Case Presentations Part I: Cloaca and Complex ARMs 2015

So the first case we're gonna present, it was a baby that was a newborn, 34 weeks gestational age female. There was a prenatal diagnosis of a pelvic collection, and this was done at the baby, the parents were actually from California and then relocated to Cincinnati for work, um, when the baby was about 28 weeks. So we didn't have access to a lot of her prenatal imaging, but this is what the parents were told. So, pelvic collection and hydronephrosis on ultrasound. So automatically, I think everybody on the sitting at the table here would diagnosis as hydrocopos. At delivery, she was small for gestational age, had a very distended abdomen, had no urine for several hours after birth, and then just on the screening test, no tether cord, no presar mass, normal echo, and normal sacral X-rays. So this is her abdominal film. Does anybody wanna comment on that X-ray? Doctor Breech. I was just gonna say ovarian cyst. I think that's the highest on my differential, um, so, uh, I think what Doctor Dickey is wanting me to add is that, um. We suspect the baby probably has hydrocopos, the antenatal ultrasounds, as well as the combined finding on the abdominal film, um, demonstrating that there is some distortion of the structures in the pelvis, and you can see that there's something shading there that's the, the mass of fluid that's distending the abdomen. So I think that's what she's wanting me to add. We as a team would work together, I think, caring for the baby, and we'll probably have some different comments on managing Hydrocopos overall. Yeah, so we'll come to the management of that. But you know, so for many institutions, um, I, we have the luxury of being here and. Uh, listening to Doctor Pena's talk many times about hydrocopus and urinary instruction, urinary obstruction in uh Cloacas, we, that's what we would suggest. But many, many institutions would think that's a distended bladder. And so at that point in time, you know, the decision making could be, um, changed. So this was the initial ultrasounds, um. You could see bilateral hydronephrosis as well as that's the distended collection um down in the pelvis. So, um, initial management, um, Does anybody wanna comment? Doctor Langer, since you're back online with us, all of us at the table know this baby, so. I think I'd move on to a physical examination. Distended abdomen, 34 week otherwise looks fine. What about the perineum? The perineum has a single opening. So it's a, so yeah, so it's a cloaca, and uh the ultrasound shows a hydroculpuss. So, uh, we would probably, we would go through the things we, we talked about earlier, rule out the veal things, but, uh, this, uh, girl's gonna need a colostomy, and, uh, we would probably move fairly quickly to, to do that. So, so, of course, well, I'm gonna um turn the slides over to Doctor Breech briefly. So she's gonna discuss a little bit about hydrocopus and she's um Right. So Doctor Langer, what, what, what you, what would you do with the hydrocopos at the time of the colostomy or reevaluate it after the colostomy, or no, we would, at the time of the colostomy, we would likely put a drain in it. Do you put drain or do you do a formal vaginostomy? Uh, we've usually put a drain. OK. So I was just going to share a few slides at this point, um, I think the slides can be seen by. Um, in our center a number of years ago, our group looked at, uh, patients with hydrocopos, and at that time there were about 411 females with cloaca that you can see on the slide, and at that time we found about 28% of the patients, um, had hydrocopos. I highlighted on the slide um just below that uh the majority of those patients had bilateral hemivaginas. I think that's important. Obviously I'm a gynecologist, so in all of my discussions I will be migrating towards the gynecologic and reproductive sequelae or manifestation. So you will find that my slides are always favoring the reproductive parts. But the importance on this slide is that more than half of the patients did not receive timely diagnosis and treatment, so we talked a bit about how to drain the hydrocopos, which is great that we're actually thinking about and draining the hydrocopos because the sequelae can be significant. Doctor Pena had previously highlighted some of those sequelae with things like infection, sepsis, recurrent urinary tract infections, and subsequent, um, even in some patients rupture of hydrocopos. Um, if we look at the next slide, um, just over the past year we looked back at patients with hydrocopos with a little bit different eye. You can see that we had increased in the number of patients in our center with 645 females who were identified at that time we had a similar number, about a third of the patients experiencing hydrocopos, and really our team was very interested in sort of the association of utero vaginal duplication with hydrocopos. Obviously pediatric surgeons are very active in managing patients initially with cloaca and hydrocopos, and it's very important to assess the reproductive structures. So it's important for the pediatric surgeon to be thinking there is highly a high likelihood the patient's going to have two hemi vaginas. So what we're wanting to emphasize really with this review was