You Okay, I'm going to get started. Welcome to the AUG's urogynecology webinar series. I'm Taji Yazdani, the moderator for today's webinar. Today's webinar is titled Congenital Genitourinary Anomalies and the Role of the Urogynecologist. Our speaker today is Dr. Maggie Mueller. She's an associate professor in the Division of Female Pelvic Medicine and Reconstructive Surgery. She joined the faculty in 2015 after completing the Female Pelvic Medicine and Reconstructive Surgery Fellowship at Northwestern. She completed her residency in OBGYN at the University of Chicago. She's double-boarded in OB and Female Pelvic Medicine and Reconstructive Surgery. Dr. Mueller's clinical interests include treatment of urinary incontinence and pelvic organ prolapse, fecal incontinence, complex urinary and rectal fistulas, and mesh complications. She has worked with expert pediatric surgeons to develop a nationally recognized program, the Collaborative Advanced Reconstructive Evaluation Clinic for Women with Congenital Anomalies, to care for young women with complex congenital anomalies. Increasing numbers of women who underwent complex surgery for cloacal and bladder atrophy are transitioning to adulthood with unique urologic, obstetric, and gynecologic needs. Dr. Mueller recently earned the ABOG Focused Practice designation for Pediatric and Adolescent Gynecology and is on staff at Ann and Robert H. Lurie Children's Hospital as well as Northwestern. She's actively involved in women's health research and has received several national grants to study complex pelvic floor disorders, and most of her research interests focus on surgical outcomes. She's also an enthusiastic surgical educator. I'm so glad she's joining us today, and she currently co-directs the Feinberg School of Medicine Reproductive and Genital Urinary Sciences module and serves as the fellowship program director. So this presentation will run about 45 minutes. The last 15 minutes will be question and answer. Before we begin, I'd like to review some housekeeping items. AUGS designates this a live activity for one CME credit. To claim your credit, you must complete the evaluation following the webinar. The webinar is being recorded and live streamed. A recording of the webinar will be made available in the AUGS e-learning portal. Please use the Q&A feature of the Zoom webinar to ask any of the speaker questions. We will answer them at the end of the presentation. And use the chat feature if you have any technical issues. Our AUGS staff will be monitoring the chat and can assist at any time. Thank you, Dr. Mueller. Awesome. Thanks, Tashi. Thanks for that great introduction. You covered a lot of bases and some of that I was actually going to say in this title slide, so thank you for that. So I, the first thing I just have to say is I'm really always very excited to speak to a urogynecologic audience about this. Because as Tashi alluded to, I truly believe that urogynecologists are really the best suited given our knowledge base and reconstructive surgical skills to take care of these women. Many women who are transitioning out of the hands of pediatric surgeons and have some complex needs that we at least can orchestrate a team to help take care of. And that's really basically what I have done at Northwestern, gotten a very collaborative multidisciplinary clinic together to be able to do this. So I'm going to highlight some of the things that we think about when taking care of this very unique patient population. So just some, you know, again, a little housekeeping, but some of the learning objectives, very straightforward. So hopefully at the conclusion of the lecture, you'll be able to identify different types of genital urinary anomalies, illustrate the evaluation and management of common genital urinary anomalies, and then anticipate the reproductive sexual health and pelvic floor outcomes for these young adolescent girls and women. So this is our typical page that maybe one of your residents or trainees might receive. 15 year old female with severe cyclical pain, no imaging. The very astute medical student takes a gynecologic history and notes that they've been having menstrual periods every month for two years. So it sounds like at the, at first flush, there might not be something that we need to be really putting our reconstructive hats on for. But I will illustrate how a clinical scenario like this can actually be a manifestation of one of the more complex GU anomalies. So just, you know, basics overview. I'm really going to divide this into anomalies that require reconstruction, reconstruction of the genital system. And that would include things like vaginal agenesis, vaginal septa and imperforate hymens or hymenal abnormalities. There are also anomalies that require reconstruction of reconstruction of both the urinary and the genital system. Congenital adrenal adrenal hyperplasia is kind of grayed out because I'm not going to speak about that today. But it is one of the, one of the conditions that will require both genital and urologic reconstruction. There are some more complex GU anomalies that I do want to touch base on because they do have pretty significant long-term reconstructive surgical considerations, including bladder exstrophy and cloacal exstrophy. So that's what we'll be covering today. So again, why do we care the name of our clinic? And I think that we, we kind of alluded to this earlier, but complex GU anomalies are, they're really complex. They're both medical and surgical problems. One kind of anecdotal example I have is there was a patient that I saw early on in my career who had bladder exstrophy, which was repaired when she was a child. She was in her twenties and she had had vaginal prolapse, which was attempted to be repaired with a pediatric surgeon. And she had recurrent prolapse and she was coming for a second opinion. And one of the reasons she stated that she was coming for a second opinion is because when she told the provider