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Colorectal Quiz Episode 24: Cloaca Part 3
Published:
Topic overview
Expert discussion on operative management of cloacal malformations, emphasizing the importance of simultaneous urogenital and rectal reconstruction to prevent reoperations and reduce long-term renal dysfunction. Surgeons from Riley Children's and Nationwide Children's review a protocol for preoperative imaging that allows surgeons to accurately assess anatomy and determine case complexity before operating.
Timestops
0:05
Introduction and Case Overview
6:01
Imaging Assessment and Common Channel Measurement
9:12
Rectal and Vaginal Mobilization Techniques
13:35
TUM Procedure for Adequate Urethral Length
21:59
Short Urethra Management and PSRVT Approach
33:13
Urethrovaginal Fistula Prevention and Surgical Pearls
40:10
Protocol Development and Outcomes Discussion
44:09
Closing Remarks and Key Takeaways
Key takeaways
- Complete cloaca repair should address both rectal AND urogenital components in one operation to avoid reoperative scarring and ongoing UTI risk
- 30-50% of cloaca patients develop long-term renal dysfunction, making comprehensive urogenital reconstruction critical—not optional
- Preoperative imaging protocol allows surgeons to define anatomy and complexity before deciding whether to operate or refer to specialist center
- Incomplete repairs leaving urogenital sinus unaddressed perpetuate vaginal voiding and continued risk of kidney damage from recurrent UTIs
- Modern imaging-based protocols have reduced redo surgeries by helping surgeons accurately assess case complexity before committing to repair
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Transcript
Click "Show Transcript" to view the full text (40685 characters)
Hey there listeners, it's Amanda Jensen from Riley Children's. Welcome back to the Colorectal quiz. Today we have a very special episode, episode 24, and it is part 3 of a 3-part series discussing Cloaca. Specifically for today, we will pick up talking about operative management. Doctor Fisher, why don't you Start us off. All right. Welcome back, everyone. Here we are with another episode of the Colorectal Quizzes. I am really excited because we are talking about Cloicas again. We are doing another recording, which means we are going to have lots of content. And lots of discussion about this condition that challenges us both in and out of the OR. And so I'm really excited, we have some guests with us today. And of course, my partner, Doctor Levitt, will uh help introduce everyone on the screen today. Doctor Levitt is my dad. Um, uh, my name is Mark. I get called on that like 2 times a year. I always mess up. That's OK. Um, and he's a very smart neurologist. And by the way, you know, he told me that when I went into surgery, I had chosen a non-cognitive specialty. So, take that, like you said, he's a smart man. Yeah. All right. Well, we're back, um, and we're continuing our discussion about CLACA, and with us is a CloaCA expert and my partner in crime in helping children as well from Columbus, Richard Wood Nationwide Children's Hospital, with whom I spent many, many, many, many hours trying to work out a protocol to make Cloaca a little bit less of a mystery. And we're gonna try to explain that today, uh, because it's a mystery to many surgeons, but I do believe with the proper thought process and proper radiology, it actually can become more of an understandable entity. Richard, thank you for joining us again. Great, thanks for having me. So I think today we're gonna go over a couple of different cases and scenarios. And so, Richard, why don't you take us through what you have planned for us, because I think we're going to discuss sort of shorter segments, longer segments, what to do with the urethra, which I think really makes a lot of us nervous. I know, I've seen a number of patients where a lot of surgeons do a great job mobilizing the rectum and putting that in the right place. But it's a urogenital reconstruction that Really challenges us. And I think Richard and Mark put together a real great protocol here. And by the way, uh, the fact that you mentioned that, unfortunately, Richard, I know, um, you have seen this as well, where a surgeon has managed the rectum and has ignored the urogenital sinus intentionally, saying, oh yeah, we'll deal with that later. And that is not a Ideal. The ideal time is to understand the entire cloacal anatomy and fix it all at once. And the urogenital complex is the complicated part. The rectum is usually the easier part, and it ought to, uh, it ought to all be done together. Mark, that is a great point because I think uh many of us at like these tertiary and coronary centers, the surgeon is capable of moving that rectum, putting it in the right spot. And leaves the eurogenital sinus, and then that's left for the reconstruction surgeon to go and attack that. But now you're going into a re-operative field, which clearly is a more challenging situation. So, if we could do it all in one setting, probably a better situation. And I think the other thing to mention is that, you know, the, a lot of these kids are still having issues of vaginal voiding when you've done the genital part. And so you haven't really removed that risk of UTIs, which