UG Sinus and Neophallus - Case Discussion

We're gonna kinda pivot now to, uh, kind of, you know, ask the experts here. Uh, I'm gonna Give you uh a few cases here. Um, Hot off the trot of Doctor uh uh Coyle, um, Discussion here. Uh, KS was an infant found to have, you know, abnormal genitalia at birth. Um, this is her second, uh, pregnancy, as you can see, first child, relatively normal gestation, uh, following a normal pregnancy. And I don't have a picture, um, but, uh, hopefully, I'll be able to kind of describe this by mouth and you might be able to Kind of figure out exactly externally because I have a good endoscopy for us to talk about. Um, there was some mild liromegaly. Uh, clitoral dimensions around 2 centimeters, 0.6 centimeters. There was a single urogenital sinus found, uh, between sort of these bifid labial scrotal folds that were, uh, fused, um, minimal rugations. Uh, I think the Prader score has some, um, deficiencies to it, but it's definitely something that we all may use in the lexicon. I would not say this child's clitoromegaly was, you know, Prader 4 or 5. We'll kind of call it a 2.5. It was definitely not, it was visible, but it was not uh one of the ones from maybe your lecture. Um, a karyotype was performed, 46 XX. If I'm talking about a Prater scale, I just told you this patient has sort of leading you in the direction of CAH, an elevated 17 hydro hydroxyprogesterone. An ultrasound and MRI showed the typical features, normal appearing uterus, no um uh testicles or any uh ambiguity for that. Started on mineral and glucocorticoid replacements, doing well, growing, no UTIs, no hypertension. And so, like many patients with these diagnosis, they come for sometimes uh a multidisciplinary or a center's, uh, Uh, opinion on what type of ongoing care. So, we have one of those centers here at our hospital. They are raising this child as a female and met with our whole host of sub-specialists. This was the genitogram, and that was actually obtained at the hospital outside our uh hospital. This is a spot image. And as I said, this was done while the child was awake. And I think we can kind of see where there is the bladder that is dilated. And then there is a structure that is opacified. And then you kind of see this virulized, uh, sort of approach. Um, So As Doctor Coyle was alluding to, uh, we had an extremely in-depth conversation about surgical management in females with CAH, the controversies. And we talked about no surgery. We talked about pros and cons of early surgery and the pros and cons of observation along with the intent of a delayed surgery given the persistent UG sinus. So, um, my first question to the audience and everybody is, what would be your next step? Um, we've kinda talked a little bit about before, but, uh, this specific case, uh, One option, as we talked about, repeat the genitogram. My mentor, Doctor Rink would say why the kid would scream the second time. So, Uh, we talked about the diagnostic, uh, sort of evaluation alone, and then come back and talk versus the all in one bundle, versus some people might say, what's the problem? The kid's growing, leave him alone. So, Uh, we'll return to kind of these, uh, results in just a little bit and kinda Jump ahead to kind of let this case kind of discussion go. But I, I want everybody to know uh what their kind of thoughts would be. Um. Doctor De Castro, what would you have done? I, I've, I hopped ahead and showed you what we did, poisoned the jury pool, probably. No, I, no doubt, I don't, I, I still don't have a, a right idea of the situation, so my recommendation is to do eval endoscopic evaluation first. And uh Marty, would it be kind of the same thing? Would you say um we think a outpatient diagnostic procedure is the best way to go and then uh reevaluate or as we're talking about, you know, how much repeated anesthetics, Should we do everything all at once? Obviously, that genitogram is leading you more towards, hey, this might not be quite the uh low confluence, but what would you do? So I think obviously much depends on your, your two slides ago slide with the discussion with the family and to their understanding of no surgery, early surgery or observation. Um, and what we would tell them is that in the old era, meaning when I first trained, we operated on day one of life. This was a surgical emergency. We don't do that anymore. This patient's already, uh, uh, 2 months of age. Um, we can talk about observation with delayed surgery, but there's really no data on that. So, do we swing the pendulum all the way to the right and say, oh yeah, this is what some people are suggesting. Or do we say, the decision is yours, give them as much information as possible. And if they elect to go with early surgery, then what, what I tend to do is say, OK, um, uh, and this comes from your institution, Brian, uh, uh, where you showed that really, once they hit about 3 months of age, the safety of anesthesia becomes, um, um. Basically, it's gonna be, I wait till 6 months nowadays because sudden infant death syndrome tends to occur in the 1st 6 months of life. And even though it's not related to anesthesia, if an act of God will occur in a child that I operated on, I don't wanna have that blame. So for something that's I would call uh surgery 6 months of age and I would do it all so I would endoscope plan if the family made the decision for early surgery, meaning 6 months of age. OK. Well, one of the questions from the audience is, uh, Brian, uh, you know, what's the role of an MRI, a pelvic MRI in here before you do anything further? Um, In my experience, uh, very limited. I think that the pelvic ultrasound, some of the biochemical assays, I think we've come up with a fairly accurate diagnosis of congenital adrenal hyperplasia. So, you finding some type of uh non-female internal genitalia. is something I've never really seen with an accurate diagnosis of CAH. So it's not a test that I've done for this condition. Um, it seems like the audience, as I'm looking to my left in the poll results, uh, around 16% said they just wouldn't, uh, sort of none of the above. So, I don't know if that equals leave the child alone or that equals a pelvic MRI in 16% of the respondents. But, um, uh, it seemed around 50%, which was the, um, uh, most frequent respondents, was, uh, somewhat of a diagnostic procedure alone. And as I said, I might have poisoned the jury pool by going to the slide too fast. So, uh, here's our operative findings. Uh, there