Surgical Management and Follow-Up: Cloaca and Complex ARMs 2015

Um, so is that the, was that the first slide that I had? That's the first slide. So we sort of touched on this already, so I'm going to go straight to your urology stuff if that's OK. OK, that's fine. All right, so we touched on the team approach. Um, if you want to talk about the goals and the definition, that'd be great. So the whole purpose here is that we all gather together and we work as a team to, you know, really have the patient have a full life, and that really means that all aspects have to be kind of looked at from nutrition, growth and development, renal health, future sexual health, continence, everything has to be looked at in continuity in order to promote this concept of holistic care and to in fact do the right thing for the patient for the long term. And unfortunately, I've always been, you know, uh, uh, a little bit of the person who tries to, to stir the pot a bit. So, I think we're gonna go ahead and discuss some of the, some of the nuances of the management and the long-term follow-up for the patients. So, um, I think it's always easiest if we define the neurogenic bladder, and I, I define it very simply as, you know, either the ability to store or the inability to store or empty urine. And um thank you. So, um, this Brian touched on this point a little bit earlier that continence is not necessarily a measure of a healthy bladder or healthy upper tracts, and voiding may in fact be possible, but in some cases, voiding can actually be harmful, so it might not necessarily be the end point that we think about. Next slide. So this, I, I always took, I took this from Doctor Pena. Um, he always said there are many ways to get to Rome, and I do agree with him. But the point that I'm trying to make is that we, if we all individualize our management based on just our beliefs or our biases, we actually don't have any demonstrable outcomes to measure, and at the end, we may never know if we're making a difference. So in practicality, in terms of looking at interruptum affirmations and trying to think about the future for our patients, I think we have to agree on some definitions and standards. Uh, it may not be possible right now, given the information we have to risk gratify patients based on the size of the common channel or based. On whether or not they have one kidney or a tethered board plus one kidney, but we should have consistent treatment algorithms, so maybe in a few years, we can re-stratify the patients. And in the hopes of risk stratifying and, and sort of doing the right thing, we also want to try and reduce variation. And in, in that vein, we want to provide support for the families, and I think the multipledidisciplinary team is actually a very important concept in order to achieve those goals. So the question that gets us a lot is whether we should cath or not cath, and I actually don't think that's the correct question. I think that we need to understand that the management of the neurogenic bladder requires a multifaceted approach. We can't necessarily heal with steel or do surgery to make things better, and our goal ultimately is to protect the upper tracts. So in that spirit, let's talk about a case study. Dr. Pena knows this patient very well. It's a 30-year-old female with a long common channel cloaca. As a child, she underwent a peace arc with a neovagina. And after surgery she tolerated a voiding trial, meaning that she was continent and she did urinate. Over the course of her lifetime, she may have been lost to follow-up from a single provider, but she had multiple urinary tract infections, was in and out of various ERs, was found to have vesico uterine reflux, and was treated by defflux again by another practitioner in another state. She had a history of a left-sided dysplastic kidney which was removed. So she came to see us and, you know, a very state of issues. The VCG, I think was later, but we did a VCEG on her, which shows a good capacity bladder. And then We did a a renal ultrasound, which did show that she had hydronephrosis of the kidney, of her solitary kidney. Her, um Bladder ultrasound showed debris in her bladder. And this layering debris was evidence of incomplete bladder emptying. So for 20 years she had painful voids, urinary tract infections, multiple emergency room visits, a questionable history of a kidney stone in a solitary kidney, hypertension, chronic kidney disease, stage 3, and after, you know, 20 years of doing this, she was diagnosed with some profound psychological and behavioral issues because of her constant complaints of kidney pain. OK. So, we tried to catheterize her to cycle her bladder, but unfortunately, as someone alluded to earlier, the common channel which was left at the urethra had a takeoff where the native vagina was, and that she was unable to get past that point. Uh, we did put a suprapubic tube in, and she underwent bladder cycling with Ditropan, and then she had a ventrofenov and Malone. Post-intervention, the hydronephrosis completely resolved. But the story was a little complicated. There were multiple issues after surgery. It was a very prolonged hospital stay. She had a duodenal um issue that was sort of missed early on and diagnosed uh post procedure. She underwent a redo Malone. Um, she, we fixed her duodenum and uh she finally recovered, but she still had flank pain. She went to Boston because she felt that, you know, we just didn't address her stone. And after another million dollar workup, they found that there was no stone. It was just protein debris in her kidney. She's doing well. Um, she's chronic kidney disease stage 3. She is continent using her metrofenil, but she does suffer from continued kidney pain. So, this is what I always kind of talk about, you know, what is, what is the cost of one nephron, you know, what's really at stake. When children are born, when they're one month of age, they have about Anywhere between 30 to 40% of their actual adult GFR. GFR is still developing. There's still an opportunity to make those kidneys better, no matter how bad the kidneys are. And by sometimes our management of tolerating hydronephrosis, tolerating