Colorectal Quiz Episode 32: Anorectal Malformations And Cardiac Anomalies

Published: 2022-05-30

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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

Rod Gerardo — host
Mark Levitt — guest
Jason Frischer — guest
Megan Durham — guest

Chapters

0:00Introduction and guest reveal — Podcast introduction, discussion of the Stay Current app, and reveal of guest Dr. Megan Durham from Children's Hospital of Atlanta.
2:07Case presentation: ARM with tetralogy of Fallot — 39-week male neonate with prenatal tetralogy of Fallot diagnosis, postnatal finding of anorectal malformation with perineal fistula and meconium along scrotal raphae.
5:23Management options: dilation vs. colostomy vs. primary repair — Discussion of whether to dilate the fistula, perform colostomy, or proceed with primary repair in the setting of significant cardiac disease and hypercyanotic spells.
8:29Workup findings and laparoscopic colostomy technique — Renal anomalies, sacral dysplasia, and VCUG findings presented. Laparoscopic colostomy performed with low insufflation pressures and cardiac anesthesia support.
11:21Cardiac repair and definitive PSARP — Emergent tetralogy repair on day of life five, followed by PSARP at three months. Discussion of prognosis and surgical technique for fistula straddling the sphincter complex.
14:23Technical pearls and closing — Debate on mobilization of anterior rectal wall when fistula is partially within sphincter complex, joke, and podcast sign-off.

Key claims

2:07Tetralogy of Fallot was diagnosed prenatally in this patient — Megan Durham
3:00Prenatal echocardiogram revealed tetralogy of Fallot with large VSD, bidirectional shunt, moderate pulmonary valve stenosis, right ventricular outflow tract obstruction, mild right ventricular hypertrophy, and very small PDA — Megan Durham
3:53The patient had a perineal fistula with meconium visible along the scrotal raphae — Mark Levitt
4:06Presence of an anal dimple, raised area, and good color change suggests a good sphincter — Mark Levitt
5:23A perineal fistula with external opening is one of the less complicated anorectal malformation lesions — Megan Durham
5:23Primary repair in the neonatal period is optimal for perineal fistula in a baby without cardiac defect — Megan Durham
6:32Dilation without operating room intervention is possible for perineal fistula, especially in females with vestibular fistula — Mark Levitt
6:32In males, perineal fistula dilation is more dangerous because the opening is near the urethra — Mark Levitt
7:24In cardiac patients with external ARM opening, dilation is typically performed as long as evacuation is adequate — Jason Frischer
7:48Concern exists about healing of ARM repair in blue babies with significant cardiac lesions requiring early surgery — Jason Frischer
8:33Patient had cross-fused ectopia of left kidney with normally positioned right kidney — Megan Durham
8:33Patient had conus at L2, which is normal — Megan Durham
8:33Patient had sacral dysplasia with foreshortened sacrum — Megan Durham
8:54VCUG showed small diverticulum along right bladder base, otherwise normal — Rod Gerardo
9:04VCUG is obtained if there are renal anomalies — Megan Durham
9:14Patient had significant tetralogy spells with desaturation to 60s when crying during dilation attempts — Megan Durham
9:14Laparoscopic colostomy was performed on day of life two — Megan Durham
9:43A turnable loop ostomy (95-5 percentage loop) behaves like an end colostomy but allows distal contrast studies — Mark Levitt
10:23For cardiac babies, laparoscopic insufflation pressures are started at 8 mmHg — Megan Durham
10:23Irrigating the distal rectum preoperatively helps keep insufflation pressures low during laparoscopy — Megan Durham
10:50If umbilical line is present, Palmer's Point access with Hasson technique is an alternative to umbilical access — Mark Levitt
11:39Patient's PDA completely closed postnatally — Rod Gerardo
11:56Patient required emergent tetralogy of Fallot repair on day of life five due to persistent hypercyanotic spells — Megan Durham
12:19PSARP was performed approximately three months after cardiac repair — Rod Gerardo
12:33Low anorectal malformations (perineal fistula) should have good continence prognosis — Mark Levitt
12:33Continence depends on sensation in anal canal, absence of dentate line, spine anatomy, type of ARM, and sacral anatomy — Mark Levitt
12:33Sacral ratio measurement should wait until three months of age — Mark Levitt
13:36Half of this patient's perineal fistula opening was anterior to the muscular complex, requiring formal repositioning into the center of the anal muscular complex — Megan Durham
13:56The fistula tract along the median raphae was left alone because the perineal fistula did not extend up into the raphae itself — Mark Levitt
14:10The fistula tract is only one millimeter deep and should not be aggressively pursued surgically — Mark Levitt
14:23When fistula opening is 50-50 (half within, half anterior to sphincter complex), leave the anterior wall and mobilize posteriorly to achieve 80-20 reconstruction — Jason Frischer
14:57If fistula is completely outside the sphincteric ellipse, full mobilization is required — Mark Levitt

