Colorectal Quiz Episode 18: Cloaca Part 2

Hey there listeners, it's Amanda Jensen from Cincinnati Children's. Hey everyone, this is Kiram from Nationwide Children's Hospital. Have you downloaded the new version of the stay current pediatric surgery app? It's in the Apple App Store and better yet in the Google Play Store. So while you're listening to this episode, you can easily bring up all the pictures and X-rays in real time while Doctors Fisher love it and would talk about them. So download the app. All right, Amanda, tell us what we're talking about today. Alright, today we have a very special episode. We are talking about Cloaca. This is part two of a three-part series. Last week we talked about newborn initial workup and management, and today we'll be talking about operative planning. Now, let us summarize our last week's review. So just to review, last week we discussed how a good perineal exam is very important for making that initial diagnosis and that in a patient with cloaca, you do not need an endocrine workup to assess gender because they are all female. Additionally, appropriate pre-op testing includes a renal and pelvic ultrasound to assess for hydrocpals as well as hydronephrosis, along with the echo to assess for cardiac anomalies and assessing for tracheoesophageal fistula with a chest X-ray and an NG tube. Additionally, if hydroculpusse is present, it is important to appropriately drain with a couple of different options that we discussed, as well as colostomy tips and tricks. All right, so for today, let's pick up where we left off with operative planning. OK. So we have a thriving young 2 or 3 month old infant in our office. That's Dr. Jason Fisher from Cincinnati Children's Hospital. When do we start doing the definitive diagnostic workup? Uh, so we usually do it at about 5 to 6 months. That was Dr. Richard Wood from Nationwide Children's Hospital. 5 months is probably reasonable to bring them in. If they come from far away, we'd often do it all in one. Like trip if you like. So then around 6 months, and we would usually have the patient come to the office, meet the whole team, you know, urology, gynecology, colorectal, and then plan to take them to the OR and do a cystovaginoscopy and examination and anesthetic. And at that time, we tend to do preoperative urodynamics. So, we place a urodynamics catheter in the bladder as part of that exam. And then they get a 3D cloacogram done. Uh, what do you mean by a 3D cloacogram? Can you take us through a pediatric surgeon's version of a 3D cloacogram? So, we start off by doing a cystoscopy. What's the first structure that you encounter when doing a common channel scope? And as Mark said, the easiest structure to get into when you do a cystoscopy of the common. Channel is you usually go straight into the vagina or vaginas. It's actually quite challenging to get into the urethra and the, and the bladder because you have to really point very far up to take that turn. And, and we'll show that in a second. So, we would scope the bladder, we'd scope the vaginal vaginas, we try and see where the, where the entrance of the rectal fistula is. It's not always that apparent. So sometimes you might inject some fluid through from the mucous fistula to define that. If there's a septum, very often you'll see the rectal fistula in the bottom of the septum on the rectal side. And once you define that anatomy, we'll measure the length of the urethra, the length of the common channel, the length of the vagina itself, and get a good sense of the anatomy. And then you want to be in a position where you can inject all three of the structures with contrast and acquire images. Uh, there are various places you can do that in our, in our hospital, it workspace to do that down in radiology. So the patients actually go down to radiology. I know other people have hybrid ORs. We don't use one. And then you acquire images with a vascular sea arm and the software of that is something which we've all shared amongst ourselves. So if anyone wants the protocol, we can share that. And then that basically allows you to acquire images. To reconstruct three dimensionally, like we're seeing in the pictures here, the structures of concern. And the big advantage of the three-dimensional reconstruction, which we'll show here in a second, is that you can get a spatial understanding of what's going on because the patients, unfortunately, don't always read the textbooks. So, Richard, let me interrupt for a sec. We do have listeners that I think this is tough for them to perform, but I I think some of the key points that you bring up are that when you're doing the cystoscopy, usually with a rigid scope, then you're looking at the vaginas, you're looking for a septum. I think it's also important to look for cervices and see if you have one vagina, two vaginas. Do you have a cervix which will sort of be the front door? Is there a uterus behind there, trying to understand the malarian development of these children. And then also looking for ureteral orifices, right? Cause in some of the more complex. Malformations, you have some anomalous ureterral attachments to either the bladder or even bladder neck, etc. And so they sometimes come in very low, and, and could be treacherous. And so, knowing that anatomy in preparation for your repair, I think is important. And then, like you said, if you have the Technology available to you using a fluoroscopic sequence, you could develop these 3D