Dr. Thomas Hamilton - Complex Esophageal and Airway Surgery in Infants and Children

you you you you you you you you you you you you you you you you you you you you you you you you you you approach to these more complex esophageal and an airway problem. So Dr. Hamilton. Thank you. It's truly a pleasure to be here to talk about something that's now taken up over 10 years in my life and most of my clinical time. Everything I'm going to talk about today is part of my involvement with the esophageal and airway treatment center. We want to always a center. We started for several years in the steep learning curve and have evolved into this really remarkable program, but very dedicated and multidisciplinary team. Rusties are fearless leader. You all know that Ben and we trained and joined us and has been a wonderful addition to the team. Mike is our GI champion and medical director. Peter is the other guest runner, Aldous and Jessica's a fellow who's been working with us that we hope will will stay around Gary and Leah or pulmonary team and over nursing. I put these names down specifically, but there's certainly several more that have helped specifically Josh Reed and your Haldago and Megan O'Connell. And from anesthesia, Carlos has really been the champion, but Neil Sullivan, Juay, Mike Hernandez and Walid have helped us tremendously and with the cardiac aspects, Chris spirit and David Hogan's in. Or else it's been important to have them involved because of the prevalence of synchronous serodidestive lesions and you'll see them highlighted later today. You won't hear much about the plastic surgery today because they're really involved with the dejunums and that's not going to be on the on the docket today, but we certainly will be working with them closely tomorrow. Dory has recently been promoted to our program director position, which is well-earned after 10 years of service to us and the NP team. I don't know if Lindsay was able to make it, but she's been really carrying the clinical weight for us on the inpatient side and keeping us up to date with the details and where we have to be and what needs to be done. And the colon was or the outpatient side and Michelle is our program coordinator, Lisa's social work and then speech and language works a lot with these kids have very difficult time with oral aversion and feeling difficulties and they help us. And Jane especially helps us grow these young along gaps that need to get to a higher waist. So what do all these people do? Well, we do a lot. It sort of started as a program with long gap of sophidula, Tricia, but it became readily apparent that these really complex kids, you couldn't really just focus on the esophagus and really take care of the kid. And furthermore, there are a lot of different problems that they can counter. We work on strictures. We work developed and revolutionized some of the treatment for strictures with Dr. Matt Freddie within seasonal therapy. We're using endoscopic ultrasound, stance steroid injections, an extensive work with congenital strictures, but a lot of complicated sapodial reconstruction and you'll you'll hear about that today. Area anomalies also. Traco, Rockamolace will be the other main topic and we'll talk about all these things that we do today. So we have all these people, but what do we do? Are we busy? Well, this slide was prepared in June and at that point we were already above our targets for the fiscal year and clinic visits well over 1600 surgical procedures. We were up to 1500 and again, this is three months old. So we're extremely busy. And again, an old slide, but it clearly makes the point that our patients come from all over and we're consistently either the highest or one of the highest specialty programs in the entire hospital for out of area zip code referrals. So what I really want to tell you about today is the surgical treatment for Traco bronchomolasia. We'll talk about post-year Traco Pexi, anterior Traco Pexi and also a anterior Traco Pexi and not just anterior, but also descending and went to do that and why to do that. And then finally, we'll talk about long gap with the South of Georgia. You can start out talking about Traco Pexi with our first paper that came out on our work with recurrent Traco Pesafidio Fisula and categorization of acquired Traco Pesafidio Fisula because really this was the birth of the Traco Pexi and the strategy here is to completely mobilize the esophagus away from the airway, identify the Fisula divided and then separate the suture lines by tacking down the post-year membrane and then rotating the esophageal repair away. And what we sort of realized was, hey, the the airways of these kids looked pretty good. Maybe we should just do that even if they don't have a Fisula. And that's how it began. So to understand why it works, you have to think about a normal Traco. The normal Traco has about a four and a half to one ratio of cartilage to membrane. When that membrane ratio changes in the membrane elongates, it can actually intrude farther up into the airway and in the most extreme cases cause compression. The adjacent bronch here showing you a normal airway, which we don't see very often in our program, but you can see that there's not a lot of secretions. It's got a nice shape and it's why they pay. In Traco bronchomalasia, however, there's conlapse or compression of the Traco. And the main stem bronchi and most dramatically with exhalation. The frequency is estimated about one in 2,500 live bursts and it presents with a barking cough by a phasic expatory strider, very frequently recurrent respiratory infections, feeding difficulties, synosis or apnea or dying spells. Historically, this was managed with positive pressure ventilation, whether without a Traco, and occasionally with stents or an anterior aorta pexy. And what you have to appreciate is when this membrane is wider, it can actually move up. And with with forceful cough, and you think about it, medical school, we're all taught that anything that interferes with the mucus, silly a, like, ciliary escalator can cause pneumonia and get respiratory problems. Well, there's nothing worse than actually choking off the