Esophageal Atresia - Arnold Coran: Update Course 2014

So, Dr. Korn Yes. like I said, you're I I I know you're at NYU and at Michigan, but to all of us, you're Professor Emeritus of the Earth. You along with Dr. Grossfeld. We have two of the most experienced and wisest pediatric surgeons with us. We look forward to. Well, what usually happens when you get to be our age is uh you do hernias and orchidopexies and uh don't get involved with big complicated cases. My life has taken an opposite turn of doing recurrent, recurrent, recurrent esophageal cases and rectal cases. Uh, what I did is uh if you can put those slides up, I'd like to talk a little bit about uh not the most complicated, but relatively complicated esophageal atresia. If we can get those slides. Yeah, he marked. You see that? Okay. Give us one second here. So, um, Abdallah, you asked a question about We're waiting for the uh Adobe to reboot here. Um, about the, the azygous. Yeah, I guess there was a question. Not VIAIR did it open? Yeah, you can. Is anyone done that here? That's why I asked, because Sakket Kazi had said, why not the Azygous? Oh, okay. So I mean, I've, I've, uh I mean, I can imagine very unique ways of getting there, but that's why I wanted to know. But this is, this I'm telling you, this is a lot simpl It's a pretty easy procedure. And it's it to me, it's it seems everybody gets anxious, but it seems really safe. And someone asked, where I do the uh puncture in the neck. And I do it just like you're doing an IJ. Basically, go where you're going to go for a supraclavicular stick of the IJ. If you tear the you know, you that's very slick and it looked very nice. If you tear the pleura, would you continue having opened up a free space for it to bleed? I would. That would be my concern. I I might put a second port in to hold pressure if I have to, but I would. You would just. I would I would. What do you think the chances are of perforating the right atrium, getting access. In getting access overall, or getting access this way? Getting access that way. I think it's very low, because you know where the tip you I think it's lower than if you're doing it totally blind. You know where the tip of your needle is and you see the guide wire go. And again, once you have once you get that blood back in, I haven't seen one that's clotted. The azygous, it always reconstitutes at the azygous. So, I call it I I I call it right atrial puncture, you know, it's a little dramatic, but it's really SVC at the azygous. So it's yeah, that's what it shrinks me is. I've never done that, but uh probably never will. So it looks like we're back up. Uh, I don't see Mark, but I'm thinking that we're good to go. So, um Okay. Uh can we see that next slide? Yeah do I move it? See if the clicker works, Mark. It may not. We need need Mark to come sit down here. Yeah, the clicker is not catching us. So Dr. Korn, we'll advance your first slide, but I can probably uh Mark, can we get that first slide advanced here? Cool. Okay. Well, it we can't talk about the esophageal atresia without putting this slide up of Cameron Hate, just to remind everybody, uh Cameron Hate was a thoracic surgeon at Michigan and an adult thoracic surgeon. And we all know did the first esophageal atresia repair in 1941 and did manage to do over 300 cases during his career. Next slide. Can we Can we Yep. Okay. Uh these are the things in in uh 2014 in my mind that I think are the major areas of interest when we're dealing with esophageal atresia. I think the most complicated form of esophageal atresia today, the one where we see the most complications is the long gap pure esophageal atresia. Uh the I put down as the second item here that we got to address and that is the H fistula, not a pure H fistula, but the how common is a proximal fistular in a standard esophageal atresia. Can the audience give me a rough idea what they think the incidence is today? Okay, so um Do you do we have Let me ask the panel here. Okay. What do you think the incidence of a proximal fistular is in a child with a standard distal esophageal atresia with a esophageal atresia and a distal fistula? It was published I think. Hey Mark, Mark can we go back to the slide that shows the question? Yeah, thanks. 