Search for tag: "u0033689"

Interviewer: We're with Dr. Stephen Fenton. He is the founder and director of the Utah Fetal Center at Primary Children's Hospital. And it is for women who have high-risk pregnancies due to a congenital anomaly with the child. There is kind of a way things used to be done. And now there's a way things are done at the Utah Fetal Center, Primary Children's Hospital, which is a much better way. Dr. Fenton, first of all, you're the founder and the director. You came in and you said, "I want to start this center."Why did you say that?

Dr. Fenton: I don't want anyone to ever think or believe that what was happening before wasn't being done to the best of its ability because it certainly was. All of these conditions were being cared for before. But the reality is it was kind of done in what I would term a physician-centered approach. So oftentimes that would require multiple clinic visits because they were separate clinics in each of the physician's clinics. And you can imagine how frustrating and how hard that must have been for that mom to kind of shoulder that burden all on her own.

Interviewer: It was a very stressful time.

Dr. Fenton: Very stressful time.

Interviewer: Just to coordinate her care kind of.

Dr. Fenton: I mean, and during all that time, of course, worried about her unborn child. And so what we've done is we've kind of shifted it from this physician-centered care to what I term patient-centered care with a multidisciplinary approach. So we, over the last five years, have put together a multidisciplinary team of all of these specialists that care for the child and for the anomaly. In addition, we've added adult specialists, so maternal fetal medicine or high-risk OBs that care for the mom, all in one place. We didn't have adult providers at the Children's Hospital. Now we have these adult MFM providers who help care for these moms. We also added a coordinator. We've added nurse coordinators that actually help the mom wade through all of this, and ancillary staff, such as a social worker, who can also help with the non-clinical aspects. We are now all in the same place. Instead of being in our individual silos, if you will, we're all located together where we can look at the images together, where we can talk about it, and where we can come up with a care plan not only for the mom until baby is delivered, but also for the child after delivery.

Interviewer: What is the objective of the center?

Dr. Fenton: We want to make sure mom and baby first have the right diagnosis. It's much easier to help the parent learn what the condition is and start understanding what the treatment, if any, will entail before the child is born. Now that's one aspect. The other aspect is some of these kids need intervention before they're born. And in order to do that, it's very specialized. It requires a team and it requires being able to take the mom to proper diagnosis and from diagnosis to intervention, and then from intervention to delivery safely. And you can't do that without proper infrastructure, and the Utah Fetal Center is that infrastructure.

Interviewer: Can you give me an example of a couple of the common conditions that you would require that would require this multidisciplinary team?

Dr. Fenton: Congenital diaphragmatic hernia is one that comes to mind, myelomeningocele, that's another word for spinal bifida, CPAM or congenital pulmonary airway malformation also known as congenital lung lesion, atresias, intestinal atresias, omphalocele, gastroschisis. These are all things that are surgical too. We oftentimes see kids that don't necessarily require surgery. So some of the genetic disorders like trisomy 21. We also see kids that have neurologic issues, so brain malformations that won't necessarily require intervention, but will require a coordinated care with multiple providers.

Interviewer: What does that initial consultation entail when they come to you? What does that look like?

Dr. Fenton: In the morning, they oftentimes will undergo an ultrasound and consultation with one of the maternal-fetal medicine physicians that work at the Utah Fetal Center. Dependent on what the original diagnosis is, and oftentimes we have already received outside imaging from the referring providers and reviewed it, they might also undergo a fetal MRI, and that fetal MRI will give us even more detail, especially when we can compare it to the ultrasound that happens on that same day. It's read by the fetal radiologists that work in the center. And then usually we give them a little bit of a break. They go to lunch, etc., and then come back in the afternoon or early afternoon, and there, they will see the specialist, the sub-specialist that will ultimately care for their child.

Interviewer: If a patient wasn't referred and they believe that the Utah Fetal Center is the place that they would like to go, are they able to call?

Dr. Fenton: Absolutely. They can go to our website, utahfetalcenter.org and self-refer. We really want to help these parents get through this very, very difficult time. We understand that there are a lot of providers out there that are doing a portion of this, and we certainly appreciate all that they are doing. We are not looking to just assume all care of these moms because we know that a lot of their care can be delivered close to home, but we do feel like it's very, very beneficial to start that coordination of care early so that we can help the parents understand what is happening, obtain expectations on the treatment plan, as well as understand the treatments involved and then initiate that plan early, and in the long run we know that doing so with the help of the many providers, not only here at the University of Utah and Primary Children's Hospital, but across the state will allow us to do that.

