Lecture: Pediatric Uveitis

During this live webinar, Dr. Tinley will talk about uveitis in children in general, as well as its profile in Cape Town, South Africa. Dr. Tinley will specifically review the new guidelines on the treatment of JIA-associated uveitis: Update of the evidence based, interdisciplinary guideline for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis. Semin Arthritis Rheum. 2019 Aug;49(1):43-55. Lastly, Dr. Tinley will briefly discuss paediatric uveitic cataract and ‘new frontiers’ in uveitis management.

Lecturer: Dr. Christopher Tinley, Ophthalmologist from The University of Cape Town, South Africa.


DR TINLEY: Hello, everybody. Very warm welcome to South Africa. It’s very exciting to see so many of you signed up from all around the world. Yeah. My name is Christopher Tinley. And I’m head of pediatric ophthalmology at a department in one of the largest children’s hospitals in Africa. And I’d be delighted to share my experiences of pediatric uveitis with you. So let’s get started. Okay. So to be quite honest, most of us are very fearful of or at least frustrated by pediatric uveitis. Mainly because there’s so many unanswered questions. Really, the only certainty is uncertainty. And traditionally, the prognosis for children with pediatric uveitis was dismal. Even blinding in 40%. But perhaps we can reach enlightenment, possibly using a systematic, multidisciplinary approach. So our first poll question is this: What is your level of interest in uveitis?

DR TINLEY: Okay. Hello, everybody. Sorry we had a technical hitch there. So… Back to the contents of this webinar. So we’re gonna talk about uveitis in children in general. And then look at recent consensus statement guidelines, as well as touch on uveitic cataract for a little while, and explore some new frontiers. So to start off with, uveitis in children in general — well, it seems it’s not a disease peculiar to humans. And that animals can get it too. And this is a picture of a cat with uveitis. In our unit, we looked at a spectrum of non-infectious uveitis managed in our pediatric rheumatology department, over the last few years, and we didn’t find a huge number. We had an equal number of girls and boys. The median age at the first visit was six years. And in total, 6% of all our JIA patients have uveitis. Of those who had associated diseases, 43% had JIA, of which the lion’s share was oligo JIA. However, we had a large proportion of idiopathic uveitis as well, which are particularly frustrating to manage. Sarcoidosis, HIV-related, and poststreptococcal syndrome make up the minority. So at Red Cross, we’re very fortunate to have a multidisciplinary team to co-manage these patients. We have combined clinics on Tuesday afternoons. The ophthalmology appointment is first. And then the patients move on to see the rheumatologist. We keep continuation sheets of patient data in the clinical folder. And we look after routine blood tests, check blood results, and do rheumatological checks if necessary. Thankfully, the motivation for biological agents and the planning of infusions is done by our rheumatology colleagues, and we’re very grateful for that. However, we work in a financially constrained health system, so there’s a limit on funding for our biological agents. So we have to be very targeted, as far as who we can prescribe them to. This is a copy of the spreadsheet, the continuation sheet we use for each visit. So we enter all the patients’ data for the day down these columns, including what bloods have been done and what follow-up is necessary, as well as the treatment plan here. So it’s possible that our poll questions are what caused the hiccup in the talk. So I don’t know if we want to try it again or just go over to the next slide. But it would be interesting to know if all of you have biological DMARDs available to you. I know in developing countries, some people only have access to corticosteroids, and then your treatment options are severely restricted.

