Transcribed by David Landsman Craniofacial biology 7 - CCP1 Craniofacial Defects, Dr.

Lai

03/26/2014

Slide 1 Title slide Dr. Yon Lai - We are gonna talk about cleft lip and palate and other craniofacial deformities. I used to work in NYU medical center when I was a student here I was a research assistant, then when I graduated I continued as a fellow, so Ive been there for 6.5 years. Developped many new techniques over there including new impressions on new born babies. The patients that start there are quite young, from 6 years old to teenage, a lot with craniofacial deformities. If you are interested in that area you should contact us, and you should contact the medical center, Dr. Graysons (sp?), Dr. Macarthy (sp?), Dr. Wishe, the list just keeps going. Slide 2 History of clefts Dr. Yon Lai - The history of clefts, the first evidence of clefts is from 5000 years ago. They have evidence on Egyptian mummies, also from Colombia there is a ceramic statue of a king with a cleft. An African mask showing cleft lip and palate but the first treatment was carried out by Chinese physicians. Slide 3 Unilateral Total Cleft Lip and Palate Dr. Yon Lai - This is a picture of a unilateral cleft baby, its on the left side and its a through and through cleft, including lips, hard palate, soft palate, all the way down to the uvula. Slide 4 Bilateral Cleft Lip and Palate Dr. Yon Lai - This is an example of a bilateral cleft. The premaxilla, which is the front part of the maxilla, is totally dissociated with the rest of the uvular arch. The only point that it is connected to is the nasal septum. So it is quite mobile, so if you hold the premaxilla, you can move it quite a bit. How old is the baby until you can wait to do surgery? What's the rule of thumb? Just remember this: 10 weeks old, 10 pounds of weight and 10 units of hemoglobin. If you verify all three you can do the so called elective surgery. Of course if it is life threatening you may have to start even before the baby is born they do surgery. For the cleft lip and palate you can wait. Why are you waiting for the baby to grow heavier? As an orthodontist we can prepare for the surgeons to do a much better repair on these cleft lips and palate. And I will go over in detail. Slide 5 - Statistic Dr. Yon Lai - I will point out which slides are important to you. Which slides are relevant to your test and also for the board exam, they used to ask these questions somehow, so I will point it out to you along the line. Statistically, the site of the cleft and side of the cleft. Slide 6 Developmental cause of clefts This is Dr. Wishes territory. The cause of the cleft. If you look at the transverse slides of the embryo, I don't know if you can see it, median nasal process, left and 1

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right maxillary processes, and tongue. What is the lower right hand corner? Anyone want to take a wild guess? They had the histology already? It is macrocartilage. The cleft can form as young as the baby is 7-8-9-weeks old, 10 weeks, 11 weeks, 12 weeks. So around 7 - 12 weeks. Can form when right and left max processes don't meet each other or they meet and separate and meet again. That is how you have posture cleft with a fistular on the palate. This is an important slide. You have to remember, failure of mesoderm to migrate from the lateral max processes into the median nasal process, they ask this a lot of times on the board exam. Lack of mesoderm in any of these processes can form a cleft. Slide 7 Cleft of Uvula This is an example of a cleft o fthe uvula. Slide 8 Cleft of Tongue Cleft of tongue. Slide 9 Rare forms of Clefts Cleft of the comissure of the mouth, this is like not very prominent on the left side, but there is a cleft. On the right side it is more prominent and goes all the way to the tragus, you remember the tragus line. You remember the tragus? Tragus is where the ear is. Slide 10 Median Clefts Median cleft, right down the middle, the cleft can be formed. In these patients the cleft can go all the way to the brain and there is leakage of cerebrospinal fluid, high mortality rate with these kinds of clefts. Mental retardation. Looks quite mild but this is the tip of the iceberg, actually, what you see outside. Slide 11 Mild form of median cleft This is a milder form of cleft, you see the depression here. So the cleft is not through and through, but you can tell by looking at the arch form here, it is so narrow and V shaped. The shape doesn't have a support from the bone in the middle so it collapsed. The functional cheek muscles and all the other structure in the mouth will collapse the arch. Slide 12 Do you see a cleft? Do you see a cleft on that slide? I dont. How can you detect there is a cleft on this patient? Palpation? Symmetry? Most of these cases are symmetrical, can't really tell asymmetry in these partially cleft patients. We call it submucous cleft, the cleft is under the soft tissue. What she said is right. You can palpate with the finger, instead of the ridge you can feel a cleft underneath. You can shine a flashlight through the nose and see the light going down. But the most obvious diagnosis on these patients is speech. Once they start talking, they have hypernasality. If you notice that pediatricians. Nobody goes to see dentists that young, by the way, unless they have visible clefts. Pediatricians should pick it up and refer it to us, dentists or pediatric dentists, then we can properly diagnose. Because a lot of times they will miss the 2

