Good morning and welcome to um this edition of uh surgical, uh, wider surgical community Super Bowl grand rounds that we started a couple of years ago. And for those who haven't had a chance to attend this, um, and welcome to all of you on Zoom. But within the next few minutes, we usually get up to over 500 people in the room and, and on Zoom. This has been very popular and and lots of reviews. What we're trying to do is twice a year, uh, highlight, uh, some of the special things that, that happen here, um, either cause it's like really new and different, and people should learn about it, and, or it demonstrates how we collaborate at Boston Children's. Uh, and, um, and so we try and squeeze two cases into an hour. Um, and we are gonna work hard at that, uh, uh, um, this morning and lots of content. So, um, usually we get invited to come to like a big speech like this, like we read your CV and we tell people like, Where you went to college in Minnesota. We're skipping all that stuff. I just very briefly introduce the primary, uh, organizer, and then they will briefly introduce the people who are gonna speak, and it's a little bit rapid-fire. uh, and hopefully there'll be time for a little bit of discussion at the end. Uh, but we wanna just, um, uh, get on to, to, to hearing the, the, the wild and, and cool collaborative things that we do here. Um, uh, one quick public service announcement. As you're watching this, and you think of, oh, I should show something that this, this would be good, or I know somebody who should show this, please email me, uh, so that, and I keep running a list of things and we, we try to sort of spread out the topics. We're gonna start this morning with actually a redo nature of a topic. Uh, we, we were gonna do this a year ago, um, when sort of uh fetal intervention got going. Uh, and, um, and, and, um, um, you know, we're catching up in the world with fetal intervention. Um, but we very quickly jumped ahead with, um, uh, first, uh, uh, concept, uh, done here. Uh, and Darren Orbach was gonna present it and he had a, a family. Emergency, wasn't able to. So, we're, we're bringing him back. Um, since then, he's become like a movie star and all that kind of stuff over this New York Times. Uh, and so, uh, without further introduction, Darren is gonna talk to us about the, a cool thing that he and the team have been doing, uh, and I'll let him introduce. Thanks, Steve, and thanks for the opportunity to be here. Uh, so, I was gonna give background and motivation, and then Shami will present the actual case, and then I'll finish up with a little bit of perspective on the clinical trial that's ongoing. And I just want to start with a comparator case. Uh, this was almost exactly a year ago. Uh, these are MRI images from a fetus that presented about a year ago. On the top row here, you see the diffusion-weighted imaging, which shows no injury to the brain, so no stroke at the time, uh, and the brain parenchyma was completely intact. And they presented here for delivery. The baby was born 3 days, I'm sorry, before that, uh, the fetal echocardiogram, as it always does, showed increased flow. The heart is big because this is a high flow lesion in the brain, but valvular function and ventricular function was totally normal at the time. So this is the end of pregnancy. The baby was born 3 days later, and the echo immediately gets worse, as it always does. So now you have the beginnings of valvular dysfunction and right ventricular dysfunction, which is always what happens first in the NICU. Uh, the BNP is elevated, and then look at the first MRI after birth. Nothing happened here other than the baby was born. The whole left hemisphere is infarcted now, and you can see the swelling on the T28 image. Uh, the, the echo continued to worsen over the next few days. We did an embolization and the echo improved after the embolization as it usually does, because we can help the heart dysfunction by decreasing the flow and the malformation. What we cannot do though is keep up with the brain injuries. So you can see on subsequent MRI now you have dots of infarct in the right hemisphere as well, and infarcts in this condition are a snowballing problem. That's part of the, uh, the underlying issue. So at this point, the parents decided to go to palliative care for this child. Um, and in fact, systematic looks at how fetuses with this diagnosis of vegalen malformation do have shown that the outcome is terrible. So this was a meta-analysis that showed that if you add together the intrauterine deaths and neonatal deaths, you get a perinatal death rate of about 28%. Another 37% do very badly neurologically. So a fetus with this diagnosis has about a 1 in 3 chance of having a good outcome, surviving childhood with a good outcome. Um, and just a quick observation, all of the bad cascade, the pathophysiological cascade seems to happen after birth. It's quite rare to have parenchymal brain injuries in utero, and it's quite rare to have actual heart failure in utero. Usually, they're fine in utero like this case was, and then they start to crash in the NICU. And why would That be? Almost certainly, it's because of the placenta. You have this, um, very blood-filled, um, organ carrying about almost half of the cardiac output that shields the heart and brain in utero. Then you clamp the cord after birth, you lose the placenta, and now the whole burden of the malformation is on the baby's heart and brain, and they start to crash. So, considering all that, all of this, this was the motivation for developing a fetal intervention to try to diminish the flow in utero, uh, and prevent this pathophysiological cascade from happening in the first place. Um, and so, first, you need to be able to predict which fetuses are gonna do badly, and we came up with a measure that very reliably does that. Um, this is the main malformation, this is the falcine sinus bringing the blood. Blood to the back of the head and then back to the systemic circulation. It turns out the width of this falcine sinus opens and closes like a windsock, and the more flow there is, the wider it is, the less flow there is, um, the, the, the narrower it is, the less flow there is, and you can literally predict how the baby will do after birth based on the width of that sinus. We have now shown, and this is under review right now, that the falcine sin is Beautifully predicts mortality after birth, so the narrow sinuses babies survive low mortality as you go up. Uh, mortality goes up dramatically. It predicts the likelihood of parenchymal brain injury at birth. It predicts the likelihood of meeting developmental milestones. So here you are at the low falcine sinus width, they all meet their milestones, and then as the width goes up, they never do. So at 1 month, at 6 months, and 1 year. So we can really identify which fetuses are at high risk, and that allowed us to launch a formal clinical trial of fetal embolization uh with IRB and FDA approval. So now I'm gonna call Shami to present the case. Good morning, everybody, and thanks for having us here. Um, I'm going to the second case, which is a future intervention