that there is a strong association of reproductive anomalies and duplication in patients who have hydrocopos. The second bullet was looking at the common channel length because I think that's another emphasis and you can see that in our group of patients that you can see that 69.4% of our patients with hydrocopos had a common channel length that was greater than 3 centimeters, so it was more than 2/3 of the patients. So again, I'm thinking about cloaca management, complexity of the cloaca. So I'm hoping that the pediatric surgeon would be thinking, wow, hydrocopos may make me think I need to assess the utero vaginal anomaly on the other side to make sure that there is not just a single vagina, but there could be two that may need to be drained. But in addition that this patient may have a longer common channel length, i.e., may be more complex in multiple regards and may need um either some specialized management or involvement of another tertiary care center. These are some of the images from patients we've cared for. We talked about patients with a distended abdomen, which you can see very easily on this particular slide. And we'll talk a bit about how you manage or how you would drain the hydrocopals. These are some images looking at open drainage of hydrocoals so that you can see on this slide that you have distention of the uterus and vagina in some patients, primarily the vagina, but the uterus can be distended as well, making it difficult to delineate the anatomy. I'm gonna go to the next slide. So this is a patient who's having a larger incision for drainage of the hydrocopos. So this is at the time the colostomy is being created and at the same time the vagina is being drained, the lower part of the baby here, head up above. So this is a fairly large incision and a large procedure for a baby with hydrocopos. So we'll talk about what are some different approaches to managing that. This would have been closure of that incision with the vaginostomy tube you can see it coming from the lower part of the incision. Again emphasizing the reproductive components, this is again an image, but I include it specifically for the GYN reason, and that GYN reason is to demonstrate that when those patients have two uteri, they often are at the same level, and those vaginas and the attachment of the uteri are at the same point. So it's important to think about there's a single cervix that you see on one side of the septum, so the cervix is located here. This is taking down the septum and draining both sides of the vagina. It's important to know that the cervix on the other side is likely at the exact same level and to not traumatize that cervix for future childbearing in the future. And then this is just an image that would show you keeping that vaginostomy tube in place until the definitive surgery is critical to make sure that both of the vaginas are adequately drained so that you can see on this image that this is us doing endoscopy to demonstrate at the time of the definitive surgery. It's not necessary to do that all the time, but this is for us to show you that that tube crosses over both of the hemivaginas and drains both sides adequately until the time of the definitive surgery. So this baby did undergo colostomy creation. I think those are her pictures actually, and, and, um, drainage of the uh the hydrocopos. Um, I think it's important to remember that, you know, sometimes when we have had our interventional radiologists drain, um, hydrocoals for us, they potentially go through the uterus or potentially come very close to the cervix, which makes. Dr. Breege very anxious and, and get heart palpitations. Belinda, I, I'd just like to add Doctor Wilcox just kind of published a paper where he's looked at, um, sort of, uh, many different types of drainage procedures of hydrocopos at his institution in Colorado, um. Not the most ideal because he didn't go into specifics of whether there were two vaginostomies or anything, but he was specifically his preferred method was more intermittent catheterization of the common channel, which I know as Dr. Pena said it is, it is tenuous, it is somewhat unreliable for, as we said, when you insert a tube into an unrepaired cloaca, there are 3 places it could go. Um, but in his study, he did not, once again, very retrospective in about 25 patients or so, he did not see an increase in Um, Uh, pyelonephritis or infectious complications with his sort of, uh, multifaceted approach and so I guess just as we're talking about hydrocopos, I felt that that might be because I know where I did my training in Indianapolis, um, Doctor Rink felt that a. External drainage tube may provide some type of tethering to the vagina that could interfere with a total urogenital mobilization or in theory make pulling the vagina closer to the perineum if its tethering effects may be more complicated. I know that's not necessarily the way. Our experience has been, but that may be what colors some other people's lack of enthusiasm for external drainage. I don't know if Dr. Elam is online because he also has some opinions on intermittent drainage. He's still in the operating room. Oh, is he still in there? OK. Or we could ask how often. Yeah, I mean, is there, can we put up a polling question, um, a quarter of