OK, so here we go. Here's a baby. We talked a bit a little bit about this diagnosis. Urethra, vagina, hole. Sphincter diagnosis? OK. A, perineal fistula, B, vestibular fistula. A, perineal fistula, B, vestibular fistula. Normal urethra, normal vagina. Whole Great. Everyone agree? OK. This is what Eva was talking about before. This is a really nice technique is to hold these labia up and out. You can see urethra, vagina. Who A perineal fistula, B vestibular fistula. Thank you. Experts now. I'm sure they're gonna get this right, right, Vicky? OK. All right, same idea, same idea. OK, how about this one? Right right at the Paula, you have an opinion? It's right at the ochette. OK, so that's urethra and vagina. Yeah. And the hole is as Paula's describing it, the forchette. Yeah, so. So, is this, I guess this is perineal at the forchette. Yes. OK. vestibular at the is when Mother Nature is up there chuckling at us when we try to make these distinctions. All right, a little bit about the technique. This is in in prone position, stitches around the fistula. Mobilization Now, let's talk about this plane here. How do you know you've mobilized enough, Don? This is a, this is a uh perineal Forchet fistula, prone position. How do you know enough is enough? Well, I don't care if I get it completely off the vagina, OK? That's not my goal. My goal is just to mobilize it enough to get it to reach the perineal skin with a little bit of tension. So, my answer is, when I pull down on it and it reaches the perineal skin with a little bit of tension, I quit. Eva, you agree? Not completely. I, but I, I can imagine, but um. I'd like to have it completely separated from the vagina because you'll find there's a. It's always very attached to it. It's like the common wall. If you finally get it loose, you can place the tension free, and I have the feeling, but it's a feeling that if you don't do that, you may get more retraction and wound problems if you don't really get it free from the vagina. So we've had the discussion before as well. I think a lot of retracted anoplasties are Related to not getting it loose enough and you sometimes do a redo, you find that this plane, this plane which separates it hasn't been touched before. So my feeling is you have to get enough to separate it. The downside is a little bit that you lose more you lose more of your internal sphincter or your rudimentary internal sphincter tissue or you will lose some of it. But I, I, my feeling is, is more not to get any wound problems and. So I, I tend to go until it's completely loose from the vagina. Yeah, me too, Michael. Uh, I, I agree, uh, with, uh, a little bit more mobilization, um, so that, uh, you can't bring it, bring the, uh, rectum down without tension, um. The other thing is, even though it seems like such a straightforward case and, and it can be, um, you need to, to be careful because if you are slightly, um, too far away from the rectal wall, you will end up in the vagina because even though there's a separate plane there, um, you know, uh, compared to the, the stibulars where it's more of a common wall. It's really, you could, you could, uh, get into the posterior vagina easily. OK, so now that you find that completely clear what to do, your current practice is A, mobilize just enough so it reaches, or B, ensure a complete separation of rectum and vagina. It's like. A mobilize just enough so it reaches B, ensure a complete separation of rectum and vagina, knowing you're gonna have to throw out a little bit. All right, so we will see this afternoon that these little details in the case. My personal feeling is, and I, I acknowledge I'm very biased on this topic because we do so many redos of females. And in every single redo of a female, I find that perineal, I'm sorry, that areolar tissue that had never been dissected by the original surgeon. We know it. We say, see, no one, no one was ever here. And so, my conclusion from that, and I may be wrong, my conclusion from that is the redo, uh, that the reason for the redo was the perineal body disrupted, and the patient's left with no perineal body. That somehow the anterior rectal wall was not free enough and it pulled back. So that's why I'm very biased to try to get to that areolar tissue that shows that the two structures are separated. I'm sure what Don is saying is right, and you can do that and get a beautiful anoplasty, but if you have any tension on that anterior wall, you can disrupt your, your perineal body. You want to comment? I'm, I'm just listening to what everybody is saying. I don't recall having those perineal complications, so maybe I'm mobilizing it more than what I'm describing. But my also my other comment was going to be. How many of those redos that you've done were done sort of in a newborn period without a backup colostomy, without a colostomy, because a lot of the newborn female vestibular fistulas that I've had to redo were done without a colostomy. Yeah, I think that's an important point, but many, many have been done with colostomy. Every, every, all conditions perfect, but the surgeon did not dissect the anterior wall to the point where you get to that areolar plane. And then again, on the other side, every single female redo, we find that plane that had never been dissected. So I believe that that is an important technical point. Um, all right, and the attacking once again, and there's the anoplasty. OK. All right, so this picture is um. An interesting one. Jonathan, I think you alluded to this before, urethra and vagina are normal here. So, what is this? We talked previously about an entity called the common perineal groove, and this seems to be in association with an anorectal malformation per se, i.e. it looks like there's a stenosis. So looking