is so damaging for kids with cloaca and, you know, if we look at the long-term outcomes of these children, about 30 to 50% will develop long-term renal dysfunction, so. I think from a practical perspective, I agree with you, Jason, it's a much easier approach when you don't have a, you know, a, a scarred field to work in, but I also think there's the, there's this thought that these kids are taken care of when they're really not, and then their ongoing risk of renal dysfunction is not taken care of either. So, I think it's like Mark said, really practical to get a holistic approach to the malformation and a sort of. Overarching view on how they should be reconstructed and then, you know, either it's, it's one you can tackle or one you need help with. But, uh, I think the benefit of the protocol, which we're gonna talk about is that you don't have to change your plan. I will say just in addition to that, if I, if I may 1 of the things about the protocol that I am most proud of is that surgeons can define the anatomy before they decide whether or not to do the case. And I actually have noticed a significant reduction in the need for redos because surgeons are following the protocol, understanding the measurements and saying, OK, this is a cloaca that I think I can handle, or this is a cloaca that I think needs to be referred. And I don't think surgeons are diving in. To cases that they probably ought not to be doing, and maybe that's why the redo number has gone down. And that's what I think the protocol is probably most valuable for, and then, of course, it of course, of course, it has value for the surgeons who are actually gonna do all types of Cloaca, knowing what type of CloACA to do, and that's was Richard's point, is that the imaging is so good, we now know what to do, and then when we execute, it's exactly what we thought we had to do. And that's a really dramatic change, cause when I got started in this, you know, as a, as Alberto Pena's fellow, I vividly remember that the cloaca would be set up for the day. We would start with a cysto, we didn't do any imaging, and then we basically said, OK, let's go for it. And then we spend the next 8 or 10 hours watching artwork play out, and that was just simply not a reproducible way to solve these kids' problems. Thanks, Mark. If we look at the first case, and I'm sorry about the psychedelic pink, but my radiologists got very creative and I thought it was a good one to show. But we have 3D reconstructed imaging here of a patient who's about 6 months old. And what we can discern from this imaging is a urethral length of 2.2 centimeters. And the cutoff we use in the protocol is 1.5. So a good adequate urethra. And then we can see a common channel length in this patient of 2.5 centimeters. So I would say this would be a fairly classic short common channel cloaca patient. The one caveat I will say, and I'd like to get comments from Mark and Jason is, in this case, we see the rectum is quite high, and I've included a picture on the right which shows us where the spine is. And so to my eye, I always like to look at the PC line or the line between the pubis and the coccyx. And in this particular case, the rectum looks a little higher than the PC line. And so, I think that's the third piece of the puzzle we spoke about in the, in the, in the discussion on the protocol. What's the urethral length, what's the common channel length, and what's the relationship of the rectum to the PC line? So Mark, I don't know if you wanna give us some thoughts on, on your ideas here with regard to the rectum. And, and, and I guess the UG sinus as well. So, uh, interestingly, I, uh, our fellow just graduated and was, today was my last case with him, Rodrigo Man is on his way to Atlanta, very proud of him, did a phenomenal job. He said, can I just ask you a quick question about Cloaca? Can you just give me a quick one-line summary of what I need to know from that beautiful paper? This is literally what he asked me about 2 hours ago. And I said, sure. Common, common channel. Less than 3 centimeters sets you up for a possible TUM. But, you need to have an adequate urethral length, and that's 1.5 centimeters or greater. Common channel greater than 3 centimeters almost always means a urogenital separation. Period. And if so, if you know the common channel length, and you know the urethral length, you can pretty much make any plan, and he said, Thank you, and he walked off into the, into the distance. But, um, and basically, That is 50 years' worth of work in this field, starting, we talked a little bit about the history on the last time, starting with Hardy Hendrin and Alberto Pena and all of that, and here we are, good radiology, common channel, 3 centimeters or less gives you a TUM option, but you need an adequate urethra, because when you pull down the TUM when you pull down the urogenal complex, You better leave yourself with an adequate length urethra, otherwise that urethra will leak. So, with regard to the rectum, I completely agree, Richard, PC line is key. The one thing I will say, as opposed to a male rectum, where you're sort of differentiating the height of the rectum and whether you need to do laparoscopy, or whether it's reachable by PSARP, one thing that I have found is if you