was around 3 centimeters from basically the urinal sinus opening in the perineum to the confluence. There was around a, once again, we talked about the accuracy of measurements inside and out. I didn't use a ureteral catheter, but that's another trick that I've learned from Doctor Rink to use the markings on a ureteral catheter to provide accurate instead of, uh, what we all think. But a very short, uh, distance from basically that confluence of the bladder neck and the total vaginal length was around 2.5 to 3 centimeters. So not very large in this. And this was performed, uh, at around 6 months of, uh, life. So I have this video here and, uh, Jay, are you playing the video? This is, or shall I just play it? How about this? I played that one. Perfect. This is the one that you embedded. So, we are beginning this endoscopy that we are in the bladder right now, everybody, and we're coming backwards, OK? And, uh, if we kind of stop around here, just to kind of take this sort of frothy, I, uh, Doctor Rink would tell me sort of a Verro Montana. I mean, we think about it, the, uh, Vera Montanum is basically the male analogous to, you know, the paramysonephric ducts, the uterus. So, this is kind of our distance, uh, that's here. We're gonna kind of come back there a little bit. Hopefully, everybody, oh, I'm sorry, I was trying to more pause it. Um, But hopefully, this video in real time, I don't have the catheters for measurements. I think we're getting a uh a gist that this confluence is closer to the urinary tract than it is the perineum. And uh we'll kind of pull back here a little bit. As I said, definitely a more virulized appearance on cystoscopy than one would expect in a, you know, female. And now we're back just into kind of the genital sinus itself. We'll come a little bit forward and uh There's the orifice. Uh, in this video, I don't have it going into the vagina, but there was a solitary cervix, and as I said, that vagina measured to be around 2. There we go again. I'm just gonna, sometimes I find it easier to scroll so everybody can kind of see uh kind of what we're dealing with here, OK? So, kind of here is our ostia and then our bladder neck there and uh So, Doctor De Castro, I think I know what your answer might be. I think my choice, I, my, my suggestion to the family will be to do, to do a, a vagin uh immediate, a, a, a full operation, a genit a genitolasty in one stage and astra in, and then, and then, uh, turn a lithotomy position and finish everything and with the Paserini flap and Pissale, uh, clitoroplasty. So, um, mm, can I ask you, do you, do you think based upon this anatomy that uh selection of, say, a urogenital sinus mobilization and a lithotomy is, is a good idea? An alternative, you say as an alternative. Correct. Like if you said, uh, oh, I'm, I'm very worried about all this bowel work. I'm, I'm just a urologist. I, I don't, I don't wanna get in there. Um, I've been shown the total urogenital mobilization. Uh, do you think that selection of that surgical technique is gonna get you in trouble with this specific anatomy? Uh, I, I, I'm, I'm no experience of total mobilization, but sometimes I do some partial. Very partial mobilization can help in the situation in which, uh, uh, I, I don't think Atra is indicated uh and I don't want to do, to do any, any special preparation and I do straight to do the perennial, perennial approach and, uh, the mobilization could be, is interesting, very interesting. The partial mobilization is something that is part of my, of my Uh, practic, uh, in a, in a low confluence or medium confluence, uh, vagina, uh, vagina ending, yes. So, uh, Marty, are, you're back on your internet or, or I see you live now. You were talking, but, uh, it's frozen in time. I know it's cold sometimes in Canada. But, um, er, question to you. Do you think, uh, based upon this video that hopefully you were able to kind of see with me there, do you think that if you selected the total euro general mobilization, that may be a less favorable approach than, say, an Astra? Well, I suppose I've, I've, uh, been blemished a little bit by Rick, uh, and, and I, I don't do TUs anymore. I, I, uh, I would only do PUMs, uh, partial eurogenital, uh, mobilizations for fear of the unknown, I guess. Um, and, you know, after, after talking to Alberto, um, I, I think he supports that even to some degree, uh, despite his experience. So what I tend to do, and I think again the beauty of the younger patient and Roberto showed how, how big some of these patients are, it's difficult to turn them back and forth. A baby, you can do a complete body prep and be prepared to do, do them in the uh uh prone or supine or um uh lithotomy position. So that, that's the beauty of saying in the, in the ones you don't know, be prepared to do the Astra, but I start with a sort of a pum approach and, and see how the dissection is going, and if I need to, I turn them over and uh then go to the Astra. So, it looks like the poll results are kind of uh 60% have uh selected option A. So, um, definitely a majority, but, uh, as you pointed out, Marty, um, as the years have gone by, I guess it is no longer this uh surgical emergency, tackle it and you fail your boards if you didn't operate. So, it'd be interesting. Brian, uh, can I just ask both Doctor De Castro and Doctor Coyle what their, um, and if you can pull up the, uh, The genitogram, I know it's not ideal, but, um, uh, cause in the video, you pass it so quickly, but the length from bladder neck to the confluence, you know, the urethral length. Um, I know that, uh, when we talk about these operations and urinary incontinence as being a very important outcome for these children, um, the, uh, urethral length is something that recently has become much more important and has been acknowledged. And I know Doctor, um, Mark Levitt published a nice paper looking at what is the normal length of the female urethra to begin with because we start talking about this, uh, you know, but if you don't have an idea what the norms are, Um, but how does that factor into your equation of, uh, your surgical algorithm? Martin. So, you know, I think it all depends on age too, and, and, um, uh, one of the things that I think genitograms, uh, sometimes look much more scary than they truly are where it looks like, like the, um, uh, sinus is coming right off the bladder neck and it almost never is. I mean, I, I think it's almost always, uh, uh, distal to the bladder neck and