Cleft Lip and Palate – PediaCast 174
- Cleft Lip and Palate
- Dr Richard Kirschner
Chief of Plastic and Reconstructive Surgery
Director of the Cleft Lip and Palate Center
- Casa Azul America
- Cleft Lip and Palate Center at Nationwide Children's
- Cleft Palate Foundation
- Cleft Palate Foundation Phone Line: 1-800-24-CLEFT
Announcer 1: This is PediaCast.
Announcer 2: Welcome to PediaCast, a pediatric podcast for parents. And now, direct from the campus of Nationwide Children's, here is your host, Dr. Mike!
Mike Patrick: Hello everyone and welcome to PediaCast. It is episode 174 for August 16th 2011. This is Dr. Mike coming to you from the Campus of Nationwide Children's. And we have another interview show for you, taking a break from our news that we did last week. And today I'm joined by Dr. Richard Kirschner here in the studio.
He is the Chief of Plastic and Reconstructive Surgery and the Director of the Cleft Lip and Palate Center here at Nationwide Children's. And that's what we're going to talk about today, cleft lip and cleft palate.
I want to remind you if there's a topic that you would like to talk about it's easy to get a hold of us just head on over to PediaCast.org and click on the contact link. You can also email firstname.lastname@example.org, and call the voice line at 347-404-KIDS, that's 347-404-5437. Basically just leave a voicemail in that case and we get the audios. So, if there's a question or comment you have we can get you on the show that way as well.
I just want to remind you that the information presented in every episode of PediaCast is for general education purposes only. We do not diagnose medical conditions or formulate treatment plans for specific individuals. So, if you have a concern about your child's health, make sure you call your doctor and arrange a face-to-face interview and hands-on physical examination.
He is a professor of Plastic Surgery at the Ohio State University College of Medicine and as co-editor of Comprehensive Cleft Palate, he is a nationally recognized authority on cleft lip and cleft palate.
Dr. Kirschner is also co-founder and Medical Director of Casa Azul America, a nonprofit foundation dedicated to the provision of free cleft palate surgery to underserved children in Central Mexico. So, welcome to PediaCast Dr. Kirschner.
Richard Kirschner: Thank you Mike.
Mike Patrick: Really appreciate you taking the time out of your very busy schedule to stop in and talk to us. So, Casa Azul America how does an American plastic surgeon get involved with the helping kids in Mexico.
Richard Kirschner: This is interesting story, I was asked to speak at a symposium down in Mexico City many many years ago. And at the completion of the symposium an orthodontist down in Monterey names Ricardo Ellisondo walked up to me and he said, "You know, I have a foundation that I put together down here in Monterey to provide free cleft and crania facial care to underprivileged children in the Monterey area."
And he was an orthodontist, he said, "You know we need surgeons to help do the surgery for Casa Azul," he said, "Would you be interested in joining the team of Mexican surgeons next month our next mission?" I said, "Well, I'd love to help you out Ricardo, but I already have some surgery scheduled back in the States next month.
But I'll do you one better, if you give me a year, I will assemble a team", at that time it was in the Children's Hospital in Philadelphia."I will assemble a team of doctors and nurses from Philadelphia and welcome down and take care of your kids." And so, it was almost exactly a year to the day later that we brought a team of 12 nurses, anesthesiologist, and surgeons down to Monterey, Mexico for our first Casa Azul mission. It became pretty clear to us though that this was an endeavor that require a fair amount of support.
And so, we returned to the States and my wife who is a nurse, she and I founded Casa Azul America which is the sister foundation of Casa Azul of Mexico. And the purpose of Casa Azul America is to raise funds to provide free cleft and cranial facial surgical care to the children of Monterey.
Mike Patrick: Great. When you go down there, how many surgeries do you typically perform during a trip?
Richard Kirschner: We typically perform a mission for a week, and during that week we operate for about three or four days. The numbers of procedures we do depends upon how many operating rooms we run based on how many surgeons we bring down. So, it's typically somewhere on the order of 15 to 30 surgeries with each mission.
Mike Patrick: And those are provided completely free to the families?
Richard Kirschner: Exactly.
Mike Patrick: Now, this sounds dangerous. We hear on the news of a whole police departments being gunned down. And do you ever run into the drug cartels and I mean, is that a fear?
Richard Kirschner: We try not to.
Richard Kirschner: When we first started working with Casa Azul our early missions, Monterey, Mexico was actually fairly peaceful place.
