Hey, everybody. Thanks for having me today. My name is Shannon Duscher. It's a real pleasure to be here with y'all, and I thank AANS for the opportunity to chat with you guys today about this really interesting topic, intracranial pressure disorders. So again, my name is Shannon Duscher, and I am the head PA for neurosurgery at MedStar Georgetown University Hospital. I'm also one of the APPs who helped start our CSF Leak Center that was recognized by the Center for Spinal CSF Leak National Foundation here in Washington, D.C. We have a large quantity of patients with CSF leaks of all types, and so the focus of my talk today is to hit on what we see in our practice as well as what's being done nationally around the country to help these really interesting patients. We'll also hit on IIH towards the end, given the opposite side of the spectrum with these leak disorders, and talk a bit about what we're doing to treat that as well. No disclosures today. And objectives. So today we'll be talking about, as I mentioned, the clinical presentation, ideology of CSF leaks, specifically spontaneous intracranial hypotension, and idiopathic intracranial hypertension, also known as pseudotumor cerebri. We'll be talking about the diagnostic pathways, physical exam, common tests for both of those conditions, and also some interesting radiologic findings you can see on radiology studies in CSF leak and in IIH. And then we'll kind of round it out by talking about some of the pharmacologic and surgical options that we have to address both these conditions. And really at the bottom of all that, what I want to drive home is the red flags and the don't miss symptoms that come with both these conditions so that when you see them in the office, you'll know what they are. I can refer these patients to centers that can help them if you are not equipped in your own practice. So we're going to start off our talk today talking about our spontaneous intracranial hypotension patients or SIH patients. And real quick, I want to ask in the chat here, if you guys can throw a response in, how many of you have encountered CSF leakers in your practice, specifically the ones that are not necessarily iatrogenic, meaning caused during a surgery, but the ones that are idiopathic and are not from a specific surgical intervention? I'll wait a few moments here to see if anybody responds in our chat. All right, getting some responses in. Very cool. Very few, some frequently, some every year. Awesome. One or two a month. Great. Wow. Really great. That's a lot. Wow. I'm surprised. Well, thanks for your responses. I appreciate that. So I want to start by, it gives me an idea of how often you all might be seeing this and kind of give some framework for this next part of my talk. So I want to share a quick story. So CSF leak patients, I find can get a little bit of a bad rap. Some of them will come in with a large quantity of information of treatments they've tried. Sometimes they can be a little histrionic, sometimes, not always. They can be complicated and really at the end of their rope, they can have comorbid conditions like anxiety and depression. And a large part of this is because their diagnosis gets missed on outpatient practice, either primary care, neurology, neurosurgery, and they get shuttled around from area to area, trying to find out what is going on with their symptoms. And so I want to talk about this patient, MG. She gave me permission to share her story. She was one of our leakers that we met a few years ago. And MG was exactly like this. She was someone that had come in and when I met her, she was laying on the floor of our lobby on a bedsheet. I was walking down to clinic and I see on the doorway of our lobby that there is a head sticking out from the doorframe on the floor. And so of course I run out and I say, oh my goodness, are you okay? Have you fainted? And she very calmly looks at me and says, no, I just can't sit upright for more than 10 minutes. And of course, we got her back to her room. We evaluated her pretty quickly and I got to know her and her story. She had some unusual symptoms and some unusual headaches. But beyond all the information that she brought into me and the hour and a half we spent together, she also told me that prior to four months of our meeting, she was a college grad, majored in engineering. She was engaged to be married. She was living independently, completely functional, and said to me that the rug simply got pulled out from underneath her. And that really resonated. She was someone that was completely, completely independent and then became very unlucky with an idiopathic spinal CSF leak. She is now doing really well, married and has a baby on the way, and we'll talk about her a little bit later. But I just want to drive home that although these patients can be really challenging and really needy because of the complexity of their condition, I always remind myself that a lot of times they are patients that weren't independent, who just got really unlucky. So I share that story to kind of hopefully give some perspective as to often how these patients are existing prior to them getting these awful conditions, CSF leaks. So a quick review, CSF pathway and purpose. So chord plexus creates the majority of CSF in our body, mostly bi-appendable cells. It runs through the ventricles. For any new grads in the room, just a reminder of kind of how things flow along