Case Based Journal Review - CPAM in 2022

Published: 2022-04-12

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Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Ellen Ancisco — host
Rod Gerardo — host
Jose Campos — guest
Speaker 4 — guest

Chapters

0:05Introduction and Case Presentation — Introduction to the podcast and presentation of a case involving a 30-year-old G2P1 with a left lung mass discovered on 24-week ultrasound. Focus is on prenatal counseling for asymptomatic CPAM.
1:35Risk of Becoming Symptomatic — Discussion of natural history study showing most children born asymptomatic remain so. Review of prospective data with 10-year median follow-up showing 0% of conservatively managed patients became symptomatic, contrasting with retrospective studies showing 3-85% risk.
3:57Malignancy Risk: Prenatal vs Postnatal Diagnosis — Analysis of Midwest Pediatric Surgery Consortium study showing 0% malignancy in 344 prenatally diagnosed lesions versus 8.7% (15/177) in postnatally diagnosed lesions. Discussion of pleuropulmonary blastoma registry data showing only 9 cases worldwide in prenatally diagnosed lesions.
6:49Impact of Prior Infection on Surgical Outcomes — Review of study comparing thoracoscopic resection outcomes with and without prior pulmonary infection. Prior infection associated with longer operative times, more postoperative fevers, and increased antibiotic use, but no difference in major complications or conversion rates.
9:03Optimal Timing for Elective Resection — Discussion of study comparing three age groups (1-3 months, 4-6 months, 6-12 months) for elective resection. Youngest group had shortest operative time (115 minutes vs 163 minutes) but higher thoracotomy rate (40%). No differences in major complications, conversion rates, or readmissions across groups.

Key claims

1:44Most children with prenatally diagnosed CPAMs are born asymptomatic — Speaker 4
2:30A prospective study followed prenatally diagnosed congenital cystic lung lesions for a median of 10 years — Ellen Ancisco
2:3857% or 68% of patients in the prospective study were managed conservatively — Ellen Ancisco
2:43Of the conservatively managed patients who were followed up, none became symptomatic — Rod Gerardo
2:49Respiratory compromise at birth from CPAM is very unusual, not seen in 20 years by one speaker — Speaker 4
2:58The argument for early resection is that CPAMs will go on to cause pneumonia, pulmonary abscesses, and potentially cancer — Speaker 4
3:37Retrospective studies show a huge range of 3% to 85% for risk of becoming symptomatic — Jose Campos
3:37Systematic reviews report symptomatic rates of 3.2% in one review and 25% in another — Jose Campos
4:36Out of 344 prenatally diagnosed lesions, none had malignant pathology when resected — Rod Gerardo
4:47Of 177 children with postnatal diagnosis of CPAM, 15 (8.7%) were classified as having a malignant tumor — Ellen Ancisco
5:49The International Pleuropulmonary Blastoma Registry has 350 cases reported worldwide and found only 9 cases of prenatally diagnosed lesions that turned out to be pleuropulmonary blastoma — Jose Campos
7:18Patients without prior pulmonary infection had shorter operative times — Ellen Ancisco
7:18Patients without prior infection had fewer postoperative fevers and less need for antibiotics postoperatively — Ellen Ancisco
7:37There were no significant differences in postoperative complications between groups with and without prior infection — Ellen Ancisco
7:50There was no significant difference in conversion rate from thoracoscopic to open between groups with and without prior infection — Rod Gerardo
8:17After infection, there is a higher rate of conversion from thoracoscopic to open surgery — Jose Campos
8:25The number of patients who will have an infection is so low that the benefit of lesser conversion rate does not justify preemptive surgery — Jose Campos
8:38If you only operate on those that get infected, you will miss those that get pleuropulmonary blastoma without a pre-existing infection — Speaker 4
9:32Operative time increased with each increase in age group — Ellen Ancisco
9:39At younger ages, the tissue planes are easier to dissect — Ellen Ancisco
9:50Between 1 and 3 months, operative time was 115 minutes compared to 163 minutes in the 6 to 12 months group — Ellen Ancisco
10:08There were no differences in major complications, conversion rates, or readmissions between age groups — Rod Gerardo
10:28The 1 to 3 month group had a 40% thoracotomy rate — Jose Campos
10:42The complication rate of operating on a perforated appendix with an abscess is the same as when doing an interval appendectomy, according to Kansas City data — Speaker 4
11:13Prophylactic operations should have nearly zero complications — Jose Campos

