Chest Wall Deformities in the Pediatric Population

Dr. Meghna Misra discusses Chest Wall Deformities in the Pediatric Population.
Chest Wall Deformities in the Pediatric Population
Featured Speaker:
Meghna Misra, MD MS- Attending Pediatric Surgeon, Elliot Hospital
Dr. Misra is originally from Massachusetts. She completed her general surgery training at Beth Israel Deaconess Medical Center in Boston. She completed a research fellowship in Pediatric Surgery at Boston Children's Hospital and her clinical fellowship in Pediatric Surgery at Oregon Health and Science University. Dr. Misra has been an Attending Pediatric Surgeon for 7 years. She came to Elliot Hospital last autumn, after spending 6 years as an attending surgeon at Connecticut Children's Medical Center in Hartford. In Connecticut, she served as the Director of the Center for Chest Wall Deformities. Dr. Misra has published and presented some of her research in Pectus Excavatum at national meetings.
Transcription:
Chest Wall Deformities in the Pediatric Population

Prakash Chandran (Host):  As parents, our children’s health and wellbeing are always in the front of our minds. So, what happens when your child’s chest looks a little bit different than the other kids their age? Today, we’ll be talking about chest wall deformities in the pediatric population and here with us to discuss is Dr. Meghna Misra, an Attending Pediatric Surgeon at Elliott Hospital. This is The SolutionHealth Podcast from SolutionHealth. I’m Prakash Chandran. So, Dr. Misra, it’s really great to have you here today. Let’s start with the basics. What exactly is a chest wall deformity?

Meghna Misra, MD, MS (Guest):  Well first I just want to thank you by giving me this opportunity to discuss this topic today. As a pediatric surgeon, I’ve been trained to operate in the chest, in the abdomen and in the head and neck and the extremities. But operating on the chest is a particular love of mine. And chest wall surgery and correction is one of my favorite operations. So, in terms of what a chest wall deformity is, the chest wall of a child encompasses their ribs and their sternum. And it can have different appearances. These appearances deviate from how we expect the typical chest to look. And I think the word deformity is a bit harsh. Because sometimes kids can just have different appearances that are extremely subtle.

Host:  Yeah, that makes sense. And so, just as a parent, just so I can get a good sense of this; how might I be aware that something might be a little bit off?

Dr. Misra:  So, really what parents notice is that either the breastbone, the center of the chest, either sinks in quite a bit or it sticks out. Sometimes that difference or that appearance is very obvious and sometimes it’s extremely subtle. But it’s just the breastbone just looks different form how we expect a breastbone to typically look.

Host:  Got it. And at what age does this normally start manifesting?

Dr. Misra:  So, some kids are bone with it and right at the get go, parents realize as babies that their child’s chest just looks a little bit different. Other times though, it doesn’t appear until kids go through puberty. One day they look fine and then overnight, it seems like all of the sudden, their chest is just caved in and that they’ve been through some sort of trauma which they haven’t. It’s just the way their chest is developing.

Host:  Understood. So, maybe let’s talk about the different types of chest wall deformities that you’ve come across.

Dr. Misra:  Here at Elliott, we see kids with all different types of chest wall deformities. The most common one we see as pediatric surgeons is called pectus excavatum and that’s the condition where the breastbone sticks in. The second most common one we see is something called pectus carinatum where the breastbone sticks out. Then there are more rare conditions such as Poland Syndrome or slipping rib syndrome, sternal defects but these are far less common than pectus excavatum and pectus carinatum.

Host:  Okay, got it and just in terms of talking about how common this is, how many children does chest wall deformity actually effect?

Dr. Misra:  Every condition occurs with a different frequency. The one I’m going to focus on today to discuss, is pectus excavatum. And this one occurs in roughly one in every three to four hundred births.

Host:  Okay and just one more general question. Why exactly does these anomalies occur?

Dr. Misra:  So, that’s a great question. And the cause actually has not been established. Pectus excavatum can be associated with other musculoskeletal anomalies such as scoliosis, which is very common, or another condition called Marfan syndrome. This relationship with these other processes suggests that the abnormalities with connective tissue may play a role in to why some kids develop pectus excavatum or carinatum. Roughly 30 to 40% of patients with pectus excavatum will have another family member with a similar condition. So, this does support a potential genetic predisposition in many cases. We also see a lot of kids that just have this as an isolated incidence within their family.

Host:  Okay. And do you find that this is more typical in boys versus girls and is race ever a factor?

Dr. Misra:  We definitely see this condition more in boys than girls. It occurs about four to five times more commonly in boys than it does in girls. In terms of race, it seems to be more predominant in patients of a Caucasian background.

Host:  So, you mentioned today that we were going to focus on pectus excavatum because it is the most common. I am a parent, let’s say that I notice in my daughter for example, that her chest cavity is caving in. What are the steps that I would take like when I for example, come to doctor like yourself, talk to us a little bit about the process.

