 It's a much better presentation in person, but hopefully you guys can pick up some pearls and things from the lecture that will be helpful for both boards and for your career, whether you're going into glaucoma or not. Pediatric glaucoma is a fun topic. It's a very small niche area, but there's a lot of crossover with a lot of different specialties. Those of you that are going to be even doing plastics or neuro, you will encounter pediatric glaucoma in your career, no matter what you do from comprehensive corneal to neurophthalmology. So let's just start and feel free to stop and ask any questions. And then we'll keep going. Okay. Now I can't see everybody. I know some of you are still probably getting up, but maybe I'd like to have some participation. Otherwise, this is going to be a pretty quick lecture. All of this is going to be question format. And we'll just kind of talk about different things, different features and things that you might find interesting, some questions that may come up. And then I'll show a video. Some of you have seen some pediatric glaucoma surgery. And so I'll just show you kind of a classic case, kind of my go-to surgery for most cases of congenital glaucoma. Okay, question one, which of the following is not a classic feature of congenital glaucoma in large cornea, also known as buphthalmus, obstrea, myopia, conjunctival injection, epiphora, photophobia, and blepharospasm. So I'm just going to start picking on people that I see in our group. Let's start with who's here? How about Cole? I think I see you're on Cole. This is just kind of an open discussion. What do you think about this list? What things are not classic features of congenital glaucoma? The classic triad would be the last three, epiphora, photophobia, blepharospasm. You certainly can see obstrea, buphthalmus, and kind of progressive axial myopia. So I think congenital injection would be too broad. Yes, that is correct. The answer is congenital injection. That triad is what you alluded to at the bottom, epiphora, photophobia, and blepharospasm. And then in large cornea, buphthalmus, everything else is pretty much classic features for congenital glaucoma. Now, is there an age range? Have you come up with some sort of scheme for determining what is congenital? How do you categorize these? How do you categorize pediatric glaucomas, in other words? I guess thinking about age range. I know juvenile is like four to 35, I think is the BCSE. So I kind of think of congenital, I guess, is earlier than four, but usually within the first couple of months to a year of life. Yeah, I think congenital glaucoma is at birth, and then there's infantile, which is in that range after birth or after a year. So, and then yes, you're correct in terms of juvenile open-angle glaucoma is a very broad, large range. Essentially in your BCSE book, I think it says what, four to 35. And just realize that anybody who presents with glaucoma at a young age is in that broad category of juvenile open-angle glaucoma. Okay, let's see. So this is just a picture of hopstria. Do you guys remember, Abigail, do you remember hopstria in terms of this is a path question that may come up? It's a crossover question between glaucoma, pediatrics, and pathology. But do you remember, these are hopstria that you see here in this photograph, but what exactly at what level are hopstria occurring? Say it again, Abigail. Sorry, this is better. I think it's at the level of the endothelium. Close. Oh, decimates, sorry. Decimates, that's correct. So, breaks and decimates is why hopstria occur, and then that's what leads to potentially corneal demyelopaspidation. Okay, all right, very good. All right, question, next question. Which of the following age ranges portends a better prognosis for patients with primary congenital glaucoma? You know, this is a more detailed question, but this comes up all the time. In fact, this came up last night. We had a patient who presented with primary congenital glaucoma at birth. The several UA's prior to today or yesterday were reasonably controlled pressures on medical therapy. And now the kid was four months old. And mom had a question about this. Which of the following age ranges portends a better prognosis? So, why don't we go with, this is kind of a, I think a senior level question. How about Ariana? You're going into glaucoma. This is going to come up a lot next year. Okay, I actually don't know the answer. I guess I would think older age, but I'm not sure. Yes, so this is the magic window that I want you guys to remember for primary congenital glaucoma. It's three to 12 months. Okay. And the way I think about it is if someone presents at birth with congenital glaucoma in signs of congenital glaucoma, that's pretty severe. This has been going on in utero. And it's often a more difficult case, very challenging case. If they present older, that also could present some issues as well. But in that three to 12 month window where they're in that they're still their eyes developing quite a bit there, even their plumbing maybe still developing, that tends to be a ripe time for response to surgery. So if they present in that three to 12 month range, they usually do pretty well with angle surgery. Okay, next question. This is just a picture of buphthalmus, just to realize that it can be unilateral or bilateral. This is a bilateral case of cloudy corneas and buphthalmus. Okay, next question. Which of the following are not distinguishing features of Axanfeld Rieger? Bilateral, autosomal recessive and autosomal dominant inheritance, sporadic, around 75% association glaucoma, posterior embryo toxin, maxillary hypoplasia, hypospatias and pituitary abnormalities. So Mike, I think Mike, you're on, you're on with us, Mike Murray. What do you think about this list? Let's just kind of go down the list and see if you can recall some of the features and then we can kind of by process of illumination figure out the one which is not a distinguishing feature. Alrighty, so let's go through these. Looks like, so pituitary I know, yes, the hypospatias that would say yes, maxillary hypoplasia, I would say yes, the posterior embryotoxin, I'd say yes, 75% association. I am not sure about that one. I think it might be a little lower, but I'm not sure. Sporadic, I would say no because a lot of them are, well, I think they can be sporadic, but a lot of them are autosomal recessive autosomal dominant. And then bilateral, I would say no. That it's not a feature? That it's not? Yeah, most of them are thank-you lateral. So actually most of them are bilateral. Okay. And axinfel reger can be inherited in many either autosomal recessive and or autosomal dominant, but actually most cases are autosomal dominant. And so that's just some, and I want you to think about this. If