming and an EMT is going to come up to them on the side of the highway. So you can also get really reasonable information from an anesthesiologist or with your anesthesiologist about what a regular DL looks like, about what ease of access to the larynx or the airway or the stomach or the hypopharynx or the trachea looks like with regular equipment and operators, meaning like anesthesia residents, CRNAs, ENT residents or fellows. This is important outside of the OR because we’re not the only people who touch these kids. And the people who touch these kids in emergencies or in presentation to the hospital at 10 p.m. on a Saturday night are not always the aero team. So you can also get good information from induction for that part. So I have a whole internal monologue of what aero digestive looks like. I tried to get it on one slide with too many words, definitely breaking rules. So in five parts, a kid comes into the room, is induced, we put monitors on, place an IV, check labs if needed. We do have a process for kids who are difficult IV access. We have a number of pokes or a time clock, whichever comes first, and then we start to activate a stepwise set of approach for difficult IV access. Part two of the anesthetic is the pulmonary bronch, which we do through a natural airway with a spontaneously breathing child. And then we do something that’s really silly, and I do describe this to families, especially kids who have pulmonary hypertension, who have severe trachea bronchomalacia or who have either controlled or uncontrolled asthma reactive airway disease. We intentionally do all the things that in my entire residency were taught never to do, which is to lighten the anesthetic to the point of essentially reentering stage two while the scope is in the trachea. That’s for a dynamic tracheal evaluation and seeing an intentional cough and trying to mimic what’s happening when these kids are awake at home and having coughing fits. This is like breaking all the tenants of anesthesia, you know, pulmonary hypertension crises happen because of atelectasis, hypoxia, aspiration, hypercarbia, and we intentionally create all of those scenarios at one time in the operating room with a camera in the airway. So what could possibly go wrong? Part three, and that’s where you really need your anesthesiologist to be comfortable and not to panic. Like, we don’t panic in the Aro OR. Part three, once we’re done with the pulmonary bronchlavage, of course we’ve acquired a lot of atelectasis. I have an entire talk on how to fight atelectasis to get kids home after their scope instead of needing to be admitted to the hospital. The third part is when we turn the bed, hand the airway over to Dr. Prager, does an ENT micro laryngoscopy bronchoscopy, also was spontaneously breathing, but we’re about to put a very large, very cold piece of hard metal in the airway, and you have to obtain instant deeper anesthesia. So we go from the reentering stage two to within seconds being deep enough and the right amount of deep to tolerate a rigid bronch and a rigid blade. Usually we rely on propofol and fentanyl to do that. Occasionally we’ll opt for gas alone, and that’s a little bit more of a complicated approach. Once we’re done with the ENT bronch, thankfully our ENT colleagues help us clean up a lot of the secretions and fight the desaturations in PACU in that way. Part four, we move to the upper endoscopy for GI. Usually at this point we’ve left behind an endotracheal tube, and I still keep a child spontaneously breathing, but I lean heavily on pressure support mode of ventilation with a lot of PEEP, again just to combat the atelectasis. Part five is extubation and exiting the operating room. We usually extubate patients deep unless a kid really has severe physiologic derangement on induction and I’m worried that they’re going to have severe tracheobronchial collapse in the PACU. I would rather all of that very bad, dangerous, scary stuff to happen on my watch because it’s not bad, scary, or dangerous when we’re taking care of it in the operating room, but to a new grad PACU nurse who’s never taken care of patients on Thursday mornings, it can be terrifying and they will invariably hit the code button even though that’s the kids’ like normal physiology. So I usually extubate deep, but I can be convinced to keep a kid in the operating room. And transitioning patients to an experienced PACU is super important. It helps us get more cases done on Thursdays because we can take them deep and it doesn’t scare our PACU team. So a couple, Paul mentioned a couple things about do we do a CT or do we do a bronchian. I agree with him, the answer is usually both. What he did say is what is seen is highly dependent on the depth of anesthesia, so totally true. What that slide should have said is what is seen is highly dependent on the depth of anesthesia, trauma, and if you have an anesthesiologist who understands what you are worried about, you will run the anesthetic accordingly and get the answers you want out of the CT scan. There have been many cases where I am actually physically on the phone with the reading radiologist describing to them what the aero team was looking for in our multidisciplinary team meeting and then I create a different anesthetic condition and he talks to the, he or she talks to the CT tech to change the protocol real time. And so when I know what the aero team is interested in, we do adjust the anesthetic accordingly. The other thing Paul said is that CT may be limited only by passive exhalation or by sedation artifacts. So that’s actually not true in my book. I can actually control the degree of active exhalation and it sounds super crazy but this is what I do. I do our CT scans with an LMA for breath holds inspiratory and then for the expiratory breath hold, I turn the flow off of the anesthesia machine so there’s no air flow. I disconnect the LMA from the anesthesia circuit and I reach into the CT scanner from the foot end and I take my hands and I squash the patient’s chest down and then if they’re not quite squashable enough, I will take the five pound sandbag and put it on their chest and then I run out of the room and I say scan and the CT scan gets a very nice exhalational image where