Hey there listeners, this is Amanda Jensen, and Rod Gerardo from Cincinnati Children's. Have you downloaded the new version of the State Current Pediatric Surgery app? It's in the Google Play Store and in the Apple App Store. The reason I bring it up is that if you're listening to this podcast within the app, you can bring up all images that we discussed today in real time while Dr. Frisher and Dr. Levit are talking about them. So download the app. Until then, enjoy the episode. Last week, we talked about the newborn anorectal malformation exam in a male baby. It's a fantastic episode. If you haven't listened to it yet, jump out of this one, go listen to last week's episode, look at the associated images in the State Current Pediatric Surgery app. Then, come back to this episode. We're going to talk about newborn ARMs in the female baby. How to do them? This is a topic that is not just for pediatric surgeons, but I would argue pediatricians, neonatologists, pediatric gastroenterologists, anyone who has the opportunity to care for newborns. This is the podcast for you. So, without further ado, here's Dr. Mark Levit from Children's National Hospital out in DC. All right, I um, I put four photos side by side. We'll describe each one, but quite intentionally, because I think they're a little nuanced and the treatment or nontreatment is different for each of these cases. So if you're listening to this in anything other than the State Current Pediatric Surgery app, that's okay, but if you're in the app, scroll down under the media player, you can look at all of the images that Dr. Levit and Dr. Frisher are about to talk about. Now, if you can't see the images, don't worry, we got you covered. We're going to describe them anyway. All right, Dr. Frisher, tell me about this first photo. So the first photo all the way on the left demonstrates, you could see a the pink ellipse we talk about, which is the sphincter complex where we anticipate where the center of the muscle is located and where the opening should be located. Now, just anterior to that, you could see a fistula. It looks like basically the vestibule of the vagina to the sphincter complex or at least where the sphincter is supposed to be. And I would call that a perineal fistula. I think the principles here are there's a hole, the hole is too small, the hole is not in the center of the sphincter, and there's an inadequate perineal body. What are the indications for surgery? So, you must achieve that. You must achieve a hole in the center of the sphincter with an adequate perineal body. And those are the indications for surgery. So in summary, thanks to ask yourself when examining a patient with an anorectal malformation. Number one, Can you locate the perineal fistula? Numero dose. Is it in the correct location? Door number three. Is it too big or too small? Four. Is it within the sphincter? And last but not least. And what is the size of the perineal body? But this particular hole, I believe is just outside the ellipse, which is the sphincter and therefore really needs to be moved back. The current hole could then be repaired to distinguish it from a situation where the hole is right at the top of the ellipse. Now, how do you do that? In theory, you could do just a posterior rectal wall mobilization, if you conclude that the perineal body is adequate, and the hole is centered by the sphincter. And if you're not sure, then you have to do an electrical stimulation. Now, that would require an exam under anesthesia. Now, let's look at the second image. Again, if you're in the State Current Pediatric Surgery app, scroll under the media player, open up the image number two. A little bit tougher to see. It looks to me that there's a mucosal lining going from the vestibule of the vagina towards the anal opening. So to me that looks like a perineal groove. Tell me more about this perineal groove. With the anal opening a little bit hard to tell if it's really within the muscle complex or again sort of incorporated within the anterior part of the muscle complex but not completely in. And that's where the actual real life physical exam could really help out. This comes up a lot. Um, whether you do anything about that perineal groove, that mucosal lined perineal body. What do you advise? Usually does not require any surgical intervention. My conversation with the family often involves that this will keratinize and look like a normal perineal body over time. Is there any indication to operate on a perineal groove? Is if it's secreting a lot of mucus causing irritation, developing ulcers in that area, um, where you could excise that mucosal, um, lining. But typically, it is extremely rare and usually with some good local wound care, um, like you're treating a rash. Okay, that's awesome so you can avoid a surgery, but then what about the actual perineal fistula aspect of this? That particular hole is also abutting the edge where the sphincter stops and starts. It might be contained within the ellipse. I'd have to check on stimulation, but it might be amenable to a posterior rectal wall mobilization only, leaving the perineal body alone to become epithelium uh down the road. Yeah, I will say this is an interesting picture because it's a little hard to tell if that opening is exactly in the center of the sphincter complex. Most perineal bodies I see and you get consulted a few times a year on this. The anus is in the proper position and you just see this mucosal lined, Yeah. Uh, groove between the vagina and the anal opening. So with the second image, remember, congenital perineal groove. The anal opening is normal and is essentially an exposed wet sulcus of non-keratinized mucus membrane. And usually this epitheliazes on its own by age two. And it can be misdiagnosed as contact dermatitis, trauma or even sexual abuse. All right, now I I I really wanted to uh rock everyone's world with the third photo here. Yeah, photo three is a tough one. That that could be a conversation in and of itself. All right guys, just tell us what do you see? A anal opening that looks to be within the center of the sphincter complex, also appears to be very close to the vestibule, thereby making a short perineal body. Now, again, you probably need to do a real physical exam for this or do an exam under anesthesia. Or the other test you could do is an MRI. Then you could just see if the rectum is correctly exiting the sphincter. You know, back to our indications for surgery, to me, that is almost definitely an adequately sized hole. Of course, you can check it with a hager. It appears to be surrounded by sphincter and there appears to be a perineal body, albeit short and therefore no surgery in my book for this patient. And I see four or five of these a year that someone as a second opinion, I see them because someone decided they wanted to do surgery and you can't get better than this. There's nuance to this. Here's Dr. Frisher. When we discussed earlier, you had mentioned one of the indications for operation is short perineal body, but if the other pieces are right or correct, I don't think you could fix that. Yep, that that perineal body is going to grow