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Interviewer: Three types of surgery can be used to treat craniosynostosis, which one depends on a few different factors. If the synostosis is caught early enough, the newer endoscopic procedure can be used.

Pediatric plastic surgeon Dr. Faizi Siddiqi and pediatric neurosurgeon Dr. John Kestle are here to discuss the three different types of surgery for a synostosis and why they would consider one over the other. So, first of all, how early does a child need to see you for the less invasive endoscopic procedure to be an option?

Dr. Kestle: So the endoscopic method we've been doing since '07, and we've found that the best time to do it is usually between two and three months of age. So we have to see the patient before that and the earlier the better. The surgery is done under a general anesthetic, and the surgery typically lasts about two hours, and most of them spend one night and can go home the next day. Occasionally, they need two nights. It's done with, for example, sagittal synostosis with two incisions -- one just behind the soft spot and one toward the back of the head. And then we work under the skin to remove the fused bone.

Removing the fused bone doesn't really change the shape much at all immediately. It just releases the bone. And then about two weeks after the surgery, they start wearing a special helmet that's designed for surgical patients, that is a little snug front to back and a little loose on the sides, and it just guides the growth so that as the baby's head growing, it's taking on a more normal shape. They wear the helmet for about six months, some shorter, some longer, and we just monitor the growth pattern and make that decision. And they'll go through several custom-made helmets over the course of the treatment as they grow.

Interviewer: Generally, I'd imagine a less invasive procedure is always better. Why is this procedure better than say the traditional treatments?

Dr. Kestle: Well, it's got a shorter hospital stay, a much lower rate of blood transfusion. It's a lot less swelling associated with the surgery. It's easier on the babies, and the results are at least as good.

Interviewer: Is there a reason, other than age, why a child would not be eligible for the endoscopic surgery?

Dr. Kestle: We don't often do it in children that have syndromes where they might have multiple malformations in other parts of the body, such as Crouzon syndrome or Apert syndrome. And in addition to those other malformations, they have synostosis. Those children usually need the more traditional, bigger surgery. But any child that has one suture fused is a great candidate for the smaller surgery.

Interviewer: Dr. Kestle, you're a neurosurgeon. So you handle that part of the procedure?

Dr. Kestle: Yes.

Interviewer: Okay. And then Dr. Siddiqi, you're a pediatric plastic surgeon. With the endoscopic procedure, both of you are in the operating room at the same time. Just kind of walk me through how the surgery goes. Dr. Siddiqi, you start the surgery.

Dr. Siddiqi: So we're in the OR together. It's a team approach. So once the anesthesia team have completed their part, which is getting the baby asleep and making sure the IVs are put in and everything is safe to proceed, that's when we position the baby for surgery. I would make the initial incisions. For example, for sagittal synostosis, we make two incisions on the top of the scalp. Again, that's one of the advantages of doing it this way versus the bigger procedure because you have two small incisions. Through those incisions, we expose the area that we want to operate on, which is that fused sagittal suture. And once everything's exposed and visible, then Dr. Kestle would take over.

Dr. Kestle: What we do is remove a little bit of bone under each incision. And that allows us to get underneath the bone. Underneath the bone is a layer called the dura, which is a covering layer over the brain. It's kind of like leather, like a thin leather. And we use the endoscope to separate that layer from the bone, and that allows us to safely cut the bone and remove it. Once the bone is removed, we look at the dura and make sure it's okay. We stop any little bits of bleeding, but there usually isn't much. And we check the bone edges, which sometimes ooze, and make sure that any bleeding is stopped. And then at that point, Dr. Siddiqi and his team continue working.

Dr. Siddiqi: Yeah. So we take out or remove additional segments of bone. There are these little triangles we take out, about four them. Again, afterwards, we make sure that the bone edges are, you know, clean. They're not bleeding. Again, that's one of the other advantages to doing it this way. The blood loss is quite small, minimal compared to the traditional way. Most of the time, it's maybe 10 or 15 milliliters of blood. So once those triangles are removed, then we close the incisions. Then the anesthesia team takes over, and the baby's, you know, woken up and then taken to the recovery room.

Interviewer: What does the recovery look like then for a child? And, you know, what kind of outcomes can parents expect?

Dr. Kestle: With the small surgery, they don't need to go to the intensive care unit. They stay in the hospital in a regular room, and the parents can stay with them. The vast majority of those children are here for one night. Occasionally, they need two nights. The criteria for going home are pain control and feeding. They get some swelling toward the back of the head, that gradually goes down over the first week at home. Stitches dissolve on their own. And within a day or two, they're back to their usual self as far as feeding and behavior goes.

Interviewer: And Dr. Siddiqi, how long does it take for the head then to regain more of what would be considered a normal shape?

Dr. Siddiqi: Yeah. So as Dr. Kestle mentioned earlier on, the shape doesn't change after the surgery, right after. It's once they're in the helmet. The helmet is critical for reshaping the head. And typically, they're in the helmet usually two to three weeks after the surgery. It's a custom helmet. You know, it just guides the growth of the head, and over the ensuing, you know, three to six months, we have a more normal head shape. And hopefully, after six months of helmeting, that's all they need.

Interviewer: And the incisions that were talked about out in the endoscopic surgery, are those visible or are those in the hairline?

Dr. Siddiqi: You know, they're in the hairline. And again, another advantage to doing it this way is the incisions are on the top of the scalp, the head, and those scars heal very nicely. They're quite thin and they're barely perceptible. You only notice them when the hair gets wet. Again, with the bigger procedure, you have a much bigger incision from ear to ear, which is much more noticeable.

Interviewer: Let's talk about the more traditional procedures in the event that a parent is in a situation where their child is older than six months old or there's other reasons why they might have to have that. What are the two procedures, and can you explain those a little bit?

Dr. Siddiqi: Yeah. So sometimes, you know, we do see kids who are, you know, two, three months old and they're eligible for the smaller procedure, but for various reasons, let's say they live out of state or they don't want to do the helmeting, they would like to do the traditional, what's called cranial vault reconstruction with orbital advancement. So then we would wait until they're 10 to 12 months of age to do that procedure. Essentially, that's a much more involved procedure, but it's a procedure that's, you know, well described. People have been doing it for, you know, 30, 40 years. You know, the results that you get are comparable to the endoscopic procedure, but, again, it's how you get there.

So with this procedure, you have to expose the entire skull. So that means an ear-to-ear incision through the top of the scalp. I would expose that, mark out where I want Dr. Kestle to make the cuts and remove the segments of bone that we want to reconstruct and reshape. Then Dr. Kestle would remove those pieces of bone, make sure that the lining of the brain is okay, make sure everything is okay. Then I would reshape all those bones and reconstruct the skull in a more normal configuration, and everything is stabilized with plates and screws. And these are resorbable plates and screws. They dissolve in about a years' time. So we put everything back together and close the scalp. That's a four or five-hour process. Much more blood loss than with the endoscopic procedure.

They typically would go to the intensive care unit for one night, and they typically would be in the hospital three or four nights. Oftentimes there's quite a bit of swelling. The eyes can get swollen shut, and it would take maybe 10 days to 2 weeks for that swelling to go down.

Again, the advantage is you don't need a helmet. It's all done in one stage. You know, the compromise is that it's a much bigger operation.

Interviewer: If parents are evaluating a center or physicians to do this procedure, what advice would you give to them to, you know, pick out the best place for them?

Dr. Kestle: I think it's a procedure that is usually done very safely, and children do very well and go home quickly. We are exposing the layer over the brain, and there is a potential for bleeding. And so I think that experience matters. And I think that you do want to be treated by people who do this a lot and people who can handle problems, which are rare, but if they arise, they need to be dealt with appropriately. So I think it's a big advantage to being treated by people who have experience with this, who are in a children's hospital with pediatric-trained specialists, including anesthesia and a pediatric intensive care unit if they need that.