that she had a history of bladder exstrophy, she saw the provider get on the computer and Google the term bladder exstrophy. So that doesn't really, you know, foster a lot of confidence. And I wouldn't fault the provider. It is a very unusual, one of the more rare complex anomalies. But it's something that I think should be in our wheelhouse and we should be able to at least anticipate some of the pelvic floor conditions that might come up for these women. So what are the things that we think about anticipating for caring for these young adolescents and women? Certainly fertility. This is front of mind, especially for a young teenager that might have just recently had a diagnosis in their family. Certainly this, any person who's had any type of reconstructive genital and urologic surgery, we should be thinking about pregnancy and delivery planning. And then there's concerns for sexual function. And then as I mentioned, prolapse, other urogynecologic concerns, including incontinence. So back to the basics, I'm not going to belabor this, but, you know, we think when we're talking about you anomalies, obviously we're talking about the embryology of the renal system, the development of the kidney, the ureter, the bladder, and the urethra, and then the embryology of the uterus and vagina. And when we're speaking more specifically with respect to external genitalia, like conditions like congenital adrenal hyperplasia, we're going to need to remember the embryology of the external genitalia. I'm not going to go into major detail, but I just want to remind everybody that the adult kidney is formed from both the ureteric bud and the metanephrogenic blastema that kind of come together during embryogenesis. And this is happening and the ureteric bud is budding out around the timeframe of five to eight weeks. The urinary bladder is also developing around this timeframe and a little bit later. And towards the end of development, this is when the allantois and the bladder are still continuous. The bladder is really arising from the vesicle portion of the urogenital sinus. You can see that in yellow here. And then I think this is probably things that from a urologic or urogynecologic background, we're probably all a little bit more familiar with. And this is just, these two diagrams are depicting the portions of the genital urinary system that are derived from the urinary, the urogenital sinus in yellow. So that would be the urinary bladder, the lower one third of the vagina. Most, most folks consider that to be a portion of the urogenital sinus, although there is some debate there. And then everything in blue is really developed from the paramecinephric ducts. So those are the fallopian tubes, the uterus and the upper two thirds of the vagina. When we talk about the development of the gonads, certainly where we have, everyone remembers that we have an undifferentiated gonad. And then in the presence of a mullerian inhibiting substance, we will go down this pathway that will cause active regression of the paramecinephric ducts. If that's not there, then you'll have development of the paramecinephric ducts and then regression of the wolfian ducts or the mesonephric ducts resulting in the typical internal female genitalia. So now getting into the meat of some of the common GU anomalies. So certainly I'm going to start with the straightforward anomalies that affect the genital system. So really, when we talk about mullerian anomalies involving the outflow tract, we divide these into non obstructive and obstructive. So non obstructive would be either a somewhat perforate hymen, somewhat perforate septum, a longitudinal septum, an anomaly that's not causing an outflow obstruction. So these surgeries for these types of anomalies are really indicated for sexual function or fertility. There's no emergency to these. So when this is identified, certainly a young woman can be counseled regarding the, you know, the need for any kind of surgical resection and the appropriate timing. Oftentimes people get, people will ask about, you know, is it better if I do this before or after my delivery? What are the complications, et cetera. Obstructive anomalies are a little bit more difficult. They typically present and are time sensitive. So they will present, this is your typical, you know, primary amenorrhea workup where a young adolescent comes to the ER with severe abdominal pain. There's some imaging and they find a hematometria, hematocole post. So these are typically time sensitive because they're obstructive and obstructed and causing a lot of pain. However, in more complex obstructive anomalies, things that we'll touch on later on, you can buy some time if either the adolescent or the family is not ready for surgery. And this is where, you know, utilizing your reproductive endocrinologist or pediatric endocrinologist can be very helpful to obtain the right tools to suppress someone. Things that certainly you can use would just be the, you know, low estrogen OCP, some sort of lower estrogen at OCP. Depolupron works really well and can be really nice for adolescents as well. But that can be a nice, a nice way to bridge someone who might not be ready for a more complicated surgery that might require, require dilation, mold in the vagina, et cetera. So for obstructive mullerian anomalies, again, just in that diagram that we just reviewed, they can occur at different portions of the vagina. So many of us are very familiar with hymenal abnormalities. Certainly these can actually pose a problem for neonates. The maternal estrogen stimulation can cause this to become kind of an obstructed, an outflow obstruction or at menarche, if there's a hymenal abnormality, this will, this is kind of the prime time to cause obstruction. A transverse vaginal septum can sometimes be mistaken initially for a hymen, an imperfect hymen. They present in a very similar fashion, typically cyclical pain or primary amenorrhea. As we know, this can really occur in any