DR TINLEY: So let’s talk about uveitis in JIA in general. So we know that children at risk are those who have a younger onset. Younger age of onset. Those who are ANA positive, girls, and children with oligo JIA. However, we also know that boys do get it as well. As well as ANA patients and polyarticular and psoriatic and entheses-related, and no lab findings consistently find a pattern of disease. So how do we go about screening in JIA? Well, there’s lots of confusion surrounding this. Americans have several complicated guidelines, and they don’t seem to be able to reach a consensus on what to do with JIA screening. The British are simpler, thankfully. And they have come up with a consensus statement, which includes all JIAs, which are screened four monthly, until 11 years old. And they feel that the ANA status does not change the risk sufficiently to determine a screening policy. And this is an easy policy to remember. And we know that in children that present with uveitic eye disease, often they are asymptomatic, and this makes it particularly difficult to pick up at an early stage. Hence the need for screening. Occasionally they do present with red eyes, photophobia, posterior synechiae, and abnormal pupils. And corneal clouding, if there’s a glaucoma issue or visual impairment. If their eye is painful, it often points to an infective etiology or a reactive one, such as poststreptococcal uveitis, which I’ll touch on a bit later. In a young child with uveitis, they may present just with unusual blinking or eye rubbing, visual inattention, preferential attention to auditory signals, or new onset squint. So be on the lookout for that. So I want to spend a little bit of time on this slide, because it’s important in the management of these children. So the nomenclature for uveitis grading changed about a decade ago, and it’s important, because you need to be able to compare your findings from one visit to the next. And in order to do this, you need to be able to use all your superpowers to make sure that the child is comfortable and relaxed for the examination of anterior chamber. So I usually do this by letting the child sit on the mother or the father’s lap, and try if at all possible to get the child to raise their chin on the slit lamp, so you can do a proper slit lamp examination. And then what I do is I turn all the room lights off, so that the room is completely dark. And then sit with my slit lamp, looking at the anterior chamber, and just waiting there for 10 or 15 or even 20 seconds to see if I can start see cells circulating in the anterior chamber. But it’s important to have the room lights dark and all doors closed. At our unit, our routine uveitis workup includes an autoimmune screen. Some infectious serology. Namely syphilis, toxoplasma, and poststreptococcal, as well as an HIV test, chest x-ray, and a full rheumatological review. I just want to touch on this entity, called poststreptococcal syndrome uveitis, which we see occasionally in our unit. Typically, it presents with an acute uni or bilateral anterior uveitis, with a hypopyon. And when you do serology, you’ll see very raised ASO titers, if the child has had a throat infection, or anti-beta-DNAse titers if the child has had a recent skin infection. But it usually responds well to standard treatment protocols. But often recurs when the child is exposed to streptococcal antigens again. It’s unclear if penicillin plays a role in the management of these children, and whether a tonsillectomy removes a reservoir of streptococcus, which prevents recurrences. More commonly, however, when a child presents with uveitis, it’s not typical of a poststreptococcal syndrome, and is more likely to be atypical. But if you find that your strep titers are raised, then you’re not quite sure what to make of them, because they could be incidental. Just because the child has an incidental throat or skin infection. We very rarely see infective uveitis in our children. We have occasional toxoplasma. Toxocara crops up occasionally, but it’s a clinical diagnosis for us, because in South Africa, the Toxocara lab testing is no longer available. I see quite a few children with TB uveitis. Namely an anterior scleritis, a huge iris nodule — all different children — and two children with posterior uveitis. And most of them were confirmed with very strongly positive tuberculin skin test. But if you have a positive uveitis, it makes life easier for us in a sense, because we treat them per infectious disease protocols, and usually they respond well. It’s the chronics and the idiopathics that give us headaches. So here’s a picture of uveitis in a rabbit. With associated cataract. So how do we treat JIA-related uveitis? And this is a template that I want to share with you, which I also find useful in extrapolating to all children with uveitis. Especially the idiopathic group. So I’m gonna skip over this poll question. And hopefully you have access to a pediatric rheumatologist, or a rheumatologist who can help you with the management — the systemic management — of children with uveitis. Because it can be very complicated for ophthalmologists to manage on their own. The paper I want to specifically talk about is a consensus statement that came out in the Seminars of Arthritis and Rheumatology in 2019. And it gives you a very nice stepwise algorithm on how to manage these children. So the aim of treatment in children with active uveitis is to eliminate all anterior chamber activity. You should not be happy seeing any cellular activity in the anterior chamber. So phase I is to institute topical steroid therapy. And these need to be potent topical steroids. Two hourly, while they’re awake, or even hourly, if there’s a lot of anterior chamber inflammation. And then keep them controlled with ointment at night, and do that intensively for the first few days. And then taper over six weeks, according to the response. Use cycloplegics to prevent or break posterior synechiae. And then chronic inflammation, consider tropicamide at night. Just be careful. In unilateral uveitis, dilating the pupil, if they’re in an amblyogenic age group. So what happens if there’s no inactivity on more than two drops per day after three months? Because then you run the risk of developing topical steroid-related cataract and glaucoma. What happens if there’s a reactivation, once you taper the topical steroids? Or if you have new inflammation-related complications? Then we have to start disease-modifying drugs like methotrexate. In smaller children, absorption is adequate if you give it orally, but in bigger children, we have to use a subcut dosage once a week and give associated folate weekly as well. Prior to commencing these medications, you need to rule out any underlying liver disease. Specifically if you’re going to use methotrexate. And if you’re planning on biologicals, you have to exclude underlying infections like tuberculosis, et cetera. It’s recommended that vaccinations be completed more than four weeks prior to commencing the treatment. And critical is to ensure monitoring, and that there is an adequate place for monitoring of compliance and infections or malignancy, as a result of these immunosuppressive therapies. And that’s where our pediatric rheumatologists are exceptionally helpful. Occasionally we reached the stage where, after four months of methotrexate and topical steroids twice a day, there is still no inactivity. Or reactivation or new complications, in which case now is the time to add a TNF alpha antibody. And the one we prefer to use is adalimumab or Humira, as it’s a humanized antibody. And you use a smaller dose in smaller children and a higher dose in children who are heavier. And it’s a two-weekly subcutaneous administration. An alternative to that is infliximab. But the downside of this is that it has to be administered under an intravenous infusion, two to eight times weekly. But it can be very useful if you’re worried about compliance of children and whether the Humira is actually being administered correctly at home. We know that methotrexate needs to be used in combination with biologicals, as this reduces the risk of drug-neutralizing antibodies. If you can’t afford biologicals, then azathioprine is a useful alternative if biologicals are not affordable, contraindicated, or ineffective. We advise against cyclosporine, mycophenolate, and etanercept in JIA-U and sulfasalazine only in HLA B27 associated uveitis. So very occasionally, and if you’re very unlucky, you still don’t get a remission after four months of biological agents. In which case, you have a few options. You can either increase the dose of the biological or reduce the interval between doses, or you can switch between anti-TNF agents. Alternatively, you can use a different biological, targeting a different cytokine, like tocilizumab, abatacept, or rituximab. Rituximab has been associated with immune-related reactions, and so often has to be administered with caution, with steroid or antihistamine cover. Systemic steroids are recommended as a bridging therapy only. So when you have severe anterior chamber inflammation, a dense vitritis, macular edema, or hypotony, and then you can use either an oral steroid pulse or an intravenous steroid pulse. We often use intravenous steroid pulse in our setting. So you use 10 to 30 milligrams per kilogram per day for three days, and you admit the child to a unit, and in the mean time, you can get all your uveitis investigations done, if it’s a child who’s presenting for the first time. We all know that videos have very toxic side effects, and I won’t go into those in detail. But they should be used systemically with caution. Periocular steroids have a useful role to play as a form of rescue therapy, if all else fails, especially if the disease is unilateral. And you can give it subconjunctival, orbital floor, or intraocular. And the steroids recommended are triamcinolone or dexamethasone. And these are associated with fewer systemic side effects. However, you have to consider that anesthetic will be required, and you can develop problems with glaucoma and cataract. Often children respond to steroids with IOP spikes.

