Neurogenic Bladder

Hi everyone. Welcome to my office. My name is Lynn Wu. I'm a pediatric urologist at Rainbow Babies and Children's Hospital in Cleveland, Ohio, and today we are going to be talking about neurogenic bladder as it relates to our pediatric patients with spina bifida, uh, which includes myelomeningocele, lipomyelomeningocele, and meningocele. I'm here today with Doctor Lynn Wu, associate professor of urology and program director at Case Western in Cleveland Medical Center. She is a pediatric urologist at Rainbow Babies and Children's Hospital. Thanks for joining us, Doctor Wu. Thank you. It's a pleasure to be here. We are also joined by Al Ray, a PGY4 urology resident at Case Western, currently on his research here. Thanks for joining, Al. Uh, thank you for having me. So, Doctor Wu, many of our listeners out there are pediatric surgeons and some are pediatric urologists as well. My main question to you to start us off, why is it important that pediatric surgeons are familiar with the management of neurogenic bladder? Thanks, Ray. Neurogenic bladder can affect a lot of different, um, um, disease states, and so we always think of spina bifida as probably the most common cause in our PES populations, but, um, truth be told, you can see neurogenic bladder, you know, in children with any spinal cord abnorm. Abnormality, a spinal cord injury, certainly after trauma, um, with which you guys are very familiar and then other, um, congenital anomalies like, uh, cloacal and bladder atrophy or even in the anal rectal malformations or children with the, the veal. Kind of complex, uh, where there's a known spinal cord problem, um, those children often have a high incidence of neurogenic bladder as well. So, you know, I think it's, it's important that, you know, you as surgeons who are managing, um, these really complex children, um, can at least be a little bit familiar with what a urologist is thinking and what their priorities are, um. In taking care of kids who have bladders that uh don't function normally for, you know, for whatever reason. So thank you for bringing that into context for the pediatric surgeons out there. um, and now as everyone um knows and loves, let's dive into some case-based discussions. So we'll start off with, OK, you're consulted on a one day old female who was found to have myelo meningocele. What is your approach for the initial evaluation of these patients? So as you know, when we talk about it, the kind of the, the layman's term would be spina bifida or that kind of encompasses a lot of these myelomeningocele defects. And, um, you know, one thing that, that, uh, we'll maybe allude to later is many times we're picking these up based on prenatal imaging. So, the families have some idea that their child's going to be affected with this issue, and I think this is really helpful to prepare them as much as possible for, um, you know, what their child might have to face. Often in the very early days after delivery, um, a point that has been brought up on a previous discussion was that sometimes these defects are being, uh, repaired in utero, so in a, in a prenatal, uh, fashion, and those are being done by pediatric surgeons, and that can be saved for another discussion, but. Um, there's been ongoing evaluation about what the outcomes of those children are versus children that are being, um, repaired in the, uh, neonatal, uh, period, which has really been the gold standard or the, the kind of the standard, uh, repair timing. So when the children are born, there's obviously a a major assessment done by the medical team and then the neurosurgeons are very quickly involved in um planning the surgical closure of the of the back um uh uh abnormality and then once that's set, um, urology should be involved early on partly so we can come and meet the families and and reassure them or counsel them and sort of prepare them. Um, and then to, uh, start the evaluation of the, of the urinary tract. So getting to the, the main question that you were asking, generally getting a baseline, um, renal bladder ultrasound and, um, uh, VCUG or voiding cystourethrogram are some good baseline, um, imaging studies that are available at, at most every, um, major children's hospital, and that gives you a good look at, uh, making sure there's two kidney. That are morphologically normal, you can detect the presence of hydronephrosis. Um, you can look at what the bladder shape looks like, um, and, and it's a kind of a good baseline picture. Um, the VCUG is a test done and on in flu, you know, with fluoro, um, with a catheter being placed, and then that gives you the ability to look for the bladder shape, um, the presence of trabeculations, which are, you know, which is a, is a word we use for like thickening within the bladder or, um. Uh, increased muscle within the bladder, which sometimes is indicative that the bladder's not normal. Um, it looks for reflux, which is, of course, backflow of urine into the kidneys, and then it can give you an idea about the shape, um, and contour of the bladder neck and urethra. So you can get a lot of information from both of these, um, studies, and, and one thing that's an important take home too is that the majority of kids with spina bifida, um, will have very normal studies at the beginning. So it's nice to get that baseline picture. Um, other than imaging, we also like to plan for a test called urodynamics, and this is very urologic, but this test, uh, most people like to do within the first month or a couple of months of life. This involves placing a catheter into the bladder, um, and then filling the bladder, and this gives you information on what the bladder. Activity is like, it gives you information on the pressure within the bladder, and that's a key thing that we'll keep coming back to you because a normal bladder should be a low pressure kind of situation as it fills, the compliance should be good, meaning that the change in the pressure as the volume changes should be very, very little, and that's the hallmark of a normal bladder. So in pathologic or neuropathic bladders, they have a tendency to, um, have higher pressures, and that is the, the most dangerous thing to the urinary tract or to the kidneys overall. So we'll come back to that again. But the, um, urodynamics allows us to kind of get a sense for if the bladder is, um. More normal in its in its function early on or if it's what's considered hostile and hostile is a term we'll use too to indicate that the bladder has a high