case, and we did the intervention here. Uh, case presentation, 36 years old, pregnant person. Uh, this was her 4th pregnancy. She had a, uh, tree-life, uh, pregnancy which, uh, was happening as a vaginal delivery at term. Um, this, uh, was a, uh, she did have an ultrasound scan at 19 weeks, which was unremarkable. And then at the third trimester, which Pain of getting usually diagnosed at the, you know, late 2nd to the 3rd trimester, was diagnosed from North Carolina and then came here. As Darr mentioned, you know, if you look at the Falton sign, you know, here, uh, the measurement is close to the, uh, 1 centimeter, which is a, you know, significantly high risk group of the patient, uh, and look at the brain, the brain at this point is completely intact. Um, This is now, uh, again, the ultrasound, uh, that we performed. You can see the Felsen sign is, is pretty big. Uh, venous varix is here, uh, trochlear is in the back. Uh, this is the, you know, in the vertex position, that means the head is down. When we put the Dopplers, called Doppler easily you can see the, uh, the, the, the blood flow inside the, uh, the structure. And um Um, again, um, this is a non-collar, you know, uh, non-collar, which I think we did have a eclipse here that doesn't work, yeah. No. But uh that should be fine. That means, here, you know, you see, you know, still without the collar, you know, you can see that, uh, the, the falsan, uh, what we do is uh ultrasound guided needle with an 18 gauge needle. We go, we drill, uh, the bone and then, um, with the needle, we get to the back, uh, here on the fals end and then we put the wire inside, which you can see something here is a little bit of the, you know, shattering. We put a wire inside and then, uh, start dropping the, uh. Uh, the codes, which is, you know, calculated prior to the intervention by Darren. Sorry, none of these clips are working here, but, uh, uh, this is what will happen. That means, you know, you, we, we drop it inside and, um, again, um, it's ultrasound guided. Majority of the time, the mom is in neraxial, um, you know, uh, uh, anesthesia. We don't put them, put them to the sleep, and we do some, uh, local anesthesia to get a little bit of the, you know, pain management after the, um, after the intervention. Again, here, you know, after the intervention, you can see that uh the shadow and the coils um nicely put in here. Therefore, you know, we can see where's the coil going um on a uh real imaging when we, um, you know, put this, you know, we do this intervention. This is the MRI after intervention, you know, as you can see, there's a completely intact brain. There was no injury after the, um, after the, the intervention. Um, on a post-operative course, uh, the mom did have a preterm premature rupture of the membrane a week after, uh, she was admitted to the hospital and then, uh, they had a, um, cesarean delivery at 35 weeks, which is a, uh, with a great of course. And the uh PH cord blood was uh completely normal. Uh, we ask that again because we do this type of the intervention late in the gestational age and usually we do it lower in the, in the abdomen and needle is there. Uh, we did a starting experiencing more, uh, preterm deliveries after this intervention, which is, you know, was expected as a general. Uh, postnatal, you know, scan, complete intact, no bleeding, and there's no brain infarct at all in this case. Um, the patient did have two rounds of the postnatal neonatal embolization at the day 2 and 9, discharged home, came back at 5 months, again, completely intact brain at the 5 months. Uh, at 5.5, uh, had another, uh, round, and, um, she's a neurodevelopment intact at this point. They So, um, at this point, we've enrolled 7 patients in the trial. We've successfully embolized 5 of them. In 2, we were not able to cross through the skull, uh, with a needle. There's definitely a learning curve in boring through the skull. Uh, and we actually had several cases before the trial started on a compassionate care basis where we just could not get through the skull until we learned the technique. We Started the procedure over at Brigham and Women's before we had fetal surgery here, and it was analogous to the fetal cardiac interventions that we've been doing there for a while. And Louise uh Wilkins Houg was doing those cases and there was a learning curve there, getting through the skull, and then when we started doing them here, and Shamiy is the fetal surgeon, there was a learning curve in getting through the skull, um, there too. But we we're technically successful in 5 cases. Based on the width of the falcine sinus, we can calculate what the expected mortality for those 5 cases would have been with standard postnatal treatment, and it was a 90% expected mortality. You can see the width of the falcine sinus. Over 80% of them would be expected to have uh parenchymal brain injuries at birth, and they'd only have about a 12% likelihood of meeting milestones. Um, so of the five, we've had two deaths. One was complete futility of the intervention. So the intervention went perfectly well, no problems. Baby. He was born, overwhelming heart failure, so sick we couldn't even get to the angio suite to do postnatal intervention. So this heart must have been sicker than, than we saw, uh, from the start. The other one was really tragic. Everything went great. The baby was doing well in the NICU and then got sepsis, uh, and died of overwhelming gram-negative bacter it was actually bacteremia with identical, um, bacteria grown from the urine. We actually did an autopsy in that case and there was no infection in the brain. So this was unfortunate septicemia. But 3 of the 5 are alive and well. Here, their ages, um, brain parenchyma is completely intact, they're all neurodevelopmentally intact. Um, in every case of the 5, there was a dramatic diminution in cardiac output on the echo, so the procedure really is achieving the goal of diminishing, uh, the cardiac output. Um, and there's a lot more we can talk about, but we want to finish on time. Um, it's obviously a big, uh, collaboration across two institutions and, and lots of, uh, groups. So, um, I'll stop there and, and take questions. The only thing I'll mention before I stop, what Shami mentioned about premature rupture of membranes has turned out to be, uh, a harbinger, and it definitely looks like this late intervention, late in the 3rd trimester, in the lower uterine segment. does cause a proclivity to early delivery. We're tweaking the protocol now to likely try to do it a little later than we've been doing it. We typically deliver the vein of galen babies at 37 to 39 weeks because we don't want them to deliver outside the hospital. So the thought is perhaps we will do the intervention at 37 weeks and then deliver just a day or two later when we would have delivered anyway. So the risk of premature rupture of membranes is not, not a big deal. Um, that's, so that's the protocol is currently being tweaked as we speak. So, I, um, I know that some of you are like wondering what he was talking about. Um, I will take responsibility for the fact that he did not, they