here. We've got our lateral ventricle, runs through our third and fourth via a network of foramen, monrose, sacral aqueduct, all that good stuff, goes all the way down towards the fourth ventricle and then out through our two apertures. So we have two lateral apertures and one medial, eventually into the subarachnoid space, and then out through our dural amino sinuses and reabsorbs into the general circulation via our arachnoid villi or arachnoid granulations, which kind of act like a one-way valve of the CSF back into general circulation. You create about anywhere from roughly half a liter a day, somewhere around 400 to 200 CCs, and you turn that amount over a couple of times as well. So the CSF you woke up with in the morning is not what you went to sleep with at night. All right, so let's dive into our first part of our topic here, which is what is SIH? And put very simply, SIH is a hole where there should not be a hole, right? The dura should be pretty much a solid connective piece here, and when we have a leak that occurs in the dura, spinal fluid can leak out into places where it shouldn't be and cause a host of symptoms. We can have CSF leaks in our brain, in our spine, and we think of some of the common causes, right? So surgical iatrogenic ones, meaning you're doing a microdisc, you have a kerosene, you take off a little piece of bone, and some dura comes with it, and everyone goes, oh my god, and then you sew it back up and take your precautions after surgery, and patients usually do fine, which can also, of course, present in a delayed fashion, you know, a couple days after the original surgery if the original leak was missed. You've got your traumatic types of CSF leaks, so clear, messy fractures, combinated fractures that rip or tear the dura along brain, the cranium, or along the spine. And then the focus really today that I'm going to talk about is these idiopathic leaks. They're the leaks that happen when the leak is not related to a surgery or a trauma. So let's launch into our CSF leaks in the spine first. So the don't miss symptom of this condition with the CSF leaks in the spine is this positional headache. It's going to happen in over half of patients, and it's going to be very specific. They're going to come in and say, when I sit up, I feel worse, and when I lay down, I feel better. And that's really because the brain is not being held buoyant from this constant loss of fluid, this constant leakage of spinal fluid out where it shouldn't be. They often usually will have a host of other symptoms, you know, hitting multiple positive symptoms on their ROS screen, five, six different symptoms, and they can include things like nausea and vomiting, tinnitus, dizziness. Changes in hearing can be either muffled as well or tinnitus. You can see photophobia, neck, back pain. Brain fog is a big one that I hear really a lot, and it's not so much, you know, perhaps the word finding, although we hear that, but a lot of folks will describe it as a processing speed thing where they're just not as sharp as they used to be, or they're having problems at work processing information. Gait disturbance as well we can see. And this usually, CSF leaks in the spine in women that happen adipathically usually happen in women, about a two to one ratio, typically anywhere from age 30 to 50, typically middle age, but can be seen younger. And in terms of a differential, we can see things like atypical migraine, vestibular migraine, orthostatic headache, patients that have headaches when they sit up or sit down. Chiari malformation is a big one on radiographic imaging. We'll explain why CSF leaks in their brain findings are different than how Chiaris look. And often you'll see some comorbid conditions. I mentioned anxiety, depression. You also can see POTS, Marfan's, some EDS, other types of connective disorders. You'll find sometimes in the literature, a higher rate of these CSF leak patients. So over the last 20 years or so, we've made a lot of progress in how we identify these leaks and types of leaks that exist. This is a really great image on the right-hand side here that kind of explains the different types and what they look like in a cartoon version, so to speak. So type one tears are going to be these dural tears that occur, usually come on osteophyte complex, so the disc osteophyte complex. So a sharp piece of bone and it gets pushed into the dura and there goes your hole. You can have them leaking ventrally. They can leak posterolaterally. Type two, meningeal diverticula. So this is the type where you have a little bit of meninges coming from the nerve root that kind of bulges outward and can leak. They're simple and complex. And then CSF in the fistulas are becoming more and more common. And it's noted in the literature that we may have been missing these for some time just because of the lack of diagnostic modalities that have existed to identify these. But they are an abnormal connection between one of the tiny veins on the side of the spine that can sort of enter the subarachnoid space and create this fistula where there should not be a fistula. They are especially hard to find because unlike some of our other types of CSF leaks, these don't create epidural fluid collections. This is a direct dumping of CSF into the venous system and then out into the body. So still a leak but much harder to catch. So our workup for these spinal leaks, and this is really