pressure, that the bladder is demonstrating signs of instability or it's contracting, um, during the filling, which is abnormal. So your bladder should be kind of quiet and relaxed during filling. Um, and so all of that information early on gives us a way to track what's going on with your urinary tract as that child grows. And it can also give you an idea about who you have to watch more closely or you might, who you might have to be more worried about early on for hurting their kidney. Um, so, so those are kind of our early, um. Set evaluation techniques and I think that's fairly standard across the board for most urologists. The timing of all of that, um, can get a little bit grainy and remains a little bit controversial. That's what I was about to ask. Uh, one other thing that I have to put out there is that the urodynamics, um, also allows you to look for something called, um, DSD, which is, uh, one of our many abbreviations but stands for detrusor sphincter dysynergia, and this is something that can be seen in any neurogenic bladder situation. So for again my pediatric surgery colleagues, you know, in the setting of a spinal cord injury or, or spinal cord trauma. Um, those patients can also develop this pattern of DSD, and DSD means that there's a discoordination between the bladder and the sphincter, and you don't think about it in normal life, but those are regulated, um, between very complex pathways between the brain and the spinal cord, and then the, the nerves that go to the bladder. And so for a normal person, when you go to void, your sphincter knows to relax and open so that. There's no resistance to that at all. But when you get into a situation where things are discoordinated, and one part of the bladder doesn't know what the other part of the bladder's doing, you can imagine that voiding, or trying to contract your bladder while the sphincter is still tight and closed, um, can lead to another high pressure situation. And that, over time, can again result in high pressure that the kidneys are seeing. So, that is why. We will talk about some of the, the strategies for trying to get around that high pressure situation or how we can, um, obviate a sphincter that doesn't open, um, in response to, to your own body because, because the, the reflex mechanism is broken. And so, it's important that, that, um, that's something that we're paying attention to, not just for spina bifida, but in the setting of a spinal cord injury where those, those reflexes can be interrupted. So, Doctor Wu, you mentioned high pressure bladders that aren't functioning well. And we know that prior to the widespread use of clean intermittent catheterization in the 70s, the most common cause for mortality in these patients was renal deterioration. So, how do you decide when to initiate intermittent catheterization and what factors are you using to base this decision? So, this is definitely key. So, um, Al mentioned the concept of intermittent catheterization, and that really is the mainstay, I think, for management of these patients. And so, um, intermittent catheterization is, is basically, um, straight catheterization, and it's done on a, on an intermittent basis, meaning you're not leaving an indwelling fully. And the idea behind CIC or, or IC, uh. Um, and catheterization is that on a scheduled basis, you're going in, or the patients, you know, as they learn to do it, or their parents are doing it, to empty that bladder. And that will, A, make the bladder empty completely. It will help with the overall pressure situation because now you've taken a filled bladder that may be under pressure and taken that out. And it also allows the patient to empty if they weren't otherwise able to do so because of a sphincter problem. Um, as we had discussed earlier. I also want to add, to make things more complicated, that just by the same effect that the bladder might be over, um, or high pressure or overly contractile, some bladders are reflexic. So, they just sort of sit there and hold the urine. Urine, but don't really efficiently empty the urine, for which you need a good to choose your contraction. So, again, basically, the concept of CIC gets around all of those things because it enables the patient to get their bladder emptied on a regular basis. So, kind of in the bigger picture, when do we start doing that? And it is definitely a controversial thing in the realm of urology. So there are some people that feel all kids from birth with the diagnosis of spina bifida and neurogenic bladder should be initiated on CIC as soon as possible. Parents should be introduced to the concept, um, and that. Can do many things. One, it can familiarize the family with what otherwise seems like a fairly complicated or daunting task. Um, it allows the child to accept it sooner. So you can imagine if the child's been catheterized for their entire life, that's much easier than if you're telling a patient who's 6 years old that it's time to start catheterizing. So, that's the second part. And then maybe the most important part is there's some data now coming out that suggests if you do it early, you may actually be able to influence how that bladder develops, and you may actually improve the overall bladder dynamics as that child grows. So, it can be potentially protective, um, in, in a proactive way to start catheterization early. Um, kind of the more old school or traditional way was you would do all the tests that we talked about in the first question, where you're assessing the urinary tract, looking at the numbers on your urodynamics, and then only starting those patients with worrisome features or hostile bladders on the CIC regimen. And there are plenty of people that still do that, large in part, largely in part because we worry, are we going to be increasing the infection rate in these patients? Are we doing something that's unnecessarily invasive? Um, do we really need to do it? Um. But the, the argument, I guess, you know, or the, the worry about starting it sort of Expectantly is that maybe there's already been damage done at that point, um, and you're kind of doing it on a later basis. Like you've, you're waiting to see something bad happen before you pull the trigger on starting this intervention. So it's less less preventive then it's less preventative, but, you know, the, you know, and then you're asking a family to adopt, you know, a kind of a brand new concept to them, um, at whatever age the child. Happens to be. But, um, but I think urology still remains