 Next, we're going to hear about the peculiar pediatric Pablo Dama from our own Rachel Patel. She's currently winding up her stint as a resident on the pediatric ophthalmology service. We'd love to have her on the service. We'll be sorry to see her go, but it's what happens. And I'm glad to get her up here to speak in grand rounds before she leaves us. So I just wanted to talk about a few cases that we've seen recently over the past few months. When I was asked to do this talk, I was looking in the list of interesting cases that I have. And there was a whole series of kids with papillodema. And I said, well, this is fascinating. I don't see this that often. But apparently, each resident collects things on call. I've apparently collected pediatric papillodema occasions. So I'm going to talk about three different cases. And I'm going to go over them fairly quickly. We don't have a ton of time. But then at the end, I'll have a little bit more of a discussion about some things to take away from what I got out of seeing these kids. The first one is an infant with sudden onset, esotropia. And so it's a nine-month-old girl. She's super cute. Unfortunately, I have no pictures of her. One week ago, she had some fever and vomiting. It went away. But then four days ago, she had her left eye turn in. And this was originally intermittent. And then for the last three days, it's been constant. She had a head ultrasound because she still had somewhat open fontanelles. And the pediatrician found that it wasn't a great view, but everything looked totally normal. And she was otherwise doing fine in terms of her visual and medical development. So on examination, she could fix and fall with both eyes. But she did have an abduction deficit in the left eye. She had about 60 prison adapters of ET by Krimsky and a very mild, hyper-opic, psychopathic refraction. And on dilated examination, her nerves looked like this. These are not actually her nerves. But they were very similar in that she had bilateral discodema. So to answer Griffin's question, to image or not to image, I think we're stuck imaging with the papillodema. So she got admitted. She had an MRI. You can actually see the esotropia on the MRI. But other than that, her MRI was fairly unrevealing. She also had a lumbar puncture. Her opening pressure was off the charts of what a pediatric little scale for opening pressure would be with over 38. But she didn't have very much else on her LP. A white blood cell count of only one protein was fine. There was no infectious etiology that was discovered there. So at this point, fairly not a whole lot of contributing information here. But just some thoughts about what a differential for this kiddo could be. Any thoughts? OK, so the ones that I had. IH. I'll have people on cases 2 and 3, sounds good. There are cases of IH have been documented in kids like one-year-olds. That's a possibility. Menegitis, her profile didn't look exactly like that from her CSF. Leukemia, of course, when we worry about leukemia in kids who are especially that age, some other ones that are less likely that I'll get to later on. But in conclusion, we kind of came to the thought that this is presumed like post-viral meningitis. She was fever and vomiting a couple of weeks ago. It did get better. She then had the intern in the eye. So she was then, she did have more of an extensive work up when she was admitted by neurology. And then she was discharged on a diamox taper over the next few weeks. She was patching the right eye just to make sure that her left eye was still being used, even though it was permanently interned for that time. And then one week later, her isotropy had completely resolved. Her optic nerve edema had almost entirely resolved. And she was doing great. One quick interesting thing about infants with elevated intracranial pressure is their head circumference. I don't really think about this, because most of the people I see with elevated ICP have fused fontanelles. But she had a six-month well-child check. A few months ago, and had been in the 60th percentile for head circumference. And then right before admission, she was up at the 95th percentile. So there are other signs that we can measure in these kids. OK, so I'm going to come back to some thoughts about her case later on, but I'm just going to jump ahead to the next one. And admittedly, I did not actually see this kid. So thanks to Tina Mamelis for helping me out with this one. So this is a kid with an ectopic pituitary. He's a 12-year-old boy. He came in for evaluation for blurry vision for the last two or three months. He has a history of obesity and this known ectopic pituitary gland that resulted in growth hormone deficiency and hypothyroidism. And he had been off growth hormone for the past nine years and was just restarted on it two months ago. So he is on that growth hormone and then an astrosol. And he says, I have no pulse feltenitis. I just have some occasional transient visual obscurations on my left eye and that's it. So his vision was actually excellent in both eyes. He had no APD, full color vision. He was orthotropic with full motility. Blood pressure was normal. And then on RNFL, he has grossly swollen nerves. Segmentation is pretty bad on that RNFL there, too. So thoughts about him. OK, differential. There are lots, what do people think? Very true, could be. It's a 12-year-old obese kid. Oh yeah. OK, what else? Of course, exactly. He's obviously got pituitary stuff. What if he has adrenal problems as well? That can also cause elevated ICP. Brain tumor is on there as well. And cerebral venous sinus thrombosis, of course. So he had imaging. His MRI was just done recently. And he, of course, has an empty cell because he's always had an empty cell. But he also had a read which showed there's a little bit of narrowing of the transverse venous sinuses, but there's normal flow voids. There's no collusion in any of his vessels. So he was diagnosed with growth hormone-related intracranial hypertension. And this is kind of an interesting entity because it's often, in kids, within the first couple of months of them initiating treatment with growth hormone. And this kid had been off of it for a long time. He was just restarting it. In one series of 23 kids who had growth hormone-related intracranial hypertension, when they all had their first eye exam, they all had papillodema. So it correlated their ICP, often resulted in papillodema. And then interestingly, when they stopped their growth hormone, their papillodema and their symptoms go away pretty quickly. And so in his case, just a few days after C-sync therapy, he's already started having some subjective improvement. And then he's going to be seen later this week. And we'll see how