Surgical Considerations for In Utero Kidney Failure

This will finish our panel for the morning, um, but again, we'll stay on for questions after we're done. Um, so our final speaker this morning, um, is Doctor Alex Bondock. He is the surgical director of kidney transplantation and assistant professor of Surgery and pediatrics. Um, At the University of Cincinnati and here at Cincinnati Children's Hospital Medical Center, and Doctor Boondock is gonna talk to us about some of the surgical considerations for these patients who require early dialysis, um, as well as an eye towards eventual renal transplantation and some of the outcomes that we've seen, um, here locally. So Doctor Bondo, take the floor. Thank you, Steph, and thanks to Doctor Askenazi. It's an, that was an awesome talk really highlighting some of the challenges in Um, caring for these patients, um, because truly one of the things that, you know, I can talk to you guys about is the multidisciplinary nature and the way that we succeed is through that is exactly what, um, he was describing, and I'll touch on all those aspects here. But from a with an eye towards the surgical considerations that Doctor Askenazi was uh alluding to, because thoughtful consideration of Um, how do you dialyze these patients via which catheters, um, enteral access, um, kids with stomas, things like that all have, has evolved very significantly in the last, even, let's say year or two for us. So the title of my talk today is the surgical Considerations for in utero kidney failure. The objectives of today's talk are just to discuss the perioperative and physiologic considerations. that are unique to this patient population that Doctor Askenazi alluded to already. Um, reviewing options for renal replacement therapy and types of catheters, which has also been touched on, um, and considering the other surgical needs of these patients and specific, um, anomalies that these patients face. And then finally, as, uh, Steph alluded to, I'll, I'll touch base on, um, uh, our experience with renal transplantation of bladder outlet obstruction patients, uh, here at Children's in Cincinnati. And so, um, the first, I, I started out as a case per Doctor Lim's request, and this is an ex-thirty-seven week old, uh, male child who was born to a G2P1 mother, whose 20 week anatomy scan revealed a massively, uh, dilated bladder with a urachal pop-off there, you can see. So it's red as megacystis, there was anhydramnios. Um, a severely echogenic left kidney with peripheral small cysts and no normal right renal tissue or left renal tissue was noted for that matter. And so the prenatal diagnosis was made of bladder outlet obstruction. And as a result, the um um mother infant died underwent an MRI at 21 weeks, uh, which, which demonstrated the very distended bladder you see there, as well as um bilateral severe cystic renal dysplasia. Um, the patient, uh, as described by Doctor Habli and Doctor Tabal underwent 9 amnio infusions over 9 weeks. And as we discussed, the baby ended up undergoing a repeat MRI at 33 weeks gestation, um, where the bilateral cystic dysplasia of the kidneys was reinforced and pulmonary hypoplasia was, um, the diagnosis of pulmonary hypoplasia was also made with Uh, the total lung volume estimated here was 37 mLs, where the normal range at 33 weeks to 35 weeks is anywhere in the neighborhood of about 50 to 130 mLs. Um, so, certainly, uh, um, a cause for concern for us and also giving us a, um, thoughts on how to prepare for this child when, uh, he was delivered. So, unfortunately, the baby was aneuric at birth. Uh, there was no urine via the urethra, and this was the ultrasound that was demonstrated at the time of birth. Um, hemodynamically, he required, uh, initiation of epinephrine and vasopressin. He was also started in on inhaled nitric oxide and hydrocortisone. So shortly thereafter, the urologists were consulted. Um, and they went ahead and placed a percutaneous angiocah to try and decompress the, uh, bladder system, a percutaneous vesicostomy, as demonstrated by the star there, you can see the catheter. So a lot of the sort of, um, hypoechoic fluid was was drained, but the hypeechoic debris, uh, was still trapped in the bladder. And so what next? Um, as we've already talked about, it's not just a balancing act between, you know, it's not a binary balancing act. We have to consider, as Doctor Askenazi mentioned, the renal replacement therapy, but then you're balancing all the other things, specifically in my, in my case, the fitness for surgery and the risk of general anesthetic. Which is then a balancing act between cardiovascular status, pulmonary status, other congenital anomalies, and how are we going to handle the genitourinary tract and uh especially in the outlet obstruction patients, but all KCU um patients. And so, I don't have to belabor this. I, you don't want to hear a surgeon talk about the renal considerations of these patients, especially after Doctor Askenazi. But the other thing I would add is how are we going to manage the bladder? What is the urinary system drainage, and what is the bladder status? Um, as we mentioned already briefly, the cardiovascular status of the patient is the patient hemodynamically stable or on vasopressors. Uh, we usually echo these babies in their first week of life to look for evidence of pulmonary hypertension, as if it wasn't, uh, abundantly clear by their ventilatory needs, uh, as well as congenital heart defects. We also