over time and there's absolutely nothing to do about it. You can actually make the patient worse. One of our professors like to say and we we trained at the same place, Jason and I, the great Mount Sinai in New York, it's very hard to improve on an asymptomatic patient. And this is a patient that will remain asymptomatic. And that's not even a joke. That that's a true statement. Yeah, well, I mean, most of what I try to say is true and serious. All right, jokes aside here. Let's change it up with a little bit of a hypothetical. Half the fistula appears to be within the sphincter complex. Half of the fistula is outside. You have a decent perineal body. How do you counsel that family? I I think that if you don't have a sphincter on the anterior aspect of the anaplasty, they will leak stool because they won't be able to close the hole. So that is uh worthy of surgery, specifically relocating the hole because it may have in effect on the child's continents and you might not even notice it depends on what the patient's doing if they're sneezing, playing sports, etc. All right, let's get back to image number three. Amanda, can you kind of wrap it up? For this third image, when considering surgery, remember the three qualities to anal location, namely the anal size, the location, AKA whether or not it's surrounded by the sphincter muscle complex, as well as the perineal body that separates it from the introitus or urinary structures. Thank you. All right, now let's do image number four. Yes, the fourth picture, another girl. It looks like you have a no anal opening within the muscle complex area, but there appears to be a fistula within the vestibule of the vagina and then there see it looks like there's a vagina and hyminl tissue. The urethra is right above it. It's I can tell you for sure it's normal. And yes, I would agree this is what I would call a vestibular fistula, very common in females and needs a formal repair, that hole needs to be transposed uh to the center of the sphincter. Um, and there are a couple ways to accomplish that. Hey Mark, when you see this patient, are you thinking colostomy or what's your approach when you see this from a from a repair and reconstruction standpoint and timing of that? I think if you make this diagnosis in the newborn period, I would do a either a primary repair in the newborn period if the baby's well or let the baby stool through that fistula for a couple of months, bowel prep them and have have do that operation electively without a stoma. However, recognizing that we have an international audience here, a lot of these patients are diagnosed much later. They come in at 6, 8, 12 months of life. They've dilated up their rectosigmoid. That's a patient I think needs a diversion as the first step then a repair. Ultimately your diversion is to try to avoid perineal body uh dehiscence. So yeah, I would I would do this in the newborn period without without a protecting stoma. So as stated with this last image, it's a rectovestibular fistula. It has three openings, the urethra, the vagina and a fistula within the vestibule. Awesome. All right, let's move on to the next image. This baby obviously has no anal opening, but there's only a single perineal orifice and that is a cloaca. And that little um hypertrophied area around the clitoral hood on the right photo is fairly typical and it is not, and I repeat not ambiguous genitalia. This patient has no endocrine problem, does not need steroids, does not need an endocrinologic workup. This is a cloaca. There's no question of their gender assignment, it's a female. And unfortunately, these patients are still even today being misdiagnosed as ambiguous genitalia and some of these babies I've met um have not had proper gender assignment for a week or two while they're trying to figure it out. Okay, so basically urogenital sinus plus a normal anus, that is an endocrine problem. But no anus and a urogenital sinus, that's a cloaca. So in summary, what do all three of these images have in common? They all have one opening and are all variations of cloaca. All right, so how do you do a perineal exam in the newborn female? I think there are some keys and and we're we're going to go into the nitty-gritty, but there's some basics. You need good lighting, you need good visualization. There's nothing wrong with bringing your loops up there to take a look if you have loops or anything that can magnify the image and getting good lighting, whether you wear a headlight or bringing a an external light because it's a little dark in these units sometimes and and you these are small holes that you're trying to see. Now nowadays I use my loops to tie my sneakers. Um Oh, okay. Someone needs to get Dr. Levit some new glasses, but in the meantime, scroll down under the media player, we put a video on how to do this exam. Dr. Levit's going to walk you through it. You really want to push down and flatten the perineal body. That is the key. You want to see if the perineal body is normal or not, and then you want to check the anal size. By that, he means use hager dilators. Start low, work your way up, get an accurate measurement, don't use your fingers because every surgeon has a different sized glove. This patient in particular, actually did not have an anorectal malformation and you can see that in the video because the anus is centered within the sphincter, the anus is of adequate size and the perineal body is of normal length properly distanced from the vestibule. One thing that Dr. Frisher noticed was not in the video. How do you evaluate for a vestibular fistula? Using both my hands, my right and left hand on the right and left labia and pulling the labia towards me and opening the labia a little bit to get try to get a good visualization of if you're looking for a vestibular fistula, looking at the vaginal opening and looking for the urethra. So, what if the exam still leaves you with some questions? You're still not sure what the exact diagnosis is, then you got to move on to radiologic evaluation. Open up the next image. There's an image on the left and there's an image on the right and both of them show a nice air column. So, what say you? What say you? Well, I say we're actually out of time. We can't talk about these cross table laterals this week. You're going to have to wait until next week. So, let's close it out. Amanda, give us a summary of today's episode. In summary, we learned about female anorectal malformations. The biggest thing with this exam is how many perineal orfices are there? If there are three, the question is, is this a perineal fistula or a vestibular fistula? If there are two, it's important to know is there a fistula at all, uh vaginal atresia or a rectovaginal fistula. And if there's only one orifice, this is a cloaca. Beautifully stated. Once again, I'm Rod and I'm Amanda from Cincinnati Children's. If you missed out on the images or the video for this podcast, make sure to download the State Current Pediatric Surgery app. It's in the Apple App Store, it's in the Google Play Store. But until then, remember, knowledge should be free.
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