All right, so Steve, uh, Steve Rothenberg is going to talk to us about congenital cystic lung lesions, thoracoscopic resection. So, uh, this is just a case presentation, um, to talk about some of the controversies, if there are controversies regarding, uh, the management of, uh, CPAPs or congenital malformations of the lung, um. Yeah, see if it works. Yeah, so this is the case. Um, this was a child who prenatally had a diagnosis of a congenital cystic lung disease on a prenatal ultrasound. The patient was, uh, referred for, um, uh. Uh, sorry. was referred for um uh prenatal consultation, um, from the perinatologist with us. Uh, prior to doing that, um, they obtained a fetal MRI which showed a 4 by 3 x 2.5 centimeter cystic mass in the right lower lobe with evidence of a feeding vessel. So the first question would be how many in the panel would get a fetal MRI for a cystic fetal lung lesion? Anybody? Personally or at our center? Well, I, I think. All right. That's your chance. I mean, I, I, I find that more and more are being, being obtained, but they really don't change what I do at all. Is that pretty much, well, yes, yes and no. I mean, you are trying to decide whether you're gonna give them steroids or not, right? I mean, someone is. So that's the reason and the indication to get an imaging study so that you're going to do something about it. Like you said, I completely agree. And if it's microcystic in a certain size, then at some centers, you are gonna get uh steroids that will change the course at a certain age. So if you're gonna do it for that reason, absolutely, you should be getting imaging studies under whatever protocol that you have. You got to check your ultrasound. Uh, the prenatally to make sure that you're within your own parameters that makes sense and that makes sense. But if you're just getting it because it's cool, that's not an indication to get it. And sometimes that's the reason people get them is I just wanted to know, that's not acceptable in, in my opinion. If you're gonna do something about it, sure. I think it partially depends on if the fetus is manifesting signs of drops or something like that. So, well, that's a different case, but if if they're not, they're not manifesting, there's no evidence of high drops or an MRI is really going to change. Yeah, but I, there are centers where everybody who has any. Cystic, uh, lung lesion found on ultrasound, do get an MRI, and I, but I think Abdullah makes a good point. If you're going to give steroids, then there's, then you need to know. Yeah, and you can get steroids even if they don't have manifestations of drops. If they meet whatever criteria you've set out in your, in your hospital, you are still intervening on the baby, on the fetus, and if you're going to do that. But like you said, if you're not, if you're not going to do that, then you shouldn't get it, correct. We shouldn't get any test unless it's going to change. Correct. Getting back to the line placement and the call. So anyway, this child had that. We, we spoke with the child. Now how many, I mean, some of you are in um Isolated children's hospital and some of your own women and children's. How many people, uh, if you had a delivery service, would have this mother delivered at your hospital as opposed to an outside institution? In your institution back you would cause you're combined anybody else feel strongly. We, we have a combined hospital and in this case, um, uh, this, this actually, this case actually was from New York, but this mother did deliver um at Columbia. Anyway, the baby was born at 39 weeks. Apgars were 9 and 9, and the baby had no respiratory distress, and a chest X-ray was obtained, which showed cystic lung disease, and I think the next side kind of shows that. So that's the, the initial ferment film at birth. Um, babies on room air, doing well. What, what, uh, what would, uh, Anybody do at this time. So this is a question, uh, for the, for the audience, but the postnatal workup at this point, asymptomatic infant, that chest X-ray, uh, would you, would you watch them, you know, just watch them. Would you do further imaging at that time, or would you go straight to surgery? Mark, we put that pole up. Great. Did that X-ray show mediastinal shift? I was a little concerning. I think it's a little rotated, so I don't, I don't think really the child had any media style shift, and it's a little hazy. Part of this is quality of the film and it's initial, but so basically a stable kid, no mediasty shift, but you can see the lesion on the uh on the X-ray. So how many on the panel are gonna book that kid for couple of days? No, nobody. OK, no, I, I, I wouldn't, I wouldn't. Well, I do, yeah, the only way I would do this, so we live in a, in, in Denver, we have people who come to deliver from a 1000 mile radius because of the, the large expanses that we cover. And if this family came in, it was a