This Live Lecture covers the differential diagnosis, evaluation, and management of bilateral optic disc swelling. Topics include papilledema and its causes (e.g. idiopathic intracranial hypertension, tumor, etc.), optic nerve infiltration, and pseudopapilledema.
Lecturer: Dr. Karl Golnik
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DR GOLNIK: Well, welcome to another neuro-ophthalmology Orbis webinar. Hopefully you’ve been to some of the ones in the past. And we’re gonna start this time — the last lecture, about optic neuropathies — I thought we tried to cover mostly bilateral optic disc swellings. So the title is the approach to the patient with bilateral optic disc swelling. And remember that questions can be typed in at any time, and then I will answer those at the end of the lecture. So my objective is that when we’re done, you’ll be able to list the differential diagnosis of bilateral optic disc swelling, you’ll be able to list the characteristics of papilledema, and describe the approach to the patient who presents with papilledema. So I’m gonna start with a question. And the question is this — and this will be an audience polling, so you guys can vote. You see the optic discs, pictured below. This could result from which of the following problems? Could it result from a tumor? Tetracycline use? Sleep apnea? Or all of the above? Here’s the polling. You can see the discs. Could this be A, a tumor, B, tetracycline use, C, sleep apnea, or D, all of the above? And I’m not sure if people are voting or not. I can’t tell. We’ll give people a few more seconds to give their opinions. This is in part to let me know sort of what you guys know about bilateral optic disc swelling. Oh, I’m not sharing my screen? Oh, I’m sorry. Hold on. Where is the… Okay. Here we go. Let’s try again. Is that working now? All right. I apologize. I thought I was sharing my screen. I don’t think I need to go backwards. I just had the objectives. So we’re starting with this question. Sorry. Now you can see my screen. And here’s the question. The optic disc below can be caused by what? Which of the following? Tumor, A, tetracycline use, B, sleep apnea, which is C, or all of the above, which is D? And I can’t tell if people are voting or not. But if they are, I think we have given them enough of a chance that… Can we close the polling? And let’s see the results. And I can’t see the results, for some reason. Ah, thank you. There we go. So the majority of you, two thirds, about, said all of the above. And that’s correct. Of course, a tumor could do this. Tetracycline use can cause increased intracranial pressure and bilateral optic disc swelling. Sleep apnea can actually do this as well. So that’s probably less known, and that may be what fooled some of you, who didn’t pick all of the above. But sleep apnea has been reported now in a number of studies to cause bilateral optic disc swelling from increased intracranial pressure. I just wanted to get an idea of where you guys are. We’ll close the polling. So this is a typical patient, probably the most common type of patient I’m gonna see, where I am, in terms of bilateral optic disc swelling. She has headaches, intermittent blurred vision, she’s gained a bunch of weight over the last six months, pupils are good, no relative afferent pupillary defect, and everything looks normal except for her visual fields and her fundus. Here’s her fundus appearance and visual fields. You can see she’s got enlargement of the physiological blind spots, and bilateral optic disc swelling. So this is the most common scenario I’m gonna see. And the question is: How do we manage this patient? So of course, there’s a differential diagnosis for bilateral optic disc swelling, other than papilledema. And that includes all of these things. So of course, anything that might cause unilateral optic disc swelling could potentially cause bilateral optic disc swelling. I think that’s kind of unlikely, but you’ve got to think about those things from time to time. Optic disc drusen, pseudopapilledema, a term I like to reserve for people who were born with small, crowded discs. Sometimes they can look swollen. Lebers, a DNA problem we discussed in a previous webinar, can cause disc swelling that’s actually pseudodisc swelling. And there are a number of medications, including the list I have put down for you here. And finally, don’t forget if someone comes in with bilateral optic disc swelling, your first thought probably shouldn’t be oh my God, it’s a tumor. The first thought should be I hope they don’t have super high blood pressure. This is something any ophthalmologist should be able to check in their clinic. It’s the first thing to check. Because you might have somebody with a hypertensive crisis that may not know it and you may save their life by checking their pressure. So compressive causes — probably thyroid eye disease is something I might see a bilateral disc swelling. Remember, most tumors are gonna be unilateral. And tumors within the head, in the skull, not in the orbit, don’t cause disc swelling unless they’re so big that they’re causing increased intracranial pressure. So compressive optic nerve lesions — you have to have bilateral orbital tumors for disc swelling from compression. So a pituitary tumor never causes that. In 20 years of neuroophthalmology, I’ve never seen it. If there’s infection, syphilis, tuberculosis, cat scratch disease, all of those could be potentially bilateral. Things that infiltrate the nerves, like cancer, carcinomatosis, often bilateral. Fortunately fairly rare as well. Ischemic problems. So giant cell arteritis, which depending on where you live you may or may not see, could cause bilateral simultaneous anterior ischemic neuropathy. Occasionally I’ll see people wake up postoperatively with ischemic neuropathy. And of course postop AION and blood loss can cause bilateral disc swelling. When these affect your vision, they cause loss of vision. So it would be unlikely that any of these conditions would present as early papilledema presents, with bilateral enlargement of the blind spots. And that’s the thing that’s remarkable, I think, about papilledema. Papilledema causes, usually, very little loss of vision early on, yet the disc may be hugely swollen. So here’s a bunch of different photos. The born-with types of problems. So you can see in the bottom left pair, down here hopefully you can see my pointer — Bergmeister’s papilla, a remnant of the connective tissue that used to be supporting the hyaloid artery that runs from the disc to the back of the lens. This doesn’t completely resorb. Once in a while these people get sent to me with bilateral optic disc swelling. Optic disc drusen, which hopefully is fairly obvious. You can see some more discrete calcified mucopolysaccharides in the nerve. Suspicious over here. Ultrasonography, B scan, can prove this. Over here, and I hope you’re not seeing — I have a bar with Cybersight over here. I’m gonna move this out of our way. This is actually buried disc drusen. That nerve looks very suspicious for swelling. Actually, there were buried disc drusen. And once in a while, I have somebody referred to me with myelinated nerve fiber layers, like you’re seeing in the upper two photos. This very pronounced myelination, and then something not as pronounced. The key with myelination is it should be pretty white-looking. And if you look at the borders of the abnormality, you’ll see this feathery appearance. And that feathery appearance is because the myelin goes further along some of the axons than others. So that can be a tip to myelinated nerve fibers. But these were all patients referred to me with bilateral disc swelling who all had born-with-type issues. And then pseudopapilledema. This is a term I reserve — in the bottom left two photographs, these are both patients who had nerves — both nerves looked like each of these. These are not the same patient. It’s two different patients. There’s no central cup. There may be some elevation of the disc — they just were born with a small hole in the back of their eyes. They’ve got small, crowded nerves that look swollen. In the upper right — I’m not entirely sure I’ve got this photo. It may be to remind me that some textbooks refer to optic disc drusen as pseudopapilledema. I call it drusen. This patient had a very suspicious nerve with concentric hemorrhage up here, what looks like disc swelling and may be disc swelling, but they actually have buried disc drusen causing this appearance and causing some folds and little concentric folds in the retina, which we’ll see more of in a moment. And then there are toxins and medications, methanol, ethylene glycol, which is in antifreeze. It’s sold in some parts of the world. Some parts of the world don’t use it. Some people try to kill themselves by drinking it. Tamoxifen, cisplatin, amiodarone, and there’s some data that the PDE5 inhibitors, Viagra, sildenafil, and others, can cause disc swelling. Usually you’ll get this in the history, and this is not gonna be something that will be occult, or — you’ll look at the medication list, or the person tried to kill themselves or something, they got exposed to something, and so it will be pretty obvious. And don’t forget hypertension. Again, if you learn one thing, it is: Just check the blood pressure in anybody with bilateral disc swelling. Make sure they don’t have — they’re not in a hypertensive crisis. So here is our second audience polling question. And the question is: papilledema is defined as bilateral optic — sorry, that should say optic disc swelling. True, which would be A, false, which would be B, and I don’t know, which would be C. So papilledema, defined as bilateral optic disc swelling. I’ll give you a moment or two to decide. Okay. Why don’t we… Five, four, three, two, one, vote! Okay. Let’s close the polling and see what the audience says. About half the people think this is true, almost half think it’s false, and a few don’t know. So the answer is false. Papilledema is not defined as bilateral optic disc swelling. Papilledema is defined — well, let’s get rid of that poll. Papilledema is disc swelling due to increased cerebrospinal fluid pressure. So what that means is if you see someone with bilateral optic disc swelling, you shouldn’t call it papilledema unless you know their intracranial pressure is elevated. That means either you’ve got a scan with a big tumor or you’ve done a spinal tap that shows increased pressure. Otherwise it’s optic disc swelling. And sometimes papilledema is unilateral. So the definition is disc swelling due to increased cerebrospinal fluid pressure. Older ophthalmologists will sometimes refer to it as papilledema. And non-ophthalmologists might think of bilateral disc swelling if you use the term papilledema, but as a neuro-ophthalmologist, it’s not appropriate. So if you see someone and their blood pressure is 200/120, they probably don’t have papilledema. And papilledema can take many forms. And certainly we see here sort of a spectrum on our far left very mild disc swelling. The margins of the disc are blurred. There’s no hemorrhagic, there’s no exudate. You get the sense perhaps in this two-dimensional view that there’s slight elevation of the disc. Remember that elevation is not the same thing as swelling. But this disc is mildly swollen. Over to the next frame, you can see now it is at least a moderately swollen disc. There’s obscuration of blood vessels, as they’re crossing the surface of the disc, because there’s swelling of each of these fibers, and that’s what papilledema is about. Axonal swelling. There may be a little hemorrhage, or maybe this is just a blood vessel up in here. And then a very swollen nerve where you can’t even see disc margins, exudate, hemorrhages, and so on. And then what you don’t want to see, which is this postpapilledema optic atrophy. So in patients who are losing nerve axons, ultimately, if it’s not treated, and they’ve lost all of their axons, well guess what? The nerve can’t swell anymore. That’s what’s swollen. The axons. If you have almost no axons, the nerve cannot swell. There’s nothing there to swell. So you don’t want to see this scenario on the far right. And watching somebody with papilledema and have it quote-unquote “improve”, hopefully it’s actually getting better, not just because you’re losing axons. So here’s some examples of different ways that papilledema might look. Sometimes there’s a lot of hemorrhage. Can’t even see where the disc head is, in the bottom left photo. And then you may even see some preretinal hemorrhages. This is fairly unusual. But certainly it can be seen. So you can see almost any sort of disc swelling with papilledema. And I don’t think you can look at a nerve that has — that is affected by papilledema, increased intracranial pressure, and then look at another nerve that’s swollen from optic neuritis, or swollen from anterior ischemic optic neuropathy, and necessarily differentiate it, simply on the basis of the optic disc appearance. And so here’s those concentric lines that we showed earlier in this patient with disc swelling. And you can see these — hopefully see these concentric little lines. These are known as Paton’s lines, named after David Paton. And when the disc swells — it doesn’t have to be swelling from papilledema. Although that’s when I most commonly see it — as the disc swells, it enlarges and pushes the retina aside. This is not the same disc. The histopathology — but you can see here here is the retina, being thrown into these folds, and that’s what is these Paton’s lines. You see these folds. Now, as the swelling goes away, that retina usually flattens back out. But this is why you have a big blind spot. It’s not optic nerve damage in any way. It’s simply mechanical. The reason you have a physiologic blind spot is that there’s no retina that overlies the optic disc. Well, if the disc is swollen and it pushes the normal retina aside, guess what? You have an enlarged blind spot. So this is not damage to the nerve. It’s simply a mechanical thing. This is a video that’s not the best clarity, perhaps. But the best I can do, video taping spontaneous venous pulsations. So if you look hopefully where my arrow is, you can see that vein. And it’s the best place to look, as the central retinal vein, the superior or inferior vein crosses the optic disc. And as you see those pulsations, you can be somewhat assured that the nerve has not been affected by increased intracranial pressure. Now, there have been some reports that people with increased pressure might have spontaneous pulsations. Now, if you don’t see spontaneous pulsations, it doesn’t mean much. A couple people just don’t have them. If you do see them, it’s reassuring. If you don’t, it doesn’t mean much. So those are spontaneous venous pulsations. What about unilateral or even more papilledema? Can you have that, if you know the cerebrospinal fluid pressure is increased? And here’s a pair of optic discs. And you might argue that this right disc couldn’t be mildly swollen. Who knows? Maybe there’s a little blurring of the disc margin here. But obviously there’s clearer swelling of the left optic disc. You can see some Paton’s lines right here. Some hemorrhage. Obviously there’s obscuration of the blood vessels. Clearly at minimum, this is very asymmetric disc swelling. And this is a patient with known increased intracranial pressure, who happens to have virtually unilateral papilledema. I also definitely have people sent to me by neurology who come in with headaches, MRI, normal, do a spinal tap, have high pressure, I see that patient the same week, no papilledema. So not everybody with increased intracranial pressure develops papilledema. And we’re not really sure why that is for certain. I think one possibility is this. If we look at this — well, it’s just a cross section of the optic nerve. And you can see that all these little trabeculations between the arachnoid and the optic disc — you can imagine that in some patients, there may be just a lot of these trabeculations — maybe that prevents transmission of cerebrospinal fluid pressure to the back of the globe. Because we think you do need the pressure transmitted behind the back of the globe itself. So perhaps in patients with no papilledema, they just have very exuberant trabeculations, and perhaps in people with unilateral papilledema, it’s asymmetrically — they have asymmetric trabeculations in the optic nerve. This is not a proven fact, but one possibility, perhaps, as to why we might see somebody with known increased intracranial pressure who does not develop papilledema. I will say that if you don’t develop papilledema, you are not at risk of damaging your optic nerve. There’s a couple of case reports out there in the literature that suggest that it would happen. I would say that one of those case reports is actually from me and published many years ago in a patient who had a history of increased intracranial pressure, treated, did well, and had recurrence of disc swelling in one eye. And we published that as a case of — I’m sorry, not recurrence. Had recurrence of… Occurrence of an optic neuropathy in one eye. With slightly elevated intracranial pressure on spinal tap, and in fact, ultimately, he was diagnosed with multiple sclerosis, in addition. And we think now that the case report, which is still out there, it was probably due to optic neuritis in a patient who had two different conditions. The other thing you can see when the intracranial pressure is increased is 6th nerve palsy. And you can see here that this patient has a marked deficit in right abduction. In this case, increased intracranial pressure was causing this. It doesn’t have to be that striking. Here’s another patient. And I’m gonna try to play this. There we go. With double vision. But a very mild abduction deficit, when he looks to the left. Here. So he had a mild… Maybe more than mild, but a mild to moderate 6th nerve palsy, and increased intracranial pressure. So of course, we look for this. We don’t always see it. Whenever I see somebody with 6th nerve palsy, either unilateral or bilateral, I look closely at the optic nerves, of course, just to make sure I don’t see papilledema, in addition to the 6th nerve palsy. So here’s a third question, an audience polling question. And the question is: Visual loss in papilledema initially is usually which? Now, I don’t have big blind spot, because I don’t consider the big blind spot to be visual loss. I consider that to be mechanical. So that’s not supposed to be in the none of the above category, if you’re thinking of that. So big blind spot doesn’t count to me as visual loss. I’m talking about — in papilledema, what’s the most common place that we see optic nerve damage? Or where is the vision loss in optic neuropathy in papilledema? Is it centrally? Which would be A. Is it temporal? B. Nasal? C. Or is it none of the above? I’ll give you a chance to think about that. All right. I’ll give you five more seconds. Five, four, three, two, one. Let’s see what the audience says. So I have kind of a mixed bag. So I’ve got a third of people that say none of the above. Central, about 22% or 30%. Sorry. 