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Melanie: Welcome to UF Health MedEd Cast with UF Health Shands Hospital. I'm Melanie Cole, and I invite you to listen in as we discuss care for pediatric chest wall deformities.

Joining me is Dr. Saleem Islam. He's a professor at the University of Florida College of Medicine and the Chief of Pediatric Surgery at UF Health Shands Hospital. And Dr. Janice Taylor, she's an Associate Professor at the University of Florida College of Medicine and a pediatric surgeon at UF Health Shands Hospital.

Doctors, thank you so much for joining us today. Dr. Islam, I'd like to start with you. Please help us to understand the reasons why chest wall deformities occur and what's the prevalence of them.

Dr. Saleem Islam: So chest wall deformities overall can be classified into, like you mentioned, pectus excavatum where it's caving in. It's like excavating and the sternum or the middle part of the chest in the front kind of goes in towards the back, if you will. The other is the opposite, which is pectus carinatum where the sternum is actually poking out, and it's also referred to as pigeon chest because that's how kind of pigeons look like.

Other things that we sometimes see in the assessment of comprehensive chest wall deformities that we see are Poland syndrome, where there can be absence of muscles or ribs on one side of the chest in a congenital deformity or even something known as slipping rib syndrome, which occurs at the base or the bottom of the rib cage and can cause severe pain in patients as they exercise or do things of their daily life.

Most of these issues, other than Poland, arise because of problems with the cartilage. This is known as the costal cartilage, which connects the ribs, which arise from the back to the sternum in the front. And for reasons that are not fully understood, the cartilage is either bent inward in the pectus excavatum or pushed outward as in pectus carinatum or slip on each other and pinch the nerve in between as in the slipping rib syndrome.

And so all of these are cartilaginous problems that we see in children. The incidence is tough to know, but we do know that they're more common in boys than girls. And that of the two, the pectus excavatum is substantially more common than the pectus carinatum defect that we see.

Melanie: Dr. Taylor, what's the clinical presentation? At what age do these present themselves? What are some of the hallmarks of these deformities?

Dr. Janice Taylor: For pectus excavatum, you can see it sometimes when the child is very young, even as a baby, and sometimes will be sent some children to be assessed. And we don't operate on children when they're babies for many reasons, mostly having to do with not wanting to stunt the growth of the chest wall as the baby grows into a child and teenager. So we tend to more often see them when they present to us when they're 12, the tween years, older, or mid-teenage years, once the child hits a growth spurt or tends to become a little more self-aware of their bodies. And so that's typically when we'll see them and then also when we'll start considering them for any type of intervention for both the excavatum and the carinatum, to talk about those in particular.

The things that often are brought up when we see them outside of just the appearance is the question of, "Can my child still do whatever they want to do, even with the way that the chest looks?" Or, "My child sometimes has difficulties. It seems like they have difficulty breathing?" Or, "They feel that their heartbeat is beating funny. Does this have anything to do with that?" And so those are the types of questions that we will typically address when we first meet a family.

Dr. Saleem Islam: And I can jump into that as well. And I think as Janice said, we see these in kids as well as actually in adults. Sometimes they can present in adulthood for reasons that are not fully understood. They don't come to our attention until they're in their twenties, their thirties, and sometimes even in their forties. And they tend to see us because we're the ones who manage these in our chest wall deformity center.

Melanie: Dr. Taylor, if you would expand a little and please correlate for us the clinical symptoms that you described and the potential long-term issues or complications that they may cause.

Dr. Janice Taylor: So the symptoms that people will sometimes come to us for them or their children will be some shortness of breath, sometimes the feeling of heart palpitations or just the complaint, from a parent's standpoint at least, that "My child's always hunched over." And so in asking more questions about how it really is affecting their function or even their personal well-being, their quality of life, that leads us to our various workups.

We will often address this by doing an echo and doing an EKG, pulmonary function test, and a chest CAT scan, in order to get more objective measurements and data to then, correlate it with what the patient's complaints are. And then what it all comes back to then, and this again is for pectus excavatum, is how badly does the child or teenager or adult really want this operation, because in many cases, it does not ultimately affect how they function and what their activity level really can be.