look at their pulmonary status, which goes hand in hand with their cardiovascular status. Because some of these children need escalation to invasive positive pressure, uh, ventilation as even the oscillator, you already heard Doctor Askenazi talk about his experience with patients who are placed on VA ECMO for the same reasons. Um, some of our patients come out and surprise us and they're extubated in the 1st 24 hours after having been intubated and the, um, Uh, immediately postnatally in the resuscitation room. And then again, as we discussed the the ongoing assessment of pulmonary hypertension. And so, in these first few days um of life, we have to consider what kind of renal replacement therapy options. Um, uh, are we gonna consider? And again, same things that Doctor Askenazi already touched on, but it sort of, as we think about that, it informs how the types of catheters I'm gonna place at the initial operation. So not only are we thinking about how stable is the patient, what kind of anesthetic considerations are we gonna have, but also what kinds of catheters will the baby be stable enough to have? What kind of urologic procedure should uh um he or she have at that time. And so typically, um, for our the way we initially proceed once baby is stable enough, Um, in this case, uh, the case that I described to you, we went to the operating room on day of life 5. the baby was still on vasopressin, which was weaning, the epinephrine had been weaned off, the steroids were still on, the baby was still on inhaled nitric oxide. So, again, we have a very short window of when the baby can be under general anesthetic. And so what we typically do here is we place a temporary right internal jugular 7 French hemodialysis catheter. Um, as well as a laparoscopic or open assisted peritoneal dialysis catheter. Exactly for the reasons that, uh, Doctor Askenazi said is that we like to have the option to do blood blood renal replacement therapy either via aqua continuous CRRT or by eventually hemodialysis as we allow for about 2 weeks, typically, the The peritoneum to heal, uh, it's incisions where we placed the PD catheter. And so, in this scenario, just some tips and tricks we've picked up over the, over time. We never place catheters in the subclavian for future reference, for future hemodialysis or fistula-based dialysis as the patient hopefully will, you know, grow older, um, and eventually, likely outlast any kidney transplant they would have as an infant. We also sort of tunnel this percutaneous catheter. Uh, we make a counter incision in the neck near the mastoid process, almost like an emo cannula, so that this percutaneous catheter is actually somewhat tunneled because these polyurethane catheters have the tendency to kink because of the weight of the lines that go to the aquaphoresis machine or even the dialysis machine eventually. Um, You know, aquaphoresis for the short term is sufficient, but over time, and it certainly in this scenario, the baby doesn't make any urine and will need formal dialysis. And again, as I mentioned to you, this time on hemodialysis, usually a couple weeks, will allow for adequate peritoneal healing in anticipation of uh peritoneal dialysis initiation. And so, from a tunnel peritoneal dialysis catheter placement, um, as Doctor Eskenazi made it clear, it's the long-term pre uh preference for physiologic and social reasons and ease reasons. Um, from a technical consideration that we typically like to do them with a laparoscopic approach. You can do it open with fluoroscopy if the baby is is particularly hemodynamically unstable. Um, this is specifically in regard to our patients who have undergone cardiac, um, procedures or have structural heart disease and have high right-sided heart pressures. Oftentimes they will not, um, tolerate laparoscopic insufflation of their abdomens. So we do that with we do that from an open approach. Uh, typically, we will place the catheter and the and the cuff through the right rectus sheath with an exit site. Um, on the left lower quadrant with a downward facing course, there's, um, a, a, a reasonable amount of retrospective data suggesting that a downward facing course for a peritoneal dialysis catheter decreases its risk of, um, uh, peritonitis and catheter site infection. We also do a concurrent omentectomy and I'll show you some data on that later. And so this is essentially what it looks like. The X marks where um uh subcutaneously or intro uh inside the fascia is where the catheter is actually going in, and then we make a gentle curved tunnel to the contralateral side of the abdomen, so that it's a downward facing um exit site. And so for this patient clinically, um, 5 weeks later, the baby was, uh, working up on peritoneal dialysis, uh, with a functional catheter to fill volumes of 30 mLs per kilo. Uh, but more recently, we're having some, uh, intermittently inadequate return volumes, uh, which is either a peritoneal issue or more likely a catheter-based issue. Um, with peritoneal dialysis, as is not uncommon, we noted a right inguinal hernia, uh, with a hydrocele, uh, that fills up with peritoneal fluid or peritoneal dialysate, and then the patient is also nasogastric feed dependent. So once we get out here and we have reasonable peritoneal dialysis is when we start to think about other surgical procedures. Now, what I would tell you for a lot of these babies with in utero kidney failure, whether or not they have a true cause, such as