21%… Okay. So the answer actually is: all of you are — only 7 got this right. The minority. Nasal is where the visual loss usually occurs. And we’ll go ahead. Let’s close that. Let’s look at some typical visual loss. So certainly you can have vision loss from papilledema that — usually we think about the optic nerve. You can have vision loss from retinal problems. Usually this is due to leakage of fluid and exudate into the macula. So if I see someone with a good — pretty good-looking visual field, but their acuity is down, and they’re thought to have papilledema, they almost always have fluid in the retina. You do not lose central vision early in papilledema. From optic nerve damage. You lose it because there’s fluid in the macula. In the optic nerve, when there’s damage, it’s always peripheral loss first. And so here are a couple of visual fields. The one on the bottom left is a fairly typical early visual field. You’ve got enlargement of the physiologic blind spot. Again, not optic neuropathy. This is a mechanical thing. You’ve got the very classic early nasal — often inferonasal — this person has a little supero and inferonasal field loss. By far the most common early visual field finding of optic neuropathy in papilledema. Unfortunately then what can happen is, in more extreme cases, in this field on the bottom right, this person is also 20/20 or 6/6 with good central acuity, but like glaucoma, it tends to affect peripheral vision. So you get this non-specific-looking constriction of the visual fields. Okay. Another question. This is an audience polling question. Causes of papilledema include all of the following, except which? So idiopathic intracranial hypertension? Venous sinus thrombosis, some medications, or traumatic optic neuropathy? So causes of papilledema. All of the following except which of these? IIH, this is formerly known as pseudotumor cerebri. A — if you’re not familiar with this term, you should be. We’re trying to get rid of the term “pseudotumor”. It’s got the word tumor in it. And then venous sinus thrombosis, some medications, traumatic optic neuropathy. Let’s close the poll. Good. So the majority of you got this right. Traumatic optic neuropathy is usually contusion of one optic nerve. There is usually no optic disc swelling. If there is damage to the globe, you can see that. If there’s damage that causes increased pressure, we probably wouldn’t call that traumatic optic neuropathy. So of course we all worry about tumors that are large enough to cause increased intracranial pressure, venous sinus thrombosis, which causes increased intracranial pressure by increasing the venous sinus pressure. CSF drains through the venous sinus. So if the pressure goes up in the venous sinus, it goes up in the CSF. Tetracycline and minocycline, which is used quite a bit in the United States for treating acne in teenagers. Vitamin A toxicity, also used in the United States for teenager acne. Lithium has been reported. And then there’s some question medications. I consider these all questions without really great proof. Birth control pills. Amiodarone isn’t really papilledema. That’s something else. Amiodarone optic neuropathy. So I have questions about all of these. I don’t think they’re necessarily true. Sleep apnea, as I mentioned, polycystic ovary syndrome has been reported. And microcytic anemia has been reported. So if you’ve got a giant tumor like this, it might increase intracranial pressure. You might have compressive optic neuropathy too. Here’s a patient with MRV, magnetic resonance videography, showing pressure in the venous sinus, in a patient with venous sinus thrombosis. There’s a number of risk factors for this. It can be spontaneous. Of course, if there’s a coagulopathy, just a tumor elsewhere can make you hypercoagulable, Behcet’s disease, cancer, SLE, pregnancy, sarcoid has been associated with this. So there are lots of things that can cause venous sinus thrombosis. You see this narrow transverse venous sinus in a patient with papilledema from venous sinus thrombosis. Medications, as I mentioned already, the cyclins are by far the most common reason that I see medication induced papilledema. I don’t really call this idiopathic intracranial hypertension, because it’s not idiopathic, in my opinion. It’s due to the medications. I think from the list that I have the question mark — maybe the most controversial would be the birth control pills. If you really look at that evidence, it’s not great. So here’s a patient, illustrating the sleep apnea issue. Here’s a young guy that I saw some years ago now. 25-year-old, overweight gentleman, worked in construction. And whenever he bent over and stood up, he had transient visual obscurations. A little blurry vision. He had had a lumboperitoneal shunt for many years, because of a problem when he was born. And his mom was a nurse who worked with a neurosurgeon. And he had recent imaging studies, because there’s a question about whether his shunt is working. And he’s had three lumbar punctures, spinal taps, all with normal pressure. So he got to me after all this, because his mom works for a neurosurgeon, and he had what looked like classic bilateral papilledema, despite having these low normal intracranial pressures. Normal intracranial pressure is up to 20, there’s a grey zone, 20 to 25, over 25 is certainly elevated. And so I said… Gee, this really looks like papilledema. You don’t have any significant loss of vision. The blind spots on the fields are slightly enlarged. You’re having these transient visual obscurations. I really think this is papilledema. Maybe you’re only having increased intracranial pressure part of the day. Enough of the day to cause the papilledema. The only way to know that is to admit you to the intensive care unit and put an intracranial bolt in. Whenever I talk about that to patients, they always say no. Forget that. His mom said — sure! Let’s do it! We put him in the ICU, put in the bolt to measure his pressure. His pressure is 12, 16. Then he went to sleep. When he was asleep, his pressures were in the mid-40s. And he had sleep apnea. And we fixed his pressure by fixing the sleep apnea. There were a couple papers out on this. This was the one I was involved with. Polycystic ovary has been part of this. There are women with menstrual cycle problems, androgen infertility, usually overweight — the question is — overweight women might be in the age range for idiopathic hypertension. Which is true, but a high percentage of our patients had polycystic ovary syndrome. There’s some debate on this. There’s some literature to it. Not everyone believes this, I guess I should say. And there’s some literature on microcytic anemia possibly being linked. I check a CBC in patients to make sure they don’t have microcytic anemia. There’s not a lot of new data, as you can see, and the mechanism is not known. So idiopathic intracranial hypertension, IIH, also called many things, pseudotumor cerebri, probably one of the most common terms — the other term I really don’t like is benign intracranial hypertension. Or BIH. Why? Because it implies — benign — that it’s not a problem. But it certainly can be a problem, and you can certainly lose vision from it. So to diagnose this, you need a few things. You need normal neuroimaging. These days, we like to get both the MRI and the MRV. If you don’t have an MRI, an MRV, CT scan is adequate to at least rule out a big tumor. There should be — there has to be increased intracranial pressure on lumbar puncture. Greater than 25. I say here 20 to 25. Sort of a grey area in overweight people — normal people — just overweight people — but normal otherwise can have a pressure in the 20 to 25 centimeter range. That said, if I see somebody who is overweight and their pressure is 23 and they’ve got big bilateral optic disc swelling, they’ve got papilledema. And then the CSF composition has to be normal. There can be no localizing signs, in other words numbness on the right side, except for 6th nerve palsy, which we don’t consider localizing. To diagnose IIH, you do not need headache or papilledema. They are not part of the diagnosis. A small percentage of people may not have headache and may not have papilledema. So typical IIH are women, child bearing age, they’re overweight or have had recent weight gain, signs of increased intracranial pressure, that usually include headaches. Transient visual obscurations. These are these brief episodes, lasting on the order of 5 to maybe 60 seconds, when the person changes position. Sometimes even when they change their gaze. Sometimes I’ll see people who wake up in the morning, they wake up every morning, their vision is blurry in both eyes until they sit up on the side of the bed, stand up, wait a minute, and the vision clears up. Pulsatile tinnitus occurs in 60% of people. Certainly frequently enough that when I see somebody with bilateral disc swelling, I ask do you hear sort of a whoosh, whoosh, whoosh noise. It’s not specific to IIH, but if I see somebody who has it who has papilledema, it’s probably related to IIH. We look for papilledema, which is what this talks about. 