didn't do an introduction to what is a vein of Galen malformation, etc. and that is in part because of miscommunication and the group you're about to see, which is huge, thought they had the whole hour. So I asked Darren and Shay to really uh uh uh accelerate, which they did. Um, and I'm sorry that your, your technology failed. Um, for those who are interested in this, uh, if you just go online. Uh, and, um, and, and, um, and, and find the Grey's Anatomy on this procedure. Um, there's, it's all they actually did a Grey's Anatomy and Darren went as a consultant and got to be in it, so you could tell whether, you could see which one is him, uh, holding the ultrasound probe in Grey's Anatomy, which it's the first time I didn't know Grey's Anatomy still existed, first time I've seen it in like 20 years, but, um, um, he's there, uh, uh, this, this is like, um, dramatic change. This is the first world. Darren has been thinking about this for. A decade, right? And doing this and, and, and this wasn't like, oh let's just go try it. There was an enormous amount of preparation for this. And we have a few minutes for questions. Um, can we, can we go a few more minutes? Yeah. Um, if anybody has any questions or, or comments or, or we can move on to the next case. Just a quick, uh, technical question, uh, what do you use to embolize, uh, the vein of galen? So we use a very large aneurysm coils, and, um, we calculate the packing density in advance based on experience with giant aneurysms and what the, uh, what the packing volume needed is needed to slow. To stop flow. In the case of aneurysms, you really want to completely eliminate flow. In this case, you actually don't want to close the malformation because closing the venous outlet up front could be very dangerous. So the goal is really to diminish the flow enough so the baby is not as sick after birth, um, but they are large aneurysm coils. And this all goes through an 18 gauge needle. Yeah, the, so the needle tip ends up in the torcular in the back of the head, and then a micro catheter and microwire go through into the into the venous varinx, and then we do the embolization that way. Thank you. Yeah. Darren, quick question, Shamsi as well. Uh, fetal positioning for this intervention, I mean, I presumably there may be times you have to do a version or something like that and it probably applies to other procedures you do. That, that, that's an excellent question, but again, you know, in our world that's so easy, that means, you know, we, we move it all around, uh. Uh, Mostly we want to bring it as a vertex, that means down in the pelvis that we can, the head can be more fixed because with the needle, you need to drail inside the, the head and you put too much pressure. Therefore, um, from our experience, it's, it's harder when the head is off that we call it a bridge because the head will be start moving. Um, but also the downside is anytime we know on the fetal intervention, you walk down in the pelvis because you're close to the cervix, then the, the chance of the pre and premature rupture of the membrane will be higher. Yeah. Uh, Casta, um, it's very interesting. Thank you for the talk. Um, I spent some time in Saint Christopher Hospital for Children a long time ago, and we used to see a lot of these malformations and for the health staff, the presentation usually was when you look at the neck, the neck is bulging and pulsating a lot, and that was very common, you know, to see. We also saw a couple of cases where in cardiac, uh, PD cardiology, they were ruling out a quotation of the aura because and you took a picture and there's no flow to the descending aora, so everything was going to the brain. So we, we went through a lot of learning, but for the house that when you see somebody with a baby with a neck that is pulsating a lot, just think about this. Thank you. OK, I know that was whirlwind, um, but, um, suffice it to say that is an example of what happens at Boston Children's that Change, literally changed the world. And there are other people in the world um looking and actually doing this now. Uh, um, something that people thought was crazy when I know when Darren first talked about it, and we do crazy things here, uh, do them safely. Um, And find the right partners to, to, to make it happen. So, um, extraordinary example, uh, of innovation, uh, and, and teamwork. And there's a lot of people, uh, beyond, beyond them. Uh, the next case, um, is, um, um, a whole, uh, different nature of what we do here and beyond here, uh, because the children have to get here. Uh, so David Mooney, uh, our long-standing. Um, I won't say how long, uh, trauma medical director, uh, is gonna feature this case, and it's actually a little bit of an opportunity to thank David for his decades of, um, of, uh, uh, leadership here and, and really building our, our trauma center. He's been twisting my arm for a while and giving me a date and he won't be, uh, doing this for too much longer, although he's not retiring, but we are gonna, uh, look to have, have somebody step into that role. So, so it's an opportunity for, for us to show what an incredible system that, that he has built with this enormous team, David. Thank you, Steve, and, and uh I was egging on the fetal team to go quickly, and they went super fast. Um. And I don't know how to get to slides, our slides. And so I just go next? OK, cool. So I wanna uh present something about the, the other end of pediatric practice, um, a teenager. And uh present a case that I think highlights, um, many of the aspects of care that we don't often talk about here. Um, It was difficult to limit the number of people involved cause this, uh, the patient who we're gonna talk about, uh, was cared for easily 16 teams, teams of people, not just individuals, but teams of people here, outside hospital rehab facility. And we don't, uh, we talk about, you know, the, we'll, we'll have a few of these people speak today. But one other thing that we don't talk about much here is the, the army of other people that care for patients who come here. Um, Therapists, comm center, all the NPs, vascular access Service. We have just layers and layers of people to provide care. And when I was looking back through uh Epic to try and look at the people who care for this, this child, um, it, it, it was numbered in the hundreds of people that were involved in this person's care. So the story here is that there's a, a teenager whose father likes to rebuild cars, Doctor Fisherman, and um they were driving a, an antique truck that the father was restoring and taking it out for a spin uh down a nice road, country road in New Hampshire, when the gas pedal got stuck. And uh they veered off the road into a tree. And the car, the truck was old enough where there were no seatbelts, prior to seatbelts being installed in vehicles. And to talk about this case, um, a couple of people have come down from New Hampshire today to talk to us. Um, you're gonna hear from, uh, Jennifer Ted Castle, who was at the scene along with uh Rob, the vice chair, but, um, I think Jennifer's gonna do the, the speaking. Um, Michael Paul, who's a