structures when you inject contrast into all of the organ systems. All right, so just to summarize, in a patient who's 5 to 6 months old with an established diagnosis of cloaca, bring them in, multidisciplinary team. And then in the operating room, do a detailed exam, scope all of their common channels as well as urethra, vagina, and rectum, measure the lens, leave the catheters, and take them to radiology for a 3D cloacogram. Jason, the one thing I'd love to stress as well, and I, I appreciate you mentioning the malaria anatomy because I think that cervix piece is really important as well as the ureters. But I think if you look at these pictures we have, you can see when we're looking at a lateral view. How the common channel actually takes a very significant turn as it gets behind the pubis. All right, and if you are on the stay current app, remember you can see the images of the cloiccogram in real time. Especially on these longer common channel cases. And so what we found when we studied it was that the cystoscopy significantly undermeasures the, the structures when you scope and you compare the same patient's 3D reconstruction to their scope. And we did a study with a number of institutions to show that because your straight scope can't measure that turn. The study that Dr. Wood is referring to is measure twice and cut once, comparing endoscopy and 3D cloacogram for the common channel and urethral measurements in patients with cloacal malformations. This is in the Journal of Pediatric Surgery from October of 2019. And if you are in the stay current app, you can click on the link and be referred to this article below. So what you'll end up doing is maybe not getting the most accurate view of what you're dealing with if you only use cystoscopy. So, it's fine if that's all you have, that's all you have, but just be aware that you may significantly underread the length of the common channel if you're doing it just with a scope. But I, but I, I think I would say that, you know, endoscopy in the hands of a general pediatric surgeon who doesn't have a lot of experience with cloaca. Certainly has value in figuring out whether it's a straightforward Cloaca, not that there are that many that really could be called straightforward or a complex one. And I think one of the major changes in cloacal management is surgeons realized that they needed to do some evaluation of the complexity of the Cloaca before they attempted a repair. And if they realized that it was a very difficult one. They didn't do the case. They referred the patient to a center that does a lot of these, because I can tell you, as recently as 10 or 15 years ago, there was a lot more redoing of cloacas that was required because I think surgeons attempted a repair and then realized that it was much more complicated than they had imagined, and now with routine endoscopy, just making sure what kind of. Cloaca you're dealing with, many surgeons realize that it's a bit too complicated and it ought to be referred. And obviously we're gonna talk about the different type of cloaca, those that are a little bit more lower of a confluence and those with a higher of a confluence. The lower confluence, if you know how to do it, is a beautiful, elegant operation. The higher confluence one where vaginal replacements and. High vaginas and all of those kind of things, ectopic ureters come into play, probably ought to be done by specialized centers, but the endoscopy is a good. Differentiator if you can't do this elegant radiology that Richard is showing. This is why it's extremely important to know your anatomy. I think the key to the algorithm which we came up with was really to try and help people identify the patients that could have a very reproducible reconstruction versus the ones that need much more significant reconstruction to be done. And I think the benefit is that you can select these patients out very accurately if you just get a couple of measurements and a few spatial bits of information. You can find that algorithm in the paper published in 2017 called COICA Reconstruction, a new algorithm which considers the role of urethral length in determining surgical planning. And this is in the Journal of Pediatric Surgery, and again, if you click on the link below, it will take you to this article. As we said before, you got to make the diagnosis. We spoke about the newborn management to keep the patient safe. Then you get to the point where you do your endoscopy and hopefully your imaging. And at that point, you get two bits of vital information. And then there's a third one, which we'll get to in a second. So the first bit is, what's the length of the common channel? And everyone's known about that for a long time. If the common channel is less than 1 centimeter long, We would generally call that a type one cloaca. If you're on the app, you can refer to the flow diagram of reconstructive strategy and cloacal malformations. This can also be seen with additional information in the article that is linked to organizing the care of a patient with cloacal malformation, key steps and decision making for pre-intra and post-operative repair. It's really a hypospadic urethra with a rectiv vaginal fistula. And in that situation, we don't touch the hyperspatic urethral orifice. And the plan here would be to do a vaginoplasty, and entroidoplasty, and a PSA. That will be a very reproducible thing to do to make a good size roidal opening, and to do a PO to put the vagina