This is really peri and postnatal management a little bit. Um, it runs forward from the previous talk. And again, I like to keep things fairly simple and what we try to do is around 32 weeks, uh, we, uh, perform an ultrasound and we try to, uh, prognosticate whether the fetus will be asymptomatic or symptomatic at birth. And if they're going to be asymptomatic, and that's really based on mediastinal shift. So, if there's no mediastinal shift and it's a relatively small lesion, Then, uh, we'll consider that child to be asymptomatic at birth. They can deliver close to home. They can take the baby home in a normal fashion, and they can come back for a CT scan and have an elective resection, uh, thereafter. Uh, if there is mediastinal shift present at 32 weeks, uh, uh, without major cardiac or lung compression, then, uh, to be on the safe side, we generally have them deliver, uh, at our center, uh, or at a surgical center. And those kids usually have postnatal resection during, uh, the first few days of life. And then, uh, the third scenario is if you have major mediastinal shift, uh, with longer cardiac compression, then in those cases, uh, they should definitely deliver in a tertiary center and have the availability of the exit procedure or advanced ventilatory support ECMO. Lung lesions, postnatal management, I sort of divide them into three categories CPAM, interlobar, BPS, and hybrid lesions. In my opinion, all of these need to be resected, and we routinely resect all of them. Uh, extra lobar BPS, uh, some cases, uh, don't need resection, can be observed. Um, and I'll go through that. And then the, uh, bronchial atresia sequence, again, you can have either main stem, lobar, or segmental, uh, complete or partial obstruction, uh, leading to, uh, a variety of lesions. Bronchogenic cysts can exist with or without the associated bronchial atresia. Um, And can be a separate entity that can be observed as well in some cases. So, the reason I think CPAPMs need resection is because they have CPAM histology, and it's, uh, well known that they're prone to infection. Uh, they have mucoid stasis routinely when you resect them. A lot of them have, already have inflammatory, uh, uh, evidence of inflammation and, uh, uh, infection when you take them out at three months of age or two months of age. Uh, they also are, uh, established to have malignant potential. And no one knows the frequency of that, but it's certainly been, uh, reported in multiple case reports with, uh, pleuropulmonary blastomas, bronchoalveolar carcinomas, rhabdomyosarcomas, and pleuropulmonary, uh, blastoma is a particularly troubling one because you can't differentiate it radiologically from CCA or CPAM. And I personally have resected three CPMs that turned out to be stage 1 pleopulmonary blastomas in my personal experience. So, those are lesions that are highly malignant and lethal if allowed to go beyond stage 1, and they could not be differentiated from relatively small asymptomatic, uh, CPAPs. And then intralobar BPSs, they can have CPA histology. What you need to realize in dealing with these lesions is there's really a continuum, uh, between the lesions. They're not, we put them in these categories, but there's a great deal of overlap. And you can have anatomical overlap, as I'll show you, or histological overlap. Uh, so, you can't, for instance, be, uh, absolutely certain that a sequestration lesion that has small cysts doesn't have CCA histology. Um, Uh, interlobar BPS, uh, mucoid stasis. There are communications via the pores of con, which can lead to infection, and there's the potential for high flow physiology. For, so, for those reasons, uh, I feel that intralobar BPS also should be resected. And, uh, just to interrupt, just because it's two blastomas and one adeno CA that, So, you know, we all hear about this being a, a relatively minor thing, but in just our experience, there are at least a significant number of cases that I think it has to be a real point of discussion and issue. And the other thing that Alan mentioned was the early inflammation and, and I think we'll discuss later about. The timing of resection, but I think you find significantly when you do a significant amount of these that even waiting until the child is 4 to 5 months of age, you can find significant inflammation within within the fissures and things of that nature that change the complexion of the dissection, and we'll talk about that later. But I think those are two really important points when you when you're having discussions with parents about. Whether or not these need to be resected and timing of resection that are really important so I don't, we don't, we're gonna interrupt your talk so um. In those kids, those were kids that had a CPAP, and then when you resected it, you saw that that was in the tissue, or these were