vascular malformations of the brain in children and...

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Vascular Malformations of the Brain in Children and Adolescents Webcast March 8, 2011 H. Hunt Batjer, M.D. Linda Byus Maggie Byus Please remember the opinions expressed on Patient Power are not necessarily the views of Northwestern Memorial Hospital, its medical staff or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you. What is an AVM? Andrew Schorr: Arteriovenous malformations, or AVMs, are the most common cause of stroke in young people, but what are the signs and symptoms? How are AVMs treated? Coming up, a leading neurosurgeon from Northwestern University and Northwestern Memorial Hospital will answer these questions and more. You'll also hear from an inspiring patient and her mom. It's all next on Patient Power. Hello and welcome to Patient Power sponsored by Northwestern Memorial Hospital. I'm Andrew Schorr. Well, I am a parent of three kids, and fortunately they're pretty healthy, but imagine if your child had terrible, terrible head pain and it turned out to be a congenital malformation of the veins and arteries in their brain. Pretty scary. We're going to learn what an AVM is, an arteriovenous malformation, and talk about it particularly in the brain where there's this concern about stroke. And we'll have a leading expert with us and we'll also have a mom who has become an expert and a young adult who has become an expert, too. First I want you to meet Dr. Hunt Batjer. Dr. Batjer is chair of the department of neurological surgery at Northwestern University's Feinberg School of Medicine and Northwestern Memorial Hospital in Chicago. Dr. Batjer, I know you're a specialist in this. Thank you for joining us. What is an AVM? Dr. Batjer: My pleasure. AVMS or arteriovenous malformations are relatively unusual congenital problems but one that has a very high profile. They likely arise when a certain area of the brain is genetically programmed to not develop. During our intrauterine life, all of our organs including the brain develop a blood supply before we develop the target organ. In this situation an area of the brain does not develop but it already has a blood supply and those arteries simply connect up directly to veins coming back to the heart. In this situation the arteries come up from the heart into the head and connect directly into veins coming back down to the heart and lungs creating a loop. In the adult form we call this a fistula or a direct connection of arteries to veins. At that point, after the initial intrauterine event the arteries become more and more tortuous and twisted very similar to what you see when you look at a lean elderly person and note the arteries in their arms that get redundant and tortuous. The

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very high flow demand that these vessels suffer results and reach a point of being a Medusa’s head of abnormal arterial blood vessels that shot blood directly back to the heart and deprive the surrounding brain tissue of that blood. Andrew Schorr: So if you looked at a picture of it would look like a big tangle. Dr. Batjer: Absolutely, a big tangle of snakes, red snakes. Andrew Schorr: And they could bleed or rupture I imagine and that would be a terrible thing where someone, a child even or a young adult could have a stroke. Dr. Batjer: These blood vessels don't appear to be built biologically to carry the amount of blood supply that they're forced to carry, and then because of that they can give way and burst, as you described, causing hemorrhage either into the brain or into the spaces around the brain. Andrew Schorr: Okay. So of course it could be a little bit of blood or it could be catastrophic and could be fatal. Dr. Batjer: Correct. Around 20 to 30 percent of ruptures result in death or disability from AVMs. Maggie’s Story Andrew Schorr: All right. Well, as everyone can gather we're talking about a serious concern, and let's meet a family that has been touched by this. Let's go up to Arlington Heights, Illinois. Linda Byus is with us. Linda, you were on vacation back in 2003, mom, dad, two children. One of the children, Maggie, age 14. There you are at Virginia Beach, Virginia, enjoying summer vacation. What did Maggie complain about? Linda: It was in the evening, and we were getting ready to go out to the pool, we were in our hotel, and she all of a sudden grabbed her head and said, “I have a headache, is this what a migraine is?” And this was a healthy, athletic kid, never had any problems, never complained of headaches. She was in agony. We tried to quiet her down, gave her some Tylenol, and she just looked at me and said, mom, you have to take me to the hospital. There's something really wrong and you can't fix it.