graduate of the Brigham, who's up at Concord Hospital in New Hampshire. Um, and then here, an army of people, I'm gonna step in as the general surgery slash ophthalmologist, cause this happens to be the day of their annual meeting. So that, we'll see how that goes. Um, uh, Doctor C and Birch are gonna present the, the spine aspects, uh, Doctor Green, the face, and leaving out, again, a lot of people, um, Doctor Siegel and Coca will be presenting, uh, what, what in the heck does pain management and physitry do for a patient like this. So as you can see from the picture, that's where the scene of the accident was, it's a dirt road out in the backwoods of absolute nowhere. Sorry, um, we were told for automated crash detection, which is where your phone senses you've been in an accident, calls 911 for you, um. When we were responding, they came back and said they can't make contact with the caller. Our fire chief is actually. Sorry. Oh, I see. No worries. How do you get to the next slide though? Down button. So when we arrived on scene, this is how you find the truck, as you said, it's an older pickup truck, you know, for a newer car, you've got your seatbelts, airbags, passive restraint, crash crunk, you know, the crumple zones in the car, you're gonna stay in the seat. In an older vehicle, that's not what happens. You either go up and over the dashboard and out the windshield, or you go down and under the dashboard and through the floorboards, which is exactly what happened to this patient. And to see how mangled the cab of this was. When we arrived, he was leaning into the tree, kind of between the tire and the axle, and his dad was holding him kind of upright. He was conscious, he was groaning at us, he wasn't really speaking. You know, one of the first things we talked about in EMS is, is the scene safe for both the patient and for us? Obviously in this situation, it's not really safe for any of us to be where we are. We're down the side of an embankment. There's a pickup truck leaking fluids. Who knows if it's really stable where it is. We just need to not be where we are. So what we did initially is just got a cervical collar on him, put him on a backboard to come up the embankment, back up onto the road, where we can actually assess him. Once we got him back up to where we could actually see bleeding from the head quite extensively, he could tell us his name, he could tell us his age, he was asking for his dad, so clearly he maintained his own airway, which for us is important. We're not an RSI service, so if we need someone to come manage that airway, we need to call someone from another town to come take care of that. Um, chest wall was intact, his lungs were clear. Heart sounds, he was tachycardic, but not muffled. He still had palpable radial pulses, so we have somewhat of a blood pressure. Abdomen was initially soft, did not stay that way on the way to the hospital, um, had a really good sized laceration on the left flank, and the extremities were grossly intact, not anything. Deformed. So things we look at on the way into the hospital, how many, you know, scene safety we need to leave. We initially talked about aeromedical transport, but where we are, that's not really an option. We usually start with Hitchcock to fly. They're 25 minutes from the scene, we're 25 minutes from the hospital. We just opted to go. Um, were we gonna need anything additional for resources? There were 4 people on scene. There was our fire chief, our deputy chief, and the 2 people on the ambulance. That doesn't leave many sets of hands to do much. Um, we ended up getting one firefighter to drive, so we'd at least have 2 people in the back for him. Um, on the way into the hospital, he was on oxygen and title CO2 monitoring, cardiac monitoring, 2 IVs. Um, one of the biggest problems we had is when we were on scene with him and Dad was with him, he was fairly calm. As soon as Dad was not with him, he was not quite so calm, so it really did take one person just to be there talking to him the whole time. You know, one of the things we look for for EMS is how long does it take us to do what we do and get him to all of you. For us, for this call from the time of the tone for the first notification from the accident till we were in the doors at Concord Hospital was 38 minutes and 20 miles of that is driving. So, for us, that's pretty fast and fairly impressive to get him to you guys to actually take care of him. Yeah. Thank you, Doctor Mooney, for having me. Um, I work at Concord Hospital. It's a level 2 center. We went from a level 3 to a level 2 about 7 years ago, really just based on community needs. Um, our volume of admissions was about 30 or 400 when I first started at Concord, and you can see it's, you know, 3 to 4 times that now. Um, we take about 300 transfers in a year from other hospitals and we send about 75 people out to, um, usually Boston hospitals, um, and about half of those are pediatrics. We don't have a, a very robust pediatric, um, uh, hospital system at our hospital. I was on call that Saturday and thankfully, we got a 10-minute head start so I was able to be in the trauma bay when the patient arrived and when the patient arrived, he was obviously in extremists. Um, most concerning was we didn't have a, a, a, a latest blood pressure. It was just 50 over pelp. That 1st 5 minutes in the trauma bay is a busy place and uh a lot of things happened simultaneously. After about 1 minute of trying to get a blood pressure, um, I just moved off my usual perch and put my hand on his uh femoral pulse. He was thankfully, he was awake and he was oriented and he was responding although very abbreviated. Um, in that 1st 5 minutes, we did primary secondary. We got a chest X-ray, the lungs looked OK. We got a pelvic X-ray that looked like he had some Rami fracture, but Um, his, his ring was intact. It did not look like an open book. And then we got a, a, a fast exam that showed obvious. Blood in the pelvis On all level one traumas, we have blood in the, in the ER at the time. So as soon as he came in and we saw his tachycardia and his uh low blood pressure, we immediately started transfusing. So after the first unit of blood, that was about 4 minutes into his stay. Um, unfortunately, his pulse was getting thready and, uh, uh, that initial heart rate of 157 was actually, um, uh, the best heart rate he had. It was actually about 180, about 1 unit into the, into the transfusion. So at the completion of about 5 minutes, I made a decision to go to the operating room, um. Fortunately, we're about 3 minutes away from the operating room and we had uh a second unit hanging. By the time we got to the OR, um, the third unit was hanging. Um, induction of anesthesia, uh, uh, just after that, we had our first, um, uh, measurable blood pressure which was about 80/40, and that was after 3 whole units had gone in. Um, we made an incision at, uh, about 15, it's actually 14 minutes from arrival, which It's hard, it's hard to beat that. Um, it's hard to beat that. Um, he had massive hemoperitoneum. Uh, he had a, a ruptured spleen, and actually he had a ruptured bladder right on the, uh, the dome