Evo Terra (Host): Chest wall deformities, or abnormally shaped chests, are a very common condition that affects about 1 in 500 people, including children. But I have good news. They're treatable. Let's find out more with Dr. Garret Zallen, a Pediatric Surgeon at Shriners Children's Portland. Welcome to Healing Heroes PDX, the podcast series from the specialists at Shriners Children's Portland. I'm your host, Evo Terra, and today we're discussing chest wall conditions and their treatment for kids. Thank you very much for joining me, Dr. Zallen.

Garret S. Zallen, MD: Well, thanks for having me.

Host: So, I know that you specialize in providing care for children with chest wall deformities. Can you explain what these types of conditions are and how they're identified?

Garret S. Zallen, MD: Yeah, there's a few different sort of categories of chest wall deformities. Most of them have to do with the way that the chest grows and shapes. Some have to do with what elements of the chest are there when the child's born and what elements actually aren't there. And so they fall into sort of two broad categories; things that we're born with and things that sort of we grow into.

The most common chest wall deformities that we see are the ones that are problems with growth of the chest wall, and it tends to be shapes of the chest wall, most common being where the sternum sinks inwards. People have probably seen people around the swimming pool whose sternum seems to sink in or their chest looks like it's a funnel chest, it's been referred to, and that's what we call pectus excavatum. That's the most common chest wall deformity that we see.

They also can have where their chest wall sticks out, so instead of sinking in, it does the exact opposite. It pushes out, and they're left with a deformity that's called a pectus carinatum. Two Latin terms. I didn't name them. People came up with these a long time ago, back when people were supposed to speak Latin and doctors took Latin, but pectus means chest, excavata means caved in, carinatum actually means bird chest because it's shaped as the sternum would be in a bird.

And then the other are the ones of growth of the chest wall. The most common of that being what we call Poland Syndrome. Just like the country, Poland, where when kids are born, they're actually missing parts of their chest wall. Some of the muscle, some of the ribs, in girls, some of the breast tissue in girls and boys, sometimes some of the nipple or the nipples can be asymmetric.

So, purely a developmental problem, not a progressive problem, but a developmental problem where kids are born with not the normal amount of tissue that's supposed to be there.

Host: Hmm, hmm. I see, yeah, one of my friends when they were babies she was born missing a pectoral muscle, so, yeah, I can understand that happens. Now, this may sound like an obvious question, because some of these things are obviously very forwarding, very front presenting, if you will, but it easy to know when your child has a chest wall deformity?

Garret S. Zallen, MD: Most of these are actually pretty visually apparent, especially the pectus excavatum, pectus carinatum. Those can be progressive over time, so it's not uncommon where we see kids who are born with relatively normal looking chests or completely normal looking chests until they go through their adolescent growth spurts.

And when they go through that really rapid growth, then the ribs can start to become misshapened. And it's not uncommon where I have families who are like, overnight, this seems to have happened. Well, none of this happens overnight, but when kids start going through puberty, you start to see them less and less without their clothes on.

And they become more independent, and all of a sudden, you see them after eight months, a year, and their chests look very different. And so, these are things that can sort of slowly happen, and it seems like it just sort of happened overnight, or how did I miss that? It didn't. It just took time to develop.

Poland syndrome, on the other hand, can be pretty subtle at birth because kids don't have a lot of muscle. Babies don't have a lot of muscle in their body. They don't need it. Their nipples are pretty small and they're kind of hard to see anyways. And so it's easily sort of missed until they start to grow and they start putting on a little bit of muscle.

Their nipples start to get bigger. Often that's when you can see these problems. And so, even though they're born with it, sometimes it doesn't really become apparent until they're a little bit older and they have a little bit more muscle mass and then you can actually really see the asymmetry. I'd say it sounds like you probably just diagnosed your friend with Poland Syndrome, the one who is missing the pectorals muscle. So congratulations, you've gone from podcast host to physician.

Host: Fantastic. I will be taking my residency at anytime now. No, nobody wants that. So, you just brought up a great point that, you know, as our, as our children's bodies change over time, something might look a little unusual. So I guess the question is, when is just, ah, that's the kids growing, or when is it something different? When do I get a specialist like you involved?

Garret S. Zallen, MD: Yeah, that's, I think, a really good question because most of these conditions don't necessarily require any treatment when the kids are young. And so getting us involved, I think, is important for sort of two reasons. One is to get a diagnosis. If your pediatrician or your primary care physician is uncertain as to what this is, they see something but they can't quite explain it, that would be a good time to come see us because then we can also sort of tell you what this is, what to expect, what this might mean, or this is just a normal variant because not everything we see is necessarily one of these particular conditions.

