Jean Martin or or or Jack. What are your thoughts on the whole thing that was discussed about, uh, pretty much the, or the management of a, of an asymptomatic lesion? Do you agree with what was said in Alan's talk? Yeah, I agree with him on most points. I think Jack is probably burning the tug, but will get his chance after he. We're having a little trouble with the fire alarm here. I hope it's run, run, run. One point I want to make that maybe Alan said but was not that clear from his slides is most people believe now that C cams do not become PPB, but PPB is a de novo tumor. It's just cystic and you cannot differentiate it at all on imaging. And there are several cases of even prenatally diagnosed, uh, that turned out to be PPB after, and we had one case recently. So I think the main thing is not that TCA may become PPB, but what you're looking at could be a PPB and you cannot distinguish. PPB has some features, um, and there's a nice summary. I, I would send people to this, uh, June issue of, uh, clinics in perinatology. Uh, there's a good review on that, and you'll see that, It's more than just a couple of case reports of malignancy. There's more than that. There's one series actually out of Toronto that estimates that cystic lesions that look like CA, about 4% of them will actually turn out to be PPB. There's other risk of bronchiolalveolar car. But that's a bit later on in the teenage years or early adulthood, and there's about a 1% risk. Those are really CA becoming bronchialveolar carcinoma. So I think it is a real risk, and if you decide to observe and not operate, you should inform people properly. I think PPB should be registered. There's a registry you mentioned Jack Priest in St. Louis, so I think it's good to have these cases reviewed and have them in a central registry because there are not so many of them, and we keep learning more about it. If you do them by thoracoscopy, I would, uh, urge you, though, to put the specimen in a bag before you extract, because if you end up mushing up the specimen in little pieces and then it turns out that it was a PPB, I think you might regret it because there's a risk of, uh, recurrence, I think. Uh, one question I have for Steve. You mentioned, Steve, when you, uh, interrupted so gently, uh, Alan, that, uh, extra lobar sequestrations could become infected. Now for me, by definition, extra lobar sequestration, we're talking about the routine one that has no communication, no air in it whatsoever by CT scan. I don't think their infection rate is, is, uh, very high. Yes, they can get, uh, hematogenous infection like any other piece of tissue in the body, but that's pretty rare. And the same thing with malignant transformation of extra lobar sequestration. I mean, there's maybe one or two cases in the world literature ever described of a squamous cell carcinoma later on in life. So, I don't think, Infection and cancer is it a good argument to resect a non-communicating extralobar sequestration. I agree with Alan. They're nice cases to do, and if they're big vessels, you're worried about, you know, hyperdynamic state and all that, maybe you should take them out. But if they're small, small feeding vessels, and they're especially if they're intradismetic. I don't, I think messing up with the diaphragm just for the fun of taking it out, you might have more complications than you have of leaving it there. Well, I, I can't, I mean, I, I think the incidence of infection and malignancy, like you say, is very low, but it, it has occurred. Um, and I think that our imaging still is not perfect. And so, I'm not sure we can always be absolutely sure, uh, of the diagnosis, uh, and whether or not it's not a hybrid lesion. Um, the infra diaphragmatic ones, some of those we diagnose prenatally, and it's never been, in some cases, it's not clear whether it's a, a, um, Extra lobar sequestration or whether it's possibly some other sort of tumor and so because the morbidity of the procedure is so low, we've tended to remove those but you know it's certainly a point that is worth discussion in this meeting and seminar, but I'm just not comfortable leaving. Those masses, I certainly, I think if you know a lot of people are talking about, you know, you could argue, well, if you're absolutely sure it's it's a completely separate extra lobar process, whether it's in the chest or in the diaphragm, you can just watch it. Some people favor embolizing these things, and that's where I would strongly say that you know that to put an infant or a child through an embolization. It's much easier to go in and resect these, um, using minimally invasive techniques. And I think there's, there's no role for embolization of these lesions. Um, whether you would just leave them and sit, I wouldn't be comfortable with that, but