for all of you folks out there who are not near a leak center or who might be out in other areas of the United States where you suspect this and want to really get a jump on if this is someone that has a leak. So the don't miss takeaway points from here are the initial workup which includes an MRI of the brain with and without contrast and an MRI of the cervical lumbar and thoracic spine without contrast. It's a lot of imaging. Side note, I have not had much difficulty getting it authorized through insurance when the CSF leak is the suspected diagnosis. So this is a note there. It has not been a big barrier for my practice, but you know, be worried. That may be something you need to think about. And the reason that we do the MRI of the brain with and without versus the spine without is because there are specific findings in the MRI of the brain that are only going to be seen with a contrasted study versus the MRI of the spine. We're really just looking for epidural fluid collections, abnormal areas and pooling of epidural fluid in the epidural space that indicate a leak at that site or somewhere nearby. And then when we have patients that have a suspected leak on imaging based on their symptoms and some of the radiographic findings, CT myelogram has really become the gold standard for determining these leaks. I should say myelogram in general has become really the gold standard, whether it's CT or dynamic, meaning that it's done under fluoroscopy versus CT. It's really site-specific. It is based on whatever center is more used to using. Digital subtraction myelography, the one under fluoro, has some drawbacks in that it typically requires some type of general anesthesia versus just sedation and the pictures need a trained eye to see them. CT myelogram has some benefits in that we can get higher resolution images, but of course it is more radiation. And the type of CT myelogram that is done is also based on the type of leak that we suspect somebody has. So for example, and I have a great slide on this later, type 1 leaks, these disgust-euphyte complexes that are causing dural rinse, you can often find by putting somebody in a CT myelogram prone, so on their bellies, because if it's a ventral leak, odds are during the myelogram, we will pick up the leak as it's happening during the scan versus a CSF venous fistula, which is typically on one side or the other. We can do a lateral decubitus myelogram on the side that we suspect there may be a leak. One question I've gotten before is, if you don't know which side it is, how do you pick a side? The answer to that is, anecdotally, we have seen more leaks on the right, but now we have graduates doing bilateral CT myelograms in our practice. So we'll do a CT myelogram on one side, flip them to the other side, and repeat. So it is a lot of radiation, but it does a great job of picking up those more subtle leak findings. I have a great video of this too. All right, so takeaways for this, for your initial workup, right out of the gate, MRI brain width without, MRI cervical, thoracic, and lumbar without contrast. So, this is great. So a couple, well, I guess, well, we'll get to this. So the radiographic findings in CSF leaks are really specific, and I listed a bunch of them here. So you can see brain sag, dural enhancement, engorged sinuses, pituitary hyperemia, subdurals, something called the pontimus isobelic angle, which I'll show in a moment, and then of course those CSF collections at the site of the leak, either thoracic or cervical typically. Most leaks that occur with the dysgastiofite type usually seem to be thoracic, so something to keep in mind. And we have this great little trick that we use to help determine whether we think someone may be a leak patient, have a leak along the dura somewhere. I'm going to flash forward to one second and talk about a burn score, and I'll come back to this picture in a sec. So the burn score is a series of criteria that a couple years ago in 2018, there was this great study in JAMA Neurology, enrolled about 152 patients, so pretty small, but all of those patients had confirmed spontaneous intracranial hypotension from a CSF leak on some type of myelogram. So they were all patients that we know had leaks, and they imaged their brains all the way, all the patients, and they found a series of things that were really specific. So some of these things on this slide, on the right hand side, you'll see that I mentioned previously. So again, that venous engorgement, the dural enhancement, some effacement of the supercellar cistern, again, you know, the pituitary can be a little big, so you see some supercellar effacement there. Subdural fluid collections, pre-pontine cistern can be closed down, and then something called the mammalopontine distance, it can also be closed down. And based on these criteria, they can help us figure out who needs a CT myelogram. The burn score runs from one through nine. A low score is anywhere from zero to two, so I guess zero through nine. Intermediate is three to four, and then a high burn score is anywhere from five to nine. So the takeaway for this slide is, if you have someone that is familiar with burn scoring, and you have an MRI of a brain of someone that you think might have SIH, you can use this burn scoring to determine, should I pursue a formal CT myelogram to see if the person has a leak somewhere? I'm going to go back one slide and show you