Lecture: Diagnosis and Management of Congenital Glaucoma

Good morning, afternoon, or evening, wherever you all are joining from. I’m going to share my slides here. We should be able to see this. My name is Dr. Claire Wright. I’m an assistant professor of ophthalmology at the University of Tennessee in Memphis. I’m going to talk to you today about congenital glaucoma. I’m going to preface this by saying I’m a fellowship trained glaucoma physician, but I primarily work with a comanagement strategy with my fellow pediatric ophthalmology. It’s a team approach and we’ll cover that a lot. I have no financial disclosures. Our lecture objectives are to discuss epidemiology and clinical features. And review medical treatment for congenital glaucoma and how these management strategies differ from adult forms. I took a chance to review the submitted questions and for the most part the questions sent to me ought to be answered with this lecture. So I have a couple poll questions to start. Here is the first one. What are the components of the classic triad for primary congenital glaucoma? Epiphora, photophobia, blepharospasm, corneal clouding. E is A through C and F is A through D. I will give you 30 seconds to answer this question. Pretty interesting distribution. We’ll have the same questions at the end. The answers of these questions will be reviewed in the PowerPoint. Next question, which of the following statements is true. A, primary congenital glaucoma is associated with a defect on the PAX 6 gene. B, the first-line treatment for primary congenital glaucoma is latanoprost. C, one should avoid using Brimonidine in children due to risk of CNS depression. D, the procedure of choice for primary congenital glaucoma is trabeculectomy. Give you all a couple more seconds to put in your answers. Okay. So pretty good distribution. Again, we’ll review these in the PowerPoint. Third question, which of the following statements is true. A, patients having undergone surgery for congenital cataracts only need ophthalmic follow up for the first year after cataract surgery, B, Peters anomaly is characterized by a central leucoma and iris strands. C, the procedure of choice for glaucoma in Sturge-Weber syndrome is trabeculectomy. And D, neurofibromatosis type two is associated with glaucoma. Which of the following is true? Okay. I’m liking the distribution of answers to these questions. This means we’ll hopefully learn a fair amount in this PowerPoint. I’m going to go ahead and proceed. Before I start, I want to plug the American academy of ophthalmology, basic and clinical science course glaucoma book. This is an excellent and up to date review of glaucoma. The way I structure my presentation is taken off the glaucoma BCSC book. For us practicing in the United States and probably elsewhere, you can get continuing medical education credits, the content of this book is what is tested on our ophthalmic knowledge test for the United States ophthalmology residents. There are videos. I’m not a paid consultant I just love the book. Now we’re going to talk about congenital glaucoma. I like to look at a historical perspective. I looked on PubMed and the first report I could find is from 1923. A case series where the term congenital glaucoma in our PubMed search was first used. I thought it was interesting to look it over because some of the observations made by the patient’s parents and caretakers and providers were interesting and telling. I’m going to read a couple quotes from this. If you’re interested you can pull up this report. The mother said that at birth the eyes protruded like marbles. The eyes were prominent and staring, pupils moderately dilated. Stony hard. It was almost impossible to open the child’s eyes and the moment they were pried apart a gush of tears came out. Lusterless cornea. The cornea was too cloudy to assess. I looked over the enucleation reports and some of the quotes in those reports were interesting. The iris is exceedingly atrophic and thinned. On section, there was an absence of the canal of Schlemm. We can learn a lot looking back to see where we’ve come from and we learned a lot in 102 years. I’m trying to put myself in the shoes of those doctors back then. This was probably much more difficult to care for then than now. How do we define pediatric glaucoma. Well, you can use this grid to help you. So we’ll start with how is pediatric glaucoma defined, so the middle column. Pediatric glaucomas IOP related nerve damage and two of the following. IOP over 21. Optic disc cupping or progressive CDC increase. The presence of Haab striae. And large corneal diameter. We’ll get into all these. Visual field with reproducible defect consistent with gluco. And progressive myopia and myopic shift out of proportion with the normal growth. You have no IOP related damage in a pediatric glaucoma suspect. IOP over 21 on two separate occasions. Optic disc appearance suggestive of glaucoma. Increased corneal diameter or axial length in eyes with normal IOP and visual field suggestive of glaucoma. Does this patient meet criteria for glaucoma or glaucoma suspect. How do we classify pediatric glaucoma? These are classified as primary or secondary. Primary implies that the abnormalities are limited to the anterior chamber. Secondary means there are other associated ocular and systemic abnormalities from which glaucoma develops as a consequence or secondary to the underlying ocular problem. This is Axenfeld-Rieger syndrome and Peters anomaly, and Aniridia and Sturge-Weber and neurofibromatosis. So word on pediatric glaucoma genetics. Many of these as we’ll get into have associated gene abnormalities. So there are gene-based panels available. The hope is for these syndromes associated with a single gene mutation, this can be a potential site for future therapies. This may be useful for patients who are childbearing years and thinking about building a family. So we can work with these patients and get them connected with genetics to counsel them about how they ought to understand the chances of passing these genetic defects onto their children. So we’re going to first talk about primary congenital glaucoma. So thankfully fairly rare but we do see it. Occurs in 1 in 10,000 to 1 in 30,000 live births. This primary congenital glaucoma accounts for the majority of pediatric glaucomas and 25 percent of pie mare congenital glaucoma cases are diagnosed during the newborn period. 