the time, half the time, never the time? I think it's very important that we always say we don't argue with success, so I'm sure some of intermittent catheterization might work, especially in the short common channel or where the folly prefers to go to the vagina, which we know. it happens sometimes. Whatever method you try or you initially provide to your patient, just follow up with an ultrasound to be sure there's no reaccumulation and that the hydrocorpus is indeed drained. We see many patients that come from other institutions with very distended hydrocorpus infected with mega ureter and they were doing. Doing intermittent catheterization, Leslie and Brian just did one that was exactly this story. So whatever you do, at least follow with ultrasound and be sure that your method is actually working. So I guess the poll question for Jennifer was, how often do you use intermittent catheterization for hydrocoupals management? And I guess your options would be, well, I think the pole question would be how do you manage hydrocoupals, intermittent catheterization or vaginostomy too. I think we're, I think with our experience here, most sorry or vaginoscopy, yeah, um, but I think if you know, often for us if the patient's going to the operating room and we see hydrocopos they're getting a drain at it at the time, so either with a formal vaginostomy. Or a vaginostomy tube placement can I, can I just, uh, that, that was the point I was going to make, um, that we have, you're, you're doing this in real time and these children generally need their colostomy within, um, you know, 24, 48 hours. So if you have hydrocopos. To me it's the most sensible thing and the safest thing just to drain it at that time of the colostomy, which raises the question of under what circumstance would you have your interventional radiologist drain the hydroculpus if you're planning to go do a colostomy anyway within the first couple of days. So we have done interventional radiology when the patient received the colostomy elsewhere and comes with us with a hydroculpus. That's one of the situations. And regarding tube vaginostomy versus vaginoscopy, vaginostomy, it depends on the size of the vagina. Sometimes the vagina reaches the abdominal wall, sometimes it doesn't. So we cannot have a recipe that fits all. Yeah, yeah, but I also think that, you know, there's been, you know, at least this baby. We cath the baby and you could see the abdomen become less distended almost immediately with a lot of urine, but that wasn't a reliable way to manage the patient, so she got a formal vaginostomy. We actually put a tube in, so a vaginostomy tube as well. One other, one other issue, some as Leslie pointed out, a lot of times there's a, a duplicated vagina, and both of them are dilated. Um, what we've sometimes done is drained one. Um, and then see how it goes with the other one. usually the one drain will drain both sides, but we have had a couple of kids where we've drained one, the other side doesn't completely decompress, but we can decompress it with intermittent catheterization from below. So it's kind of a hybrid approach. Mhm. You know, so I, I, I want to say that the we should all agree that the the um the our goal is to be sure that the hydrocorpus is decompressed and that remain decompressed and even when we leave a catheter in place we must be aware of the fact that sometimes reaccumulates with the catheter in place and becomes infected with the catheter in place so that's why we have to follow the patients to keep monitoring the emptying of the bladder. And and and sometimes we recommend to do irrigations with gentamicin solution into the giant vaginas because we have seen infections even with the catheter in place. So, um, everything is relative of course it depends on the specific case. Richard, did you have a comment? I was just gonna say that, I mean, the one advantage of doing the open, uh, open vaginostomy is if there's a septum there, you can open the septum and then make 100% sure that everything's gonna drain versus if you do the tube, you know, as Doctor Lange explained, you sometimes have to wait and see, and then you're stuck with one tube in already. You don't want to put in a second tube vaginostomy, but you want the child to drain. So I think that's the one advantage if you're there already. To just open it up and divide the septum to a degree and allow both sides to decompress. So this is a question as well, how many people would now do a colostomy laparoscopically and drain the hydrocopus laparoscopically versus doing a formal open exploration? I think it again depends on the patient, how distended the patient is, how large the hydrocopus is, and Doctor, do you have comments. Um, we, we have not done that laparoscopically here, but, um, I know some people have, and, and, uh, there's no reason why you couldn't do both the colostomy and the drainage of the hydrocarpus, uh, laparoscopically. Yeah, I, you know, there, it's always a concern whether to do laparoscopy on a baby that's distended or not, but I think it, it can be safely done. Um, I think you just have to be able to do what you think you're comfortable with. So We're gonna, if we go back to the slides, um by the way, just so you know, so 80-90% do a vaginostomy too. One