at the two things separately, I, I, I wouldn't, I haven't found patients becoming symptomatic from a common pineal groove. But let's, let's ask, let's ask everyone. I will tell you there's a normal urethra and normal vagina here. That hole is in the center of the sphincter, and it accepts a number 1112, Hagar easily. In a neonate, is that like, huh, in a neonate. Neonate, neonate. OK. So, A. Surgery, be observation only. Do Because they're in the group and eventually people are. After the first year relationship. But I also OK, would any of the 12.7% of you want to comment on surgery? Uh, Doctor Teitelbaum. By the way, just so you know, I, the fellows are doing very well with their answers. There are 2 of you that will not be able to participate in the afternoon session, and we have booked your, uh, we have booked your flights. Connie will let you know the new arrangements, OK? So I, I throw this out to the panel about this mucus and if people observed, if you don't. If you years later, do you find any problems if this is left alone? So again, normal urethra, normal vagina, anus in the well in the proper location and good size, and there's this thing, this mucosal lined channel between vagina and anus called a perineal groove. Jonathan, the only problem that I've seen is in an older child that was referred because it caused confusion. In the GPs' minds about whether or not this was a standard fissure in a. Um, so once it's got a name and a label, um, for me, um, I've always been treating them just based on symptoms. My colleague Ian Sugarman has had a patient who was troubled because it produced a fair amount of fluid, and the procedure that he did was just to excise the mucosa and just close it, and it was very straightforward. Yeah, I think the vast majority of them, if they're observed, will become normal skin over time. Yeah, yeah. And the ones that don't, it's a very simple fix to just unroof the mucosa and suture it up if it's causing mucus production. But it's, it's an interesting entity, and it's often associated with a perineal fistula. So then if you're going to fix the anus for the perineal fistula, you might as well just unroof the, or get rid of the mucosa. Question. Doctor Downey, how are you? Thanks for coming. Isn't this just uh a picture of embryology? I mean, if you think about the the analogous structures in the male, this is just an opened up. If you will, form fresh fistula that goes up to the scrotum. Yeah, maybe. Like you would. I mean, that's what it's, I've always thought of it. Yeah. Conceptually as being, that's. It's clearly mucosa. So, you just leave it alone and it. Yeah. It'd be fine. Uh, you just leave it alone. Well, I also make a point of just going through the clinical aspects of B1 with those just because I think it is part of the spectrum. I wouldn't submit them to a lot of radiation, but I'd listen to the heart, etc. OK. A perineal fistula, B, vestibular fistula. It's a test to understanding. It's a test. A perineal fistula, B, vestibular fistula. Right. And then the mobilization of the, and we'll talk about this in the OR so we don't have to spend a lot of time, uh, today, but dissecting the posterior rectal wall and then the lateral edges before going anterior. And here's a nice separation of rectum from vagina with no holes in the vagina. So hopefully we can achieve that today, but. If there is a hole today, it's for instructive purposes, of course, of course. If you have to make a hole, which would you choose, vagina or rectum, Evo. Don't ask the gynecologist, but the vaginal opening in the vagina, vagina, yeah, so it's, it, and why is that? What is the technical reason for your much rather making a hole on the vaginal side than on the rectal side? Well, it heals, it heals very well, and you don't, you don't really get any much complications, and I would also add that the rectal blood supply is intramural. So the last thing you want to do is injure injure the rectal wall because then you're hurting its blood supply. Any tricks, uh, Michael, for making this a beautiful, uninjured posterior vaginal plane? Well, I think, uh, you have to do. Coming and starting laterally is really the key, I think. Um, and you really wanted to find that lateral plane before you even attempt to start separating, uh, or creating two structures out of one common wall there anteriorly. Um, the lateral defines the anterior. I think that's a very important guiding principle. And then here's the back to this discussion. Is this too much dissection or is this the appropriate amount of dissection? So we sort of talked about this already. I personally like to go to the areolar plane that exists here, knowing that I'm, I'm going to have to throw away about this much rectum, but I'm willing to accept that because these patients are going to be continent on the grounds of having normal skeletal muscle, Mark. So could you go back just one another comment or follow up to what Michael was just saying is that coming in from lateral to anterior is great, but also the more proximal you are, the easier they are to separate. So if you start right down at the perineum, they're going to be a little bit harder to separate than if you get a little bit higher up and then come from proximal to distal, and, and that's, um, I, I, I think that that's a really nice place to find the right plane. Is on the is on the posterior wall. You lift up that fascia and then you come around lateral. So, I think that's a very good point. So, I have a question. Do you do this primarily without a colostomy? Yes, we, yes, I talked about that earlier. I personally would do a vestibular repair primary without a colostomy, either newborn or a repair in the next 3 or 4 months, depending on the child's condition, but