Urologic intervention: Cincinnati Fetal Center

So, um, our last talk is, um, related to pediatric neurologic, uh, intervention, and you have all met, uh, Doctor Reddy, uh, earlier, um, except those that just tuned in. Uh, he's currently our director for the Pediatric Urology Program in Cincinnati Children's. Doctor Reddy, thank you. So, uh, you know, now that you have a baby who's born with posturethral valves, what do you do next? And I think before we get into this, I do want to highlight one important thing, which I hope you've been realizing as this conference has been going on. Is that it really takes a team, both a team prenatally and a team postnatally, to ensure that these patients have the best outcomes, and I'm fortunate to be part of an awesome team here at Cincinnati Children's Hospital. We try to meet on a regular basis, uh, from the prenatal standpoint to ensure that we are looking at how we're performing as a team in terms of caring for these patients. And making gradual improvements so we can constantly be asking ourselves questions, how can we do things better? And also from a postnatal standpoint, we do have monthly conferences with our transplant and nephrology colleagues to again review the outcome of these patients, how our patients are doing as we're getting them on the journey towards renal replacement therapy or hopefully avoiding renal replacement therapy even. And uh and that's something that I would encourage any of the institutions that are watching to uh try and mirror as you develop your own programs is really get those teams working together. OK, so you know you've got a baby who was born with postrethral valves. What do we do next? I stuck. OK. Sorry, I'll let you get froze. So, um, you know, I, I think that, uh, once you have a baby who's born with, um, posturethral valves, that you have to look at the newborn management. You've already heard some of that from Doctor Kingman in terms of pulmonary stabilization and, uh, you know, uh, support that they may need, but I think that's one of the most important things is that you have to stabilize the patient as a whole. Oftentimes initially it is with the pulmonary stabilization, but also the electrolyte imbalances that they may have. And um from a urological standpoint, our job is very easy upfront. Uh we like to decompress the bladder. We typically recommend using a um a feeding tube rather than a balloon catheter and the reason is that these bladders are so hypertrophied that if you put a balloon into that bladder, that bladder is going to contract down around that balloon, and you're going to get obstruction of the UVJs from the balloon being squeezed by the bladder. And so we like to use a feeding tube rather than a balloon catheter. We then try to assess the anatomy of the bladder and the urinary tract with an ultrasound and a VCUG. Whenever the baby is stable enough to travel down to radiology, you can get the VCUG, uh, you know, obviously doing a full scale neonatal survey of the baby to look for any associated anomalies. If there is a sacral pimple, we do ask for a spinal ultrasound. So all of these ultrasounds can be done on the bedside, um, the, uh, VCG down in radiology suite. And then we sit down, meet with the entire team, and we come up with a care plan that is specifically designed for the patient at hand. And uh during that consultation with the family, we make them understand that this is not a one and done sort of deal, but this is a journey that they are gonna be going on and we're there as a team to support them through that journey. So from a urological standpoint, the goals of management of a child with postferal valves are to protect the upper tracts. Now you've heard a lot of discussion about why do some of these children who have seemingly normal creatinines at the time of discharge then progress to renal disease and renal insufficiency, ultimately end stage renal disease. And it's because we are now understanding that there is preventable harm and harm that we can't prevent, and some of that is just a fact of growing as Doctor Goebel rightfully pointed out that as a child grows, their demand on those kidneys, if they have appropriate reserve is gonna be met. But in a kidney that has no reserve to deliver to the child's body as they're growing, they're going to outstrip their ability of the kidneys to provide the renal replacement, sorry, the renal support that they need. So we want to protect the upper tracts from this preventable harm, and the preventable harm is um the barrow trauma, the pressure-related injury to the upper tracts, the urinary stasis, the urinary tract infections. These are things that we want to try and prevent and that's what we as the urology team will be doing for these patients. So we protect the bladder and provide an adequate low pressure storage unit for urine that protects the upper tracts. We allow for emptying, whether it's volitional emptying or emptying with a uh intermittent catheterization program. Now some of these patients, as we talked about earlier, will have a renal injury that is minimal but manifests as polyuria, and these children make large volumes of very poor quality urine, but those large volumes of urine overdistend the bladder and make that bladder dysfunctional. So in those instances we do resort to doing overnight catheter drainage of these bladders. That's something that, however, manifests a little bit later in life, not as a neonate. We want to minimize the risk of urinary tract infections. Both we know that when you have a high