1% was what uh was published through that Australian series. Very good. 20 years ago. You think you think that's correct? No. No. No. probably higher. What do you think it is? 5%. The audience. I I think it's about anywhere from other reported cases uh from other series anywhere as low as 2% to up to 9% in one series. I think it's probably 10% now and and the best series was the one from Netherlands showing that the incidence is much, much higher than we think. And that's extremely important because uh I don't know that everybody fully evaluates the upper pouch adequately in order to uh uh determine whether there's a fistula there. So I'll ask the panel here, uh how do you evaluate the upper pouch before you go ahead and do a standard esophageal atresia. What do you do to uh rule out an esophageal fistular in the upper pouch? Bronchoscopy. Is it bronchoscopy? Okay. Does anybody use any other techniques? So Mark, can you advance the slide? I or give us the clicker? Do we have it? Hey Arnie, you have the clicker there. Oh, I have it now. Is it the top one? Far right. No, far right. Far right one. Far right. There we go. Okay. Okay, so the incidence is really about 10% and that's the best study as they said is from the Netherlands a few years ago. And you're saying it's 10% in type C or pure atresia? Uh in a in an esophageal atresia with a distal fistula, what do you think the incidence of the? I I think that's high. I I think it's that's way I don't think it's that high. I think it's I think in pure esophageal atresia, the incidence is much higher than we thought. I think you have a pure esophageal atresia, you have to look for the proximal fistula. But I think if you're you're doing a type C and I was just with Ed Kylie for the last five days and we sort of had this discussion, he agrees, the incidence is so low, I think the incidence it's much lower than that in a type C. Well, the numbers that I'm uh looking at are the numbers from the the uh Netherlands study. Uh but the point of the question is really not whether it's 10% or whether it's 5%, it's not 1%. It's much much higher and for that reason, one has to absolutely prove you don't have it. Now, the pure atresia is higher than the standard C type of esophageal atresia. But the key thing is, uh you don't want to miss that upper fistula and I think everybody should do a bronchoscopy. But in addition, I often will get a pouch pogr pouchgram, which everybody thinks is old hat. And if we can get uh one of the next slides, uh does the clicker work or not? Give it to me. I'll see. There you go. Yeah. Yeah, I just want to show a picture here. Uh, of a pouchgram. There it is. And the pouchgram is still I know Mac Hammer will probably critique me for being an old fud and getting that. Uh, but I always did those uh in when I was uh chief of surgery at Michigan uh because it helps you in two ways. One, you can see whether or not you have enough fistula, but two, if you're not able to get cardiology uh evaluation in a timely fashion, you could term determine what side the aortic arch is on with a good gram. So it's a very helpful study and in my own experience, I've seen a fistula missed by an experienced pediatric surgeon doing bronchoscopy that was essenti of eventually picked up with a pouchgram with a repeated uh branc uh bronchoscopy by by myself at the time I did the case. So I think uh you want to be sure you rule out that upper fistula. I think I think you can get clues to that. Hey. Arnie. Yes. May I ask you a question? Benno over here. Yes, Benno. Um, uh what do you think is the sensitivity and specificity of your pouchgram? That's one. Did you miss? Are you sure you always see the fistula? And the second is do do you see some aspiration of your contrast media because we saw in former times, we saw that from from time to time and that was the reason not to do it. Yeah. How would you comment on that? Uh, one, we usually put about a cc and a half, so the risk aspiration is small. The other thing you want to look for on that pouchgram is not just whether or not you see the fistula, but if you don't actually demonstrate the fistula, if the upper pouch is narrower and smaller than you normally see, you should be suspicious that there is a fistula and be more uh uh insistent on trying to prove it's there with either repeating the bronchoscopy, even repeating the pouchgram. And Okay. And so that that's a uh uh that's a good question, but I don't think that's a risk with a small dose of uh water soluble contrast. What was your other question, Benno? That was the two. Those were the two questions. Sensitivity. Did you miss a a fistula by the pouchgram? How are you always sure to find it? Uh, I don't I mean if I see a uh uh fistula with a bronch bronchoscopy, obviously I won't get the pouchgram. So I can't say if I ever missed one. I don't think so. Okay. I was going to say, I think to your point about it being narrowed frequently, not maybe not frequently, because it doesn't happen that often. But I think you can get an idea just on the plain film and typically before we shoot the initial X-ray, we shoot air into the tube in the pouch and if I don't see a big dilated proximal pouch, then I'm very concerned about a proximal. Yeah. That's a very good point and I I think that's that's also helpful, but I'd still probably. Just do a study. Still a study anyhow. Have you ever injected um into the pouch while you're doing the bronchoscopy to see if you can demonstrate a fistula? Well, in under different circumstances, if you're dealing with uh going to the OR to do an H fistula without esophageal atresia, we'll