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ABSITE Review: Pediatric Surgery
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ABSITE Review: Pediatric Surgery
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Language: EN.
Segment:0 .
DR. KNIERY: Welcome back to Behind the Knife's ABSITE review. Today, we'll be reviewing pediatric surgery. I'm going to continue with my theme of reading a quote of the day, and this quote is from Teddy Roosevelt. This was the favorite quote of one of my good friends, Christopher Goeke, who was killed in Afghanistan in 2010 while leading a platoon of the 82nd Airborne. Chris embodied this quote, and I feel this quote has direct parallels to a career as a surgeon.
DR. KNIERY: "It is not the critic who counts, not the man who points out how the strong man stumbles, or where the doer of deeds could have done them better. The credit belongs to the man who is actually in the arena, whose face is marred by dust and sweat and blood, who strives valiantly, who errs, who comes short again and again, because there's no effort without error and shortcoming, but who does actually strive to do the deeds, who knows great enthusiasms, the great devotions, who spends himself in a worthy cause, who at the best knows in the end the triumph of high achievement, and who at the worst, if he fails, at least fails while daring greatly, so that his place shall never be with those cold and timid souls who neither know victory nor defeat." Okay, best of luck, enjoy this episode.
DR. BINGHAM: All right, so today, we're going to be doing a pediatric surgery review. It should be pretty brief. Pediatric surgery is kind of a daunting topic, but when you look at it with regard to the ABSITE, it's only about 2% on the exam, so that should be telling you about how much time you should be spending on it. There's some big key things that we need to cover, because a lot of the same stuff shows up on the test every year, so these are pretty high yield.
DR. BINGHAM: So let's get right into it, let's start with trauma. So John, what's the number one cause of death in children 1 to 20 years old?
DR. MCCLELLAN: So the number one cause of death is trauma, although I'm curious what the opioid overdoses have done recently on that.
DR. BINGHAM: Yeah, those kids out there on opioids, kind of sad. Yeah, number one cause of death in children, trauma. Okay, with regard to trauma in children, let's talk about shock. So John, walk us through, tell us about shock in kids. What's the best indicator of shock and what are some key things we need to know when looking at a kid in shock?
DR. MCCLELLAN: So when you look at a kid in shock, it's kind of a well-known thing that kids actually look really, really well until they decompensate very quickly. So your best indication of shock in a pediatric population is tachycardia.
DR. BINGHAM: Yeah, so tachycardia is the best indicator of shock. It's going to be late when they drop their pressure. So they look well until they don't. So typically look very well, decompensate quickly.
DR. MCCLELLAN: You have heart rates and for like, if you want to look at actual heart rates for a lot of different types of pediatric population. So a neonatal patient in shock would be a heart rate greater than 150, anyone less than one years old, anything greater than 120, and then all other pediatric population, so up until the teenage years, anything greater than 100.
DR. BINGHAM: Perfect. That's a general rule of thumb. That was exactly what I was leading into. We'd say tachycardia but you have to know what the different parameters are for kids to know if it's in fact, tachycardia. In a neonate 150, that's normal. Let's talk about fluid resuscitation. So targets for fluid resuscitation, this sometimes shows up, what's the rate for a bolus of fluid, if we're giving blood? How do you calculate that?
DR. MCCLELLAN: So for a bolus of crystalloid fluid it's 20 cc's a kilogram. And the easiest to remember that for blood is you take that number and half that. So a blood bolus or blood dosing would be 10 cc's a kilogram. And typically if you're getting asked this in a question, you give this after about two fluid boluses in a patient who is hypotensive.
DR. BINGHAM: Okay, what's your target for fluid resuscitation as far as urine output in kids?
DR. MCCLELLAN: So urine output in a neonate or infant population is 2-3 cc's a kilogram an hour, and in toddlers and everybody older than that it's closer to adults which is 1 cc a kilogram an hour.
DR. BINGHAM: Great. So that stuff will kind of show up, the other thing that shows up is calculating maintenance IV fluids and if you're not familiar with it, you should look up the 4-2-1 rule. I think most people are pretty familiar with that from medical school and internship, but let's stay on the topic of trauma in pediatrics. Sometimes you'll get asked how to choose an endotracheal tube in children under 10, John, finish up the trauma for us. What are some general rules of thumb there for endotracheal tubes in children less than 10?
