We've been doing this podcast now for about 18 months and we already have over 20,000 listens. And by far the most popular topic has been colorectal. Mark Levitt, one of our previous faculties, said he gets numerous emails about his podcast, but mostly relating to CloACA. And so since we haven't done a podcast on CLACA, we're doing that now. So today's podcast is what's new in the Management of CLACA with Doctor Mark Levitt from Nationwide Children's. Stay Current is a multimedia publication designed to keep healthcare professionals up to date with standards of care and new emerging ideas. Stay Current is created and edited by Todd Ponsky, Ian Glenn, and Sophia Abdulhai and is recorded and produced at Akron Children's Hospital in Akron, Ohio. Welcome to State Current in Pediatric Surgery. This is Todd Ponsy recording live from Akron Children's Hospital. And today we're going to be talking about what's new in the management of Cloaca. With us today we have not a stranger to stay current, Dr. Mark Levitt, who is the surgical director for the Center of Colorectal and Pelvic Reconstruction at Nationwide Children's Hospital and professor of surgery at the Ohio State University. Mark, thanks for joining us. Pleasure to be here again. Thanks, Todd. We also have a guest host with me, Dr. Aaron Garrison. One of my partners here at Akron Children's Hospital and head of our colorectal center. Aaron, thanks for joining us today. Thanks for the invite, Todd. Good to talk to you again, Dr. Levitt. It's actually really fun for me to have both of you on the phone here to talk about something that I know very little about because I have have not had many opportunities to work on Cloaca, and that's, I think, the same with a lot of people, and this is going to be a very helpful session. I know that you've worked with us on the Hirsch Brunk session in the past as also you've helped us with complications of anal rectum malformation. So thanks for joining us once again. You're welcome. All right, Mark, let's jump right into this. So let's talk about the prenatal evaluation and maybe even intervention in Cloaca. So let's say you're sent to your, to your office, a patient from the fetal treatment center. It's a mother that has a 22 week fetus that had an ultrasound and then an MRI that suggested cloaca. Can you tell me what do they see on ultrasound and then, and then who gets an MRI and when do you decide that you need to intervene? So yeah, so I'd say I would say the most common finding on the ultrasound was a pelvic mass. And often the perinatologist doesn't have much more delineation of it than that. Many pelvic masses, I think, get referred to MRI, but I'm certainly not the expert in telling you who deserves a fetal MRI. But there are some things about a fetus that should make the sonographer suspicious that they might have an anorectal malformation and specifically that they might have. A cloaca on their hands and a pelvic mass is key because that could be something big in the pelvis. Often they think it's the bladder, but in the cloaca, it's a dilated vagina, i.e., a whole hydrocopos. Sometimes they can actually see that there's a wall in the middle, so it's two cystic masses, obviously a bilateral hydrocopo. And in the presence of other anomalies associated with ARM such as a missing radius or an absent sacrum or a single kidney or hydronephrosis, and particularly if it's also a female and they know that. That's pretty clear that it's a diagnosis of a cloaca. And then intervention might be necessary. Unlikely, but maybe. What do you tell the mom? So I think this is a fairly common prenatal consultation, certainly in the colorectal world. Most colorectal problems are not diagnosed prenatally. Straightforward ARM and Hirschprung's are almost never diagnosed or even even suspected prenatally, but the possible. A conversation is one that happens. And basically you tell the mom that um there's a female fetus with a large pelvic mass. It could be a hydrocopos and the baby should just go to term. There's almost never a situation where you would need an intervention fetally, and then that baby needs to be managed in the newborn period with good imaging and a well-done colostomy by a good pediatric surgeon. With the knowledge of how to drain a hydrocopos if one is present and with excellent collaboration with urology to manage the high incidence of associated urologic problems, so you haven't had much experience of patients getting such severe. Obstructive neuropathy or hydronephrosis that they need to have early delivery or fetal intervention. Myself personally, none, no cases. I do know of a case report out of Japan where hydrocophus was drained, very similar to a bladder being drained for urethral valves and for similar indications, namely massive hydronephrosis with impending renal loss. That to me would be the only reason to intervene. On a hydrococos, and it has been done at least one time that I know about that was reported. So this this mom goes ahead and she delivers, and now the