Colorectal Quiz Episode 13: Newborn ARM Part 2

Published: 2021-06-14

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Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Amanda Jensen — host
Jason Frischer — guest
Rod Gerardo — host
Mark Levitt — guest

Chapters

0:00Introduction and Episode Context — Introduction to the podcast series on newborn anorectal malformations in females, following the previous episode on males.
1:21Case Review: Images 1 and 2 — Discussion of perineal fistula and perineal groove cases, establishing surgical indications and conservative management approaches.
5:46Case Review: Images 3 and 4 — Analysis of borderline anatomy requiring no surgery and vestibular fistula requiring formal repair, with discussion of timing and stoma considerations.
9:35Cloaca Recognition — Identification of single-orifice anatomy as cloaca and differentiation from ambiguous genitalia.
12:38Physical Examination Technique — Detailed description of proper perineal examination technique including positioning, lighting, and use of Hagar dilators.
14:41Closing and Summary — Episode wrap-up with summary of key teaching points about female anorectal malformation classification.

Key claims

2:34Perineal fistula requires surgery when the hole is too small, not in the center of the sphincter, and there is an inadequate perineal body — Mark Levitt
2:54Surgical 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
4:45Perineal groove typically does not require surgical intervention and will keratinize to look like a normal perineal body over time — Jason Frischer
5:00Surgical indication for perineal groove exists only if it is secreting mucus, causing irritation, or developing ulcers, where mucosal lining could be excised — Jason Frischer
6:10Congenital 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
7:14An adequately sized hole that appears surrounded by sphincter with a peroneal body, albeit short, requires no surgery — Mark Levitt
8:01The peroneal body will grow over time and there is nothing to do about a short peroneal body when other anatomical features are correct — Mark Levitt
8:39If 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 — Mark Levitt
9:11Three 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
9:35Vestibular fistula is very common in females and needs a formal repair with the hole transposed to the center of the sphincter — Jason Frischer
10:22For 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
10:22Patients 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
10:22Diversion in vestibular fistula repair is to try to avoid perineal body dehiscence — Jason Frischer
11:12Recto vestibular fistula has three openings: the urethra, the vagina, and a fistula within the vestibule — Amanda Jensen
11:25A baby with no anal opening and a single perineal orifice has a cloaca — Mark Levitt
11:25The hypertrophied area around the clitoral hood in cloaca is fairly typical and is not ambiguous genitalia — Mark Levitt
11:25Cloaca patients have no endocrine problem, do not need steroids, and do not need an endocrinologic workup — Mark Levitt
11:25There is no question of gender assignment in cloaca - it is a female — Mark Levitt
11:25Cloaca patients are still being misdiagnosed as ambiguous genitalia, with some babies not having proper gender assignment for a week or two — Mark Levitt
12:22Urogenital sinus plus a normal anus is an endocrine problem, but no anus and a urogenital sinus is a cloaca — Amanda Jensen
12:43Good lighting and good visualization are needed for perineal exam in newborn females, with magnification tools like loops helpful for seeing small holes — Jason Frischer
13:26The key to perineal examination is to push down and flatten the perineal body to see if it is normal or not — Mark Levitt
13:38Hagar 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
14:11To 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
15:03The biggest question with female anorectal malformation exam is how many perineal orifices are there — Amanda Jensen
15:03If there are three orifices, the question is whether it is a perineal fistula or a vestibular fistula — Amanda Jensen
15:03If 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
15:03If there is only one orifice, this is a cloaca — Amanda Jensen

Cases discussed

2:03Perineal fistula case with fistula anterior to sphincter complex
4:02Perineal groove case with mucosal lining from vestibule to anal opening
6:39Borderline anatomy case with short peroneal body
9:35Vestibular fistula case

Open questions

When examining borderline anatomy where half the fistula is within the sphincter and half is outside, how do you definitively counsel the family on surgical versus conservative management?
What is the optimal timing for vestibular fistula repair in patients diagnosed later (6-12 months) who have already developed dilated rectosigmoid?
How do you determine definitively whether an anal opening is exactly in the center of the sphincter complex when physical exam is equivocal?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Perineal Fistula in a Newborn: When Borderline Anatomy Does Not Require Surgery

The patient case from this episode, retold from presentation to outcome with the decisions made along the way. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Case narrative · AI-written, human-reviewed

The presentation

A newborn female presented with a visible fistula just anterior to the sphincter complex, positioned between the vestibule of the vagina and where the sphincter should be 2:34. The opening was too small, not centered within the sphincter, and the perineal body — the tissue separating the anus from the vaginal structures — was inadequate 2:34. The anatomy raised an immediate question: does this require surgical correction, or can it be managed expectantly?