DR. MCCLELLAN: Yeah, I think the one, the biggest thing to know the difference nowadays is that with children less than 10 years old, there used to be an indication for an uncuffed tube. Now we're putting cuffed tubes into the low pressure of the actual endotracheal tube balloon. So now we're doing cuffed tubes in everybody, in all children, except for neonates who still get an uncuffed tube. Typically you want to do, there's an equation for the size of the ET tube, but you can also use the pinkie finger or the size of the nasal passage as well, that's kind of a fast rule, but the equation is the number four plus the age divided by four.
DR. BINGHAM: Okay, four plus age divided by four or the size of the pinky, perfect. And you can also get out those - in real life - get out those Broselow tapes to help guide the sizing of these various things. Okay, moving on from trauma, let's get into emesis in kids. Emesis is another thing that shows up very frequently on the ABSITE, having to know differentials based on age, bilious versus non-bilious so Meg, let's go through a differential by age for emesis.
DR. BINGHAM: We're talking about neonates, infants, toddlers and then adolescents, what's on your differential for emesis in those different age groups? [Dr. Kashyap] So when we're talking about emesis, it's always important to remember that not every emesis in these children is surgical. So thinking about the nonsurgical issues in neonates, it could be infectious, it could be allergic reactions, it could be a metabolic disorder, but surgically there's a lot of issues in the neonates.
DR. BINGHAM: They can have GERD, which might require a fundoplication, pyloric stenosis, or intestinal obstruction, or an atresia anywhere along the esophagus or intestinal tract. In infants and toddlers, again, they can have gastroparesis, infectious etiologies, or even neurologic and psychologic issues, but surgically the common findings are intussusception or volvulus, and then finally in adolescents, they can have functional intestinal disorders, they can have IBD, again, they can have psychogenic causes for emesis and then surgically the most common cause of emesis in adolescents is appendicitis.
DR. BINGHAM: Yeah. So you can kind of narrow your differential just based on the age. So either in the question stem or in real life when you're looking at a patient, you look at how old they are and that can kind of help you narrow your differential based on what the most common causes for emesis are. Now let's talk about bilious versus non-bilious. This is another key distinction that can help you kind of figure out what's going on.
DR. BINGHAM: So Meg, let's first talk about non-bilious emesis. So what's on your differential for a child with non-bilious emesis? [Dr. Kashyap] So the first thing I'm going to think about is pyloric stenosis. I think it's the most common one we find. And then a little less common are tracheoesophageal fistulas and intussusception.
DR. BINGHAM: Okay. So let's tackle that first one that you mentioned, pyloric stenosis. What's going to be in the question stem, what are some buzzwords for pyloric stenosis? [Dr. Kashyap] Yeah, so this is the one where it's usually a younger infant or toddler that comes in with projectile vomiting. And then sometimes they might describe that you can feel a palpable, maybe olive shaped mass in the abdomen.
DR. BINGHAM: And this patient usually looks a bit dehydrated, maybe a little bit tachycardic as well.
DR. BINGHAM: Yeah, and along those lines, the dehydration, there's a very characteristic metabolic disturbance that you see in children with pyloric stenosis. And sometimes this is the question on the test. What kind of metabolic disturbance are they likely to have? They give you a classification with pyloric stenosis. So what is that, Meg? [Dr. Kashyap] Yeah, so it's the same with anyone who has emesis for prolonged period of time, you're going to get a hypochloremic, hypokalemic, metabolic alkalosis, and you'll also get a paradoxic aciduria.
DR. BINGHAM: And what's driving that paradoxic aciduria? [Dr. Kashyap] So I think it's because you're losing the potassium, so your kidneys are trying to retain it and so instead it's excreting the hydrogen ions.
DR. BINGHAM: Exactly. That's exactly right. So diagnosis, ultrasound, there's a cutoff for diagnosing pyloric stenosis. What is that? [Dr. Kashyap] So I like to remember the 4s, similar to that equation for ET tubes. There's a lot of 4s in children. So pylorus being more than four millimeters thick and being more than 14 millimeters long is diagnostic. and being more than 14 millimeters long is diagnostic.
DR. BINGHAM: Yeah, right. The one I remember, I remember a little bit differently and choose which one works for you, but I remember pi for like pyloric stenosis, pi equals 3.14, so greater than three, I think the technical thing is four but greater than three millimeters thick or 14 millimeters long. What do you do with these patients as far as treatment? [Dr. Kashyap] So this is one where you can get tripped up.
DR. BINGHAM: The first thing is always resuscitation, because like we said, there's a metabolic disturbance. These kids need to be resuscitated first, which is crystalloids, normal saline. And then if you're switching over to maintenance IV fluids, it's always important to remember that pediatric patients need some glucose, some dextrose in their maintenance fluids. And then you can proceed to the OR once they're resuscitated.
