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SNI, Surgical Neurology International, a 2D Internet Journal, and SNI Digital Innovations on Learning, a video journal interactive with discussion in association with the Sub-Saharan African
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neurosurgeons
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are happy to present the Sub-Saharan Africa International Neurosurgery Grand Rounds held on the first Sunday of each month.
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These Grand Rounds are devoted to global solutions, to clinical challenges in neurosurgery, the moderators are Estrada Bernard and James Oussman,
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and this has an international audience. A guest lecturer will will be John J. Wellens, the third.
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MD,
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MSPH, who will talk on posterior fossa tumors in pediatric patients, these from Vanderbilt University and the Department of Neurosurgery in Nashville, Tennessee. Dr. Wellens is chief of the
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Division of Pediatric Neurologic Surgery, the Cal-Turner Chair in pediatric neurological surgery, the professor of neurologic surgery, pediatrics and radiology, and radiological sciences, and the
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Department of Neurosurgery at Vanderbilt University. Dr. Well, welcome everyone. I think I've started recording. We're privileged to have Professor Jay Wellens join us. He's the chief of
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pediatric neurosurgery at Vanderbilt University He's got wide expertise in all aspects of pediatric neurosurgery, but we asked him to just focus on posterior fossa tumors today. He's a well
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accomplished author having published a memoir and is often contributing to outlets like the New York Times and NPR. He's reputed to be a great storyteller.
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So, Jay, welcome. And please get started after Jay's presentation We'll have a presentation from the, from Professor Hagguland's group from Nigeria regarding their posterior,
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their posterior, pediatric posterior, fossil tumor experience. I hope we'll have very active participation and please proceed.
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All right, William White, it's great to be here. Thank you all. It's a big honor for me to be asked to do this. I do have some disclosures. Most of them are research-based and don't have
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anything to do with this. At the bottom, I do have to say that I have a contract with
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Avidas Creative Management
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and Penguin Random House. I'm still
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under contract with them. This is our division, myself and Rob Naffdall, who does our epilepsy spasticity, Chris Bonfield, who does our craniofacial and complex spine We call him our epidural
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pediatric neurosurgeon. And Michael DeWann, who we hired several years ago to really cohort all of our brain tumors in with Michael. And so we're just a terrific surgical group. Haley Vance is a
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nurse practitioner who partner who works with us. And Charles Markroom, it's our physician's assistant. But I'll show you this because Chris Bonfield Michael DeWann and Haley Vance have made a
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significant effort into the global neurosurgery space in terms of collaboration with several groups across the world, but particularly in Africa. But before I talk about them, I wanted to talk
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about Astrata just for a moment. Oh no. I met many years ago, Astrata was the chairman at UNC when I was a resident at Duke. And we were also friends through Tim George, who was a pediatric
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neurosurgeon, and he was Astrata's friend and my mentor. And one thing I remember, there was a get together that we were at many years ago, and Astrata, you may or may not remember this, but
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what I remember is that there was the limbo.
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And everybody was doing the limbo, and everybody could do the limbo relatively well, but Astrata, that's not him, but it's a picture of exactly what he could do. He was amazing at the limbo I was
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started thinking like I wonder if this drought is still doing the limbo. So I looked up and it turns out there is something called limbo dancers bursitis. And so I didn't know Estrada if you've been
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attacked by limbo dancers bursitis, but apparently this is really the only injury you can get from limbo dancing that's published in the literature. So kudos to you for having been a limbo dancer.
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And if you have any bursitis issues, I'm happy to send you this brief note from the new in the journal. Yeah, the joints, once you pass 60, the joints just don't have that flexibility anymore.
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So.