Cases discussed

2:0739-week male neonate with prenatal tetralogy of Fallot and postnatal diagnosis of perineal fistula ARM

Open questions

Is colostomy creation more or less stressful than a one-hour mini-PSARP in a neonate with significant cardiac disease?
At what age should sacral ratio be measured in neonates with ARM?

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:

Tetralogy of Fallot and Perineal Fistula: When Cardiac Spells Force the Decision

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

Presentation

A 3050-gram male neonate presented with prenatally diagnosed tetralogy of Fallot and a postnatal finding of anorectal malformation 2:07. The prenatal echocardiogram had revealed a large VSD with bidirectional shunt, moderate pulmonary valve stenosis, right ventricular outflow tract obstruction, mild right ventricular hypertrophy, and a very small PDA 3:00. On perineal examination, the infant had an absent anus but a visible anal dimple with good color change — findings suggesting intact sphincter anatomy 4:06. White beads of meconium were visible along the scrotal raphae, and a small meconium smear confirmed an external opening: a perineal fistula 3:53.

The VACTERL workup revealed cross-fused ectopia of the left kidney with a normally positioned right kidney, conus at L2 (normal), and sacral dysplasia with foreshortening 8:33. VCUG showed a small diverticulum along the right bladder base but was otherwise unremarkable 8:54.

The Decision Point

A perineal fistula with an external opening is among the least complicated anorectal malformations 5:23. In an otherwise healthy neonate, primary repair in the neonatal period would be optimal 5:23. In a female with a vestibular fistula, or even in some males with perineal fistulas, dilation without operating room intervention is a reasonable alternative — the opening can be serially dilated with Hegar dilators to allow stool evacuation, deferring definitive repair until the cardiac situation stabilizes 6:32. In males, this approach carries slightly more risk because the fistula opening lies near the urethra, but it remains feasible with careful technique 6:32.

The question facing the team was whether to dilate and wait, perform a brief primary repair (essentially a one-hour mini-PSARP), or create a diverting colostomy. The cardiac lesion complicated the calculus. One discussant noted that in cardiac patients with an external ARM opening, dilation is typically performed as long as evacuation remains adequate 7:24. Another raised concern about wound healing in a cyanotic infant who might require early cardiac surgery 7:48. If the baby was going to be a "blue baby" with poor oxygenation, would an ARM repair heal properly?

The decision was made for the team by the patient himself. During attempts at dilation, the infant had significant tetralogy spells with desaturation into the 60s when crying 9:14. Dilation was not going to be tolerated. A laparoscopic colostomy was performed on day of life two 9:14.

What They Did

The team created a turnable loop ostomy — a 95-5 percentage loop that behaves like an end colostomy but allows distal contrast studies through the smaller limb 9:43. For a cardiac patient, laparoscopic technique required modification: insufflation pressures were started at 8 mmHg 10:23, and the distal rectum was irrigated preoperatively to decompress the bowel and help keep insufflation pressures low 10:23. (If an umbilical line had been present, Palmer's Point access with Hasson technique would have been an alternative to umbilical entry 10:50.)

Postnatally, the PDA closed completely 11:39. Despite medical management, the infant continued to have hypercyanotic spells and required emergent tetralogy of Fallot repair on day of life five 11:56. Three months after cardiac repair, the team performed a PSARP 12:19.