complete trachea at the maximal force of expiration when your body is trying to clear all those secretions. You can also have associated extrinsic compression from abnormal vasculature. So in active respiration, you can see that it's a dynamic process and that if this is collapsing, because there's such an elongated or soft membrane, that again, that's going to impair clearance of secretions and lead to respiratory or breathing difficulties. This is a bronc that looks at the static airway and the patient was sort of bow-shaped cartilage is. You can see they're sort of rounded. There's some secretions and it's not very patent. And when you look, if you wait long enough for them to actually have a forced exporter, expiratory phase, you can see what I'm talking about, about kinsing and trapping this secretions behind. And this leads to recurrent infections and pneumonia. And ultimately, if those go untreated, which we've seen with many out of country or even out of the state referrals, is that that can result in end bronchial, bronchiacctasis and end lung damage. So one of our first papers described the initial 20 patients. And I think the most important part of this paper was really thinking hard about how to really communicate between providers and other people about the precise anatomic level of abnormality of the airway. And Dr. Fienz, who is not with us today, but was our illustrator for this. And as you can see, we separate the airway into three main locations. T1 is above the clavicle T2 is the most proximal interest aracid component and then T3 extends down to the carina. Now, when you think about that, you really have to think about the anterior anatomy if you're going to try to design any procedure that's going to help because that's going to be in your way. And I like to see Fienz and Net are side by side because to me they're sort of in the same group. But this is sort of hot off the press that we just, it's e-pub right now, but we took a look at this and looked at great vessel anomalies in our population. And we identified over 209 patients that had an airway CT before and went underwent repair for trade-go Malaysia. And 57% had some type of great vessel abnormality. And those those, those, uh, enamored it was the most prevalent at 34% and we identified that the non-EA patients had a later time of repair and a more likely hood of needing a vascular pair of which that was 25 and 6 required by pass. So for these other centers that are out there, there's a lot of important points that we can help them with in terms of patient selection. So how do we evaluate these airways? Well, one thing that we really want to know is if the cords work and many of these kids are coming from another place and we want to be sure. So the best way to do that is an awake flexible airway exam. We have our clinic on Wednesday, which is today. They usually go to ORL in the morning and they're get their flexible airway awake exam. Then they come to clinic and then we schedule our bronch as needed. The most important thing about the bronch is that it, we incorporate three different phases. We want to look at them shallow breathing. We want to look at them active breathing or coughing because we really want to reveal the maximum level that that airway collapses because then we can tailor that any therapy to direct it exactly at that level. This has been a long and steep learning curve with our anesthesia colleagues. They have really embraced it now and understand that we really need to do this to get the airway examination. And they really understand now that this is part and parcel of and critical to what we're going to do and how we're going to play in the procedure. At the final phase, we want to look at the airways distended. We try to get that pressure up to about 40 and that's going to really help you identify abnormalities in the airways such as Aberdebrakeye, a trigusophagyofistuli. And then we occasionally will, if we really suspect the fish love that we can't totally prove it will inject a little contrast and do that. I think that the synchronous airway abnormalities are highlighted by this paper that Rusty is on that race was a senior where they actually identified 20 to 35% of our kids have an associated Laran geoclapte either one or one or one is most prevalent but two is fairly common as well. So all those things are going to be important for the management of the child to help them ultimately be able to eat and breathe normally. So when we look at the airway, we're going to describe the collapse. We're going to describe anterior collapse. We're going to describe posterior intrusion again. It's a, it's something that we want to know exactly where that level is and that this if it had run right, we're just going to show you that the airways actually collapsing. This is a kid with a trakiostomy when you look inside. It's not pretty hard to figure out why he needs one because his airways completely collapse below that level. And if you're patient and you keep moving to a distention phase and you move down towards this, you'll see why they had so much trouble intubating this child from above because they fall into this giant crater of a diverticulum that we're clearly accommodated and then to track you to and then be very difficult to ventilate the patient. So all these things are critical to help you plan the operation and this, this is one that we would, would strive to repair and get that trakiostomy out. So when we look at our airway, these are all the points that we're trying to look at on Friday when we're, we're got five or six bronchoscopies with a, soupier, whoever else is with us. This is what we're looking for. Ben has done a nice job now incorporating this to a ad hoc form and power chart, which I'm learning about, but it's going to be very helpful to look at our data going back and make it a lot more easy to follow. Follow these patients. So in summary, the three phase dynamic bronch, it really allows for precise definition of the anatomy and it also helps you characterize the severity of the traki bronch amalasia. It facilitates our ability to communicate among the disciplines and it's really an