you guys have ever rotated on pediatric glaucoma service or pediatric service, we have groups of families who are coming in for axinfeld regers. And so that should let you know that this is a very strong gene and very strong inheritance pattern. And so most of the cases are autosomal dominant. When you see groups of families with axinfeld regers, you can kind of remember, oh yeah, for Mendelian inheritance, most of these are autosomal dominant, but you can have some sporadic cases. The other theme that I want you to take away with, and this is kind of throughout glaucoma for many things in terms of risk for when they talk about things like pseudoxfoliation, pigmentary glaucoma, axinfeld regers. The general ballpark, if you have to guess for your test is about 50%. So all of these are features, bilateral is a feature, autosomal recess, autosomal dominant, most are autosomal dominant, some sporadic, around 50% are associated with glaucoma. So the answer here is around 75% associated with glaucoma is not a distinguishing feature. I know, tough question. I'm just picking some things out with some nuances, but otherwise might be picked them out really well. Okay. Okay, so here's a classic picture of a posterior embrotoxin. This is a very traumatic case. Sometimes it's very subtle. And sometimes you may not be able to see this until you actually do gonioscopy, intraoperative gonioscopy, like with a microscope and a gonioprism where you can actually see all these different features. And sometimes it's very subtle, just a little bit of purling around Schwab-based line. So you may not see this kind of dramatic case. It's just, you know, very, very overt. Why is that important? I'll go back to Catherine. This is a great cornea question. Posterior embrotoxin, why is this, what's the pathology of a posterior embrotoxin? And why, what types of features may you see in a posterior or an axinfilt regress patient that is related to the posterior embrotoxin? It's mostly, I know it's basin, just anterior segment dysgenesis. That's the basis of it. So I'm not quite sure. What are some other features other than, what are you, remember that this is a common entity that we confuse with ice syndrome, but what's the dead giveaway in demographics between axinfeld versus ice? Ice syndrome is in a female and it's unilateral. Okay, but what about age demographics? And I'll give you a clue. We're talking about pediatric glaucomas. Ice is in adults. Yeah, young adults. You guys remember vanilla ice, that's kind of the picture you should have in your mind of young adults who present with ice syndrome, but axinfeld regress is a pediatric glaucoma. Okay, you're going to pick these up early on. And there's a whole spectrum of anterior segment dysgenesis that we kind of lump in here with axinfeld regress because of incomplete penetrance. So what types of things are, can you see though, in terms of the physical exam, let's say you don't see a posterior embrotoxin, what are some other classic features that you can see on an axinfeld patient? Anybody can chime in. Choreotopia. Okay. And how that might, how might that be related to the posterior embrotoxin? Oh, I'm not sure how it's related to the embryotoxin. I thought it was just in general for axinfeld reger. Okay. Anybody else want to chime in? I mean, iris atrophy, polychlorio. Yeah. So this is, this is a, an abnormal membrane that can start to pull on the iris and the angle structures. So hence you see choreotopia and hence you see perfluentia synegia. Okay. So the idea is, is that this abnormal membrane that you see the posterior embrotoxin has contractile features on it. Okay. Similar to an ice membrane that can cause contractile features and pull on the iris and where you can get choreotopia as well. Okay. Okay. Next question. Which of the following are not distinguishing features of a Peter's anomaly? Catherine, go back to you here after I'll read the list and then you can just kind of work through this list. Leucoma, corneal and ticular adhesions, erotocorneal adhesions, sporadic inheritance, unilateral and around 50% glaucoma risk. So which of the following are not features of a Peter's anomaly? Let's see. Not distinguishing features. Yeah. So which ones are first? You can do process of elimination here. Yeah. Um, is Leucoma, is that corneal winding? Yeah. Okay. So yes, that is a feature. Um, corneal and ticular adhesions. I'm not quite sure about that one. Um, erotocorneal adhesions. I'm not quite sure about that one. I think yes. And I think it is sporadic and unilateral and actually not quite sure about glaucoma risk. Okay. Sounds good. All right. So yes, Leucoma is the white lesion that you see of the cornea. And if you remember a Peter's anomaly, um, the dead giveaway is that you get this, you know, big white spot right in the center of the cornea. Uh, you're thinking it's just trauma, but you know, usually these cases are bilateral. Okay. So the answer here is actually unilateral. It is not, it's not unilateral Peter's. Most of the time it's bilateral Peter's. Gotcha. And that's kind of the dead giveaway. And if you remember that the Leucoma is really a Pandora's box, if you were to lift off the Leucoma, there's a lot of badness typically underneath that Leucoma. And that's where you get into the lenticular adhesions or the erotocorneal adhesions. And so sometimes it'll just be these sprouts, these, um, roots that come off the iris that go right to the Leucoma. And they can be very difficult to deal with, um, during surgery, especially for cataract surgery, where you have to kind of lice all these adhesions off. And obviously your view is terrible when you have a giant Peter's anomaly too. So some of these patients may have to undergo a corneal transplant, but it is kind of the Pandora's box on the top of the cornea. So this is a typical feature. Well, it doesn't look too bad, right? But if you were to do UBM here or OCT, you would see that underneath this are strands of iris tissue coming up to this Leucoma. So it's not simply something that you can just scrape away or do a limited PKP in that region. There's usually going to be adhesions underneath. And if you remove them, there's going to likely be some anterior segment disorganization. And so you might need some anterior segment reconstruction after this. Okay. And once again, 50% glaucoma risk. So if you have to guess on your test about the risk for glaucoma, guess 50% for most of your questions. Okay. All right. Next one. All right. A parent with a one year old aneridic child is wondering about the long-term risk of glaucoma, which statements are not true about aneridia and glaucoma? So why don't we go with who's been on pediatric glaucoma service? I only want to pick on those that those of you that have actually