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Terrific. Okay, so this is kind of a slide that represents to me what kind of pediatric surgical neuro-oncology is all about. Across the top, you see many people, there's
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pediatric oncologists, There's pediatric neuro radiologists on the right. There's our surgical team on the left, and Mike DeWann, Michael DeWann, who is kind of the lead on this now. You know,
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it's not just the imaging and the taking out of tumors. It's the, you know, collaborating with other groups across the world, and it's also the publishing piece, which is really important to
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establish and then be able to reflect, and then be able to pass on your learning that you've had an opportunity to be a part of. I'm also just wanted to give a brief shout out to Dr. Bonfield and
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Dewan and Hailey Vance for our global neurosurgery program here. It involves attendings and senior residents and pharmacists and our nurse practitioner colleague, Hailey Vance goes, but many of our
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senior residents have gone from places ranging from Peru to Sub-Saharan Africa,
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to numerous other places across the world. I won't go too deep into it, but right now Chris Bondfield and Haley Vance are coming back from Tanzania, and then they also spent a week in Nigeria. And
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it's such a tremendous benefit for our team to be able to go. I love the different places that they go and the collaborations that you see and all the patients that they're able to help take care of
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with the teams there. And, you know, when Chris talks about the benefit, it really is a benefit to us and to our teams and about how not to waste so much and how to be able to do operations in all
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different kinds of areas. And so, and a lot of benefits, teaching and collaboration, but meeting new people and learning new ways of operating and thinking about problems differently. getting out
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of your routine and comfort zone and seeing new things. These are just pictures of a lot of our residents who've had an opportunity to be there. And I can just tell you that it's really had a
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tremendous impact on our department to be able to spend time with other centers learning how they do things and then bringing that back to us. And I'm just very proud of Dr. Bonfield, Hailey Vance
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and Michael Dewan for that work And then Michael has done, Michael Dewan's done a lot of work in this sub-Saharan African neuro-oncology consortium and some of the folks on here may be part of that
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and you can see a recent publication from that group. But it's just, it's been something as chief that, you know, when there's just one or two of us, we really couldn't do that work. But when
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there's four of us, you know, We have an opportunity to have somebody gone. almost all the time and helping and learning in other parts of the world. So I just wanted to point out that there is,
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as you all probably know, a lot of collaborations and one of them is with us here in Vanderbilt and we're very grateful for the opportunity to do that. So with that being said, and with me already
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talking about astrotis possible percitis, I'll go ahead and move on to kind of the outline of what I thought we would talk about today. And I'll also say as astrotis knows, your career as you
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move through this, I'm sure
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many of you know, it evolves over time. And so I spent my training years at Duke, as I mentioned with first Bob Wilkins as chair, but then Alan Friedman as chair while astrotis was.
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My first, my fellowship and first job for a decade was at UAB, University of Alabama Birmingham with Jerry Oakes. And I did the majority of brain tumors and vascular malformations there. And then
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when I moved here to Vanderbilt 13 years ago, really to kind of build the program and start recruiting people in, I kind of found myself doing everything. And then, as you want to build and build
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epilepsy programs and you bring somebody in who is good at surgical epilepsy, like Rob Naftel, or if you want to build a spawn or craniofacial program, you hand that part away and you have Chris
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Bondfield. And the same thing with tumors, now that Michael Dewan here is that we cohort most of them in him, so some of these slides will be from Michael because I thought it was important to
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represent him as well. But I do have a long career of taking care of kids. I think at the end, I'll show a picture brain tumor I took out. I look like I'm about 12 years old. I look about the
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same age as the patient, honestly. But anyway, so we'll talk about brain tumor classification and epidemiology, a little bit about hydrocephalus and acute management just because that is such a
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common way that children with posture fossa tumors present, talk some about surgery and then post-operative management and then the adjuvant therapy and survival and how those things are important So,
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you know, some epidemiology brain tumors are the second most common childhood malignancy. The first most common are blood cancers like leukemia, but that would make a brain tumor being the most,
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it's the most common solid organ malignancy in kids. And I'm sure many of you all know that. It incidents six per 100, 000 person years and it's the most common cause of death among childhood
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cancers
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This is a nice. Um, just a kind of flyer that, uh, side care, um, uh, a group of hospitals puts out for the 10 common symptoms of brain cancer. Uh, and you can see at the top, uh, headache
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is common, vomiting, uh, headaches are vomiting, particularly first thing in the morning, visual issues, hearing loss or balance problems, issues with lethargy or confusion, uh, and then of
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course seizure Now these are for obviously for brain tumors of, of all over, all over the brain, not just the posterior fossa, but it's nice to see some folks putting out some public health fliers
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for people to be able to kind of understand how folks present. Now, uh, this is a complex slide and I apologize for it, but the reason why I wanted to bring it up, this is, this is for all brain
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tumors. But if you look on the left side of the slide from age zero to 14,
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You see, in brinal tumors, in which medulloblastoma is
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part of that, and polycytic astrocytoma, and then high-grade gliomas. And then if you look at tumor types from age 15 to 19, your in brinal tumors really shrink down. And you still have
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astrocytomas, but you begin to see meningemas, you see a lot of tumors at the pituitary that includes craniofringiomas and also pituitary tumors. And then of the in brinal tumors, the majority of
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these are medulloblastomas. So this is
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an important point, I think, and that is, you know, when you see a slide like this, you see a CT scan that shows a tumor here and hydrocephalus. And then as you scroll up, you can see worse and
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worse The patient's somnolent, their blood pressure, systolic's 165, their heart rate is low. And, you know, the classic question, what is this patient immediately dying from? It's not so much
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brainstem compression or tumor progression, it really is the hydrocephalus. And so, you know, that's the most common presentation. Post-year fossa tumors is with hydrocephalus. And, you know,
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the mechanism is, you know, really basically thought to be obstruction. So it's obstructive hydrocephalus. Conceptually with
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malignant or high-grade type tumors or some major low blastoma is also, you can see disruption of the ability to absorb spinal fluid. So you get a, what's called a communicating hydrocephalus or
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absorptive-based of hydrocephalus, but really the most common reason is to have, is to, if for, is it for to be, it is to be obstructive hydrocephalus. And there's a couple of different ways to
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treat this. There's some data in the literature about people treating it upfront with an ETV.