objective tool that we can correlate the pictures, the video and the description to look for outcomes and absolutely critical for surgical planning. So what's our algorithm so how do we get these patients to this day after you know, 11 or 12 years now doing this Facebook is by far the number one referral center these patients cannot wait for you to get the paper out and publish these families are online all the time and even last week ahead and three patients Facebook is where they where they got us. What are they looking for they're searching for recurrent barking cough prolonged infections exercise intolerance or acute life threatening events are unrelated explained events and and it's really you know every Wednesday again will be there today at one o'clock it's like a broken record they get bronchidilators they get in health steroids they get sometimes. Pio steroids and antibiotics every four to six weeks so when they come we're going to look for those signs and symptoms we're going to do our dynamic bronch if they don't have traco bronch amalasia will will they fall out but when they do have it then we're going to define the anatomy we're going to get a CT scan we're going to identify the great best anatomy we're going to look for the dark artery of a damn points. And then they fall into the category of well do they have congenital cardiac defects that need intervention if they don't then we usually start with a posterior with or without a descending or to pexy and we'll talk a little bit more about that shortly. And if their symptoms are resolved and they're done if they don't resolve and they'll be candidates for anterior work for those with congenital cardiac defects we often will do the anterior work in a combined fashion with the cardiac team. And then if the symptoms persist then we'll do the posterior. So post your trachepexy really fixes the membrane to the anterior longitudinal spinal ligament and it's critical that this is under direct bronchoscopic guidance because what looks good to you sort of like the lymph nodes looking at a lymph node you can't tell if it's positive or not. But when you pull that membrane back you have to be sure that you're not angle dating the airway in a way that's actually making it worse and that's very hard to do without the assistance so we rely heavily on our anesthesia colleagues to do that for us. The pexy can be done at the time of the sausage of the soft to your work can be done at the time of primary a repair can be done minimally and basically or open. It stabilizes the membrane and it still allows for an anterior pexy which in our experience now is about 10 to 15 percent I think also will go on to need anterior work. It's also very good as you saw previously for management of recurrent tracheosophageal fistula. The main risks are the thoracic duct and the injury to the recurrent legular nerves often these are done in very complex reoperative fields. The diagram isn't good enough for you to picture what that really means we're going to show you a video now this is something that Ali and Jason presented at the I think sage's meeting I'll answer it and so what I'm going to let this one and I just talk to you about I better let it run. There was doing it. Talk about the steps of the repair with us now remember this this is the Bronx showing yes indeed this child has collapse and that is a candidate. This is a very select population out of the 400 we did about 12 so 2 to 3 percent at a virgin chest were over 10 kilos and didn't anticipate having a or to work need to be done. The first stage here is we're going to harvest the azygous vein and we're going to use that as our autologous pledges for the actual tracheopaxi in in the reduced situation that's often not there anymore. And when we incise that plural we try to stay a little anterior so that we can recapitulate that at the end to keep the esophagus to the side after the repair. And that is the incising the plurum. He's going to take it up you know you get great visualization and optics here which you know this is typically 2 or 3 hours of dissected in the redo field before you get to this point. But in this particular case the isophagus was leftward and Jason pushed it that way and kept it that way one of the advantages here is that the surgeon has a coaxial view of the Bronx down below. And the the operative field and that that isn't the same in the in the open approach when we do this. Again you can see that he's checking to confirm that that for here he's just completing the mobilization of the sovagus but as he places the sutures he's looking down and making sure that that's really giving him the opening or the. The airway that he's setting out to achieve that's the it here spinal ligament that's getting the the suture and he'll set them all in. And then tie them down sequentially after they're all placed. In many ways it's sort of like the lab coli when it took over the easy gallbladder the robot takes away all the easy to take your pecs you know so. But it's a really helpful helpful tool now. At the end let it run because the doctor munos helped us develop a technique of applying negative suction at the end to sort of recapitulate that exploratory phase or active calls that we can ensure. That after we placed all the stitches and open it to our capacity that we're able to keep that airway open as our objective was. And you'll see that coming up. There it is the munos maneuver and indeed once the sutures are placed and then you apply it it remains open that's a good predictor that this this kids going to have a good result. In the open technique it's not the same rusty and I usually joke that once this spot area we're both snipers you both can shoot but only one can actually take a shot. So rusty is placing the sutures and the whole team usually not to me is telling them whether that stitch is actually too deep and penetrating into the actual element or whether you're seeing a suture but again the bronchoscopic guidance is critical to ensure that you've got the opening at the level that you want an inner fashion that that actually improves the airway. So that's great here's the before and after