Interesting Case Presentations Part II: EA/TEF

As your webinar is teaching everybody today, so I'm really glad you're doing this and thanks for hosting us. The next slide, please. I don't see any arrows. OK, I'll do it for you until you see the arrows. Yeah, and you can just move along. OK. This is a child we met at birth, full term baby who was diagnosed with a type 3 esophageal fistula. We took him to the OR and did a bron. Bronchoscopy where we saw a single fistula. He had an open TF repair through a thoracotomy, and then on about one week we did an esophagram which showed contrast in the trachea. My heart sank, and we took him back to the OR, repeated a bronchoscopy, and could not see a fistula, neither a fistula at our repaired, nor any additional fistulas. So we said, well, we'll feed him, and he fed great, and he came back for his follow-ups, didn't have any problems until about one year of age where he had significant coughing, gagging, and episodes of aspiration. So we do an upper GI, we see a mild shoulder at the anastomosis and a questionable hiatal hernia. So we called our ENT colleagues. They did a laryngolaryngoscopy. We repeated the bronchoscopy, did an esophagoscopy, couldn't really see anything. This is an image of his upper GI at that point. And um you see kind of in the mid portion where it repairs, the esophagus is actually quite patent, and at the lower portion of the esophagus, he has a significant narrowing. So we thought for a while what this might be, thought this could maybe be a, um, congenital esophageal cartilaginous ring, and not knowing exactly what it was, we tried dilating it a couple times, which did not work, and so we decided we had to resect this. So we performed. A thoracoscopic resection after endoscopic localization. Here's a few pictures we got around the esophagus with a thoracoscope. Then we entered the endoscope, saw the stricture of the lower esophagus because it wasn't visible with the thoracoscope. And then here this is a brief movie. I don't know if you can turn that on. Yeah, we're gonna play it for you in a minute here, right. So the thoracoscope. And the endoscope at the same time and then we turned the light off. And it shows nice transillumination of the esophagus. And so that then allowed us to localize it. Um, we went, you know, here you see nice little light transillumination. Um, and go to the next slide. We took a thoracic, Wolfgang, we're just going to let it play because some people have a little bit of a delayed bandwidth, so we wait about 5 seconds and then we'll put your slide up. Absolutely. So we're very excited to do this kind of hybrid type of procedure and we placed aidural or spinal needle through the chest wall and put it in the position of our light reflex where we saw this. Here you can see the spinal needle. And now we knew exactly which segment to resect, which through the thoratoscope was not quite obvious. So we did our resection thoracoscopically, sewed it together, we did our anastomosis, and here's the pathology where you see this little cartilaginous ring, this, um, segment of the esophagus that was removed. So postoperative day 5, we do an esophagram, and at the same time, unfortunately, a bronchogram. Where you see the contrast um in the tracheobronchial tree, so my heart sank again, we started over, we did another esophagoscopy and um. Bronchoscopy, tracheoscopy, and we finally see these little ridges here at the upper esophagus, and this time around, we managed to introduce a small little catheter and realized that this child also had a H-type fistula on top of his two other problems. But at the time, this is now a few years back, we tried fibrin glue, we tried roughing it up. We did not try any of the cautery that was described today, and we did that several times, and it failed. And so subsequently, the patient had a surgical repair of his H-type fistula through the neck, and since then is doing well and had no additional problems. He's now over 3 years old and is doing great. Comments. Uh, I think that the endoscopic approach to, uh, tracheoesophageal fistula has got a reasonably good track record with recurrent ones. Right. I think the track record with congenital ones is not as good. And it seems to me that the surgical approach to a congenital one is a lot easier than the surgical approach to a recurrent one. So, my only comment would be, I, I would have started with that rather than fiddling around with the endoscopic approach. Yes, and, and, and we had, uh, I, I had the almost identical patient, Wolfgang, uh, did the repair, had a distal esophageal cartilaginous ring. I tried dilating it. For a while and it kept going right back and I, I should have never even tried it because it wouldn't have worked, um, and then we did a, uh, a thoracoscopic resection and then our patient, and then my patient, uh, developed a, uh, recurrent, uh, TEF which we ended up fixing. So then I wonder if the distal, um, you know, it's interesting. I don't know if that was, you're pretty sure that was an H type but not a recurrent, right? Yes, absolutely. Yeah, so that was, that was a different fit than your initial distal fistula. Yeah, yeah, I think. So this isn't an H-type fistula, this is a missed proximal fistula, exactly right, a missed proximal twice, which is wondering if those two initial bronchoscopies were flexible broncs through the tube or were they rigid broncs. We did two rigid broncs and then we asked our ENT. They did a DLB. I don't think he ever had a flexible bronch, actually. You know, I think this is a good point. One, you know, the resection of a distal cartilaginous ring is a great thoracoscopic case, and the hybrid approach is definitely the way to go. I mean, whenever you do these cases, it is almost impossible to tell thoracoscopically where that where that stricture or cartilaginous ring is. And so it's important to have. An upper endoscope in to help you identify at the time of surgery. And you should be careful because I had one where there was actually a second ring just below it, and I didn't realize that. I cut out the first piece and then tried to pass an NG tube down into the stomach and wasn't able to do it and actually needed to cut out a second segment. So just, just to be aware that, make sure you can pass something into the stomach. But the other thing is, I, you know, I've had a number of, of. Either H type. I, I guess I would call them H type, but, you know, I wonder, recurrent, where the patients have had multiple studies, upper GIs, bronchoscopies, uh, you know, where they, these fistulas have been missed. Um, and so I think it's very easy to miss them. And so, I'm not sure even if you choose to evaluate these at the initial, time of the initial, you know, you, you think you have a, A type C and you bronch them to see it because these are so difficult to see the upper pouch fistulas or if you will, or the mist H or