the kid’s chest is pretty much a forced exhalation because I’m squashing them. The only kids in whom this doesn’t work are the really older ones who are not as cartilaginous. They’re a little more bony and they’re kind of hard to squish. In that case, I just used two sandbags and it still works. So again, that sounds a little nuts. The poor residents that are with me on an arrow day really think I’m crazy but if you have an anesthesiologist who understands what you’re looking for, you can accomplish some of the things that don’t seem possible and I did mention we use an LMA. So a couple other things. I just was told I have two minutes so here we go, one minute. Doing the dance is a rescue maneuver we do during the P flex. You can see here the flex scope is in the nose. We’re masking over. That’s a good way if a kid does drop to that desaturation of 70 or 60 or 50 or 40. We don’t stop our exam. We actually just mask right over. We can also turn that mask sideways and just mask over the mouth and I close the contralateral nostril with my thumb and I can actually get a little bit of CPAP across the patient’s mouth and around the scope while without touching the scope so that the pulmonologist can still drive around. We did talk about the danger zone and light entry, reentry into stage two. What this does in our OR we don’t consider this a complication. We only consider a pulmonary scope complicated if the pulmonologist has to come out of the nose at any point but what this does is it allows for passive and dynamic airway evaluation, minimizes the time that we’re in the airway which prevents atelectasis and also preserves efficiency in the operating room. This is what it feels like sometimes when we’re around the patient and I have a few schematics drawn up for this is what our normal aerodigestive day looks like when we’re doing a PEXI it looks a lot different but we have schematics for each one. So a couple other things Paul said, “Inadvertent PEEP is a concern with mask insufflation and LMA.” That’s true but there should never be in the aero operating room inadvertent PEEP because that means your anesthesiologist doesn’t know what you’re looking for and what you’re up to. So you need an anesthesia team who can design that spontaneously breathing open airway plan that’s appropriate. So there should be no such thing as inadvertent PEEP. And then it is absolutely correct the PEEP trial is bogus unless you have an aero savvy anesthesiologist who can tinker on this with you. So you imagine that picture that you saw of doing this dance. So I have control over that mask and the pressures going through that plastic tubing and the flow and we can actually I envision a time where we can get a little catheter down in there that has a pressure sensor on the end and we can actually measure what kind of PEEP trial we’re doing. So tinkering is what we’re still doing. I think it’s important and if you do it as a team it works out quite well. Our pearls about tracheopexy essentially are the same. This is usually an open surgical procedure but with trans-oral intra-tracheal, intraluminal assessment by the ENT team. And so you really have to think about designing a nimble short acting medication, clean anesthetic, keeping a patient spontaneously breathing and leaning on PSV pro for ventilatory parameters for the duration of the surgery which can be quite long and a spontaneously breathing anesthetic is not feasible. The thoughtful, proactive, ergonomic schematic for what your operating room looks like makes all the difference. And then also training your pediatric surgical teams to really get comfortable with a multidisciplinary approach. So no such thing as like anesthesia time is done, ENT is finished with their bronc and now I’m going to operate. That’s not how it works for a PEXI. And I think to get all of our friends on the same page about what multidisciplinary really means is important. My one plug which is partly why I asked the panel our question earlier, choose a champion. I guess I’m the aerodigestive person here for the meeting so hopefully I can help you guys all find and recruit your own champions and there will be another anesthesiologist here next year. And then if any of you are interested in some anesthesia related but also non-anesthesia related fun daily reading, half a page of an email comes to you three times a week in the form of the PEDS anesthesia article of the day and I encourage you all to check it out. And thanks to Dr. Prager and the team for inviting me to speak. Thank you. [ Applause ] [ Inaudible ] All right. So we did want to have some time to work through some cases that have some different aspects. So this child is a five month old with a right side dominant double aortic arch who has presented with lifelong noisy breathing and chronic cough. It’s common at male to do a flexible oncoscopy at the time of vascular ring repair and looked to me to have approximately a 50% right sided trachal compression in the distal third with some mild additional dynamic collapse. The patient went to left posterior thoracotomy, ligamentally much ligated in the left arch was resected which has been a common approach. Postoperatively you can see that there’s no longer a left arch, there’s a bit of a circumflex right aortic arch and descending aorta down the midline where it crosses over from the right side of the chest into the down here over into the just to the left side of the spine. So this child presented too much later with still persistent noisy breathing which you always counsel can be commonly persist for while after vascular ring repair. Again typical trachal militia kind of sound with raspy, extra wheezing, coughing fit especially in the typical situations, awake, excitement, agitated. Difficulty swallowing particularly when getting into longer runs of chain swallowing would get got treated for potentially having a tracheal bronchitis and had some improvement and the cough dried up became less wet but then it still really persisted. This child did not respond at all to ipratropium or oral steroids and again the wet cough just returns. Video swallow study however was normal with thin liquids and pudding. Repeated the flexible bronchoscopy at nine months old. The right