divided and there's a lot of studies that are coming out that maybe will, um, shed more light on what's the right thing to do for these patients. Um, but I think in general, um, most of us would agree that we'd have a low threshold of starting it, and it's always easier to say after the family's gotten used to it, that if you've done all the tests and everything otherwise looks reassuring. Then maybe you can feel more comfortable about withdrawing that for a period of time while you watch the child closely. But, but, you know, I think in my practice, I tend to have a low threshold to get everyone on board with it, have the families learn to do it, and then if we find that that particular child doesn't necessarily need it, or is having problems with infections or, or, or other reasons to not do it, it's easier to sort of stop it for a little while after that period. So Doctor Wu, are there any strong indications, it sounds like there are people in both camps, um, on whether or not you should intermittently catheterize, but are there patients that you absolutely should be intermittent catheterizing? That's a great question. Yeah, so I think most of us would agree that if the, the pressures on neurodynamics are very high, and historically we use, uh, 40 centimeters of water as our cut-off point for, for pressure. Uh, that's a worrisome situation, so those patients should be initiated on CIC if there's already evidence that there's hydronephrosis or, um, uh, a small capacity bladder, um, or evidence of reflux because of the pressure, those are reasons to do it, um, or if the urodynamics suggests that condition of DSD where we know that the bladder and the sphincter are not coordinated, those would be reasons, um, to do it. Um, so Doctor Wu, we've talked a lot about managing the patients, but in the pediatric population, we're also gonna be managing the anxiety and expectations of the parents. So what is your approach to counseling the the parents of these patients and discussing goals of care? Super key. And I know my pediatric surgery colleagues will understand that because you are not just managing the child, you're managing, um, their guardians as well. So, I think this is like any major congenital anomaly. It can be extremely overwhelming for for parents who have a newborn, um, and then they're being told that there are all of these things that they have to be, you know, worrying about as that. Child grows. So, um, from the get-go, I think it's about providing clear education, um, and support. And this is one of those entities that's really well managed in a multidisciplinary setting, because there are so many different parts of the body that, that can be involved. And so, getting patients and parents plugged into these support services within the hospital or within. In the community, um, is really important. And then I do try to start by explaining, you know, for, for urology, what our priorities are. And that would be making sure that we're safeguarding the, the renal function or the kidney function as a secondary part, um, as the child grows, then we'll have that discussion about social continence. And for every family, that's a different priority as well. And then, kind of hitting home that this is something that's going to require lifelong management and follow-up. So, this family is going to get to know their doctors very well. Um, and, and I think that conversation sort of has to be had multiple times in multiple stages because You can't, you can't expect all of that to be absorbed or accepted, um, after one conversation or after two conversations. And, and many parents sometimes even after a few years of management will still come up with a question or will demonstrate some behavior that makes it clear that they still don't quite grasp all of the things that, that you're trying to emphasize to them. So, I think it's about patience, clear communication, um, and, and, um, Repeating, uh, what your priorities and goals are, uh, with that family and patient. Doctor Wu, we've touched on, like, even just now, the multidisciplinary. Care that these patients undergo and I think earlier you also touched on some of the times where you interface with the pediatric surgeons. Can you expand on that? How are these patients managed um with so many disciplines working together? So most hospitals, most children's hospitals do have what's called a spina bifida clinic or a Mlo clinic, which, um, enables a team of, of practitioners to get together to see these kids in a, in a one day kind of comprehensive clinic, and clinics can be different and modeled differently, but the general, the general clinic would have a neuro. Surgeon, um, an orthopedist, uh, neurologist, and then there are some other adjunctive, um, specialists that are, that are very useful. So, having, um, pediatric surgery involved, having GI involved, having social work and a developmental pediatrician, um, those are all, uh, I'd say bonus and, and good things. And not every program necessarily has, um, time or, um, faculty to always, uh. Uh, devote to these kinds of clinics. Um, but you can imagine these children may require other things in addition to what's, you know, specifically related to their bladder or, or back. They may require G tubes, they may require, um, um, Orthopedic, uh, procedures and, and other things that, that, um, that complicate the picture. So, so again, it's overwhelming, but having that team approach I think is the best way, um, to manage these patients since none of us are experts in everything. Um, having a, a, a care coordinator is also good to sort of rope it all together. Just like our comprehensive care patients who, who are, who have, you know, many, many issues and where the families benefit from putting all of the experts, uh, advice into. To a cohesive plan for that child, knowing that that's gonna change as time goes on. And I think it's good for the providers to also be on the same page about, oh, so and so's gonna have a surgery involving this. Is there something that I wanna do at the same time? Or does that affect something that I'm doing? And so, um, so I think really the, the optimal management of these patients has to involve several caregivers who are in good communication with each other about what's going on. Um, so we've had a lot of discussion about the initial management for these patients in those first few weeks of life. Um, I just want to circle back. You did mention that the timing for studies such as the BCUG and neurodynamics