And now as you said, Mike it is fairly dangerous. And in fact we took the entire year of 2010 off from our missions in order to protect our teams. We really can't let that get in the way of doing good work down in Monterey. And so, we've noticed after taking 2010 off as there are large number of undeserved children that are just not getting taken care of. So, we will be returning this September and we'll just be careful.
Mike Patrick: Just kind of under the radar, right
Richard Kirschner: Exactly.
Mike Patrick: You don't drive in limousines and flashy clothes or anything.
Richard Kirschner: No. No. And you know, we travel to the Monterey Airport from Monterey Airport to our hotel, from the hotel to the hospital and that's it for the week until we fly home. So, we pretty much maintain a fairly low profile. We're on a pretty safe area as long as we're in the hospital.
Mike Patrick: Sounds like a great program and it's a kind of thing that you wish more people would be able to do to help folks out in the world.
Richard Kirschner: My wife and I say it's really the best thing as doctor-nurse that we. And it's remarkable because we go down there and the families are really so grateful. But what most people don't realize is we take the same team that we worked with here now in Columbus everyday doing cleft lip and palate care on American children, we take the same team down to Monterey, Mexico.
And there's really something transformational about going down there and just doing nothing, but purely taking care or patients. And so the families are very grateful, but what they don't realize is they give us far more than we give them.
Mike Patrick: So, when we brought PediaCast in Nationwide Children's I was excited and shared with the audience that this is an institution that really cares about kids. And so here's an example even reaching out across the border and doing that. All right, let's talk about cleft palate. If you could just sort of start by defining for our audience, what exact is cleft palate?
Richard Kirschner: OK. I suppose we just start by defining what the palate is. So the palate plain and simple is roof of the mouth and is made up of the heart palate which is the front part of the palate which is you can feel what your tongue is made up of primarily bone covered by mucus membrane.
And the back part of the palate which is the soft palate and there's muscle in the soft palate and that's really the business and that's the part of the palate that really does the work. The whole function of the palate is to separate the mouth from the nose during speech and during swallowing, so that when we eat or drink food or fluid doesn't come through our nose.
And when we speak, air doesn't come through our nose. So, the lip and the palate normally the lip and the palate developed during the first trimester, the first three months of pregnancy. And in the embryo the lip and the palate, the right and left halves formed separately. And during the course of development the right and left halves of the lip and right and left halves of the palate gradually come together or fused during tat first trimester.
So, a cleft lip or a cleft palate occurs when that process of fusion is somehow disrupted and the closure of the lift or the palate, or both is incomplete.
Mike Patrick: Now how common does that occur? Is this a common thing. Obviously you see it a lot because folks congregate.
Richard Kirschner: Right.
Mike Patrick: But you live down a small American town, what's the percentage rate?
Richard Kirschner: Well, that's actually one of my favorite questions because there's a common misconception that cleft lip and cleft palate is really a third world disease. And I think a lot of that has to do with the high profile of organizations like Casa Azul that provide so much care to children overseas. But the fact that maybe startling to many is that cleft lip and cleft palate is very common in the United States.
And in fact it's the most common birth defect next to congenital heart disease. So cleft lip and or cleft palate occurs in about 5,000 births per year in this country or one in 700 births.
Mike Patrick: Is there a difference between male and female ratio?
Richard Kirschner: There is. Cleft lip is more common in boys, and cleft palate without cleft lip is actually more common in girls.
Mike Patrick: And how about ethnic trends, do you see different ethnic groups with it more often?
Richard Kirschner: Yes. Because of the genetic nature of cleft lip and cleft palate we find that clefting is actually twice as common in Asian population, and half as common in African American populations as in Caucasians.
Mike Patrick: So, you've got a kid now that has a cleft palate or a cleft lip, how did that happened exactly? Is this something that's genetic or are there environmental factors, combinations? What exactly why does this happen?
Richard Kirschner: Well, the short answer is yes and we don't fully understand.
And so, there is a genetic component to clefting and the exact genetics of cleft lip and cleft palate had yet to be defined. There are some clefts that are associated with other abnormalities as a part of a syndrome. We also know that there are environmental factors that play a role in clefting and there are certain medications that can play a role as well as smoking.
Mike Patrick: Cigarette smoking is one of those factors that can?
Richard Kirschner: Yup. Cigarette smoking by a pregnant mother has been associated with an increased risk of clefting.
Mike Patrick: Now what set of symptoms and problems then does a cleft palate create for the baby?