NICU calls you, the neonatal intensive care unit calls you and says that Dr. Levitt, we have a newborn when it looks like they have one opening. It looks like they may have a cloaca. How do you evaluate this patient? What do you look for? Uh, and then what are your next steps? So let's talk about how do you do the initial evaluation. Yeah, so that's a great question, and I think something all pediatric surgeons and all trainees need to be able to answer well because this happens a lot and the neonatal. And it often doesn't give you much more information other than there's no anal opening. Um, so when you come to the bedside, you have pretty good confidence whether you're dealing with a boy or a girl, and obviously in this circumstance you're dealing with a girl. And then I basically like to describe what do you have one hole, two holes, or 3 holes? And are the holes in the right place. In a cloaca, you have one hole. The hole is in the area just below the clitoris. And no other holes, and this is a very important distinction because if you have that same single hole underneath the clitoris, but yet you have a completely normal anus that is not a cloaca. That is what's called a urogenital sinus, which can be with or without. A virulizing component, meaning a hypertrophied clitoris would make you suspicious that there's an androgen effect, and those patients need to be evaluated for adrenal hyperplasia and making sure you don't have an urgent electrolyte abnormality. But let's say there's no anus. There's no anus at all, and there's one hole, that's a cloaca. That is not ambiguous genitalia. That is not in any way a eurogenital sinus with imperforrate anus. That's a crazy term that I've heard, but it's, it's a cloaca. There is no adrenal problem, and the baby is a normal female with two normal ovaries and will be hormonally normal. Um, you could have, uh, two holes. You can have, it's quite rare, but you could have a urethra and an anus, and the anus in the wrong place. That could be a, let's say a vestibular fistula, ARM, vestibular fistula with absent vagina. And of course, the most common ARM in a female is 3 holes, normal urethra, normal vagina. But the third hole is in the wrong place, either in the vestibule or in the perineum, i.e., a vestibular fistula or a perineal fistula. OK, so you look and examine this patient. Can you give me some tips on how best to examine a patient with a cloaca? Do you, do you pull out the labias to get a better look? How do you examine? You need very good lighting. You grab the labia. And you lift them up and out so that you can really accentuate the single hole. So you can peer in and see a, if in fact there's a single hole. When you do that, often you can see an obvious urethral orifice. And a hymen and a vagina and another hole in the vestibule that is not a cloaca, that's a vestibular fistula. There have been many patients that have been considered cloacas, but with a better exam, you would have been able to see three holes and know that. All you need to do is deal with the rectal component. And then, of course, Mother Nature hates our distinctions, so she puts things in the middle of the vestibular and cloaca that there's a little bit of depth to the cloaca, but the urethra is sort of visible. I would still consider that a clolaca, but obviously a lower type, and easier to fix type. The bottom line is you want to pull up and out on the labia and see, can you see distinct holes, or is it one hole and you have no idea where the other orifices are? You would need an endoscopy to know for sure. OK, so you do that. You don't see a. fistula. What do you order? What do you do next in your workup for this patient? So I think making a cloaca diagnosis is a clinical one. You don't really need much more information other than a good physical exam. And then the typical ARM evaluation to make, make sure that you've had a relatively quickly done ultrasound to See if there's hydronephrosis, see if you have two kidneys. Most importantly, do you have a hydroculpos associated with bilateral or unilateral hydronephrosis? We do a spinal ultrasound to see if there's a tethered cord. Obviously, you listen to the heart and make sure there's no murmur, and most centers routinely get an echocardiogram, um, although I'm not sure if that's absolutely vital, but most centers tend to do it. Um, I like to get a plain X-ray of the spine to make sure I'm not dealing with any hemi vertebrae and sort of get a sense of how good is the sacrum, and then you need to take a determination of when to operate on the baby. So if in fact it's a cloaca, they're going to need their stool diverted, and they're going to need their hydrocopos managed. And not necessarily operatively and only operatively or intervened on if there is hydronephrosis and of course if there's a hydrocopus on the ultrasound. So how do you decide if you're going to have interventional radiology drain the hydrocopulus or if it's something you'll address at the same time as the colostomy? Yeah. That's