Cerebrovascular neurosurgeon Tomoyoshi Shigematsu, MD, PhD, reviews the two classifications of vein of Galen malformations (VOGM) and presents recent cases of VOGMs treated at Mount Sinai using the trans-arterial and trans-venous embolization techniques, as well as other treatment approaches. He also describes how to diagnose VOGM and monitor the condition in utero.
Chapters (Click to go to chapter start) Dr. Shigematsu describes his experience as a neurosurgery resident in Japan and how he learned to understand neurological anatomy A review of vein of Galen aneurysmal malformations (VGAM) Classification of VGAM and its presentation, plus important points to note Trans-arterial embolization technique for treatment Trans-venous embolization for treatment and coiling for cure Antenatal and neonatal classification and evaluation VOGM and angiogenesis in both medical school and residency and neurovascular fellowship in Japan or beginning as an attending there and then moving to the United States to complete a second neuron vascular fellowship here And then joined the department in as an instructor and then an assistant professor in 2018 and 19. And he's here to speak to us today about the vein of Galen broke. Yeah thank you for the introduction introduction then. Um Then I'm the one of the several vascular nurses and working here. Um And today again I want to talk about the kind of gal and more information but not the basic but a little bit updates and oh please you can show and share my screen. All right. And I don't see any residents in the panelists. But if I saw that we can move them up if you like we'll move them up for you just if okay they can help me present my presentation in the first half of the presentation. But um again yes I'm gonna start to talk about information but before starting my clock um just peter give them my introduction from Japan and I'm from Osaka. and my neurosurgical residency started 2006 has been 16 years already. And this is the the semi arrow. This is a hospital in Osaka University, the biggest tallest building here with the medical school. This this week. I started here. It's a large university hospital. So we had in the neurosurgical department. We had all the spine group. We have a tumor group, vascular group and function group. It's this hospital is the um active function. Uh There's two functional neurosurgery department. Part of the it's very big. So um we had all the groups. So during the residency I was able to experience all the all those um expertise and I also did the little presidency and the smaller affiliated passed overhead in the middle which is also the nearest neurological institute. It's just a 120 beds small hospital just with neurosurgery department. Um So basically we handle the mural emergencies and like mainly stroke, we didn't do spine here. So mainly stroke and other new emergency like trauma just focused on that brain and some tumor emergencies. And the second half of my residency for three I spent three years in court. The city medical center General hospital. The Kobe is just next to Osaka. So it's in the same region and this hospital is really really big stroke center has a stroke center and a leading hospital as a neurosurgeon in the vascular surgery and as well as several vascular surgery. So um as you see probably I had the experience of pediatrics only in the university hospital. And I found presentation I made In 2006 in my hard drive. So uh in our residency for every case is a we experience we present in the weekly conference. This was a case where the subarachnoid hemorrhage it looks like and it looks like you had the income. This was a film and we scanned it into the presentation and then we needed to organize and this is a post up. It looks like we did the by frontal craniotomy in into the inter hemispheric approach. And it looks like there's a paramilitary oedema. But it improved and then the patient is doing well something like that. And we will hopefully this video works. It doesn't work. It's okay. But for all the surgery I we needed to what we did edit the operative video. As we usually I see the resident does here as well. Our doctor Benson does and then this is my first series last did the clip and I didn't do the cliff. I did it the video and but this was a very good training to understand anatomy. To the basket anatomy and then to review the case is because we didn't have many cases that's here per one resident. So we have time to review and this is all up notes that I really need. We really needed to write and then attending school science. This is in the japanese but as just not just writing. We for each case is we draw a picture looks like we did the by frontal craniotomy in this position and then open up the frontal sinuses open. I don't remember how we if I read this I can no but I don't know how it reconstructed the frontal sinus. But and we opened the juror and then took off the crystal golly by frontal opening and we went into the inter hemispheric and blah blah blah. Get into this is how I learned the same vascular anatomy. And then I found that the drawing picture is a very very good way to review the cases and the understand anatomy. I just wanted to show a little bit to our residents that that was my training and I'm based on this kind of training. Can you, can you go back to that tomo? Yeah, I cannot think of a better way to learn how to operate. You know, there are many ways to do it and I'm sure you see that in our program, One of the ways is we throw a lot of volume your way. And so as a resident you get to see, You get to choose from 3000 cases a year, 3000 open cases every single year. But you know you could see a whole wave of cases and not get as much detail or learn as much