aren't set in stone. So I guess for the sake of discussion, let's say we have a patient that's now 4 months old. They've been doing very well in their clean intermittent catheterization. Um, they haven't had any urinary tract infections. Would you get any additional testing now that this patient's a little bit older? So, um, we'd make sure that the urodynamics, um, had been completed by this point. So 4 months old, um, baby's already getting older. It's not unreasonable to consider repeat imaging with a renal ultrasound. So, for most of us in pediatric urology, the kids are growing quickly, the kidneys are growing quickly, and so trying to do surveillance. On the upper tracts, um, is important, what, what we call the upper tracts, the kidneys, and so getting another ultrasound, usually we would do that probably every 3 to 6 months for the first several years of life just because the child's growing rapidly, and many, many clinics have that, um, available to the patients, um, when they come for their normal urology follow-up. And so, um, uh, so that testing would be involved and I, I, um, would definitely praise the family on their ability to, to keep up with the CIC. I probably wouldn't change anything right now since it sounds like they're doing very, very well. I would say if all of the numbers on the urodynamics, the imaging, and again, the family's response to doing the catheterization is great, I would say let's continue, um, doing that for now. Uh, one other thing that may not be clear is, um, in addition to doing intermittent catheterization, sometimes we'll find that the, the pressures are still high or concerning. And one other adjunctive measure that we'll frequently do in neurology is add a medication, um, which is called an anticholinergic, and, and many people know it as oxybutynin or Ditropan, and this is a safe, well-accepted, uh, medication that, um. Helps to sort of relax the bladder or, or increase the compliance of the bladder. For non-neuropathic patients, we use it to treat overactive bladder, so that, that feeling of needing to go all the time. And so we found that in this population, the anticholinergic medication in combination with intermittent catheterization is sort of our, our key combo therapy for how you manage a hostile bladder. So. I, just to continue to dig up at, you know, the controversy. Some people even suggest that all patients with spina bifida should be started on oxybutynin early, again, as a prophylactic. I haven't seen enough evidence to say that that is necessarily supported. Um, because it's a medication and can have its own side effects. But, um, but that is the, the second weapon, sort of in our armamentarium, when you're doing CIC and there are still some concerns about that, the bladder pressure, or, or findings on imaging that suggest a high bladder pressure. So if you were to have that patient with the high bladder pressure, the concerning findings, and you're on anticholinergics and intermittent catheterizations, how often are you re-imaging them and reassessing them? So, great question. So, in general, if things are going well and the patient's stable, um, most people would say trying to do the renal ultrasound every 3 to 6 months, and there's various protocols for that. And then doing urodynamics somewhere between the, the neighborhood of 6 to 12 months. And I think that, the. Intervals depend on how worried you are. So, in, in a, in a patient who's clearly demonstrated some hostile characteristics or in whom you question the parental compliance with the plan that you've laid out, you'd want to keep a closer, closer tabs on that and maybe repeat, um, imaging and testing sooner. But in general, um, from a practical standpoint, trying to do it, you know, annually, um, ends up being kind of what happens for most of the patients and hopefully can help you catch any major changes. Um, also, if you're making a change to the regimen, so if you add in a medication in response to a concerning result, you might want to follow up sooner to try to get some quantified, quantifiable data that what you've done is making a difference. Um, so we've talked a lot about the, the patient that's doing pretty well on these conservative measures with the catheterization and oxybutynin. Um, so let's move forward with another case. We have a, a one year old child. They've been compliant with the catheterization, they've been compliant with the medication, um, but when you repeat the urodynamics, you see that the bladder pressure is greater than 40 centimeters of water. Um, so what's going through your mind at this point and how are you changing management for the patient or maybe not changing management and why? So this is definitely one of the thing, the scenarios that you worry about, and I wish there was like a, yeah, we know exactly what the answer is. So far, I'm not getting any indication about the kid is in trouble per se. I don't have any, um, you know, all I know is that the pressures are not reading the way we want, but that the, the family is. Is, is doing the things that we've asked them to do. So, what, how worried I am sort of depends on how long they've been aggressively managing with the intermittent cathing and the, um, medication. I'd wanna make sure that the that the that the medication was maximized, um, to the, to the greatest dose tolerable by the child. And then, probably, this would be, and if, and if those things are, are being adjusted, then this is that child that needs to have very close monitoring and close follow-up to see if there's worsening of that parameter. The, the hard part is, we don't know over what time period you're gonna lose kidney function. So, there's not a Hard and fast rule to say, oh, if your pressures remain above 40, this is going to lead to loss of X amount of GFR in, in what period of time. We don't know. And so, um, that's where it also, you know, this is why the, the art of medicine is important. And, and again, talking to one. Urologists might get you one management option versus another one. And I think that also plays into the dynamic of how that family is. And if you know that that family is going to return for all their follow-ups and allow you to, to, to closely monitor them versus are they going to miss the next 3 appointments that you schedule for them. Um, but suffice it to say for that particular scenario where the family's doing these things, and all I have is that the pressure's high, um, probably I would say needs to have close monitoring