DR TINLEY: Okay. Steroid responders. So if you find a child’s intraocular pressure is spiking due to the steroids, then rather treat the intraocular pressure spike than reducing the antiinflammatory medication. And I often think of uveitis as like playing a game of chess. You make one move and then you wait for the disease to respond. So it’s important to change the medicines or adjust the dose just one at a time, because otherwise it’s difficult to gauge the effect of changing each move. A lot of people are concerned about using prostaglandin analogs in uveitis. But generally speaking, they’re okay to use. Be careful in children who have had complicated cataract surgery or in aphakes. Just a word about other steroid alternatives. Fluorometholone does not penetrate well into the anterior chamber, and therefore has no place in the treatment of uveitis. Be careful about using Alphagan or brimonidine in children less than five years of age, as this can lead to apnea, hypotension, and somnolence. Also it’s important to remember that there’s very little evidence for the use of topical steroids in uveitis. And often we think we can control the inflammation by non-steroidal agents, when this is really not the case. If you have managed to get a remission with whatever step or stage of treatment you’ve decided upon, you need to consider a period of at least two years before you can start de-escalating treatment. Two years of inactivity before you can start de-escalating treatment. However, because biologicals are so new to the treatment, there’s very little data to support the abrupt or gradual dose reduction of these agents. What we often do is we stop increasing the dose, according to the child’s weight gain with age, or we prolong the intervals between the biological treatments. Remember, when you’re starting to wean the child off biologicals, it’s incredibly important to do regular ophthalmic screening, to see if the child flares up again. So now we’re gonna touch on some pearls and pitfalls of uveitic cataract. So this is a uveitic cataract in a horse. We’re gonna skip over this poll question. So how do you go about doing cataract surgery in children with uveitis? Obviously we do a lensectomy. But whether to implant an IOL or not remains controversial. There is some evidence to suggest that we should only insert an IOL if the suppression of inflammation can be guaranteed. And that’s not always possible in developing countries. Some people suggest you do a lensectomy and a pars plana vitrectomy, but only if it’s warranted after B-scan. In our practice, we do PPV, lensectomy, capsulectomy, and trim the cyclitic membranes, if we see them intraoperatively. Some of you are lucky enough to be able to visualize cyclitic membranes before you go in, but we don’t have that luxury. So we do it in this manner. It’s difficult to know how aggressive you need to be in infective uveitis, however, because it’s an acute etiology, and you wouldn’t expect chronic inflammation to persist, after the infection has been treated. However, I have been caught out in the past by doing a lensectomy and inserting an intraocular lens in a child who has Toxocara uveitis, and she developed chronic inflammation after that, and I haven’t been able to get her off topical steroids. So there may be an element of the fact that an infected eye is primed to become inflamed afterwards, even though the infection has resolved. Here are some quotes from a consultant in the UK called Clive Edelsten, who is very experienced in the management of children with pediatric cataract. He says: A single aphakic amblyopic eye rarely adds to visual function, but failing to quickly remove a rapidly forming unilateral cataract during the first years of life will not only produce bipolar, but also prevent adequate monitoring of posterior segment disease. The family should be informed that in some circumstances, all one can aim for is an eye of normal appearance. IOLs are very easy to put in, but extremely difficult to take out. JIA-uveitis is an inflammation like no other. With high rates of IOL cocooning, posterior synechiae, and membrane formation. And the drawbacks of aphakia can be trivial, compared to the profound visual loss that may result from complications of IOL implantation. So in our setting, we do a lensectomy, capsulectomy, and PPV. If cyclitic membranes are present, they are trimmed. Perioperatively, we use systemic steroids, 1 milligram per kilogram, which is started a week before the surgery, and we taper them off only after a month, and this is over and above all the other steroid-sparing agents the child may be on. And then it’s important to review the child regularly, post-op. As well as to keep the pupil dilated. Sometimes we give intravitreal steroids as well, but I prefer to use just systemic steroids post-op. So let’s explore some new frontiers, as far as future management of uveitis is concerned. So this last picture is of a dog who’s got a terrible uveitis with hypopyon. We are beginning to learn that there are several cytokines which drive uveitic inflammation. Over and above the TNF alpha cytokine, there are S100 interleukins, which are responsible for generating the uveitic cascade. We are beginning to learn more about the role of the intestinal microbiome in ocular inflammatory disease. We know there’s a community of microorganisms, and all of its genetic material, which live inside of us. And these actually outnumber mammalian cells by 10 to 1. And they live in our GITs. And we’re learning that there’s an association between intestinal dysbiosis and immune system derangement, such as multiple sclerosis, ankylosing spondylitis, and rheumatoid arthritis. Certain bacterial strains can promote T-helper cell differentiation in the gut. And can be highly associated with autoimmune diseases such as uveitis. And interestingly, a fecal microbial transplant has been shown to be curative in resistant clostridium colitis, in large clinical trials. However, there are unique challenges in defining healthy or appropriate donors. We’re beginning to learn about the notion of a gut-eye axis, and that altering the intestinal microbiome with antibiotics can reduce severity of uveitis in mice and that bacterial metabolites can also reduce severity of uveitis. In a case series of chronic posterior uveitis, those with uveitis were shown to have significantly increased populations of fusobacterium and enterobacteriaceae in their guts. However, it’s quite difficult to prove a cause and effect. However, this is further food for thought. So in summary, we’ve explored uveitis in children in general. We’ve had a look at the recent consensus agreement guidelines. Namely, first stage topical steroids, second, methotrexate, third, biological agents, specifically adalimumab and infliximab. And then rescue biological agents, if the first line aren’t effective. We’ve also had a look at the pearls and pitfalls of uveitic cataract surgery, as well as explored some new frontiers. So I think the ending is happy. We really are what we eat. And thankfully, many unanswered questions are now being answered. The prognosis is also improving for children with uveitis. With far fewer ending with blinding disease. And it’s critical to have a simple, practical, and multidisciplinary approach. Many thanks.