6th nerve palsy, which can be unilateral or bilateral, and then visual loss, which most often is optic nerve, can be retina if there’s enough disc swelling to cause leakage of fluid or exudate into the macula. Usually it has about a two-year course. It can definitely last longer than this. Most of my patients, it’s in that kind of ballpark range. Not all. And it can be sneaky. The visual loss. So you can have people who present with a visual field on the left that we saw earlier, with just a big blind spot. They may be totally asymptomatic, with that type of field. And then as it progresses, they become symptomatic, with peripheral vision problems. And ultimately lose vision entirely. If the person is not that observant, they may damage quite a bit of their optic nerve, in this visual field here, just to the left — to the right of center — you can see this person’s 20/20 and didn’t come in with visual symptoms until this point. And then of course they can present, thinking they just need glasses, as they’re going blind. You also have to be careful, because there have been at least one report of non-organic vision loss. Non-organic meaning the patient has a headache, they’re just not good at the test, and you see this generally constricted field. So it can be tough to know if a field like this one left of center is a real constriction, or is this just a person who can’t do the test very well? Because this is very non-specific. I see people with no loss of vision who do the test and they look like this, and I give them a pep talk, and they get better. So you’ve got to be real careful basing your treatment decisions on the visual fields. That can be a challenge. Beware non-organic loss of vision. Always repeat a visual field test if you’re about to make a treatment decision based on that field. And if the loss is asymmetric, there will be a relative afferent pupillary defect. As you can see in this person, who has a right relative afferent pupillary defect, which you’ll see as you swing the light from eye to eye — there it is — the reason I’m bringing this up is sometimes my residents will say — oh, they’ve got papilledema and some vision loss, but there’s no relative afferent pupillary defect. Remember, vision loss in papilledema is often bilateral and often fairly symmetric. So of course, there will not be a relative afferent pupillary defect, if the loss of vision is symmetric. Right? If you damage each optic nerve about the same, there won’t be a relative afferent pupillary defect. What are the red flags? Red flags — I’m not sure if that’s a term that’s used around the world or not. Meaning: Warning. You’re about to make a diagnosis of idiopathic intracranial hypertension. If you see any of the following, you should take caution. If the patient is a male. Men rarely get idiopathic intracranial hypertension. There’s some articles in the literature that suggest that it could be as often as one in nine patients. There is no way that’s true. Those articles are from before our current knowledge. I’ll bet you it’s maybe one in 50 at most. So with any man I see, I’m gonna think about other possibilities. Like what? Like sleep apnea. Of course tumors and so on. Venous sinus thrombosis. But if it’s a man, overweight, I’m gonna really think twice about making that diagnosis. If they’re over age 49. People usually don’t get IIH — women don’t get IIH in that age range — and if they’re a woman who has it and they go through menopause, they shouldn’t have it anymore. So this is not a lifelong condition. And if you’re trying to make a diagnosis of IIH in someone who’s 50 or over, take caution. If the person has normal body weight — people with normal body weight don’t get IIH. You’ve got to think again: What medicines are they on? Do they have sleep apnea? Do they have venous sinus thrombosis? What else is going on? Be extremely hesitant to make a diagnosis of IIH in any of these situations. What’s the treatment? Well, of course, lower the spinal fluid pressure. That’s the treatment. We know that weight loss can do this. And in this study, from almost 20 years ago, which wasn’t a great study, but it was a study that looked at women who on average lost about 7% of their weight. So for me, that’s a good number. 7%. So that’s 7 pounds per 100. If I see someone who weighs 300 pounds, or whatever that would be in kilograms… 150 kilograms… If I tell them to lose 7% of their weight, so for a woman, 7% of 300 pounds would be about 21 pounds. That’s way different than telling the patient — get down to your ideal body weight. The patient is gonna say forget it! Are you kidding? But if I tell them to lose 21 pounds, or 8 kilograms, they say well… That might be possible. And I have patients who do. And once they do lose that amount of weight, they realize — hey, I can lose weight. And then they lose 100 pounds, or 50 kilograms. And they cure themselves. So weight loss is always… I always discuss weight loss with the patients. Medications can lower the pressure. Acetazolamide is certainly my still go-to first line medicine. Topamax or topiramate I guess I should call it. Furosemide… Octreotide has been reported. That has not been duplicated in the study. But there are medications. Furosemide and topiramate are second tier to me. I usually start twice a day with the time released form. In the recent idiopathic intracranial hypertension treatment trial, they pushed patients up to a total of 4 grams of acetazolamide a day. And there are a variety of surgical treatments for IIH. I won’t go into great detail, but they’re cerebrospinal fluid diversions, like shunts, there’s nerve sheath fenestration, there’s venous sinus stenting, and then bariatric surgery. If patients bring it up to me, I’ll write them a letter, saying they have a condition that weight loss can cure. So I mention the idiopathic intracranial hypertension treatment trial, or the IIHTT. This was relatively recently completed. This was a prospective randomized trial of weight loss versus weight loss plus acetazolamide. And to get into the study, you had to have of course — meet the diagnostic criteria. Which we’ve discussed. But you also could not have severe or moderate to severe vision loss. So you had to have between a mean deviation between -2 and -7 decibels. This is a representative visual field, as you’ll see. By far the most common early visual field defect you’ll see in idiopathic intracranial hypertension. You had to have this sort. In the -2 to -7 range. They enrolled 165 patients, only 4 men. And all the patients were obese. This was published in JAMA in 2014. There’s the reference. And what they found, to make a long story short — they used a low sodium diet in both groups. But one group got placebo and one group got acetazolamide. It was pushed in that group up to 4 grams per day. And the bottom line, the primary outcome of this study, was looking at improvement in visual field test. They also looked at papilledema grade. But as I often tell my patients — I care what your nerves look like, but I really care how they work. And how they work is the visual field. So what they found in the test, and they reported that patients on acetazolamide plus diet did better than diet alone. And here’s the data. So if you know about automated visual fields, you know about mean deviation. So what they found was that both groups had an average mean deviation of -3.53 at the start of the treatment trial. The group who got the diet and the acetazolamide had improvement to a mean deviation of -2.10, versus the group who only got diet, who improved to -2.82. And they found this difference, 0.72, to be statistically significant. Now, anyone who does these visual field tests knows that a mean deviation of 0.72 is a trivial difference. You could do the test yourself every day this week, and believe me, you’re probably gonna find more than a 0.72 decibel difference. Although