this nifty trick. Four, five, six on this picture on the right hand side. So four refers to the supercellar cistern, so less than four supercellar cistern effacement, indicative of SIH, that's the point there. Pre-pontine cistern being closed down, less than five millimeters, and then the mammalopontine distance being less than six. And really, when you think about these image findings in a gestalt, on the whole, the entire brain is just sort of sagging, it's just down, it's not buoyant, and it's because they're losing this fluid out from a dural tear, or CSF, venous fistula. But somewhere along the line, they're losing CSF, and the whole brain is just tired, like Jabba the Hutt, just very, very, you know, just sort of not buoyant, right? Okay, any Star Wars lovers out there, I apologize, I love Jabba the Hutt, okay. And I also want to mention one common question that comes up, which is, why aren't these Chiaris? And the answer to that is, with Chiaris, usually you'll see that cerebellar tonsillar descent, but you won't see all the sagging midbrain signs I just described. You also don't usually see the venous sinus engorgement either. So although Chiaris and brain side can mimic each other, you won't have any of these other findings from burn scoring on here. I'm going to rock through these pictures real quick. These are just nice ones I pulled from, most of these are from my patients over the years, so normal T1 posts on the left, and then an engorged transverse venous sinus on the right here, more on this side than the other. This is the example of diffuse dural enhancement. This is really one of the big reasons that we get referrals from the community, is that somebody will get an MRI of the brain for headache, and they will see this dural enhancement, and they won't have meningitis, right? They won't have any other obvious causes of dural enhancement, and they will send them to us, and we will do a leak workup. So here's an example of some brain sag, okay? This is not a Chiari patient, they have less of these other midbrain findings, but this is an example of someone that eventually did have a leak, and was found to have some of this brain sag, but again, not that classic descent of the tonsil into the foramen magnum. I mentioned earlier this thing called a pontomacencephalic angle. This is an example of someone that had a positive finding here. So someone that has, again, this sort of sagging of the midbrain can cause a decrease in the angle here, less than typically 55, so more acute than 55 degrees is indicative, or is suggestive of, again, a CSF leak, the whole brain sort of sagging down. This is a great shot from Walter Scheivink, who is largely considered to be the most, if not certainly one of the world's leading experts on CSF leak. Had a chance to see him present earlier this year. It was awesome, like meeting your favorite superstar. Didn't ask for his autograph, though. That's one level too nerdy for me. But this is an example of someone that he treated, and this is on the left-hand side here. So you can see a bunch of those positive signs. So pituitary hyperemia, pre-pontine cistern effacement, that's here, sort of sagging of the tonsils, again, the ICRE, and then post-surgically after his was repaired, you can see the pituitary hyperemia has improved, the pre-pontine cistern has opened, and also the ventricles are actually back to normal size, which is kind of fun. So you do see some improvement there. I'm going to hurry up here. So some more pictures on the left-hand side. This is a CSF venous fistula on the left at T10. This is some abnormal dumping of contrast out here. Someone I just saw last month or two months ago, we did a myelogram on who had some dumping into the paraspinal vein. Just the top of that picture there of the arrow is sitting. We just recently repaired him. He's doing quite well. On the left-hand side, this is an example of a leaking meningeal diverticulum on a digital subtraction myelography. And this is an example of what's called a SLEC, or a spinal longitudinal epidural collection, or an abnormal CSF collection, where there should not be one. Quick video from one of the patients from last year. You'll hear me say go, and then as we're doing this lateral decubus CT, we see this dumping out here of abnormal contrast coming out through one of the veins on this patient's left-hand side. No, right-hand side. Yeah. Okay. So interesting. So treatments for this non-operative, of course, we have blood patches. And there is some suggestion that an earlier intervention with blood patch, these patients seem to do it better. They can require multiple blood patches, sometimes up to three, before they start to get any relief. In general, targeted seem to work better. So if you have suspicion of where the leak is you want to target, that seems to be a little better outcomes. But in a blinded blood patch, meaning that we don't know where the leak is, but we think you have one, they still seem to respond sometimes. And then, you know, fibrin glue is just another way of doing a targeted blood patch with fibrin that can seal off a leak and can be curative in some situations when we can really specifically identify the leak and go after it, usually done in interventional radiology. And then surgical treatments for this, again, I can't drive home enough the role of CT myelography here. The approach is dependent on the site of the leak and