In this podcast, we cover the basics of malrotation including embryology, presentation, differentiation, diagnosis, surgical approach, and post operative management with Dr. Meera Kotagal.
Host: Em Tombash
Animations to understand the topic better
https://www.youtube.com/watch?v=vJA1A0v6Aa4
Type: podcast · 13 min · posted 2023-05-04 Canonical: https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621 ## Chapters - [0:00](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=0) Introduction and Epidemiology - [1:17](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=77) Embryology and Anatomy of Malrotation - [3:39](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=219) Complications, Presentation, and Diagnosis - [8:33](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=513) Surgical Treatment and Controversies - [12:17](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=737) Summary and Closing ## Statements - "Malrotation occurs in about 1 in 200 to 500 live births" — Em Tombash (epidemiological) [0:28](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=28) - "In the fourth week of gestation, bowel development begins with the bowel herniating into the yolk sac and along the umbilical cord and SMA axis" — Meera Kotagal (clinical) [1:17](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=77) - "Normal bowel rotation involves a 90 degree rotation of the duodenal-jejunal loop followed by a 270 degree rotation of the cecum and colon as the bowel returns to the abdominal cavity" — Meera Kotagal (clinical) [1:44](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=104) - "Normal anatomy results in the duodenal-jejunal junction to the left of midline at the ligament of Treitz and the cecum in the right lower quadrant" — Meera Kotagal (clinical) [1:44](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=104) - "Non-rotation results in the colon on the left and small bowel on the right without the problematic Ladd bands that cause obstruction in malrotation" — Meera Kotagal (clinical) [2:22](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=142) - "Non-rotation anatomy looks similar to the anatomy of a malrotated patient post-Ladd procedure" — Meera Kotagal (clinical) [2:22](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=142) - "Malrotation involves incomplete rotation preventing formation of a broad-based mesentery, which is important to reduce the risk of volvulus" — Meera Kotagal (clinical) [2:22](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=142) - "Complications of malrotation fall into two categories: obstructive symptoms with feeding intolerance, and midgut volvulus resulting from narrow mesentery" — Meera Kotagal (clinical) [3:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=229) - "About one in 200 children have a risk of malrotation, but only about one in 30 of those children with malrotation will develop a volvulus" — Meera Kotagal (epidemiological) [3:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=229) - "About 70% of children who will have a midgut volvulus will present in the first year of life" — Meera Kotagal (epidemiological) [3:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=229) - "As children with malrotation get older without having had a volvulus, their likelihood of having a significant clinical event related to malrotation decreases" — Meera Kotagal (clinical) [3:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=229) - "Children with congenital cardiac disease and heterotaxy commonly have associated malrotation, but their likelihood of symptomatic events is much lower" — Meera Kotagal (clinical) [3:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=229) - "Rotational abnormalities are associated with congenital diaphragmatic hernia, gastroschisis, and omphalocele due to the nature of the anatomy of these diagnoses" — Meera Kotagal (clinical) [3:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=229) - "In the neonatal period, bilious emesis is the most critical symptom requiring evaluation for midgut volvulus even if the x-ray appears normal" — Meera Kotagal (clinical) [4:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=289) - "Other symptoms of malrotation include feeding intolerance, abdominal pain, and abdominal distension" — Meera Kotagal (clinical) [4:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=289) - "Late signs of volvulus include bloody stools, peritoneal signs on exam, and abdominal wall erythema, which are suggestive of intestinal ischemia" — Meera Kotagal (clinical) [4:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=289) - "The differential diagnosis for bilious emesis includes atresia, Hirschsprung disease, meconium ileus, meconium plugs, incarcerated hernia, anorectal malformations, and necrotizing enterocolitis" — Meera Kotagal (clinical) [4:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=289) - "Malrotation must be ruled out before pursuing other diagnoses in a child with bilious emesis" — Meera Kotagal (clinical) [4:49](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=289) - "Upper GI is the most frequently used imaging for diagnosis of malrotation, looking at the position and course of the duodenum" — Meera Kotagal (clinical) [6:40](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=400) - "On upper GI, normal anatomy shows the C loop of the duodenum coming back across the midline to the left, and on lateral view going posteriorly and cephalad" — Meera Kotagal (clinical) [6:40](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=400) - "Contrast enema can sometimes determine cecal position but current sensitivity and specificity are not sufficient to rule out malrotation definitively" — Meera Kotagal (clinical) [7:30](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=450) - "Upper GI remains the definitive test for malrotation because missing midgut volvulus is considered the number one surgical emergency in pediatric surgery" — Meera Kotagal (clinical) [7:30](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=450) - "Surgical treatment of volvulus involves exploratory laparotomy with evisceration of the bowel and