Scott Webb (Host): Pectus or chest wall deformities range from mild to severe, and experts like my guest today specialize in helping patients with operative and non-operative treatment options to address their medical issues and cosmetic concerns. And I'm joined today by Dr. Marisa Schwab. She's a pediatric surgeon with a clinical and research interest in chest wall deformities. And I'm also joined by Dr. Stephanie Chao. She's a pediatric surgeon and program director for the chest wall program at Stanford Medicine Children's Health. 

This is PedsTalks, I'm Scott Webb. Doctors, it's nice to have you here today. We're going to talk about chest wall defects and how you help folks. So from diagnosis to treatment, surgery, all of that. It's great to have you here, great to have your expertise. And Dr. Schwab, I'm going to start with you. Just have you provide a brief overview of chest wall defects in children and maybe highlight why they are so important for all of us to understand.

Dr. Marisa Schwab: Thank you, Scott. We're so excited to be here to talk all things chest wall with you today. So, the two most common types of chest wall defects we encounter are pectus excavatum characterized by the posterior depression of the sternum and then pectus carinatum, so anterior protrusion. And while historically these were often dismissed as purely cosmetic or psychological concerns, our evolving understanding of thoracic biomechanics suggest that there's a much more significant physiological impact. So, the critical thing to understand is how these structural abnormalities can alter cardiopulmonary physiology.

And beyond the common pectus deformities, there's a complex spectrum of rare chest wall conditions that require highly specialized multidisciplinary approach. So, Poland syndrome is a rare, typically unilateral congenital anomaly that's characterized by either the underdevelopment or total absence of the pectoralis major muscle. And then, thoracic dystrophy and sternal clefts are other rare chest wall anomalies that we see in our clinic.

Host: Yeah. Dr. Chao, I want to have you talk about the Stanford Chest Wall program and the role maybe it plays in the treatment of these conditions.

Dr. Stephanie Chao: We really designed the Stanford Chest Wall Program to serve as a medical home for any patient with a chest wall condition. We know that these are not common conditions and the average family practice provider or primary care provider may not encounter these conditions very often. But it is a very visually obvious condition, and a lot of times patients and their families have quite a bit of concern and anxiety over these conditions. So, we are happy to take these on behalf of the primary care provider. So, we have created a multidisciplinary program where it starts with the surgical team of surgeons and advanced practice providers. So, those are our nurse practitioners and physicians assistants. But in addition to our team, we have radiologists who are dedicated to helping us image, these conditions in the best possible way while minimizing ionizing radiation for children. We work with a team of cardiologists and pulmonologists to do cardiopulmonary function testing, echocardiogram for complete evaluation of structural anomalies of the heart. And we also work with our anesthesiologists and pain clinic to ensure that, postoperatively, we provide our patients with really the smoothest, fastest functional recovery.

Host: Yeah, a real team effort as you would expect at Stanford Children's. And Dr. Schwab, learning from you today from both of you, that it's not just cosmetic or psychological, that there's, you know, some real health implications here. So, I want to have you talk about how these defects impact the child's heart and lung function, and maybe what are the potential risks if it's left untreated.

Dr. Marisa Schwab: Pectus excavatum, we commonly see a tangible compression, especially of the right ventricle. So, this can manifest as a reduction in stroke volume, impaired diastolic filling, maximal oxygen uptake, also known as your VO2 max, especially during peak physical exertion. And while often if you ask teenagers who have even severe pectus excavatum about shortness of breath or their ability to do the same activities as their peers, they may not have noticed any difference.

But a lot of that has to do with this is, all they've ever experienced, right? And while they may be able to compensate while there are these healthy teenagers, it's common that later in life, even in their, you know, 30s, that they start noticing that they just can't push through the way they used to. And at this point, they've really hit a ceiling.

And then, at that point in their life, they start wishing that they had done something about their pectus earlier on, when the surgery is much better tolerated when you're a teenager. We are also increasingly recognizing the long-term potential for early-onset atrial arrhythmias when you get this chronic right atrial compression.

So, all of that was pectus excavatum. With pectus carinatum, the primary issue is really one of chest wall compliance, so the stiffer thoracic cage increases your work of breathing and can sometimes even mimic restrictive lung disease on your pulmonary function testing.

Host: Yeah. Dr. Chao, I'm sure that surgery's on the table for a lot of these patients, but maybe there's some other treatment options before you get to surgery or instead of surgery in some cases. So, I want to have you talk about the different treatment options and how do you determine the best course for each patient?

Dr. Stephanie Chao: Yeah. I am a parent myself, and I would never want to walk into a doctor's office and be told your child has to have surgery and there's no other option. We really try to individualize the treatment plan for every patient. And I tell all of my patients, "I am not in the business of selling surgery to you. We have to make sure that this is the right choice for you and your lifestyle." So for patients with mild cases, we have something called the suction bell. We're the first center on the west coast to start using the vacuum bell in a very structured format that is medically supervised.