of the bladder that was probably bleeding as, as much as the spleen. Um, in a trauma laparotomy, we, we tamponna, tampona, tamponna, that's what we say, just get the bleeding to stop. So he was filled up with probably about 30 or 40 laps at that point in time. And actually, as soon as we had the, had the bleeding tampon knotted, he responded immediately. We were able to stop transfusions after I think he had 6 units total. Um. And we warmed him up for about 15 minutes and by the time I went to re-explore him, um, you know, his vitals, his heart rate was in the 90s, his blood pressure was in the 110s to 120s. His, um, his splenic injury required a splenectomy, went down to the hilum. Um, any sort of salvage procedure, uh, I, I don't think would have been. Successful. Um, we went ahead and repaired his bladder. We found some mesenteric bleeders that we ligated and then we ended up repairing a sigmoid, um, a traumatic sigmoid injury. Um, and at that point, um, he was stable and I called Doctor, uh, Weil who was down here and we, uh, had a conversation. Normally in a patient who's had a, a, a, a, a, a setting of shock, I will leave open for 6 to 12 hours. To have a second look. Um, and in his case, I don't really have anywhere to bring him. Uh, you know, it's, there's times when it's snowing up there and I can't get the child out. And so, you know, I have to, you know, sleep, sleep with them, managing the vent in the ICU. But in this case, you know, we, we were flying helicopters so we made the decision just to um do a temporary abdominal closure called an phthera and um uh we, we flew him, flew him down here for you guys. So again, just to highlight um what was done in the operating room at Concord Hospital. Um, they removed his spleen, fixed his bladder, um, noted that he had a non-expanded hematoma in his pelvis and fixed his colon. And, um, a little bit more blood, 8 units, so about 80% of his blood volume. Right, uh, about 80% of his blood volume was replaced, uh, in the OR there, along with some other clotting factors and some TXA. So We, these patients arrived to us, and again, we try to fill in like, what actually happens in that big black box we call outside hospital or, you know, scene, and uh we just see them when they get here. Well, there were a number of things that happened before the patient arrived here. One was communications with one of our surgeons. Thank you, Brent. Um, also, communication center got involved. Uh, Concord Hospital activated the helicopter crew well before the patient was ready to be sent, so that the crew would be there and ready, so there wouldn't be a long pause. It just, thing after thing happened, uh, the way it's supposed to happen. Uh, the child arrives, and this is some picture I pulled off the web. No one took a picture of the kid's belly in the, uh, IC ICU arrival. One of the unique things we have here is the child was greeted by a surgical attending who was staffing the ICU. Um, Doctor Damiri, and, uh, who's at his bedside on arrival. The kid arrived, heart rate was 110, blood pressure was also 110, but their hematocrit was 41. They were fully resuscitated, platelets count was fine. And their lactate was, they're a little bit acidotic, but not so bad after what the child had been through. Basically, the kid arrived warm, resuscitated, ready for us to patch up what was left. And the injury, so then, and they hadn't had a CAT scan. They went to the OR so quickly, the rest of their body had not been evaluated just to save their life. They just took them to the OR. So then we started rolling out the evaluation, scanning different parts of the body, finding different injuries, things that were identified. Um, the left eye, um, was had an open injury with the iris poking out through a hole in the eye. Um, some amazing, uh, pelvic fractures were identified, uh, which we'll hear about. Um, some run of the mill, you know, quote-unquote, uh, orthopedic injuries, metacarpal fractures, heel fracture, cuboid avulsion fracture, and, uh, knee tibial spine avulsion fractures, which, uh, we're not gonna discuss much, but Doctor Livingston from, um, orthopedics got involved with ortho trauma to repair those. Uh, some pretty impressive facial fractures, and also a C1 to T3 spinous ligamentous injury, and a fairly boring grade 2 kidney injury. They get tossed into the mix. But a number of, uh, basically a whole body, uh, set of injuries were involved, were identified on evaluation here. Well, uh, my new career as an ophthalmologist. Um, so, basically, the, the first step in the care of the child is once they're resuscitated is we find all this stuff, well, then, You gotta pick an order. Like, what do you fix first? So, Concord Hospital did all the, stop the bleeding, which is obviously number one. Then what comes first? Well, the eye injury first up needs, you know, gallons of antibiotics cause it's an open globe. And then that needs to be fixed quickly. Uh, well, in the, in the morning after all these, you know, other injuries are identified. Things are laid out according to, you know, some sort of an order. And then it's determined that then the following day, the child goes to the operating with ophthalmology and general surgery. And so I think I went first, just to make sure there's always that fear if there's some anesthetic issue or some instability, get the important one done first before, cause we could do the belly the following day if need be. The, um, so there's a thing, turns out there's a thing called the limbus. And And it's in your eye And it's that dark rim we all have on the outer part of our iris. It's uh, and it, the term limbus means like the, the edge, or the, or the margin. And I guess limbo as a Catholic limbo must mean like you're kind of not going to heaven and not going to hell. Like you're somewhere on that edge in between the two. So, his limbus was torn, they take him to the OR they You know, with a, a very carefully examine his eye, they see a tear in his sclera, uh, a tear in his cornea, and right through the limbus. And as they're probing away, and they actually took sterile, they sterilized air with this filter, and they filled the anterior chambers, the thing underneath the cornea, and they fill that full of air. They get it to poof out. And as they can see better, which is, looks really cool. And, um, but anyway, so then they found this, this tear in that area. Um, and when they're poking around, they feel something hard in there. And it turns out a piece of glass had cut his eye. And so they pull out this piece of glass, and then they repaired his sclera, repaired his cornea, um, did some other magic, and then closed the cuts. And then that was basically a first eye, and that red line is sort of where the tear was. That's a picture of his eye, but it's kind of blurry, but that's, you can kind of see something going on immediately. Then that got done, general surgery got involved, and Doctor Modi um did the procedure. Basically, uh, took the big foamy dressing pack thing off, removed the pad, the foamy stuff from his belly, looked around. Bladder looked great. Spleen area looked fine. No