urinary retention, we may be in fact hurting the kidneys. And with these certain children who pass voiding trials that have future problems down the road, are we actually causing problems? Are we just trading one disease for another? And sometimes this concept of watchful waiting may act of harm, and are we actually creating a new cohort of adult patients with hydrogenic injury? And I don't think that we can answer those questions just yet. So the key points from this particular presentation are that a successful voiding trial is not predictive of future health, and bladder management to protect upper tract injury is actually what we recommend, not simply catheterization or not catheterization. And as part of the catheterization question, bladder management to allow for continence or dry intervals, as well as a yearly measurement of GFR and blood pressure measurement. I'm gonna just interrupt you there for a second. When you talk about bladder management, what do you mean by that? So bladder management sort of encompasses sort of looking at the entire patient. Number one, is the bladder capacity adequate, and we can obtain that information from urodynamic studies. And does the capacity of the bladder, meaning the storage or the emptying of the bladder, put the patient at risk from an upper tract perspective? And that's another thing that we can look at with urodynamic studies. There's not a lot of data. On voiding pressures, but it appears there's certain certain cohorts of children with, as, as Doctor Vanderbrie referred to Cet, that do have deterioration of renal function long term with voiding. So, urodynamics will help us understand that, and the bladder management comes into play when once we make that decision, should we put the patient on catheterization? Is this a patient that would benefit from a vesicostomy? Is this a patient that would benefit from a short-term suprapubic tube? Or is this a patient that would benefit from a Foley, or is this a patient that we can follow carefully and allow them to void? So I use bladder management. This is very vague sort of um concept to kind of see how the bladder matches the patient and the long-term health of the patient. I think I just switched your slide again. OK, uh, it's not up. Oh, it's coming. There it is. So, this concept of risk ratification based on the common channel, I think that uh Doctor Pena's work initially showing the difference between a 2 centimeter common channel and a greater than 2 centimeter common channel was actually very, very important and has helped a lot of people. Sort of not do bad things from a surgical standpoint and understand CAA better. But from a urologic standpoint, the risk stratification may not work. I think that it's not the only risk for the patient, as everyone's alluded to earlier, so the next case is going to sort of outline that. So this is a child, again, another one of Dr. Pena's patients with a 2 centimeter common channel and a normal spine. The only thing that was outside the ordinary is the child had a transverse colostomy at birth, but they did a birth ECG and an ultrasound, and both were completely normal. After the colostomy, unfortunately, she developed multiple febrile urinary tract infections, one episode of sepsis and several episodes of pyelonephritis. In the short term of a few months, she developed significant bilateral hydroureterine nephrosis. The patient finally got to Doctor Pena, who performed her repair at about 18 months of age. And curiously enough, during cystoscopy, we found a very massively trabeculated bladder, again, a short common channel of the spine. So this is a picture of a BCG. It's a side view which shows some pretty significant dilating reflux, and as you can see, the bladder itself, there's evidence of trabeculation of the bladder. Here's her pre-op ultrasound, which shows pretty significant hydrourea nephrosis. The post-op ultrasound, you can see that the hydronephrosis completely improved. The Follow-up BCUG shows complete, complete resolution of the reflux, but it wasn't just the operation that did this. After surgery, we put the child on intermittent catheterization and Ditropan. Again, not common for a child with a short common channel, but with those two interventions alone, massive reflux and massive hydronephrosis resolved. Unfortunately, the cost to the child was that she was CKD stage 3. Her GFR was a little less than half of what it should be, and all this morbidity was acquired because she was born with normal kidneys. But unfortunately, the story gets worse. She said she went home, she was thriving and growing, and the urologist together with the family said, well, we do note that she does try and pee between catheterization, and the question was, does she have a good stream? And they said, yeah, she has a good stream, so we're gonna let her void. So they stopped catheterization. Unfortunately, the reflux recurred. She had multiple urinary tract infections, and the parents just refused to stop to restart catheterization because they claimed the child could void. Child Protective Services was called in. The family were lost custody of the child. A vesicostomy was performed, and the patient is now unfortunately on the transplant list. And so the summary is, is that the 2 centimeter common channel, the normal spine, the normal kidneys at birth, and, you know, a little late to the game, but passing a voiding trial did not necessarily portend a good prognosis for this child, and I think the transplant was probably avoidable, and this child was not cared for by either Dr. Pena nor myself for the long term for all of these problems, so everyone knows. Oh, sorry. So, um, you know, family support is critical. Uh, these patients are resource intense. We have to have nurses and specialized teams to teach catheterization. We have to manage their DMEs, make sure that they get the catheters that they're ordered. There are multiple phone calls from the families. Addressing problems and setting parental expectations is very important and may have helped this patient in the long term. And as well as what happens. When the patient develops UTI. We need to