At operation, half of the perineal fistula opening was found to lie anterior to the muscular complex, requiring formal repositioning into the center of the anal sphincter 13:36. The fistula tract along the median raphae — those white beads visible on the initial exam — was left alone, as the perineal fistula itself did not extend up into the raphae 13:56. The tract is only one millimeter deep and should not be aggressively pursued surgically 14:10. The surgical principle applied here: when the fistula opening is 50-50 (half within, half anterior to the sphincter complex), leave the anterior wall intact and mobilize posteriorly to achieve an 80-20 reconstruction 14:23. If the fistula had been completely outside the sphincteric ellipse, full mobilization would have been required 14:57.

What Happened

The patient tolerated both the cardiac repair and the PSARP well. The outcome was not discussed in detail, but the expected prognosis for continence is good 12:33. Continence depends on sensation in the anal canal, absence of the dentate line, spine anatomy, type of ARM, and sacral anatomy 12:33. This was a low lesion — a perineal fistula closely approximated to the anal muscular complex — and sacral ratio measurement, deferred until three months of age, would help refine the prognosis 12:33.

What the Case Changes

The plan to dilate was sound. The physiology overruled it. When a neonate with significant cardiac disease cannot tolerate the stress of a bedside procedure, the decision tree collapses. A colostomy is not more or less risky than a brief primary repair, but it buys time — and in this case, it allowed the cardiac team to operate on day five without the added variable of an open perineal wound. The transferable judgment: in ARM patients with major cardiac lesions, the default pathway may be dilation or early repair, but the patient's physiologic tolerance — not the algorithm — determines what is actually possible.

Takeaways from this story

Perineal fistula dilation is feasible in cardiac patients, but hypercyanotic spells during the procedure may force colostomy.
Laparoscopic colostomy in cardiac neonates: start insufflation at 8 mmHg and irrigate the distal rectum preoperatively.
When a perineal fistula opening is 50% anterior to the sphincter, leave the anterior wall and mobilize posteriorly (80-20 repair).
The superficial fistula tract along the median raphae (visible as white beads) should not be aggressively pursued — it is 1 mm deep.

Managing Perineal Fistula in a Neonate with Tetralogy of Fallot

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

When Cardiac Disease Changes the ARM Algorithm

A newborn with perineal fistula and tetralogy of Fallot forces a decision tree most colorectal surgeons rarely traverse: does the cardiac lesion mandate colostomy, or can you dilate and defer? The discussants opened with the ideal scenario — dilation without operating room intervention — but this patient's hypercyanotic spells during attempted dilation closed that path 9:14. The teaching moment begins where the textbook answer fails.

Perineal fistula is the least complicated ARM, but cardiac instability redefines "complicated." In an otherwise healthy neonate, primary repair in the first days of life is optimal 5:23. When the patient desaturates to the 60s with crying 9:14, that option evaporates. One discussant emphasized the concern about wound healing in a blue baby requiring early cardiac surgery 7:48. The question becomes whether a one-hour PSARP is less physiologic stress than a colostomy — and whether either operation is necessary at all. The consensus leaned toward colostomy when dilation proved intolerable, accepting two trips to the operating room rather than risking a fresh anoplasty in a cyanotic infant who might need emergent cardiac repair within days.

Laparoscopic technique in the cardiac neonate demands pressure discipline. For this population, insufflation starts at 8 mmHg 10:23. Preoperative distal bowel irrigation reduces intra-abdominal contents and helps maintain low pressures 10:23. If an umbilical line is present, Palmer's Point access with Hasson technique avoids the umbilicus entirely 10:50. The turnable loop ostomy — a 95-5 percentage loop that behaves like an end colostomy but retains a small distal limb for contrast studies — gives functional diversion without sacrificing diagnostic access 9:43.

Timing the definitive repair hinges on cardiac stability, not a fixed interval. This patient required emergent tetralogy repair on day of life five 11:56. PSARP followed approximately three months later 12:19. The discussants did not suggest a minimum waiting period after cardiac surgery; they waited for clearance from cardiology. The sacral ratio measurement also waits until three months of age 12:33, so the prognostic conversation with the family remains incomplete until that point.

