# Colorectal Quiz Episode 13: Newborn ARM Part 2 — GCMD Library

Surgeons, neonatologists, pediatricians, and anyone who cares for the newborn child needs to understand how to evaluate anorectal malformations. To clear things up, Dr. Marc Levitt and Dr. Jason Frischer explain how to make sense of various ARM presentations in this 3 part series. Featuring Amanda Jensen

Type: podcast · 15 min · posted 2021-06-14 Canonical: https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142 ## Chapters - [0:00](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=0) Introduction and Episode Context - [1:21](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=81) Case Review: Images 1 and 2 - [5:46](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=346) Case Review: Images 3 and 4 - [9:35](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=575) Cloaca Recognition - [12:38](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=758) Physical Examination Technique - [14:41](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=881) Closing and Summary ## Statements - "Perineal fistula requires surgery when the hole is too small, not in the center of the sphincter, and there is an inadequate perineal body" — Marc Levitt (clinical) [2:34](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=154) - "Surgical indications for anorectal malformation include: locating the perineal fistula, determining if it is in the correct location, assessing if it is too big or too small, confirming it is within the sphincter, and evaluating the size of the perineal body" — Rod Gerardo (clinical) [2:54](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=174) - "Perineal groove typically does not require surgical intervention and will keratinize to look like a normal perineal body over time" — Jason Frischer (clinical) [4:45](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=285) - "Surgical indication for perineal groove exists only if it is secreting mucus, causing irritation, or developing ulcers, where mucosal lining could be excised" — Jason Frischer (clinical) [5:00](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=300) - "Congenital perineal groove has a normal anal opening and is an exposed wet sulcus of non-keratinized mucous membrane that usually epithelializes on its own by age two" — Amanda Jensen (clinical) [6:10](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=370) - "An adequately sized hole that appears surrounded by sphincter with a peroneal body, albeit short, requires no surgery" — Marc Levitt (clinical) [7:14](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=434) - "The peroneal body will grow over time and there is nothing to do about a short peroneal body when other anatomical features are correct" — Marc Levitt (clinical) [8:01](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=481) - "If half the fistula is within the sphincter complex and half is outside, the patient will leak stool because they cannot close the hole, making surgery worthy to relocate the hole" — Marc Levitt (clinical) [8:39](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=519) - "Three qualities to assess in anal location are: anal size, location (whether surrounded by sphincter muscle complex), and the peroneal body that separates it from the introitus or urinary structures" — Amanda Jensen (clinical) [9:11](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=551) - "Vestibular fistula is very common in females and needs a formal repair with the hole transposed to the center of the sphincter" — Jason Frischer (clinical) [9:35](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=575) - "For vestibular fistula diagnosed in the newborn period, primary repair can be done if the baby is well, or the baby can stool through the fistula for a couple of months followed by elective operation without a stoma after bowel prep" — Jason Frischer (clinical) [10:22](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=622) - "Patients diagnosed with vestibular fistula at 6, 8, or 12 months of life who have dilated their rectosigmoid need diversion as the first step, then repair" — Jason Frischer (clinical) [10:22](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=622) - "Diversion in vestibular fistula repair is to try to avoid perineal body dehiscence" — Jason Frischer (clinical) [10:22](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=622) - "Recto vestibular fistula has three openings: the urethra, the vagina, and a fistula within the vestibule" — Amanda Jensen (clinical) [11:12](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=672) - "A baby with no anal opening and a single perineal orifice has a cloaca" — Marc Levitt (clinical) [11:25](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=685) - "The hypertrophied area around the clitoral hood in cloaca is fairly typical and is not ambiguous genitalia" — Marc Levitt (clinical) [11:25](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=685) - "Cloaca patients have no endocrine problem, do not need steroids, and do not need an endocrinologic workup" — Marc Levitt (clinical) [11:25](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=685) - "There is no question of gender assignment in cloaca - it is a female" — Marc Levitt (clinical) [11:25](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=685) - "Cloaca patients are still being misdiagnosed as ambiguous genitalia, with some babies not having proper gender assignment for a week or two" — Marc Levitt (clinical) [11:25](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=685) - "Urogenital sinus plus a normal anus is an endocrine problem, but no anus and a urogenital sinus is a cloaca" — Amanda Jensen (clinical) [12:22](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=742) - "Good lighting and good visualization are needed for perineal exam in newborn females, with magnification tools like loops helpful for seeing small holes" — Jason Frischer (clinical) [12:43](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=763) - "The key to perineal examination is to push down and flatten the perineal body to see if it is normal or not" — Marc Levitt (clinical) [13:26](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=806) - "Hagar dilators should be used to check anal size, starting low and working up for accurate measurement, rather than using fingers because every surgeon has a different size glove" — Rod Gerardo (clinical) [13:38](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=818) - "To evaluate for vestibular fistula, use both hands on the right and left labia, pulling the labia toward you and opening them to visualize the vaginal opening and look for the urethra" — Jason Frischer (clinical) [14:11](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=851) - "The biggest question with female anorectal malformation exam is how