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diagnosis. So submucous cleft is a missing diagnosis often. There are speech problems. Slide 13 Submucous cleft Lack of tensor veli palatini, there's a muscle called tensor veli palatini and the function of that muscle is to contract the soft palate so that when the patient speaks it touches the posterior wall of the pharyngeal space. If you dont have that function you call it hypernasality, air escaping through the nose. Which is the opposite of the hyponasality. When do you hear the hyponasality? It is when you have a cold. So this is the opposite of when you have a cold. Too much air escaping through the nose. Slide 14 Submucous Cleft Sometimes you see a full soft palate there, but is hypomobile or not mobile. Soft tissue. So this soft tissue cannot reach the posterior pharyngeal wall and air escapes through the nose when they speak. Certain ways that we test these kinds of patients is ask them to say Popeye or Coca-Cola and they will say it like Popeye, Coca-cola (nasal sound) like air escaping through the nose and it is very obvious. Slide 15 - Etiology Etiology, mutant genes. Chromosome problems. Environmental teratogens. Usually it is multifactorial. Inheritance plays a big part there. If you have one parent and one child with cleft, most likely the subsequent sibling will have cleft. The agent that really damages is viral diseases, vitamin deficiency especially folic acid, B6, thalidomide, which is very powerful drug to treat certain types of diseases, Kaposi sarcoma. Suppress the HIV positive symptoms. In the late 50s and 60s that drug was prescribed to pregnant women with morning sickness so babies were born without limbs, craniofacial defects, all sorts of problems so they banned the drug after that. But in the 80s it came back because we needed it to treat certain conditions on AIDs patients. And they have to sign a consent form that they have to use at least two types of birth control methods before they can use it. So those are the examples of the teratogens that can cause the craniofacial defects. Slide 16 - No significance in cleft production These are effects that dont do anything in producing cleft. Slides 17, 18, 19 Problems with newborn cleft babies. Now Id like to present the problems that are encountered with newborn cleft baby. As a parent, as a mom, it is a big shock that the baby is born with a cleft. They have a problem with feeding. They cannot create a negative pressure in the mouth because the air is leaking, so they have to cut the nipple a bit larger and let it drip. Hold the baby at a certain angle. Slide 20 - Speech The whole team is there to train the family how to take care of these cleft babies. When the baby reaches 2.5 years old they learn to speak and we need to evaluate that speech by our speech pathologist. If its necessary the surgeon can perform the 3

Transcribed by David Landsman

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Pharyngeal flap surgery, and I'll go over it in detail. Just remember that how the speech is affected because soft palate cannot reach the posterior pharyngeal wall, just remember that because thats important. And we go over how to fix that. For the older patient we make obturator which is sort of like a denture with a speech bulb in the back. Slide 21 Congenital Palato-pharyngeal Incompetence So, CPI, congenital palato-pharyngeal incompetence. Very often you'll hear CPI, talking among the surgeons, orthodontists and the speech pathologists in a team. This is the exact phenomenon we describe, agenesis of soft palate, foreshortened soft palate or immobile soft palate. That can cause the functional position of the soft palate, preventing the contact with the posterior pharyngeal wall during the speech. Why is it so important that you need a sealed mouth to speak? For certain languages it is not very important, for Yiddish there is a lot of air escaping through the nose and these patients would be ok. But for English, almost every vowel and consonant requires the sealed mouth, except M where you need to let the air escape through the nose. But all the others, everything needs a sealed mouth, like P Q R S T, anything. So its very important to have a sealed mouth to learn to speak English, and Chinese and Spanish too. Slide 22 Diagnosis of CPI Simple way of diagnosing the CPI is to take the set film and while the patient is saying out loud, EEEEE, we call it E film. If you take the film and press a button while the patient is saying EEEE you'll see the difference. Here and here. Here it is closure of the posterior pharyngeal wall and this whole palate, here you see the white opening. Slide 23 Pharyngeal Flap Surgery Now how do we correct or improve the situation of this patient. You take a piece of the soft palate and take another piece from the posterior pharyngeal wall and suture them together, so instead of one big hole we create two small holes. So physically the hole becomes smaller. It is two fold, another benefit the patient gets is they can use the pharyngeal wall muscle to tuck (?) the hypomobile soft palate so it becomes mobile again. This method works on a younger age, it means before the patient finishes growing so they can retrain themselves to pronounce a lot of words. If the patient grows older this doesn't work anymore because it is very difficult to teach them to use that. Perfect example is new immigrants who speak spanish or chinese as first language. They come here as a late teen and adult, after you correct this, their second language, which is English, improves very much but their first language is very lousy. A lot of hyponasality, so that shows it is best when its done 2,3 years old, 4,5 years old, around that age. Slide 24, 25 - Obturators For the older patient, 30 - 40 years old, who came here without the benefit of that we made a speech ball. Called obturator attached to the partial denture type of