Anterior rectal wall mobilization makes the fistula tract disappear — aggressive dissection does not. When half the perineal fistula opening sits anterior to the sphincter complex and half within it, the instinct to chase the tract along the median raphae is wrong 13:36. The tract is only one millimeter deep 14:10. The correct move is to leave the anterior wall alone and mobilize posteriorly, shifting the opening into the center of the sphincter complex to achieve an 80-20 reconstruction 14:23. One discussant stated it plainly: "Do not dive in and try to find that fistula's tract. It will disappear, provided you have a good anoplasty with a good anterior rectal wall mobilization" 14:10. If the fistula is completely outside the sphincteric ellipse, full mobilization is required 14:57.

Continence prognosis in perineal fistula depends on factors beyond the fistula itself. The discussants listed the determinants: sensation in the anal canal, absence of the dentate line, spine anatomy, type of ARM, and sacral anatomy 12:33. This patient had sacral dysplasia with a foreshortened sacrum 8:33, which complicates the prognosis despite the low lesion. The presence of an anal dimple, raised area, and good color change suggests a good sphincter 4:06, but the sacral ratio at three months will carry more weight than the perineal exam at birth.

The case underscores a principle that applies beyond ARM: when two organ systems are critically ill, the one that can kill the patient fastest dictates the sequence. The heart won. The bowel waited.

Takeaways from this story

In cardiac neonates with perineal fistula, start laparoscopic insufflation at 8 mmHg and irrigate the distal bowel preoperatively to minimize hemodynamic stress.
When a perineal fistula opening straddles the sphincter (50-50), mobilize posteriorly rather than dissecting the anterior wall near the urethra.
The fistula tract in perineal fistula is only 1 mm deep and disappears with proper anterior rectal wall mobilization—do not chase it surgically.
A turnable loop ostomy (95-5) functions like an end colostomy for diversion but retains a small distal limb for contrast studies without a separate stoma.

Topic overview

A male neonate with prenatally diagnosed tetralogy of Fallot presented with an anorectal malformation—specifically a perineal fistula with meconium visible along the scrotal raphae. The discussants debate management options: dilation versus colostomy versus primary repair, weighing cardiac risk against surgical intervention. The patient developed hypercyanotic spells requiring emergent cardiac repair on day of life five, after which a laparoscopic colostomy was performed at low insufflation pressures. At three months, a posterior sagittal anorectoplasty was completed; the fistula opening straddled the sphincter complex, requiring partial mobilization. The case illustrates decision-making when significant cardiac anomalies complicate ARM management.

Key takeaways

Perineal fistula dilation in males risks urethral injury; cardiac patients with tet spells may need colostomy first. (6:32)
Laparoscopic colostomy in cardiac neonates: start insufflation at 8 mmHg, irrigate distal rectum to minimize pressure. (10:23)
When perineal fistula opening straddles sphincter 50-50, mobilize posterior wall only to achieve 80-20 reconstruction. (13:36)
Definitive PSARP for low ARM can be safely delayed 3 months post-cardiac repair in cyanotic heart disease patients. (11:56)

Keywords

tetralogy of Fallot anorectal malformation perineal fistula ventricular septal defect pulmonary valve stenosis right ventricular outflow tract obstruction right ventricular hypertrophy patent ductus arteriosus cross-fused renal ectopia sacral dysplasia bladder diverticulum hypercyanotic spells echocardiogram voiding cystourethrogram laparoscopic colostomy

Hashtags

#AnorectalMalformations #PediatricSurgery #CongenitalHeartDisease #VACTERLAssociation #PediatricCardiology #CongenitalAnomalies #ColorectalQuiz #ColorectalSurgery

Transcript

Click "Show Transcript" to view the full text (15039 characters)

