Transcription:
Correction of Chest Wall Deformities

Bill Klaproth (Host):  Chest wall deformities are congenital conditions which means they are present at birth. Now sometimes these deformities are apparent. You can see them. Other times, they are not. So, what are the different types of chest wall deformities, how would you know your child has this as he or she is growing up and most importantly, how can they be corrected? Let’s find out with Dr. J. Duncan Phillips, a Pediatric Surgeon with WakeMed Children’s in Raleigh, North Carolina. This is WakeMed Voices, a podcast from WakeMed Health and Hospitals. I’m Bill Klaproth. Dr. Phillips, than you for your time. First off, what causes chest wall deformities?

J. Duncan Phillips, MD (Guest):  Well the exact cause is not entirely known but, in some children, for unknown reasons, the ribcage starts to grow in a somewhat abnormal fashion. That is, as the ribs get longer and longer, instead of growing in sort of a flat direction, they curve inward and as a result, they push the breastbone, that is the sternal bone in toward the heart and lungs. That’s pectus excavatum and in a few other kids, again for reasons that we don’t quite understand, the ribs start to grow in the opposite direction. That is instead of growing flat, they grow outward pushing the sternal bone outward and that ends up causing pectus carinatum.

Host:  Okay so, the ribcage either is either raised or sunken, that’s what we’re talking about basically when it comes to chest wall deformity?

Dr. Phillips:  Those are the most common ones by far. Both pectus excavatum that is going in and pectus carinatum that is going out. So, although there are a number of somewhat less common chest wall deformities, the most common one we see are the ones that I’ve just talked about.

Host:  So, when you say see, is it true that sometimes these aren’t apparent at birth. Is that correct?

Dr. Phillips:  That’s true and in fact in most kids, they aren’t apparent right at birth and they slowly develop often during middle parts of childhood perhaps beginning around age 9, 10 or 11 years. in fact the most common time we see these kids is when they are about 12 or 13 or 14 years old where the deformity has gradually worsened over a period of several years and the parents are starting to get concerned about it.

Host:  So, are there symptoms that go along with this as well that would indicate there’s a problem?

Dr. Phillips:  Sure. Initially, many of the kids are asymptomatic meaning they have no problems at all. But what often happens is very gradually over time, as the deformity gets worse; the kids start to develop symptoms such as shortness of breath with exertion. So, they for example may have in the past been able to play an entire soccer game and not have to rest. They may notice that they are having to stop and rest and catch their breath and sit out for a period of time. And they don’t have the same ability to continue playing sports often whether it be soccer, football, basketball whatever or swimming.

The second most common symptom would be chest pain and that’s often brought on by exercise, by exertion where a kid will with exercise, start to hurt a lot in the front of his or her chest. Other less common symptoms can include asthma, recurrent respiratory tract infections and even episodic attacks of pneumonia.

Host:  So, when you mentioned that pain in the chest, you said it’s more towards the front; that’s different than the side ache pain that I know a lot of kids get when they are kind of growing and exercising and running around?

Dr. Phillips:  Yeah, the pain is common. We are not entirely sure why it happens. It may be because the nerves on the front of the chest wall are getting stretched and twisted in some sort of way. Along the lower edge of each rib, there is an intercostal nerve which sends signals of discomfort of pain up to the brain and it may be that those nerves are getting pulled in or pushed out in the case of pectus carinatum and those nerves therefore are being irritated and that may be the reason that the kids have the pain.

Host:  So, then how do you ultimately diagnose this Dr. Phillips?

Dr. Phillips:  Well part of it is physical exam where you – and it’s funny. A lot of the kids have become quite self-conscious and have not been taking their shirts off in front of their parents. And so, for example the family will go on a trip to the beach and the boy will keep his shirt on the whole week. Or they’ll go to the swimming pool and he’ll want to keep his shirt on and not take it off. So, oftentimes, parents are caught off guard. Because the kids are older, they are no longer dressing their kids in the morning or helping them in the bathtub or in the shower.

