Transcribed by Erica Manion Craniofacial Biology Lecture 8 Prenatal Craniofacial Development IV by Dr.

. Wishe Slide set: 2014 FACIAL DEVELOPMENT 3 3-20 post.ppt

3.27.14

[Slide 18] L FIG. 17.15 (18.15) SPINAL BIFIDA 11TH ED. Dr. Wishe: Good morning. So yesterday we had gotten to the point in our discussion of defects that can occur in terms of the brains in essence. Today we are going to continue that discussion and turn to the area of the spinal cord. Keep in mind that the neural tube gave rise to the whole central nervous system. In the head region the brain develops. Below the head you get the spinal cord forming and any defects that occur in the spinal cord are known as neural tube defects, and they can be moderate to severe. And these type of defects are generally referred to as spina bifida. Youll see the word on the screen, spina bifida occulta, and thats generally less of a defect than spina bifida cystica. So spina bifida occulta, essentially you have one vertebra being effected. And in this picture (Image A) youll see a vertebra with the transverse processes, and heres your vertebral processes, your arch. And youll notice that the two sides are not fused together. Therefore in this part of the region there is no bone protecting the spinal cord. About ten percent of the population has this condition and it usually effects only one vertebra. Homework assignment, of course you need two people to do the homework assignment, is just take your finger, run it down the vertebral column, youll find all of a sudden the finger goes in and it comes out, and it goes in because there is no bone over here protecting the spinal cord. And for some reason, oh by the way that blue indentation is called a dimple, sort of cute term. And in that particular area you get hairs developing. For whatever reason I have no idea. As I see people reaching behind them to find if they have it or not. But thats not a bad type of condition. Now you can have neural tube defects where your survival rate is really zip. (Image B) We saw this particular name (underlined Meningocele) in connection with the skull where essentially you had a little opening. By the way -cele means opening. And when you see the word or prefix Mening- you know its referring to the meninges covering the neural components. So in essence what youre seeing here, the red region, are really the meninges which are protruding from the opening. Going a little bit later on, next picture (Image C), meningomyelocele, its not only the meninges but the actual neural tissue, the spinal cord thats protruding through this opening. There are no ventricles associated with the spinal cord, but you still have cerebral spinal fluid associated with the spinal cord. And they dont seem to give that a separate term by inserting the word hydro which means fluid. Then there are two take offs of these neural tube defects which are much more serious than what we see in picture A. And I love the name, Rachischisis. If you recall what normally happens, is that you get two neural folds forming like this, the folds come together, join. And this region, which is your potential neural tube, seals up, and eventually you get a 1

circle in here representing that neural tube. Well in picture D, youre getting the beginning of the formation of two folds, but thats where it stops. So you dont get a fusion of the two folds, and you dont get a neural tube. In E, which has the same name, a single neural fold appears but not two. So again you wind up with the same condition. Absence of an actual neural tube. Whenever you listen to the news, watch TV, whatever, sooner or later youre going to come across the discussion of folic acid. And Dr. Lai mentioned that in his discussion of the CCP. Giving folic acid to pregnant women really helps reduce the percentage of neural tube defects. He was talking about it in terms of clefts of the face where he mentioned there is a 50% reduction in the clefts. But the same thing applies to the neural tube. Folic acid reduces these type of neural tube defects by at least, lets say, 33%. Certain vitamins besides folic acid also help, and on the same side you have to be careful. Something like vitamin A tends to promote the exact opposite effect and increases your neural tube defects. [Slide 19] L FIG. 18.16 SPINA BIFIDA 12TH ED. Here we have a typical example of your spina bifida. And you can see it right down here. Its hard to tell from this picture how many vertebra were involved. And I dont really see anything protruding from this area. So this is a little bit less severe neural tube defect than what we saw on the previous page, except for the fact that a single vertebra might not have the vertebral arches fusing together. [Slide 20] L FIG. 19.16 NEURAL TUBE DEFECTS 9TH ED. Now we are getting in to much more severe type of conditions. And if you look at B first, its a very large neural tube defect. So the spinal cord is wide open here. And by bringing the two edges of the skin together, thats not going to afford any protection at all. Now people sometimes have a part of the skull removed for some sort of surgery, what have you. And you need the brain protected by something, so some people walk around with these steel plates in their head and thats protecting the part of the exposed brain. What they do in this scenario Im not sure. They might have special gizmos where they do insert some sort of neural plate to cover and protect the underlying spinal cord. Now because you have a neural tube defect doesnt mean you must have defects in the brain. And heres another neural tube defect (Image A) but this one, its not that its wide open but the meninges and the spinal cord is actually pushing out. So that actually finishes our discussion of the nervous system Slide set: 2014 FACIAL DEVELOPMENT 4 2-23 post.ppt [Slide 1] FACIAL DEVELOPMENT 4 2014 And now we are going into a discussion of eye and ear development. [Slide 2] L FIG. 19.1 OPTIC VESICLE 11TH ED. 2