70 percent of the cases are bilateral. More than 75 percent are diagnosed in the first year of life. More common in males and patients who have a sporadic form have a 2 percent chance of having a child with primary congenital glaucoma. More on the genetics. So primary congenital glaucoma, 40 percent of the cases are inherited in an autosomal recessive pattern. And there are gene defects associated with primary congenital glaucoma. The first is a CYP1B1 gene on the GLC3 locus. This gene encodes cytochrome P450. And involved in the anterior segment development and regulation of aqueous humor production. The other gene is LTBP2 gene on the GLC3C and GLC3D loci. This gene encodes with extracellular matrix protein associated with cell adhesion. If you have a defect in that, you have cell adhesion problems. Another gene associated with primary congenital glaucoma is ANGPT1 and TEK genes. The ANGPT1 gene is associated with blood vessel and lymphatic development. Abnormalities there you will have abnormalities of blood vessels and lymphatics which have implications for the drainage area and angle. So it follows that problems with these genes would have implications on patients with problems with the angle. What is the pathophysiology of primary congenital glaucoma? We don’t know the exact pathogenesis. There was a thought back in the day that was perhaps a membrane obstructing the flow of aqueous through the angle but it’s never been identified. This is most likely an arrest of neural crest cells that are responsible for angle development. This photo shows three ways of it developing in the eye and an aberration of any of these ways can result in intra-segmental dysgenesis and most likely glaucoma. What are the features? There is a classic triad. Epiphora, photophobia and blepharospasm. That is commonly tested on board exams and clinically relevant. Other findings associated with PCG include high pressure that results in a number of findings. Enlarged corneal diameter, globe enlargement or Buphthalmos. Increase in axial length are progressive myopia. Breaks in Descemet’s membrane and corneal clouding. Optic disc cupping. These are the primary features of PCG. The picture on the left demonstrates Haab stria and the photo on the right, a significant buphthalmos. When we see a patient comes in and it looks like they have congenital glaucoma. Practice your differential diagnosis. What your differential is varies depending on the presenting symptom. If you’re seeing a patient with corneal clouding, the differential diagnosis is the mnemonic is STUMPED, scleral cornea, trauma, ulcer, mucopolysaccharide steroid acidosis, and Peter’s anomaly, congenital endothelial dystrophy and dermoid. Patients with tearing, a obstruction, conjunctivitis and corneal abrasion, uveitis. A patient with corneal enlargement, it could be congenital glaucoma or megalocornea, axial myoma, craniofacial dysostosis. If you have a patient you’re seeing with optic nerve abnormalities it can be congenital glaucoma or disc pituitary, coloboma, optic nerve hypoplasia. It depends on the presenting symptom or sign. How do we manage these patients? We’re going to get into this again later. We’ll talk about meds and surgeries: But just taking a step back, many times these patients present as infants or babies or toddlers and these patients need an examination under-anesthesia for a comprehensive assessment and the treatment is primarily surgical. Medications can temporize and improve corneal clouding but these patients need surgery. Contrast this with juvenile and adult forms of glaucoma, treatments many times are laser. As far as my understanding is, the angles are developmentally normal usually in these cases so you have more to work with. There is more wiggle room versus a congenital glaucoma, the angle never developed normally. So let’s talk about JOAG or juvenile open-angle glaucoma. This presents between 4 and 40 years of age. Mostly inherited autosomal dominant. The patients present with elevated IOP but not with Haab stria or corneal development. They can have axial elongation and progressive myopia. There is the wall of the eye, the sclera is still kind of malleable but not as malleable and subject to change as the eyes of infants. So that’s why you can have axial elongation but not the corneal enlargement and buphthalmos, et cetera. You can start drops and we’ll get into that but for refractory cases you think more surgical options. So we’re going to talk about the secondary pediatric glaucomas. These are glaucomas associated with different ocular and systemic abnormalities. These are listed here. So let’s talk about Axenfeld-Rieger syndrome. It’s a spectrum of diseases characterized by anomalous anterior segment. No sex predilection. The classical presentation is posterior embryo toxin of the cornea. This is an anteriores displaced Schwalbe line. There is a white ring just if front of the limbus, that’s the posterior embryo toxin. You can have iris adhesions and abnormalities. Abnormalities can include hypoplasia, corectopia, poly cornea or ectropion Uveae. The bottom picture is poly coria. The Axenfeld-Rieger patients can have systemic signs. Abnormalities of teeth or facial bones, like rudimentary teeth or redundant periumbilical skin. I’m not lifting up kid’s shifts to look at their belly button but you can ask if your suspected child has it. Hey, any abnormalities elsewhere on the body and a parent would note this. 50% of Axenfeld-Rieger patients go onto develop glaucoma. Typical onset is middle to late childhood. It’s thought to be due to abnormal development of the angle. It presents later. The treatment is the same as open-angle glaucoma. Angle based surgery may not be possible due to iris adhesions. We’ll get into that later. We’re going to talk about Peters anomaly. This is characterized by a corneal opacification Leukoma in the central visual axis. These cases are typically sporadic and most often bilateral. And the classic clinical presentation is that leukoma, the central corneal opacity with iris strands to the leukoma and absence of the corneal endothelium and Descemet’s membrane and posterior stroma. This is a photo demonstrating bilateral Peters anomaly. Patients with Peters anomaly, 50% have developed glaucoma. And the rate is higher in those with cataracts and corneal adhesions. The glaucoma is thought to develop due to abnormal angle development. You can also develop glaucoma secondary to ocular procedures required for Peters. Treatment for Peters anomaly, you can — many times refractory because the angle is abnormal. There is a lot going on in the anterior segment. If you have a few you can start with angle surgery or trabeculotomy or move onto larger procedures like tube shunts and we’ll talk about that. I’m going to talk about Aniridia. This is characterized by high resistance hypoplasia. They can look normal but then you get into retro illumination and there’s — you see a lot of that red reflex through the iris because it’s hypoplastic. And patients that have almost no iris. So the spectrum is broad. Clinical features are iris hypoplasia. And with or without micro cornea, limbal stem cell deficiency. Anterior polar cataract, optic nerve hypoplasia and foveal hypoplasia. I need the talk about genetics of aniridia. 