So, often mom and dad haven’t seen the child with his or her shirt off in many months or even years. And suddenly for some reason, they notice this. So, physical exam is very helpful. And we can measure the depth. If it’s an excavatum, we can actually measure how deep it is. And then in addition to physical exam; there are a number of ways that we can test and quantitate the severity of the pectus. We can do some internal measurements.

One of the most popular is a CAT scan, a CT scan of the chest and there are ways to measure the internal dimensions of the chest. There was a famous pediatric surgeon many years ago, up at Johns Hopkins in Baltimore, Dr. Alex Haller and he came up with the Haller Index which allows us to do a rough measurement of the inside. Other tests that we often do are things like echocardiograms where we bounce sound waves off the heart to see if it is being squished by the pectus or stretched out in some way. We can also measure pulmonary functions with pulmonary function tests and then finally, we actually have the ability to do exercise physiology tests. We actually have a way to put a teenager or an older child on an exercise bike and measure their heart and lung function as we have them exercise on the bicycle.

Host:  Right because a sunken or raised chest either one, can affect both heart and lungs. Is that right?

Dr. Phillips:  Right and it’s somewhat complicated because in some kids, it’s diminished lung function and in others it’s more diminished heart function. And remember the human heart has four chambers and it’s the right atrium and the right ventricle that pump the blood out to the lungs and so sometimes, the shortness of breath is not because the lungs are squished but because the heart is not able to push as much blood every minute to the lung tissue.

Host:  Right. Okay so, that makes sense. Got it. So, once you do diagnose this; what are the types of treatment? How do you correct chest wall deformities?

Dr. Phillips:  Well, that’s a good question. In the 1950s, 1960s, 1970s, the operations that were done to correct these deformities were very aggressive and very invasive. They involved making a large incision, removing a lot of the abnormal ribs and the abnormal cartilages and taking an electric saw, a power saw and cutting the breast bone, the sternal bone to lift it up or to push it down in to make it flat. So, that’s not a bad operation but it’s an operation that has to some extent become much less popular since Dr. Nuss came along in the very late 1990s and came up with a different way to treat these problems.

So, Dr. Nuss was having, apparently his daughter get braces on her teeth and he had a little light bulb go off above his head and saying why don’t we come up with a way to basically bend the ribs and bend the breastbone and just push it into the correct shape. And just like braces on the teeth, you remove them two years later, come up with some sort of internal braces, put it on the chest, correct the deformity and then take that brace out. So, the Nuss Bar which has become really the most popular operation for chest wall deformities was purely his idea thinking about how we correct crooked teeth.

Host:  Really interesting and the Nuss Bar works on both pectus carinatum and pectus excavatum?

Dr. Phillips:  Well that’s in evolution. Sort of. Right now, it’s primarily for pectus excavatum. It works really well to push the sternum out.

Host:  Okay and that’s for sunken chest. Pectus excavatum is sunken. So that’s to pull the chest up.

Dr. Phillips:  Right, so for sunken chests, probably about I would say 90% of the kids who are getting that fixed in the US and in multiple other countries are now having the Nuss Procedure. That is I would say by far the most popular way to correct the sunken chest. For the chest that’s actually sticking forward; we often avoid surgery entirely. We use a brace that the kids wear under their shirt that pushes the sternum down and they wear that brace under their shirt for a couple of years and in most cases, we can actually correct pectus carinatum without any surgery at all.

Host:  And that’s because the child is still growing, so you have time for the bones to correct themselves with the brace?

Dr. Phillips:  Right. It’s an individualized approach. A lot of what you can and cannot do depends on the flexibility of the chest and that depends on well partially the age of the patient and partially just how flexible that individual child is. So, for example, there are some 16 year olds who are extraordinarily flexible and can be easily corrected with an external compression brace if their chest sticks way out. There are others, who are very stiff and may actually require surgical procedure. So, what we like to do is individualize the approach to the individual child.

