Bilateral Wilm's Tumor - Complex Gastroschisis - Complex Ileal Atresia:...

So next we're going to move on to a new person in a new format, so. Doctor Tony Sandler is gonna talk to us about several different topics, but about only 5 minutes on each one. So we do this rapid fire. So I'm asking the faculty here if we go around, just give a short answer. If you have an explanation, go brief because we wanna get through what, what the opinions are of the faculty here. So go ahead, Tony. Thank you very much, Todd. It's, it's great to be here. Um, this is the best, one of the best parts is I'm learning more than I'm actually gonna teach today. Uh, but it's, uh, yeah, I congratulate you for putting on an incredible symposium, uh, for the rapid fire session, uh, a child presents who's 5 years old with what I call giant bilateral kidney tumors, almost kissing in the midline, uh, and on CT scan, uh, there's positive lymph nodes that are large, as well as a lung metastasis. Uh, this is what the tumor looks like on the MRI scan. This is the left sided tumor, as you can see, it's a, it's a very large tumor encompassing. Uh, 3 quarters of the abdomen and then on the right side you've got 2 tumor lesions on the right kidney. So my question to the, to the group is, uh, what's your next step? Do you uh biopsy this? Do you try and resect it, or do you just start chemotherapy? That's that's chemotherapy. Any other opinions? So no one here would biopsy it. So, correct, uh, the, the current status is just to start chemotherapy. Probably 2 cycles get shrinkage, and when you stop seeing any shrinkage, and when we speak about shrinkage, you're talking about a decrease in 50% of tumor size. Well, this child stopped shrinking, and at this size, after 2 cycles, there was no further shrinkage. Uh, and you have what, what you can see on residual, the very still large left renal tumor. And on the right side, you can see the, on the right side, you can see the two tumors in this area over here. So, what is our, our next step here? I ask the panel, is there, uh, what would you do here? Would you, would you go in and resect both tumors? Would you remove the left kidney and try do nephron sparing surgery on the right kidney? Um, or would you continue with chemotherapy? Oh I heard a hm. And that was me. So I want Dan to answer this because he knows this much better. Would you, Dan, would you, would you remove the primary and, and, I mean, the left side and do nephron sparing on the right? Or would you, is that what approach would you take? So the concept, obviously with chemo, right? So the concept is obviously to make sure that you preserve as much kidney tissue as possible and without just seeing that one cut, don't know whether you could do nephron sparing surgery. It certainly doesn't look like you could do it on the central one, although you might be able to. The other issue is whether if you change to a more aggressive chemotherapy regimen, if there are, uh, because it has not been biopsied, if there are anaplastic elements in there that would respond with a more aggressive chemotherapy regimen, then you might move more toward being able to preserve kidney tissue. So I think if you can't do nephron sparing tissue, I would biopsy it and make. The problem with biopsy is they're heterogeneous tumors and you might miss foci of of um. Um, anapplasia, so some would argue just, just change the chemotherapy regimen to something more aggressive. What about taking out the left because that's Doesn't look too promising and using that as your biopsy. Um, to take out the lift and leave the just leave the right, so take out the left, find out if there are any elements that would guide your therapy and hope that you can further shrink the right to allow for nephron sparing. Uh, you could do that. I um. Um, I suspect that if it was only 2 cycles of chemotherapy, we'd probably change to a different chemotherapy regimen. It's hard to know that it's stopped responding after 2 rounds though, so it's um. So, so in general, what we, what we do is, uh, if it stops shrinking, and because most of these are favorable histology Wilms tumor, we will then approach a surgical intervention. Um, when they often stop shrinking, it's usually because of mesenchymal differentiation and not so much an anaplastic tumor, but Dan makes a good point that there could be a, could be a heterogeneous tumor with different phenotypes. So what we do is we always try to do bilateral nephron sparing, and most of the time, surprisingly, you can actually do it. And what we do, our, our technique is, is actually to put the kidneys on ice, especially the left side, clamp the vessels, and then the sharp dissection, uh, remove the tumor, and it's quite, quite interesting, and we've got a, quite a big series now because we have, uh, uh, Jeff Dome who's sort of the head of the Worm's tumor Study Group in, in