Interviewer: Pectus carinatum is a condition where the chest and the ribcage press outward. It has received over the years the nickname "pigeon chest" because of the way that it presses out, and wanted to find out more information about that today.

And we're with Dr. Stephen Fenton. And I wanted to find out about the condition and about some of the treatments of this condition. So, first of all, what causes it?

Dr. Fenton: That's a great question. It's really unknown. It's a congenital pectus deformity. There are two of them, excavatum and carinatum. And, like you said, carinatum goes outward like a pigeon's chest. It must be some interaction between the costal cartilage, or where the ribs come together with the sternum, and with development of the chest. Those cells push the sternum outward.

It can be symmetric, it can be asymmetric, so it can be to one side versus the other. It can encompass all of the sternum. Sometimes it just encompasses a portion of the sternum. And there's a large range of mild to severe as well. People don't often also recognize something called costal flaring, and that's where the bottom of the ribcage, or what we call the costal margin actually flares out or pushes out as part of this defect.

Interviewer: And is this defect possibly dangerous to a child's health? Because normally you notice it in adolescents.

Dr. Fenton: Correct. I said congenital, meaning the child is born with this defect. But, especially with carinatum, it's usually not noticed until adolescence when the child has gone through puberty and started having growth spurts. And that's when, because the chest is developing one side or the other, starts to push out more. Is it dangerous? That's usually the first question parents want to know. It's not. The chest will be every bit as strong as their peers. And so we don't give any limitations on the child's activities. We want them to play sports, we want them to be active, and certainly don't tell them otherwise.

Interviewer: But I would imagine that that kind of chest deformity can cause some self-esteem issues for a lot of people, especially adolescents where, boy, it's a tough time of life, anyway, right?

Dr. Fenton: That is definitely one of the aspects of these pectus deformities that children often experience. And sometimes it takes the parent by surprise, especially carinatum, which usually isn't seen in the small child. Excavatum can be seen because there's a dip, and so parents oftentimes notice that, especially when the child is young and they're the primary caregiver.

When the child gets older and starts to develop, they often take over their own body. Sometimes they hide their body, if you will, from their parents, they're not taking baths with their parents anymore. And they might be someone who just wants to wear a shirt when they're in the swimming pool, and, lo and behold, oftentimes it's because they don't want others to see what their chest looks like. Obviously, the more mild form of this is much less recognizable than a more severe form and/or kind of an asymmetric form which is going to be the most obvious.

Interviewer: If it's not physically threatening, I guess the treatment would be for cosmetic reasons?

Dr. Fenton: For carinatum, that is usually the case. Now, again, we're talking about the routine kind of run-of-the-mill. In some kids where it's very asymptomatic, deforms the chest quite a bit, that's another story. Even with carinatum, I would say, they should be evaluated and we should determine whether treatment is right for them or not. For most carinatum, though, oftentimes the kids are not symptomatic in another way. So the treatment really is along cosmetic lines.

And it used to be that we would offer this surgically. It required oftentimes a big surgery with a prolonged hospitalization and recovery time. And you can think about the cost and expense of that, and so it wasn't oftentimes pursued. Nowadays, much like correction of teeth with braces, there is bracing that can occur to help correct the chest. We like to do it in kids that have a fairly young body type because their chest is still very malleable, and initiation of bracing usually at that age will allow the chest to correct, and then we, kind of, help them maintain that correction through continued bracing until they've, kind of, finished growing.

Interviewer: What's the earliest point you might consider treatment for a child, and what's the point where you're like, "Well, it might be a little late at this juncture?"

Dr. Fenton: We would like to see the kids, first of all, whenever the diagnosis is suspected. Even if they're young children, we'll see them in clinic, talk to Mom and Dad, and the child about what this is, and then come up with, kind of, a timeline on treatment.

Oftentimes we'll gauge when we should repair based on severity, and based on what the body type is. But, obviously we want to do it not necessarily as soon as it's recognized, but once it's recognized and the child is starting to initiate puberty and growth, because, again, the chest is much more malleable, kids tolerate this a lot better. The older the body type, the older the person, the treatment itself can be much more difficult and not as successful.

