Cloaca - Urologic Concerns

OK, well, I really like to thank Belinda for putting this together and inviting myself. So, um, obviously, uh, we've heard that the cloacal malformation is a spectrum and, uh, we're trying to artificially make boxes and in the urology world, we are trying to do the same thing at times to say. Are all cloacas created equally, and do they keep us awake at night the same amount? And if you think about Sort of risk stratification systems. This is what we do as clinicians in neurology daily. The grade of reflux influences my counseling families about UTI risk and renal injury. The grade of hydronephrosis helps me predict whether or not there's a high probability of an obstructive phenomenon and further testing. And measuring somebody's glomerular filtration rate or renal function helps me to understand what the risk of future renal replacement therapy may be. So, um, I'm going to humbly submit what I utilize for uh identifying risk factors. None of this has really been validated with our cloacal cohort as of yet, but, uh, as we are, you know, examining our database, I hope in a multivariate fashion to try to understand what there is and potentially identify ones that may not even be on this slide today to help me take care of these children better. So I think uh many people will understand that the common channel length not only influences the surgical planning but also the counseling and uh that is one parameter at the time of presentation that I utilize to help discuss with families. An abnormal spinal cord also helps me whether that's a tether cord or we have even seen patients with cloaca and myelo meningocele. Uh, I use this heading called structural anomalies, um, a solitary kidney and a hydrocopos. We've already discussed about the hydrocopos, and I'll get into why I think a solitary kidney is somebody who even without cloacal anomalies has some need for further observation. And then if we're fortunate that the patient may be presenting at an older age as the last case, uh, are there histories of UTI, incontinence, are there voiding symptoms, and is there a existing history of chronic kidney disease that may be helping us? And then as a tertiary referral center we see a lot of reoperative cases, and I always worry whether or not if we're going back in the tissue planes that. Uh, are so nice to see in a primary repair are somewhat obliterated and we are in an area where sometimes, uh, and this may go back to Doctor Woods' uh, statement, these are cases where I do think preoperative urodynamics are very helpful to understand how the bladder is operating before a re-operative case. So what's the rationale? Is this by uh feelings? Well, uh, yes and no. Uh, I'm going to, you know, present some existing literature. You know, Dr. Pena has a seminal paper that looks at 394, if I remember the title, but, uh, he, he subgrouped looking at 193 of these patients that were old enough to look at continents and with a common channel less than 3. Uh, as you can see in that series, almost a third were on intermittent catheterization, and that was, uh, either at the discretion of the surgeon or the urologist, um, mostly for either infections, radiographic imaging, or incontinence, and that number, as you can see, was much higher in that series, greater than 3. So this is helpful for me at the bedside to kind of set the stage for the family so they're not hearing about a catheterization for the first time, uh, at age, say 4 or 5. A tethered cord. There's a study in the urology literature out of Italy that kind of looked at all comers of anorectal malformations, and they found with urodynamic evidence that there was abnormalities in the bladder in up to 40% of patients with an abnormal spinal cord, and that was higher than those who had similar malformations with normal. And lastly, completely not part of the urology or surgical literature, there was a study over in Europe that looked at children with solitary kidneys, whether they were congenital or acquired. Wilms tumors, renal agenesis, and then they tried to separate those children into those this acronym CAKUT stands for congenital anomalies of the kidney and urinary tract. These are children who may have posturethral valves, reflux. Um, what they found is that if you only had one kidney and one of these associations by age 9, 50% of them had some chronic kidney disease, an antihypertensive medication. And it was about 33% in those that didn't. So these are the ones that if I'm worried about a marginal bladder that we don't have a lot of nephrons to play around with. So as I said, I humbly propose this is one of the ways that I kind of help clinically and practically look. I put in parentheses lower because I don't think there's low, high, and once again it's a, it's a spectrum, and there may be patients who are in these low that behave like high, and then those that are in high that don't act like high, so. I think a common channel less than 2 centimeters, those are ones typically repaired through a posterior saggitical approach. Preoperative and postoperative urodynamic studies have not demonstrated very deleterious effects with that isolated surgical technique, but still, I think the cloacas are our highest at risk in our anorectal malformation patients we see. And if you have a normal spinal cord, I think that minimizes your chances of bladder dysfunction. The higher risk, uh, I think then we're going to obviously have a much longer. I'm picking 3 just to kind of make it a nice little box, as we said, 3 to 5 may be a surgical categorization, but greater than 3 we've seen, and I