Laryngeal Clefts

Hey there listeners, this is Rod Girardo, research resident at Cincinnati Children's Hospital and host of the Stay Current Pediatric Surgery podcast. I'm so excited because today, not only do we have the audio version that you can listen to on the Stay Current Pediatric Surgery app, on Apple Podcasts, on Spotify, on Stitcher, but we're also video. So you can find us on YouTube starting, I guess, today with this episode. So without further ado, enjoy the podcast. Is there anything that's beaten into our heads more during surgical training than the ABCs? I'm talking about airway, breathing, and circulation. And as you know, airway reigns supreme, which is why we're kicking off a new series on aerodigestive disorders. To do that, we're going to talk to Dr. Mike Rudder. He's the director of the Aerodigestive Center at Cincinnati Children's Hospital Medical Center. Really quick, a little bit of background. What is a laryngocleft? A laryngocleft is a congenital condition in which the posterior wall of the laryngotracheal tract is kind of open and then food or liquids can kind of pass from the esophagus into the trachea. As you can imagine, that can lead to aspirations. So with that, Dr. Rudder, let's kick it off with a question. Type 3 laryngeal clefts. How do you like to repair these? Trastracheal layered closure? Lateral pharyngotomy? Endoscopically or endoscopically unless there's a reason to do an open approach. Okay, so let's break this down. Type 1 means that the opening is above the vocal cords. Type 2 means that it extends below the vocal cords. Type 3 means it extends down into the trachea. And then type 4, well, here's what Dr. Rudder thinks. Laryngocleft classification. This is the Benjamin Inglis classification. We've been nagging Andy for years, Andy Inglis, to actually modify that classification. Oh, Andy. To include a type 4 long. Because think about it, type 4 could be really proximal above the crina. It could be at the crina or it could go straight through the crina. And of course, Andy responded by retiring. So there's nothing we can do at this point in time. Let's talk about some diagnostic dilemmas, specifically really small clefts. So look at the video on the left. A flexible bronchoscopy being done by Paul Besh, who's about the closest thing to a savant with a flexible scope that I've met. And this kid so has a history that would go along with having a laryngeal cleft. And Paul is desperately trying to find it and utterly failing. And now look at the video on the right. Clear as day, there's a type 1 cleft. So what's the punchline? Flexible bronchoscopy is not adequate for diagnosing a posterior laryngeal cleft. All right, let's talk about endoscopic management. The technique that we've developed in Cincinnati is a mass closure technique. It's the same concept with tracheoesophageal fistulas if you're repairing them endoscopically. You want raw against raw. Mucosa is a nonstick surface. Get rid of the mucosa. You want a wide strip of raw against a wide strip of raw. Wait, wait, wait, wait. What about the lasers? Well, to explain that, here's Dr. Alessandro Dialicon. So what's really nice about a laser is this is where you can take advantage of that part of removing all that mucosa broadly. So this is using a KTP laser. You can use a CO2 laser, whichever laser you have in your armamentarium. It doesn't really matter which one it is. And like they were saying, you want to make sure that you go nice and wide. Using the laser is often easier to use, also easier to teach with. Now, what about the open approach? So this is really reserved for your cases where you've got failed endoscopic repair. So this may be for your, with type, some type 2s and type 3s. It's a laryngo fissure approach. When we're thinking about our type 4s, it's a cervical approach. And then the type 4 lungs. Now, these are really challenging. So that means they give you anesthetic challenges, which may mean a double lumen tube, single lung ventilation, ECMO or bypass just to do the surgery itself. They often have associated microgastria and multiple other congenital anomalies. And those pieces may actually drive your decision making about even attempting a repair if some of those other things are not compatible with life. And very important to counsel your families before even making the attempts that there is a very high mortality rate with these patients. And they mean high, like 50% mortality rate. Now, keep in mind, some of that literature is based on older techniques. We talked about the concept of mucosa being a non-stick surface. We developed a technique for basically a reliable, simple, fast method of endoscopic repair of a type 1, type 2, types, often type 3 laryngeal cleft, and a deep notch. Again, the concept is not an anatomical cleft, but a physiological cleft. We don't care if you've got a type 1 versus a deep notch. We care whether you're aspirating. All right, here's a video in real time of this procedure being performed. If you're in the audio version, just scroll down under the media player, click on the link, and it'll open a video for you. What you're going to watch is the V-shaped area of mucosa getting excised. And then you're