side of the compression was similar. Here’s the dynamic collapse on the left side around the level of the aortic arch and then because I’m not good at doing only what I’m supposed to do and staying in my lane I looked in the esophagus and there was, you could see the vascular compression of esophagus as well. There was concern about that crossover of the circumflex aorta that was descending and crossing over to the left side causing a significant compression on the esophagus. So she kind of went through the same kind of cycle of sicker than weller than sicker than weller though she was growing well but as she decided, started taking more solid foods developmentally she just had worsening and gagging episodes. They would, I mean she would, her breathing would become terrible. She would choke but she also would have rest of her distress and even become cyanotic. The family was managing this on their own by either giving her only very small amounts of solids, lots to drink to get things down. Daycare was sufficiently terrified that they would not give her anything solid and so they stopped it altogether and this was of course becoming very concerning to the family. So another repeat bronchoscopy, again the right arch looks about the same, the dynamic collapse looks about the same although this time I did find a chunk of green bean in her esophagus that was consumed three days prior and removed that. Because trachomyelitis is a dynamic problem, I want to play a video of how it looks still so I don’t think it captured very well. So the proximal trachea is normal, you can see some pulse compression on the right side, right anterior a little bit. I’m going to try to skip ahead a little bit because this is not as well edited as it should be. There’s dynamic collapse but it’s also pulsing posteriorly and this can be something that’s hard to differentiate between the airway pulsing against like the vertebral column for example versus actually having pulsations coming from behind. I think that’s easy to mix up but when the child reacts to the scope near the crina there is a distal collapse that’s dynamic with force exhalation, extends a little bit to the right main bronchus and then things are really better down there. And then on the esophageal side there’s the chunk of green bean sitting up above where the aorta is crossing behind the esophagus. [ Inaudible ] Which part? [ Inaudible ] Yes, yes we did. So okay. So it had been a little bit of time so scan got repeated again because we were planning surgical interventions. The original plan was to do consider a right thoracotomy and do a posterior descending aorta plexi hoping to have the aorta cross over further down the chest away from the airway structures and hopefully more space to cause less esophageal compression. However intraoperative look during that procedure showed that there was improvement in the posterior trajectory compression when the distennial where it’s pulled posteriorly however the anterior arch pull created some compression on the left main bronchus and did not relieve the esophageal compression. So this patient then underwent division and re-anestimosed the esophagus, flipped it to the other side of the aortic arch and re-anestimosed it. And then did three posterior trachea plexi stitches plus one going on the right main bronchus. There was a complication of some chial leak which was managed with a low-fat diet after NPO and discharged 12 days later and has been really fine. All symptoms are essentially resolved since that may unconventional approach to this patient. More recently than just three weeks ago I had a similar 18 year old who presented with a similar anatomy who had prepared H2 similar approach and then but had lifelong continuing dissident exertion that’s what she was here for. Does not have dysphagia really however she was getting by with the combination. She had a fixed x-ray obstruction but normal inspiratory loop on spirometry. So older kid presenting with an initial same kind of problem, similar right sided compression, posterior bulge near the leveled arch in the corona and a little bit different descending aorta position. So similar but not the same. And this is a good segue to ask the other, the surgeon at the table, you know, they’re thinking about patients like this and the approach. [ Inaudible Remark ] >> We need to be switched over. >> I think we can just quickly go through a case. I think we see a pageal atresia that can have severe tracheal malacia and one of our common referrals is failure to extubate and whether the move is to go with the tracheostomy or not. So this was an 8-month old small birth weight at type C plus an imperforate anus. Initially they divided the TEF into the G tube. The baby was OK in that interval. On CPAP a lot of it but not intubated for most of that interval period. These four months had esophageal atresia repair but really was not able to extubate after that. Developed an esophageal structure and spent the next four months on high vent settings. They were worried about BPD, pulmonary hypertension, the PEEP was high, getting paralyzed a lot of the time. And on our, you can see on this EGD study with fluoro, the esophagus now has a gap. This structure is sort of completely occlusive for this 2 centimeter length. This is the tracheomalacia, just kind of typical wide flat tracheomalacia. This was the CT scan which I’ll skip through. In this case what we decided to do is focus on getting a good airway outcome as the first step and deal with the esophagus later. So we actually did a spit fistula for that part and we did a posterior tracheopexy in the neck and the chest. And interestingly enough with that approach we were able to extubate on postop day five from that following sort of a four month intubation and sort of get to the point of going home on nasal cannula. It’s probably the case that the pulmonary hypertension quote crises were more airway collapse crises. Those two things can be a little bit hard to sort out. But that’s just a brief glance of sort of a surgical approach sometimes for tracheomalacia with the steroid extubate. I don’t think we have any time for questions so we’ll just end it here. Sorry. Thank you. [Applause] (audience applauding)