portion of the vagina, low, mid position or high, which can cause confusion at the time of diagnosis, if there isn't the appropriate imaging. And then more complicated things would be things like the ovirus spectrum. So ovirus stands for obstructed hemivagina and ipsilateral renal agenesis. This is a condition which we'll get into, which certainly can delay the diagnosis because these young girls and adolescents can go quite some time with having one side of their outflow tract obstructed. But their other side is patent, which allows them to continue having menstrual cycles, which really confuses the picture. So they will get a, they won't, they usually are having a pretty large delay in diagnosis because imaging hasn't been obtained. And then there are other obstructed mullerian anomalies like non-communicating horns, which you'll run into as well, which can cause a lot of obstruction and pain. If the non-communicating horn is actually, has functional endometrium, and sometimes that can be difficult to sort out as well. So this is again, the depiction of the ovirus syndrome, which is a spectrum, but this small little diagram here just shows you exactly what we're talking about. So what is typically included in this diagnosis would be a uterine didelphys and obstructed hemivagina. And then on the, um, that same side, there is ipsilateral renal agenesis. So almost always you will find these things together. Um, and I mentioned that, um, these, um, patients typically have a delay in diagnosis because they're having menstrual cycles. So think back to that presentation, that little guinea pager that I showed everybody that clinical vignette. So this is a 15 year old that's been having cycles, but she's having this intermittent pain. Um, she might come to the ER several times before she has any imaging on the essential views of the MRI. You can see in the first plane on your left side of the screen, you can see the outline of the uterus here. So there's a hematometria and then there's a hematocle post. And then you can see this portion, um, where it does not look like there's a patent vagina. And then maybe there's some suggestion that there's a patent distal vagina. So this would be suggest maybe suggest suggestive of, um, a transverse vaginal septum or that something obstructing that hemi vagina. And then you can see in this other side, it's actually a kind of nice image. You don't see it a great image of the endometrium in this picture, but you do see a cervix, um, right here, and then you see a completely patent vagina. So this is probably her functioning side, um, which is allowing her to have a patent outflow tract on that side. This is another patient that unfortunately got all the way to 23, um, who was diagnosed with chronic pelvic pain, um, for a long time before she had imaging very similar images here. Um, so again, on the left side of your screen, you can see what appears to be a pretty normally shaped, um, uterus, a very large hematocle posts. Um, and then, uh, in these two different, um, series, you can see two uterine cavities. Again, one maybe looks a little bit bigger than the other and a large medical posts. And for this patient, um, once we took her to the operating room, she actually had kind of a perforation in the septum, which was allowing this kind of old hematometria to, and hematocle posts to drain through the other side of the vagina, which was probably there for a while. Um, given the fact that she had gotten to the age of 23, um, without suppression. Um, so what, what are the management strategies for obstructive mullerian anomalies? Um, well, they're really the excision of the obstructed septum. Um, our goal is always to provide an adequate outflow track, but at the same time, minimize future need for surgery. Um, so you'll, um, if you end up taking care of this patient population, you'll run into, um, a lot of adolescents or younger women who had an obstructive anomaly, um, at an early age presented to a hospital and underwent a surgical procedure to drain, um, the hematocle posts hematometria. And sometimes this is really just making a very small incision and not resecting the septum, which causes a lot of trouble later on. Sometimes they have a small incision made and then they have serial dilations as well. But this is fraught with a lot of trouble with scar tissue, pain, et cetera. So when we talk about the goal, we wanna provide an adequate outflow tract, but we also wanna provide a functional vagina. Without the need for additional surgical intervention. When the transverse vaginal septum is pretty thin, it's very easy to reconstruct. You can see in this little picture, we really just wanna excise the septal tissue and reconstruct the vagina just by bridging that gap. Obviously, if it's very thick or there's really not much of a distal vagina, this is where we are talking about utilizing a graft to help bridge the gap where there's no intervening vaginal tissue. Again, I mentioned that you can get tricked here pretty easily, if you're just examining a patient and take a patient to the operating room without imaging, certainly you can have a very low transverse vaginal septum that you might've thought was an imperfect hymen and even unfortunately, vaginal agenesis with a hematometria. And again, just a reminder, just avoid the temptation of IND even in an adolescent. So for non-obstructive anomalies, really I mentioned the indications are for function. So they're either for sexual function or reproductive health. Sometimes I guess one of the other things that can come up, women will say they have a lot of difficulty inserting a tampon or the tampon doesn't work because it's really only occupying one side or portion of the vagina. So those would be indications to be resecting longitudinal septum, a longitudinal septum, certainly Meyer-Rocatansy-Kuster-Hauser where an adolescent is born without a vagina would be an indication to reconstruct a vagina so that they have a functional