I can't, uh, criticize someone who wants to have a diversion in place. My advice, if you want to. Try to do this primarily. You may want to do it primarily with a diversion at the same time and then a colostomy closure. I don't think these patients need a colostomy in the newborn period and then a repair and then a colostomy closure. And if you do it primarily, how long do you put the patients on NPO, I mean. How long that's a good question. All right, let's poll the audience for that one. So, if we do a primary repair with no colostomy, vestibular fistula. Let's do it both ways. Newborn, newborn vestibular no colostomy, who feeds the patient in 4 days or less? Who waits greater than 4 days? A is 4 days or less food. B is 5 or more days. So, if, if it's a nice wound, otherwise you go longer. I can't do it. Vicky, are you keeping up? Right. Look at that. You do the. So, my personal practice is I wait, um, until the perineal body is healed, which usually is around day 6 or 7. Yeah. And I think that many people think that's radical. And again, I admit that is based on my bias of having to do a lot of redos, and in large part the redos are in patients that were fed early. And again, there's no science behind what I'm saying, and I admit that, but I believe if you are watching the perineal body carefully, you have a way to intervene without a dehiscence. You can actually take the patient back to the operating room on day 6 or 7 and re-suture the perineal body. As opposed to a patient who was fed, was sent home, and then no one's looking, they come to clinic at 3 or 4 weeks and the perineal body has fallen apart. I, I, uh, uh, I know this is a bit of a controversy, by the way, in many parts of the world that insist on never keeping people NPO because they don't have Hyper-al, what I've tended to use is 10% dextrose, which they do have, and only use Hyperal if there's been more than 7 days NPO. And when I started to do that a few years ago, a lot of my colleagues around the world were very happy because it meant that they could try an NPO period because they have D10, they just don't have hyper. I wouldn't do that in under one or in a not so greatly nourished child, but in a healthy, robust kid, I would definitely do that. So this is a bit of a controversy, but any, and there's a comment from Todd actually a comment from me. I just throughout the whole morning here we've heard so many things. That you have a bias on that someone else has a bias on, and, um, you know, how much dissection for the laparoscopy, minimal or just pull it through the cutback, the, when to feed and we keep saying there's no science, it's your experiences, it's what you've seen, but you are a referral center. Why not start doing prospective randomized trials or are you so convinced by your, um, anecdotes that you don't even feel comfortable doing a prospective trial? Uh, I think, I think there are a number of opportunities for a prospective trial, but there are a lot of situations where you really can't do that. I don't know, maybe only you could because you have enough cases to do it. No, what we, we recently did, we had an, uh, an, uh, who had an abstract in the episode last year, uh, we did a systematic review on the perioperative nutrition. And if you look at literature, it's all retrospective, but actually giving early enteral nutrition seems to be better than later nutrition, just as in adult surgery has been proven as well. The problem is they're all retrospective studies. If you look at you score them on quality, they're all poor quality. So what we need, maybe not need is just a prospective study, but a good follow-up study, good follow-up cohort studies on the outcome, is the first thing we should do. The second thing is, are you willing to To change good results you need a good power analysis to do it and this will have to be a very large multi-center trial so the evidence is very difficult to get, but I think you could start with a good Perspective study and most studies are all and there's about 1000 patients have been studied, but they're all retrospective and bad quality studies. I completely agree and I just have always thought when I look at Mark and I see him as an example of a place that's a referral center, it's so difficult to get good data in pediatric surgery and there are a few places where we actually have a center that has enough patients to actually get to it, but that's going to mean that the person there might have to do what they're not comfortable doing. In a prospective randomized trial, so I, I, I, I, I wanna respond to that and then I'm gonna have my colleague Kate Deans who's really, uh, with Pete, the expert in this area, and I can tell you one of the major deficiencies I felt in my previous practice was this exact detail because I, um, as uh one of my professors like to say, the, the, the plural of anecdote is not data. Right. So, um, we as surgeons deal with anecdote a lot and sometimes we have a lot of anecdote and then we get biased. Um, one of the cool things about getting together like this is, and I know it's frustrating, but people have to find their own way sometimes. There aren't protocols for everything. Um, even appendicitis now, the protocol is changing, right? So, as a surgeon, you have to have a feeling and you have to do it the way you're used to and and feel like you're giving your patient a good result. But one of the clear deficiencies, in my opinion, in colorectal was exactly this point. And one of the reasons why I've come to try to collaborate on this exact topic is for, is to do better in this, in this area because I think, I think the technique is pretty clear. We know how to do it well, we know how to not do it well. Now the question is, which, which