without attending to that detail. The example I like to use is to ask ask someone which letters correspond to which numbers on the telephone dial. And you know you think about it for a moment. You may not realize that there are no letters on the first number and not all the letters are represented equally. Some of the numbers have four letters, some have three um You probably have made what 10,000 phone calls in your life. A 100,000. I don't know. But unless you attend to what you're seeing, you won't learn it. Um So I think this is beautiful. You did this as a resident tomo. This was one of your cases. This is yes. And first year I found this is from 2006. So this is it. This is great. It's great. And go go back to the positioning. The first drawing. Yeah. Oops. Sorry, mm hmm. This one, there's so much detail here. Um Look, he's got the front of the frontal branch of the facial nerve and the that's on and the S. T. A. Um you know, he's putting the positioning in where he wants the nose and the face relative to neutral all of those, all of those details are absolutely key to learn. So um I don't know if maybe we ought to have the residents do drawing on one case per week, something like that. And maybe give it, give you a chance to show it to us. It is time consuming. That is also true. But it's very good. It was a very good way to review that. Yeah, thank you for sharing this. So that was my six year, seven year residency. Again, I didn't have an uh enough experiences pediatrics or spine and um that's why I feel a little bit. I didn't expect to talk about the pediatric and at that moment and I was interested in. So in Osaka City of Osaka City General Hospital which is not affiliated to our Osaka University but um this is a big center with the pediatric, several basket with dr and thankfully we were we got it, we became a good friend and I was able to you know go there. It's a very close, it's very close to go there and then observe cases and discuss cases there. So that's my basics and I want to get some updates about the van of talent. But first of all I spoke about the vein of Galen malformation a little bit in june last year. So I want um this is the regimen today. Um gallery rebuilding, Gallimard commission and then slowly go to the details so to treat them. Usually the trans arterial embolization but there must be another treatment. So I will show you some other treatments and dr Fifi talked about the details about it. Yeah I touched a little bit about the trans venous and recently um some progress in the prenatal management and neonatal management and in our practice we see a lot of and your genesis with the advent of Gallimard formation cases. So I want to show that as well. But first again um well let's review the or information. It is rare and from Germany they gave us the data that they are one out of 58,000 live births. It's um and I told last time that it's not that rare that I I thought before and here and I see Alex right in front of me. So if you can ask if you can help me um This is arteriovenous shunting between you know, can you tell which area in which pain it's so um okay you're you're saying specifically in this case correct information. Typically information is what kind of batteries from which can which been it's drained into. I'm training to the sandy Guillen as far as arterial theaters. Um Yeah, can be variable but we often see Islamic perforate ear's uh colloidal proliferators. Yes. So yeah, criminal arteries is the main theater sometimes the we call it a arteries can contribute it into the more information. But corridor arteries are the main theaters and it is not the vein of yelling itself That they found in 19 At the beginning of 1980s. It's not that the debated event is exactly in Alabama, no yelling but its precursor of the vein of it's called median vein of president cephalon or medium presidency phallic vein of Makowski. Um It is not then on the island itself. This is one point that it's I wanted to emphasize and again Alex do you know exactly which kind of what kind of space? The reign of Valdemar information is located in the super accurate actually the subarachnoid space. Where is the typical a VM located it's not any more more right more in the prank and I think maybe in in the literature they said it's sub pile space. Yes it's it's located supply away and all the direct and all the features of pile theaters and then it just goes to the the brain drainage. So it's a little bit from different, it's a little bit different from the pen of A. B. M. And the gallery is a little bit different and as opposed to being a intravenous smart mission to be an umbrella and does not have what typically the Vietnam gallon does not have what kind of structure that usually A B. M. Has like a true true native. Exactly. So it doesn't have the night. So it's it is theoretically it's a v. Fish lights like a zero A. V. Fisher but it's not on that, it's not on the draw. It's not a fish shot. It's not on the but it's on the it's on the medium grain of president cephalon official. So these are the points of anatomical points that it's good to know at the beginning. This is a picture from dr Bernstein's textbook but it shows there's a brand stamp here and the market mission itself there's no night. And so it's very hard to tell where exactly it is. But the fish to this point is here it's on the the precursor of the venom gallon. It's located on the uh usually in the Vietnam into positive or behind the victims. So the original cistern and there are two types of bane of gathering more information. I see Brandon there. Do you know what type? And yeah. Have you ever heard of types more information? I think that there's a steroidal type and a mural type. Exactly