Eagle-Barrett. Um, of their in utero kidney failure, um, what our urologists have termed this, termed what a lot of these babies have is pseudo prune belly. And the reason they say that is typically because of how distended the bladder was in utero, um, with little, uh, um, um, Amniotic fluid surrounding them, that bladder ends up distending and distorting the development of the abdominal wall. And I would tell you that the vast majority of these patients, the boy patients and the female patients for that matter, have inguinal hernias, and a lot of the babies also have undescended testes. And so when you think about surgery in these children, sort of distant from birth, distant being weeks, um, what we're trying to do is prevent disruption of peritoneal dialysis with minimally invasive approaches, laparoscopy, tiny. Incisions, incisions that are higher up on the abdominal wall, and then all the procedures where we will locate G tubes, where we will locate stomas, things like that, all are done with an eye towards future transplantation. And so in this same baby, we show you the ectopic testis. And so I talked to you about omentectomy. Um, because this is a very common reason why peritoneal dialysis catheters fail in young children, and this is a, um, a, uh, data that has been published or has been presented at meetings and is now currently in revision from Meredith Shi and, uh, a consortium of, uh, pediatric. Nephrology groups in the Midwest. Uh, the senior author is Donna Clayes, who's also on staff here in Cincinnati. And what they found was that, um, when you look at the adjusted, uh, multifactor multivariate analysis of reasons why peritoneal dialysis catheters fail in children, is usually age at the time of peritoneal dialysis catheter insertion. In this case, it's 4 times more likely to fail if the patient's over the age of 6, and that When you have omentectomy, that dramatically decreases the risk of this happening, of, of PD catheter failure or infection. And so you could see here in one of these patients, um, omentum was clogging up the catheter, and it can wrap up and and distort the catheter, the pigtail catheter. You can also get fibrin, protein proteinaceous deposits inside the catheter, and so here, we just use a blunt wire to um sort of roto root that tissue out of there. So the other surgical considerations I told you about were the inguinal hernias. Um, and so, here, what we've started doing, one of my partners who's particularly facile, even in small children with laparoscopy, uh, it started to fix these hernias laparoscopically. Um, other things to consider are enteral access, um, such as G tube placements, um, undescended testes, and more recently, um, We started after some um data came out at, at a meeting that Doctor Stu Goldstein, uh, who's one of, who's our head of pheresis here, um, saw was that traditionally, our center had not offered peritoneal dialysis at all for patients who needed stomas for other congenital anomalies such as um anorectal malformations, uh, or even Hirschprung's. But more recently, because of, um, intraabdominal catastrophe for one patient who had Hirschsprung's disease, Um, we were able to sustain her on, um, on peritoneal dialysis for a period of time with an ileostomy, and that's shown in the top panels. And then, um, also recently we've, we had a patient with an, uh, um, anorectal malformation who had his stomas made at one day of life and had developed subsequently worsening chronic kidney disease to the point where he needed Um, renal replacement therapy. And because he already had stomas and we already had to go back in for surgery, we went ahead and tried peritoneal dialysis based on our experience with the first patient, and he's been sustained on peritoneal dialysis and at the same time, we put a gastrostomy, uh, tube in his stomach. And so here's the picture of that patient's abdomen, stoma in the left lower quadrant, gastrostomy in the left upper quadrant is peritoneal dialysis catheter. We exit site we flipped uh for for hygiene to the right side. And so what happens long term? This was a paper, uh, or this was a poster that we presented at uh PAS just, uh, last, a couple weeks ago, uh, done by one of my research fellows, Carrissa Lake, and Braxton Ford, who's one of the maternal fetal medicine fellows, uh, working with Doctor Taba at the university. And this covers um our experience from 2010 to 2018 on patients with bladder outlet obstruction who've survived uh to renal transplantation and their outcomes. And um what you see here is we tried to break it down, each panel to the multidisciplinary to reflect the multidisciplinary nature of the care of these patients. And so, um, you can see that 80% of the patients required ventilator support at some point in their life, in their nursery stay. Um, but luckily, only, um, 2 patients required minimal oxygen support out of the 18 we've presented here. Um, Postnatally, uh, I'm sorry, post nursery, um, we had an average of 6 hospitalizations, uh, with 8 operations. And sorry, I should have hit that earlier. And from a dialysis standpoint, 9 89% of the patients or 16% of the 18 patients required dialysis at some point, and the duration of pre-transplant renal replacement therapy was 1.7 years. And to speak to Doctor Riddle and Doctor Askenazi's um um points about how important nutrition was. Um, was that we were able to supplement these patients, um, 80% of whom needed gastrostomy tubes, um, where they were able to