bleeding. The colon was well approximated. Basically it was all done. There's no leaking from anything, no bleeding from anything, no pee leaking from his bladder. Washed it out, closed the belly. So again, no, nothing more was needed from that point of view. And then started to walk down the body on the next injury up. To sell that it needed 2 people. We actually always stand and walk together. Yeah, we do everything together. We'll finish each other's sentences, every other word, so that's why we need so much time. Um, uh, no, so we'll talk about spinal pelvic dissociation, which is the injury he had. It is orthopedics, so we have to make the words simple. So spinal pelvic dissociation, the spine becomes separated from the pelvis. It's a very unstable injury, uh, in the lumbosacral spine with through a fracture, uh, separates itself from the pelvis. Really high energy trauma, uh, classically a fall from a substantial height, uh, a little bit more than half the time in a big, uh, systematic review meta-analysis, but also see them in high speed, uh, MVCs, about 1/3 of the time. Uh, and then a very high associated rate with neurologic injuries with about 70% having some type of a neurologic, uh, injury, a little bit more than a third of the time, that's cauda equina. Uh, and then about a 65% neuroimprovement rate, uh, again, with the kind of big multi-center studies that we have. Um, there's a couple of different classification systems. The one on the right is, uh, the one that we use most commonly, which is really based off of the direction and amount of displacement. And so this highlighted one on the left is the classification that, uh, our patient had. And so the sacrum, uh, went through its fractured segment is displaced into flexion and then posteriorly displaced, and then in our case, also shortened from an axial load, uh, which then obviously compromises the canal that we'll talk about with Doctor C in just a second. Uh, there's a couple of different ways of treating this. There's the one on the far left, which is the kind of pelvic trauma, pelvic ring bias, maybe, um, uh, that's done predominantly by trauma surgeons, uh, where the, there's a direct reduction that's performed, and then SI screws going, uh, across the pelvis through the sacrum, uh, are used. There's not a lot of rotational stability with these, and so people have then shifted more towards a spine-based fixation where you almost bridge, uh, the sacrum. And you put pedicle screws into the lumbar spine, and then put screws into the pelvis, and then attach those with rods, and that stabilizes the relationship of the lumbar spine to the pelvis. People are shifting more towards the image on the far right, which you can also see here, which is triangular fixation, which is essentially just a combination of the two, where you also put in your uh Uh, pedicle screws into the lumbar spine and then screws into the pelvis, but back that up also with an SI screw. We were unable to do that, just there wasn't a safe corridor to be able to put an SI screw, so we used the spine biased technique, uh, where we put pedicle screws into the lumbar spine and then stabilized it to the pelvis. So one of the questions that uh was presented before was what order do things happen in and when can they actually happen safely. Uh, so not to forget about some of the other injuries aside from the lower pelvic injury that did require uh uh instrumentation and uh surgical approach. He also did have a cervical spine injury. And we do have good data on this, uh, so Fellings, uh, did a prospective trial in about 300 patients back, published back in 2012, showing that there is, uh, definitive time frames where you have improvement in function. So people who had um surgical decompression of a cervical spinal cord injury within the 1st 24 hours had twice as likely uh improvement in their uh Asia grade compared to people who were operated 24 hours or after the 24 hour mark. So time does matter and kind of our, our goal is really within that next day, right? Uh, this child did not ultimately need cervical spine, um, surgery, but The lower spinal cord and ultimately the sacral nerve roots, uh, did require evaluation and surgical management and decompression and so there is also some data, although it's not quite as robust, um, and, uh, mostly it's based on a large, uh, national registry of about 4000 patients as well as subsequent, um, retrospective studies and smaller cohorts of about 300 patients and it's, uh, interestingly, Somewhat similar, they also use 24 hour but then also 48 hour and then 72 hour cutoffs and compare the function in those groups and there's one additional distinction and that's whether or not there's urinary retention involved in that patient's uh neurologic compromise. Unfortunately, in this case, we had no idea because they had a bladder repair, so we're a little bit limited, but I would then presume that this person does have urinary retention and Interestingly enough, the data then suggests actually that there is no benefit to earlier surgical repair, but I think the caveat in interpreting the data is that that's because they just had such worse outcomes. Uh, it's somewhere in the order of 40 to 50% bad outcomes and lack of recovery. So then we're still kind of pushed then to move as early as possible. So as soon as the abdomen was closed and the patient was able to be position prone, we then proceeded with surgical decompression of the cauda quia in this case, and that also is. A kind of dose dependent uh response, so people who had it less than 24 hours, uh had uh 90% recovery of bladder function, uh, in the incompletes meaning they didn't quite have complete urinary retention. If you add another 24 hours that drops down to 85% and then if you go beyond 3 days. Then you're getting, um, then you're getting into, uh, I think it was around 60%, so you see that dose dependence, so it's really convincing that the biology there is real and that the data there is real. Um, and then ultimately what still keeps, uh, wakes me up at night, uh, every few weeks, uh, for this kid is whether or not he's gonna recover his function and whether we had adequate decompression. I think, uh, ultimately, um, we'll have some additional discussion on this, so, uh, suffice to say that, uh, several years later when you ask patients, uh, what is or is not, um, important and what is or is not bothering them, pain is actually still something that's very important to them and that's still Uh, requires a lot of work and that they struggle with. Urinary retention is kind of mixed, uh, in terms of how likely it is to recover and be functional. Um, luckily, uh, bladder, uh, bowel function is something that actually recovers fairly well and most people don't really have, uh, disabling bowel dysfunction. Um, move on. OK, great. Uh, so I get to talk a little bit about the facial trauma our patients sustained. Um, oftentimes, families are concerned about this a lot, um, but the reality is it comes a little lower on the totem pole of, you know, the care that's already been discussed and provided. Um, but our patient did have several, uh, facial