Transcribed by Erica Manion Craniofacial Biology Lecture 5 Prenatal Craniofacial Development I and II by Dr. Wishe Slide set: 2014+FACIAL+DEVELOPMENT+1+2-23+post.

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[Slide 16] L FIG. 16.16 FACIAL DEFECTS 11TH ED. Dr. Wishe: Ok, we might as well continue. So this defect that you see on the picture of the child. Its a very uneven type of defect. And we can see a little bit better. What I did was I blew up sets of two. [Slide 17] L FIG. 16.16 TREACHER COLLINS SYNDROME & ROBIN SEQUENCE 11TH ED. Doesnt help us anymore, but [Slide 18] L FIG. 16.16 DIGEORGE ANOMALY 7 HEMIFACIAL MICROSOMIA 11TH ED. You can see, its complete malformation. And this usually affects one side of the face, although it can effect two as well. [Slide 19] N 1-87 MANDIBULOFACIAL DYOSTOSIS Now heres a picture of somebody who has Treacher Collins Syndrome, alias mandibulofacial dyostosis. And as you look at his face, you can see that the palpebral fissures slant down. You can see little notching of the ear. Now hes got pretty long hair and that covers up his defect in the ear itself. And it looks something funny in terms of the chin. And as you look at a slightly lateral view, you cant see the defect anymore because of the hair but what you do see is that the maxilla and the mandible are not quite in full occlusion. Theyre not bad. The severity of the defect varies from one person to another. Mental retardation is generally not associated with this particular syndrome whereas others it can be. [Slide 20] L FIG. 17.17 TONGUE DEVELOPMENT 12TH ED. Tongue development. The tongue develops from all four branchial arches. From the 1st arch you get two lateral lingual swellings and a small central component called the tuberculum impar. These lateral swellings actually wind up extending up and growing. Now sometimes the two tubercles dont meet. Heres where they do meet and youve got a normal looking tongue. If they dont meet, the tongue is split into two components. Thats called a bifid tongue. If any of you have been brave enough to look a snake in the mouth thats the way the tongue of a snake looks. And the expression He speaks with forked tongue, is a take off of this. What that means, which most of you probably dont know, is 1

the person is saying one thing, but doing something else. So hes not telling you the truth. Anyway. This is all derived from 1st arch. Looking at diagram A you see blue, yellow, and green. Arch 2, 3, and 4. But when you look at B, what happened to the blue? It disappeared. So a lot of folks say that the root of tongue only comes from arches 3 and 4. But what the blue part, arch 2 contributes to, is the formation of the chorda tympani. So if the nerve develops off of arch 2, the root of the tongue is involved with arch 2. So therefore the root of the as far as Im concerned is derived from arches 2, 3, and 4. And arch 1 gives rise to the body of the tongue which is really 2.3 of the tongue. And on the dorsum of tongue where you get your various papilla. Well talk about that later on. And the root of tongue basically has your lingual tonsil. And that lingual tonsil is the last one that could possibly become infected. Because everything is exposed to your saliva flushing out the area. [Slide 21] N FIG 1-19 ANKYLOGLOSSIA Here we have a senario where the tongue remains attached to the floor of the oral cavity. Its called ankyloglossia. Glossia tongue. Ankyl, ankylosis. Its attached to something. And this is an easy scenario to correct. Snip there, snip there, stop the bleeding, and thats a fairly simple procedure. But if tongue was attached to floor of the oral cavity along the ventral surface, the complete ventral surface, thats a more intensive type of oral surgery because you now have to separate the tongue from its attachment point. [Slide 22] N FIG 1-18 MACROGLOSSIA We have conditions like macroglossia. Not only is tongue enlarged, so is the face and the rest of the body. This could occur because of a pituitary tumor. Youve seen individuals like these. I dont know if hes still alive. The guy that played Jaws in the James Bond movies. Everything about him was gigantic. There used to be a wrestler, Andre the Giant. They all had pituitary type tumors [Slide 23] N FIG 1-16 MICROGLOSSIA Heres the opposite. Microglossia. Tongue is small. Not only is the tongue small, but notice the position of the teeth, the way they are situated. Means the whole oral cavity is small. [Slide 24] N FIG 1-17 MICROGLOSSIA And heres another situation of your microglossia. And again youll notice the teeth are not lined up properly. Theres a limited amount of space. The tongue itself is unusually small. [Slide 25] L FIG. 17.18 THYROID GLAND DEVELOPMENT 12TH ED. What we are looking at here is development of the thyroid gland. Heres where you would have the anterior 2/3 of the tongue, alias the body of the tongue. And this would be the root of the tongue, the posterior 1/3. In between the two areas theres an opening called foramen cecum. By the way, the ant 2/3, the body of the tongue, is covered by epithelium 2