get these babies to a similar Z score to their peers, uh, by weight, but interestingly enough, height was, we could never achieve, um, uh, similar height by weight, uh, growth parameters. And so from a transplant perspective, uh, the median age of transplant was 2.3 years. Um, a lot of these patients had complex genitourinary needs that the urology service helped us with, um, or, you know, they're, they're critical collaborators in this whole thing. Um, and then long term, the median, the mean follow-up, um, Uh, it was 6 years for these patients with a median follow-up of almost 7 years, and we report a, you know, a survival over that time period of 94%, which I think is relatively good. Unfortunately, we had one patient, um, experience graft loss from chronic rejection and then one death, um, uh, in a patient with unrelated to their renal disease. So that's about it, and I'm happy to take any questions or make any comments um uh on the data we presented. That was great, Alex, thanks so much. Um, I think it was great that you mentioned the experience that we've had with the patients with ostomy and you know, you alluded to the data from the North American, um, registry that was presented a few years ago that sort of led to this shift, but any sort of, um, learnings that you guys have had. Out of those few patients that we've done so far, um, and things that, that we need to consider for the future as we expand, um, the option for postnatal therapy. Yeah, no, I think it, I mean, it's interesting because both of those patients, as you might surmise, have not had simple postnatal courses. The first patient perforated her colon. Um, after initial PD catheter placement, which because of her Hirschprung's, that was her initial presentation of Hirschprung's. So we had to biopsy her rectum and make her ileostomy, and she was able to sustain on PD after she, we sanitized her peritoneum for a period, but then it failed, more than likely because of uh peritoneal failure, and then the second patient actually um had a complication with his colostomy. So, at that point, we, because of our experience with that first patient, we elected to revise that surgery, um, and then place a PD catheter. Luckily, there was no peritoneal contamination. So I think it really ends up being, you know, those were our feasibility patients, and then what we've learned in that regard is keeping the peritoneum sterile, but also meticulous, um, technique when you're building the stomas. So that you are completely isolating the outside from the inside that you're not gonna, you know, contaminate the peritoneum. But from a sur purely surgical perspective, you know, when we make stomas for anything else in a patient who does, in a neonate who doesn't have renal failure or in utero kidney disease, it's not like we're contaminating their peritoneums. So I think it stands to reason that if you, if you make stomas in a, in a Durable, meticulous way, those patients can be sustained on peritoneal dialysis. Yeah, it's certainly shifted the way we talk to families prenatally. Not all of these things are able to be diagnosed like with your patient with um Hirschsprung's and we've seen them with imperforated anus and things. So, um, it has shifted the way we've counseled these patients. So, um, it'll be interesting to see their experience moving forward. And, uh, one last thing to add is sort of from a, from a system standpoint, as has been alluded to, the, the people who take care of these patients and their surgical problems, uh, are, it's a specialized team, just like it is with you guys and with Doctor Lim and the nephrologists, but, um, the transplant surgeons, the pediatric transplant surgeons here in Cincinnati, and essentially take care of these patients' surgical needs from birth to transplant and beyond. So, I have, just as a matter of course, I have two other partners. We're both trained in pediatric surgery. We're also board certified in abdominal transplant surgery, and so that allows us What Doctor Askenazi was referring to, building that experience, building that comfort level, where to put the catheters, how to handle these surgical problems, and that's really the way we've, we've learned is, you know, we try to be as specialized as possible, but it also gives the, the, the families and the teams familiar faces to, to help put with, um, you know, from birth to transplant. So, um, there was a question from the audience, obviously, um, you know, not all centers are like, um. Some of our, um, presenters here. Especially, you know, what we can offer, um, comprehensively from the get-go. Um, there are, there are plenty of places that, uh, resources are low, uh, and inadequate. Um, how would you approach those patients in those areas? I mean, it's not just, you know, uh, in third world countries, certainly we see that pockets also in United States, right? And also some of the um The advanced, um, uh, and developed countries. Anyone from the panel. Yeah, I'm happy to, to answer that. I think that, you know, like anything else, I mean, as physicians, we're, we need to know what we can do and what we can't. And we need to reach out to our colleagues for, for help. And so, You know, we, if there's things that we can't do at our institution, you know, we should be sending them, you know, do the right thing for the patient and, and finding those resources that are out there. Uh, at the same time, um, you know, institutions should be looking at how to