Welcome to another podcast. I'm your host. I'm Goddy, research fellow at Cincinnati Children's Hospital Medical Center. Along with Stay Current, we're sharing knowledge to improve child health around the globe. This time we're talking about oesophageal atresia, challenges and techniques for reconnection and replacement methods when conservation isn't possible with Doctor Danvon Alman. He's the surgeon in chief at Cincinnati Children's Hospital Medical Center. Let's jump right in. When we talk about oesophageal atresia and tracheoesophageal fistula, you often emphasize the importance of using the correct terms. Can we start today's episode on that? We have a tendency to say TEF when we mean esophageal atresia, so just be very careful when you're talking about these things that you're clear about what you're talking about, because when you say, oh, the patient had a TEF when really. They had is a pure esophageal atresia. That's just not correct. Yeah, that's a great point. Now let's talk about a little bit of the epidemiology of esophageal atresia. It's 1 in 4500 in the United States. There's a slight male predominance. Chromosomal anomalies are fairly common, and we'll talk about. Vacterol, which is listed there, which is not a chromosomal anomaly really, and then the recurrence risk is about 0.5 to 2% among parents who have one affected child. And how effective is prenatal ultrasound in detecting esophageal atresia? Prenatal ultrasound only detects about half of these patients, and this is a little bit old data, so it may be better than that now. But the ones you detect are primarily the ones that have a pure atresia because then they have significant dilation of the proximal pouch. For those not familiar with the different types of esophageal atresia and fistulas, could you provide a quick overview? This is the diagram that I think anybody who's done pediatric surgery is very familiar with. It is a demonstration of. The different types of esophageal atresia and the different fistulas that you can get from that, and as we know, by far the most common is a proximal atresia with a distal fistula. If you'd like to follow along and check the diagram that Dr. Wen Alman is talking about, you can check the description below on the Stayer app. Let's get back to our topic. Many of these children also seem to have other associated anomalies, right? Lots of associated anomalies. Cardiovascular are very, very common, genitourinary, gastrointestinal, neurologic, and skeletal. The veal. And why is the distinction between association and syndrome important? It is an association. It is not a syndrome. Those are the defect. That can be associated in order to have the diagnosis, you have to have 3 of those to be classified as having a veal association, and we know that oesophageal atresia has a very high mortality rate in certain cases. When it's associated with particularly significant cardiac anomalies and low birth weight, it still has only 27 to 30% survival rate. So how should we evaluate a patient presenting with these issues? When it is associated with particularly significant cardiac anomalies and low birth weight, still has only a 27 to 30% survival rate. So what's your approach to evaluating this patient? Let's say we have a child with type C fistula experiencing respiratory distress. Our primary concern is that air is continuously going into the intestine, causing an increase in the stomach size, which can affect respiratory compliance. Basically, the problem with this is that the low resistance pathway is into the intestine, and that's why the air is going into the intestine instead of into the lungs. You can put a G tube in and decompress the stomach or decompress it with a needle, which will help temporarily, but that's also still the low resistance pathway, so the air is still going to go out and just come out of the G tube instead of going into the lungs. People talk about putting the end of the G tube underwater like a water seal to give it some resistance. That is. still doesn't completely fix the problem. It's a very interesting approach. And how about vessel loops? How important are they to this procedure? Putting the vessel loop around the distal esophagus is, if you're really in trouble, that's a little bit easier than trying to divide the fistula itself. So why is bronchoscopy vital in children with tracheoesophageal fistula? We believe quite strongly that every child with a tracheoesophageal fistula or esophageal atresia needs a bronchoscopy. And the reason for that is there is a probably 10 to 15% association of some associated airway issues with tracheoesophageal fistula and esophageal atresia, and it also can help you plan your surgery. And how can a bronchoscopy reveal the nature of these issues? The trachea is supposed to be an oval or a circle. It's not supposed to be this flat D-shaped. Shaped or C-shaped thing, depending on how you describe it. Virtually all kids with esophageal atresia have some element of tracheomalacia. The question is whether it's significant or not. You can't tell, I guess, based on the bronchoscopy, whether it's clinically significant, but you can certainly see it anatomically in the bronchoscopy. There's often mention of assessing the gap between the two ends of the esophagus. What does this mean for us? I personally believe that there is some Growth in the esophagus and typically our approach is to wait several weeks. I think once you get past probably 4 to 6 weeks, there's not going to be any more growth. Let's say the gap is quite significant. The way we do it here oftentimes is to just support the child with enteral feedings. bolus feedings often are good because we want to put whatever stretch we can on the distal. And then get sequential gap studies. You get to 6 weeks. Our approach would be to take the child to the OR. As far as I know, the positioning during teroscopy is key for successful visualization. How do you ensure proper positioning during the procedure? You want the patient almost sort of 45 degrees from prone, pushed way over to the one side of the bed so that you can actually access the patient more comfortably. And then the port sites, you, we typically put the A camera port looking straight down at the esophagus and the visualization you get is phenomenal. It's, it's better than open for any of you who have not seen it. Next, I'd like to ask about the role of the esygu vein in these cases. How does it typically approach during surgery? Oftentimes you start at the zygous vein because that will in many or most cases is actually guide you to the level of the fistula, because the fistula typically occurs right about the level of the zygous vein. Speaking of the fistula, what precautions do you take while dealing with the trachea during the operation? When you put that clip on, it's really important to make sure that it is flushed with the trachea. You don't want to leave a long. A pouch because that pouch can pool secretions and would potentially cause problems. Communication with the anesthesiologist is clearly crucial during the