rotated on service so far. Sean, have you done pediatric glaucoma service yet? I mean, I've been on pediatrics. I guess some there's maybe saw a little bit of glaucoma, but I'm not sure that's going to help me with this question. It's okay. So, so which is not true about aneridia and glaucoma? I mean, you know, you've got that 50% number there. I would imagine that it's higher. I think it's higher than that. The risk of glaucoma and aneridia. I think the second one, well, let's go with yeah. Yeah, yeah. I think I'm really not sure, but I think that the mechanism is this genesis of the angle rather than sneak you'll closure. And I think that glaucoma probably could develop after birth. Although that would probably indicate something more like sneak you'll closure. But I really actually don't know. Okay, that's okay. I think the risk is higher than 50%. Yes, you're correct that it's around 50 to 70% risk for glaucoma patients with aneridia. Now, there's a theme here in pediatric glaucoma that I want you guys to remember in terms of when children present with glaucoma. If they're fairly young, okay, it's usually just genesis of the angle. Okay, now aneridic patients are interesting because they can develop glaucoma over time. And the second decade, sometimes that may be their chance when they develop glaucoma doesn't always have to present at a young age. But I think what's important is here is gonioscopy and figure out exactly what's going on. As you remember in aneridic patients, there are sometimes often some residual stump. It's not like they're completely aneridic. And so by doing gonioscopy, that actually helps to determine what your surgical approach is. So if I have a patient who presents at a very young age with aneridia, one year or less, then I'm going to do a gonioscopy. I'm going to check and most of the time when I've examined these patients, there is no anterior synechia. And so I'm going to give it a chance. We're going to perform angle surgery. These cases are a little bit tough because you've got no protection. And you've got to be very careful that you don't nick the lens and cause a cataract. But it is possible to do angle surgery in these patients. And they often do pretty well. But over time, if the patients progress, and you start to see perforants here, synechias, and that stump starts to rotate and really close off the angle, then you're going to be moving into a different type of procedure like a bypass, either a trapecolectomy, a zen, or a tube trend surgery. So the mechanism can be either of those two. Early in life, it's this genesis, but if it occurs later, it's more synechial, more likely. That's right. Yeah. And so, and that's true of all glaucomas. You can pretty much, if someone comes in with an early type of glaucoma, it's usually angle dysgenesis. But once you, for primary glaucoma, if you've already ruled out of the causes such as trauma, et cetera, steroids, these cases are going to be dysgenesis of the angle. So we're going to give it a try. We're going to do angle surgery in these patients. All right. Next question. All right. An infant with Sturge Weber syndrome presents with congenital glaucoma. Which of the following is not correct? Why don't we go with, Abigail, you're going in the plastics, you'll probably be seeing a lot of these cases. Can you hear me okay? My audio's weird, sorry. Yeah, I can hear you. Okay, perfect. So, infant with Sturge Weber presenting with congenital glaucoma. Which of the following is not correct? I think eridotribecular dysgenesis is the right thing to do. You feel that that one is not correct? I think so. So I'll give you a clue. And the last question, we just talked about these themes that you'll see in pediatric glaucoma. If kids present early on, it's usually due to eridotribecular dysgenesis or angle dysgenesis. Okay, and this is an infant, so that would be the reason. Yeah, so the clue here, this is an infant. Oh, is it the lower eyelid? So, now we're talking about just the lesion in a Sturge Weber syndrome patient, right? And the port wine stain is actually in the upper eyelid. It's more likely if it involves the upper eyelid. That showed up on my board's question, and I couldn't believe it, but ever since then, I've remembered that it's the upper eyelid. It is more likely to port 10 glaucoma if the port wine stain is on the upper eyelid. I don't know why, but it's just one of those things that you kind of tuck away a little pearl. Okay, is anything else not correct here? An infant with Sturge Weber? The tube shot is preferred over angle surgery. So this is a tricky question, but what I'm trying to tease out here for you guys is to understand that. Is it the first one? If it's congenital glaucoma, because it would be higher EVP would develop later. That's correct, yes. So what I'm trying to get out here is another theme, or there's a theme just kind of reiterating the same thing. If you have an infant that presents with glaucoma, angle disgenesis is the most likely cause, right? Even though this patient has a port wine stain, you're thinking, oh yeah, elevated episcopal venous pressure, but in this case, because it's an infant, the things that are not true is that elevated EVP is the most likely mechanism of glaucoma. That's not true. That's true later in life, okay? And then the other part of this is that a tube shot surgery is preferred over angle surgery. That is not the correct approach for this, because it's an infant, because it's angular disgenesis, we're going to do angle surgery as a preferred approach, even in a patient with a port wine stain and Sturge Weber syndrome, okay? So I'm not really worried about the risk of a crudal effusion and crudal hemorrhage. Those are the things that we worry about in patients with elevated episcopal venous pressure, okay? Does that make sense, that question? You guys have questions about that? Yeah, I have a question about angle surgery. Is it that angle disgenesis? Is it that we're targeting the trabecular mesh work and doing TM surgery, or we're doing something else in the angle that addresses something other than the TM? No, I mean, at present, I hope one day I'll be able to intervene in collector channels, but as of right now, the only thing I can do is either strip or stent the trabecular mesh work. So, I mean, we could, you can viscodylate, I guess, but usually we're doing viscodylation in the setting of doing a trabeculotomy, okay? And so, when I refer to angle surgery for kids, I'm referring to basically doing either a goniotomy or a trabeculotomy. Okay. Yeah, I have yet to put any stenting devices in kids, and I don't know if anybody has done that before. I think the most progressive thing that people have done is a co-hook dual blade, or they basically just peel off the