person just finally said intermittent catheterization and then we're putting up the poll for the laparoscopic vaginostomy. I think it's the people from Colorado. Well, I mean there, there are people who do it and, and we, I think we've had one patient together where they successfully did intermittent caths and we got routine follow-up ultrasounds. There was no reaccumulation of hydroco. Or hydronephrosis. So I think like you said, what uh Dr. Bishop and Dr. Pena said, the critical part is, however you do it, make sure your, your, your intervention is actually doing something. And as a segue into our slides, you can see that on follow-up ultrasound there has been resolution of the hydronephrosis and hydrocopals. So this is, um, now the picture of the 3D cloarogram on, on our screens it's, it's very dark, um. It's brightened for the audience. All right. So, so, I'm gonna point out um what we can see here. Which, which I can't really see on mine, but. But here, here's, here's the rectum coming down here. There was this triangular shape here. There's almost seemed like a septum there that nobody could explain because when we scoped, we couldn't see a septum type thing. Um, the vagina was here and then the bladder was in place with a 1 centimeter common channel. So, at this point in time, there's another picture of it with that sort of septum and triangle. Um, so, the question was, is that triangle another vagina, which, you know, when we did the laparotomy, there was a, actually a single vagina with hydrocoopus that was drained. Is this a flap? Is this, nobody knew what it was. So, at this point in time, we'll pose it to the panel. How would you approach this patient, uh, in the repair of this malformation? And then secondly, counseling of the family on potential fecal and urinary continence, knowing that no tethered cord and normal sacral X-rays. So, Doctor Fishisher just joined us. Um, we'll, since he hasn't spoken yet, we'll have him input on. So not being able to actually see the images, it's usually helpful to see the images, but how long of a 1 centimeter, so this would be an excellent, excellent case to approach from a posterior sagittal, uh, perspective. And so, um, that would be my initial approach with. If I trusted you in that saying it's a 1 centimeter common channel. And Richard, would, how would you counsel the family? Of course, everybody wants to know, is my baby going to poop normally? So, um, as part of the workup, I think, um, Doctor Bischoff already said, you know, you want to get a sacral ratio, you want to have a look at your spine. If you've got a good sacral ratio, you've got a good prognosis malformation, and you have a normal spine. I think in this situation, you counsel the parents, there's a very good chance of, of bowel control, but, um, but that you would take them through the process when it came to the time of potty training and help with whatever inputs they needed to, to get them there, but you'd be fairly confident to predict a good outcome. Jason, can you tell me when, what lengths of the common channel would. Decide which which lengths would would determine which approach. Taking just general anatomy and not having any other complications or issues, uh, our studies have shown that approximately 3 centimeters is our basic cutoff for when we're going to say that we could confidently say we could approach from a posterior sagittal approach versus when we have more concern and how we prep the patient, how we discuss with the family. Uh, possibly needing an abdominal approach and then discussing an abdominal approach, um, is, would it be an open approach or depending on skill sets and anatomy, can you do it with laparoscopy? So Todd, I think the cases are going to get more complicated as we go through this in different lengths of common channel, but you know. With all of Dr. Pena's work and his, his series and our experience here, I, they, we sort of break them up into 1 to 33 to 5, and greater than 5. I think you can reliably say that 1 to 3 centimeters common channel you can approach with a posterior sagittal maneuver. I think as we get more experience, you know, there's more things during the endoscopy now that I will look at as well, to, to depend on the posterior sagittal. It's not just the. Common channel length anymore. I think it's the common channel plus what is the length of the urethra, where does that take off from? And then also you may have a 1 to 3 centimeter common channel, but the rectal takeoff is way up on the back of the vagina, so that would then alter your approach. So I think it's, you know, we, you can very nicely put them into compartments, but then at that point in time there's also deviations as. Doctor Penny often says God doesn't make boxes. So, uh, the, the first sort of common maneuver and is, is really, if it's a very short common channel, and usually the short common channels we say less than 1 centimeter, you can potentially leave the urethra in place, except a little bit of the female hypospadius, and then mobilize the vagina only and the rectum only. So, you can see in this picture here, um. The urethra is quite near um where it should be. The vagina, we can mobilize the posterior wall um and bring it to the enteritis and put the rectum in place. So