huge hardship for them to come back and forth, and they said, we really want this done. Um, and that was going to be an issue in the management or if I was somewhat concerned based on, I've had a couple of kids who are asymptomatic but have giant cysts and I'm worried that sending them home is going to be a problem, then we will operate early. So, uh, quick, quick question from the audience and I open this up to anyone, maybe Abdallah, whoever, or Dan, whoever feels they can answer this. Quick review of indications for prenatal steroids. What, what is your, you said whatever your hospital does, right? Yeah, I was actually just typing it up now while, while, while we're discussing. But since you're asking, I mean, the indications differ depending on your institution, but you got a prenatal ultrasound and you want to decide, OK, is this something worrisome or not? And if it looks like a big mess, and it really is just kind of reading the prenatal ultrasound once you get comfortable with that, the radiologist, the, uh, uh, MFM, the internal fetal medicine, or the surgeon. And you're like, OK, fine, it's kind of a big mass. And then you measure out pretty much the CVR in it. And for most people, you know, you kind of use a 1.6 cutoff to see if this is big enough, um, to be worrisome. You don't want to wait for the high drops per se. And then, um, you want, you want it. Decide if this is a microcystic lesion or a macrocystic. My understanding is it doesn't really work on the macrocystic lesions. So you really got to try the microcystic, and this was a coincidental kind of uh finding from the folks at UCSF that sought for different indications and they said, oh, these lesions literally did not either the baby grew and the mass stayed the same or the lesion stayed less. So that would be an indication to to do it. And then, you know, there's one other point, if you're not at an institution that's comfortable with that. Then you should be able to manage with your prenatal folks. A protocol of some kind so that if you find that patient, you should refer them to someone that does. So that may be another reason to get a fetal MRI to be like, OK, this is a big lesion. I don't know. Do we give steroids or not? We don't do this with us, but send them someplace that does. And there are good centers that I mean, Cincinnati is one as well that does that, that does that. Sorry, Steve, good point, that's good. Do we have the poll results? Is there? Yeah, so Stefan, can we throw those poll results up? It looks like 62% would do observation and 37% further imaging. Nobody would go to the OR. Uh, I think that's appropriate. So this, this child was actually was doing well, lived in the New York area, was sent home and came back at 3 months, um, and the CT was obtained, which showed this. Would anybody do anything other than a CT scan? So we had this discussion at our radiology conference last week and surprisingly, one of the radiologists actually suggested why get a CT scan. If you can see it on the plain film, you know, it's there. What is, what, how does the CT scan change the fact that at 3 months or 64 months, whatever your cutoff is, that you would just take it out? So why get a CT scan? Why not just get a plain film if it's still there, which presumably it would be, you just take them to the OR. I think, I think that's a good question. I mean, I feel very strongly about not getting studies. I don't know. I think that it, but when you're doing something that's a bit more complicated like this, and I think it can help a little bit in the planning. I think it's not always clear on the chest X-ray what lobe it's in. Parents like to know. They don't want to just have you say, Well, I'm going to go ahead and take out one of the lobes of your children. I'd like to know what lobe it's going to take out, how much of their lung. What are other issues or, or indications, um, if you're concerned about a combined lesion, you know, is there a systemic vessel, but again, I always look for a systemic vessel, whether there is one or not. So I think you can make that, that argument. Um, I think that. I would tell you medical legally that if you went in and did this and you had a complication and you hadn't gotten a CT scan, that someone would get up and say you should have absolutely gotten a CT scan when you did it. I will argue somebody would get up and say anything if you have any complication. Somebody would argue the opposite too, and I mean my practice has been to get CT scans, but I think it's an interesting challenge I think. You find multifocal disease, because, because occasionally in a certain percentage of these kids you're gonna have multifocal disease and you want to know if you're dealing with more than one lobe or sometimes you'll have disease in all three lobes and that may change your management. Those, those are the complicated patients you sit there and