Host:  So, since you mentioned this individualized approach; let’s talk more about the individualized approach at WakeMed. In fact your Chest Wall Deformity Center has been established to offer individualized treatment to all patients. So, can you talk more about this individualized approach?

Dr. Phillips:  Yeah. We have some newer technologies as well that we’re using that are not widely available at most centers in the US. Let me give you an example. A German engineer has developed a vacuum bell which looks sort of like a giant toilet plunger that you can attach to the front of your chest and suck the chest out. And so there are some little kids, I would say probably under age 12 that have an extraordinarily flexible chest that can actually respond to the vacuum bell. The vacuum bell has not yet been recognized by the US Food and Drug Administration. It’s a foreign project. It’s not illegal to use it. it’s just not covered by health insurance.

But we do have a relationship with the man who invented the vacuum bell, so we are one of probably ten or twelve centers in the US that help patients get the vacuum bell and then work with them, if it’s appropriate to try and fix those kids without any surgery at all.

Host:  That’s amazing. So, I want to touch on this too. Currently, WakeMed does more chest wall reconstruction procedures than any other center in North Carolina. Can you talk about that for a minute Dr. Phillips?

Dr. Phillips:  Yeah. my partner David Hoover and I have sort of a special interest in chest wall deformities. We’ve both been doing this kind of surgery for a long time. So, when he joined us at WakeMed, he and I together put this center together so that we wanted to be able to offer all different treatments. I think some surgeons whether it’s right or wrong, specialize in one way of doing things. and for kids with chest wall deformities; we didn’t think that was right. We thought some might benefit from a vacuum bell, others might benefit from compression bracing, others might benefit from the Nuss Procedure. We even have occasional patients who need the old fashioned operation, they need the Ravage Procedure.

We also have young adults who might be better candidates for a modified Nuss Procedure with more than one bar perhaps two or three bars if they are over age 18 or over age 21. So, the reason we put this sort of center together at WakeMed was so that we could offer all those different treatments. We didn’t want to find ourselves stuck doing just one thing. We wanted to just do what’s right for the patient. And in all honestly, some people don’t need anything done. Some people have a very mild chest wall deformity that really doesn’t require any treatment at all. And we want to be able to tell families that.

Sometimes people get sent to us who really don’t need anything done.

Host:  Well that’s a really good point to make too. And for a parent listening to this who obviously would have concerns; what’s your best advice for that parent?

Dr. Phillips:  I would see someone who is an expert on chest wall deformities. So, what we understood in the 1960s and 1970s is dramatically different than what we understood in the 80s and early 90s. And that’s dramatically different that what we understand now. And so, sometimes your primary care physician whether it be a family practice doctor or pediatrician may have done his or her training back in the 70s or 80s or 90s and so what they learned may be somewhat out of date. Because things have changed so dramatically in the last 20 years that it might be worth your while if you are nervous about your child to just go see someone who is up to date with the latest treatments.

Host:  Yeah, that makes a lot of sense. So, then lastly, Dr. Phillips, what is the long-term outcome for these kids? Most go on to lead normal lives. Is that correct?

Dr. Phillips:  Absolutely. In fact long-term follow up studies show that for the vast majority of patients; the quality of their life is much better. That is their ability to run around, to do exercise when they are young adults to catch up with their toddlers who are running around in the yard, to take their kids to Disneyworld, whatever. Their quality of life improves because their cardiovascular function improves.

Host:  Yeah, really important to know and remember that. Dr. Phillips, this has been fascinating. Thank you so much for your time today.

Dr. Phillips:  Thank you.

Host:  That’s Dr. J. Duncan Phillips, a Pediatric Surgeon with WakeMed Children’s in Raleigh, North Carolina. And to learn more about WakeMed’s Chest Wall Center or to get connected with Dr. Phillips or another provider please visit www.wakemed.org. And if you found this podcast helpful, please share it on your social channels and check out the full podcast library for topics of interest to you. This is WakeMed Voices, a podcast from WakeMed Health and Hospitals. I’m Bill Klaproth. Thanks for listening.