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Andrew Schorr: And this was not a kid who complained. Linda: Not at all. Not at all. So we loaded her up and went off to the emergency room, and the emergency room doctor started talking to her, and her complaint was this terrible headache, stiff neck, just lethargic. And the doctor was actually looking for meningitis and ended up doing a lumbar puncture and found blood in her spinal fluid and at that point did a cat scan and found the AVM. Andrew Schorr: Found this tangle. Now, Dr. Batjer, this symptom of maybe what Maggie was describing as the worst headache of her life, would that be one of the typical symptoms? Dr. Batjer: Absolutely. For a ruptured AVM that's very classic. Andrew Schorr: Now, I don't want to say she was fortunate to have this, but it sounded like it was bleeding rather than rupturing, but yet blood would leak into her spinal fluid. Is that one of the clues there? Dr. Batjer: Well, I think those words are a little redundant. Any time an AVM bleeds it essentially has ruptured from some place. Typically the bleeding doesn't go on for very long or the person dies. The pressure inside the head gets so high that you don't survive it. It bleeds for a few heart beats only and then stops for reasons that really don't make any sense. It's hard for me to believe that anybody survives a hemorrhage from a AVM. It just shouldn't stop, but it does in the majority of patients. Andrew Schorr: Well, your daughter was lucky, Linda, because as we'll hear along this program she has done quite well with help with from Dr. Batjer and a wonderful team and support from you and your husband of course. So let's go on. So there she is at the hospital. You're told she has a AVM. Now, you'd never heard of this, right? Linda: Never heard of it, didn't really know what they were talking about. Andrew Schorr: Other than as you began to understand, it's terrifying. Linda: Right. Well, any time there's bleeding in the brain, they're talking about a tangle of blood vessels, and I guess I still didn't get it until they loaded her into an

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ambulance, you know, with lights and sirens and took her to a nearby pediatric hospital in Norfolk, Virginia, where there was a pediatric neurosurgeon on staff. Andrew Schorr: So she's in intensive care there, and he starts telling you how you treat this. And I understand he starts talking about specialists in this condition, and one of a handful is the physician, the neurosurgeon is with us today, Dr. Batjer, who happens to be in Chicago, your home town. So does that then lead to you saying we've got to get Maggie back to him? Linda: Well, the doctor we saw in Norfolk, you know, explained to us how he would treat it, which was basically how it was treated with embolizations and surgery, but because it's a lengthy treatment it's hard on the family, there are a lot of demands on the family, he thought it was best if he could get us home and we were just very fortunate that one of the leading experts in the country was at home so we could bring her home to have her treated. Andrew Schorr: Okay. And ultimately Maggie had what we're going to learn about, embolizations to sort of wall off this tangle and then ultimately open brain surgery. And she is now years later doing well, right? Linda: She's doing great, yep. Dr. Batjer: Mr. Schorr, could I make a point that Ms. Byus brought up? Andrew Schorr: Yes, sir. Dr. Batjer: Unlike ruptured aneurysms, which are little blisters on arteries of the brain, with AVMs the rebleeding risk is not very high in the first few days and weeks. With aneurysms the bleeding risk is the highest in the first day or two after the first hemorrhage, and that is a very important but subtle distinction, but the fact is most AVM patients that either bleed or have other types of symptoms can move. And there are certain areas, as Mrs. Byus suggested that treat a lot of these, and they really should be the ones that should do that for reasons that we'll get into. Andrew Schorr: So it was not unsafe then to have Maggie as she recovered in Norfolk to come home to Chicago. Dr. Batjer: No, it was not unsafe.

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Andrew Schorr: Now joining us is 22-year-old Maggie Byus, who is a senior studying advertising at the University of Illinois. So, Maggie, when you think back to that day on vacation in Virginia Beach, how bad was the headache? Maggie: Oh, the headache was definitely something I had never felt before. It was a sharp pain and it happened suddenly, so I knew right away that something was not right. Andrew Schorr: Now, your mother said, you know, you try, you know, Tylenol and other simple things that you can do, but you wanted to go to the hospital. It was that bad. Maggie: Yes. I knew right away, I could tell by the feeling in my body that there was some gut feeling somewhere that I needed more help that Tylenol could provide. Andrew Schorr: So you go to one hospital and then ultimately to the Children's Hospital and a pediatric neurosurgeon. This had to be pretty scary. Maggie: Yes, it was. I just, you know, looking back at my 14-year-old self I was definitely just thinking, you know, whatever's wrong I hope they find it out really soon and can fix this so I feel better. Andrew Schorr: So you go back to Chicago. You and your dad fly back, and then ultimately you see a renowned specialist. And you have to go through these procedures, embolizations to, you know, wall off from bleeding, and then ultimately neurosurgery. What was that whole experience like? Maggie: Well, when I was in Norfolk the neurosurgeon that we saw said there are four neurosurgeons in the country I would send you to because they're the ones that specialize in this, and Dr. Hunt Batjer in Chicago happens to be one of them. So when they said, you know, you need to go see Dr. Batjer, I was really happy that someone in my home town could be there to be one of the specialists that could help me. So that gave me a little bit of comfort. Andrew Schorr: And going through all the this, was it difficult, or I mean the recovery and all that going through these procedures?