think Dr. Allam's going to highlight later on in the afternoon some, you know, low risk patients that actually had really terrible outcomes. So, um, an abnormal spinal cord once again helps me to kind of have my antennas up and reoperative surgery. So management, what is this? I think at uh preoperatively once again I think this is what we hope to do prenatally to some of these families as well as in the NICU and then again after the NICU and then again when follow up because these are complicated patients, dynamic things change, imaging changes, and I think that's what's going to influencing your decision. So telling the families what we say and. In the first week of life just like any other chronic medical condition may not always be relevant 3 months, 6 months, 2 years later. So intraoperative, um, I'm, as I said, have the luxury that we have a very uh good, uh, interdisciplinary collaborative approach. Um, I was a Boy Scout, so I got to be prepared. Uh, I think anatomy varies and that uh you need to understand that. With all of these excellent preoperative imaging, there can be times when we have the need to perform concomitant neurologic procedures at the time of a primary repair, and that is actually pretty rare. I think what we try to do is restore some of the, you know, say urethral, vaginal and rectal anatomy and then do a functional assessment afterwards rather than. Saying that reflux that's present at the time of our initial evaluation requires anti-reflux surgery that may not necessarily result in a good outcome for the patient or might not even be necessary. Uh, Doctor Pena brought up the point about, uh, assessing the, uh, urethra, and Doctor Bischoff also talked about its ease for catheterability. At some point all of our catheters that are placed need to come out and we want to find out whether or not spontaneous voiding is a good thing or a bad thing and if it's not possible, how are we going to drain the bladder. So at times we will leave a suprapubic tube if we feel that the family may benefit from a proactive. Intermittent catheterization based upon some of these variables and so this allows the family to know that the bladder is being trained or there's a safety valve while we're teaching them this and then even in some areas where we have a lot of concern over say the urethra, a vesicostomy, I think is a very reasonable way, although I would tell you my first choice is to keep the bladder closed rather than have a vesicostomy where bladder capacity and its management can sometimes be a little more difficult. So follow up, um, I think there's some separate time points where the urologist or urologic. Assessment can be done postoperatively. I think, you know, after the primary repair prior to the colostomy revision, uh, colostomy closure, sounds like a great idea for us to get a picture of the ultra ultrasound of the kidneys and bladder and see how things are going. Uh, I think annual surveillance with a renal ultrasound and a history, uh, is a good idea up until the time of toilet training at our institution we have a bowel management program that's another time point where usually I will see some of the, uh, patients and reassess how they're doing, say, from a urinary incontinence standpoint. Adolescence is, I bring this up because sometimes our gynecologic issues that can become manifest can affect urinary issues. Also, there's a tremendous amount of growth that can occur with adolescence, and that can sometimes help us unmask when either chronic kidney disease is showing itself. And I also think lifelong is a good idea. So, uh, what are the components in my opinion, obviously everything starts with the history and physical exam. Blood pressure measurement is important because hypertension can be a manifestation of chronic kidney disease. And so the other thing would be measuring the glomerular filtration rate. I'm not certain measurement of a serum creatinine alone is going to help a patient because. That value, depending upon the age that you take care of patients may not be quote unquote abnormal, but unless you calculate, and there are many formulas and many blood serums that you can use, and on the internet is pretty much sometimes what I'll do, plug in the patient's weight, height, and serum creatinine, and it can give you an estimated, which is far more helpful when you speak to your nephrology colleagues than just this isolated serum creatinine. I think a renal bladder ultrasound with the bladder emptied and full can be a good thing and then uh we really reserve, I think, uh, VCG and urodynamics for select patients. I think some of the shorter common cloacas with normal um renal ultrasounds in the absence of any radiographic findings or history, I think it may be very reasonable to omit these in a routine follow up. So why are we chasing all this? Because I think long term renal outcomes in this patient, uh, group that have been published over many different centers are quite alarming. Um, Great Ormond Street, Riley Hospital in Indiana, uh, sick kids, and then Doctor Alam, when he was on faculty here, presented some of the long term follow up and as we could see anywhere from 44 to 75% had evidence of chronic kidney disease. Now some of these studies are based upon either serum creatinines alone, need for transplant, so it's, it's heterogeneous, but I think the last column is if you have end stage renal disease, there's really no variability there, and as you can see, 10 to 15%, that's much, much higher I think than the stable normal population. Uh, Dr. Alam and I looked at some of the patients, uh, that were at our