Interviewer: And you mentioned a third procedure, a cranial vault distraction, when might that be used?

Dr. Kestle: So there are some children where their brain is in trouble or potentially in trouble because they have presented very late or they have multiple sutures that are closed. And in that situation, we want to make the skull bigger to give the brain room to grow. Probably the best way to do that these days is a procedure called distraction, where some implants are inserted and then the skull is gradually expanded over time.

Dr. Siddiqi: You know, with cranial vault distraction, again, the idea is to give the brain as much room as we can because of the fact that more than one suture is fused. And the way that's done is I would ask Dr. Kestle to make some cuts on the bones. And then I would put these little devices, they're called distractors, on either side of the cuts. And then three days after surgery, we would have the family start turning those distractor devices. Typically, it would be total of one millimeter a day. So over the ensuing three to four or five or six weeks, the bones are slowly being separated. And what happens is that, as they're separated, there's new bone being formed in the gap. And over time, that new bone will solidify. So we're not only expanding the volume for the brain, we're also creating new bone. This is really the only way we can expand the brain to this degree using these devices. We couldn't do it as a single-stage procedure.

Interviewer: Between the two procedures, the endoscopic procedure, the less invasive, and the cranial vault reconstruction, are there any tangible differences and outcomes or how the head is going to look or anything like that?

Dr. Siddiqi: I would say like the overall head shape is probably going to be comparable, just the head shape itself. But again, as I said, it's how do you get there? You know, how long does it take? What are the risks involved? In terms of the shape itself, with the endoscopic procedure, overall the head feels and looks quite smooth at the end of the day when everything is healed. Whereas with the bigger procedure, you know, we're taking all the bones out or in multiple pieces, putting it back together. So when everything is healed in a year, two, three years' time, you do feel some irregularities over where the bones are joined together. But overall, the head shape is probably comparable. But, you know, I think you get an overall smoother head shape. And I think it probably looks a little bit better as well.

Dr. Kestle: And obviously, the scar is different as well. In the endoscopic, there's two scars on the top of the head that hide really well. And the bigger surgery has an ear-to-ear incision, which also usually heals really well. But as people age and start to lose hair, it's a lot more obvious.

Interviewer: Craniosynostosis. It's a condition that causes a newborn's head to be misshapen. And it should be treated. To learn more about the condition, how to identify it, and why it needs to be treated, we've got Dr. John Kestle, he's a pediatric neurosurgeon, and Dr. Faizi Siddiqi, he's a pediatric plastic surgeon. And they are experts. They do the surgery that actually treats craniosynostosis.

So let's start with the first question, which is very basic. Dr. Kestle, what is craniosynostosis?

Dr. Kestle: So it's a condition where the bones that are normally separate are fused. And it restricts growth and creates abnormal head shapes. The pattern is usually present at birth or very soon after birth. That's different from the benign conditions where the head shape gets distorted because the baby is laying on one side. Those babies have a normal shape in the beginning and their head shape gets distorted over the first couple of months of life because they're lying on one side.

Interviewer: How does a parent generally find out that their child has craniosynostosis or synostosis?

Dr. Siddiqi: When they're born, they're usually told that after delivery, as Dr. Kestle mentioned, the head is going to be a little misshapen from the birthing process. And that usually corrects within two to three weeks. If that doesn't correct, then they're kind of suspicious and they visit their pediatrician. And then hopefully that's when they're referred for further evaluation by us.

Interviewer: And then what does that head shape look like? We do have a link to a pamphlet that you have that can help a parent. But just describe it briefly.

Dr. Kestle: So the typical head shapes, number one, most common is sagittal synostosis. It makes the head long and narrow, and the forehead and the back of the head kind of stick out. And the back of the head is narrower than the middle of the head. You can see those features when you look down from above.

Probably the second most common type is metopic synostosis. And that's when the suture down the forehead closes early and the forehead looks like the bow of a boat, or a triangle.

The other two types are less common. One is coronal synostosis, and that misshapes one side of the forehead so the forehead is pulled back, and the nose is sometimes crooked, and the eye socket on that side is usually a little bit elevated. And then the very rare one is the lambdoid synostosis, where the back of the head is flat on one side and the ear tends to be pulled back toward the flat side.

Interviewer: So how is it diagnosed then? So a parent recognizes that their child might have a misshapen head, they're concerned, they would go to a pediatrician first?

Dr. Siddiqi: Certainly they visit with their pediatrician and then they're referred to our synostosis clinic for further evaluation.

Interviewer: Okay. The pediatrician doesn't do any sort of imaging or anything like that generally?

Dr. Siddiqi: Sometimes they do. Oftentimes they don't because they don't want to subject the child to a CT scan unless they've seen a specialist and they're confident of the diagnosis. So we would see those kids in the clinic. Most of the time, it's a clinical diagnosis.

Interviewer: Meaning it's just visual, you're visually confirming it?

Dr. Siddiqi: Yeah. But once we decide it is and we talk about surgery, then we would want to get a CT scan, generally speaking, to be definitive about the diagnosis. And the scan also gives us information about the brain, which is helpful as well.

Interviewer: Why do you choose a CT scan over, say, an X-ray or some other sort of imaging?

Dr. Siddiqi: A CT scan gives much more detail of not only the bones, but, as I said, also the brain as well.

Dr. Kestle: The X-ray is very unreliable in making the diagnosis. And the CT scan has been changed over the years so the dose of radiation that's received is lower and lower. So now it's a very reasonable thing to do. It gives excellent anatomy, helps us with planning a surgery, makes the surgery safer.

Interviewer: And, Dr. Siddiqi, when do you generally like to treat a synostosis then?

Dr. Siddiqi: So if we see a baby with, let's say, sagittal synostosis that comes in at 2 to 3 weeks of age, then we've seen that the optimal time for surgery would be somewhere around 3 months of age. Between 2 and 4 months of age. That's why we stress the importance of early referral.

And the reason for that is if we can get the kids in by that time, we can get the surgery done, and then we can get them in the helmet and the duration of a helmeting would be as short as six months. So if we see these kids later on, the helmeting duration is much longer.

Interviewer: And, Dr. Kestle, if a child doesn't receive treatment for a synostosis, what could be the potential outcome then?

Dr. Kestle: So the natural history is that the shape will stay the same or get progressively worse because everything is growing except the fused suture. So number one, it's an issue of shape and appearance.

Number two, there are potential effects on the brain if it's left untreated. There's an incidence around 15%, maybe 20%, in the kids with sagittal synostosis that the brain growth will be restricted. And that can lead to brain problems, chronic headache, possibly visual problems.

With the other types of synostoses, that number is a little higher. And so it's nearly impossible to predict which baby with synostosis is going to get into those brain problems later. But that incidence of raised pressure is enough that we worry about leaving it alone.

Occasionally, we'll see an older child who had a CT scan for another reason, like maybe a concussion, and we identify a fused suture. But their shape is normal, and they're healthy, and their brain is developing normally. That's the situation where we might just follow them. But the baby that has the abnormal shape soon after birth is the ones where we recommend treatment.

Dr. Siddiqi: I would just add that . . . echo what Dr. Kestle said. The two indications are the shape and the risk of pressure on the brain. But the shape is important. A lot of families ask, or even other providers say, "Is this cosmetic?" It's not cosmetic. Cosmetic means, by definition, it's normal and you're making it better. It's not normal to have craniosynostosis. I just wanted to make that clear.

Dr. Kestle: I just want to really emphasize that it's visual. It's the shape that makes the diagnosis. So much so that what we do for almost every patient is have the family send photos. And we can pretty much make the diagnosis from the photographs and then decide if they need additional testing, how quickly we need to see them, and so on. But it's really a visual inspection of the head shape that tells you the diagnosis 90-plus percent of the time.