TM, right? But most of the time, we're just doing a cutting procedure and doing an otomy of some sort, either goniotomy or trabeculotomy, okay? All right, next. Dr. Triad, just to clarify, you said a tube chin is not preferred over angle surgery in this case. Yes, because it's an infant. Okay, gotcha. Okay, so again, just that pearl, that clinical pearl that you guys are going to see throughout your life. Infants with glaucoma, angle dysgenesis that should just come to mind, and we're going to try to do angle surgery. Right. Even though I threw in these confounding things that, oh, this is a patient with Sturge Weber, and you're thinking, oh, Port Weinstein elevated episcletal venous pressure, that's something that you want to categorize for patients when they're older as a possible cause, right? So for example, I might have a patient that we follow, or you guys might have a patient that you follow with Sturge Weber and Port Weinstein, and they don't have glaucoma as an infant or as a child, but maybe later as a teenager they present, and how would you decide, should I do angle surgery or should I do 2% surgery? I would be cautious to do a 2% surgery in a teenager or in a young adult patient with Sturge Weber because of the risk of paroidal hemorrhage. When you rapidly decompress the eye, you put them at risk of having a paroidal effusion and paroidal hemorrhage. So these are cases where we typically would put a tube in, in a stage process, or we would ligate an almond valve in order to kind of stage the decompression. So if we were doing, the way we used to do is we'd put an almond valve in just sew the plate in and not actually put the tube inside of the eye. Then we would wait for maybe six to eight weeks and to allow a capsule to develop over the plate, and then we'd come back for stage two and then put the tube actually into the interior chamber or the sulcus. And the idea being is that if you have a biological valve, the capsule, the tenons it forms over the shunt, you're going to reduce the risk for kind of rapid decompression, right? Absolutely. So because if I just put a tube in immediately, it's going to start working from day one. And if you drop them too fast in a patient with elevated episcovial pressure, there's a larger risk for cortifusion and hemorrhage. Okay, next question. All right, which of the following is true regarding a fakia after congenital cataract surgery? Which of the following is true regarding a fakia after congenital cataract surgery? Cataract surgery 15 to 50% or more developed glaucoma. The patient is only at risk of developing glaucoma within the first, sorry, within the first three years after surgery. Irritable trabecular dysgenesis is the most common cause. Removal of all residual cortex during surgery may reduce the risk. And small coronal diameters are risk for developing glaucoma, and cataract surgery in the first year of life is a risk factor. Okay, who wants to take this on? A lot to cover here. And there are multiple answers. I can give it a shot. So 15 to 50% or more developed glaucoma, I think that's true. The patient is only at risk of developing glaucoma within the first three years. That's not true there. I think they're at risk the rest of their life. Irritable trabecular dysgenesis is the mechanism. I think that the mechanism, I think it's not like definitely known, but that the patient had a cataract. And so anterior segment dysgenesis is considered possibility because something led to their cataract in the first place. And so their angle may not be normal either. And then removal of all residual cortex reducing the risk, I don't know, a small corneal diameter as a risk that seems true to me in like that anterior segment dysgenesis category, but I'm not sure. And then cataract surgery in the first year of life is a risk factor. Yes, that's true earlier surgery is a risk. Okay, great. All right, so we'll start at the top. There's that 50% rule again shows up a lot in on your on your questions. If you have to hedge, you can usually say around 50%. The patient is only at risk of developing glaucoma within the first years, three years after surgery. That is false. Okay, you can, as Arianna said, this can be something that can develop, you know, within the first few months after surgery, or could be developing later in life. Okay, so iridotrabecular dysgenesis is not the most common cause. And you really have to do a good exam to figure out exactly what the cause is. But many of us in glaucoma understand that, or we don't understand truly how the exact mechanism, but there is something that seems to be very important for the development of the angle for a patient to retain their lens. When you remove that lens, there's either some postulated theories about stretching of the scleral spur, and how that relates to the angle. So really, we'd like to think of the lens angle as a complex, and there seems to be something that's really detrimental to the development of the angle, if you remove the lens early on. As you know, the eye is growing. And so there's some, there seems to be a role in terms of a beneficial role for the lens to be retained in the development of the angle. So by removing it very early in life, you definitely put them at risk of developing maldevelopment of the angle. But it's not really your classic example of an iridotrabecular dysgenesis, where there's maldevelopment early on. Now, you have to do good gonioscopy because if a patient presents with the history of cataract surgery as an infant, you need to figure out what the mechanism is. Is it truly open angle? If it's truly open, then I would consider doing angle surgery to see if removing the trabecular mesh work may help to decompress the eye. However, these patients may develop synechia, and you can get kind of a creeping crawling peripheral anterior synechia. And so if I have an angle that's completely zipped up in a patient with a history of infantile cataract surgery, we're going to be moving on to a different procedure. I won't even try to take these down. I think it's kind of a mess to try to take down these types of synechia. If it's limited, you can, and then do some angle surgery. But usually we're moving on to a bypass surgery. Okay. So, and then removing all the residual cortex, why is that helpful? You guys know the kids are very pro-inflammatory. And so if there's a lot of postoperative inflammation, that could cause disruption or to the angle and to the trabecular mesh work and cause dysfunction. So it's important to be meticulous. When you do a pediatric cataract surgery, you really want to remove all the cortex used by manual. If you have to get out to the phoenix and really search for cortex, removing the lenticular material, the core nucleus and epinucleus is a piece of