Washington DC, uh, but it's quite surprising how you can save this. So that's what we did. We saved both sides, uh, and did the left upper pole, and this is the pathology that came back that you can see on the screen. Uh, left upper pole was favorable wombs. The left lower pole was anaplastic. The right tumors were both favorable, and the lymph node had anaplastic tumor. So now that you know that there was some anaplastic tumor in the left side, uh, what is your next, your next stage? What do you do? Do you just keep going with changing chemotherapy? The kids, the child is gonna get radiation on both sides. Either way, because this is rarely stage 3 local tumor, although this was a stage 4. So what, what would your, what would anyone, uh, recommend doing, uh, at this point? Let me, let me cut to the chase. I was away. I'd left town because I never like to stick around for post-op complications. I, I'm that's a joke, sorry. Um, I, I, uh, I actually, uh, went away about 4 days later and in the 5th or 6th day, the pathology came back as anaplastic. I was pretty confident that we got the tumor that looked like gross tumor out and, uh, but the, our oncologist felt that because there was anaplastic differentiation, it was necessary to go get the left side. I argued that the lymph node had anaplastic tumor, so it was really outside, and we did the nephron sparring. Nevertheless, one of my partners went back, I did the left kidney, and there was no tumor in the remaining left kidney, neither favorable wilms nor anaplastic tumor. Nothing they identified, right? How do you know for sure there wasn't? Well, they, they do section through the tumor. They gotta look a lot. You have to look a lot. That's very true, because recurrence for an anaplastic recurrence would be a very, would portend a very bad outcome. This is true. Salvage is difficult, especially with an anaplastic tumor, despite the chemotherapy. This is true. So I think in general, although we have one now, uh, who does have some anaplastic elements, but it's in the central part of the tumor that I removed. And so I think it would be appropriate, we'll see, not to go back and take it out. But I think that was a, that was a reasonable thing to do. Any comments on that case, Dan, do you, would you have done things differently? Um, I don't think so. I, again, you have to go with the philosophy that you're doing the best you can to preserve renal tissue when you have bilateral tumors, and that's why we no longer biopsy them upfront. They're treated. If they don't respond, and Bob Shamberger's made the point that if you have a tumor that's not responding, then you do need tissue to make sure it's not got anaplasia in it. I think when you take the tumors out, you preserve both kidneys, which is, you know, a tribute to the technical experience that you all have. But you get anapplasia, then it really becomes a question because, as I said, once you get a recurrence, it's very hard to salvage that job. One of the issues is you don't really know, and this is a debate and an argument from a pathologist's point of view. Was, was the anaplasia there initially or secondary chemotherapy, did it differentiate into an anaplastic phenotype? I think most pathologists think that the anaplasia was there primarily, so you have really a heterogeneous type of tumor. You also have multifocal tumors, which is also another poor, you know, I think. And I can't quote you the data on it. You may have it, but for multifocal tumors, you would worry about the underlying embryology of that kidney and that you're going to be at risk for developing additional tumors. And certainly if there's mutations in WT1, WT2. So I do want to, I do want to say that the two highlights, and this is, this is what the child looks like now, the left kidney is gone and no residual tumor in the right kidney. Another child, just briefly before I sum up, a 12-month-old child who presented from, from the Middle East with these large tumors after chemotherapy that failed to decrease in size. Uh, we resected them both and saved kidney on both sides, and 7 months later you can see there's really good kidney on both sides with some nephrogenic risks that we are continuing to follow but are unchanged. So, so the message from this is for large bilateral tumors. Um, nephron sparing surgery, you don't need chemotherapy upfront because 97% of these cases are Wilms tumor and there's only a, a very small percentage of cases that are not Wilm's tumor. OK, great. Next, gastroschisis. So a child presents with gastroschisis inflamed bowel. If I can caucus the, the panel here, how would you, how would you manage this child? So, uh, I would try to reduce at the bedside, um, if I couldn't, I would put it in a silo, and if I could, I would use your technique of reducing it and, uh, putting tape over it. Can, can we get the slides? Intimated or not