say, well, do you take this out, see what happens in the other two. Do I take, you know, do I just watch them? I mean, I think that's a good point. I've, I've, I've had 3 patients. It, you know, in a few 100 that have had bilateral disease, so we had to alter their management based on. Yeah. Finding what appeared to be evidence of bilateral CPAPs. So, I think that's a very good point. So this scan was done at 3 months of age. Um, so how many in the panel would operate now? 3 months? Why wouldn't you operate 3 months? It's just so much easier when they're 9 months. Oh, absolutely not. It's so much easier. I teach you anything. I did it I did it. I mean, the size increases, the circumference of the baby's chest increases dramatically. I wouldn't wait past 9 months. I mean, we can talk about this, but there's no question, and we've actually published a side study now that shows the operative time is less, the complication rate is less when you do these patients earlier, but it's, it's because these, these, the thing with these kids is they have. They will have subclinical inflammation and infection. And when the difference between 3 months and 9 months, is the, the amount of inflammation in, in a fissure or the number of enlarged lymph nodes. Um, is significant and can make the procedure significantly more difficult. I mean, I agree if you wait years, between 36, and 9 months, you really think there's a major difference. Absolutely. Did the patient have any symptoms at this point? Patient's asymptomatic. Totally asymptomatic. Totally asymptomatic. Would anybody never do an operation? Would anybody just follow this kid? Jack Langer is not on the phone line. It'll be interesting to see, I mean, we have a very international audience. Yeah, I know. I mean, is this, and I think could we poll, if we do a poll question like do you do do you do you observe or do you operate, right? So the way I would phrase that, Mark, if we could do it on the fly, or who are we sending this to Zach or Jen or whoever, can we open a question saying, do, do you always operate on a congenital lung lesion, an asymptomatic congenital lung lesion? You want to put a size in there. Because if they're real small. Does that affect you? It certainly affects Jack, um, you know, is that right? Yeah. And, and, you know, they'll continue to observe very small ones. Todd, can I ask you a question? If the child's still asymptomatic, that's the X-ray. What's the indication to operate on the child's asymptomatic asymptomatic, you know, you're now at 6 months, you get that X-ray tell me, yeah, it is a setup. What is the indication to do an operation? Well, to be the devil's I don't remember if you were on that faculty. This is the debate that is my favorite film to watch between Alan and Steve fighting with Jean Martin and Jack, but the risk of infection, the risk of cancer. What's the risk of cancer in that? Well, we're going to get to that. All right, so I think, you know, that's. That's that's the debate and that's the question around the world is do you need to operate on these if this was a little bit smaller or if there was an issue. So while we're getting the poll, actually I just for the, so we have the answer is 62% always operate on asymptomatic lung lesions, but it keeps increasing here now it's 70% always operate on asymptomatic lung lesions, 30% do not. Can we have the video? So I, this, this is just, I think we have time. It's a quick 3-minute video. I thought the audience, this is this child's uh uh thoracoscopic lobectomy. So this is a 3 month old, um, 5 kg baby. So there's a huge amount of space in here. I mean, the space is not an issue. These are 3 millimeter instruments. This child ended up actually having an unseen sequestration associated with it. You can see the sequestration sitting down. Along the uh lower lobe right at the inferior pulmonary ligament, um, this is, this kid had an incomplete fissure and already what you'll see at three months as we open up the fissure and We're using ceiling technology here to open it, but you can already see the enlarged lymph nodes. You can see that large lymph node right in the major fissure, and I will tell you I've seen a number of kids who are totally asymptomatic, 9 months to 1 year of age. You get in there and the lymph nodes are massive, and the inflammation in the fissure is massive, and it's not that you can't do the operation, it's just it makes it more difficult. In this kid, there's larger lymph nodes, but it's easy to dissect. Samir Pandia is anxiously waiting to see, are you going to take it. With a 5 millimeter stapler, you're going to take it with a clip. The bronchus. Well, in this particular case, I did not have the 5 millimeter stapler. That's what I've done. I've done since when I did this case. The stapler wasn't available yet, but this is a 3 millimeter sealer, and you can see how easy it is to dissect out all