Super Bowl Grand Rounds (10/2/2024)

It is. OK. Turn it off. Just wanna make sure the Zoom is working. Uh, welcome to Super Bowl Surgical Plus grand rounds. Um, this is something that we started doing a couple of years ago, a twice a year event, which we've had a really, uh, excellent, uh, response and feedback from, uh, and the idea here is for us to share across our, I hate the word silos, but, um, our, our, our sort of separate specialties where each of our Uh, groups is so deep and so broad that sometimes we consider another specialty someone who actually is in the same department. Uh, and this is an opportunity for us to hear from each other across departments, across interests, and across specialties. Um, not only to see wild cases and see what, what sort of what's exciting, but to get ideas about how to cross-fertilize. Most of the cases that we've had presented so far have been, uh, uh, actually within themselves cross-disciplinary, although that's not a necessary requirement. Uh, we like to have that, makes for lots of chairs on the, uh, on the, on the stage here. Uh, unlike the typical academic round rounds where we spend about 6 minutes going through people's CVs and talking about their history and all the great accomplishments, we're not gonna do that today cause we want you to hear from these people. Uh, so, uh, there won't be any formal introductions, um, and the groups will sort of quasi-introduce themselves to move along. We have two cases for today. We're gonna ask them to stay on time. So, I'm gonna off the podium. You're gonna go first. So, Megan Anderson is gonna introduce her team, uh, and, and, and lead the first case. Thanks for the invitation. I really appreciate the, uh, it's a real honor to be able to present in front of all you guys, and I'll have everybody on the team just introduce themselves as they come up. Um, so we're gonna talk about hemipelveectomy today. Um, I'm an orthopedic tumor surgeon here. Uh, this patient initially came to us when she was 12 back in 2012 with a large Ewing sarcoma involving her left ileum. She had normal systemic chemotherapy and her, her local control which can either be surgery or radiation for Ewings, she decided on radiation because it was pretty close to her acetabulum and the thought was that without doing, with the, the surgery would have decreased function um compared to radiation. Um, that was OK until 2016 when the Ewings relapsed in the left ileum. But unusually, it, she didn't have any relapse anywhere else. And so she went back on to some chemotherapy, and we then removed the ileum surgically and uh kept the leg, on an internal hemelveectomy. did great again until what brings us to the, the current or the recent issues is that, unfortunately, she developed a radiation-associated osteosarcoma in the residual parts of her pelvis, uh, the pubis and the ischium, and into the soft tissues. And so, that's the case that we're gonna be talking about today. Here's her initial presentation and you can see how very large her Ewing's is, very, very typical. And then with chemotherapy, you can see how much it really reduces in size because it's so cellular, all those cells die away. After radiation, this is sort of how it looks, and it sort of stays that way until the 2016 relapse of her Ewings when there was a marked change and she had new tumor there. With the, with the chemotherapy, uh, that reduced in size slightly but not as much as the earlier one. And then this is what we, what I was able to, to remove with her first internal hemelvectomy. And uh she did well until, um, and the bone, uh the bone shifted, of course, because you're missing part of your ileum. Uh, this is the appearance of that part of the bone that's tilted now. And then, this is where the osteosarcoma developed. You can see this bone is all full of tumor and the, then the soft tissue extension of the tumor and how close it is to very critical structures. So that's what we're gonna talk about. Leading up to the surgery, uh, she is still has non-metastatic disease, and so the goal is cure. She's very limited in what chemotherapy she can get because she's already had so much chemotherapy. So the, and then the only option for local control is surgery. Osteosarcoma does not respond to, to radiation and she's already had radiation. So, in weighing that amputation versus limb salvage, which the amputation we call external hemelvectomy and limb salvage we call internal hemelvectomy, there were a lot of surgical, surgical challenges that I think weighed in on our recommendation to her and her thinking about it. The tumor was really, really large, involved the bone and the soft tissue. Its location was extremely challenging because it involved in the critical large vessels of the pelvis, the nerves, the nerve roots, femoral sciatic, the bladder, the uterus, the vagina, and the rectosigmoid colon. And then, osteosarcoma, unlike Ewing's, has a lunch, a lot of matrix in it, so it doesn't reduce in size with chemotherapy, even with um a good response. So that's a problem. She also had pre-existing massive lymphedema of that leg, massive, and a, a DVT. Uh, the vessels were largely, basically encased in the superficial part of the tumor. And then the biggest challenge and remains the biggest challenge is the scarring from her prior surgery and radiation, making it very challenging and very vascularized to be able to remove this tumor. It's really important. Uh, so with all that, she decided preemptively that she wanted to have the external hemmielvectomy. Um, she, it's really important for these kids to get longitudinal psychosocial support, and she and her parents both have that and have continued with that. I wanted to just highlight our periooperative support group called Copes Sarcoma, and it's a multidisciplinary group where we focus on the stress and the pain of surgery for sarcoma patients, and it's really, really been helpful for these kids. And all of us are really important, but I honestly think one of the biggest and most important thing is that patient meeting. We have all of our patients who are facing a big surgery like this meet another patient and family who have gone through it. And just to get that connection and to talk about like, where do you get a prosthetic made when you've had an external hemelvectomy? Do you mostly use your crutches or do you use your prosthetic? All those questions that are just really difficult come from the patient meeting. Uh, Doctor Cypress is on paternal uh maternal leave, so I am going to present her slide. Um, the issue with the, uh, the vaginal stenosis is that there was an obstruction and so there was this hematomerocopus that did not result in a withdrawal bleed with with hormone replacement therapy. So, the sur the surgical procedure started with um Doctor Cyrus doing an, an, an evaluation under anesthesia and the, there was really just this scarring of all the vaginal tissues, the cervix and the uterus that prevented this, it created this blockade to any of the tissue passing. So, she wasn't, you know, resection of, she notes that resection of a radiated vaginal obstruction is a humongous risk of injury and not part of that, of this tumor resection surgery. So the, the plan was basically to suppress the menses and then later on, have her think about reconstruction or, or vaginal dilatation at a