Cleft lip and palate is one of the most common birth defects in the United States.
James Y. Liau, MD, FACS discusses what the treatment options are like for a child as they grow, and how it should not keep a child from having a healthy, normal, productive life. He will also discuss plagiocephaly, which is when a baby’s head is extremely flat on the back or one side.
Cleft Palate Care and Plagiocephaly
From the Show: UK HealthCast
Summary:
Air Date: 2/19/19
Duration: 10 Minutes
Transcription:
Melanie Cole, MS (Host): If your child needs surgery to fix a deformity caused by a birth defect, injury, or illness, a pediatric plastic surgeon has the experience and qualifications to treat your child. My guest today is Dr. James Liau. He’s an associate professor of plastic surgery at UK Healthcare. Dr. Liau, before we talk about cleft lip and palate and plagiocephaly, I’d like to ask you what kind of training do pediatric plastic surgeons have? What makes you so unique that you can work with children with these kinds of sensitive issues?
James Y. Liau, MD, FACS (Guest): Well thank you for the opportunity. Actually, for pediatric plastic surgery is a subset of plastic surgery. In order to complete plastic surgery here in the United States, you need to do at least a one-year internship for general surgery. Then depending on the program, five to six years of plastic surgery. After that, you then proceed on to doing a pediatric plastic surgery fellowship, which is one year of length. That usually focuses on congenital anomalies such as cleft lip, cleft palate, skull anomalies, vascular tumors, and other sort of associated congenital issues.
Host: Then let’s start with cleft lip and palate. How common is it? Really tell us what it is and how it happens.
Dr. Liau: Sure. It’s relatively common, even in the United States. Often times you associate cleft lip and palate with conditions in other countries. For example, global health trips and mission trips are very commonly focused on cleft lip and palate. The reality is in the United States it’s quite common as well. It’s usually about 1% to 2% of live births. No one really knows why cleft lip and palate happen. There’s a lot of other issues which can occur, including family history. Sometimes, obviously environmental factors such as maternal factors can cause a cleft palate for example. No one has a smoking gun, so to speak, as to why cleft lip and palate occur.
Host: Can you tell if a fetus will have it? What are some complications if it’s left untreated? Is it difficult for children to eat and drink with a cleft palate?
Dr. Liau: Absolutely. So, the cleft lip and cleft palate, they're usually diagnosed in a prenatal ultrasound. Cleft palate, by itself, is a little more difficult. It depends on sometimes luck of the draw. A lot of kids like to hide like their faces when you try to do the prenatal ultrasound. Routinely, I would say, 99% of my cleft families that I meet, I meet prenatally because we have it on the ultrasound. I can meet with them and tell then what is going to happen.
A lot of issues occur with cleft lip and palate. Feeding, as you mentioned before, can be a real issue. These children cannot generate suction. As a result, they need to be fed from special bottles or special techniques. The other issues which can occur with time is that obviously the nose area and the mouth are connected basically. There’s no palate in the way so to speak. So, eating solid foods can be an issue. It can come out of their nose consistently.
Ultimately, the thing that is probably the most traumatic for a child is obviously not only the appearance but the function. Without a palate, a child cannot talk well and cannot talk in a way that can be understood. Obviously, as social beings, that is a devastating deformity. As a result, they do not socialize well. They don't have friends, et cetera, et cetera. So, it can be a very functional both socially and physiologically.
Host: So then when is the right time to consider surgery? When you're counselling parents about this, when do the surgical procedures start? I said procedures because I know it does sometimes, and quite often, take many procedures. When do you start that discussion with the parent?
Dr. Liau: So yeah. The prenatal visit, I think, is extremely key. It gives the parents a little bit of, obviously, information. The unknown is very scary, but also gives them a timeline. We used to try to fix cleft lips by two months of age. Now, I think, with the current standard of cleft care, that’s pushed out to more to be the six months of age. A lot of the rational behind that is simply the children are bigger, it’s easier to fix them, and there’s absolutely no rush once we get over the feeding difficulties of the child. The first year is the busiest year. The cleft lip repair around three to six months of age. The palate repair is a little bit more stringent in that I like to get it done before a year of age. So anywhere from 10 to 12 months of age.
The reason for that is, the will actually be doing fine with the cleft palate once we can get over the feeding as mentioned before. But what a child can't do, as we mentioned before, is talk well. Generically speaking, a child will start talking about a year of age. So, we would like to have the palate fixed and in place before the child’s brain really starts to begin the process of speaking and sort of the familiarity of speaking. That’s just the first year.