vagina for sexual function. And then I'm gonna just kind of very briefly go over the different uterine anomalies just so that you're familiar with that because they oftentimes are present with the vaginal anomalies. So I mentioned Meyer-Rocatansy-Kuster-Hauser syndrome. This is really a spectrum of mullerian anomalies in 46 XX individuals, typically presents with vaginal agenesis and then uterovaginal agenesis. But certainly this is a spectrum. So you can, I have seen vaginal agenesis with a portion of the uterus. Most commonly this is without the development of the majority of the uterus. And it really happens due to a cessation of mullerian duct development, which is caudal to the round ligaments. Very commonly associated with other urinary and skeletal system defects. So that's really important in terms of surgical planning. You'll wanna know, and the adolescent and her family will wanna know if she has a renal anomaly at the time of diagnosis. So primary management. So I think there's a lot of debate here, but there are many ways to create a vagina. There are non-surgical ways and surgical ways. A lot of, this diagnosis requires a lot of counseling because usually people are taken aback when they're hearing this for the first time that there is no vagina. This might be an adolescent that also doesn't have a uterus. So there's a whole slew of concerns that the adolescent and her family members might have regarding reproductive strategies, outcomes, et cetera. And we talk about the creation of a functional vagina when the adolescent is ready to maintain a functional vagina. And that's when they'll be ready, either mature enough to dilate their vagina or ready to have somewhat regular intercourse. So the non-surgical methods would be pressure methods. So that diagram there is in a depiction of the simple pressure from a bicycle seat, which is the Ingram method. Believe it or not, regular intercourse is actually a method to dilate the vagina. When you look at literature that evaluates this, most often these are women or adolescents that have a pretty significant vaginal dimple to be able to attain a vaginal length with regular intercourse. But most commonly, this will just be in the form of, an adolescent will come, they'll want to try dilation. We will set them up with dilation teaching to basically dilate a vagina over the course of six months to 12 months. It requires a lot of dedication. And certainly this can seem like a large feat to an adolescent. I don't require it before moving on to surgical reconstruction, but if we do talk about and plan surgical reconstruction, we do have patients learn to dilate beforehand so that they're very well aware of what is in store for them in the future. When we talk about surgical reconstruction of vaginas, there are a variety of different methods. Some are still in use today, others really people have moved away from. Things that you'll see less commonly would be bowel vaginoplasties. There are just more complications that occur there. There are mucosal abnormalities, a lot of strictures, and then the discharge that women have with that can be quite distressing. But you'll still see a lot of the graft vaginoplasties, either buccal mucosal grafts, artificial grafts, split thickness grafts, and then surgical traction, which I have a video on the Vacchietti method, which is a great option for women that creates a very nice and natural looking vagina. So I'm going to go ahead and play that video. In this video, we'll demonstrate the surgical steps when performing a laparoscopic Vacchietti procedure. The procedure was first described in 1965 and allows for the treatment of vaginal agenesis without the use of a graft. A neovagina is called a vaginal agenesis because it's a vaginal vaginoplasty. It's a procedure where the vaginal vagina is created in seven days by exerting continuous pressure on the vaginal dimple. The patient is a 21-year-old with complete vaginal agenesis. Laparoscopic survey reveals normal bilateral ovaries and fallopian tubes. However, the uterus is absent. Retraction is placed on the pelvic peritoneum while a second surgical team places through the vaginal dimple into the abdominal cavity. To prevent inadvertent injury to adjacent structures, this step is performed using simultaneous cystoscopy and the surgeon's finger in the rectum. Next, two sutures are pulled through the vaginal dimple. We utilize an orthopedic suture called Fiber Wire. The polyethylene core provides high tensile strength while its braided jacket of polyester provides abrasion resistance. The vaginal dimple is passed through an external acrylic olive. This olive will create the neovagina by exerting continuous pressure against the vaginal dimple. Be sure to secure this in place using knots at the proximal and distal aspects of the olive. The proximal knots will facilitate removal of the olive once the neovagina is completed. While this is taking place, the laparoscopic team creates retroperitoneal channels The proximal ends of the suture are passed through the retroperitoneal tracks and are pulled out of the body through the laparoscopic incisions. This is performed bilaterally. Next, we secure the suture to the abdominal tensioning device. As it exits the body, we thread the suture through a segment of red rubber catheter to protect the tissue of the abdominal wall. A piece of foam is placed between the device and the abdomen. The sutures are secured under tension, thereby allowing the olive to exert continuous traction against the vaginal dimple. A Foley or suprapubic catheter is placed for continuous bladder drainage. The patient is admitted to the hospital. Daily vaginal exams are performed to assess progress. The device is re-tensioned by 1 to 1.5 cm each day. The device is removed and the patient is discharged home with instructions to perform daily vaginal dilation to maintain vaginal length and caliber. 