on upper tract imaging and, and urodynamics. But I don't think you can really increase the frequency of the intermittent cath at this time. And if you've maximized the dosage of the medication, there's not much you can do at that time. So, it may lead us down the road where if subsequent follow-ups don't demonstrate any improvement in the, in the parameters, we may have to do other aggressive management options at that point for that child. So you mentioned, uh, aggressive management options and. You need something other than just the high bladder pressure to consider going down that route. Um, so if this patient were to have additional findings such as, um, high-grade reflux or recurrent UTIs, would that be an indication to do something more aggressive or, um, choose a different route of management for this patient? No, so that's great. And so again for our non-urology listeners, so reflux in the urology literature, it can happen for a couple of reasons. Some kids can just, kids with non-neuropathic bladders can have reflux, and we feel that that happens for a, uh, uh, a reason about the, the bladder and ureterral anatomy. But in kids with a neurogenic bladder or abnormal functioning bladders, reflux can be what we call. Secondary. So, the pressures within the bladder are so abnormally high that it overwhelms the valve mechanism where the ureter meets the bladder, and then you get back up. And to us, that's a very poor sign. If, if it's, the pressure is so high that it's actually forcing urine back up into the kidneys, this becomes a very, you know, visibly dangerous situation. And then And combining that with recurrent UTIs is also, or urinary tract infections, is, is equally deleterious because now you've got backup of a high pressure situation. So you've got, you're hammering the kidneys with high pressure, and you are potentially showering the kidneys with bacteria, which now have an escalator to the kidneys, if you will, through this reflux mechanism. So both of those things are, are terrible because now you're setting yourself up for kidney damage and or pyelonephritis and kidney scarring, um, which is also gonna take down future kidney function. So in this situation, you need to kind of act aggressively and figure out how can I. obviate that high pressure situation or how can I fix kind of what's going on. And so now we're gonna go down the road of what are our, what are our get out of jail cards for a patient in this situation. So, going back to this scenario, it's a one year old, so it's an infant. Um, um, they're still, you know, they're still small. Uh, one of the gold standard ways that we in neurology will get around this is a, is a, uh, cutaneous diversion. So we will do a cutaneous vesicostomy, um, and we can talk more about that later, but that's, uh, a, a fairly straightforward procedure where we will open the bladder. At the dome and sew it to the skin in the form of a an incontinent stoma. And we do it kind of at the level just above the, the pubic bone, so it drains to the bladder, but this, it essentially prevents the bladder from filling and so it takes care of that pressure situation. And if you take care of the pressure situation and the bladder's not filling, then no more reflux. And so, it's not necessarily an ideal long-term situation, because you can imagine as the child grows, um, you know, the diapering may be a bit more difficult. Um, but that being said, there are some older patients and even adult patients that might benefit from this sort of diversion. Um, We try to avoid placing tubes or catheters long term for these kids. suprapubic tube, you gonna be ideal. It's gonna lead to encrustation and, um, infections, um, and it's gonna get dislodged by an active toddler. Um, one of the ways in which, um, a hostile bladder could be managed in a one year old patient. Um, where there really is concern about, uh, reflux and upper tract damage is performing a cutaneous vesicostomy in which, uh, the bladder dome is opened and brought to the skin in the form of an incontinent stoma, which then drains to the bladder around. On the level of the pubis and this can be left in place, um, for as long as the parents and patient are comfortable, but you can imagine as the child gets older it can be a little bit messier, um, to, to maintain the diaper for this situation and so in general we don't view it as a permanent option. But certainly when you're worried about a child's upper tracts, this is our easiest and quickest way to decompress the bladder, um, and a bladder that is freely draining through the vesicostomy won't have the opportunity to fill and reach high pressure and won't have the opportunity to reflux, um, and hopefully will prevent further cases of pyelonephritis. Doctor Wu, when you're making these vesicostomies, you said they're, it's a temporizing measure. So when do you decide to take that down and then do you continue to follow that patient? Um, so, in general, it's thought of as a temporary kind of, um, situation. It can definitely get messy, um, to diaper, um, particularly as the child gets older and bigger, um, and, and is potentially making more urine. The timing of takedown really is, again, it plays into The family dynamic, the parental, um, acceptance of, you know, what the next stages are, which we're gonna talk about, I think, um, um, later on in our discussion. But when you are deciding to close your vesicostomy, there has to already be thoughts about How are we gonna manage this bladder? Because once I put things back, it's going to likely, um, be the same situation we were, um, when I first made the vasicostomy. So, we have to start having a plan for how are we going to continue to manage this bladder safely once we close the vasicostomy. So I think you are alluding to this in the, in that response, but let's move on to another case. I think that will help dive into that a little bit more. So, we have a 5 year old um female with neurogenic bladder. She's about to start kindergarten, and parents wanna have better control over her incontinence or over her continence. She leaks despite cue for hour intermittent catheterizations and being on, I'm assuming max dose oxybutynin. Dude, you sound like a radio, I mean a urologist at this stage. I'm learning, yeah, you sound like a urologist, um, so. We, we're changing gears. This is an older child now, and this is a classic thing that comes up because parent parents have been, you know, hopefully very faithful about doing all of the things