After that, there are several other surgeries, unfortunately, for the child. Usually around five to seven to nine years of age, we may do a lip revision or a nose revision if they child has a full cleft lip. Once again, we focus this on speech. For some reason even though we put the palate and everything in the right place, the speech is still problematic. As a result, they may need a secondary surgery on their throat to help them speak well.
If they have a cleft gumline, which is usually the case with cleft lips and palates, then around 9 to 12 years of age we’ll put graft in from their hip using the bone marrow there to allow the tooth to grow in normally. Then we usually do another revision like a rhinoplasty or nose surgery because they're more finished growing around 12 to 16 years of age. Occasionally depending on the severity of the cleft they may need some jaw surgery. That would also happen around 12 to 16 years of age. So, as you can see, it’s, unfortunately for the children, a lifelong process.
Host: Dr. Liau since you mentioned feeding and talking, and because this takes so many years to really get to the point where the child is really comfortable with it all, what type of specialists are involved in treating the child with the cleft lip and palate as they grow?
Dr. Liau: There is very large multidisciplinary approach. Not only do you have these physicians which would include a plastic surgeon, an ear, nose, and throat surgeon to make sure that the hearing is doing well. Often times with a cleft palate you have hearing dysfunction, and that’s just from fluid buildup in the ears. You have oral surgeons who will also do the jaw surgery when they're a little bit older. You also have a speech pathologist. That is probably one of the most critical personnel in the multidisciplinary approach. From there, they can diagnose any sort of speech disorders, stratify whether it’s simply a physical issue with speech or mental or if it’s both. Obviously institute the therapy required to make sure that their speech is doing fine.
Pediatric dentistry is often involved because there can be anomalies of the teeth, that’s not uncommon. Then orthodontists are also involved to make sure that the teeth start getting lined up, especially when we look towards bone grafting to get the adult teeth in place. That’s pretty important. On the more social side, you definitely need a social worker. There’s a lot of visits. A nurse coordinator is integral because there is a lot of appointments to make and it’s very difficult sometimes to figure out where to go and just to kind of maneuver your way through the healthcare system.
Then nutrition. Nutrition is a big key, especially in the beginning. They work closely with speech pathologists to make sure that they're feeding well. As you can tell, it’s a whole host of personnel and healthcare specialists in order to bring them the best care.
Host: Well it certainly is multidisciplinary Dr. Liau. So now let’s talk a little bit about plagiocephaly. It’s a little bit more common even than cleft lip and palate. What is it and why does it happen?
Dr. Liau: Yeah, absolutely. So, I would say probably a good third of the kids I see in my clinic are kids with what we call plagiocephaly or deformational plagiocephaly. Luckily, it’s not surgical. That’s one thing I want to emphasize right off the bat. However, it can be sort of a problem.
Usually what it is… Basically what we’re talking about is when you see a child, their forehead is very asymmetric. One side juts out more. The back of the head is quite flat, and oftentimes at an angle. The ears, like I mentioned before, jut out in a different way. Also, sometimes the jaw can be smaller on the effected side. Basically, what’s happening is that while the child is developing in the belly, their neck is usually tucked in a certain way. When they come out, they are sort of fixed in that position. If it’s very severe, it’s basically torticollis, which you basically have a neck muscle spasm. The overwhelming majority of kids sort of work out of it.
However, in this country, we’re a back to sleep nation. Subsequent because of that, the skull is quite flexible at that age. We put them on their back to sleep. So, their neck is tilted a certain way, they're a back to sleep child, so they flatten out in that area. It’s oftentimes not symmetric. The skull does what it’s designed to do, which is to sort of accommodate pressure and allow the brain to grow. As a result, it grows asymmetrically or, as I mentioned before, the forehead is more forward on one side. The ears are a little bit off. As a result, I often see a lot of these kids coming in for this sort of deformity.
The treatment is pretty straightforward though. As long as you get them off the back of the head, in that region, the head will round out. You see the biggest difference around four to six months of age, up to eight months of age. The reason is because at that time, kids have great neck control. They’re looking around. You come in in the morning to the crib and they're on their face, even though you put them on their back. So, they're wiggling, they're looking around a lot more. Belly time obviously helps because it gets them used to being on their belly, off the back of the head. As a result, it alleviates that pressure and subsequently allows them to grow more in a round fashion.
Host: So, it goes away.