do a TUM the rectum comes to. So a high rectum like this might actually be doable posterior sagittally. With a good mobilization of the of a TUM. Now, you need to sort of get a feel for that and decide before you go, because I don't know if you would have started this laparoscopically to do the rectum, but I might have given this a go posterior saggitally, knowing that the rectum is high, but I think it's probably reachable posterior sagitally with the assistance of a TUM. Yeah, Mark, I must be honest and say like we've, we've done a lot more laparoscopy in our cloacal repairs, um, of late. I do think there's really good benefit in releasing the rectum from above. I mean, you obviously having done lots of this will know that those. Lateral attachments posteriorly on the vagina, where the blood supply lies, is like such an important safe plane to be able to get to and to get a good release of the TUM. And I think with a high rectum like this, it's not always that easy, and we're talking here practically for people who maybe haven't done hundreds of these, to, to see that attachment blood supply piece. And I think an important point to make is that you have to really go into the peritoneum with these releases because. You, you've got to get the rectum out of the way to be able to mobilize the posterior vagina. So, my, my preference here would be to be laparoscopic, get the rectum freed up. The one thing I will say in agreement with Mark is I usually don't divide the fistula off the back of the vagina, I just mobilize the rectum. And then when I do the TUM then you can divide the rectum and uh off the vagina and repair it and the rectum. right there rather than separate it. And then you have to go and try and find it in the pelvis and make sure it's not twisted and all that. So, mobilize, but leave it on, and then you can just take it off. But as Mark said, you could do it either way or try TUM first. And if it doesn't reach, you can always mobilize more with laparoscope. That's a very good discussion. And just to give some credit, I know Belinda Dickey in Boston is also doing a fair bit of work on. Uh, laparoscopic mobilization even of the um UG sinus. For completion's sake, Richard, do you wanna talk a little bit about your experience with the laparoscopy for the UG sinus part? I'm happy to. Do you want me to do it when we do the separation, please? Yes, that's probably wise. Yeah, so, OK, so let's, uh, let's continue with this case. It meets criteria for a possibility of a TUM. It's less than 3 centimeters common channel. The urethra is greater than 1.5 centimeters. Richard, you would agree that you would, you, you would believe prior to surgery. That a TUM would be successful. The rectum is another discussion at the moment. We talked through that. OK, so we're going to go for this from a TUM point of view, total urogenital mobilization, correct? Yeah. So I think the first step is to expose the, the open the posterior sagittal incision, uh, expose the coccyx and take that incision all the way through to the common channel. The illustrations we've included are ready to make the point that while in the separation, we leave the common channel intact. When we do the TUM we, we open the common channel until you can see where the urethra. Enters, uh, or leaves the common channel. Um, because I think the approach is really different. I know when I was training initially, we used to open the common channel for the separation patients as well, but then you have to reconstruct it. And I think, you know, that recent publication where you showed 97% urethra is intact. I think a lot of that's because we're not opening the common channel, and then re-suturing it up and lining up with, with strictures and whatever. So, I, I do think leaving the common channel intact when you're doing a separation makes sense. But for TUM the plan is to open the common channel widely, to expose where the vaginal entry is and where the urethral entry is. I, I, I think it's a good habit to get into, to remeasure everything at that point and make sure that you haven't got things wrong. Because to be honest, at that point, you still haven't reached a point of no return. And if you now find, wow, this common channel's actually 4 centimeters or 4.5 centimeters and we mismeasured, you could still do a separation at this point and re-suture the common channel closed. So I, I think it's an important thing to mention, you can remeasure your common channel now because you can measure where the urethra goes off. And um And you want to fully mobilize the rectum like we discussed a little bit earlier, because by fully mobilizing the rectum until you reach the peritoneum, um, I think what you really see there is the blood supply and the attachments to the back of the vagina. And that really allows you to mobilize the vagina adequately posteriorly, which allows it to drop when you do your TUM. So, I think, you know, there's a lot of talk now in PSA repairs that you only mobilize the rectum just enough because, you know, there's that work that Restorantalis groups done showing that there's some benefit to keeping as much of the rectum as possible. But I think in this case, you really have to mobilize the rectum all the way to the peritoneum. So you can see all those