at a later time. So, I'm gonna turn over to Brent now. Morning. For those of you who don't know, I'm Brent Weil. I'm one of the pediatric general surgeons here. I do a, uh, a large, uh, portion of our group's, uh, surgical oncology. Uh, Megan and I work together, uh, frequently for cases, uh, like this patient's. Um, as she described already, this was a particularly challenging case, uh, given the prior therapy she had received. Uh, just for some perspective for, um, uh, uh, radiotherapy as primary local control for Ewing sarcoma. Uh, we're talking very high dose, uh, probably the highest dose that we really give, uh, any pediatric patient, uh, for local control. Uh, that combined with, uh, prior operation in the same field, uh, we basically have to go into an operation like this expecting the worst, uh, and prepare accordingly. Uh, so, uh, we had a few, um, sort of goals, uh, with, with the operation, as, uh, Doctor Anderson, um, indicated, uh, uh, best we could tell from her workup, this appeared to be, uh, a localized, uh, uh, secondary malignancy. Um, so cure is, uh, uh, difficult, but at least in the cards, so it has to be, uh, uh, a part of the goals, uh, for us. Um. We also knew that, uh, given the, um, uh, radiation damage to the tissues, uh, given the DVT, given all of those challenges, uh, that, uh, needing to optimize to any tissue coverage and any soft tissue flap that we would create, uh, that would be very critical. Um, so we, we have the goal of, uh, uh, preserving the appropriate vascular supply, uh, in this case, which would be the left internal iliac, uh, vessels, which, uh, Uh, generally can be ligated, uh, if necessary, but again, as the primary blood supply for the anticipated flap, uh, we did, uh, uh, want to make this, uh, a goal of the resection. Um, and just from the still shot that you saw and, and, uh, just, uh, an appreciation of the general anatomy, uh, we, we, uh, just anticipated that all of the, uh, pelvic organs in that area would come into play from the, the prior operation and, uh, uh, prior radiotherapy. Um, when we do these cases, uh, it's actually quite straightforward in most cases to separate all of these things, meaning the bladder, uh, the uterus, and females, the ureter, the blood vessels, um, because you can get into a very nice, uh, retroperitoneal space where all of the peritoneal contents just, uh, Uh, quite literally sweep away or fall away. We, we generally have these patients in a, a lateral positioning, so just gravity kind of makes it all fall away as you can get into that space kind of bluntly. And so it's actually a very nice, uh, very straightforward dissection, uh, most of the time, and, and it's rarely a problem, uh, keeping all of those important things safe and out of harm's way. Um, but we anticipated that would not be the case, uh, given, given this patient's prior therapy, and indeed, uh, it was far from it. Um, so, so, uh, our, our goals were to save all of these critical structures and separate them from the tumor, uh, and I would say that proved to be the, uh, among the more challenging parts of the operation. Um, we were able to identify, uh, uh, the ureter, um, and it was, uh, very challenging to separate this from sort of the scar overlying the primary tumor. Um, we were able to take that down to the bladder, but in order to have sufficient, uh, uh, kind of space away from the eventual surgical, uh, specimen, Uh, that required, uh, a complete mobilization of the bladder, where, where there were really no identifiable planes, and we, uh, and, and the portions had to essentially do an intramuscular, uh, kind of dissection to get the bladder off of the specimen. We found the sigmoid was firmly adherent to the surface of the sort of scar overlying the tumor as well. Um, so this was challenging, uh, to remove, uh, and actually required a, a relatively straightforward repair of the anti-mesenteric surface of the, uh, uh, sigmoid, uh, as well. Um, all of this, uh, took a very long time, uh, and as you'll hear, uh, in, uh, uh, uh, a little bit, uh, from Doctor Butler that A lot of time and a lot of scar equals a lot of blood loss. Um, so very, very, uh, uh, challenging, uh, to, to accomplish all this. Um, At the conclusion of the kind of mobilization of everything, we were able to identify the external iliac vessels, uh, ligate those, and save the internal iliac vessels as we hoped to do, so that was, uh, good. Uh, we were able to peel everything off of the, the scar. Um, but that was very challenging, as I noted. Um, I'm very fortunate that, uh, Doctor Thacker, who you'll hear from shortly as well, um, was, uh, on call and not only came and helped with the bladder mobilization and, uh, repair of a muscular portion of the bladder, um, but really took on the long-term care and, uh, subsequent issues that developed from that. Uh, uh, so, uh, a really, uh, uh, a nice thing about this place that we have, uh, uh, uh, people who will take on these challenges. Um, and, um, as Megan listed here, I don't know if you wanna say, uh, anything more, Megan, um, but after we were able to get this exposure, uh, after a long day and a lot of blood loss, we were able to accomplish the external hemelbectomy, um, as you, as you see there. OK. Um, Carolyn Butler, I'm gonna talk about some of the anesthetic considerations for this case. Um, so we had two priorities from an anesthetic planning perspective. The first was pain management. This kind of procedure puts patients at risk for severe postoperative pain, and this is a patient who had long-standing chronic pain from her malignancy. So we really wanted to optimize pain control. We worked with Doctor Bobby Riley, who's here with the COP team to create a multimodal intraoperative strategy, including a um nerve catheter for regional anesthesia, um, neuraxial anesthesia with an epidural placed by the interventional pain team under fluoroscopic guidance because she had a history of spinal fusion. And, um, uh, Opioid sparing agents such as a ketamine infusion and methadone that could be consi uh continued after the procedure. Um, we did have a long conversation about in the setting of possible large blood loss, coagulopathy, the risk benefits of the epidural, and after Um, meeting with the patient, we elected to place that. Um, and then, as, uh, Doctor Weil was describing, you know, we knew with the radiation and the location of the tumor, there was a risk for hemodynamic instability, um, and large blood volume, uh, needs. So, we had multiple large bore peripheral IVs and an arterial line for patients who might have, um, The need for lots of transfusion, it's important to consider blood conservation strategies such as cell salvage and uh anti-fibronolytics such as TXA. Unfortunately, because of patient uh patient concerns, we weren't able to do this because of the hematology and oncologic considerations. So, as mentioned, the intraoperative course was notable for significant uh blood loss during a tumor resection and bone resection prior to amputation of the limb. During this time, there was excellent and clear communication by the surgery team. They were really great about letting us know, OK, this is gonna be a period of higher blood loss, and even holding pressure if we needed to stabilize the patient and get more blood products in the operating room. I had additional anesthesia