whatever is so difficult to see that, you know, I'm not sure you necessarily see it in that newborn patient, um. Under sort of all the things that are going on in that, so I just think you need to keep it in mind that even if you don't see it at that initial procedure, that there may be another fistula there that was missed, and it can take repeated investigations, even by very good endoscopists to find them sometimes. Well, I think also that's one of the reasons in the prior sessions that I think you always should keep in mind the use of a contrast study. Because what may be missed with the bronchoscope could be picked up with a contrast. And so you're saying that after because his first one, he wasn't suspecting it, so he didn't have a reason to do that. The second time when he saw the contrast go into the airway, I'm assuming, Wolf, Wolfgang, that your assumption was, which I would have assumed too, is that maybe it just aspirated and that's why it went down the airway instead of actually a fistula. But if you're pretty convinced it's a fistula. Um, maybe at that point try the methylene blue or other tricks, yeah, that we talked about. So, you know, like everything else, if one thing doesn't work, have a few other things you can do to try to prove what you suspect. Any last minute quick comments, uh, before we move on to the next case for Wolfgang or just a quick, a quick thing, yes. Just, just to say that although that the patient has an open TF repaired the first time, the second procedure wasn't thoragoscopically with no problem. That reminds us always to, even if we have a patient that is referred from outside, uh, that we can do it thodoscopically the second time with no problems. Well, I may answer this. The first repair of the oesophageal atresia, tracheoesophageal fistula was through the right chest, and then the distal cartilaginous ring was through the left chest, so it was virgin territory. But we have, we, I, I've been back into, to chest arthroscopically multiple times that had had previous open procedures. Um, and it's not that difficult, uh, so I, I would agree, and I, I think if you had wanted, you could have approached this through the right chest as well. I didn't believe you. Remember the case we did? I didn't believe you, and you said, trust me, it's not going to be that bad. We went in and it took a minute to move the lung away and we saw a beautiful view of the esophagus. So, so, uh, I was convinced. Any other, uh, comments or questions before we move on to Doctor Reyes's case? What side did you do the, uh, what side of the neck did you fix the H fistula from? I actually didn't do it because I had moved on, but they did it through the right side. You ran away. Does anybody do it through the left side? In the faculty. Yeah, right side, OK. I agree. I'm just curious. OK, um, great case. Well, thanks for, uh, sending that in, and we appreciate you always sending us cases because your cases are always a very good ones. So thanks for sending that in very much. All right. Uh, Cynthia, are you there? I'm here. Can you hear me? Yes, welcome. Thank you for joining us and presenting your case to us. Uh, sorry, sorry, go ahead. Um, I, I'd just like to thank you and the faculty for letting us, um, present this interesting case from the University of New Mexico Children's Hospital. This, uh, presentation is about a 2.1 kg female infant that was delivered at 35 weeks' gestation at a local level two neonatal intensive care unit. Um, at 7 hours of age, she developed respiratory distress and was intubated. Um, about, uh, 24 hours later she was extubated and a sepsis workup was performed, and they did not find an infectious etiology for her respiratory, uh, problems. On the 3rd day of life, uh, they started to feed her and they noticed she was choking with feeds. So a barium swallow was performed by an adult radiologist at their institution. Baring was given to the infant through a bottle, and a proximal tracheoesophageal H type fistula was immediately evident. For unclear reasons, uh, the study was continued and the infant was allowed to drink. And drink And drink barium to the point that she filled her lungs. At this point, uh, she, uh, was transferred to our institution in mild respiratory distress, so she was not intubated and had fairly good oxygen saturations with minimal nasal prong cannula, uh, supplementation. We treated her with IV antibiotics and aggressive pulmonary toilet for this aspiration pneumonia. She was supported with trans pyloric feeds until she was taken to the operating room on day of live 12 for repair of an H-type TEF. A rigid bronchoscopy revealed a fish mouth tracheoesophageal fistula in the cervical trachea. The trachea distal to the TEF appeared normal. A guide wire was passed through the TEF and, uh, through the esophagus and into the stomach, and we confirmed the position of the guide wire with fluoroscopy. A low right cervical incision was created to expose the trachea and esophagus. Separation of the trachea from the esophagus, however, was difficult. During this maneuver, her oxygen saturation felled to approximately 60%, and we were not able to regain normal oxygenation until the endotracheal tube was advanced into the right main stem bronchus. A space between the esophagus and trachea was ultimately found at the level of the thoracic inlets, and concern for a laryngeal tracheoesophageal cleft prompted a repeat bronchoscopy. Uh, this bronchoscopy revealed a laryngotracheoesophageal cleft that extended to about 1.5 to 2 centimeters, um, above the carina. Interestingly, we had invited the pediatric otolaryngologist to assist us with the initial bronchoscopy because he was such a small infant, but unfortunately he was unable to attend at the last minute. And the other two otolaryngologists were also unavailable. So my two partners and 3 anesthesiologists and I huddled to determine our next move. Let me stop you, and that's what we might like to see. What would you do? OK, what would you do? Great, this is great. And uh Patricio, you know I'm going to call on you for this. So, uh, Patricio, tell me what would you do in this situation, and then. Uh, we'll talk to everyone else to see what everyone else who doesn't work on airways would do, but, but Patricia, what would you do in this situation? Uh yes, in this situation, OK, this is a grade 4 laryngotroesophageal fistula. Are you agree with that? I think it's, I think it's, I think it's a grade 3A, maybe a 3B. It did not extend onto the bronchus, and we had about it extended in the thorax. Yes, it went into the thorax. Yes, inlet, yeah, that's the difference between 3 and 4. But anyway, it's a long, it's a long cleft. Uh, if you, if you are not able to repair. Um, Uh, with a, with a good oxy oxygenation, uh, the best way for me would be to do the repair and bypass. That, that would be my way. OK. So would anyone have a different option? So, uh, you