66 percent of cases are inherited autosomal dominant but the remaining third are sporadic due to mutation of PAX 6. 20 to 30 percent of sporadic cases are associated with the WT1 gene mutation and they have a high chance of developing a Wilms tumor. So it’s really important to ask these patients and families, hey, is there a family history of aniridia. You look at other children and look at the parents. If there is not, you better get an abdominal ultrasound because this — if this is sporadic mutation, then there is a solid chance they can have Wilms tumor. Aniridia can have other systemic abnormalities and syndromes. The WAGR syndrome is autosomal dominant associated with Wilms tumor. And aniridia and GU abnormalities and Gillespie syndrome, which is autosomal recessive. It’s the WAGR syndrome that you want to have your ears perked up for if you are making the diagnosis of aniridia in what looks like a sporadic case. So aniridia glaucoma occurs in 50 to 75 percent of cases. The timeframe is variable. Presents early it’s thought to be due to albumin development of the angle structures. The treatment of glaucoma is similar to other forms of pediatric glaucomas. If you can do an angle-based surgery you should do it. You may need to use larger incisional glaucoma surgeries. But for these patients it’s important to remember clinically and on boards you have to remember to treat or refer out for other associated conditions. Cornea can be associated with limbal stem cell deficiency. Recommend lubrication. Limbal stem cell transplant. Glaucoma, you treat that. WAGR disorder, you better monitor for Wilms tumor or work with the pediatric team. Many of these patients have hearing loss so refer out to audiology and there is an inheritance pattern you work with the patient and parents on family planning. Next is Sturge-Weber syndrome. Encephalo facial angioma ptosis. This is a phakomatosis characterized by a port wine stain. Ipsilateral choroidal cavernous hemangioma and lepto meningeal angioma and cerebral calcifications. They can have seizures and neurological deficits and cognitive impairment. Sturge-Weber syndrome have 30 to 70 percent incidence-over developing glaucoma. Bimodal age distribution, close to 2/3 present 0 to 3 years of age and the remaining later in life. This bimodal distribution probably implies those presenting early in life have an angle anomaly. The second could be due to the later in life could be due to increased episcleral venous pressure. Glaucoma is more likely to occur if any of the following are present on the exam. Cutaneous hemangioma of the upper eyelid, hemangioma, iris heterochromia and choroidal hemangioma. They have high risk of choroidal — tube shunts are preferred over trabeculotomies. And intraoperative considerations, you can consider making scleral windows and work on IOP control before they go to the OR. If you get a high preop pressure and low postop pressure, there is a high chance they will get a corral hemorrhage. Leave a fair about of viscoelastic device in the eye before you finish the surgery to reduce the pressure differential to minimize the risk of choroidal hemorrhage. Next neurofibromatosis type one. It’s the most common phakomatosis. There are type one and type two. Type one is peripheral and type two is — type two is not associated with glaucoma. NF1, 50% of cases are inherited and 50% sporadic. NF1 is a tumor suppressor gene. You’re going to get tumors. So what do you see, Lisch nodules. The iris lumps and bumps in the second photo. You can see ectropion Uvea. Choroidal lesions. Optic nerve gliogliomas and eyelid neuro fibromas and glaucoma. Thankfully there is a low incidence of associated glaucoma in NF1. But if they have a neuro fibroma, the risk of glaucoma development increases. The pathogenesis develops on age of glaucoma presentation. At birth probably dysgenesis. Later in life probably due to the neuro fibromatous tissue or secondary angle closure due to thickening of the ciliary body. Treatment of glaucoma in NF1 is typically surgical. Less successful than PCG cases. I want to talk about glaucoma following surgery for congenital cataracts. Risk factors for patients who develop glaucoma having undergone cataract surgery for congenital contacts are cataracts in the first six weeks of life. Small corneal diameter relative to age appropriate values. And the risk of developing glaucoma is independent of if they are left aphakic or not. Another name for this is aphakic glaucoma. Not exactly accurate. Because a lot of surgeons at least in this country put an IOL in the time of cataract glaucoma. Glaucoma following surgery for general cataracts is more specific and accurate. The pathophysiologies of developing glaucoma after surgery for congenital cataracts. Could be related to congenital anomalies of the aqueous outflow or postop inflammation or it’s not normal in a human to have cataract surgery as a child or baby. Fundamentally altering the ocular anatomy post cataract surgery maybe results in glaucoma. We don’t know exactly. Many times the angles are open. Patients who have undergone cataract extraction — it should say congenital cataracts require lifelong monitoring for glaucoma. You diagnosis a child with congenital cataract and do the surgery and you need to monitor them for life for glaucoma development. You have to monitor for life. So how do we evaluate the pediatric glaucoma patient? First you got to get a good history. Talk to the parents and caretakers and ask about the gestational history. Any intrauterine infections. Did she get regular prenatal care. What was the delivery. C section, vaginal, complications, forceps. How have the patient’s developmental milestones been. Are they meeting them or regressing. A family history of glaucoma, eye diseases, consanguinity increases the likelihood of getting congenital glaucoma. The prior medical and surgical history. Have they been on medications or steroids. What’s their behavior like. Do they act age appropriate compared to their peers and older siblings. Are they irritated. Are they eating okay. Do they navigate a room okay. The history is so important. Do the caretakers notice squinting, tearing, sensitivity to