a great question, Erin. I think there is some value in passing a catheter into the single perineal orifice and see if you can decompress things. Sometimes you get a lot of urine out, the baby feels better, the abdomen is softer. The hydronephrosis actually can start to improve that way. And there are actually some centers that will do intermittent cath of the common channel. And if that works, then congratulations, that's a successful intervention without any operative fix on the hydrocopos. However, you need to realize that passing a catheter into the single perineal orifice might have it enter the. urethra might have it enter the right or the left vagina or might have it enter the rectum. And what you really need to do is make sure the hydrocopos or the bilateral component of the hydrocopose is decompressed at least daily. So intermittent cath of the common channel is not reliable. And if it works, I would actually confirm. It's working under ultrasound and actually make the the caregiver show you that they can pass the catheter through the common channel and confirm on ultrasound that they're actually decompressing the structure because as soon as you don't decompress the hydrocopose, it it refills with urine again and the hydronephrosis returns. So if you, if that's not successful, and it's usually not, I just drain the hydrocopos if present at the time of the colostomy creation. I don't really need interventional radiology because we're in the OR anyway. There are some circumstances where a colostomy was done. And hydrocopa was not properly managed or even recognized, and at that point I would probably use IR to help me decompress things. We've talked previously about making a proper colostomy for ARM patients, but can you give us some technical tips on placing a vaginostomy? What size tube do you use? Do you purse string it in? How do you, how do you secure it in place? Sure, so at the time of the colostomy creation, is an ideal time to drain the hydrocopos, and it's important to know whether it's bilateral or not, because if it is bilateral, then what you need to do is get to the dome of the vaginas, open into the vaginas, remove a little bit of this common wall, the septum. And then one tube will drain either side. And so I tend to bring the vaginostomy tube out the right lower quadrant if the colostomy is on the left side. If the hydrocorpus is particularly large, meaning above the umbilicus, you can actually sew it into the right lower quadrant like you would a G tube. You can leave a tube across that, and ultimately, you don't need a tube. The parents can intermittently cast that more often it drains well on its own. And that's really nice, but only if the hydrocorpus is quite large, and then, and then you get to avoid leaving an indwelling tube. If you're gonna leave a tube, my suggestion would be to use an 8 French or perhaps a 10 French. I like to use a pigtail catheter that I get from IR. The reason for that is if you give us, if you put it in a straight catheter, like a pesser or a Malanco, as the hydrocorpus recedes off of the abdominal wall as its inflammation goes away, You end up getting the tube to fall out, whereas a curled catheter doesn't fall out, and I've learned to do this, of course, the hard way because tubes had fallen out on me and I haven't had a curled tube fall out on me. It's nice to have that too because it drains the urine well. The hydrocohoseros, and then of course it's of value during the cloacogram. I want to go through a couple of other things. When would you need to do a vesicostomy? A vesicostomy is to drain the bladder, and the problem with the hydronephrosis in these patients is not the bladder usually. The problem usually is that the hydrocopos, which is behind the bladder, leans forward and pushes on the trigon where the ureters enter. Compressing the distal ureters. How do you get the distal ureters to drain? Not by doing a vesicostomy. In fact, if you do a vesicostomy, the hydronephrosis persists. If you drain the hydroculpos, there's nothing pressurizing the ureterral orifices. They can now drain into the bladder, and the bladder usually then can drain out the common channel or out the vaginostomy tube. Why the urine does not get out is a bit of a mystery because there's no actual obstruction. It's got to be a mechanical thing that it's easier to fill into the vagina than it is to exit the common channel in certain circumstances. Now, if you successfully drain the hydrocorpus. And the bladder still does not drain. i.e., the hydronephrosis ought to improve, but the bladder still does not drain, then that's the time to consider a suprapubic tube or a vesicostomy. That's exceedingly rare, but it happens when the common channel is very, very. And narrow and in very rare circumstances there's no urethra at all, so you have to get the bladder decompressed and it may not decompress adequately out the vaginostomy tube. And so how do you evaluate that? Do you get an ultrasound in a day or two? Yes, so I would follow the