kids that just happened to have those lesions and you think it was related to a CPAP. The, you said you had pregnancy, yeah, no, they were, they were prenatal. Two were prenatal diagnosis of the CPAP, or, or one was prenatal diagnosis. One was picked up later. Um, it was just a kid came in with a cough. Had, uh, got a chest x-ray, looked like a CPAP and it was a pulmonary blastoma and we resected it. And the third was just we found it within the tissue, the, the. Within the CPAP? Yeah. Two of mine were asymptomatic, uh, prenatally diagnosed CPAPs that I did thoracoscopically and found pleuropulmonary blastoma. And one of them, uh, presented with a viral pneumonia, um, at 8 months of age and got an x-ray that looked like a CPAP and then had pleuropulmonary blastoma. OK. You know, I think this is one reason to, to remember that we refer to it as a congenital lung lesion rather than a CPAP. Right. For those exact reasons. Yeah, you're right. So, uh, on the timing. So, uh, on the timing, um, so, I think all these should be resected. The indications, uh, are mass effect, respiratory symptoms at birth, obviously, you would do that in a neonatal period. No controversy there. Uh, some, some lesions will be asymptomatic at birth and then air trapped, uh, within the first few weeks of life or months of life. They should be, uh, resected when symptoms develop. And finally, obviously, if infections, uh, are malignancy, Are, uh, believed to occur on a frequent basis, then it would make sense to prophylactically resect these. I agree with Steve. Uh, I think the earlier you resect them, actually, the easier it is surgically and the less traumatic for the infant it is. And we'll go through thoracoscopic resections or even open resections. Um, The kids do much better and they're easier, resections if you do them early rather than late. So, I don't see any reason to wait unless you have concerns about your anesthesia support or other reasons for waiting. Um, thoracoscopy versus thoracotomy. Steve will talk a lot about thoracoscopic resection. I'll vouch for it being a difficult learning curve, but once you learn it, it's absolutely a superior operation, in my opinion, shorter hospital stay, less pain, no thoracotomy morbidity and better cosmesis in the long term. So case presentations, um. Asymptomatic CPAP. This is a prenatally diagnosed lesion. CVR 0.4. It was never large, never concerning. Um, and no mediastinal shift, asymptomatic at birth. So, this is really a garden variety sort of CPAP, uh, predominantly microcystic. You can see one, predominant little cyst there. And that's an important observation, you know, the, the segmental bronchial stenoses really have a very confluent appearance. They're almost like just emphysematous pulmonary parenchyma. And I think that's a very controversial lesion that we don't really understand yet and that may not require resection on a routine basis, although, uh, certainly, if you see any cysts in it, that's usually indicative of CPA histology, and, uh, a lot of them do have that. So, do you, do you think we're good enough? Based on our imaging studies to be able to differentiate. No. So, that you possibly you could, you might think it's bronchial atresia, but it could have CPAP elements within it. Yes. Or vice versa, you may think it's a CPA. And it's a bronchial atresia. So, this is just a pulmonary lobectomy. I have to show a video or two, or two just to compete with Steve. This is a, Left lower lobectomy. And uh basically taking the vessels and you can see the the beautiful magnification you get. Uh, we have, uh, tools now like the ligature, particularly in, in infants that you can take the majority of the vessels with. It's another advantage of doing it early is the ligature can take care of just about everything. I tie off the bronchus and put a clip on it. Um, That's probably overkill. And then the, uh, pulmonary vein, and I show you this video. It has a perfect fissure. It's, uh, you know, you know, an easy case once you get used to doing thoracoscopic lobectomies. Hm. I'm tying off the vein for some reason. I used to tie off the vein. Now I just usually clip it or, and ligature distally. Are you using pneumothorax? Are you, are they doing the right thing, I do a single, a single lung ventilation, yeah. OK. So, that's, uh, one asymptomatic CPAM, an easy one. Here's one that's a middle lobe lesion. I consider the middle lobe the hardest lobe. Um, some people consider the left upper lobe harder, but for some reason, middle lobes have been, uh, difficult for me and this is just an example. So, here's another, Video of a, and I'll just show the anatomy on this, uh, just to convince you that they're not all easy. So, this is a very visible, uh, CPAP. And you can see the fisher, the major fisher here is very nice, beautiful fisher. But then you go