would do a, an, an immediate repair at that time, potentially on bypass. Yeah, yeah, yeah, if, if it's type 13, I will do it through the neck without bypass, but uh if it's the 4 type lung, I will do on bypass. I would say that this is, this is a 3, a 3, not this is a 3. OK, if it's 3, I will do without bypass. I think each of you has to define what you mean by 3 and 4. I'm reading this as what I would call a 4 because it goes all the way down to the trachea almost to the carina, uh, and, and doesn't seem to go onto the right main stem bronchus. So that's, you know, that's, that has, that's an emergency situation in a newborn. Uh, where did, Cynthia, where did this thing actually stop? This thing stopped about 2 centimeters above the carina, and I think this infant had a foreshortened trachea because I was, I was able, we were able to reach the carina through the cervical incision. So, um, it, it's possible it's a, it's a 4, but my understanding, um, um, is that, and there are several classifications, and I guess the classification that I'm using is, uh, when the cleft extends to the trachea and, and, and even extends onto the one of the bronchi. So in one classification this would be either a 3A or a 3B since it didn't quite reach the carina. So what would you do in this situation? 2 centimeters above the carina, then you've got to treat it as an emergency. It's a cleft that has to be closed. The kid's going to die. And I think in most cases it's probably safer to do something like this on bypass. I don't do that, but that's probably what, but these have been done. Uh, not on bypass, uh, but the, uh, the, the survival with this is very, very low, even with, uh, an adequate repair. All right, so Cynthia, go ahead and tell us, well, does anyone have any, uh. We have anyone have any more comments? Professor B. Yeah. Uh, now, we, we met a similar case two years ago in, uh, Type 3 of geriaresia, uh, and the baby was, uh, was, the diagnosis was made, uh, preoperatively thanks to the bronchoscopy. So all surgery was stopped. The informed consent was recalled, and the day after, The baby was operated on to repair the type 3 laryngoesophageal cleft using a flap of the upper esophagus to close the posterior aspect of the trachea, and only after 10 days the the the the esophagus was repaired by a typical transthoracotomic right approach, so. I, I, I think that in this case, if the diagnosis as, you know, has been made in the preoperative period, probably it would be more easy. To approach it and once again I think that the endoscopic preoperative assessment is absolutely necessary nowadays in this difficult also if we believe that it's a typical type 3. Yeah, I mean, I, and then, and Cynthia, I want you to continue here, but I want, you know, the question here is we have a situation where it's something that we're not accustomed to treating, uh, unless you're someone who does these, who does a lot of complex airway management, and how do we do, how do we get out? How do we, uh, do damage control and get out, is that right, Cynthia? That would be my, that's correct. We were, we were between um. 3 ollalarenga just over the phone, 3 pediatric surgeons and 4 pediatric anesthesiologists, we had the cumulative experience of one case. So, um, so what we did was get on the internet. And uh, thank God for um internet access in the operating room. Um, um, we were able to, um, find some literature that suggested, um, uh, A conversion of a type 3 laryngeal tracheoesophageal fistula cleft to a type 2 cleft, exactly how was described earlier by repairing the cleft with an esophageal flap. This would allow the trachea to accommodate the the tracheal tube safely, and then after an adequate amount of time we could transfer the child out of state to an institution. With more experience in this lesion than we have. And why I'm showing this illustration of the classification of laryngeal laryngotracheoesophageal clefts shown by Dr. printed by Dr. Deboer in 1990, and I don't know if my pointer, can you, is my pointer showing on your screen here, if you, yeah, there you go. Yeah, OK, so, um, basically the. What I would say is, is, let me see if I can grab that click and push, yeah, there you go, got it. midway between what we're seeing between a 3 and a 4, our lesion was about right, right here. So that's why I hesitate. I called it either 3A or 3B because it wasn't quite a 4. It didn't quite reach the carina as described in this paper. OK. Yeah, I agree with Cynthia, but there are many new classifications, so but it's at the end of the day it's almost the same. So, unfortunately this was intended to show an esophageal flap repair and it and it just doesn't project well, but we performed an esophageal flap. We left the infant intubated for several weeks. Cynthia, I'm gonna see if we can in a, in a few seconds try to recreate a picture because I want you to explain that specifically, but keep going. I'm just to let you know we're trying to work on getting a better picture of that. OK. All right, so she was, uh, so, um, she did, she developed, um. She had positive pseudomonas cultures in her trachea. Fortunately, she did not develop a pseudomonas pneumonia. We were very, very nervous that our repair was not going to heal, um, but we, we got our nerve up and on post-op. 23, she was transferred to Primary Children's Medical Center in Salt Lake City with a pediatric anesthesiologist at her bedside. They endoscoped her and found that our repair was intact, and we were very, very happy about that. And ultimately they performed an endoscopic repair of a type 2 laryngotracheoesophageal cleft. Her vocal, she had a transient vocal cord dysfunction, but a tracheostomy was required because of her significant tracheomalacia. They performed a fundal placation and a gastrostomy tube for feet and to prevent reflux, um, and she's done well, very well at home. Um, a couple of weeks ago she was admitted for an aspiration, um, pneumonia after emesis. She was endoscoped and they did not detect a recurrent fistula, and she's scheduled next week to have. An outpatient sine esophagram to see how she swallows. So I see our slide is up. So basically what our flap actually would start here. Our fistula did not extend this far. However, we basically performed the same thing. We took an esophageal flap and then wrapped it. over the defect in the trachea and did a linear anastomosis with interrupted, I believe it was 5 OPDS sutures, and then closed the esophagus, left some drains, and, and this repair was, was performed up to the level of the cricoid cartilage. So you just do a, then you have this long hole in the esophagus. You just do a uh running or interrupted sutures of the to sort of bring it in a longitudinal fashion. Yes, we just close it in a longitudinal fashion and uh uh subsequent esophagrams I'm told by the. The, um, the surgeons in, in Utah, look, looks very good, the