I, I think it's certainly worth debating. Just, uh, I don't want to give the impression I resect all of the infra diaphragmatic ones. You know, the, the differential prenatally is adrenal hemorrhage or neuroblastoma. Right. Cystic neuroblastoma. Um, and you can generally, uh, uh, a small one I will follow by ultrasound because you can use ultrasound in that circumstance. And, uh, if they stay the same or get smaller, I don't worry about them. Uh, adrenal hemorrhage will evolve and you'll be able to recognize it. Um, so, I don't generally resect infra diaphragmatic ones unless they are causing symptomatology of some kind like the one that I, uh, demonstrated. I agree. The problem with neuroblastoma, I think, has been dispelled a little bit with the COG study that showed that it was safe to observe those, um, adrenal masses. Uh, I think it's safe. You have to watch them, of course, but you can observe. Right. The other thing that you mentioned was, uh, you know, with the, with the PPB issue and malignancy issue is, uh, you know, if you choose to follow them, then counsel the parents accordingly. The trouble is there's no good way to follow them, as you know. And, uh, the, the best method is CT scan, and that induces a certain incidence of malignancy in and of itself. And so, you really have limited ability to follow pulmonary lesions. And because you can't differentiate a CT scan from a PPB, uh, it doesn't really matter whether you follow them or not. You're not going to be able to differentiate any clearer until you have a stage two or three occurrence. So, It's, it's an area I don't think you can follow them in any kind of a reasonable manner that's going to prevent the incidence of malignancy or allow you to catch it even earlier than you would otherwise. I, I don't think you can counsel a family and say the word cancer and have them watch. I mean, that's just almost universal. That has not been a frequent request. Jack, you don't have any thoughts on this, right? We're waiting. Thank you. Go ahead. So I think, uh, when we talk about, uh, making a decision to operate on something or not, we have to always weigh the risks of not doing the operation versus the risks of doing the operation. And there's been very little discussion about the risks of doing the operation during this panel so far. The risks are low, um, especially in experienced hands, but there are still children who die from pulmonary lobectomy, particularly done, uh, thoracoscopically, and, uh, we can't ignore that before Jack, where you've just made a comment that I think you need to. I, I would, where's the data for that? Well, I know, I know of two cases, um, that I have been asked to review, uh, not in our institution, thankfully, but, uh, there are two that I know of that, um. That I was asked to review and I'm sure that there have been others as well that people don't report. Well, I, I just think, I, first of all, you shouldn't be doing a lobectomy thoracoscopically unless you're experienced. And I think in experienced hands, uh, thoracoscopic lobectomy should have no more morbidity than open. So, I just. Yeah. I worry about, I hear that comment all the time, and that bothers me. Um, you should not be doing this operation unless you have advanced thoracic and, and minimally invasive skills. And if you do, there should be, there, there should be, The mortality should be 0. Well, that's a, that's a bold statement, Steve, and, uh, I'm glad that, uh, your mortality is zero so far as is mine, but, um, I think we have to admit that you do enough of any kind of operation, you're gonna have significant complications, and that can be open orthochoscopic. So I can be open orthoroscopic. I, I'm, I'm talking at this point about operating versus not operating, and, um, I think. Uh, maybe, maybe you took exception to my sin, particularly using the thoracoscopic approach, but I think, you know, Alan described a situation where he had massive bleeding. I think if you have that kind of massive bleeding when you are already open, your chance of salvaging the situation is probably better than if you have that kind of bleeding in a thoracoscopic case. I can't back that up with data because fortunately there aren't enough cases, but I think that most of us would agree with that. Anyway, what I want, the point I'm trying to make is that, that there are complications and potential complications that can be serious to doing a lobectomy. And when we're talking about the benefits, we have to really look at what are the frequency of the bad outcomes from watching. And I think we, we all agree that that PPB can be indistinguishable radiologically from a CCA, but when you look at the incidence of CCA or CPAM and the incidence of PPB. It really is, is quite markedly different. Their PPBs remain