up to the. Uh, minor fissure, and there is no fissure to speak of, and it's really very densely inherent there. And I, uh, even with the section, you can't really identify a decent fissure. Um, and they're often abnormally, uh, globulated and have abnormal fissures related to the developmental effects of the secam, I think. So, you can't depend on normal anatomy when you're, uh, when you're doing these, uh, lobectomies for, uh, sea cams, particularly. And I won't share this whole video, cause you'll see a lot more of. So, this is a lesion that's good to see how to do it right before. Yeah, exactly. I wanted to show you a bloodless video. All right. Of thoracoscopic lobectomy. Um, So, this is a kid that is an example of what I mentioned earlier. So, this child had a very, uh, large secam prenatally, and you can see it there on the left. And then, uh, by birth, it had regressed predominantly. There was no mediastinal shift and it was hard to see. And so, we predicted this child would be asymptomatic at birth, which he was. But then about two weeks of age, he started getting tachypnik and an x-ray showed that this had, uh, air trapped and enlarged and we went ahead and did a thoracoscopic resection. Uh, at that point. So just different scenarios here of CPAP. Now, this is an interesting lesion that addresses what you mentioned earlier. So, here's a baby that had a bilobar CPAM, uh, diagnosed prenatally, uh, thought to be a microcystic CCA. And he was born and had a CT scan and, uh, I couldn't see any cysts in this. The radiologist couldn't see any cysts in this. My concern was that it was segmental bronchial stenosis. Do I really need to do a bilobar resection on the baby? Um, and I discussed it all with the parents. They wished to go ahead with the resection and I did a thoracoscopic, uh, Blobar lobectomy here and the baby did just fine. So, It's uh, and it turned out to have CPAM histology, which made me feel better about it. And that's not, I mean, we've, we've had, um, a few cases. Um-hum. Like that where, uh, I've not, I've yet to have one where we didn't find some. Well, you, you see some funny things. When you do a lot of these kinds of lesions. I had a lesion that had a, a, uh, bronchial stenosis that had pneumocystis carina. Yeah. Grow out of it, OK? At three months of age. It had a complete immune workup and had no findings of immunodeficiency, but, You see all kinds of, kind of odd things. Yeah. But you frequently do see CPA histology within them. So, here's another type of lesion that, uh, we frequently see, asymptomatic, uh, CPAM, and this is a hybrid lesion. You can see the vascular reconstruction there. This is no small, uh, uh, feeding vessel. It's a big feeding vessel and it's clearly CCAM histology. So, uh, this is not an interlobar sequestration. It has, uh, macrocystic, uh, abnormality and is a CPAP. And just a video, and this sort of uh shows you a couple of hybrid lesions and how you deal with them, um. So, actually, this is, uh, the second one in the series, but it's OK. Um, Can go ahead and continue that one if you want. So, that's a, that's what a, a very large feeding vessel looks like, uh, thoracoscopically. You can see it's, in this case, about half the size of the aorta. Um, these are big vessels. They're kind of scary. So, a lot of them have abnormal integrity. They're sort of like PDAs. Uh, and you have to be very careful with how you deal with them, uh, because you can cut through them if you tie a suture too tight or clip them to, uh, Uh, firmly. And so, in this particular case, I'm using a clip and then ligaturing distal to the, to the artery. I think I was still a little nervous at that point with the ligature. I probably could've taken it, uh, with the ligature alone. Just so perspective, the instruments Salon's using are 3 millimeters, except for the ligature, which is a 5 millimeter in diameter. So, that vessel is at least 5 to 6 millimeters in diameter. And just, just so you have some sort of frame reference for what the sizes are. And the important thing is to always keep your, uh, Maryland close by, uh, and leave a stump on the aorta so that if something goes wrong, you can grab that stump, um, And salvage uh yourself. So, I actually have three hybrids there with three different methods of, uh, Uh Securing the artery, but I'll spare you that. OK. So, this, I'm just going to show you a series of cases here that just show you the variation that you can see in these lesions and sort of highlight what I mentioned before about the overlap between anatomy and histology. So, this was an interesting prenatal case. It was thought to be a right upper lobe bronchial atresia with, uh, hyperplastic growth of the distal right upper lobe. Uh, there was respiratory distress requiring 4 days of inhalation. It was delivered a little early at 2400 g. And