3D ultrasound is becoming more popular and can really help with the diagnosis. This illustrates the difference between an HSG where you might not be able to see much more than just the contour of the endometrial cavity but in the ultrasound, you might be able to see the contour of the endometrial cavity but in the ultrasound, you can actually see where the myometrium is here and you might be able to discern what's going on, is this an arcuate uterus, etc. Unicornate uterus, these are anomalies that really can affect reproductive outcomes. They come about from failure of the development of one of the mullerian ducts. Typically, if there is not an obstructed rudimentary horn, there is no surgical management that is recommended for these anomalies. Uterine didelphys we briefly touched on with the ovira spectrum. It's almost always present in ovira. It's also almost always present in the cloacal extrophy patients and the pathophysiology is really complete failure of fusion of the mullerian ducts and normal differentiation of each to form the cervix and the hemiuterus. I think that mullerian anomalies are really more of a spectrum. The common teaching was that if there are two cervices, then it's definitely a didelphys. There is actually a really nice review on the ovira spectrum and actually bicolis, the double cervix, can really be 30% of the time it is a complete septate uterus, 30% of the time it's a bicornuate uterus and 30% of the time it is a didelphys. It really enforces that need for that high-level imaging like an MRI before you start to counsel these patients. to support any surgical treatment with a bicornuate uterus, or excuse me, a didelphys uterus. Bicornuate is just incomplete fusion of the mullerian ducts, typically at the level of the fundus, which creates two separate uterine cavities. This can be seen with one cervix. As I mentioned, it also can be seen with two cervices. There are some reproductive outcomes that metroplasty was recommended and is no longer really in favor for this anomaly. I wanted to get to some of the more complex anomalies that I have alluded to because you will see these in more increasing numbers as these infants that were cared for many, many, many years ago started to survive much longer without being completely taken care of. This is a picture of classic bladder exstrophy, which is, again, a spectrum typically seen with a low-set umbilicus. In this picture of the infant, you can see that the bladder is completely splayed open and fused with the anterior abdominal wall. You can see the internal ureteral orifices. It's always seen with a separated pubic synthesis and then typically the anus is deviated anteriorly. There are different time periods and considerations for timing the surgical repair, but in general, the goals of this repair would be adequate low-pressure urine storage. They need to reconstruct the bladder to be a storage container, if you will. Certainly, there are considerations for urinary continence to make that happen, preserving renal function. This is probably the most debated aspect about when to do it, how to do it, the development of functional and cosmetically acceptable genitalia. Cloacal exstrophy is definitely a more severe, more rare form of a midline abnormality. This is seen with an omphalocele, which you can see in the picture here, the exstrophy of the bladder, so the bladder is bivalved and then there's intervening bowel that you see in the picture here. This is also seen with an imperfect anus and a blind-ending colon. There's always a diastasis of the pubic synthesis similar to the classic bladder exstrophy. As I mentioned, it's always seen with the uterine didelphys. As you can imagine, the mons clitoris labia is located and then the vagina is either in two or displaced anteriorly. The goal of this, it's a little bit more intense and very individualized, but again, we want to have that storage facility function of the bladder, preserving renal function. There's much more consideration with what to do with the bowel, so we want, certainly the pediatric surgeons that are reconstructing this are thinking about other bowel complications and then again, this is debatable at exactly what time point to do it, but the creation of functional and cosmetically acceptable genitalia. And the timing is typically really, varies with management and the different strategies that are there to narrow and secure the pubic diastasis. So there are different osteotomies that need to be performed in order to reconstruct the hip and the sockets to make sure that the symphysis can close. I just bring this up so that you're aware that there is a need for the vagina to be created at some point in time, and sometimes this is done when the neonate is still an infant and it can be done, you know, in the very initial surgery or later on. Typically with the initial surgery, these are either a pull-through vaginoplasty or a bowel vaginoplasty or the last option would be in a patient who is not going to have, who will have a non-functioning urethra or the bladder neck will be closed and they'll have a continent stoma. The vagina can actually be reconstructed with, using the native vagina or the native bladder. I think with the bowel vaginoplasty and native bladder vagina, there are different considerations for the long-term care and safety of those. But you need to know that those are the potential options, especially in a patient who is considering fertility, might get pregnant and need a delivery or might need some sort of revision vaginoplasty. So this is where we kind of need to know all of this information. So when the patient comes back saying, you know, my vagina is strictured, I'd like to have a functional vagina, you'll need to get the notes from the pediatric surgeon, try to figure out what type of material was used for the vagina, how was this done, et cetera. Pediatric surgeons are usually pretty eloquent in their descriptions. They typically will draw a picture of exactly what they did. So getting those records can be incredibly