in the correct position. I will say though, that the imaging in this case is really important because the true rectum can still be high even in a type 1 cloaca. And so knowing how high the rectum is, is important, even if the common channel is short. The next patient group is where the common channel is between 1 and 3 centimeters long. Again, you can refer to your flow diagram here. The other vital bit of information here is the length of the urethra. So, a normal urethra, we want to be at least 1.5 centimeters. So if the urethra is more than 1.5 centimeters, this would be amenable to a total urogenital mobilization and a piece of. All right. So to summarize, you can do TUM if you have a urethral length greater than 1.5 centimeters and common channel length less than 3 centimeters. If the urethra is less than 1.5 centimeters, we'd advocate for a urogenital separation. And the reason for that is if you were to do a TUM on a patient with a 1 centimeter urethra, and although these patients aren't common, they do exist. Then you will have the bladder neck sewn right near the perineum, and that could render the patient incontinent. So we would prefer to advocate for your genital separation in those cases, but the majority of 1 to 3 centimeter common channel cloacas will have a normal length urethra, and they'd be amenable to a to a gentle mobilization. And a piece of. And then if you get to the greater than 3 centimeter common channel group, those patients would in many circumstances have a urethra of less than 1.5 centimeters. And if either of those things are the case, then we would advocate for a urogenital separation and a repair of the common channel which we leave as the urethra. And mobilization of the vagina down to the perineum and a piece of For common channel length greater than 3 centimeters and urethral length less than 1.5 centimeters, we strongly advocate for your genital separation. And if the vagina or vaginas are unable to reach the perineum, then the patient may need a vaginal replacement in order to bridge the gap and we can talk in more detail later about what the options would be for vaginal replacement. Any tips if the rectum is too high? The other caveat that I would add would be that if the rectum is high, you may want to consider doing that with an abdominal approach to mobilize first. And so that may change your piece of approach to a or laparoscopic assisted PSA in order to mobilize um length. And now for all of you interested in the history of Cloica. So I just, I feel compelled to give a little bit of historical context because That was Doctor Mark Levitt from Children's National. It is absolutely amazing to me to see this level of detail on a single slide. Which I hope everyone recognizes, and I suspect many don't, is probably something like 50 years of work culminated in a single slide, and I wanna give due deference to um Hardy Hendren, who was really the father of cloacal management. In the late 1960s and 1970s with a very specific focus on the urology part and urethral reconstruction. And then, of course, Alberto Pena, who I was blessed to be mentored by from a young age as a medical student, who in 1996, made a major advance in the care of Cloacass with the development of the total urogenital mobilization. Prior to that, all patients had a urogenital separation. Which we're gonna discuss on the next podcast, but he said, why don't we keep the urethra and vagina together as a unit and mobilize that forward? That was in 1996, not that long ago, and then 21 years went by. Before the next major change in the CLACA protocol, and Richard, you were there presenting this paper. What year was that at ABSA? It was 2017, and Jason was there too, because he, he asked me some questions. Yeah, Jason, Jason and I were there. Presentation. It was a good one. And they, it was, it was absolutely amazing experience, and I, I just want to tell you it from my vantage point because you guys both know I was in the. Room in 1996 as Alberto Pena's fellow when he showed Hardy Hendren the TUM and he drew pictures and Hardy Hendren's eyes lit up and said, wow, this is a major advance in cloacal management. Then many years went by, and Richard and I really worked very hard on figuring out what was the problem with doing a TUM in a patient that had a higher confluence. And that's when the whole concept of the UG separation came back into play. Again, that was the Hardy Hendron approach, the UG separation. And then Richard, you presented this at ABSA in 2017, and I was there watching and amazed, and Hardy Hendri himself at the age of 91, got to the microphone and we shuddered a little bit, wondering if he was gonna say something that our work was no good. And he got to the microphone and he said, I have no question. I just have a statement. I agree with everything that's been said. And he sat down. And it was the last session, the last presentation in a session. There were 200 people in the room and I got up to the microphone and there's a photo of Richard at the podium, Hardy at one microphone, and me at the other microphone, and I said, I just want everyone in the room to know who just asked that incredible question, and I want to give him credit for 50 years of work on this very challenging problem. Um, and, uh, so I just think we need a little bit of historical context because this algorithm that Richard just described is really for the first time, and I've been looking at this stuff for 25 years, the