 So, I'm going to present a case of a kid I actually just saw in clinic last week, Tuesday, and the kid came in and I kind of started to discuss it quickly with Bob, and he said, oh, you should present that case. So, this is a six-month-old kid who was in my 9.30 slot last Tuesday, came in because he had some left orbital swelling. He had been treated by his primary care doctor with a course of augmentin. He'd been on that about two days. Mom felt like it got worse, and she went to the emergency department where they saw him and said he has a little bit of injection of his conjectiva, some swelling with erythema of the upper lid, thought the pupils were equal and reactive to light. They thought he looked around, looked fine, told him to go home and come back and see his primary care doctor in two days, or follow up with them if things got worse. But nothing got better, and so they were a little bit concerned and talked to their primary care doctor and decided that they needed to come see ophthalmology. His past history is pretty unsignificant. He was born full-term. There was no family history of any eye problems, no past medical history. On exam in the clinic, he would follow things with his right eye but would not follow anything with his left eye and got mad when you covered his right eye. His pupil on the right was round and reacted. The left I had a hard time seeing, but it certainly didn't seem to move at all. His pressure was normal in each eye, which I was able to get with the eye care. He had pretty small palpebral fissures. He was a half Asian kid, so he didn't open either eye a lot, but the left eye barely opened at all. The anterior segment exam on the right eye was clear. When I could get the left eye open, he had a very, not a very, but he had a hazy central cornea there, and it was difficult for me to look at the anterior chamber. So at this point, I was a little bit concerned that he had kind of a fuller, it didn't look so much like a swollen orbit to me, but it made me concerned that mom was saying that it was swollen. What's going on here? That in addition to an eye with a cloudy cornea that I can't see to the back, couldn't really dilate the pupil and the pupil was unresponsive. The bad thing that I was really worried about was this retinoblastoma with extraocular extension, and that's why he's got some orbital changes. Mom said it kind of comes and goes. I looked at her phone and it didn't look like it, she said it wasn't there right after birth, but it had come later, and I was wondering if maybe this could be something like a lymph angioma, which has kind of a course that comes and goes, but that didn't really go with the cloudy cornea. Could this be a tumor like a rhabdomyo sarcoma, something else that's very worrisome, but always thinking of? We see these commonly, but that also wouldn't go with the cloudy cornea norwood congenital ptosis. Always think about congenital glaucoma in any infant you see with the cloudy cornea, but his pressures were normal and that kind of rules that out in a kid. Or is this a retinal detachment with the pretisical eye and that maybe it's not really a swollen orbit, maybe it's just a small eye that doesn't open up as much. I was thinking about those things while I was letting the kid dilate, and I took a brief look at the dilated exam and that right eye and thought it looked pretty normal, but was quite concerned about the left eye. I couldn't get a view at all, couldn't get that pupil to dilate at all, and fortunately that day I was at primary, so I was able to send him over to see Dr. Harry for an ultrasound, which showed a total retinal detachment. And interestingly, an axial length in the normal eye of 20 millimeters and an axial length in the left eye of 14 millimeters. So I thought, oh, this is PHPV. This is a smaller eye with a retinal detachment, you know, it just maybe a little bit of a, and the eye is smaller, so it just doesn't open as much. This, you know, doesn't have anything to do with it being swollen at all. And kind of started to talk to the family a little bit about it. They were understandably quite upset. But I also recommended that we do an exam under anesthesia just to get a really good look at the right eye to make sure we weren't missing anything else, but I was pretty sure this was what was going on. They were quite concerned that we, you know, although there was a retinal detachment here, that I didn't think we could fix that retinal detachment. But I said, let's just get an EUA. I have OR time tomorrow, and then we can have a better idea of things. So we put the kit under anesthesia here, and these aren't the greatest photos, but the right eye has a nice clear cordia. The left eye and a normal anterior segment. The left eye here, there's a haze here in the middle. The pupil is, you know, about a millimeter big. And the iris is all the way up to the endothelium. And I think that's why the cornea is cloudy. It's just started to... The anterior segment doesn't look particularly... No. No, no. The anterior segment actually looks pretty normal. It's just that