a CT scan showed two lesions. One was a large apical sequestration, which was what was interpreted as the bronchial atresia. And then there was also a right middle lobe, uh, CPAM, and you can see them both there, uh, on the, on the scans. You can see the indominate artery feeding this, uh, sequestration. And sequestrations can draw their blood supply from essentially any systemic source. Uh, and they're often very interesting when you get in and look at them. So, this is the right middle lobe abnormality there. I didn't cause that hemorrhage on the lobe. Yeah, right. Yeah. Yeah. And then as you go up, you see this thing, uh, and it's actually got a plural cap on it. And you can, and a lot of, you see these abnormal pleural, uh, investments of some sequestrations. So you actually had to pull that thing out of the pleural cap. Um, wow. And you can see the vasculature as I pull it back here coming off of the innominate up there. And it wasn't trivial vasculature. It had a real, uh, sort of a bag of worms there, of blood vessels that you can see going to it. And this is actually the venous drainage coming off the inferior side of it. Very small or friable veins and. That's sometimes I think the same as an azagous lobe, that, uh, membrane that divides it. It's just there's a lot of questions from the audience about your technique as you're going on, um, and I think a lot of them are asking the same questions that we'll address it at the end, which is how you take vessels, uh, for people all over the world who may not have all the same instruments. Uh, questions about clips and be good enough. I've evolved over, uh. My series of lobectomies and that all partially because of a mishap I had with the older version of the ligature. It's called the ligama because it, uh, I did a pulmonary artery with the earlier version of the ligature and I lifted up the, uh, lobe to look for the vein and suddenly the field turned red and my seal had broken down. It looked like a good seal. All the right sounds went off. And I had a pulmonary artery hemorrhage, and so I crashed into the chest and controlled it, but it, it took at least, you know, years off my life and gave me a lot of gray hair and chest pain. Um, and after that point, I tied every vessel, every pulmonary artery, and then ligatured distally, um, until the new, um, force triad came out, which is a much better energy source. And I felt a lot better about, uh, the ligature, and I've gradually gone to, back to just ligaturing. But if you, if you tie all the vessels, uh, you learn intracoreal not tying very well because of the small space and the motion. Um, and, uh, it takes about twice as long to do your cases, but it's, uh, one alternative, uh, that's useful. I mean, you can do a lot of different techniques and we'll spend a lot of time in the second half. Talking about technique and, How to take vessels. But. Yeah. I, I would agree with Alan. Um just so, so for the audience, put your questions up, but, uh, the second half of this is pretty much all on technique, and we'll get much more into detail on that. So, this is another. It actually had was a mid thoracic extra lobar, uh, BPS and a right lower lobe CPAP. And you can see how big the BPS is, uh, there in the mid thorax and that's what looks sort of like the lower lobe, uh. Um-hum. CCA prenatally. And then there was a lower lobe CCA, uh, as well. And so, just, uh, I'm going to show you about 10 different types of BPS's here, just to, my goal is to show you every possible permutation of a, uh, sequestration. And you can see this is a big edematous sequestration. A lot of sequestrations are edematous because of, uh, I think, restricted outflow, both, uh, venous congestion and lymphatic, uh, congestion. In this case, this was a piece of cake. You just take it with the ligature, it's small pedicle. All the edematous BPSs have small pedicles. Um, they often have pleural effusions associated with them. And they're very easy to resect. Yeah. So, that's all there is to it, then went on with the lower lobectomy. This is one of the cases I referred to. This is a 4 month old who presented with a viral pneumonia. Uh, a chest x-ray showed the cystic mass and it turned out to be, uh, a pleuropulmonary blastoma. I won't show you the video on that. Here's an interlobar bronchopulmonary sequestration. I showed this just to illustrate the, uh, similarities of that to hybrid lesions. And here's, uh, a massive blood supply that you can see coming off of the infra diaphragmatic aorta. Um, and you notice that it, it goes into the lung parenchyma and then drains almost directly into a pulmonary vein. So, this is a very high output potential shunt. Um, well, it is a shunt, but it's potentially a very high output shunt that would put the child into cardiac failure. Usually by the time they're 3 or 4 