esophagus looks good, slightly narrow, but, um, not bad, and they're going to repeat a study, uh, next week to assess that further, assess the esophagus further. Has she got, has she got a, a, a long term tracheostomy tube and now she home, yes, yes, she's on, uh, she has a tracheostomy tube and a gastrostomy tube. And what, what's the degree of her tracheomalacia now? They say Dr. Smith thinks it's fairly significant. He doesn't think that her tracheostomy tube will be removed till she's about 2 or 3 years of age, because the esophageal flap tends to collapse into the tracheal lumen, and then you have tracheomalacia collapsing anteriorly, and it's, uh, you know, it's, that's one of the things you have to deal with at this point that is almost impossible to deal with, really. Yeah, you know, years ago, they might have considered putting a stent in there at some time, but those are, you know, those are very risky in terms of erosion and things. Well, uh, Pato, or does anyone here have any last comments about this case? Uh, Doctor Spitz, is that you raising your hand, or yeah? No, no, I'm not raising my hand. I'm only, only to say, uh, I will come in now and say that, uh, recently they've done a tracheal transplant at Great Ormond Street, which has been very successful. Hm. OK. And I'd like, I'd like to share some of the, the things that I've learned from this, from this case, from, from our, um, pediatric laryngologist. Um, first, if, if one sees rapid spillover of trachea of contrast into the trachea with less than 1 millimeter of contrast, um, one should be suspicious for a laryngotracheoesophageal cleft. um. And at the time of the bronchoscopy, uh, one should try to separate the posterior commissure of the vocal cords to identify a laryngotracheoesophageal cleft, um, particularly in a case like ours when the folds have not just separated. Um, it's a rare event that this occurs, but I would, we bronchoscope all of our kids with oesophageal resia and tracheoesophageal fistula, but I never, I did not learn to do this technique, and I will do this in the future. And, and last, there are 3 approaches. The cervical approach is what's most familiar to the pediatric surgery for repair. But then, uh, there's, uh, there's a risk of, uh, injury to the, uh, recurrent laryngeal nerve, and then there's an anterior transtracheal approach, um, that basically is done through a midline incision of the trachea, and that spares the recurrent laryngeal nerve, so that's good. But the ENT surgeons are now using an endoscopic approach, and they, and they've been able to repair this lesion down to the cervical trachea, basically 2 to 3 centimeters down to the cervical trachea, and anything distal to that level would require a thoracic approach. So I really learned a lot from this case and And I, I thank you for letting us share this experience with you. Well, I, I thank you because I certainly learned a lot. Um, this was, it gives you a little, uh, reality check that we got to always be prepared for something we're not expecting, and, uh, this was, uh, I think I've been convinced as the day goes on to start doing routine bronchoscopy and uh. I think that your point about the huge amount of contrast that goes through is really important. I mean, everyone here said, Whoa, and to think, yeah, that's probably why we're not used to seeing so much is because there's a big cleft, not an H type. So that taught me that was confusing. That was the problem that was confusing to us. Because the study we were operating on a study that wasn't performed in our institution and clearly the child was allowed to drink way too much contrast. They, they, I, I suspect that they were trying to see the stomach because the stomach was full. They had images that was full of barium, but had this occurred in our, in our hands, you know, being done with a slight trickling of contrast into the esophagus, perhaps we would have. been suspicious for this a bit sooner, but you know, be it as it may, we're very, very blessed and thankful that we were able to get this baby. The child was lucky you were there to take care of her because you did a great job. Thank you for that case. That was very helpful. Uh, we're gonna move on to the next case, and, uh, I don't know, Mathias, we're gonna do your case last because it has a video and I don't want to kill everyone's camera, um, for that. So what we're gonna do is we're gonna do, uh, uh, Amber Shata and Sarah Rasmussen's case from the University of Virginia. Amber, can you hear me? Hello. Yes, can you hear me? Yes, OK, perfect. So Amber, uh, welcome, and, uh, we're gonna go ahead and put your talk up. And the title is Long Gap esophageal atresia in an infant with Charge Association and Tetrology of Flo. Um, we're gonna get that pulled up in just one second here. Give us a minute. Come in. OK, speaking in advance. OK, and then we're gonna give you access in a second, so you'll see 2 arrows on the bottom there to control your slides in a minute. Great. So this infant was born at 36 weeks. Again, similar to the first case that was presented today. She, uh, was very unstable at birth and was, um, intubated in the delivery room and brought to the NICU. Um, Prenatally, she'd been diagnosed with congenital heart disease and uh they were unable to pass the NG tube uh day of life one and um we were called at that time. The echo also done day of life one demonstrated tetrology of flow, absent pulmonary arteries, and a right-sided aortic arch, and also um possible in nominate vein duplication on the initial echo. Day of life 1 and into 2, she became increasingly unstable on the oscillator and declined and was brought emergently for ligation. I just did a primary ligation, did not repair the fistula at that time. She was too unstable to do so. Uh, we approached the ligation, uh, via right thoracotomy and did go retropleurally and, um, had no trouble with the aorta, um, with that approach, but did, uh, find the fistula. Off of the left mainstem bronchus rather than off of the trachea. Can I stop you there, Amber, and then we're gonna continue on with your case in a minute. I wanna talk about this cause it's been, there's been questions from the audience and this is a common question that I want to address. So, straightforward, uh, we keep calling these as, as, uh, uh, John Folker calls it, yeah, we keep the quote unquote straightforward type C, but, um, Steve, I'll start with you, uh, right-sided arch. How do you approach it? You know, I think you can approach it from either side, but I, I've done 4 of these now thoracoscopically through the left chest, and it works fine. You can see it fine and the aorta is out of your way, so. Left chest. OK, Doctor. I, I think it's easier through the left chest. It's been done and there are papers that are support either way. It's been done through the right chest and left chest. I think it's easier because you