baby with ultrasound. The hydronephrosis is often a prenatal finding, so it's not going to resolve immediately. You just want to make sure that the baby is making good urine, is draining out the urine well, and that the hydronephrosis is not worsening. And then over several days, if not two weeks, then the hydronephrosis eventually recedes. So don't panic. As long as the hydronephrosis is stable and the baby's making good urine and all structures are being decompressed, you are doing well. If you have a situation that the hydronephrosis is massive and the bladder, most importantly, continues to be filled in every ultrasound you do, then the The bladder needs to be intervened on. It's very rare that you need to do that. Traditionally, if you asked urologists, they would tend to drain the bladder first, and many Claas I meet come to me with vesicostomies and have undrained hydrocopo. The one exception to this rule is if they have massive bilateral reflux. If they have massive bilateral ureteral reflux, Things, you must decompress that system and often a vesicostomy is a very safe way to get those ureters out of the equation until later, later in life. Again, a rare circumstance. Mark, why do you get hydroculpose? So that's a very good question. I think there are a couple components to it. I think the bladder tries to exit the get the urine out through the common channel and doesn't, it doesn't go, but there's obviously a fistula into the vagina, so it preferentially fills the vagina. So the vagina fills with urine. The vagina also has mucus in it, and of course there's the maternal estrogen that can fill or make that mucus more prevalent. And that's one of the reasons, by the way, some centers do intermittent cath of the cloaca, and then they say a couple of weeks later the hydroculus is gone. It may be because the estrogen effect has gone away. I haven't personally seen that, but some people have told me that, and I think that's a very reasonable thought. Actually seen hydrocopos patients with blood, blood inside the hydroculus, of course related to the estrogen effect. Most of the time it's clear, sort of a turbid kind of fluid which is a combination of mucus and urine. So if the vagina is filled with urine and mucus, I don't understand why it doesn't just drain out. It's a mystery to me too. I think it's, it's got to be a mechanical thing. There's, it's no, there's no obstruction usually. Obviously you can do a cystoscopy and get right in, but I'm sure you realize when you do cystoscopies on cloacas, it's very hard to get into the urethra. You almost have to put the scope on the on the floor and point to the ceiling. It takes quite a steep turn often to get up into the bladder. So if you think about why isn't it draining out, it makes sense. It's not draining out easily because the urethra is so far away from the perineum, and there's a beautiful pop-off valve goes right into the vaginal fistula, so the urine just goes into the vagina, fills, fills, fills, and then the vagina is huge and the vagina has nowhere to go. Presses forward, compressing the trigone which obstructs the distal ureters. Mm, OK. Let me see if I got this. You go to the operating room with this patient. You're gonna do a colostomy, maybe a vaginostomy, and I'm assuming you do a cystoscopy at the same time. No, I actually don't scope the patient in the newborn period. I don't think there's any advantage. I don't think there's any advantage to taking the time to do that. The cystoscope you need is tiny. Your visualization is not good. The perineum is swollen. Frankly, I like to minimize the amount of time I'm in the OR with a newborn, and I'm in and out. And one nice technique which the Michigan group described is to laparoscope that patient. And drain the hydrocopos and do the colostomy with great visualization from the laparoscopy. That's a very nice approach. The other approach is to do the traditional left lower quadrant incision that you would for ARMs. And if you're not, if you're not frequently using laparoscopy, and then deal with the hydrocopos at the same time through that same incision. And then the only one exception to that if you have a massive hydrocopos. That goes above the umbilicus, I will use a lower midline because it's very hard to get above the hydroculpos through a left lower quadrant oblique incision, and it's also hard to get a laparoscope in in such a patient. But if you make a lower midline, you get on top of the hydroculpos, and then you can do your opening of the of the common wall and The dome of the vagina and making your, um, making your colostomy. The nice thing feature about that actually is you don't need a skin bridge between the two stomas cause you just bring the 22 stomas out in the, um, in the in the lower quadrant and you don't need a bridge in between because you already have a midline incision, but that's the rare circumstance of when you have a very, very large hydrocopos. And by the way, in that case, as I mentioned earlier, I would do a tubeless