Understanding Pectus Excavatum & Carinatum
Understanding Pectus Excavatum & Carinatum
Scott Steele: Butts & Guts, a Cleveland Clinic podcast, exploring your digestive and surgical health from end to end.
So welcome to another episode of Butts and guts. I'm your host, Scott Steele, the chairman of colorectal surgery here at the Cleveland Clinic in beautiful Cleveland, Ohio. And we're very pleased to have Dr. Anthony DeRoss, who's a pediatric surgeon, and Dr. John DiFiore, who is also a pediatric surgeon and the Director of the Center of Excellence for Pectus Excavatum and Carinatum. Gentlemen, welcome to Butts & Guts.
Anthony DeRoss: Thank you.
John DiFiore: Good morning.
Scott Steele: John, we'll start with you. Tell me a little bit about your background and where are you from? Where'd you train, how did it come to the point that you're here at the Cleveland Clinic.
John DiFiore: I grew up in the Northeast. I'm from a town outside of New York City called New York. That's where I grew up. I did all my school and training in the Northeast, went to Dartmouth College, Columbia Medical School in New York, back home. Then I spent 10 years in Boston where I did all my surgical training, including general surgery and pediatric surgery.
Scott Steele: And Tony?
Anthony DeRoss: I grew up in Pittsburgh and went to medical school and college there. Did my training in New England and then a have been in Cleveland now for about nine years.
Scott Steele: Well, we're very glad to have both of you here. We're going to talk today about one of the disorders that affect children and that's pectus excavatum and carinatum. And so let's start with you John. Can you just give us some descriptions, a background of what is each of the conditions and what's the difference between the two?
John DiFiore: Pectus excavatum and carinatum are a group of chest wall abnormalities or deformities, where there's an abnormal contour to the shape of the chest and the sternum in particular. Pectus excavatum, which we see much more frequently, about 85% of the time is an indentation of the chest, where the center part of the sternum or the breastbone is pushed backward by several centimeters. Pectus carinatum is the opposite where the sternum actually projects too far forward or anterior. Pectus excavatum is sometimes called a sunken chest. Pectus carinatum is also known as a pigeon chest. Those are common terms that are used or that people might recognize.
Scott Steele: Tony, we're all built a lot differently in life. Everybody's a different way, shape, and form. So I'm assuming that there's a degree of normal versus "abnormal" here. How do you figure that out? Is there specific symptoms that go along with either one of these disorders that parents or kids should kind of watch out for, or primary care docs that see these children or pediatricians and say, "This is pectus excavatum or carinatum and look to seeing a referral for this"?
Anthony DeRoss: Sure. The biggest symptom is just the appearance of the chest. That's the symptom or sign that patients and primary care docs both usually will notice first. When you speak patients more, especially patients who are in their teenage years, when we see this condition become more noticeable because they're going through growth spurts. Those patients oftentimes with both conditions, but more commonly with excavatum compared to carinatum, will start to have a oftentimes symptoms of chest pain, shortness of breath, fatigue, and decreased stamina compared to that of their peers. A lot of these patients are very tall, thin, athletic individuals, and they start to notice that they need to take frequent breaks when they're playing soccer, or baseball, football, whatever it is, swimming. And so those are some of the symptoms that patients tell us that they're having whenever they come in to see us.
Scott Steele: So John, I know Tony talked a little bit about some of the symptoms and especially I can understand how with sports this may be a problem, but is this a cosmetic deformity in and of itself or is this something that truly is an aspect of having decreased functionality?
John DiFiore: It's really both. The term cosmetic is a little misleading because of what can really be very serious impact on the development of self-image and self-confidence and self-esteem in a developing young teenagers. So the appearance of the chest is really critically important to the psychosocial development of growing teenagers. So even though it's often seen as cosmetic, there are real implications in terms of development for that.
On the other side, what I think is underappreciated is really the physiological aspects of pectus excavatum in particular, the cardio-respiratory component that we are really trying to unravel here in our program. The shortness of breath and fatigue that Tony mentioned are probably a combination of two factors. One is simple mechanical restriction of the chest wall by the indentation of the sternum and of the pectus excavatum.
The second component is likely due to cardiac compression, typically of both the right atrium, and the right ventricle by the sternum pushing in or indenting the heart. And that may have some effect on pulmonary blood flow with oxygenation playing a role in the fatigue and in the shortness of breath. So I think the simplistic view is that it's just the chest is pushed in, and it doesn't expand very well. But what we're learning in our program, and we can talk a little bit about the testing, is that there is a significant cardiac component as well.