haven't got the aortic arch in the way. OK, Jack. Oh, is that, Yeah, Langer, uh, let's, let's do Fokker first and then I'll do Folker first and then Langer. Anyway, through the left chest, why make it hard? The baby's depending on this anastomosis. OK, Jack Langer. Yeah, I mean, if I know it's a right-sided arch, I go in the left chest, but we've had somewhere where the echo was wrong and you go on the right side and there's the arch, and, and you can fix it quite satisfactorily on the right side, even with the arch there. OK, yeah, uh, Holger. I've just been in that same situation recently, went through the right chest because I saw that publication, and every time I tried to retract the lung, uh, the heart responded with decreased capacity. It's called bradycardia. Well, OK, thanks, John. By the way, anyway, um, we had to abort the situation and turn the chairs over and come from the left chest, which technically was just easy. Um, a lesson I learned. OK, Miguel, have you ever had that, Miguel? Definitely, yeah, from the left, it's much easier steroscopically, mainly I think. So from the left, Dr. Spitz. Uh, I think if you diagnose the right aortic art, you should go from the left, but it may be just as difficult on the left as it is on the right, uh, Professor Bagalo. Uh, probably in emergency, I would have, uh, chosen the, the right side, but, uh, once again, um, after a bronchoscopy in emergency, probably I would have seen, uh, the, the fistula in the left bronchus. So, uh, in the right side, probably in emergency radiation. OK. So, Amber, continue with your case. OK, just briefly, um, bronchoscopy was not an option given the instability of the child being on the oscillator, and we were hoping to save the left chest, uh, for future repair, and we had no plan to repair at this time. Um, we also did place a gastrostomy tube at this original, uh, surgery, and the infant did quite well. She, as I mentioned before, had tetrology of flow that was affiliated with a uh PDA stent, um, done by our, uh, cardiology colleagues. And we spent a long time after that planning her ultimate reconstruction. She um did have a gap study preoperatively that showed uh the fistula. Um, being what we consider to be intermediate, got 2.5 centimeters, uh, on maximal traction of both dilators, so we were concerned about primary repair, and you think, um, you can see, uh. I don't know how to give you a pointer here, uh, if you click that pointer at the top and then drag it with your mouse, you, you have to push while you drag. Yep, thank you. You can see here that there are clips, uh, from the previous ligation here, and we have our coronary dilator right up to the clips. Um, so we feel like we are in a decent position in the, um, distal. Um, esophagus. Uh, so she had several months of relative instability, a lot of episodes of supraventricular tachycardia, an aspiration incident, and some central vein thrombosis that delayed her repair. She also had a right-sided chylothorax, um. That uh came up postoperative day 47, after her initial ligation, and we felt that that was probably too far out to be a postoperative, um, pylothorax, but instead, um. Was a result of her central thrombosis. Um, we also were trying to kind of, um, repair her as early as we needed, given she had a the PDA stent that was not gonna last forever, and we wanted to have her esophagus dealt with, uh, prior to her, uh, tetrology repair. And this is also preferred by the cardiac surgery. Um, eight weeks after her original ligation, we repeated her gap study and her gap was unchanged, uh, still 2.5 centimeters, and. As you can see here, the proximal pouch maybe was a little bit left of midline, um. And we felt that if we did a repeat right thoracotomy at that time, that that may make things more complicated. Also remember the official came off the left main stem bronchus. So we considered several options. A primary repair via left thoracotomy, um, the 2.5 centimeter gap should be, uh, amenable primarily, but we also thought about a staged repair, um, as well as an esophageal diversion. Um, she did have, uh, charge association that, um, we were worried would. Um, we were not sure what her neurologic outcome would be, and her swallow was poor at best. It was hard to tell, uh, whether she was showing any signs of swallowing at all. Let me, so let me stop you. So, Doctor Corn. I would attempt a primary repair through a left thoracotomy, Steve. Primary left thoracoscopy. Primary left thoracoscopy. Primary left thoracotomy. Doctor Folker. Yes, what would you do next? I'm sorry, we're, we're, we're having microphone problems. I, so I've missed a little bit of this. I think I better pass. OK. All right, um, it looks like Dr. Langer's. No, I'm here. OK. What do you do? We have a few more people here. I think I would go for option one. I'd try primary repair. I'd probably go through a thoracotomy in this kid. OK, Holger. Keep it simple and safe.Rootomy. Sorry, Miguel, I agree with Hoger. OK, Doctor Spitz. I would also go for a primary repair if possible, but this is the one situation I would not use a gastric transposition if I had to replace the esophagus because the child will have swallowing problems throughout its life, and I think under those circumstances, it's better to have a stomach in the abdomen where you can feed through the gastrostomy. So I would do a colon if a replacement was required. Do you want to respond to that, Doctor Corn? Um, You're speechless. I think you're going to be able to do a primary repair. I wasn't even, I didn't even have a, a replacement in the back of my head at this point. Uh, but that's, that's a point. Uh, but you could, uh, if you had to use a feeding, uh, axis in a child like this, you could still do a gastric transposition and then do a feeding ostomy. Doctor B. I would have attempted probably a primary repair as soon as possible via a left thoracotomic approach. OK. Um, all right, so, Amber, what did you do? So we did a primary repair by a left burotomy um on day of life 75. And uh the baby did great, she um had a swallow study that was delayed somewhat by some respiratory difficulties post-op, but ultimately on postoperative day 16, uh, had a swallow study with no leak, however, we were unable to feed her initially because of her residual chylothorax. And so she was managed conservatively with TPN, and that resolved 37 days after her formal repair. That complication that she had and the last complication she had was uh SVC syndrome um requiring thrombolytics, but that ultimately also resolved. And she do the And she's uh due to be discharged home next week. Great case. OK. Go ahead. Did you wanna say something about that slide or that's just for? Oh, I mean this was published 20 years old. This, uh, the previous series that we have looking at oesophageal atresia and CHARGE syndrome is, as you can see, very dated, uh, but had a 70% mortality. We were