many perineal orifices are there" — Amanda Jensen (clinical) [15:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=903) - "If there are three orifices, the question is whether it is a perineal fistula or a vestibular fistula" — Amanda Jensen (clinical) [15:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=903) - "If there are two orifices, it is important to know if there is a fistula at all, vaginal atresia, or a rectal vaginal fistula" — Amanda Jensen (clinical) [15:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=903) - "If there is only one orifice, this is a cloaca" — Amanda Jensen (clinical) [15:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-13-newborn-arm-part-2-4142?t=903) ## Transcript 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. Frischer and Dr. Lovett are talking about them. So download the app. Until then, enjoy the episode. So 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 Levitt from Children's National Hospital out in DC. All right. I put four photos side by side. We'll describe each one, but quite intentionally. And because I think they're a little nuanced and the treatment or non-treatment is different for each of these cases. So if you're listening to this and 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. Levitt and Dr. Frischer 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. Frischer, tell me about this first photo. So the first photo all the way on the left demonstrates, you can see 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 can 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 peroneal 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 peroneal 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 peroneal body. And those are the indications for surgery. So in summary, things to ask yourself when examining a patient with an anorectal malformation. Number one. Can you locate the peroneal fistula? Numero dos. 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 peroneal 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 peroneal 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 peroneal groove. Tell me more about this peroneal 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. Whether you do anything about that peroneal groove, that mucosal lined peroneal 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 peroneal body over time. Is there any indication to operate on a peroneal groove? Is if it's secreting a lot of mucus, causing irritation, developing ulcers in that area, where you could excise that mucosal lining. But typically it is extremely rare. And usually with some good local wound care, like you're treating a rash. Okay. That's awesome. So you can avoid a surgery, but then what about the actual peroneal 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 peroneal body alone to become epithelium 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 peroneal 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 mucosally lined groove between the vagina and the anal opening. So with the second image, remember congenital peroneal groove, the anal opening is normal and is essentially an exposed wet sulcus of non-keratinized mucous membrane. And usually this epithelializes on its own by age two, and it can be misdiagnosed as contact dermatitis, trauma, or even sexual abuse. All right. Now I really wanted to rock everyone's world with the third photo here. Yeah. Photo three is a tough one. 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 peroneal 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 Hagar. It appears to be surrounded by sphincter, and there appears to be a peroneal 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. Frischer. When we discussed earlier, you had mentioned one of the indications for operation is short and peroneal body. But if the other pieces are right or correct, I don't think you could fix that. Yep. That peroneal 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 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'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 peroneal body. How do you counsel that family? I think that if you don't have a sphincter on the anterior aspect of the analplasty, they will leak stool because they won't be able to close the hole. So that is worthy of surgery. Specifically relocating the hole because it may have an effect on the child's continence, and you might not even notice it. Depends on what the patient's doing, if they're sneezing, playing sports, et cetera. 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 peroneal 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's, see, it looks like there's a vagina and hymenal tissue. The urethra is right above it. It's, I can tell you for sure it's normal. And yes, I had 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 to the center of the sphincter. And there are a couple of 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 repair and reconstruction standpoint and timing of that? I think if you make this diagnosis in the newborn period, I would do either a primary repair in the newborn period of the baby's well, or let the baby stool through that fistula for a couple of months, bowel prep them, and have to 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 six, eight, 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 dehiscence. So yeah, I would do this in the newborn period without a protecting stoma. So as stated with this last image, it's a recto vestibular 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 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 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 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 an external light, because it's a little dark in these units sometimes. And these are small holes that you're trying to see. Nowadays, I use my loops to tie my sneakers. Oh, okay. Someone needs to get Dr. Levitt some new glasses. But in the meantime, scroll down to the media player. We put a video on how to do this exam. Dr. Levitt'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 Hagar dilators. Start low, work your way up, get an accurate measurement. Don't use your fingers because every surgeon has a different size glove. This patient in particular actually did not have an anorectal myoformation, 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. Frischer 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 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 anal rectal malformations. The biggest thing with this exam is how many perineal orifices 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, a vaginal atresia or a rectal vaginal 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 Stay 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. --- Not medical advice · citation policy: https://library.globalcastmd.com/ai