Scott Steele: Tony, we have a lot of parents that listen to this podcast and having a teenager myself, you can go up to the teenager and say, "What's wrong?" And they'll say, "Nothing." And so if I'm a parent that's out there, I guess, what is the threshold to seek an evaluation on these patients, especially if you get that typical response, "Nothing's wrong. I'm fine, dad. I'm mom." Something like that.
Anthony DeRoss: Very good question. I think it's interesting in this day and age of ease of access to information, we find that all the times the parents, but even more often, the patients are actually seeking knowledge about this condition when they see it. They notice that their chest looks different than their friend's chest. They go to the internet, they Google it, they find out information. And most of the time when parents and patients come to see us, they already have a fair amount of knowledge and most of it pretty accurate about what's going on and what they even want to do about it. The truth of the matter is if they're not having symptoms, and they're not upset with the appearance of their chest, there's really nothing that absolutely needs to be done. There are plenty of people who have lived their entire lives with excavatum and carinatum and have had no real consequences later in life.
We don't know that... And we're trying to find out in patients have or who develop heart and lung conditions later in life, who have pectus carinatum or excavatum, are those functions negatively impacted by the chest wall? We don't know if they have worse cardiac function because of their excavatum later in life. We don't know that. We don't have the data for that yet. But as far as we know, there's really nothing that absolutely has to be done, unless the patient is having symptoms and/or wants to have something done about it.
Scott Steele: So it's pretty scary to go to the doctor. So John, I'm a parent, I take my child in to see you. Walk me through that first initial evaluation. What can they expect in your office and what sort of tests can they expect to get?
John DiFiore: The initial evaluation is a fairly laid-back conversation with what's typically a young teenager and we ask them why they came to the office, what's bothering them about their chest? Is it just the appearance, are they having symptoms of shortness of breath and/or chest pain or other symptoms? It's a fairly a stress-free discussion. Then there's a physical examination where we look at the chest and make an assessment of the severity of the pectus, and then we'll talk about the testing involved. And at Cleveland Clinic, I think we have a fairly unique testing program, a fairly standard evaluation across most of the country is a CT scan, an echocardiogram and pulmonary function test to evaluate lung function.
Scott Steele: The last of which pulmonary function test involves what?
John DiFiore: That's essentially breathing into a machine and measuring different lung volumes to see if there's any impingement by the pectus.
What we found over about 15 years is that the vast majority of those tests other than the CAT scan that shows the indentation, most of the times those tests are going to be normal because young teenagers have tremendous cardiopulmonary reserve. They got great heart, they got great lungs, and they can overcome a lot of the issues that we might be looking for. So what we started doing about three years ago when Tony did a great really transitioning our evaluation process was we now do a cardiac MRI and a cardiopulmonary stress test where patients get on a treadmill. And what we are really starting to see is that we are picking up a lot more subtle abnormalities when we stress teenagers in terms of their cardiac response, something called cardiac work rate and cardiac efficiency. That's a much more detailed examination than just a pulmonary function test, and we're really starting to understand the cardiac component in addition to the pulmonary component and picking up on those abnormalities.
Scott Steele: Tony, is there any non-surgical treatment for either of these conditions? Is there a brace I can go out and buy or can I work extra hard on my breathing or do anything like that?
Anthony DeRoss: Sure. We actually do have a very, I think now well developed and established bracing program, particularly for pectus carinatum. You can't brace the excavatum but carinatum, we do have a brace that we've been very happy with, and our patients have had excellent results with. It's manufactured by a company in Argentina that was founded by a pediatric surgeon there, who was interested in trying to find a more nonsurgical management for carinatum, which happens to be more common in that region of the world. It's a dynamic compression brace. We can measure the exact amount of pressure that we put on the carinatum so that it is just the right amount to actually correct the chest wall shape, but not enough to cause any damage to the skin. Patients wear that for a period of months to years depending on how severe their condition is, and we've had excellent results with that so far.
For pectus excavatum, there is a vacuum bell. It's basically a big suction cup that goes on the chest and can actually reshape the chest when the patient uses that for a period of time every day. It takes a lot of determination and focus from the patient to be able to do that every day. We have found that it works well for patients who have very minor pectus excavatum, and it can sometimes be a bridge to partial correction and then sometimes patients will still want to proceed with surgery after that, but the brace for carinatum does work quite well.