very trepidatious and trying to consider um what. It would be best for this child and this family. This family is, uh, on their 3rd miscarriage and desperately wanted this child, uh, to survive. The primary and this paper is the primary reason why cervical esophagoscopy was even brought up in this case. We wanted something that was. Any, uh, any last comments from either Amber or from the, uh, faculty? No, OK, Amber, great, thank you, uh, Sarah, thank you both for that case. Uh, fantastic job, and that brings up a lot of great issues, and I appreciate you presenting that. Thanks, thank you. We're gonna move on, uh, to the next case. Uh, Doctor Brizzoni, can you hear me? Yes, hello there. Hello. And then Mark, can we pull up his uh video or we're gonna have to close down the cameras. We're gonna, um, have to close down cameras. We'll ask you guys to open them up at the end. Is there any way I could pause the video from here, or, or should we have Mark pause it? Mark, just ask Mark to pause it whenever you want him to, yep. Sounds good. Well, thanks so much for letting us present it. I'm here with uh James Wall. He's our fellow. He's also part of the operation, um, great symposium. We're sure we learned a lot. So thank you very much. Thank you. Um, let's see if we can get the video. I, I'm not seeing it. Are you guys seeing it? Uh, they're pulling it up right now. months We can, uh, why don't you do that? Yeah, go ahead and we're gonna shut your camera down just to make it bigger. There you go. And, uh. Why don't you hit. Actually, it looks like I, looks like I can pause it from here, huh? No. Now Marco control for you there. OK, so if you, if you can just back up and start it over, that, that'd be great, thanks. Uh, so we did a, um, thogoscopic treatment of, of, uh, both, uh, a proximal and distal tricheesophageal fistula, um, with esophageal atresia, and this was a baby, a premature baby, 35 weeker who also had CHARGE syndrome. He had complete choanal atresia. Uh, he was 2 kg, and he was born in, in respiratory distress, so he was intubated. Um. Go ahead. Let's see right here. I'll tell him to, I'll tell him to pause and stop, so, OK, keep going. If you can pause it here, that'd be great. OK, pause. A few key points of this video is that. You know, we'll show the x-ray, uh, right after this, this slide, but it was pretty straightforward, uh, type C trichoesophageal fistula as far as we could tell on the X-ray, um. The kid, this baby had choanal atresia, was in respiratory distress, so we, we didn't feel like extubating him in the operating room, doing the rigid bronchoscopy and intubating him again, so we, we gave the, the, um, the flexible bronchoscopy a try, and, uh, we went through the ET tube and showed the distal fistula, and then we started backing up the ET tube as far as we could and we couldn't see a proximal fistula. Um, then we went ahead and did the operation thoragoscopically through the right chest, and, um, after ligating the distal fistula, we still had issues ventilating, uh, the patient, so we repeated the bronchoscopy intraoperatively and finally we, we found a small proximal, uh, trichoesophageal fistula. And this actually was an interesting one, just by looking at the classification. Um, and it'd be interesting to see what the rest of the faculty have to say, but it wasn't a side to side. It was an H type instead of an N to side on the proximal pouch. Uh, we can go back to the video, Mark. Thank you. All right, uh, Stefan, you can hit play. So this is the x-ray you can see distal tear and the orograph orogastric. Hello Matthias. Can you hear me now? Can you hear me? Uh, we can hear you now. All right, so I, I was just saying that we ligated the distal fistula with, with a vicral suture as you can see there on the video. Um, and right after ligating it, you know, we're still struggling with tidal volumes. So then we repeated the bronchoscopy and then you can see it on the right side of the, the screen that there's a a small pit uh describing the proximal tracheoesophageal fistula, and uh we, we got lucky because we were able to advance the the uh bronchoscope through it into the upper pouch just to confirm we're in the right place. And then we turned back to our thoracoscopic view and turn the lights off. And and you can see how the pouch lights up and then we're gonna turn the light back on, and you're gonna see the the bronchoscope inside the upper pouch. But as you can see, it, the, the, the end of the pouch is free, but this goes side to side from the trachea. So then we went ahead and dissected the upper pouch. You know, we always thought it was just the common wall they share, but this time we did have a fistula, and it was a really short track, so we ligated it the same way we ligated the distal fistula, but we felt uncomfortable ligating it and cutting in between. So we just cut. On the esophageal side and and uh then proceeded to repair that primarily uh with a few interrupted stitches. As you can see now, now in the video. We put some traction with a wet rubber catheter that helped a lot with exposure. We just put 3 stitches and fixed the side hole and uh the anastomosis had very little tension and and we just did it with interrupted uh vicral sutures on a TS needle um. Through um anastomotic, uh, tube and, um, and completed it. I think towards the end you'll see the two fistulas that are ligated on the bottom part of the screen and then the anastomosis with the site repair of the of the esophagus. This, the postoperative esophagram, which looked good, and eventually we did a rigid bronchoscopy when the ENT took him back for his coal atresia repair, and, uh, and we saw that amazingly there was no tracheomalacia, that the repair looked good, and, um, unfortunately, you know, she has a very poor swallowing, uh, reflex and coordination, so we ended up having to do a Nissan and G tube, um. About 2 weeks postoperatively, so maybe more, a little bit 3, like 3 or 4 weeks post-operatively. It's an interesting case. Um, I mean, I think you handled it pretty well. Would you have, would anyone have done anything different? I mean, it sounds like things, I think there's two points I learned from this is, uh, and it's been verified today already, is you can get an H fistula, congenital one through the chest, uh, if you know it's there, uh, and it's very commonly missed when it's a second fistula in an esophageal atresia, and that's. Uh, the paper from, I think it was, uh, Holland a couple of years ago, I think Klaus Bach and his group at Utrecht, uh, showed that the incidence of a, uh, uh, proximal fistula in patients with, uh, pure esophage atresia is extremely high, much higher than, uh, we thought in the past. Do you think this would have been in the same location as a pure H type? Looks like a little lower. Yeah, I, I can't tell because you can do those obviously through the neck or through the chest, and