the type. So again, those ventral leaks versus CSF venous fistulas versus ventral ligands in particular, they all have different approaches. And having that CT myelogram in your arsenal to identify where those leaks, again, is so important when it comes to fixing these things surgically. In general, type three leaks, so those venous fistulas can be embolized. They also can be ligated. For our ventral leaks, we have a number of different options. We can do, believe it or not, transdural approaches, which just makes everybody in the room go, oh my God, number one rule, don't touch the spinal cord, right? But we can actually skirt around it or in cases through it and drain and then do a ventral leak that way, a ventral leak repair directly that way. I've seen it done. Of course, we have monitoring going the whole time with some success. We have laminectomy infusion for direct repairs, so posterolateral leaks. When we know we can see it not responding to fibrin patches, we can do a laminectomy, go after the posterolateral leak, do a direct repair, and occasionally fuse, although we're doing more minimally abrasive these days, and do direct repairs that way. If the amount of bony laminectomy or bony depression that's required is enough, then we will sometimes fuse, but it's less common these days. And then finally, I'll mention just we are doing more commonly these days these dural onlays, so we will put synthetic dura over a leak site if we are not convinced that we can fix it directly, and those patients are also still doing well, which is interesting. I mentioned lumbar drains here. They're kind of falling out of fashion with these leakers, but they can be useful in patients that end up with rebound headaches, right? Some of these folks have been living with these headaches for a long time, and we go and we plug up the hole, and all of a sudden they go into high pressure, and they get terrible high pressure headaches. If they are not responding to things like Diamox or other things that can kind of drop the pressure a bit, we will sometimes put lumbar drain in to give them a more gradual return to a normal pressure system. And then here's a great shot of a quick and dirty table that I came across that kind of does a pretty good job explaining when you might want to get a CT molegram versus not. So a quick description. So on the left here, we have head positive, select positive, meaning positive MRI of the brain findings, so burn score maybe five or six or something of that nature, plus an epidural collection. Those are probably going to be these type one or type two leaks that have those epidural collections associated with them, and you can consider pursuing either digital subtraction myelography or a CT molegram versus those type two leaks where you see a meningial diverticulum. You may want to consider those lateral types of CT molegram to really get a better picture of it. And then on the right here, you can see, you know, head positive, select negative. So earlier, we talked about CSF venous fistulas and how they don't have those epidural fluid collections because they're not dumping into that space, right? They're dumping directly back into the venous system. So these would be patients that have leak symptoms and leak findings in their brain but no epidural collection. Those folks who want to think more, okay, CSF venous fistula type problems, and you can pursue the lateral D-cube, DSM, or CT myelography. This is a great picture, someone we treated. This is actually, this is MG. I'm so sorry, this is not DG, it's MG. We did treat her, and on the left-hand side, you can see all that crazy amounts of dural enhancement, and on the right is a year later. Most of it has resolved. She did really well. And then same thing on this side, you know, it's really satisfying to see that dural enhancement get so much better pre and post operatively. It's one of the quickest things that we see that seems to improve. Cool. So that is sort of idiopathic CSF leaks in a nutshell. We're going to switch gears really quickly here with the last 15 minutes or so of our time, talk about cranial CSF leaks, and then also IAH. So cranial CSF leaks, right, these are going to be things that you see, I'm sure you've seen them, you know, in the unfortunate moments when they are post-operative, so someone's had a pituitary surgery recently, or, you know, something in the ear. They can be surgically created, they can come from trauma, right, so skull fractures that are severe enough can cause CSF leak. They can also be spontaneous, so they occur just because, and they can be associated with things like idiopathic intracranial hypertension, which we'll close out our lecture with today. And also, interestingly, OSA, so patients with sleep apnea seem to get these too. They are most common in females, up to 79% of patients will be female. And in some situations, they actually recommend screening for OSA patients that you think might have a CSF leak from the cranium, so interesting. I'm going to skip the deferential right now just for the next slide here, and we'll get back to that. So these patients are going to be very different how they present. These are not going to be your positional headache folks, even though leak is still the problem, right, so these are the patients that have primarily, first and foremost, drainage. Drainage from their nose, drainage from their ear. We've all been taught