light or things like that. How do we evaluate the pediatric glaucoma patient. Check age appropriate vision. External exam which includes looking at the patient from across the room in the chair and lids. Do they have frank evidence of buphthalmos. Epiphora or blepharospasm. Fake ma ptosis. What is their behavior like, are they playful, agitated. Able to navigate the exam room. Now we’ll talk about what do you look for with anterior segment. We usually perform examination under anesthesia. If you have a lot of 1finesse in the pediatric exam room what do you look for. Corneal diameter is going to be enlarged in patients with congenital glaucoma. If it’s over 11 in a newborn and over 13 mm at any age, it’s highly suggestive of glaucoma. Look for corneal clarify. Are there Haab stria. Check the pressure. Many times we rely on rebound tonometry because you don’t need the numbing drops. During examination under anesthesia, it’s important to check IOP immediately after induction of general anesthesia and before intubation. All of the anesthetics, the medications given by the anesthesia team for putting them to sleep are going to drop the pressure. You want to get as accurate pressure as possible from the onset. I use the tono pen. Normal Pachymetry is 540, 560 that range. If it’s thick, that can apply corneal edema or have implications on IOP measurements. Gonioscopy, good luck doing this on a child in a clinic. This is usually under anesthesia. What’s the normal infant angle? Typically, less pigmented trabecular meshwork and less prominent Schwalbe line and less distinct junction between ciliary body, band, and scleral — compared with adults. In the PCG angle, you have a deeper anterior chamber, high anterior iris insertion. This photo it’s hard to get the images in general but more difficult in children. This is a photo demonstrating a poorly, partially obscured scleral spur. The white line is difficult to visualize because of high iris insertion. JOAG angle is usually normal. You can sometimes see an iris stump. Optic nerve exam is so important in all glaucoma patients including the pediatric glaucoma patient. Typical cup to disc ratio is less than 0.3 and should be symmetric. This does increase slightly until 10 years of age to about — but this disc ratio varies by ethnicity. They are larger in African, Middle Eastern and Hispanic and east Asian decent. Say you start treatment, cupping can be reversible if IOP lowering is initiated before three years of age. So that’s very interesting. Sorry, I just got distracted. So cupping can be reversible with initiation of treatment at an early age. And then patients with primary congenital glaucoma typically on nerve fiber layer assessment have diffuse RNFL loss. That is in contrast to adult forms of glaucoma. It’s diffuse symmetric cupping of the optic nerve head opposed to involvement of the superior and inferior polls. This is demonstrating asymmetry of cup to disc ratio. Other testing you can perform for pediatric glaucoma patients are cycloplegic refraction. You’re monitoring to watch for progressive axial elongation from high pressure. A scan to measure axial length. Management strategies I have with my friends here, part of the standard EUA exam is getting A scans and we compare the axial length to the previous axial length and see has it changed more so than you expect for the change in age. So we monitor that. You can try to do visual field testing in children. Age appropriate. Usually, try to wait until the patient is 6, 7 to try a Humphrey visual field. Play it by ear depending on the patient. And then if you can get nerve fiber layer imaging, that is great. You have to remember the normative database for children doesn’t exist in commercially available platforms. We look at the global thickness scores, the trend, the green, yellow, red, that is a database of adults. So you have to almost just look at the global thickness scores of the different sectors of the nerve and subtract from your brain, like the colors. Because you can get fooled. And look for change over time. What is important and I learned this when preparing this lecture, RNFL thickness for children over five is comparable to adults. If you have a kid over 5, 6, 7, you can look at the colors a little bit. But in general, you look for kids you just have to remember, look at the global thickness scores and change over time. The other thing I wanted to mention on the visual field testing is that there are not really age adjusted corrections for visual field. So if you’re doing a Humphrey, the mean deviation is not really affected. It’s less than one decibel per 10 years of life. There is not much influence that age has on the visual field measurements. So just get it when you can and wait to get it until they can take it. There are other ways to test visual fields in children. There are fun pictures on the internet. But I try to just usually wait for Humphrey. How do we manage congenital glaucoma. We got into this a little bit but we’re going to go into more detail now. Management is primarily surgical in primary congenital glaucoma and early onset pediatric glaucomas. You can use medications to temporize and reduce clouding and that potentially would have enough to perform a lens assisted surgery. Temporizing with medications, keep in mind the age and comorbidities when choosing which medications to start. Drug classes, you can use with caution beta blockers. Like a low dose. You have to make sure they don’t have asthma because that can exacerbate wheezing. We know about bradycardia. And start at the lowest concentration, 0.25 adult dote. Start with pediatric dose. You can do cardio selective medications. Typically, my first line is beta optic. And punctal occlusion. Basically, apply pressure to the punctum 2 to 5 minutes and that will limit the systemic absorption. You can use topical preparations of anhydrase inhibitors. Be careful with systemic preparations. Because these have so many side effects. GI upset, anorexia, diarrhea, weight loss, hypokalemia, sickle crisis risk. I try to avoid those in children. Use topical. You can use prostaglandin analogues in children. Useful in JOAG. Older children respond better than younger. So you must avoid alpha adrenergic agonists in children under two. This is Bromonidine because of the risk of CNS depression. You can get hypoxia, altered mental status. And proceed with caution in children under 6 and look at the weights. Maybe the youngest patient I have on this is 8 years of age. But you just don’t want to run the risk of CNS depression. If you’re starting this, talk about nasal lacrimal occlusion. Drops. The