This child's not intubated. No, I'm asking time. So I know he talks about not intubating. I've tried it and I haven't been successful, so I always intubate. So, uh, doing rectal Tylenol, a tiny bit of fentanyl, silo for a couple of days, and then tape closure without you silo, even if you could reduce it, you still sigh, um, because I'd like to tape them closed. So I mean if it's very little bow out and it's doable, I would try, but Everyone so far has been silent pretty good argument, Todd, that that would be that one's going to be difficult to reduce. They look like, you know, you said very little ball out, though I actually think I've repaired surgically, maybe one or two in the last 8 years, because I do them all this way, um, and so even when they're all out, I'm, I would say 80% successful at getting it in. The question is, should I be pushing, like, is it, I'm pushing it in and it's tight. I make sure that their peak airway pressures aren't high. I know that uh you measure bladder pressures. I don't, I, I. I maybe I push it in too tight, but I can almost always get them in. The question is, should I? What do you, what does everyone else do? Hm At least in my practice, uh, these children are being born early, and I, I do not see this thick matted bowel as much. The, the perinatal, you know, high risk people that see them are tending to deliver them for whatever criteria, um, that they use. You say early. How early? Well, I'm saying before I see the bowel matted, and, um, they'll take them in and they will do C-sections despite, you know, discussions that doesn't need to be done. But that's the way they manage them, um, so at least the vast majority. As a result, I'll see a lot of bow out, but it won't be thick and matted. I treat them though, you know, by trying to do it at the bedside. Um, I have the babies intubated and paralyzed. No, I put a stitch in the fascia and put a leave a little bit of cord hanging out as a little kind of biological patch or whatever, and I put an opsite on it, take the drapes down, as long as the peak pressures aren't very high and there's no differential cyanosis, I'll watch repair. Basically, and I do this, um, you know, and we'll, we'll keep him paralyzed for a day or two, and that's it. Any other Comments, what do you guys do? rapid fire. It seems that most of the ones that I've got so far are all thick and matted, and so I've gone with a silo and reduced it. So thick and matted determines what you do, yeah. OK. I think unless easily reducible, we place them in a silo, a serial reduction. And we've now converted over to the Sandler style of closure primarily, although we have a decent number of large umbilical hernias to repair. My question how many hernias do you get afterwards that you have to fix? We can discuss that, but we haven't fixed any in the last 6 years. Initially in the first series, we reported 60% of the patients got umbilical hernias, but most of them closed. If you cut the fascia, they will not close. You will have a defect. So if you don't have to cut any fascia to widen the thing. I don't remember fixing. So I treat them as umbilical hernia. So you wait. How long do you wait until you cry mercy? 34 years, 5 years. So it seems to be in our country that either you take the baby to the operating room and try to get it all in, or you do a bedside silo. So we've got a trial going on looking at those two arms. It's, uh, it's prospective randomized with putting a silo on in the delivery room, uh, versus the other randomization sequences taking the patient to the operating room and trying to put everything in. And if you can't get it all in, then you put the silo on and then what do you do? You can actually put it in the in the OR? Well, that's, that can all be a difference of opinion, but that's the, that's the way immediate closure versus silo, whether you do surgical repair or tape because I think you can go up to the bedside and see if it goes in, and then if it goes in, it's in, and then you just close it right there. If it doesn't go in, you pop on a silo, right? That's what I do. We do it all at the bedside. Agreed. OK, um, I have a com a quick comment is that who uses the spring loaded Bentech silos here? Does anyone use those? You know, it's interesting, those I love, but if you squeeze down the, the physics, the, the pressures, the forces are going out. Because it's squishing down here, the ring is here, so the forces, I think it makes the hole bigger. All right, I've got some, I have some nice slides to show that put them in. You must, you must have gone over this before. I set you up. I set you up. Thank you. So, so, so we, we generally do it this way. We exactly as as spoken about, we reduce the bowel if we can. We put it in and we just put a tegader over. I used to put Betadine on and all these things. You don't need to do anything like that anymore. Uh, and I