institution with Cloaca and given our, as I said, admittedly referral bias with some of the more complex Cloacas, when we looked at what, uh, CKD stage some of the patients we were seeing, so the average age of these patients is around 4.5 years old, we found that almost half of them had CKD stage 2 or 3. Just so you all know, that category of the stages, as you see, is anywhere from 30 to 90 mL per minute, so normal would be above 90. So even at a young age, some of this can be present and so I think this gets to this slide that, you know, the chronic kidney disease could be prevalent, we know this, but could it be done to congenital non-modifiable upper tract causes? I only got one kidney, that's what my lot in life was. It's not a good kidney. There's nothing you can do about that, or could it potentially be an acquired phenomenon, recurrent infections, a dysfunctional lower tract that hurts kidneys, and I think it's this. Part that makes us as urologists have hope and aspirations that we can influence and I think we've also seen some children in retrospect that this renal injury wasn't present. And then Dr. Lam, I think will uh expound upon that. So with a mind that we're having a proactive approach, uh, one of our colleagues here published what we, uh, as a collaborative institute have done since Dr. Pena and the Colorectal Institute came here is that uh we looked at 55 patients with Cloaca. And we excluded 11 of them because of their follow up at elsewhere or they were not survival or we basically lost a follow up, leaving us 44 and at the time that we were involved with their care from the straightforward we can see on the bottom right hand side the majority of them fortunately did not have high stage chronic kidney disease, but. That I would show you that over about a 5 year mean follow up, none of them had stage progression. Now granted that's pretty early and we may need to reexamine this cohort as they enter into adolescence because as I told you that's usually where we will see children start to outgrow their kidneys but with a common channel length of about 4 centimeters and a very high percentage of these Cloaca patients who are diagnosed with a neurogenic bladder on active management having that you can hopefully prevent progression. At an early age, so in summary, I think the take home message is there's a known association between some bladder dysfunction and cloaca. I believe that that risk is much higher in the more complex. So be prepared. Uh, extensive counseling is needed for not only the families but other providers, uh, as Doctor Pena said, we have, um, more experience in seeing how some of these patients, uh, can behave in long term, and some of my other urology colleagues may equate voiding incontinence as the ultimate outcome. So as I said, I'm not going to steal a lot of my colleagues uh uh. Presentation, but he will uh kind of expound what his own personal observations has been and what his management is out in New York. So while CKD progression may not become evident until adolescents, I think we really need to emphasize that, uh, either measuring kidney function, taking a blood pressure, or evaluating a history and an ultrasound of these patients can be very worthwhile in minimizing the effects of chronic kidney disease. Thanks, Brian, I think, uh, you know it. It's, the surgery is one thing, but the long-term care of these patients and the surveillance, especially with regards to kidney and renal function, um, is what will potentially make them sick in the long run. And so, we're very lucky and fortunate to have a very um invested urology team where we can work closely with them to follow these patients. Just, can you expand on the mention, you mentioned suprapubic tubes and vasicostomies and Which, which are the patients that you would potentially do a vasostomy at the time of repair? So for me, um, it's a variety of factors. I think I already told you vesicostomy is not something that I really like to do from as my first a priori. um, I think the uh psychosocial factors can sometimes play into this is I think, uh, intermittent catheterization is extremely stressful and difficult for families, and, uh, I'm very fortunate to work with a lot of experienced nurses that help to. Um, teach families and give them that support, and I think other families can also prove to be reliable resources. So sometimes though with a, um, solitary renal unit, a massively dilated, um. You know, renal unit or hydroureter and a bladder, sometimes I will use that as a uh. Uh, as a way to help me anatomically independent of the psychosocial scene to make that, but it's hard to kind of go in there a priori and say you're a suprapubic tube or you're a vesicostomy, um, in the, in the cases where potentially there may be intraoperative findings where we're very worried about the urethra, um, then I think a vesicostomy makes a lot of sense there because is this going to be a viable bladder outlet for catheterization or voiding. But fortunately that's not something we encounter frequently. I just Can I say something about the. The, uh, the decision about leaving a Foley catheter that only no suprapubic is one alternative. The other one is suprapubic tube, and the third one would be vasicostomy. And the if let's, let's consider a patient that has a common channel of 2 centimeters, normal sacrum, not at the cord, and everything went well. I will leave a just a catheter and not a suprapubic. Because I have the feeling that that patient is going to pee and I'm leaving the urethra in a perfectly visible place. I will leave the catheter for 2 or 3 weeks