enhance their programs, enhance their ability to take care of patients. But in the meantime, uh, don't, don't hesitate to reach out. And I think too, Fong, um this is where the importance of the fetal, the prenatal fetal counseling, um, portion of things comes as you sort of go over what you think is the severity of the disease process with the parents or the family of that, um, unborn child that You know, you're looking at, we can cite you data of how long your baby will be living in the nursery, and how long your baby will be living in the hospital, and what it looks like on the back end, and the problems that can happen, how many hospitalizations, what transplantation looks like. And so I think if, you know, depending on the resources of the persons of the institution in general and plus how involved that care can be, then you can at least the parents can make the most um Informed decision possible. And I think one of the things that we've learned, maybe one of the upsides to the pandemic has been the availability of, uh, virtual consultation and the ability to counsel patients that are not physically present with us. Um, and I think that could be a resource, um, certainly, we've had referring Physicians join some of these um sessions, as well as the family itself to um try to understand the resources from, from where they're coming from, and I think that could be, you know, a benefit that comes out of the pandemic as these, uh, virtual resources have improved. Well, we definitely have had a wonderful, uh, panel this morning or this evening to some of you. And um I really hope that, um, You know, you all, uh, find this um type of live webinar, um. Not just interesting but also um Benefiting, you know, Your center and also your patients and we, we would love to hear back from you if you have any feedback how to make something like this um more useful uh and also more interesting. Um, we, we have session number 3 coming up in June and certainly hope a lot of you and your colleagues, um, as well as your, your friends. Uh, can join us. Uh, I apologize that some, uh, countries, uh, were not able to view this live webinar, uh, smoothly because of the platform, um, in those areas, uh, but hope that, um, you are able to log on to the recorded, um, series. And able to view that, you know, hopefully, uh, in a, in a easier fashion or hopefully smoother um when you come back and view the recorded session. And I want to take this opportunity to thank all of the um the speakers um in the panel as well as uh people that help us to make this successful from our global cast crew, um, Jay, John, you know, especially, uh, and all of those that help us to put, you know, this webinar together, um, and More importantly, I'd like to thank all of the um the audience uh from around the world to, to make this uh successful without, without the audience, we will just be talking with each other on uh on this Zoom meeting. Um, so thanks all of you again for taking your time joining us, uh, even, you know, I know some of you had to get up at 4 a.m., 5 a.m. to join us and others, um. You know, uh, uh, putting away your, your important Saturday night, uh, agenda, uh, to join us. So thank you again. If we don't have any um additional questions for the panel here, we will conclude here a few minutes early. And remember, so remember that David, you, you wanna say one more time about your um your quarterly um course. Yeah, thank you. So, um, our next course is May 23rd and 24th. Uh, we have just like two or three spots available, so just email me if you wanna do it. Um, and then, uh, again, it's, it's a full day of, uh, didactics, and then it's a half a day of simulations. Um, and, uh, again, we have, uh, neonatologist speak and surgery speak, and, uh, nutrition and pharmacist to really try to be comprehensive about The care that, that, uh, is needed to support these, these children. So, just send me an email and I'm happy to, to, to, to get you guys hooked in. I think the, the next one is middle of June. Middle of July, sorry. Um, Alex, actually there's a question just posted. Uh, by Doctor Kaskas. About tricks you would recommend for insertion of PD catheter by open method? Yeah, I responded um for the first part of it, but typically when you do any PD catheter, um, our, the, the ones we have are pigtail catheters, and they measure the distance from the cuff, um, to the tip of the catheter, and usually it's by infant, pediatric, adolescent. Usually the way I estimate that is the distance between the bottom of the belly button to the pubic bone. So I would tell you for most neonates, it's actually a child's catheter, a 6.5 centimeter catheter. Um, you can go through the same incision I described in the slides, but in this way, if you don't have fluoroscopy, you, you have to insert the catheter over a metal stylette. And you aim, you use your hand and you feel the anterior superior iliac spine, you touch that and then you walk it down the pubic bone and try just by blind feel, try to get behind the bladder and then deploy the catheter. You can also, before you, you know, push your, push, place your catheter, you can pull the baby's omentum up through that incision, and you can just do a, a sort of blind omentectomy, not obviously not getting too close to the colon. So that's, I, and if you have fluoroscopy, that's the best way to confirm where the tip of your catheter is. So those are sort of the my thoughts on open um PD catheter placement.