lateral and posterior attachments of the vagina. I don't know if you have any other thoughts on that, Mark. Yeah, and then, and, and, and of course, remember, yes, I think that was really well said. You're opening the common channel. Until you can see clearly the urethra and the vaginal opening, that often requires opening up into the vagina. Don't worry, you can always close it if you open up too much, but you wanna see the urethral orifice. That's the time to put the Foley catheter. That's the time to confirm the common channel length from that urethral orifice to the perineum. As Richard said earlier, our imaging is so good that we've measured it right every time. We've never had that disconcerting feeling that we had the common channel measurement wrong. And then, yes, get the rectum off, full visualization of the TUM and then we're gonna go for the TUM with sutures all around the um roidal opening and the urethral opening. And remember, the TUM requires that ultimately you will split. The common channel down the middle, and the two sides of that common channel become the labia minora. And the urethral orifice goes to the perineum, and that point to the bladder neck is the urethral length, which of course, you've pre-measured, and that needs to be at least 1.5 centimeters in order for that not to leak. And that's essentially the TUM once you fully mobilize the rectum and fully mobilized uh the urogenital complex. Now, maybe Richard, just go through a couple of tricks. I know this is a part that people get a little nervous about. Is the suspensory ligaments of the urethra, the anterior dissection for the TUM? How do you find it? When do you know you've done enough? Just give us some of your, uh, uh, game day decisions there. Yeah, Mark, I think, uh, first of all, I would say, like any other time in this field, you always work laterally first. And so I think finding the plane as a full thickness plane on the sides is really beneficial. I think sometimes people are nervous and don't do a full thickness dissection. And I think that can get you in a lot of trouble because then the lab, the, you know, the common channel starts falling apart, which means you don't have good tissue to sew. So you really want to do a full thickness dissection and there's a bloodless plane that you can get into laterally. And then you want to divide the common channel, usually 0.5 centimeters behind uh where the clitoral tissue is. So you make sure you don't damage any of the nerve supply of that. And that would be a good landing place for your urethra. And then, yeah, if you do a full thickness dissection laterally, and then you come around the corners to the front, you can get into that bloodless plane, uh, in front of the urethra. And then, you know, with good tension, um, you're able to expose that plane as you go down. And I think it's important also to keep feeling where the pubis is. So you make sure you get to the bottom of the pubis where the suspensory ligaments start. I know there's been a lot of discussion about partial versus complete TUM, uh, especially in the neurological literature. There's never been really any great demonstration that partial TUM is better functioning than full TUM. But I'd say from my perspective, uh, I mobilize enough. I know that sounds silly, but, um, I don't think you always have to go to the space of Rhetsius in order to get an adequate TUM without tension. I think sometimes you can do the lower suspensory ligaments and you're reaching tension-free, and I think it's fine at that point to just do a partial TUM. And then in, in other times, you would have to go all the way to the space of red Cus. You see that fat, and you know that you're in, in good tissue, and then you'd be tension-free there too. But I don't think I would prescribe that you have to always go to Retzius's space. I think it's you, you go as far as you need to, to get a good, a good repair, because I think if you can leave some ligamentous attachments on the front, there's probably some benefit to that. That's excellent. And I think, honestly, anyone who's about to do a TUM should listen to that. I do like the pushing with your finger on the anterior portion of the urethra to feel the little bands. And know what to release. Lateral sets you up for the anterior, no question. And I like to see, I really wanna go until I see the um retropubic fat. You see a little uh fatty layer, which you know is covered by a little whitish fascia, and when you incise into the whitish fascia, the TUM releases, it gets nice and free. You gain about 22, 2.5 centimeters at that point. And then you split the common channel as I described before. And then I think it's really important to make sure that the introitus is a good size and fits nicely into the space where you need to place it, and it's not too small and not under any tension. And that's tricky cause you gotta do maybe some more lateral dissection of the vagina. And then you've marked your anterior limit of the sphincter. So you know how much perineal body you get to have based on how much inroitis you want. You don't want the introitus filling the the perineal body. Sometimes you actually have to close the posterior wall of the vagina a touch to get a nice-looking introitus. You wanna keep your