Interviewer: And I love the analogy about teeth because we would not hesitate to take our child into the dentist and have their teeth straightened because that's just such a self-esteem thing, and it's so important. This could be very much the same self-esteem thing. It might not necessarily be causing any pain or any other sort of conditions, you know, since it is just a brace. How long would somebody have to wear that brace to make that correction then? And is it like braces that, you know, other people are going to see it?

Dr. Fenton: Most of the braces can be form-fitting. So what I tell parents . . . and we have a list of providers that we've used in the past and parents have been satisfied with what they've had. I tell parents, "First of all, you want the child to wear it." Now, it is a brace. It's not perfect. But, certainly, if it looks like a coat of arms or armor, they're not going to want to wear it. But if, you know, it fits well, it's fairly comfortable, they're going to be more apt to wear it.

Second, you really want the place where you get the brace from to be somewhat convenient. If they're driving from Logan to St. George for fine-tuning of the brace, that's not convenient and probably won't happen. And some kids will have to go in and get adjustments every few months, especially while they're growing in order to make sure that the brace continues to fit. So convenience is the second thing.

And then, thirdly, I tell the kids the amount of time required for correction really depends on how often they wear it. And if it sits in their closet under a pile of clothes, their chest is not going to correct. If they wear it 24 hours a day, sometimes we've seen kids correct in as little as 6 months, that's hard mainly, and it depends on the timing.

So, for example, in the winter, it might be easier for someone to wear a brace to school because they're wearing a hoodie, they're wearing baggy clothing, they might not have gym, etc., whereas in the summer it might be more difficult because they're wearing a T-shirt and shorts. And so I kind of tell the parents and teenager, you know, "You need to gauge it so that you're optimizing your time with the brace. And so, if it's every day after school and all day during the weekend, then great. If during the winter months it's every day, all day long, awesome. You know, if you have to miss the days that you have gym because you don't want to take off your shirt and have a brace on in front of your buddies, that's understandable. You have to do what will actually work." And the more frequent they wear it, the faster it'll correct.

Now, the other caveat with that is, let's say they're very diligent and they wear it every day for six months, and it totally corrects in six months, but they still have a year and a half of growth, there's going to have to be some sort of maintenance. And so, you know, then I usually discuss decreasing the frequency when it's worn, usually wearing it maybe on the weekends, making sure that they're, kind of, self-assessing the chest and seeing if it's starting to come out a little bit more, if it's staying corrected.

And we actually like to see the kids back about six months after they start bracing, and then about every year after that, or when needed to, kind of, help, again, make sure that that bracing is working.

Interviewer: And after the growth period is done, then that's when it, kind of, settles in and it's not going to change anymore, it sounds like?

Dr. Fenton: Correct. I mean, once the cells have stopped really, you know, causing growth to the adult-size chest, it doesn't become more prominent.

Interviewer: Is it an expert such as yourself that parents and the children should go to? Could a general practitioner provide the brace? What would be the process if a parent recognized their children had pectus carinatum?

Dr. Fenton: If it's recognized, we would ask that, you know, you can go and verify it with a general practitioner, or pediatrician, family practice doctor, etc. Most of them will then refer these children to us because the correction does require thoracic surgical expertise, which is what we do. And when we see the child, we can discuss with them and the parents the different options, and decide whether bracing is the right way to go.

Announcer: Health information from experts, supported by research. From University of Utah Health, this is the scoperadio.com.

Interviewer: A teratoma is a congenital anomaly that can affect newborns. But what is it, and how can it be treated? I'm here with Dr. Fenton. He's an Assistant Professor of Pediatric Surgery at the University of Utah. And he's also the Director of the Utah Fetal Center. So Dr. Fenton, what is a teratoma?

Dr. Fenton: A teratoma is a collection of tissues that develop with the developing child, and it's oftentimes a remnant. So we don't exactly know why they form, but we know that they form as a result of extra developing tissue, if you will. So it can contain all types of tissue. muscle, skin, hair, teeth, has a very characteristic finding on ultrasound and on a CT or MRI because of the different types of tissue within it.