Interviewer: So really at 3 weeks, if a parent suspects that their child might have a synostosis, they should get in contact with a couple of specialists or a specialty center such as yourselves. You would encourage them to do that as quickly as possible. Is time really of the essence?

Dr. Kestle: For sure. And we can make plans based on photos, and we can see them in the clinic and talk about it, and then targeting between 2 and 3 months for corrective surgery.

Interviewer: Your child was diagnosed with pectus excavatum, which is also referred to as funnel chest. It's a caving of the chest. And you want a second opinion about whether your child should have surgery or not.

Dr. Stephen Fenton is going to help us better understand when surgery might be the best option.

So when the chest area is caved in, the condition is called pectus excavatum. Does that offer any other sort of health threats, or is it purely just a cosmetic issue?

Dr. Fenton: No, it actually does. So it's part of the congenital pectus deformities, which encompasses funnel chest as you talked about it, pectus excavatum. I oftentimes refer to parents or kids as think of a backhoe that's excavating. It's digging, and so the chest goes inward. The other disorder is pectus carinatum, which is where the chest comes outward.

Excavatum is associated with other conditions. And so, whenever I see a child in consultation, I always try and ascertain whether any of those conditions are present. So, for example, scoliosis is associated with it. Mitral valve prolapse, which can oftentimes be brought on, or recognized by a heart murmur, is also associated with pectus excavatum. Also, Wolff-Parkinson-White, which is an abnormal pathway in the atrium of the heart, which can cause a heart arrhythmia.

So some of the questions I always ask are, "Is there a history of murmurs in the child, or any of the parents? Is there a history of scoliosis? And has he or she ever felt their heart race just out of the blue, not with exercise?"

So the last thing is a connective tissue disorder, Marfan syndrome. These individuals that have this often have laxity of their joints. Sometimes they can lens dislocation of their eyes. They often have dilation of their aorta, as well as some cardiac valve problems. They're oftentimes very, very tall and have some features that we would refer to as Marfanoid. In that case, if we're ever worried, and I do ask parents specifically if there's a history of Marfan syndrome in their family, we would refer them to a geneticist to make sure whether that's present or not.

Interviewer: The heart conditions you talk about, and some of those other conditions, is that because the chest is caving in and putting pressure on that cavity? Is that what's happening?

Dr. Fenton: So we do know that there is pressure on the chest, and oftentimes a murmur can happen because of the heart being displaced and/or pushed on. But these entities are associated with pectus excavatum and not really caused by them.

Interviewer: So fixing that condition does not necessarily fix the other conditions?

Dr. Fenton: Correct. And so, in those conditions, if we feel like there might be mitral valve prolapse, we would get an echocardiogram, or an ultrasound of the heart, to look for that valve disease, because that's something that a cardiac surgeon would have to fix, or follow and maybe subsequently fix, but not something that would be corrected by correcting the pectus itself.

Interviewer: When might a parent notice that this is a condition that their child might have?

Dr. Fenton: Pectus excavatum is actually noticed oftentimes when kids are young. In fact, most parents come in and they'll say, "Oh, yeah, we saw it when he was a baby. It was like a little divot or dip in the chest." But most kids, it really starts to become prominent and more noticeable once they start their growth spurts around or after puberty. And that's what causes it. It's actually growth from the costal cartilage where it attaches to the sternum, and there is a differential in growth, which causes the sternum to go inward.

Interviewer: So let's talk about now what you would do for somebody that has pectus excavatum, the sunken-in chest. It's usually taken care of by surgery, if I understand. How do you know if surgery is something that you would do for a particular patient?

Dr. Fenton: So, first of all, there is a range. Sometimes it can be very mild. It does go inward, but the child doesn't have any symptoms. They don't complain of chest pain or shortness of breath, back pain, all of which can be associated with it. They feel like they can keep up with their classmates or their teammates when they play sports. And in that case, I usually say, "Don't do anything."

In boys, especially, I'll tell them, "Hey, work your upper body, build up your pec muscles, and a mild pectus will actually make you look more ripped." You'll look like . . . you won't necessarily have to do anything, especially if it's not limiting to their activities, and especially if they are totally symptom-free.

Now, on the other end, with severe pectus, it's interesting. I've seen kids who, let's say, have played competitive sports, basketball, football, soccer, track, etc., and even when they notice it before they hit puberty, or right when they're hitting puberty, they'll often tell me adamantly they can keep up with their classmates or their teammates.

And then as we follow them, and the pectus becomes more severe, even they and their parents will definitely start saying, "You know what? They're putting in as much effort as they were before, but they fatigue early, they complain of chest pain, they complain of shortness of breath, and they tire out much quicker than their teammates do."

And a lot of that can be attributed to the severity of the pectus. It does displace the heart. It can displace lungs, etc., and there is this sense of not being able to catch your breath or to breathe appropriately. In that case, with severe pectus and with symptoms, I would typically recommend surgical repair.

Interviewer: Is there an optimal age to do the treatment, or is it more just watching the progression of the condition?

Dr. Fenton: We usually say around 13 to 15 years of age. That does not preclude kids that are earlier or younger or older. But around that age, the chest is just very malleable and they usually tolerate it a lot better.

Interviewer: So it sounds like, with symptoms, you would definitely consider the surgery. Without symptoms, if everybody is feeling okay about it, then perhaps surgery would not necessarily be the option?

Dr. Fenton: Correct. And I think the important part for that is for the young adult and the parents to seek consultation. And along with the surgeon, we can talk about what the options are, whether we want to continue to follow, or whether we would recommend repair, or whether nothing needs to be done at all.

Interviewer: And the surgery, is it a pretty complicated surgery? I mean, is it a major surgery?

Dr. Fenton: Dr. Nuss and colleagues came up with a procedure that we would term "minimally invasive." So a small incision on either side of the chest, about an inch and a half to two inches, and then using a camera we can actually skirt the bar underneath the sternum. And then when we turn the bar around, it brings the sternum out. And we secure the bar in place.

Even though it's minimally invasive, and that's nice because whenever it's minimally invasive the body can recover quicker, the sternum does not want to be pushed out. And so there is pain associated with it. We work closely with our anesthesiologist to help make sure that we can mitigate that pain as much as possible.

And we've really done a much better job in the last several years. Kids that are undergoing this are leaving the hospital quicker and are recovering faster. This approach is, again, a great approach. It has a very low infection rate. It has a very low complication rate, and the outcomes in the long term are very good.

Interviewer: Does that pain continue during the treatment, then, after the procedure?

Dr. Fenton: No, usually the worst of it is for the first few days. So they're oftentimes in the hospital, and, again, our anesthesiologists help us control that pain. Most kids I say, by the time they come see me about two weeks afterwards, are for the most part back to normal. As with any major surgery, I always say, "There might be a little bit of pain or a pull for about six to eight weeks afterward." But once you've made it past, really, a month or a month and a half, those young adults are back to normal.

Interviewer: So this sounds like a very specialized individual that would do this. If somebody is more in a rural area and they want to have this procedure done, they're going to have to go to a metropolitan area it sounds like?

Dr. Fenton: Absolutely. I mean, the procedure itself is important being able to know how to do it, how to manage outcomes, etc. It's also very important knowing when to offer the operation and when not to as well, and the further workup around it.

And so, when seeking a surgeon, you want a surgeon who does this often, who cares for these kids often, and also works in a system that does, because it's much more than just the surgeon. It's the OR team. It's the anesthesiologist. It's the nursing staff, etc., that all contribute to good outcomes in these kids.

Interviewer: Final thought for somebody who is listening to this, who they believe that their child might have this condition?