this is statistically significant, I don’t believe this is clinically significant in any way. They did say that when they looked at the grade of papilledema, maybe the people with worse papilledema did better if they got the Diamox and diet. So that’s the results of the IIHTT. The other thing I wanted to just mention, that’s newer, is the venous sinus stenting. So there is an interesting literature on this. Back some years ago, there were patients reported who had narrowing of their venous sinuses. Not thought to be thrombotic. Just narrowing. And the question was: Is this the chicken or the egg? Is the venous sinus narrow because there’s increased intracranial pressure in the fluid that’s pushing on the venous sinus and narrowing it? Or is this narrowing of the venous sinus causing increased intracranial pressure? And indeed, the answer is… Probably it could be both. So there are patients reported who are found to have narrowing of the venous sinus, they get a spinal tap, they lower the pressure, and then they repeat the MRV, and they find that the venous sinus narrowing is gone. And so in those patients, it probably is increased spinal fluid pressure causing narrowing. But there are also patients where it doesn’t go away. And if you do an angiogram, and you put your catheter across that narrowing, you can measure a gradient in the pressure. And if you can measure a gradient in the pressure, and you can put a stent in there, you can actually cure the venous sinus pressure and the increased intracranial pressure. So in this review article I quote here, they looked at 143 patients from a number of studies and reported high rates of success, technical success, and improvement in conditions. So this is not a first line treatment of IIH, in my opinion. I would not consider this. Like I would not consider other surgical interventions, in most patients, until they fail medical therapy. And we’ll talk about that in a moment. So the treatment… As we talked about… Weight loss. I always recommend that. I start people on Diamox or acetazolamide, a gram a day, increase as needed. I reserve surgery for times when there is severe headache, perhaps, or moderate or severe vision loss at presentation. I reserve optic nerve sheath fenestration for patients with moderate or rapid severe vision loss without headaches. Here’s a flowchart. It’s hard to do a cookbook kind of a thing for this. So my approach to papilledema or bilateral disc swelling — obviously you have to make sure it’s papilledema. Not any of the other entities we talked about. So you’re gonna establish a diagnosis, get an MRI and a spinal tap. If you establish the etiology, meaning there’s a brain tumor, venous sinus thrombosis, a problem in the cerebrospinal fluid — so all of these things mean you don’t have IIH — then fix it. Whatever that problem is. However, if you don’t identify an etiology, now you have a patient with, by definition, idiopathic intracranial hypertension. If there’s no vision loss, or very mild, like we showed you, you could use weight loss and/or medications. Your choice. Certainly always recommend weight loss. However, if the patient presents with vision loss, or if they are losing vision on your medical treatment and weight loss, now you’re gonna talk about more aggressive treatment. That could be shunting or stenting. And if the patient has some loss of vision and headaches, they might prefer the shunt or the stent. If they have really no headaches but loss of vision, then optic nerve sheath fenestration is an option. So this is not 100% absolutely gotta do it this way. And there are — some people just really don’t want a shunt or a stent. Some people don’t want a fenestration. So it depends, of course, on the patient as well. So this is sort of the way I approach it. I’m gonna run through a few cases to try to illustrate the thought process. So here’s our patient. This is the one we started off with, thankfully. This is sort of my typical patient who comes in with papilledema. Far and away the most common. Young overweight woman, has some headaches, intermittent blurred vision, TVOs, or transient visual obscurations, they’ve gained some weight recently as well, their exam is normal, except for the visual fields and the fundus appearance. I’ve shown you this before. So they’ve got the big blind spot in both eyes. In the right eye, there’s this primarily inferonasal loss of vision. So what are we gonna do with this patient? We’re going to… Whoops. I’m sorry. I guess I’m asking you. So what we’re gonna do, of course, is… Since I didn’t tell you the MRI and MRV is already done — we’re gonna do an MRI and MRV. That’s normal. And the CSF pressure is 36. So the question is: now what are you gonna do? How would you treat this patient? And so let me see what people think. Some of this is opinion, but let’s ask the question on the poll. So would you do weight loss first? Would you do acetazolamide first? Would you do optic nerve sheath fenestration or venous sinus stenting in this patient? All right. I’m gonna give you five, four, three, two, one. Let’s stop the poll and see what people said. Very good. So the majority of you are gonna either use weight loss or Diamox, or maybe — I didn’t give you the option of both. Maybe most of you would pick both. I think you could go either way. You could go weight loss only. And I talked to the patient about this. You could go weight loss only and say — listen, we could go just with weight loss, if you think you can lose weight. A lot of my patients say… I think I can. Some say — I’ve been overweight my whole life. I know I can’t lose weight. You’ll say try to lose weight, and we’ll start the acetazolamide. If the patient says I think I can do it with weight loss, I’ll say great. See you in six weeks. And we’ll repeat the visual field test. If they say they can’t lose weight, I’ll say try to lose weight, but we’ll start the medication. Or if they have really bad headaches, I’ll start with the medication. I’m not gonna start with the shunts. They’re not foolproof. Neurosurgeons don’t like doing shunts much. They can fail and require revisions, and so on. And I certainly won’t go to venous sinus stenting. There was no narrowing of the venous sinus. So the one thing for sure you’re not gonna do is venous sinus stenting. And I’m not gonna do nerve sheath fenestrations or shunting, because this person has not failed medical management and does not have severe vision loss. So the patient had a normal MRI and MRV. Pressure was elevated at 430 millimeters. Composition was normal. We made a diagnosis of IIH. Recommended weight loss, discussed Diamox. The papilledema resolved over 3 months. I usually with treat the patient, if they’re being treated with Diamox, or acetazolamide, at least a year before I try to get rid of it. Another patient. 52-year-old man has headaches. Notes tinnitus. Memory difficulties. The MRI is normal. He’s obese. He’s on no medicines. He has a similar sort of visual field. He’s 20/20 OU. He’s got no APD. Visual fields show big blind spots, bilateral disc swelling. And the question is where are we gonna go from here? I don’t think I’m gonna ask the audience. Looks like papilledema. He’s got bilateral disc swelling. You’ve got some big red flags for IIH. He’s a man. He’s over 50. I’m not thinking IIH. He got an MRV, because he only had an MRI to begin with. We added the MRV. It was normal. He had a spinal tap. In the morning, his pressure was 29 centimeters. His spinal fluid was normal. So we did a sleep study. He was a snorer. And he had lots of apneic episodes. In fact, the sleep study doctor called me and said — you saved his life. Wow. I don’t get to do that very often. We treated him with continuous positive airway pressure, or CPAP, and he improved over time. The third case is a 22-year-old woman who has headaches, already had an MRI, but she’s not overweight. Not on any medications. And she has what looks like bilateral optic disc swelling. So the red flag here is that she is thin. She’s not overweight. So we’re thinking strongly this is not going to be IIH. And so in her case, she hasn’t had an MRV yet. Just the MRI. So clearly we’re gonna get the MRV. And in her case, she had venous sinus thrombosis. Yes. All right. Last case. Another 22-year-old woman. Who’s overweight. She’s on no medicines. She has headaches. She already had MRI. She’s 20/50 and 20/80. So she’s got decreased vision. This is not my usual patient. Bilateral disc swelling, no relative afferent pupillary defect, and her visual fields look like this. So this is someone who has probably — probably has chronic papilledema, has lost a lot of their vision, and they’re now beginning to lose their central vision. That’s what’s brought them in for exam. So fortunately this is not a common scenario. So we need to establish that she does have increased intracranial pressure. She already had the MRI. You could do the MRV. In her case, we just went right to the spinal tap, showing increased pressure. And she underwent optic nerve sheath fenestration, because of