parts have some variability and which is better for which scientific reason. So with that intro, maybe Kate could make a comment. Um, that's a great question. I think your observation was similar to mine that we're talking a lot about anecdotal things, and we've seen from audience response and through Globalcast that we have a lot of variability in what we do, what we recommend, how we screen all of those things. Unfortunately, like all things in pediatric surgery, we're talking about rarity. Diseases and rare diseases are very difficult to study in clinical trials, because the more institutions you introduce, the more variability you introduce, the harder the statistical approach becomes. So there is, um, a concept which is about 10 years old, which we're going to be talking about tomorrow in the lunch session, which is called a rapid learning healthcare system. And it allows you to continually accrue your experience and perform statistical modeling rapidly so you get real-time point of care results to the patient who's right in front of you to answer some of their questions about prediction or a choice of two procedures or two approaches. So we're gonna have a little more conversation about that tomorrow. Mark, could you just comment on your primary, uh, repairs valid diversion, your, uh, bowel prep? Sure, yes. So, um, in the newborn period, we don't, uh, bowel prep. I don't bowel prep them, but if they're delayed repair, I do a full, uh, go lightly, um, bowel prep until the effluence is so clear that the resident can drink it. Um, I, I'm, I'm just kidding, we don't make our residents do that, um, but, uh, um, and then, um, plus or minus oral antibiotics. I know, uh, lately it's been shown that oral antibiotics may be, uh, uh, of an advantage, and that is our, we have a sort of bowel prep routine that all bowel preps are done the same way, so we actually do that here. I'm not convinced that that is absolutely vital, but we definitely, we do it. Um, and the patient comes in the day before. They come to surgery. So, this baby for today, for example, uh, had a full bowel prep last night, um, and is going to get surgery today. We'll get a PICC line. Um, we'll be on, um, because it's a baby, will be on Hyperal, and in 7 days, so next, uh, Tuesday afternoon, we will, we will take a very careful look at the perineum. And if the perineal body is socked in and, Good healing. The baby will be fed and discharged, which is the vast, vast majority of the situation. And on occasion we start to see that the perineal body is a little separated, maybe once or twice a year this happens, and then we take the patient back to the operating room and put some reinforcing. Perineal body stitches and of course and this hasn't happened to me but if the perineal body falls apart then they need to be diverted and I think that technique has avoided lots and lots of diversions that probably would have would have been needed in the past. The alternative approach is to. Do a sub, a different bowel prep than that or no bowel prep at all. Feed the patient early and send them home. And I think a number of those patients, and again I have no data to support this other than the anecdote of seeing redos. A lot of those patients are falling apart at home and no one knows because no one looks and then whenever they come back to clinic, perineal body is gone or 3 months later perineal body's gone or no one ever notices and then they can't potty train at age 4 and in fact there's no perineal body. So this this technique has come out of of learning how to do it to try to try to improve that result. During, during the 7 days NPO, do you keep a urethral catheter? A what? Uthra. Oh, no, yeah, I don't use a Foley at all. Uh, um, uh, for those in the audience, so vestibular repair, Foley A, no Foley B, or female perineal for that matter. Always use a Foley in the case. Never use a Foley. I, I keep it. I keep it, it's a style, but it's still moist. split. Jonathan, do you use a Foley catheter? I'm afraid I do. Me too. There's there's nothing wrong, nothing wrong with it. I just don't think, I don't think urine leaking on the perineum is a big deal, but there's nothing wrong with it. I think, the, uh, the bias or the prejudice that that that I've had comes from. The idea of keeping um alkaline urine away from the wound, um, I just don't like the idea of that on there. I don't know what BXO exactly is, but I don't want the idea of, of having a fresh wound being bathed in something, it could go either way. I personally don't, uh, don't, don't use a Foley. All right, just a reminder that a certain percentage, about maybe 2 to 5% of vestibular fistulas have a vaginal septum. So you need to look. I honestly don't believe you need a formal vaginoscopy, but you need to at least look. You need to look into the introitis, spread with a mosquito or forceps, and prove that there's no septum, because the ideal time to take care of that septum is when the rectum is being mobilized out of the way and the perineal body is open. Many patients have come back years later and the septum was never noticed or touched. And then you have to do a a removal of the septum later, later in life. Jack has a comment. There's nothing wrong with that, by the way. You can not touch the septum and then say, please come back after puberty, but you don't want to leave the septum untouched because then this girl will have issues with uh tampons and of course intercourse. Jack. Well, I, I guess my, it's not so much a comment as a question, but our, our gynecologists don't think a septum is a problem. And they, they tell us you don't have to do anything about it. So maybe I can get a comment from, uh, you know what, why don't we hold that because Jerry, Jerry is not