and then I remove the catheter and I would bet that the patient will be voiding with no difficulty. And on the other hand, if I see a patient that has a little bit more complicated situation. Common channel 3 centimeters or more aesthetic cord is an abnormal sacrum. I would not hesitate to leave a suprapubic tube, which is a great advantage because then the urology will be able to do urodynamic studies and you won't have to worry in the middle of the night because the patient cannot pee. And in addition, if the patient has a very poor bladder that we already know that is very poor bladder and has mega ureter and single kidney and hydronephrosis and reflux and that patient needs a vasicostomy. I'm putting just examples in between maybe difficult situations, but that has been my philosophy in general. I want to, to, to, to mention a specific group of patients that are very, very high risk patients are those patients that are born at birth. They have a single kidney with hydronephrosis in mega ureter. Those patients most likely will end up with a kidney transplant and therefore I think they in the colorectal there was a recent evaluation of those patients who ended up with kidney transplant. And I think it's pertinent now to mention that Andrea, you want to mention that. So that was gonna be my comment actually. We reviewed our colorectal database and always also the transplant database at Cincinnati Children's to look at what patients ended up with a transplant, a kidney transplant, and the high risk group that we found were patients with Kidney failure at birth, uh, ectopic ureters, cloaca, and a stricture common channel. So if you see that patient, you should carefully follow urologists and nephrologists should be on board because those are the high risk patients for kidney failure. And I, and I think it's important, which Brian had talked about, you know, a lot of these high risk patients, we counsel them from the beginning. I mean, it's not. You know, you start counseling once they go into failure or have issues, but they're counseled prenatally if we meet them prenatally and then subsequently if we meet them after, um, the potential urological implications, the need for intermittent catheters and catheterization, potential further need for urologic reconstruction. So there is one question in the poll. What do you think about doing amirofenov in patients with cloaca and neurogenic bladder? I think that's a great idea. I, I usually don't do it at the time of the primary repair, so I think the Metrofenoff has been a fantastic surgical technique to allow for intermittent catheterization when either the urethra is not a viable option or if the family um requests an alternative way. Um, I always say girls are smarter than boys when I'm talking to um. Families, because we have a lot of intelligent young 67 year old girls that are able to catheterize themselves with supervision per their urethra, and the family's not interested and as the father of two girls, neither would I, more scars in their belly if the urethra seems to be a reliable mechanism. But if you are having a patient that has an incompetent bladder outlet that's contributing to either incontinence later on. I have not found placement of the Metrofenov to be any different than other patient populations such as spina bifida or posturethral valves or bladder atrophy, so I think it's. A very good alternative. I would tell you that there will be an arm wrestling match with your colorectal colleagues unless you were the one who are doing it the same because one surgical technique may be that you're allowed if vascular anatomy and appendiceal length permit, you can then divide the Appendix so that it can be utilized for a Metrofanov and a Malone antigrating continents channel. So that would probably be my only other thing that the question didn't address, but let's make sure we're not making reconstruction piecemeal and trying to collaborate and figure out what. What the child requires. So I think that's an important thing to bring up because um for us when we do them alone it's usually at an older age and you're, you know, if we're gonna do a reconstruction for a Metrofanov and a Malone we like to do it together in the sense that we can collaborate and either split the appendix or um you know, decide on which piece goes where um between Brian and myself I think I usually win or I'm very nice and give it to him so. I, I, I definitely won't disagree with the course director. I think the other thing to comment is you won't know about fecal incontinence. I think we all have an idea who's going to be fecally in continent or incontinent at that point in time, but bowel management, we usually don't do until they're toilet training age, so we wouldn't do a primary metrofenoff, if you want to put it that way, at the time of the repair, but we would wait until they're older. I've, I've heard some centers in the United States that were putting Malone's in at the time of a colostomy closure because they have felt that that was going to be the best way, but, uh, I, I once again had the luxury of hearing and authority with many years' experience, and Dr. Pena has told me about successful laxatives where he had zero confidence ahead and sometimes the patients tell us what they need. All right, so we're gonna take a few minute break here. Why don't we do uh 10 minutes? Is 10 minutes enough for you, Mark? OK, let's take a 10 minute break. So we'll, uh, we'll reconvene in 10 minutes and we'll put the clock up on the screen now. And actually, Doctor Lam I heard is back, so he'll be here for the uh next session.