that's sort of the most common, most simple sort of cloaica if you wanna put it that way, um, to be able to repair. And this is really what we would call a type one cloaca. Um, does anybody have any comments on how much female hypospadius you would accept? I think you have to accept enough that the patient can catheterize if needed, so the patient has to be able, or the doctor has to be able to identify the meatus. In case catheterization is needed. Yeah. So, interestingly enough, I didn't have a picture of this one, but that little triangular flap was actually a flap, uh, from the vagina, um, that halfway covered the vagina. So, urine could get into the vagina, I think with posterior sort of reflux into it, but couldn't get back out. And that's why she ended up getting the hydrocopous at that point in time. So we're talking about the the origin of hydrocorpus and there is no explanation as to why patients have hydrocorpus. It's a mystery. One would think immediately with our surgical simplistic mentality that patients with hydrocorpus accumulate liquid in the vagina because there's an atricia or some sort of obstruction. I have never seen an obstruction when we operate on these patients, so it's a mysterious why they accumulate that fluid under real tension and yet there is no atricia so that would be a fascinating the uh mystery to uncover in the future for the young generations of pediatric surgeons. All right, so we're gonna go on to our second case. So the second case now we were talking about the older cloaca and does the common channel length change at all. So, uh, this is a 4 year old female, um, at birth was found to have a cloacal anomaly, single opening. They had never been repaired, um, has left-sided, uh, colostomy at birth. She voids spontaneously but's not continent of urine, so she's still in diapers. She had previous posterior, uh, previous perforation of her posterior vaginal wall during an endoscopy, and this sort of frightened the family away to do anything else. So she, at 4 year old, came to us for a second opinion on whether a repair was possible. And whether um what would the repair entail. She had no tethered cord, no presacral mass, had a sacral ratio of 0.7, and no other cardiac disease. The rest of her history, she had left renal agenesis. She had cross-fused right renal ectopia and right uh vesicoureter reflux, and she had two hemivaginas, uterine didelphis. So on our exact initial EUA she had a common channel of 5.5 centimeters, which, you know, on reports from when she was much younger, it hadn't changed much. So, the report said long 5 centimeter common channel, which we thought, OK, maybe it would be longer, but it was still only 5.5 centimeters. The urethra length from the takeoff from the common channel was 2 centimeters. The vaginal septum leading to the two cervices, um, was approximately 4 centimeters in length, and there was a rectal fistula at the top of the vaginal septum. Um, there is also a bladder diverticulum with, uh, multiple stones in it. So Doctor Penny, can you comment on where you would usually find the fistula, um, when you have bilateral or director, director, when, when, um, the, the fistula is usually located in the posterior aspect of the, of the vaginal septum, um, it sometimes a tiny orifice that you have to look intentionally for that. But I have seen in cloacas everything is possible. Sometimes I have seen the rectal orifice located immediately behind the urethra and the vagina is located posterior to the rectum, so everything is possible. But in general, the rectal orifice is a small, sometimes it is large, but sometimes it's small, located. Look for that in the posterior aspect of the meat and the vaginal septum. So, once again, I, for our screens, it's very dark. So hopefully, it's, it's much lighter on your screens. Um, this was her 3D loacogram. The little markers here are at the end of where the catheter is, which was inserted into the um bladder. The BB here is where the anal sphincter that we can see. This is the rectum coming down, and you can see that it ends posteriorly, um, at Approximately 5 centimeters from the common channel. Things to note, um, to try and decipher which approach you're gonna take is the end of the sacrum. Is there anything there? And you can see that at the end of the sacrum, you sort of run into uh rectum. Vagina is here, and bladder there. It's another view. This is the anterior posterior view. Here you can see the rectum coming down. These little things, initially, we didn't know what they were, but we sort of, uh, knowing that she had bladder stones, figured out that those were all the bladder stones that she had. Um Uthra here. Coming down to the common cha, sorry, it's full, uh, bladder here, common channel. So at this point in time, Richard, I know those pictures were hard to see. How would you start from front, back? How would you prep the patient? So we do a total, a total body prep so that you can, um, go posterior surgally and transabdominally. Um, and I think, um, this child is kind of right in that lovely borderline area that we spoke about earlier where there aren't really boxes for this. So, a very obviously long patient, you'd probably do a transabdominal approach, but You've at least got a sufficient