Interviewer: So you can actually see it while the fetus is developing?

Dr. Fenton: Yes. So they can be found on the screening ultrasound. Typically, it's noted to be an abnormal mass either in the chest or let's say at the end of the tailbone, more commonly at the end of the tailbone than in the chest. And because of that mass, they're usually referred to a high-risk OB or a maternal fetal medicine physician and/or a fetal center like the Utah Fetal Center, where the mom will undergo a more formal, specific ultrasound and sometimes an MRI to further characterize this mass.

Interviewer: So when you find this teratoma, what is the course of action from there on? When do you get in there to try to help treat it?

Dr. Fenton: First of all, we want to confirm what the findings are. And so again, typically they will go to, say, the Utah Fetal Center, and they'll be seen by a maternal fetal medicine physician, who will have them undergo a specialized ultrasound, which will look at the mass further. Obviously, other masses can develop. These have characteristic findings because of the multiple different tissues that are within it, which will help us kind of determine whether we feel like it's a teratoma or something else.

In that case, when we feel like it is a teratoma, we will follow the mom very carefully because we know that the teratoma will grow. And it can cause a couple of problems with growth. So let's say it's hanging off of the tailbone. The growth is not really restricted because it's not in a contained space.

But what can happen is because of excessive growth, it can actually have an increased blood flow through it, which could lead to strain on the heart and could actually cause the child to go into heart failure. We call it hydrops. So you start seeing swelling of the child's tissue fluid within the chest, fluid within the abdomen. And hydrops is obviously very dangerous for an unborn child. And so we would want to try and intervene on that before really hydrops is discovered.

And so, in the case of a sacrococcygeal teratoma, we can look at other things that tell us that the child is starting to have strain. And if that's the case, if the child is very young, let's say less than 28 weeks gestation, then we would advocate for a fetal surgery, where that portion of the child is actually delivered outside of the womb, and the tumor debulbed, or the majority of it is removed, not completely but removed to the point where it should decrease the chance or eliminate the chance of further cardiac compromise on the kid.

Interviewer: And what about if it forms in, say, the chest because that's the second most common, in the mediastinal area?

Dr. Fenton: That's correct, the anterior mediastinum, so it's in front of . . . it's right underneath the sternum or that middle chest bone. And the problem with that is usually later on in pregnancy, because as it grows, it starts to compress a couple of things. So first of all, it can compress the airway. Now, a child in the womb doesn't breathe because it has the maternal support from the placenta. But when the child is born, that can obviously cause some real problems, especially when the child tries to take the first breath.

But also, it can push on the vessels of the heart. And so the cardiac function is compromised in that the heart is really trying to pump hard but can't get blood out. And, again, we can start seeing signs of hydrops or heart failure on a child, and we would want to intervene sooner than later.

And if the kid gets to about 28 weeks, we want to try and get them as close to term as possible, but when they get to at least 28 weeks, we would advocate to deliver them early. And the way we would deliver them is by doing what we call an exit procedure, or it's an ex utero intrapartum therapy. So it's outside of the uterus, but still within the birthing process. And so we can actually deliver the head of the child in this case, leave the rest in the uterus so that the placenta still works, and put a tube in their throat to make sure that they can breathe properly before we deliver them completely. Or I was going to say in extreme cases, we could actually open the chest and take out the tumor while on placental support as well.

Interviewer: And for the mother whose newborn child may have a teratoma, what can her expectations be for her child?

Dr. Fenton: I think a lot of that depends on when it's found and the state of the child. So what I mean by that is if it's found very early in pregnancy, and there's no real compromise to the child, we can follow them closely to the point where something can happen and help that child get to delivery. A child that has hydrops has a very high mortality rate or death rate, and we want to try and intervene before that happens. In a newborn, so a child who has been born where a teratoma is found and the child is doing well, most of those can be removed. And when they're removed early, there's a smaller chance that there will be a malignant component to it or require further treatment and a higher chance that the surgery is all that is necessary.

Announcer: Have a question about a medical procedure? Want to learn more about a health condition? With over 2,000 interviews with our physicians and specialists, there's a pretty good chance you'll find what you want to know. Check it out at the scoperadio.com.

Announcer: Medical news and research from the University of Utah physicians and specialists you can use for a happier and healthier life. You're listening to The Scope.