vaginostomy. I would actually suture it to the abdominal wall like you would a G tube. OK, I want to also back up and say, let's say you saw the patient in the NICU and They did have an anus, but they had a urogenital sinus. What do you do with that patient? Yeah, so that's a patient that needs a, um, uh, workup to make sure that they don't have any electrolyte problems. They likely have an adrenal problem that caused the virulization. You can have your adrenal sinus without virulization, and that's a patient that doesn't obviously need anything done to the rectum. And UG sinus is managed very similarly. If the urine comes out, great. If it doesn't, you may need to drain it. Again. Often it's the hydrococcos that's the problem. It's if there's hydronephrosis, and then at some point in the future, the UG sinus can be managed. It's a technical challenge. Urologists are often the ones taking care of those patients. They may need some clitoral work to reduce the clitoral hypertrophy. And there are a number of nice techniques of getting a UG sinus to reach. Very few of them involve the rectum, but the particularly high UG sinus cases may require a transrectal approach, which urologists would describe as an astra approach. The term I've always used is a trans anal rectal approach, same concept, both prone position, but often most UG sinuses can be managed with just A perineal urogenital mobilization without touching the rectum at all. OK. The timing of that repair and also I guess the timing of the definitive repair for the cloaca. Yeah, so there's no rush on either of those. Obviously you need to get the baby out of the newborn period. I don't think there's a reason really to delay a cloacal repair. Typically we will, we would do a colostomy, plus or minus a vaginostomy, let the baby grow, do an endoscopy. Cystoscopy, vaginoscopy, and a cloacogram, all at the same setting at about 2 to 3 months of age, and then the cloacal repair anytime thereafter, um, certainly within a year. I mean, I love to do them even before 6 months if I can. If the patients referred to me, and it often takes a few months to get them through the whole process of the colostomy creation and the growing and all those kind of things, I try to get this, get the operation done before 1 year of age. If I take care of the newborn, I try to get it done by 6 months of age. You mentioned some of the imaging like a cloacogram. Can you talk a little bit more about that since all centers don't do that? What kind of information does it provide to you that you don't get from the cystoscopy? Yeah, so the, the cystoscopy, well, I mean, let's go through the endoscopy. I think that's really important because And this is really a change in my practice in the last 4 or 5 years that I look for different things endoscopically. When you, when you scope a patient, and again I avoid the newborn period because I want, you know, a bigger scope, better lighting, a healthier baby, etc. out of the newborn period, but I scoped the common channel and I really am looking through two very important measures. One is the length of the common channel. And that measure has been traditionally described in papers about Cloaca and how to plan for them, and the 3 centimeter or less is the straightforward Cloaca, and the more complicated cloaca is greater than 3 centimeters. But I think the other very, very important measure, which is not mentioned in any of those papers, including my own, um, um, which I'm going to really, which I've really focused on in the last few years, is the length of the urethra. Because from the perineum to the urethral takeoff is the common channel. But then there's a very important measure, and that's when the urethral takeoff into the bladder neck, because that will determine what operation you do, because you must leave the patient with an adequate length urethra, and we can go over the details of that when we talk about the surgical technique component. I also like to know if there is one or two vaginas and how reasonably reachable they are. And it's all, it's really nice to know if you see the rectal orifice, but I think the one major advantage of, of a contrast study at that point is you can be falsely lulled into the confidence that the rectum is reachable. And in fact, it's a very long narrow fistula and the healthy rectum is way in the abdomen. There's no way you'll know that on endoscopy. You need a contrast study to do that. If you have the ability to do a coaiogram, which is not hard because you can inject basically all the structures that you have, you have a distal colostrogram to inject. If you have a vaginostomy, that's your other injection. If you have a vesicostomy, we talked why you might not have a vesicostomy, but if you did, obviously you can inject that. If you only have a distalcholostogram to inject, distal colostomy to inject, that's helpful in and of itself to know how low is the rectum, just like you would for any ARM. But you would also inject the common channel, and if you inject the common