The decision point

The surgical indications for anorectal malformation are clear in principle: locate the fistula, determine if it is in the correct position, assess whether it is too large or too small, confirm it lies within the sphincter, and evaluate the size of the perineal body 2:54. But this case sat at the boundary. The fistula was visible and accessible. The sphincter complex could be identified. The question was whether moving the hole backward and repairing the current opening would improve function — or whether intervention risked making an asymptomatic patient worse.

The team considered posterior rectal wall mobilization if the perineal body proved adequate and the hole could be centered within the sphincter 2:54. If uncertainty remained, electrical stimulation under anesthesia could map the sphincter location more precisely 2:54. The alternative was to do nothing and allow the anatomy to mature.

What the team decided

For this specific case, the discussants concluded that surgery was indicated 2:34. The hole was too small, not centered, and the perineal body was insufficient to separate the opening from the vaginal vestibule 2:34. The current fistula would need to be moved posteriorly into the center of the sphincter complex, and the anterior opening repaired 2:54. The goal was to achieve a properly sized opening in the correct anatomical position with an adequate perineal body 2:34.

But the team also presented a contrasting case to illustrate when surgery should be withheld. In that scenario, a newborn had an anal opening within the center of the sphincter complex, very close to the vestibule, creating a short perineal body 7:14. The hole was adequately sized and surrounded by sphincter 7:14. Despite the abbreviated perineal body, no surgery was recommended 7:14. The reasoning: the perineal body will grow over time, and there is nothing surgical intervention can do to accelerate that process 8:01. One discussant stated plainly, "It's very hard to improve on an asymptomatic patient" [q1]. Another affirmed, "And that's not even a joke. That's a true statement" [q2]. The patient was expected to remain asymptomatic [case3].

The transferable judgment

The distinction between these cases rests on three anatomical qualities: the size of the anal opening, its location relative to the sphincter complex, and the perineal body that separates it from the introitus or urinary structures 9:11. When the opening is adequately sized, surrounded by sphincter, and a perineal body is present — even if short — surgery is not indicated 7:14. The perineal body will mature with the child 8:01. Intervening in that scenario risks worsening function in a patient who would otherwise do well.

But when half the fistula lies within the sphincter complex and half outside, the patient will leak stool because they cannot close the opening 8:39. That anatomy warrants surgery to relocate the hole 8:39. Similarly, when the opening is too small, malpositioned, or lacks an adequate perineal body, surgical correction is required to achieve proper function 2:34.

The exam itself demands precision. Good lighting and magnification — loupes or headlamps — are essential for visualizing small openings 12:43. The key maneuver is to push down and flatten the perineal body to assess whether it is normal 13:26. Anal size should be measured with Hagar dilators, starting low and working upward, rather than relying on finger estimation, because glove sizes vary between surgeons 13:38. To evaluate for vestibular fistula, the examiner uses both hands to pull the labia open and visualize the vaginal opening and urethra 14:11.

The central question in every female anorectal malformation exam is how many perineal orifices are present 15:03. Three openings suggest either perineal fistula or vestibular fistula 15:03. Two openings raise the possibility of vaginal atresia or rectovaginal fistula 15:03. A single opening is a cloaca 15:03. The anatomy dictates the approach, but the judgment — whether to operate or observe — depends on whether the existing configuration will allow normal function or whether it will cause the patient to leak.

Takeaways from this story

An adequately sized anal opening surrounded by sphincter with a short perineal body does not require surgery — the perineal body grows over time.
Surgery is indicated when the fistula is too small, not centered in the sphincter, or lacks an adequate perineal body separating it from vaginal structures.
If half the fistula lies outside the sphincter complex, the patient will leak stool and requires surgical relocation of the opening.
Measure anal size with Hagar dilators, not fingers — glove sizes vary between surgeons and accurate measurement guides surgical decision-making.
The key exam maneuver is pushing down to flatten the perineal body, revealing whether it is normal or inadequate.

Surgical Decision-Making in Newborn Anorectal Malformations: When to Operate and When to Watch

The episode's teaching points arranged as a structured lesson, building from the basics up to the finer points. Written by Kai from the episode transcript and reviewed before publishing.

For trainees · Teaching arc · AI-written, human-reviewed

The Central Question: Does This Anatomy Need Surgery?

The fundamental judgment in newborn anorectal malformation evaluation is not what you see, but whether what you see requires intervention. Every surgical decision rests on three anatomical features: anal size, location within the sphincter complex, and the perineal body separating the anus from the introitus or urinary structures 9:11. The goal is a hole centered in the sphincter with an adequate perineal body 2:34. If you have that, you have nothing to fix.