eye on the labial fold, the skin fold where it meets in the midline. Then you give yourself a perineal body and then you put in the anoplasty. So Mark, do I interpret then that you would always do a full TUM? Yes, I, I, I want it nice and loose and free and no tension. So, yes, I, I've not, I've not done a partial cause I'm worried it's gonna be under some tension. And the blood supply is excellent cause you've kept everything intact, and of course, you haven't separated the two structures, so you won't get a fistula. So, Um, as long as your full thickness on the vaginal side, blood supply should not be a problem. So that's a TUM and I think that's pretty reproducible and a well-trained pediatric surgeon with some experience with this should be able to do it. And provided you know in advance from good imaging that you have a TUMA bole case. And to repeat, less than 3 centimeters common channel, and a 1.5 or more centimeter urethra at the end, once the common channel has been split. That's why it's so important to define the urethral length. All right, so let's keep going and talk about the scenario where you don't have enough urethra, or you have a very long common channel. Obviously, the two of them go hand in hand. I will say also, by the way, a short common channel almost always has a good length urethra. On occasion, you get a short urethra with a, with a low common channel. So those usually go hand in hand. But if you were to do a TUM in a patient with a short urethra, you're gonna have basically the bladder neck is at the perineum, and that's a, that's a miserable result. All right, so let's talk about the higher situation. Yeah, so here we have a scenario of a patient again, 7 months old, who has had the imaging done and we see the 3D chloacogram again and a couple of interesting points here. Number one, the common channel is 5 centimeters long. Number 2, the vagina is 5 centimeters long. And number 3, the urethra is 1 centimeter long. In addition, you can see that the most posterior structure is actually the vaginas. And the rectum actually enters in between the vaginas and the urethra. And that's not actually that uncommon a scenario. You do see it from time to time. And I think it's really where the three-dimensional imaging is incredibly important because it gives you a spatial understanding of what you're seeing. And I mean, we do a lot of these surgeries, and I still look at the 3D cloacogram just before I start the case, and I always turn it so it's in prone position. And you try and get in your mind's eye, like, where am I gonna find where? I think the other point to make in this case is that the Vaginas, because they're the most posterior structure, are often quite stuck to the presacral fascia. And that can be more difficult to take down than you might perceive because with the rectum, you've got that nice thick wall. Once you get through that white fascia, it delivers very nicely. Doesn't do that quite the same way with the vagina, so just be aware when the vagina's posterior, that freeing it up properly takes a little bit more work, I would suggest. So in this scenario, as Mark said, we've got a patient who does not have a short common channel. They also have a short urethra. So for my money, this is a separation case every single time. And. I think the question you're gonna ask now would be, how am I going to start the separation? Am I gonna start it from the posterior sagittal position or I'm gonna start it through the abdomen? And I, and I, I know, I know what Mark's gonna say cos I've had this discussion with him many times. But um, Mark, do you wanna give your thoughts and then I can chat a little bit about your other scenarios? Yeah, well, I mean, this, this is the, there, there is really no debate here that you need to do a separation cause there's essentially no urethra. The harder ones, which we don't have an image of, are the borderline cases, but this one, there's no question we need to do a separation. The one thing I really would want to know is an image showing where is the sacrum relative to this spot, and then, You already described it very nicely. I'd like to start posterior sagitally no matter what, even if I know that there has to be abdominal work. And the reason why that, unless there's a situation where everything is above the peritoneal reflection, which is exceedingly rare, but does happen. But if I know that the separation of the vagina from the common channel is reachable posterior sagitally, There is, I think, great value in starting that separation. Again, we're doing a UG separation. We, we're no longer talking about TUM. We put some stitches in the, in the vagina, open the vagina, leave the common channel untouched. Do not open the common channel. Make the uh meatoplasty just enough to slip a catheter in, maybe 1 or 2 millimeters, but do not touch the common channel, go high, right under the coccyx. Um, you find the bulge, that's the vagina. You put stitches on the vagina and open in the midline. Then you put your stitches along the anterior vaginal mucosa. You start your separation, that's the tricky part. The ureters are coming in from the side, so be careful, stay very midline. And do as much as you can comfortably, laterally and midline. And as soon as you feel