pressure bladder, that does increase your risk of infections as it reduces the urothelial libidity to fight off infections appropriately, and then urinary stasis. As the child grows, we're now moving from protecting the upper tracts, protecting the bladder, to looking at how do we allow this child to achieve social continence, and we define social continence about 3 to 4 hours of dry interval during the daytime with emptying of the bladder and 8 to 9 hours at nighttime being able to sleep without having to empty the bladder. It is critically important that we continue to monitor the renal function of this patient as and the child as they grow. Ultimately our goal is to provide for this child the ability to have independent care of their own condition and appropriate psychosocial and social body image. So the diagnostic testing that we do right away as soon as the baby's in the new NICU would be a renal ultrasound to assess the quality of a tracts. Specifically here we're looking at the echo texture and echogenicity of the kidneys, the degree of hydronephrosis. We like to use the SFU, the Society of Fetal Urology grading System for hydronephrosis. Goes from grade 0, no hydronephrosis, 1, less than 10 millimeter dilation of the renal pelvis without any celiectasis, grade 2. Greater than 10 millimeter dilation of the renal pelvis with no associated calliectasis and normal parenchyma. Grade 3 is any degree of pelvic calectasis with normal parenchyma, and then grade 4 is where there's parenchymal thinning. And obviously if there's any cystic dysplastic changes, that puts them out of grade 4. With regards to the ecoexcture, you know, we have built-in control. So on the right side, we look at the liver and say, is the kidney as echogenic as the liver? If it is, that's not a good sign. On the left side, the spleen. This is a little different than what I've learned from my, our maternal fetal medicine colleagues where they look at the kidneys in utero and they're comparing them to the bones, and they're saying if they are as echogenic as the bones, that's not good, and that's a sign of renal dysplasia. Obviously, if there's a lot of um parenchymal cystic changes, that's not good and that. Tells you that there's already been a significant irreversible damage to the kidney. Uh, if there's sacral dimple present looking for a spinal ultrasound, if this, we've had a few children who have had both post retal valves and tethered spinal cords, so that's a double hit to their urinary tract because now they have both a myogenic damage to the bladder but also a neurogenic damage to the bladder. In terms of labs, uh, we want to do a CBC, complete blood count, renal profile. Uh, it's very important here to understand that the creatinine for the 1st 3 days of the baby's life is gonna be reflective of maternal creatinine. So, while that is a good guideline to keep in mind, if the baby's creatinine starts off at 2, you know that there is some significant injury because the maternal creatinine is not 2. And when stable, we will get a VCUG. Our philosophy here of taking care of these children is that they are simply a pair of kidneys and a bladder to us. This is a whole child that we're taking care of, and we're taking care of them at the beginning of their life's journey, and the goal is to give them this potential to realize a full and functional, healthy life with normal quality of life. And so at the top of that pyramid is gonna be obviously their kidney health because that is going to dictate a lot of their quality of life, but we want to minimize complications from any interventions that we're gonna be undertaking. Allow them to have normalized anatomy, urinary incontinence at an age-appropriate time. Rewarding sexuality, we do have a lot of data now that suggests that children with chronic illnesses who do have to use intermittent catheters to manage their lower urinary tract, whether it's spina bifida patients, patients with traumatic spinal cord injuries, or our patients with valves do tend to have some issues with sexuality as they become teenagers and young adults. And so we do work on that and ultimately the goal is to allow these children to become independent contributing members of our society. So, uh, once we have diagnosed the presence of the valves, because remember, not all of these babies that are billed as being posturethral valves in utero end up being posturethral valves, they could be Eagle-Barrett syndrome. They could be urethral atresia. Uh, we've had patients with high grade vascular reflux come in suspected of having valves, but once we've made the diagnosis with the VCUG, we're able to then move forward with the care plan. We want to take them to the operating room at a point when they're stable from a pulmonary standpoint and from an overall health standpoint to be able to sustain their ability to undergo anesthesia, and we do an incision of the valves. We attempt to do this endoscopically in babies who are either too premature or if their anatomy is not normal, then we will divert them with a vesicostomy. And then you want to, you know, develop, uh, follow these children going forward to determine their extent of bladder injury and also the kidney injury. And I think here the ongoing management basically falls into two buckets from a urology standpoint. So you've got a child with postrethal valves. We want to protect the kidneys, and we want to protect the bladder, and I have to emphasize here that the management of the bladder is often not given the degree of importance that it should