DR. BINGHAM: Right, and it is important to say, you resuscitate first with normal saline. So you wait to add potassium until you have return of urine output. So I've seen that question on the test, they'll ask you what fluid do you want to resuscitate this kid with pyloric stenosis and the first thing is normal saline. And then what's the procedure in the OR? [Dr. Kashyap] You're going to do pyloromyotomy.
DR. BINGHAM: Okay. So let's move on to the next thing that can cause non-bilious emesis and this is a big topic. So we'll go over to John for this one. So John, tracheoesophageal fistula, there's five different types, what are the ones that we need to know?
DR. MCCLELLAN: So the two types that you need to know are, mainly what you're going to be tested on is type C and type A. So type C is the most common, almost 90% of patients who have a tracheoesophageal fistula have this type. It is a blind-ending esophageal atresia. So your esophagus stops and then you have a distal tracheoesophageal fistula. Type A is the next most common, at about 8%, and this is actually the only one without a tracheoesophageal fistula.
DR. MCCLELLAN: It's an esophageal atresia on the other side. So there's no connection to the trachea whatsoever.
DR. BINGHAM: Okay, so John how do you make the diagnosis?
DR. MCCLELLAN: So in kids we do an abdominal X-ray, so the distinguishing factors between the two types, so type C, you'll have gas present within the abdomen with a distended stomach because you do have that distal fistula, whereas type A you have a gasless abdomen.
DR. BINGHAM: Okay, and what else do you have to look for when you see a patient with TE fistula? They'll ask you for some associated anomalies, what are you looking for?
DR. MCCLELLAN: Yeah, see this is where you need to remember your VACTERL workup as well, and the components of the VACTERL syndrome include your vertebral anomalies which you can diagnose with a sacral ultrasound. So A stands for your anorectal abnormalities, most commonly an imperforate anus and you diagnose it with an exam. Cardiac is your C and you can diagnose with an echocardiogram.
DR. MCCLELLAN: T stands for your TE fistula which we already talked about, R is your renal issues and you can diagnose this with a renal ultrasound and then L stands for your limb abnormalities which will be apparent on a physical exam.
DR. BINGHAM: Great, yeah, so they'll ask you for those associated disorders and a big important one there is the cardiac so, I've seen it asked before where you have a person with TE fistula and they ask what the next step is and you need to rule out any cardiac abnormalities that need to be corrected prior to taking this patient to the operating room to repair their TE fistula.
DR. BINGHAM: So that's a very important one to remember. Treatment?
DR. MCCLELLAN: So you want to resuscitate them like all patients and you can place a Replogle tube which is a double lumen tube to suction out the saliva and also keep the esophagus patent. You can also place a G-tube for decompression distally and then obviously the repair is in the course of surgery. So we need to do a repair, you're going to have to select usually a right extrapleural thoracotomy.
DR. BINGHAM: Okay, great. Common complications for that procedure?
DR. MCCLELLAN: You have a strictured fistula as your most common type of complications, and also later down the line is reflux disease.
DR. BINGHAM: Yeah, so you think about esophagectomy essentially. So you can have a stricture there, you can form a fistula, you can have a leak which can be pretty devastating but long term, as you say, GERD is a very common complication. Okay so Meg, back to you, we're talking about now intussusception which can be a source of either bilious or non-bilious vomiting, obviously if you're obstructed from intussusception, you can have bilious emesis, but what are going to be the buzzwords you see on the test for intussusception in a child?
DR. BINGHAM: [Dr. Kashyap] So the one term that I basically always associate with intussusception is the currant jelly stools and then the one other is if you're given imaging that they talk about the target sign which is the bowel inside of the bowel, so you see two concentric circles.
DR. BINGHAM: Great. What causes intussusception in children? [Dr. Kashyap] So the number one cause is after a viral illness, that's going to be the common presentation that the child comes in after a viral illness, and like a URI and then they have this abdominal pain or emesis and it's due to the inflamed Peyer's patches as a lead point. The second most common is a lymphoma and the third most common is a Meckel's diverticulum.
DR. BINGHAM: Perfect. So I think the point there being that most common in children it's a viral illness and inflamed Peyer's patches and it's a benign etiology. So you can get away with treating it non-operatively which we'll get into here in a second, versus adult where if you have a true intussusception, not just an incidental small bowel intussusception seen on CT scan, a true intussusception, it's more common that that's from potentially a mass and so those adult patients are treated differently than children.