Interviewer: Dr. Stephen Jay Fenton is a pediatric surgeon and also an expert in congenital diaphragmatic hernia. So, most of the time, it's diagnosed before the woman has given birth. So, this diagnosis comes in, what are some considerations that the woman, the mother, the family are going to have to do at this point?

Dr. Fenton: Once this woman has diagnosis they need to be referred to a maternal fetal medicine physician, so someone who deals with high risk pregnancies. And one of the things associated with that is that they need to be followed closely during their pregnancy. One is to ensure the safe delivery of the child and then also in preparation as far as what will happen once the child is born.

I think it's important for the mother to know that this is something that will require treatment for a long time after the child is born. So I typically would say three, four, to six months afterwards, that child will need to be in hospital that takes care of these types of kids. And so sometimes it might require relocation or making sure they have a support system away from their house. For example, if they live quite a bit away from the children's hospital that deals with this specialty, they may need to start thinking about the Ronald McDonald House or finding some other place that they can live.

Interviewer: Wow, so this diagnosis is a serious thing.

Dr. Fenton: Yeah, I mean over the past 10 to 20 years the survival rate has improved, but it still is one of those diseases that takes a lot as far as to treat the child once they're born and then afterwards to help the child continue to grow.

Interviewer: What's the conversation that you have with these families? Take me through that conversation, some of their concerns, their questions, the things that you tell them that are important to consider.

Dr. Fenton: So, after getting past the basic questions of what is a diaphragmatic hernia and how is it treated, the things that I really focus on are we need to find out a little bit more about the child. So, one of the things that we'll get when they come to our office is what we call predictive or prognostic predictors.

So, a lot of times they will have already undergone ultrasounds, several ultrasounds, to look further at the child. One of which is of the heart, so a fetal echocardiogram. We want to know whether the child has a congenital heart disease as well because that does complicate things when the child has not only a diaphragmatic hernia, but has congenital heart disease as well. They are at a much higher risk of not surviving.

And it also limits some of the things we can do, especially as far as the ECMO or the heart lung bypass, if the child has a structural heart defect we can't put them on ECMO. So, the families need to know that.

Additionally we want to know what their chances of going on ECMO are and what their survival ability is. So, we look at these different things. One is the lung to head ratio, which is obtained by the ultrasound. Then the other is the total lung volume which can be obtained by the MRI. And those, depending on what the lungs look like, so the ratio of the lungs to the heads and/or the observe to expect of total lung volumes.

We can give parents the percentage of whether their child is going to require ECMO support and/or what their survivability is. And so the parents, when I talk to them, I almost talk to them much more about the support that the child will receive before and after repair, than the repair itself. Because the repair is really a step and only one of many steps in the care of the kid once he's born.

Interviewer: Wow. So, how do you help parents take all this information that you just talked about, head-lung ratios, and how do you make them make sense of what they should do at that point?

Dr. Fenton: Well, I think it's hard, especially for the initial visit, because a lot of information is given and so we will talk to them upfront and then we'll see them once they come into the hospital again to give birth. And talk to them once the child is born as well, and we can gauge. Obviously all of these are predictive.

They're not definitive and so it's after the child is born that we see how they're doing, that we kind of give them a little bit more of a definitive plan as to whether we feel like the child's going to need ECMO or whether we feel like when the child can be repaired. And some of the consequences as far as neurologic cardiac long-term pulmonary support. A lot of times we can't necessarily give them that until we are in the midst of taking care of the child.

Interviewer: What options do parents have at this point?

Dr. Fenton: I honestly refer my patients out to websites as well. "I feel like I've given you a lot of information. We want you to know more." There are support groups, CHERUBS is one, as far as the parents can Google. I also had them look at WebMD. I've actually found most parents to be very well informed when they come in even to the prenatal interview. So the very first kind of clinic visit that we have, I found that most parents who come in have already read up quite a bit about it.

Interviewer: Yeah, because they were told about the diagnosis and they're very curious and want to know.

Dr. Fenton: Exactly. And I think that helps. I mean having the parents be informed already about what it is and having specific questions as far as okay, so, tell me about ECMO. Tell me about reflux, and tell me about when you're going to do the repair and what the repair options are. It helps with the conversation already.