pictures but you know those are great pictures but how they do well three really busy surgeons and a lot of patients to look at you take a really busy research fellow who's very productive and give her a big task. And here's what you got. Hester wrote up this experience that we had of the first 98 and if you look at the percentage of patients before and after a tragiorepexy that remarkably better in terms of symptoms of cough, heart and cough, noisy breathing, prolonged infection, recurrent infection, exercise and tolerance and it gets outlier because a lot of these are babies that the older kids really can subjectively feel a difference. Transient respiratory distress, oxygen to dependence and ventilator dependence, loose bells, apparent life, threatening events. It makes a huge difference for these kids. Dory worked with the marketing department here, any of you who would like to learn a little bit more about it. There's a free ebook that you can get on this at this website. The patient is Kate Elliott who is a typical patient. She's eight years old, spine a bifida, a soffordial atreasia, bad malaysia, just told that she would grow out of it and it wouldn't be a problem but she was very limited in her activities. After the pexy, she's now got a lot more energy and is doing after school activities and is no longer getting the frequent recurrent respiratory infections. You can make a real big difference on these kids' lives. So childrens we always love to talk about our history and when you talk about a orthopexy, then you got to talk about a rubber e-growth. Robert e-growth is original description of compression of the tracheum and the anomalous enamor, an artery, an operation for its relief. We described how if the enamor takes off too far down that it can go across and compress the tracheum and that to do that you need to place the stitches just so that if they bleed to take them out and hold pressure and do it again. They must be in the end of the tisha and you should place all the sutures before time. A lot of these principles we're still hearing today. We don't use silk and we frequently use a transverse incision even though we're going to make a vertical hemispher not any sometimes we'll do a vertical depending on the extent of the work but the principles are the same. He only used sutures on the aorta however. For us we look at the problem. If you look at the sages of you, especially in the infants, the thymus can be taken up a lot of that space that you really want that real estate to lift up the order and do it. So we discard the thymus and then we place the sutures on the aorta. This particular cartoon is sort of after the cardiac guys have done it. I always sort of cringe when they peel off all the tissue that keeps the nice trache in a order together because they sort of take away some of your fruits of your labor but we've had an innovative solution to that which is that we go directly to the trache itself. Also, if that doesn't work, we still can do the post year work to have opening on both sides. So it's anterior pexy and posterior pexy again that's only about 10 to 15% of the population. So great, but does it work? Well, Rusty wanted to get this to a broader audience. So he had Claire who was at the time one of the fellows with the suki. And she wrote this up and presented it at the ASPO, which is the American Society of Pediatric Oodle Lair and Gology. And I guess they liked it because she won the paper for the best clinical manuscript and it is now subsequently been published in the Leningu scope. So this paper looked was a retrospective review of the patients who underwent anterior and posterior trache bronchopexy for a four year period. We didn't exclude anybody. There were 25 patients, 26, 28 months of follow up. They had life threatening events, ventilator dependence, tracheotomy, recurrent respiratory infections, increased work of breathing and exercise and tolerance. Many of them had associated synchronous aerodestic tract lesions. Many had other complex abnormalities that you can see here. So anterior post year pexy was performed at the same operation with cardiac in nine of the patients at a 25 and post year first and anterior in 12 and an anterior first during post year and four. And they were separated. They were about 184 days apart. The results were that 21 out of the 25 had significant improvement in the respiratory symptoms. So 84%. Three of the five patients who previously needed a tracheotomy were decanulated and no longer needed the tracheotomy. There were no post operative deaths. There were two of the five patients did not get and continue to airway collapse with chronic lung disease. Large airway surgery doesn't help the smallest airways. It does clear the secretions and help them clear them, but the collapse is truly distal. It's not going to be affected. There was a one recurrent neuro injury. Unfortunately, one who had had a previous cord out on the other side. A new trache in two patients for vocal cord immobility. So it's not perfect, but it's a really big changing event. And these are things that we're all working on to improve now. So decennial or depexy. I put this up with the already a damp woods because you can't think about doing it unless you know where this final cord blood supply is. And and fortunately, Sunday, Prebu has done a lot of great work for us helping us identify this in these patients and let's us know whether it's safe or not to proceed. So the real conundrum is that the left main stem specifically L2 is where the impact is in a typical left sided arch. You can see that very clearly here where the extrinsic compression lays in this airway. So descending or depexy is what we do to gently push the order back again, and then the initial test stitches you're not going full thickness and you're just trying to tuck it down to the level because if you pull the trachea level down at the carina and it still has extrinsic compression high, you're going to exacerbate that extrinsic compression on the left main stem by pulling them down together, you can improve the opening of the of the airway. And Dr. Shagg looked through all these patients for us and