often use what's called the methylene blue study to see if we can get die to go through. Uh, but in a standard esophageal atresia, especially a pure esophageal atresia, you you don't do that. Um, at any rate, um, what I wanted to move on to from uh the upper pouch and the uh fistularies to the issue of long gap esophageal atresia and what the management of that should be today. Uh, I'd like to ask the audiences, if you have a full-term baby born with uh pure esophageal atresia, you have uh uh ruled out an upper pouch fistula and you uh are concerned about what the real gap is. Uh, what do you do to evaluate that uh in terms of uh studies to see whether or not it's a significantly long gap, small gap, and then determine when you decide to go ahead and operate on the baby. With the pre-existing G-tube in place? You put a G-tube in place in the first 24 hours. Yeah. So what we do and Marcus can confirm this is we uh we take them to interventional radiology and uh do contrast studies and also put uh probably a bakes dilator in from the G-tube site. Yeah, when I when I take him to the OR on that first one, I do the G tube, because they all need a G tube. I'd have floral there and do the Bakes dilator up the uh up the lower. Can I ask both you, what do you think the probability is with the Bakes dilator that you get into the lowest segment? So you have to be careful and what you can do is you can take a little spaghetti uh scope and uh you can like a little spaghetti bronchoscope and you hook it up to a little insufflation air to give a few puffs. If you if you really have a hard time finding it, so you can see the esophagus, because I've been fooled. You can push the fundus up. Correct. And be really fooled that you're a lot And you can push that thing way up there and think you have a short gap and then you find out it's a lot harder. We do the same thing with the scope, but that's also one of the reasons we do it in interventional radiology, because you can inject it and see that you're actually in the tuber in the the distal pouch as opposed to in the fundus of the stomach and just pushing it up. Yeah. Yeah. I I think that in fact, probably the times that you end up pushing up the fundus of the stomach, which is like a rubber balloon in a baby all the way up into the left chest, uh as I say, uh you can almost get it up to the kid's nose if you want to when you're pushing and think you have a good a short uh gap is very, very high. To prepare for the gastrot transposition. What's that? Yeah. It's a pre-op stud. Right. Hopefully you won't have to do it with all uh these long gaps. But I think the important thing in my mind is you've got to visualize that lower segment. What I like to do is put the neonatal gastroscope in and when you put it in and see how small that GE junction is, you're amazed that anything would go in blindly. Now, I won't argue that maybe you can blindly get a bakes dilator in, but I think most of the time you can't. The other thing about a contrast study, do you think you see the full extent of the lower segment with a contrast study map? No. No, you don't at all. So putting the scope in in the OR under floro, Yeah. where you drive your scope up up about and then you push down. That is your best way to assess gap. Absolutely. I agree. Now and then then what do you do now? You got a baby that you've measured the gap on. What what gap is reasonable to go ahead and do the? But you knew that. You do that on day one when you put the G tube in? No. Because that's what you're saying. You take the kid to the OR, you try to put a G tube in and with that delicate stomach, you're trying to ram a scope and. Not ram. Well, but it is. I mean, you have a limited access. You have a. And if it's if it's a tiny little baby, I'll wait. But if it's a bigger baby. And to be honest, you don't have to know it that first. Right. Exactly. You know, how you you how many when would you wait to do that study? The the initial, the initial evaluation. I wait at least three weeks. I want the gastronomy site. My experience has been these babies are almost like 2 kilos or smaller. And they got to eat and grow a little bit before I think if you try to put even that little tiny pede neonatal scope in before three weeks, you're going to push the stomach away from the s the gastrostomy site. And as Mack said, you you're not forced to do anything. You're feeding them by gastrostomy anyhow. So now you're at three weeks and you put the scope in and you uh definitely get it into the lowest segment. Uh, you got a little bougie in the upper segment. What is a reasonable gap between the two to make you decide you're going to fix the kid right then and there or in the next day or two. Steve? Well I I mean anything my experience has been, the part of it depends on how big the upper pouch is. I think the bigger the upper pouch is, the more likelihood you have of getting it together. So if I have a