DR TINLEY: Okay. So the first question is: How do you manage — extra step in managing uveitic cataract? Would you advocate intracameral dexamethasone injection at the end of surgery? I tend not to give intracameral dexamethasone. So usually managed by our pediatric vitreoretinal colleagues. They sometimes give an intravitreal dose. But I prefer to cover the children with systemic steroids, rather than giving intraocular steroids. The second question is: How do you manage cases who present with late hypotony? And 360 degree peripheral anterior synechiae? So that’s an interesting question. By implying 360 PAS, you’re saying they have closed angles. So do you mean closed-angle glaucoma? Or do you mean hypotony? I think once you’ve got hypotony, you’re really on a losing wicket. And it’s going to be very difficult to rescue an eye, when you have hypotony and 360-degree PAS. So that’s a difficult one. Okay. The next question is: When would we stop screening or follow-up for JRA-associated uveitis in two clinical scenarios? First, a child without signs of uveitis in multiple visits. Okay, in this scenario, if you have no signs of uveitis in a child with JRA, you have to continue screening them four monthly, until they’re 11 years old, and then you can stop screening. But you can’t be reassured by the fact that there haven’t been any signs in the past. The second part of that question is: A child with a history of JRA-associated uveitis, who has been stable without treatment for a considerable period of time. So the child has had uveitis in the past, but has been stable without treatment for a considerable period of time. It would be interesting to know what age group of child you’re thinking of in this question. But as I mentioned earlier in the talk, you should monitor them for at least two years, before considering forgetting about screening and monitoring. So the next question is: Is there need of parenteral treatment for uveitis? I’m not sure exactly what you mean by parenteral. Do you mean oral steroids? Or intravenous steroids? I’m not sure… Are you talking about intravenous steroids, then there is definitely a place for that, and especially when you’re worried about a child with acute uveitis or at first presentation, when they’re presenting with complications, then I often admit the child for a three-day intensive intravenous prednisone, or methylprednisolone pulse. So it does have a role to play, definitely. An 11-year-old girl presents with reports of floaters in her left eye and mild decrease in vision. On examination of anterior chamber, you notice a mild anterior chamber inflammatory reaction and corneal changes shown in figure 8-3. Dilated fundus examination reveals whitish fibrous material in inferior periphery. I’m not sure what the question is here. Perhaps you can rephrase it, Ajay. So HLA-B27 anterior uveitis is anterior uveitis associated with some of the spondylo-arthropathies, like ankylosing spondylitis. And if you test for HLA-B27 and they’re positive, you know this is most likely the cause of the uveitis and you can manage it as such. Usually it involves rheumatological investigation for underlying rheumatological diseases. Okay. I’m glad you asked this question. Do you prefer portable slit lamp in cell detection? Because it’s often very difficult to look at the anterior chamber in very wiggly, busy children. I find that the slit lamp chair is the best for examining children with uveitis, but as I said before, you have to use your superpowers to make sure they’re calm, comfortable, and relaxed when looking at their anterior chambers. If the child is particularly cooperative, you can try using a portable slit lamp on the mother’s lap, but that can be difficult as well. I don’t think you can see the cells that easily in a child with a portable slit lamp as with a proper slit lamp. Remember that if you do see — if you only see keratic precipitates in a child and you can’t convince yourself that they’re cells, the keratic precipitates themselves are a sign of activity associated with the fact that there is uveitis going on. In a worst case scenario, I’ve had to either sedate the child, to get a good look at the anterior chamber, or even do an examination under anesthesia, in severe cases. But it’s very important to be able to examine eyes in quite a lot of detail. So the next question is: Do you use paracentesis of aqueous for PCR, in cases of suspect herpes or TB? So this is something which I find our adult uveitis colleagues do very regularly. They do PCR specimens of the aqueous for viral causes, and for toxoplasma. But I don’t find it particularly useful in my children, and it’s also quite an invasive test to do. Because you don’t want to do an intervention unnecessarily. And very few of the children that present to my unit actually do have infective causes, which is completely different from adults. Where the majority of adults with severe uveitis often have an infective cause, which needs to be investigated.