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thing, will partially block the big holse in the posterior region. Here's another example. Slide 26 Fan shaped rapid palate expander (RPE) Remember the slides that show a very narrow maxillary arch in that patient, a lot of cleft patients are like that because the lack of support in the palate so this is typically the arch form in the maxillary arch. As an orthodontist, we have to expand it so it will look like a normal U shape arch form before the surgeon can do anything. Before they do a bone graaft, soft tissue graft, we have to expand this for first them to gain access and receive proper amount of graft materials. And this appliance is called fan shape, will pivot around this groove and when you crank this groove it expands more in the anterior region than the posterior region. Slide 27, 28 Lubit Palatal Exerciser; Palate exerciser to improve speech Dr. Lubit is my predecessor. Hes been teaching and treating cleft patients for decades. He passed away 4-5 years ago. He invented this little device called a palatal exerciser. This little balloon is inserted into the soft palate an you pump to release the pressure, do that 20 min, 3x a day, and try to revitalize the muscle that isn't working properly. Just like physical therapists try to revitalize certain parts of the limbs. It has a limited success, this device. It should be used, whatever we can do non-invasively we try to do. If it doesnt work we can always put these patients through surgery. Slide 29, 30 Hearing; Myringotomy The next problem that these patients encounter is hearing. The cleft patients tensor veli palatini cant function properly so the Eustachian tube is a little tube that connects the inner ear to the oral cavity is blocked. You need muscle to contract, open and close it to clean up all the fluid there, if you dont have that function these babies get ear infections all the time. It could be serious. At the time of the pharyneal flap surgery, the plastic surgeon or EMT surgeon will insert a myringotomy tube to drain the fluid. It is a draining tube, very simple draining tube inserted into the ear drum. This is the size of it. Slide 31 - Mastication The next problem that these patients will encounter is chewing, mastication. We do the early orthodontics at 5-8 years of age, as soon as their primary teeth erupt, we can insert some palate winder (sp?), RPE, to establish the arch form. Alveolar graft, normally these patients require repair of the lips, they have to be treated for the cleft on the alveolar bone. Even though we expand the arch it tends to collapse easily because there is no support. As soon as you finish expanding the maxillary arch you have to put the bone graft in there. The most likely donor site is the left iliac crest, if you are right handed, take out some cortical bone, some spongy bone, from the area and they autograft and patch it up. If it doesnt stay you have to do it again. It's just like if you lose a tooth. For quite a while you get bone resorption, right. If you open the mouth of a 60 year old who lost the first molar, there is no ridge. No root or tooth to support this bone. Just like the astronaut in space, they do have to do 5

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exercise in the spacecraft otherwise they lose bone density. Osteoporosis is the biggest problem they encounter in a weightless environment. It is a very similar situation, the bone doesnt have a stimulus it will resorb. Just like your muscle, if you dont exercise you get a weak muscle. The bone needs gravity to maintain the mass. If you dont have a tooth, the natural bone or graft bone tends to resorb quite a lot. So long term retention is important to these patients. So lets recap. When the baby is born, the parent discovers a cleft. So what do you do? They bring it to your office. Lets say you are working in a hospital based environment where you have to work with a surgeon, orthodontist. You prepare the arch form for the surgeon to do surgery. At what time? The rule of 10. 10 pounds, 10 weeks, 10 units of hemoglobin. You gotta remember these! 6 months old, they do the surgery. Usually they do the lip surgery first, in the old school. In the new school they do lip surgery, the alveolar graft, and the suture of the fistula that you see in the mouth at the same time. Because surgeon techniques have improved so much you dont see scar. Thats what they were afraid in the old days, the scar tissue created of the surgeon would limit the growth of the palate. A lot of these patients develop class III crossbite because of the scar tissue on the palate. Then we treat them we they have primary teeth, second phase, expand again, bone graft again if it is necessary. When do you do the bone graft in the second time? Before the permanent tooth erupts into the cleft site. If any tooth that erupts into the cleft, that tooth is a gonner, you gotta take it out. A bone graft will never work with a tooth in the cleft. Remember that. Pleases remember this fact. When you do the bone graft? Before any tooth that erupts into the cleft site. Once its erupt, its gone, you have to take it out. Usually either lateral, canine or central incisor, one of those. When the patient reached the end of the growth, we do the orthognathic surgery. We move the upper jaw back, lower jaw forward. Maxilla, touch up surgery for lips and nose. All these procedures are really tiring to the parents, not only patients. A lot of them run out of insurance, they have a maximum of lifetime insurance and they have to pay out of pocket or borrow from the state. By the way a lot of these patients are under the handicapped program in the NY state. Any problem in the mouth that causes speech is a handicap and insurance and state has to pay. Slide 32 Cleft patient in mixed dentition anterior/posterior crossbite Anterior crossbite or posterior crossbite. Thats a very typical cleft patient that has been ignored in the early age. Slide 33 - Esthetics This patient from Costa rica. The mothers would put her in the closet when a guest would come to their home. It was shameful for them. After the surgery their life changes. Slide 34, 35 = Role of orthodontist in craniofacial team; Multi-disciplined team So the role of orthodontists in the team is we take records. We analyze the records, do the tracing, pre-surgical orthodontic preparation, mock surgery on the cast, fabrication of the splint, help surgeon get the best result, post-surgical care and 6