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there's issues with time and timing and ability to get a tumor taken out. And some places we'll put a shunt in and then bring the person back. Here at our institution, we'll put an EVD
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in either through the standard coronal point, which you see here, or through a occipital bur hole. I tend to do this in the OR, through a separate incision, just right at the beginning of the
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case. Dr. DeWann likes to
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use stealth and put it in from an occipital bur hole. That's just really dealer's choice. And it depends on if you wanna do one or two preps. But in general, we will put an EVD in upfront because
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we believe this helps reduce the need for shunting and hydrocephalus afterwards. And we published a paper several years ago that shows that when we got here in 2012,
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The shunt rate for post-geophosphatomers was around 70 percent, and after five or six years of being here, we reduced that down to around 10 to 12 percent. So there's some things that you can do to
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help reduce the incidence of permanent shunt placement, and I think one of them is putting an EVD in pre-op and leaving it for several days after surgery. So after a resection, what's next? In
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terms of how to treat hydrocephalus, the tumor resection is the favorite definitive management. And as I mentioned, external ventricular drains are temporary, not all need CSF diversion. And then
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let's say they do need long-term CSF diversion, there's two options. There's a VP Shunner and ETV. And again, these are decisions now that you're making after surgery, so I'm skipping ahead just
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a bit. But I just wanted to talk about, you know, the pros of shunt is that they reliably relieve even Trichinomegaly, they're familiar, they're inexpensive. The cons are it's a lifelong
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commitment. They're depending on what you read. There's a three to 15 infection rate. And the obstruction risk doesn't stop accumulating. The versus an ETV, pros for that is that it tends to be
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safe, minimally invasive. Failures occur in the first six months. And after that, you can see the failure curve flatten out. And there's a near zero infection risk unless you have a leak, it
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avoids the shunt, which is a need for permanent, you know, a silastic. And the cons are, you know, there are some exclusion criteria, meaning, you know, not all people are eligible for
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ETV. The ventricles don't always come down. Oftentimes they don't, and rarely the stomach can close or scar over. But, you know, as we all know, Ben Worf did a great deal of work in the world
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of ETV CPC, looking at CSF diversion in kids under the age of two. And that is really, that work done in Kanjabi is really impacted how hydrocephalus is managed in North America. And our center is
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part of the 14 centers of the hydrocephalus clinical research network. And we're right now doing a study where we randomized patients ETVCPC or SHUNT between two under to see
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which one is the better long-term
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treatment plan. So anyway, those are just kind of things to think about in terms of after,
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you know, resection of a tumor. But the role of surgery is super important. You know, we like to say post-op day zero is the most important day. And extended EOR is extended resection, and
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that's the most important modifiable factor. And so here you can see operating room ongoing. And then pretty odd, weird looking tumor over here. But on the post-op scan, there's a little bit of
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residual here and a little bit of residual there. And depending on what that pathology is, you probably need to go back and take those things out. And you can do that. Some places have what's an
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intraoperative MRI. We do not. We do a scan on the first day and keep patients MPO in case we need to go back and take more out. But that is a very modifiable factor. And I think it's important
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thing to be able to get an image afterwards to be able to tell how things look. The role of surgery with medulloblastomas is basically maximal safer section. Same thing for high-grade gliomas.
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For
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a low-grade glioma like a polycytic astrocytoma or a palomixoid,
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the extended resections depending on location. So that means in the posterior fossa in general, we're able to get most of those, they're all of those out. But if they're hypothalamic or optic
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nerve type gliomas, then those are not off and able to. to be completely resected and therefore they're treated with chemotherapy or sometimes radiation therapy afterwards. But for a polycytic or a
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JPA, a complete resection equals cure. For an appendamoma, same thing, complete resection can equal cure. Often there's not really a chemotherapy that works right off the bat, so radiation will
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be done at least locally to that area. But when an appendamoma recurs, that's usually a bad sign. So you really try to get as most, the bottom line is your goal is to get as most of it out as you
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can safely. And I think that's no surprise to anyone. Here's just some examples that I had from a large midline medulloblastoma. You can see that is filling up the fourth ventricle, and you can
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imagine it hanging down from the superior medullary vellum where it has its origin. Here is
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a cystic tumor, which is more consistent with the JPA. Um, and that's right at the junction of the, um, of the middle cerebral, P-dunkle, uh, um, and the cerebellar P-dunkle, uh, and the
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lateral aspect of the fourth ventricle. So you're really kind of interperincomable there, almost in the brainstem more than it's in the cerebellum itself. Uh, here's an example of a JPA out, uh,
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further out within the cerebellar folia, which makes that operation a much simpler one, as you can imagine. Here's a vestibular schwannoma that can occur in kids.