here, but she is coming back for the noon session. Oh, is Jerry here? Oh, because you said you were stepping out. You stepped back. OK, we'll get you a microphone. OK, let's keep, let's keep moving, but, um, let's make sure to talk about, well, answer it, answer it now. Because it's. Everyone's thinking about it. If it's. And while she's, uh, taking her to the microphone, you don't think, uh, formal vaginoscopy, uh, is, It's, uh, necessary. You think if there was uh Didelphus you could just see that I think if you look into the perineum and Jerry Pierce, your comment about this too, if you look into the, into the introitus, if you see a septum. There's two hemi vaginas. I think at that point you could take out the vaginal scope and see, do you see two normal cervices? Are there, is there mucus at both of them, etc. But if you, but I don't think every single girl needs to be scoped, is my point. Um, because most of them are normal, but if you look in and you see a septum, then you need to recognize that there may be some Eulerian anomaly. Jerry, so the reason that you might, so I would argue that I would give the opportunity to take a, take advantage of getting rid of a longitudinal septum. The reason being, oftentimes, you know, these women go undiagnosed, and we see a lot of adult women that oftentimes they are asymptomatic with intercourse, and the women kind of learn the septums and they kind of use one side of the vagina more often than the other one, but oftentimes they're, they're, they're not really bothered with. Intercourse, which is probably why your colleagues are saying you don't have to worry about it. For delivery, if they have a longitudinal vaginal septum, we'll let them labor, and oftentimes they either blow the septum out during labor, which is OK. We resected the time, we repair everything else. It's oftentimes relatively avascular. But the problem is, and women work around this, is tampons. So oftentimes a kid will come in and say, I can't use tampons, or women will say I had to put a tampon in each side, or they'll say I have to use a tampon, but I need a pad. So menstrual hygiene is a huge reason to actually remove it. In my experience with adults that have septums, you know, they're not too terribly bothered with intercourse, and we'll let them labor. Hm Could you comment on, uh, resecting that septum in an adolescent girl if you, if you do decide to resect it, um, you know, later down the road, uh, how involved is that operation? You know, it's, it's, it's not, it's not hard at all. Yeah, yeah, yes, you can, uh, use anything that has power in it, so, you know, electricity, and you want to get up as much as you can as close to your cervix as you can without damaging your cervix. So the question internationally, so given that, what is the role of vaginoscopy? Um, I, I think, uh, in, in a female perineal or female vestibular, you're obligated to look into the introitus visually with a little spreading of an instrument. Um, and if it's normal, it's normal. Um, if it's abnormal and there's a septum, and maybe Jerry can comment, I think at that point it's very reasonable to define the septum, define the cervix or cervixes, maybe take out the scope at that point. No, I, I agree. If you have any suspicion in your mind that there might be a septum there, I think it's, it's imperative that the entire length of the vagina is evaluated as well as the presence of cervix or cervix. I will not, uh, today's case is not scheduled for vaginoscopy plus PSA, but I will be looking at the introitis and I will show that to you, hopefully, um, and to see that there's anything there. If we see a septum, we'll take out the vaginoscope, but 97% of these patients are normal. The only point is I think you have to look because you don't want to miss this. You don't want to ignore the possibility that this could be there. The other, um, now, here's a, here's an interesting one. Normal urethra, normal vagina. What is this? A vestibular fistula. B vaginal fistula. What's A vestibular fistula. B, rectal vaginal fistula. And it looks good behind me. This is a, this is a rare picture. You will not see many pictures like this, but I, I, I, Jerry, you would agree that this is within the introitus. This is not a vestibule problem. So what everyone agreed, so this is a, a true vaginal fistula, which is very much like a vestibular but just probably a little bit more mobilization of the rectum. But we don't like the term rectal vaginal fistula because most of them are recto vestibular fistula, but occasionally you may find one like this. OK, we talked a little bit about this before, urethra. Vestibular fistula. Nothing vagina nothing. Nothing. Right? So this is a vestibular fistula with absent vagina. How about this one? Urethra. Vestibular fistula. Nada. Vestibular fistula with absent vagina, right? OK. Another example. So many, many of these patients have had rectal repairs and the vagina was ignored. And then later in life, there was, uh, so Don, how would you manage the situation if you uh in fact noticed this to be the problem? So, you're going to ask me to go out on a limb again, but I'll tell you what I do. So, the first thing is I think it's really, really, really important to make this diagnosis in a newborn. So every time a newborn is born with imperforated anus, I go to the bedside with the fellows in Q-tips and or the residents in this case, and, and look to make sure there's a vagina there. But assuming there's no vagina, then my preferred method of repair is to use sigmoid colon as a vaginoplasty. So this would be a case where I might delay the repair, dilate the rectal fistula. And not do a colostomy. Cause if you do a sigmoid colostomy, you are potentially interfering with your blood supply