DR. BINGHAM: You mentioned that the diagnosis is by ultrasound, what's the treatment for intussusception in a child? [Dr. Kashyap] So in the child the treatment is an air contrast enema.
DR. BINGHAM: Okay, so you do that and it recurs, what do you want to do next? [Dr. Kashyap] Another air contrast enema.
DR. BINGHAM: Okay and it recurs and what do you want to do next? [Dr. Kashyap] So I forget what the threshold is but, the answer is usually again to try an air contrast enema but if you're unable to reduce it, then that's when you have to go to operation or typical reasons to go to the OR like peritonitis, or if they are obstructed and it is bilious emesis.
DR. BINGHAM: Perfect, I think you'll see some old, some kind of old dogma that if they, they fail twice then they go to the OR but in reality as long as they're stable and they respond to the air contrast enema, you can keep doing it. If you're unable to reduce it with the air contrast enema or they demonstrate signs of a threatened bowel or peritonitis then that needs to go to the OR.
DR. MCCLELLAN: The one thing I've seen come up before is that when you do reduce it, say they are in an emergency room and you reduce it, how long do they have to stay in the hospital? Can they be observed in the emergency room or do they need to be admitted overnight? Does anyone know the answer to that? [Dr. Kashyap] They have to be observed I believe for four hours and I think also you give them some food and test how they're doing.
DR. MCCLELLAN: All right, okay.
DR. BINGHAM: Yeah, I'm not sure at that. I've never seen anything like that on the ABSITE. So I'd be surprised at something like that, where there's probably quite a bit of variability in practice. Okay and we already mentioned, again, in adults intussusception more likely from a malignancy so those patients need to go to the OR, don't air contrast an adult with an intussusception. Okay, moving on we've talked about some causes of non-bilious emesis.
DR. BINGHAM: Now let's move on to differential for bilious emesis. This is a favorite of the ABSITE. So John, differential for bilious emesis.
DR. MCCLELLAN: So I'd be looking, immediately my mind goes to malrotation with some type of midgut volvulus but I also need to be considering duodenal atresia and meconium ileus as reasons for it.
DR. BINGHAM: Okay, so again, let's come back, let's tackle that first one. Malrotation with midgut volvulus. What's the typical etiology of this?
DR. MCCLELLAN: So it's a failure of the normal 270 degree rotation during intestinal development so the bowel just did not rotate as it comes back into the abdomen and the volvulus portion is actually caused by the Ladd's bands which are adhesions to the right retroperitoneum.
DR. BINGHAM: Okay, and when does this typically so-- let's say you have malrotation, at what age group do these present with volvulus?
DR. MCCLELLAN: Almost all of them, 90% or so present within the first year of life.
DR. BINGHAM: Okay, perfect, I've seen it in adults so occasionally you'll see it in an adult, somebody in their 20s or 30s will show up with some problems and they have a malrotation and volvulus. How do you diagnose a malrotation?
DR. MCCLELLAN: So for pretty much all the bilious vomiting diagnoses, the first step you always do and should be like almost a go-to answer is your emergent upper GI and then on this upper GI you'll see that the duo does not cross the midline.
DR. BINGHAM: Perfect, associated syndromes? Testing loves associated syndromes with these childhood disorders. So what are some associated syndromes with malrotation?
DR. MCCLELLAN: So I've definitely seen that congenital diaphragmatic hernias can be present up to 20% of these patients and it's also associated, I think, with omphalocele more so than gastroschisis.
DR. BINGHAM: Perfect. Treatment?
DR. MCCLELLAN: The treatment is you resect the Ladd's bands counterclockwise detorsion so you want to take the intestine back to where it belongs and place the small intestine, as your final anatomy would be a small intestine on the right side of the abdomen and a large intestine on the left side of the abdomen and the last thing you want to do to avoid any diagnostic dilemma in the future is perform an appendectomy.
DR. BINGHAM: Right, so resect those Ladd's bands counterclockwise detorsion, counterclockwise you want to turn back time. Counterclockwise detorsion, place the small intestine on the right, large intestine on the left, appendectomy, perfect. That's your Ladd's procedure. Okay, so Meg, let's move on to our second cause of bilious nemesis and that's duodenal atresia, buzzwords for duodenal atresia.
DR. BINGHAM: [Dr. Kashyap] So this is something that you should Google search the image of it because it could be an X-ray finding that they show but it's the double bubble sign, you see two gas bubbles, one is the stomach and one's the duodenum.