para sympathomimetic agents. There is like a small but pretty — small group of individuals who respond well to this medication. It’s refractory glaucoma cases. Congenital glaucoma. But it does have associated iris cysts, inflammation and the like. It’s like gone on and off the market in our country. Any time our existence is threatened there is group of people that say this is the only medication that works for my patient. We have to keep it available. There is direct para sympathomimetic agents like pilocarpine. It’s not used that often because of the intense dosing schedule. Has associated myopia, risk of retinal detachment. I have seen with prolonged use of pilocarpine, it can cause miosis and they stay that way and makes eventual cataract surgery challenging. I usually don’t use pilocarpine in general in any patient. Rho kinase inhibitors. There is not a lot of data available. Cost prohibitive. I use it as refractory treatment leading um to surgery. Here is a sample, we need to do surgery: You can start it. Congenital glaucoma surgical management. First line is angle based surgery. This includes goniotomy or trabeculotomy. And which surgery depends on how clear the cornea is. If it’s clear, you can get a view for an internal approach and do goniotomy. If the cornea is cloudy despite trying to clear it you can do antibody extern know trabeculotomy. Angle based surgery has a good success rate. 70 to 80 percent chance the they’re diagnosed within a magic window of time, 3 to 12 months. They have a pretty good success rate. You can perform these procedures a couple times. Second line therapy, trabeculectomy or tube stunts and refractory cases a diode laser. I’m going to talk first about goniotomy this. Is an incision of the trabecular meshwork tissue using a blade or needle. You can only do this if the cornea is clear. You have to see what you’re treating. If you don’t have a clear cornea, you can’t do goniotomy. And considerations for this is the landmarks are going to be funky and distorted us if they have buphthalmos. Something to keep in mind. Risk of goniotomy is hyphema, infection, iris damage, lens damage and uveitis. I want to talk about trabeculotomy. This is cannulation of Schlemm’s canal. You can do it from the outside in or ab interna. You can do this or goniotomy. I think the goniotomy is technically easier. Trabeculotomy, you use an instrument, suture, you thread it through the Schlemm’s canal, 360 once you get all the way through, then you can take the ends and pull it and you can basically create an opening. Basically you shred the trabecular meshwork so there is an opening from the anterior chamber to Schlemm’s canal. This is in adult patients we call this GAT. Omony (ph.) is another device used in this country. It’s cannulation of Schlemm’s canal and you can perform a goniotomy. You want to be careful about creating false passage. Something to keep in mind while you’re doing it. The risks of this procedure is hyphema, infection, iris damage, lens damage and uveitis. Say you have performed a couple of angle based surgeries and the IOP is back up. What now? Think about trabeculectomy or tube shunt. There is a lot of things to consider. You have to think about the age of the patient. Success rate for trabeculectomy is low in children under two. Ability to follow up regularly. Mostly I care for adult patients and even with them, very careful about who I perform a trabeculectomy in because the postop Bleb management is intense and requires close follow up for injections. It’s an intense follow up. What is your IOP goal. What are the concomitant eye diseases. Is the sclera thin, you would be hard to make a scleral flap. What’s the lens status? If they are aphakic, you have a lower success rate for trabeculectomy in those patients. And behavior and lifestyle. If there is a history of eye rubbing, that is one example, I wouldn’t go either of these procedures because of the risk of infection. But one thing that I want to bring up at this point for an adult patient I had, she was given a recommendation for trabeculectomy and came do me for a second opinion: I swim a lot. I don’t want this surgery because it would alter my hobbies. So I did something else for you. You have to ask these questions so you can make an appropriate management decision for your patients. Trabeculectomy is where you bypass the trabecular meshwork with a scleral flap. Proceed with caution in young children. If you do this in children under 2, high risk of failure but you also have risk of infection. The anti-fibrotic agents help with the success of the procedure but you run the risk of infection. This, trabeculectomy can be combined with trabeculotomy. The thought is it’s combined procedure, reduces resistance to the trabecular meshwork and enhances outflow. There is a higher rate of success with trabeculotomy versus trabeculectomy. If you’re trying to maximize the OR time, this is a child, a good candidate. Over 2 and things like that. Maybe just do a combined procedure while you’re in the OR and then you may have a higher rate of success. Risk for trabeculectomy is bleeding, infection, mainly blebbitis, and endophthalmitis and scarring and cataract. Let’s talk about tube shunts. This is an implant that shunts aqueous to a reservoir, valved and non-valved. Ahmed valve is one available in this country. The risk of infection in tube shunts is lower compared with trabeculectomy but there is less IOP reduction. When you’re doing a tube shunt, you do have to keep in mind if you’re doing a tube shunt in a child, what’s the axial length. Because you got to be careful you don’t put that tube shunt to close to the optic nerve. So there’s a calculator available, I think it’s via Duke University. It’s the online calculator. You put in the quadrant that you plan to put the tube shunt in, the axial length, the distance you want it to be, I shoot for 8 and it tells you how, if you need to trim the back portion of the plate to mitigate damage to the optic nerve. Risk for tube shunts is bleeding, infection, double vision due to effects of potentially damage to the extra ocular muscles or effect of that, the plate develops encapsulation around it. Sometimes if that encapsulation is prominent, it can abut and effect the extra ocular motility. It’s an implant, so risk of device exposure migration, cataract formation and corneal decompression. It can touch the cornea and cause decompensation. Next diode laser. Reserved for refractory cases. The old school G6 probe is difficult to tie rate. You can use, if you’re going into the eye for cataract surgery, you can perform