DR TINLEY: Next question is: Do you think the SUN classification is enough, or do we need a more accurate tool, like fluorometry? Well, I think if you’ve got fluorometry, you’re very fortunate. But most of us don’t have that facility available to us. So I think that… I do think that some classification is good enough. It’s very difficult to remember the classification off by heart. So what I’ve done is I’ve printed it out and stuck it on my notice board in my examination room, so you can refer to it and classify it accurately. And that’s very important. The next question is: What is the characteristic appearance of the iris nodule for TB uveitis? Well, this was a child I saw at the end of last year. And she presented with mild symptoms. But she had an absolutely massive nodule in her iris. Which… We were wondering whether it might even be neoplastic. But we did an investigation for her. But it was just a huge, absolutely huge nodule in the iris stroma. It was not marginally significant in any respect. It was just a massive nodule. And she responded very well to antituberculous treatment. So the next question is: What is the most common cause of acute non-infectious hypopyon iritis? So in my setting, as I mentioned earlier, the most common cause is poststreptococcal uveitis. They’re the ones that present with an acutely painful red eye and hypopyon, and they’re the ones I would classify as typical poststreptococcal syndrome uveitis. We hardly ever see Behcet’s in kids or sarcoid-related uveitis in kids. I can count on one hand the number of kids I’ve seen with sarcoid uveitis. And HLA-B27 associated iritis is uncommon with children in my setting. The next question is: Which form of uveitis is most common in ocular sarcoidosis? I’m not sure, actually. I would have to defer to my adult uveitis colleagues. But they can present obviously with all of the above. I couldn’t give you an answer of which one is the most common, however. So a dense vitritis is when you have a lot of vitreous inflammation. So much so that you can’t view the posterior pole. And if a child has a cataract, you can pick up whether or not the vitreous is involved by doing a B scan and seeing if there’s vitreous opacification in the vitreous. But if a child does have dense vitritis, it’s unlikely that topical steroids will manage to control it, and then you need to start implementing a systemic treatment. A lot of patients wonder… A lot of doctors wonder whether prostaglandin analogs are not contraindicated in uveitis. I’m fairly relaxed about using them, to be honest. I know that they can cause maculopathy and CMO in adults who have had complicated surgery. And in diabetics. But I’m fairly happy to use it when I’m stuck, as far as a glaucoma medication is concerned. The next question is: If you advise leaving the child aphakic, do you advise a contact lens wear after the post-op inflammation has subsided? Yes, ideally. If the child manages… Tolerates a contact lens, then a contact lens will reduce the amount of anisocoria. But in some children, it’s impossible to manage them with a contact lens. In which case you have to use aphakic specs, even though it’s unilateral. And then try and patch them as best you can. But ideally contact lenses would be your first option. The next question is about: How about using intracameral triamcinolone if you’re not performing PPV at the same time? I presume intracameral triamcinolone is an option. I wouldn’t like to comment on that, because I don’t have much experience with that. And I don’t do that routinely. So IOL cocooning is when you have a severe thrombotic reaction of the capsule around the intraocular lens. You have dense posterior capsule opacification, and anterior capsule phimosis, and it contracts, and fibroses the intraocular lens into a cocoon. And that is a particularly difficult situation to manage. And invariably, one needs to go in and take the whole IOL, as well as the fibrotic capsule, out. So this is a complication best avoided by not inserting an intraocular lens in a child with uveitis. That’s poorly controlled. If you do decide to use an IOL, I would always recommend using an Alcon AcrySof lens. They have the best track record in children. Okay. The next question is: How can we view the cyclitic membrane? There are two ways of doing this. The first is by UBM, if you’re lucky enough to have it. And the second way is intraoperatively. So my vitreoretinal — pediatric vitreoretinal colleagues tend to approach the uveitic cataract anteriorly. And they use the cutter, and the aspiration to aspirate the cataract, and then they take the posterior capsule and introduce a light source into the anterior chamber. Sometimes you need iris hooks to pull the pupil out of the way. And then they do deep indentation to look for cyclitic membranes. Before they place the ports for the PPV. And this is important, because if you place a port through a cyclitic membrane, then you can induce retinal breaks, which are difficult to manage. So I’ve had a few children who have developed giant tears after uveitic cataract surgery, and I’ve needed silicone oil. So you have to be very careful about where you’re putting ports, if you’re putting in ports for a PPV. The next question is: Do we follow the same treatment and follow-up guidelines in traumatic uveitis? Well, no. Traumatic uveitis is a completely different condition. And that does not usually become chronic, and responds to the usual treatment with antiinflammatories topically. Like topical steroids, for a few weeks. But it’s not something which tends to become chronic. And so is usually self-resolving. The next statement is that it’s difficult to rehabilitate with a contact lens in a young child. Which contact lens do you prefer? It is difficult to rehabilitate a young child with a contact lens. We use soft contact lenses if we can. In small children. I think most of us have steered away from the use of hard contact lenses, for pediatric cases. But whichever one you have available to you. And usually the soft three monthly contact lenses, like SilSoft. So the next question is: How would you manage pre-op cataract in uveitis pediatric patients? So when you’re planning surgery for these children, I usually bring them in to my clinic a week before. And give an oral steroid pulse of 1 milligram per kilogram, leading up to the surgery. And that you must sustain for a month post-op as well, just to make sure that the uveitis is preemptively controlled, before you incite inflammation with your surgery. Some people say you should wait for three months or quiescence before you attempt cataract surgery, but sometimes your child is forced. If you’ve got a child with bilateral cataracts and they’re non-functional because the cataracts are so visually significant, you need to intervene earlier, but just make sure the child is very adequately immunosuppressed before surgery, if you do that. So again, which IOL do you prefer for uveitic children with cataracts? As I said before, I would be very cautious about using an IOL at all in children with uveitic cataract. Be very, very cautious about using IOLs. They end up causing more trouble than help. So the next question is a good one. You have suggested a stagewise treatment plan. Can we use biologicals directly to avoid steroid complications? I think you have to be careful about using biologicals directly, because they’re not without risks in and of themselves. And also, they’re incredibly expensive. So I think it’s probably more prudent to have a stepwise approach, and remember, you’re not gonna use steroids long-term. Systemic steroids you’re only gonna use for bridging purposes only. And topical steroids you’re gonna use initially for the first six weeks or so, and then taper them down to a maximum of two drops a day, which are unlikely to cause complications. What is the differential of iris nodules in children? Is it common? No, iris nodules are not common in children. But often go hand in hand with granulomatous causes of inflammation in the eye. But that’s something you can read up about. But it’s not common. We see them occasionally. And the only TB nodule I saw, as I explained earlier, was a child who had a huge nodule in the stroma of the iris, which was spectacularly large. We’ve had this question before. I’m quite relaxed about using prostaglandins in uveitis. I think you’ve got to be more careful in adults. Who are more prone to cystoid macular edema. Okay, guys. We’re just gonna take a few more questions and then our time is up. So let’s have a look. So this is a very important question. When steroid responder starts, do you shift them to a weaker steroid? The answer is definitely not. You’ve got to control the inflammation. That’s your first priority, is to make sure the inflammation is under control. What you need to do when you have a steroid response is to start antiglaucoma treatment. And not shift them to a weekly steroid. The inflammation needs to be controlled as your first priority. Okay. I’ll do two more questions. How much toxoplasma do you have? We see it occasionally. I can think of maybe… Three or four or five children I’ve diagnosed with toxoplasma uveitis over the last five years. It’s not common in our setting. And the last question is: Is there any role of ketorolac in maintaining remission or preventing recurrence of anterior uveitis? And the simple answer to that is no. There’s very little evidence to suggest non-steroidals have a role to play in the management of uveitis. So steer clear of them if at all possible. Okay. I’ll take one last question. In poststreptococcal arthritis, do you recommend uveitis screening in those children? There are a lot here in Egypt. No, we don’t tend to screen the eyes of children with poststreptococcal arthritis. Usually if they get poststreptococcal uveitis, they present with an acutely painful red eye, plus or minus hypopyon. Okay, folks. Let’s wrap it up. It’s 4:01. Thanks for joining me. Be safe. And if there are any further questions, you’re welcome to post them to the website. And I’ll try to answer them as best I can. Cheerio.