embryologically that's what you'd expect it to be. Um, what Roberto brought up previously about the Paserini, uh, is, uh, I, I don't get too worried about the, the length of the urethra as long as that, you know, I know that I have a bladder neck. Um, my former mentor when I was a urology resident, Ben Gittas said, there's not a good reason for a woman to be continent in the first place based on the anatomy, which is a little bit of hyperbole. Uh, uh. But, but realistically, I don't know if there's a perfect amount and especially when you look at an infant compared to an adult female, that's the problem is you're comparing an apple to an orange. But the Passerini approach where Giacomo Passerini suggested using The, the common GU sinus as the anterior wall of the vagina and the lining of the vestibule, I think gives us a lot of um ability to really make a more anatomically normal area and to, to also make sure we're not cutting back too much because I think there is a worry. I just don't know what the worry is as far as length is. Hm. Yeah, I think that, um, if I might comment, you know, Doctor Reddy was referring to how uh our colorectal colleagues were um in a, in a uh cloacal anomaly population, seeing whether or not, um, urethral length should influence their surgical repair of a cloacal anomaly because obviously, This case is a urogenital sinus and, you know, somebody's got to do a rectal pull-through and then, as Doctor De Castro said, we are uh recruited in to assist with the persistent UG sinus, hopefully at the time of the rectal pull-through and not, you know, post-op. But, um, I think the reason why I asked our esteemed faculty here whether a total urogenital mobilization was appropriate for this patient. I think there is a limitation on how far you can um pull a bladder, bladder neck, uh, to the perineum without starting to make a funnel and starting to basically surgically create stress urinary incontinence for the goal of getting a vagina to the perineum. Um, and I think the multi-center papers that are out there on, say, a Use of a urogenital immobilization technique in a non-Cloaca patients has not revealed as much stress urinary incontinence as people are espousing or, or worried about. Um. And once again in another apples to oranges is the persistent UG sinus in a cloacal population is very different than the UG sinus in a CAH or maybe even just an isolated, as we all know, there's a large amount of sacral and nerve anomalies such that the neurogenic incidence in the cloacal anomaly group. Uh, sort of out of the gate is higher, but we all don't want to create problems for our patients urinary wise while trying to achieve a vagina at the perineum in a baby, which is important, but maybe not important at that age. Well, I think that that's uh pretty clear too from uh Mark and, and Alberto's work too that the 3 centimeters of a common GU uh sinus uh in a cloacal abnormality, not an isolated GU sinus abnormality, tended to portend a higher chance of uh needing a metrofenoff or whatever at the same time to assure that uh that their bladder would have to drain. But you brought up the key point, Brian. 46 XX DSD isn't associated with other abnormalities. Cloacal abnormalities, the higher they are, the more likely there is to be a vertebral, uh, or other abnormality, uh, that might be associated with the neurogenic bladder. Does um Uh, have either of you run into a vagina that was so short, you were like, uh oh, I can't get this to reach. And, uh, once again, I think Doctor De Castro, before I put this on, you said vaginal length is an extremely critical part of your surgical decision making. So, uh, What, what do you do with your endoscopic or intraoperative um assessment is, is, wow, this vagina is a lot shorter than I thought. I, I postponed the vaginoplasty. I, I don't have another chance. I have, I have to postpone the vaginoplasty in that case. Uh, I never use the, um, the suggestion to of, um, my one, my mentor, Philip Ransley to close the, the, the vagina, but, uh, waiting, waiting for the, uh, I have a, I have a big number of patients. That was treated without vaginoplastic, congenital adrenal perplasia treated routinely by, by some surgeon without doing anything for the vagina, and they referred to me in a big number. 89 patients in this situation. So I have this experience that uh in, in the age of 1213, 14, after puberty, the vagina, the, the vagina was much easier to, to pull through. And so this is my experience. So if the vagina is very small and the pediatric age, I don't do anything for the vagina and leave everything, but I like to do the job, the, the, the cosmetical, the cosmetical job for the clitoris and the labia. I don't leave uh the uh male appearance in a female. This is my point, yeah. So that's interesting, Roberto, because, uh, at least in North America, most of us, we, if we do a clitoroplasty, we, we don't touch the glands anymore or we do something very minor, uh, that just in case, and you talked about PIPI's technique of preserving the corporal bodies which we don't know be worthwhile or not, but it certainly, uh, seems like a, an alternative. Um, I, I have a problem with Phillip's idea, and I've spoken to him about it, that we don't know much about or, uh, because at least 15% of females, adult females will have ectopic endometrium. If you're closing off the, the, the junction and you don't have an egress and you're allowing them to menstruate. My worry is, uh, de facto, are they gonna, um, uh, reflux, uh, you know, their uterine contents through the fallopian tubes into their abdomen, and are we creating to make something potentially surgically better, are we creating a problem, uh, later on? So I personally am against that. I can tell you, uh, with an end of two of two very complex adults, uh, one that I did in Vancouver and one that, uh, a former, uh, Indiana resident sent to me from the Marsh Clinic, both adults, where I, and I regret it because I, I had more guts than brains in doing the surgery. They were small vaginas, adult, very masculine CAH. I figured there's enough GU sinus that I could just do passerini's, uh, on them. I had to add bowel to both of them, and I wasn't prepared to do so at the beginning. I did it with unprepped bowel. Fortunately, they both turned out OK, but believe me, I think preparing for the unexpected is the key to any surgery. I don't care how simple it is, and don't go in there and be a hero unless you know what