length of urethra that if you are able to do a trans, a total uregenal immobilization that you would, would have sufficient urethra not to have the bladder neck on the perineum. So, I think there is certainly a case for doing a total genital mobilization in this child, if you feel you can get it down. Uh, we tend to try and start posterior sagittally because even if you are gonna separate, you can keep the common channel intact. And if you start that separation process from behind, I think it prevents you from pivoting into the common channel if you come from above. So, we would certainly start posterior surgically, hopefully find the rectum. It looks like one will from based on the imaging. You should go if you go under the sacrum and actively look high, you should get it. And then, um, And then I think one will get a better feel of exactly whether you're gonna get a total genital mobilization once you've done that. So I'd probably keep the common channel intact to start with, and then you can make that decision when you get a feeling of how well the vaginas are going to come down as to whether you're gonna do that or whether you're going to keep the common channel as urethra and separate. So, you know, knowing that it's 5 centimeters, that's sort of our border of, you know, can you do a total urogenital mobilization or not? Um, 3 to 5 centimeters for me, I think is the hardest to decide. 5 centimeters, you know that things are probably coming apart. The takeoffs are high. I think 1 to 3, most likely you can do your to your genital mobilization. 3 to 5 is that gray area, you know, and I think those are the points, um, if you're repairing these that you need to become fully armed, being able to do everything. Uh, go ahead. Just one other point. I mean, I think one should also choose the option which fails best and, um. I know that sounds like a funny thing to say, but it was advice one of our old professors always used to say, think about the option which fails best, not always the option that works best. And I think you've got to be mindful, if you go for a total genital mobilization and you can't get it down, then you're gonna separate and dissect both sides of the urethra, which you don't really want to do. So, I think. You got to be very careful if you're gonna go for a total unit of immobilization on a 5 centimeter cloacre. And I think it's important what you mentioned it, and I sort of briefly mentioned it. You, you need to know where your sacrum is and what's the first structure you're gonna run into. We know that the first structure we're gonna run into is the rectum, and you know where to look for it. So as you're dissecting down, you know, you always have to look right underneath the sacrum, which, um. It's very helpful, so. These are just sort of diagrams. Let's go to. Doctor Pena's animated video. So for this baby, um, we were actually able to do a total year genital mobilization. She's not a baby, but 4 year old, we were able to do a total your genital immobilization, um, knowing, you know, we looked at 5 centimeters and we thought, hmm, it's rather long, but she was also 4 years old, so she was bigger than our usual patients, and we were able to do a full total your genital immobilization from behind and get things down. Linda, the other thing, sorry to interrupt, is when looking at that imaging, you want to see, do you have enough rectum and colon to bring down, which it looked like that was the case in this, um, situation. But if you think you need to potentially replace vagina or do something that requires more work, do you typically get proximal imaging of The colon So I think if I know there's a short amount of rectum, then I'll ask them to image the proximal colon. If it looks like there is, you're going to reach and you don't need vaginal replacement, I don't always. But what about in this case, 5.5 centimeters? I probably would just to make sure that we have sufficient colon because I think we've been fooled in the past that, oh, there's a lot of distal colon. Then you image the proximal colon and it's only like. 10 to 15 centimeters and then Andrea, what do you think we've gotten into trouble with this before, so what's your advice? You have to be prepared for everything. So ideally you wanna know how much proximal column you have and you also wanna have the proximal column prepared in case you need to use. I think it should be. Prepare that for everything because even when we look at the studies and I always try to look back in the studies so I can learn from my uh previous case but uh sometimes we are, we have to misled to the to a decision so I think you should be prepared for everything. Right, so I, we typically um get our distal cholostogram during the time of our exam under anesthesia, but then we So I asked the radiologist, can you squirt some dye approximately in the proximal colostomy as well so we can get an understanding of the anatomy in case we need that proximal colon for either pulling it through or using it for a replacement of the vagina. And in order to use the proximal column, you have to be able to study the blood supply. It's always different. You're not the one that did the colostomy, so you don't know which vessels were ligated, so you have to be prepared for