procedure. And can you explain how can they help us with the case? When you get to the upper esophageal pouch, it's really important to be connected to your anesthesiologist because you need them to push down. And often repeatedly to help you find the proximal pouch. And how do you ensure the safety of the esophageal pouch during the operation, considering the close proximity to the trachea? You would much rather put a hole in the esophageal pouch than in the trachea. Uh, we had one case where we were operating along and everything seemed to be going great, and then the anesthesiologist said, you know, it's really weird. The CO2 has just skyrocketed on the ET tube, and then we looked very, very carefully, and we had put a hole in the trachea. And the CO2 from the thoracoscopy was going up the trachea and out the end title, and it was causing this very, very high end title CO2. So if you hear that and you're doing this case, you should think there's a hole in the trachea. In most cases, uh, you can repair that. So let's move to a situation where reconnecting the esophagus is challenging. What alternatives or methods do you consider? There's the Fokker procedure, another procedure that was described by a guy named David Vanderzee who's in the Netherlands. The Kimura is a much older procedure. I've actually never done that. And then you get to the replacements. The Folker procedure involves putting traction sutures in, bringing those traction sutures out through the chest wall, and then using sequential tightening of those sutures. You can put traction on it, and traction is a stimulus. esophageal growth. And when the esophagus can't be saved, what are the most common replacement techniques? Primary are the cases that they operated on first, secondary were cases who had had a previous operation and came as a secondary approach. In the primary approach, they were able to get the esophagus together 96% of the time, whereas only 2/3 of the time if the patient had a previous operation. Importantly though, this is not a benign event. This procedure has. Significant morbidity associated with it. Even in the primary case, they spent more than 1 month in the ICU on average, and they were paralyzed for more than 2 weeks. In the secondary cases, they spent a mean of 110 days in the ICU and were paralyzed for more than 1 month. So that's somewhat morbid. I would say that none is any better than the other. The most common one used is a gastric pull-up, but there's a reverse gastric tube, a colon interposition, a duodenal interposition are the most common. Types. So we have a few options. First, can you describe the reverse gastric tube method? It involves creating a tube from the greater curve of the stomach. If you're ever going to consider this, when you're putting in your initial G tube, you have to make sure that you don't compromise the gastroepiploic artery because that's the blood supply to the reverse gastric tube. It has a lot of problems with it, and we certainly wouldn't do it. What we use primarily here is a colon interposition. How does this differ and what are the advantages and why? Prefer this one. The advantages of the colon are that you can get tremendous length. We've had caustic congestion patients where we literally pushed down on the pharynx and we anastoize the colon to the pharynx because there was no esophagus left. This is amazing, but I'm not going to lie, this sounds very hard. What are the challenges associated with this technique, colon interposition? The biggest challenge with the colon interposition, which is a bad one, is that they dilate over time, and this shows one of the classic. Problems with it is that they elongate and they dilate and food collects in this and doesn't empty. We were taught initially that these are hard to revise because you damage the blood supply to the colon, but that's actually not true. Now let's go back in time. What kind of success have you seen with this method? This is some really old data, but I threw it in there. Our 1st 13 colon interpositions, 12 of the 13 were NPO at the time we saw them. There was everything from caustic ingestions to esophageal atresia. Not had a graft loss since this initial one, so it can be a very robust conduit. One of my patients coined the term the callophagus. Her daughter came to us with a red rubber catheter sticking out of her neck because there wasn't enough esophagus to actually bring up an esophagostomy, and we replaced her with a callophagus, and she no longer requires anything other than just eating by mouth. Looking ahead, they're hoping for Dr. Helmrath to figure out how to grow an esophagus in the lab. For our audience who doesn't know who is Doctor Halrath yet, he is the director of the Center for Stem Cell and Organic Medicine, or CUTO at Cincinnati Children's Hospital, and they have developed in vitroculture techniques to maintain and expand individual human intestinal stem cells derived from human tissue samples, pluripotent stem cells, and surgical animal models. The other issue that we didn't touch on because it's a whole lecture. In itself is how do you deal with tracheomalacia. I am a believer in the posterior tracheopexy, which is putting some sutures into that membranous trachea to hold it, uh, back. Uh, we don't know who needs it and who doesn't need it. When you're doing a baby, and you have it all dissected out, it's right there. So my approach is to say it's all right there. Let's just put those 3 stitches in. It takes another 15 minutes. And then you don't have to wish that you had done it when you're going back through a previously operated field to do it later. Here Dr. Juan Alman has a final advice for surgeons regarding management of these cases. As surgeons, we're frequently trained that we need to be the captain of the ship, and it's all about us, and we need to do everything. And one of the best things that happened to me when I came here and started doing these esophageal cases was partnering with our ENT group. And we now do these all as the colon interpositions or any complex case we do jointly with the ENT guys, and I think that if we put our egos aside, it's the best thing for the patient to have the people with the most experience do the operation. And there you have it, a fascinating insight into oesophageal procedures with Doctor Dan von Alman. Together we delved into the complexities of esophageal atresia, importance of correct terminology, its epidemiology and diagnosis techniques. Also, we learned about various surgical approaches, techniques, and the challenges involved, especially in reconnecting the esophagus. Thank you for listening and stay tuned for more. If you like this episode, give us a rating on wherever you listen to your podcasts. Don't forget to subscribe to our YouTube channel, follow us on social media, and download the Stay Current app for tons of content in pediatric surgery. I'm M. Goddy, a research fellow from Cincinnati Children's Hospital Medical Center, and along with Stay Current we're sharing knowledge to improve child health around the globe.
Comments