you're doing. Yep. So, our, our center's sort of recommendation was to be a little bit more conservative. Um, uh, Doctor De Castro, do you want a 90th patient for your case series if I give him a ticket to Bologna? But, um, remarkably, uh, we've all seen where the parents have a lot of, a lot of distress over this diagnosis, which, as I said, was not known prenatally. Um, I think that the external appearance was not so virilized and our discussion with them, and I offered. Uh, outside opinions, so that they didn't feel like, uh, oh, the, the Cincinnati experts said we can't, so we can't. Uh, fortunately, we're short driving distance to an extremely experienced busy surgeon, and, uh, that person actually answered the phone when I called them too. So, Um, and, but this family is actually coping very well. Their child's growing. We see them annually. So, we are supporting them. We're caring for them. It's just not with a surgical, uh, treatment arm. But we do know that it's likely that surgery will need to be performed and just hard to know what's that right age when that's gonna happen. So, Um, I have 3 cases, but I'm gonna jump to number 3. So we're gonna, Brian, before we leave, uh, one of the questions is, um, from the audience is, uh, what's the association of DSD and an anorectal malformation? Do they coexist or can they coexist? Um, well, uh, trust me, how many times I had to listen to Alberto Pena say, I can't tell you how many cloacal patients got labeled with ambiguous genitalia. Uh, I personally have not really seen any type of endocrinopathy, uh, with a anorectal malformation to result in such bizarre. It, it usually is the cloacal anomaly spectrum, whether it's more virulized, feminine or prophallic. That I think that the neonatologist or the birth hospital just throws it in that basket of ambiguous genitalia and you cross your fingers, somebody recognized there was an anal problem. So that it jumped out of that basket to get to the surgical division. So, in my experience, and I would ask the other panelists if they've seen similar things or I've seen ectopic labia and things like that which people have uh construed as being ambiguous, but it, it uh with anal rectal malformations, uh, but not true association of 46 XXDSD with an ARM. Yeah, sometimes DSD, DSD, if, if you look at the, at the, at the list of DSD, you can put everything, I mean, but, uh, even, even, even the simple, the simple, uh, understand the testis is DSD. So, is it exaggeration then, so DSD must, must, must have some more to be, to be in, in that, in that field. Don't tell our politicians that because they're trying to legislate against anything that related, that sounds like a genital abnormality. So I, I, I, I know we only have uh probably around another 30 minutes uh before we're gonna take that lunch break. And, uh, I, I lied. The title of this was Your General Cases. But, uh, as Doctor Reddy introduced Doctor De Castro, uh, I think his, uh, Uh, the eponym associated with the De Castro technique for dealing with aphalia, we would be, uh, remiss as to not extract your thoughts and knowledge. So, this was a patient that was referred to our center. It's a twin newborn male who was found to have, let's talk about anorectal malformations here, uh, and an aphalia and a low-lying conus. Um, He had, on imaging at the outside hospital, solitary left kidney with uh hydroureter nephrosis. This patient underwent a diverting colostomy and a vesicostomy on the first day of life at that hospital. Um, That, uh, upper tract urinary dilation did not really resolve. In fact, it started to worsen in sort of the first few weeks of life after the vasicostomy. So, a nephrostomy tube was then placed, that was then internalized with sort of a double J stent once they arrived to our hospital and was seen by our colorectal colleagues in urology. I got a little picture here. Um, as you can see, there is a, uh, formed scrotum, but aphalia, prolapse. This is the vesicostomy. This is the divided, uh, colostomy, the mucous fistula, as well as the end colostomy, and umbilicus. And this is the back of the patient. And as you can see, an imperforated anus. And, uh, this little dimple here was just at the top of the sacrum. There was no sort of urinary egress. Interestingly, uh, this brother, uh, is, or this twin should be probably very, uh, happy that he had a monoamniotic, uh, twin because I imagine pulmonary development was relying upon his brother's fetal urine and, uh, development. So, Uh, but as you can see, there was no urethral opening identified. So, uh, Doctor De Castro, uh, we're gonna ask for you to comment in just a little bit, uh, with that, uh, fantastic video. But, uh, to start out with, A falia grabs a lot of people's attention as well as the other anomalies. Where does the Where does the timing of that fit into this, in your experience? I saw many patients, I saw some patients with anorectal malformation together with, unfortunately, with the tragedy of uh uh penallogenesis. And of course, if you think on the possibility to use abdominal flap to create a penis. The colostomy should be a little bit more high to leave, to leave the lower abdominal wall intact. Vesicostomy is also another problem. I had patients with vesicostomy and I with penylogenesis, and I was able to do the flap anyway, thanks to, um, skin expander. Uh, using a skin expander, uh, is possible to even with the, with the mitrofenil on one side and colostomy on the other side, you can even. Uh, have an acceptable amount of skin to do, to do, to do, uh, to do a phaloplastic in early life. Uh, so it's, it's, it's important to, thinking about phalloplastic. It's important to, to, to, to, to making the colostomy in the right position and possibly, possibly to avoid the vesicostomy or even the vesicostomy can be moved, can be done in, in, in more close to the umbilicus than we usually do in, as, as in this particular case. Roberto, um, with the skin, use of the skin expander, um, cause, you know, we, the flap that you described and you're gonna show, it's not just a cutaneous flap. I mean, you're taking that subcutaneous tissue with it. Um, with the skin expander, are you able to get that subcutaneous tissue also to be as robust, or is it just a, um, you know, a thin skin flap that you're gonna be able to get? The, the, the extra skin that you can gain thanks to the skin expander is most, uh, is very important to reconstruct the abdominal wall after you use the, the