With regard to the eye I did point out previously with regard to facial development.. oh ok. Thats not what I want to do. Ah. Thats why. Ok, now I have my pointer. So the eye actually begins to form completely in a lateral position. And so this is the beginning of the eye (indicated the optic vesicle, Image A), and over here youre getting the formation of the ear (indicated the Otic vesicle, Image A), which is supposed to be in a lateral position to begin with. So coming off the diencephalon, and thats what youre seeing in this particular picture, the diencephalon (Image B). Youll find these two structures called optic grooves. The optic grooves enlarge and spread out and they form this structure known as an optic vesicle (Image C). The optic vesicles expand to the point that they touch the surface ectoderm. What you see in red is neural ectoderm and what you see in blue is actually the ectoderm associated with the formation of the skin. Once these two layers touch each other theres an inductive effect that takes place. So the presence of the optic vesicle against the surface ectoderm... You have an induction where the red stimulates the blue. And as a result youre going to get a thickening of the surface ectoderm. And this is the thickening (blue part of Image D). And with increased mitotic activity the thickening sort of pushes inward, and once you have a thickening of the ectoderm, we saw that happening with the formation of the nose. The nasal placode. So were going to have a lens placode and an otic placode. So this structure (indicating the lens placode of Image D) is going to invaginate inward, putting pressure on the optic vesicle. So if you compare diagram C to D, the shape of the optic vesicle is now changing, and its gone from a somewhat circular structure to something resembling a horseshoe. And this inside layer (indicating the Invaginating optic vesicle of Image D) will get pushed further inward. [Slide 3] L17.1 EYE DEVELOPMENT DAY 22 OLDER EDITION And this is just an older version of the same picture. But I put it on because it does show you an actual slice through, probably, a rodent head (Image D). And its also comparing this picture (Image E) to the overall longitudinal view. So again we have your forebrain, your midbrain (labeled M), and your hindbrain (labeled H). And as soon as you see an opening or a hole (white arrow), you know those are the cerebral ventricles, and there are two of them. And this would be the diencephalon from which these optic grooves really originate. [Slide 4] FIG 19.2 LENS VESICLE WEEK 6 WEEK 6.5 11TH ED. At first these pictures are a little confusing so Im going to draw an additional picture. Lets see how do I do this now. Not too well done but This now shows you a C shaped structure which in essence is this (Image B) and the optic vesicle becomes the C shaped structure which is now referred to as an optic cup. And with this optic cup youre going to get two layers, an inner layer and an outer layer. Heres the inner layer and theres the outer layer (indicated on image C). The inner layer is much thicker and thats what becomes the retina. The outer layer being much thinner becomes the pigmented layer of the eye. And you still have this little space between the two layers, and its known as the intraretinal space. The lens placode which pushed inward, further develops and you get this structure known as the lens vesicle. And when you see the word vesicle there has to be some sort of space as part of the structure. A solid mass is not a vesicle. And what you have developing in this 3