Dr. Liau: It can go away. The trick is the timing. For kids, and there are some kids for whatever reason… For example, a more severe case would be a child with cerebral palsy, you just cannot get them off their back or their neck straightened out enough. Unfortunately, in those cases, we do a shaping helmet. How that works is I send them to a prosthetist and the prosthetist who specializes in this gets an image of the head. Then they make a helmet. The helmet doesn’t squish them down or anything like that, it doesn’t compress them. What it does do is it blocks the areas that are asymmetric. As a result, the head will continue to grow into those areas and round out.
The issue is that the time that the helmet is most effective is before one year of age. The brain actually slows down in velocity and the head growth circumference range around a year. So, if you're going to use a helmet, you harness that growth and the velocity of the head growth to round it out. So, if you put a helmet on after a year of age, it tends not to be as effective because the brain’s just not growing as quickly any more.
For me, and different practices vary, but for me if you shape a kid at six months of age and you shape the kid at eight months of age, at a years’ time they're both going to be the same. The number of kids that require therapy at eight months of age drops dramatically. So, it’s just that natural growing, looking around, getting off their back. Those two months in itself can make a huge difference.
Host: So interesting. Dr. Liau wrap it up for us. What you’d like the listeners to know about cleft lip and palate and plagiocephaly and why they should seek out a plastic surgeon and ask these questions if their child has one of these issues.
Dr. Liau: Absolutely. Cleft lip, cleft palate, in my mind, my practice, my experience, there’s no reason why your child should have any sort of disability or anything to hold them back in life from being a very happy, productive person in life. I think a lot of that comes from the special training that we received and the focus that we have and different perspective we have on cleft lip and cleft palate. So definitely if your child has a cleft lip/cleft palate, find a center, find someone who specializes in cleft lip and palate versus someone who more or less “dabbles” in it.
In regard to plagiocephaly, there are a lot of conditions that can mimic plagiocephaly. They're not very common, but that’s why you come to a pediatric plastic surgeon. They're going to be the ones that catch the uncommon one which can be very detrimental to your child.
Host: Thank you so much for joining us today Dr. Liau. And for sharing your expertise explaining these things because parents, we get so scared so easily about things. You’ve really given a lot of hope and cleared everything up so very well. Thank you so much again for joining us. This is UK Healthcast with the University of Kentucky Healthcare. For more information, you can go to ukhealthcare.uky.edu. That’s ukhealthcare.uky.edu. I'm Melanie Cole. Thanks so much for joining us today.
Melanie Cole, MS (Host): If your child needs surgery to fix a deformity caused by a birth defect, injury, or illness, a pediatric plastic surgeon has the experience and qualifications to treat your child. My guest today is Dr. James Liau. He’s an associate professor of plastic surgery at UK Healthcare. Dr. Liau, before we talk about cleft lip and palate and plagiocephaly, I’d like to ask you what kind of training do pediatric plastic surgeons have? What makes you so unique that you can work with children with these kinds of sensitive issues?
James Y. Liau, MD, FACS (Guest): Well thank you for the opportunity. Actually, for pediatric plastic surgery is a subset of plastic surgery. In order to complete plastic surgery here in the United States, you need to do at least a one-year internship for general surgery. Then depending on the program, five to six years of plastic surgery. After that, you then proceed on to doing a pediatric plastic surgery fellowship, which is one year of length. That usually focuses on congenital anomalies such as cleft lip, cleft palate, skull anomalies, vascular tumors, and other sort of associated congenital issues.
Host: Then let’s start with cleft lip and palate. How common is it? Really tell us what it is and how it happens.
Dr. Liau: Sure. It’s relatively common, even in the United States. Often times you associate cleft lip and palate with conditions in other countries. For example, global health trips and mission trips are very commonly focused on cleft lip and palate. The reality is in the United States it’s quite common as well. It’s usually about 1% to 2% of live births. No one really knows why cleft lip and palate happen. There’s a lot of other issues which can occur, including family history. Sometimes, obviously environmental factors such as maternal factors can cause a cleft palate for example. No one has a smoking gun, so to speak, as to why cleft lip and palate occur.
Host: Can you tell if a fetus will have it? What are some complications if it’s left untreated? Is it difficult for children to eat and drink with a cleft palate?
Dr. Liau: Absolutely. So, the cleft lip and cleft palate, they're usually diagnosed in a prenatal ultrasound. Cleft palate, by itself, is a little more difficult. It depends on sometimes luck of the draw. A lot of kids like to hide like their faces when you try to do the prenatal ultrasound. Routinely, I would say, 99% of my cleft families that I meet, I meet prenatally because we have it on the ultrasound. I can meet with them and tell then what is going to happen.