to ask about, you know, post-op, pituitary patients, hey, it's all the taste in the mouth, right? These patients are going to come in and say, like, my nose is constantly dripping, or when I lean forward, it comes out. They will sometimes wake up and say my pillow is always wet because there's stuff coming out of my ear. It's typically the first tip off that something isn't right and that they have some type of CSF leak from the brain. Sometimes they can have no headache. Sometimes it's just a low-grade mild. They often can be not even patternistic. It's just sort of random. They can get tenderness. They can get vertigo. They can come in with meningeal signs if the leak has been missed for a long time and there has now been an infection that's occurred. So the workup for these, for outpatient, when I have a patient that comes in with this, generally the consensus is you start with beta-transferrin testing. So you get someone to give you a sample, if they can, from their nose. We usually need at least one or two cc's on ice. Very important, put it on ice. And you test for beta-transferrin, which is a very specific protein that is in the CSF that will confirm that the liquid that is coming out is in fact CSF versus, for example, allergies or something else that's causing fluid from the nose or the ear. We also will get an MRI of the brain without contrast, again, looking for any symptoms or signs associated with contrast, so infections, et cetera. And then also a non-con CT with skull-based protocols. Very important. Most CT heads that are just written as is will not include the thin cuts you need through the interior skull base. If you have an ear leak, we often will pursue CTIAC as well. But the gold standard really is CT cisternography. I put it at the end, even though it is the gold standard, because sometimes we don't need it. If I have a patient that comes in with an ear leak, and I've done an MRI, and I see an encephalocele somewhere, or I can see a Tegman defect, then I know that's where my problem is. And cisternography isn't necessarily indicated. But technically, it is considered the gold standard. These findings are going to be also different than our spinal leakers. These folks will have pneumocephalus, skull-based defects. I mentioned encephalocele, meningocele, meningeal enhancement when they have meningitis. And of course, the passivation of air sinuses because they're leaking into those spaces. This is my patient, TS, also getting permission to share with you all today. This is a gentleman that had fluid leaking through his nose into his sinus, sphenoid sinus. You can see in the middle of the picture here. It's not supposed to be there. And on the right-hand side, also, you can see fluid accumulating in the sphenoid there. And the treatment for these is really dependent on volume, location, and cause. So low volume, closed-head injuries, these can sometimes spontaneously resolve with bed rest, temporary lumbar drain placement, although it's, again, falling more out of favor, elevation of the head of the bed. Those are the big ones. When surgical repair is necessary, again, dependent on the site, ear or nose, we can go either endonasally. We can go through a middle cranial fossa. For patients that you're concerned may have rebound pressure issues, you can place a lumbar drain. Just takes a lot of pressure off of the leak site to heal. And it's somewhat controversial to use that, but I have seen it done. For your rebound headache patients, you can consider the use of acetazolamide, Diamox, again, to drop that pressure down a little bit. It seems to help with the symptoms. And the takeaway from this slide is that if you have a patient that you think might have a CSF rhinorrhea or CSF otorrhea, it's important to get them into a center that has all the bells and whistles. Someone's going to have an ear, nose, and throat guy or lady on with a neurosurgeon that can work together to treat the problem. I just realized I forgot to mention halo signs. These are really quick and dirty, again, not diagnostic, but certainly suggestive. You can take a small amount of the fluid on a piece of filter paper, and you'll see that double ring sign, you'll see if it's blood in the sample, you'll see it create a ring and then an outer ring of clear fluid, which is a CSF. Not very specific in that if you do that with blood and saline or blood and a couple other types of fluid, you will still get a double ring sign. It's not super specific, but it can be a quick and dirty bedside if you're in ER, for example. So that is just a really quick overview of leaks that occur causing intracranial hypotension and cranial CSF leaks. I am going to run and just finish up today's talk with obligatory. We talk about what happens when you have too much pressure inside the brain. So these are pseudo-tumor patients, IIH patients. So what is IIH? Essentially, IIH is symptoms and signs related to elevated intracranial pressure that you can see on, you actually can see some evidence on MRI of the brain, and it's a clinical diagnosis. So it's people that have elevated intracranial pressures that don't have any other obvious cause. So they don't have a sinus thrombosis, they don't have a tumor, they don't have some other cause of elevated intracranial pressures. This is just sort of, they are just baseline high. We use a couple of different things to diagnose it, symptoms, and then also, I