there's no AC. It's about a half-millimeter difference in corneal diameter in the oil. Yeah, no. It's attracted to pressure. Yeah, it was 17, normal. Yeah, pressures were normal. And we re-checked it in the OR, and it was even less. It was like 13 and 11 or something like that, very normal. I took a picture of the kit under anesthesia, and you can see that this was at the end of the case. But apart from the lid speculum marks, it doesn't really look like a swollen eye. It more looks like, you know, the orbital swelling was just kind of a red herring. This is just a smaller eye that doesn't open as much. We also did some electrophysiology and electro-ERGs here. And you can see that in the right eye, looks pretty normal. Single flash here in the left eye, almost nothing. And VEP is very abnormal as well. So at this point, we kind of looked around inside the eye and taking a little closer look now that the kids are under anesthesia. And, you know, Bob and I both took a look here. And we could see that there was this depigmented area here, which made us a little bit take a little pause and think a little bit more about what's going on with the retina. Also, if you look a little more closely here, you can see that the vessels don't seem to completely go all the way out to the edge. But at this point, we were still kind of thinking about it and kind of still thought it was PHPV until we got in the fluorescein. Glenn was nice enough to come over at the very last minute and do a fluorescein for us. But you can see very nicely, which, I don't know, you can't, the lights are on a little bit here, but this showed up much better on my computer. Yeah, if we can hit those lights off here. Oh, this picture up at all. Oops. It shows up much better on my screen here. But the vascular retina ends right here. And this all branches out into fans. Which showed up much nicer on my computer here. But pretty, and as well down here, you can see a little bit. It just ends here. Very dramatic. Very dramatic. With your vascular areas in that eye. So pretty clearly looks like something like fever now, which we were completely thrown off until we did this under anesthesia. There's no leak. These are the later frames here at two minutes. There doesn't seem to be any leakage. It's just all peripheral abasca retina here. You can see it just kind of fans here into a ear pattern looking here. So very glad you can also see it here as well. We put this kid under anesthesia to kind of answer that question a little bit better. So just to talk a little bit about fever, which is something I didn't see so much in residency or fellowship, but I've seen a couple of cases and picked up on a few cases as in attending. It's characterized by peripherally vascular retina in patients without a history of retinopathy or prematurity. Really, it kind of looks like ROP. Can be very asymmetric and has variable patterns of inheritance and penetrance. This is actually one of my patients here who had kind of a similar story born with retinal detachment in the other eye. This eye actually was initially doing much better and then just suddenly took a turn for the worse when the kid was a couple of years old and started pulling more and more and more although she had many times been lasered. So certainly can have a course that's very unpredictable and can change very, very rapidly. There's a paper in Ophthalmology in 2014 done by Mike Tracy's group that suggests that the prevalence of fever is quite underestimated. They looked at 74 subjects of 17 families and found that 58% of asymptomatic family members had stage one or two fever and 21% had stage three, four, or five. So our patient would be a stage one just because they just have peripheral vascular, avascular retina. Stage two is when they can start to have some exudate and a little bit more concerning. So 35% of these asymptomatic people had stage two which is kind of that stage that can really take a turn and start causing a lot of problems in terms of retinal detachment and macular dragging and things like that. So the question for me is how do you pick up on these kids when you see them in clinic and when can you identify them? I mean, this is one of the few retinal dystrophies that we can do something about and we can prevent vision loss. So what are things we look for? We can look for straightened vessels. We can ask about a family history but that's not always so helpful unless it's positive. If it's negative, it doesn't really help rule this out. We can look for some vitreous opacities or things. This is actually one of my other patients who was seen by another pediatric ophthalmologist in town. He had a little bit of nystagmus, had a Chiari I malformation, had actually seen Neuroop at one time for his nystagmus and his Chiari and questions about whether they wanted to decompress that. He came to my clinic because the other pediatric ophthalmologist, he had a head turn and they were considering doing a Kestin bomb so they came to me for a question of whether we should do surgery for the Kestin bomb and I said, well, what is your ophthalmologist