again was able to retritulate some of the findings that we found in the larger study with the trachea pexy that indeed they had improvements in cop, bargain cop noisy breathing prolong infections and recurrent infections. So all these things have made a big difference for these children. And we need to descending or depexy when they presented this at absent that 50th celebration of some of us were on call. And basically we wanted to know is there any clinical predictors that we could use preoperatively to help identify it specifically did we have to ask Neil to put another a line in the foot or not so that we could monitor blood pressure above and below and make sure we weren't impeding blood flow. And the bottom line is that when you looked at all these patients that that really the predictor was greater than 50% collapse of the left main stem on the wrong and a greater than 50% anterior displays deorta on the left. And I think this is important because since Jason has put out that first video article the robot there are several people now publishing about their case reports of minimum invasive trachea pexy and they need to know that are some of these difficult clinical situations that you're going to encounter that you need to consider before just embarking on it. So that's the airway portion and the last part is going to be about how we're doing with long aposophageal treason. So when you if any of you're going to New Orleans in a couple weeks that you'll hear about this but that the background is that the focal process uses tension accelerated natural growth tang. I've been trying to knock stretch out of the vernacular in this institution for 10 years and I'm still working on it every day that tension somehow is a mechanical force that is translating to a cellular signal that is causing growth and I'll show you some examples here why I truly believe that that's the case technically demanding if there's a definite learning curve for this procedure and outcomes clearly very among centers. So we sought to look at the evolution of our treatment algorithms and our outcomes for these patients. So we divided them into the long gaps that were 2014 to 2019 that's our contemporary cohort then we compared them to historical controls from 2005 to 2014. And long gap is always one of the difficult definitions but it's really the inability whether perceived or real by the operating surgeon at the time to achieve a true primary and asthmosis due to inadequate the salvageal length. And that can be a pure type A it can be a type B that has a proximal fish flow to the upper pouch and a long type C that there's just not enough length to get it together. We've also included patients who are transferred from outside hospital just inability to connect the esophagus for whatever whatever means. We looked at demographics in terms of gender, birth, weight, prematurely, associated heart disease and genetic abnormalities which as you know are high in this population. So when we have the unrepaired long gap we have three avenues we think about the folk are we always think about primary and asthmosis if it's possible. And sometimes if there's no esophagus present or if they've had a prior esophagus to me we will just move those to to genome. And again those need to be 10 kilos. So when you look at the separation of the focus we put those into ones that are primary that we got to start from the beginning or the rescue failed attempts at outside places and then we're going to look at our techniques. Minimally invasive versus open or a combination and how we did. So there are different ways to measure the gap length. The first is simply using the big styleators and I think the most useful thing about this is with your pressure in the banks that's what you're going to get when you're done mobilizing. You're not really going to get much more so that really helps you plan from the beginning what you're what you're up against. When you you can also measure it and is graphically looking at it and injecting some contrast you have to here unless you have two endoscopes you can't get a picture in one with with both of those. You can do just static contrast without the endoscope. And when you're doing the long gap it's really like to say caveat emptor you really got to think ahead what are you going to do if you cannot get it together. It's a different situation if the ends are closed if they're closed you can simply tack them to the spine under tension. If they're less than a centimeter you can probably just come back in four to six weeks and you'll have enough of solvigates to put it back together. That works best in the kids that are three to three and a half or less kilos. If you have closed ends you could also place them on traction and put that accelerated natural growth and that's easiest when they're greater than three or three and a half kilos. If you got open ends you got to close and tack them to the spine very them in the spine don't let them just sit there out in the breeze because they they can't leak. You need to drain the stomach and you need to give them a total brain role nutrition. You can also close them and put them on traction again that works best with a bigger kids that don't have a lot of other associated problems going on. What we don't want you to do is in the saffagostomy and we don't want you to do a myotomy. We want to wipe that out of the textbooks because that's what happens over time you get this big dilated mucosal tube that's the area for stasis, vegetation and really sub optimal feeding outcomes. When you're looking at the long gap we like to sort of think them in different categories. There's the really hard ones or the ones with just the sapodio primordium. The difficult ones have a little bit of a segment and these can be a fake out. Often that little stump isn't really good at saffagost and you pull on that end alone you're going to have it a bolster or leak. You really need to get down low on that distal segment to apply your attention to induce growth. The less difficult ones are simply the ones where you have more saffagost to work with whether they have associated fish or not we can take care of