In the case of the pectus carinatum, it's a little bit of a different approach. Usually, the complaints are a little bit different. It's not so much a feeling of difficulty breathing or keeping up with physical activity or heart palpitations, but it's more the overall appearance and the psychological effects of the carinatum.

Melanie: Dr. Islam, how do you determine then when to treat? What's the right time during their skeletal development? And tell us some of the options for treating these chest wall deformities, and please first start with some non-surgical options that might be used.

Dr. Saleem Islam: So I'll address carinatum first. Carinatum, which is where the chest is poking out is dealt with non-surgically right off the bat. We have an external brace device that use. These are constructed by our colleagues in various orthotic development agencies. And they basically set them up for the brace. The brace pushes the chest inside and basically, allows the costal cartilages to assume the correct position. One can think of it as bracing for the teeth in a way. So that bracing involves using metal braces to align the teeth in the orientation that we want them to be.   And that's the same concept here, that you're compressing the chest slowly.

So that's a completely non-surgical thing. We compress the chest for about six months aggressively and then six months less aggressively as a retainer kind of concept. And then usually by then, the chest wall has formed in a more acceptable way in the sense that the sternum is no longer poking out.

A similar concept is there for the other one, the pectus excavatum, where again we are molding the costal cartilages to where we want them to be. In this case, though, we are using a metal bar in the minimally invasive repair of pectus excavatum where the bar is partially underneath the sternum and partially on top of the rib cage, underneath the skin and subcutaneous tissue and muscles. And in that fashion, it's pushing it up and holding it in place. The correction in that situation is achieved immediately. So as the patient wakes up from the surgery, they are completely corrected.

Now the question on when to do this and when is the ideal age?   It really depends. A lot of times the ideal age in terms of how much pain the patient is going to have postoperatively because the correction is done immediately is around the 12, 13, 14-year age group. By the time a lot of these patients present to us, however, they're already 16, 17, 18 years of age. And so that's the age when we're doing them. So we won't do them, I won't do them at least, less than 11, 12 years of age. And we certainly have no upper limit necessarily. We have 30, 40-year old's who come and have this done as well. So we take them at all kinds of times of skeletal development, even before puberty is done and after puberty is done,

Dr. Janice Taylor: I'd like to add to that too. That as far as when we have the younger patients, tweens, early teenagers that present to us for a chest wall correction, that I think that's really important to make sure that child who is the patient is really involved in the conversation because I think that, they need to really understand what they're going to be going through. And in the case of the bar procedure, the Nuss procedure that Dr. Islam described, I think we owe it to them to make sure that they have as good of an idea of what they will be undergoing.

Melanie: Dr. Taylor, thank you so much because that segues beautifully into my question for you, is how do you help parents? And what would you like other providers to know as they're counseling their patients and getting ready for referral? How do you help your patients, the parents, and the children, to understand this condition, how it could affect them in the future, if at all? How do you work with families to prepare that child for potential physical and social impacts? And what are these treatments like for the kids and their families? How well do the kids tolerate them, the brace, or the procedures? Tell us about that.

Dr. Janice Taylor: I think that you need to really be realistic about setting expectations. And I find myself a lot of times being very redundant in the counseling sessions with families when I first meet them, because I want them to really understand that, in the case of the brace, it's not just the one and done thing. This is something that the child needs to be committed to, that the teenager needs to be committed to.

In the case of surgery, you don't just have the surgery and you leave the next day and then everything's fine. You can go back to, for example, playing football the next week, it doesn't really work like that.

So I think, expectations and really, honest conversations are important. I tell my families when I first meet them in a room and I tell them, "I'm getting straight to the punchline. I'm not offering you surgery right this very second. This is a fact-finding mission as far as I'm concerned. I'm not pushing surgery on you. You need to understand and go back and have a dinner table conversation about the surgery, how much you want it, how you think it will affect you, and then come back and see me after that."

Melanie: Thank you for that. And Dr. Islam, you mentioned your colleagues in orthotics. Then tell us how important this multidisciplinary approach is for these patients and who's involved in the care team.