support, including multiple anesthesia attendings. Um, the patient remained hemodynamically stable despite this blood loss. Uh, we did end up activating the massive transfusion protocol. This is a clear and efficient way to get the blood bank to send you a fixed ratio of blood products. Um, so in this situation, it was really helpful to just get blood in the room faster. And I'm not gonna talk about this, but I wanted to just show that there's an official massive hemorrhage and massive transfusion management clinical pathway that's created by Doctor Susan Guy, who's gonna talk later. Um, but this pathway is a really valuable resource for anyone managing a patient with clinically significant bleeding. Um, the goals during this event are avoiding hypotension, avoiding, um, hypovolemia, and, um, maintaining adequate, uh, tissue for fusion and avoiding coagulopathy. So, here's the total number of blood products that our patient received. Um, despite periods of rapid blood loss, she remained stable on an intermittent phenylephrine infusion. Um, and she had good signs of end organ perfusion, such as good urine output when we were able to measure it, and lactate. Um, and more importantly, her ventilation remains stable despite receiving a pretty significant volume of crystalloid and colloid. She was taken to the ICU and extubated on post-op day one. Uh, good morning. My name is Hunt and I'm a urologist. As Doctor Anderson and Doctor Wang mentioned, this was an extraordinarily difficult case. And around 8 or 9 p.m. I got a phone call saying that there was gross hematuria in the Foley catheter, which is very common for a consult for us. Um, so, I scrubbed in and I had the opportunity to help control the severe pelvic bleeding that the patient was suffering from. But more importantly, able to inspect that the ureter was completely intact despite the difficult dissection. And as Doctor Weil had mentioned that the only surgical plan that was allowed for this was actually through the bladder. And thankfully, the bladder mucosa was also uh intact. So, this was repaired, uh, primarily, and whether we knew it or not at the time, uh, based on her history of radiation, the therapy she had received, her prior surgery, and this massive, massive resection that she had. The bladder defect that she suffered from, along with the um defect in her bladder neck and her pelvic musculature, would later be the source of her urinary incontinence, and I'll come back to that in just a moment. I'm Amir Tina. I'm one of the plastic surgeons. Um, thanks for the opportunity to participate in this case. Um, after, uh, Doctor Wil and Doctor Anderson, the rest of the team were done, my job was actually pretty easy. Uh, we mobilized the gluteus maximus and the fascia and performed a skin closure, repairing it to the abdominal wall and, um, remaining, uh, pelvic floor. Um, but I also want to highlight two things that, two themes that seem to keep coming up, which is that this was a very difficult case, and in our, our case, the patient was high risk for wound complications because of the scarring, the XRT and the extremely difficult location of the wound. But the second theme, which I think really um highlights the importance of good communicate is, is good communication, is that the, the, the surgical team was able to communicate well and Um, Doctor Anderson, Doctor Wild got me involved early so we could help design the incisions to maximize healthy soft tissue before starting. And I think that was really important. Um, and then subsequently, when we had excess tissue, we were able to remove the tissue that was less healthy because it was more irradiated. And secondly, To communicate the importance of preserving the internal iliac vessels, which did take a lot of extra time to do, uh, but was super important in terms of enhancing the recovery of this patient. So, thank you. OK. So, as mentioned, as mentioned, urinary incontinence was a major issue postoperatively for this patient. In the short term, despite having a Foley catheter in place, the patient was dribbling urine down her leg, and it was very difficult for us to not only have any sort of genital hygiene for the patient, but in order to protect Doctor Tagena's flap, this became a major problem. Uh, this was made much worse because once she started adjuvant chemotherapy with etoposide and methotrexate, hyperhydration is required. So, she was creating liters of urine and completely unable to be managed. So, with the help of Sue Hamilton, we started to get a little bit creative and use the Purewick. External catheter. This is a catheter that's kind of affixed to the genitalia, attached to a wall suction, and this will wick away urine. We don't actually have any of this equipment here. So, Sue spent a significant amount of time finding this for us. And even though it was meant for just one patient at Boston Children's Hospital, it was completely worth it. Long term, about a year later, her urinary incontinence still persisted. And so, at this time, we had to really be um uh sure about what the diagnosis was in terms of why she was having this urinary incontinence. It's the only institution in the nation to have the opportunity to do external sphincter EMG. We were able to determine through urodynamics that it was her external sphincter and the pelvic floor musculature that was completely denervated, and this was the cause of her incontinence. You can see on the tracing here that her bladder capacity and her bladder pressure was completely normal otherwise. On cystoscopy, you can see on the bottom left image here that her bladder neck and urethra was completely wide open, and in order to treat this condition in a minimally invasive fashion and avoid major surgery in a radiated field again, I injected a bulking agent, which uh increases the resistance, improves continence, but doesn't put her into complete urinary retention either. So, I think, um, as, as we move on, you, this was just an absolutely massive amount of surgery um and uh the communication and teamwork was uh uh critically important, not only preoperatively and obviously intraoperatively, but also postoperatively with all of the repercussions that flap that Doctor Tina created allowed her to have a prosthetic fitting and had enough bulk where she could have even a suction device onto her prosthetic. She was able to go back to college and um after rehab stay was starting to use her prosthetic pretty well. Um, she is, uh, we then went back into surveillance and sadly, um, she has relapsed, uh, the osteosarcoma, uh, so not the best oncologic outcome and sadly, uh, almost always the case. Uh, but I think we gave her, uh, with it all of this devotion, and I probably should also mention the pediatric oncologists who are predominantly the captains of the ship here. Uh, gave her a year and a half of a great quality of life, uh, despite losing her leg and, uh, despite the, the critical issues that we faced, um, in surgery. Thank you. Uh, I thank the team, uh, for demonstrating this extraordinary collaboration, uh, with extraordinary challenging case. Uh, we're gonna see two really complicated, difficult areas, uh, to resect. Um, and these people are also difficult to get cause both of these cases have been over a year in scheduling, uh, to, because everybody's so busy. Um, we have a couple 100 people