that. This is my case which I think really convinces and should convince all of you that there really is a translational signal that mechanical force gives to this cells of this saffagost and tells it to grow. Here's in this case with a sapodio primordium. This is what it looks like of contrast. This is what it looks like through the gas thrust to me. All you see is a little mound where it should be but there's nothing there. What do we do? You make a poster lateral incision usually on the right and then you identify. If you can't see the segment follow the vagus it will take you there. Usually has little stocking cap on it that you can pull. If you still can't see it get Mike to put a scope in and find the segment for you. Place your tracks and sutures just so deep enough to get the submicosa where there's some strength but not into the lumen itself so it doesn't leak. Usually you can get four if you're lucky sometimes five but sometimes these are very small. The technical points again avoid the lumen. One of the lessons that we learned was that even though we felt like we were doing pretty well that there's verification and discovery that you're not through the lumen is a good thing and and replace the tracks and sutures if they break repair the leaks if they leak. We used elastic sheeting around the esophageal segments so that they don't adhere to the long as you try to pull them or meet other media and the separate film slurry has been a really effective way to prevent adhesions. The minimum of both minimally invasive and non invasive and basically that's just instead of trying to lay that sheet all over you just cut it up into small pieces and then and turn it into a slurry and you can put it where you want it. For the enastimosus we use non reactive sutures and take generous bites. This is how it looks. The attention and discs that we apply and this is how they look in the ICU is usually been at about you know five thirty in the morning when they're here he's in there doing that if you want to see it. Important technical points about the enastimosus are to lay them all in first and and don't try to put all that tension on on one individual suture and this is where I have a hard time with a orcoscopic. Enastimosus because I think it's very hard to do that what we do it we we place all these numbered al does that usually makes the or team cringe a little bit that yes we need the number of al does and we place them all around issues about 15 in the back row and 15 in the front row. And we slowly apply sequential tension so we can see if we can get them approximated and together. So here we go back to that primordium here's what it looks like after a fabrication of tension usually for ten fourteen days and there's no way that that little numb is stretched to that level that is new cell growth. I think dr. Kim did a little abstract that was published showing that DNA actually changed in this tension and the model the and there's the ultimate a software and in the end. So again we went from that to an actual lumen that's functional and able to eat. So for the internal technique we're doing the mobilization the orcoscopically you're applying the sutures but to the segments but you can actually just park them there and then you sort of have a stage sequence of going into to adjust them. And this although it looks all that sort of yellow crunky stuff is that separate film slurry and and it can be accomplished in the minimally invasive technique. So when you look at all these patients we had seventy five long gaps forty seven had focus eighteen of those we were able to get together in the first operation and ten of them we deemed should just have an immediate to do them. There's similar in birth weight prematurely. The general heart disease was more prevalent in the ones that needed a folk or they all had similar genetic abnormalities and the type of a tree said I think there's a misconception that most of our kids are failed long out or failed type c somewhere else it's only you know ten patients that got focus for failed type c somewhere else we have a large number of pure long gaps that we deal with. And if you look at what surgery they had prior either no surgery at all as ideal but for the focus that that's only five and then primary two and immediate zero and then if you look at the G2 plus ligation or previous attempts at a true folk or an attempt at a primary repair those are the ones that more likely go to the June when you look at the pre out variables it makes sense that the longer gap needed. More a folk or or weren't as amenable to primary repair whether you measure that length by pressure static or virtual bodies the age differences really that our our fastest colleagues won't let us do a jujune and less throw over ten kilos and because we'd like to super charge those to junums dog met the blood supply and we've had a really good good luck with that so we hold off until they're their ten kilos. If you look at the technique that this is really where we've we've evolved there's of the thirty two if you look at the open door economy is fifty five focus thirty two and primary was thirteen immediate to June was ten but we have done sort of all one or a hybrid and some on the right side some on the left side and the left side really is a question of either avoiding all the prior mess in the right chest for the right. So we've got a lot of work from the third economy or to try to keep that airway encroachment from happening as you pull that proximal segment behind the trachea during the growth induction when everything is a demodus and a swollen and eating time and we followed these kids up as you can see for some time now. So when you look at the subgroup analysis external versus internal there's no difference in the demographic variables including the tight the gap length of the weight when you looked at primary versus secondary or rescue folk or the rescue focus tended to be older and the tape send it away more but there was no difference in the demographic variables. And when you look at external versus internal here's where you start to see why we're still interested in trying to do this is