derived from ectoderm whereas the root of the tongue is covered by epithelium derived from endoderm. And these endodermal cells begin to invaginate and push in to the underlying tissue through the foramen cecum, and it almost resembles blowing up a balloon. Theres the thyroid bud, thats your thyroglossal duct. Heres another view. Showing you the migration of the endodermal cells. This is your thyroglossal duct and eventually heres the thyroid gland by the thyroid cartilage. So a lot of embryological development involves migration of one type of tissue or another. [Slide 26] L FIG. 17.19 THYROGLOSSAL CYSTS 12TH ED. Any time you have movement of tissue you can get some sort of defect. And what we see here are four circles and they represent potential thyroglossal cysts. As the cells are moving down to their final location, get huge dribbling of cells being dropped off in different areas. There used to be an advertisement on TV, bush hair-cut, thick rimmed glasses, black frame, walking across the kitchen floor with a slice of chocolate cake. He probably consumed only 50% of the cake, the rest of the cake was on the floor. Thats what I mean by dribbling. The cake represent the cells that are migrating, and these cysts represent pieces or remnants that have been left behind. They could be cysts, or they can develop fistulas as well. [Slide 27] L FIG. 17.19 THYROGLOSSAL CYST 12TH ED. So here we have a case of a cyst, smack in the midline of the neck. Thats where the endodermal cells are migrating through foramen cecum. Whereas the lateral cervical cyst is located over here by the sternocleidomastoid muscle. [Slide 28] A FIG. 4-17 THYROGLOSSAL FISTULA 3RD ED. This situation is not only a cyst but a fistula. And its hard to see where it actually opens up, but its going to be some place in this area [Slide 29] N FIG 1-20 LINGUAL THYROID This is a little different. This is development of the thyroid completely in the wrong place. It develops in the oral cavity. And the thyroids necessary gland for development.. I dont know really how they correct this type of situation unless they have some sort of technique where they make an incision and push the thyroid down a little bit. I really have no idea. But you can see with this where it would inhibit food swallowing and possibly oxygen/CO2 exchange. Slide set: 2014+FACIAL+DEVELOPMENT++2++2+23+post.ppt [Slide 1] Facial Development 2 Ive got to wire up these seats with electrical stimulators and when I show a picture I hit another button that causes you to show life! Ok now we are going into facial development. 3

[Slide 2] L FIG. 17.21 FACE DEVELOPMENT 12TH ED. So these two pictures you saw before. Lateral view of the arches (Figure A). This is a frontal view (Figure B). And what we see again is this frontonasal prominence. This represents your maxillary prominence or process, and here are the two mandibular processes or prominences, or as the book calls it, mandibular arch. And heres the future stomodeum, or oral cavity. Were going to turn our attention to this area in here where you see arrow pointing to the nasal placode (in Figure B) and there should be on the other side as well. [Slide 3] L FIG. 17.22 FACE DEVELOPMENT WEEK 5 & 6 12TH ED. And now we can look at this picture. So you had thickenings, a placode, on each side developing on the frontonasal prominence. And this represents the nasal pit, future opening of the nose to the outside. On each side of the pit you have a blue and yellow component. The yellow component represents the medial nasal process, the blue component, the lateral nasal process. Here you have your two maxillary processes. The primitive stomodeum is wide open. If we go a short time later, a week later. If you were to measure this space and compare it to this space, this is shorter (figure B). That means these two regions being pushed closer together. Theyre being pushed medially. If you look at the maxillary process in Diagram A and Diagram B, you can see the maxillary process is getting larger and larger and causing these two areas where the nasal placode was located to move closer to each other. Heres the eye by the way, and the eye actually develops in lateral part of face. Once these areas move close to each other, the eyes will then shift to the medial position. [Slide 4] L FIG. 17.23 FACE DEVELOPMENT WEEK 7 & 10 12TH ED. So the next picture shows you the maxillary processes at their maximal growth. There is no separation of this whole apparatus by the frontonasal prominence anymore. What has happened is the two medial nasal processes have joined together. Fusing and merging. Two terms that require explanation. Here we have two processes. Merging is when you have more mesenchyme cells being released and pushing this groove, making it smaller and smaller. So a later picture could be something like that. These two areas merge together. And thats what happens between the maxillary and mandibular processes. This is what happens to narow down the size of the opening of the stomodeum. Then we have the word fusion. Fusion works a little differently. Here are 2 processes. Mesenchyme cells keep developing. These two processes wind up being pushed closer together. Finally you have the two processes fusing. That means the epithelium covering those two processes disintegrate. And the mesenchyme can now flow back and forth, and this is the final thing. This is fusion. Where do you have fusion? Joining of the palatal 4