that you would then use to mobilize a sigmoid vaginal graft to do a sigmoid vaginoplasty. And then I would do the repair a little bit older, laparoscopically. In fact, Michael and I did one of these, um, together. And you can use, uh, laparoscopy to harvest a piece of sigmoid and bring it down to the perineum and do the perineal repair all in one stage. So, uh, repair the imperfored anus and do a sigmoid vaginoplasty, um, and then do a backup colostomy. So, um, there are a couple of really interesting features about this anomaly. Um, first of all, the technical, um, um, Don gave one of the options, and that is to do a sigmoid neovagina and use the rectum as rectum, as you would normally do a vestibular or fistula repair. Uh, the alternative approach was to, um, use the, um, rectum as vagina. And immobilize a more proximal portion of the rectum down to be the neo rectum. The downside to that is you're not using rectum as rectum, and the rectum has value for its continence. So I would only do that if I knew that the patient was unlikely to be continent, like they had a spinal anomaly or an absent sacrum. Otherwise I would do exactly as Don described. The other thing we found in this group, and maybe Shammael can get a microphone, is it's a unique group of patients from a urologic point of view. So we presented this back, I think it was, uh, 2009. Of the 33 patients that we had in our series who had, um, absent vagina, 75% of them had urologic problems, including neurogenic bladder. Of that cohort, 50% of them had grade CKD stage 3 or greater. So, solitary kidneys, reflux, hydronephrosis, neurogenic bladder, urinary tract infections are all long-term sequela that have been observed. In this cohort of patients. So, once you make the diagnosis of absent vagina, um, a pretty aggressive screening of the urinary tract has to also be employed. So I would add this to the group of patients to be very nervous about and make sure you have urology collaboration. I would say that the male ARM with any associated urologic problem gets a urologic collaboration. Obviously cloaca, and then vestibular fistula with absent vagina is an important category because a large percentage of those patients had serious urologic things going on with them. Um, this is some technical points here. This is a Patient like that where the urethra is normal, the rectum separates actually very nicely from the urethra. It's a very thick, fibrous type of tissue. It's not nearly as adherent as a rectum is to the posterior vagina, which then allows you to have this space. To then bring through, as Don suggested, your neo vagina. Excuse me, what is the age of surgery in these patients? So what is the age of surgery? So I can tell you if we had a room filled with gynecologists, they would say teenager. But as a pediatric surgeon, well, let's put it this way, what anyone have an opinion about age or vaginal reconstruction? Paula, you have an opinion? Is, is different from us to the urologists and gynecologists. We, we, we tend to do it at the time of the rectal, uh, correction. Yes. So, I think that's the, that is the key point is, um, gynecologists often deal with vaginal anomalies alone, and the rectum and the urethra is fine. So, the timing of that is, is, can we, we can discuss the timing of a Roatansky neovagina. But in the patients that we deal with, we have to fix the rectum. In my opinion, the ideal time to fix the vagina is when you fix the rectum, because you have an open perineal body. It's a great opportunity. I also think from a technical point of view, it's easier when they're younger because that pedicle, this pedicle reaches much easier when the child is young and doesn't have as long a pelvis. So I'd much rather do a neovagina in a younger, uh, in a younger child. This is, this is that neovagina sutured behind the normal urethra, and there's a nice video of that that maybe we can show if there's a gap between, just so you know, there's a file here called Key Videos, and there's a nice video of of this case. There's urethra, neov vagina and anus. All right, here's an interesting patient that had a Um, absent vagina, but normal urethra and normal rectum. And there was this this was a slightly older child filled with blood, and what we did here, normal anus, normal urethra, and through the perineum, and this can also be done laparoscopically, but this one was so close that we were able to open it perineally and make a neovagina. And there's the menstrual blood, and there's the neovagina. So this will, you will see two normal urethra, normal anus, distal vaginal atresia. And there's your vaginoplasty. All right, so, um, A, a little bit about reoperations. How's this? She needs. How did the surgeon do here? Looks like that's probably, uh, Uh, no problem. Uh, abnormally located and, uh, may have even had postoperative wound infection there. Looks a little anterior. Yeah, so this is that what we were talking about. This is the urethra, the vagina, and then the dehist uh perineal body. Many, many examples of this, I'm gonna skip through. This is what a cutback looks like, but a cutback in a vestibular. So this does not leave a proper cosmetic result. And here is a anoplasty, but the vaginal septum was ignored. Nothing wrong with that. You just have to go in now and uh remove it. Jerry, do you have a comment as far as timing? Let's say we have this situation. And the child's 6. Would you do it now or would you wait until puberty? I mean, you could. You, you caught me talking with Kate about another clinical situation. So here, so here's an anorectal malformation patient. Anus was repaired. Vaginal septum was not diagnosed, and you meet the child at age 6. Yeah, so I don't think unless she's going to sleep for another