Dr. Fenton: Final thought is, first, it's not dangerous. Don't restrict the activity of your child. And two, we're always happy to take a look at it and determine whether it's something that's not going to cause any problem for them or that might require surgery, and certainly can provide the expertise to do so.

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.

Interviewer: One in 5,000 children in America develop an anorectal malformation. What is that, how is it treated, and results you can expect on today's Scope.

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Interviewer: We're here with Dr. Michael D. Rollins, Professor of Surgery in the Division of Pediatric Surgery at the University of Utah Medical School and Primary Children's Hospital. We're talking about congenital colorectal disorders, specifically anorectal malformations. So, Dr. Rollins, what are some of these conditions, and what does it mean for the child?

Dr. Rollins: Basically, an anorectal malformation is when a child is born with an anal opening anywhere other than the normal location within the center of the anal sphincter complex. This may be an opening of the rectum on the bottom or perineum, which is anterior to the sphincter complex, or in a boy, the rectum could open into the urinary tract, such as the bladder or the urethra, or in a female, the opening could be into the back of the vagina. The most severe defect that a girl may be born with is where the urethra draining the bladder, the vagina, and the rectum all join together and empty on to the perineum as a single opening or common channel. A child may also be born with a blind-ending rectum and no apparent opening on the perineum.

Interviewer: Dr. Rollins, what causes these types of conditions and malformations?

Dr. Rollins: That's a great question, and it's largely unknown. There are a few syndromes that include anorectal malformations and genetic mutations for those particular syndromes have been identified, but by and large, the cause or the ideology is unknown. There seems to be some familial inheritance, familial contribution to this, 2% to 4% having an affected family member somewhere along their lineage. The incidence in the United States is approximately 1 in 5,000 live births, so that's roughly 800 children a year born with that.

Interviewer: And when does a parent or, you know, a new mother first find out that this condition is developing, and how do you, as doctors, see it?

Dr. Rollins: That's one of the unfortunate parts. Right now, we don't have reliable prenatal detection of this malformation, because most women receive their 20-week ultrasound, and if everything looks good at that point, they don't have follow-up imaging. And these malformations frequently occur later in gestation. So most of the time, these children are born as a complete surprise to the family which, as you might imagine, is pretty overwhelming.

Interviewer: So if most of these are detected after birth, what kind of symptoms would a parent see in their child that would point to one of these malformations?

Dr. Rollins: Fortunately, it's pretty easily picked up on the initial exam on the child by the delivering physician or pediatrician because there's no anal opening, which is one of the things that they routinely check for. As I mentioned earlier, this is a spectrum of malformations, so the anal opening may simply be located outside of the center of the sphincter complex. So a parent or a physician may notice that there is an opening on the bottom, although it might look small, might not look like it's centered where it should be on the bottom. And so those can be a little more difficult to detect. In those patients, that particular malformation is called a perineal fistula. It may be several weeks or even several months before that's diagnosed, and usually, the parents will note or the primary care provider will note that the child is having trouble passing stools.

Interviewer: How do you, as a doctor, as a surgeon, kind of treat these different types of conditions? What are some of the common ways that they're treated?

Dr. Rollins: The more severe malformations, I'll just address those first. So if the rectum is connecting, for instance, to the bladder in a boy or if the rectum is blind-ending, within the first one to two days of life, we create a colostomy so that the child can pass stool into a colostomy bag. Then allow the baby to grow, get older, and then do more definitive imaging to determine the exact location of the rectal fistula, whether it's connecting to the bladder or to the urethra, which drains the bladder somewhere along its course, or in a girl, to the vagina. And then we can make plans for a definitive repair which, depending on the malformation, could happen anywhere from three months of age to a year of life.

Now, the other course of treatment would be if the patient, the baby, does have a perineal fistula, one treatment option that we may use is a dilation of that fistula to dilate it up to where the size is large enough to actually pass stool and then have the parents do that at home until the child has a chance to grow, and then do the repair in a single stage, which would be around three to six months of age. And those that are treated with colostomy first, they'll have their definitive repair, as I mentioned, generally between 3 months to 12 years of age. And then once that repair heals, their colostomy is closed, so they end up with three operations.

Interviewer: And after all of this, these interventions, all these surgeries, all these treatments, what's the aftercare like? How long and what kinds of things do parents need to keep in mind with their child?

Dr. Rollins: Parents need to keep in mind is that this is a lifelong condition and this is a lifelong relationship that they're establishing with their care team. Postoperative care is fairly intense, and the fact that these can be associated with a number of other anomalies of the esophagus, of the spine, of the spinal cord, of the kidneys, of the genital tract, two-thirds of children will have one or more associated anomalies, and so the care requires a multidisciplinary approach. And you know, it's not a one-surgery and you're finished type of problem.

Things that will affect sort of long-term function, the character of the spinal cord and the sacrum or the lower spine, we know that if there are spinal cord malformations or severely abnormal sacrum, that those children have less development of the muscles required for fecal continence and the nerves required. And so they're more likely to be incontinent and will need to be on an intense bowel management program throughout childhood and potentially throughout their life.

Interviewer: So what do you tell parents after their child is diagnosed with the malformation?

Dr. Rollins: I think we've made tremendous strides in treating these kids by approaching this in a multidisciplinary way. Decade or so ago, often the renal anomalies were not recognized or addressed, and those children went on to experience renal insufficiency, renal failure, or even need a renal transplant. Now, you know, by approaching this in a multidisciplinary way, we identify kidney abnormalities, for instance, early, manage them proactively, and avoid renal failure. That's just one area.

The other thing that I want parents to know is that their child can go on to live a completely active life. Even if they are one of the children that suffer from fecal incontinence, we have a structured bowel management program at Primary Children's, and there are other places in the country that have a similar program, we can artificially keep these kids clean and in a normal underwear, with either a large volume enema program or, in some situations, a high-dose laxative program. But we're able to keep them clean and in a normal underwear, and they can spend the night with friends, they can play sports, they can go to camps like their friends.

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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.

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Interviewer: Hirschsprung's disease is a rare intestinal disorder that can severely impact a child's ability to pass stool. What treatments are available and what you can expect for your child on The Scope.

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Interviewer: I'm here with Michael Rollins, a professor of surgery in the Division of Pediatric Surgery at the University of Utah Medical School and Primary Children's Hospital, and we're talking about Hirschsprung's disease. Am I pronouncing that right, Hirschsprung?

Dr. Rollins: Yeah, that's correct.

Interviewer: So what exactly is this disorder?

Dr. Rollins: Hirschsprung disease is a disorder where the nerve cells of the rectum and/or part of the colon don't form normally, and these nerve cells are responsible for relaxation of the rectum and colon to allow the passage of stool. So basically, a child is born with an intestinal obstruction because they're unable to pass stool.

Interviewer: So that can be pretty serious, I would think. How common is this type of disease?

Dr. Rollins: It occurs in about 1 in 5,000 live births, and yes, it is serious. It's a problem that most of the time will progress to severe abdominal distension or vomiting, feeding intolerance, and can even progress to a life-threatening infection called enterocolitis or an intestinal perforation if it's not recognized within the first few days of life.

Interviewer: What are some of the ways that physicians, surgeons kind of treat this disorder?

Dr. Rollins: The majority of babies will pass meconium, the first stool, within the first 2 days of life, up to 98%-ish. So probably the most common way that this is diagnosed is the baby is born and they don't pass stool in the first two days of life. Second most common way would be that the baby doesn't tolerate feeds in the first one to two days of life, becomes significantly distended, the abdomen becomes distended, or they're vomiting, and also probably not passing stool or passing very little stool.

Those are red flags and those prompt further either consultation to a pediatric surgical service or further investigation, which most commonly the first study would be plain abdominal x-ray or what's called a contrast enema, where a small tube is placed in the child's rectum and contrast fluid, contrast dye is infused to look at the rectum and colon.