cake. You just slurp it out. And really, most of my time spent during infantile or pediatric cataract surgery is removing the residual cortex and polishing the lens epithelial cells, which I'm a big fan of, particularly for pediatric patients. Because these kids, if you don't, they end up with huge cell rings rings that become problematic later, either as adults or as either as juvenile adolescent or adult patients. In small corneal diameter, that makes sense. Microphalmia, micro cornea, these patients have a tight angle just by sheer volume. The anterior chamber volume is small. The iris is closer to trabecular mesh work and at risk of developing a significant closure. Okay. Okay, next question. Which of the surgical approaches is the preferred method of treatment in a one-year-old with primary congenital glaucoma and cloudy corneas? I'm going to give this question to anyone who's rotated. I think, Cole, have you been on the pediatric glaucoma service? Right. So hopefully, now here's some clues as you read this question. It's a surgery question. It's a one-year-old with primary congenital glaucoma. So we already talked about angle dysgenesis as being the primary mechanism. And this patient also has cloudy corneas. Yeah, so we're certainly not going to put in a tube. And to do agoniatomy, you need visualization of the angle. So you can't do that with the cloudy cornea. And so I think then you need to do a trabeculotomy or ectomy. And the trabeculotomy abexterno I think would probably be the first choice. Yes, that's correct. So these terms are confusing. And I want you, if you're going to remember this for a test, agoniatomy requires you to use a gonioprism. And hence, you would need to have a clear cornea. So you answered correctly that for agoniatomy, you need a clear cornea to be able to visualize the angle. Now, sometimes you get lucky. Last night, we got lucky. We had a patient with a cloudy cornea. And the view was marginal. And I had one small window. If I tilted the patient far enough away from me, I was able to look between the cloudy central cornea and the limbus, which had cleared since birth. And so I had a very small window where I was able to do agoniatomy. Now, you guys have heard of the GAT technique. The GAT technique is gonioscopy assisted transluminal trabeculotomy. And so this is confusing because for this type of procedure, for the GAT procedure, you do need to have a good clear cornea, relatively clear, because you are using agoniatomy or gonioprism. Now, in the classic sense, for this question, a trabeculotomy, before we had angle surgery techniques and before we had catheters and sutures and things that we could use to cannulate slumps canal, we were able to do a trabeculotomy ab external. And that's where we do a cut down. We do a pyrenomy. We make a flap or a slit. And then we basically dissect our way from external to internal into slumps canal. It's probably the one of the most challenging but one of the most fulfilling procedures to be able to do because once you get down to that anatomy, you really have to, there's clues to know that you're in the right space and that you're unroofing slumps canal. And one of them is the scleral spur is a very, the scleral fibers that represent the scleral spur become parallel right at the limbis. So if you're wondering, am I there yet? Am I there yet? What you're doing is you're looking for the fibers. If you see a very irregular fibers going in every different direction, you're not at the spur yet. The fibers become very organized and parallel to the limbis once you reach the spur. So once you've identified the spur, you just keep unroofing a little bit more and getting through those corneal layers until you're literally right just above slumps canal. And you have to use a Utrata forcep or a non-tooth forcep, something that's not too sharp and just basically peel off the layers as you unroof slumps canal and then you just start to see aqueous fluid percolate into the canal or into that space. And from there you thread the catheter or suture and you may be able to achieve 360 treatment by doing an otomy. Or we have something that we rarely use. We have these in our toolbox. We were almost ready to use it last night, but it looks like a pitchfork. And this pitchfork is called the harm's trabecula tome. And the pitchfork works by you put one part of the probe and then you essentially just tear through the trabecula meshwork. And you can do that for 90 degrees or 180 degrees. We have probes that go to the left and probes that go to the right. But that's an approach where we would do it as an ab external technique, a trabeculotomy ab external. Trabeculectomy is actually the right procedure if you're in other parts of the world. When I've traveled around and I've seen how people have managed pediatric glaucoma, they've done a trap-trap. And what a trap-trap is, is you do a trabeculectomy along with a trabeculotomy. So they'll dissect a trap flap and then they'll do a trabeculotomy with a harm's trabeculotome. And then they'll make a punch and then convert that into a trabeculectomy. And so, but these are tough. Why do we not do, at least why do I not do trabeculectomies for kids? You can imagine if we're going to manage your trap in a child and we're going to have to cut stitches or do suture lysis, that's pretty much impossible in a young child because of cooperation. And that would require you to take them back for an exam under anesthesia every time you wanted to adjust the flap. Now, there are ways to be able to do it where you leave the sutures a little bit loose, but then I think you also risk hypotomy, etc. And so I'm not a huge fan of doing trabeculectomy in kids unless you absolutely have no other choice. We are doing Zen, though, on this list is, not on this list, is the Zen, which is a small gelatin stent that we use to bypass and to create a blood forming procedure. I think that's a reasonable approach for congenital glaucoma and for kids because you don't have stitches to manage postoperatively. So the answer to this question is the first choice would be a trabeculotomy of external or the cornea is too cloudy. We can't use a gonioprism that's why goniotomy is not the correct answer. And trabeculectomy is the right answer if you're other parts of the world. But I think for your board exams, the answer that you would put here is trabeculotomy. Dr. Chai, a quick question. If you were trying to buy a kid time, would you ever do an ab-interno Zen or would it just scar off so badly with all the tenons there? Does anyone do that just to buy time and say, oh, this might scar off, but we want to get the pressure down so that our trap will work better in the future? So because you're saying that if I do an ab-external and do a pyromede that