And while that is not as successful as surgery is, it is an option. And I think as parents, we want to know that we've exhausted all those non-invasive options for our children before we kind of make the next step for surgery. Now, surgery certainly is the most tried and true treatment, and it is minimally invasive surgery. It's called the Nuss procedure. Where we make two small incisions. And we insert titanium bars to basically push out on the pectus excavatum to push out on the sternum and instantly correct the chest. So in the course of a 60 to 90-minute surgery, the patient comes in with a sunken chest. And when they wake up, their chest is completely corrected.

Every patient and their family comes in with a certain mindset. Some come in as an empty slate and really just want to learn about it. And that is why we have our dedicated chest wall clinic time and our program to really sit down with every patient, understand where their at today, what their concerns are, and kind of revisit the various options. You know, today there may not be a great treatment option or patients may not feel a need for treatment, but we follow them over time to ensure that everyone has kind of an individualized plan.

Host: Right. Sort of a surveillance, right? You know, move along with them through this journey and maybe, you know, eventually, they get to the point where surgery is on the table

Dr. Stephanie Chao: Exactly. I have some patients who I've met before they went into puberty, so they were young and in elementary school, very flexible chest walls, but they always noticed that they had what appeared to be a sunken chest. And over time they've tried certain options, non-operative options, and some progress eventually to surgery.

And after surgical corrections, three years after surgical correction, we actually have to remove the bar. And usually by then, they're ready to go off to college. And so, I've seen them grow up in front of me. We've become part of their lives. And so when they graduate from me, it is usually around the time they graduate from high school. And it's kind of like a bittersweet graduation for both of us.

Host: Of course. That's so cool. Dr. Schwab, let's talk about the stay with surgery and talk about some of the recent advances in surgical treatment of pectus excavatum. You know, what do we want pediatric providers to be aware of in terms of the advances?

Dr. Marisa Schwab: Yeah. I echo Dr. Chao. Really early referral to a pediatric surgeon is essential. Not necessarily for intervention, but to establish that baseline, and then follow patients over time. So, we now have really sophisticated tools like cardiopulmonary exercise testing, rapid MRI, that quantifies exactly how much a patient's anatomy is limiting their physiology or their potential physiology. Moreover, we've seen a massive shift in how we're managing these kids.

For carinatum, external bracing has become the gold standard. It's really, really rare to need to operate on these patients. For pectus excavatum, we've moved towards the minimally invasive Nuss procedure that comes with cryoablation. So, that's fundamentally changed the postoperative pain profile, it's shortened hospital stays. At Stanford, we use titanium bars, so we no longer have to worry about allergic reactions. And we use stabilizers between the bars, so that eliminates the need for the painful suturing that used to be done.

And then, finally, there's a huge emphasis on early functional restoration with enhanced recovery after surgery pathways or ERAS. So at Stanford, in addition to ERAS, we also have a NUS target-based care pathway. And basically, what that means is that everyone from the anesthesiologist, the surgical team, the nursing, the patient, the family, everybody has this shared mental mindset of what the postoperative milestones are and how we can get there as a team.

Host: Yeah, Doctor, I'm just picturing, you know, without any real frame of reference, I'm sort of picturing what it used to be like, right, for these patients And what you're doing today, just the multidisciplinary, minimally invasive, and it just feels like it's so much better, right?

Dr. Marisa Schwab: Yeah. I mean, patients used to spend a week in the hospital, and now almost every patient at Stanford goes home the next day.

Host: Amazing. Yeah. I'm doing a lot of head shaking today listening to you both just like it's amazing. I love it. And Dr. Schwab, can early intervention—I'm assuming yes—but can early intervention or specific lifestyle factors even maybe help mitigate the progression of chest wall defects?

Dr. Marisa Schwab: The concept of mitigating progression and chest wall defects has shifted significantly from this wait and see approach to more proactive management. For the pectus carinatum patients, the window of opportunity is really early adolescence. So, doing the bracing is most effective when the chest wall is still pliable. And then, for the patients with pectus excavatum, the vacuum bell that Dr. Chao mentioned has really emerged as the non-surgical tool to mitigate deepening of the defect ideally in patients who are 11, 12 years old, with a mild to moderate defect.

And then, while exercise itself cannot cure pectus, specialized physical therapy can modify the appearance of the chest. So, many pectus patients will develop a slumped or kyphotic posture. So, strengthening your core, your upper back, helps the patient maintain an upright thoracic frame. So when we see our patients in the chest wall clinic, we give them a set of exercises that was developed by one of our physical therapists, specifically for patients with pectus.

Host: Yeah, I see what you mean. We could all have better posture, me included right now while I'm talking to you both into the microphone. But you can see how it would really help these patients. And it's so cool my daughter wants to be a physical therapist and it's so cool that one of the PTs sort of created this program.

Dr. Marisa Schwab: Yeah, it's incredible. It really speaks to the multidisciplinary nature of our team.

Host: Yeah. And sticking with that, Dr. Chao, that is exactly what I wanted to ask you about, is have you talk about the multidisciplinary team at Stanford and how it contributes to comprehensive chest wall defect care, you know, including physical therapy, but even beyond.