online. I'm gonna prioritize questions to the in-person, but go ahead and enter the Q&A if you have questions on, uh, on Zoom, and we'll get those secondarily, uh, if there's time. Um, we have just a couple of minutes for questions for this team, and, and then we'll switch. Um, I know, like retrospectively is more 2020 but like, the primary source control, you know, how you said it can be radiation or surgery, but the risks of osteosarcoma with the radiation, does that, Outweigh the benefits of like upfront. Um, XRT. Thank you. Yes, um, does this work? Uh, So, it's really a really difficult discussion, and you want to talk about like multidisciplinary. Um, when kids have Ewings and that discussion comes up of local control, we have, once they start their chemotherapy, we have 12 weeks, and then that really needs the local control really needs to happen. And 12 weeks sounds like a long time, but it's a very short time for a kid who's facing, should I have surgery or radiation for my tumor in the family, and it's, it's a lot involved, especially in growing children. Um, and so, in, in pelvic Ewings, we often would lean a little more towards radiation, probably more so back in the beginning when we were treating Ewings, but even now, so, especially if it involves the acetabulum because our ability to reconstruct the acetabulum is very poor. And so the function of the limb with that shortening and all that is poor. Now, of course, in hindsight, had we known that she was going to develop massive Of lymphedema and a radiation associated sarcoma, we would never have given her, we would have done the surgery upfront. Uh, but it is a, it's, so we, we basically sit down with the family after several meetings that always involve the three arms, the the surgeons, the radiation oncologists, and the pediatric oncologist. And it's a really good group discussion, and we try to help them with meeting other patients who have had both options to decide which one they want. I have one more question. If the family had chosen uh surgery for local control, uh, do you think that the intramural dissection in the bladder would have been, uh, unnecessary, or, or was that necessary just to have a margin? If, if you mean if, if for her original Ewings at the outset, it, it did not involve the lower pelvis initially. It was only the ileum and the upper pelvis, so the bladder would have been completely fine. Um, it would, it would have been the smooth dissection like Brent was alluding to where he comes in, we get into the retroperitoneal space that the Ewings has shrunk down to be a bone only. Sort of resection, you make a cut just above or through the acetabulum, you make a cut through the sacrum, the bone comes out easy peasy. Um, it's definitely very different than what we had to go through or that's a crucial factor in the balance of discussion and obviously there's so much advantage to radiation that that was the choice. You might make the operation harder. It wouldn't make the operation harder in the future, right? And, and I think in, in her case, it was, it was, um, mainly just losing that acetabulum and affecting the, the, the, the function after internal humulectomy is, is good, but it's not normal, you know, your leg really, really shifts and you know, who you don't, you don't think, you don't base your decision on, I'm gonna get a horrible secondary malignancy, even though we talk about it. If there's no further questions, I wanna thank this team. We'll do a, a, a quick switchover, um, and I think you could see, thank you. I think we can see what extraordinary uh collaboration and cooperation we have. Um, all of the preparation that went into this case. And despite the preparation, still some people being called in on call at night. Uh, and Amir described his, his part of the case being easy. I'm not even gonna ask what time of the night his, his part started. Uh, the so-called, so-called easy closure. Uh, uh, uh, so the, the next case, I, I think will be, uh, similarly fascinating. Uh, I think, I think Lissa Barrett's gonna start and introduce her team, uh, and I think this will be another case. OK. Great. Well, thanks so much for having us um here to present. This case of a clivalcardoma. This case, uh, requires really a lot of moving parts to get through the surgery and so we have, it's gonna be a, a little bit different. We're gonna talk about all the different things that led up to preparing the patient and to getting the patient through the surgery in an intraoperative MRI for what was a very complex skull-based case. Um, these cases are particularly challenging because choomas are so locally invasive and are surrounded by neurovascular structures that are very critical. And are very difficult to access at the anterior skull base. Um, historically, we would have accessed a tumor like this through a very morbid surgery like a transfacial approach or a very wide lateral approach that requires a lot of removal of bone and sometimes um sacrifice of some neurologic structures. So, uh, that has really evolved over time to these. Um, far more elegant and less morbid approaches where our, our ORL team can get us to the ventral face of the tumor in a much more um Much less and much less invasive and less morbid way, so. I'm going to go through, through that case with this patient. Um, these cases, uh, as I mentioned, require a lot of, a lot of moving parts, but this patient in particular was challenging, excuse me, because he was a Jehovah's Witness and it was important to him. Sorry, um, not to have a blood transfusion. And so, Doctor Gubi, who is one of our anesthesiologists who manages the bloodless surgery program, is going to talk about what, what goes into preparing a patient for a case like this. And, um, in this case, in particular, we were drilling around the paralival internal carotid artery, so there was a lot of potential for really massive hemorrhage and we needed to be prepared for that in a patient that really did not want transfusion. It's really important in these cases and to, for survival to really get out the entire tumor and these tumors can kind of grow through little channels in the bone and through little nooks and crannies in the intradural space and having an intraoperative MRI which we're so fortunate here to have two of these, um, is very critical for their survival so we can really get these tumors out. And Kevin Carroll, who is our MR tech that runs our MROR program and is going to talk to us about what goes into, to doing that safely in an operating room. Um, and then our, our nursing team, um, who is amazing with these cases have really a lot of challenges, and these cases require many, many instruments that are only used for these cases. And so they're not instruments that people are as commonly familiar with. Um, and so it setting up the room and, and, uh, scrubbing these cases really can be, um, challenging. Um, our skull-based program here is, I think, probably unique for a pediatric program. It, it may be the only pediatric skull-based program in the country where we don't bring in any adult skull-based expertise. We really have everybody here. Um, we have Elon Medill who is just masterful at these endoscopic and anterior skull-based approaches. Um, we, we recently had Edith, um, Reshef join our team who can do our transorbital approaches. Um, we