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Maggie: Yes, it was definitely a lot longer process than I originally thought it would be, but as I look back now this many years later, eight years later, it almost seems--it seems a lot smoother and it seems like it actually went by quickly, but at the time it was definitely, it was rough. Andrew Schorr: Let's talk about it now, though, because the result is so important. You're a senior in college. How are you feeling and what are you able to do or is there anything lasting from the AVM or the procedures you had? Maggie: The only side effect that still persists today is that I have some slight loss of some peripheral vision in my right upper quadrant. Other than that I'm just like any other student, and in fact the past two years I've run two half marathons, so physically and mentally I'm about where I was eight years ago. Andrew Schorr: Wow. That's neat. How do you feel about the care you got from Dr. Batjer and just that you got to the right people and serious a neuro condition was treated it appears successfully? Maggie: I always felt pretty confident with Dr. Batjer and Dr. Bendok, two of my doctors in Chicago, because they always seemed very knowledgeable, and because Dr. Batjer deals with this type of thing a lot, since he specializes in AVM, I felt, you know, as comfortable as anyone could going into these serious procedures. Andrew Schorr: So are you ready to just go on with your life? Your mom can maybe breathe a sigh of relief, maybe someday you'll, who knows, you'll have children, give her grandchildren, but go on with a successful life. Maggie: Yes, I'm really excited, looking for my first real job, and hoping to move to the big city of Chicago by this time next year. Andrew Schorr: Linda, we hear Maggie, she makes it sound like it was no big deal. Linda: She does, doesn't she? She was 14. Her conviction all along was this was just a problem that we could fix, and that's how she treated it. She said do what you need to do and fix it so I can get back to my life.

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Andrew Schorr: And she has. How fortunate. Dr. Batjer, she mentions you and Dr. Bendok, of course, who I imagine is a physician who works with you? Dr. Batjer: Yes, he is. Understanding the AVM: Diagnostics Andrew Schorr: Okay. So take us through it. So, first of all, so she gets to you in Chicago. So how do you know what you're dealing with? So whether it's Maggie or somebody who comes to you now what do you do to understand what you're dealing with? Dr. Batjer: Well, the first things that go into it are you try to get a good history. In this case it was an obvious one. She had a catastrophic presentation with a hemorrhage into the brain. And you also look to see, well, one of the other things these can do is they can preceed seizures. They can produce long-standing headaches. Maggie had a sudden, severe headache, but some patients have a long-standing almost migraine sounding headaches from other effects of AVM, so you want to get a good understanding of the patient's course. A rare presentation of patients is that they can present like a progressive stroke over some weeks or even months, a progressive weakness or limp that you might see depending on where the AVM is in the brain, or a visual loss can also be there. So you want to find out kind of all you can about the background of the patient. Andrew Schorr: One of the things we didn't discuss yet is age. So Maggie was 14. So we talked about the problem developing in utero and then you talked about maybe the trauma of heart beats over maybe many years. When does this typically show up? Dr. Batjer: This is not an uncommon age for these to manifest. This is a cause of stroke that in general affects a younger age group than other kinds of stroke, either ruptured aneurysms or blockage of arteries that we see more in the seniors, from 55 on. AVMs tend to present in young patients, so that's not a big surprise. Andrew Schorr: But a parent or child, they'd have no way of knowing. You're not usually imaging a child's brain routinely so we wouldn't even know. Dr. Batjer: That's correct.

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Andrew Schorr: So you do imaging and you and your team then understand what you're doing. Now, these AVMs could be at different places in the brain. That makes a difference, where it is, doesn't it? Dr. Batjer: It makes a huge difference in terms of what the, you know, what the natural course of this condition is going to be. We don't treat every patient that has a ruptured AVM. And all these decisions are based on the specific anatomy, where is it in the brain, where are the important functions of the brain arising from. And there are types of imaging we can do now that can take a patient, put him on an MRI table and with the magnet going have them do language tasks or have them do motor tasks, visual. Any other thing imaginable you can test and as you do it--for example, if I'm just taking my right index finger and moving it up and down, that creates activation in the motor cortex on the other side of my brain. That exercise causes more blood vessels to dilate in the area and more blood supply to that area of my brain, and we can see that on an MRI, so we can see where that function is coming from very precisely. So that's a critical tool in evaluating folks with AVMs, and what you not infrequently see because these are congenital is that you see an MRI and it's right in the motor cortex or language area of the brain and in fact when you do a functional image you find that particular function has been switched even to the other side of the brain, which is sort of intuitive, as you think about it, because you know that their language is fine, you know that their language can't be coming from exactly where that AVM is because there's no brain tissue in that area, so it's intuitive that it had to move somewhere else and it does. But it helps guide therapy. Andrew Schorr: How remarkable. We're going to get into much more detail about, first, the decision-making on whether to proceed with intervention or not and what those interventions can be and how you approach it at Northwestern Memorial. We're going to continue our discussion on AVMs with Dr. Batjer, Linda Byus, we'll hear more from Maggie. All that is coming up as we continue Patient Power right after this. Surgical Intervention Andrew Schorr: Welcome back to Patient Power sponsored by Northwestern Memorial Hospital. We're learning about arterial venous malformations in the brain and how they're approached at Northwestern Memorial with a specialist, and he is actually the chair of neurological surgery at the Northwestern University Feinberg School of Medicine and Northwestern Memorial Hospital, Dr. Hunt Batjer. And we also have with us a mom who has lived it with her daughter. We have Linda Byus, and we'll hear more from daughter Maggie in just a little bit.