shelves initially. Fusion occurs between the lateral nasal process, thats the blue, and the maxillary process. Also occurs between medial nasal process and maxillary process. The end result is the same, its just how it goes about doing it. Ok. So when two medial nasal processes merge together, you get this little part here, labeled philtrum. We are going to change that, and just refer to it for the time being as the intermaxillary segment. Well come back to that momentarily. [Slide 5] A 4.1 HUMAN EMBRYO WEEK 4 3RD ED. This is just another set of pictures showing us the same thing. Frontonasal prominence, the maxillary and mandibular prominences, and the hyoid arch. And theres of course the stomodeum. [Slide 6] A 4.2 HUMAN EMBRYO WEEK 5 3RD ED. Here we have maxillary processes getting larger, pushing these two nasal areas closer together. By the way if you look at the blue and yellow golden color, you see little tiny segments. Thats early formation of the ear called auricular hillocks. Well go over that when we discuss ear development. The space in between is your external auditory meatus which came from pharyngeal cleft or groove 1. So the external ear really develops from arch 1 and 2. [Slide 7] A 4.3 HUMAN EMBRYO WEEK 6 3RD ED. You can see two components of nose getting closer and closer together, the eyes are moving more medially, maxillary process is getting larger and larger. [Slide 8] A 4.6 HUMAN EMBRYO WEEK 7 3RD ED. Here we have maximal growth and joining of processes. Theres your intermaxillary segment which came from the two mesial nasal processes coming together. And so fusion occurred here, fusion occurs here. Now some place on one of these diagrams Ah. [Back to slide 3] I missed showing you this. See this structure called nasal lacrimal groove? That actually exists between the lateral nasal process and the maxillary process. And it extends from medial part of eye down to the upper lip. That fuses together but then what happens is that you have a solid chord of epithelial cells detaching itself from this area and technically speaking, new ectoderm should form to reseal up the area. Sometimes it doesnt, and that leads to the formation of oblique cleft which goes from the middle medial portion of the eye all the way down to the upper lip. [Back to slide 8] I like to title this picture only a face only a mother can love. and following suit, now we have this (now pic of dog). In case youre having trouble detecting it, heres the tongue, and the ears, and the rest is your guess. Somewhere in here the eyes are located. Anyway, moving right along. 5

[Slide 9] L FIG. 17.24 INTERMAXILLARY SEGMENT 12TH ED. Now we are back to intermaxillary segment. Thats this, formed by the two medial nasal processes. Theres your two maxillary processes, theres where you had the fusion. The intermaxillary segment forms the following items. One is the philtrum of the lip. And when you look at yourself in the mirror or look at your neighbor you see this little indentation. That comes from the intermaxillary segment. The four incisors, two centrals, two laterals, forms from the intermaxillary segment, as well as the jaw component. And finally you have a little triangular portion here known as the primary palate. [Slide 10] L FIG. 17.24 INTERMAXILLARY SEGMENT 12TH ED. Gone back to the nasal pit. We have this area, and cells are pushing in, just with any placode, and they push further and further in. And they are giving rise to a primitive nasal passage. Meanwhile we also have a primitive oral passageway. This particular area which separates oral cavity from the nasal cavity is known as your oronasal membrane. And that begins to break down. We come to Diagram C, it is no longer a blockage between oral and nasal cavities. This looks like, this little primary palate, is just hanging in the breeze. Its not. Theres the medial nasal process, and theres the lateral nasal process so its being held in position. And finally we get to the formation of the secondary palate, which well do momentarily. And now youve created a big nasal cavity and nasopharynx. Theres your oral cavity. The oropharynx and the nasopharynx join up and become one tube. And so where we started with the nasal pit, thats the opening to the outside, your external nares. And where you see this part called the definitive choana, thats your internal passageway leading into the oral cavity, the oropharynx. [Slide 11] L FIG. 17.25 PALATE FORMATION 1 WEEK 6.5 12TH ED. Now for the secondary palate. Coming off the maxillary processes, you get two more processes developing. Thats these two bulges, and theyre known as your lateral palatine shelves or lateral palatine processes. Now one thing you notice which is kind of strange is that this is the tongue, and this is the nasal cavity. So literally, the tongue is sitting in the nasal cavity. As such, these lateral palatine shelves will grow, but they only can grow down along the side of the tongue. Theyre supposed to be up here. So what must happen is the mandible must lower itself, pulling the tongue out of the nasal cavity. Heres a different view. Shows you the two shelves. And what youre seeing in this part is really the nasal septum and here in gold color is your primary palate which is derived from the intermaxillary segment. [Slide 12] L FIG. 17.26 PALATE FORMATION 2 WEEK 7.5 12TH ED. Diagram A, the mandible and tongue have dropped. Now the two palatine shelves or processes elevate and rotate up. And they are now opposing each other. Now they can