Transcribed by David Landsman

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retention. In a team you see surgeons, usually oral surgeon and plastic surgeon. The oral surgeon in this university is Dr. Robert Gleichman (sp?) and Dr. Caliss (sp?), those are the leaders of the team. Four year and six year program for oral surgery. Constantly have inter-relationship with our department, orthodontics, so usually Thursday is the best day to come to observe these craniofacial patients. Im there the whole day, usually start at 7:30 in the morning until 9:30, in the 8th floor seminar room we go over a lot of these craniofacial cases. Presentations, progress cases, we discuss. I find residents learn the most from that section because you get to see 3-5 cases in the morning. If you want you can attend those seminars to get a feel of how these dentists are taking care of these patients, not just the M.Ds. After 10 oclock we start seeing these craniofacial patients in the clinic, 6W. But older patients. The much younger patients are seen in a hospital based center, NYU medial center, in Bellview. Slide 36 Team work, good results In order to get the good results such as this symmetric nose. You mentioned about asymmetry. Asymmetry is frequently present on the unilateral cleft, not the median cleft. And a unilateral cleft such as this, either on the right side or the left side, can cause a growth asymmetry on the nose, lips and sometimes face. From here to here we cant just send the patient to the surgeon to suture the lips, wont achieve a symmetric nose like this. We have to go through many steps, within a few weeks time we can achieve this. And Im going to show you. Slide 37 Infant Orthopedic Its called infant orthopedic treatment. The early age, 1 10 weeks old we started these pre-surgical orthopedic splints. The objective is to align the cleft segments to minimize the bone graft, we reshape the alae base of the nose, elongation of the soft tissue and to minimize the skin graft. To achieve the excellent results from early age by doing GPP, gingival periostalplasty, in short GPP. Slide 38 Oral-nasal impression to make molding plate with nasal stent This is the impression technique that I developed when I was a resident. I used to play with Legos. So why not incorporate the lego pieces into oral and nasal impression. We cant do both at the same time, would choke the baby, can do one at a time. Attach the other piece and load a fresh load of cytricon (sp?) Polyvinal that you use to take the impressions on crown and bridges. And it worked. Slide 39 Adhesive/adhesive removal suture strip for skin After we got the impression for the models we dispose of the articulator. It shows babies nose lips, can fabricate the molding plate out of that. You know acrylic. We usually do it ourselves instead of sending it to the lab. These are the adhesives and surgical strip we use.

