World–renowned Vein of Galen Malformation (VOGM) expert Alejandro Berenstein, MD, Professor of Neurosurgery and Pediatrics at the Icahn School of Medicine at Mount Sinai and Director of the Pediatric Cerebrovascular Program at Mount Sinai Health System, reviews a VOGM case on a neonate with severe heart failure. Dr. Berenstein and the Mount Sinai team performed 16 neuroendovascular embolizations on the child beginning when he was six days old up to six years of age, utilizing an innovative transvenous embolization that reduced the fistula flow and cured the malformation. Dr. Berenstein has successfully treated the highly lethal condition in more than 300 pediatric patients. Dr. Berenstein will be co-directing the Fifth Bi-Annual World AVM Congress at the Icahn School of Medicine at Mount Sinai September 26-28, 2021. Learn from leading experts from around the world about the diagnosis and treatment of arteriovenous malformations like VOGM. To learn more, and to register or submit an abstract, visit avm2021.org.
Thank you. We're going to present a case of a young newborn child with a vein of gallon malformation and how the technology has taken us uh to save this child life and eventually cure the Children. Uh This is uh case down at the Mount Sinai Health care System in new york city new york. Uh These are some of the uh conflicts that disclosures. This is a six day old baby boy born full term through a caesarean section. On day two was in severe congestive heart failure, pulmonary hypertension and an enlarged heart. A cranial ultrasound revealed a vein of gallon malformation. An immediate postnatal M. R. Imaging of the brain. T. Two weighted images demonstrate both in the actual and corona all projections a typical image of a vein of gallon and a reasonable malformation in the midline, with prominent feeding vessels representing a correctional type. Whenever we do the immediate post natal M. R. Imaging, it is important to look that the brain is normal uh As this will have predicting value. The brain is very normal. The baby is now transferred to the Mount Sinai health care system. Uh And in our neonatal intensive care unit We were able to calculate the two umbilical arteries and the umbilical vein. And this is the access to this baby body. And this is a technique developed at the Mount Sinai health care system in which we use the umbilical artery as a means to reach the brain. And what you're seeing now is the use of a night and old guide wire place in the uh arterial line for an exchange. And now we hold with our hands the umbilical court and are now advancing uh the guide wire and bringing the catheter out to exchange it for an introducer sheath which could permit us to get into the descending aorta. And through that we now have access to the arterial system. This is the setup in which a four french introducer sheath is placed in future in the umbilical court and it gives us access so that multiple characters can be pain introduced. And if there is any obstruction of the catholic we can easily change it. In addition we can keep the umbilical access For up to 5- seven days. Here is the control angiogram of the left vertebral artery in frontal and lateral projection to demonstrate a typical colloidal type pain of gallon malformation in the newborn period. These Children's are very sick are in severe heart failure. So a full angiogram is not done as we do have the M. R. Imaging which is excellent. So we know that the major officials are coming from the right posterior cerebral artery. So we're going to try to close this in the early phase during this newborn period. And here is a micro catheter advance from Into the postures are large and here is that fistula seen in the frontal and lateral projection. These are the same images. And here is the video of the liquid anabolic injection of 90 Syahnakri laid mixed with tantalum powder. And you can see how the flow is quite strong and you can see how the feeling of the fistula. This is the arterial political. And now you see the glue entering to the venus side, which is an expansion in the diameter. We take them the catheter out. Looking at the level view, you can see the nearly pure glue and here is the official inside itself and this is the removal, have the catheter, another fistula in the same procedure. Here we have the Catherine de ap and here is the glue injection in the ap with selling of the pinnacle and closing the fish to itself. Will see that this is the lateral angiogram. And we're going to see that now in the lateral video this is the injection of the new syahnakri lit again 90% and it's entering its closing the fistula and now we will seal off and close the pentacle. This is a third Fischler down to the same period and here is the injection of the soundtrack relate. This is on your left the control angiogram in ap and lateral. And here is the injection of the group in the lateral projection. So this is the third Sana Accolate injection that day. And you can see how the previous glue help us to retain this. An immaculate small amount is in the vein but it's not significant. Now this is the angiogram. After that session you can see on the left side the major officials have been occluded. Of course there are still significant flow but this was necessary to decrease the floor and take the child out of heart failure. This is now in a second day. It's also trans umbilical where we are advancing another micro catheter into another fistula. And here you see again the liquid symbolic injection. And you can see that actually we're closing more than one fistula at this location and we'll see it also in the large projection his arterial to arterial anastomosis. So we're able to close a number of fistulas in that case. And he was seen in the lateral projection. You can see that there is one fish volunteer lee. One is in fairly the vein is in this area and this area so that permit has two additional close malformation. And again you saw you see now the decrease in the floor to this malformation. Now several years later the child coming for additional treatment and now we're gonna go to the anterior cerebral artery. And here is the catheterization with age. A guide wire. You can see how we advance into the anterior cerebral artery. Here is in a lot of projection. We use a J. Wire to be as a traumatic as possible and we can relate the anti is allowed to on the right and now we advance all the way all the way until the side of the visualization. Here is a detachable tip micro catheter you see to tip marker. This is the angiogram. This is the injection of design immaculate in the frontal and this is in the latter projection. Here comes the glue. Again you see the artery to vein fistula. It's a perfect demonstration. And now in this type of catheters we can actually bring the glue to this area and the catholic will be detached at this steep. So we're showing you as the progression of the treatment. Also the progression in the technology. As we continue the treatment the Charlestown developing Koronadal supply. And here is the micro catheter again to tip marker, turning all the way into the anterior Kuroda lottery. Here is the angiogram. The position is a little too deep. We bring the tip of the cap is slightly back and now we do an angiogram again and we can see a much better position. This is the angiogram and this is the actual injection