Hey everyone, this is Rod Gerardo from Cincinnati Children's, and before we get into this colorectal quiz episode, I wanted to let you know that whether you are watching us on YouTube, listening to us on Apple Podcasts, Spotify, Stitcher, or Amazon Music, really the best way to get the colorectal quiz is on the Stay Current Pediatric Surgery app. It's free, it's in the Apple App Store, it's in the Google Play Store. I tell you this because Dr. Levitt, Dr. Frischer, and their guests always talk about images, articles, guidelines, all sorts of stuff that we want to give you. We can give it to you on the app, you can click on it, read it while you're listening to the podcast, it's the best way to go. Download it today, but until then, enjoy the episode. It's been a while since we've done one of these, I forgot what you look like, Mark. Handsome as ever. Same old me. Alright, let's get started. That was Dr. Mark Levitt from Children's National and Dr. Jason Frischer from Cincinnati Children's Hospital. Now, Dr. Levitt is a huge fan of the podcast Smart List. I mean, I listen to it too, but they always introduce the guests in kind of like a reveal fashion. So Dr. Levitt wanted to try that today. Let's see if you can guess who it is before he's done with the introduction. A special guest from the great city of Atlanta, Children's Hospital of Atlanta, in fact. She's one of the leaders of the consortium, Pediatric Colorectal Learning Consortium, and has been very actively involved in the research mission of that fantastic organization. And a wonderful clinician herself. So we'd like to welcome Megan Durham to the colorectal quizzes. Megan, thank you so much for joining us. Well, thanks for the invitation, Mark and Jason. I'm looking forward to this. It should be a lot of fun. And thanks for all the collaboration over all the years as well. Tell me that you guessed it was Dr. Durham. If you didn't, that's fine. I'm just, I'm a fan and she's done a lot of other podcasts with us. She came with a case. Let's jump right into it. 39 week gestation little boy. He was born to a 34 year old mother. So first time mother, G1P0. Birth weight. 3,050 grams. The APGAR scores were eight and nine at one in five minutes. No significant prenatal history. Some medications mom had been taking. Nothing really significant, but there is one thing. This baby was prenatally diagnosed with tetralogy of fallot. Maybe we'll just stop there before you tell us more. Cardiology involvement early and them knowing about this baby prenatally is where to start. Yeah. Great point, Dr. Frischer. Now remember Dr. Durham is in Atlanta, Georgia. Whenever a baby in Georgia gets diagnosed with a prenatal cardiac disease, Sibley, that's our group, gets called pretty early and they get involved. And involvement means going over the echocardiogram, meeting with high risk OB. This patient's echocardiogram revealed the following. He had a tetralogy flow with a large VSD, bidirectional shunt. They did see moderate pulmonary valve stenosis and what they thought was an out, right ventricular outflow, tract obstruction. So prenatally, there's already mild right ventricular hypertrophy. I guess of one other note, they'd notice a very small PDA prenatally. Now before the pediatric cardiologist gets too carried away here, keep in mind this is a podcast on pediatric colorectal disease. So at the outside hospital, they realized there was not only a problem with the heart, but they noticed that there was a missing anus on the rest of their physical exam. That's fantastic that they noticed. We're already ahead of the game here. Actually, the topic of missed ARM, that's a totally other podcast altogether. You can see a photo now of the newborn baby and the exam. Scroll down under the media player, click on the first image. I see a male baby with no anal opening. But there is an anal dimple. That means... A raised area and a good change in color. Which means there's probably a good sphincter. What else do you see, Dr. Lovett? And then I see these tiny little white beads in the scrotal rephae. Now, if you listen to this podcast regularly, you've heard all the episodes, you probably know what we're getting at there. But before we dive too deep into all of these findings, Dr. Frischer had a question. Do you automatically get an echo on every one of your anorectal malformation babies? Let me say that I don't stand in the way when the NICU gets an echo. I routinely get them all. I do try to stay consistent with all the prenatal screening and make sure I kind of check the box. But keep in mind, you're listening to three pediatric colorectal experts at like major hospital centers. So the reason Dr. Frischer brings it up is because... We have international listeners and people with less resources available. And it could take sometimes moving a mountain to get an echo at some hospitals. So I think a good physical exam and a good listen to the heart with a stethoscope can bypass that need if necessary. All right. Let's