detorsion, usually requiring 270 degree counterclockwise rotation" — Meera Kotagal (clinical) [8:33](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=513) - "After detorsion, the bowel is given time to rest to assess viability and determine if ischemia has been prolonged" — Meera Kotagal (clinical) [8:33](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=513) - "You can have malrotation without midgut volvulus, but you cannot have midgut volvulus without malrotation" — Em Tombash (clinical) [8:33](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=513) - "The Ladd procedure involves widening the mesentery by removing Ladd bands, straightening the duodenum to avoid obstruction, and often performing an appendectomy" — Meera Kotagal (clinical) [8:54](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=534) - "Appendectomy during Ladd procedure is performed because the appendix will be in an abnormal anatomic position, and families need to be informed if it is left in place" — Meera Kotagal (clinical) [8:54](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=534) - "After Ladd procedure, the bowel is placed in non-rotation configuration with small bowel on the right and large bowel on the left to keep the mesentery as broad as possible" — Meera Kotagal (clinical) [8:54](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=534) - "Some believe that part of the benefit of Ladd procedure is causing scar tissue that helps the bowel adhere in a configuration preventing midgut volvulus" — Meera Kotagal (opinion) [10:01](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=601) - "Laparoscopic Ladd procedure may have decreased adhesion rates, potentially reducing the adhesive benefit but also potentially reducing postoperative bowel obstruction" — Meera Kotagal (opinion) [10:01](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=601) - "About a quarter of patients who undergo a Ladd procedure will have intestinal obstruction related to adhesive small bowel disease" — Meera Kotagal (epidemiological) [10:01](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=601) - "In older children with symptomatic malrotation, laparoscopic approach may be started to assess mesentery width and duodenal anatomy before deciding on approach" — Meera Kotagal (opinion) [10:01](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=601) - "There is controversy about whether laparoscopic approach can successfully broaden the mesentery as wide as it should be" — Meera Kotagal (opinion) [10:01](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=601) - "There is controversy about whether prophylactic Ladd procedure should be performed in asymptomatic children with diagnosed malrotation or in conditions like congenital diaphragmatic hernia" — Meera Kotagal (opinion) [10:01](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=601) - "In younger children with malrotation, the risk for volvulus or symptomatic malrotation is higher, so Ladd procedure is performed more commonly" — Meera Kotagal (clinical) [10:01](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=601) - "Decisions about prophylactic Ladd procedure should involve informed consent discussions with families about risks of both doing and not doing the operation, considering factors like access to care" — Meera Kotagal (opinion) [10:01](https://library.globalcastmd.com/watch/malrotation-with-dr-meera-kotagal-6621?t=601) ## Transcript Hi, everyone. Welcome back to Stay Current Podcasts. I'm Em Tom Bash, a research fellow at Cincinnati Children's Hospital Medical Center. And along with Stay Current, we are sharing knowledge to improve child health around the globe. In this podcast, we are going to talk about malrotation anatomy, presentation, diagnosis, and treatment options with Dr. Mira Kodagal. My name is Mira Kodagal. I'm a pediatric surgeon at Cincinnati Children's. We have a lot to cover today. So if you're looking for a specific part like diagnosis or treatment options, make sure to check out time stops in the description. They'll take you wherever you need. Malrotation is a congenital condition that occurs in about 1 in 200 to 500 live births, making it a relatively common issue in pediatric patients. It is one of the most important causes of intestinal obstruction in infants and children. The severity of symptoms can vary, and early diagnosis and treatment are critical to prevent long-term health problems. But before we start talking about diagnosis and treatment, I want to take a step back to understand how malrotation happens anatomically. What are the differences in fetal development that cause malrotation? Now, Dr. Kodagal will explain. In the fourth week of gestation, that's really when we start to see development of the bowel. And as the bowel grows in length, it actually herniates into the yolk sac and along the umbilical cord and the SMA axis. We've all been there. I know it's hard to visualize the anatomy, especially challenging one like malrotation. For this reason, we added some animations linked below in the description. Don't forget to check them out. I promise it'll help you understand the concept better. Dr. Kodagal is the last one. It develops outside and continues to grow in length and then will actually go back into the abdomen with sort of two pieces of rotation. The first part of the rotation is a 90 degree rotation where the duodenal-jejunal loop sort of rotates. And then you get the return of the rest of the bowel into the abdominal cavity with a 270 degree rotation of the cecum and colon. And that allows it to end up in the conformation that we consider it normal, where you have a duodenal-jejunal junction to the left to midline at the ligament of triads. And then the cecum and colon around the abdomen starting from the right lower quadrant all the way around to the left. Now is the time to check the animations link below in the description if you haven't yet. We're now moving on. You can get errors at any stage in that sort of rotational return of the bowel to the abdomen. And those can result in that wide spectrum of anomalies. We used to describe rotation as either normal rotation and