flap for, so the donor site, the donor site for the flap for the the penis is covered by, by the, by the, the skin that you gain with the, with the, with the, with the skin, with the skin, with the skin expander. And we start using the skin expander in case of cloaca, in case of uh complex, uh, um, associated anomaly, but then we decide that possibly to use skin expander every, in every case, even if the, if, even if the Aphelia is just a, a, a single anomaly and there is no other problem. Skin expander is very, is very nice, it's very useful. And how long does it take you to get that expansion? Do you do a rapid expansion over like 6 weeks? We, we, we develop an introduction of a skin expander through the umbilicus. Just a small incision and just using sometimes the laparoscopic instrument to get a nice area and, and put the skin expander in a very, very, so with a very, very small incision. This allowed to start the expansion immediately. And have a very short period, 3 months, 2.5, 3 months before the operation, before the phaloplastic. Uh, if you do the classical approach, inguinal approach to insert the skin sponder, you need at least 6 months before, before the phalloplastic. So, Brian, a couple of questions from the audience are, uh, is there a distal cholostogram for this patient and also, did we do cystoscopy via the vesicostomy? Yup. So, we did, uh, I'm in, in the interest of time and for where we are, I, I'm not gonna show you the images, but basically, the, uh, fistula connection, uh, for this imperforated anus was very high. It was at sort of the rectal bladder neck. Um, and then cystoscopy also did not reveal any type of leakage of urine outside any other type of, uh, external orifice. So, um, we took to the operating room and we did a, um, uh, also, at this point in time, uh, we kind of diagnosed actually a, uh, solitary kidney with a Uh, a UVJ and a UPJ cause at the time of the nephrostomy tube placement at the outside hospital and the internalization, there was a UPJ, uh, obstruction. OK? So, at the time of the, it was a transabdominal pull-through that was kind of done laparoscopic assisted. And, uh, I basically did a, uh, left open pyeloplasty. They revised that very, you know, prolapsed vesicostomy. And uh then at the time of the um Um, colostomy closure, uh, through that same kind of incision because I left a, uh, sort of like a KISS catheter, and I did an antigrade, uh, study, and that's how I diagnosed the UVJ obstruction, or at least that there was not flowing. So, I actually did a, uh, a refluxing reimplant to just get him out of dodge cause we were leaving him with a vesicostomy at that point, and I didn't want to go creating trouble with the tapered reimplant. And um so now we're up to where, uh, Jay, you're gonna play this video. Um, this patient was um seen at a center here in North America outside of us cause the parents were pretty early on recognizing that the aphalia with their twin male brother may have been sort of an accentuated. Stress, let alone with the aphalia alone. So, um, I had, uh, referred them to Doctor Reiner in Oklahoma, who had then also seen Doctor Crop. But, uh, so this patient did have sort of a, uh, a phaloplasty outside of our institution, but I think this would be a great time for us to pivot to, uh, the video, Jay. And Doctor De Castro, please comment during the video. Yes, yes, sir. Thank you so much. So this is a Scratch of a child with penile agenesis. And it's not clear, but, uh, in my mind, just think that the skin expander was uh inside and uh filled already, and this is the flap. Stop the video. You stopped the video, Jay? Did you want the video stopped, Doctor De Castro? No, it was jump, jump from, uh, it's, it's, it's a pity not to, not to have shown the, how, how we make the, the flap, but this is the most important part. We jumped to, to, to the hand of the, of the. Uh, OK. Doesn't matter. So the flap is already done in the, at this stage, and we take a piece of uh rectal muscle fascia to make uh. A tube And this tube is not the urethra. It's just something to put inside of the flap to make some sort of suspensory ligament, something like that. So let's see, this is a case of penallegenesis, congenital ephalia. The urethra is ending very, very close to the rectum. And this is the flap. The rectangular flap is going to be the penis, and this is going to be the, the, the glands, and this little extension is going to be the urethra. Stop again. What happened? I'm sorry for that. I think we may have a little bit of a delay with what you might see in the Zoom. Do you see it now? We stopped the video No, no, please go ahead. OK, sorry, we heard you say stop. That's why we stopped. No, no, no, sorry, sorry, I didn't see any, any, the video, so just go ahead. So we are developing the, the, the, the, the, the flap. And we start approximate the cylinder of skin. To make an acceptable appearance of penis. It's not my fault, but the video stopped again. I don't know why I can hear Roberto. We can see the video. Yeah, we can see you can see the video. I'm not anyway. So what, what, what, what we're up to now is the, I think the rectal fascial uh harvesting and the tubularization. So I don't, I can't, I don't know what you're able to see. Excellent, excellent. And then, and then this, this, uh, this tube is introduced inside of the penis. Again, this is not to make any attempt to reconstruct the urethra. It's just something to put inside to make uh uh to, to work like a suspension ligament. And to keep straight a little bit the penis, because the only skin, uh, there is nothing inside. And we connect the, the flap with the, with the, with the rectal fascia. And then we go ahead to, to complete the appearance. Of a cylinder You see, it's go ahead because I see the video stopping again, but probably you right now, right now on the, on the superior part of the incision you are, uh, defattting, I think the skin for the coronal sulcus. That's the big you're already there, OK. Very good. We like to have a little, a little bleeding, that means that the flop is uh still alive. And then we insert this free graft. It looks like a circumcised penis. Are you using monochryl suture for most of this or Vicryl or Vicryl 6 or 70. Now, we are up to the part where I think you're uh trying to close the lower abdominal defect by circumscribing the umbilicus and Yes. Next time we'll have the meeting in Italy, the penis looks acceptable most of the time, but