Scott Steele: So just for my own information, I'm picturing these smile commercials where they put in these implants, and it changes your teeth over time. Do you want these braces actually change the chest wall or does it only work when the braces on?
Anthony DeRoss: No, it actually changes the chest wall shape over time. In the initial period, patients will tell us that they'll have it on, they'll take it off, and their chest is corrected, but then after about half an hour or an hour, they noticed that it comes back out. And so patients do have to be vigilant in wearing it, we actually recommend in most cases, up to 23 hours a day taking it off for sports and showering and hygiene purposes and things like that. But it does over time then correct. And we wean them off just like you would with braces on your teeth. For example, we have a retainer mode where they wear it and we don't tighten it, and when they wear for fewer and fewer hours.
Now patients can develop sort of not really a recurrence, but an increased development in the carinatum after they've been off of it for a while and then we just put the brace back on. That typically happens when they're going through growth spurts, but once they're done growing, theoretically it should be corrected.
Scott Steele: John, let's jump into the surgical management of it. So walk me through what that involves, and a little bit about the risks associated with it and how successful is it?
John DiFiore: Historically, pectus excavatum in particular was repaired with an open surgical procedure with an incision across the chest and removing the abnormally shaped cartilages that are pushing the sternum backward. Over really the last 20 years, I would say upwards of 95% of our patients are now candidates for a minimally invasive repair for pectus, also known as the Nuss procedure after Dr. Nuss, who invented the operation and presented it first about 20 years ago.
The advantage of the Nuss procedure, which we do in very high volume here, is that it's a two very small incisions out in the axilla or the armpit area. We put a very tiny camera into the chest and we create a tunnel behind the sternum, and behind the sternum we place a curved metal bar that will push the sternum forward into the normal position. Essentially that works. The best analogy, as Tony alluded to all ready, is that it works like braces on your teeth. The bar stays in place for a period of three years and during that time, the chest wall, the ribs, and the sternum will reshape and remodel in that normal position so that when the bar is removed, the chest stays in the normal position.
The difference between braces and the Nuss procedure, if you will, is that with the Nuss procedure, the chest wall is immediately corrected. So when families are in the recovery room, and they're looking at their child's chest wall, it looks completely corrected, 100% right after surgery. It's typically about three to four days in the hospital after surgery, and we're working on improving on that, but overall the results are excellent.
Scott Steele: And when that bar is in there, how long does it stay in there? Is it a permanent type thing, can patients walk, talk, play sports?
John DiFiore: The bar is in place for three years. Eventually they can return to complete and full activity in any type of sport. For three months, there are restrictions on most sports. We wait six months for contact sports like football, hockey, soccer, things like that. But eventually Tony and I have patients playing all kinds of sports including full contact sports like football and hockey.
Scott Steele: And then for carinatum?
Anthony DeRoss: For carinatum, patients can undergo a surgical procedure, which John already described, the ravage procedure where we go in and take those abnormally shaped cartilages out. There are really two different ends of the spectrum, same disease process with these abnormal cartilages excavatum and carinatum. And so with carinatum, we take out the cartilages, we put a small bar in to stabilize the sternum while it's healing. That bar stays in for about six to 12 months and is then removed.
Scott Steele: There's similar type of restrictions associated with that type of repair as well?
Anthony DeRoss: It is. For both the ravage procedure, which is the open procedure for both conditions and the Nuss procedure, the return to activity is pretty similar, at least for me and I think for John too, over a period of three to six months we get patients back to doing everything they want to do.
Scott Steele: John, I can imagine there might be a child with this out there that's excited about the procedure, but then is like, "Whoa, a bar in my chest and what's going on?" What's the risk of not correcting either one of the conditions?
John DiFiore: What I tell patients and their families is that it's not unsafe to leave it the way it is. And it really comes down to the factors of how much does the appearance of the chest wall bother a patient. And some patients come in and say, "You know what, I'm good. I don't want to not play soccer for three months. And it doesn't really bother me that much." But it's the appearance combined with symptoms of shortness of breath and chest pain. So it's really a lifestyle choice. And most patients that come in to see us are coming because one of those three things is bothering them enough that they want to improve it.
So can you function well with the pectus the way it is? The answer is yes, but most patients find they can't function optimally. And even patients that say they're not short of breath before the surgery when we see them in the office, I would say at least half of those after the surgery come back and say, "I remember telling you, I wasn't sure to breath, but after the surgery now I breathe completely differently and so much better."