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We didn't really talk about dermoids or epidermoids, but here's one, epidermoid in a child. And so, you can just kind of imagine, you know, some people think, oh, well, all approaches are all
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approaches for the posterior foster are the same. And that's what's really important to kind of get is that they're really not. There's all different of ways to get into the poster fossa and they're
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not all the same. And so, you know, this would be a midline incision that you'd have to make sure went, you know, far up enough to be able to,
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you know, at least do a Tilo Vilar death section so that you could resect this from a very midline approach. This is one where it's midline, but would probably hockey stick off to one side to allow
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you to maybe sneak into that through the folia without having to get too much into the, into the, you know, perincoma of the brain stem or cerebellar peduncle there. This is would be more of a
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lateral incision where you'd sneak in through the folia to take that out. This, of course, would be either a trans lab or a retro sigmoid. And, you know, this would be a TransLab, Transcochlear
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with.
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the, you know, resection to remove this and obviously keep the basilar and the rest of that brainstem intact. And so lots of different ways to get into the postdoc, post your faucet to deal with
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these things. But I like this to do list, you know, number one, take out the bad stuff. That's good.
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Number two, leave the good stuff in place and then try to minimize collateral damage on the way in and on the way out. I think that's a good checklist for us all to remember. So post-operatively,
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we like to
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extubate quickly and get an early neuro exam in the operating room to see how the kids see how they look and plan on getting the post-op MRI either on the first or second day after surgery. Blood
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pressure control usually try to keep this to solids less than 120. Q an hour neuro checks. And like I said MRI on post-op day one oftentimes we have people on steroids at least for a few days after
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surgery to just to help with any kind of a little bit of swelling here and there. And then like I mentioned, if they presented with a hydrocephalus, then they're going to have an EVD in at least
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for a few days.
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When we look at the MRI post-op, we want to see what the extended resection looks like. And I mentioned before, do we need to return to the OR or not? And then we want to see if there's any
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collateral damage, meaning is there hydrocephalus that's come on that wasn't expected before? Has there been unexpected bleeding into the tumor bed? Are there any areas of ischemia that have
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occurred or cortical collapse like you can see in that lower picture there? We'll make a decision about whether or not to continue them in the ICU or are sent them to the floor and that just depends
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on what their EVD status is. And then we have early mobilization with PT and OT one of the
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most frustrating things about. posterior fossa tumor resection is this concept of
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posterior fossa syndrome or cerebellar mutism. Um, and you know, the definition here, state of language impairment following resection of a PF brain tumor, you can see a taxi, a dysmetria
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irritability, emotional ability, often present. They can be sad. They can be happy very quickly. Cry out neurocognitive dysfunction out of proportion to their tumor therapy. Kind of the old
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concept was that it's a transient condition that resolves over weeks or months, but I think it's really believed now that it's something that persists along a longer spectrum of disease. You can see
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here a
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medulloblastoma in the midline, and then
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a curse seems to occur most commonly after midline tumor resections. You know, people have hypothesized if it's the, you know,
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You go through the vermis, as opposed to going to La Vilar. Is that it? Is it because the tumor is somehow involved in the middle cerebellar peduncle? Is it because somehow it's involved in the
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brainstem? Is there a difference between posterior fossa syndrome and brainstem injury from resecting it off the floor where you get a six and a seventh and the patient takes time to recover? We do
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know it's more frequently with inbronal tumors like medulloblastomas or ATRTs And more frequent and younger patients. And there's a lot of research that's gone into this.
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And we still struggle with it. It's still something that, you know, is seen and depending on what you read can be seen and anywhere of five to up to 20 of patients that undergo resection of a
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post-refaucet tumor. Unfortunately, there's really not an effective treatment. You know, luckily we have rehabilitation groups with speech OT and PT.
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There's not really a benefit to steroids, and maybe a SSRI or some type of medication can anecdotally help, but still it's a problem that we're still doing our best to avoid and then treating with
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basically intensive rehabilitation afterwards.