they mobilize that esophagus, so it might have looked like it was lower. I mean, Steve, you've done these thoracoscopically. Is that what it is that pretty much they're usually right at the thoracic inlet, which I think if you're doing an open thoracotomy is extremely difficult to get at. So, you know, I, if I was doing it open, I would always approach it through the neck because I, I, when I was in training. We went at one through an open thoracotomy and couldn't reach it and had to close the chest and go back up through the neck because my attending thought that it was, it was lower and we would get through the chest, but the advantage thochoscopically is that you can work up in that space. The problem is, and they did a beautiful job, is that it gets very narrow up there and so to be able to. Suture up in that angle, especially in a newborn, is tough. No one should think that's easy. That's probably the toughest place you'll ever throw a suture is up in the apex. Most of the H types we've done have been the kids are a little bit bigger, so you have a little more room to work, but that looked very good. But I think it certainly can be done and you can see it extremely well. The advantage I think of doing an H-type. Thochoscopically is that I think it's easier to protect the nerve because you're not getting around the esophagus. I mean, at least the way I was trained, you know, you get around the fistula, you're doing a lot of. Manipulation in that tracheoesophageal groove and when you do the thochoscopically, you're doing it all under direct vision. Um, what's the, what's the experience in the panel with flexible, uh, bronchoscopy instead of rigid bronchoscopy? It's just so much easier when you have an intubated patient. A flexible bronchoscopy through an endotracheal tube, you're gonna miss proximal fistulas because uh the tube is in too far for you to see it. Yeah, but you know, we, we withdrew the tube as we, you know, under direct vision with a bronchoscope, and we eventually saw it because we were really looking for it. The first time I put the scope in, I withdrew the endotracheal tube and tried to go as high as possible, but of course I missed it initially. But then the fact that the baby wasn't recovering after the ligation, then that made us look a little bit in more detail. But uh um I guess the learning point for us is like, you know, a rigid bronchoscopy would have shown it right away, I think. I think in general, you see more with the rigid, uh, Stort's bronchoscope than you do with a flexible scope, whatever you're looking for. I think it's a better examination. So, uh, we, we, let me tell you what's going on now. So now we have come to the end of our time and, uh, but we're not done. Steve, uh, has a flight to catch, uh, and so, um, he is going to leave our panel, which is great because we now can talk about him when he's not here. So, uh, hold your comments till after he's out of the studio and then we can talk about reality. Thanks everybody. Actually great, great seminar. Thanks, Todd. Thank you for, uh, joining us as always. You're, uh, yeah, you, uh, give us a nice angle on, on how we should be doing things in the future and, and, um, you. It looks like you already got another case in, uh, Virginia now, so, um. Thank you very much and have a safe flight and I'll talk to you when you get back from Denver. We have, uh, we actually were done, but we do have one more case that was sent to us and then we'll be finished in about 5 minutes, um, but uh we have a case from Saudi Arabia being presented by Dr. Paul. Um, and we're getting him on the phone in a minute. He's done. Uh, OK. Uh, I don't see his, uh, talk though, Mark. OK. Doctor Paul, are you there? Uh, we're getting him on the phone in a minute. Hello. Can you hear me? Yes, we can hear you. 2. Yeah, and go ahead and, and, uh, I can advance your slides for you. OK, so, uh, we, uh, we made these slides for you. The title is Long gap esophageal resia and tracheoesophageal fistula with a right esophageal lung in a preterm, uh, small for gestational age baby, a surgical Challenge. Yeah, that's right. So, uh, would you like, if you'd like, I can read the slides for you. Or would you like. OK, so it seemed, uh, this was a baby that was referred to the, the NICU as a 6 day old male, 29 weeks preterm. He weighed about 1 kg. He had a diagnosis of esophageal atresia and a distal tracheoesophageal fistula. This was from another hospital. The pediatric surgery evaluation confirmed the diagnosis and revealed a collapsed right lung. 13 pairs of ribs. He had a patent foramal valley and an ASD suggestive of the Vactral Association. He had a, uh, you guys did a right thoracotomy, uh, and division of the fistula and a modified VY flap, end to end primary esophageal anastomosis was performed for a long gap. There was a 3 centimeter gap. Post-operatively, uh, he was managed in the nursery with elective paralytic agents, sedation, and ventilation due to the long gap, which is an interesting thing to discuss. Do you need, is there a benefit of paralytics and sedation in that situation? Do you, do you do that for long gaps? Do you, uh, do paralytics? Uh, not, not, not before we fix them, but, uh. After you, after we fix them, we would, yeah, if there was a lot of tension. OK. Uh, postoperative contrast study revealed a patent esophageal anastomosis without any leak. However, the right. I'm missing, oh, here it is. OK. Yeah. The right lung remained collapsed. Uh, bedside flexible and bronchoscopy revealed one patent ostium on the left suggestive of a left main bronchus and another collapsing ostium with evidence of sutures suggestive of what they were seeing was the ligated fistula fistula. On the right side, absence of a definite patent ostium for the right main bronchus and distortion of the carina created suspicion of the anatomy of a tracheal bronchial tree. A high resolution CT was performed. Um, there was no aberrant vascular ring, but there was an air bronchogram, and I think we have a picture of that coming up, right, Mark? What's coming up in the next slide. Um, from the collapsed right lung down towards the lower part of the esophagus, almost near the diaphragm level. The patient underwent an exploratory right thoracotomy, which revealed an intact oesophageal anastomosis, a patent left main bronchus, and an absent carina or right main bronchus. The exploration of the right lung hylum revealed a thick cartilaginous tubular structure suggestive of the right main bronchus. Traversing almost at the supra diaphragmatic level towards the lower esophagus. The right lung was unilobar without any fissures and was of liver-like consistency. The lung had a pulmonary artery and vein, uh, pulmonary artery and vein supply which clearly ruled out any extra lobar sequestration. He's on the phone. OK, Doctor Paul, are you there? Yeah, OK. So you, uh, you basically, do you want to continue