Interviewer: And then how do you actually treat or correct? What intervention do you do with this disorder?

Dr. Rollins: Hirschsprung disease requires surgery for correction. So as I mentioned a little earlier, it's a spectrum of a disease. So the very end of the large intestine may be affected, so the rectum only may be affected, but variable lengths of the colon upstream, or even in more or the most extreme cases even part of the small intestine may be affected, so that stool can't pass. So the treatment options would be different really based on the level of the disease.

So 80% affect only the rectum, so what we call a short segment. And in those babies, the treatment option, they can be treated in three different ways. The first thing is a rectal biopsy is performed at the bedside to make a definitive diagnosis. So the pathologist won't see the nerve cells when they look at it under the microscope. And so once you have that definitive diagnosis, then you can either proceed to the operating room shortly thereafter and determine the level of the normal intestine, resect or take out the abnormal portion, and bring the normal intestine down to a small, little rectal cuff just above the anal canal. So a one-stage operation.

Another treatment method may be to perform daily irrigations or washout of the rectum and colon. Parents often do this at home. And then once the child has a chance to grow, get a little bit bigger, we'll come back around three months of age or so and do that one-stage operation at that point. Sometimes, it's technically a little bit easier, and then, you know, the babies had a chance to go home and be with parents and develop that normal relationship.

And then the third way to treat it would be with a colostomy at birth. So go to the operating room, determine the level of the normal intestine and bring that up to the abdominal wall so that stool empties into a bag. And then once the child has grown and gets older, go back for the definitive operation, where the abnormal portion is resected and the normal portion is brought down to the bottom.

Interviewer: And what kind of care should a parent expect after this procedure?

Dr. Rollins: So the tricky part of Hirschsprung's disease is intuitively you'd think that, "Okay, the abnormal intestine is gone, my baby is cured." And we thought that for a long time. But we've begun to realize that we as a surgical community, medical community have begun to realize that that's not actually the case.

You know, in the past, we would do the operation, we would see the child back a few weeks afterwards, everything looks great, great operation, you know, good to go. And those children would develop problems with infections of the intestine called enterocolitis, can get a bacterial overgrowth that can cause severe infection, which the symptoms of that are fever, abdominal distension, vomiting, severe explosive diarrhea. And the treatment for that is washouts, rectal washouts, which parents can do at home and which here, at Primary Children's in the university hospital, we teach all of our families how to do that so that if those symptoms develop, they can contact us, let us know, and begin that treatment at home.

So that's kind of the short term, you know, what we worry about after we're satisfied with the surgical side healing and things like that. So we worry about enterocolitis initially. Seems like that becomes less of a problem after the first couple of years of life.

And the issue that seems to be most common during childhood, and even into adolescence, is constipation. And it's constipation beyond what you think of when you think of constipation, maybe hard stool that's infrequent that the pediatrician or primary care provider treats routinely each day. This is constipation that requires often a formal bowel management program with a high-dose stimulant laxative therapy to get the large intestine to squeeze and empty normally. If this constipation is not recognized, left untreated, over time, it can result in a dysfunctional colon. So we stress to families that, you know, this is a long-term relationship with us as the care team in order to avoid these complications.

Interviewer: And with this kind of long-term care, what kind of quality of life can a parent expect for their child with this disorder?

Dr. Rollins: The great news is, as I mentioned, our thought process in the past was not complete. Now, we are recognizing these issues, and with early management, these children going to be highly functioning, you know, normal functioning children playing sports, going to regular school, sleepovers, camps, things like that, they just may have to take medication each day in the form of a laxative, which you know, I mean, I explain to children and to their families that you wouldn't think anything about taking your inhaler every day if you had asthma. So you know, this is the same thing.

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Announcer: Examining the latest research and telling you about the latest breakthroughs. The Science and Research Show is on The Scope.

Interviewer: All right. Here's a question. How can you reduce the use of imaging tests for patients that you are evaluating for appendicitis? That's what Dr. Eric Glissmeyer's research focused on. Thank you for coming to help answer that question. First of all, why did you want to answer that question?

Dr. Glissmeyer: Well, particularly when we're evaluating children that are being seen for abdominal pain and they may have appendicitis, we like to not do dangerous things or harmful things to children. It's not uncommon that a CT scan, a test with ionizing radiation that exposes the child to potential risk of harm, is done, especially in uncertain cases. So we wanted to try to set forth the protocol with the support of our surgeons and our radiologists that helps us one, standardize our approach and two, reduce CT use and that was our primary objective.

Interviewer: How have people tackled this problem before?

Dr. Glissmeyer: Well, there are a number of places around the country that have done great work in establishing protocols that many times have been just developed iteratively in a quality improvement kind of way, that have had the focus of reducing CT use.

I think that one unique approach that we took here is we didn't want to just know when is CT scan used in patients who are ultimately proven to have appendicitis, but when is it used in that larger demographic, that larger denominator of patients who are evaluated for suspected appendicitis? The doctor goes and feels their belly and says, "They may have appendicitis. I better rule them out for appendicitis."

What do you do next? We have a lot of tools at our disposal and there's a lot of variability in what people do. We wanted to standardize that with that objective of reducing advanced imaging tests like CT scans.

Interviewer: All right. Here's the moment of truth. What do you do?

Dr. Glissmeyer: What we believe the best thing to do is to take a standardized approach driven by physical exam and utilizing a scoring scale. We use something called the Pediatric Appendicitis Score and there are a number of other scores being used. We don't really think it matters exactly what the score is. We don't think there's any magic in one particular lab value or one particular physical exam finding.

Appendicitis is sneaky and it likes to present many different ways. What we've found though is with the support of our surgeons and our radiologists all working together to determine an algorithm, that as we go down and we do a blood count and we do an exam and we see what some of those initial results come back as, we can either put the patient into a category of low risk and, "Gosh we're done." We really don't need to have much worry about appendicitis unless things get worse, intermediate risk, where perhaps an ultrasound test and certainly you have to have an organization that does ultrasound well.

Pediatric ultrasound for appendicitis is not an easy test. Our ultrasonographers have been doing this a long time at primaries and are very good at it. We actually know based on what they see on the ultrasound there are four different grades for the ultrasound result. We know what the likelihood of appendicitis is for those different grades.

So we've got some great data to drive our decision making out of the results we get. You know what? There are some patients who don't even need an imaging test to diagnose their appendicitis. If it's a classic case, straightforward, the labs support it, you're done, call the surgeon, take the appendix out. This was done 20 to 25 years ago with no imaging tests regularly because they didn't have any. So we kind of need to go back to that in some ways.

Interviewer: And in your research, how accurate was this method?

Dr. Glissmeyer: For patients who come in and we see for having appendicitis, I want to first say how do we know when we were looking at this retrospectively that the patient was actually being evaluated for appendicitis? How did you crawl into that doctor's mind and determine, "Well, did they actually suspect appendicitis or were they just coming in for gastroenteritis?"

We developed a surrogate definition, whether they had an ultrasound done, whether they were coming in for a chief complaint or abdominal pain and the word appendicitis was used in the note and a CVC was obtained that 95% of those patients were evaluated for appendicitis.

It had a sensitivity and a specificity of 95%. We're confident that we could evaluate the patients or rather identify the patients that were being evaluated for appendicitis. So in that group, I think the real question people would want to know is what were you doing before you did this protocol and what did you achieve after in terms of the CT use?

Interviewer: Yeah, did it reduce it?

Dr. Glissmeyer: We were one of the lower utilizers in the country in CT. Our baseline data showed we were doing CT scan only 15% of the time in this cohort of patients being evaluated for appendicitis. If you look nationally, it's more around 30%. So we were already a low utilizer of CT.