The other is if it's starting to cause some symptoms. Again, that would be a good time to come see us because that's when we can start talking about potential treatment options and what sort of possibilities there are to help manage the symptoms that the child's having or to sort of allay some of the concerns that come with some of those symptoms because some of those symptoms are things that, that eventually over time won't go away.

So I think those are good times to come see us. I'm happy to see kids who are little, years before we would even consider doing any type of interventions, just to talk to the families, let them know what's going on, what they can expect, because sometimes this can produce a lot of anxiety for families.

You know, when they see their kids chest looks funny, they don't know what this is going to mean. Is it going to affect them? Should they not exercise? Should they not play sports? You know, should they be worried about impacts to the chest? Those kind of questions. And we can go through all that and talk to them and sort of help them understand what it is that their child has and what the future looks like for them.

Host: And I would assume parents and certainly for the children themselves that one of their big questions is what are we going to do about this? I mean, am I going to be walking around with a giant cast around my chest for the next three years? What do treatment options look like?

Garret S. Zallen, MD: Right. I mean, the good news now is the treatment options are really good for most all these problems, which is vastly different than when I started my training 30 years ago. The treatment options were pretty minimal and actually pretty miserable and so things have gotten significantly better. The treatment for the most common problems that I mentioned, the pectus excavatum and the pectus carinatum. The pectus excavatum can really become a problem, not just of an appearance for the child, which is can become a really big issue as these things get deeper. It can become a real problem with their self-image, but it can also cause cardiac compression.

And when the heart starts to get squished because the sternum is sinking in, we start to see a lot of problems with exercise intolerance, shortness of breath. They used to like love to play sports. Now they don't because it doesn't feel good. They don't like it. They can no longer keep up with their friends.

And so we do a surgery that repairs that problem, that literally lifts the sternum up and gives the heart the room that it's supposed to have that the sternum was taking away from it. The surgery that used to be done for this 30 years ago was a really, it was not a very good surgery, totally different technique, outcomes were really poor, and so a lot of these kids parents who may have had this, or have heard that, you know, do anything with this, don't operate on it, it's not a condition that you want to mess with, that's completely changed over the last 20 years.

We now have a much better technique, completely different surgery. Much better pain control management techniques that we do. We literally go in and freeze the nerves on the inside of the chest with a special probe that knocks out the sensation of the chest wall for about two months so that the post operative recovery is really enhanced in these kids.

 We do everything we possibly can to minimize narcotic use for the surgery and what was used to be an inpatient hospital stay for up to a week or more is now down to particularly one day. Occasionally two days. So it's much better tolerated surgery with really good outcomes. The pectus carinatum, so for those kids whose chest stick out, the good news is, is that doesn't typically cause any type of physiologic problems because it's not compressing the heart.

But it can cause a lot of body image problems. You know, you can imagine walking around with your sternum sticking out. The worse it gets, the much more obvious it is. And for, a lot of people, that's a big problem. And so we actually have braces now that fix this problem. One of the biggest issues I have with the kids who need to get a brace is in their mind, they have this picture of a, this tremendous brace that like, you know, you're just sort of saying encompasses their whole body and they can't do anything.

These braces are actually pretty small now. They're compact. They sit on the chest wall. easily tolerated, can be worn to school, can be worn at home, they can sleep in them and they work and they work really well. They can completely eliminate the pectus carinatum without any surgery, no scars, no risk, no, no real pain with it.

So, as far as that goes, those treatments are really come a long ways and lots of options for the kids to have.

Host: That is great to hear.

Garret S. Zallen, MD: Yeah, and then the last ones are the Poland Syndrome, which is sort of the other common one that we see is there's not always a treatment that needs to be done. Most of these kids still have the same strength in their upper extremities. They can be just as active. The loss of muscle tissue is not really a physiologic problem for them. It can be an appearance issue as they get older and they get more developed. In males, it tends to be less of a problem. In females, it can become a significant problem if they have one breast that really doesn't develop at all but just like we do breast reconstructions in adults, they can have a similar reconstruction done once they're through puberty and they can have, you know, a very nice cosmetic result once they're done with it.

So lots of options for these kids and they're all, as I said, options. These are things that are really left up to the individual child and family. It's how they want to proceed. There's never a time where they're going to come into my clinic, I'm going to tell them they have to have something done. It's always their choice.

Host: Well, dovetailing off of that, what if they do decide to do nothing? Is that a good idea? Is just leaving the conditions untreated acceptable?