channel, you can light up the urinary tract and the vagina. Often it's difficult to light up the urinary tract, so what we do is we do the endoscopy and we leave catheters in the bladder and The distal cholostogram and then one in the perineal orifice, and then while the patient is still under anesthesia, we have our radiologist in the interventional radiology suite inject those structures, spin the CR, and create a beautiful 3D cloacogram. If you don't have the ability to do that. You can still do straightforward fluoro. Most important image in my view is the lateral image. I want to see how low is the rectum, how reachable are the vagina is the vagina or the vaginas, and where is the urethra. I think you also at your center get 3D. Printing sometimes, is that right? Well, we've done that actually once. It's very, very cool. I'm not absolutely certain it's going to be the way to go with these, but we actually did a very interesting study where we had experienced surgeons look at a 2D cloacogram, a 3D cloacogram with the reconstruction I was talking about. A 3D printed cloacogram and then a virtual reality cloacogram. We had our mathematicians recreate so you could actually put on goggles and walk inside the Cloaca. And interestingly, the more complex the modality, the more correct the answers were on the different anatomy descriptions. So it's clear to me that 2D is not nearly as good as 3D. And it turns out that printed 3D, where you can hold it in your hand, is actually a very valuable modality. Now, is that practical? Not sure. We've done it once, but who knows? It might be a very valuable thing, um, because I think the, the brain sees things in a very visual way. And my ability, and I would compare this to your, Todd's ability to read CAT scan. When you learn how to read CAT scan, it was coronal. Now, probably like me, you often look at the sagittal reconstruction, sort of the AP view that they used to never give us when we were trainees, right? It's a different visualization of CAT scan. Well, we've gotten better probably looking at CAT scan because of that. So how do you speed up the learning curve for cloacograms? I think we've got to get more sophisticated imaging. And 3D is definitely better than 2D and printed might be better than 3D. I don't know. I've seen pictures of it and it's a very cool looking, so I'm curious to see how it progresses. Mark, let's finish up the discussion by talking about the actual repair. Talk to me about where these should be done, when they should be sent out, when not, and who's part of your team? I know you have a multidisciplinary team. Let's get into that first before we get into the specifics of how you do the repair. Well, you know, I think these patients very much benefit from the collaborative approach. We like to joke that it's a Cloaca by committee. I even have a slide of a committee room discussing a Cloaca case. Um, I, I think the days of a single surgeon being able to handle a case of this complexity are over. I am so thankful that I have urologists to help me and gynecologists to help me through. Uh, these coal repairs. The pediatric surgeon's role is obviously paramount with regard to the creation of the colostomy, but there is a lot of urology, management of hydronephrosis, um, management of renal function. The gynecologists in our team play a role even at that juncture. That's that's not absolutely vital. I know not everyone has access to pediatric gynecology of that, of that level, but They're, it's really nice that our gynecologists have learned about the newborn gynecologic anomalies. It's definitely helped them care for patients when the child is 12 years old and having trouble menstruating and certainly when they're 25 years old and considering having a baby. And so we get them involved up front, even in the newborn period, and the families are very grateful to talk to them about their gynecologic future. A lot of centers can't can't organize that. I totally understand that. So I think that most, most good general pediatric surgery teams can handle a cloaca. They can certainly do a nice colostomy. They can drain a hydrocopus if appropriate. They can do the endoscopy later in the next couple of months and get the data. And then they need to make a definitive decision about whether it's a case for them or a case to be referred out, and that has to do with the surgical planning. So with regard to surgical planning, I think if you are able to do your general mobilization. Which basically is mobilizing of the rectum in the traditional way you might for a vestibular fistula. And then doing a urogenital mobilization where the urethra and the vagina are brought down as a unit. That is a beautiful, elegant operation described for the first time in 1996. I still remember that I was a trainee standing behind Alberto Pena as he explained to Hardy Hendren this concept, this new concept he had. Oh, you're a general scientist and immobilization. He was drawing pictures. I remember like it was yesterday. It was a moment