I'm just potentially causing more scar tissue and why not do an ab-interno, is that what you're saying? I'm just meaning if often in adults we say, oh, their pressure is high and so the trap will be unpredictable in healing. And so sometimes we say, oh, we're going to do an angle surgery like a hydrus or whatever just to bring down the pressure and make it a little more stable. And I think I'm not like super up on the data, but I think even when you put in like a hydrus in an adult in which they're going to get a trap, they do better if they've had some sort of mix. So I was wondering if you ever do that with a Zen in kids to like prep them for their future trap? That's a good question. I will tell you that my general approach is to start on the angle for patients with congenital glaucoma. If that fails, we move on to a bypass. My first choice as a present would be a Zen and I would do that as an ab-external technique. I'm not a huge fan of the ab-interno technique for the Zen. That's how we were all trained how to do it, but particularly for kids who have very thick tenons, I think it's ripe for failure. And unless you can get that Zen in perfectly in the subconjunctival space, well, let me, I'll tell you my bias. I don't think the Zen in the subconjunctival space is a very safe place to protestant. And I've seen several cases of Zen erosion, conjunctival erosion, where they're placed simply under the conjunctival, which is how we were taught to place them if you're not opening the conch. I prefer to do an ab-external technique. We'll bring back the conjunctival, we'll pull back the tenons, and then we know exactly that that Zen's going to be placed under the tenons' tissue. Now, can that patient get a trap later? Absolutely. If you're careful with your dissection, you're not too aggressive, there's still multiple tissue where you can put a trabeculectomy in later in life. Now, my new approach for patients who, let's say, fail a Zen surgery, and now I'm forced with the decision to put a tube shut in this patient, I like the approach that Sharon Friedman has espoused. Sharon Friedman, some of you know her, she's a pediatric glaucoma specialist trained both in pediatric ophthalmology and glaucoma, who runs the Duke pediatric glaucoma service. She's a huge fan of, if you have to put a tube shut surgery in, or a tube in a patient with congenital glaucoma, or even a pediatric patient, a juvenile patient, is to put the tube down in fear and nasal quadrant so you can save some superior conjunctival for a future filtration surgery. I don't know if that answers your question, Mike. No, that makes sense. Yeah. Yeah, so I'm always thinking about the next plan, the contingency plan. In fact, when I see parents, when I counsel them for congenital glaucoma, I often tell them this is not going to be their first rodeo. This is going to be the first of many interventions over their lifetime to be able to control the disease, right? Now, in the future, I hope that we'll have pressure flow. Pressure flow is a new device that has not, unfortunately, about three weeks ago, the FDA ruled that they are not going to approve it here in the United States. It's a long, complicated story. I won't get into it, but we're all disappointed. So presently, it's only available in Canada and Europe and parts of Latin America. But I think that material has an advantage over the zen material. The zen, as you know, is a gelatin-based stent. It tends to degrade over time. I've seen it in some adults just completely melt away, almost disintegrate. But the pressure flow is a special type of polymer made out of a polymer called SIBS, which induces less tissue reactivity. And our hope is that we can make this available one day for patients in the United States with all types of glaucoma. But I'm particularly excited for patients with congenital glaucoma. Are they putting this in kids internationally? Yes, yes. Ike Ahmed has done probably, he hasn't done a thousand kids, but he's done a lot of kids with glaucoma juvenile open angle and primary congenital glaucoma, and they've used it successfully there in Canada. He has about, in his cohort, he has over a thousand patients, but that's all comers. That's adults, children, but he's had some great results with pediatric glaucoma and the pressure flow. Okay, next question. We talked about this a little bit, but let's see if we can have an answer, because parents are going to wonder, the parents of a six month old child with PCG, primary congenital glaucoma, want to know how successful angle surgery will be for their child? What's the appropriate response? 50 percent. 70 to 80, 50 percent. Better than 95 percent. Wow, that sounds like almost like cataract surgery, or 25 percent. So who wants to take a stab at this based on what we talked about? Remember, we talked about that magic window, right? Is this patient in the magic window? Yes. Yes, three to 12 months. 78 percent. That is correct. So pretty good. I mean, I'm optimistic. When I see a patient who presents in that magic window, I am very bullish with the parents, and I will tell them that there's a very good chance that this first initial surgery is going to do really well, and hopefully we won't need another major surgery for a while. Okay. Okay. Next question. All right. Which of the following class of medications should be avoided in infants with glaucoma? I'm going to give this one to Tony. Beta blockers, CAIs, alpha adrenergics, PGAs, or myotics. So definitely right off the bat, alpha adrenergics. We don't use them. For beta blockers, we do want to be careful if they have bronchospasm, if they're very small. For CAIs, I think you can use them, but just to be careful to check their electrolytes and make sure they're not having like lethargy. For PGAs, I think you can, but they're not very effective. And myotics, usually mostly just used for, I think it was I think aniridia. Okay. So you're correct about alpha adrenergics. That is one class that we're, so the clue in here, though, is infants. Okay. We're talking about really young kids here. And why is it that we are avoiding alpha adrenergics? So they can cause CNS depression and apnea. Yes. That is correct. So, you know, thymologists, we can kill patients too. And this is one where you could kill the patient. So alpha adrenergics, that's definitely on your board's question. It will likely maybe even show up on your orals. Those of you guys that are going to be taking your orals soon. I don't think, mainly this is for our fellows, they've got orals coming up this weekend. But should we avoid alpha adrenergics for all pediatric patients? Let's say I have an eight-year-old. We put them on cosopt and a PGA. And is it okay to use an alpha adrenergic for a patient who's older? Yeah. I think by