malrotation. And now we've gotten more into the language of talking about intestinal rotation abnormalities because they tend to fall more along a spectrum than the black and white of either normal or abnormal. Those can include both non-rotation and malrotation. With non-rotation, basically the gut goes back in but doesn't rotate at all. And so you end up with the colon on the left and the small bowel on the right. And you don't tend to have the problematic lads bands that can cause obstruction as you see in malrotation. And so, non-rotation anatomy actually looks a lot like the anatomy of a malrotated kid post lads. Malrotation means that you get some portion of the rotation but you don't necessarily end up with full rotation to allow for the broad-based mesentery, which is what's important to reduce the risk of volvulus. So you can end up with some rotation meaning that the small bowel is still all on the right and the cecum makes it part way towards the right lower quadrant but not the whole way. And so you get lads bands that can be obstructing of that duodenal junction or you can get different sort of anomalies within there. The cecum can still be on the left-hand side but the small bowel mesentery is narrow. And that's what ends up causing your predisposition towards volvulus. Since we mentioned volvulus, we need to look into malrotation complications. What are we afraid of for this kids? Complications of malrotation, there's really two buckets that you have to think about. One are sort of obstructive symptoms and you tend to see that with feeding intolerance and kids that may have issues around food getting past those lads bands in the duodenum. And the other one is midgut volvulus, which is the result of narrow mesentery. When you have malrotation and because of the way that the bowel aligns, the small bowel mesentery is not spread out from the left upper quadrant to the right lower quadrant as you would expect and you can get a narrow pedicle. It's easier for that pedicle to twist on itself and then you get a midgut volvulus and ischemia of the intestine. About one in 200 children have a risk of malrotation, but only about one in 30 of those children with malrotation will develop a volvulus. A lot of kids with malrotation may never be identified to have malrotation or they may have mild symptoms of feeding intolerance and that may result in their evaluation and diagnosis. It's important to recognize that about 70% of those who will have a midgut volvulus will present in the first year of life. So as kids get older, if they never had a volvulus, their likelihood of having a significant clinical event related to their malrotation goes down. Very commonly kids with congenital cardiac disease and heterotaxy especially can have associated malrotation. The data seems to suggest that in those kids their likelihood of having a symptomatic event, whether that is feeding intolerance and biliosemesis or actually progressing to volvulus, is much lower. So we don't tend to fix the kids who have heterotaxy and malrotation with the same frequency that we might for another child in their infancy. The other thing to remember is that rotational abnormalities are also associated with certain diagnoses that are very commonly seen in pediatric surgery and they're just the result of the nature of the anatomy of those diagnoses. So that includes congenital diaphragmatic hernia, gastroschisis and emphalocele. In the neonatal period, the most critical symptom to be really aware of is biliosemesis. Even if they have a normal appearing x-ray, those kids really need to be evaluated for midgut volvulus. You have to be able to really understand that because the risk to missing it is so high. And so biliosemesis is the most common criteria for us to evaluate a kid for malrotation. But there are other things that you can see like feeding intolerance, abdominal pain, abdominal distension. You can get some late signs associated with volvulus, but at that point it's usually reflective of the intestinal ischemia. So you can get bloody stools or peritoneal signs on exam, abdominal wall erythema. All of those are more suggestive of a later stage in the process and concerning for progression of intestinal ischemia. So the differential diagnosis for biliosemesis is actually quite broad and includes basically all kinds of intestinal obstructions. So it includes atresia, Hirschsprung's disease, meconium ileus or meconium plugs, any other cause for intestinal obstruction including things like an incarcerated hernia and anorectal malformations in kids who have no obvious fistula or who have an intestinal fistula to their urinary system or kids with necrotizing enterocolitis. All of those diagnoses in kids can present with biliosemesis and actually for many of them malrotation may be less likely. But it's the thing that you have to rule out before you go down to the pathway of thinking about other diagnoses. The imaging that we use most frequently for the diagnosis of malrotation is an upper GI and you're really there looking at the position and the course of the duodenum in order to determine whether or not it is normally or abnormally positioned. You want to see the total of the C loop and actually watch the duodenum come back across the midline to the left. Also when you look at it from a lateral perspective you want to see it also go posteriorly and a bit cephalad in order to determine that's really normal position of the duodenum and decreases your risk or concern for malrotation. But what about using contrast enema for diagnosing? Some people also think you can use contrast enema sometimes if you're able to determine whether the position of the cecum is normal on a contrast enema. I think our sensitivity and specificity for those studies currently is not sufficient for us to be comfortable using them to rule out a significant pathology, right? We think of midget volvulus as the number one surgical emergency in pediatric surgery and so missing that is a significant problem and so to this point they haven't superseded upper GI as the definitive test. The surgical treatment of volvulus is to go to the