But what I was so pleased to find was that as we instituted this protocol that has not just the standardization but this follow up option that we'll talk about too, we were able to drop that low rate in half again, from 13% to 6%, from 13% to 6%. So we were really pleased to see we were even able to cut that in half further.

Interviewer: Is there an ability to even cut that more, do you think?

Dr. Glissmeyer: I think potentially. But I think you achieve a certain baseline minimal rate where you get your unclear cases, you get your cases where your patient has been having pain for a week or so and you really suspect that this is a ruptured appendicitis and CT scan is really the optimal test to use. I think around 5% or so is probably about the minimum you want to achieve.

Interviewer: All right. Explain the follow up option you were talking about.

Dr. Glissmeyer: Probably somewhere around a third of patients who have abdominal pain are being evaluated for appendicitis. It's not totally clean, a third, a third, a third, but let's talk about it in those terms because it makes it easy.

If a third of patients come in and it's pretty obvious after your exam and some labs they don't have appendicitis, you're done. You don't need imaging tests. About maybe less than a third, somewhere around 15-20% and they come in and they have pretty clear evidence of appendicitis, labs support it, you call surgery, they get their appendix taken out, no imaging tests needed there.

Then there's this larger middle where the fall into an intermediate range of a Pediatric Appendicitis Score that we have called between 4 and 7. If they have that score, you do an ultrasound scan first. Boy, when it shows the appendix and it shows it's normal or it shows the appendix and it shows it's not normal, that's really helpful. Then you can make your decision based off of that, but if it doesn't see the appendix, what do you do then.

The patient still has some tenderness, you're still a little uncertain. Do you just go stick them in the CT scanner to get your answer? That's what I think historically has been done, when the ultrasound fails to give you the answer you want, we go and scan them. We work with our surgeons to say, "You know what? We've observed this patient for a couple of hours here in the ER. Their pain is not really getting worse. It's not a clear cut appendicitis. Why don't we have them come see you tomorrow morning in clinic?"

So if it's Sunday night between Sunday night or Thursday night where they could go the next morning, that being between Monday and Friday, they can show up at about 7:30, 8:00 a.m., come into surgery clinic, get their belly pushed on, be examined, get lab tests repeated if they need to, perhaps do another ultrasound if necessary and find themselves in the hands of another expert the next morning also with not a second ER charge, which is nice, but being able to come into the clinic.

We thought, "Are we going to go and be doing surgery clinic follow up now? Are people going to just be like all the time overrunning the surgery clinic the next morning?" Actually it's only about one patient every ten clinic days that utilizes this resource. But the fact that you can offer it to them in the emergency department the families love and the docs love because in the faces of uncertainty, it gives them a plan. That's been the magic of the approach.

Interviewer: What's the next iteration then of this research? Where do we go from here?

Dr. Glissmeyer: We've been pleased with what we've been able to achieve at Primary Children's Hospital. It's to take it to other hospitals within Intermountain Healthcare, University Healthcare, having the support of surgeons at those other hospitals is key and radiologists as well, we're working on that. But only half of the appendix case present to the tertiary children's hospital here, Primary Children's Hospital. We want to address that other important half and that's where we're going next.

Interviewer: Getting that feedback from the surgeon afterwards for the patients that didn't get the imaging but had the operation and you find out how successful you've been or haven't been. Have these tools proven to be pretty successful?

Dr. Glissmeyer: That's a great question. You wouldn't want to be going and admitting to the surgery service a lot of patients who you as the ER doc are convinced have appendicitis and then they go in there to take it out and then, "Uh-oh, it looks normal." That happens on a rare occasion. Nationally about 5% is the rate of negative appendectomy. You go in and, "Oh, we thought it was appendicitis and it's not."

Our rate here is about 2-3% and it's not increased since the use of the protocol. So going in and taking out patients' appendix without ultrasound test or any imaging test, it still is successful and doesn't increase that negative appendectomy rate.

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Interviewer: You notice that your child is starting to develop a flat or misshapen head. Is that something to really worry about? And what should you do? We'll discuss that with Dr. Barbu Gociman next on The Scope.

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Interviewer: Dr. Barbu Gociman is a plastic surgeon who specializes in pediatric plastic surgery at the Craniofacial Program at the University of Utah Health Care. If your child is showing signs of a flat or misshapen head, could be a couple of things going on. We're going to find out more about that right now. Is that always something to worry about?

Dr. Gociman: We see two different patterns. One would be the parents are really worried, the other one, the pediatrician is very worried. In either instance, there are two different causes. The more common deformity that you see is actually acquired after birth, is due to positioning in the uterus. As babies, we have a position of comfort. That is whatever the position of the uterus is. So once the baby is born, they tend to reproduce that position and preferentially just lay on one side.

As the brain pushes the bones and the head keeps growing, because the baby preferentially lays only on one spot, that becomes flatter and flatter. The rest of the cranium grows, and suddenly at three, four, five months of age, your baby has a flat head. Usually, it's on the back of the head one side. Obviously, there's a different degree of severity. We say usually about 1 in 300 babies has a very severe deformity that will need some time of aggressive management. That is the least severe problem because it's just an easy fix. Basically, you have the baby not lay on that spot. We will have to have the parents aggressively trying to rotate the baby from that spot. The challenge is the baby will always want to turn back into the same position.

Interviewer: How do you prevent that? What are some strategies you have to prevent that?

Dr. Gociman: Unfortunately, there's no one perfect strategy. All the pillows and things that try to make it through the FDA were to kind of reposition the baby in the crib did not make it because of safety hazards. They can act as chokers, basically. So there's no good device that will keep the baby from rotating so it's just the diligence of the parents. Some parents will be more diligent than others.

Interviewer: And you just have to go back in there and just . . .

Dr. Gociman: Keep rotating. Obviously, once the baby is growing a little bit more, more tummy time, more sitting position and that will alleviate the problem.

Interviewer: Gotcha.

Dr. Gociman: If the deformity is really severe, then we recommend a helmet, which the helmet is worn for 24 hours a day, basically. That will take off all those problems where the parents will have to go repeatedly and change the position.

Interviewer: The harm is not only a misshapen head, but it could actually affect the brain?

Dr. Gociman: There's absolutely no influence of the developing brain. It's just an aesthetic problem.

Interviewer: Oh, really? Okay. Well, I guess that's a relief on some level.

Dr. Gociman: This is the more common problem of a flat head. Unfortunately, there is a much more severe problem that could lead to a flat head or misshaped head. The technical name is craniosynostosis, in which one of the sutures, which normally separates the different bones into the cranium, fuses prematurely. The sutures usually fuse when we are in our 20s or 30s. If a suture fuses prematurely, either in utero or as a very, very young baby, the head will be forced to compensatorily grow and you will deal with a really misshapen head.

One suture can be fused, or multiple sutures can be fused. The more sutures that are fused, the more severe the deformity, and the higher the chance that misshapen head will put pressure on the growing brain and lead to developmental problems. That is a condition that will require surgical intervention. We do some surgical intervention, minimally invasive, in which the operation is done in conjunction with your surgery that is much quicker and the patients recover much easier.

We also, for more severe forms or for delayed presentation, the patient will require really very, very significant operations and the patient will require prolonged hospitalization, intensive care unit care after the operation. So, definitely, the way to tell the difference between the two, although we highly have a suspicion on physical exam, is to do a CT scan. The CT scan will tell us exactly if a suture is fused or not. We'll know 100%. We usually, on clinical exam, we can have a 90% to 99% suspicion of if we deal with one or another. Usually, for positional plagiocephaly, it's very easy to make the diagnosis just on physical exam.

Interviewer: That's the one where sleeping in the same position too much . . .

Dr. Gociman: And which is the more common one.

Interviewer: Gotcha.