Richard Kirschner: Well as I've mentioned the function of the palate is to separate the mouth and the nose during speech and during swallowing. So, the primary problems that we see early on in infancy with children with cleft palate is the difficulty with feeding. And fortunately there are special bottles and special nipples available for infants with cleft palate to assist them with feeding. Now later on clefting may influence the sound of the child's speech. And so, some children will require an additional operation to improve their speech and many children with cleft palate will require speech therapy.
Mike Patrick: And ear disorders as well? Ear problems?
Richard Kirschner: Yes. It's very very common for the eustachian tube to which is the drainage tube that allows the ear behind the eardrum to drain fluid so that fluid doesn't build behind the eardrum. Malfunction of eustachian tube is very very common in patients with cleft palates. So, many of these will build up fluid by in their eardrums and that can interfere with hearing and predisposed to ear infection if it's not treated properly.
Mike Patrick: And then of course there is the cosmetic issues as well and social issue especially with cleft lip.
Richard Kirschner: Exactly.
Mike Patrick: Now we talked about some of the disease processes you'd mentioned that can be associated with cleft palate. What are some of the specific examples of that or some other diseases where you see cleft palate as a part of that?
Richard Kirschner: Well, as I've mentioned cleft palate has actually been associated with more than 250 different syndromes.
Mike Patrick: We won't list them all up here.
Richard Kirschner: So, we can't list them all, but in some of these syndromes have other features such as heart disease or kidney disease. But two most common syndromes that we see associated with cleft palate are syndromes known as Stickler syndrome which is associated with abnormality in collagenic can lead to joint problems as well as nearsightedness.
And the second most common is 22q111.2 deletion syndrome wherein cleft palate can be associated with fairly severe hear defects as well as immunologist defects, and really abnormalities and virtues in every other organ system.
Mike Patrick: 22q is a big topic and I know we have a multi specialty clinic here in Nationwide Children's to see kids with that. So, we're going to have you come back at some point because we could do a whole another show just on 22q.
Richard Kirschner: Absolutely.
Mike Patrick: All right, so you have a kid a cleft lip or cleft palate, can you describe just sort of in general that surgical treatment that you do sort of procedures and timing. How exactly do you fix this?
Richard Kirschner: Sure. Well, if we have another few hours.
Mike Patrick: Yes.
But we can generally repair a clefted lip within the first several months of life. The timing of lip repair is not terribly critical but we like to get that done early because of the appearance related issues, and it's important to the parents. In terms of repair of the palate it is since the palate is so important for the development and the sound of normal speech.
There is something critical to the timing of the repair of a cleft palate we really want to get that palate restored to its normal form before the child develops a whole lot of speech. And so, we will repair the lip within the first three to six months of the life. We typically repair a cleft palate by about 12 months of age.
Now some patients if they have a cleft that involves both the lip and the palate, the gums will also be involved. And so that will be a cleft in the gums where the teeth will eventually develop. And that will require that we place additional bone in that area usually around the age of seven to nine.
Mike Patrick: Now, is this something with the palate that you do, is it one procedure or does this take multiple surgeries in order to finally get it closed?
Richard Kirschner: Typically for a cleft lip we'll repair it in one or sometimes two stages. Cleft palate repair is usually performed in one stage. And as I have mentioned before these children are followed very closely for this speech development and the sound of their speech after cleft palate repair. And approximately one in ten to two in 10 will require an additional procedure on the palate to achieve normal sounding speech.
Mike Patrick: Now, the surgical procedure to close the cleft lip and cleft palate is just one aspect of what these kids need from a medical standpoint. Talk a little bit about the multidisciplinary approach and what are some of the other specialties and like if a kid was coming to the cleft palate center here in Nationwide Children's who else would they see other than the plastic and reconstructive surgeon?
Richard Kirschner: Sure. So, from what we've already discussed Mike, you can understand that a cleft is more than just an appearance related issue. And so because of the involvement of the palate it can affect speech. We've already talked about its impact the potential impact on hearing and function of the middle ear.
And we talked about how clefts can also involve the gums so they can have a very significant impact on dental development as well. And so, there are number of medical professionals that need to function together as a team to coordinate the care of these patients and that includes the plastic surgeon, the oral surgeon, the ENT surgeon.
In addition to our specialized cleft lip palate nurses, our speech and language pathologist, our geneticist. In addition our children will see our pediatric dentist and our orthodontist. In addition to ENT they will also be followed by our team audiologist to check their hearing on an annual basis. Now that's just the medical issues.