operation, I don't think that you would need to take her just for this individual procedure to the operating room to fix it. Um, but I think if she's going to asleep for another operation that and you were gonna take advantage of her being asleep already, that'd be reasonable. But prior to that, I don't think there's any rush if you've already sort of missed your window of her primary repair. Um, I don't think there's any rush when she's premonaral. Well you would do it at puberty, at puberty. Only sooner if she was going to be having some other procedure done. Can I, can I just ask something, is the primary determinant. Of whether or not this is a vaginal septum or a double vagina is the primary determinant number of services in terms of your differential diagnosis, meaning, do you, so right, so she could have just a longitudinal vaginal septum with one cervix on top of it, or she could have two, so that's why I think we talked about doing the vaginoscopy to identify what's at the top of the vagina, whether you see one or two cervixs is so important. Um, but they can't have an isolated longitudinal vaginal septum with one malarian system. So we knew, we know that fish have cloacass, but we weren't sure about sharks because they have, um, live births. So we sent Rama to Florida recently. Is Rama here? They here so your um, your assessment was they have Cloacas, right? OK. All right. And then the other thing we learned recently, what about whales, Vicky, whales. Chloea or no? Anyone? All right, whales have a cloaca. B, whales do not have a cloaca. They shouldn't because A, whales have a cloaca. B, whales do not have a cloaca. They shouldn't maybe. What else we don't know. Can, can we send someone? Interesting. Who in person are gonna send Chris back to do this investigation, right? All right, Vicky, what are people, oh, look at that. Vicky, what's the answer? They do not have a cloaca. That's correct. So they're mammals. See how much knowledge you're getting. OK, see you. So cloaca, single perineal orifice, and we'll end with this, um, and by the way, can you just, what is the, Karen, do you know the lunch is going to be outside as a box and you come back inside. So we need a little time to, to do that, um, um, and then we'll set up for the urology and gynecology portion while you're eating. So I think it's really important to distinguish that there really are two different types of cloacas, the lower ones and the really complicated ones, um, and on the left side is a, uh, artistic diagram of a lower 13 centimeters or less, and on the right side is a picture of the higher 13 centimeter common channel or greater, and I think if you make that distinction you're gonna avoid a lot of trouble. Obviously, hydroculpos is a concern, um, and it may in fact um obstruct the distal ureters and cause bilateral hydronephrosis. And then about 50% of cloacas have this BID uh gynecologic system. So here is a newborn, so maybe Michael could describe. How you would manage this newborn female with a cloaca and abdominal mass. So, um, it's most likely a hydroculuss. Um, I, um, Would, uh, do an open, uh, divided colostomy in this particular, uh, patient, and, uh, at the time, I would decompress that vagina with a, uh, pigtail catheter, rather than creating a formal vaginostomy. Um, The, uh, uh, sometimes the, uh, doing a colostomy in these patients can be challenging because that's so dilated. Uh, so, occasionally I have to decompress the vagina, uh, first. And the type of. One mistake, uh, I have made a mistake before trying to, trying to do cystoscopy, vagina, or not, you know, to do, uh, cystoscopy at the time of creating the colostomy, um, in a patient with cloloica and that, uh, makes your, Uh, colostomy creation, uh, very difficult as well. Uh, yeah, I, I, I don't know, uh, if others in the audience have had this experience, but my personal recommendation is to just divert the patient and deal with the vaginostomy and don't worry about scoping them at that time. You have an opportunity to do that at 2 or 3 months of age. It's much more pleasant. You'll get a better, better visualization. There's no rush to scope the vagina at that time. It's important to. What incision? So, um. OK, well, who would do a, uh, a, a left lower quadrant oblique incision? And B, a transverse incision. On a transverse or midline transverse or midline. Well. And I'm curious to know how many would approach this laparoscopically. So it's the way that you going to use it later. So A, left lower quadrant, oblique, B, midline. North or south. So about split. So there was a lovely paper I had the honor of reviewing and the author is in the audience about laparoscopically managing hydroculpos. Would you like to comment? Doctor Speck with the microphone. long enough. OK, well, let's, uh, wait for the microphone so they can hear you out. Doctor Speck is a, uh, Michigan trainee, but now at, now at Vanderbilt. Thank you, Doctor Levitt. Um, Doctor Teitelbaum and I actually at the end of my senior fellow year had this very patient, and we, um, did do a cystoscope and vaginoscopy at the initial, um, portion of the procedure to decompress, and then we put in our laparoscope through a left upper quadrant incision. And we could actually very well visualize all of our pelvic structures. We placed a right lower quadrant vaginostomy tube percutaneously and then a left lower quadrant diverting colostomy. So it's a, it's a nice technique. The alternative approach is through an incision. Obviously, and then the judgment of whether to drain with tube or no tube, as Michael alluded to, I would pretty much base on how big the thing is. And if it's a monster hydrocopos and it comfortably reaches the abdominal wall, you can do a sutured tubeless vaginostomy. And