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2018 AANS Annual Scientific Meeting
Scientific Session IV: Pediatrics, Question and An ...
Scientific Session IV: Pediatrics, Question and Answer VI
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We'll now invite any questions you have for either of our speakers. Did we scare you all away? Yeah. As far as the Queen Infant Joma and what our today's plan, try to get a GTR or try to maximum resection and get radiotherapy and what's the long term quality of life of the child, especially in the cry, Herman, follow up the Queen Infant Joma. What did he ask? Can you repeat that? I'm sorry. First is Queen Infant Joma. Our surgical plan, we try to get a gross total resection of Queen Infant Joma and sometimes difficult and sometimes we try to leave some residue and get radiotherapy because we try to preserve maybe the hormone or something. The approach is we take the craniofringeal medorbazygomatic. I use a two-thirds, what's called a two-thirds Fukushima FRAP. We take the muscle down on block. I always take out the anterior clinoid extradurally. I take down the doropropia extradurally. I'll usually drill off the optic nerve, free that up, take the optic strut. Once we get inside, that allows for mobilization of the optic nerve where you get that seven degrees and you can actually look underneath. I used to also do some work a little more posteriorly, which I get Hunt Batry's call a half and half approach. I don't do that any longer. I don't see any utility in that. I opened up Pernetsky's ring around the proximal carotid and I think that gives us the best look. I think that's the best way to get a good resection of craniofringioma. On the downside, it's extraordinarily rare that we preserve pituitary function. I would say that preserving pituitary function from any approach is probably a challenge. I think certainly from the endonasal approach, one of the things that you so frequently appreciate is that even when you have a totally normal pituitary sitting in the cella, the entire stock is nothing but tumor. If you make that decision, and this is a conversation Jeff Wissoff and I had when I first started, if you make that decision, you're going to peel the tumor out and leave those few strands of stock left. Often you end up with no pituitary function and you often leave an itis for your recurrence. I think one of the parts of the question you were talking about was gross total versus subtotal and adjuvant therapy. I think one of the things that we very much embrace is that if we can't safely take the whole thing out, and by that we're really talking about preserving hypothalamus, talking about preserving optic nerves, and we're talking about occasionally we'll see these really caked into the posterior fossa vessels, and that sort of happens to do with the referrals of what we see. If you can't, then absolutely I think that the right answer is to preserve that function, particularly hypothalamic function, and opt for adjuvant therapy. I think even in the setting, I think there are some people who have been looking at doing what looks like a gross total and still doing adjuvant therapy. I don't know that the answer is clear yet whether or not that's beneficial or not. Thank you. Great discussion. Elizabeth, a question for you. In one of the slides you showed that trans-nasally your kids are in the hospital for only three days. So either we are all, I mean I'm certainly not as good a surgeon as you are, but how do you manage all the pituitary problems in three days? So if I look at that, there's a skew there, and what I really need to do is go break that out between the fresh cranios and the redo. We do a lot of redo craniopharyngiomas, whether they are sent to us from other institutions. And when those kids come in already well established on their endocrine medications, typically I have them back on all of their endocrine medications in less than 48 hours, and they're out of the hospital on day three or four. Certainly if we look at the patients who are not, who are fresh patients, those patients are going to have a longer stay. But I think if we were, if I'm really honest, we're definitely skewed to more of the redo. Also those particular numbers are skewed to our later cases where we had full financial data. So that doesn't really look at a good cross-section of our craniopharyngioma length of stay for all comers over the last 15 years. So my second question is, and I think this comes down to resection versus using adjuvant treatment. And, you know, what are the consequences of giving a two-year-old child severe endocrinopathy and then managing them with adjuvant replacement medications? Is that worth it? Is it better to do a conservative approach, let them grow up, use adjuvant radiation? I don't know. I mean, this is, look, I love doing this operation. We all love doing this operation. And, I mean, I'm the one who does all this in my institution. And each time I do it and I have to put a pause on all that and say, do I do it or not? So I will say that, you know, this is, I think certainly this is one of my questions in that, you know, we have been very, very protective of the hypothalamus. And the endocrinologists I work with assure me that we've definitely seen a change in the hypothalamic injury. When I go to my endocrinologist and ask them about not operating to preserve pituitary function until the kids are older, most of the time they're telling me, no, go ahead, we can manage hormone replacement. I think that really the long-term quality of life is going to be the answer, and that's going to take us a while to answer. You don't have any data on that, right? Not yet. We're working on it. My worry is about schooling, intellectual outcome. This is a great operation. I love it. Yeah, the tumors are extraordinarily difficult, especially the large ones. We tend to see some large ones because we – No, I mean the large tumors, which have already done the damage. And the reason we go for complete resection is because where I usually hurt a person is with the recurrence. Thank you. So what's the current status of interferon? Oh, well, let's see. Reggie Joukaki, who was my great champion, is no longer in clinical practice. And the formulation that we had available was then taken off the market. So I know a few families that are doing their – have pharmacies that will dilute it to the appropriate dose, so I do know a few families that are still on it. But I have a whole new set of oncologists who are not very excited about it, so I'll have to see where we end up with this. I'm going to get into a little bit more of our experience on that. We use interferon, you know, where we have a lot of these collapses in a small area. And, you know, the results are that you can delay the intervention. The best data is Sergio Cavallaro's international study. It's basically at 18 months, one-third of the patients will have stable disease on intracystic interferon. It's a tool. I'd like to offer a couple of philosophic comments. First, I thought it was interesting that this was less a point-counterpoint than a meeting of the minds somewhere in the