here? I would like all the and then probably we would like to have the expert opinion on this. OK, um, we're having a hard time hearing, but I think you said you want to get expert opinion on this, but this, um, the subsequent literature review revealed about an extremely rare clinical entity where the main stem bronchus connects directly to the lower esophagus called an esophageal lung. Have you heard of that? OK, akin to our case, most of these are associated with esophageal atresia. Only 20 cases have been previously reported so far in the world literature. Diagnosis of such cases are extremely intriguing, and management involves pneumonectomy. The baby's plan for a right pneumonectomy. So these are the pictures. Um, this is the collapsed right lung when the baby was born. Uh, they, they confirmed the diagnosis with uh upper pouch, um, contrast. Tracheal deviation to the right. This is pre-op, postoperative, right thoracotomy and primary esophageal anastomosis, and that's the contrast study which looks good. But on the one on the right, um, the contrast study shows a patent esophageal anastomosis, but faint bronchogram onto the right lower eso from the right lower esophagus. And I'm having, if you look, it's kind of hard to see because the image is so small, but on that image, you can see in the chest, there is a, I don't know if it's possible to blow that up, Mark, that picture on all the way to the right, but that is, you can see that there's contrast in the right chest there, uh bronchograms. Um. So, here, it's, this is going to be too hard to see because the pictures are a little too small, but, um, all right, but I can't read those because my eyes aren't good enough, so, um. Here, I can read that. Right esophageal lung, right bronchus connected to the distal esophagus, um, and query the sub diaphragmatic cysted malformation. So basically this is what I want to get to. This is the right bronchus. Um, there's the lower esophagus, the right bronchus. In the right lung there. This is crazy anatomy. You've heard of this before, huh? I had one. You've had one too. Talk to us about it. We, it's, in fact, it's published in the Journal of Pediatric Surgery several years ago. One of my fellows at that time, uh, uh, Hernandez, uh, uh, wrote up the paper with one of the medical students. This was a, a, this was twins. One twin was born with esophage atresia and a right esophageal bronchus. And the other twin did not have esophageal atresia, but had a left esophageal bronchus. And in both, uh, we repaired the esophageal atresia, uh, and we did the, uh, uh, resection of, it's a lobectomy, a pneumonectomy, whatever you want to call it, it's one lobe. And then in the other twin who didn't have the esophageal atresia, we electively went and took out that other lobe on the other side. OK. And that, that was reported several years ago. I don't, it's in Journal of Pediatric Surgery, but I don't recall the exact date. Has anyone else seen this before? We have a kid right now, uh, who didn't have exactly this, but had bilateral pulmonary sequestrations, uh, both of which had bronchi that went into the esophagus. Can you hear me, Todd? Yes, Jack, with those, can we go back to the, we can hear you perfectly now. Jack, can we go back to the normal lung in those cases too clinical picture, but congenital heart disease associated with it, which has made things very complicated. No, no, what I'm asking on those two, in that case was their lung, normal lung in the in the chest along with the esophageal lobe. So, so let me try to organize things here, Doctor, uh, Doctor Langer, I'm gonna have your answer in a second, but I want to make sure, Doctor Powell, we can hear you now. We're gonna have you talk in 1 2nd, but Doctor, uh, Corn and Dr. Langer want to finish this discussion. So, Jack, go ahead and answer, uh, Arnie. Yeah, so Arnie's question was whether the child with bilateral sequestrations emptying into the esophagus, whether there was normal lung as well. And the answer is yes, there was normal lung on both sides. But she also had a complex congenital heart disease, which has complicated things as well. This here and the one we had was, uh, the whole lung on the side with an esophageal bronchus was, that was the lung. There was no other lung, normal lung there. Obviously the lung on the other side was normal. And can I ask you a question? Yes, please. Yeah, if we can go to the coronal section, yes, just a slide prior to this. Yeah, and the one after this, OK, uh, yeah, right there, yeah, it's the one I, I've said less 2.2 or 3. Uh, cystic structure which is almost at the supra or we could say supratic or in, which is just close to the junction of the bronchus to the esophagus. My question to the panel, uh, what was their opinions about that particular cystic malformation. And how to approach that. OK, so I think the question is the cystic structure above the diaphragm, and I think I don't see a On this X-ray, something cystic. But I, but is this the area you're talking about, uh, that is, and can we go to the, uh, unfortunately your phone is breaking up and we're having a really hard time hearing you. I'm sorry. I mean, if you want to type your question you can type it and we can ask, ask the faculty, but I think, I think this is the question was what would anyone think of with this, uh, coronal section. I think it's all part of the esophageal lung there. OK. I, I would guess that that's all part of the oesophageal lung. Uh, you really have no real lung in the right chest, and, uh, you need to, you should take out that esophageal lung. you know, resect. Um, may I have a comment? Yes. Uh, I, I, um, I met a case like this, exactly like this last year, and the baby was, uh, Mark, I don't know. Yeah, hold on. Can you hear me? Can you hear me? Yes, go ahead. So, and the baby, uh, had a absolutely a normal lung, not a lung, a lobe. And the same story, exactly the same story, and the baby experienced a very severe postpermonectomy syndrome also before lobectomy, so we had to Insert a skin expander into the right hemihorax to save him, and 15 days after we could perform the right lobectomy. Now the baby is well, but have you ever seen a posterior morectomy syndrome in those babies with with the esophageal lung? That's my question. Uh, are you asking me? I only have experience with that set of twins, and they did not develop that, but in fact, that is a risk. And in fact, I remember a few years ago at the ski meeting in Kutai where Jack Langer presented a patient, older patient, I think Jack, isn't it, who developed that syndrome, and he had to put an expander in to stabilize the mediastinum. The younger the patient, the greater the risk of that happening, and you have to sometimes put something in to stabilize that mediastinum. Thanks. I think that, yeah, so I, I want to, um, we're going to have to conclude here. Um, I want to thank everybody for