injuries that need Need to be addressed. He had several abrasions, lacerations, bilateral41 and the 42 fractures. If you look down at the bottom there, you'll see what those fracture patterns look like, um, and then had some minimally displaced orbital floor fractures, uh, some septal, nasal septal bone fractures, and then some, uh, bilateral mandibular fractures were probably the most substantial. And this is him on presentation, and so they're often very edematous, very swollen. A lot of ecchymosis and so oftentimes it's hard for us to really determine uh what extent of his injuries are gonna be clinically noticeable later on. So we have to wait for the swelling to subside. This is just his CT scan of the face. This was the initial one done, uh, and there are several fractures on the top right. Uh, you can see the displacement of his mandible fracture on the lower right. As you move across, these are all axial cuts in the bony window on the left side. There's a ramus or subcondylar fracture involving the mandible. The second one over, we start seeing some fractures along the Reform plates, uh, and then further over, we start seeing some of the maxillary, uh, wall fractures that are incorporated into that Leo fracture. Further down, again, these are just showing some minor dis, uh, minor fractures involving the orbital floors, uh, the LaFort fractures kind of coming across. So, no, none of the fractures were really that badly displaced, but they, he had several of them. And so as we start considering how are we gonna manage these fractures, there are a few things that we always like to think about the airway, is it stable? Um, do these fractures, uh, pose any potential risks to the airway? Uh, also, in order to fix some of these mandible fractures, we need to have access to the oral cavity and so, are we able to successfully intubate the patient nasally or would we have to consider something like a trach or or some mental intubation? Uh, fortunately, Mike Hernandez was our anesthesiologist that, that, this day and was able to do a nasal intubation despite the La4 fractures, so we were able to get away with it. Uh, next is, do we have access to our fractures? This patient had a cervical spine collar, had unstable C-spine, uh, and so we had very limited access specifically to the left mandible fracture, and so we had to take into account, uh, what are some ways in which we can manage these fractures with the access that we have. Um, Maocclusion is just something that we always talk about. It's how the teeth are gonna fit together, can we get him back to what his pre-morbid bite was, and we felt that we could. These are not his pictures, uh, but I did wanna put these, uh, up because, uh, we didn't know whether this patient was gonna have to get MRI's and stuff, so the other thing that we always have to think about is what type of Um, imaging there, he's gonna eventually need to get, and if he's gonna need to get MRI's, we don't wanna put a lot of stainless steel in his mouth and then have to go and remove it the next day. And so the lower archbars are some titanium archbars that we actually use for our patient. Uh, in order to help stabilize the bite. So he had open reduction internal fixation of his Lafort fracture on the right side. He had a plates placed in the right mandibular fracture as well as had a wisdom tooth removed, and then we did a closed reduction technique using those, uh, orange arch bars that I showed you, uh, in order to treat the left fracture. And so, I'm gonna skip through this one cause it's just some pretty pictures showing some of the hardware, but I think you can see it a little bit better here. Uh, and so, basically, we put on some plates, uh, for the right La4 fracture. The left, uh, La4 fracture was quite high and extended up into the orbital rim and it was actually quite stable once we exposed it, so we didn't feel like we needed to fixate it. Uh, especially because we knew we were gonna keep him into intermaxillary fixation with some rubber bands. And so, he got a plate for the right mandibular fracture, and then we were able to interdigitate his teeth back to the way they were before, and then using a lot of heavy elastics, we're able to fixate his teeth back together. His left subcondylar fracture on the mandible, is just going to remodel over time, and we allowed him to be clamped down for 4 weeks, and then he's transitioned to lighter and lighter elastics and his bite looks excellent today, uh, and he'll get some post-operative imaging, uh, actually pretty soon. And the nice thing about this is all this hardware is MRI compatible, so if you did need to have any type of um MRI done, nothing would need to be removed. We also travel as a pack, yeah. Um, so I'm gonna talk a little bit about the, um, how we treat pain in, uh, patients who have spinal cord injury and don't worry, I'm not gonna go into the details of this slide. Um, but there's a, there's, uh, many things that we consider. We, we, we really need to understand the path of physiology and the time course of the injury when we're thinking about treating pain. So, when I met this patient, he was in the acute to subacute phase of spinal cord injury. Um, and this, uh, this phase is characterized by inflammation, cytotoxicity, which is mediated by glutamate receptors, and then progressive axonal injury and cell cellular necrosis. So, our goal was to be very aggressive in treating his pain because we want to treat his acute pain, so we don't have to, or we try to minimize that transition from the acute to chronic pain. So, in the acute injury phase, the patient has both, um, Has both no susceptive pain and neuropathic pain. So, the nociceptive pain is the somatic pain, the visceral pain, um, and that is, uh, we treat that aggressively with opiates, NSAIDs, acetaminophen, steroids. Um, but when the, when the injury progresses to the chronic phase, um, most of his nociceptive pain, um, will have resolved, hopefully. Um, but then, as we talked about, there's a, there's a great chance of developing chronic pain and studies have shown that in 70 to 80%, Um, of these patients, they'll develop chronic neuropathic pain after an acute spinal cord injury. Um, so the Neuroplasticity is crucial in the transition from acute to chronic pain, and we have many tools to address neuromodulation. Um, one of the, uh, to treat neuropathic pain, we commonly use antidepressants or anti-seizure medications, but the role of these medications in the pediatric population has really been extrapolated from adult studies. Here, I just list one study that is a meta-analysis and the objective was to compare the efficacy and safety of um pregabalin, gabapentin, carbamazepine, um, and amitriptyline in treating spinal cord injuries related, um, Related neuro uh neuropathic pain. So, they looked at 8 randomized trials, um, 8 randomized controlled trials. They had about 600 participants and it showed a good safety record and a good side effect profile for the long-term use of, of these medications. Um, I think that gabapentin showed the highest pain relief, but it was also a good co-pharmacologic