be given, and it's the bladder that is going to be your enemy in taking care of these children because it will be the cause of silent demise of those kidneys. These children who have been billed as being OK, we don't need to see you back, we'll just get a periodic ultrasound every couple of years. Those are the children that will creep into end stage renal disease before your very eyes because you're not managing the bladder appropriately. And this is why the bladder is such an important thing, and it's because when a bladder functions appropriately, you can think of the bladder along Starling's law, where you know you have appropriate bladder volumes, you have appropriate bladder contraction, you have appropriate storage pressures, the bladder's going to work well. If you have a very hypertrophied bladder, which almost all of these babies with posturethral valves have early on, they're going to have very high storage pressures which will then cause upper tract damage, and that high storage pressure in the bladder causes ongoing injury to the bladder itself. These bladders will then go to the other side of the normal curve. They'll go all the way over to the right side of the Starling's curve and become the end stage bladder, or what we used to call the um not the valve bladder, but the teenage valve bladder, which is a large floppy bladder that doesn't empty well. We know that there's 2 reasons for that hit to the bladder. One is the high storage pressures. The second hit is the polyuria that the upper tract damage causes to these bladders, and they cause them to become really overfilled, and the child doesn't have the myogenic capacity to be able to contract an empty well, so they always have a post-void residual that causes ongoing injury to the bladder. So if you have that high pressure bladder early on, our management strategy is either a vesicostomy, we all start almost all of our patients on Ditropan early on. In patients who have a very high bladder neck, we'll start them on an alpha blocker to try and alleviate that bladder outlet resistance at the bladder neck level. And we also have a low threshold for starting these children on intermittent catheterizations. If we see that someone has high grade reflux, we are going to start them on intermittent catheterization along with the anticholinergics to drop the pressures down and prevent any ongoing injury to the kidneys. Now then go to the other side, go to the, the end stage bladder where we've got this bladder that doesn't work well. Same thing, intermittent catheterizations, and again our goal is to help the bladder protect the existing nephrons that the patient was born with, or we're now protecting the bladder in the child who has already got end stage renal disease to keep it healthy for the future transplant. So, I think, again, you know, I just want to say that it takes a team to get these children to a point where they can really have that full and productive life and be as healthy as possible. Thank you very much. Thank you promote. Um, that's a question for you, and, um, I'll let you read about that while um Bill is continuing on, um, carrying out some of the discussion. Um, I don't know if you can see that on your Uh, so, um, there were, there was a period in the evolution of our understanding of these bladders where we did do reduction cystoplasties to try and make these bladder work better. Our experience has been that if you actually manage the bladder appropriately, some of that excess volume is going to go down, and by allowing these bladders to cycle better with intermittent catheterization, we found that to be a better, uh, long term strategy than going in and surgically excising part of the bladder. OK. Another question that was asked if the prenatal echogenicity that we can see on ultrasound and maybe Ron or Mark Greg, the prenatal echogenicity, do you know of any studies that correlate that with the kinds of stage 4 or 5 criteria that Doctor Reddy was referring to? I mean, I think it's pretty Pretty accepted that poor, highly echogenic kidneys or irregularly echogenic kidneys are more likely to be functioning poorly, but that's probably because there's a high incidence of poor function in these patients, and so we end up with this recall bias of of correlation. But I'm not aware that anybody's looked at it. Gregor bar. I think, you know, there have been studies early on that tried to correlate ecogenicity with, you know, renal function, and a lot of that is in the literature, but, you know, a lot of times, and Greg, you can comment on this as well, when you get a fetus that comes in and gets 20 weeks or 22 weeks and has severe, you know, pelvic calectasis, the brenchyma is so compressed that I think just the compression of the tissue itself. You know, must lead to some kind of increase in echogenicity, so I think, you know, when we decompress the bladders and the upper tracts drain, you know, we've seen where the echogenicity goes down significantly. I would agree, Mark. I think the exogenicity is a very subjective assessment. I think the best study again comes from the Birmingham Group and Katie Morris looked at all of the studies looking at how good we were using ultrasound to predict function and unfortunately, you know, whether we're talking about gestational age or echogenicity or cystic dysplasia, it doesn't. They don't work as well as we hope they should work, so I think we're not, I think our tools are still inadequate in assessing dysfunction. I'd also like to hear your guys' views. Particularly on the