vesicle, and thats represented by those blue cells if you will (Image C), those are the lens fibers. Keep in mind that when the lens vesicle developed, its sort of a round to oval shape. But by the time the lens is completely formed you have something that looks like that (drew an oval). And these fibers will get longer and longer, and what elongates the structure to give you this really flattened oval shape is that there are more fibers that get added on at the end. So thats going to increase the length of the structure and change its shape. This is the part thats a little confusing. Maybe we should look at B first where you can see the C shaped optic cup. And heres the inner layer of the retina. And when I mention inner layer, were talking about the layer that really has the rods and cones. The elements that pick up the image. And the outer layer is your pigmented layer. So in the center of this optic cup youre going to find a groove or a fissure. Called a choroid fissure. And theyre trying to show that to you by showing this slit like structure. The significance of the fissure is youre going to find blood vessels present. Theyre called hyaloid vessels. Here you can see these vessels and they are located in the optic nerve. Keep in mind that youre only looking at part of the optic cup here. There should be another portion down in this neck of the woods as well (drew in on image C, see image below). We started off with an optic groove and now its become an optic stalk. And this is the stalk. And what the stalk really becomes is the optic nerve, which connects to the brain.

[Slide 5] L FIG 19-7 OPTIC NERVE 11TH ED. And this is just the C shaped structure on a larger scale. Again the outer pigmented layer, the inner visual layer (Image A). And heres the choroid fissure with your blood vessels. Of course the inner layer gets thicker (Image B) and thicker (Image C). And what youre essentially seeing at the back of the eye is your optic nerve fibers and here you can see the blood vessels, which pass through the center of the optic stalk. [Slide 6] L FIG 19.3 PARTS OF EYE 11th ED. Now here we have further development of this particular area. Heres our optic cup (tannish brown color) and you can see how thick the inner layer has become. Some people refer to it as the neural layer. The outer dark part is your outer pigmented layer. But they are both part of the retina. And there is a little space in between. From your study I assume you look at an eye in anatomy, did you? The retina consists of many layers. I think its a bout 9 or so, and there are blood vessels in here as well. You can develop certain problems as I did many years ago. One morning I got up opened up one eye, then opened up the other eye, and wasnt seeing exactly the same thing out of both eyes. So both eyes had the same vision capacity but at that point it didnt. So I knew something was wrong and went to the eye doctor. And he couldnt find anything. Looked carefully, called his partner in, who couldnt find anything, and the consensus was come back in 6 months. Cant always trust doctors opinion. I knew something was wrong, and they just didnt know what it was. So I went to another eye doctor who recognized I had a problem dealing with the retina but didnt know exactly what it was, and I got sent to a retina specialist. Did certain tests including dye tests. To make a long story short, I had a leaky capillary. And as a result, the fluid leaking out was separating the 9 layers, and if left unchecked you would loose vision in that eye. This required laser surgery. There are all different types of lasers. First laser I ever saw was in the movie Goldfinger, and that laser was meant to cut through gold. This type of laser is meant to burn a certain specific region. He started to work and finally said to me, Do you trust me? So I said yes. Then stop squirming in the seat! I cant focus the laser on the spot that I want to! All the tests that he was doing or had done pinpointed where the leaky vessel was. And then with the laser he burnt the capillary, sealed it up and knock on wood I havent had a problem since that time. But it had been around for a while and I just hadnt been aware of it until that one day. I did lose a small percentage of the vision in that eye. In wearing corrective glasses, I have two different prescriptions, one to accommodate 5