have Aaron Remenschneider. And Reza Rabar who can do our lateral skull-based approaches. We really cover the gamut of what, what is needed for expertise in skull-based approaches and can take care of all of these diagnoses in our program, um, just with pediatric specialists and are really fortunate here to have all of that expertise in-house. Um, it's, it's very unusual. So, I'm gonna turn the time over to our brain tumor center fellow Casey Jarvis, who's going to go through the clinical history, um, and then we'll go from there. Hi, everyone. As Doctor Gray said, my name is Casey Jarvis. I'm a neurosurgery resident at the Brigham and the current Brain Tumor Center fellow here at Boston Children's. I'll be presenting this case today of patient JR, um, who is a 16 year old previously healthy male, notably a Jehovah's Witness, who presented with double vision and vomiting, was found in an altered state by his parents, and brought to an outside hospital ED. Um, where he had a CT, um, image, which demonstrated a skull-based mass, um, for which he was transferred to Boston Children's Hospital for evaluation by our specialized skull-based team. Um, on arrival, he was noted to have a left-sided sixth nerve deficit, but was otherwise intact, and we obtained an MRI that demonstrated a large clival skull-base mass that was concerning for fordoma. So here you can see some of the representative MR images. We have a um large lil-based mass that is hypo-enhancing and T2 bright, involving the internal carotid arteries, cavernous sinus, and six nerves bilaterally, um, and also exerting a significant amount of mass effect on the pons, um, and the basilar artery and really effacing the prepontine cistern. Hi, everyone. Um, I'm Susan Goodby from the Department of Anesthesia. I'm gonna talk about the perioperative management, uh, and preparation for this patient. Uh, so, I'm gonna talk to you mostly about, uh, what we do preoperatively. It's definitely a team-based approach, discuss, discussion of bloodless care, informed consent and patient-centered care, and optimization of red blood cell mass, and then some of the intraoperative management. Uh, so, for those of you who don't know, the concept of bloodless medicine originated, uh, with the Jehovah's Witness in 1945 in the modern era when they, uh, interpreted the, this, uh, this statement from the Bible and Leviticus, for the life of every creature is its blood, it's blood is its life, you shall not eat the blood of any creature. And furthermore, the Watchtower, uh, doctrine, uh, said that When sugar solutions are given intravenously, it's called intravenous feeding, and then, therefore, the transfusion is feeding the patient. So because of this, the Jehovah's Witness, uh, faith, uh, developed a, a doctrine where allogenetic blood transfusions were not acceptable to these patients. Now, in the subsequent years, uh, there's many more reasons that, uh, families and patients and the healthcare, uh, team, uh, are, um, trying to decrease blood transfusion exposure in our patients. I won't go into that, but I just wanted to give you a little bit of the background about where bloodless medicine started. Uh, so, this is basically the concept of bloodless medicine and patient blood management. It's really about improving patient outcomes by managing their anemia, using blood conservation modalities, which are, uh, multimodal and interdisciplinary, optimizing the Patient's coagulation, but most importantly, uh, I think is patient-centered decision making, especially for these Jehovah's, uh, Witness patients. Uh, so, this, uh, article, uh, was part of our themed issue of patient blood management in anesthesia and analgesia, and Um, the important thing is that we, uh, all worked as a team, uh, to have, um, uh, informed consent, uh, with this patient, informed choice, and we wanted to make sure that their ethical and moral, uh, beliefs were, uh, considered and respected and, and upheld. Uh, so the patient came to us with this, uh, durable power of attorney, uh, document, which, uh, many of you have probably seen, uh, even though this was a 16-year-old. Definitely the 16-year-old had the capacity to consent for treatment and the capacity also to refuse treatment. Uh, and it stated that I'm one of the Jehovah's Witness and I direct no transfusions of red and whole blood, uh, platelets and plasma under any circumstances given healthcare providers believe that such are necessary to preserve my life. Uh, so I'm very grateful for, uh, Doctor Adele and Doctor Beard for bringing me into this conversation. Uh, we had, uh, a lot of meetings with the family together with our hematologists, our social workers, and, uh, and, and, and, and our legal team. And this, uh, this patient was, uh, where this concept of having a letter of understanding came, uh, into play. Boston Children's Hospital was one of the first hospitals, uh, children's hospitals to have this letter of understanding. And what we wanted to do is have a nice discussion with the patient about their, um, about their, um, considerations and beliefs and try to, uh, come together. As a team, uh, to have an understanding about what's acceptable to them and what's acceptable to us. So, the old way was a court order, uh, but now our, our new way is to try to, uh, go into an agreement of a letter of understanding. And this is where they, the family and the patient don't consent for us to give blood products, but they recognize that. In, uh, life or limb situations, threatened situations that we may, uh, ethically and morally feel that we need to give it to save life or limb, and they're releasing Boston Children's Hospitals and all other physicians and entities of liability. So, the, our legal department was very happy with this document. Subsequently, we've shared this with many children's hospitals across the, um, The United States. Uh, here's the basic position of, uh, blood and blood components for the Jehovah's Witness patients. They don't accept whole, uh, blood, uh, red blood cells, and, uh, plasma. They, uh, definitely accept self-salvage if it's in continuous, uh, circuit with the patient, recombinant, um, erythropoietin, and synthetic blood products, and Um, this is the discussion that we need to have with our patients about what, um, because this is variable, they may consider, uh, albumin clotting factors, cryoprecipitate, uh, prothrombin complex concentrates, cell salvage, and auto-transfusion from predated blood. So, this is the document that we have, um, formulated to have this discussion. Uh, we had many meetings with the family on Zoom, uh, and in person, uh, with our hematologists, our nurses, our social workers, our legal department to go through all of this, uh, and I think they felt heard. Uh, we were, we, um, uh, agreed that albumin, cryoprecipitate, uh, cryosupinant. Uh, recombinant concentrated factors, uh, and normal volemic chemodilution, which is harnessing the patient's own blood, whole blood beforehand, and cell salvage were all acceptable to this patient, uh, with certain, um, uh, prerogatives. So, this is a snapshot of what, you know, bloodless care is. We don't have a patient blood management program nor a bloodless care program. As a matter of fact, there's been many patients who have been turned away. Because we can't offer, uh, a guaranteed bloodless care here. Hopefully, that