grow together and join. And heres the other view. What happens is the epithelium of one merges with epithelium of the other, and the epithelium disappears. [Slide 13] L FIG. 17.27 PALATE FORMATION 3 WEEK 10 12TH ED. And now you see the two palatine shelves have fused together. But theres another fusion that takes place, and thats between the two shelves and the nasal septum. And wherever you have these three items fused together, youre going to have the hard palate. If you look at your nose, the inside of the nose, you dont find a straight passageway like this. Instead, the passageway angles up. So eventually, the nasal septum is from the upper part of the nose, completely separate from the two palatine shelves. So when the two palatal shelves fuse together by themselves, thats where you have the soft palate. So lets just say this represents the soft palate leading into the uvula. So instead of having bone present, youre going to have glandular material present. And heres your junction point between your primary and secondary palate, your incisive foramen. [Slide 14] L FIG. 16.28 PALATAL CLEFTS 11TH ED. Now up to the point of what can go wrong. Heres a normal picture (Figure A). Whatever you see in blue came from the intermaxillary segment. Whatever you see in red, reddishbrown came from the lateral palatine shelves. Diagram B, if you look right over here, you see theres a separation between the intermaxillary segment and the maxillary process. By the way, the intermaxillary segment plus the two maxillary processes gives you your maxillary or upper lip. But in this diagram there seems to be a separation. Thats a cleft, an anterior cleft. From what we see, its going through the lip and thats it. It occurs on one side. So these clefts can be unilateral or bilateral. They can be incomplete or complete. Thats incomplete cleft. Diagram C, now youll notice the cleft here not only goes through lip, but is separating the primary from the secondary palate. This is considered to be a complete unilateral cleft. If we had an actual picture, you would see that the cleft is also separating the jaw components. Diagram D, here we have a double whammy. A bilateral cleft lip, complete. And so you have the area of philtrum, sort of if you look at what Im doing on me, flopping in the breeze but its attached. Next to last picture E, is a cleft palate. Cleft palate could just be the hard palate, could be the soft palate, anything is possible. Diagram F: And finally we have a unilateral complete cleft lip plus a complete cleft palate. So you never know who shows up in your office and with what. [Slide 15] L FIG. 16.29 FACIAL CLEFTS 11TH ED. Pictures of children. With this condition (Figure A), this is a little notching of the lip but it still leads to certain distortion. Maybe the child as he/she develops it will fill in and look better.

Here (Figure B) we have something going on bilateral. And its really hard to tell these two clefts are complete. Could just be a bilateral incomplete cleft lip. However when we look at picture C, not only is the cleft through the lip, its also gone through the jaw. And theres part of the jaw component. Figure D: 2 palatine shelves never fused together. Heres the nasal septum. Figure E: And heres this not too pretty picture of an oblique facial cleft. This is where you develop the nasolacrimal groove. Thats why I went back to the picture to show you that. So you saw a chord of cells detaches, falls inside, become the nasolacrimal duct. Then the ectoderm should have resealed itself up, but it didnt. So you got this nasty looking cleft from the medial part of the eye all the way down to the lip. Last picture (Figure F) shows us, the mouth is sort of triangular in shape. This is like a midline cleft but what happens is you dont have proper amount of mesenchyme in here. So as a result your mesoderm doesnt develop to the proper amount. And therefore youre going to have defective nose, defective brain, everything thats in that pathway. [Slide 16] A 4.18 UNILATERAL CLEFT LIP 3RD ED. Heres your unilateral cleft lip. And youll notice even with something like this which is more of a minor problem, the nose does become a little bit distorted. [Slide 17] LA FIG 8.3 INCOMPLETE CLEFT LIP This is also incomplete cleft lip. Youve got the lip separated, but theres the jaw. Its not separated. So youre going to get varying degrees of clefts, from clefts barely noticeable to something like this. [Slide 18] Image Or something like that. Definitely through the lip. Theres the jaw, you can see two teeth sticking out. Im just going to assume this is incomplete and the maxilla is intact. [Slide 19] Image Here we have another patient. You can see the tooth. And it sort of looks like there might be a cleft right over here (underneath the patients right nares). This could be a potential complete cleft. [Slide 20] LA FIG 8.4 BILATERAL INCOMPLETE CLEFT LIP Now the only thing different between this and earlier picture is that youre getting a bilateral incomplete cleft lip. One, two. But theres the jaw, intact. And you can see some teeth. Also notice this nose isnt distorted as weve seen in other pictures. Now all these 8