the vessels. There's no inflammation. This operation with the fellow. Assisting and doing about half of it took about 90 minutes and the vessels are small, so the sealer will take vessels, 3 millimeter sealer will take vessels safely up to 5 millimeters. So all these vessels fit within that, so we're able to just seal them and divide them easily. The dissection is easy and again I'll just tell you. Out of pure volume and experience, when you wait and do even these asymptomatic kids around a year of age, this dissection becomes much tougher because there's a, there's often a lot of inflammation in it. You will ask the parents and they say they never had had so much as a cold, let alone a chest infection or anything they needed to deal with. That's not every case, but it certainly happens. Uh, what we're doing here is just because we didn't have a 5 millimeter stapler, so this is a superior segmental bronchus to the, to the, um. Lower lobe, and we're taking that separately and then we take the basal trunks. Um, and in this case, again, at this age, you can safely use clips. It's just like using, uh, you know, if you go to the segmental, um, level and take them, uh, and then, um, Um, and I generally will take in a lower lobe, we'll take the bronchuss first, and then that just leaves the, the vein exposed, um, and makes it, uh, quite easy to get at. Um, these are, you can see, if you're used to doing endoscopic surgery in babies, there's a huge amount of space, and, and it's, and movement is not a problem. It's all about the setup, and there's the vein being exposed. Um, and we'll just seal that and take it as well. Um, at the end of the case, we simply morsellate the specimen through the trochar site, so we take it out, uh, piecemeal. Um, it's hard to get any kind of bag in here, especially with a specimen that's big. That is one of the difficulties. So, I would say that when I trained. I did the same thing that you described, waiting a little longer. When I went to Steve's place. I thought it was going to be a Steve-ism, like, oh, you could do it at, you know, 3 months, and I thought that's just because Steve could do it at 3 months. But the truth is this does not at 3 months does not feel like a limited space operation. I think that at 3 months you get good lung collapse. It's, I never felt like I wish I had more space, but the vessels are small. It's a nice, easy dissection, so I actually am a believer now. And that it's easier to do it when they're smaller. And sure, I mean, I didn't have a lot of problems when I waited to 9 months. I just felt comfortable. But if it's easier, I'm not going to mind that. And you know, Dan Elsley on, on the chat also said it's, I'll quote him here, it is not even comparable. 3 months is way easier. So it sounds like there's, that's fine with me. If something's easier, I'll take it. So, so let me ask Benno or or Philip or anyone else on the virtual faculty, does anyone Have any, uh, do you guys do things differently, especially the European guys. Uh, do you, are you more the, uh, the, the watch and wait or would you operate on these kids? We had extensive discussion in Europe also about this, and there are different approaches. The Netherlands, the attitude is much more conservative than in Germany, for example, and we in my place, we operate 3 to 6 months of age, any kind of this lesion, any kind of cystic lesion, because of potential malignancy later on. And we also think that the operation is most easily done when the patients are about 3 to 6 months of age, as Steve says. So there's not much difference to that. I knew I liked Benno for a reason. Philip, do you do anything different in Lucerne? No, we use the same devices and we have tried just to comment on the stapler. We have tried the stapler. It just didn't work properly. Oh, wait, wait, wait, you know, be very careful about this. You're not talking about the same stapler I am, so I know there are different devices, but just a comment to be cautious with any device or stapler. I agree, but it's a different stapler. What did the upper lobe look like when you got done? Was that normal? Yeah, it looked totally normal. What would happen if you saw in the lower part of the upper lobe? What would you do? So that's happened occasionally. If I could do a segmental resection at the same time, but I, I, I would do an anatomic segmental resection, and I think that's key. And we printed up a series, um, uh, that uh. We got, we printed up a series of segmental resections and we're following those in cases that on CT scan and at the time of surgery looked to be limited to just an anatomic segment, uh, but one child has shown evidence of recurrent disease, cystic disease, um, and, but that, but I am concerned and I've had a case where I went in. And I thought it was a lower lobe CPAP, and the child ended up having no fissure. I mean, it was just one giant lobe, and