Kuroyedov type of neural type um prototype is a little bit more complicated. She has a complicated structure than the mural type. Mural type is a single hole fisher. They're likely into that, you know. Uh Makowski, yes. If we want to explain which about different between the corridor type and the mira type, this will maybe the way to easier to understand. So this is the um fetal a picture of the fetus and this network, this is a precursor of the vein of gallons. So based on this, the vein of gallant itself is going to form and it's a network, this is the vein of person settle in and if the shand happens like in this network there's gonna be completed, the structure is going to be complicated, complex and it's gonna happen and it's gonna be become a prototype and the for being a person's cephalon start disappearing from the front and then eventually it's going to go away. But so if the sound is here, that means the shunt developed in the earlier stage. And if the shunt develops in the back part, it becomes a mural type and it's less structurally. It's less complicated. So they see the corridor type involves a lot of different kinds of peters and mural types? Uh usually less feeders contributed and these are the two types but what about the other very dilated vein of yellow and this is a little bit different then. We called this vein of yelling aneurysm dilatation P. G. A. D. And the baby currently in Nicu is not a typical wayne of yelling more information. And this is the three D. Picture. A little bit hard maybe hard to understand. But this is three D. Images from the M. R. Angiogram and you see the dilated pain of gallons but you can follow the and richard greatly and then it becomes it comes all the way to the right side, right side until like uh around the hippocampus or medial temporal lobe. If you follow this M. R. I. T. Two. This it's around here and this is not a difficult man of god. And more information but this baby has applied Phil peel. Maybe official. It's a little bit different but this isn't as opposed to that that the oldest just chill out venom gallanar information usually exists in the midline and supplied by by tomorrow which is Ontario corridor arteries or arterial corridor arteries or some theater from bacterial cerebral artery period colors the lottery. Let's see here. It's located in the midline. That's that's typical. There are some um exemption but this is a typical man. I got my information. Just wanted to show the pictures. Okay the presentation is as as you know um It def it's different when depending on when the baby present to us if the baby is and so we can divide it into this. These periods is Anthony. If the feel screening shows a big a round structure with the pulsation of the ultrasound. Um we can say that there may be event of the MARS mission. So the routine screening or that screening work up of the high jobs. Can b 11 the presentation with the anti NATO nao NATO three main symptoms gonna be heart failure and infant. They failed to thrive. Increasing circumference is or hydrocephalus like this kind of symptoms that have to monitor our city if the baby is having the market, this is a good point for good mark to follow up. All right. Yes. Afterwards. Usually like they are found incidentally for the head injury or of course it can show developmental delays, seizures and can bleed and if want to make it simple. Usually the corridor type present in the new Penelope or it was crying, congested congestive heart failure and the mural type present is the hydrocephalus and in front it's not always. But if you um if you think that way that can make it's simpler to understand. So again, especially on the median vein of prison Switzerland is a corridor type and mural type and truly in the brain and gather more information. We need a lot of providers involved pediatric cardiologist unit pathologists. P cute and or of course O. B. G. Y. And even including the family. So as a nurse in the vascular surgeon or neurosurgeon um you need to be a good conductor too. Our team leader to manage the venom gallon mark mission. And as I said this gold standard is stage transfer material embolization and targeting. The officials don't just don't close the theaters. I don't think it's important. But I see matt there due to manage the hydro syphilis. What you should do for this kind of uh if you see the venom hydrocephalus and venom gathering baby was what's the first thing you should do? Do you need to have to jump onto the shunt? Do you have to put the DVD? I don't I don't think you have to. I think you can monitor I think you monitor clinically you know follow head circumference and you know only jumped the CSF diversion of genetics I think Like what 4 4 months old. The venture goes getting better bigger. It's a patient is vomiting and in that case I would I would think CSF diversion it's warranted. Yes you I think it sounds like you need to place the DVD or it sounds like you need to shout but this kind of patient. The venture card is not getting bigger picture. Usually it's not because of the obstruction of the sometimes it does a big mural type of venom really market mission can obstruct the aqueduct and then called the hydrocephalus so they E. Tv might work. But usually it's hydrogen dynamic. So the it's venus hypertension causing the less absorption of the CSF that causing hydrocephalus. So usually hydrocephalus in vein of Galen is managed by the embolization first. So if you see the patient with venom gathering more information, started vomiting inventor codes getting bigger. That's the urgent sign to proceed. Not the shunt but the embolization that way you think it's a little bit different. It's a good point to remember I think so these are the important