panel, a lot of time. My question is, you know, with our better and better transducers and higher and higher frequencies, you know, when we look at these kidneys, you're starting to pick up such detail that now the question is, are we really seeing small cysts, or are we just picking up the normal parenchyma and, you know, how big of an area do you really call a cortical cyst versus normal parenchyma, and I think we struggle with that a lot because using these, um, you know, high frequency. Transducers, you can talk yourself into that there's mic microcystic stuff developing and yet I'm not sure that that's real or whether that's just artifacts, um, because our our imaging is getting so much better. You guys have any experience with that? Do you see that you're picking up small things that you wonder whether they're So, um, uh, Doctor Johnson, to answer your question, you know, we're struggling with that on the adult, uh, I mean, postnatally too, you know, the imaging. Um, ability of the current generation of ultrasounds is so good that we are picking up things that we're not quite sure what to make. Uh, are these artifactual? Are these real? Um, especially with pediatric stone disease, you know, we pick up these, um, very echogenic lesions within kidneys and all those, uh, vessels that are we're seeing and not now with the improved enhanced ultrasound imaging, or is this a real small stone that's not clinically significant, so. I think that our learning curve is gonna have to keep up with the um with the advancements in technology. Yeah, I, I would perhaps add on the prenatal side. At least my experience when I go to these consultations is that 90+% of the imaging that seems to matter is the MRI. We barely look at the ultrasounds, um, so I, I, I don't have much, um, they got, they. Barely look at my pictures, so I, I learned a lot since I started going to the fetal care center, but I didn't learn much about fetal ultrasounds and renal echogenicity on them. That's what I'm trying to say. I'm not trying to make you cry and then postnatally. I'm with Pramode. We've certainly seen very lousy looking, very echogenic kidneys work surprisingly well, and we've seen the opposite where you look at an ultrasound and say, wow, there isn't very much echogenicity, but those kidneys aren't working worth a whole lot. So I would second that we're. Uh, we have such good technology now that we are even more on a learning curve and in an area of uncertainty. Can I have something here. I just like to to make Ron Ron feel better that we, we really don't do MRI for for affective neuropathy because we don't really believe that we get any better information out of it than a really good ultrasound, but we do use it for if we worry about cloacal malformations because I think it does benefit a lot, you know, MRI plus a high resolution ultrasound. So I, we still base all of our decisions. And we on ultrasound and we rarely use MRI's for just a bladder outlet obstruction. Yeah, I think we would go ahead, Greg. we would have the same approach here. We don't use MRI at all, and our MRI folks have not. have not felt they contribute an awful lot over and above what we're seeing in the ultrasound. I think the points you make about the ultrasound are very good, but, but sometimes when you, it comes back to the rationale of whether or not we should test the urine, if you have a crappy looking ultrasound and you've got crappy electrolytes, you've probably got crappy kidneys, um, and sometimes, you know, the two of them together are useful in prognosticating. I step into that. And, and to go back to a point I made earlier, the reason why we continue to show MRI both to our nephrology colleagues and families is the big, the big image of the baby allows parents to see it better. I don't think it changes our ability to, to diagnose, but it does sometimes make it easier to get the story across to the parents. I think if we use the MRI uh prospectively with the lung volumes that we can get to help guide families in their decision on whether to be aggressive with fetal intervention. Uh, that's something that we do peripherally. It isn't part of our central consultation, but I think it can be helpful. Yes, I have a question for Doctor Reddy. Yes sir. Can I ask Doctor Reddy a question? Yes, yes please, yes please. Yes, I mean, you mentioned in your lies, in your slides about sexuality and stuff with these patients. 20 years ago when we were looking at the histologic changes in the pathophysiology within the bladder, one of the things that we noticed is that on all of these autopsy specimens, the um prostate glands were very, very small and abnormally developed. You, is there any information out there now that we have shunt survivors as to fertility in this population? So, um, I have to tell you that I'm not up to date on the literature in terms of fertility in patients with post fetal valves per se. We have a few patients who, uh, actually fathers who had post fetal valves, who have had babies with post fetal valves that we take care of in our center. So there is that. I know that this particular father needed assisted reproductive techniques to help achieve that pregnancy with his wife. So whether or not fertility de novo is normal or not, it's, uh, hard to say, but, uh, that certainly is a good question, and I will have to look at that with our adult colleagues here because a lot of the patients that were born here in Saint Saint John's Hospital with valves. Uh, 40, 50 years ago are probably patients over at the University of