the loss of vision in that eye. So anything is possible that can happen. And why did the vessel leak? No one can give you an explanation. But if you have high blood pressure, the pressure effect is not only visualized in the arm the doctor uses to take the pressure, but all through the body. So the pressure problem that I had must have broke the capillary and lead to this problem. Thats my sad tale of woe for today. And heres the central part of the cup and this is where you have the choroid fissure. And what its showing you here happens to be the hyaloid blood vessel that was in the choroid fissure, and you can see its feeding directly into the optic nerve. Surrounding the developing eye is the pink region and that is essentially undifferentiated mesenchyme, which will give rise to other things as well. In this picture if you look at the lens vesicle, the space is much smaller, these posterior cells are much larger, and there have been more lens fibers added on here and here (Indicated very top and bottom of lens). So the shape of the lens is changing slowly but surely. Now on the outside of the eye youre going to have your surface ectoderm, and you get a little invagination on top and bottom, and youre forming your eventual eyelids. These two will grow towards each other and sort of seal up temporarily. So when you are born the lids have to separate once again. [Slide 7] L FIG. 19.6 FURTHER DEVELOPMENT OF EYE 11TH ED. And this particular picture, heres your upper lid, lower lid, and you can see both of them have attached to each other. And now look at the lens vesicle (blue structure). This little dark line in here represents the remainder of the space. So technically we can now call this structure the lens. And what youre seeing in the lens are all the lens fibers. The shape isnt quite as flattened as it suggesting that it will become but that happens in due time. If we look at retina youll see the pigmented layer and your neural layer. Then, right above the pigmented layer youll see this vascular layer, which is referred to as the choroid and its associated with the pigmented layer of the eye. Over here, an extension of the choroid will become the cornea of the eye. And thats a fairly transparent covering of the eye and youll follow this cornea completely around, and youll see it merges with a structure called the sclera. And the sclera is the white portion of the eye. And both of those develop from mesenchyme. Now the retina itself, the neural layer has lengthened a little bit. And we can divide this retina into two parts. Lets say from about here to here, thats the anterior 1/5 of the retina (see image below). The rest of the retina is the posterior 4/5.The posterior 4/5 has your rods and cones, and is known as the pars optica retinae. The anterior 1/5, which I outlined in red, represents the pars caeca retinae, and thats where you have your iris and ciliary body forming. In addition, at the same time, youre creating a number of spaces associated with the eye, and a space behind the lens is your vitreous body, which has a gel like substance. Then we have your anterior and posterior chambers of the eye, which is filled more with a clear liquid fluid substance.

[Slide 8] L FIG. 19.4 RETINA 11TH ED. And this just shows you various layers of retina, and so youll have blood vessels present. And you can have leakage from any of those blood vessels. And you can see that the various layers are integrated with each other, and if you start pushing the layers apart, you are going to have a problem. [Slide 9] L FIG. 19.35 SYNOPHTHALAMIA OLDER ED. As with anything else you can get defects associated with the eye, and this particular case is called synophthalmia. In essence you have two eyes developing which fuse together. And so thats going to happen in this region. And if the two eyes fuse together, the nose has to have defects. And so youll find parts of the nose not formed into a well organized structure at all. And obviously the vision is impaired, if youre having this problem, youll notice theres a problem in terms of formation of the mouth, there are defects of the ear, and this type of individual of course would be mentally retarded. A take off of this occurs in good old Greek mythology with the Cyclops who develops the one eye in the center. Thats a similar scenario. In that case youre developing one eye, not two eyes fused together. Theres a condition where the eye is missing, anopthalmia. And again whenever you see the letter a, it means absence of. [Slide 10] L FIG. 19.12 SYNOPTHALMIA 11th ED. Heres another version of synopthalmia. This by the way is supposed to be the nose, and this is the nasal cavity. And literally the nose is up in the air and the eye is behind here. So 7

once you start having defects in the midline it must affect the brain because it extends all they way up.

[Slide 11] L FIG 18.1 EAR DEVELOPMENT 11TH ED. Ear development. We go back to the same picture we started off with before (Image A). Heres our nice little embryo, again, heres the optic vesicle, which we just covered. And now we are going to turn our attention to the otic vesicle. First lets look at picture B. Its showing you the neural folds, theyll fuse and youll get your neural tube, and theres your notocord. If you look at the surface ectoderm, right over here (Image A, Otic vesicle) youre going to get a thickening, and thats your otic placode. [Slide 12] L FIG. 18.2 OTIC VESICLE FORMATION 11TH ED. And with any placode theres going to be a vagination of the ectodermal cells, and so heres your invaginating placode (Image A). Its pushing in to the head mesenchyme etc. And you can see at this point the neural tube, at least what we see as half, has been completely formed. So the placode continues to push in, and just think of these areas as, oh. Ok. Uh, why dont we take a few minutes break at this point. Have you noticed that when most people lecture, sooner or later theres a problem?