Deborah Howell (Host): Medical conditions affecting the chest wall of patients can sometimes go undiagnosed in females resulting in prolonged symptoms and a delay in receiving treatment. Shriner's Children's Portland specializes in orthopedic care, including chest wall conditions in children and teenagers. Today we're joined by Dr. Kimberly Ruscher, a pediatric surgeon who specializes in chest wall conditions. Welcome to Healing Heroes PDX the podcast series from the specialist at Shriners Hospitals for Children in Portland. I'm Deborah Howell. Dr. Ruscher to get us started, can you tell us about chest wall conditions? What are they and how common are they?

Kimberly Ruscher, MD, MPH, FACS (Guest): Well, Deborah, thanks so much for having me to talk about chest wall conditions. Here at Shriners Hospital in Portland, we see hundreds of children every year for a chest wall problem. Most commonly pectus excavatum and pectus carinatum. These are two conditions of the chest wall that affect the shape. In one, the chest wall and the front sticks out and the other one, it sinks backward or is depressed. Pectus excavatum is the depression of the sternum or breast bone sometimes called funnel chest. And then the opposite problem, pectus carinatum, where the breast bone or part of the chest wall sticks out is called pigeon chest. These chest wall conditions used to be thought to be more common in males and effecting one in four to 500 people.

However, we've found that these conditions are often underdiagnosed in females, especially in teenage females. And we now believe that as many as one and 200 or one in 300 people may have a chest wall deformity.

Host: Wow. Incredible. How might a diagnosis differ between girls and boys?

Dr. Ruscher: That's a great question. Historically, the thinking was that males are more commonly affected. However, that is probably due to the fact that it was easier to see the problem in a male. As children go through puberty and they go through growth spurts, that's when these conditions very commonly show up and in girls, of course, that's also, when we see breast development. The breast development can make it harder to appreciate changes in the shape, shape of the bones of the chest wall.

The development of the breasts can make it harder to see changes in the shape of the chest wall. Also, there is sometimes hesitancy in the primary care offices for girls to want to take off their bras and or shirts thus harder for their physicians to identify the problems at the right time.

Host: Oh, that makes perfect sense. Now, in terms of symptoms, what should parents look for? Is there a certain age range when patients usually receive a diagnosis?

Dr. Ruscher: Some children will have abnormalities in the shape of their chest early in life. For example, we very rarely will see infants who have an apparent problem, especially a depression, or excavatum seen quite early, but more commonly children begin to show symptoms of problems in the shape of their chest through the late elementary school or middle school years.

And they become especially prominent through puberty. Anyone who has a change in the shape of their chest can be diagnosed at times that the symptoms or problem appears, but that's usually in puberty.

Host: Okay. And how does a chest wall condition impact the overall health of a patient?

Dr. Ruscher: The answer is, it depends. If a patient has a pectus carinatum where some part of the front of the chest is sticking out or protruding, that shape does not affect the overall function of the heart or the lungs. Thus patients with carinatum will usually have no health problems. However, a patient with pectus excavatum who has a depression of their sternum can have problems because the heart can be compressed.

What I mean by this, is that the sternum or breast bone, as it gets deeper and deeper will exert more and more pressure upon the heart, specifically the right ventricle. The more pressure there is on that heart, the less, the patient is able to tolerate exercise. The more pressure there is on the right side of the heart from the pectus excavatum the less, the heart can work hard during times of exercise.

So for example, a child may be very active running around in PE, active in sports, but over time, their heart can not work as hard as their peers, thus they'll tire out more quickly. So, compared to their friends, they may get short of breath more quickly, have racing heart or chest pain and have to stop their fun or exercise more quickly than kids their own age.

So we sometimes see kids who have such severe compression of their heart, that they can't happily participate in normal family activities. I had a girl who who really enjoyed hiking with her family. It was their family's thing to do. And her pectus got worse throughout her teenage years, to the point where she had to stop so much, she lost the joy in that activity. And after her pectus was corrected and she was recovered from her surgery. That was a message she sent to us. I can hike my favorite hikes again.

Host: Oh, that is so wonderful to hear. Now, do children need any studies like x-rays if they have a chest wall condition?

Dr. Ruscher: Sometimes they do. And sometimes they don't. For children who have a protrusion or pectus carinatum, we usually don't need any studies. It's just a good old fashioned physical exam by a physician can identify the problem. And we can get those patients into needed treatments. However for patients who have a depression problem of their sternum, for patients who have a depression of their sternum or excavatum, we do want to do additional studies to try and identify how severe is this problem.

You can tell from the outside, they have the diagnosis, but we need extra studies like x-rays or perhaps a CT scan, and also an echocardiogram to know is the heart being compressed. How deep back is the sternum? Is there any effects on the function of the heart from the compression? And we occasionally are also looking for other problems with the heart or vasculature in those patients.