agent when we're thinking about anxiety and depression in these patients. Um, Some of the non-pharmacologic tools that we have are the most important in treating pain in these patients. Um, we, we emphasize trauma-focused therapy and, you know, that's around the ax, can be ACT, DBT. Cognitive behavioral therapy has the greatest evidence for it in treating, um, many of these, uh, in treating many of these symptoms that the patient experiences. Um, this is one. Um, a study that showed that was a multidisciplinary cognitive program, cognitive behavioral program that was individualized, uh, to these patients, and it showed, uh, they showed a good reduction in, um, pain intensity, but also an improvement in pain coping strategies. Um, there are many areas of ongoing research, so spinal cord stimulation for, for the neuropathic pain, uh, TMS, and visual illusion. So what is rehab medicine? So we're physicians who care for patients with new onset or chronic disabilities and a change in function. And so, in this particular patient, we actually met him when he was in the ICU. So accident had just happened, had all of these fractures, had all of this change, had weakness in his legs, also had a foley and wasn't peeing, and so we come in and kind of work. On the inpatient side, PT OT feeding, communication, working on pain, evaluating disposition, thinking about other things related to disability. So spasticity, we work really closely on pain, spinal cord injury specifically, neurogenic bowel, bladder, sleep, agitation, PSH. We also talk about delirium and other things that can help it happen in the ICU. And so for this patient specifically, he had a lower motor neuron spinal cord injury. It was incomplete, so he had some function below that level. He also had neurogenic bowel, neurogenic bladder. He had neuropathic pain. Um, and so this patient went to acute comprehensive inpatient rehab, and he was there for a short amount of time. He actually ended up coming back, um, to Children's for a time and then went back to rehab. And so for this kid, when he originally went to rehab, he was m assist for stand, for standing and transfers. He was supervision, walk. with a rolling walker, he was modestest using steps. He was modestest with all of his bathing and dressing and ADLs. By the time he left rehab, he was independent with all of his transfers, independent in for mobility in the home, me assist for walking outside of the house and with crutches because that was difficult. Um, and he was independent with all of his ADL. We made huge gains at rehab during those two stays. Um, bowel and bladder function. He had a Foley in when he came into rehab. When he left, he was independent in caffeine, and he was timing his bowels, so he was independent with his bowel program. He was actually seen in clinic most recently and his caffeine need has decreased. And so he's not actually needing to cath quite as frequently as his was, and we are seeing some return of that bladder function and he's actually able to control some of his bladder. Um, and then the other interesting thing with gabapentin specifically is, so there are a couple of studies that look at spinal cord injury patients, and patients who start gabapentin early might have some neuro nerve recovery, motor recovery. And so we actually see that looking back at studies, patients who have started on gabapentin have nerve recovery. And so there's a mouse model that actually shows Some nerve recovery in the, in the spinal cord that can lead to some meaningful gains. What that really means, we don't know, but as we, as we talked about, the side effect profile is really low. So a lot of times, even if patients aren't conscious or talking to us, but they have a spinal cord injury, if they're not even having pain, we'll start gabapentin early, and that's a medication that we use just to kind of hope that we can do something from a nerve recovery perspective. So how's he doing now? And um, well, he's home, uh, back in school. Um, he has pain if he sits for a long time. He's got a little bit of numbness in his leg. He's walking around. Uh, he's doing his catheterization, just follow up with urology, and it turns out his bladder, he had an EMG of his bladder and it shows, uh, it's actually functioning fairly well. Shockingly to me, his vision's 20/20 in his right eye and 2025 in his left. Uh, he does have the one, it's a suture that's kind of itchy on his eye, uh, but his, his, uh, open globe is healed, he's back to school. He needs some hardware, orthopedics is doing something with hardware, and he wants to wait until after Boy Scout camp this summer to, uh, have his hardware adjusted or redone or something like that. And um, So for him, um, terrible injuries. Um, all kinds of things, super specialists down here that have been like, you know, slowly chipping away, fixing the things, the various things that were broken. Um, first thing for me about this, one of the reasons I wanted, I'm really happy to present this kid, the amazing work that we do here, repairing things that don't seem repairable, having this kid return to his normal life. But it all starts with the fact that Jen and her, her squad saved his life at the scene. Michael Paul and his crew saved his life at Concord, that allowed him to get here in the first place, cause otherwise, he would not have made it to get all this fine care. And that's what I mean by systems approach to care. Uh, thank you very much. And we actually shockingly had 8 people present in, uh, beautiful people, thank you very much. Sorry, the videos didn't work, but the, um, in a, in a historic short time. Well, well, thanks to all of you, and, and for all the many, many, many people who helped care for this child that obviously, uh David could not include, uh, even if he had 4 hours. Um, and, um, it's remarkable how much you got into such trauma time. It is truly remarkable that you got a patient from the scene. To the hospital, to the operating room. Everybody hear this? Boston Children's 14 minutes from arrival in the emergency room till they're on the table and 3 units of blood were already in, right? Well, uh, in, in just incredible right at an outside hospital like, so just remember how, how, how, um, high the standard is, um, uh, but all the incredible things that were done here could have been here and that's what the system is all about. Um, we are out of time, so it's not good time for questions, but, um. Uh, I, I think it's a nice contrast of like, um, two cases of the like super esoteric presentation of a completely innovative new treatment in the fetus of an essentially a uniformly fatal or devastating, almost uniformly fatal devastating condition, um, done in a in a high tech, uh, on a condition that most people in this room had never heard of, um, and then. Um, something like, um, uh, essentially fatal injury, um, from those seatbelts, um, that, uh, is saved because of incredible teamwork. Um, uh, so, um, this is, um, Boston Children's and, and Beyond. And thank you guys for getting up so early to come down and join us in Boston.
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