helpful. The truth is that the majority of women with complex GU anomalies will need some sort of revision vaginoplasty or genitoplasty. And this is oftentimes required for sexual intercourse or just sexual health. That's really where this debate on when to do it kind of occurs. So many thought that this should be done right away. And now there's more of a developed kind of like an individualized pathway for parents, et cetera, to make these decisions because why would we want to do a surgery that will inevitably need to be repeated in the future if there's no need for it at this point in time? And it goes into a lot of the debates about genitalia surgery in infants and minors, et cetera. So to create a new vagina, this would be the same option for even a primary repair of somebody with vaginal agenesis, but there's certainly mac and dough, which would be just the creation of that potential space between the urinary bladder and the bowel. Typically you're utilizing some sort of graft material, either split thickness skin graft of non-hair-bearing skin or things like intercede repliform. We typically use Oasis and typically wrap an Oasis material or graft in a soft dilator, which would be left in the vagina for about a week, and then require post-op dilation. I believe I have a video up next, which shows the revision vaginoplasty. In this video, we will demonstrate the application of extracellular matrix graft in two pediatric vaginoplasty cases. The use of various biologic grafts has been described in pelvic reconstructive surgery. We utilize a graft to drive from porcine small intestinal submucosa. It will hyper porcine develop subsequent scarring and adhesions. She was taken to the operating room for resection of vaginal scar tissue, expansion of the distal vaginal canal, and vaginoplasty utilizing SIS matrix graft. Exam under anesthesia reveals a severely shortened and narrowed vagina with dense scar tissue present approximately. Total vaginal length is approximately four centimeters. The vaginal adhesions are systematically taken down with bovine cautery. We confirm adequate vaginal length and caliber. In addition, the cervix is now able to be visualized. Next, to help prevent future vaginal adhesion formation, graft material will be secured at the site of the vaginoplasty. Suture is passed circumferentially at the level of the mid vagina to serve as the attachment points for the graft. The ends of the suture are then passed through the sheet of graft material. The sutures are tied down sequentially to secure the graft in place. Next, a vaginal mold is inserted to prevent the walls of the vagina from adhering to one another. This mold also keeps the vaginal graft in place during the healing process. We create this mold. We don't have to create that mold anymore because it's back on the market. So I'm just going to speed through this just so you can see the next portion of the video. Obtained, which showed a fluid-filled upper vagina measuring approximately four centimeters across. She was subsequently taken back to the OR for resection of vaginal septum and vaginoplasty using SIS matrix graft. Examiner anesthesia reveals a fluid-filled cavity at the proximal vagina on the patient right. The location of the cavity is confirmed by aspirating fluid using a needle and a syringe. The needle is exchanged for a flexible catheter so as not to lose the opening into the cavity. Sutures are used to tag the wall of the septum and provide counter-attraction. The wall of the vaginal septum is incised open, granting access to the proximal vagina and cervix. The cut edges of the septum are sutured to the vaginal mucosa. And then similarly, the graft is placed along with the mold and kept in place for about one week. The mold was left in place for one. So I think that the next aspect is just maintaining sexual health and sexual function. So as I mentioned, reconstructive vaginoplasties, when should they occur, childhood versus later in life, the things that come along with these like scarring, stricture, the need for an adequate length for intercourse, certainly pelvic floor dysfunction can happen after these procedures are done. And then there's a lot of emotional considerations like body image that go along with taking care of these women and adolescents. So the goals are really preventing sexual dysfunction as a sequelae of the reconstructive surgery, and then preparing a woman for a healthy sexual relationship, educating women on their anatomy, and then addressing sexual function and the concerns when they arise. I think one of the things that I can't stress enough is that sometimes these young girls and adolescents really have only been managed by pediatric surgeons who are fabulous at what they do. But sometimes things like counseling for sexually transmitted infections, your Gardasil injection, all that stuff, it needs to come from somewhere. And I think that, again, with backgrounds in urogynecology, that can be a very helpful add to the team. So I mentioned that we always have patients dilate prior to any surgery. We have a nurse practitioner that really dedicates her practice to this, and we call it dilating one-on-one. So we set these expectations up pre-op. We introduce the dilating very, very soon post-op. This involves close follow-up, any kind of hand-holding that needs to be done, weekly visits. And then we always ask the patient to demonstrate self-dilation in progress. If a patient's struggling, you can pre-medicate with liocaine gel. That's really helpful. Our nurse practitioner puts these little color-coded tabs on the dilators and gives everybody a guide, and they graduate, et cetera. So it's really tailored towards a younger population. Vaginal estrogen can be pretty helpful. We use this post-feciati, and it can help with scarring and septum resections. And again, I think we're all pretty comfortable with using vaginal estrogen. And then pelvic floor physical therapy has been a great adjunct to