extremely rare. The paper that Peter Kim, um, published in our institution that Jean Martin mentioned, um, was histological evaluation of, of these lesions in the using the new Stalker classification in which one of those classifications has been termed PPB, but we don't really know what the natural history of that histological finding is. What we do know is in a in a center like ours, which is a pretty high volume center, we see PTB coming de novo extremely rarely, like once every 3 or 4 years. So, and we see probably 20 or 25 new cases of CCA every year. So I think the incidence of cancer remains extremely low and has to be balanced against the risk of the operation. The risk of infection, uh, we, we have published on that, and the estimate that we came up with based on our data was somewhere around 20 or 30% lifelong risk of infection, which is not insignificant. But the question is, should you put every patient through a lobectomy to avoid the risk of a 30% risk of infection, where most of those infections can be treated and the lobectomy can be done afterwards. I think, I think that's a questionable figure, Jack. What the 30%. Well, that's the only thing. Nobody, nobody really knows that number. The only prospective study that followed patients for a long period of time, it was a small study, uh, showed 18 of 21 asymptomatic patients developed symptomatology. Uh, during that, uh, interval of anywhere from, I think the average was 2 years up to 13 years after their entry into the prospective observation study. So, that, that number is questionable. Uh, I don't know what it. I'm not claiming to know what it is, but I don't think you can, uh, state that you can absolutely say that a lifetime risk of infection with a CCA is 30%. I believe it to be higher than our data was 10% with a mean follow-up of 4 years, so we tripled that, right? Yeah, but we, we tripled that and we, we, we tripled it just. Come on, I don't know that there's any evidence that I didn't, if it was only 4 years follow up, you know, and you live 70 years, maybe you should have, uh, multiplied by 35 or no, sorry, 15, maybe, OK, maybe I, I, it remains, which would have given you an over 100% instance of. You know, the thing is that, uh, we, we now know how common these lesions are because we see them so frequently prenatally, and in the days before prenatal diagnosis, it was not that common for people to come in during their teenage years or even in the adult thoracic surgery units to come in with symptomatic infected, uh, secams. It happened from time to time, and the adult thoracic surgeons knew it existed, but we weren't seeing. The kind of frequency that we would expect if every one of those C cams that was asymptomatic and had not been picked up prenatally had, had just been followed. So we can argue you're not older than I am, right? So you're saying, OK, we've both, uh, grown up in the prenatal diagnostic era, and even early in my career, I remember doing sea cams that were infected um. Several occasions in my first few years in San Francisco, it wasn't a rare event to see a sea cam that was infected. I'd like to see some data on what you just stated. Well, let's, OK, I mean, you know, what, what was the frequency of infected se cam surgery back before prenatal diagnosis? I've never seen any, anything that would give me a number for that. Well, would you accept that if, if we see, let's say for the sake of argument, we see 25 new prenatally diagnosed asymptomatic cases a year in the Toronto area, that you would expect if, if none of those were operated on. And, and let's make the assumption that all of those patients stayed in the Toronto area. We should be seeing 25 infected cases. We should, all of those should come back if it's 100%, should be coming back infected. So shouldn't we be seeing 25 cases a year? Of infected depends on the time depends on the time course and the frequency, right? So suppose it's only 50% within the 1st 10 years of life, all right, you're gonna see a low number on a yearly basis. John Martin, you had a comment. Yes, I was going to say to Jack they probably go to Hamilton to get operated. Exactly. Well, the other thing is I think my point, as it turned out, I worked in Hamilton, you're, you're really reaching here with speculation, Jack. No, but my point also, it's true that we don't have a long term cohort of patients to know the exact incident. The only thing that's out there in the literature that people keep quoting is actually a letter to the editor saying, oh, we're following 100 patients and only a couple have got into trouble, but there's no data, there's no, you know, there's nothing there's a pretty good paper. It's a small number of patients. It's by Wong. It was published in Pediatric Surgery International a few years