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How should we treat the patients after they get surgically resected? And that depends. If you look, there are probably the best ways to divide this out or based on standard risk and high risk. We
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are gonna talk about a little bit about the molecular aspect of it in a minute, but this has been the classic way to look at things which then determines as of now what type of adjuvant therapy
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people get. Standard risk is greater than three years of age with a gross total resection, no metastatic disease Um. and no, sorry, that should say no anoplastic histology. That gives you
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standard risk. And then high risk would be if there is greater than 15 centimeters cubed residual that you cannot go back and take surgically. If that's the case, you can treat that stereotypically
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or if there's Mets or if there's an anoplastic histology. And then of course, if you're very young, you're less than three years old, which means you can't get radiation therapy, then that
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changes things. And so for standard risk, the treatment, as you can see here is radiation to cranial spinal with a local posterior fossa boost and non-cycles of very intensive chemotherapy. For
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high risk, you can see that the cranial spinal radiation goes up with the same degree of boost. And then chemo is slightly different and a little stronger and with six cycles of chemotherapy And
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then if you're less than three years of age, uh, you know, we say the kitchen sink, you know, because you really try not to to give radiation therapy to kids this young, um, you can do, um,
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high dose chemotherapy with stem cell rescue to try to basically, you know, reset their bone marrow, um, and, uh, and give a great deal of chemotherapy There are some protocols now that involve
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stereotactic radio surgery with kids this young, and then for ATRTs, uh,
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COG has a protocol that the Children's Oncology Group that does involve radiation in some way, just because ATRTs are such a difficult tumor to treat Um, you know, so here is just a touch on, uh,
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this concept of the molecular aspect of, uh, of, uh, of, uh, of medulloblastomas. Um, you know, there are started off as four different subtypes and has gone up as high as 14 different
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subtypes. but the major subtypes to look at are WINT,
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WT, Sonic Hedgehog, Group 3, and Group 4. And you can see that also, most importantly, a gross total resection does have a substantial impact on outcome, but there are some types like that WINT
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and Sonic Hedgehog that have better outcomes, even if you don't have a full-on resection. And that's because of the nature of the molecular disease and its ability to be treated by chemotherapy.
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And I often wonder if people who don't live in the US know why that gene is called Sonic Hedgehog And it's actually because there is a video game made by Sega. years ago that involved this little
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creature, this little very fast-moving creature. And the person who discovered this particular this Sonic Hedgehog gene called it that because his son loved this game. So that's why this gene is
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named after Sonic Hedgehog, which actually makes no sense unless you know the background to it
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And I should have put a picture of the little guy up here. He's blue, he's fast. I don't know what it has to do with molecular biology, but there you go. This gets us lucky to have a smart dad.
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You can see that the therapeutic effect of cytoreductive surgery and irradiation of posture fossa appendamomas.
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You know, you can see here the progression-free survival with the growth total resection versus the subtotal resection. and then you can see the overall survival. So like we were saying before,
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resection, and again, this is talking about epinamomas, really matters in a substantial way, both progression-free survival and overall survival. And then what about for low-grade gliomas? So
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again, you can see, that's like JPA's or polemicsoid tumors. You can see gross total resection This is for progression-free survival, versus if there's less than 15, or what's a near total versus
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a subtotal. Now luckily, because JPA's are not highly malignant tumors, you can see the overall survival remains really good. But there has been progression within that time, which means the
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person then has to have either radiation or chemotherapy, which then affects their quality of life. Another reason, particularly in the posterior fossa to try to get out as many of these as much of
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this as possible.
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And then of course, this year for, if we've talked a little bit about medullos, we've talked about JPA's, we've talked about appendamomas,
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we have to talk about kind of malignant gliomas at the posterior fossa, which are very rare. And you can see just a profound difference in gross total convection versus subtotal resection here.
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You know, so, you know, I grew up in the era where we looked at
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normal risk versus high risk tumors. And when Dr. Dewan who trained with us here at Vanderbilt went off to Toronto and did his fellowship and became a mentee of Peter Dirks and that amazing group
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there, he came back and I made him make me a cheat sheet so that I could best understand. all the molecular issues because they're so complex. And at the end of the day, I think the things to
33:26
remember is that functionally most medulloblastoma subtypes, they're really three types, went sonic hedgehog and non-wint non-sonic hedgehog, which is group three and group four. And the wintz and
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the sonic hedgehog tends to have better outcome and the group threes and fours tend to have worse outcome. Most posturephosphopendomomas or PFA with histone alterations and most
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posturephosphate JPA's are BRAF fused, which means they're not mutated. And this is a
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much like completely boiled down version of what the complexity of the molecular aspects are. But I'm happy to provide anybody with the Michael Duan neural onk tip sheet for JW, which you see right
34:15
here. I'm happy to provide anybody with that
34:20
So, you know, here's that picture of me about the same age as the patient back in 2002 with the first tumor I took out. And yes, the six neuro palsy did get better. Thank you very much. But
34:32
there are many different types of tumors. The most important thing, and those different types, medulloblastoma, JPA, appendamoma, those are the most commons. And then you have the less common,
34:43
the malignic leomas, the ATRTs, and then the things that occur in the kind of the anterior part of the posterior fossa, like weird things like acoustic neuromas or in kids, or, and that's really
34:57
CP angle, or epidermoids. The most, you know, the thing that our job is, is to figure out how much to take out, and how much to take out safely. And that's always hard, you know? I mean, I'm
35:10
sure everybody's had that feeling as a tumor surgeon, which is, did I take out too much, did I take out not enough? And, you know, too much is. where the child wakes up with a deficit. Not
35:22
enough is the child wakes up perfect, but there's tumor left on the image. So it's really a razor's edge, like Somerset Mom wrote. It's really a razor's edge that we have to walk in order to
35:38
navigate the world of tumor resection surgery. But surgery comes with cost. And if you have a child that has an epinamoma and you know that that epinamoma needs to come out, and it looks like it's
35:52
partially in the brainstem, then you really have to make sure you speak with the parents up front and talk to them about, if we're gonna get this out, then
36:02
there is a chance of there being difficulty swallowing, there is a chance with there being, you know, some
36:10
cognitive issues afterwards. So these are things that you have to just be mindful of and really speak with the families beforehand.
