 Making sure this is on correctly. Thanks so much for giving me the chance to talk to you this morning and to share with you an interesting case that I saw when I did my neuroophthalmology month back in November. Hopefully there'll be some interesting pictures and we'll be reminded of some things that you already knew. So to start our case, I'm involved, no surprise, a 12-year-old girl. She was a right-handed female, which I'd like to talk about in neurology, who presented with the afterword. It's fallen off. Yeah? OK. Three to four months of redness and swelling of her left eye. She had, in talking with her a little bit more, she talked about having left facial numbness for up to three years. When I asked about pain, she'd only had some intermittent burning, which was worse with eye rubbing in the morning. And then also one episode of left jaw pain when she was chewing popcorn a couple days prior. They had gone to see her family physician who told her that it was probably a Bell's palsy, showed her some horrific pictures, was scared 12-year-olds quite a bit. But parents weren't really convinced. And so they pushed and a couple months later went back and said, we really like to see an eye doctor because we're concerned there's something going on with her eye. And otherwise, her past medical history and neurological history were not really concerning at all. The patient was most concerned actually about a little skin tag that she had under her eye that people at school made fun of. That was her biggest concern. So on exam, her vitals were normal. Her general exam was normal. Vision was almost normal. Stereopsis was affected. And she did have proptosis on the left. Also, on just looking at her appearance, the left upper eyelid was a bit tautic. And her lower lid was retracted. What I was most impressed by and what parents hadn't mentioned yet in going through the whole history was the fullness of the left side of her face. I said, well, when did that start? And they said, oh, you know, her grandparents came to visit last week. And they asked about that. But we hadn't really noticed it. She had no tenderness to palpation. And here's a picture. It's not a great picture. But you can appreciate that the left side of her face looks much fuller. And it's really tough on the quality of these images to tell the ptosis of her left eye. But that's there as well. Otherwise, totally healthy, not in pain kid who's doing her usual things. So the rest of the exam, which we do a thorough exam, slit lamp was normal. Dilated fundus exam showed a little bit of fullness of the optic nerve on the left, as well as an OCT. She had a thickened nerve fiber layer on the left. And everything else was normal. So what else would you want to know? Anything else that I think I'm holding back on you? So one thing we really like to do is look at old pictures to see, even though parents see her every day and they're noticing it's a problem now, how long was this going on? So we went and dad happened to have their Christmas picture from a year before. And again, I'm seeing the patient in November. And he pulled it out from Christmas. And sure enough, the left side of her face looks swollen there. And parents' stomach drops because they just weren't aware of it going back that far. So just to kind of summarize her, she's a 12-year-old girl who has left-sided proptosis, subtle left optic nerve edema. This is probably been going on for a long time. And it's not really causing her any pain, just maybe a little bit of change in her appearance. So came up with a wide differential, but knew that we weren't really going to know until we did a bit more testing, largely imaging, and eventually labs. So we talked about retro bulbar processes, extraconal masses, graves. Although she did not have any, again, restriction of her extracular movements, you might expect a little bit more lid retraction as opposed to ptosis and graves. And here's the first image. So she went and got an MRI. Fortunately, they were able to schedule her the following day. As you can see here, there's an extensive skull-based tumor, which is centered, appears to be centered in the left cavernous sinus and the medial portion of the left middle cranial fossa and the left pterigopalatine fossa. Some more images in coronal view. So the tumor is extending posteriorly through the trigeminal fraymen into the enterometeal portion of the left cerebellal pontine angle. It displaces the brainstem, as these tend to do, and eventually goes through the fraymen of alley into the left upper neck. So the radiologists gave a differential, probably, of a small blue cell malignancy, such as a rhabdo or granulitic sarcoma or amniuings and recommended biopsy. Prior to going to biopsy, ENT was involved and they wanted to get some additional imaging, so a CT was performed and here are those images. Again, not as helpful as the MRI, but again, indicating an extensive destructive skull-based mass with high attenuation indicative of a neoplasm. And so biopsy was performed. I believe that the ophthalmologic surgeons, neurosurgeon, plastics, ENT all had a discussion and eventually ENT were the ones who went in and performed a biopsy. The biopsy results were reviewed both here and at CHOP in Pennsylvania. They were found to be CD-99 positive. CD-99 is expressed on all leukocytes but highest on thymocytes and is known to play a role in T cell adhesion, migration, activation. It is positive in ewing as well in granulosis cell tumors. Picture over there is vimentin, which is a member of the intermediate filament family along with microtubules and actin makes up the cytoskeleton and that is usually used as a marker for granulosis cell tumors. Also the fish was positive for the EWS-FL-I-1, which is a marker for Ewing's and I'll review that translocation shortly. So the fish was positive but the PCR was negative. So, but based on the CD-99, the vimentin and the fish, the leading diagnosis at that point was Ewing's sarcoma. So that would be consistent with the radiologists that thought in terms of a round cell tumor of the bone and soft tissue. Usually affects males or the population they say to think of as male teenagers or maybe you've anecdotally seen that more in that population. They're difficult to classify and so that's why additional immunotesting is necessary and you wanna be really sure of the pathology