Dr. Gociman: In order to make the exact diagnosis, we'll be happy to see you to our Craniofacial Program at the University of Utah, to see you and discuss what the problem is and what the next steps will be.

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Interviewer: You found out your ultrasound that your child possibly might have a cleft lip. What should you do after that? What's going to be coming up and what are some things you need to know? Dr. Barbu Gociman is a plastic surgeon and a specialist in pediatric plastic surgery at the Cranial Facial Program at University of Utah. First of all, what is a cleft lip exactly? What's going on?

Dr. Gociman: Yes, it's not an extremely easy question to answer. It's anomalous and biologic developmental problem in which the forming face and particularly the lips, do not come together. It can be associated with a clefting of the palate. There are different degrees of clefting present. What a cleft means is basically an opening where, instead of the tissues coming together, they do not, and the bigger the defect, obviously the wider the gap between the elements that should come together.

Interviewer: So in the ultrasound meeting, they may indicate the there is a cleft lip possible. It could be very minor to very major. And what's that extreme?

Dr. Gociman: Yes, exactly. There is a whole spectrum. It can start with just a furrow that you can see on the lip which is not going to be present on the ultrasound. To mind comes, Joaquin Phoenix as an actor that has that deformity, as a personality that you can always look up. From that point you can have different degrees of deformities, just small degrees of notching in the lip. It can go all the way to the nose and extending to the alveolus, which is the bony part carrying the teeth, and all the way into back of the palate, which is the roof of the mouth, can be completely clefted.

Interviewer: And do the ultrasounds, do you have a pretty good indication of how bad it's going to be at that point? Or you could a parent be even surprised even more after the birth?

Dr. Gociman: Yeah, the ultrasound, obviously is a good modality to diagnose, but as any diagnostic modality, it's not 100%. So you'll not completely know the degree or exactly what structures are affected until you actually see the baby and you can examine him.

Interviewer: And that degree can mean the difference between maybe a single operation or many operations through the child's childhood, up until 18, 19 years old.

Dr. Gociman: Yeah, exactly. So there is a stepwise process that has been validated over the last 50 years or so, and obviously it continues to undergo refinements in how we treat these patients. So if only the lip is involved, usually one operation is enough to correct the deformity. The sequence of treatment for the more severe deformities involving the lip and the palate would be we start taping or do what we call a nasal alveolar molding, which would be reshaping of the structures and bringing them in a more anatomic position to facilitate the surgical repair. That is done by our orthodontist, Dr. Duane Yamashiro, and requires multiple adjustment. That will last for approximately for the first three months of life.
We usually do the lip repair around three months of life. At the same time we examine the palate, if the palate defect is really wide, we insert a palatal prosthesis, which is an obdurator that will facilitate feeding.
The second operation is done at around 10 months to one year of age, which is the soft palate repair. That is done right before the speech develops. We exchange usually the palatal prosthesis at the time if a large hard palate cleft is present. We repair the hard palate at around four years. At six years of age we do a repair of the clefted alveolus with bone grafting, if the baby has that problem. And finally we will do a formal rhinoplasty operation at the late teenage years if the nasal deformity is still persistent.

Interviewer: I would imagine one of the major concerns parents have is physical appearance. It sounds like there's also a lot of functional things that aren't going to be right if these surgeries don't happen. But as far as physical appearance, can you bring somebody back to the point where you couldn't tell they ever had a cleft lip?

Dr. Gociman: Unfortunately to completely, scarlessly repair somebody, it's not possible. We as humans we heal by scarring, so you'll always see a scar. The quality of the scar can be from a very, very thin line, which it's imperceptible to a person that is not very attune to what he's looking at, to a fairly thick scar that will require revisions or steroid injections or scar treatments. That is at the level of the lip.
As I said, because the nose is mis-shaped by the deformity, usually the eyes are going to be really drawn to that deformity. That's why we put a lot of effort in reconstructing the nose in a very anatomical fashion to try to avoid that from happening. We do the cleft lip repair at three months of age, just because we want the parents and everybody that comes in contact with the baby to see a normal baby. We do not want them to get used to the clefted baby.
All the other functional portions, the palate actually, functionally, the palate repair is much more important because the palate is involved in speech, is involved in swallowing. So functionally that is more important. But what we see, and it's human, we judge, where most of the people, the lip repair is the most important step and that is why we do it first, and that's why we pay a lot of attention to it.

Interviewer: I'm sitting here, hearing this and in a way my heart is breaking for parents that find out that this might be something that their child will have to face. How do you even address that? What do you say? How can people find comfort?

Dr. Gociman: It's definitely not an easy process. Fortunately, especially nowadays there are all the support groups. We can always put you in contact with the families that went through with our group. That brings a lot of comfort. It's very difficult, especially the first moment when you feel like you are all alone confronting with such a big, challenging problem. But our Cranial Facial Program at the University of Utah has the answers and we are very happy to address all of them multiple times. Our phone number that you can reach us is 801-581-7719. The Cranial Facial Center is the way to go.

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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.

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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.

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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.

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Dr. Gellner: A common concern parents have, is that their child's feet turn in. I'm Dr. Cindy Gellner, and today's topic on The Scope is Intoeing.

Dr. Gellner: So what is intoeing? It's just like it sounds. Intoeing means the toes point inward. There are several different causes, but it's very, very common in babies, and young children. A lot of people will refer to it as pigeon toed.

So what does cause intoeing? Intoeing is observed in children less than 2 years old most often. It's caused by the shin bone, which is the tibia, the bigger of the two bones in the lower leg. That bone may be actually turned in a little bit. It's called tibial torsion, and it's caused because of how the baby is positioned when it's still inside the mother's womb before birth. For children over 2 years old, if this intoeing is still present, or it's new, it's most common cause is due to the thigh bone being turned in at the hip. That condition is also caused from birth. It causes the knees, feet, and toes to point inward. So it's not just the feet, like you would see in intoeing from the shin bone, but it actually is more the whole leg.

If your child has just the front part of the foot turned in, that's not intoeing. That's called club foot. That's usually something you pick up at birth.

So how is it diagnosed? Usually as pediatricians, we just need to watch your child walk to see if they have intoeing, and what it's from. X-rays normally are not needed to diagnose intoeing. Once your child is diagnosed with intoeing, how do you treat it? Most children really don't need any treatment at all. The intoeing gets better on its own. When it's caused by the shin bone being turned in, it usually gets better once the child starts standing, and walking for a while, usually around age 5. If it's caused by the thigh bone being turned in, the main treatment is simply having the child not criss cross their legs while sitting down. The problem usually gets better when children start school, and they have to sit in chairs, and they're not sitting on the ground as much.

So how can you take care of your child, if they do have intoeing? The thing I recommend most, is just making sure they have supportive shoes, and being patient. Waiting for it to get better, or see if it gets worse. There's really not much you can do about intoeing, and there's really nothing you need to prevent your child from doing. Let them be active, let them play, and often this corrects on its own. I'm often asked, "What could I have done to prevent this from happening?" The bottom line is, nothing.

Children are born with intoeing. There's nothing you could do to prevent it. It's just because of how they are positioned, and curled up inside the womb before they're born. So, if your child is walking a little funny, they've got their feet turned in, chances are, it's going to be just fine. Just keep an eye on it. If your child continues to have the intoed feet, and it actually is causing a lot of problems with running, and walking, and daily activities as they get older into their later elementary school ages, at that time, then your doctor may refer you to a specialist.

updated: January 4, 2021
originally published: February 16, 2015

Interviewer: A study finds that when it comes to choosing health care procedures, cost matters. More next on the Scope.

Announcer: Examining the latest research and telling you about the latest breakthroughs. The Science and Research Show is on The Scope.

Interviewer: I'm here with General Pediatric Surgeon, Dr. Eric Scaife. Dr. Scaife, tell me about your study.