of history. That has been extremely valuable. Prior to that, the operation involved separation of the vagina from the common channel and repair of the common channel to become the urethra. And the problem with that is about 10% of patients got a urethral vaginal fistula, which was very difficult to fix. So the urogenital mobilization that does not operate on the wall between the urethra and vagina was a godsend because it stopped that problem from happening. However, I believe the application of the urogenital mobilization to most cloacas then has led us to other problems, and the problem has been You don't leave the patient with an adequate length urethra. And that's why I think it's so important to understand the length of the urethra before you attack this patient. So, if you have a patient that has a common channel of 3 centimeters or less, and above that urethral takeoff, you have at least 1.5 to 2 centimeters of urethra, when you bring that urogenital sinus down. And you split the common channel until you get to the urethral orifice, you now have a repaired cloaca with an adequate length urethra above. That is an operation that if you have experience with, if you have training in how to do it, by all means. Obviously if you do it a lot, it's a lot easier of a case, and if you don't do it a lot, it's much more technically demanding, but that is a beautiful case that I think a good, well trained general pediatric surgeon can do. If you have an inadequate length urethra, you do not want to do a total urogenital mobilization. You then want to leave the common channel alone to become the urethra, and then your job is to separate the vagina from the common channel. That is a technically demanding operation that is not easy to do. And once you've separated from the common channel, you then have to repair the common channel. And I like to put a fat pad, anorectal fat pad to cover it and even SIS if necessary to make sure you have a well-healed urethra and avoid the problematic urethral vaginal fistula. If you do a urogenital mobilization and you guessed wrong and the urogenital complex does not reach. You have only one choice at that point, and that's to go into the abdomen and deliver up the urogenital complex, hoping that that will gain you enough length to pull it through. However, that often doesn't work, and then you are in trouble because you have to then separate the vagina from the common channel, but the common channel has already been circumferentially dissected. And what we have seen is a number of these urethras get lost because they've been devascularized. And then you have a patient with no urethra who ultimately will need a metrofenoff. The alternative option is you've done a urinal mobilization and you've left the child with an inadequate length urethra, and now they leak, and there's no way to gain control of the leakage without tightening or closing the bladder neck. However, if you've made the right call and moved the vagina off the common channel and left the common channel to become the entire length of the urethra, you give the patient about a 4 centimeter urethra, and that's great. And they can do intermittent cath through that, and they can remain dry. So, in a case where you're able to visualize a, a urethra from the common channel, and it's maybe 1 centimeter visible, uh, the patient may have no neurogenic bladder, they're able to void. You basically just leave. That alone, leave the urethra alone and mobilize the vagina down with the PA. Oh, so you're describing a situation we haven't talked about yet, but that's a patient with a slightly hypostatic urethra, like a common channel of 1 centimeter with an adequate length urethra to the bladder neck. Yes, and I would call that a type 1 cloaca. And in that circumstance, all you need to do is mobilize the vagina, and you can leave the urethra a little bit hypostatic, and they will void. What you need to be sure of is they don't do vaginal voiding because then they could leak later after they've had a good void. And if they have a neurogenic component to their bladder, and many of them do, whether they have a tethered cord or not, but certainly if they have a tethered cord, they may have a neurogenic component to their bladder, you need a visible urethral orifice that's easily catheterized. If you know for sure that they're going to void and they won't need intermittent cath, then having a slightly hypostatic urethra is not a problem. All right, Mark, so what do you do if the vagina doesn't reach? What are your options? Yeah, so the scenario that you're describing is not uncommon in the higher cloaca. The scenario I'm mentioning here is when you decide to separate the vagina from the common channel, repair the common channel, make that the urethra. Now you have a vagina, a native vagina, and it doesn't reach. You even go into the abdomen, you fully mobilize it, and it doesn't reach. What are you gonna do? Well, often it reaches. That's good, good news. That's lucky. Um, and then you have a