presenting with really bad optic neuropathy right off the bat. We didn’t waste time trying to lower pressure with medication or weight loss. We went right for optic nerve sheath fenestration. So I don’t have a summary slide — sorry, I should. But that’s what I have to say about bilateral disc swelling. Remember, not all bilateral disc swelling is papilledema. You’ve got to consider the other entities that we talked about. Sometimes it can be just born-with small optic discs. Sometimes it can be buried optic disc drusen. Maybe it’s one of the unilateral things that presents bilaterally simultaneously for some reason. So you’ve got to first make sure that it is papilledema, meaning bilateral disc swelling due to increase of the intracranial pressure. Once you’re pretty sure you’ve ruled out the other things, in the US, at least, getting MRI, MRV, to make sure there’s no tumor or venous sinus thrombosis. If you don’t have that ability, hopefully at least a CT scan. And then you’re gonna treat, as we talked about, if there’s a — if you identify a cause of the increased intracranial pressure, treat it. If you don’t, and you think the patient has IIH, you’ve got weight loss, medications, and the various surgical procedures that we’ve talked about. Okay. So I am going to answer questions. If you have other questions, feel free to type them in at any time. And I’ll answer the first question, which was actually sent in before we even started, which is: I have two young patients with bilateral disc swelling, secondary to renal failure, and arterial hypertension. My question: How long should I follow them as an ophthalmologist? That’s a good question. I guess most of the patients I see — and I don’t see a lot, fortunately, who have disc swelling from hypertension, of course, you have to fix the hypertension. Hopefully the hypertension is fixed. But once the hypertension is fixed, the swelling should go away. And so I have not had a patient who had chronic disc swelling, chronically elevated hypertension — systemic hypertension — that I couldn’t fix. If I did, I would probably follow them, wonder why they couldn’t fix it, but I would probably follow them on a gradually decreasing frequency with visual fields, to make sure they’re not losing vision. The problem is I’m not sure what I would do if they were, other than fixing the underlying arterial hypertension. Okay. Please explain about the Foster-Kennedy syndrome. So the Foster-Kennedy syndrome is when you see a patient, and one optic nerve is swollen, and one optic nerve is pale. And a true Foster-Kennedy syndrome is due to an intracranial tumor that initially is probably causing just compression of one optic nerve. So that optic nerve is damaged. It becomes atrophic. And eventually, that tumor gets big enough, intracranially, that it causes increased intracranial pressure. So you see papilledema in the swollen contralateral nerves. That’s a true Foster-Kennedy syndrome. It’s very rare these days. Because of imaging studies. The most common reason you see a pseudo-Foster-Kennedy syndrome is an AION. So if they have an AION in one eye and their disc swells and they become atrophic, you know that 15% of people develop an AION in the other eye, where it looks like a Foster-Kennedy syndrome. It is not a true Foster-Kennedy syndrome. It is a pseudo-Foster-Kennedy syndrome. If a papilledema patient is controlled with acetazolamide, how do I wean her off it? A lot of my patients will start on the acetazolamide, I’ll see them in fairly short order, to make sure they’re not rapidly losing vision. I’ll see them in the 6 to 8-week ballpark. I don’t expect the papilledema to be gone in 6 or 8 weeks. I’ll say great, your visual field is stable or improving. I’ll see them in two or three months. I expect the papilledema to be gone at that four month check. If it’s not, we talk about increasing the Diamox. If it is gone, I’ll say great, you’re doing well, keep losing weight, stay on the medicine. We’ll see you in three or four months. Then if you’re doing great, we’ll consider decreasing the Diamox dose. Usually I ask them — if they’ve been doing well for at least a few months, my question is: Hey, do you ever miss a dose of the Diamox or acetazolamide? Usually if people are honest, they’ll say… Once in a while. I’ll say what happens? If they say I don’t even notice I missed it… We could try stopping or decreasing your dose to 1 a day or 2 a day. Sometimes we do that, the patients call me in a week, and say the symptoms are coming back. Okay, it’s too soon. We’ll repeat in three or four months. If the patient says sometimes I miss a dose and within hours I can tell, it’s too early to start tapering the dose. The question about meningitis. So meningitis can cause increased intracranial pressure. So the MRI will probably be normal. But of course, the spinal fluid won’t. And so you can see a papilledema — well, you can see bilateral disc swelling or papilledema with meningitis. So meningitis can cause increased intracranial pressure. It can also just cause disc swelling itself. The key is that you diagnose it when you do the spinal tap, which is one of the reasons you do the spinal tap. And meningitis is not gonna present usually like idiopathic intracranial hypertension. Usually the person is sick, they have sudden onset of bad headaches, and so on. So it’s usually not the same presentation. But that’s where the spinal tap comes in. Could systemic steroids increase ICP? So there is some literature on steroid use, either using steroids, or withdrawing steroids, causing increased ICP. It’s not the greatest literature. But the answer is that we think that it could. So the answer is yes. And also, could IIH present with optic disc edema and some small hemorrhages, bilateral? Definitely. So you can see any degree of papilledema with IIH. It can be mild, it can be moderate, it can be severe. I’ll leave it at that. You can have marked papilledema with exudate in IIH. Next question. In optic disc swelling, is bevacizumab or… I have trouble pronouncing that… Ranibizumab… Are they effective? The answer is… Of course, well, I guess it depends on what kind of optic disc swelling is it. There’s some literature — this is a big question. In papilledema, no. You’re not gonna treat it with… You’re gonna get rid of the increased intracranial pressure. So I have no knowledge about using that in papilledema. In unilateral or bilateral disc swelling, from AION, there’s some literature. It’s not great literature. I personally never use either of those medications for optic disc swelling. That is primary optic nerve problem. If that answers the question. How long is acetazolamide prescribed for? And are there any markers to taper it down? So, again, I think I answered that question. I guess let me go a little further. And that is that… So in that scenario I described, let’s say we’re tapering the Diamox, and the person is on — we’ve treated them now for 8 months or so. Disc swelling is gone, headaches are gone, they’re doing great. Hopefully they’ve lost some weight, to boot. I’ve already decreased them from 1,000 to 500, still doing great, I stop the Diamox, see them in a couple of months, just to make sure they’re doing well off the Diamox. And then I cut them loose. If they’re doing well, we tapered the Diamox for a year, I say listen, you’re doing well. If you gain weight, it’s possible the condition will come back. It could just come back. So don’t assume you’re cured forever. Let me know if you’re having symptoms. I would much rather see you and say nope, you’re doing great, then have you assume you’re cured forever. So don’t do that. If the patient comes back, and there is disc swelling or recurrent symptoms, which happens once in a while, I’ll say okay, it’s too soon. We’re gonna bump you up and do the same over the next year. All right. Do you consider neuroimaging in all cases of disc swelling, even the unilateral ones? No, I don’t in all cases. Although when you consider the causes of unilateral disc swelling, certainly a lot of them I’m gonna image. I don’t image someone I think has a typical non-arteritic typical optic disc neuropathy. Who else do I image? I image all my patients with typical optic neuritis. If I think it’s atypical, I don’t necessarily image them. So I think that would be the answer. Is there a place to follow a patient with IIH on treatment with MRI or MRV? None whatsoever. I never repeat imaging. The only reason I might repeat imaging in someone I think is IIH is if they’re being treated and we’re having trouble controlling them with medicine. And the question of venous sinus narrowing comes up. Because we’re considering venous sinus stenting. That would be the time that I would repeat an MRV, to see if there’s venous sinus narrowing that would then cause me to refer the patient to our interventional neuroradiologist. Because if they don’t have venous sinus narrowing, I’m not gonna send them to the interventional neuroradiologist. How long after lowering CSF pressure does papilledema resolve? That’s an interesting