operating room and do an expletive leparotomy. You have to actually detour the bowel. So there's multiple steps when we think about doing a LADS procedure. So there's multiple steps when we think about doing a LADS procedure and a LADS procedure is the last part of an operation for volvulus. You're going to make an incision, you're going to eviscerate the bowel in order to be able to determine what you're dealing with and then de-rotate or de-volvulize the bowel. Which usually requires a 270 degree rotation counterclockwise. And once you've done so then you're able to give the bowel a rest and actually try to see whether it can pick up again or whether that ischemia has been going on so long that you're concerned about the viability of the intestine. So Dr. Cartergal believes that it is really important to distinguish malrotation from volvulus. You can have malrotation which is not a surgical emergency or a mid-gut volvulus which is a surgical emergency and malrotation predisposes you to that mid-gut volvulus. You can't have one without the other but not all kids who have a malrotation are volvulus. So you can have malrotation without mid-gut volvulus but you can't have mid-gut volvulus without malrotation. And so once you have done the operation in order to de-volvulize the bowel then you can deal with the LADS procedure which is an operation for malrotation. And that involves doing a couple of things. The initial thing is widening the mesentery. You want to try to take off as many of those LADS bands that have created a narrow mesentery in order to broaden it as much as possible. Sometimes there are also LADS bands that cause obstruction of the duodenum and so you want to make sure that you free up and straighten the duodenum to avoid concerns for feeding intolerance related to duodenal obstruction. And in this part of the surgery a lot of surgeons choose to do an appendectomy. The real driver for taking out the appendix is just knowing that it's going to be in an abnormal anatomic position and if you leave it in place you have to remember to tell families that if they present with signs they need to tell people that their appendix is not in the normal location that the kid is not rotated. And then after you do that you're going to put the bowel back into the abdomen in a non-rotation configuration. It's a small bowel on the right, large bowel on the left as a way to the intestines and try to keep that mesentery as broad as possible. We know a significant amount of debate as to whether or not a laparoscopic LADS procedure is a good procedure or not a good procedure. And Dr. Carter Gall says it depends a little bit on your theory of why you think a LADS works. Some people believe that part of the benefit of doing a LADS procedure is A you widen the mesentery. But you also cause other scar tissue helping to the bowel to scar at a conformation that prevents midgut valvulus. So if you're doing a laparoscopic procedure you have decreased rates of adhesions related to that it's possible that you might not get that adhesive benefit. That being said one of the other downsides to doing a LADS procedure is the high rate of intestinal obstruction. So about a quarter of patients who undergo a LADS procedure will have an intestinal obstruction related to small bowel obstruction related to their OTCU bowel disease from their LADS procedure. And so the thought is maybe that doing it laparoscopically reduces that risk of postoperative bowel obstruction. So it's a balance. What I often will do is if I'm concerned in an older child, not a kid who's presenting with midgut valvulus but a kid who has symptomatic malrotation, I might start laparoscopically and determine whether or not I think the mesentery is broad or very narrow and then look at the duodenum. If I think the mesentery is actually fairly broad but the biggest problem is bands around the duodenum causing obstruction, I might do that laparoscopically. For cases where the mesentery is very narrow, sometimes Dr. Cartergaul thinks that's a harder procedure to do laparoscopically. There's some controversy about whether you can successfully broaden the mesentery as wide as it should be by a laparoscopic approach. There is some controversy as to whether or not you should or should not do a prophylactic LADS. In a kid with diagnosed malrotation or kids like congenital diaphragmatic hernias who we know by definition have malrotation because of the long-term risks of bowel obstruction and the complications that can result from that. In general, in younger kids when they present with malrotation their likelihood or risk for volvulus is higher or for symptomatic malrotation is higher and so we tend to fix them or do a LADS procedure more commonly in young kids. But again those are opportunities to really have good informed consent discussions with families around the risks of both doing or not doing the operation and what they might prefer and particularly access to care. For families that are much more remote they might opt for an operation accepting the risk for bowel obstruction knowing that in the event of an acute volvulus they would have a much harder time getting to the hospital in a timely fashion. That was all regarding the diagnosis and treatment of malrotation. In this podcast we reviewed using upper GI for malrotation diagnosis by looking at the position in the course of the duodenum in order to determine whether or not it is normally or abnormally positioned. Also we talked about surgical treatment of volvulus which is going to the operating room and doing an exploratory laparotomy. Also mention some controversial topics like using laparoscopy for LADS procedure or if prophylactic LADS procedure is necessary in an asymptomatic child. Thank you for listening to this podcast. Don't forget to follow us on social media, subscribe to our youtube channel and download the StayCurrent app for more content including videos, podcasts and infographics. Cincinnati Children's Hospital and StayCurrent are sharing knowledge to improve child health around the globe. --- Not medical advice · citation policy: https://library.globalcastmd.com/ai