Dr. Misra:  The process begins first in the surgery clinic where I would see a patient a boy or a girl. And at the very first encounter, I just try to make patients and their families understand that the appearance of the chest is nobody’s fault, and we don’t know why it occurs in some kids and not others. But just like some kids are tall, some kids are short, some kids have chests that look a little bit different than others do. The first thing that I do every evaluation begins with a history and a physical exam. I try to get a sense of when the difference started to be noticed, whether it was something that was noticed right when the child was born or whether it’s something that occurred as the child was going through puberty. And after I do get a better sense of the history, I try to get a sense of the symptoms that the child is having. Typically we see kids complain of shortness of breath with exertion, decreased endurance and sometimes they develop chest pain as well when they are trying to exercise. And other times they have absolutely no physical or no physiologic symptoms whatsoever. But what is bothering them the most is how they occur, and we feel that as surgeons, we feel that psychologic impact that the child’s having because they are worried about their appearance of the chest matters just as much as the other symptoms that they could be having.

Host:  Okay, so after you perform that initial assessment, and you review all the potential side effects that the child might be having; talk to us a little bit about the treatment options both surgical and nonsurgical.

Dr. Misra:  I get a sense of how severe the pectus is based on the child’s physical exam and after the history and physical, I then recommend that every patient at least start with a chest x-ray, a two view chest x-ray which has minimal radiation exposure. And that gives me a better sense of how deep the pectus is. Once the chest x-ray has been obtained I then, that with my physical exam I can then get a sense of whether the pectus appearance is severe or mild. The more mild cases, we can talk about nonoperative management and the most popular nonsurgical option is something called a vacuum bell. The vacuum bell was developed in Germany by Eckart Klobe back in 2005. And the bell uses a suction cup to create a vacuum seal on the chest wall. So, by applying the suction, the bell pulls the sternum up and this have been shown to correct mild pectus excavatum in some adolescents who still have flexible chest walls.

And this correction can actually last through a patient’s adolescence. So, that is one option for patients with mild pectus. Other patients who have mild pectus can just do some exercises to actually build up their pectoralis muscles in their chest wall and just doing those exercises and building up that muscle can actually minimize the appearance of the pectus if they are not having symptoms and if the appearance is really not that bothersome to them.

Host:  Okay, that makes a lot of sense. Let’s say that you try the most mild treatment first like the vacuum or those exercises and that doesn’t work; when do you actually establish that surgical treatment is necessary and what does that look like?

Dr. Misra:  If a patient has a chest x-ray that and based on my assessment in terms of my physical exam and the chest x-ray, the pectus is on the more moderate to severe spectrum; then I typically don’t recommend the vacuum bell because it’s not going to be the gold standard treatment. The gold standard treatment for patients with moderate or severe pectus excavatum is a procedure called the Nuss Procedure. And this Nuss Procedure was devised roughly 25 years ago. So, if I feel that a patient is going to be on more of the surgical end of the spectrum, after the chest x-ray, I then go ahead and get a CAT scan and with the CAT scan, there’s something called the Haller Index that’s calculated. The Haller Index is a ratio of how wide the chest is to how narrow the distance is between the back of the sternum and the front of the vertebrae. If that index is over 3.2, then the patient’s chest wall appearance is thought to be severe enough to warrant consideration of surgical intervention.

So, if the patient and the family and I decide that a child will ultimately benefit from surgery, I’ll advise at that point that the patient undergo the Nuss Procedure.

Host:  Okay. I know that for some families, well pretty much for everyone; surgery can be quite a serious issue and something that they don’t want to necessarily expose their children to. I’m curious as to what happens if they choose not to have their child undergo the surgery even if the recommendation is there.

Dr. Misra:  Well the good news is that pectus excavatum is not a life threatening condition. And there are several people with this condition who choose not to undergo this operation for one reason or another. And a child’s life will not be shortened or put at risk for major cardiac or lung problems down the line if they choose not to undergo surgery. However, there is more literature that suggests that patients with severe pectus excavatum as adolescents who do not have the operation performed in that time period, do have a compromised quality of life as they get older. And this is manifested by progressive lack of endurance and chest pain with exertion as they get older. And the reason for this has to do with the depression of the chest wall from the pectus that keeps applying pressure on the right ventricle of the heart for such a prolonged period. So, it’s not life threatening but it can impact the quality of their life down the line.

Host:  Understood. Yeah, I was just going to ask you around the best time to get this treated and my sense is obviously the younger you are, the more malleable your chest wall is and so if it’s something that you notice early, and you do want to get it addressed, earlier the better. Isn’t that correct?

Dr. Misra:  To an extent. It’s actually best performed, the Nuss Procedure, when children have started to go through puberty. Typically after their first major growth spurt. So, at this stage, there’s enough flexibility of the chest wall that the operation is not as painful as it could be if the bones are more rigid. And for girls, this age range is typically 13 to 16 years old and for boys the ideal range is about 14 to 17 years old. So, they have enough flexibility to tolerate the Nuss Procedure without too much pain. That bar that is placed during the time of the Nuss Procedure, stays in for three years. And by the time that bar is removed, then there’s enough solidity of their chest wall that the pectus wont come back. So, those are the ideal ages but that being said, I’ve performed this operation on young adults into their early to mid-20s and there are even centers in the country that will perform this operation on adults beyond their 20s. I think at the beginning of puberty and later, you can really get it done at any time but the optimal age range is in that adolescent period.