years of age. And if you look at the parenchyma, there's not much parenchymal abnormality. You see a little bit of, uh, hyperinflation. Um, Uh emphysematous appearance, but no real, uh, cysts or masses. So, interlobar sequestration. And uh again, just a view of the feeding vessel. And again, a lot of these vessels are abnormal. You can see how torturous this one is. And you'll see a lot of arterialization of the vasculature in these lesions. So, um, even the pulmonary veins, the outflow becomes, becomes thickened like a, uh, Uh, an artery. This just shows the inseal device. It's another device I use frequently, although the, the dissecting or the tip of it isn't nearly as fine as the ligature, but it does, uh, seal very nicely and divide the vessel. And I think you get less collateral, uh, injury with this device than you do with the ligature. So, I like it for, for areas where you don't have to, uh, you're not in a tight space and you have room to use it. And you need to be careful and watch cause there's often multiple feeding vessels. Yes. It looks, it looks like, may look like one on the CT scan. Um. But you get in, and there, I've been in cases where up to three or four vessels. Wow. Have actually come off. Yeah. Or they'll branch very early on, right after. So, so, you really need to look at this area very closely when you take it. And that's true of all lobectomies, you know, irregardless of whether, of what you think. When you take the inferior pulmonary ligaments, you need to be very careful about looking at it. And and the blood flow through some of these small systemic vessels can be. Uh, phenomenon. If you, if you take the vein, for instance, and you don't know, and you've missed a, uh, systemic feeding vessel, you'll, uh, have tremendous congestion of the lobe Prior to taking it. So, the unsealed device. OK, and this is just a CT on a three year old that did have high output physiology and, uh, pending cardiac failure. You can see has increased, uh, cardiac to thoracic ratio and you can see that huge pulmonary vein coming into the heart. The pulmonary vein dilates dramatically and, uh, Yeah, it gets huge. So, extra lobar BPS, uh, have a separate pleural investment, no bronchial connection, systemic arterial supply from almost any source, and have systemic or pulmonary venous drainage, which I didn't really appreciate until I was taking a lot of these, uh, lesions. They can be supra, intra, or sub-diaphragmatic variants. So, there's all kinds of interesting BPSs that you encounter. Um, they can have CPA histology, as I said. Usually, they have a visible cyst if that's the case. They have mucostasis, but no communication with the airways, so there's really not an infection risk. There's potential for high flow physiology for lymphedema and pleural effusions and occasionally an esophageal bronchus. So, um, why do you need to resect extra lobar BPSs? These are the reasons that I resect them. If I'm worried about high flow physiology, that is, they have a large feeding vessel, and particularly if they have pulmonary venous outflow, um, it represents a potential low resistance, high flow circuit. Um, large size, so mass displacement effect, uh, or interval growth, which I've rarely seen after birth. Uh, visible cysts by ultrasound or CT because, again, that's CPAM, uh, histology, uh, almost, uh, always, and pleural effusions. And I would just add to it, I have seen these get infected. Even though they don't have a, uh. Yeah, I have two, and it must be. A hematogenous. Yeah. So, they, they actually do get infected and present as pneumonias. And then, again, once any of these lesions get infected, they're much more difficult to resect. A, a purely extra lobar sequestration that's prenatally diagnosed is, is a relative chip shot. Yeah. Um, in doing these cases. And in fact, a great way, and, as you're learning to do these, uh, a great place to start. Uh, but once they get infected, they can be much more difficult because the planes are really distorted. Yeah, I really think these could be done as an outpatient procedure, you know, two ports, no chest tube. I send them home the next morning. Right now. And so, it's really almost, uh. Yeah. So, just some examples. Here's an extra lobar BPS, sort of garden variety. You can see the feeding vessel. It's, uh, Not particularly big, but big enough for me to take it out. Doesn't take much for me to take it out. But you can see how edematous this lesion is. Um, and you can see the pleural effusion associated with it. There's fluid pooling at the bottom there. And you can, you know, it's a lymphatic congestive problem. And there's the small pedicle. And Steve said, nothing's easier than just, uh, coming across that with a ligature and. Taking care of it. There's the feeding vessel. So that's garden variety. This one, Cool Is, uh, I