that, that need to be done and the child's starting to be very socially aware and, um, continence can become a. Bigger issue and surprisingly, this is very personal, so it's not a priority for everybody and I think a lot of it depends too on um kind of the cognitive status of the child and whether there are other medical issues that take precedence um, but continence is is definitely a very important issue. So, I will tell, um, all of our listeners that this is one of the most challenging parts of managing, um, these patients. So, uh, in general, continence is very important. That's what we do as urologists. But it's important to know that continence in this situation comes at a price. So, anything. That we're going to do to try to help that patient gain continence could adversely affect how their bladder, um, works. And you can take a situation that was previously non-hostile and create a hostile situation based on something that you're surgically doing for that patient. It is very important that you select your patients carefully and that you do a lot of counseling with them. As you're starting to dance around the idea about urinary tract reconstruction, as it relates to continence and independence for this patient. So, we talk about all of these things, and the, the next hard part is to determine what is the reason for the patient's incontinence. It can definitely be multifactorial. We know that the bladder may not be normal, so maybe the leakage is happening because the bladder's squeezing when it shouldn't be squeezing. Maybe the leakage is happening because the urethra is not normal. So the sphincter may not open or close, uh, at the right times. It may be always open, and in that situation, the patient will leak all the time. Um, it could be that the bladder itself's just not large enough to store the urine that's needed. So, after a very short period of time, it's already at capacity and then the urine leaks. And it could be a combination of all these things. So it's up to the urologist to put all of the tests that we've been talking about together and try to figure out what is the, the main reason this patient is wet. And then that leads you to what your surgical plan would be. So, our surgical reconstruction can consist of, and any combination of. Physically enlarging the bladder and that's done through an augmentation and the and augmentation is typically done with um bowel. So a segment of bowel enter your pediatric surgeon. Exactly. Um, there is also, uh, the second part which is doing something to the bladder neck or sphincter mechanism. So we can't create a sphincter, but we can do something that hopefully will increase the resistance to the outflow of the bladder. So that's tightening up the bladder for, for just to keep it simple. Um, and then, um, a final part of that is, if you do something to increase the size of the bladder, and you do something to tighten up the bladder, you need to give that patient a reliable way to empty. Now, some patients will be able to continue to catheterize their urethras, um, using their intermittent cath. But depending on the type. Of bladder neck procedure you do for them, or depending on their habitus or gender, it may not be ideal for them to cath through their native urethra. And so for that, there's a variety of catheterrizable channels that can also be done, and most surgeons will do that at the same setting of the bladder and bladder neck surgeries. And so there's a variety of those that can be done. And so at at various centers, um, the pediatric urologist tends to perform these fairly independently. I, um, have a really good relationship with, with our pediatric surgery colleagues and I really like to make it, um, a very multidisciplinary collegial experience in the OR so they. my colleagues are happy to come in and help me with the bowel work, which I find, you know, very reassuring. And also if there are complications, you know, that relate to the intestinal surgery or the, the exploratory laparotomy, um, it's nice for them to, to be on board helping me manage those patients. So we can talk about each one of those surgeries, but generally, it's sort of a menu of options and picking and choosing what's appropriate for that family. And you can imagine it's very individualized for each patient. Um, some patients are more mobile than others and can walk. Um, and are more dependent. Some are more wheelchair-bound or, or more paraplegic or quadriplegic and may not have full use of their upper extremities, and a lot of that, um, factors into your decision-making as well. And I think the final situation that's becoming more apparent is that In the beginning, the parents are usually very, very involved and, um, doing all of these things. And I think the goal is to try to get the patient to be as independent-minded and independent in their care as much as possible. And, and that's not always feasible, and, and sometimes you have to also look at the maturity of the patient, but in this, in this current, um, Day and age, our myelo patients are living a lot longer. And sometimes, um, you know, the parents are aging, um, as their, as their children are getting, um, into young adulthood. And so, there has to be some understanding that if a child, you know, the parents may be very excited to sign up for any and all of the procedures you're describing, but who's going to manage that? When the parent's not there. And so, I think that that opens up a whole another discussion that can be had, you know, in, in a separate, in a separate podcast, if anyone's interested about what happens to these patients later. Um, and if they do go into a nursing home type situation, is anyone going to be able to catheterize them? And, um, And if we don't have good, a good idea of that, what, what are the ethical considerations about moving forward? There are all these amazing, sexy sounding surgeries that can be offered, but whether or not that's a safe option for that particular patient, um, is again, at the discretion of the physician. And again, relates to, um, kind of the art of medicine. So, you can, can, you can. Can you can always, but should you? And for that particular patient, is that the best thing for them? So, so getting back to some of the nitty gritty for the surgery, so enlarging the bladder typically consists of an augmentation cystoplasty where a small segment, uh, I'm sorry, where a segment of, uh, bowel, either, uh, ileum typically or even colon can be harvested, detubing. lar ized um, and