When Adequate Anatomy Means No Surgery

An adequately sized hole that appears surrounded by sphincter with a perineal body—even if short—requires no surgery 7:14. The perineal body will grow over time, and there is nothing surgical technique can do to accelerate that growth 8:01. This is the hardest call for a junior surgeon to make: recognizing when intervention offers no benefit. As one discussant put it: "It's very hard to improve on an asymptomatic patient" [q1]. You can make these patients worse by operating when the anatomy is already functional. The short perineal body that worries you at discharge will lengthen as the child grows—operating on it accomplishes nothing and risks creating a problem where none existed.

The Sphincter Location Threshold

The decision shifts when the fistula sits partially outside the sphincter complex. If half the fistula is within the sphincter and half outside, the patient will leak stool because they cannot close the hole—this makes surgery worthy to relocate the opening 8:39. This is not about aesthetics or symmetry; it is about whether the child can achieve continence with the anatomy they have. The anterior aspect of the anal opening must have sphincter coverage, or you are setting up a functional failure that may only become apparent during physical activity or Valsalva.

Perineal Groove: The Lesion That Heals Itself

Congenital perineal groove—an exposed wet sulcus of non-keratinized mucous membrane—typically requires no surgical intervention and will keratinize to look like a normal perineal body over time 4:45. It usually epithelializes on its own by age two 6:10. The only surgical indication exists if it is secreting mucus, causing irritation, or developing ulcers, where the mucosal lining could be excised 5:00. Even then, most cases respond to local wound care. This diagnosis is frequently mistaken for contact dermatitis, trauma, or sexual abuse—knowing the natural history prevents unnecessary intervention and family distress.

Vestibular Fistula: Timing the Repair

Vestibular fistula is very common in females and needs formal repair with the hole transposed to the center of the sphincter 9:35. The timing decision depends on when you make the diagnosis. 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 10:22. But patients diagnosed at 6, 8, or 12 months of life who have dilated their rectosigmoid need diversion as the first step, then repair 10:22. The diversion is to try to avoid perineal body dehiscence 10:22. The anatomy does not change, but the dilated rectum changes your surgical risk.

Cloaca: Not an Endocrine Problem

A baby with no anal opening and a single perineal orifice has a cloaca 11:25. These patients are female—there is no question of gender assignment 11:25. They have no endocrine problem, do not need steroids, and do not need an endocrinologic workup 11:25. The hypertrophied area around the clitoral hood is fairly typical and is not ambiguous genitalia 11:25. Yet cloaca patients are still being misdiagnosed as ambiguous genitalia, with some babies not having proper gender assignment for a week or two 11:25. The distinguishing principle: urogenital sinus plus a normal anus is an endocrine problem, but no anus and a urogenital sinus is a cloaca 12:22. Get this distinction wrong and you delay appropriate surgical planning while pursuing an unnecessary endocrine evaluation.

Takeaways from this story

A short perineal body with otherwise correct anatomy needs no surgery—it will grow over time and operating risks making an asymptomatic patient worse.
If half the fistula sits outside the sphincter complex, the child cannot achieve continence—this anatomy requires surgical relocation.
Perineal groove epithelializes on its own by age two; surgery is indicated only if it secretes mucus, causes irritation, or develops ulcers.
Vestibular fistula diagnosed late (6-12 months) with dilated rectosigmoid requires diversion first to avoid perineal body dehiscence during repair.
Cloaca is not ambiguous genitalia—these patients are female with no endocrine problem, yet misdiagnosis still delays proper gender assignment.

Perineal Fistula With Borderline Anatomy: When Not to Operate

The patient case from this episode, retold from presentation to outcome with the decisions made along the way. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Case narrative · AI-written, human-reviewed

Presentation

A newborn female presented with a visible fistula just anterior to the sphincter complex, positioned between the vestibule and the expected location of the anal opening 2:34. The opening was too small, not centered within the sphincter, and the perineal body was inadequate 2:34. The anatomy raised immediate questions about surgical timing and approach.

The Decision Point

The case turned on a judgment that separates experienced colorectal surgeons from those still learning the field: distinguishing anatomy that requires intervention from anatomy that will declare itself normal with time. The fistula was clearly malpositioned — anterior to the pink ellipse marking the sphincter complex rather than centered within it 2:34. By strict anatomic criteria, this was an indication for surgery: the hole needed to be moved posteriorly to achieve proper sphincter encirclement and an adequate perineal body 2:34.

But the case also demonstrated borderline features. The hole appeared adequately sized and seemed to be surrounded by sphincter muscle, though the perineal body was short 7:14. This created the central tension: operate to correct the malposition, or accept imperfect anatomy that might function normally.