I had to do kind of a bi-segmental resection, and that child has had recurrent cystic disease, but I couldn't do anatomic resection. So I don't know that I can recommend that to everybody, but I think it is important to to keep in mind. I just want to, can we go back one slide? Is Mark, we go back the clickering. Yeah, no, no, goes forward there. So I just want, so, no, go back. There you go. So the surgery was uncomplicated. The chest tube was removed on post-op day 2. could have been removed on post-op day 1. The child went home on post-op day 3. But what's interesting here is that the path is that it showed lung tissue with cystic congenital pulmonary airway malformation, type 1 and type 2 changes, but with exuberant neoplastic mucinogenic proliferations and bronchial margin is negative, and this is a finding that I had actually not seen reported before. Um, I will tell you, in my, I've done about a little over 300 lobectomies for cystic lung disease. Some of those have been out of the country, so I don't have really good follow up. I've had two pulmonary blastomas and one adenoCA that, that I know of. But this is a new finding, and this is, um, maybe associated with the CRAS mutation, which is, um, also a marker in adult small cell, um, carcinoma. As well as colon cancer, um, and now this is a Colombia and they're going back and review it and they found 4 others with this. So we get into the discussion about, um, you know, when and why to operate. So I believe the literature fairly clearly shows that 30-40% of these kids who have this kind of cystic lung disease will have a significant pulmonary infection at some point during their life. Um, and that's often how we used to diagnose these before it was prenatally diagnosed. Once these lesions become infected, they're much more difficult to do that. This, I think, and, you know, I think all these operations personally should be done thoracoscopically at this point. Getting to your point is, you know, there are many good centers who do that now, and if you can't do it, maybe you ought to send them to a center who can because I think avoiding the morbidity of a thoracoscopy, a thoracotomy. Um, especially when you do it young, young in life, the one thing we don't really have are good pulmonary function studies that show long term that we've actually, we need to do that to document that if you take a low bout, we all say if you take a low bout, it's an infant, that the rest of the lung will grow, they'll compensate. They really don't have any significant disability from that, but we should do the studies to, to show that. But if they become infected, they're much more difficult. Um, and then I think, uh, this risk of malignancy and now with this possibility of a new mutation being identified, so in my personal series incidence, it's almost 2%, certainly 1%, um, but it may be 2%. So I, I kind of opened up the discussion about real quick, real quick discussion. I think that that actually I just like to ask you a little bit about that because I think um. If one of the indications for doing the lobectomy is risk of malignancy, which I agree with, then Um, the fact that you're morselating the tissue to pull it out raises some concerns, and are those things a contrary, are those sort of competing interests? Maybe should we not be morselating? Should we be putting them in a bag, right? So it's a good, it's a good point. So in the cases that we've done, the the, the hematologist, oncologist has said by doing that, we've not upgraded the tumor and that it doesn't change the treatment and nobody's getting chemotherapy or anything else, but we are watching those kids. But it is a reasonable point. You know, should we, now, it's hard to know because you never know. You don't know those kids. It would be difficult in, in a lot of these kids to get that specimen in a bag, um, especially if you're doing them early like we are. Um, that is, but it's a, it's a good point. Steve, what's the, uh, you said you saw 3 tumors in 300 cases. So that's much less than 1% incidence of cancer. It's 1%. It's 1, you know, 3 of it's, it's 1% incidence. But if these mutations, you know, if this is now I'm including these cases now, 4 cases that I know of that have this may have this CRS mutation, and there needs to be more work done. But again, it's the morbidity of the procedure versus you take that plus a 40% chance of infection versus the morbidity of the procedure in the long term, um, issues associated with it. But, I mean, that's where the debate comes in, right? That's where. How, how old were the kids, when they had the tumor, when you did them? Were these all kids under three months of age who had the tumor? These were all kids. Um, the, the kids, the blastomas and the adeno were all under a year of age. So let's, we can continue this conversation online. We're gonna, that's a great, uh, great job Steve. Thanks.