usually leave the Tegaderm on for about 3 days. When things are stuck, I convert it to a dry dressing, and these are some examples. You can actually get quite inflamed meconium stained bile back in the abdomen. We don't want to be too aggressive, and so I think this technique is also used for the child who needs a silo. And so you, you put the silo in first, you put the get the bile back, and then just cover, cover the umbilicus with the Tegaderm until it's stuck down there. Um, just, just to show you the extent, and this is to Todd's point, uh, some of them are really big and you, and you struggle and you push the silo in, and then what happens is, uh, the silo doesn't stay in anymore because your, your, your defect dilates up. And so in my more youthful days, I said, well, let's let's push the limits here and and see what happens. And so we actually just put Tegodome over this defect that you can now see we couldn't keep the silo in anymore and at 3 weeks. 4 weeks, and 8 weeks, this is the outcome. We usually start feeding when bowel function returns. We don't wait for complete closure, but it's just to show you that even though you dilate the defect up, this technique can still work by cicaterization of the umbilical defect. All right, so, more complex gastroschisis, how do we handle that? This is a child who potentially has a atresia, uh, with a dilated segment of bowel. Uh, what, what do you do for these cases? What does the, the group feel, uh, is the appropriate management? The bowel looks pretty good. I'll say that up front, and you have an atresia. Well, if you've identified an atresia, uh, I have in the past done this 2 or 3 times, and the bowel is not mad at its pristine, then I have done an ostomy at the site of the and brought it out of the umbilicus on a temporary basis with the downstream side tacked right next to it. Wait a few weeks, 2 or 33 or 4, whatever you want. Uh, and then go back, core out the, the, um, ostomy at the site of the umbilicus, put it together, and then close the ostomy, and that's worked very nicely in a few select cases, but you don't want to do that if it's matted bowel, it's thick inflamed bowel. If that's the case, then you ought to just drop it back in the abdominal cavity, let the inflammation resolve and come back in 4 weeks, 6 weeks, some period of time like that. I've done a similar thing, uh, similar management, although I was not smart enough to bring it out through the umboli because I brought it out through a, you know, a right lower quadrannostomy site, and Brad Warner accused me of just transposing the gastroschisis. Yeah, the, the, the, the fascial ring is a very nice place to sew the bowel. The bow is usually quite big. The it's about the size of the fascial ring, so I've just sewn it to the fascia ring. If it's really clean bowel, would anyone fix it? Just fix it and put it back in. I mean, since my algorithm, I, I, I've done it, but I've regretted it. No, no, in a, in a 1 again, in maybe 2. It's, it's doable, but, uh, I like the ostomy idea, yeah, because my, my algorithm. I like I, I, I don't like the idea of the ostomy next to the gastroschisis. Obviously it, it can work and it sounds like a good option. I may consider if it comes up, but my algorithm is, if it's, if it's fixable, if the bowel looks pristine, go ahead and fix it right then and there. But if anything else, just put it back in and wait, and I've never had any problems waiting. The thing is that they are within aresia, the bowel is so big proximally and so decompressed distally that you're doing a. Very tenuous and tedious anastomosis. In an air bowel that's been outside and so you worry about the quality of the anastomosis and for that reason, the ostomy is an attractive idea, about a 5 millimeter stapled anastomosis. I would agree with that, yeah, weight limit that you would consider a primary ostomy, uh, primary anastomosis? What's the volume on the computer? I knew you'd say that. So I, so I would agree with most, most things spoken here. Um, if you can tell that there's an atresia, you can often, it's often pristine bowel. Most of the time with gastroschisis, when you're unsure where there's a resia, it's inflamed and you don't know what's going on, and that's obviously the situation, you put it back. If you can clearly see there's a resia, I do repair it and if necessary, and, and I think one of the benefits of just putting it back and not closing the fascia, the ambient pressure in the abdomen is a little less than when you close the fascia. You do get cases like this though where you get massive dilation and some necrosis of the wall, and in these cases, even though it looks pristine, uh, in this case specifically, I did pull out an ostomy, uh, as you, as you umbilicus or a new site. I wasn't smart enough into the umbilicus. I put it into a new site. Uh, what, what do you do with this case? This