36:18
PT, OT, speech therapy, rehab, those things are super important and, you know, as we get better with the treatment of this, not just radiation, but understanding how the molecular aspect of
36:33
tumor then relates to what chemotherapy the patient's going to get and how aggressive to be, rehab becomes more important. You know, we have
36:44
colleagues who believe that one day the treatment of a posterior faucet tumor will be to biopsy it and then see which type it is and then potentially treat that type with chemotherapy before any
36:59
resection occurs to see if any resection is even needed. Because if it's a wint or a sonic hedgehog, it may be more likely to be able to be shrunk or substantially treated with chemotherapy. So
36:60
it's an interesting time to be a tumor surgeon.
37:16
And there's a lot of things that are starting to move in terms of different types of chemotherapy agents. So, you know, our job again as the surgeon is to make sure the family understand, you know,
37:28
is to, is to, from a public health standpoint, I think it's to disseminate what our primary care doctors and pediatricians need to look for and understand what is a brain tumor and what symptoms
37:42
come with brain tumors It's then to be able to create an environment where we can get patients in and get their tumor resected. We have to have surgical skill in order to do that. We have to have
37:54
communicative ability to speak to the families about what to expect. And then we really need to have, you know, need to take care of these patients afterwards with physical therapy, OT, speech,
38:07
or at least a highly involved family they can help because rehab becomes more and more important.
38:15
being said, I'll say thank you from Nashville. It looks like this, but it hadn't looked like this in a long time, because it's been raining a lot. But I think the rains finally stopped. And so
38:28
I'd love to take any questions or hear the next presentation. But thank you so much, Estrada for asking me to speak today. It's nice to speak about
38:41
something other than the book. No, I love talking about the book. But it's really an honor to do this work to be quite frank. My dad was in the Air National Guard. And he retired a two star
38:57
general, he's passed away many years ago. But he always talked about how important it was that people be able to to be in the Air National Guard, but also equally as important were the people that
39:10
stayed home and, and, you know, stayed at work so that people could go be in the National Guard. And that's been something that I felt is important with my partner's really pivot and interest in
39:29
global neurosurgery is not just the visiting, and the learning, and the teaching, but also
39:37
the research piece. And I've felt like for a long time that my role here is to support them as a leader, but also to cover their patients, like my dad said about the National Guard so that they can
39:51
go do that work. So that's been my approach for a long time. And so now the opportunity to actually do a talk to you all in Sub-Saharan Africa is just really pleased for the opportunity to do it.
40:04
So thank you, Genestra, and it's nice to meet you all, and I'm happy to take any questions. Thank you so much, Jay I really appreciate it, you're nice for. presentation was great. I have one
40:17
question for you in talking about Meduloblastoma management. What are your thoughts about the concept of doing an ETV at the time of resection? Yeah. So Jay Riva Cameron, who was at Utah, who's
40:38
now at Calgary. He has a master's of public health and his entire basically thesis was on a scale that would help you predict whether or not somebody would benefit ETV an from upfront. And I think
40:56
that
40:58
it's not unreasonable. And I think it's not unreasonable in places that may not have the opportunity to manage external ventricular drains in a unit or in a setting, it's relatively straightforward.