Dr. Stephanie Chao: We get to live and work in such a rich environment here at Stanford that we really want to bring the expertise of other specialties in addition to our pediatric surgical expertise. So that starts at diagnosis. We used to get a CT scan for every single patient who was being evaluated for a pectus excavatum condition. And while it generates really nice images for surgical planning, it is quite a large radiation burden for the patient. And so, we switched to a rapid MRI that was really developed just for our chest wall program where our MRI radiologist helped us develop a functional MRI that looks at patients during various phases of inspiration and expiration. And they're actually able to look in part at the cardiac function in these various phases of respiration. And then, you follow that through to surgery.

So, Dr. Schwab mentioned our ERAS pathway and our targeted clinical pathways, And that includes how we administer even anesthesia. From before the time the patient goes to sleep, they'll receive anti-inflammatory, kind of preventative pain medications in the preoperative area through what they receive during their anesthetic until when they wake up and are in recovery. We try to standardize this in a way so that we can find kind of the best mix for our patient population without over anesthetizing or exposing them to perhaps unnecessary opioids.

And then, postoperatively, our multidisciplinary care extends to the partnership with our pain management and anesthesia teams. So, the clinical pathway for how we manage pain after surgery was developed after looking at how our patients respond to the various pain regimens that we've used in the past and really try to target it down so that we minimize their long-term use of opioids and use other adjunct medications, like medications that target the nerves or muscle relaxation or anti-inflammatories. And even before surgery when we have some patients who are extra apprehensive about undergoing surgery or maybe struggling with other emotional issues, sometimes we develop a treatment plan with them for their pain management long before they have surgery. They work with our team of pain specialists, which includes a pain psychologist so that they are emotionally prepared for surgery as well. So, I think we try to bring in our multidisciplinary team and kind of every aspect of the evaluation and treatment.

And then, finally, we want people to return to full function as soon as possible. And that means engaging with our physical therapist even as early as the day of surgery, and every day they're in the hospital and if needed, when they return home after surgery as well.

Host: Yeah. Just listening to you both today and learning about this topic, you know, and it just really does seem like it is a physical issue, obviously, but also psychological, emotional. You can really get a sense, Dr. Chao, of the importance, the impact of the team, this multidisciplinary team coming together and working together. Everybody pulling on the same end, if you will, to help patients to help their families, right?

Dr. Stephanie Chao: Exactly. We all have this goal center, patient-oriented care, where really the patient's maximal social and physical recovery is at the core. And so, it takes a village to raise a child and it takes a village to heal a child. And that's one of the beauties of being here at Stanford Children's, is that we're able to rally the team around the patient.

Host: Dr. Schwab, a chance here. Final thoughts, takeaways, A key takeaway, if you will, that you want listeners. And this is a podcast obviously for other providers, for your peers, if you will, about chest wall defects and their management.

Dr. Marisa Schwab: I think we have three essential takeaways for you regarding chest wall defects today. The first is that these defects are not merely cosmetic. They really represent a physical mechanical alteration of the thoracic cage. And when the chest wall is restricted, the heart and lungs are forced to work in a constrained environment.

The second is that the paradigm of management has dramatically shifted. We've moved far beyond the wait-and-see era between bracing for care and the vacuum bell and minimally invasive Nuss surgery combined with cryotherapy. We're rarely able to give patients many more options. And then, finally, it's never too early or too late to refer a patient to a chest wool specialist.

Host: Yeah. That's what I was going to followup with Dr. Chao, you know, Dr. Chao, like for other providers, what's in your opinion the optimal time to refer patients to specialized care like the Stanford Chest Wall Clinic?

Dr. Stephanie Chao: I think whenever you or your patient or their families notice that there is something different that you can't explain about the chest wall, we are happy to see them. I think a lot of times families and patients have noticed that something's not quite right for some time, but they're not sure if they should mention it. And I've heard a lot of providers worry that they don't want to refer to early or they haven't done a full enough workout. And the reality is that this is a very sub-specialized condition. And so, we are here to really answer those questions and take that burden off the primary care provider. So, no time is too soon. We will figure out the optimal interval of following up with our patients. And, you know, anytime it crosses the patient's mind or the provider's mind, go ahead and send a referral. We are always happy to see these patients.

Host: Yeah. As you say, it really is a subspecialty and, you know, pediatricians are the best. I get to talk to them all the time for Stanford Children's. But, you know, we have experts like yourselves and the team there who work with these patients and why not? It's never too early. Dr. Schwab, last word to you today. How can listeners—in this case, other providers perhaps—get in touch or learn more about the services offered at Stanford Children's Chest Wall program?

Dr. Marisa Schwab: So, you can go to chestwall.stanfordchildrens.org. And then, on the website you can actually, submit a referral for your patient, or there's also a number you can call us directly to speak to one of the providers, and get your patients seen by us as soon as possible.

Host: Well, it's great to have you both here. Great to learn from you, have your time, your expertise. I feel like I know a little bit more today and that's good. Knowledge is power And so forth. So, thank you both. Really appreciate it.

Dr. Stephanie Chao: Thank you for having us.

Dr. Marisa Schwab: Thank you.

Host: And for more information, visit chestwall.stanfordchildrens.org. This is Peds Talks from Stanford Medicine Children's Health. I'm Scott Webb. Stay well, and we'll talk again next time.