Cincinnati, so we could certainly look at that and get back to you. Great question. I would like to answer a previous question that Doctor Karno had put in and I'm just looking at the, um, the, uh, the history that you provided in the um chat room and you know, wow, I, it's a rather challenging case and I think that the history that you present, I would suspect that this patient is on some form of um. Enteral nutritional supplementation overnight or during the daytime. So if you've got this big floppy bladder that's not responding well to a vesicostomy, one thing that we do with our patients who have vesicostomies is the parents are taught to dilate the vesicostomy using either a 16 French catheter or 18 French catheter. Over, um, you know, twice a day just to keep the stoma patent and prevent the complications from stomal stenosis in some patients where we've seen poor emptying of the bladder with a lot of stasis and a lot of complications with infections, we have had the parents catheterize the vesicostomy with a catheter overnight. So I think if this bladder is really that atonic and causing issues with uh intestinal obstruction or getting intertwined with the loops of bowel, you may want to try that first before you do a reduction cystoplasty. Uh, have we done a reduction cystoplasty? We've had one female patient who was born with a cloacal malformation that came in with a massive, I mean, 2 L bladder capacity, and, uh, that patient, uh, necrosed part of her bladder. So in, in managing that part of, uh, in managing her, we did take off about half of the bladder. So, um, we've done it in that one patient, she's actually done pretty well. I think anytime you do a reduction cystoplasty, you have to make sure that you're taking off just enough so that you don't have to go back and augment this patient later in life if things do improve. So that's kind of the. The fine line that you have to walk there, but I think you could probably mitigate the issues of the overdescended bladder by having the parents catheterize that vesicostomy and decompress that system. Are there any other Questions here with the panel or other things that have come in through the chat room that we're missing? Otherwise, I think we're ready to close. We wanted to thank our audience today. Uh, we know that they were checking in from, uh, I think we were over 40 different countries. And for those of you participating, we hope this has been a valuable learning experience. Uh, I think you'll get some information on the fact that this will be archived and posted and available to review. I wanted to thank our panelists in Philadelphia, Doctor Johnson and Doctor Ryan in Toronto, as well as all our faculty here, uh, Doctors Jaco and Goebel and Alonzo, Doctor Lim, Doctor Reddy, Doctor Kingma. We wanted to thank the, um, uh, support teams that we've had here to put this on and, uh, welcome any feedback that you have so that in the future, we can make these opportunities even better for you. Uh, I think we have a final concluding word from Doctor Ponsky. So I just wanna second that and then add one more thing. Um, this was a spectacular panel, and I wanna thank all of you for, for someone who knows not much about this. This is really good for me, and I, I really wanna go back and watch the event again when it's been categorized by its topics so that it's more applicable for me in practice as we're trying to build our, uh, fetal prac fetal practice in Akron, um. I wanna again reiterate that, click the link to take the CME test to get your credits, and we'll send you an email with the link. You can always go back and watch this, show it to your colleagues at any point in time. Give us 48 hours to put it up on the web. Um, and now I wanna do a shameless plug. We have now released, uh, by people asking us podcasts, and I'm gonna ask you all a favor here. So we've released a podcasts. The idea is called Stay Current, that people should be able to listen in their car, very brief vignettes, 3 to 5 minutes on a particular topic, sort of board style questions. And next is Doctor Rich Falcone from Cincinnati Children's who will be doing trauma, and we have had requests from people saying, can we put one out on fetal. And uh we will, we released this to 50,000 people. So, we're hoping that we can put together, maybe as a group or, or, or one on one, maybe 10 topics that we can release a couple of times a year uh for free to anyone in the world who can listen and stay current to what's new in fetal uh surgery. So, I wanna thank everybody, yes. Uh, I, uh, yes, yes, so I'd like to take this opportunity to make a plug too, and that is that, uh, the division of pediatric Urology here is gonna be holding a workshop next month where we have dedicated a whole day to fetal urology. And we have uh our colleagues from, uh, maternal fetal medicine, from neonatology, from transplant, from fetal surgery all participating. We've got some experts coming from around the country and around the world to help us with this, uh, workshop. So, um, you'll see that popping up on your screen there. There's a link that you can follow. It'll give you information about that workshop. We would encourage anybody that's interested to, uh, come and participate. Uh, we hope for this to be. Not just another conference, but truly a true exchange of knowledge and a dialogue where we can then move the knowledge forward. So thank you. Fantastic, and thanks again to the virtual faculty, and we will see you next time. Thank you very much. Good afternoon, good morning, or good evening.