everything. Right. image everything. Um, I'm gonna, Doctor Pena just stepped out, but we'll play his, um, so Doctor Pena has the animated videos. Of a total urogenital mobilization. This is the right one. So here is the separation of the rectum posteriorly. And then, um, you can see that there is some movement, but we want to try and get adequate movement. So, multiple silk sutures are placed around. I think to note that's important and Richard touched on it, and I sort of touched on it. The length from the takeoff of the common channel to the urethra, so your to the, to the bladder neck is an important length, because what you don't want to do in a total urogen, the total urogenal mobilization is bring the bladder neck right down to the perineum. So I think if you, if that's done, then you're probably doomed to have that patient be incontinent of urine. OK. What, what number are you guys using for your? They're fucking nice. I don't know if there's a cutoff or not. I mean, you want, I think at least 1 to 2 centimeters. Let's say in another population with a neurogenital sinus anomaly alone, like congenital adrenal hyperplasia, uh, which is another way that we'll use, say, genitography. To assess, the majority of the patients that we've seen at our center have kind of been in about 1.5 to 2 centimeters, so it's not a cloaca population, um, but I know that other surgeons that are that are publishing a lot on um. These distances and correlating and how the importance is the definitely I think from a urologic standpoint, I worry more about the distance from the bladder neck to the confluence of the common channel as far as potential uh continence. What about judging the competency of the bladder neck at that time, or I don't think it's very reliable endoscopically because you're usually using, um, you know, hydrostatic pressure to, so it's not a reliable mechanism for me. But I must say about about that that there are no real studies to demonstrate anything like that. Nobody compare one thing with the other concerning your question is, um, the only evidence that we have is that the com we know for sure that the length of the common channel is important. There's no question. That when the, the vaginas are attached to the bladder neck or the the trigon, that's a very, very serious problem when you separate everything you may end up end up not having a bladder neck, but below that, um, of course, usually long common channel means shorter distance between the bladder neck and the bladder, so the vagina, but to compare one or the other in terms of prognosis, I don't, I'm unaware of anybody doing a study like that. So I think for the interests of time, we may just um. Does the panel think we should keep going with cases, or we have a couple of presentations from gynecology and urology. We can still talk maybe one or two cases and then do. The gynecology and neurology portion. So, sort of the, so that was the, the next step, you know, you can leave, um. urethras, uh, common channel is urethra, like we talked about in the last 1 centimeter. Then the next step is do the total urogenal mobilization. So, what happens when your total urogenital immobilization doesn't work? So, you can still cannot get the vagina down. And so, sort of the next step in that, and I'll have Doctor um Pena go through his video, is the total urogenital mobilization with extended. Intraabdominal approach. So let me stop this and go from the beginning. You ready? Here, and pushing play. Here we go. Doctor Pena, that's, that's very ugly. I know. I don't know what happened. It was fine before. Here we go. Here we go. It's ruining my reputation. That's it turns of the domino. Yeah, when, when the, when the, um, when the, you try the total neurogenital mobilization, they say that the patient is 34, 4.5 centimeters, and sometimes you can reach, you, you can repair the malformation even with 4 centimeters because the tissues are more elastic or something, but sometimes it's not possible. Then you have to go into the abdomen and. We do what we call a transabdominal um mobilization and you basically you divide all the avascular attachments that fix the, the, the bladder and the vagina and the pelvis and sometimes by doing that you reach the, the pelvis. I want to say something about, about very important. The um if you do a total urogenital mobilization in a patient with a very long common channel and then you go into the abdomen and bring everything, we lose the urethra by doing something like that because if the common channel is very long, the blood supply will will come from the bladder and if you devascularize by separating everything that, that patient will have for sure a closure of the bladder neck so. I still believe that we have to start all the cases posterior sites because you need the space anyway to put the vagina and the rectum. But when you open and you see a very long common channel, I suggest not to do a total eurogenital immobilization because it's not gonna work and you will sacrifice the blood supply of that common channel and that common channel may be very useful to do intermittent catheterization in the future. So you, if you see that it's very long, let's say 5 centimeters, you, I suggest to go right into the abdomen without attempting the