get back to the image. Dr. Durham, what are the options? With the pearls along the median rephae and going up on the scrotum. And then in the picture, you can see a small smear of meconium. So you know the lesion that you're dealing with, the interrectal malformation lesion you're dealing with, is one of the less complicated lesions. We know that there is an opening somewhere along that perineal body. So, you know, you could ideally do a primary repair on this patient in the neonatal period. There also would be an option to potentially send the baby home and dilate. But I think most likely primary repair would be the optimal choice. You said that's a suboptimal choice in a baby with no cardiac defect. But I think it's important to know how to do that because it might be a good choice in a baby who you don't want to take to the OR. Now, Dr. Durham's hospital takes care of a lot of PEDS-CARDS patients. So for them, pediatric patients who have an ARM and a really significant cardiac anomaly. An ostomy is probably the standard choice. There's always discussions, but I think that's a standard choice. I think we have to talk about two things. One is, does the baby need to go to the OR? Perhaps if you could dilate this patient, you don't need to go to the OR at all. Let them deal with the heart. Especially if this is a female patient. There's no rush on a vestibular. There's no rush on a perineal. In a male, the perineal hole isn't always so easy to see. And the dilation is a little bit more dangerous because it's near the urethra. But with care and Hagar dilators, you can definitely get egressive stool and never go to the OR. The second thing he wants to address is that if a patient has a really significant cardiac defect... Is creation of a colostomy a less stressful operation than a one-hour mini PSAR for a perineal fistula? Because think about it, then you're saving trips to the operating room, right? Well, here's what Dr. Frischer says. In a cardiac patient that has an external opening from an anorectal malformation, we typically dilate. As long as they're evacuating okay, I think a perineal fistula or a vestibular fistula in a female, one can consider just dilating, making sure you have good evacuation, and letting the cardiac situation play itself out. Now, when you're deciding, you know, is it dangerous to do a colostomy? Is it dangerous to do, like Dr. Levitt said, a one-hour PSAR? Well... My concern would be if you're going to have a significant cardiac lesion that's going to likely require early surgery, you have a blue baby. You're having a lesion that a patient isn't oxygenating well. And then I worry about the healing of that repair. And Dr. Levitt agreed. So I actually don't think there's a need to do a colostomy in a baby like this. You can dilate and then do the repair primarily later. And I don't think the colostomy is any riskier or more or less risky, I should say, All right, let's go through the rest of the bacterial workup for this patient. And the patient had a cross-fused ectopia of the left kidney with a normally positioned right kidney, had a conus at L2. Reminder, that's normal. Now, there is also some reported sacral dysplasia. It does look a bit of a foreshortened sacrum. I think the baby's obviously too young to calculate a sacral ratio. You can look at the image with Dr. Levitt. Scroll down under the media player. Open up that picture. The VCUG showed a small diverticulum along the right bladder base, otherwise normal. Now, does Dr. Durham always get a VCUG? I do if there's renal anomalies. Now, for this particular patient, Dr. Durham decided to move on with colostomy on day of life too. Well, I think our preference would have been to dilate. But this baby tended to have significant TET spells. And initially, the cardiologists were expecting to have cardiac repair around six months of age. But every time that they would try to dilate, the baby would cry and then desat down to like the 60s. So we went to the OR and did perform a laparoscopic ostomy and decided to create a turnable loop ostomy for the baby. And what's the benefit of that? Sort of a 95-5 percentage loop. It behaves like an end. And no one knows except for you that there's another side there you can do a contrast. They had pediatric cardiac anesthesia there. The baby already had a PICC line, so they didn't need to put an extra venous access. And then for arterial, they had an ART line to help monitor the baby. Kept the pressure below and just identify the site and then maybe tolerate it just fine. Can you share with us some tricks you have for a baby like this in laparoscopy? What pressures do you set it at? Where do you put your ports? How do you do it quickly? So, I mean, we initially always start out with cardiac babies around a pressure of eight, if we can get away with it. This baby also, because the anus was able to be irrigated, we were able to irrigate the bottom and evacuate some stool, which probably helped keep pressures low. So, she