you have to see that in the long term, of course. It looks more than acceptable in the video I'm looking at, so. So, we, we finished the isolated and uh now we're onto the uh traumatic penile amputation one. Yes, and, and, and this is very, very interesting because we call functional phaloplastic because usually we are able to have the patient passing urine in a short penis but uh but in an acceptable way. So the flap is exactly the same, but you see the difference. The difference is there is some residual corpora cavernosa there. This is a case of trauma, is in one year, one year old boy, one year old boy that lost his penis because of a dog attacks. So, in that case, you know how you're talking about the um the, the, the expander. When did you put the expander in this child? The expander was, was put inside 3 months before. But what, how, how long after his um Attack, you know, because at that point, no, the attack was, was, was, was at the age of, of a few days of life. Oh wow, OK. In Vietnam. This is from Vietnam, and uh when we saw the patient, we, we decide to, to insert a skin expander and then delay the operation three months later. So, I don't know if you, the video is still playing for you now. Can you see? No, it's not playing for me. So it looked like you basically did the skin, uh, incision and kind of find the residual functional tissue and you're kind of buttonholing it out. And we found a, a, a good amount of, in this particular case, we found a good amount of corpora cavernosa and urethra. And, and we leave everything there. I think it's 3 centimeters. Yeah, it, it definitely. Sort of like a proximal hypospadius. Once you start dissecting the penis, you, you'll find it. But anyway, I think the flap is important. Uh, to Because sometimes you can, you can, you can isolate the corporate, then you don't know how to cover it. So my, my flap in this case is just to cover the residual corpora cavernosa, and I don't know if you are looking at the video because it's stopping again in my, in my screen right now. I think you are trying to get the distal lens of the flap to the uh remnant urethral meatus exactly and make it large, as large as possible meatus because the stricture there is a, is a, is danger. You can have a stricture of the meatus if you don't do a very large uh uh anastomosis between the flap and the urethra. Did, it did not look like you spatulated when you did your skin? Do you try to spatulate the native urethra like we do for other kinda Yeah, sometimes, yes. In this particular case, I don't, I don't remember, but sometimes, yes, we had to open a little bit more to have as large as possible and then move the two little flap inside to reach and then again. Some boys that have undergone, uh, say, isolated hypospadius repair, depending upon the technique. I've seen them in clinic and they say, my urine sprays or I don't like the way my urine comes out of my reconstructed urethra. What do some of these children say that have a voiding urethra after your phalloplasty? Do they have some of those similar complaints after urethral reconstruction or? Uh. No, they have any, anyway, they have a acceptable short urethra, so they don't, don't, don't have the trouble of a long new urethra, no, they have a, a an acceptable short ureter, that's the original urethra. They own, they own urethra. Just that, the distal part is made by the flap. Yep, yep, we get to watch a video, spontaneous voiding, one month later. And uh you know, you see, you see the scar is even present, so this is. This is why we call functional, functional phalloplastic because we are able to have. To have a short penis, but uh uh working penis. Yeah, I mean, this, this little boy has something to grab, so. That was, uh, that was an amazing video. Yes. We're truly amazing, uh, Roberto, one of the modifications from your original description of your flap to what you showed today is the addition of the little, um, Z plasties. Is that because you were seeing contracture on the uh along the anastomosis exactly, exactly. Sometimes are disappointing because the, the, the ventral aspect is quite good, but the dorsal aspect of the new penis is short, short. And, and so we try to make something to, you know, to have a, to have a more, um, a less reaction, less contraction of the, of the, of the um scar uh after surgery. But I think the most important modification is that the flap is large, as much large as possible. It's a rectangular flap, not a quadrangular flap. And all the extensions we made have some, uh, made, made some advantages. The rolling of the rectus fascia to act as a neo-suspensory ligament. Do you feel like that uh rolled fascia stays intact or does it just scar down and disappear and get reabsorbed by the body? The Some, some parents are so nice, are so good that they are continue to keep open, to, to make a catheter once a day inside of, of that uh tube that we create, and, uh, you know, this can, can make you thinking about some possibility to You know, to introduce something inside there like Dflux, like something like that. I mean, just to have, to, to make, to make the penis grow, uh, we never tried that, but, uh, this is something that can, can be done maybe in the future. Roberto, I, I've, uh, watched you do these now for close to 20 years and, um. Uh, some of whom are now, uh, in their late teens and probably into their twenties. How many with non-functional, so have no corporal bodies, um, have people tried to put erectile, um, artificial cylinders or whatever in there for erections since then? And, um, have you, have you looked at their, um, semen analysis, their ability to ejaculate everything else, uh, in the ones who've, uh, uh, who have passed that age group? In my, in my series of 47 patients that received the phaloplastic, I have only 2, only 2 patients that reach the age um of puberty, much after puberty actually. One patient that was treated in, uh, in Brazil together with the Macedo. Uh When he was 11 years old, when he reached 22 or 21 years old, Antonio in Sao Paulo inserted the prosthesis, a single prosthesis, and he's reporting an acceptable outcome. I think the paper is coming in the, in the, in the Journal of Pediatric Urology in, in a very, very, in the, in the future, very, very soon. And I have another patient. That, that was my number 2. The second patient treated in my series, this an Italian boy, and he reached the age of 19, and uh we send the patient to To to Ghent. To