then, uh, anastomos to a widely opened bladder to kind of create a new roof on the bladder, if you will. Um, and this can increase the capacity, uh, of the bladder and then help with the pressures, obviously, and the detubularization, um, helps with any of the inherent contractile, um, properties of the, of the bowel segment. Um, colon tends to be, as you can imagine, much more contractile. Um, and so, that's sort of the, kind of been the, the mainstay. There are historical uses of like the, the, the stomach, um, which, uh, has really fallen out of favor because of a lot of complications, and we'll talk about complications, but, Um, typically like a 20 to 30 centimeter segment of, um, small bowel is the, the is typically what's used based on how much bladder capacity you are starting with and how big of a, of a, of an augmented bladder you want to have to end up with. Um, for the bladder neck procedure, there's a variety of things you can use, uh, rectus fascia. It's where you harvest a strip of rectus fascia and you create a sling mechanism that kind of lassos around the bladder neck and you tighten that up to effectively kind of, um, uh, tourniquet the bladder neck, um, and then that scars it into place. Um, there are a variety of artificial sphincters which we use in adult urology for, for, um, patients with incontinence, and that that involves some, some hardware. Um, and there are a variety of procedures that we can do to, um, actually lengthen the urethra on the inside by sort of, um, tubularizing the distal part of the of the, of the bladder and sort of elongating the urethra on the inside, and that probably goes beyond. On the scope of this, but elongating the urethra may increase the amount of resistance and decrease the chances that a patient's gonna leak. And then the final, the, the most definitive way to, um, eliminate any leakage is to close the bladder neck completely, where you completely divide the bladder neck from the urethra. And like anything, you need to provide multiple layers of closure and hopefully some sort of interposition layer to prevent, uh, a fistula connection, which, which surprisingly, um, can happen. Somehow the, the urine always likes to find a way out. So that's something that, that we always have to struggle with. But there's multiple things, and again, it has to be individualized for that patient. And then in terms of the channels, um, the, the most common one is the metrofenop, which is, um, better known as an appendicovasicostomy, where you would remove the appendix. So whenever you pediatric surgeons are taking out appendixs, I always cringe because it's a waste of an appendix. Um, I wish those could all be kept, um, for rainy days, but the appendix is, um, preserved on its mesentery and disconnected from the cecum. And then it's tunneled into the bladder, um, in a way that it won't leak, where one end goes to the bladder, and then, um, the, um, uh, proximal end is brought up to the skin as a cutaneous stoma. And then the patient catheterizes this. And this is a An alternative to the urethra, but allows them to empty. And certainly, if you, um, do a bladder neck closure, or you do a very complicated bladder neck procedure in which catheterization would no longer be possible through the urethra, um, having a, a channel like a metrofenoff is, is, is required. Um, if there's no suitable appendix, um, you can actually create a, um, another sort of, sort of tube using ileum where you, you, uh, this is called a Yang-Monty tube, where you harvest a small piece of ileum, and you also detubularize that in a fashion that Allows you to close it back up. So, if you, you open it sort of transversely and then you close it longitudinally, and I, I should probably show a picture of that. We can, we, we can do like a little drawing of it. Um, so you create a tube that you then plug in the same way you would as an appendix. And sometimes the appendix is not available as we mentioned, or the appendix is being utilized for an antigrade continence enema or a mace, a Malone antigrade continence enema, um, and oftentimes if we do that, uh, the pediatric surgeons may be doing that with us as well, um, as they will often manage, uh, the GI, um, the, the enema solutions and, and the irrigation protocols afterwards. So these are all ways in which we can kind of work together to do that. But those are the most common things and, um, and as you can imagine. All of those things come with their own complications. So, I'd mentioned that when I talk to families, I say that all of this stuff comes at a price, and I wish it could be. You know, as, as perfect, um, as, as we're taught in our textbooks, um, but each of these things has its own specific complications and morbidities that can result because of it. So, Doctor Wu, we have a 5 year old patient. Um, she's also trying to start kindergarten. Her parents are worried about continence, and she's already doing intermittent catheterization. She's on the oxybutynin, on the maximal dose. They aren't quite ready to move on to reconstructive options to manage her incontinence. What are some other options besides a full reconstruction? Further management of the bladder at this point can fall into several different categories, and depending on what is going on with the overall bladder dynamics, um, and sphincter mechanism will lead me to counsel them, you know, on various options. The gold standard for surgical reconstruction, um, would consist of. Augmentation of the bladder, a potential bladder neck procedure, and creation of a continent catheterrizable channel. More recently, uh, urologists are offering Botox injections done cystoscopically. Um, and the Botox, um, is a method by which we can sort of paralyze the diffuser muscle and help potentially with some of that overactivity or high-pressure situation. And This is being viewed favorably because it obviously may help delay, um, the need for a major reconstruction. Um, it may allow some kids to, to manage their bladders safely for a very long time, um, and may obviate the need for, for major surgery. Um, but it's, you know, like, like any Botox, it's not, um, a permanent situation. It does have to be repeated. Um, and ultimately, it may not solve all the problems related to continence. Um, it may help with the overall pressure and, or capacity and, and, and those features on neurodynamics, but may not be sufficient, um, to get the, the child, uh, as dry as they want to be. Or, or it may not be effective. in, in reducing