total eurogenital mobilization. Now when the common channel is extremely long right from the beginning preoperative, you, you find that it's a very long common channel and endoscopically you see the little vaginas coming into the bladder neck and the rectum into the trigon and so forth. Those cases should be managed through the abdomen only. You don't have to you open posterior side but that later just to bring the vagina and the rectum down. But those cases actually are easier to manage than those that are. In the 5 between 3 and 5 centimeters common channel, those are the difficult ones. The decision making is difficult, particularly if, if it happens that the patient has large vaginas and a 4 or 5 centimeters common channel, then they, they, they, those patients have a, a common wall between vaginas and the trigon, very wide common wall, and the separation of those structures is the main challenge in the repair of cloacas. And the ureters go through that common wall, so that's the most deeply challenging situation. So Doctor Pena, when you, when you start posterior sagitally and you know you have all your preoperative imaging, it's between 4 to 5 centimeters, when is that decision point making that you say I'm gonna leave common channel as common channel, or I'm going to start my total urogenal mobilization? That's, there's something in surgery that, that we, that is politically incorrect to talk about is called feeling, that feeling is extremely important in surgery because we are in the area of uh evidence-based surgery. But actually when you are in the operating room alone with your case there are many situations in which you cannot go to the library and look for any evidence based uh whatever you have to make a decision based on your own feelings and uh that's as far as I can go that's that's the that's the art that's the art of surgery and doesn't matter how much progress we we see in surgery. The surgeon will still be facing that situation of consulting his feeling. I think just one important point to leave the common channel as a urethra, it's important that you have a straight shot from the catheterization because sometimes even when the patient is completely open, we cannot catheterize the bladder, so that will not work as a future urethra. So we're gonna talk a little bit about the very long common channel in the next cases, but Richard, yeah, I just wanted to ask Doctor Pena, so you know, you got into a situation where you've decided to separate, and obviously the thing we're all worried about when you separate is um fistula because that was the whole, you know, try and get away from the urethra vaginal fistula. Do you, what, what do you use fat pads and materials to put between the two structures or, you know, sometimes you have to divide a vaginal septum as well. So then you've got to repair in the vagina, you've got a repair in the urethra, in the urethra. Do you think there's a case for the vaginal septum in place and rather dividing at the time of colostomy closure so that you don't have a repair on both sides, or do you, when you say vaginal septum, you're talking about the septum between two hemivaginas vaginas. No, I don't see the reason why the resection of the vaginal septum may contribute to create fistulas. I, I, I, I thought you were talking about the separation of vaginas from the urinary tract. I think both. So you're separating the two and you're going to divide a vaginal concerning the separation. Once you to embark yourself in separating those structures, you have to be absolutely sure to leave a completely normal wall of one structure in front of a suture line and never leave one suture line in front of the other because that would be the, the that's the problem. Um, we're going to have Doctor. Can I ask you, can I ask a quick question about the septum? Uh, our gynecologists have sometimes told me that you don't need to divide the septum, and I wonder what Leslie thinks about that. I think Leslie just rolled her eyes, but luckily I wasn't on camera for that, but Belinda shares everything, as you can tell. Um, so that's a great question, Dr. Langer. I think that um we all as gynecologists have met young women who potentially present with an isolated vaginal septum, a longitudinal vaginal septum, who may not have had other associated anorectal or uh urogenital anomalies. Thus they weren't having other surgeries performed, and I would say in those cases we don't need to do anything about the vaginal septum, but these are patients who are having. An extensive surgery, we're just talking about going from the perineum to the abdomen back to the perineum, it would seem a reasonable time to unify the vaginas that we know would be more comfortable for her sexually, more comfortable for menstrual hygiene. Could you leave them separated? Certainly that's a possibility, and I would say that would probably be for cases where patients aren't having all this other surgery performed. So we would recommend in patients who have septated vaginas and cloacal anomalies to go ahead and take the septum down under a coincident anesthetic. It's not extending the procedure, and we haven't seen by doing that that that's increased the risk of things like urethro vaginal fistulas by just resecting the septum.