women that are struggling with dilation. As I mentioned, with vaginal agenesis, there are some unique challenges, and these include body image, when is the adolescent or young woman kind of ready to have intercourse, the decision of surgical versus non-surgical, and then many are very apprehensive because they've either started to self-dilate or had a bad experience with self-dilating or had a lot of procedures where somebody was trying to find a vagina, and they're really, really reticent to actually do anything. So as I mentioned, anticipation of pelvic floor disorders, this is where we can kind of shine. So I have a couple of cases that outline this, and then I'm happy to take any questions. So this is a patient, this is one of our first patients. She was a 23-year-old with cloacal atrophy who spontaneously conceived, again, was told that she couldn't get pregnant, but she was still a fertile 23-year-old patient. She had many pregnancy complications, including prolapse, pyelonephritis, a small bowel obstruction, and ultimately was delivered at 34 weeks via a classic cesarean section for help. She unfortunately continued to have very symptomatic prolapse postpartum, and a pestry wasn't able to manage her symptoms. So we actually planned this surgery with the pediatric surgeon and performed an abdominal sacrocopalpexy through a midline incision. You can tell that the external genitalia, certainly there are some differences in her external genitalia, and this was a copalpexy of the prolapsed vagina because she had two hemivaginas, so it is very deviated to one side, which is exactly what you would expect with cloacal atrophy. This is also a patient who had prolapse with vaginal agenesis. This is a patient who actually presented after self-dilating. She self-dilated and was able to have vaginal intercourse, but then ended up developing prolapse. She gave a very vague history of having some kind of surgical procedure on her kidney. So we obtained a CT eurogram, and as you can see, this was very helpful in planning her surgery. You can see she has a horseshoe kidney. And when we go to the sagittal view, you'll see exactly, it sits right over the sacrum. This is not a patient you would want to be doing a sacrocopalpexy in, unless you had a lot of vascular help. So you can see that kidney is sitting right over the promontory, and more importantly, her great vessels are right there as well. So this patient had a sacrospinous. Then we have erectovaginal fistula. I mentioned some complications that can arise after the initial surgeries for transverse septum. This is a 22-year-old that had come back with bleeding per her rectum, only with her period. She described a history of a transverse vaginal septum excision, which was complicated with hysterotomy for hematoclopos and hematometria. There was a rectal injury at the time, which was repaired. And then unfortunately, at the age of 17, she had erectovaginal fistula, which she was symptomatic from, and had two subsequent repairs. We had gotten some imaging, and as you can see on this image here, this is a satchel view with gel in the vagina, and you can see that it communicates to a portion of the rectum. So she was able to have a robotic rectovaginal fistula repair and vaginoplasty with an Oasis graft, which took care of her symptoms. I don't think we have time for that one video, so I'm just gonna go on to a couple of the reproductive concerns. So obviously, with vaginal agenesis and myorhepotency Kuster-Hauser, pregnancy can be achieved through IVF. This is really important to note that in most cases, the individual would need a gestational carrier, and certainly there are cost considerations, which vary by state. IVF can also be very challenging, secondary to the location of the ovaries. It can be very high in the pelvis, and if there is no vagina, the procedure might need to be performed through an abdominal oocyte retrieval. Everyone asks about uterine transplants, so it's kind of helpful to know where we stand on this. It's been a long time in the making, but in 2018, the first live birth occurred from a deceased donor. It's a very complicated surgery, takes many, many hours. There are some skilled surgeons that are setting up centers around the country. So if there's a patient that's really interested in this, I would send her for a consult at one of these skilled centers, but this is really a very emerging technology. And then in terms of reproductive outcomes, I mentioned that sometimes these women and adolescents aren't really fully counseled about their reproductive potential. They can get pregnant. So this was a small series of 52 women over 35 years. They all underwent a primary bladder closure surgery, so in infancy. Many of these women required an augmentation cystoplasty. About 20% had concomitant pelvic osteotomies to help close that pubic symphysis. 54, just about half of them had a continence stoma. Most underwent vaginoplasty and entroitoplasty, and 81% were sexually active. Out of the women who attempted pregnancy, 19 were successful. So 28 attempted and 19 were successful. The majority required ART, so they had some help in this. Unfortunately, these pregnancies and deliveries can be really complicated. I think I draw everybody's attention that these were all delivered by a cesarean section and should be. So if there's anything that we can help with, it's to advocate for a cesarean section at the time of delivery with a very skilled surgeon. Obviously, you'd like to know what kind of surgeries these patients had in reconstruction, but even in this small case series, there was a ureteral transection and one ureterocutaneous fistula. So you're really thinking about urologic complications that can occur at the time of these deliveries. So to close, my take-home points are that GU anomalies are common and they affect sexual health, fertility, and the pelvic floor. The care really should involve a multidisciplinary approach for focusing on the transition