pull-through vagina after you've repaired the common channel. One option is to do a vaginal switch. Where you disconnect one of the sides, preserving the ovarian blood supply and switch down the vagina, so the dome comes down and you remove the septum. It's called a vaginal switch. Alternatively, you can leave both, um, Round ligaments intact, so you have good blood supply to the uterus. And the vagina needs to be bridged to the perineum, and you need to put something in between. Your options include uh rectum. Colon or small bowel. My personal preference is the colon. And actually the left colon in my view is ideal. Sometimes the sigmoid, depending on the arcade, where you bring out a segment of the colon and then sew that to the proximal vagina and then pull through the neovagina to sew it to the perineum. Of course, I hope one day soon we don't ever need to do a vaginal replacement. Because on the horizon, I think is tissue engineering of vaginas. There's already been a fair bit of work done on this, both in at Wake Forest in Mexico. Um, and the hope is that you could use a stem cell from the patient and grow yourself a 5 by 5 or 10 by 10 centimeter piece of vagina that you sew in and that will revolutionize the care of cloacas once it happens because then we won't ever have to do a vaginal replacement. But when you dive into a case like this of the complex type, and I would say that includes the greater 3 centimeter common channel patient or the patient with a very short urethra, meaning the urethra take off the bladder neck is less than 1.5 centimeters. Those are the very difficult cases that you really need to go into with expertise in urology and Vaginal replacements and be able to do those if one is necessary. So what is the one thing that you see most commonly when you're doing redo cloacass done here or internationally? Well, I would say the most common problem after a cloacal repair is the surgeon never realized they were dealing with a cloaca. So all they did was fix the rectum. And the patient comes with a repaired rectum, good or not, maybe it's in the wrong position, maybe it's prolapsed, and there's the urogenital sin that's sitting there, never touched. And that's a patient that needs the rectum immobilized out of the way, the uroginal sinus dealt with with the techniques already described, and then the rectal repair done. That's probably the most common. Second most common is they made an attempt at the collaal repair, but they did not adequately mobilize the structures, and they left them with left the patient with a stenosed or lost vagina. And that patient obviously needs a redo as well. All right, Dr. Levitt, that was very good. I can tell you that I think the summary here is, is that this is quite a complex condition, and it's great that people have someone like you who specializes in this. And, and if you could. Help us out here. Give us some, some contact information so that people listening to this would be able to reach you if they had a patient with with this condition. Oh, absolutely, I would love to help. A lot of times you just need a little, people just need a little guidance on planning, and they can get through cases like this with a little, with a better plan. Feel free to email me anytime, M A R C. Levitt L E V I T T at nationwide Children's, that's one word with an S at the end.org. Um, also, if you'd like, and we can, uh, post this to, um, uh, your site, uh, Todd and Aaron, um. If you go to www.ccpr.video and the password is CCPR, you can actually watch a whole bunch of our videos, uh, including a coacal repair. I'd also offer that if your radiologists are interested in learning how to do a coacogram, which is quite easy, email me and I will connect you to our terrific radiologist. Uh, Doctor Greg Bates has perfected the, uh, technique in our institution. And we're happy to share how we do this because it's really um if you have a floral suite, if you have an interventional radiology suite, you can definitely do a cool echogram. That, that's gonna be very helpful. I know that from previous podcasts that we've done, uh you've received quite a few uh questions and and um. And comments. So we definitely encourage that. Also, if you're listening to this on the Stay Current in pediatric surgery app, the Stay Current in Surgery app, which you can download from the App Store, both in the Google Play or the iPhone store, you can watch Dr. Levitt's videos which we'll post and as well we'll also have his email address listed there. And a summary of what he's said today. Mark, as always, we love having you on, uh, as a guest on this podcasts, and uh we hope to hear from you more in the future. Great. Love doing it and uh ask me anytime. We hope you enjoyed this episode of Stay Current in Pediatric Surgery. You can listen, watch, or read all content by downloading the Stay Current in Surgery app. Please send questions or comments to us attacurrent podcast@gmail.com. We'll see you next time.
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