or what have people said in the past about that tuft of vitreous that's in this, you know, right over as an optic nerve and the family said, well, we don't know what you're talking about. And I sent this patient up to Emmy and it turns out this kid has fever and the nystagmus was probably due to the Chiari and he probably had both things, but he ended up getting some laser in the periphery. Vertical stribismus, I have a couple of fever kids that have kind of funny vertical stribismus, but we often see that too in kids who are dragged in RLP. So these are kind of some things that I kind of think about when I'm looking for these kids. The other thing that, you know, is a huge part of our exam, sometimes the biggest part of our exam is the retinoscopy and so I always kind of think about when I see kids with really high refractive error, oh, sorry, this should be mine, yeah. Do these kids have fever? I have a couple of kids with fever who have really asymmetric, you know, this is a case report that was actually written by my mentor, Boston Children's, where they was published in 99, but looking at, you know, this kid who had some anisomatropia and this is actually the eye with the worst fever and they conjectured that maybe the disease could be associated with this axiomyopia. I have seen this in a few cases. I have at least one kid who's a minus nine with fever and one eye and nothing in the other eye, but I also, you know, this other kid here is, I showed as a, you know, Plano in each eye, so certainly doesn't always have to be that. Also high myopia, kids with really high myopia, I kind of wonder about that as well. This is, there was a Taiwanese study that was published in 2002 that looked at kids and said that all of them had, it looked at nine patients actually with fever that they saw consecutively in their clinic and all of them had myopia greater than five diopters by age eight. This is another kid I saw who came into my clinic just after failing a vision screening and maybe a little bit of straightening. This is from his first EUA, but he was 19 months old and had this refractive error. Also had a little bit of exudate here and I sent this kid up to Emmy and ended up having fever as well. So bottom line is I think it's a lot more common and I think sometimes we miss it and I think it is something that we can do, something about some of these really sad cases like it can cause devastating vision loss, but I think we're missing some of these cases. It's good, you know, this is the, one of the reasons I'm so glad we have our pediatric rep and service and Dr. Hartman, I mean the issue with this case that she presented is that without knowing the diagnosis, this child's only eye is a significant risk of vision loss. We're gonna go back and do a combined EUA for pediatric rep service. That eye will probably get laser treatment to the abascular retina. So this is making a big difference and these are cases that was tough to sort this out that we likely would have missed years ago and so I think that this is a clear case where having a pediatric retina specialist has brought this disorder into our consciousness and made us a lot more aware of it. So do you have questions on the left eye? Yes. Is it feeding, this was bilateral fever or is there a certain PHPB in the other eye? No, I think it's- Is fever sometimes associated with that mark difference in regards to overall globe size? I don't know the answer to that. I mean, once you have the rep, this is an eye that probably didn't thrive, it had a retinal detachment very early on in vascular- We don't have to affect globe growth. That's what I attributed the very short eye to was that it probably, if we don't have a prenatal ultrasound, what would be fascinating is that they had one to go back and look and see at what point in development- So this thing could have- That retached potentially even in utero. Oh, I think it did. Yeah, I always did. I think it did. That's what we would have- It was a- The size difference, too. I think it's a pretty tight funnel in there. I think it's been there a long time. So the other thing you're telling is you look at these signs, but it sounds like in these mild cases, the only way you can know for sure is a forcing angiography. A mild one may not, right? No, you're correct. That's right. And if you hadn't had a forcing angiography- No, and I almost didn't. Because to me, it looked pretty like a- Like it looked like a PHPV to me, you know? And that with, especially with the globe size, I almost didn't do one. But then I just kind of been thinking, and I've seen kind of, I have a collection of these fever kids now, and I'm thinking about it more and more. So, yeah. You're right. Because even in the ore, looking at the peripheral retina, she and I agreed something isn't quite right here. Let's get an F-A. And the F-A, night and day difference. So- It was just that one depigmented area. And then the vessel, it just looked a little funny, but not really straightened. I mean, you can, it didn't, it's not advanced enough. It's just a little salt. Yeah. Okay.