is almost the vanishing gastroschisis. There's really very little defect, and you have this markedly inflamed bowel. How would people approach this? There may not be much bowel left inside the abdominal vivi. Uh, in this particular case, so, so what would you explore the kid or how would you manage this then? Yes, I would explore the child to see. The other thing is the, the abdomen's fairly, uh, scaffold or flat, it looks like. So I'm skeptical there's a lot of bowel left in the abdominal cavity. I think you need to explore to try to see how much is in there. And I, I didn't get that good of a look at that picture, but if that bow is OK, you might try to put something together if that's feasible in order to save Val down, down the road. Can we get back to the picture, please? Um That doesn't look like gastroschisis. um, so, so, so yeah, that's what we're saying that's not going to be a bow you put together, but I, I think it's hard to tell sometimes how much what is in that. You can't tell. I wouldn't take that out. I would let, no, I, I would, I'd open it up, put it in, yeah, put it in and let the inflammation resolve. So that, so that is critical. So that's the critical point. You cannot tell what's going on with this bowel, whatever you, whatever you do. Now, something strange we did and absolutely not take it out, never take it out. Um, we actually, I, I wasn't sure how much this was going to be. There was shortcut, so at the first opportunity, I did a, believe it or not, a step, maybe a little silly in retrospect, uh, and I put everything back in. Just did a step, didn't bring out an ostomy, put everything back in. Uh, when would you go back and take a look at this? So you obviously haven't got the newborn period. I mean at the at the time when I opened the belly up to see what's inside. I did a step because of this massively dilated bowel. I didn't bring out an ostomy. And I popped everything back in after you've been to church several times, I might go back in. I'd go back in when the leaks 2 Sundays, 4 Sundays and 6 Sundays. I would say if there are no problems, I would say 6 Sundays. So I went back at 2 weeks. And that's what we found. Very interesting. At 2 weeks, the, the bowel was suddenly real bowel, and it was the extent of most of this child's bowel did an anastomosis at that point, uh, and the child had a very good outcome. So I, I think we're always taught that you have to go back at 4 or 6 weeks. I'm not saying you need to go back sooner, but if you go back within 2 weeks, you, you get this gastroschisis bowel that actually becomes intestine. Uh, in that, uh, two-week period, were you forced to go back at 2 weeks or I felt very uncomfortable because I didn't have an ostomy. So I thought I'm gonna go back and if I cannot make what's going out, I'm gonna pull out an ostomy because I felt my NJ tube or ND tube wasn't decompressing adequately. It was actually an ND tube. We're already 10 minutes over, so let's go to ileal atresia. So apple peel deformity, this will be very quick. We'll run through this child with the apple peel ileal atresia, how do you manage this? This is a child, as you may see here, has a clear atresia between two ends of the bowel, and then this ischemic or necrotic looking apple apple peel. How would the group handle this one? Well, that's a, that's a tough one because of the schemy looking. Ilium, in general, if the ileum looks fine, you'd, you'd put it together and very gingerly and carefully put things back into the, into the um abdominal cavity. If, if you're concerned about that one though, I'm not sure I wouldn't put anything together. You might just drop it back in, um, and, um, and see because you've got to make sure that that bowel is. Appropriately vascularized in order to do anastomosis. So I mean, this is not gastroschisis, you've just taken it out, OK. And so, you know, this is an atresia. I'm not sure you'll learn much from leaving it back in. OK. I guess somehow in my mind I was thinking it was a gastroschisis type valve, but, but anyway, I don't think you can put that together if, if there's, if you're concerned about the vascularization of the distal ileum. That's exactly right. So it was ischemic and it's easier to tell obviously on the table than on a, on a picture, um, and we did 4 anastomosis and put it all together. Uh, but, uh, you know, the point is these children have relatively short gut. The, um, apple peeled bowel is not, not always that healthy, and some, and this is also another clear case that needed one anastomosis. And then, uh, we have these cases where you do have a good distal small bowel. Um, that needs to have an anastomosis. I usually, what was the reason for the vascular insufficiency in that first bowel? Was it just twisted and you untwisted and it was fine? The 1st and 2nd 1, it just wasn't adequate, even though it wasn't twisted, just was