from a pediatric to an adult provider. As I mentioned, urogynecologic providers are really well-suited to act as this bridge and orchestrate a team around a woman to meet her needs. And I would just encourage everybody to anticipate the obstacles that these young adolescents and women face. So with that, I'd like to thank everybody and I am happy to take any questions. Thank you, Dr. Mueller, for a great, great talk. So informative. And we have our first question here. How early should that transition between the pediatric doctor and the urogynecologist occur? So when should we, as urogynes, take over some of the care of these children with anomalies? So there's a lot of data on this, not in this subset of our studies, not in this population, but in other pediatric surgery populations of when someone is ready to be kind of transitioned to an adult provider. Because I will say that pediatric care is much more focused around the patient and it's very patient-centered. I think we do a great job as adult providers, but it's really a different world. So I think it's very dependent on the kind of setup as a urogynecologist that you have. So if you're having someone come to a dedicated clinic that's really taking care of these patients and holistic and has a reproductive endocrinologist, a physical therapist, a sexual counselor, a psychologist, that can probably be transitioned very early. If you're just incorporating this into your urogynecologic practice, I would probably offer to see the patient, but maybe not completely take over that transition until the patient and her family are a little bit more ready to transition. And there are certain transition checklists to make sure that they understand the diagnosis, they understand what they need to do, they understand what kind of medical records they need to have, they understand the vaccinations they need to have, et cetera. And there are some great guidelines in the pediatric surgery for that. Thank you. It's great, thank you. I think I agree, like as urogynes, we're probably gonna see some of these kind of recurrences or complications for revisions, things like that. I'm just curious in your experience when you've done these revisions, what kind of complications have you seen? Like what should we be worried about if we're using like a graft some of the procedures that you were mentioning for these revisions? Kind of what are the things to look out for? I think the complications that occur, and one of the videos I had was a complication that occurred in a pediatric patient that underwent a septum resection by a pediatric surgeon when she was like 11 or 12. And it's the things that we're all kind of, we all learn about. So resecting too much of the vagina and getting either a bladder or a bowel injury would probably be the worst complication. So we've seen fistula from those types of complications, but more commonly it's really stricture. So stricture and need for revision surgery. So that's why I mentioned there's really, if you're not at a tertiary care center and there is a surgeon who's gonna take care of a pediatric patient with some sort of obstruction, their knee-jerk reaction might just be to drain that area and make a small incision. But the likelihood of that sustaining even an outflow tract for a long time is low. And the likelihood of needing a revision surgery for either a re-obstruction or pain or stricture or inability to use a tampon, et cetera, is very, very high. So those are typically the types of complications that we see, which can be avoided by just taking kind of a multidisciplinary approach to these patients. I have one more question. Just with the Vickie Eddy procedure, it's such an elegant procedure. It just looks kind of nice to perform. Is that kind of the go-to procedure right now or are there other ones that you kind of decide upon as you're thinking about these surgeries for eugenicists? I mean, I think that's a great question. And there is some literature looking at outcomes, so specifically outcomes in vaginal eugenicists with respect to the different types of surgeries. And honestly, most of them are pretty similar. I think that if you look at the literature, the kind of surgical traction procedures typically tend to have the least amount of complications, which make them an attractive option for a kind of first shot. You would never be able to do that on someone who's already had a surgical procedure. So it's really kind of that, you know, first time where you're able to utilize that. There are instances where we're able to, there's a Wharton Shears method where we're able to identify the two dimples in the, they're like teeny tiny, they almost look like Skeen's glands and dilate those and then place a mold. So it's kind of a surgical dilation. And that's a really nice way to create a vagina. And there are some benefits, like you don't need to hospitalize the patient for a week. You'll keep the mold in for a week. They don't have to do the kind of the crank, which might cause a little anxiety in a younger patient. So that can be a really nice option as well. Okay, well, on behalf of Ops, I'd like to thank Dr. Mueller and everyone for joining. Be sure to register for the upcoming webinars. There's two of them, October 25th, which is Incorporating Pelvic Ultrasound into Your Practice by Dr. Choubary. And then December 21st, Tis the Season for Good Relaxation, How to More Effectively Treat Individuals with Chronic Constipation. And that will be by Darren Brenner. So how else to ring in, you know, holidays with a little bit- Make sure to tune in. Yeah, exactly. Enjoy. Thanks again. Thanks, Taji. Thanks, everybody. Have a good one.

congenital genitourinary anomalies

urogynecologist

anomalies affecting genital and urinary systems

management and surgical options

multidisciplinary approach

transition from pediatric to adult care

reproductive and sexual health concerns

surgical techniques

complications and revision surgeries