the pressures like we, like we, we hope. But, um, Botox is a viable alternative that's being offered by many, um, to try to stave off that, that eventual need for, for surgical reconstruction. So you've followed these kids sometimes for 18 or 20 years, and you've probably developed a close relationship with them. Um, but ultimately they're gonna have to transition to care with, uh, adult provider. So what are some challenges that you face in transitioning these patients from a pediatric, uh, center to an adult clinic? So, those are the very challenges that we are all, um, struggling with now. So, you know, the pediatric management is as, is as clear-cut as, as we can make it. And I think that we at least have some sort of guidelines to follow. But it It is definitely uncharted territory as you're getting into, um, adulthood. And again, we, we discussed the challenges of gaining independence, um, and, um, having less reliance on their, their parents or their guardians, um, and taking charge of a complicated health history. Um, they, as they grow, will have the normal questions about, um, about sexuality, about fertility, about, um, self-image, and I think that it's important that there are. Um, mechanisms to support this. And I am embarrassed to admit that this isn't well, well fleshed out, um, at many, at many hospitals. I think that that's the next priority, um, to, to get a good transition. A transitional plan for these patients. I will say that there are a number of programs with really good, um, um, transitional clinics planned out where there's a literally a handoff or a slow, a slow handoff, um, starting in the teenage years, getting these patients. Introduced to the adult providers, um, and trying to, um, make them take charge of their appointments and understanding what's important in, in their health. Um, disappointingly, though, there's multiple barriers, which would involve the patients themselves sort of getting lost to follow up. Um, Being, you know, a young adult, you, you don't necessarily think about all of your health concerns. And then, um, a general, you know, mistrust of the healthcare providers. And, and I think there's also that sort of rude awakening when you get into the grown-up world where you're not necessarily being shepherded or, or, um, hand. Held through every process. Um, and then on the flip side, there's the challenge of our adult providers not necessarily being comfortable managing, um, these patients with complex congenital anomalies. And I think that our pediatric surgery colleagues can probably sympathize because, um, many of these patients do have. You know, a long list of surgeries and, and, and, and complications that, um, make it, uh, fairly daunting for a, you know, a, a general adult provider to want to take on. And so, you know, the key, I think, is, is Finding a next generation of providers who, um, are interested and passionate about, um, reconstruction, um, and, uh, young adult medicine and, and ideology and then, um, also wanting that continuity of taking these patients through then from young adulthood into, um. Um, even to geriatric ages, you know, where they're gonna have other issues that, that come up related to their health, um, that just come with the normal aging process. But I think getting providers who understand all these key issues the way we think we do impedes, um, on the adult side is, is key. And I, and I don't think it's, it's necessarily a, you know, a general provider that wants to do that. I think that there has to be a, Um, a, a specialty almost that wants to devote itself to congenitalism or a, a transitional type, uh, practice, where they're going to see the challenges of, of these, of these patients and their surgeries, and have to redo some of these surgeries or revise these complex surgeries. I think the emphasis is just gonna be trying to transition at the time of adolescence, um, so that they can be better prepared to take charge of themselves as, as adults. And then that's easier said than done. Dr. Wu and Al, you've both given us a lot to think about and learn from the urology perspective. I was, I was wondering that in these last few moments, if you could highlight some key clinical takeaways for our learners out there, like that can just be a little refresher to bring home the main points at the end of this, this hour or so. So, key factor number one. Um, neurogenic bladder is a dynamic and, and changing situation, um, particularly in our pediatric populations who are growing, um, physically. Um, and so, uh, close observation and, and, uh, surveillance of the urinary tract is really important in this population with the goal of maximizing, um, renal function and preventing loss of renal function as that child grows. Um, challenges to this include, um, um, the amount of testing that's needed in the form of, um, imaging and neurodynamic studies, um, the need for parents and patients to be compliant with catheterization and or medications to help relax the bladder. And then, um, if we're in a situation where we feel that the neurogenic bladder is adversely affecting renal function, or has the, the, the possibility of affecting, uh, renal function adversely, um, or is causing quality of life issues with incontinence, um, then there are a variety of surgical options, some minimally invasive in the form of Botox, some maximally invasive in the form of Urinary tract reconstruction that can be offered, um, to, to help with all of these problems. Um, these procedures can have, uh, morbidity and complications that are important to know. Um, and patients will continue to require lifelong follow-up, um, especially in the setting of, um, reconstruction. And, uh, a need for Uh, comprehensive transitional, uh, care, uh, in this patient population is sorely needed, since many of them are living well into adulthood, um, and, um, are experiencing older parents who can no longer provide the kind of care they did when these patients were children. And that remains an ongoing, um, clinical challenge for all of us in, in neurology and medicine. That was a wonderful summary. So thank you, Doctor Wu. Thank you, Al, for both being here and educating us on neurogenic bladder today. Thank you so much, Ray, for setting this up. This is a lot of fun. Appreciate it. Happy to participate, Ray. Thank you for having me. Now that we've discussed the management of pediatric neurogenic bladder and the setting of spina bifida, what are your key take-home messages from this talk today?