Ubike, to Professor Ubike, and uh he made a new a new phalloplastic for him. So the, my phalloplastic was acceptable looking, was made an acceptable looking penis till the age of 1516, but then the boy needs something more. And uh since from the very first operation, we did the first operation together with Pie Ubike in, in Bologna and then there was the plan from the very beginning to send the patient to him and he made the, the, the, the, the, you know, the traditional uh uh standard ph adult phaloplastic for him. So you're gonna hate me for this, but, uh, I've continued to use, uh, scrotal uh uh phalloplasties as temporizing procedures, and they just look like an uncircumcised, not a very pretty, uh, penis, but it, it creates an appendage that's easy to do. And the difference is, uh, the urethra, we can usually move maybe into the scrotum, but rarely, uh, and sometimes even to the neo uh penis scrotal junction, but not further than that. Uh, so we have a paper coming out with, uh, Juan Prieto and another Brazilian, uh, pediatric urologist, Francisco Dinas, who, um, between us, I think we have 11 now that we've done. Now, none of these have had ARMs, which is a, uh, a little bit different too. Uh, so they've all been, uh, patients with normal rectums, and every one of them has had, uh, their urethra opening in the anal verge, essentially. OK, OK, I mean, I accept, I accept that, of course. I mean, the most important, they don't look as beautiful as yours. Yours look like a penis. Ours looks like an appendage. The most, the most important point is to leave this patient as male, not to make any change. This is the most important point. Then you can. Do whatever you can postpone every surgery to puberty or you can try to do something like you do, like I do, uh, to, to, you know, to give them some penile appearance during, during infancy and then, and then think about something else maybe at puberty or after puberty. Thank you, um, Doctor De Castro. That was an important question that, uh, one of the audience members has asked is, when would you consider a gender reassignment in this instance? Is there any role at all for that? And, uh, I know in North America, we would say no role. Never, never, never, like, but as you, as you have traveled around the world doing this, have you seen culturally that maybe, uh, because I know the male child is always more valued in many, many cultures, and so it's very hard for me to imagine that a parent would say, take my son and make them into my daughter, but have you ever experienced that or seen that, and what are your thoughts? Well, unfortunately, my, my only experience is my own experience because when I was a young doctor, I saw, I saw 3 or 4 cases of penallogenesis when I was a resident in Bologna and one of them, we made a wonderful operation, a wonderful operation to make a female. And this was a disaster. I know, I know the story of the, of the, of the patient. This was, was a, a, a great disaster for the, for the life of this patient. In my first patient, is, is, was an 11 month old, a patient from Yemen. I was working in Saudi Arabia. And even in Saudi Arabia, many doctors suggest to do a, a, a gender reassignment in female, even in Saudi Arabia, and the, and the, and the I mean, I don't know, luckily or not luckily, but the, the father came to my hospital and said this is my son and this is going to drive my car and this is going to take care of my family when I So, so I cannot think to make him, to make him as a female. So doctor, can you do something? And this was the beginning of thinking to do some little things to help to keep, uh, uh, the gender, the, the right male gender, uh, and this is the main point, no doubt, this is the main point. So there is no, no, at the moment I think. There is no way to, no reason, no, no, no, no doubt anymore, uh, to make any gender reassignment in pinine Genesis, and we can't underemphasize. That the um virilization of the brain has already happened and so even though, you know, you may think that uh the gender reassignment might be OK from a cosmesis, external genitalia appearance, you cannot uh affect the realization that's already happened and that's one of the most uh biggest casualties when you do gender reassignment. And that will, that psychosocial pathology that develops in those individuals is significant if it, it is lifelong, and it can be very, very, very devastating. So, I wanted to give a, just a, a follow-up. So as I said, this patient underwent um Uh, the, the outcome didn't exactly look like the video, but, uh, there is definitely, uh, a phaloplasty where there, that I, I did not take a picture to share with you, but, uh, exactly, I think like what, uh, Doctor Coyle said. There is some Some, uh, tissue that is there instead of it being the blank Ken doll that, um, you know, we've all seen, uh, poorly performed circumcisions in normal male infants. Um, I, I won't say it's exactly like that, but, uh, the family was very pleased. Um, and, uh, this patient's vasicostomy needed to kind of be moved during that lower abdominal. And the vasicostomy was replaced. And this family decides to come to Cincinnati for both their urologic and um colorectal care rather than kind of segregate the urologic and color care between the two institutions. Um, interestingly enough, this patient's renal function, uh, their GFR is 85. This patient's now 5 years old. Uh, we've discussed, you know, nephrologic surveillance, and in fact, their nephrologist is closer to their house in Memphis. Um, but the vesicostomy kind of stenosed a little bit after being replaced, probably cause it's his 3rd vesicostomy revision, sort of since, uh, birth, or, or bladder. This child is able to remain continent through a somewhat stenotic vesicostomy. He's not suffering from infections. Given the refluxing reimplant that I performed, he definitely has high-grade dilating reflux. But this patient seems to be not suffering from fecal incontinence due to an enema program from his ARM. He is urinary incontinent by having his family kind of come to school and cath him around every 2 to 3 hours. And they're very happy with their phallic, but, um, What I wanted to kind of then go over with these two questions of the non-surgical psychological support that maybe you can say, what does your center offer these families? Cause I know they're coming for your surgical expertise, but you've probably developed some of the other non-surgical, um, holistic