Garret S. Zallen, MD: Taking all three of them, the Poland Syndrome, I think it's totally acceptable if it's not a, an issue for them from an appearance and they're happy with it. I think that that is a very acceptable option. The Pectis carinatum, they start to get more severe. I worry that sometimes these kids, when they get older, they're going to wish they had done something about it.

Teenagers sometimes live in a world where they kind of see themselves invincible. They're, time is infinite. Their choices are always open, which with this particular problem is not necessarily true. Once you get to a certain age, the braces don't really work very well because the ribs, the chest wall get too rigid.

 Adults, if you push on your chest, you notice your sternum doesn't move. A kid, you push on their sternum, it moves a lot, and that's what enables the brace to work. So sometime in the teenage years that the brace becomes really no longer feasible. And so for some of those kids, I let them make their choice, but I will sort of counsel them that they do have a time limit on it. It's not an infinite decision that they can come back and change later. And so to really think about it, and I do have kids who at first decide they don't want anything and then they'll come back in a year or two and after they've thought about it and matured a little bit more, and then, they decide they want to get it done.

And then that works fine. And they, once they're motivated, they wear their brace and then they fix their problem for themselves. As far as the pectus excavatum goes, that's always going to be a decision up to the child as well. Most kids who have really severe pectus excavatums who are having the exercise intolerance, they elect to have a surgery to fix it because they want to feel better.

They want to exercise. They want to go do the things that they used to be able to do. They want to do stuff that their friends are doing. So, for them, it's not a hard decision, but there are kids who still wait and they don't necessarily want to have it done. And the good news with the pectus excavatum is the surgeries can be done as adults.

So they don't have to have it done as a child or as a teenagers. We don't do their pectus excavatum surgery until they're done with their adolescent growth spurts. So there's kind of a, a floor to that surgery.So we won't do it before they're done with that. And I can get into some of those reasons in a little bit as to why we wait until they're done. But I have done these surgeries on adults too other institutions we operate on adults. So, the decision doesn't have to be made as a child. The surgery works better when they're done as a teenager.

It's a little bit better recovery, a little bit easier to recover. You don't have to tell anybody. The older you get, the harder it is to recover from anything. And so teenagers, have a natural ability to recover well, but also their chest walls are more compliant, they're more flexible. So when you are changing the shape of it, it, the body tolerates it better than in an adult. But certainly they can change their decision and do it later if they want to do that.

Host: Yeah, and lots of things are left to kids and teenagers, they're going to change their mind, a lot the process. And it sounds like a lot of progress has been made in the correction, which I'm sure a lot of people don't know about, so that is good news. On a similar, vein are there some misconceptions? I assume there are. I mean, there's some people who are thinking that this is the old way of doing things, but there are new things now. Are there some common misconceptions regarding chest wall deformities you see in your clinic?

Garret S. Zallen, MD: Yeah, the biggest common misconception is, these are cosmetic issues. And I hear that term sort of bantered around quite a bit. I disagree with the terminology just in general, cosmetic. Cosmetic problems are things where you have a normal body part, but you want to change it to look the way that you want it to look.

These are not normal body parts. These are sternums that are depressed, that are pushed out, ribs that are misshapen, muscles that are missing, breasts that are missing. We're kind of in the world of reconstructive surgery or reconstructive processes. And so I try to shift the conversation away from plastics because that sort of seems to put a little bit of vanity into it.

You know, well, just get used to your body. It's okay. In these cases, it's not a normal appearance. So, my job is to bring it back to normal. And so for me, that's one of the misconceptions. The other misconception and that came about not just from sort of the public's perception, but really actually came out of medical science.

When I was looking at pectus excavatums when I was a resident years ago, we did research to look to see how doing this surgery affected the lungs and how it affected the kids ability to breathe after surgery. And what we really found is there wasn't much effect. And the problem was, is we were looking at the wrong organ.

What we really should have been looking at was its effect on the heart. And the reason why is when we were looking at these kids, you know, you see their chest sunken in, you would think that this is really going to affect the lungs because they're not going to have much space in their chest. And it turns out it doesn't really, for most kids, affect their lungs.

And we would send these kids to the cardiologist. They'd get echocardiograms, and they'd come back and say, well, echocardiogram's normal. We don't see any problems. So, whatever symptoms they're having must just be related to something else, but not the chest wall. And then after time, we as doctors got a little bit smarter and figured out wait a minute, let's start studying the heart and see if that's what's being affected.