going to see some sutures placed. So check it out. Thank you. Thank you. Now, when would you lean towards doing an open repair? We tend to be looking at the more severe clefts. So usually the 4s. None of us has been brave enough to tackle a 4 endoscopically yet. Most 3s, we will try and do endoscopically unless there's a reason to go open. And so this is the transtracheal technique where you form layers between the trachea and the esophagus. You sew up the esophageal layer, knots and lumen. You sew up the tracheal layer, knots and lumen. You can use an interposition graft if you wish to. And this is a coronal section of the same. So check this out. Here is a type 4 cleft repair. The cleft was about a centimeter above the carina. Here are the sutures. And then here is the post-operative view. And then this is the same patient 5 years later. So type 4 clefts. Dr. Rudder and the team actually developed a new surgical technique for the type 4 longs. Let's hear about it. We transect the trachea at the lower border of the cricoid. We peel the trachea off the esophagus. So you right to beyond the cleft. And you can actually keep the patient intubated into one bronchus while you do this. And then you're looking down on the hole in the esophagus. You sew up the esophagus. You can do a second imbricating layer if you wish. You can lift up the larynx and continue the repair up to the arotenoids through that approach. Place an interposition graft. Typically we use sternal periosteum. You can use tibial periosteum. Then we sew up the back of the trachea with the endotracheal tube still in it. And then reconnect it to the larynx. Intubating from above. Reconnect the trachea to the cricoid. Let things heal. And then place a trache relatively late. Two or three weeks later. And so I'm going to show you an example of this. This is a girl who was referred while all of us were in Portugal. And the weather in Portugal was fabulous. It was a great meeting. And we got a phone call about a 2.3 kilo kid with quite a cleft. All right, let's take a look at it. Dr. Rudder, walk us through the anatomy. So on the right, this is the right bronchus. This is esophagus. Over here, that's the left upper lobe. Still esophagus. And if you look around, you can actually find the left lower lobe. So this is a cleft to the end of the left bronchus. That's left lower lobe. So as laryngeal clefts go, this is long. And we used the described technique to do the repair. And there are some nuances here. So for starters, they waited three months until the child was greater than five kilograms. That's based on outcomes data from previous research. Next, the patient would undergo regular flexible bronchoscopies with a whole team. And once you do place an ET tube, think big, like 4.5. And so with her, we actually did this on ECMO because it was easy. It was such a long cleft. We did an aortopexy as we came out. This is the trach. We had to put the trach in incredibly high because all of these children have a very short trachea. So the trach went through the cricoid. We're still sorting that out. And you've basically got right bronchus, left bronchus, left upper lobe, left lower lobe, and a girl who's not on positive pressure support, who can't eat yet, but will get there, still has a trach, but will get there. Now, when it comes to laryngotracheal esophageal clefts, the biggest risk is that the distal end turns into a trachea esophageal fistula. And that's exactly what we're looking at with this patient. And again, we're using a cuffed endotracheal tube in his esophagus to show where the fistula is. And what this is, the second slide or the second video is after we've done a transtracheal three-layer closure of extremely thin mucosa. This is what we would now do a slide tracheoplasty on. And he's still got a very small residual tracheoesophageal fistula. And so what we're going to try and do is fix this endoscopically. And this boy's six. So this is difficult in a younger child. This is using a bugbee cautery to demucosalize the tract, as we previously talked about. And we're then going to place an endoscopic suture to, like, gate that tract and get raw against raw without things, saliva moving through it or air moving through it. And this endoscopic suture, let me just say that this is fairly edited because this is a difficult thing to do. And this is using a P2 needle on a 4-0 PDS and then tying the suture afterwards. A surprisingly difficult thing to do. And this is the post-operative view. Three months later, you can see a little bit of the residual suture left. No tracheoesophageal fistula. So there you have it. Laryngeal clefts from Dr. Mike Rutter, the director of the Aero Digestive Center. Did you love this? Did you hate this? Do you want to go back to audio only? Let us know in the comments. But in the meantime, download the Stay Current Pediatric Surgery app. Get ready for August 27th, 2021 when we're going to have our next pediatric surgery update course. There you can watch lectures just like this live online. And you can ask people like Dr. Mike Rutter questions in real time. So until next time, I'm Rod Gerarder from Cincinnati Children's. And remember, knowledge should be free.