will change. Uh, so, preoperatively, what we did is all the patient-centered care and informed consent. We optimized the red blood cell mass together with our hematologists by using erythropoietin and iron and, uh, got hemoglobin up to a, a good level and intraoperatively, we used Normovolemic hemodilution, presumab, uh, predominantly with antifibromolinics. And of course, there's nothing, uh, that we can, uh, do without our surgeons and good surgical hemostasis, which, uh, which was a big part in this. Um, here's normal volemic hemodilution. So, uh, what we do is keep the blood in continuous circulation with the body, kind of, um, Uh, like a setup of almost like an ECMO, I guess, where we actually take, uh, blood, uh, from the patient, um, anticoagulate it and replace it with, uh, with albumin. Uh, we can do an allowable blood loss calculation, so the patient's hematocrit starting was 40. So we can calculate if we go to 30, we were allowed to take off over a liter of blood, and that's what we did under careful, uh, um, Um, consideration in the operating room and, uh, we have a protocol, which is based on AABB guidelines that, uh, I wrote for Boston Children's Hospital for autologous, uh, whole blood. So, the good thing about this is you have whole blood to give back, which has all of the good clotting factors, red blood cells in the patient's own blood. So, intraoperatively for this 72 kg, 16 year old male, we had good IV access. We maintained normal bulimia, we gave TXA and we gave, we had uh one whole liter of hemo diluted whole blood removed. We replaced that carefully with album and we had all our recombinant agents and clotting factors available if we needed it. Um, and, you know, in total, um, there was luckily only 500 cc's blood loss, uh, thanks to a good surgical, uh, control and hemostasis, uh, but we did give the whole blood back at the end. Uh, and as you can see, it was a very long, uh, procedure where the, uh, we started in the operating room at 8:30. It took us a little while to get the lines and the blood off. Uh, 10:30 was incision and, um, after midnight, we came out of the room. So here are the five perioperative bloodless care, um, rules. Uh, I think first and foremost, communication, informed consent, and careful planning, uh, was what was, uh, really important for this patient and then of our, all of our, um, meticulous, uh, care, uh, perioperatively. And in the end, uh, we received, each received letters from the patients, uh, I think the nursing team and the surgeons and everybody involved. This is the one I got, very proud of it. Uh, it's been a year since my surgery and completion of my radiation treatments. I'm doing better than expected in light of the seriousness of my diagnosis, chodoma, and surgery. I want to thank you for the care you gave me during the surgery. It was a rough period for me and my family and my friends. Your professionalism and respect for our questions and beliefs gave us some time and peace during that time. I'll go on to Judy, are you next for the nursing perspective or? Hm Good morning. I'm Christine, and uh Doctor Baird said, I, uh, we do collaborate with all the teams for this case. It's a lot of preparation to get ready for. Um, These cases take place in our MRORs 303, 304. We, as you can see from this picture, um, we do use navigation for these cases. We have the option of brain labs, stealth axioms, stealth optical, um, And we It does, we start preparing for these cases typically the day before, um, if we can. So as you can see from this picture, we have a lot of trays and equipment that we have to gather for the ORL standpoint. We basically set up as a fess. We use their Endo scrub. The cameras, scopes, everything. Um, we have about 8 trays that we use. They're specialized trays, each has about 50+ instruments in them that have all been selected by the surgeons for these cases. Um, this setup, it's a rough view of how we set up the room each time. Um, we have the reconstruction team table is really just for a fat graft. Um, so we always have that prepared. We have our navigation system, we do have 2 tables, 2 mayo stands. As you can see, closer look at all of our instruments, we have a circulator, a safety nurse, and a scrubber, surgical technologist every time for these cases. Um, we count multiple times. It's critical that we are very organized and we know the instruments very well when we do these cases, um, because they are specialized and we do count. Before or after scans, the case after the case, constantly. And it's also critical that we keep our rooms sterile and set up until the patient leaves the room because these are specialty trays that we use and we do not, unfortunately, have full backups of everything. So we do have to keep in mind that we have to protect these trays and our tables at all costs. Good morning. I'm Judy Jackson, one of the staff nurses. Um, you know, I'm, I'm not a public speaker, but I'm, you know, so I'm gonna use the checklist that I had, had, uh, prepared here. Um, first of all, I wanna thank, uh, Doctor, uh, Beard and Doctor Adell for inviting us here to, uh, to talk about, I guess I'm gonna be talking about the historical perspective of nursing, uh, in the endoscopic, oh, I guess I just, um, Perspective of these surgeries. Um, I wanna pay homage to my former neurosurgical nurse, uh, colleagues, Jeannie Richard, Rita Tolchinskaya, and the late Sue Lovell from the Brigham. Um, I know many of you in the audience probably don't know who I'm talking about, um, but they were an honor to work with and to learn from. I also wanna talk about, uh, Doctor Ed Smith, who in my mind was a pioneer in the early days of transferhenoidal surgery and is still a trailblazer in performing these surgeries. He was always a great communicator and teacher. Um, he would invite Doctor Laws over from the Brigham. Uh, to assist and is needed, where, uh, which is where Sue Levell would come in. She come in the night before with 6 to 7 kits that needed to be processed, um, and, uh, but the just in case he needed his, his instruments, um, because we all know that surgeons like to have what they want for their, their instruments. Um, we always tried to schedule these cases in the old, now, uh, room 16 because of the equipment involved. Um, microscope, CA, irrigating endoscope, microdebrider and or the drill, specialty kits. Uh, we had 2 Mayo stands and at least 3 tables full of instruments. So, as you can see, not much has changed from the, uh, the, the pioneer days. Um, so, when the charge nurse would used to call in and wonder what, do we really need two circulators, uh, we would always say, yes, we really need two circulators, uh, to, to start these cases because just as the exposure for the nurse uh the surgeons is important, the room set up for, uh, these cases is important to the nurses and the scrub techs. Um, some of the nursing considerations for this type of surgery are gonna be going over the labs with the anesthesia team and paying, uh, particular attention to the endocrine and hematological state, making sure there's a type and screen available, uh, keeping the lights dimmed and the noise. Level to a minimum when the patient enters the room because of just their vision issues or um headaches. Um, having the ordered meds as well as hematological agents available in the room including