Host: And what types of treatments Doctor are available for these conditions?

Dr. Ruscher: If a patient has a pectus carinatum, we will use a brace, which is an extremely effective therapy to gradually apply pressure and push that sternum back to a flat position. This brace can be made at our bracing clinics at Shriners and is 90 to 95% effective in correcting pectus carinatum when used correctly.

Host: That's incredible.

Dr. Ruscher: It is incredible. Isn't it? It's so great that we have a safe, durable non-invasive therapy for pectus carinatum that can help so many patients. For children who have pectus excavatum we generally don't offer any treatments until the teenage years. The reason being is that the treatment is a surgical implant of a stainless steel bar that lifts up the sternum back off of the heart.

The surgery is called minimally invasive repair of pectus excavatum or NUSS procedure. We only offer this surgery for patients who have moderate to severe cases, who have evidence of effect on the heart from the problem. This surgery is done at Shriner's Hospital, Portland, and most patients stay about one day after the hospital, perhaps two. Most patients spend one or two nights in the hospital after thw surgery.

Children who have this procedure done in the teenage years, have the bar that stays in for three years. They're able to be active like normal once they're fully recovered from the surgery. And when the bar comes out, it's an outpatient surgery. And the great thing that patients tell us is that they get to take the bar home and keep it as a keepsake. The reason-

Host: I love it.

Dr. Ruscher: When patients have pectus excavatum surgery, the goal is to alleviate the pressure on the right heart so that they can live a normal, healthy life. They can hike, they can participate in sports, they can be active, but more significantly to me, there are a lot of patients who are bullied or teased or who feel extremely self-conscious about the appearance of their chest. And the transition we see in these patients to confident teenagers who are more willing to participate in normal activities that are age appropriate is incredible. We've seen kids would barely make eye contact with us at their initial consults. And by the age of 17 or 18, they are just blossoming. And as a physician, what more could I ask in terms of professional gratification and feeling like I'm doing the right thing for my patients.

Host: That has to be the best part of your day.

Dr. Ruscher: It really is.

Host: Dr. Ruscher, why should a family seek care at Shriner's Children's for chest wall? In other words, what makes our care unique?

Dr. Ruscher: Shriners has such a long, fantastic history of wanting to serve every child, no matter what. And this program serves that mission. Our program for chest wall deformities is led by experts who have some of the longest term experience in the Pacific Northwest, with these conditions. We offer compassionate care. We follow the best practices in terms of safe provision of effective care for these conditions. And we're happy to see children of any age with these problems and help get them on the path towards needed treatment.

Host: And what, in your opinion is the optimal outcome of a child receiving chest wall care?

Dr. Ruscher: As a pediatric surgeon, I think it's so important that every family walks away from a visit with their surgeon with a clear understanding of what the condition is and what their options are. And from there, the outcome of that consult or visit would be if a family chooses to undergo therapy for the condition, that they receive a safe treatment, that's durable, that lasts the child throughout their whole life. And with excellent attention to every little detail of pain control, physical recovery from surgery and the experience of being in the hospital, making it as easy as possible for everyone involved in that child's care.

Host: And once these procedures are done, that's it? The, the chest wall remains in that current position?

Dr. Ruscher: That's a great question. We take advantage of a couple physical changes that happen to the body in the teenage years when choosing when to do the surgery for pectus excavatum. If a teenager has a NUSS bar or stainless steel bar placed for pectus excavatum in the teenage years, it then stays in three years. At the end of that time, the body has remodeled the shape of the chest wall and that remodeling and scar tissue from the surgery holds the chest wall in that position for their entire life.

Host: Absolutely incredible. Is there anything else you'd like to add to our conversation today?

Dr. Ruscher: I would just like to say, I'm so grateful to Shiners Hospital for allowing us to continue this program after such a long-term. We really do offer services to a broad group of patients in the Pacific Northwest. And we're happy to see kids of any age with these problems. And if it's an adult hearing this podcast who thinks they're also affected by this condition, Shriners can help guide you to experts for adult pectus consults.

Host: Good to know for sure. Well Dr. Ruscher, thank you so much for being with us today and for all the excellent information we really enjoyed having you on.

Dr. Ruscher: Deborah, thank you so much for having me.

Host: For more information about care provided at Portland Shriner's Hospital, please call 503-221-3422. Or visit our website at portlandshrinershospital.org. And that concludes this episode of Healing Heroes PDX, the podcast series from the specialists at Shriners Hospitals for Children. Head on over to our website at portlandshrinershospital.org for more podcast episodes. I'm Deborah Howell. Have yourself a terrific day.