eight would be okay. That was kind of where I was thinking of cutting, having a threshold. Yeah. And so I think the youngest that I've used it in is probably a six-year-old. But I was very careful to tell parents, you need to watch the patient when you first start this medication. And if they're very somnolent, we're going to have to stop it. And so it's appropriate to use in older kids, but definitely avoid in your infants because of CNS depression. Myotics, I don't know how many of you guys are familiar with myotics. These are becoming a dying class of medications, but can be very powerful for patients. The one that I'm thinking of that is commercially available, actually, this is a medication that was recently sold by Pfizer. Pfizer was the main producer of phosphiline iodide, colonist-race inhibitor. And by doing so, you allow the pupil to constrict and you really put some tension on the spur. And myotics tend to be the magic drug for patients with Afakic associated glaucoma. We don't really use them for most patients. I mean, we use them in practice. If a patient has angle surgery, I will definitely put them on a myotic agent for a few weeks, but usually I'm not putting them on a myotic agent for long-term administration. The one caveat is patients with Afakic glaucoma. It is a magic drug. I mean, you can try the PEGs and everything and you might get marginal improvement. And you put a patient on phosphiline iodide with Afakic glaucoma, and it can be just remarkable the amount of pressure lowering that you get. And again, this has to do with the role of, you know, by putting stretch on the skeletal spur, you may be able to open up the trabecular mesh work and improve the outflow. So as I think about Afakic glaucoma and the role of the lens and the development and the angle, by removing the lens, I think you actually reduce the contraction on the spur. Give me just one second. Let me turn on this alarm. Okay. All right. Sorry about that, guys. Some of you may know my grandmother lives with me now, and that's a bed monitor. Every time she gets up, I got to go make sure that she's not going to fall. So apologize for that delay. Next question. You have decided to use a beta blocker in an eight month old with PCG, PCG patient status post-goniatomy. Which of the following are important considerations? Beta blockers are contraindicated in kids less than one year old. Punctile occlusion can be helpful. 0.25% Timolol or a beta blocker is the preferred concentration and should be avoided in patients with asthma or significant cardiac disease. Anybody want to take this one? I can take this one. Okay. So should be avoided in patients with asthma or significant cardiac disease? Definitely. 0.25% is the preferred concentration. So I think 0.5 is the adult. So sure, let's go with it. Punctile occlusion can be helpful always to prevent systemic absorption. Beta blockers are contraindicated in kids less than one. I believe that is not true. So that is correct. In many of you that have been on the pediatric glaucoma service, we love COSOP. That's actually 0.5% concentration. If you have a choice, it would be ideal to start with a lower concentration in kids just because of the risk for bronchospasm and stuff. But it's not an absolute thing. I mean, we use 0.5% even in infants. So you just have to be careful. But we do encourage family members to do punctile occlusion for their children after the administered drops. Okay. So for this one, punctile occlusion and should be avoided in patients with asthma or significant cardiac disease are the answers for this question. Okay. All right. Which of the following is not true regarding juvenile open-angle glaucoma? Presents between ages of 4 to 35, autosomal dominant, most common inheritance pattern. The angle can appear dysgenic, like in PCG. And progressive myopia can continue to develop until 10 years of age. And buphthalmus and obstrea are common. I can take this one. I think that the angle is not dysgenic, like in primary and congenital glaucoma. And then buphthalmus and obstre would be more common in congenital glaucoma. So I feel like those are two are not true. And I'm not sure about inheritance or progressive myopia. But I don't think that it's actually I do think it's autosomal dominant. Correct. Okay. Yes. So you answered correctly. The ones that are not true for this question. The angle can appear dysgenic, like in PCG. No, that's not true. The angle in a juvenile open-angle glaucoma looks like a normal angle with your normal features, your normal landmarks, a little bit of pigmentation. But when you look at a patient, if you've ever seen a case with a primary congenital glaucoma, it would be described as featureless. You can barely see any TM and there's no spur. It just looks like iris. And then it kind of just blends into cornea. And you might see Schwalbe's line, but that's it. You really don't see any obvious features of the trapecular meshwork or the spur. Okay. We talked about obstre, or I'm sorry, buphthalmus. This is a very important question or just an important clinical pearl. You guys remember when buphthalmus stops? Say by three years? Exactly. Yeah. Around three to four years old. So if you have a patient that's older than that, that's not going to be a dead giveaway clue for you. But myopia can be. So, but realize that buphthalmus is something that we typically only see in that setting. We're only going to see in younger kids. Buphthalmus is really that corneal stretching that really occurs only early on. And then after age of four, so there's really no longer any stretching of the cornea. Okay. All right. Next question. We're just about done. The role of CCT is well established in pediatric glaucoma and should be routinely measured in all children with glaucoma. True or false? False. That's correct. We don't do pochimetry all the time for kids because there's no established norms. Is it helpful? It might be. We have a patient who's now a teenager in our practice who has pressures of around mid-20s on max drops with dimox, nurse local K. We took her for an EUA. She has axon felts. I believe she has axon felts. And her corneal thickness is like 700. And so maybe that's why her pressures are a little bit on the higher side. The reason we're concerned for this patient is that she's had a previous history of having much lower pressure. So there's been a very dynamic change. You know, one of the things about glaucoma is it's really changed over time. And so realizing that a patient who has thick corneas, if you're measuring the pressure consistently over time, if they have suddenly a dynamic change, you have to take that seriously. Just because they have a cornea thickness of 700 and their pressures 25, that may be a cause for panic. It really depends