VLOG: NeuroOp Guru: Unique case of 15-year-old who showed signs of meningioma in eye

Andrew Lee, MD, and Andrew Carey, MD, sit down on another episode of the NeuroOp Guru to discuss a unique case of a 15-year-old who showed signs of meningioma with epithelial whirls and some psammoma bodies in the eye.

Video Transcript

Editor's note - This transcript has been edited for clarity.

Andy Lee, MD:

Hello, and welcome to another edition of the NeuroOp Guru. I'm here with my good friend Drew Carey. Hi, Dr Carey.

Drew Carey, MD:

Hi Dr. Lee.

Andy Lee, MD:

And today we're going to be talking about a very strange case of a 15 year old who had blurry vision and had this fundus appearance in this OCT and imaging, and a pars plana vitrectomy was performed and Drew what did it show?

Drew Carey, MD:

So, yeah, I think this is a really strange case where the patient had kind of presented with, you know, mildly elevated optic disc, pretty good visual acuity and one of the challenges in pediatric ophthalmology is it's hard to get reliable visual fields. I think as a neuro ophthalmologist, we rely greatly on the visual field as part of our diagnostic evaluation because it, you know–pattern recognition. But it also tells us about the visual function and maybe how serious the patient's condition is. And so as this lesion grew, it obviously became more concerning and on the biopsy, it showed findings typical of a meningioma, with epithelial whirls and some psammoma bodies, which is not something we usually find inside the eyeball.

Andy Lee, MD:

And so how does one get a meningioma inside your eye?

Drew Carey, MD:

Yeah, so I think that's the really fascinating question about this case. So meningioma, we can have primary optic nerve sheath meningiomas, which is probably the the scenario in this one. Although we don't have contrast enhanced imaging to see if it extends outside of the orbit. But you know, the optic nerve sheath has to fuse with the posterior sclera. And meningiomas can be exophytic where they grow out into the orbit. Or they can be endophytic, where they grow inside the optic nerve sheath and into the optic nerve. Or, you know, into that subarachnoid space. And when they do that, then they can grow through the lamina cribrosa and have intraocular extension. So super rare manifestation and complication. But it can happen. I am not sure how one would make the decision to do an orbitotomy for biopsy versus a vitrectomy for biopsy. Certainly, I guess if you're a retina specialist vitrectomy seems simpler. But orbitotomy would probably give you access to more tissue if you felt like you needed it.

Andy Lee, MD:

I would have thought orbitotomy would be easier and what we normally would do for these strange optic nerve sheath tumors. But I guess they saw the big white thing and they said maybe we should go get that.

Drew Carey, MD:

Yeah, it may have been the concept of well, we can see it pretty easily. And I think there's a good chance for getting, you know, good yield from it. Sometimes you get into the orbit, and it's harder to find things than you'd want it to be. Also, I guess, if you're really trying to, you know, do a biopsy right up against the optic nerve head where, you know where it inserts into the ... where the nerve sheath inserts into the sclera, you might be at higher risk for biopsing like a posterior ciliary artery, or having a central artery occlusion as a complication, although those are rare events. Probably the other option from the vitrectomy standpoint is you could biopsy on the nasal side, and so you're less likely to have damage to central visual fibers. I think a lot of times when you're doing orbitotomy you're coming either superior, superior lateral. And certainly you can do a medial approach, but I think a lot of folks, you know, prefer another approach, and then you're closer to the central visual pathways.

Andy Lee, MD:

And what do you think accounted for the delay and why it was so stable and all of a sudden, bigger?

Drew Carey, MD:

Um, you know, I think probably the good vision was the reason that they decided to monitor at first and at age 9, looking at it and you say, well, maybe that's a pseudo disc edema. I don't know. Although, you know, certainly elevated on the B scan. You know, probably at age 12, I would have been nervous, but I think he still had pretty good vision at that point in time. Sometimes the patients say, you know, I'm not too crazy about having eye surgery or the parents say that about the child. Certainly it's very slow growing so we're not particularly concerned that this is, you know, an infection or cancer. You know, earlier diagnosis means you probably could have done radiation earlier and maybe had a better visual outcome although parents are usually not too excited about having their kids undergo radiation treatment. So I think, you know, certainly that monitoring is what gave it the chance to grow more and have that intraocular extension that gave us that really strange appearance at age 15.

Andy Lee, MD:

Did they say they treated this with radiation or what happened to the patient?

Drew Carey, MD:

The patient did undergo image guided radiation therapy. So about, I think it was 50 Gray over 30 fractions or so. They said the final visual outcome was around 21/50. I think at the time they did the biopsy, it was 20/40. But we don't know what the division was in between that period of time. But they said it remained fairly stable for at least 6 years on monitoring after radiation therapy.

Andy Lee, MD:

And do you think there's anything about this imaging or OCT that would have predicted this was a meningioma? Certainly the age wouldn't. I would have thought this would be more likely to be glioma.

Drew Carey, MD:

Um, I think it's it's difficult to say from the B scan, ultrasound, you know, if they could have detected it was specifically nerve sheath thickening as opposed to nerve thickening. That would have, you know, led you more towards a meningioma type process. You know, I think optic nerve pathway, glioma would certainly fit this kind of picture. It's a little strange to have it progress if it's syndromic with NF1. But the sporadic cases certainly can progress and you do kind of expect that maybe 60-80% of the cases. I think, you know, good MRI scan with fat-sat and contrast enhancement should be able to tell you the difference. We try to avoid CT scans in kids, although if that had shown calcification that certainly would have told you it was a meningioma. But because of cumulative effects of radiation, we try to avoid those certainly more in retinal blastoma patients who are already at risk of radiation toxicities and secondary cancers.

Andy Lee, MD:

So what do you think the take home messages for our audience? Or is this just an interesting fascinoma?

Drew Carey, MD:

I definitely think it is an interesting fascinoma. I think, you know, take home messages, if you're seeing you know, optic disc edema in a child, unilateral optic disc edema, we should be thinking, you know, a lot of cases it's going to be pseudo papel edema, but if you're seeing progression, or any other red flag features, you know, it doesn't look like drusen on an OCT, we really do want good quality neuro imaging of the brain and orbits in MRI scan with and without contrast. And they're not routinely doing fat saturation on your orbital imaging at your institution, ask them why. And make sure that you get that, so that we can have the appropriate diagnostic techniques to make the appropriate diagnostic. And even if you made the diagnosis at age 9 and 12. And you said hey, it's good vision, you might have made the same decision to monitor the patient and then do treatment later down the road. But I think everybody sleeps better if you know what the diagnosis is.

Andy Lee, MD:

Well, that concludes yet another edition of the NeuroOp Guru, Dr. Kerry as always a pleasure.

Drew Carey, MD:

Thanks Dr. Lee.