have some guidelines in partnership with the team because maybe the first person who's called, what if it's Doctor Bree, called for another reason and found that, oh, there have been a couple of UTIs. When the team is involved and we all have sort of consistent goals and consistent guidelines for management, we can probably make a difference. You wanna say something, Doctor Bree in response to that comment. I'm feeling it, I'm feeling a little challenged there. Let me just tell you, before we talk about pregnancy, we ask about their kidney function, so. When they have a menstrual problem they call that the doctoralla. There you go. Oh. We'll go back to the slides again. So, um, I think the individual management of the patients is exceedingly valuable, but sometimes difficult to interpret. The literature is confusing. Um, Brian went over a number of different studies. And there's not a lot of consistency out there, and I think that dogma can become paralyzing when it comes to these patients. I think that the opportunity to put everyone's heads together, to meet in sessions like this, and to really speak to a wider audience may get us one step closer into understanding what the long-term consequences of all of our actions and management are for these patients. So, in summary, we follow carefully with ultrasounds. Not all of my patients, despite popular belief, are on catheterization. Yearly measurement of GFR is advised and that can be done through whatever uh system that the hospital that you work at has. I do actually do a baseline form of your urodynamics after about a year of age in selected high-risk cases. Um, delayed urinary control from a concept standpoint is not normal in interectumformation, and sometimes families come to us with the belief that it is normal. Urinary tract infection is also not normal in interrectum formation, and I think this is another opportunity from an education standpoint. And then lastly, incontinence or volitional stream or strong urinary stream does not necessarily mean that the children are healthy, nor does it suggest the absence of a problem. Shamal, Richard had mentioned that they're trying to do urodynamic studies before repair and then do it again after. You may have heard that conversation earlier. I know you have some thoughts about that as well. So urodynamics are a funny thing, and they're highly dependent of, you know, when I was in Cincinnati, I got used to reading neurodynamics, and now that I've moved centers, I actually am the one performing neurodynamics, and I will tell you that being the one who does the urodynamics has tempered my Sort of desire to do a lot of urodynamics, not because they're time consuming, but more so because the information that's given is not necessarily gleaned from a urodynamics. So I do understand the idea of wanting to do a pre and post procedure urodynamics, but I would posit that you may not think that, you may not get the information you think you're gonna get. Um, a lot of information can be gleaned from a simple ultrasound as well as a VCG, especially in the below 12 month age group. Unfortunately, your dynamics in under 12 months of age is pretty challenging to interpret. High pressure voiding is actually normal in that cohort of patients, and so sometimes you may not necessarily get the information you're looking for with urodynamics. I think there may be other tests out there. Are you doing VCUGs and ultrasounds in conjunction in the study? Well, they'll, they'll all get VCGs as part of their cloaiogram. So I mean that takes care of that, uh, in the, but they, at the cloogram, they don't necessarily will have them void or do you have them void because we really just inject contrast and then take the 3D pictures. No I think like, well. I don't know that every single one of them is voiding, but I think you certainly would get quite a lot of that information. Um, I think, I, I, I mean, I take the points completely, but I, you know, we've had a lot of discussion today about when you separate and when you do a total genital mobilization, and that's all based on trying to get an anatomical result, but we don't have any idea. On what those procedures do to the functional function of the bladder, and I'm not saying that it's perfect, but I think the more information we can gather on a cohort of patients might lead us to believe that yes, maybe it's a slightly easier repair, but. With, with a different risk profile either way, but I think we have to try and understand what the effects of the two different techniques are in that. And, and we're really just talking about that like Doctor Pena alluded to that, that challenging group in the middle, the sort of 3.5, 4 to 4.5, 5 centimeter group in any of the patients that we are. Doing surgical repairs on, you know, we can do an anatomical repair, but what is the eventual physiological functional outcome of those patients which, you know, we can only glean from these prospective studies and looking at these patients as they get older and at the moment we have, we don't know exactly. Well, I think you also have to remember that the, the cloogram is done under anesthesia. So generally, that there isn't a, a voiding component to it and it doesn't necessarily give you the information that you would think. The, the trigone seems to be the area. Where the magic happens, at least that some of the literature looking at Botox to the bladder has suggested that the trigone may be this area where the confluence of nerves may have some interaction with the rest of the bladder. Now that being said, that's the part of the anatomy that's altered, especially with separation. And so I think you have to go in with the question. So what, what's the question that you want to know? Are you going to interrupt continents? Well, in that situation, there's typically is no um external sphincter, so that's not easy to test with your dynamics. The intrinsic, the function, the intrinsic sphincter is not necessarily easy to test with your dynamics, and then you're not necessarily gonna affect capacity unless there's a lot of dissection at the trigone. But if you're looking at bridging a long gap, I think