a 16 French Foley just in case we get a major bleed. Um, positioning the patient. These are long surgeries, so making sure that, you know, you have good positioning, uh, for the patient for that length of, of surgery. Um, they're usually gonna be in a fixed head frame, um, and be 180 degrees away from anesthesia, so keeping all of that in mind. Um, and going over, um, during the case with anesthesia as far as monitoring maybe perhaps for, uh, diabetes insipiditus, so you wanna make sure that you're keeping good communication with your anesthesia team. So, around 2020, Doctor Beard started performing more and more of these surgeries, and at that time, he, she introduced our esteemed ORL colleagues. Um, this is where our newest neurosurgical team, uh, took it to another whole level and, um, You know, but again, these surgeries are complex and long and um still requires two circulators and a scrub to set up. And um that's my perspective. Yeah. Mm Thank you for the privilege of presenting today. I'm Elon Medill. From an otolaryngology perspective, our role is to provide transnasal endoscopic access and a watertight closure at the conclusion of the case so that there's no postoperative CSF leak. In this case, we elected to take a transhenoidal approach which allows access from the plainum sphenoid alley or roof of the sphenoid down to the clivus, which is our surgical target. Following the resection, we typically perform a multi-layered closure. Uh, the final layer is a nasal septal flap. This is a local rotational flap based on the posterior septal artery, which runs between the sphenoid sinus and the nasal coina. And I'll go through the approach in some more detail. This is a transnasal endoscopic view through the right nasal passage. We'll start with some injection of local anesthetic. Next, we're gonna lateralize the inferior and middle turbinates to identify the superior turbinate, which is our landmark for the sphenoid sinus. We'll resect the lower half of that and just deep to that, we'll find the sphenoid ostium. We're gonna expand this slightly and then perform the same procedure on the contralateral side. And next, we'll go into our nasal septal flap. So this will start with an incision along the arch of the coina, which extends to the posterior septum, down to the floor of the nose, and then anteriorly to the edge of the cartilaginous septum. Next, we'll make a superior incision starting at the level of the sphenoid sinus and then extend that incision along the septum. And we'll connect this anteriorly with our prior inferior incision. Now, we're gonna raise our flap off of the underlying septum. And we'll continue this dissection posteriorly all the way to the sphenoid sinus. Once the flap's completely mobilized, we'll lay it in the nasopharynx for safekeeping during the case. I'll usually place a pledget to protect it during the rest of the resection. And now we'll perform our posterior septectomy. So once we remove the posterior bony septum, we now have binasal access, so we can use instruments through both nostrils. And I'm gonna resect the sphenoid rostrum, so that we can have one large sphenoid cavity. Now you can start to see the sphenoid. There's some remaining inner sinus septations which we'll take down. And I'm just resecting the anterior wall of the sphenoid right down to the floor of the sinus so that we have as much access as possible. Just ahead of you, that's a bony osteophyte, so probably not the tumor itself, but just some reactive bone from the tumor. Just removing some final inner sinus septations, as well as the osteophyte. Once we have as much transnasal access as possible, um, Doctor Barrett will step in for the resection. So, these are just some illustrations and cadaveric um dissections of our surgical view. So, we're, we're, once, once uh Doctor Adde has finished the exposure, um, I'll drill off the bone and this is basically what we'll be looking at. This patient's tumor extended from about the level of the 6th cranial nerves um down to about here, but, um, Just to kind of note some of the really critical neurovascular structures we're working around, we have the vertebral arteries, the basilar artery, um, and we're surrounded by cranial nerves, and we knew in this case, the 6th cranial nerves were already involved because the patient did present with the 6th nerve palsy. Um, but also have surrounding facial auditory nerves, hypoglossal, hypoglossal nerves, the lower cranial nerves, um, a lot of landmines in this area, this, this very small anterior spinal artery which is in the field, um, an injury to that would cause paralysis. All of these tiny little brain stem perforators are feeding very, very densely eloquent parts of the, of the brain stem, and these tumors, um, tend, tend to be very sticky and invasive tumors. So, um, Why these, why these, uh, cases take so long, just a lot of really careful dissection. Um, so we'll start out with drilling through the clival bone, um, using navigation pretty frequently to make sure we know where our internal carotid arteries are and kind of the extent we want to expose. Um, in this case, we're already looking at tumor as the bone is coming off because the tumor had really kind of obliterated the dura, um, which is the normal layer we'd be seeing there. And, um, the 6 cranial nerves are, are coming off and going through these dural sleeves at the top and, and we really started with that under the limit of, limit of our dural opening extent. Um, and then we'll, we'll use kind of these angled instruments to slowly open the dura and free up pieces of the tumor from the cranial nerves and any vascular structures um before we deliver them. And take this tumor out. We are doing neuro monitoring during this case, so EMG monitoring of the cranial nerves by monitoring the, the lateral rectus muscle in the orbit and monitoring the lower cranial nerves so that if something is being irritated or is attached to the tumor, it's, it's going to trigger and also so that we can stimulate and find those nerves through the dura or in the tumor mass um as we're working. So you can start to see some brain stem anatomy here, um, we're looking at the vertebral artery. That was too big to put on. Looking at the vertebral arteries here, um, anterior spinal artery, vertebral vasar junction. Um, and then the pontine medullary surface of the brain stem, um, just as we continue to, to dissect. Um, we, we do have the ability to go a little bit lateral, um, with our angled instruments. Um, the, the tumors that extend very far lateral are, are inaccessible from this approach and sometimes we have to do combined approaches from the side, but that wasn't the case, uh, for this patient, and we were, we were able to slowly get all of, all of this tumor out, um, just from this anterior approach. And I think we, we started out, um, kind of being quite judicious with our superior approach because of the 6 cranial nerves that are going to be coming off, um, about, about there. We really wanted to identify those, um, prior to removing that last top part of the tumor, um, to really try to protect them and uh we were fortunate with this patient that he did not have any new cranial neuropathies or neurologic symptoms. His 6th um cranial nerve deficit did not. Um, worsened, but it also did not improve afterwards and he eventually