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Slide 40 A type of molding plate and tape Put on patient's cheeks and teach mom or dad to exercise, the soft tissue. Only have 6-7 weeks to do it. If you miss the opportunity, that's it! You aren't gonna have a symmetric correction. Slide 41, 42, 43 lip repair surgery. So the lip repair surgery, the most common one is a Z-plasty its called. Makes a Z pattern like this. Fortunately, the bone you can harvest from a lot of places but for the skin and soft tissue it is hard to get a donor site so stretching prior to the surgery is important. See the difference. So what we do is, in a loading plate, we build up a little spot that we keep adding to every week to make it taller and taller to prop that base of the nose, to stretch it. After 8-10 weeks the patient is put in surgery and the result is tremendous. I have to give credit to my teachers, DR. Bary Grason, Mccarthy, are all retired or retiring very soon. The new batch of surgeons are there. You hear that NYU doesn't do anything without the money, that's not true. Sometimes we do the charity cases. This is one of them. Slide 44 4 year old Philipino girl This 4 year old philipino girl was flown to New York, NYU paid for everything the hotel the airfair, the treatment. She has nasal and frontal bone defects. Orbital hypertelorism, that means very far apart. Again, if you don't see this defect on a nose, just this, this patient looks quite normal. But the underlying cleft is huge. Slide 45, 46 In the mouth it looks pretty normal. 4-5 year old with problems. You hear of milk bottle syndrome, parent keeps bottle in the mouth and you get all kinds of decay. Its pretty normal, dont see cleft in the palate. Slide 47, 48 But if you look at these orbits, they are very far apart. Slide 49 So I did a tracing, and we required the removal of a triangle piece of bone and collapsed these 2 halves, so the patient would have a normal face. Ostectomy of a wedge contains part of the frontal bone, cribiform plate, concha, part of the nasal spine and maxilla. While doing the surgery, found some lipoma also, so it was removed. Slide 50 Again the detail, pre-surgically you measure 43 mm and post surgically was 18 mm. That's between the medial orbital wall. And intermolar distance was actually expanded, because when you collapse the upper half of the face, the molars come further apart. That's why its 34 40 mm.

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Slide 51 construction of surgical splint On a cast, you take the model and split it accordingly. That's why the molars are getting wider. But the upper face is getting narrower. This is a splint that we fabricate. Slide 52 The surgery takes about 13 hours. 3 teams of surgery will come in. A neurosurgeon will do the transcranial flap. Will draw these holes on the frontal bone and sort of connect the dots and remove the frontal plate. Then they found a lipoma, removed the lipoma. [] And then, we went in. Dr. Gracin and I, insert a splint. Put in a maxilla, tied in. Like a template now. Two halves of the maxilla are being held by the splint that we put in. Then the plastic surgeon came in. Put the screws and plates in. So you see, a huge surgery but not too many screws and plates. So thats before and this is one week post op. OK. Future treatment modality and craniofacial anomalies That's one of the extreme cleft cases that I just presented. Most that you see in the clinic aren't that extreme. We manage them by putting braces, expand the palate, do an alveolar bone graft, maintain the arch form, maintain the bone, until the patient is 18,19 years old when we do some touch up surgery on the nose, lips. Patient is a college graduate, meets someone, gets a job, leads a normal life. What's her name? Naiomi, the model, tall black girl. She has a cleft. I first notice from her speech. I noticed form her hyponasality. It could be a submucosal cleft but it is there. It is interesting that you notice some of these celebrities, especially from their speech. The future of medicine and dentistry will be so different in 10 years from now. The textbook will be very much different 10 years from now, and its been changing, when I was here. And 25 years later, it is so much different. Fetal surgery in humans Fetal surgery was performed in a lot of animals. In humans it is performed in certain parts of the world but not United States yet. Fetal surgery is performed to save lives of the fetus, but not treat the cleft palate because thats a cosmetic feature. Patient who has hernia of the diaphragm, hernia backwards or upwards, so they dont develop lungs properly, these patients can die in a few weeks if they are born. So fetal surgery is done to insert a balloon, through the mother so they can push the diaphragm into the right place. Thats been done for at least 25 years by surgeons. But to repair the cleft thats not yet. Fetal wound healing process is very different. Fetal wound Healing In fetal wound healing it is very different, very rich amniotic fluid in growth factor, warm temperatures without exposing to the environment, rapid tissue turnover without the oxygen. Less differentiated cell, so there is more undifferentiated cells. They can take their time, heal the wound.

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Human genome mapping In the not so far future, human genome mapping was done 14 years ago. We can clone limitless supply of DNA from a sample. Prevention of Oral-facial cleft Any child bearing women should take this vitamin, 0.4 mg of Folic Acid, thats B6, which would reduce the cleft in other orofacial defects by 50%. The study was done, two big studies, one in California and other in Eastern Europe. Parent has history of cleft should take even more. Up to 10 mg of Folic acid a day. In the early 90s, I was attending this cleft palate conference in Atlanta, Georgia at the childrens hospital. In 1991 they started pushing the wonder bread to incorporate folic acid in the bread and cereal. And thats when it started. And now if you read the label on the boxes you see this vitamin in these products. Just like fluoridated water in the united states. Cost of Orofacial clefts The cost, its very costly, about 100,000 per patient. If you take these vitamins it is a few pennies so is a bang for the buck. Thank you very much, these are my kids. When they were born I checked their palates first.

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