And what we've figured out since that is that that's the problem. The echocardiograms that we've been getting are normal in a lot of these kids because when they do the echocardiogram, they're looking to see the heart underneath the sternum. Well, if your sternum is pushing into your heart, you can't look under it with an echocardiogram because it's an ultrasound.

An ultrasound doesn't look through bone. And so they were being fooled. And it's not that the echocardiograms are wrong. They just can't see what they can't see. So then we started doing CAT scans on these kids to look and see how deep this was and we started to see a lot more kids with cardiac compression, that were had normal echoes, but when you look at the CT scan, which can penetrate the bone and can look underneath the bone, you can see the clear compression. And then we started doing exercise tests on these kids where we looked at is the heart able to really increase the cardiac output the way a normal heart does?

We found it does not. And so, we've now learned that, yes, there is true physiologic consequences for some of these kids. Not everybody, but for a number of these kids and fixing them gets rid of it. So that was the other part of the question we had to answer, well, if we lift up the sternum, does it make it better. And that question's also been answered. Definitively, yes, it does make people better.

Host: When families come in with a child which has a chest wall deformity, I'm assuming they've got a slew of questions from what are we going to do about this, to how long is it going to take, but talk to me through some of the common questions that you get from families.

Garret S. Zallen, MD: Yeah, so, you know, most families, have been on the internet and they've read about it and they've seen some websites. And so, one of the big questions I get, because these chest wall deformities can be associated with other sort of more serious connective tissue disorders. And connective tissues are the tissues in our body that hold things together, that compose our ligaments, that holds our blood vessels together, holds our muscles together.

And some of these kids will have problems with connective tissue problems, but most kids don't. And so that's one of the first questions I have to answer is, well, does my child have Marfans? Or does my child have one of these other more serious problems? And the answer to that question is almost usually always no, although some do, but the majority of kids who have these don't.

They're in isolation. There is no definable connective tissue disorder that we see with these that put these kids more at risk for serious health problems. We do see a fair number of kids who have a condition called Ehlers Danlos. which is another connective tissue problem. The particular subtype that is the most common doesn't have any real consequences in terms of long term longevity.

It can have some quality of issue by problems in terms of some ligaments and joints and those sorts of issues that can be very real, but it's not something that is going to impact the child's longevity, which is one of the big questions that these families have. And so that's one of the things I sort of help put to rest is that probably just in isolation, we're going to do some tests.

We'll make sure we'll look to see if there's any associated problems. But for the most part, these are things that are just affecting the chest wall, sometimes some other bones, the spine but not the other sort of more serious health problems. People want to know, where did this come from? Why did they get it? I wish I could answer that question. We don't know why these bones start to grow abnormally. There's lots of different theories. Probably has something to do with the connective tissues, with other structures, but we don't truly know why the bones grow in a direction that is abnormal.

You know, I tell most families, we don't even know why bones grow in the direction that is normal. You know, we don't know what tells your finger bone to grow in that exact shape and what tells your rib bone to grow in that exact shape. We just don't have that understanding of chromosomes and genetics yet, and at some point maybe we will, but at this point we, we can't really answer the true question is, why did this happen?

Oftentimes there's other family members who have it, either a relative or somebody in the family. I've also had parents who have been like, oh wait, I had the same thing too, but when I was a kid I was told don't do anything about it. Like, well, that was probably true when you were a kid, but now that you have a kid, things are different.

And so, those are some of the other questions that tend to often come up. And then they also want to know what we just talked about. Well, what can we do about it? What are our options? When do we need to get concerned about this? And then when we do, what can we do about it?

Host: You mentioned earlier that this has changed a lot in the 30 years you've been doing it, and it probably will continue to change. But let's talk about what's changed at Shriners Children's. What makes getting this kind of care at Shriners Children's different than it might be at a different facility?

Garret S. Zallen, MD: Yeah, so this is a hospital that has been doing this surgery now for over 20 years. We have kept up with all the changes in terms of the modernization of this surgery, as I mentioned, and also the modernization of the bracing and other treatments that go along with chest wall deformities. We see kids from all over the western part of the United States, and so the experience here is really on the West Coast, one of the best experiences in terms of volume and longevity in programs that have been really successfully managed for well over two decades.

And so I think when it comes to some of these issues, the experience is really hard to, minimize in terms of outcomes, in terms of, how families feel once they get through the program. So, I think that that's really one of the big benefits of the Shriners Hospital. The other is this is a surgery that is not always covered by health insurance.

We haven't talked about that yet, but, this surgery used to never be covered by health insurance because as I mentioned before, it used to be considered a cosmetic operation. And as I said, that was largely some of our problems. You know, we weren't finding the physiologic problems that we were trying to correct, even though we knew that they had to be there.