>> Thank you so much, Dr. Tinley.

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Remaining Q&A Questions

Question Answer
If child a already has cataract and high intra ocular pressure. What can we do? Urgently get the inflammation under control, manage the raised pressure medically, and then do the catarac surgery
Sir how do you trim cyclitic membranes? Do you use vitrectomy cutter? If yes, what parameters? PPV with vitrector, yes. Ask your VR colleagues what parameters are appropriate for your particular machine.
Some of this complicated cataract has thin membranes in front of them what are those membranes and if the eye is soft with cataract with cylitical membrane still you go ahead with lensectomy? The membranes in front are inflammatory. If the eye is soft, then it is likely that the ciliary body has shut down due to uncontrolled inflammation. Get the inflammation under control first before proceeding with cataract surgery.
What is the consclusive indicator for starting anti tubercular treatment? i have patients with serpiginous choriditis with quantiferon and montoux positivity. but physicans dont approve of starting ATT. Agree. I would have a very low threshold for starting anti-tubercular treatment in this case. I’m not sure why they do not approve.
Is there a role of intrvitreal steriods at the time of cataract surgery ? Yes. No harm in giving at the time of surgery, but maintain oral steroid cover for a month post-op.
Do you implant an IOL at the end of PPV and PPL In our setting, never.
Do you have any experiance of using intraVitreal Methotrexate in CME for those who r steroid responders? I don’t have any experience of this, no.
What IOL is best for Uveitic cataract for child? No IOL is best in my opinion!
Is it possible to operate a children with active uveitis if it is not reponding to medications? Yes, but you are run a very high risk of severe post-op complications. Rather escalate the level of immunosuppression if possible.
What management do you suggest in low income setting where you don’t find Methotrexate or biologicals? Then you have no option but to use topical and systemic steroids.
Can you tell me which type of IOL do you use in non JIA uveitis? I don’t tend to put in IOL’s for any type of uveitis. I have used Alcon Acrysof lenses in the past, but even these exacerbate the inflammatory response.
Toxoplasma Gondii. Do you have it there? This is by far the most common children uveitis here in Mexico. We do see it here, but not very often. Thankfully there is usually good treatment available for this!
What are accepted signs of uveitis that continue with the patient after cure from the attack of uveitis? KP’s can become ‘old’ – pigmented and flatter, PS can remain, as well as band keratopathy. Some children with JIA continue to have AC flare, even once their inflammation is controlled.
What about the role of Bekhterev´s disease in pediatric uveitis? We know ankylosing spondylitis is an HLA-B27 spond-arthropathy associated with hypopyon uveitis. It is not common in children in our experience.
Do you do PPV  + Silicone OIl injection for JIA with cataracts? Any indications for Oil fill? We only put silicone oil in if we have induced iatrogenic breaks.
Pediatric  Uveitis may be congenital? Perhaps if associated with neonatal syphilis?
What are your views with regards persistently raised IOP in such patients? Any experience with shunts? Yes, tube shunts are a good option if medical therapy fails.
Is it possible to leave 11 years old boy with intermediate uveitis on 5 mg prednisolone for one year? metotraxate was ceased and only prdnosolone left Yes. 5mg of Pred is a low dose, unlikely to cause side-effects.
Do you give steroid pulse therapy pre, intra & post phaco surgery? Yes. 1mg/kg oral pred one week prior to surgery and then continue for 1 month post-op
Hello, I live in Argentina, it was not very clear to me, should the lensectomy and PPV be performed only with commitment from the posterior segment? Yes, ideally ask your vitreoretinal collegues to help in performing the surgery. If you don’t have access to a retinal surgeon, then use an anterior approach.
What is the limit of ecogaphy choroidal thickness you take to decide perform cataract surgery? I usually decide to perform cataract surgery based on the density of the cataract, not on choroidal thickness.
We see children with unilateral granulamatous uveitis, may be shistozomaisis! Recommendations? We don’t see schistosomiasis much in South Africa, so can’t advise you on this as a possible differential.
Biologic agents are not available in our country They are not available in many countries, so you have to make to with whatever you do have.
Before you start IV steroids, what are your precautions in these pt? We don’t wait for TB/infectious screening results before starting the 3 day IV steroid pulse. The children are admitted to our ward for monitoring, while we await these results. If it turns out that the child has TB, then we immediately commence anti-tuberculous treatment.
What’s the dose of pulse steroid therapy (methylprednisolone) you give in children with JIA? The dose is IV methylprednisolone 10-30mg/kg x 3/7
Comment: the age to stop screening JIA uveitis was set at 11 years, since the feeling is that an 11 year old would be able to verbalise any symptoms and therefore go for screening. Risk is also thought to be highest in the first seven years from diagnosis. This is correct.
How to treat CNV if present? I have not yet seen uveitis-related CNV in a child. Perhaps refer to your medical retinal colleagues for advice?
Any role of hydroxychloroquine? Not in uveitis as far as I am aware.
What is the management for panuveitis? I would give a 3 day IV steroid pulse at diagnosis, while you await for investigation results, and then manage as per standard protocols.
For patients with uveitic cataract, we usually wait for 3 months after a patient is uveitis-free before doing cataract surgery. For children with uveitic cataract not responding to treatment, do you recommend doing lensectomy to prevent amblyopia? 3 months seems a reasonable time to wait it seems from the literature. If they are not responding to maximal treatment, then you can do a lensectomy, but prepare for post-op inflammatory/fibrotic complications!! And don’t put in an IOL.