on historically where they've been. If their pressure has always been 25, then maybe that's totally normal for them. But if they at one time were well controlled and now pressures are getting out of control, I don't really look at that corneal thickness as a thing that's going to sway me and to dissuade me and to say, oh, you know, corneal thickness is elevated, a patient's going to do fine. Okay. All right. Let's see if this is the last question. I believe the normal corneal, corneal diameter of a full term newborn is which of the following nine, 10, 11 or 12. And why is this important? Because you guys are going to be called to figure out, Hey, is this you Thomas or not? What's abnormal? I'd say 10, 10, nine, going to vote for nine, two for 10. Anybody else? I don't know. But this is the OCAPS question like directly from this year and I probably got it wrong. So it was on OCAPS this year. Yeah, it was on OCAPS. This is like was on OCAPS this year. Okay. Well, here you go. Here's a free bonus point for next year. It's 10. Yes. So 10 millimeters for a normal corneal diameter of a full term newborn. Okay. Just tuck that away because it might show up on OCAPS maybe not next year, but it seems like they alternate years and they cycle around. This was definitely on my OCAPS though. Okay. All right. Let's see. These are just some things to tuck away. I save this for last because I really despise genetics and having to remember all these little things, but these might be important. And I'll try to tease out some threats here that might be important. What are the two main genes associated with PCG? Anybody know? CYP. Yep. There you go. There's the CYP1 or cytochrome P450, family ones, subfamily B, blah, blah, blah. But if you have to guess, if they give you this, GLC3A locus, just remember that GLC has to do with all these glaucoma loci and just guess GLC something 3A, 3B, whatever. The next one that's associated with it is the LTB2 gene. And I can't even remember what it stands for, but I think it's some sort of transport gene or it's a transforming growth factor gene, I believe. Okay. And so that has to do with development again, that transforming growth factor is important for development of collagen and different structures. And so that's why I kind of tie it in with this association of primary congenital glaucoma and dysgenesis of the angle. Okay. Which gene is associated with Joag? Anybody want to take a stab? Anybody remember? Tiger Myok. There you go. Okay. Tiger Myok is the one to remember. Okay. Which gene is associated with aniridia? This is definitely on your boards at some point. I got it at least once or maybe even twice during my O-CAPs. AX6. That's correct. All right. Which gene is associated with AXmFeld Rieger? AX3. AX3, AX2. You're close. AX5. No. No. Pitex2. Okay. Or Foxy1. I like that one. Sounds kind of cool. So those are the two that you need to remember for AXmFeld Riegers. Is Pitex2 or Foxy1? Okay. Now there is a large spectrum of some crossover. And so we get this kind of waste basket of associated genes and anterior segment dysgenesis. So why don't we just do a little drawing here? So which one is for Pitex2? AXmFelds. AXmFelds. Which other one? Foxy1. Foxy1. Okay. How about for Peter's anomaly? Also Pax6. It's actually, I believe, all three. Okay. And then Pax6. Aniridia. Okay. And then now PCG. So 1v1. That's right. Okay. So the one, you know, if you have to remember one to remember, I would say the Pax6 and Aniridia. That's shown up multiple times for me over my career. Let's see. Is that Pax6 dominant? What's that? Is that Pax6 dominant for Aniridia? Oh, you mean in terms of versus sporadic? No, no, versus recessive. Oh, good question. I don't know. I don't know. Okay. My final public health announcement to all of you. Big eyes are not cute. Okay. I think Disney has done a disservice to humanity and in most of their characters having really big, beautiful eyes, but big eyes are not cute for pediatric glaucoma. So that is all I have for today. Do you guys have any questions though? Just in general about pediatric glaucoma, remember some takeaway themes, kind of that 50% risk is a big deal. Remember the magic window for primary congenital glaucoma, three to 12 months, and those patients do well with angle surgery. Pediatric glaucoma surgery rarely shows up on boards. It showed up once for me, and it had to do with that issue about what the best surgical procedure was. And you had to know the difference between agoniotomy versus a trabeculotomy. Remember that agoniotomy requires agonial prism. Hence, you need a good clear corneal view. Okay. Medications. Oh yeah, medications. I was just going to ask, in somebody with anterior segment dysgenesis or I guess more corneal pathology in that sense, do you ever avoid like CAIs? Because I know that we use a lot of co-sobbed in kids, but has that ever led to more endothelial dysfunction that you've seen or cloudiness? I have not experienced that. This is, I think, something that might see more in adults who have kind of corneal decommunication with chronic CAIs, but I have yet to see it. And I think our options are more limited with kids in terms of what we want to. There are some things that we have used, some newer medications such as Visolta that might be helpful. And we are using some rock inhibitors now for kids too. So some new classes of medications that we've had experienced with adults, we've been able to use them in kids as well. But in general, I don't avoid CAIs in kids, unless I guess I were to see some sort of significant corneal and deep compensation. Yeah, I was just wondering, out of curiosity, because I haven't seen that either, but just wondering if in your experience, cool. Thanks. Yeah, I know our corneal colleagues frown when we're putting CAIs on their transplant patients, but you know, you got to do what you got to do. You got to save the nerve. I mean, we can always replace the cornea again. I can't do it, replace the nerve yet. Any other questions? Okay. Well, I hope you guys have a great Memorial Day weekend, short lecture today, but please reach out. I look forward to having those of you guys that have not rotated on service yet, so that you can see some of these surgeries. And we have some great pathology here at the Moran in terms of big groups of families of Acksenfeld's and Sturge. And it's fun to be able to kind of tie these things in together. And to really educate your primary care colleagues to let them know, you know, to be able to screen for patients with congenital colicoma and refer them early. Kids do better if they're referred early, you know, rather than what you see oftentimes in low and middle income countries where the cornea is completely scarred over already and very tough recalcitrant cases.