inadequate. So you resected that resected because it was 4 resected, right? Yeah, resected. This, this one over here, we resected as well. You can see it's non-viable bowel. And then this one, of course, nice and healthy. We do the anastomosis. I'm not sure what the group's experience is, but these are difficult cases. The only thing I would argue is, is if it was ischemic, not necrotic, you could wait till tomorrow and see if it pinked up at all. But you said, but then again, you did say it wasn't twisted or anything. It wasn't twisted. It didn't pink up on the table. It was what we found this kid. No, but that's a good point on this kid over here. I did do a placation. I find that, that a plac application of this, uh, bowel is often helpful. Who does placation? So no, I was gonna say if you have that same patient but less small. bowel and you're worried about short bowel. I have done steps to that proximal intestine in this scenario where you didn't have all the distal small bowel. Yes, and that's a good point. I mean, my, my preference is actually Bianchi procedure. I find it smoother. I find you get 22 equal chambers. You don't get these blind ending out pouchings, although the step is a very ingenious operation and a very good one when there's no good blood supply. But I, I wouldn't do that in the neonatal period. I would hook it up together, usually do a placation, then come back because these placations usually unravel with time. Come back and use that bowel for whatever your preference for bowel lengthening. Last update from the step registry was to discourage doing um uh steps in the perinatal periods, that those outcomes were. That those outcomes were difficult at best, and then also that steps in patients with gastroschisis and independent of these atresias, um, the patients have such motility disorders that they really aren't all that beneficial. What we're doing is we prefer a Bianchi as a choice because you can Bianchi and then step, um, whereas once you step you can re-step, but your, your ability to lengthen has been altered. So at least for the way we kind of think about it, and I'm we're working with Mike Humrath and Jamie Nathan all my other partners who focus on this disease process. We don't try to step early because it doesn't, it's not particularly favorable. I don't, I think location is certainly a choice, um, but it's not something that we, we generally will try to decompress that and salvage as much bowel length as possible. Yeah, I would, I would agree with you, Greg. Um, I, I, I also like to hear that you prefer the Bianchi because that's my philosophy as well. Um, we have a very big series in Washington DC, um, and the neonatal step is kind of, uh, unusual. I don't think I would, uh, I would, I wouldn't, uh, suggest that that's the right way to go. Uh, you do sometimes have to do something for that very dilated bowel that's dysfunctional, that becomes a cesspool of bacterial stasis. Yeah, I think that's, that's correct, but I think the point is we don't want to send out a message that the idea of a newborn step is something that is something that's being favorably looked upon in terms of at least my understanding from the last step registry report, which I think was just a couple of years ago. What about application versus table? So that's, that's another great question. Um, in general, in these newborns, and especially one with, uh, a questionable shortcut, I would not, uh, taper because you want to save that bowel for, for potential lengthening later. Uh, certainly if a kid's got an extensive amount of, of, uh, bowel and you've got a segment, uh, that, that's dilated, then a plaque, then a, a tapering is very reasonable. OK, I think, um, we're running a bit over. Is it OK if we, we're done. I, that was a great recession, um. Like you, I picked up a lot of uh new, new ways of doing things. Uh, we'll take a shorter break than planned. We'll take a five-minute break. I just wanna say that this happens, this, uh, You want a 10 minute break? So we're gonna be then continuing to be 10 minutes over. We'll, we'll, we'll try to catch up as the day goes on, uh, although I, I don't know how that's gonna happen. Um, but, uh, I do want to let everyone know why this is happening. This is happening because there are a few hospitals that are dedicated to education, and, um, I wanna make note of the hospitals that put this together. Uh, and the same hospitals from last year, uh, Kansas City, Mercy Children's Hospital of Kansas City, Children's Hospital of Atlanta, Women and Children's Hospital of Buffalo. Cincinnati Children's Hospital and Akron Children's Hospital, have, have, uh, been able to support this, and I, I wanna thank all of you for making this possible for the rest of the world, uh, and continuing to support this. So, uh, we will take a brief break and we'll come back with session number 2. Thank you.