41:13
procedure to do, and so I do think that it's a good option for people
41:22
to do upfront. We do not choose to do that. We choose to do that with, we choose to manage the hydrocephalus with the drain, but it does mean the patient stays in the hospital longer, and drains
41:33
do have complications So I think personally, if your choices are,
41:41
for whatever reason, you can't put a drain in, and your treatment would be to take out the tumor and then close everything up and hope that hydrocephalus didn't occur, that
41:57
the sequela, just having when I arrived here seeing so many tumors be taken out, go home on post-op day two or three, come back on post-op day seven leak and CSF out of their incision, require an
42:08
EVD to make sure it wasn't infected and then require a shunt. three or four, you know, it's like four operations. And so I think doing an ETV up front, if that's your choice to, if you, for
42:24
whatever reason, can't have an EVD after surgery because of maybe a lack of nursing or a lack of unit, I would rather see an ETV done up front than not anything done with the risk of coming back,
42:36
leaking CSF and all the sequelae that comes from that. Okay, well, let me take it one step further All right. What about doing an ETV, putting in an external ventricular drain, doing the
42:49
resection and then managing them pose up. My
42:55
concept is just looking towards reducing the need, the probability of requiring permanent CSF diversion with a shunt. Yeah. Well, we,
43:10
yeah, I mean, I think that's very reasonable.
43:13
been able to reduce our, as I was mentioning before, the rates were really high of shunt placement. So we've been able to reduce ours pretty substantially by EVD
43:30
alone and have published that. We've not added an ETV upfront to that. The
43:38
issue is that, and the reason why I think we've not pivoted to that, Estrada, is that
43:46
most of the time when you have hydrocephalus, it's not there because of an obstructive issue post-op. You've taken that tumor out. If
43:58
you look at the ETV success score, just the presence of obstruction alone really raises your percentage of it working if there's obstruction that's present. if it's a non-obstructive etiology, then
44:15
that lowers your chance of it working. And so I think that's the reason why we just roll on to the OR and put the EVD in and take out the tumor and then see what's small proportion afterward ends up
44:28
needing a shunt or ends up needing some type of CSF diversion. Because in general, based on ETV success scores, we would go on and do a shunt if there's not any obvious area of obstruction of
44:40
structured hydrocephalus But it's a great point. And maybe we could use J. River Cameron's predictive scale and do an ETV at the same time and then put the EVD in and do surgery and see if that
44:55
reduces. So it's a great idea for sure. Particularly, I think it's a great idea for places that may not be able to take care of
45:05
EVDs for three or four days after surgery, if there's a different general, all that, yeah.
45:13
I mean, let's say I'm in in most of the world, which is the low to middle income country and I can't do any genetic testing on the tumors. What do I do?
45:27
Well, I think as the surgeon, you take out the tumor as best as you can and you treat it based on if it's a medulla blastoma, you talk to your pediatric, if there's an oncologist there, and you
45:42
treat it based on that. The other option is to get in touch with some of these larger Michael DeWann sub-Saharan African research group and get accessed by sending tissue off, but you also have to,
45:58
and it depends on the resources of your particular community, are you able to do radiation therapy? Are you able to do chemotherapy there? Because if not, then the next step is to get that. get
46:10
that person to a place that can do those things. But there are ways now to be able to send tissue out and around to get it looked at and get the molecular subtype done. So I think you're most
46:26
important job as a surgeon at a place that doesn't have a lot of resources is to focus on what you can do. What can I do? I can communicate with the family that there's a lot of risk here and what
46:36
the expectations are after surgery And then I can do as good of a job as I can and taking this tumor out, getting as much of it out safely. And then I can spend my time making sure on rounds
46:48
afterwards that the patient's cared for as best as possible. I think those are the main things that you can do. And then everything else is an ask for help. If you have somebody there who can do
46:58
that, who can do the chemotherapy radiation, great, but you need to send the molecular stuff out, you can do that. And there are ways to do that. And I think Michael DeWann and a lot of other
47:08
places collaborations that would be able to make that work. Sam, do you have any thoughts about anything you want to add? Thank you, first, I want to thank Jay for the great talk, always great
47:23
to learn from you.
47:26
From the limited time I spent with colleagues in different parts in Africa, every time I go, I feel that the field of pediatric brain tumors should be centralized. And one of the examples are there
47:42
are two examples that have fun to be very successful. One in South Africa, the other one is in Egypt. Egypt has a population of close to 100 million people and 90 of pediatric brain tumors go to
47:57
one hospital in Cairo, children's cancer hospital. And 90, 100 of the tumors Same thing in Africa, most of the tumors go to Cape Town or Johannesburg. Victoria. And in many other parts in Africa,
48:13
I wonder if centralised healthcare when it comes to pediatric brain tumors will give the kids the best outcome possible. I do remember very well working with a great friend, John Mugamba, whose
48:27
friend to many of you, when I was at Cure Children's Hospital in Bali, Uganda, I went there to learn from the best and ECV CPC. But while I was there, two brain tumors came in. One of them was a
48:42
highly vascularized meningioma in a 10-year-old child. And the whole hospital had one unit of blood. And luckily things went well with limited resources. The other case was actually an embryonic
48:56
tumor, a middle blastoma. And
48:58
I'd love to question earlier, Professor Osman, about if there's a way for molecular profiling to help the surgeons decide. I do believe in some parts around the world, if we know upfront this
49:12
middle blastoma is going to be grade three or four, probably the surgery should not go through maximal resection if we do not have nearby guaranteed adjuvant therapy to follow. And I wonder how Jay
49:29
feels about
49:31
that. If there's a way we can do spot testing in the future, and I know some people working on it, where intraoperatively you can find out years from now that this is a group three or group four,
49:44
now the WNT or sonic hedgehog, will you as a surgeon go for maximal resection with the high possibility of post-optimal morbidity requiring longer rehabilitation? If you know deep in your heart,
49:58
this child will not receive the best adjuvant therapy Alvin is only neurosurgeon in Liberia. Alvin, how does this,
50:10
what do you think about all this? Is this practical?