Nobody has a sense that they've got a bar in their chest. So a lot of kids are worried about, "I've got this piece of metal." You really don't feel it at all. Most pectus patients are pretty thin, so they can feel it out in the armpit area if they really try, but they just go about their business and really have no idea the bar is even in there.
Scott Steele: Tony, I know we're focusing on kids, but there's going to be some adults out there that maybe didn't even know they had this, but they knew something was up. Only something that can be addressed in a pediatric or young adult time, or is this something if an adult comes in that they could get addressed at that stage?
Anthony DeRoss: Absolutely. We definitely see some adults in our practice, particularly for the Nuss procedure. There are some adult thoracic surgeons who offer a different type of procedure and correction for adults, but particularly the Nuss procedure, we're the only group that is doing that here at the clinic. And so anybody who's interested in the Nuss, even adults, and we've done some patients... I've done some patients in their 20s and 30s, and I think John has done some patients older up to 50 for the Nuss procedure. So for carinatum for the bracing, it becomes a little more difficult for patients to tolerate whenever their chest becomes a little more ossified and less compliance. So we can offer the brace to older patients, but it takes a lot longer for things to correct.
Scott Steele: And so to either one of you, what's on the horizon for the future treatment of pectus?
John DiFiore: What we're really working on at this point is optimizing the details of the procedure and in particular our pain control. We have gone from a spectrum of using epidurals, which we no longer use, to intravenous medications, to now starting a new trial of doing intercostal nerve blocks during the surgery to basically numb the chest wall on the postoperative period, and we've had some excellent results with that. So patients are going home more comfortable.
We're also really focusing on minimizing opioid use. That's a big important issue for our patients and for patients in general. So we've been able to really cut down on the need for narcotics and opioid medications postoperatively.
Scott Steele: Any other things that you can see if you were to go out 20 years in the future and think, "Is this bar going to be something that's still there or are we going to have something else, some new cartilage in surgery?" Anybody working on anything that's maybe a little outside of the box, whether or not it kind of comes through fruition or not? We all know how those things go, but have you heard of anything?
Anthony DeRoss: There are some different trials going on with some different devices. There are some magnetic devices that are seeking FDA approval now. They still involve an implant procedure. So to my knowledge, been anything done, particularly with the cartilage itself. But we do know that these conditions, pectus excavatum and carinatum are associated with our connective tissue disorders such as Marfan syndrome, for example, that some patients share those diagnoses. And so perhaps some of the research in those areas will spill over and help us to better control patients with excavatum and carinatum.
Scott Steele: Well, that's pretty incredible stuff, and we appreciate you coming on and talking about it. So we always like to end up with all of our guests on a couple of quick hitters, and so we'll go kind of back and forth style. John, favorite food?
John DiFiore: Steak.
Scott Steele: Tony?
Anthony DeRoss: Tough one. Yeah, pizza.
Scott Steele: Tony, Favorite sport?
Anthony DeRoss: Football.
Scott Steele: John?
John DiFiore: Rugby.
Scott Steele: And the last nonmedical book that you've each read.
John DiFiore: I read the Lord of the Rings trilogy recently.
Scott Steele: Wow, that's got to be long.
Anthony DeRoss: My neighbor writes mystery novels. And so the last book I read was hers.
Scott Steele: Oh, fantastic. And then to each of you, you've spent some time here in Cleveland, so what is it that you like about Cleveland? John?
John DiFiore: Cleveland was just a fabulous place to raise my family. My daughter was born in Boston, but my two boys, my twins were born here in Cleveland, so they grew up their entire lives here and it was just a fabulous place to raise a family.
Scott Steele: Tony?
Anthony DeRoss: It's funny, my wife came here to train at the clinic in her specialty, surgical specialty, and we never thought we'd end up staying in Cleveland, but we really love it and it's a fantastic place.
Scott Steele: Well, that's fantastic. And so for more information about pectus excavatum and carinatum, please download our free treatment guide at clevelandclinic.org/pectus. That's clevelandclinic.org/pectus, P-E-C-T-U-S. And to make an appointment with a Cleveland Clinic specialist, please call 216.442-4378. That's 216.442-4378.
Gentlemen, thank you for joining us on Butts & Guts.
Anthony DeRoss: Thank you very much.
John DiFiore: Thanks Scott.
Scott Steele: That wraps things up here at Cleveland Clinic. Until next time, thanks for listening to Butts & Guts.