if it's a little bit lower than that, your ideal is to do a tube vaginostomy, but I would suggest using a curled tube. Rather than a straight tube like a pesser or Malanot. And I can tell you I learned that the hard way because what happens with the hydroculpos is it recedes from the abdominal wall, the inflammation goes away, and then at about 2 months, the tube falls out. Whereas if you have a curled tube, that doesn't, uh, that doesn't happen. Dan, do you want to add anything to your ex-fellow's comments? Did she, did she graduate? We, we passed her. Yeah, she's a real attending. OK, good. I, I think she's absolutely right. I think, remember, a lot of this fluid can be vaginal secretions, but a lot of them can be urine reflexing up. And so you, you may wanna, so, so the urine is often a big, as big of a problem as the. Vaginal dilatation. I wanna, I, before you raised your hand, I was gonna call on you guys about the decision about whether to drain the vagina or to drain the bladder, um. Doctor Curry, please. Mark, I think the issue of putting a tube in the vagina and leaving it in for a couple of months is not the most ideal situation for the baby and the family. One of the things that you may want to try is to have the family just catheterize the cloaca intermittently. And what that does is it drains the vagina and because a lot of, like you were saying, a lot of this fluid is urine after the initial newborn period, the uterus stops secreting and most of the fluid trapped in the vaginal part of the cloaca is urine that's refluxing back into that system because it's a serval mechanism. As the vagina distends, it obstructs the urethra. And that results in more of the urine leading into the vagina, and that keeps that cycle keeps perpetuating itself. So if you just put a catheter in the cloaca 2 or 3 times a day, it drains all that urine, and you don't have to, in many cases you don't have to do anything with the vagina. I would say that that's a very skin or or put a tube in it, a very reasonable approach, but I would add to that that that first, the teaching should be under ultrasound. Because, um, you can have that tube go into the right vagina, the left vagina, the bladder, or the rectum, and you may go three days without draining the right vagina, for example. Whereas if you are, uh, under ultrasound showing that the catheter is going where you want it to go, then that's a very reasonable approach. In general, though, you're not draining the structures you want to drain if it's a blind passage through the perineum. Which is why I've advocated for a formal drainage tube or no tube. But if you can get that to work and you prove that the patient is your job is to decompress, as you've said. If you can do that, by all means. Steve, you want to talk about that one case we had that, um, the patient came in very, very sick. Can you give it to Doctor Krauss the microphone. Patient came in very, very sick and we did a bedside ultrasound. So as Mark said, you have to have a crazy radiologist like me who goes up to the ICU with them and brings my own ultrasound machine to a newborn baby. And I don't know if you have the image, but I, I do have it on my laptop. And it actually shows the very, very dilated hemi vaginas and very echogenic fluid, probably uh meconium and urine, and the patient's creatinine was about 4. And so we went up there. Uh, Doctor Pena put a tube in, and I just followed it under ultrasound guidance, and you could do it even in the ICU. Now they have ultrasound machines. You could do it yourself, get the probe, watch the tube go in and see it being drained. And so it's a very nice elegant. One of the things that was very cool about that case is we, when we first did the ultrasound, we could not see the bladder. And then once the hydrocous was drained, the bladder filled beautifully. Which demonstrates the physiology. So the hydrocopo is compressing the ureters and the bladder can't fill. Now once the bladder fills, then the question is, can the urine get out of the bladder? And then on rare occasion you have to drain the bladder too. But I think in large, most of the patients, you can drain the patient successfully by draining the hydrocopose only. Be aware, like this image shows that there's often a right and left side, so you have to be able to drain both sides, otherwise, only one of the hydronephrosis will, will improve. And, um, this is an image of hydronephrosis before and after drainage of the hydrocopos, and this is what some of these, this is what one of these hydrocopos things looks, looks like. And then what you can do is surgically during the opening of the colostomy is open the dome and then remove a little bit of the septum, and then it becomes a single chamber. All right, I think we're going to quit. I have a question. Please just wait for the microphone. I wonder when if you put a, as Doctor Corey was mentioning, if you put a tube vaginostomy, uh, and you want to do an operation next like couple of months, would it be difficult to bring the vagina because it's fixed not to skin. Can you bring it down? It would be difficult because sometimes I find it difficult to bring it down. Yeah. But then, but doing as what Doctor Cody was saying, CIC, mostly it goes through the channel. Yeah, that's true. But if it's one of these very huge hydroculpuses, you're almost always going to need to be in the abdomen anyway, and then you can take down the vaginostomy. I think his way is very good if you can prove that you're in fact emptying the structures. If you're not emptying the structures, you'll have pers persistent hydronephrosis. So, um, there's some more talk about we hopefully we'll have some time maybe to discuss some of the technical details of Chloe.