ago that followed 21 patients that were 8 of them were prenatally diagnosed. The rest were picked up serendipitously and were asymptomatic. And of those 21 patients, 18 of them developed. Uh, infections or other symptoms requiring resection. But the counterpoint, Jack, to the fact that you don't, you don't know how many get infected early, there's several old autopsy series of. Cases and we know for example that a small asymptomatic extra lobar sequestration is a relatively known finding at autopsy. It's something you can see just like in situ papillary carcinoma of the thyroid, but a C cam on autopsy series, asymptomatic C cams, it's just nonexistent, so they present. Later on with infections with different things, but I think the majority do become symptomatic. It's just not a normal variant. Of your 25 new, what you call CCAs per year prenatally diagnosed, are they really all cystic lung lesions confirmed by CT postnatally, or are they some, you know, echogenic lesions that then disappear and there's nothing left? No, we follow them all. We, we CT them all, and, uh, the, and the ones that, uh, we don't operate on, we, uh, we follow with chest X-ray and then with another CT, and that is, as Alan suggested, uh, is also problematic, the, the follow-up of these patients. I, I also want to make it clear that we are not advocating non-operative management of all CCAs. Um, I mean, I, I'm often put into the position of, of taking that side in this argument, but When I talk to these families, I am very clear about what the risks are, and I mention the word cancer every time, and many of them, as you've said, as soon as they hear the word cancer or even as soon as they hear, um, the, the word infection, um, they say, you know what, I don't want to live with that risk. I'd rather have the, the very low risk of the lobectomy. And, uh, and so I actually do quite a few thoracoscopic lobectomies for these lesions. All I'm advocating is That we give a balanced approach to the families and allow them to decide and not, I, I don't believe that we should be taking an approach where every single one needs to have an operation. OK. Um, and just to, to accentuate that point, you know, there's a comment from Ramesh who, uh, is unable to call in but that they, they selectively decide who to operate on based on resources, based on the family, the patient, and they don't have a, uh, an, uh, an, a dogmatic approach of, of operating on everyone, and I think that's an interesting point that it depends on, uh, also the, the patients, the families, and the resources and the location. I was actually very interested to see the little poll that you put up there a few minutes ago that 25% of people do not advocate uh routine reception. I was surprised by that. Here, um, let me pull that poll up. We actually still have it live. Um, it seemed like it was about, was it 30% that did not, uh, Jack, I'm gonna pull it back. It looked like around 25%, uh, the last time I looked at it. Here's, let's see, it was, uh, 76 to 23%. Um, well, I think this brings up the point that, that, uh, Doctor Langer mentioned about the fact that there are, there is a hidden mortality with a lot of what we do because people don't report bad results. The only way you know about them is through lawsuits and the like, and I guess the 10 years from now if we all participate in the American College Pediatric NQuIP. Where it is unbiased, there'll be large amounts of data, we'll have some idea about thorascopic surgery and open surgery and how often the complications and the death really occur. So, I think a lot of this is, we don't have the data to really make an informed consent. So, we have to give the families the whole. Um-hum. The, the whole picture and let them decide, which is a tough thing for them to do. Yeah, and I, and I, it obviously depends on your perspective and the volume of these lesions that you see and your comfort, uh, with, uh, treating them. I, You know, doing thoracoscopic lobectomy shouldn't be taken lightly. It's, it's, it is a, you know, a major investment in, uh, time and learning to become proficient at it. And, but I do think, uh, centers that do these procedures routinely and see a volume of CCAs can treat them with an extremely, extremely low morbidity, and no mortality. There's always a potential mortality out there somewhere, but. You know, it's amazing how well, uh, infants do. They're generally in the hospital for two days and they go home and I've had, we just reviewed the last 100 thoracoscopic lobectomies that I've done, lobectomies, not sequestrations or otherwise. And, uh, there were two transfusions and two latent pneumothorases, and that was the only, those were the only complications. So, we're, we're actually running over, so, I want to try to do rapid,