What about the more sophisticated devices, like inferometry? I’m sure these would be interesting to look at, but we don’t have access to these in our unit.
Timing of surgery of cataract after treatment start? Ideally 3 months after control of inflammation is achieved, but you may need to intervene sooner if there is bilateral cataract and the child is functionally blind.
Future reserach prospect on JIA associated uveitis -treatment targeting microbiome? Absolutely! That is where we are all heading.
Is it common to see herpetic uveitis in children? Not common at all in our setting. Quite common in our adult HIV positive population however.
What characteristics of the KPs determine activity if no cells are seen? ANY new KP’s are suggestive of activity.
I think using of Prostaglandins analogue anti glaucoma drugs will in increase more the risk of CMO formation in Uveitis. Not so much in children. Definitely in adults.
Which lab tests are mandatory for a child who presents with first episode of uveitis? Slide 15 of my talk is on our routine uveitis work-up. https://cybersight.org/portfolio/lecture-pediatric-uveitis/
1) When the eye is going towards hypotony and the cyclitic membranes can not be removed completely; do you advocate putting in silicone oil following PPV?
2) In paediatric VKH cases do you advocate biological right away?
1) I am not a retinal surgeon, so unfortunately can’t advise you on that question 2) I have not seen a case of paediatric VKH yet. Have you? Do you work in the Far East?
Do you prefer to give intracameral triamcinolone in 360 PAS? Not sure I understand this question. Do you mean when doing uveitic cataract surgery? Do you mean PAS or 360 degree posterior synechiae?
Please give me some tips to rule out the uveitic children in rural areas, whom doesn’t have that much hospital or eye screening facilities…without slit lamp how can we find out uveitic children’s? What are all the questions we have to ask the children to rule out the disease. One cannot screen for mild uveitis in small children without a slitlamp examination. In rural areas, you may only be able to pick up severe disease, with cataracts or cloudy corneas due to band keratopathy.
Please kindly summarize the major differences in the Management of Adult vs Pediatric Uveitis. This is an entirely different talk. I think there is a lecture on adult uveitis on the cybersight website. You could compare and contrast using that.
What would be the roll of an optometrists in pediatric uvitis? Optometrists mainly help us managing the aphakia after uveitic catarac surgery.
In hypotony: do you think ppv helps to remove cyclitic membranes and to improve some aqueous production? In hypotony, I am more concerned about active uveitis, with ciliary body shut down, so make sure the inflammation is well-controlled before doing PPV. You may find the IOP recovers when the inflammation is better.
In treatment of Uvitis , why is FML not affective? FML does not penetrate the cornea and reach the aqueous in sufficient levels to control the intraocular source of inflammation.
Which IOL should use in complicated cataract in patient with uveties with posterior syncheia? Preferbly none, but if you can guarantee control of inflammation post-op, then Alcon Acrysof.
What is the further management for anterior uveitis if the patient doest not get respite since 2 to 3 years? Then you need to follow the treatment algorithm and increase or switch immunosuppressive agents.
How do u diagnose the post streptococal infective uveitis? Raised streptococcal antibodies (ASOT or antiDNase), with all other investigations negative.
What are the characteristics of a streptococcus uveitis? There is a slide about this in my talk and an article in BJO 2012. “Post-streptococcal syndrome uveitis in South African Children”
Sulcus implated IOL – should we explant it if the patient develops progressive bsk? No. If there is progressive band-shaped keratopathy, first make sure the inflammation is controlled and then do EDTA chelation of the cornea.
What about diabetes and paediatric uveitis? We hardly have any children in our unit with diabetic retinopathy, and certainly none who also have uveitis.
How do you manage recurrent anterior uveitis in a case of hlab27 jia , previously managed with immunosuppressives? Have you tried sulfasalazine?
In your practice do you modify the diet or use broad spectrum antibiotic to alter the gut microbiome in managing uveitis? (gut-eye axis) Not yet, but this is definitely a prospect for the future.
Post PPV + capsulectomy. Do u do SFIOL at another sitting or same sitting? Scleral fixated IOL’s are possible to do in children, but I would not advocate this in a uveitic child. A contact lens or aphakic glasses are so much safer.
How long can we give steroids in children without growth retardation or other systemic side effects? They will get weight gain and Cushingoid almost immediately. Growth retardation and diabetes come later, so try not to use oral steroids for more than 3 months.
Do you so complete pars plana vitrectomy as it is sometimes hard to do so in children and lift the posterior Hyloid? Our VR colleagues restrict it to a core vitrectomy if they can’t induce a PVD.
Is treatment with antibiotics with anti-inflammatory in cases of an infectious posterior uveitis done concurrently? (e.g. toxocariasis and or toxoplasmosis infection) Yes.
Would you raise the topical steroid dosage when starting PG analogs to decrease possible reflare or increase in cells? No. Increasing the topical steroid dosage may worsen the glaucoma.
In poor socio eco conditions, can we go for cataract surgery with intravitreal triamcinolone at same setting as patients are not compliant to oral therapy? You can yes, but what happens if the inflammation flares once the triamcinolone is absorbed?
I had a child 10 year old. Acute onset unilateral total cataract. Ultrasound b scan had vitritis. Anterior segment was normal though. Other eye clear lens with gd1 vitritis.