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Dr. Batjer, so you made the comment that it's not always that there's a surgical intervention with an AVM. So how do you decide, and maybe even thinking about it, and Linda, we'll hear from you too in Maggie's case, whether to do more? I mean, she survived fortunately the bleed or the rupture, and she was doing okay, but then you look at really some pretty significant intervention. How do you decide, Dr. Batjer? Dr. Batjer: Well, it's one of the hardest tasks. In fact I believe it's a much harder task than actually treating the malformation. You consider a number of things. The first, whenever you see a patient with a complex lesion like this you have to try to come to grips with what you expect the natural course to be untreated or the natural history. You'll hear the docs refer to that as the natural history. Well, it's going to be a hemorrhage rate of 10 percent per year, two percent, four percent. A lot of that is imprecise, but there are certain things that we know increase risk over time, and certainly young age is the most important one. They simply have more decades of life ahead of them, and if you take a fixed annual risk of hemorrhage it means a lot more to a 14-year-old than it does to a 74-year-old, if that makes sense. So age is important. The size of the malformation with some caveats is very important to the risk of treatment. So that's something we have to really think about. Where it is in the brain can have a very important decision-making influence. If it's down in the deep areas of the brain, for example, the basal ganglia, children or anybody else will not tolerate surgical treatment for those, so we have to think of other possibilities. We look for little things on the angiograms or the imaging studies that suggest a higher or lower risk of bleeding going forward, and those things might be narrowing of some of the veins draining it, like tightening a hose. It makes sense that if you've got your water turned on and you put a stricture in the hose at some point the pressure backs up behind it, and those are malformations that we think have a higher risk of bleeding, and they're pretty easy to identify on the imaging studies. Pregnancy, based on the data we have from Finland, this was done in the mid portion of the last century, it looks like pregnancy has up to a threefold increase in the risk of hemorrhage during that year of a woman's life. So, you know, a 14-year-old girl approaching her child-bearing years is even a higher concern than a young boy in the same situation. So that's another element. In Maggie's case, obviously, biologically her lesion was active, it had ruptured. It was in a pretty forgiving part of the brain. It was large, but it was confined to a lobe of the brain. It was compact. The AVM tissue was favorable as opposed to being very wispy or angiomatous at the edges, blending out into normal brain tissue. That was a favorable feature, and as I recall, Maggie had significant visual disturbance from the hemorrhage, which I thought we would probably make worse for the short run, but she was young enough that the chances of her recovering that would have been very good.

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Andrew Schorr: So, Linda, you heard the recommendation, now he recapped it, but years ago, and you decided to go forward. Linda: My husband and I are both kind of statistical people, and as Dr. Batjer said, you know, there's a statistical probability of another bleed and there's risk associated with every bleed, and it was just enormous that over her lifetime, over Maggie's lifetime there was a very high probability there would be another bleed, and then you combine that with the risk at pregnancy, it just seemed like the right thing to do. The risk of treatment seemed, sounded like it was less to us than the ongoing risk of not treating her. Andrew Schorr: It would be throughout her lifetime like the elephant in the room or maybe the fear of a ticking time bomb, right? Linda: That's how we felt, yes. Dr. Batjer: Well, it is, but even if you say the risk is only four percent per year, I'm going to make that up because that's one of the important studies, that's the number that came out, that's four percent each year for a whole bunch of decades, and each time it bleeds the chance of death or disability is in there at 20, 30 percent. So I think that's the thing. And the thing that patient's families always ask is what would you do if it were your daughter, and that one in Maggie's case was a very simple one. It comes out, period. Linda: And the reality is we asked that question. One of the first questions I asked Dr. Batjer is do you have kids and how should we think about that. Andrew Schorr: So, Dr. Batjer, we talked about one option, no treatment. The other option is treatment, but I imagine there are different approaches. Take us through that. Treatment Options Dr. Batjer: That's absolutely correct. The treatment possibilities include Dilantin therapy or some other anticonvulsant to prevent seizures if the patient presented with a seizure, for example. That is treatment. That's intervention. Then how to treat the lesion itself. There are some that are very simple that are located on the surface of the brain that can simply be treated with a single operation. That is a relative minority, at least in my practice, but my practice is usually more complicated lesions because the simple ones are treated elsewhere. Of the more