think I'm, this is the second PowerPoint, but in any case, this is a, uh, again, an asymptomatic prenatal diagnosis. And this is one that's down, uh, down, uh, and it's hard, it's often hard to tell whether they're supra diaphragmatic, intra diaphragmatic, or subdiaphragmatic when they're down in this area of the GE junction. And you can see the feeding vessel coming up into this one. Um, and it's right down there next to the esophagus, and that always makes you think, uh, you know, that there may be an oesophageal bronchus involved. I hope the videos are in sequence here. By the way, while we're taking the time here to get the next video, there's two comments I wanna make. Um, it seems like 99% of you guys are having a great, uh, image, um, but if you're not, um, we are also broadcasting this as a live stream, and we will put up that website so you can watch it in a, a higher sound quality, higher definition. Um, so check that out. We're looking to see if that's something we wanna do in the future. So we want your comments on what you think about that stream versus what you're watching here. Uh, we, uh, Jesse just posted the website for that, uh, right there, and there it is there. It's, uh, Ustream.tv. You can look at that, and you could watch it as a live stream. Uh, the other thing is that we have an immense number of questions coming in. Uh, so if you want your question to take priority, call in, call into the studio and ask your question, uh, over the phone. Those questions will get priority over the written questions. So, all right, so our videos are out of sequence, but I can, we'll see them anyway. Um, so go ahead and put the video up that, uh, you had there. Stefan, and this is actually an extra lobar sequestration with pulmonary, uh, venous drainage. Uh, and you can see this very large venous branch that I'm dividing now that heads up, uh, under the lung and into the pulmonary vein. The majority of these that you see will have systemic venous drainage. And I always thought you could differentiate intralobar from extra lobar by their, the presence of pulmonary venous drainage until I encountered a bunch of these that, in fact, have pulmonary venous drainage. So you can see this is almost a pure, uh, AV fistula. In reality, it's a little piece of lung tissue with big blood vessels going in and out of it. Hm. And there's the artery. You can see it's a big artery. Hm. Wow. And I think this is that lesion here. Um, and you can see as I go through here, you'll often see these abnormal, uh, densities on the arterial or, or venous phase of the CT. And if you pan back and forth here a little bit, you can see the lesion. And then you can see this pulmonary vein going up the empty end of the pulmonary, uh, inferior pulmonary vein. And then over here is the artery. Sorry, it's a little, as you can see the, the, uh, artery coming across from the aorta here as I go back and forth into the lesion. So, We'll see what this video is. Um he said one second. OK. This may be the, uh, Bronchial Esophageal bronchus, by the way, I, I found out something totally cool, completely unrelated while we're waiting to, I think I sent it to you. If you wanna show in a PowerPoint, uh, a CT scan and you wanna scroll through the images, you can make a video as you scroll through. Have you done that? I will at the end of this presentation, I figured, why don't I do that with my iPhone? Yeah, but I did it with my iPhone, so it works, but I didn't have time to put it together. So this is the esophageal bronchuss one, and you can see I've actually When I looked in, I couldn't see anything. And then I made an opening in the diaphragm where I saw a little bit of prominence. And that's the diaphragm that's opened. OK. And I'm basically dissecting around the base of this, um, Um, sequestration right now. And you can see there are a lot of blood vessels and things, but as you come down to the base, you begin to see this, um, it's a little hard to see on the video, but you begin to see this bronchus that's going directly into the esophagus. There it is. You can see that doesn't look like a blood vessel and it's got sort of a bronchial wall on it. Um. And, uh, none of the prenatals or the studies, uh, pre or postnatally have demonstrated this. I'm just putting a ligature on it after I get it all, or not a, a, uh, endoloop on it after I got it all dissected. And when I cut into this, you'll see, um, What cesspools these things are. Because uh this thing was just full of of mucoid garbage. So, you encounter these and you have to be very careful. Whenever a sequestration is against the esophagus and you're having a little bit of trouble separating it, then think esophageal bronchus. Um, sometimes they're not real big. Sometimes you can actually miss them if you're not, uh, Uh, aware that they may be there.