50:15
Thank you. And I like the questions that I think it was the last question. It was not really a question. It was a statement that the presenter made regarding about wherever
50:30
you find yourself with what you have, you make use of what you have Like now, in Liberia, most of these cases, what we normally do is to put in, because most of these children, they come in at
50:45
the late stage. They are really sick when they present. So when they come in, in order to prepare for them, for their surgeries, we usually
50:57
shun to them. When we shun them, that is to do a VP shun, because we do not have a lot of EVD, We sum them, and most of them, they improve clinically. and we have the time to discuss with the
51:09
parents for two more rejections. But what we have observed in doing that, most of the patients that usually come with lesions of the postural cranial force that are in the midline, right after the
51:26
shunt, that
51:29
conditions do not improve, but rather those with lateral lesions, they're in the hemisphere, they usually improve right after the VP shunt. So I think in the results limited certain, I think what
51:46
we do is since we have a couple of VP shunt, we usually wear various acute super contoural hydrocephalus, we
52:01
usually place in the VP shunt and then later on discuss with the relatives.
52:09
on tumor remover. So that is what we usually do here. And now regarding radiation,
52:16
now we have pathologies in country that I can do
52:21
histopathology.
52:24
But the limitation we have is regarding radiation. And most of the patients, their relatives, can I afford to have them evacuated to Ghana or Nigeria? So most often, surgery is done for them.
52:40
And after three or six months, tumor reoccur and the next thing your year, they die. So it is among the many challenges we face in this part of the world and LaBira to be specific. Thank you. Ben,
52:57
do you see a lot of cases in Kenya and you're in
53:01
the pediatric center? Any thoughts you have about this?
53:07
Yeah, thank you. Thank you for the presentation, Dr. Jayo Manle. I think we see a lot of these cases and a lot of the things we
53:22
talked about are quite similar.
53:25
I think one of the things I would pick out is how we manage hydrosoftness. Yeah, there has been water, I would say, is a shift in the practice of the management of hydrosoftness in children with
53:40
posterior post-achumas. And you find that
53:43
initially and in my early ideas of training, you find that
53:50
most of the children were shunted. You know, before you would offer them suddenly for a section of the pre-achumas. But now we
54:06
really try to avoid placement of the patients And what we do is that we.
54:10
There are key references and charts associated with this talk. We suggest that you take screenshots for your records and for further information in the
54:25
Dr. Wellins references can be found in two sources, one on the, on PubMed
54:34
and secondly in a book that he published is memoir, all that moves us, reflecting on his experience operating in children facing critical illnesses and injuries. Completely boiled down version of
54:49
what the complexity of the of the molecular aspects are. But I'm happy to provide anybody with the Michael Dewan, neuro on tip sheet for JW, which you see right here. I'm happy to provide anybody
55:02
with that because
55:06
Dr. Osmond has added some comments on this presentation.
55:14
There were three interesting observations in this presentation from Memphis Hospital in Enugo, Nigeria. One is the slide which showed the ethnic diversity and the occurrence of tumors globally.
55:31
What does that tell us? It's a genetically based distribution of this disease or it's just a sampling effect.
55:41
Secondly was the change in histology of the tumor they operated on three times initially from a neurocytoma
55:51
which then became a medulla blastoma and then after the second operation the patient developed a meningitis and then came back for a recurrence and the tumor was a neurocytoma. There are a number of
56:06
explanations for this which we'll detail on the next slide. The last point is to discuss when there's enough surgery, enough.
56:19
What is your thinking about this question?
56:25
Dr. Osmond indicates that there is an abscuple effect on tumors, it's an effect referring to a phenomenon where the localized radiation treatment not only shrinks the tumor, but leads to shrinkage
56:40
one treated tumors elsewhere in the body, likely due to an immune response. This effect has been observed in various cancers and is thought to be enhanced when combined with immunotherapy.
56:56
The other observation has occurred and many watching this program have had the experience where after an infection,
57:07
the tumor can be suppressed by activation of the immune system. There are other possibilities or it could have just been a change in histology.
57:21
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57:34
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