part points in the vein of yellow. More information. And um so I go start from here and start talking about the updates. Thank you all the residents. So says the material globalization is the go go's standards and it's asymptomatic. We don't we want to keep the babies developing normally. So we go relatively conservative, minimal risk. We don't want to cause any complications but still want to close the more information that they eventually if the symptoms if they're symptomatic it's they want to go urgently and very aggressively to reverse the symptoms and targeting official. Not that we tried to close the venus side of the market mission. That's important like these kind of if more information uh we have to first figure out where the, so we have to figure out where the official is. Probably around here probably around here It looks like two major community major officials and then we have to find the route It looks like as far far away to go. It looks like the A. C. A. Might be the way and it looks like PC A. Might be the way it looks like about it. And and also maybe these small Harris May thanks to the small capital. We have these are maybe the way we can get in but we see multiple small routes. So it's very very difficult and challenging and risky to close everything in one session. So that's that's the reason we take multiple sessions and um well we're taking the bigger route we can use that uh detachable tip micro catheter that Doctor Fifi talked about just now and inject the glue and take close a big fisher and we try to inject the glue as far as possible. Close to the bank or any into the bank. It's all the uh igloo flew into the bank genocide. We don't want them. Yeah put the glue into the van too much all the way to the lung. But just a little bit is okay. And if we need to take the smaller vessels, see how far the micro catheter goes. This micro catheter is flow guided micro catheter. We usually don't need a wire to get. We have to direct it when I point to the way we need the point. Use the wire. Two point where we want to put the micro catheter. But basically it's low. Uh huh navigated. So it goes wherever the high flow is. So this catheter navigates all the way to the far end and we can inject the glue. But this is not the detachable dip capture. So we have to take the capital help quickly. Otherwise the cattle can get stuck in the brain. In this way we have been achieved total of like 80% of the cases we can get to total collusion and 80% of good or fairer outcomes. But even though with the technology recently uh no because of the technology recently as Dr. 50 showed we've got the smaller boulder and guiding chapter and in this kind of my venom information with a lot of angiogenesis um We started using Onix for the and it can be it can spread the annex from a little bit far away so we can inflate the balloon and then let the onyx go penetrate, penetrate, penetrate through this fine vessel and then it eventually gets to the vein. It was very difficult with just a glue and smaller micro catheter to penetrate far. But we this is a new technology that we're getting and but still it's very risky. After the multiple session of the transcultural NBC embolization like this. Um What kind of options do we have is just is it just the trans venous segment? Ah Is it just the transformation of civilization. And when we reviewed when I reviewed our cases from 2004 there were three ways to finish to cure the more information I found one is of course The 80 and 80% we were able to cure. It was just NBC a ah embolization from material side. But there are some cases developing some general anastomosis. And sometimes we can cure that more information from the dural anastomosis with onyx. We see the ceremony angel artery contributed to the more information and then we can go this route with that small and micro catheter and then inject onyx from far here. Eventually it goes here and then completely occlude the more information. If there is no route to the venom gallery, more information then The kid needs to be older like more than 10 years old I think to predict the brain. Um Start tactic, radiosurgery is one way to go. And we had two cases with good cure. But again after 50 already talked about it. But the trans venus embolization is also of course one of the options and a little bit talking about the trans venus symbol. Ization. The trans venus symbol. Ization. They started inventing gallon reported in venom gallon in 1st 1986 like this way trans tubercular coil embolization. But those cases were like coiling in this big sack and had a higher risk for hemorrhagic complications and worse clinical outcomes. So this coiling was being done in some institutes but in our practice we haven't done it. We attended avoid this way and doug. We took the gold standard way. But once the more information is small like this that was this is the same case from here to here now it should be we should be able to close it again. It becomes very very attractive. We can close it so easily to get from the venus side and why don't we do that? And we we did first coil m belies this small residual mark formation And it worked in 80%. 