Dr. Scaife: It occurred to me that unlike many other parts of our economy, the consumers don't really understand the cost of medicine despite the fact that it represents about a quarter of our economy. And so as a pediatric surgeon one of the most common things that we deal with is pediatric appendicitis. We take out one or two appendixes a day. And we have two different ways of taking out your appendix. One with an open technique and one with a laparoscopic technique which requires specialty instruments.

And the outcomes in children with non-perforated appendicitis turns out to be the same, but the cost is quite different. And so it presented a really unique model for us where we could have a procedure with similar outcomes, but a very big difference in terms of cost. And we thought why not allow the patients to consider the cost in terms of which operation they get.

Most of the time we actually make the decision for them. And so it would be an interesting experiment that a patient could actually behave like a consumer as they would in any other part of the market.

Interviewer: Right. And what kind of difference in cost are we talking about?

Dr. Scaife: So for our study the open operation was about $3,000 cheaper than the laparoscopic operation. And the primary difference there is that the open operation requires a scalpel and some sutures and some instruments to provide exposure whereas the laparoscopic operation requires a number of gadgets, most of which tend to be disposable.

Interviewer: Does the patient end up paying a difference in cost?

Dr. Scaife: Well, this is where medicine gets really complicated and it's hard to peel out all of these differences, but I can tell you when I spoke with Primary Children's accountants, so basically their answer is that at the end of the day these are direct cost differences in terms of materials that are charged to the patient. So theoretically they should be exposed to that difference in cost. Whether or not that actually happens or not gets a lot muddier.

Interviewer: What were the results of the study? What did the patients decide?

Dr. Scaife: We randomized patients to either be exposed to just the difference in the procedures versus other patients that saw the difference in procedures plus the

Host: Now a shorter hospital stay and a faster recovery for children that need an appendectomy. We'll examine that next on The Scope.

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.

Dr. Skarda: Traditionally, in pediatric surgery and, in particular, with younger patients who have appendicitis, we were very worried about the potential for complications and, in particular, infection inside the abdominal cavity. Because of that high degree of concern, we essentially created protocols for treating these patients after their appendectomy, which included relatively long durations of IV antibiotics lasting 7, 10, 14, sometimes 21 days and associated in-patient, in-hospital stay for that entire period. This did decrease the rate of infections, we think, but we just treated patients for a very long time with in-patient IV antibiotics.

Host: So at one point, that was a solution to a problem that somebody saw.

Dr. Skarda: That's correct.

Host: And then you kind of started reexamining it. What caused you to think, "Well, maybe we can actually shorten these stays?"

Dr. Skarda: We noticed here at Primary Children's that a fairly large percentage of these patients were ready to go home two or three days after their appendectomy. Because of the protocol that we had in place, they were required to stay in the hospital well beyond that point.

Host: Yes. So they were feeling fine, and everything looked good as far as you were concerned. But they had to stay.

Dr. Skarda: They had to stay because they were on IV antibiotics.

Host: Yeah. And because that's what the protocol was.

Dr. Skarda: And that was consistent, I think, with most children's hospitals across the United States.

Host: All right. And then what made you think, "Maybe we can shorten this"? I mean, how do you go through that process of suggesting that this is a better way to do it, maybe?

Dr. Skarda: Well, we had talked about it as a group of surgeons for some time. Looking at that issue very closely, we realized that there was a very high likelihood that these patients who are ready to go home probably no longer needed antibiotics. And I think that the critical issue here was that not all patients with appendicitis necessarily respond the same way, and there are may be some that do not need antibiotics as long as others. If we can identify who they are, stop their antibiotics, and get them out of the hospital, they'll likely do better.

Host: Yeah. And actually, the data showed that.

Dr. Skarda: Yeah. Once we initiated our new protocol, which is clinical response based, meaning from the moment of the operation, we monitor patients every two hours at the bedside to see if they're drinking enough, eating enough, their pain is well controlled, and if they don't have a fever for 24 hours, they're ready to go home. Once they meet those criteria, then we send them home. We do check labs on their way out the door, basically, to see whether or not they need any oral antibiotics at home. Some of them do. Some of them don't. Then we follow them back in clinic in one week and make sure that they're doing well.

Host: And the hospital stay is shortened how much?

Dr. Skarda: This protocol decreased our hospital stay on an average of two days. So from 7 days to 5 days, and this decreased the cost of care by about $5,000 per patient with appendicitis and improved their outcome in terms of abscess rate from about nine percent to about five percent.

Host: Awesome. So it just went all the way around. Shorter hospital stay, less money, and better outcomes.

Dr. Skarda: That's correct.

Host: All right. And this was just all the post-operative procedure?

Dr. Skarda: That's right.

Host: But there's more to this story? You actually said, "Can we maybe do more?"

Dr. Skarda: Yes. Exactly. So although we accomplished a lot with the post-operative component of this, we realized that there are other areas where we could potentially improve. In most children's hospitals, there are many different surgeons performing appendectomies, and each of them perform their appendectomy in a slightly different way depending upon where they were trained, who trained them, and what seems comfortable for them.

Oftentimes, though, the instruments used to perform the appendectomy are relatively expensive. Given this variable nature of a lot of ways to make a taco or do an appendectomy, what we figured out is that there's probably a good way that we can perform appendectomies. We can essentially standardize the procedure so that everyone's doing it the same way. I then spent a lot of time and identified a specific set of devices and a specific way to do the appendectomy that was cost-effective and efficient. I then got all of my partners to agree to do that at Primary Children's.

Host: Was that hard to do?

Dr. Skarda: You know, it actually wasn't. I think once they saw the data, they saw the variability, they saw the cost involved, and they saw what was possible in terms of doing it all the same way, they actually agreed to it fairly quickly. Then once the procedure itself was standardized, we were able to decrease the cost per appendectomy at Primary Children's from about $800 or $900 in disposable devices that would never be used again to about $150 to $200 of disposable devices and, quite frankly, reusable devices. We've had this in place now for more than a year, and given our rate of appendectomies here at about 500 per year, that ends up being a cost savings of around $200,000 to $250,000 a year.

Host: Not an insignificant amount of money, especially in today's day and age when everybody's talking about healthcare costs and insurance premiums.

Dr. Skarda: Right. Well, it's really all about value, which incorporates the importance of having good outcomes. We monitor those as we made these changes, and, again, our outcomes have actually improved. We're having fewer abscesses, shorter duration of hospital stay, shorter operative times, and our physicians are happy. And more importantly, the parents and our patients are happy.

Host: Yeah. That's exciting stuff. Is this kind of a common thing, that procedures are standardized and we don't necessarily look at the equipment used as closely as we should throughout medicine, or was it just kind of unique to this procedure?

Dr. Skarda: I suspect there's been some drive towards standardizing procedures in other countries. However, in the United States, to my knowledge, this is the first successful attempt to do that.

Host: Well, that's exciting. Are other hospitals finding out about this, asking you how you did it? Is this something you're passing along, paying it forward?

Dr. Skarda: Yeah. There's no question there. Other facilities are very interested in this. This data will be presented this fall at the AAP Conference in San Diego, and we anticipate that as this information and this idea is disseminated throughout the United States that there will be a significant degree of adoption. There's a great deal of interest, of course, in value, improving care, and decreasing cost. And this certainly fits into that category.

Host: Where are you looking at fixing next?

Dr. Skarda: We've also standardized cholecystectomy, or removal of gall bladders at Primary Children's. And we're considering moving onto other procedures.

Host: And final thoughts?

Dr. Skarda: I think there's an enormous opportunity in medicine and, in particular, at Primary Children's Hospital to standardize and improve care and improve value.

Announcer: We're your daily dose of science, conversation, medicine. This is The Scope, the University of Utah Health Sciences Radio.