No systemic complaints. TB ruled out (montoux and chest x ray -normal). Complete blood count normal.
TORCH was postive fr herpes.

He’s been operated for cataract with IOL under steroid cover and fundus postop has only gd1 vitritis, no other findings. he has taken oral antivirals fr 1 month. The picture is still the same. He still has gd1 vitritis with normal ant seg. Please suggest where i am missing and next step now should be what.

Were you able to take an aqueous or vitreous specimen during the cataract surgery for viral PCR? This would have been useful. If there is mild ongoing vitritis and the child’s vision is good, I may just monitor closely. If there is visual impairment or worsening vitritis, then you need to immunosuppress.
Do you routinely use TB quantiferon gold in suspected TB uveitis? No, we do CXR and Mantoux or gastric washings in our children with suspected TB.
9 years child with only few snowballs in vitreous with floaters symptoms. Do you treat? How long is the history? If the vision is good, I would probably monitor closely.
How many millimeters behind the limbus do you use for PPV in children? There are normograms for port site placement in children, depending on their age.
We are managing hypotonus JIA uvitis with 5000 cS silicone oil longterm. We are a bit short on follow ups of such cases though. What is your take on that sir? We have not yet had to manage hypotonous JIA uveitis and unfortunately have little experience with silicone oil.
Patient using IMT in times Covid 19. What should I do? Continue if possible, but omit an infusion if they develop viral symptoms.
Bilateral complicated cataracts: can we leave child aphakic & give aphakic spectacles? SFIOL later as child reaches teenage/adulthood. Yes. Good plan.
I have seen pars planitis more often in children that live near mining area. Could water be a cause? Interesting theory. You may have to do an environmental study to look at that!

April 2, 2020

Last Updated: October 31, 2022

2 thoughts on “Lecture: Pediatric Uveitis”

  1. I tend to handle children with uveitis/iritis a little differently than adults. In a child, unless I think it is a traumatic iritis, I would at very least obtain RF and ANA labs since there is such a high rate of anterior iritis (frequently with posterior synechiae) associated with childhood rheumatoid arthritis which may be undiagnosed. The other most common form of uveitis that I see in children in Pars Planitis but I can usually see characteristic vitreous cells, vitreous condensations and inferior “snow-banking”


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