A lot of issues occur with cleft lip and palate. Feeding, as you mentioned before, can be a real issue. These children cannot generate suction. As a result, they need to be fed from special bottles or special techniques. The other issues which can occur with time is that obviously the nose area and the mouth are connected basically. There’s no palate in the way so to speak. So, eating solid foods can be an issue. It can come out of their nose consistently.
Ultimately, the thing that is probably the most traumatic for a child is obviously not only the appearance but the function. Without a palate, a child cannot talk well and cannot talk in a way that can be understood. Obviously, as social beings, that is a devastating deformity. As a result, they do not socialize well. They don't have friends, et cetera, et cetera. So, it can be a very functional both socially and physiologically.
Host: So then when is the right time to consider surgery? When you're counselling parents about this, when do the surgical procedures start? I said procedures because I know it does sometimes, and quite often, take many procedures. When do you start that discussion with the parent?
Dr. Liau: So yeah. The prenatal visit, I think, is extremely key. It gives the parents a little bit of, obviously, information. The unknown is very scary, but also gives them a timeline. We used to try to fix cleft lips by two months of age. Now, I think, with the current standard of cleft care, that’s pushed out to more to be the six months of age. A lot of the rational behind that is simply the children are bigger, it’s easier to fix them, and there’s absolutely no rush once we get over the feeding difficulties of the child. The first year is the busiest year. The cleft lip repair around three to six months of age. The palate repair is a little bit more stringent in that I like to get it done before a year of age. So anywhere from 10 to 12 months of age.
The reason for that is, the will actually be doing fine with the cleft palate once we can get over the feeding as mentioned before. But what a child can't do, as we mentioned before, is talk well. Generically speaking, a child will start talking about a year of age. So, we would like to have the palate fixed and in place before the child’s brain really starts to begin the process of speaking and sort of the familiarity of speaking. That’s just the first year.
After that, there are several other surgeries, unfortunately, for the child. Usually around five to seven to nine years of age, we may do a lip revision or a nose revision if they child has a full cleft lip. Once again, we focus this on speech. For some reason even though we put the palate and everything in the right place, the speech is still problematic. As a result, they may need a secondary surgery on their throat to help them speak well.
If they have a cleft gumline, which is usually the case with cleft lips and palates, then around 9 to 12 years of age we’ll put graft in from their hip using the bone marrow there to allow the tooth to grow in normally. Then we usually do another revision like a rhinoplasty or nose surgery because they're more finished growing around 12 to 16 years of age. Occasionally depending on the severity of the cleft they may need some jaw surgery. That would also happen around 12 to 16 years of age. So, as you can see, it’s, unfortunately for the children, a lifelong process.
Host: Dr. Liau since you mentioned feeding and talking, and because this takes so many years to really get to the point where the child is really comfortable with it all, what type of specialists are involved in treating the child with the cleft lip and palate as they grow?
Dr. Liau: There is very large multidisciplinary approach. Not only do you have these physicians which would include a plastic surgeon, an ear, nose, and throat surgeon to make sure that the hearing is doing well. Often times with a cleft palate you have hearing dysfunction, and that’s just from fluid buildup in the ears. You have oral surgeons who will also do the jaw surgery when they're a little bit older. You also have a speech pathologist. That is probably one of the most critical personnel in the multidisciplinary approach. From there, they can diagnose any sort of speech disorders, stratify whether it’s simply a physical issue with speech or mental or if it’s both. Obviously institute the therapy required to make sure that their speech is doing fine.
Pediatric dentistry is often involved because there can be anomalies of the teeth, that’s not uncommon. Then orthodontists are also involved to make sure that the teeth start getting lined up, especially when we look towards bone grafting to get the adult teeth in place. That’s pretty important. On the more social side, you definitely need a social worker. There’s a lot of visits. A nurse coordinator is integral because there is a lot of appointments to make and it’s very difficult sometimes to figure out where to go and just to kind of maneuver your way through the healthcare system.
Then nutrition. Nutrition is a big key, especially in the beginning. They work closely with speech pathologists to make sure that they're feeding well. As you can tell, it’s a whole host of personnel and healthcare specialists in order to bring them the best care.