that Linda is correct. What's your, what's your long term outcome? Want to make space for three openings when the top opening is never gonna work properly? Or do you, and maybe impact the vaginal opening, the perineal body and the rectum, or do you want to maybe just quit and divide the urethra and the child a very nice droitous, nice perineal body, and a nice anus. Um, it's not, I'm not suggesting I have an answer. I'm just putting that out there. Does any of the panel have any comments with? What Doctor Shama, Doctor Alamm just brought up. Yeah, no, uh, Shamel, I mean, uh, I don't think we're gonna get to it, but we have a case of a like 6.5 centimeter, uh, common channel, and, uh. In fact, I had asked some of the other faculty here about um is that going to be a useful conduit for catheterization because we know that that and this patient has an abnormal spinal cord, prenatal hydroureter nephrosis, a really severe malformation. So, uh, I know Belinda and I were having conversations about whether or not at the time of the primary repair is this, like to use your words, is this a useful top. Uh, orifice or is the child better served by, uh, this child I ended up doing a vesicostomy on actually there's a very retic common channel, um, and, uh, there was a little bit of some discussion, but, uh, what we have opted in this child with while the vasostomy is trying to progressively dilate this urethra to see whether or not that can serve as a, we're not trying to make a. urethra for voiding. So I think the thing that I struggle with at the time of the primary repairs is exactly what you said is trying to make those potential decisions of either closing off the bladder neck or creating a urinary diversion that sometimes have long standing ramifications when either the individual patients are different and so you have to kind of go by obviously your experience from what you've seen. Discussions with other experienced persons, but uh when you're talking about dividing the bladder neck as you said, what, what, what, uh, instead of trying to keep a patent urethra, what type of factors do you make for your decision at the primary care to close the bladder neck? Well, I, I think that's to, to quote Doctor Fena from earlier, that's the moment, right? That's the moment when the surgeon has to make a decision that may not necessarily be in the textbooks. Um, it's good to have that moment in a prepared standpoint, um, you know, maybe counseling the family beforehand and looking at the, that urethra. Uh, Andrea mentioned it earlier that urethra has gotta be straight, right? That common channel has to be perfectly straight, otherwise the catheter won't go in. And, um, you know, I think those are the anatomic sort of decisions that have to be made at the, at the time of surgery. Uh, if you're looking at a patient with the posterior cloacal variant that has a very thickened pubis where the urethra is probably never going to serve a purpose for catheterization. Those are the patients that you may want to divide it, and sometimes you know that going in. And so I think the anatomy really dictates what you want to do, but also, you will have some idea from a long-term, um, functional standpoint based on your testing, what the eventual outcomes are. Unfortunately, catheterizing long common channels long term. It is not always without complications, and, you know, a lot of the patients I, you know, I've chosen to stick with all of us for long term and, you know, we get these phone calls two years later that they're in an emergency room and they can't catheterize their urethra anymore, and you know very well it's a cloaca, and uh, you know, a few months later, you're making a metrofenoff. I don't think we know, have the answer for these um sort of questions just yet. And I think as you know some of the longer ones where we do leave the common channel alone, they are the ones with the thick pubis and and so it's, it, they aren't always the easiest one to, to catheterize, um, so it's, it's something that we all sort of consider when we're doing our repair. So I'll just mention a little difference because I think we got so much discussion here. We have a group of patients with posterior cloaca, those are patients that the single perineal orifice instead of being located at normally where the urethra is, it's actually posteriorly deviated, so you can even think that the patient has a normal anus. Those patients, they have a very short common channel and a very thick pubic bone, so that's when it becomes a challenge to have the three structures, but the actual mobilization is not usually a difficult one because the short, the common channel is short. Now, the long common channel cloaca, that's a different story, and then I think the all the discussion is pertinent. See, let, let me say that, um. From 560 cloacas, these are personal series. I can tell you that we have had an enormous task. Because we are talking about a spectrum of defects that goes from very short common channels to very long common channels from teta chord to normal cord to normal sacrum to abnormal sacrum born with hydronephrosis or without hydronephrosis with reflux or without reflux, some patients have a. Giant bladder even since birth with low pressure at birth. Some others have a small bladder. Some patients switch from big bladder to a small hypertonic bladder. So there's many variables here but in addition, they have a bad surgery or good surgery or repeated bad surgery in many procedures. So if we try to put all those variables into it we need a mayor computer to analyze that and come up with valid valid conclusions. I'm very happy to see that Shumaya, you are, you are getting old. Or mature because you are not so aggressive about Eurodynamics when you when you were here I was afraid that you were going to do a Eurodynamics to me and now you are you are becoming more eclectic and that's what happens exactly with with uh with time so. I agree, I agree with Doctor Pena because I don't think you ever shared the perineum with the vagina so, so kindly. So I think it's New York, New York has been good, right? Well, you know, I mean, oh my. I think what