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complicated lesions the options are occasionally to be cured with embolization. In my own experience that is effective in a tiny minority, three or four or five percent at the most. Andrew Schorr: And what is an embolization? Dr. Batjer: Embolization is a procedure, it is a surgical procedure done through a catheter. Almost always the patient is asleep, under anesthesia. That catheter is advanced up into the blood vessels right at the margin of the malformation, and some material is injected to block off a portion of the--of the circulation there of the malformation. During my career we have evolved through a lot of very difficult compounds now to one that I think is really terrific called Onyx, that's O-n-y-x, and I think it's the most versatile compound that we've used to date that has a high likelihood of safety when deployed properly and doesn't adversely affect our ability to take the malformation out in surgery. Andrew Schorr: Okay. Just let me make sure I understand that. So a catheter, is it typically inserted at the groin? Dr. Batjer: Yes. Andrew Schorr: So you snake your way up there and you get to the malformation, and then you're injecting this material. And what is the goal of injecting the material? Is that to sort of wall it off or what are you doing? Dr. Batjer: That honestly depends on the case, and part of the strategy that is developed relates to that factor. For a few patients our goal is to cure the AVM with embolization. The interventional neuroradiologist or neurosurgeon fills up the entire AVM with this compound and shuts off all the blood flow through it. That doesn't happen very often, but that could be a strategy. It may be a strategy whereby we want to attack certain blood vessels that feed the malformation that are going to be a problem to us in surgery. For example, I mentioned some people have bad headaches. They can be a result of feeding arteries that come through the skull from the scalp and penetrate the dura or the covering of the brain and then get into the AVM, and if you can imagine trying to do an operation with all those, all that blood flow coming through the scalp, it is a mess, and we like to get that closed down before we do any kind of surgery.

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In addition, you might have circulation to the AVM coming from the bottom of it or the base, and those are blood vessels that we would like to be embolized or shut off before surgery because we can't access them until we get to the bottom, and that can set the stage for complications in surgery. Andrew Schorr: Now, just so we understand, I'm trying to visualize this and I'm sure it varies. Are we talking about a tangle of vasculature that is an inch across or two inches or three inches? I know they vary, but typically what might it be, let's say, in a 14-year-old? Dr. Batjer: It's age indiscriminate. They can involve half the brain and be huge. We're treating a young patient with a situation like that right now. They can be tiny, the size of a dime or even smaller, and everything in between. Andrew Schorr: Now, in Maggie's case there were three embolizations. Tell us about that. Why would there be more than one? Dr. Batjer: The reason is not totally straightforward. When we elect to go with embolization prior to surgery as a strategy I think of it in several different paradigms, and one of those if you've got a large AVM, and Maggie's was large, it is potentially dangerous to shut off huge amounts of that blood supply suddenly. In the old days before embolization we took all these out in a single-stage operation, and we had big trouble with the big ones. Things would go well for a number of hours, and usually into the evening we would start getting into trouble, and it would be big trouble around midnight. That was a very predictable scenario in the 1970s and 80s, before we had good embolization. The reason for those struggles are that, think of the brain around the AVM, not the AVM. That brain tissue has been deprived of circulation. Blood selectively goes into the AVM, and all of a sudden if you shut that shunt or take the AVM out suddenly the heart continues to pump at the same volume. Where is it going to go? And the problem is it goes into that surrounding brain tissue which is not prepared to deal with it. The thermostat or the barostat is not reset suddenly, and I like to embolize a little at a time to let the brain get used to a new environment, and we often will separate those embolizations procedures by a week or sometimes as much as three to four weeks at a time. The Multidisciplinary Team Andrew Schorr: Dr. Batjer, you mentioned along the way the word "strategy." While I know you're a strategist, you don't do the strategy on your own. Tell us about the multidisciplinary team that comes into play for instance at Northwestern…