needed some Botox, um, in that eye. And this is just at, at the very end, we'll look laterally over into the cerebellop pontine angle, um, you can see the vagus nerve there, um, some of the lower cranial nerves and just make sure that we've, we've cleared everything, everything out. And at this point, if we feel the tumor is out, we're gonna turn the, um, The room over to our MRI and nursing team for a scan. We'll have them kind of talk about that process. Good morning. I'm Kevin from MRI. Uh, the system we have at Boston Children's Hospital is quite amazing. It's one of the first in the country. Uh, it involves three suites. We have where the magnet, a 3 TV to magnet lives in the garage, and then we have the capability to turn the magnet and go into two rooms, either a 304 or 303. And, uh, it provides beautiful images. But that being said, being a, bringing a 3T magnet into a situation where nursing talked about all the instruments. All equipment in the room can be a little terrifying, but everybody from neurosurgery, anesthesia, nursing, everybody is so good about keeping the room safe for the patient and safe for all the staff in the room too. So, we've got, uh, here's what happens with the patient. And if you're looking at that picture, you see the patient is draped to keep them sterile. Uh, and then we, uh, Go ahead and that's Joe. He's one of our MRI technologists. He's measuring the patient's head to be in the proper position for scanning. We wanna make sure they're in isocenter. And then I'm just gonna You know, before we open the garage doors, those big silver doors is where the magnet lives. It'll come out over the patient and we'll start imaging. But to do that process, we go through several checklists to make sure everything in the room is safe. Uh, as you can see, we've moved everything out beyond the five Gauss lines. And, uh, so here's what happens. And if you ever want to come and see the system, come on up. It's really quite amazing. And here, we're gonna have the doors opening. And the magnet will come out and What happens at that point is we image the patient, and I got another video here. But that's what the magnet looks like, and it, it's totally free floating, it's riding on those rails in the ceiling, uh, and let's see, and then we'll Yeah, let's see, where is my other, there we go. And now the magnet's coming out and it's gonna go over the patient. And so, As you can see, everything's away. Patient's sterilely draped. We do the scan, bring the magnet back, and then most of the time, Doctor Baird and the surgeons are quite happy and don't need to resect anymore, but, uh, sometimes we will. I mean, the most we've, I personally have scanned in the case is 5 times, you know, just in and out, just trying to get all the tumor out and helping the surgeons with that, and that's it. So after our MRI we're actually happy with our resection, so we'll start our closure. So this will start with a layer of Duragen, which is placed in an epidural fashion. And then tucked underneath the bone. And given the size of the bony defect, we elected to place a free abdominal fat graft in this case. And then lastly, we'll raise our nasal septal flaps. So, uh, it's important here to make sure that the septal mucosa is lying intranasally so that there's no postoperative mucocele formation. So we'll carefully unfurl the edges and make sure the entire sphenoid is covered. Also important to make sure that there's bony contact all around. And once we're happy with its positioning, we'll use a few layers of some absorbable materials. So this is just surgicel being placed around the periphery. And some to seal tissue glue. And lastly, a large piece of Nasacort to help keep everything in place. That's it for the closure. So, just to wrap this up, I'm gonna briefly discuss our postoperative course. Um, on the right-hand side of the screen here, you can see our postoperative imaging that, um, after resection demonstrates that the mass has now been completely removed. The, um, mass or the pon pons itself has been decompressed, and the pre-pontine cistern has been restored. Um, here you can see our multilayered reconstruction, see the mass here, um, with the Epidural, uh, durigen and then our abdominal fat graft and the nasal septal flap placed by our ORL colleagues. Um, postoperatively, we also take a number of measures to ensure appropriate wound healing and to avoid any sort of cerebrospinal fluid leak. Um, and that includes the placement of a lumbar drain intraoperatively, which is kept for a number of days after the procedure. Um, and that allows us to kind of divert the, the fluid and, um, Reduce the, the pressure on the reconstruction. Additionally, we take out of bed precautions, so we keep the patient flat for a number of days after the procedure, um, as well in order to reduce that pressure. Um, we have nasal splints placed by our ORL colleagues. We also take sinus precautions, um, which is also meant to kind of, um, avoid any um Any increases in intracranial pressure, um, so that means no straws. Um, we usually give the patients laxatives to, uh, prevent, um, bearing down, um, and obviously no blowing of the nose or anything of that sort. And, um, in the immediate post-operative period, we also have our patients go to the ICU for very close monitoring. So, this patient was discharged home on post-op day 4 with a stable exam. He did have that um slight left uh cranial nerve six palsy as Doctor Barrett had mentioned, um, but was otherwise intact. He subsequently underwent proton beam radiation therapy, which is standard of care for these chodoma patients. Um. Um, and then, as Doctor Bird also mentioned, he did have Botox injections in the left medial rectus for some mild diplopia. Um, on most recent follow-up, the patient is doing well. He's back in school and back to his normal activities. I think that concludes our presentation, so thank you all very much and we're happy to take questions. Well, I think we're gonna all see what an extraordinary, uh, Orchestration, uh, this case and the prior case, uh, was, uh, and it really highlights what we can do, uh, with our, with our teams here of, uh, not only the greatest experts in the world at what they do, but the ability to collaborate across disciplines. Uh, I wanna thank all the presenters. We don't have time for discussion, we're at time and everybody's OR needs to start, and there will not be any surgical delays. Uh, and, and so, uh, I wanna thank everybody. Um, we've gotten great, uh, response. The auditorium was full here. We had a couple of 100 people on Zoom. Uh, I had a great response to priors. Uh, please let me know if you know of yourself or your team members who should be highlighted here. We're gonna keep doing this, uh, a couple of times a year as long as people find it of value. We, we get to see things here, uh, that we don't even know exists. I'm probably most people have never seen. Our intraoperative MRI, I walk in all the time and I'm always amazed by it, uh, it's not restricted to neurosurgery. It could be used for, uh, osteosarcoma or, or, or what, what else we might need to do. So, uh, it's a Doctor Proctor's waving no. All, all the neuros are say no way. The institution built the resources for everybody. All right, thank you all for your time and its extraordinary presentations, all of you, thank you.