care for this. And then, I wanted to ask, um, You know, how do you determine the bladder management? Uh, As we said, voiding. I think you showed that great functional phalloplasty. But what about the patients where, as I pointed out, there's a sort of no bladder outlet or And I think you've explained, there really hasn't been any intermittent catheterization from a neo urethra. Like our other, uh, adult counterparts, and you've said you've had to perform this procedure in patients who had existing metopfeals or vesicostomy. So, I figured we'd finish up before lunch, trying to see if there was some dialogue about these two things and maybe that's psychology. Can you comment on that, uh, Doctor De Castro first and then Doctor Coyle? Uh, in my experience, uh, Mitrofanov was used. I'm not sure if 5 or 66 times many of my patients with born with penilelogenesis with the rectal urethra, we move the urethra up to the perineum. Sometimes it's possible to do intermittent catheterization through the, the the perineal urethra, only one actually, only one patient. Uh, but, uh, some, some patients, uh, uh, need, uh, a vesicos, a, a continent vesicostomy. Uh, I did a Casale technique once and mitrofanov, classic mitrofnov in all the others. So, the, the, the, the urethra reconstruction is, is very challenging. Uh, in the very beginning, I tried to make a simultaneously reconstruction of the urethra with bladder mucosa of uh buccal mucosa graft. But, uh, it was a disaster, so I stopped doing that. And, uh, we, we, we moved the, we moved the, the urethra from the perineum at least to the scrotum, no more than that. And, uh, I know that some, some friend of mine, I did the phaloplastic and they tried to do, to do some kind of BRCA, two-stage procedure to have the urethra at the, at the, at the tip of the penis. So some, some is work, some of them made a good job, but uh in my experience, the distal, I mean the penile urethra reconstruction is a real, uh, a big, a big problem. So I, I think that uh again with our series, since they're all without ARMs and all have had urethras at the anal verge, they're all voiding spontaneously, and I think obviously the question you're asking about uh need for CIC is going to be individualized based on the anatomy and, and uh bladder size function, etc. Remembering too that Um, aphalia has a 50% mortality rate. OK, it's a large number of these kids are dead or on birth or dying very quickly because of the associated abnormalities. So, you gotta remember that some are gonna have renal dysfunction, they may have polyuria, they may have all sorts of things that you have to deal with that become a little bit more urgent. So in the neonatal period, you might choose vasicostomy, whereas on later, later on, depending on their capabilities, it may be a metrofenov. So again, individualize your care with the first point on psychology, I think. Roberto hit the nail on the head when he and I trained in the old days. These kids were converted to females. OK, we were paternalistic, no different with the other DSD patients. These were emergency decisions to make sex of rearing, which was done on day 1 to 5 of life, and something was done surgically for them. That was wrong. We've learned from our history and as we evolved as a specialty. And probably there's nothing more important other than informed consent and disclosure is the multidisciplinary care of all these types of patients and the integration of a strong psychology and social support system for them, where there's constant support, not only of the patient, but the families because there's a A lot of dysfunction that occurs in many of these families. A lot of blame, and the kid tends to be the one who tends to be the repository of that damage. So again, this is something that we've evolved as physicians in general, but especially with these types of patients in embracing multidisciplinary care. Doctor De Castro, one of the questions from the audience is, what's been your worst complication in taking care of this particular subset of patients with the, with the technique? Ischemia, ischemia of the flap, unfortunately, in the, in the beginning of my experience, not, not anymore because now I'm using a very large flap, but in the beginning of my experience, I had, uh, 2 or 3 cases, uh. Uh, of severe, severe ischemia of the flap, and it was very, very difficult to find the, the skin to replace, uh, uh, and to make an acceptable penis later. And one other question was, have you used this technique to create a neopallus in a 46 XX individual? And how have you incorporated the clitoris into that for some sort of sensation? Yes, the, the, the, any, yeah, I have some patients with uh a partial androgen insensitivity syndrome and with a very, very severe form of partial androgen insensitivity syndrome. It's a very challenging to, it's, it's a very challenging to ask yourself many times before to do phaloplasty in such cases, but in 5 of them, in 5, in 5 cases we did, we did the phaloplastic in, in pies. And, uh, of course, the clit the clitoris, the, the remaining, the, the, what's remaining is very, very important for sensation, sensation and er for erogenous purpose and we usually are able to leave the little glands in at, in the scrotum, at the, in the, in the rafi of the scrotum. And uh this leave the, the sensation, the good sensation, yeah, of course, we have to preserve, we have to preserve, absolutely to preserve the, the clitoris. Perfect. Well, I, I mean, this, uh, discussion was fantastic, um. I really appreciate Doctor De Castro, you, uh, sharing that video, uh, as well as your presentation on Astro, Doctor Coyle, where your commentaries are always great. I'm glad you got a necktie on, you know, uh, but, um, we are, uh, gonna take a break for lunch, OK? Cause the bio break was too short and I couldn't get up to go to the, you know, but, um, at 1:30, we are gonna resume. But from 12:30 to 1:30, Uh, please, all your attendees, you can kinda, uh, check out that social room, the social lounge. Hopefully you found that font. That way you can kinda connect, uh, sort of on this video or chat platforms as well. And we also have, um, These opportunities to speak with some of our faculty, uh, Doctors Reddy, Doherty, and Shrine, if you have any specific questions. And, uh, but everybody, uh, we are gonna kind of take a, a pause here and uh see you all back at 1:30 Eastern Standard Time. Thank you, everybody. Thank you, Roberto.