uncomfortable or it's too high to reach. That's the time where you could have, um, you could, you could close the vaginal urethral fistula. Usually you have enough, enough exposure to do that at that time. We do that with urology. Urology closes it, and I will say, another thing we learned fairly recently in our process, Richard, is leaving a little bit of cuff, a little bit of vaginal tissue, so they can get a really nice urethra, urethra closure. I think in the past, we didn't get that little nuance. We didn't leave enough tissue and it was a little tight. Now, you get a nice closure. And that gets covered with SIS and a fat pad, but you were asking me specifically about the separation. Then, it's time to go into the abdomen, and in my hands at the moment, I would go into the abdomen via a lower midline laparotomy. Laparoscopy may be very appropriate at this juncture for the continued separation. And get the vagina to mobilize. And then that's where your decision-making comes in, whether that vagina is going to reach, um, usually, yes, or if the vagina is going to need a replacement to get to the perineum. All right, can I bring up a question? Great conversation. I, when I get to that, Abdominal approach to that separation, get very worried about the ureters, and we made very little mention of them. Yeah, Jason, I, I'm pleased you brought that up. I think there are a couple of points, uh, I would add to what Mark said. I think we got, we got a lot of good information from our scopes. So we knew this kid's urethra was 1 centimeter above the common channel. So if we can see where the urethral opening is, then we know that the bladder neck lies 1 centimeter above that hole. And if we've scoped the patient, we know where the ureters are in relationship to the trigone and the, and the bladder neck. Many times these higher common channels, the urethra, the ureters actually coming quite close to the bladder neck. And so I think you have to map all that out in your mind when the patient's posterior saggitally. So, you can see the hole, you go 1 centimeter above, measure it, say, OK, I'm at bladder neck now. My trigone is here, my ureters are there. I think once you get within 0.5 centimeter of where your ureters are coming, you really can't do any more safely from below. And if you want to be careful, you could even say, once I hit the bladder neck, I really shouldn't do more than that from below, cause you've got no way of knowing where the ureters are in the posterior sagittal position. We have tended not to put catheters into the ureters when we've done these separations from above, uh, with an open case because you have to open the bladder to do that. But of late, we've been doing more where we scope on the day and put in a urethral foley and put in ureteric stents prior to starting the case via a cystoscope. And I do feel it gives you a little bit more reassurance, especially if you're gonna approach these laparoscopically. It's quite nice to have the ureter extension cause it just gives me a little bit more reassurance. I, I, I fully support the stuff Mark said about if you're going from below. I think the one caveat in this case is that the rectum's anterior. And so there can be some benefit to going in with a laparoscope up front and basically coning down the rectum and taking it off like you would in any other. And erectile malformation male that you do a, a, a laparoscopic or lap on. And then I think you can see very nicely where the attachments of the vagina are to the back of the urethra and the bladder. Um, I think it's important to try and chase the ureters at that point because they are gonna come through anteriorly to where the tubes come off. And I think if you're gonna do your separation, like Mark said, you want to be really in the midline. And I think Belinda and I have discussed this many times. Most, both of us generally use the scissors just with the laparoscope for the separation because any burning makes us both really nervous. And so, you can put some heat on the scissors, but generally you just use um sharp dissection to do that separation. And you can get, you know, a really nice view of doing that. And I will say the other thing is we seem to be doing less vaginal replacements and Belinda and I have both found this with doing the laparoscopic dissection. I just think you can see slightly better deeper in the pelvis. The one thing I mentioned in this case was the patient's vagina was 5 centimeters long. And, uh, we wrote a paper several years ago looking at factors that predicted the need for vaginal replacement. And in that, we found the length of the vagina was the most significant predictor, not hydrocopos or anything else. Unfortunately, there's no like cut off like 1.5 or 3 for this. But if it's less than 4 centimeters, you're much more likely to do a replacement. And if it's more than 6, you're much less likely. This one's 5. In this case, we did do laparoscopically from above with the separation, and we were able to get the vaginas to reach. Now, I'm sure they probably would have reached with open approach too, but we chose to do it laparoscopically. And we're able to meet our dissection pain from below nicely with the, with the laparoscopy from above and that, that was how we did it. But