Interviewer Do some parents at this point after they get this information choose not to continue the pregnancy if they think... if you think I should say, it's going to be really bad?

Dr. Fenton: Some do. Yeah. I mean some will discuss options of whether to be able to continue the pregnancy or not. Obviously depending on the gestational age of the child, that might not be an option anymore. More often what is talked about is palliative care. So, meaning how aggressive to treat the child afterwards and how much should be pursued.

I would tell you that most parents say, "I want everything done. We'll go forward and certainly want to help the child, after they're born in whatever way is possible." But there are some times where we can't do that. And so I think it's important to have that discussion before, of what are the limits and the possibility of needing to do either palliation immediately after the child is born. And/or when have we exhausted all medical means to help this child.

Interviewer: So, after a diagnosis of CDH, can a child live a normal life after you've done what you needed to do to rehabilitate?

Dr. Fenton: Yes, I think that's the key. We've done what we needed to do rehabilitate or to treat the child, and obviously there is a pretty high mortality associated with CDH now. It has improved greatly over the past two decades. But it's still a serious disorder. There are some kids that have lungs that are so small and so underdeveloped that we're not able to treat those.

Now, the children that we are able to treat, they can go on and live a very productive lives. Some of them may have some challenges secondary to the treatment themselves. They might need oxygen support. They might not be able to exert themselves as much as a consequence of treating their underdeveloped lungs. But most of them can go on and live great lives even if they require some support.

Announcer: TheScopeRadio.com is University of Utah Health Sciences Radio. If you like what you heard, be sure to get our latest content by following us on Facebook. Just click on the Facebook icon at TheScopeRadio.com.

Interviewer: Congenital diaphragmatic hernia, what is it? What causes it and what can you do about it? We'll cover the basics of this terrible birth defect next on The Scope.

Announcer: Medical news and research from University of Utah physicians and specialists you can use for a happier and healthier life. You're listening to The Scope.

Interviewer: Congenital diaphragmatic hernia, also known as CDH. You might not have ever heard of it but it's likely more common than you thought. It's estimated about 1 in 3,000 children are born with it, which puts it with diseases you've heard about like spina bifida, which is one in 1,000 or even cystic fibrosis, which is about the same, one in every 3,000. Dr. Stephen Jay Fenton, the pediatric surgeon is also an expert in congenital diaphragmatic hernia. Thanks for taking time to talk about this somewhat unknown disease. Is that a fair assessment?

Dr. Fenton: It's obviously well known to us. We take care of it often but I think maybe some of it is secondary to the survivability. I mean it isn't something where kids will live as long as some of the kids with cystic fibrosis. So there are not as many families out there that are dealing with these types of kids. There is about 40 percent to 50 percent mortality rate associated right off the bat with congenital diaphragmatic hernia.

So now the children that survive, most of them will go on and live normal lives as a kid but it's something that's dealt with in the neonatal period primarily and not later on in life and so that might bring a little less notoriety to it.

As far as cystic fibrosis there's a lot more kids although it's one in 3,000. There are a lot more kids with cystic fibrosis kind of walking around out there. Some of it may be who is the champion for it, whether there's celebrities that are champions for cystic fibrosis or whatnot. I mean I think that might play a part as well.

Interviewer: Yeah. So a pregnant woman is told her baby as CDH. What does that mean for the child?

Dr. Fenton: First of all, we should just kind of talk about what it is which is basically a hole in the diaphragm. And the diaphragm is the muscle between the abdomen and the chest. And what happens is this hole develops early in the development of the child and causes bowel or solid organs to herniate up into the chest.

When it does this it arrests the lung in an earlier stage of development. And so what ultimately happens is not a matter of the intestines or liver, let's say, being up into the chest but it's actually result of very hypoplastic or underdeveloped lungs as well as thickened pulmonary arteries. And that's really the big consequence of a CDH.

Interviewer: I see. It's not a physical hole you see though in the stomach. It's all internal, right?

Dr. Fenton: It's all internal. Yeah, you would never see it on the child. The only thing you really see on the child is that their belly looks really, really flat and it looks much flatter than normal because some of their abdominal contents were up in the chest.

Interviewer: So if I'm understanding correctly what happens is because of this membrane is not there, the organs move up and is that what stunts the growth of the lungs?