Cases discussed

0:4530-year-old G2P1 with left lung mass discovered on prenatal ultrasound

Points of disagreement

5:21Management of prenatally diagnosed asymptomatic CPAMs
- Jose Campos: Would find it very difficult to operate on prenatally diagnosed asymptomatic CPAMs, but would find it very difficult not to operate on postnatally diagnosed lesions
- Speaker 4: Does not understand why anyone would wait to operate if they are someone who removes these lesions, and does not understand the argument against early intervention
8:17Justification for preemptive surgery based on infection risk
- Jose Campos: The number of patients who will have an infection is so low that the benefit of lesser conversion does not justify preemptive surgery in all cases
- Speaker 4: Operating only on infected cases will miss pleuropulmonary blastomas that occur without pre-existing infection; screening and removing all lesions avoids this but results in many unnecessary lobectomies
10:42Interpretation of equivalent complication rates across age groups
- Speaker 4: Even if complication rates are the same, surgeons prefer to do cases that are easier and less stressful, so should operate when it is easiest
- Jose Campos: If doing an elective preventive resection, it should be easy and have nearly zero complications; prophylactic operations should be close to zero complications

Open questions

What is the true risk of asymptomatic prenatally diagnosed CPAMs becoming symptomatic, given the wide range (3.2% to 25%) reported in systematic reviews?
Should all prenatally diagnosed asymptomatic CPAMs be resected prophylactically, or is conservative management with surveillance appropriate?
What is the optimal age for elective resection if surgery is chosen, balancing operative ease against thoracotomy rates and institutional expertise?
How should the 0% malignancy rate in prenatally diagnosed lesions versus 8.7% in postnatally diagnosed lesions inform management decisions?
Does the low infection rate in conservatively managed CPAMs justify waiting for symptoms before operating, or does the increased surgical complexity after infection warrant earlier intervention?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Prenatally Diagnosed Asymptomatic CPAM: When Identical Complication Rates Don't Settle the Question

The points where the speakers disagreed, with each position presented side by side. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Points of disagreement · AI-written, human-reviewed

The Central Question

A prenatally diagnosed CPAM sits unchanged on serial imaging. The child remains asymptomatic. Do you operate now, wait for symptoms, or never operate at all? The discussants agree on the natural history data — most children with prenatally diagnosed CPAMs are born asymptomatic 1:44, and in one prospective study following these lesions for a median of 10 years, 57% were managed conservatively and none of those followed became symptomatic [c2,c3,c4]. They also agree that respiratory compromise at birth is vanishingly rare 2:49. Where they diverge is what to do with that information.

The Case for Selective Intervention

One discussant draws a sharp line between prenatal and postnatal diagnosis. Out of 344 prenatally diagnosed lesions, none had malignant pathology when resected 4:36. Of 177 children with postnatal diagnosis, 15 (8.7%) were classified as having a malignant tumor 4:47. "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" [q6]. The International Pleuropulmonary Blastoma Registry has 350 cases reported worldwide and found only 9 cases of prenatally diagnosed lesions that turned out to be pleuropulmonary blastoma 5:49. These are almost two different diseases — prenatal is 0% malignancy, postnatal is 10% [q7].

On the question of infection risk justifying preemptive surgery, this position holds that the number of patients who will have an infection is so low that the benefit of lesser conversion rate does not justify preemptive surgery in all cases 8:25. The argument acknowledges that after infection there is a higher rate of conversion from thoracoscopic to open surgery 8:17, but rejects using that as grounds to operate on everyone. "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" [q9]. The standard for prophylactic surgery is near-zero complications 11:13, and if you cannot meet that standard, the intervention is not justified by the low event rate.

The Case for Early Intervention

The opposing view does not contest the natural history data but questions the logic of waiting. "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." The argument for early resection is that CPAMs will go on to cause pneumonia, pulmonary abscesses, and potentially cancer 2:58.

On the malignancy question, this position points out that if you only operate on those that get infected, you will miss those that get pleuropulmonary blastoma without a pre-existing infection 8:38. The way to avoid that is to screen everyone and remove every lesion, which results in many unnecessary lobectomies but eliminates the risk of missing the rare malignancy. Even if the absolute numbers are low, the consequence of missing one is catastrophic.