first time that it is reproducible, what to do for Cloacas. So I really appreciate, Richard, you putting this together in this beautiful format. And I look forward to the next time when we talk about the actual cases that you have brought on whether we do a TUM or UD separation and how we make those decisions. Mark, thanks for saying that. I, I will say that I was just relieved that Doctor Hendry didn't have a question for me, if I can be completely honest. But, um, the other thing I will say is that to, to Jason's point earlier, um, but, you know, if you just stick to the algorithm, it really works. And we've looked at this. And I think it's now 116 patients in a row. And if you follow the algorithm, we haven't once had to change the plan. So I think we feel very comfortable that if you just follow the rules using 3 and 1.5. You can stay out of trouble. And just to Mark's point earlier, if you're not an experienced cloacal surgeon, it gives you a really good guide as to which ones are reproducible and which ones you might want to get help from a friend with. So, and I, and I, and I just wanna mention, I don't think I said it clearly, the major change in this presentation was to make sure you measure the urethral length, because before that, it was common channel less than 3 or greater than 3, and that was it. But now we know that we got to know the urethral length because that will obviously influence what to do for the surgery cause the patient needs appropriately lengthed urethra at the end of the operation. Doctor Fisher, can you clarify how we determine urethral length? That is not the length of the common channel to the bladder neck. The definition is of urethral length because I think some people may consider that from. The single orifice all the way to the bladder neck, and I don't think that's what you're alluding to. Yeah, so what we mean is that it's when your common channel splits, it's the distance from where the urethra leaves the common channel to where it enters the bladder. And so that's what's difficult to measure really accurately with a cystoscope because you're around that curve that we can see in the picture. And so because you're around that curve behind the pubis in these longer ones where it's actually really important, you, you can seriously under and overread that and that's what that study that we did showed. Getting an accurate measurement of the urethra is fundamentally important because what we want to do is land up with a bladder neck above the urogenital diaphragm. Which is where the external sphincter or where the sphincter complex, the urethra lies. And also just then your intraabdominal pressure doesn't mess you up. So, measuring from where the common channel splits to the urethra and vagina is the key startoff point and getting to the bladder neck. And, and you can use a ureteric catheter, which you can watch and measure it that way, or you can measure it on your scope, but your most accurate measurement probably comes from your imaging because it's not straightening all the structures out and then falsely measuring them. Really fantastic work. Thank you so much for sharing. All right, and now for our joke of the day. Doctor Levitt. Did you, um, Jason know that there was a competition for the best samurai in the world? Oh, I thought you were gonna say the best looking person on this podcast. I'm like we're both losing. No, the, the best samurai. There were 3, the competition for that. Yeah, yes, there was a, there were 3, there were 3 finalists. The competition was held in Tokyo in front of the Emperor of Japan, and the 3 finalists, one was a Japanese samurai. One was a Chinese samurai, and believe it or not, the 3rd uh contestant that made it to the final was a pediatric surgeon. You didn't hear about this? Yeah, it's crazy. So, um, the Japanese samurai in his final act takes a little box out of his pocket, and a butterfly is released. And he takes his samurai sword, and he slices the butterfly in half. Amazing, and everyone claps and thinks that that can't possibly be beat. The Chinese samurai takes the box out of his pocket and he releases a bee. And with two slices of his sword, he cuts the bee into 4 pieces. Amazing. And so obviously he's now the winner. And the pediatric surgeon steps forward and he releases a tiny little gnat. And it buzzes around and he takes his sword and he slices it through the air, and the gnat keeps buzzing and lands on the wall next to the emperor. And the emperor says, that was a really valiant attempt, nice try, but you didn't kill the gnat. And the pediatric surgeon said, kill the gnat? That was a circumcision. Oh boy, you never, you never cease to amaze me. All right, and we're out of time. That was Cloaca, Part 2, operative planning. To summarize, we discussed the importance of the Cloacogram and intraoperative measurement using a cystoscope for cysto and vaginoscopy. So using that flow diagram to differentiate between a less than 1 centimeter common channel, 1 to 3 centimeter common channel and greater than 3 centimeter common channel and then determining your repair based specifically on the length of the urethra and whether or not it is greater or less than. 1.5 centimeters. With the next episode, we will plan to discuss surgical management and exactly what goes into the operative repair. So make sure you tune in for the next time. This is Amanda Jensen with Cincinnati Children's and here are Ammad from Nationwide Children's.