middle, which I think is truly what's happening with the endoscopic and open procedures in these very challenging tumors in kids. I agree that the end stage of what we're trying to do is the quality of life in a child 10, 20, and 30 years out. And I would just like to make a plea that we don't have the answer. We don't have it with surgery, and we definitely do not have it with a partial resection, irradiation, particularly for craniopharyngiomas. We have very little outcome data. And for those of you who are younger, that's anyone other than myself and Mike Scott. And Derek, excuse me. I would urge you, look at your historical series. We're never going to run a randomized trial of these patients. The numbers are simply not there for a national study, and an international thing is difficult because of socioeconomic concerns. But look at your studies. Try to do case cohort analyses. Look at the 20-, 30-year outcomes for those of you who do have access to that data. I think that's critical, and that's a huge gap in our knowledge base. Elizabeth, can I ask you about that pericranial flap that you talked about and how helpful it was? Exactly how do you do that? So we do do it with an incision behind the hairline to raise the pericranial flap. Like typical? Like typical. And then because you've already brought it down, basically you drill a hole into the frontal sinus so that there is essentially a male slot that you can pull it through, and you leave that so that that is your vascularized pedicle. Then you come back through the nose and you pull it down and basically just take it and drape it all the way down. But the opening you do it through is where? So the actual bony opening. So the incision to raise the flap is behind the hairline, and then you just make a bony opening across here just so that you can get into the frontal sinus. Or in a patient who doesn't have a frontal sinus, we use image guidance to make sure we're going to open into the nasal cavity and not into the anterior fossa. Do you have an incision on the nasion? No, because with the bicoronal incision, between that and coming from the nose, you can make that connection. I have a question about the low clivus lesions. Yes. Because doing these open, it's very, very complex anatomy because there are multiple ligaments, et cetera, and you have frequent little pockets of tumor all over the place. And I'm interested as to can you handle that with the endoscope? So we actually have been very comfortable doing that. I mean, the one you showed us was a nicer section, but is that the general setting? As you know, these low cardomas are the hard ones. Yes. Almost anywhere else are easy. So one of the things that I would say is that we do the bony landmarks ahead of time to make sure we're really going to be able to get as low as we wish to be able to get. We often, in that particular case, we were actually able to preserve the apical ligament was probably gone, but the transverse ligament was intact because we actually had little pockets of tumor that sort of stuck down in between everything. And you are able, I mean, you have to agree, the anatomy is difficult. You have to be comfortable with that anatomy, and then you can work with it. If we are concerned we've destabilized them, I just go ahead and put them in a halo and I fuse them either the next day or a couple of days later. If we feel like we were able to successfully preserve the ligaments, then we'll come back. The other thing that I will tell you is for the chordomas, particularly those low ones, and actually probably most of them, I always book them for two days of surgery, and we do a post-op MRI, and we look at that post-op MRI, and if there is a knuckle that we are worried about, we will go back and look. And actually we are hoping that our intra-op MRI will go live in June, and I will do all of our chordomas with the intra-op MRI so that instead of booking them for two days, I do my last scan and go back and look at that one little knuckle. Because what I found with these ones, the bits that went into the dura, that at least with the open you could usually tease that out and then just put little clips on the dura like you would a zip, and that would take care of the leak without having to do much else. Anastoclips. Yeah. As you can see, we fused the first patient. Elizabeth, I'm interested that that first case you showed, the lack of pneumatization of the sphenoid did not deter you from an endonasal approach. I found that if they really don't have any pneumatization, not only have you lost your landmarks and rely solely on navigation, but there's so limited working space it just becomes such a struggle to do those. So it's funny that you say that because my two adult partners, when they come over, will take a look at the 19, in particular that 19-month-old and some of these other kids with growth hormone deficiency that are very small, and they all go, how are we going to do this? They're so tiny. But I always remind them that they're used to working through a wide, long tube, and their motion is very limited. When we do these little kids, it's actually a narrow tube, but it's very short, so it almost becomes working with a fulcrum. And actually, if you kind of take it out of your head that you don't have any room, you have tons of room. You just have to sort of think differently. We find that as you do the drilling, when you don't have these pneumatized sinuses, it really is almost like we create the sinus because we will skeletonize the whole cell. We'll do the same thing where we come all the way out to the carotids. And so before we actually open through the bone into the intracranial compartment, it looks like they have a sphenoid sinus. And I think probably part of the reason we have adopted that technique is it becomes a constant reminder of exactly where the anatomy is. I would say with the really little kids, actually the biggest difficulty is that sometimes those carotids are so close together that now that's where your narrow working space is, not so much the narrows are so small. All right, I think that concludes the session. Thanks to all the speakers and the organizers. Enjoy your travels.
Video Summary
In this video, two speakers discuss the surgical plan for treating Queen Infant Joma, a type of brain tumor, and the long-term quality of life for the child. They discuss the various approaches and techniques used during surgery, such as the craniofringeal medorbazygomatic approach and the use of a pericranial flap. They also touch upon the challenges of preserving pituitary function and the use of adjuvant therapy in cases where complete resection is not possible. The speakers also address questions from the audience regarding the length of hospital stay, the management of pituitary problems after surgery, and the use of interferon in treatment. The discussion concludes with a plea for more long-term outcome data and research in this field.
Asset Caption
Scientific Session IV: Pediatrics, Question and Answer VI
Keywords
brain tumor
surgical plan
long-term quality of life
pituitary function
adjuvant therapy
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