10 out of a patient got a cure without any issues. But on the other hand in this way we had to hemorrhagic complications. one may remember what yes. Uh not too recently but we in this case series we had one mortality with hemorrhagic complications like after A few hours later after the procedure. And one more abilities has 48 hours after the procedure leading. They had the patient had a salami Cambridge. So we were looking for that another way. And as we learned a lot from dr chappelle um we started doing the trans venous support pressure cooker embolization. He started calling that way with uh not just a coil with the help with symbolic material like this. I saw this. I like drawing um using multiple micro catheter and the detachable micro catheter with the coils your metabolic materials. These are the new things that we started using in this kind of um Reddit still rare but complicated diseases. Support pressure cooker technique has we use a combination of coils, liquid metabolic materials versus transitional still I think there are some cases that we can just coils but we'll see. We have um still keep looking. But adding the anabolic material, it seems safer because um we can symbolize the angiogenic genetic vessels or network as well from the venus side to prevent the rupture. Those rupture like a. Bms. We don't want to leave. Then itis the angio genetic vessel might be the cause of the bleed. So we can immobilize those vessels together and then add some safety. But if there is a normal thing then we have to spare it. And in the end the Beatles and tornadoes and tornadoes. We in the M. R. I. We are able to see this is the same picture I showed half a year ago. But if we see our cases the. Okay, so these are all the moms, not the babies. See the red cases. The baby didn't get the treatment because the brain was already bad and the blue cases, the baby needed the treatment because the heart was bad. Um And only the three cases we didn't need to do anything during the neonatal period see And the vascular treatment was needed and more around the half of the cases. And then these neonatal cases it's it's it's not It's not 100% safe to do the procedure. The patient has this heart failure that we needed. We need to do it. It's it's working. but and it was still the hemorrhagic complication rate is high. The cases we didn't need. We didn't do the treatment was seven cases but clinically Doing well it was only three cases. Brain damage is more than that. So can we predict any deterioration of the brain damages or anything? But we the only two ways we have is we the modality we have is the echo or M. R. I. Um head out to restaurant having four. I'll think about it later. About five minutes left. Yeah the feel. Echo evaluation is important. We can read it reported our case series and it looks like um combined cardiac index is high and this may be one of the ways to predict or show. Yeah you give some information and but there are some reports from others from the said hospital and in France. Major major hospital has a lot of they reported like there was a pseudo feeder signs that the M. C. Is getting bigger and those maybe contributed to the more information. Those cases can Dettori a rate. And from boston they measured the federal out just the size of this straight sinus or the helping scientists the size of the surrender feed or M. R. I. And if it is bigger than it is there 88% that the baby may need neonatal treatment or the brain can get worse. Those are the good information that we can use and management of the gallon mark missions. So that maybe give us some indication of the fetal treatment we in our institute unfortunately we haven't developed for it construct any thing but this sort of thing we may think about in boston there start recruiting the fetal treatment for the patient can get worse. And the final thing that angiogenesis, this fine vessels. These vessel makes us more challenging to treat the spain of calamari information because it's hard to get in and it can start to caramelize at least. Okay. And it's it's interesting. It's it's not just there. You see this is the case without anything seven days. We didn't see any fine network. But this M. R. A. In one month develop this fine network. What is what is, what is this? Unfortunately I don't have the answer to this is a good point that we want to um start working on. If you if you follow the angiogram it looks like at the beginning it looks like this. But like it looked almost looked like a VM. Maybe the A. B. M. Is not A P. M. If there was official. But this is the true pain of the other markets. So this is a very good point that we can study. There's nothing and then I just defined vascular network develops and then this is a good clinical observation were reporting and we know that once we close the bank, those fine vessel goes away eventually we know that that's that's what we reported recently. So once we close the fish lower van the fine vessel disappeared. It's very interesting. And some patients develops this network but some doesn't it's a genetic so we are conducting right now um genetic studies collecting the blood from the babys. That's one exciting. Uh And then we started and we also can find the potential biomarker during the get some blood from during the prestige. And then you can uh find the mile marker. That's a next stage. So these are the venom gallon updates from me. I revealed these things and that's about it. Our pediatric service basket program with DR and Dr Bernstein. And we I want to thank everyone including Michelle, our nurse. And we now got a new P. A. Jessica joining and max is helping us a lot in the research. Um I want to thank everyone. And this is my and that's it from me. Thank you. Gemma was a great presentation. I hope that residents and everyone listening can appreciate that. Before all of these techniques were introduced. This was essentially a lethal condition really despite the complications that you talk about. You know, it's a dramatic shift from what this was. Mhm. Yeah. Thank you. Maybe we have time for a question or two before we have to sign off. If anybody has to go. We understand it's 9:00. Are there any questions? Please feel free. Mhm. All right, well thank you everyone. Um Great talks today. We'll see you next week. Thank you.