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Dr. Batjer: Yes. Andrew Schorr: …in helping decide the best strategy for each patient. Dr. Batjer: You know, you never make decisions by a committee, but you use a committee to guide decisions. And what we try to do is get all of our people on Wednesday evenings into a room and have others connected by internet and other means and present cases. And the people in that room would be folks like me and Dr. Getch and Dr. Bendok who are surgeons. We would have our endovascular team there. Dr. Bendok and our interventional neuroradiology colleagues are there. We have our radiosurgery colleagues there. In this case that would be Dr. Getch, who does the radiosurgical treatment which we've not discussed yet, but that's another option, treatment option to be considered in some of the more difficult lesions. We have our critical care folks there who are going to be helping us manage during those acute difficult first day or two after treatment. Tight management of the blood pressure and other things that can go wrong, and occasionally in complex AVMs we'll actually keep the patient under anesthesia for several days to allow that brain to adapt with a little bit of help with some medications to help control the blood flow. Andrew Schorr: Linda, did you have a sense that there was quite an elaborate team supporting your daughter? Linda: Yes. The way we came to see Dr. Batjer was actually through one of the neurosurgeons at Children's Memorial, and she explained to us that this was the procedure that would take place, and Dr. Bendok is actually the surgeon that did the embolizations, and we met with him as well as with Dr. Batjer, and we were very aware of the support team that was needed because it was somewhat of a challenge for a 14-year-old, not a grownup, not a little kid. How do you support this, what pediatric specialist do you need involved? So it was pretty complicated. Types of Surgery Andrew Schorr: Dr. Batjer, let me ask you, so we're going to get into the surgery. You mentioned radiosurgery as well. So one kind of surgery is with a scalpel, I imagine, or micro surgery. And another part is using radiation in a very targeted way, correct? Dr. Batjer: Correct. And radiosurgery is very helpful to us in certain circumstances. It is helpful in malformations that are intimately located with important functions of the

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brain where removal of the malformation would put the patient at high risk of neurological damage. That may be related to language or it may be deep in the brain. I've talked about a basal ganglia lesion or a brain stem lesion where surgery is not an option, and in those cases radiosurgery can be. The principle of it is that using modern techniques of radiosurgery, Gamma Knife is what we use here at Northwestern typically but there are others, you do an imaging study to get a perfect target of the lesion in three dimensions. Our radiation physicists and engineers work with the surgeon and radiation oncologists to target that lesion. And it sounds simple. It's really not because there's a lot of background noise in AVMs that has to be excluded from the treatment plan, and you target just the true nidus of the AVM. Andrew Schorr: And what is the nidus? Just where it all comes together? Dr. Batjer: The nidus is that Medusa's head that I talk about it. Andrew Schorr: Oh, okay. Dr. Batjer: And you avoid some of the extraneous venous drainage that make it look complex but in fact aren't really germane. So that targeting is planned and executed in a single dose. The patient lays down in a frame, and the treatment is administered. The frame is taken off, and the patient goes home. So it's like drive-through surgery. The problem is that the impact of that radiation on the malformation takes time. There's a latent period of between one to even five years where the patient is not protected and the radiation over time takes all those blood vessels and begins to restrict them and fibrose them. And what we see is they become white, thick-walled, empty blood vessels. And for the right sized AVM, the smaller the better, that can be a curative procedure. Andrew Schorr: In Maggie's case she then proceeded to open brain surgery, and so that was the approach. Tell us what you do there. Dr. Batjer: Maggie's lesion was too large for radiosurgery, as background. In her case we wanted to slowly knock down some of the blood supply with endovascular techniques, which Dr. Bendok did very well, and then she was brought for a single-staged resection. That involved a putting her under anesthesia, positioning

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her. It's been a number of years. I'm pretty sure we positioned her face down and chest down on the table, and constructed a scalp incision and bony incision that generously covered the malformation. And you utilize such things as gravity. You think about how the brain is going to sit. Sometimes we allow it to sag and use space created by that brain sag to advantage to get to certain areas. But it's a complicated process of thinking through exactly what we're going to see and what are our surgical strategic goals at each phase of the operation culminating in the lesion being circumferentially dissected, all the arterial supply interrupted, and the malformation brought out hinged on its last venous drainage which is then sacrificed, and the thing comes out. Andrew Schorr: Linda, I know you were sitting there, I'm sure, in the waiting room. Do you recall how long that surgery took? Linda: From the time we left her until we talked to the doctors when it was done it was, gosh, it was probably about 12 hours. Andrew Schorr: That's a long time, Dr. Batjer. You have a lot of stamina, and I'm sure it's very intricate. But the good news is Maggie has done well. We're going to talk about that when we continue our Patient Power discussion right after this. Recovery Andrew Schorr: Welcome back to Patient Power as we continue our discussion about arteriovenous malformations in adolescents, young adults. Maggie Byus has lived through it. Linda Byus, mom and family have lived through it. And with the help of a wonderfully specialized team at Northwestern Memorial, and there are not many around the country where these complex cases go, and that's where Maggie was helped by Dr. Hunt Batjer, the chairman of neurological surgery there, and the whole team. Dr. Batjer, so we've learned that Maggie had that, had the three embolizations and then open brain surgery, and you were able to take out the AVM. Now what happens next as far as recovery? What is recovery like, and also can there at times be some neurological deficit that happens from the surgery? Dr. Batjer: That's correct. The most important early risk to the patient is hemorrhage into that friable brain around the AVM. Now, these things, you know, when you actually see them in the operating room and I know as I was growing up around Duke Samson in Dallas, it's such a fragile situation it's a wonder anybody gets through it well, but