I think to your point, Jason, the key to this dissection is You want to really know where the ureters are. So stick in the midline to start with. And once you're through in the midline, you can very carefully work laterally and make sure you can see the ureters so we don't injure them. And then, I think the other point that Mark made was, when we started doing these upfront. Uh, separations, we had two patients in the first year that got urethrovaginal fistulas. And that was obviously very frustrating to us. We knew that that was well reported to be 10 to 15% risk, but we then sat down with urology and Mark and myself and the 4 urologists sat right around the table and came up with a strategy of leaving more tissue so they could do a double layer urethral repair like a Like you would for any kind of bias flap idea. And then putting a fat pad and SIS on top of that. And I must say since that meeting, we have not had a urethrovaginal fistula since and that was 5.5 years ago. So, I think that change of doing the double layer repair of the urethra, the SIS, which is a single layer SIS and then the fat pad, those changes have definitely helped. Um, But I, but I, I just think you need to make sure that that dissection of the bladder neck is done really carefully. And, and the other thing which we try and illustrate is that while when you're separating the rectum off the, off the, you know, the, the bladder when you're doing a male case, the rectum tends to stay in its lane, so to speak. So you can kind of follow it really carefully, like as a tubular structure. Unfortunately, the vagina tends to sort of envelop the bladder neck a little bit. And so it often sort of wraps around it. And you have to be pretty careful when you're doing the separation that you keep working around it and making sure you're not getting into the bladder neck because it's not quite as um a straight line as you might want as it is in the male patients. By the way, I just want to point out, we um have, there are some really beautiful images that you'll be able to see on the app, and this entire summary. was really nicely written up. Uh, Richard first authored the paper in seminars in pediatric surgery in December of 2020, and in there are the published, uh, artistic diagrams of UG separation and the TUM. And we, Richard and I went painstakingly through dozens of revisions with the incredible medical illustrator to try to make these points. So, I really want you to take a look at those uh pictures. They're really, really beautiful. We're very proud of them and one of the things that they do show is this attempt to try to avoid a uh urethrovaginal fistula. Uh, that was a, a good lesson in, in, in life and of course in medical care. We had a problem. We put about 6 brains on the problem, sat down at a devoted meeting, and came up with a list of things that we were gonna do to try to avoid urethral vaginal fistula. We implemented, everyone on the team agreed, haven't had a fistula since. So, you know, we have to identify our problems. I, I, I can't emphasize that enough. We gotta be honest about them and then we need to work to Solve them and come up with a protocol, and then respect the protocol. I actually think of those things that you mentioned, probably the most important was giving a little bit of cuff, so you can get a really nice urethral repair with good mucosa and no tension. It's probably the most important um aspect. Anyway, I hope we didn't lose um you all in the complexity of this discussion. I think the goal was to try to make this particular podcast of interest to anyone who's interested in tackling cloacas. To give a sense of understanding of the complexity, particularly of the higher types that will need a urogenal separation, and to give a sense that the TUM is reproducible, and to make sure that everyone getting their imaging and making their measurements in advance, so that they can uh make a proper plan. Mark, can I make one point about the, the separation piece? Um. I, I know that there are some folk out there, um, maybe not necessarily in North America, but who are approaching these long common channel cloacus by closing the bladder neck up front because of the fact that the urethral outcomes of these patients have not always been fantastic. And I think one of the points we made recently was to show that if you use this protocol and you repair the urethra without opening the common channel, We were able to have 97% of patients have a catheterizable urethra. And I think one can't underestimate the benefit of having access to the bladder via the perineum because even if you land up one day doing a metrofenoff or something like that, the fact that you have a pop-off in the in the perineum that allows patients to empty. is really valuable to have. And so, I think it was good to Use the protocol, show the repairs, but then also show that the outcomes were that you could maintain perineal access to the bladder. When we know that not all of these patients are gonna empty their bladders and like you said, we could maybe talk about it in another talk about, you know, who needs clean intermittent catheterization. But having perineal access is important. And so I think. This approach really does allow you to maintain that and I think that