On timing, even when data show no significant differences in postoperative complications, conversion rates, or readmissions between age groups 10:08, this position argues that surgeons prefer to do cases that are easier and less stressful. Operative time increased with each increase in age group 9:32 — 115 minutes between 1 and 3 months compared to 163 minutes in the 6 to 12 months group 9:50 — because at younger ages the tissue planes are easier to dissect 9:39. The comparison is made to perforated appendicitis: the complication rate of operating on a perforated appendix with an abscess is the same as when doing an interval appendectomy 10:42, but surgeons still prefer to do the operation when it is easiest.

Where They Agree

Both positions accept that prenatally diagnosed asymptomatic lesions have an extremely low malignancy rate and that most will never cause symptoms. Both acknowledge that retrospective studies show a huge range of 3% to 85% for risk of becoming symptomatic 3:37, and that systematic reviews report symptomatic rates of 3.2% in one review and 25% in another 3:37. Both agree that patients without prior pulmonary infection had shorter operative times 7:18, fewer postoperative fevers and less need for antibiotics postoperatively 7:18, though there were no significant differences in postoperative complications 7:37 or conversion rates 7:50 between groups with and without prior infection. Neither disputes the technical facts.

What Would Resolve It

The discussants did not identify a specific trial or dataset that would settle this. The disagreement is not about missing evidence but about how to weigh known risks — the very low rate of malignancy and symptoms against the morbidity of prophylactic lobectomy in infancy. One side sets the threshold for prophylactic surgery at near-zero complications and argues the current data do not meet it. The other side argues that even equivalent complication rates justify earlier intervention when the operation is technically easier and eliminates all downstream risk. The question remains unresolved.

Takeaways from this story

Prenatally diagnosed CPAMs have 0% malignancy in 344 cases; postnatally diagnosed have 8.7% malignancy — these may be different diseases.
Prophylactic surgery should have near-zero complications; if infection risk is low, conversion rate benefit may not justify operating on all.
Operating at 1-3 months takes 115 minutes vs 163 minutes at 6-12 months with no difference in complications or conversion rates.
Waiting to operate only on infected cases will miss pleuropulmonary blastomas that occur without pre-existing infection.

Topic overview

A case-based literature review discussing management of prenatally diagnosed congenital pulmonary airway malformations (CPAMs). The discussion examines four key clinical questions: the likelihood of asymptomatic lesions becoming symptomatic (ranging from 3.2% to 25% in systematic reviews, with one prospective study showing 0% in conservatively managed cases over 10 years), the risk of malignancy in prenatal versus postnatal diagnoses (0% in prenatally diagnosed lesions versus 8.7% in postnatally diagnosed lesions), the impact of prior infection on surgical outcomes (shorter operative times and fewer postoperative fevers without prior infection, but no difference in major complications), and optimal timing for elective resection (1-3 months showing shortest operative time at 115 minutes versus 163 minutes at 6-12 months, with no difference in complications). The speakers debate whether prenatally diagnosed asymptomatic CPAMs warrant prophylactic resection.

Key takeaways

Prenatally diagnosed CPAMs have 0% malignancy risk vs 8.7% in postnatal diagnosis, supporting conservative management. (4:36)
In prospective follow-up, 0% of conservatively managed prenatal CPAMs became symptomatic over 10 years median follow-up. (2:30)
Prior infection increases operative time and postop fever but not major complications or conversion rates. (7:18)
Elective resection at 1-3 months has shortest operative time (115 min) vs 6-12 months (163 min) with no difference in complications. (9:50)

Keywords

congenital pulmonary airway malformations CPAM respiratory distress syndrome pneumonia pulmonary abscesses pleuropulmonary blastoma perforated appendix thoracoscopic lung resection lobectomy thoracotomy interval appendectomy antibiotics Cincinnati Children's Hospital Pediatric Surgery Society of Chile Midwest Pediatric Surgery Consortium

Hashtags

#CPAM #PediatricSurgery #PrenatalDiagnosis #CongenitalLungLesions

Transcript

Click "Show Transcript" to view the full text (11895 characters)

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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