question. Probably the best evidence for that is, if you do an optic nerve sheath fenestration, you know exactly when the spinal fluid pressure is zero. And in that case, usually I see the disc swelling resolve in the 1 to 2-week ballpark. The alternate question is: How long does it take for CSF — for papilledema to develop? We don’t know for sure. There are various reports of as short as a day. There was a study in Germany where they looked at people admitted to the ICU with head injuries, who all had intracranial bolts to measure head pressures 24 hours a day, and they looked to see who developed papilledema. And not all developed papilledema in the first week. Of course, some people died because they had head injuries. But very few people developed papilledema in that time frame. Please discuss optic nerve fenestration. Well, in the United States, at least, it’s typically done either by neuro-ophthalmologists or orbital and oculoplastic surgeons, typically. And I used to do nerve sheath fenestrations, but when I moved to Cincinnati, where I have my current job, we had several oculoplastic surgeons do them, so I let them do them. So the most common is oculoplastics, neurosurgeons, orbital ophthalmologists. You can use a lateral or temporal approach or a medial approach and just get back behind the globe and grab the nerve sheath with forceps and cut a little hole in the nerve sheath, and you see this initial gush of CSF and know that you’ve done it, and that’s it. For those of us who do not have routine access to spinal taps, can we make the diagnosis of IIH only with a CT scan? So that’s really tough. There are findings you can look for on the CT scan. And I didn’t really go over those. The problem is: The findings on neuroimaging and the findings are mostly described on MRI — but the findings on MRI you might see on CT. And the findings are flattening of the back of the globe, so there’s — just a little flattening of the globe in the back. At the disc. Because there’s a distended optic nerve sheath that puts a little pressure on the back of the globe. So that’s one finding. What’s called “generous” fluid in the nerve sheath, but of course, you’re supposed to have fluid in the nerve sheath. And then partially empty sella. Are the findings that have been reported. The problem is that all of the findings you might see in a normal individual. And so there have been papers reported in Neurology — and one of the problems is that these days, at least in the US, I routinely see patients who — they have an MRI for whatever reason, and at the end of the report, it says… By the way, there’s a partially empty sella, and there’s fluid around the optic nerves. Rule out papilledema. And then the patient gets referred to me, and they’ve never had papilledema. But without a spinal tap, very difficult. You have to go on your clinical impression. Which, again, would typically be… I mean, the time where I would be most comfortable would be someone who presents bilateral, moderate, or worse optic disc swelling, no significant loss of vision except big blind spots and nasal field loss. Not much will do that. But if they come in with bad vision in both eyes, central vision loss, it’s really tough, and you really need a spinal tap. All right. Please explain the difference between disc swelling and disc elevation. So about 10%, 15% of everybody has elevation of their disc. And it simply means — that’s the way I explain it to patients — if I could run my hand along the back of your eye, in most patients, your optic nerve would be flush with the back of the eye. In other words, my hand would just pass smoothly right over the top of your optic disc. But in a percentage of people, there’s slight elevation, because the nerve fiber layer is heaped up a little bit. That hole in the back of your eye is small, and you have a small, crowded disc. There’s a little elevation of that disc. So to me, there’s a big difference. When I use the term elevation, I mean normal. When I use the term swelling, I mean not normal. And the problem is, as I tell patients, if the nerve is swollen, it all still looks elevated. How often do you follow up patients with papilledema for visual function? It depends on the field. If the field looks good, I’ll see them in 6 to 8 weeks. If they come in with borderline vision where I’m almost ready to recommend surgery, I might see them in 4 weeks. As they prove they are stable with minimum vision loss, I’ll gradually put more time in between the fields, and as they resolve the papilledema, once the papilledema is resolved, I don’t (inaudible). How often do you follow up new cases of papilledema? I think I just answered that. What would you do with a 20-year-old female patient with pseudotumor cerebri since three years? I assume that means it has occurred for the last three years. No or little weight loss, topiramate, Diamox not tolerated. If they’re not having a lot of headaches and the fields look pretty good despite the papilledema, I think you can follow them. But you’re probably gonna be following them for a long time. If there’s any progression on their visual field test, or bad headaches, then I’m gonna think about surgical options. And the surgical options would include shunting, I probably — depending on the headaches — I may or may not recommend fenestration. But if their vision is getting worse, I would consider fenestration, I would consider shunting, and if their MRV shows venous sinus narrowing, I would consider venous sinus stenting. All of the above. Duration of action of acetazolamide is about 6 hours. Is it enough to give it twice daily? The answer is no. I see patients quite frequently who are started on the non-time released form of this twice a day, and I tell the patient — look, you’re getting it, your blood level goes up, and six hours later, it goes down, and six hours later, you get nothing. You need to use it four times a day, if it’s the non-time released form. The non-time released. If it’s the time released form, which I prefer, but it’s more expensive, then you can do it twice a day, and it’s a lot easier for the patient, of course, because everyone knows that if you tell someone to take a medicine four times a day, they’re probably not going to. If the patient doesn’t tolerate Diamox, dizziness, bad kidney function, can’t lose weight in early stage of IIH, what is your next step? Topiramate, furosemide, and if none of that works, and the vision is worsening, bad headaches, the surgical alternatives we discussed. What is the role of steroids in the treatment of optic disc swelling? In the treatment of papilledema, there’s no role of steroids. That’s old literature. The only time I’ve ever used steroids in papilledema is in someone who presents with terrible vision, and I want to… They’re gonna be getting surgery and lowering their pressure in the very near future, but they come in legally blind, recently. I might use some IV steroids while we’re getting them ready for surgery. I don’t think there’s good evidence for that. But in terms of general papilledema, there is no role. In terms of steroids for optic disc swelling in general, that’s a whole nother lecture, because we would have to include things like optic neuritis. And I believe there’s a recorded previous webinar that covers that topic. Which I think was optic neuropathy part I. What blood tests do you routinely do, to work up for bilateral disc swelling that you don’t think is papilledema? Bilateral disc swelling — first of all, that’s a fairly rare scenario for me. Bilateral disc swelling that I don’t think is papilledema is pretty unusual. Because it’s gonna be either something they’re born with, in which case I don’t get any blood tests, some of the things we mentioned, if I’m worried they have unilateral… I’m sorry, bilateral simultaneous presentation of a condition that’s often unilateral — for instance, giant cell arteritis presents with sudden bilateral vision loss, I’m gonna check CBC, sedimentation rate, C-reactive protein. If it’s atypical optic neuritis, I’m probably gonna check a neuromyelitis optica antibody. In my world, I’m gonna check for sarcoidosis, with angiotensin converting enzyme. If you live in an area of tuberculosis, you’re gonna check for that. Syphilis, you’re gonna check for that. Any other infectious etiology, depending on where you live in the world. Check for those things. Trying to think of what else. I guess those would be the main things I can think of. All right. If I see a child with presumed pseudopapilledema, those borderline cases, which are really common, where there’s some elevation, but not really necessarily swelling, and the person can’t do visual fields… So those are tough. Because especially if the kid is too young to do visual fields, which is really the most important test to ascertain the vision, because of what we’ve talked about, because it can be peripheral — how often should I follow this appearance and any other tips to manage? Clearly, if you haven’t, you’re gonna do a B scan. Hopefully you have a B scan