types of cleft lips are correctible. You dont want to walk around like this. So you have to fill in this area, possibly with tissue from some other parts of body so the lip goes completely across. [Slide 21] LA FIG 4.19 COMPLETE UNILATERAL CLEFT 3RD ED. This should be no doubt in your mind that this is a complete cleft. Theres the lip, theres the jaw which is separated from the other part of the jaw, and theres the tongue. So you see right into the oral cavity and of course the distortion is humongous, of the nose. [Slide 22] Image Another scenario of the same thing, and youre seeing the tongue back here. [Slide 23] L FIG 16.29 BILATERAL CLEFTS IN UPPER LIP 10TH ED. Through special photographic applications, this is meant to show you a bilateral cleft lip in the upper lip Hard to see but if you look here and here you can see little indentations. Those are the clefts. [Slide 24] Image Another patient where the jaw component is intact. The teeth are fine. Theres just a matter of bilateral cleft lip. [Slide 25] N FIG 1-4 BILATERAL COMPLETE CLEFT LIP Now we have a bilateral complete cleft lip on both sides. You have this flap of tissue, which by the way, is really the intermaxillary segment. And its hanging on to the medial nasal process. Look at what Im doing now, getting this piece of tissue right over here. [Slide 26] Image Another bilateral, and youll notice the two central incisors, the lateral incisors should be to right and left. [Slide 27] N FIG. 1-8 DOUBLE LIP This I found which I thought was kind of funny and cute. Case where this particular person developed a double lip. I cant tell you what caused it, but maybe the precursor cells for the formation of the lip, something went wrong and just encouraged the development of a secondary or double lip. [Slide 28] N FIG. 1.5 CLEFT PALATE Cleft palate, nasal septum 9

[Slide 29] N FIG. 1.2 CLEFT PALATE This is also cleft palate, but I want you to take note of the dentition. This is pretty crumby dentition here. Nothing but amalgams. The way these teeth look, they are going to fall apart, no time flat. So the cleft palate could be somehow linked to development or under development of your dentition, so you can have more than one thing happening at the same time. [Slide 30] N FIG. 1-6 BIFID UVULA This is good if it occurs around Valentines day. Thats a bilateral cleft of the uvula. A couple years back I showed this to dental hygiene class. One of the young ladies came up and told me afterwards and said she had this. And what do you think the question was that she asked me? I guess you dont have a wild imagination at this point in time. She was worried that the cleft uvula which looked like this would affect her romantic and sex life. And thats the question she asked me. So I kept a straight face and said no. What I should have said to her, that might mean that youre going to have a very romantic relationship! But I never did. Anyway. [Slide 31] N FIG. 1-3 BIFID UVULA Theres another cleft uvula. Bifid. So anything is possible. [Slide 32] A FIG. 4-23 EXAMPLES OF CLEFT LIP AND PALATE 3RD ED. And this one summarizes all the various clefts. [Slide 33] A FIG. 4-4 COMPLETE UNILATERAL CLEFT LIP AND PALATE 3RD ED Theres another complete cleft lip and palate. Theres the cleft palate, and this is going through the whole jaw. So the degree of all these clefts depends. You can have almost nothing to a major deal. [Slide 34] L FIG. 17.30 NORMAL MIDLINE CLEFT 12TH ED. And this is your midline cleft that we showed you before. Thats where you are going to have a deficit of mesoderm. And you notice the hair, the head, is all kinda funky like. [Slide 35] A FIG. 4-21 MIDLINE CLEFT 3RD ED. And theres the midline cleft close up. The nostrils are to close together. The oral cavity, the lips are triangular shaped. And this goes all the way up to the brain, so these individuals usually have mental problems.

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[Slide 36] A FIG. 4-22 MIDLINE CLEFT OF THE MANIBLE 3RD ED. And you could get a midline cleft of mandible. This doesnt necessarily imply mental problems, but you have to surgically correct this. Probably put in some bone grafts. And its about time for this. See you on Wednesday, to be continued. And good luck tomorrow. Its only organ systems. Next week its probably both. Or maybe not organ systems next week. Im confused, I dont remember anymore.

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