of the glue material as you can see as it enters into the malformation. Again we have a two catheter tip so we're going to detach the tip of the catheter. And here is another injection and you can see again the frontal and this is the lateral injection. And again we go from artery to vein. So there has been a number of additional procedures and additional injections. Another glue injection on your left. You will have the baseline angiogram on your right, the video and the detachment of the tip of the catheter seen after the injection of the sand rock really so that we are seeing how we are progressively obliterating the malformation over a number of procedures and years. Here is a control angiogram where you can see it is strong and down but there is still a procedural supply. We we now have uh the catheterization of another uh session uh in which we let things mature. And we're now going to come in the center of where the fistulas are. And again uh this looks more like a malformation and you can see the penetration of the sinovac relate the child. Now is child becomes four years of age, uh Sergeant having dural supply parasite. Ation of dural supply coming into the midline. And here we're using another technique which is the technique of advancement of a balloon tipped catheter to permit us to inject another liquid and publication that you can see here uh the injection of the anterior femoral artery, residual fistula with excellent penetration and excellent occlusion. And you can see how control the injection can be done when you have control over the floor. And again you see the fistula side excellently demonstrated in this video. And now we're going to detach that tip Catherine. You can see we gently advanced and you can see how it's attaching. Look at the lateral that is the detachment of the tip in this new type of technology benefiting the treatment of this child. And here is the dural contribution. This is the middleman angel artery on the left, in the frontal and lateral projections. And we can navigate all the way here using a mini balloon catheter which has two limits. You see here the catheterization uh in the frontal and lateral projection, here is the micro catheter and we see all the way this is the two tips of the balloon and this is the tip of the Catherine. And with this technology we now can reach the dural supply. This is the contrast injection. This is actually the liquid anabolic. The liquid anabolic here is now A D. M. S. O. Base called onyx. And what we do with this D. M. S. O. Base is we can continue the injection, continue the injection until we reach the venus fistula. Additional injection with the same catheter ST balloon. This technique permits you to continuously inject progressively including the malformation. And this is the result of the angiogram where you can see that we have completely eliminated the deferral contributions to the malformation. This is now at the age of six years the child comes in and you can see there is still a residual malformation but the venus system has gotten a lot smaller. In addition to that, we can see that there are perforating arteries that come in to the area where the vendor gallon was. And this is a very dangerous territory. This is called a transmit sensitive phallic. So you have the the midbrain is in this area. So injection of any liquid symbolic can be very dangerous. So what we're going to do now is use another technique which is a trans venus approached and we're going to come from the juvenile vein and come up through the sigmoid sinus. coming to the confluence sinus Catherine's there's a cyanosis here but we're gonna try to get it all the way inside the vein with two separate catheters. Here is the video of the tri axial. That means one Catherine at the jugular vein, another bigger Catherine, the uh sigmoid sinus. And now we come in with a five french parents thing coming in uh to the straight sinus, there is a straight sinus and then using a guide wire there is some glue here uh and we have to find the channel to be able to reach the deep part of the vein. So here we have the guide wire, looking for that space there it is we are able to penetrate. And now we advance the five french catheter. And now we're going to advance the eight french, which is a much larger bore Catherine. You can see a turning here and this Catherine will give us access so that we can put more than one catheter to treat the malformation. Now we're going to be injecting against the floor. So now what we're gonna do is put a set of characters. Here is another detachable tip catheter. Here is the guide wire In latter projection and now we're going to advance, this is the touching tip. So we're gonna put this catheter as deep as we can right here. And here is the position here is the three cm that we have to navigate. And here comes a second detachable tipped catheter that we're gonna put in between Those two in this location. So here is coming with a micro wire. We again looking for the channel and once we find the channel we advanced the guide wire and here comes the second micro cather you see the tip, the two tip marker. And we're going to put this in the middle of those two. And this is to do a thing called a pressure cooker technique described by dr chapel. And what we're gonna do is put some calls first here and then we're gonna put some glue. So now once we close that here uh in this segment we can then inject liquid anabolic through the distal tip of the first catheter. And once we're done, we were separated by the touching it here and the touching both catheters. Now here we have the lateral view. We're going to put sinovac relate to seal and prevent the liquid symbolic to come backwards. So here is the NBC. A. It is placed too close that segment and then we detach the second catheter. And we still have access to be able to inject at this location. So this is what's called the pressure cooker technique because it prevents backward uh migration of the liquid anabolic. And now we're going to show you how we inject the liquid anabolic. And we're going to feel those, look at those perpetrators, But we're feeling them retrograde against the floor. So here is one and here is the 2nd 1 and we inject the onyx material, DMSO base. And as we start injecting we can see another of the perforating vessels that comes back to its origins. So here is where it came in to the malformation. Now we're going to detach that original catheter. Uh and you can see how we're pulling and there comes the Catherine. So once it's detached we now can do a control angiogram and see the total complete elimination of the malformation. And look at the perforating arteries. You will see how they are stagnant as they can go to the Malformation. So this permit a complete cure of this child. The child at the age of seven is neurologically normal and benefited from 16 separate procedures from the newborn period to the age of six years of age. Uh And it shows the cameras of how we have evolved in the technology of treating this most difficult type of cases. Thank you very much for your attention. And uh we are available at Mount Sinai to help all Children with vascular malformation. I want to thank Joanna 50 and Tomoyoshi Shigematsu, My partners and Michelle are Soldier and Evelyn Dear, which is the fabulous team that we have to treat these Children. Thank you.