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Scott Webb (Host): Congenital lung malformations are not terribly common, but they are nonetheless concerning for parents. And my guest today wants us to know that if they're diagnosed and treated early, a majority of kiddos are going to be just fine. I'm joined today by Dr. Kathleen Dominguez. She's a pediatric surgeon with Carle.

Doctor, it's nice to have you here today. We're going to talk about congenital lung malformations and what does that mean exactly, and certainly rely on your expertise. So, let's start there, just have you discuss lung development and compensatory growth.

Dr. Kathleen Dominguez: Congenital lung malformations are a variety of different types of malformations that can occur in babies as they develop in utero. They involve everything from something called a congenital pulmonary airway malformation where there's an abnormal area of lung that is still connected to the bronchial tree, so still gets airflow, but causes a variety of problems with infection that can be recurrent or even severe as well as a risk of cancer. That's the most common thing we see in terms of types.

There's also something called a bronchopulmonary sequestration, that is similarly an abnormal mass of lung tissue, but it's not connected to the bronchial tree. It can also get infected or causing problems with blood flow. Over time, that can hurt the heart or cause fluid overload.

Other types of lung malformations are much less frequent, but include things like congenital lobar emphysema. Congenital lobar emphysema is abnormal lung tissue, where basically air trapping occurs. And then, the least frequent is a type of malformation where we actually have cystic lesions off of the major airways. Then, that can occur anywhere from the trachea to more distally into the lung tissue or even in abberant locations in the body.

Host: Yeah. And I'm curious, Doctor, you know, you say some are more common than others. I'm curious about how you sort of diagnose these malformations in utero. You know, how does this work exactly?

Dr. Kathleen Dominguez: You know, it's interesting in that these appear to be much more common than they used to be. But the thought by and large is that we're picking up so much more of them now because we do routine prenatal ultrasound in the vast majority of pregnancies. The current incidence overall for congenital lung malformations is about four in 10,000 patients. But the most common of those is the congenital pulmonary airway malformations, which are abbreviated as CPAMs.

Host: Yeah. And you say, you know, four in 10,000 doesn't seem like a lot out of 10,000, unless of course you are the parent of one of the four, of course. So, let's talk about treatment options both during pregnancy and after birth

Dr. Kathleen Dominguez: For most patients, these are identified prenatally at the usual 20-week kind of growth and development ultrasound that's done. And initially, as long as it's not a very large lesion, we simply monitor these. They calculate out something called a CPAM volume ratio or CVR, and that number determines whether or not they need treatment based on the likelihood of complications developing during the pregnancy. So if there were to be complications or signs of complications with that elevated number, the mother's treated with steroids during pregnancy. Otherwise, it's pretty much watchful waiting. We do, as pediatric surgeons, usually meet these patients during the pregnancy so that parents know kind of what to expect over time. And then, once baby is born, the baby's evaluated. And thankfully, most of these children are not symptomatic at birth, though some of them do need treatment more urgently. For the vast majority of patients that I see, we plan to treat them when they're in their kind of mid-infancy between six and nine months of age.

Host: Yeah. I want to have you discuss outcomes. I'm a parent, my kids are older 17 and 22, and i never went through anything like this. But I'm sure this is pretty alarming for parents to hear about these lung malformations, and then want to know, "Okay, well then what are we going to do either in utero or afterbirth?" So, I want to have you discuss the outcomes and what do you look for long-term.

Dr. Kathleen Dominguez: Thankfully though, these are something we do recommend treatment for. Most patients do very well long-term. Majority of patients do not need any sort of intervention in utero or during the pregnancy, and even it's a minority that need treatment right away at birth. But we worry about long-term issues such as pneumonia that can be recurrent or severe as well as there's a potential for malignancy particularly with the CPAs. It's not common at all, but it's very difficult to tell until it happens. And if we don't remove these, it means recurrent radiographic studies to monitor the patient for the rest of their life. And so, most parents, though we do talk about the other option of potentially watchful waiting with the recurrent x-rays and CT scans, most families agree to move forward with resection of the lesion to avoid all the associated risks.

Host: Sure. Yeah, and anything obviously parents can do to reduce the, you know, worry long term. This is a really interesting, interesting topic. I'd just give you a chance here at the end final thoughts, takeaways when we think about how the lungs form and these potential lung malformations. What are your final thoughts and takeaways

Dr. Kathleen Dominguez: That reminds me, one of the most important reasons that we tend to treat these earlier in life is because there's enormous potential for what's called compensatory lung growth in young children. And what that means is when we remove part of the lung, that abnormal tissue that we take out. Though the child doesn't regrow that lobe of the lung per se, the remainder of the lung on that side actually continues to grow and develop it so that their eventual lung function is essentially normal. You might be able to get a couple percent if you did very detailed testing. But in terms of day-to-day living in their life, usually completely normal because they make up for what we removed.

Host: Yeah. That definitely brings a smile to my face. You know, whether it's my kids or other people's kids, knowing that for the most part, especially with early diagnosis, early treatment, watchful care, they're going to be okay. So, thanks so much for your time.

Dr. Kathleen Dominguez: Sure. Thank you. I appreciate it.

Host: For more information and to get connected with one of our providers, please visit carle.org. And that wraps up this episode of Expert Insights with the Carle Foundation Hospital. I'm Scott Webb. Stay well.