Host: Well it certainly is multidisciplinary Dr. Liau. So now let’s talk a little bit about plagiocephaly. It’s a little bit more common even than cleft lip and palate. What is it and why does it happen?
Dr. Liau: Yeah, absolutely. So, I would say probably a good third of the kids I see in my clinic are kids with what we call plagiocephaly or deformational plagiocephaly. Luckily, it’s not surgical. That’s one thing I want to emphasize right off the bat. However, it can be sort of a problem.
Usually what it is… Basically what we’re talking about is when you see a child, their forehead is very asymmetric. One side juts out more. The back of the head is quite flat, and oftentimes at an angle. The ears, like I mentioned before, jut out in a different way. Also, sometimes the jaw can be smaller on the effected side. Basically, what’s happening is that while the child is developing in the belly, their neck is usually tucked in a certain way. When they come out, they are sort of fixed in that position. If it’s very severe, it’s basically torticollis, which you basically have a neck muscle spasm. The overwhelming majority of kids sort of work out of it.
However, in this country, we’re a back to sleep nation. Subsequent because of that, the skull is quite flexible at that age. We put them on their back to sleep. So, their neck is tilted a certain way, they're a back to sleep child, so they flatten out in that area. It’s oftentimes not symmetric. The skull does what it’s designed to do, which is to sort of accommodate pressure and allow the brain to grow. As a result, it grows asymmetrically or, as I mentioned before, the forehead is more forward on one side. The ears are a little bit off. As a result, I often see a lot of these kids coming in for this sort of deformity.
The treatment is pretty straightforward though. As long as you get them off the back of the head, in that region, the head will round out. You see the biggest difference around four to six months of age, up to eight months of age. The reason is because at that time, kids have great neck control. They’re looking around. You come in in the morning to the crib and they're on their face, even though you put them on their back. So, they're wiggling, they're looking around a lot more. Belly time obviously helps because it gets them used to being on their belly, off the back of the head. As a result, it alleviates that pressure and subsequently allows them to grow more in a round fashion.
Host: So, it goes away.
Dr. Liau: It can go away. The trick is the timing. For kids, and there are some kids for whatever reason… For example, a more severe case would be a child with cerebral palsy, you just cannot get them off their back or their neck straightened out enough. Unfortunately, in those cases, we do a shaping helmet. How that works is I send them to a prosthetist and the prosthetist who specializes in this gets an image of the head. Then they make a helmet. The helmet doesn’t squish them down or anything like that, it doesn’t compress them. What it does do is it blocks the areas that are asymmetric. As a result, the head will continue to grow into those areas and round out.
The issue is that the time that the helmet is most effective is before one year of age. The brain actually slows down in velocity and the head growth circumference range around a year. So, if you're going to use a helmet, you harness that growth and the velocity of the head growth to round it out. So, if you put a helmet on after a year of age, it tends not to be as effective because the brain’s just not growing as quickly any more.
For me, and different practices vary, but for me if you shape a kid at six months of age and you shape the kid at eight months of age, at a years’ time they're both going to be the same. The number of kids that require therapy at eight months of age drops dramatically. So, it’s just that natural growing, looking around, getting off their back. Those two months in itself can make a huge difference.
Host: So interesting. Dr. Liau wrap it up for us. What you’d like the listeners to know about cleft lip and palate and plagiocephaly and why they should seek out a plastic surgeon and ask these questions if their child has one of these issues.
Dr. Liau: Absolutely. Cleft lip, cleft palate, in my mind, my practice, my experience, there’s no reason why your child should have any sort of disability or anything to hold them back in life from being a very happy, productive person in life. I think a lot of that comes from the special training that we received and the focus that we have and different perspective we have on cleft lip and cleft palate. So definitely if your child has a cleft lip/cleft palate, find a center, find someone who specializes in cleft lip and palate versus someone who more or less “dabbles” in it.
In regard to plagiocephaly, there are a lot of conditions that can mimic plagiocephaly. They're not very common, but that’s why you come to a pediatric plastic surgeon. They're going to be the ones that catch the uncommon one which can be very detrimental to your child.
Host: Thank you so much for joining us today Dr. Liau. And for sharing your expertise explaining these things because parents, we get so scared so easily about things. You’ve really given a lot of hope and cleared everything up so very well. Thank you so much again for joining us. This is UK Healthcast with the University of Kentucky Healthcare. For more information, you can go to ukhealthcare.uky.edu. That’s ukhealthcare.uky.edu. I'm Melanie Cole. Thanks so much for joining us today.