endocyclophotocoagulation. That’s tie ratable or use this newer more recent micro pulsed laser, the MP3 which we have had close to 10 years in this country. Both the end do cyclophotocoagulation and micro pulse are less inflammatory and more titratable. Less hypotony and inflammation. The risks are pertinent to the cyclophotocoagulation. We don’t use cryo laser much anymore so I didn’t talk about that. Now, what’s the long-term prognosis for congenital glaucoma? I got to it a little when comparing primary to other secondary pediatric glaucomas. But primary congenital glaucoma has a better prognosis than others. Patients have 20/70 or better vision. That is functional. That is great. They can live a close to normal life. Maybe they can’t drive but they can go to school and work and whatnot. Maybe with some accommodations but that is functional vision. The best prognosis for PCG is those diagnosed between 3 and 12 months. These patients do well with angle based surgeries. Poor prognosis for patients with PCG are those who were diagnosed as a newborn, diagnosed after one year of age and those with large corneal diameter greater than 14. When you’re counseling a patient and family on long-term prognosis, watch out for other ocular morbidities including amblyopia and corneal scarring and strabismus. And cataract and things like that. Not just the glaucoma. So how do you counsel these patients and parents? Emphasize the need for lifelong glaucoma care. It’s a long road. You’re counseling the patient and yourself. This is a long road. It’s a marathon, not a sprint. Use whatever phrase speaks to the patient: This is a long road. And it’s normal. You are going to have multiple surgeries in your life to control your pressure but I’m here for you and we’re going to take care of it together. You counseling the patient and you’re also counseling yourself. These are not adult patients this is a different can of worms. Refer these patients for their associated systemic disease. I mentioned earlier Wilms tumor, association with aniridia and others. Make sure your physician, many of us are physicians on this call, we’re treating the whole body. There is more than just the eyeball. Utilize low vision services if available and when needed. We are lucky in Memphis we have a large school of optometry that has a low vision department. So I very commonly refer patients there. And they work with them to optimize their function. So my job is to ensure the disease is stable but you also want to help them see better, help them make the most of what they have with their vision and improve their quality of life. Work with low vision. I remind patients and families that I’m available as a resource to help. That availability, that resource includes communication with other services like genetics. Coordination with school systems to fill out necessary paperwork to develop independent education plans based on visual limitations. Communicate with the employer. This can include in our country filling out FMLA paperwork and disability paperwork. I give it to the patient and their family, hey, I’m here for you: Apart from working with genetics and low vision consultation, any of this paperwork, you need me to fill out, I will do it. Just bring it to me. I emphasize that a lot. So that’s really the bulk of the presentation. I do want to give a final plug and say, you can’t, these patients require a village to take care of. I’m lucky I can comanage them with my pediatric ophthalmology peers. I have wonderful mentors in Dr. David Walton and Helen Young in Boston. Almost every time I have one of these cases, hey this is my plan, I run things by them. They have been encouraging of me in care of these complex patients. For those feeling alone, don’t feel alone, because Cybersight, this platform has free consultation services where you type in a question and send all of the information, I’m one of the mentors available to offer consultation in terms of next steps in patient management. These are complex patients and sometimes you just need to run things by someone else to make sure you’re not missing anything. Please use this tool. Last thing, we’re going to review the poll questions. So question No. 1. What are the components of the classic triad for primary congenital glaucoma. Epiphora, photophobia, blepharospasm, corneal clouding, E, A through C or F, A through D. All right. We got most of our responses correct answer. The classic triad is epiphora, photophobia and blepharospasm. Great. Next question. Which of the following is true? A, primary congenital glaucoma is associated with a defects on the PAX 6 gene. B, the first-line treatment for pie mare congenital glaucoma is latanoprost. C, one should avoid using Brimonidine in children due to risk of CNS depression. Or D, the procedure of choice for primary congenital glaucoma is trabeculectomy. Which of the following is true? All right. This makes me really happy. The correct answer is one should avoid using Brimonidine in children due to the risk of CNS depression. Last question, which of the following statements is true. A, patients having undergone surgery for congenital cataracts only need ophthalmic follow up for the first year after cataract surgery. B, Peters anomaly is characterized by a central leukoma and iris strands. Leukoma is a whitish opacity of the cornea. C, the procedure of choice for glaucoma and Sturge-Weber syndrome is trabeculectomy. And neurofibromatosis type 12 is associated with glaucoma. Which of the following is true? Good. Okay. So right. Yes, the correct statement is Peters anomaly is characterized by central leukoma and iris strands. I meant to go through the other answers but patients who have undergone surgery for general cataracts. I apologize because the phrasing isn’t quite correct in English. Patients that have cataract surgery as children need to be monitored for life. The procedure of choice in Sturge-Weber is tube shunts. And D it’s neuro fibromatosis type one that is associated with glaucoma. Not type two. Great. These are my references. Thank you, I’m available as a resource. I’m one of the glaucoma mentors on the Cybersight platform. I appreciate everyone’s attention. I’m going to hit stop share and what I’m going to do now is get into my Q&A. I’m going to read through some of these questions. Best option for clear — okay, a lot of these we have answered. Congenital glaucoma treatment if there is a hazy cornea, trabeculotomy antibody extern numb. Can we treat closed angle glaucoma with medication? Sure. With the medications I mentioned. Beta blockers, carbonic anhydrase inhibitors or prostaglandin analogues. What a