Mike Patrick: Right.
Richard Kirschner: And so, the one thing that I think is very important for all of us as professionals that treat children with cleft lip and cleft palate to remember is, we're not simply treating a cleft, we're treating a child with a cleft. And that child is a part of a family, and as the child grows, that child is part of a growing social network.
So, I think that for families the most important tool in achieving the best outcomes that we can for their child is simply information.
And so, cleft lip and palate teams are really equip to provide a tremendous amount of educational materials to our families and in addition I wanted to mention that the Cleft Palate Foundation functions nationally to provide information to parents and so families of children with clefts can call the cleft palate foundation at 1-800-24-C-L-E-F-T, or log on to their website at www.cleftline.org to get the kind of information that they need to work as a part of their child's healthcare team to achieve the best outcomes.
Mike Patrick: Right.
Richard Kirschner: Now as the children grow then, there are other issues not related to the medical issues, but really related to what it is like to grow up with a facial difference. And so as children begin to socialize through school we do start to see some very real problems in some children with teasing and with bullying.
And for those children again having their family armed with a good amount of information, education materials by cleft lip and cleft palate as well as knowing that every American Cleft Palate Cranial Facial Association approved team in this country is equipped to provide these children with access psychologist and social workers to help them through the rough times when it comes to bullying and teasing.
Mike Patrick:Now you've given the link and the phone number, I'm sure there are some people out there right now scurrying to write that down. But we will put that information in the show notes over at pediacast.org, so if you folks listening just go over to pediacast.org click on the show notes for our episode 174, and we'll have the phone number and the link for you.
And you actually kind of jumped ahead and talked about sort of long term prognosis and what that looks like. But the psychological aspect of it is important and obviously speech and hearing as well. Would you expect a kid who has their cleft closed by say year of age.
Do they typically then have normal speech or is that I mean working with speech pathologist, are they able to attain normal speech?
Richard Kirschner: Right. Our goal for every child born in this country and abroad with a cleft lip and cleft palate is to achieve normal speech. Now that may require single operation the cleft palate repair at the year of age. As I had mentioned one in two out of 10 of those children will require a second operation to get the palate to function more effectively to keep air from coming through the nose during speech. And many children will require speech therapy as well. But at the end of the day, any child born with a cleft palate should achieve completely normal speech.
Mike Patrick: That's good news. So, the cleft lip and cleft palate center here at Nationwide Children's, you know we're here in Central Ohio, but we certainly see kids from all over the place.
Richard Kirschner: We do.
Mike Patrick: And parents do they need their doctor? Let's say you're a parent and you wanted be seen here we have a Ronald McDonald house and you know, have support.
So, if you don't have a specialist in your area or multi team approach in your area. Can parents just make an appointment on their own or do they need a referral from their primary doctor?
Richard Kirschner: Most parents just go ahead and make an appointment on their own by either calling the Cleft Lip and Palate Center here at Nationwide Children's, are you are working through our welcome center.
Mike Patrick: And we will put links in the show notes too to the center as well, so parents and doctors can get a hold of you. All right. We really appreciate you stopping by and talking to us about cleft lip and cleft palate. One thing with all of our guest here on PediaCast, it's kind of our goal to see families doing more things together and not just the kids in front of their screen and the parents in front of their screen but really interacting as families.
And so, one of the things that we're talking about right is board games. So if you can kind of remember back from your childhood or in adulthood now. What is your favorite board game?
Richard Kirschner: Backgammon I suppose.
Mike Patrick: Backgammon? You know I have never played that.
I think we need to have one of the kids put that on their Christmas list.
Richard Kirschner: So we could play sometime.
Mike Patrick: Yeah, that sounds great. All right. Well, thanks again to Dr. Richard Kirschner for stopping by and of course to Nationwide Children's for putting up with this here at PediaCast and listeners like you. iTunes reviews are helpful as our mentions in blogs and Facebook pages and tweets and make sure you tell your regular doctor about PediaCast so they can spread the word.
Again of you want to get a hold of us just go to pediacast.org you can click on the contact link or email pediacast.gmail.com or call the voice line at 347-404-KIDS that's 347-404-K-I-D-S. So, once again thanks for stopping by really appreciate it. And until next time this is Dr. Mike and Dr. Kirschner saying stay safe, stay healthy, and stay involved with your kids.
So long everybody.
Announcer 2: This program is a production of Nationwide Children's. Thanks for listening! We'll see you next time on PediaCast.