Dr. Fenton: Exactly.

Interviewer: There's just no space for them to grow.

Dr. Fenton: That's exactly right.

Interviewer: Is this something that can be detected before the woman gives birth?

Dr. Fenton: Oh, yes. Yeah. The great majority of these are detected prenatally, during the screening ultrasound let's say. So ultrasound is very good at detecting the herniation.

Interviewer: So give me an idea of the degrees then. How many children are born and it's not super serious. It's something that can be... or is it always super serious?

Dr. Fenton: Yeah. Very, very rarely. Every now and then I would say we get a child maybe one or two, every couple of years that come in and are great. Most kids though come in with a pretty big defect and because of the size of the defect they have very underdeveloped lungs and have something called pulmonary hypertension which is also a consequence of the underdevelopment of the lungs and they're sick.

They require ventilatory support so they need to be on a ventilator. They require medications sometimes to help with their blood pressure and how well their heart is working. And they also require medications to try and help open up the pulmonary arteries in order to reduce what we call pulmonary hypertension which is really something that we did as challenge immediately after birth.

Interviewer: As a surgeon then, what do you do to try to solve this problem?

Dr. Fenton: Well, the surgery is a major step but it's not the first step as far as these are concerned. Once the kids are born, the key is really to stabilize them from a medical standpoint. So we want to try and help them breath, but we want to do it in a way that won't cause long term damage to the lungs. So we want to kind of do this gentle ventilation if possible and sometimes that can be very difficult because they require quite a bit of support.

We also want to make sure that we're supporting them from a cardiac standpoint. So we need to make sure that their heart is working well, that their blood pressure is normal. And then we need to work them up for any further anomalies. And then once they've stabilized their pulmonary hypertension is under control, we feel like they've been stable for at least 24 hours then we repair them. And this does require surgery, so we do make an incision in the abdomen and we go in and we go find the hole and there's really kind of three options that we can do to fix it.

So one is the hole really isn't that big and we're able to bring the two edges together without tension. When we can do that, then we just close it what we say primarily. But most of the time the holes are bigger than that and we can't close it without tension. So in that case we can either put a synthetic patch in, something that has called it a dual mesh. It has cortex on it so it protects the lung on one side and kind of allows tissue to grow into it on the other side. And that will allow us to close the hole without tension.

One of the things that we do here at Primary's is what we call a muscle flap. The abdominal is made of three muscles to the side and we actually take the internal-most muscle and rotate it inward and sew it in. So it's what we call a biologic fix, meaning that it's actually part of the baby that is helping repair the hole. And we like that because we found that it reduces the risk of it coming back. So reduces the risk of recurrence and then it also grows with the child.

Interviewer: Okay. So after the repair is done, the organs are back where they belong. What's the recovery process at that point?

Dr. Fenton: So it's still quite a bit. Now I usually tell parents they should plan on their child being in the hospital for three to four months.

Interviewer: Will the lungs ever fully develop properly?

Dr. Fenton: Yeah. Well the lungs actually continue to grow until the child is around eight years of age. So with support, and if we've been able to use gentle techniques to the point where the lungs aren't damaged, then they should be able to grow to the point where the child can live a pretty normal life. Now some of them are so severe that they will have some pulmonary consequences later on in life. They might still require some oxygen. They might not be able to exert themselves as much without being tired.

Now I think something that is important is that these kids are kids that should be followed for a long period of time. So they will still have some consequences of pulmonary hypertension. So it's not a child that after their repair and after they leave the hospital, we don't see. They're actually children that are still followed by specialists.

Interviewer: Do you have any thoughts for parents or families that found out that this is a birth defect that's in their family?

Dr. Fenton: I guess the biggest thing to say is that they can be well supported. Even though it's not known commonly throughout the community, it is something that we treat very often at Primary Children's Hospital. There are a lot of specialists that deal with this not just from the surgical standpoint but from the lung standpoint, from the neonatal standpoint, etcetera. And they can come and see these specialists and get the support, that information that they need that will help them not only through the difficult time prenatally but also through the time immediately after birth and then throughout the child.

Announcer: The ScopeRadio.com is University of Utah Health Sciences Radio. If you like what you've heard, be sure to get our latest content by following us on Facebook. Just click on the Facebook icon at TheScopeRadio.com.