mentioned Dandy criteria moving away from this. Most neurologists I know don't use this anymore, but it sort of quantifies what I just said, which is these patients will have these symptoms of intracranial pressures that are elevated, so they'll have headache, visual changes, they'll have vision loss, but they won't have any structural findings like a tumor that would explain the IIH. As part of the Dandy criteria, they also have to have normal CSF composition as well. I thought I would just mention it in case you see it in the literature anywhere. Signs and symptoms. So again, headache. This is sort of a headache talk in a little bit, right? So headache, you're going to see that. You're going to see the don't miss sign of papilledema. These folks have increased pressures in their head. You're going to see papilledema. They're going to complain of visual disturbances, everything from floaters to loss of vision to, it can be kind of subtle. But you also will see things like pulsatile tinnitus, neck and back pain, cranial nerve palsy, specifically cranial nerve six, the abdicens, because of the increased pressure you can see in abdicens palsy. And then also there's this field cut in vision loss. But really the smoking gun, so to speak, of this condition is papilledema plus this headache. It's important to recognize this quickly, because even a mild decrease in vision or even a mild visual loss can cause a decrease in quality of life. So important to keep this on differential. When you have somebody who has chronic headaches and visual complaints, put this in the back of your cap as a possible diagnosis here. It can be hard to differentiate between this and migraine, right? Migraines can do all sorts of interesting things, especially ocular migraines and vestibular migraines. But again, the differentiator here is that papilledema. You're not going to really see that with migraines. You're going to see it with IAH. Thanks. And the folks that get these are typically larger ladies in their middle age. So 40, 60 years old, usually with a history of obesity, and typically female. All right, we're wrapping up here. So workup for this. I often do all these things at the same time. So lumbar puncture, ophtho eval, and then MRI brain width without and an MRV. I usually will have a list of things to say, patient, we're going to do all these things at the same time, okay? So we will order the LP, we'll order the ophtho eval, and MRI the brain, and the MRV. MRI brain, MRV, mostly to rule out any other structural causes of the elevated intracranial hypertension. So tumors, venous sinus thrombosis, little things that can cause increased intracranial pressures. The ophtho eval. So if there was ever a patient, I was going to walk over to our ophtho clinic with a bag of cookies and say, please see my patient quickly. It's this patient. It's the one that I'm worried about that might have papilledema NIH, and I have definitely done this before in my practice. And they will usually understand the urgency and get them in within a couple days to a week. And then lumbar puncture is also really suggestive here. So we'll do a lumbar puncture. They'll have an elevated pressure, usually above 20, although above 25 is more specific. And we can also repeat these more than once. If we think that on the first time it was elevated, but not so elevated, and they're still having very obvious symptoms, we can repeat it and try again. All right, and of course, when you order a lumbar puncture, always make sure you mention the opening pressure. And I know we should usually get CSF labs because, hey, I'm in there, and I want to make sure I'm not missing something else in the CSF composition that could be causing an elevated cranial pressures. All right, we'll walk through some RAS findings real quick. You will see tortuosity of the optic nerve. You can see an empty cella, optic nerve enhancement, transverse sinus stenosis, and of course that papilledema. I affectionately call these patients that they have wiggly optics. They have optic nerves that are a little bit squiggly. So example on the right here of a big empty cella on a patient at NIH. She's doing really well. Example on this right-hand side picture, all that papilledema, wow, check that out. Lots of papilledema on this patient. Some little bit of tortuous optic nerves, too. And then this image I added. It's not in your talk. I might have skipped one. No, I didn't. This is someone that has actually vertical tortuosity, which is why it looks like there's a break there. It's because on this single flat image, the optic's actually kind of going like this. It's sort of vertically tortuous, which is interesting. And then this patient, we actually saw earlier with that CSF rhinorrhea from his nose. He ended up having IAH, and it caused a right-sided tegmental defect, a tegment defect that filled into his mastoid after we fixed him. We are fixing this next week. So you can see IAH again causing not only leaks in the brain, but also leaks in the CSF rhinorrhea through the nose as well. Treatment options. So treatment for this is going to be really focused about a couple things. Weight loss is a big one, and most neurologists that I have talked to will say that's the first go, weight loss. Low-sodium diet seems to help as well. We can put them on Diamox to see if they, usually we'll start around 250, 500 milligrams, BID. See if they tolerate it. It