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they obviously do. Trying to control those tiny blood vessels, making them secure enough that, what, for example, would happen if two hours after surgery if the endotracheal tube was still in and the patient got agitated and coughed and bucked and strained? Well, that could actually be a complete disaster in an AVM patient resulting in potentially fatal hemorrhage into the brain. So the first hours and the first 12 hours are utterly critical to control the blood pressure, and we leave the patient sedated, often intubated overnight under anesthesia just to get some hours under our belt and let the hemostasis or those tiny, fragile blood vessels get a little more secure. We help that in surgery by leaving tiny little clips across the larger ones, but there are hundreds or thousands even, that are so tiny we coagulate them good and cut them, but you're always sort of sleeping with one eye open after that. So the risk of hemorrhage is highest in those first hours. Then usually middle or late in the day on the first postoperative day we get a better neurological exam. We've already gotten imaging. We get a CAT scan to make sure there's no bleeding, and we get an angiogram done, typically do that first case in the morning after surgery to make utterly certain that there is no remaining AVM. If there is we go straight back to the surgery because it's at high risk of bleeding. So we confirm that it's all out. Once that's done, the patient is stable and we're now 12 hours out, say, from surgery, then we want to get a good neurologic exam, and to do that we decrease the sedation or allow the patient to emerge more so that we can get a general sense, is the patient waking up, is he or she moving the right arm, the left arm, those kind of gross functions, and then the more subtle ones like in Maggie, of the visual system. As you can imagine, the patient has to be pretty awake to cooperate with that kind of a subtle examination. Andrew Schorr: Now, Linda, there was some visual change, right? Linda: There was some visual change, and as she woke up there was weakness on the right side of her body, which was the first obvious issue to work through. And then as she started regaining function it became apparent that there was a deficit on the right side. She just couldn't see things on her right. Andrew Schorr: Right. And Maggie has shared with us that she has lost some peripheral vision on the upper right, but, I mean, she's running, and it sounds like she's leading a pretty normal life. Linda: She is. It seemed like a long process to me. She needed some physical therapy and occupational therapy and speech therapy just because the trauma of the

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surgery was significant, and as she was healing I guess she had to rewire things a little bit, so it probably took three to six months before she was pretty much functioning the way she had been functioning before all this started. Andrew Schorr: How much school did she miss? Did she miss like a year with all this, or? Linda: She actually finished the school year. She missed a couple months, and I worked with the school, and the school was very helpful to keep her going and get her caught up so she did not fall behind in school at all. Andrew Schorr: Dr. Batjer, now, you talked earlier about the amazing brain and the brain itself moving functions around. So will some of the deficits that might be apparent right after surgery, will some of those subside? Dr. Batjer: Absolutely, and especially in young people. If I could choose between taking care of an AVM on a 12-year-old and a 42-year-old, give me the 12-year-old every single day of the week. Kids are resilient. Their brains have what is called plasticity, and you can think of that as the capability of rewiring, just as Linda just stated. And it's not exactly rewiring but it's pretty darn close. They just develop new mechanisms of providing for certain functions. Weakness in an arm and leg, if it happens in an elderly person that is bad news for their long term. In a child it doesn't mean bad news yet. And the process can take six to 12 months of therapy and just time keeping the joints limber before recovery occurs. With the more subtle things like visual field involvement from an occipital or malformation in the back part of the brain, the recovery typically starts occurring within the next few months. It can be incomplete, as it's been with Maggie. She's got a quadrant of her visual field that's impaired, but kids work around it. And back to sports, back to all reading activities, driving, the works is totally an expected outcome. Advances in Treating AVMs at Younger Ages Andrew Schorr: Dr. Batjer, you've been at this quite a while. You talk about your history and… Dr. Batjer: Thanks for not being more specific than that.

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Andrew Schorr: …training. So and you've talked about the materials you use, the techniques you use, the sophistication of the family, the other tools that other members of the team have. It sounds like in the treatment of AVMs in young people we've come so far. Dr. Batjer: Absolutely. It's in various elements. It's the groups of people that put together strategies that have a high likelihood of success with a wide safety margin, new technology that we use in the operating room, our localization, our intraoperative navigation, functional imaging to help determine who can and should not be treated, radiosurgery, new embolic techniques, much better critical care. I mean, I think that's been a real paradigm shift. And we see it in our children's hospitals, my gosh, they keep these little people under such incredible control for prolonged periods of time and they walk right out of there. It's just incredible. Andrew Schorr: Now, although in Maggie's case or other patients you've had over the years, do we consider them cured or does this there need to be ongoing monitoring? Dr. Batjer: That's a very good question. For an adult, let's just take a 25-, 30-year-old person and treat them as Maggie was treated, that person, with a postoperative angiogram that's clear, that's a cured situation. In the young folks there is an incidence of recurrence. The number varies on the series and you can kind of pick your number, but I think between five and 10 percent of AVMs do regrow in children, and sometimes they don't regrow in the same place, which is really bizarre. But I have had a few young folks that fall into that category that have to be retreated when they get to be, you know, in their late teens or early 20s. Monitoring and Follow-up Andrew Schorr: So is that annual visits and MRIs, or what would you do just to monitor? Dr. Batjer: Yes, following with MRI is a good way to do it, noninvasive, no radiation, but I think they should get an angiogram right at around the 20th birthday or at least at the time they get out of college. Andrew Schorr: Well, Maggie is going to get out of college pretty soon, Linda, but you're aware of this. Linda: She had an angiogram a little less than a year ago, last summer.

