OK. Welcome back to the Stay Current podcast. We are doing another recent literature review with Doctor Jose Campos. Yes, on congenital pulmonary airway malformations or CPAMS. Did I get that right, that acronym? Yes, I'm Rod Gerardo and I'm Ellen Ancisco, a research resident at Cincinnati Children's Hospital. Jose sent us another list of amazing articles that we're gonna give to you in a case-based discussion. So let's go ahead and jump into it. Jose does it with a whole team, Pediatric Surgery Society of Chile. They help him pick out all these articles. We're gonna give a link below to their social media. You can follow them. They have some great content. So, this time, we're talking to a 30-year-old female, G2P1. She's coming into the clinic after her 24 week ultrasound revealed a left lung mass that looks like a heterogeneous mass that hypocoexists. And in this case, we're not going to review the differential diagnosis. We've talked about CPAs on a prior podcast, but really this time, we're gonna focus on the conversation we're having with the mother prenatally about this finding and um what to do about it or, or how to think about what to do about it. This baby was delivered and it's completely asymptomatic, but because there is a, a case, uh, a small percentage of patients who, who will present with respiratory distress syndrome and, and those Of course, we're not discussing whether they're getting an operation or not. So this, this patient is diagnosed prenatally and remains asymptomatic. Let's say you're having this conversation prenatally, and the mother asks, how likely is it that this patient, that this child is going to be symptomatic of this lung lesion? We usually have had that conversation before the child is born, but most children are born, born asymptomatic. I was always told that. This should be operated because the risk of them becoming symptomatic was pretty high. Well, we got to study for that. OK, so this article is the Natural History of prenatally diagnosed congenital cystic lung lesions. It's a long-term follow-up of 119 cases. It's from 2017 and is published in Archives of Disease in Childhood. And we're gonna give you the link to this article below the media player. Go ahead and click, open it up, read along with us. Some of these children are born asymptomatic, go out throughout life, asymptomatic. What are the numbers here? And that's what this is trying to figure out. Yeah, and this was a prospective study, which I don't think there are many of for regarding CAMs. They are followed up for a median of 10 years. They actually managed 57% or 68% of the patients conservatively. And, and of that 57%, the ones who were able to follow up, none of them became symptomatic. So this is talking about not symptomatic at birth, like respiratory distress. We're talking about do they get respiratory compromise at birth? which I haven't seen in 20 years, it's very unusual, or are fine at birth, which is usually the case, and then the argument to resect it early is that they will go on to get pneumonia and pulmonary abscesses and potentially cancer. So, the, the good thing about this study is that I think most serious that support operative management are retrospective, but actually this is the, the only one I found that is. Completely prospective. And also the only one that deals with the issue of short-term follow-ups. The question that we actually originally asked was how likely is it that the patient will become symptomatic. I know you get a huge range from 3 to 85% on retrospective studies, but all of their biased, but when you get to a systematic review of mess, actually one of them comes to the number 3.2% and the other one comes to the number 25%. That's still a huge raise. I guess also worth noting, we're gonna give you links to other articles that we don't even talk about, like these ones, they'll be under the media player. OK. So the second question that we need to talk about with the patient's mother is this risk of malignancy. This article is called Pleuropulmonary Blastoma in Pediatric Lung Lesions. It was published in 2021 out of Pediatrics, and it was a study done by the Midwest P Paediatric Surgery Consortium. Does the lesion at the time of birth have malignancy in it that we need to take out? Is there a risk of malignancy developing in the future? They retrospectively looked at The histology of 521 lung lesions that were resected across the 11 children's hospitals in the consortium. Out of the 344 prenatally diagnosed lesions, actually, none of them had malignant pathology when they were resected. Right, but then of the other 10, 177 children who had postnatal diagnosis of a CPM, then 15 or 8.7% of those Were classified as having a malignant tumor. Basically what they're saying is that the risk approached 10% for lung masses that were diagnosed postnatally, 10%, which is probably too high of a percent for most parents and surgeons. So Jose, does this change your management then if it's postnatal, that doesn't really tell us much. No, I'm surprised by the numbers, but it does not change. Change my management. To me, my opinion is that prenatally diagnosed asymptomatic, I would find it very difficult for them to give them an operation, very difficult, and postnatally diagnosed, I would find it very difficult not to do an operation. These are almost two different diseases then, right? So prenatal is 0 and postnatal is 10%. These are two different populations. These are two different things. The International Pulmonary blastoma Registry has 350 cases reported worldwide. They, they have the most, the, the largest collection of cases worldwide, and they've only found 9 cases of uh prenatally diagnosed lesions that actually turned out to be pleuropulmonary blastoma. So Jose, what do you not In prenatally diagnosed lesions, do not do any, do not resect them. The common practice in Chile is to resect them more, particularly in my hospital. We've