is an important goal to have because long term, many of these children will need bladder management, which is why I think doing these things with urology makes a ton of sense. Um, so they are involved from the start, but maintaining perineal access is actually, I think, really important. And so that's why I think stressing. This approach to try and maintain that is important. Yeah, I think that's very well said. Um, this has been great. This is really, um, uh, a really nice way to summarize a very difficult, a very difficult area of what we do. Uh, this is clearly, at least for me. The most challenging operation we're asked to do, and, the most gratifying from an anatomic reconstruction and always keeping your mind on the goal of getting a good functional result. Oh well, we have a, we have a question here from Hira Ahmad, the, uh, new Seattle Children's Fellow. Uh, who spent 3 quality years at Nationwide Children's Hospital as the research fellow and quite the expert herself on colorectal. She usually asks very modestly, I, can I have a question? But usually in the question, it demonstrates that she has the answer already, but let's see what she has to say. No, I was just, thank you. I was just gonna say, are we able to reemphasize why it's not a good idea to go from a TUM, start a TUM and then go to a UG separation, and which is why it's so pertinent that we're talking about this algorithm over and over again. Ah, Hera, that is great. We forgot to talk about that. So, um, let me, let me tell you, again, we talked a little bit about the history last time, and just a quick review for those who didn't catch that podcast. Hardy Hendren would do a UG separation on every patient with a Cloaca, and then Alberto Pena did the same for years. And then came up with the idea in 1996 to do the total urogenital mobilization. Which was beautiful, and it saved many patients from that separation. However, I believe then the TUM was applied to too many patients. Um, and some of them ended up with the leakage problem that we talked about if the urethra was not quite long enough, cause in those days, we were not measuring the urethra. But the other problem is that if you go for a TUM, And it doesn't reach, what do you do? Well, then you need to go into the abdomen and continue your dissection of the urogenital immobilization. Now, on occasion, that's enough, and it does reach, but if it doesn't reach at that point, now you are in some serious trouble, because now you have to do the separation. And guess what? You've already dissected the anterior urethra. And therefore, you may have a reconstructed urethra now dissected on both sides that unfortunately, in a number of cases became ischemic and left the patient with essentially no urethra, and then they have to have a metrofenoff one day. So, here I point out very importantly, that you do not want to go for a TUM that is not gonna succeed. And I think getting this protocol organized in advance. That never happens because you know, it's a TUM and it's gonna work, or it's not gonna work, you must go for UG separation upfront and preserve the common channel. And Richard can attest that since we started doing these measurements, we never got into a Cloaca and said, oh my gosh, this is not what we thought. Every single time we implemented the plan for the case, and that's thanks to good measurement. So, here, I thank you for bringing up that very important point that we, we neglected, and it's a good example of why you need trainees and fellows around you to keep you at 100%. When you're only able to get 95% and it's not quite enough. Hera comes in and adds 5% and you get an A+. Thank you, Hira. Um, Jason, do you have a, do you have a joke for us? Cause I, I have a really bad one. If you'd like me to contribute, I'm happy to, but yours are usually better. Well, I think we're gonna be in competition today, Mark. Uh, this was a great talk for us, Richard, awesome work. I think great descriptions, great. Discussion on preoperative planning being key in these procedures. So my question to you. Is unfortunately another knock knock joke, knock knock. Who's there? HIPAA, HIPAA who? I can't tell you. Such a good one. OK, that's, that's pretty bad, and this is probably worse. OK. Amanda Who takes care of chickens? So a chicken tender. That requires some barbecue sauce. That's terrible. I don't know which one's worse. All right there, folks, that wraps up episode 24, Chloe Waca, Part 3, and operative management. Uh, please refer to the articles attached to this podcast in the Stay Current app, um, if you're listening with us there. And remember, Specifically with operative planning and operative management in Cloaca, that the length of the Cloaca common channel is important, as well as the urethral length in determining if you're going to be able to do a total urogenital mobilization or if you're going to have to do the urogenital separation. And again, remember the importance of the SIS and the ischiorectal fat pad to prevent uh Urovaginal fistula. Um, please refer again to these articles, uh, with any further reading that you may want to dive into. This is Amanda Jensen with Riley Children's. Remember, knowledge should be free. Until next time.
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