Host:  Okay, let’s talk a little bit about the recovery and the pain after surgery. Talk to us a little bit about what that looks like if they get it performed in the age range that you just mentioned.

Dr. Misra:  So, it is certainly a painful operation no matter what age you get it done. With the surgery, the child’s chest contour changes essentially instantaneously. So, that means the child’s sternum or their breastbone which has been used to being depressed for 15 years or so is all of the sudden pushed out considerably. As a pediatric surgeon, it’s probably the most painful operation that I do. But at the Elliott we take many measures to minimize the amount of pain that children are having. So, the first thing I do is use catheters called ON-Q pumps in the operating room at the time of bar placement and these are pumps that are tunneled under the skin and deliver topical pain medication over the area where the bar rests on the side of the chest. And I actually even wrote a paper about the use of these pumps for the Nuss Procedure. Specifically and found out the duration of the hospital stay is decreased with the use of these pumps. And in addition to these pumps, I give patients IV pain medication around the clock for the first few days after surgery and I really try to rely on the use of nonnarcotic agents, but I will use narcotic agents carefully if necessary.

And often by the time children are ready for discharge home, they’re comfortable with just the use of the Tylenol, ibuprofen and a muscle relaxant and many of them no longer require any narcotic medications at the time they are discharged. And along these lines, it’s important for families to know that the pain improves considerably over the course of the first few weeks after surgery. Many children can return to school within two weeks of the operation. And there are no restrictions on activities or sports at three months following bar placement. So, while it is a lengthier recovery than other surgeries I would typically do; children bounce back from the operation very well.

Host:  Yeah, that’s really good to hear. And another thing I wanted to ask is are there other measures that Elliott Hospital takes to just ensure that children are as comfortable as possible throughout this process or procedure?

Dr. Misra:  Absolutely. As a surgeon, I try to make the experience as pleasant as possible for every child and their family, so I try to build that rapport as soon as the patient comes in for their first evaluation in my clinic. And if we are going to go down the surgery route; I try to make this as good of an experience starting all the way from the time, they get their first CT scan. The radiologists and techs at Elliott really dose the CT scan appropriately so that they minimize the radiation exposure to the pediatric patients. From there, if we are talking about surgery, I bring the child and their parents back to clinic to show them the actual CT scan pictures so that everybody has a better visual understanding of why this surgery will help. And then on the day of surgery, the child is brought to the preoperative area where our child life team is able to greet him or her and ease some of the stress of the procedure just by being a very friendly familiar face and trying to distract them. The child life team follows the patient throughout his or her hospital course. Once the patient is recovering on the ward, he or she has their own private room. The parents are allowed to stay with their child throughout the course of the hospitalization. The pediatric hospitalist service which is the pediatricians who stay in house 24/7, they are involved in the management of all these patients as well to assist with any other medical needs the child may have beyond the usual postoperative issues.

And then the nurses on the pediatric ward at Elliott are just extremely attentive and caring. We never want a family or a child to feel alone or scared throughout this process.

Host:  Yeah, that is very reassuring to hear. Just as we close here, one of the things that I wanted to ask you about is how we might best talk to our children about a potential deformity? I think that how kids appear to their peers means so much to them. So, what is the best way to talk to them about it and if they do choose to go through with the procedure; how do you walk them through that in a way that’s going to feel comfortable to them?

Dr. Misra:  So, that is a great question and it’s amazing. So, I’m increasingly surprised by how many children actually already know what the Nuss Procedure is and what pectus excavatum is and that actually comes from watching YouTube and while I usually advocate for patients to not necessarily go Google search and watch YouTube to get a better understanding of their surgeries; I think it helps in this case because the kids who get this operation tend to be older and because this operation is painful and it is a lengthy recovery; I never want to do this or I never advocate doing this operation if a child is not onboard for the surgery. So, the more information they have coming into the office, in my eyes, the better.

But for those children who are not aware of what they’re getting into, I just recommend that parents and I’ll help as much as possible, just first explain that again the chest appearance just like some kids are tall, some kids are short, some children have chests that sink in a little bit and others have chest that stick out a little bit. It’s no one’s fault. It’s just the way that we’re all formed. And the purpose of the procedure and surgical evaluation is just to improve that child’s quality of life now and in the future so that they can do whatever type of sports, activities, whatever they want to do, they should never feel limited by how their chest looks.

Host:  All right Dr. Misra. Well I believe that’s the perfect place to end. Thank you so much for your time today. That’s Dr. Meghna Misra an Attending Pediatric Surgeon at Elliott Hospital. For more information please visit www.solutionhealth.org. If you found this podcast helpful, please share it on your social channels. Thanks so much and we’ll talk next time.