ultrasound, to rule out buried optic disc drusen, which would be a very common other possibility. But if you have the B scan, and the nerves just look funny, you don’t have visual fields, that is a tough situation. I mean, I definitely have kids in that age range where they get the imaging, it’s normal, and depending on how suspicious I am, they get a spinal tap. And as long as of course the spinal tap is normal, then it’s pseudopapilledema. But there are scenarios — and probably once or twice a year I have a patient where I just can’t tell, and they get an LP, MRI, everything is normal — you’ve got pseudopapilledema. I’ve got some real outliers where I would look at those nerves and say boy, this is darn suspicious. What about management of Diamox dependence? How long can it be continued? Forever. As we’ve discussed already — you’ve probably heard my answer. But I’ve had people on Diamox for a decade or longer. How frequent visual fields done in a follow-up case — again, it depends on the vision. If they’re doing well, I gradually put more time in between. I covered that. Another how frequent — same thing. Is there a risk of hypoperfusion after optic nerve fenestration? So the main risk of optic nerve sheath fenestration isn’t hypoperfusion afterward. It’s that there have been some reports of central retinal artery occlusion. And again, we think this is pretty rare. And especially with improved surgical techniques. But in the past, you had to pull on the eye to get that nerve, and the pressure on the globe during surgery — where people woke up with worse vision and a CRAO — I think that’s pretty unusual these days. I have not had that happen in any patients, at least with my surgeons, in the last ten years. But we don’t think that the nerve sheath fenestration has some other long-term risk to the optic nerve. What is a success rate? Well, the success rate is hopefully 100%, for getting rid of the papilledema. It’s certainly not 100% for reversing and allowing full recovery of vision. It depends on where you start. So if the patient already has an atrophic nerve, and a visual loss for a long period of time, the main goal of the fenestration is to prevent further loss of vision. The second goal is hopefully to have some improvement in the vision. What is the critical time period to avoid permanent vision loss in papilledema? The problem is it’s extremely variable. So most people with idiopathic intracranial hypertension, which is by far the most — I mean, I have hundreds of patients with this condition. Most people, it takes a long time. But there are those occasional patients who seem to have what’s been called in the literature malignant pseudotumor cerebri, where over a month they get worse. That’s why when I first see them, they have pretty good vision. I want to see them in the fairly near future, after establishing diagnosis. 6 to 8 weeks. And I tell them — if you think your vision is worsening in the mean time, let me know. Don’t just wait the 6 to 8 weeks to come back. Spinal tap in IIH is therapeutic? Almost never is it therapeutic. The person with high pressure will have resolution of their headache on the table. Your spinal fluid turns over twice a day. So typically the next day the headache will be back. If you have a leak, that’s another story. But almost never is it therapeutic. On rare occasions is it therapeutic. I never recommend repeat lumbar punctures. It’s an old and in my opinion barbaric treatment. Hopefully not being done any longer. What would you do with a patient who is referred with longstanding papilledema and very poor vision? Lower the pressure. If I see someone with longstanding papilledema and they come in, and their first visit, they’re hand motion or count fingers, 20/200, 6/60, or worse, constricted visual fields — even not that bad — I would recommend surgical intervention. Probably optic nerve sheath fenestration. How do we manage a patient with bilateral disc swelling, due to acute mountain sickness? That’s interesting. I’ve never seen that. Well, I guess the question is why. I don’t know the answer as to why they have bilateral disc swelling. Is it… If it’s because they have increased intracranial pressure, then I would lower their pressure. And of course, I know that acetazolamide is used for altitude sickness. I would use it that way. And I assume if they have acute mountain sickness, it would get better. I’m sure you’re more experienced than I. But it would get better fairly rapidly after they come down from elevation. But it would depend, I think. If they had terrible vision on presentation, I would consider being aggressive with — provided it’s due to increased intracranial pressure, I would think of surgical options. If it’s mild, I would think of medical options, if it’s gonna go away shortly, because altitude bilateral disc swelling goes away rapidly, I just don’t know the answer. How long can we give acetazolamide? There’s no time limit. Thank you for the lecture. Good. Will the lecture be uploaded. Yes, it should be. The recorded lecture should be uploaded. On the Cybersight website. How long do you follow vision up with papilledema? I think we’ve discussed that. Again, it depends on… I follow up with patients who have persistent papilledema. I follow them ’til it’s gone. Have you had any experience of treating optic disc drusen with field defects with Diamox? No, I wouldn’t do it. I can’t think of any reason and certainly there’s no evidence base on it. Theoretically, why would you use Diamox? Theoretically, there’s some… The answer might be getting back to… Well, why do disc drusen cause optic neuropathy? And the answer, we think, is probably that the optic disc drusen are crowding the axons. So one line of thought is… Well, if they’re crowding the axons, and maybe crowding the blood supply, if we lower intraocular pressure, would that help? And so sometimes I see people who are put on drops to lower eye pressure. And then the other side of the coin, for acetazolamide, would be… Well, what if there’s… Maybe if you lower the CSF pressure, there would be less pressure on the posterior part of the optic disc. And so theoretically, those would be the reasons to consider Diamox. I certainly have never treated somebody with disc drusen, with Diamox. Is there a role for OCT in monitoring patients? Ah-ha! The answer is probably. I am too old… I’m not an OCT fan. That said, I believe I’m gonna have to become an OCT fan. I think that following the OCT patients, the problem in following papilledema in OCT is… If you’re looking at thickened nerve fiber layer, because the fibers are swollen, and it’s going away, the question is: Well, is that because you’re losing axons, or because the papilledema is getting better? I do have colleagues who are big fans of OCT and papilledema, and OCT in even helping differentiate mild disc swelling from elevation. I think the jury is still out on that. But I’m becoming more of a believer in maybe ordering more OCTs in papilledema. Can I follow a child with OCT if I suspect idiopathic intracranial hypertension? The answer is you can follow them. And I guess the question is, if it’s a child that can’t do visual fields, perhaps that would be a good way. I don’t know of any publications about that, but that’s an interesting idea. Maybe the kid who can’t do it, who can’t could the visual field testing, maybe the OCT. Again, I think you run into the problem of: if the nerve fiber layer is thickened from swelling, and the nerve fiber layer then is getting thinner, is it because you’re losing axons — bad! Or because the swelling is getting better — good! And I’m not sure how you would answer that question. But I’m not an expert on OCT. And the final question. Do all visual fields improve completely after lowering the CSF pressure after early intervention? No. Because it depends, again. The question is: What is early intervention? Some patients just don’t come in, until they have significant visual field defects. So the answer is you can sometimes see fairly dramatic improvement in visual fields. But early is the keyword. If the person comes in and there’s no loss of vision or mild vision loss, you can have resolution. The question is: When does the patient become symptomatic enough to seek help? Okay. Well, that was a lot of questions. I hope that was good for everybody. I think there were 42 questions. And I hope everybody learned something. And I don’t think we’ve discussed yet what the next neuro-ophthalmology topic is. If you want, I think there’s a postwebinar… I think it’s gonna be imaging, actually. There were some requests for a neuroimaging webinar. So I think that will be the next one. But yes, additional questions can be asked on the consult service. Also there’s a webinar evaluation form that asks about topics for a future webinar. So answer those things. And I look forward to seeing everyone in the next Orbis/Cybersight webinar. Thank you.
April 17, 2017
1 thought on “Lecture: Approach to the Patient with Bilateral Optic Disc Swelling”
This was awesome! Thanks, Doc!