is the visual prognosis for congenital glaucoma. If diagnosed between 3 and 12 months of life, they have good vision. What type of laser do we use? Diode, micro pulsed. I’m not sure about SLT honestly, selective laser trabeculoplasty. Because the angle is anomalous. Can congenital glaucoma be diagnosed in utero. I think I have seen patients with buphthalmos in utero. Hard to confirm what it is but I’ve seen it before in the literature. What role do you think AI and machine learning will play in glaucoma management? I’m optimistic that utilization of AI can help with screening and to appropriately shunt patients or direct patients, high-risk patients to academic centers or glaucoma providers. To help triage the patients appropriately if something can be managed via optometrist or ophthalmologist versus patients that require glaucoma fellowship training. AI and diagnosis and management are beyond the scope of this lecture. Can we give acetazolamide to children? Yes. Don’t strongly recommend it. I call pharmacy. Usually I give it in the hospital. And adult dose is at least what we orally hear is 250BID, 500BID adults. But children, I basically call pharmacy at the Children’s Hospital. That’s a good question. I defer that to a pharmacist so I don’t cause side effects. What is done in visual field testing and A scan. Someone answered this. I agree. Visual field testing tests the peripheral vision. A scan measures axial length which can get larger if the IOP is uncontrolled in children. Thank you for helping me answer that question. Is the treatment of — I have seen for black Africans its may be better to start with surgery. In preparing this lecture, I read my books and articles and I think, I don’t think — it’s hard to be dogmatic in glaucoma care. There is a lot of nuance. My approach to — the corollary I would give is my approach to glaucoma management in adult patients, I think if you can — if you minimize dependence on drops that is better for patients in terms of compliance and side effects. In my adult patients who are amenable to drops, I push for selective laser trabeculoplasty. So I kind of gave you the textbook answer. But if they’re open to surgical intervention, I say you can start with that. Especially if they’re open to it. In your vast experience, at what age does optic disc cupping reversal stop with IOP control in pediatric glaucoma. IOP treatment before 3 years of age you can reverse cupping. But afterwards probably not. What role do you think — someone asked that. Do you take the cornea thickness measurement in pachymetry when you check IOP? Pachymetry is helpful in determines if your IOPs are over or under estimated. People have tried to come up with corrections, like, hey, if it’s this, subtract this. There is no correctable factor. Is the pachymetry thin or thick or average. Well, I’m measuring 21 but it’s thick so it’s overestimated. Or I’m measuring 21 but it’s thin, that is a lot higher. I don’t make corrections because none of that has been proven to be accurate. What type of patient data is used to predict or detect glaucoma? We covered this. Going back to the table I mentioned. You look at IOP, cup to disc ratio, corneal enlargement, visual field, things like that. Part of this was in the childhood glaucoma research network and world glaucoma association grid of how do you define pediatric glaucoma versus pediatric glaucoma suspect. Can PCP be considered first choice in some general glaucoma cases to postpone other glaucoma surgery? I wouldn’t recommend it. It’s very proinflammatory and causes a lot of scarring and inflammation, it recks the anterior chamber. It trashes the conjunctiva almost precluding sometimes trabeculectomy and tube shunts. I wouldn’t unless the patient is essentially blind in that eye and uncomfortable. Then I would do it. What age would be the least to use antimetabolite in glaucoma surgery. If I’m doing trabeculectomy in any patient, I use antimetabolite. There is a procedure called orphan trabeculectomy, it’s a situation where if it’s a temporizing measure you don’t need long-term bleb use or success over time. If thyme doing a trabeculectomy I’m 100% of the time using antimetabolite. You can adjust your concentration. Typical 0.2 mg per ml. You can go down if you’d like. What factors affect the choice of the type of glaucoma drainage device? At least in this region, many of my patients need valved procedures because you want immediate IOP lowering. I could probably discuss this with my pediatric colleagues that do this every day. But I would think putting a non-valve G DD in a pediatric patient wouldn’t be really smart. There is so much nuance to a postop care of a valve device. A suture you might have to lyse around 6 weeks. You’re waiting for this encapsulation of the plate. You can get hypotony. It’s so dynamic of a postop process, I would prefer a valve device in children. Maybe I’m just scared. What factors — we talked about that. Doing A scan to measure and compare the axial length, what time would it be useful from the first time? What is the timing for repeat A scan. Depends on the patient age. There is risks with repeated trips to the OR for examination under anesthesia in terms of prolonged anesthesia. I mean thinking about just a couple cases I have. Some of my pediatric colleagues do. Maybe an EUA every four to six months. Weigh the risk of putting the patient to sleep. Just depends on how concerned you are that the patient can be progressing and that’s very individually determined. Based on patient appearance, behavior, parent’s concern, pressures you’re measuring in the office. I would say maybe in the 3 to 6 month range. Risk factors for trabeculectomy. Infection, bleeding, hypotony, scarring. What is the place for deep sclerotomy in treatment?I don’t perform deep sclerectomy. It’s a compelling procedure for adults and it’s something I can’t answer. But I will have to look into it. The axial length and optic nerve injury calculator. There is a calculator available. The Freedmam-Margetta on the Duke website. You Google search the calculator and you input the patient’s axial length, the tube shunt you’re utilizing, distance from the limbus and desired distance from the optic nerve. The calculator says, this is how much of the back plate of the tube shunt you need to trim to minimize risk to the optic nerve, the tube shunt rubbing the optic nerve. If the patient has a long axial length you don’t need to make adjustments by trimming the back plate. But it’s helpful if you’re working on a baby eye with axial length that is abnormal. So you can input the axial length, and input the tube shunt and