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Andrew Schorr: Okay. And looked good? Linda: And looked good. We'd like to say she's cured. Hope for the Future Andrew Schorr: I hope we can. Dr. Batjer, though, we think back over Maggie's story, we hear from Maggie, you've got to be, just feel very gratified as a physician that you can help in this way. Dr. Batjer: Absolutely. You know, there's nothing like a young person, I mean, I'm just thinking of the challenge of being a doctor and the blessing of being one where you have somebody with so much at stake and a complicated algorithm to work through and you end up with a healthy person with a great future. There's nothing better than that. Andrew Schorr: So, Maggie, we've heard from you earlier and your mom and your doctor. Now, you can look back on this now as a young adult. There is someone listening who maybe is worried about an AVM for themselves or for their child or teenager, and they're saying, oh, my, how should we handle this. Any advice for them about getting the best care? Maggie: I think it's really important in this type of situation, just be confident in--you know, you should trust that with Dr. Batjer and Dr. Bendok that you have the best care and really just trust that, you know, six months to a year from now you'll be looking back on this experience just like I was thinking how it was something, just a hiccup in life and now I can go on living life like I was. Andrew Schorr: Right. We surely hope that for you. And clearly you agree that getting to a subspecialist in this case, not just a neurosurgeon but a team where they specialize in this, that's pretty important. Maggie: Yes. I was very happy that when Dr. Batjer was giving us the numbers about dealing with this exact condition, you know, his success rate, and it just gave me a lot of confidence that I too would be yet one more success story in treating of AVMs. Andrew Schorr: Well, here you are and we wish you great luck in getting a job as you graduate.

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Maggie: Thank you. Andrew Schorr: And you've got to run some full marathon sometime there, Maggie. Maggie: Yes, that's the goal. Andrew Schorr: Okay. All the best with that, and thank you for being with us on Patient Power. Maggie: Thank you. Andrew Schorr: Linda, your daughter talks about all this. She's very grateful but, you know, again no big deal and she says I'm ready, I just want to go get a job. So you're looking forward to a bright future too, right? Linda: We are, absolutely. We're thrilled with the outcome. I thought it was a terrifying experience. It is really a responsibility for parents to understand what is going on to get the best treatment possible, to do your homework and really be an advocate for your child. And when everything comes together we get a terrific outcome, and for us everything came together. Andrew Schorr: And, Linda, we talked about with you as an advocate you got to one of the leading specialists in the country. He's with us here. Of course, he represents a whole team. Is there anything you want to say to Dr. Batjer publicly? Linda: Absolutely and I've told him this before. Every day I think of Maggie and I look at Maggie, and I just thank god that we had the doctors that could take care of her, Dr. Batjer and Dr. Bendok, absolutely. Dr. Batjer: That is most kind. Linda: Well, you gave us our daughter back.

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Andrew Schorr: Thank you, Linda. All the best to Maggie. And Dr. Hunt Batjer, thank you for the work you do at Northwestern and specialization you have in this to help kids like--I should say young women like Maggie and other young people, and thank you for all you do at Northwestern. We appreciate you being with us. Dr. Batjer: Thank you, Andrew. Andrew Schorr: This is what we do on Patient Power is connect you with really such memorable stories of people who are unfortunately touched by serious health concerns but, boy, when it can turn out like this, as I hope it will for any family that may be listening, it just--it makes you feel so good that there's the technology and the talent and the advocacy from parents and patients to bring it all together. I'm Andrew Schorr. Thank you to Northwestern Memorial as being such a long-term sponsor to bring these programs to you. Remember, knowledge can be the best medicine of all. For more information or to schedule an appointment with a Northwestern Memorial physician, please contact our Physician Referral Service at 1-877-926-4664 or visit us online at www.nmh.org. Please remember the opinions expressed on Patient Power are not necessarily the views of Northwestern Memorial Hospital, its medical staff or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you.

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