We just couldn't find them. Now that we've found them and we've been able to modify some of the insurance processes and the Oregon Health Authority and some other bigger, government groups, they now recognize this as a Covered Procedure but that doesn't mean it's always covered. There are certain criteria that need to be met and sometimes with individual insurance companies and whatnot, we can't meet those criteria even though it's something the child wants to have fixed, the family wants to have done, and so, the Shriners Hospital, they get it done.

They're able to work with the families, work with their processes to get these done versus, you know, some of the other hospitals, if you can't get your authorization, then it just doesn't happen. So, really, that's one of the magic of the Shriners Hospital and how they do it and how they continue to do it, I don't know, but I'll keep working here because they make it happen.

Host: Well, this has been a fascinating conversation. I want to turn the focus for the last couple of questions on to you and maybe on to me since you told me I was going to be a doctor earlier. How does one become a chest wall specialist?

Garret S. Zallen, MD: Well, I hope you have some time on your hands because it takes a while to get here. But to become a pediatric chest wall surgeon, you have to go through a adult surgery training program, and then followed by a pediatric surgery training program, which takes somewhere around in the neighborhood of about 10 years to make it through that.

And that's all after medical school. I got really interested in chest wall surgery because where I did my pediatric surgery training, where I was learning how to operate on kids instead of adults, we had a specialist who really worked on chest wall surgery. And then while I was there, the actual inventor, Donald Nuss, who, invented the modern operation that we do, came to speak at our program, and I was just really intrigued by what he had to say and his thought process that went into the surgery and creating the Nuss Procedure and his outcomes.

And after meeting with him, I, decided that that was going to be an area that I, I wanted to specialize in because I think it's an area that deserves attention. It deserves conscientious surgeons who are experienced and know what they're doing, but also know when not to operate. So I, think those were really sort of formative years for me that helped me decide this.

Once I got out to Portland, Oregon to start my career, the Shriners Hospital was right across the street from the hospital I started working at. So I came over and started working at the Shriners Hospital and I have been coming here for over 20 years because it is just such a magical place to work and the things they do here and the other surgeons. It's really been pleasure to continue working here.

Host: When you decided medical school was for you and surgery was for you, what drew you to pursue a career specifically in pediatric surgery? You talked about chest wall, I understand that, but why kids?

Garret S. Zallen, MD: Kids have problems that they didn't create, that they are either born with or that happened to them. They can be very complex problems down to really sort of simple problems. But kids, they don't see life in the same way that adults do. They see their bodies as something that they want to have work better, want to be what enables them to have a more normal life.

It gives me an opportunity to try to do that for them. And the issues that they have are not because they have been neglecting their health for years, but because it's something that they were born with or a genetic problem. And kids are just fun to work with. When they get better, they let you know.

You see them in the playroom. They're having fun. They don't have agendas. They just want to be kids and enjoy life. I enjoy working with the families. It's one of the big things when you go into pediatric surgery, everybody tells you, well, that's gotta be really hard working with the families.

It's, you know, they have to be a lot of pressure, a lot of stress. And what I've found is uh, especially in chest wall surgeries, this is the one area in the pediatric surgery that I do in addition to the chest wall where the families will come and thank me after we have finished this whole process and tell me that the chest wall reconstruction we did has changed their child's life.

It has made them more confident. It's made them more secure. They are now going out and exercising. And you see it in the kids. You see these kids come in as skinny kids who are sort of timid a lot of times. And then when they come back after you're totally done with the process; they're standing upright, they're tall, they're proud.

They have big muscular chests and they are wanting the world to see what they have done. And there's no other surgery I do where I get that type of feedback from the families as to how it's really changed their lives. So, to me, it's a pleasure to be able to do it and it's a privilege.

Host: And that's got to be one of the greatest feelings in the world. Dr. Zallen, thank you very much for joining us today.

Garret S. Zallen, MD: thank you for having me and I appreciate the chance to talk about these problems. I think they are often overlooked and I want to be here to try to help people understand them and help them understand their options. So whatever I can do, I would love to help.

Host: Once again, that was Dr. Garret Zallen, a Pediatric Surgeon at Shriners Children's Portland. That concludes this episode of Healing Heroes PDX with Shriners Children's Portland. Head on over to our website at ShrinersPortland.org. If you found this podcast episode helpful, please share it on your social channels, and check out the full podcast library for topics of interest to you. I'm Evo Terra. This is Healing Heroes PDX, the podcast series from the specialists at Shriners Children's Portland. Thanks for listening.