# Colorectal Quiz Episode 32: Anorectal Malformations And Cardiac Anomalies — GCMD Library Podcast Episode · Stay Current in Pediatric Surgery · May 30, 2022 · 17m Type: podcast · 16 min · posted 2026-07-29 Canonical: https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857 ## Chapters - [0:00](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=0) Introduction and Guest Reveal - [2:07](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=127) Case Presentation: Neonate with TOF and ARM - [5:24](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=324) Management Decision-Making in Cardiac ARM - [8:33](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=513) Workup, Colostomy, and Cardiac Repair - [12:10](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=730) Definitive Repair and Prognosis ## Statements - "In Georgia, when a baby gets diagnosed with prenatal cardiac disease, the cardiac group gets called early and gets involved, including reviewing echocardiograms and meeting with high-risk OB" — Rod Gerardo (clinical) [2:44](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=164) - "Missed anorectal malformations are a topic for a separate podcast" — Megan Durham (opinion) [3:53](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=233) - "An anal dimple with raised area and good color change indicates there is probably a good sphincter" — Marc Levitt (clinical) [4:06](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=246) - "Tiny white beads in the scrotal raphae indicate the ARM lesion is one of the less complicated lesions with an opening somewhere along the perineal body" — Megan Durham (clinical) [5:24](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=324) - "For a perineal fistula ARM without cardiac defect, primary repair would be the optimal choice" — Megan Durham (clinical) [6:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=363) - "Dilation without going to the OR might be a good choice in a baby you don't want to take to the operating room" — Marc Levitt (clinical) [6:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=363) - "For patients with ARM and really significant cardiac anomaly, an ostomy is probably the standard choice" — Rod Gerardo (clinical) [6:13](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=373) - "In a female patient with vestibular or perineal fistula, there is no rush to operate" — Marc Levitt (clinical) [6:32](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=392) - "In a male, the perineal hole is not always easy to see and dilation is more dangerous because it is near the urethra" — Marc Levitt (clinical) [6:32](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=392) - "With care and Hagar dilators, you can get egressive stool and never go to the OR" — Marc Levitt (clinical) [6:32](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=392) - "In a cardiac patient with an external opening from ARM, the typical approach is to dilate as long as they are evacuating okay" — Jason Frischer (clinical) [7:24](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=444) - "The concern with significant cardiac lesions requiring early surgery in a blue baby is worry about healing of the repair" — Jason Frischer (clinical) [7:48](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=468) - "There is no need to do a colostomy in a baby with perineal fistula and cardiac disease; you can dilate and do the repair primarily later" — Marc Levitt (clinical) [8:13](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=493) - "A conus at L2 is normal" — Megan Durham (clinical) [8:33](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=513) - "A baby is too young in the neonatal period to calculate a sacral ratio" — Megan Durham (clinical) [8:33](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=513) - "VCUG is obtained if there are renal anomalies" — Megan Durham (clinical) [8:57](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=537) - "Every time they tried to dilate this baby, the baby would cry and desat down to the 60s due to TET spells" — Megan Durham (clinical) [9:14](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=554) - "A turnable loop ostomy behaves like an end ostomy with a 95-5 percentage split, and no one knows except the surgeon that there is another side where you can do a contrast study" — Marc Levitt (clinical) [9:43](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=583) - "For cardiac babies undergoing laparoscopy, initial insufflation pressure is set around 8 mmHg if tolerated" — Megan Durham (clinical) [10:23](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=623) - "Starting flow rate for insufflation in babies is set at 1 L/min" — Megan Durham (clinical) [10:23](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=623) - "If the anus can be irrigated, evacuating stool helps keep insufflation pressures low during laparoscopy" — Megan Durham (clinical) [10:23](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=623) - "If the baby has an umbilical line, consider Palmer's Point access rather than umbilical access, using a Hasson technique" — Rod Gerardo (clinical) [11:00](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=660) - "When accessing the umbilicus, dissect in with a mosquito, make sure you are in without touching any vessel before insufflation, and clear the line of air" — Marc Levitt (clinical) [11:08](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=668) - "It is important to irrigate the distal bowel as much as possible during colostomy creation" — Jason Frischer (clinical) [11:21](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=681) - "A technique for distal irrigation involves having someone look laparoscopically while passing a tube into the distal segment and performing irrigation under direct visualization" — Marc Levitt (clinical) [11:21](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=681) - "This baby's PDA completely closed postnatally" — Rod Gerardo (clinical) [11:39](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=699) - "Despite maneuvers, this baby continued to have hypercyanotic spells and required heart surgery sooner than the initially planned 6 months" — Rod Gerardo (clinical) [11:39](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=699) - "Emergent tetralogy of Fallot repair was performed on day of life 5" — Rod Gerardo (clinical) [12:10](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=730) - "PSARP was planned for about 3 months after colostomy" — Megan Durham (clinical) [12:14](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=734) - "A baby with a low ARM lesion where the perineal fistula is closely approximated to the anal muscular complex should do really well for continence" — Megan Durham (clinical) [12:33](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=753) - "Important factors for continence include sensation in the anal canal, absence of the dentate line, quality of the spine, quality of the anorectal malformation, and quality of the sacrum" — Megan Durham (clinical) [12:33](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=753) - "Sacral ratio measurement should wait until 3 months of age" — Megan Durham (clinical) [12:33](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=753) - "When half of the perineal fistula opening is anterior to the muscular complex, the entire opening should be formally moved back into the center around the anal muscular complex" — Megan Durham (clinical) [13:36](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=816) - "The pearls along the median raphae should be scraped off at about one millimeter depth; do not dive in to find the fistula tract as it will disappear with good anoplasty and anterior rectal wall mobilization" — Marc Levitt (clinical) [14:02](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=842) - "Pearls along the raphae can persist into teenage years and young adulthood if not addressed" — Marc Levitt (clinical) [14:02](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=842) - "When 50% of the fistula is within the muscle complex and 50% is anterior, leave the anterior wall (the danger zone) and mobilize posteriorly to fill the anal sphincter, creating an 80-20 reconstruction" — Marc Levitt (clinical) [14:23](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=863) - "If the fistula is completely outside the sphincteric ellipse, a full mobilization must be performed" — Marc Levitt (clinical) [14:23](https://library.globalcastmd.com/watch/colorectal-quiz-episode-32-anorectal-malformations-and-cardiac-anomalies-13857?t=863) ## Transcript 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. --- Not medical advice · citation policy: https://library.globalcastmd.com/ai