because the length of paralysis is significantly less if you do it internal the length of intubation is significantly less the IC use data significantly less and the hospital state significantly less so all these are good things and importantly there's no difference in leaks dilations or strictures section rate after putting their. So primary versus secondary there was a greater leak rate and rescue focus that sort of makes sense those are usually messy chest that we've had to go into for the umpteenth time there's a greater stick to rate and I think the more that you operate and take away the collateral blood supply the more challenging that blood supplies going to be. At one year 33% of the focus are fully orally fed and none of the rescue of focus and that's that really speaks to the oral aversion that these kids get when they when they can't associate PO with with feeds again the feeding outcomes full oral intake 13 consists in oral intake is 14 and then predominantly to bed usually for either a bad clath or neurologic abnormalities or excursion. So we do. When you look at historical controls versus the contemporary outcomes for post op outcomes we we have had some improvement the time on traction the length of or else this is less the length of intubation is less the ICU like this day is less and the hospital like this day is less so we are improving over time. And if you look at complications again we're we're learning something from our previous mistakes. Anastomatic week strictually section venous thrombome embolism Kyle leak and fractures fractures really were a product of the paralysis in the early experience where we we intensively have managed now the calcium and vitamin D and and the boy did lay six as much as possible and has really dramatically improved our fracture incidents. So this is it the conclusion is our experiences the crew post op of outcomes and pralysis times intubation times unlike the stay of decreased and over a year over half the patients have consistent oral intake. The things that we're working on now for the future are identifying whether left side is best whether internal traction is best and we've really incorporated the e-back as part of our strategy for leaks and and ice by where we're sort of using it a lot but trying to correlate that now with with outcomes and seeing that make a difference to help us guide individual operative strategies that in real time. And so he's been helping us with now trying to do with currently or in jail and they're monitoring on his may of these patients possible it's challenging with the smaller kids to insert the probes and have a work but it's certainly helpful and we're we're learning to adapt this technology to our to our patients. This is just the team at the block time at 11 o'clock on Friday rusty gets photo credits will be doing this again tomorrow that's been in some all and I close in to June. Again, I'd like to thank all these people it's been a real wonderful journey for me individually. I really made a career for me and the other people that you didn't see before that we have to thank our doctor smithers who's now doing his own e a clinic and shop and Florida. Dr. Folk or really helped us in the beginning and and it was Dr. Shamburg who weathered that focus storm and also supported us to listen to the early growing pains and allowed us to continue to move forward to what we heard today. The treatment was our program champion as his vice chair role and is now in his chairman role is helping support us through and then all the other people on the team who really do a remarkable volume of work and take incredible care of these patients and and Lindsay I see showed up now in the back we shout it out before but now she's here she's done an incredible job on the floor helping keeping us in in line and in tact with all the details for these complicated patients. I don't think any questions or or comments that you have. Thank you. I think it's a quite remarkable I can say from personal experience having scrubbed on a couple of cases with you guys the concept of the poster tracheore proxy makes total sense I think it's when you see it in the or. I'm glad to see that you guys are using your resources now to get the data out and so it's good to see that as well I know it's taken some time to gather that data and put together so let's get to see that I do have a question about what you know often when you develop a new technique it's good to compare to history. The historical patrols not not your own historical patrols necessarily because those are the tools you're talking about where so your early versus late experience but more what was done in the past because these patients existed most of them kept their trade yeah some of them kept their trade the ostomies someone on to get bronchia ectasis and end stage lung disease and some probably did improve as they grew but we really get the most severe ones so I think we're we're offering something to kids that previously we're going to be able to do that. We're only given an interior pexy and if you have really bad lower airway disease that may actually exacerbate the lower area of disease if you just pull up on the top so I think it's it's you know when you look at the natural history that we really can make an impact for these kids. So I mean just throw it out there I think it might be worthwhile to do a real historical comparison with what was done before so the you know the old standard of care and I don't know if you have any comments about this but you know I think what was the old standard of care what happened to those patients and then what's happening to you know your current cohort I think that would be useful to know. Thanks Tom for an amazing journey it's so much fun to watch that I'm so glad I could participate. As I travel around the world talking about the center there's absolutely no question where the world's leader in the softener reconstruction now vascular reconstruction and soon to be in chest wall reconstruction it's a blistering exposure to realize the part of an incredible team that's going places nobody's ever gone before. It's a great question about historical