inhibits carbonic anhydrase activity, so it actually decreases the amount that you're producing. Other diuretics, furosemide is typically the second line. Popmex can be helpful. And then in patients who are out of options with medications or who don't tolerate them very well, rarely we can also do surgical shunting, either an LP or a VP shunt. I mentioned ONSF, which is optic nerve sheath fenestration. Again, it's center-specific. If you have someone who knows how to do it and does it all the time, great. But most neurosurgeons I know, if they're really convinced and they've had a good talk with neurology and optho, this is someone that has IAH that's not responding to drugs, we typically will pursue a VP shunt. Sinus stenting I have heard of, and I don't unfortunately know anyone in practice who's doing it. I know they exist. I just, in DC, not someone that I'm familiar with, but stenting of the sinuses to keep them open, those are another option for a surgical treatment. And then this last slide here I'll pull up is a really quick and dirty from UpToDate. I love UpToDate, man. It is sometimes simple is the easiest way forward. So this is a great slide. This talks about kind of how you want to manage these patients that come in with these symptoms, specifically with the vision loss, right? So the one thing I may have not driven home in this last slide is that patients with IAH can go from okay to not okay really quick. And so in patients that have rapidly worsening disease and rapidly worsening vision, it's important to get them on Diamox ASAP, as soon as you can. In some situations, actually even doing a temporizing LP just to get some of the fluid off, which we've also done. And you want to drop that pressure down as soon as you can if you are concerned for a rapid progression. But most patients that come in in the practice and outpatient, not always, but have a slower progression that has been picked up on an MRI or they have other symptoms that suggest about this IAH diagnosis. And we start them on a slower path of Diamox and symptom management. And you'll see at the bottom here, you know, long-term monitoring weight loss as part of the treatment options. So that's all I have for you today, folks. I have tons of references if you want to look up any of them. It's been so great to chat with you guys about this really interesting topic. I'm a big leak nerd, so if you want to learn more about this, you have questions, that's my personal email. Please feel free to hit me up with questions. And I will open it up for questions now if there are any. Yeah, there are a few questions, Tana. Thanks for the talk. The first one, we're running into break, so if people want to filter out, that's fine. But we'll go ahead and try to get at some of these questions. Is anyone still using LP shunts? And do you find any benefit over those? I don't see anybody doing them. We're still managing a few. But what is your kind of practice looking like, Shannon? Pretty rarely, especially for IAH. We're not really doing them anymore. They're very finicky and a lot harder to change in the office with the programmables, at least. I don't know about you, Mike. I know you're in neurosurgery as well. But especially because patients are often a little heavier trying to get to that shunt reservoir to change the valve can be tricky. Yeah, I have found them challenging. And I'm not seeing many anymore. One question, post-leak repair, do you have advice or restrictions on patients in flying? Great question. Yes, but I don't have any data on that. But we don't like them to fly until at the very least their post-op visit, but usually it's closer to six weeks. Got it. And this same person wanted to also point out that Jabba the Hutt was an aneurysm from another patient's talk. And last question, how often are you using, do you get to the point where you need a cisternogram to look for a leak? You know, a lot of patients that come in with that, those florid signs of CSF rhinorrhea, or sorry, of otorrhea, at least, there's almost always a tegment defect. That is one of the most common sites. There's another small bone, which, oh my God, there's so many small bones of the ear, but it's like tegmen media. Anyway, I'll look it up for you. But primarily tegmen defects in the ear seem to be where those occur. And they're really obvious on CT, especially with CT IACs or CT of the skull base. We can see them. So it's really, I think it's less common. I only order them once in a blue moon if the MRI, if for example, if the MRI is not very suggestive and the CT is not very suggestive, but the patient has like been a transparent in their fluid, I'm going to keep looking because I know that there's CSF somewhere, right? Because it's coming from somewhere. You can't just leak for no reason. Well, as we wrap, Shannon, most of the comments in the chat have to do with kind of this being complex and something we see rarely. So I think people are grateful for kind of a breakdown of something that, again, that's a don't miss, but we don't see very often. So thank you for your talk. And everybody else I'll see at the top of the hour for Dr. Wang. Thanks everybody. And thanks AANS. We'll see you next time.

intracranial pressure disorders

cerebrospinal fluid leaks

idiopathic intracranial hypertension

diagnostic strategies

treatment options

specialized care centers

neurosurgery

MedStar Georgetown University Hospital