the quadrant, desired distance from the limbus and it tells you how much of the back plate you need to trim. How long can we monitor the eye pressure of a premature baby with low birth weight and pathologies with drops? It’s tough because child born low birth weight, you don’t want to — there is risk with general anesthesia. But if the patient has congenital glaucoma the treatment is surgical. Once you get the green light from pediatric service, the risk of anesthesia are outweighed by the need for performing glaucoma surgery, you do glaucoma surgery. You temporize with drops. Topical beta blockers and carbonic beta anhydrase are useful for temporizing. Knowing the three clinical triad as a thing and knowing the no normative database for instruments is another thing. What clinical tests … What would I do for a patient under one year of age. Accurate history, and observation of the patient. Do they have tearing, blepharospasm, photophobia. Is there corneal enlargement or pressure. The pressure is the big thing. There are other steps to diagnosing pediatric glaucoma. Probably hard to get like an infant to a retinal nerve fiber scan. I don’t do those formal tests until they can cooperate in clinic. So utilize your exam skills to make the diagnosis and determine if they need treatment. When you say I can do angle surgery multiple times, how many times do you think is appropriate. And do you mean performing surgery over the same area? If you do this goniotomy, typically it’s ab interna: You do a couple of clock hours. You can go back and performance additional clock hours in a different area. Trabeculotomy. You can do, typically you’re canulating Schlemm’s canal, you go 360. Sometimes it can close. You can bring them back and try to reopen. It’s mainly goniotomy, the visual number of clock hours if you’re sitting, depending where you’re sitting. You can bring them back and open up with goniotomy, open up more clock hours. That is what I mean by multiple times. You treat different areas of the angle. Or if it’s closed and it looked like the trabeculotomy is sealed, you can repeat it. There is not a maximum number of times but maybe like, if you triangle based surgery twice and you’re still getting high pressures, probably time to move on. What drug is the first line choice for congenital glaucoma. Beta blockers, prostaglandin analogues. What is the dose of acetazolamide to be given to children? Call your pharmacist. I don’t know. It depends on birth weight and current weight. Low as possible. In general, if the general condition of the baby doesn’t allow for eye surgery, what medication do you prefer until the time of surgery. Topical beta blockers. You can do goniotomy, a couple clock hours and antibody externa trabeculotomy 180. That’s fine. So it might be tricky to do antibody extern know trabeculotomy in two clock hours because of the way you have to do it. You might need to have visualization of the angle and do ab interno. You can treat however much it makes sense. My default is 360. It implies you don’t have a good view through the cloudy cornea. Treating 360, it comes all the way through the end of the catheter or suture and you pull both ends and you get 360 treatment. All right. Do you consider the rate of axial length increase to assess the glaucoma progression in children? Yes. Sure. Offhand I can’t think of the lengths but if there seems to be a progressive increase in axial length out of proportion of the age expected values, you better windchill the pressure. How common is angle closure glaucoma in children. That’s beyond the scope of this discussion and frankly I would have to look it up. Is there a — I primarily focus on glaucoma treatment in my practice. I can’t speak to the use of atropine and drop related ambiopia treatment. Not sure I can answer that question. What age do you start asking for OCT nerve. The age they’re able to come to the slit lamp. It depends on the child. Many times my little girl patients are more cooperative than the little boy patients. I don’t know why. Usually try around 5 or 6. If they can come to the slit lamp over a period of multiple visits. What are your thoughts on trabeculotomy combined with trabeculectomy. It works well. What anti-fibrotic agent regime do you use. 2 mg per ml. 0.1 or 2 and combine with lidocaine. I would do it the same as the documents unless I was concerned that the sclera looked thin. It’s customizable. I have many cases are large cupping discs with no other signs of glaucoma. How do I follow these patients. It’s so tricky, myopia and glaucoma is tough. There is a different Cybersight lecture on that. It’s weighing the risks and benefits of initiating treatment, pressure lowering treatment, their follow up ability. Can they come for serial exams on the regular to monitor. It’s very, it’s very individualized approach when it comes to patients with high myopia and you’re concerned about glaucoma. Can untreated congenital glaucoma result in nystagmus in the long run. It can result in buphthalmos, clouding, low vision which results in century nystagmus. Do the normal glaucoma assessments on infants, yes. How do you diagnosis glaucoma, you look at the optic nerve, you look at the pressure. Pleasure the corneal diameter. That is usually enough to make the diagnosis of glaucoma. That is based on the chart from the beginning for diagnosing pediatric glaucoma and pediatric glaucoma suspect. Must all tests be done for pediatrics — not necessarily. I gave an all inclusive list of things you want to monitor which include exam. The exam portions are critical. But with regards to do I need to do a visual field. No. It’s individualized. Cycloplegic refraction is important in the pediatric population. Please comment on the management of pediatric uveitic cataracts. We didn’t talk about uveitis or cataracts but uveitis can cause glaucoma. And congenital cataracts can cause glaucoma. So lots of different modalities you can develop glaucoma. You have to be sure to treat both conditions, underlying uveitis and cataract. It’s beyond the scope. What’s the pathophysiology of aqueous outflow obstruction in congenital glaucoma. You tell me. Some sort of anomaly of the angle. So mechanism of action of the cyclo diode laser. You’re applying energy to the ciliary body and it results in IOP lowering. We are out of time. O my gosh, I didn’t mean to go this long. Thank you everyone, I appreciate your wonderful questions and thank you for joining. This will be available later as this recording and if you have any questions you can reach out to me. And please utilize the Cybersight mentoring platform. Thank you, very much.