before you take any additional steps. Picture down below shows the translocation. So the EWS protein from chromosome 22 and the FL-I protein from 11 or rather gene are translocated and that translocation, the EWS FL-I is found in 95% of Ewing's and then there's a different translocation, the EWSERG that's found in the other 5% or 5 to 10%. And again in Ewing's, CD-99 is positive and CD-45 is negative and that's because CD-45 is usually a lymphocytic tumor marker. So I'm a little bit more about Ewing's. If this were Ewing's, the treatment is usually tri-fold using chemo, resection and radiation and as is with most tumors, the prognosis is based on the staging. So in the survival for Ewing's is about five years, 70 to 80% when treated with chemo and then long-term with mats can be less than 10% but in some places they talk about it being as high as 25 or 30. So to do further work up on this patient, we performed an Ossia survey and that to no surprise showed increased uptake in the left facial bones. She had a normal echo, a normal chest CT, all these things were reassuring. She had a central line placed and was admitted with the idea that she was most likely going to be starting chemo and had a bone marrow biopsy performed just to make sure that her marrow had not been affected. And then the day before she was supposed to start chemo, the path came back and it was found to be EWSERG PCR negative and confirmed to be a meningioma. So meningioma. A third of all brain tumors, so most frequent primary brain tumor, usually benign but obviously in the CNS can cause significant mortality and morbidity. Incidents usually increases with age, usually rare in kids unless they've had previous radiation or have another syndrome such as NF2. Usually affects females more than males and some of the other risk factors that I read about. I guess radiation's used for treatment of tinea capidus or has been in the past and atomic bomb survivors is also some literature suggesting that head trauma can increase the risk of meningioma but I don't know that they're totally convinced yet. I included this picture to show that when they talk about primary tumors in kids, they don't even list meningioma, so it wasn't that I thought it was there. So often meningiomas arise from the dura, usually at places where the skull has a dural reflection, so there's a fold. So things like the Fox cerebride, centaurium cerebelli, also next to the venous sinuses, nerve sheets, chloride plexus, and one study noted the incidence including autopsy just in the general population to be about 0.9%. They can present as seizures, focal findings, which is how she essentially presented, well she didn't have visual field changes but actually a third of patients with meningioma, when tested, will present with visual field lost defects and diplopia. And so you can have focal signs, they can also be asymptomatic if found incidentally when people have imaging for other reasons or again on autopsy. So in terms of diagnosis, grading is based on histology. This is just again showing you her MRI because on T1 usually they're iso-intense or hypo-intense, which I think here is probably iso-intense to the gray matter, and then on T2 they're usually iso-intense or hyper-intense, and have a strong enhancement with gadolinium. In reading about it, meningioma often they refer to the tail sign, which is the dural thickening that kind of tapers off into a tail peripherally. And differential, so MRI often is helpful in the diagnosis as we saw in this case. CT, as again shown in this case shows that it displaces the normal brain, and PET is often used for prognosis or recurrence especially in case of maths. So differential included other infectious process, oncologic processes, maths, and inflammatory lesions, although this girl had been feeling so well that we were less concerned about an inflammatory process. Treatment for meningioma depends on grading location. In her case, complete resection is a goal. A lot of times they'll aim for partial resection or even surveillance if it's really in a place where it's not bothering someone and we can continue to monitor. Radiation therapy is also used more frequently as I've seen in people with multiple meningiomas who've already had multiple craniotomies. And then we know that survival wise people with higher grade lesions, so WHO grade two and three are more likely to have invasive disease, local recurrence, and shorter survival. So the timing could not have been weirder on this patient who I saw back in November and did go to the OR yesterday. And so prior to the surgery, they met with neurosurgeonian T, planned to do a resection attempting to remove as much of the intracranial portion of lesion as they could. They prepared parents that likely this is gonna need to go on stages because of its extent that they were gonna start at the front, try to get the part that was affecting the orbit and move posteriorly. And again, she had pre-op MRI for improved navigation in comparison to her previous and it didn't appear to have changed much in the two months and probably had been there for an incredibly long time. She's only 12. And so yesterday, she had neurosurgery. They did left frontoprital craniotomy. They drilled into the orbital root and lateral orbital wall to decompress the orbit and down into the fossil floor as well for resection. They did interoperative monitoring and the frozen path conferment in geoma. And she was transferred to the ICU and to the best of my knowledge, she's doing okay at this point. Interoperatively, they commented on transient bradycardia when they would stimulate the fifth nerve. Okay, some of my references. And I just wanted to thank all the people that helped me during my rotation. I also wanted to, you know, all of them. Highlight that when you look up neuro-optimology in Google, the third image is this. So, we're all pretty lucky. All right, but thank you for your time and I'm letting me share this interesting case. Questions, comments, people? Yeah. Yeah. Yeah. Yeah, and in retrospect, obviously, we have all the information now. It does make sense for it to have been a meningioma which we know can be incredibly slow-growing and insidious and change. Yeah, especially when you were told your kid had a Bell's palsy. Yeah, that's why I showed the picture to show. I mean, I can see how this would happen.