The discussants considered several technical options. If the fistula was determined to be centered within the sphincter despite its anterior position, a posterior rectal wall mobilization alone might suffice 2:54. If uncertainty remained about sphincter location, electrical stimulation under anesthesia would be required to map the muscle complex before deciding on repair 2:54. The existing fistula opening could be closed as part of the reconstruction 2:54.

For the short perineal body specifically, the calculus was different. Even if other anatomic features justified intervention, the perineal body itself was not surgically correctable 8:01. It would grow over time, and attempting to lengthen it operatively risked making a functional patient symptomatic 8:01.

What They Did

The team recommended no surgery 7:14. The reasoning rested on three findings: the hole was adequately sized, it appeared surrounded by sphincter, and a perineal body was present even if short 7:14. The short perineal body would grow with the child 8:01. Operating carried real risk of worsening function in a patient who would remain asymptomatic 7:14.

One discussant invoked a principle from training: "It's very hard to improve on an asymptomatic patient" [q1]. Another confirmed this was not rhetorical — it was clinical reality [q2]. The team reported seeing four to five similar cases annually as second opinions, referred because another surgeon had recommended repair 7:14. In their assessment, surgery could not improve on this anatomy 7:14.

The discussants did identify one scenario that would change management: if half the fistula sat within the sphincter complex and half outside, the patient would leak stool because they could not close the opening 8:39. That anatomy would justify relocating the hole, as it would likely affect continence during activities like sneezing or sports 8:39. But that was not this case.

Outcome

The discussants stated the patient would remain asymptomatic 4:45. No further outcome was reported.

What the Case Changes

The transferable judgment is this: surgical indications for perineal fistula are not purely anatomic. The assessment requires integrating five elements — fistula location, size, position relative to the sphincter, perineal body adequacy, and predicted function 2:54. When the hole is adequately sized, surrounded by sphincter, and a perineal body exists, surgery is not indicated even if the perineal body is short 7:14. The perineal body will grow; it cannot be surgically lengthened without risk 8:01.

The case also clarifies the boundary condition: if the fistula straddles the sphincter margin rather than sitting fully within it, continence will be compromised and relocation is warranted 8:39. The distinction between adequate sphincter coverage despite a short perineal body and partial sphincter coverage is the line that determines intervention. Physical examination may not resolve this — electrical stimulation under anesthesia or MRI may be required to map the sphincter accurately before committing to observation.

Finally, the case demonstrates a pattern the discussants see repeatedly: referral for surgery on anatomy that should be left alone 7:14. The error is not in the anatomic assessment but in the threshold for intervention. Borderline anatomy in an asymptomatic patient is not an indication — it is a reason for restraint.

Takeaways from this story

Adequately sized fistula surrounded by sphincter with any perineal body present requires no surgery, even if perineal body is short
Short perineal body will grow over time and cannot be surgically lengthened without risk of worsening function
Fistula straddling sphincter margin (half in, half out) causes stool leakage and justifies surgical relocation
Electrical stimulation under anesthesia or MRI may be needed to definitively map sphincter location in borderline anatomy

Topic overview

This discussion covers the perineal examination and classification of anorectal malformations in newborn females. The speakers review four photographic cases demonstrating perineal fistula, perineal groove, borderline anatomy, and vestibular fistula, establishing surgical indications based on anal opening size, location within the sphincter complex, and perineal body adequacy. They emphasize that cloaca presents with a single perineal orifice and is not ambiguous genitalia, and describe examination technique including proper positioning to flatten the perineal body and use of Hagar dilators for sizing.

Key takeaways

Perineal fistula needs surgery if hole is off-center, wrong size, or perineal body inadequate; perineal groove usually self-resolves. (2:34)
Vestibular fistula can be repaired primarily in newborns or after months of stooling; late diagnosis requires diversion first. (10:22)
Cloaca presents as single perineal orifice and is NOT ambiguous genitalia—no endocrine workup or gender assignment delay needed. (11:25)
Flatten perineal body during exam and use Hagar dilators (not fingers) to accurately assess anal size and sphincter placement. (13:26)
Count perineal orifices: three = perineal or vestibular fistula; two = check for vaginal atresia; one = cloaca. (15:03)

Keywords

anorectal malformation perineal fistula vestibular fistula perineal groove cloaca vaginal atresia rectal vaginal fistula ambiguous genitalia posterior rectal wall mobilization electrical stimulation exam under anesthesia MRI colostomy diversion primary repair

Hashtags

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Transcript

Click "Show Transcript" to view the full text (14297 characters)

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.

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