started at the umbilicus, keeping the pressures low, she found where she wanted to put the ostomy and then strategically placed the ports based on that. I always start on the babies at a flow rate of one. I'm probably a little more slow than others, and sometimes I'll increase it. Now, if the baby did have an umbilical line of some sort. Maybe go into Palmer's Point and not access through the umbilicus. That would be another option and do more of a Hassan technique just to get in the belly. In for umbilical for sure. Dissecting in with a mosquito, making sure you're in, not touching any vessel before you insufflate, and also clear the line of air. Now, Dr. Frischer wants to emphasize that it is important to irrigate as much as you can, and Dr. Levitt actually has a really interesting technique to help irrigate distally. Dr. Tim Jackson Looked in laparoscopically as I passed the tube into the distal segment and did the irrigation, watching laparoscopically. So, then you create a laparoscopic ostomy, which everyone on the call really loves. The baby's doing great. Now, we have to continue with, you know, watching the heart. Follow-up echoes. The PDA completely closed. The baby continued to have hypercyonotic spells. Dr. Tim Jackson And unfortunately, regardless of all of the different maneuvers that they attempted, this baby proved to the team that he needed a heart surgery sooner than later. Dr. Tim Jackson On day of life, five, they went ahead and did an emergent petrology flow repair. Dr. Tim Jackson So, the baby did well with the heart surgery. Now what? Dr. Tim Jackson At what time do you go and do your definitive repair? Dr. Tim Jackson So, this baby actually did really well postoperatively. So, they gave us the okay to go ahead relatively quickly. Dr. Tim Jackson So, in about three months, they were going to do a PSARP. But, you know, you got to talk to the family. Dr. Tim Jackson What did you tell this family relative to future bowel control potential? Dr. Tim Jackson This baby has a low lesion. So, basically, just had a closely approximated perineal fistula to the anal muscular complex. So, should do really well. Talk to the family about sort of what's important for continence, which is sensation in the anal canal and absence of the dentate line. Dr. Tim Jackson What's the weight of the spine? Dr. Tim Jackson What's the weight of the anorectal malformation? What's the weight of the sacrum? And, of course, we'll wait until three months to measure the sacral ratio. Dr. Tim Jackson Dr. Tim Jackson Did you open up that fistula along the median RAFA? Dr. Tim Jackson Half of the perineal opening was anterior to the muscular complex. So, we did actually formally move the entire opening back into the center around the anal muscular complex. Dr. Tim Jackson Did you open up that fistula along the median RAFA? No, we left that alone, sorry, because the perineal fistula, it did not extend up into the ref A itself. I've seen teenagers and young adults with those beads that never went away. So, yeah, you've got to scrape that off. It's one millimeter deep. Do not dive in and try to find that fistula's tract. It will disappear, provided you have a good anoplasty with a good anterior rectal wall mobilization. You're saying if you have a 50-50, right, half of perineal, half of the fistula is within the muscle complex, half is just anterior to the muscle complex. Leave that anterior wall because that's the danger zone. You'll still have a little bit of your anal opening outside the sphincter complex. Mobilize enough posteriorly so that you fill the anal sphincter and then complete it. So you become like a 80-20 in your reconstruction. But if the fistula is completely outside of the sphincteric ellipse, then you have to do a full mobilization. Well, I think this is awesome, Mark. Megan, great case. Don't worry. We're not going to leave you without a joke. In fact, this time it comes from our guest. So how is a tapeworm and the Eiffel Tower similar? How is a tapeworm and the Eiffel Tower similar? Similar. You got me. They're both parasites. Ah, that's good. That is legitimate and of worth to this podcast because it's as bad as anything else we talk about. Fantastic. There you have it. Complex cardiac anomalies in the setting of an anorectal malformation. How do you make those tough clinical decisions? What do you do surgically and non-surgically? If you love this episode, if you don't love this episode, whatever, just let us know. Leave a comment whether you're watching us on YouTube, listening to us on Apple Podcasts, Spotify, Stitcher, or Apple Music. Or, my favorite way, the State Current Pediatric Surgery app. Download it today. You're going to love it. But until then, I'm Rod from Cincinnati Children's. And remember, knowledge should be free. I'm going to let you. I'm with you. I'm I'm a my next one. I'm a not- I'm a you, I'm a I. I'm a I. I'm a I. I'm a I. I'm a I. I'm a I. I'm a I. I. I. I.

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