big dilated upper pouch, if I'm doing this at at two or three weeks after a study and and the gap is less than um certainly if it's three or less vertebral bodies. So put it together then. Certainly two vertebral bodies is is the classic teaching I think of of something that you're probably going to be able to get together. Jay, what would you do? Yeah. Three vertebral bodies I think is stretching it maybe a little bit literally. Um, my I guess one of the questions you have to ask is about that proximal pouch does it grow by itself or do you have to dilate it? That's always a question that people talk about. Whether you have to do anything if you except just watch and see if it grows and be patient. I don't think you need to rush to do this in the early neonatal period at three weeks. How how patient should you be? Well, I I'd wait 12 weeks to stretch. I I see nothing beside that. Babies grow pretty good with gastronomy feedings in between. Parents learn how to handle the upper pouch at home if they're intelligent parents. You have to discern that of course, if they're not, you have to keep them in the hospital, that's a problem. Let's let's say the uh when you measure the gap, uh it was four vertebral bodies at three weeks. agressive observation. I I still think a little observation wouldn't hurt for another few weeks, but would you do a Foker maneuver? No, I don't use the Foker operation. I I know there are people who think it's a great thing, but and I know they don't get any complications when they write about it. But you know, I've fixed a couple of Fokers and I'm sure everybody in this room has seen a post-op patient who's had a Foker procedure that came and it was a failure will have massive GE reflux and aspirated. I I quite frankly, I don't like the Foker procedure and if those kids followed out 10 and 20 years, their esophageal motility and their esophageal function may not be very good. Worse than the usual esophageal atresia patient. I I don't like the Foker procedure. All right, so so if you don't do a Foker maneuver and you've got four vertebral body gap, let's say at three months, that magic number that we picked to say we're going to try to put a pure treasure together. What what would you do now? Well, our teaching in the in the past was that you shouldn't try to do a replacement until the kid is upright, so they don't reflux, because all the replacements usually reflux. And so either they have to be able to sit frequently or or up walking, somewhere between six and 12 months to do a a replacement procedure. I know it's being done earlier by some and um we we still like uh the colon interposition, but a gastric pull up would be a reasonable alternative. And if you ask our friends in the Netherlands, the jejunal interposition might be the way to go. So let me use that. So I'm sorry. Go ahead. I I would like to and then I'll come back to Dan. Um, Benno uh or or Phillip over in Europe, um, how are you managing these long gaps? I I usually wait three months uh as it was said and I would also with four vertebra, I would try to get an anastomosis. And if not, we will switch to the gastric pull up. At at at three or four months? At three or four months. Yeah. Go ahead and do it then. So do people think I mean, I don't I don't I don't have as much experience as those in the room. After three months, you don't think it's going to grow. If you wait another three months, you don't I mean, does that work or by that time? It's a great question. I don't know I I I's the lore. Yeah I'm done. I'll myself. I I I don't think anybody knows the real answer to that. I think most of us sort of believe that at about three months you should start your load and it's not going to get any bigger. And how does it get bigger? It either stretches or it grows. We don't even know that and that's why we don't know what the Foker maneuver really does. But I think at three months, you sort of have to make a decision. Yeah, we do sequen sequential gap studies and if we don't see it changing anymore, then you're waiting longer and I don't think it's worth doing because you're not getting any change with it. Right. Yeah. I usually do a gap study every three weeks after we've done that initial one at three weeks uh since the gastronomy site. Okay, so now you got this not a three vertebral body gap one, but you got the four vertebral body gap that unfortunately didn't cooperate with you and uh and get lesser during that period of time. Are you going to go operate on the kid at that time? Yeah, we reported our the first three cases of reverse gastric tube without spit fistula between three and six months and I've done that three or four times and followed those kids. I think if you go longer than six months, you should probably put a spit fishlin because the child will never eat. If you go too long with waiting on the uh proximal pouch to grow. That's that's been a pretty good experience for us.