had the discussion. We've, we've read all of this, and we are currently, as I said, having the discussion with the parents, but actually the, the acacademic discussion we laid on to them, it's more uh not to. Have an operation. We said the CPAM was diagnosed prenatally. We talked about the risk of that child becoming symptomatic from that lesion. Well, let's say if we wanted to do conservative management, we want to leave it. What's the chance that it will then become malignant? And now we're going to say, could this become infected? And so this paper is asking the question, does that matter for when you go to do surgery? And so it's called thoracoscopic surgery for congenital lung malformations. Does previous infection really matter? And this paper was published in 2021 in the Journal of Pediatric Surgery. So this was a retrospective study of thoracoscopic lung resections for congenital lung malformations. They looked at about 90 patients. They divided the patients up between Whether or not they had had a prior pulmonary infection before surgery or not, and they compared operative characteristics and outcomes for those two groups, and they found that if you did not have a prior pulmonary infection, then the operative time is shorter. They had fewer postoperative fevers in that group, less need for antibiotics postoperatively, but they did not have any significant differences in postoperative complications. Right. Oh, I guess it's probably also was saying that significant difference in the conversion rate as well from thoracoscopic to open. Nothing new here to add to the argument other than the question is, do you operate or not? That's the first question. I would say, I really don't understand why anyone would wait. I don't, I don't understand the argument. If you decide you're, you're someone who who removes these, that debate I get, do I operate or not? If you're someone that removes these, I don't understand the argument why you wouldn't do it early. This paper says that after infection, you have more hootomies, like you have higher rate conversion, that, that's, that's demonstrated. It's being used as an argument for preemptive surgery to operate them all, and the number of patients that will, will have an infection. It's so low that you don't get the benefit of lesser conversion. If you only operate on those that get infected, you're gonna miss those that get PPB without a pre-existing infection. And the way to avoid that is to screen everyone and take out every single lung lesion, which has a lot of unnecessary lobectomies in a group of people that have a very low rate of having a problem. So, this is a very esoteric argument. There's no good or easy answer, but we're getting More and more data over time. We want to avoid malignancy, we want to avoid infection. So if you do decide to operate, then the next question is, when do you operate? Well, we have an article for that. It's called Optimal timing for Elective resection of asymptomatic and genital pulmonary Airway Malformations. It was published in 2018 in the Journal of Pediatric Surgery. I had to, to group up the patients less than a year of age into three groups, between 1 and 3 months, 4 to 6 months, or 6 to 12 months. And they looked at the differences between those three groups, about their operative time. And their outcomes. The operative time increased with each increase in age, which actually matches kind of what Todd said, is that at younger ages, the planes are way easier to go through. So between 1 and 3 months, they had the lower, the lowest operative time at the shortest operative time at 115 minutes compared to 163 minutes in the higher age group between 6 and 12 months. OK. So, like, you know, an extra, almost an extra hour basically. Almost, yeah. Almost, but luckily there were no differences in major complications, uh, conversion rates or readmissions between these groups. I think it's the strongest one that supports, supports earlier intervention because, uh, well, they, they showed the same complications. The surgery is actually shorter, thoraroscopy is less offered. So the, the 1 to 3 month group, they had a 40%. Thoracotomy offers. So I think it, it has to do with, with your own expertise. If you're able to perform a safe thoracoscopy. The same exact thing is true with a perforated appendix. Kansas City showed that the complication rate of operating on an, on a perf appy with an abscess is the same as when you do an interval pi. Even if the complication rate is the same, surgeons in general like to do a case that's easier and less stressful, right? So, I would want to do the operation. When it's the easiest to do. I, I see your point, but actually if you're doing an, an elective preventive resection of something. Uh, it, it should be easy. It should be easy. It should have nearly zero complications like prophylactic operations should be close to zero complications. I, I see the point in doing that. I think there's a lot of nuance you'll have. In this conversation with the patient's family, decision making, the surgical team, the institution that you work in, and hopefully, some of this literature can help guide you and help you make decisions with that family about Are you going to operate? When are you going to operate? Why are you going to operate? All of these things that families are going to want to know and could help ultimately end up with, hopefully, better outcomes for the baby. If you love this article review podcast, or if you don't like it, either way, leave us a comment or review whether you're watching us on YouTube, listening to us on Apple Podcasts, SoundCloud, Spotify, Stitcher, or if you downloaded the Stay Current Pediatric Surgery app. It's free. It's in the Apple App Store. It's in the Google Play Store. Download it today, but until then, I'm Rod. I'm Ellen. And remember, knowledge should be free, hey.
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