Dr. Saleem Islam: it truly is a multidisciplinary approach that we apply. And this kind of comprehensive plan of dealing with them goes from the planning of the surgery to a comprehensive way of dealing with the pain that Dr. Taylor mentioned that these patients can have. So in our multi-disciplinary approach of taking care of patients with chest wall deformities, we have, of course, us in pediatric surgery. We have our physical therapists who play an integral role, both before and after surgery. We have our pulmonary and cardiology colleagues who get involved when we need them to for looking at the pulmonary assessment and whether the heart's being compressed or not. We use our acute pain services and chronic pain services.   And of course, our radiologists who help us in identifying the issues and, the severity of the deformity itself.

Finally, we've got a very interesting collaboration that we've developed with our aortic disease center in the adult world where patients who have Marfan syndrome and who have aortic conditions who are having a heart surgery, open-heart surgery done, we combine that operation with the repair of their pectus excavatum as well. So it truly is a multidisciplinary approach.

When we look at the comprehensive way of dealing with pain, which is a big stumbling block in this operation for the pectus excavatum, we really try to use this via a multimodal pathway. Again, using our colleagues in the pain service, our physical therapists. We use preoperative preparation. We use non-steroidal drugs like gabapentin. And then we use some novelties, the use of cryoablation, which is the freezing of nerves in the intercostal areas on both sides, which provides a numbness and really reduces their amount of narcotics that they need in addition to the use of analytics and other drugs, which help really help to reduce their hospital length of stay and their narcotic use. So it really is a team approach.

Melanie: Thank you for getting to that question about pain management, because I was going to ask you that anyway. So thank you. Dr. Taylor, as we get ready to wrap up, tell us a little bit about your outcomes and when you feel it's important that other providers refer.

Dr. Janice Taylor: I'll get to your second question first. I think that as far as the referral, we're happy to see people at any time. I think it's always good to start having the conversation when it first comes up, because I think what is really helpful for the families is having the conversations, getting questions answered that you didn't know you had until you came and started talking to us.

So even if surgery is not the right time or it is the right time age-wise, but the patient is not ready emotionally or physically or mentally to have it quite yet, I think again to get all the data, go on that data gathering and then go home, have those conversations and decide when is the right time personally for each patient to get it done. So from a referral standpoint, we're happy to see patients at any point in time. and then take it from there.

The outcomes at our center for satisfaction have been excellent. The patients are happy with how they look cosmetically. A lot of times the first question I ask when I see the patients after they wake up is "What do you think?"

And I think in setting up expectations for them in what pain is going to be like, what activity is going to be like, what they're going to do and how they're going to manage themselves after they go home, I think in having all those really frank conversations upfront is part of the outcome calculation if you will. It's not just physically how does it look, but how is the patient doing quality of life-wise and managing things once they get out of the hospital?

Melanie: Okay, Dr. Islam, last word to you. What would you like other providers to know and take forward to their patients on this topic?

Dr. Saleem Islam: One is to be able to recognize this. A lot of patients end up coming to us sometimes a little bit late, even when, like I said, in their twenties and thirties where it can become a little bit more challenging to do. To know that we are here, that we provide a comprehensive approach of taking care of patients with chest wall deformities in almost any age, in any kind of just wall issue. And then to know that as an institution and as a university program, we constantly strive for the better outcomes and improved techniques that Dr. Taylor was talking about. And as part of that, we're actively involved in research and in looking at innovations that can make this operation better for the patients and be tolerated better. We are at any point happy to talk to any of our providers and inform them about all of these procedures and things that we can do for these patients and really help them in their outcomes for this.

Melanie: Thank you, doctors, so much for joining us today and telling us about chest wall deformities and what you're doing for patients at UF Health Shands Hospital. To refer your patient, please visit UFHealth.org/pediatricsurgery. Or to learn more about other healthcare topics at UF Health Shands Hospital, please visit UFHealth.org/medmatters to get connected with one of our providers.

That concludes today's episode of UF Health MedEd Cast with UF Health Shands Hospital. Please remember to subscribe, rate, and review this podcast and all the other UF Health Shands Hospital podcasts. I'm Melanie Cole.