his vision is going from there. OK, last case, this guy. So it's an 18-year-old. He has a history of Bell's palsy, two concussions. He was weightlifting. And then shortly after that, he developed right-sided weakness, tingling, and aphasia for 30 minutes. His parents flipped out, brought him to the ED. He had another episode of headache and aphasia in the ED. And so he got TPA. He had a normal CT, MRI, TTE with bubble study. And he was discharged on amatric, lean, and sumentript. And this was outside of the university. The next week, he had intermittent headaches. He kept having this tingling. He was constantly vomiting. And then he developed laryvision. And so another ophthalmologist diagnosed him with bilateral dyskidema. So he got an LP. Opening pressure was 29. Interestingly, he had a white count of 122. His protein was also high at 99. Glucose was fine. He was started on a pseudazolamide and two pyramids. And then he was referred to neurophthalmology. So when we saw him, he had 20-30 visions, right eye, 20-60 on the left, no APD. He's had some estropia that was more prominent in right gaze, but also in left. And his nerves actually did look like this. Pretty dramatic. And then let's see. Catherine, do you want to read this macrosyth for me? Yeah, absolutely. So dramatically, yes, you can see the swelling of the nerve over there. And then there's a little bit of intraretinal fluid there and then some subretinal fluid as well. And so he was seen by a retina specialist who said, you know, although there is subretinal fluid, this is most likely spillover from the nerve. Probably not something that's actually coming from the macula itself. He had enlarged blind spots on his visual field. And he had an extensive million-dollar work up. The abnormal parts were an ANA and RPR, an alignment of audio screen. The second two were thought to be biologically false positives. He had a repeat opening pressure of 33. His whites were again elevated. His protein was again elevated. He had a CTA head and neck, which was normal. And an MRI which showed maybe some subtle enhancement in the ninjies and some fluid in the optic nerve sheets that otherwise was fairly unremarkable as well. So his is a little bit more complicated, but differential for him. I'm not going to ask anyone to do this at this point. But by the meningitis, he's, of course, got the white count. But all of his infectious workup from the CSF was normal. Handle. Neurosophilus, of course, Lyme norobereliosis. Someone threw out the idea of VKH at one point, but this doesn't look like VKH. So the diagnosis that we came to was this headache with neurologic deficits in CSF, lymphocytosis, or handle. And for a full discussion on that handle, I will refer you guys to Tara Hahn's presentation from last year, which was most excellent and goes into this in more depth. But just to tell you a little bit more about it, it's a syndrome of episodic headaches, transient neurologic deficits that tend to come, they're temporarily related to the headaches which tend to come in clusters. And then a CSF with an elevated white blood cell count, but there's no, nothing on MRI or any other diagnosis that would explain this. And so it is kind of a diagnosis of exclusion here. There can be some, leptomine angiolansin, but that's about all that you can have. It's typically in young to middle-aged adults, so this guy does fall in that age. 15% of them do happen in kids though. They often have an elevated opening pressure, and they can, but don't always have papillodema. And they spontaneously resolve with supportive therapy over the next few months. So in his case, he was given diamox, he was eventually tapered off of it, his vision improved, he ended up being 20-20. He did have residual distortion, of course, which is most likely from this outer retinal irregularity from the leftover edema. So just some closing thoughts about dyscadema in kids, in particular papillodema in kids. The differential for this, although I've been bugging guys about it all day, is pretty broad. So there's of course primary, like IAH, idiopathic, and then there's all these secondary causes for it. And there are some that are more common in kids, so of course, I didn't show you a case of this, but like hydrocephalus or cerebral venous thrombosis, kiara malformations, as we've kind of seen as well, tumors, leukemia, and then a whole bunch of meds, including growth hormone, not to freak anyone out, but at one tertiary referral center of the kids who came in with papillodema from a secondary cause, over a third of them did have a brain tumor. So it is there that we do image these kids for a reason. So does papillodema correlate with actual intracranial hypertension? And so this was actually interesting. There was an intracranial hypertension registry in the Pacific Northwest, and they enrolled 203 pediatric patients. And they found that papillodema was present in 89% of those who had IAH, and about 78% of those who had secondary intracranial hypertension. So there's a strong correlation with it. And then normal opening pressure in kids. So when the first kid was sent into the hospital, neurology called me and said, I don't even know what we're looking for for an opening pressure for her, like in a nine month old, what do we do? And so there are studies that suggest that for kids less than eight, a normal opening pressure should be less than 18, whereas if they're older than eight, it's closer to adult values. But actually recently there was an article that was a prospective anesthesia study about determining a reference range. And they said 90th percentile for routine LPs and below should be considered normal. And they found that for kids who are sedated or obese, less than 28 centimeters of water was considered normal. And if they're not sedated and not obese, then it would be a little bit less than that. Okay, so finally, last closing dots, pearls. MRI with and without contrast, of course, super helpful. And kids maybe like who have bilateral dyskinesia, you wanna make sure it's not also bilateral optic neuritis and with and without contrast could evaluate for that. Venus imaging is also super important because there of course is the possibility for occult venous sinus thermosis, even in kids who don't necessarily have symptoms making you think that that's the number one on the differential. You don't wanna be missing that. Don't forget to check blood pressure that can also cause dyskidema, of course, even in kids. And kids also take medications, so make sure growth hormone, retinoic acid, tetracycline is all on that list of things that you should be reviewing. All right, thanks to these people who helped me see these patients. And I'll take any questions. Thank you.