controls the biggest problem with tracheobronkel Malaysia is it's not diagnosed correctly there are no good historical controls because throughout history nobody's diagnosed it well except in the very very extreme. So there's a huge cohort thousands and thousands of patients out there who are being treated for recurrent group a typical group a typical asthma all these other diagnoses and all of them are told they had a little barkey car from the young we all went to doctor school you're going to outgrow by age to they don't they go off to the pulmonologist to treat him for a typical group a typical asthma a typical pop of all recurrent pneumonia say you're prompt X as they become trickiest independent. There's no accurate group that's way had to develop the dynamic three phase bronchoscopy for an accurate diagnosis and we had to develop the classification system so we put a number on it and now that we understand this disease process on all the associated anomalies. It's even more complicated but now we're we're getting to the core but I don't know if we're ever going to go back to historical controls because there's a bunch of crippled kids out there who can't exercise chronically ill recurrent pneumonia is I wanted the little issues that I'm fighting against is the tracheol diverticula I talked to you talk about tracheol divertic electemies to key element to reconstructing the airway. And yet every time I bring it up to pediatric surgeons they say oh it's not a problem what's not a problem because they've never diagnosed it accurately and they've never been able to treat it so they ignore it and you talk to the ENT surgeon is a couple of people who do the click line opening of it and they really don't understand the significance of the tracheol diverticula so. It's really tough when you're in a new sort of you when you opened up a brand new field that people want to know what was it like then what you all have to do is go to your clinic and look at all these sick kids that you've never diagnosed and don't know how to treat and that is the cohort were treated so we had a new diagnosis and a new therapy simultaneously developed and that's the the challenge of historical control so I don't know how to get a good historical control. Does anyone have any other questions for Tom or any of the team members? I'm actually curious you know so Tom's here doing all the work and it's rusty you're traveling around the world now talking about this. One of the signs that you actually have changed the world is that other centers start doing this work successfully and I know you're trying to do that and you brought people here and you had a very successful symposium here a few months back which was very well attended. What's what's it look like out in the rest of the world in terms of who's doing this have people published about their experience you know what's happening out there. If you can do a PubMed search there's three new papers that aren't us on post your Tegipex team in only a base or not and people are trying it with very low numbers and very well result. That's a great question how are we going to roll this up? Very painfully because this is not pediatric surgery this is non cardiac pediatric thoracic surgery and I think which is going to roll out to be brutally honest the cardiac surgeon is going to do it everywhere else. Because pediatric surgeons are scared of the airways and. As I travel around all I operate with pediatric cardiac surgeons. The pediatric surgeons want to deal with the esophagus the cardiac surgeon to dealing with the blood vessels and the trachea and all the associated anomalies and the ENT surgeons are working with trade gastro ames and the nerves so I think pediatric surgeons have a real opportunity to enter. Sort of this field. I think they're blowing it as I watch them they're just shying away from it and the cardiac surgeons are jumping on it with both feet. I presented the ATS in Toronto and I ended talking for an extra hour because they were so interested in the airway because that's the biggest problem for a big problem in many of their kids getting successful cardiac reconstruction for things like absent home devils and things where the airway is markedly compressed and deformed. These kids can't breathe afterwards and they go on to dive very high mortality rate. Well now just Chris bear travels around teaches cardiac surgeons how to do and to your imposed your pixies and slide bronca pixies and the last days and things like this. They're getting survival and a lot of them so they're quite enthusiastic about traveling out to Netherlands in February to present to the European group. The European group of a pediatric cardiac surgeon teach them how to do it so I know where it's going to go. I'm clear that there's two components I think the really important one is it's not just to post your pixie that's one small element of a complex thoracic care of these children including blood vessels just while part. They're just so much less soft against everything else and it's right now taking a multi-disciplinary team and so we have to train so we went out to to Israel our entire team went out there we trained them we did a bunch of cases. Clearly we're going to go back. I was just in Brazil we did a couple of cases, such as seven cases and they want a program so they're going to invest in it and that's going to have to be how it goes. We have to go out there and teach them chop is very interesting this this talk that Tom just gave. I talked to chop about two years ago and they said they're completely uninterested they don't want to talk to me about this they're too busy. Tom went out there and talked to him and now they're going they want a entire multi-disciplinary team come out and visit us for one day. Well those one day visits never work so they failed to start a program like this like the each center in Denver and in Utah and a couple other places. San Francisco is trying right now but it's not going to work because it's not just one operation it's sort of holistic care and moving it with the around in a safe fashion. Last words Tom. Thank you very much. Okay, thank you all. Thank you.