DR. BINGHAM: Perfect and I would encourage everybody, so all these common kind of radiographic findings, Google image them, so go to Google, go to Google images, type it in a lot of times the one that shows up on the test is the first image that shows up on your Google image search so know what that looks like. So it's important to know that this is the number one cause of duodenal obstruction in neonates and let's come back to some associated abnormalities with duodenal atresia, Meg, take it.
DR. BINGHAM: [Dr. Kashyap] So polyhydramnios is one because the fetus is not swallowing the amniotic fluid, and then they can have cardiac, renal, GI anomalies and then one of the big associations is Down syndrome, about 20% of these patients have Down's.
DR. BINGHAM: Right, and I've seen that question asked as straightforward as that is, what's associated with Down syndrome. Okay, how do you diagnose it? [Dr. Kashyap] So you can do an abdominal X-ray like we said and see that double bubble sign and then upper GI will also show the blind-ending duodenum.
DR. BINGHAM: Perfect, treatment? [Dr. Kashyap] So again, with all of these, resuscitate first and then go to the operating room to reconnect, either to the duodenum, if possible or to the jejunum.
DR. BINGHAM: Perfect, so yeah, duodenostomy is the procedure of choice. Important to know that while you're in the OR these are often associated with other atresias at other portions of the intestinal tract so you need to be sure that you look for other intestinal atresias. And what causes these other atresias and where are they most common? [Dr. Kashyap] So they're usually caused by vascular accidents in the uterus and most commonly they're in the jejunum and they're usually multiple when there due to these vascular accidents.
DR. BINGHAM: Yeah, so intestinal atresias more distally are caused by intrauterine vascular accidents, but what about the cause of duodenal atresia? What's the underlying kind of pathology of that? Yeah, I think it's, John, correct me if I'm wrong, but it's failure of recanalization during--
DR. MCCLELLAN: Yeah that's the buzzword.
DR. BINGHAM: Yep, so that's your embryology so for duodenal atresia, and this is going way back to, I don't remember step one, step two failure--
DR. MCCLELLAN: Not that any of us knows what that means but it's what the word is.
DR. BINGHAM: Yeah, failure of recanalization whereas your distal atresias are due to intrauterine vascular accidents. I'm not sure if that'll show up but it's just fun to know.
DR. MCCLELLAN: [chukles] Okay, Kevin, meconium ileus, how does that present?
DR. KNIERY: So you want to be concerned for this if the infant doesn't pass stool within the first 24 hours from being born.
DR. BINGHAM: Yeah so no meconium passed in the first 24 hours, how do you diagnose?
DR. KNIERY: So you'd get an abdominal X-ray and you'd likely see a bowel obstruction, dilated small bowel, air, fluid, bubbles. And then you'd want to confirm your suspicion that the patient might have cystic fibrosis with a sweat chloride test.
DR. BINGHAM: Sweat chloride test, perfect. Again, as with all these, there're some associated syndromes or associated conditions, what's associated with meconium ileus?
DR. KNIERY: I mean the the biggest concern by far is cystic fibrosis and I think about 10% of the patients with cystic fibrosis will present with meconium ileus.
DR. BINGHAM: Okay, how do you treat it?
DR. KNIERY: So this is actually, most of the time you're able to get these kids open with a gastrografin enema, and if that doesn't work, you can progress to the mucomyst enema or the N-acetylcysteine enema and then just like everything else, if they develop peritonitis then you have to go to the operating room.
DR. BINGHAM: Okay, so when to operate? Operate for emergency, perforation peritonitis and also if you need to create an ostomy for antegrade N-acetylcysteine enemas would be another indication. So Kevin, what's another disease that doesn't pass meconium in the first 24 hours?
DR. KNIERY: Yeah this is a high Board probability question, it's the Hirschsprung's. This would be your other major concern in a patient not passing meconium.
DR. BINGHAM: Okay, tell us a little bit about that, Hirschsprung's disease.
DR. KNIERY: So it's the number one cause of chronic obstruction in infants and some patients present later in life with chronic constipation, it can be kind of more severe.
DR. BINGHAM: How does it present?
DR. KNIERY: It presents in a neonate with the same kind of presentation as meconium ileus with no stool in the first 24 hours and they have kind of an obstruction but in the more sub-acute versions they will have kind of chronic constipation that can be diagnosed later.
DR. BINGHAM: Okay and how do you make the diagnosis?
DR. KNIERY: So the classic thing here is you need to do a suction rectal biopsy, this is done at the bedside and the pathologists look to see if there's ganglion cells in the myenteric plexus, if they're absent, that confirms the diagnosis of Hirschsprung's. So that's the Board answer.
DR. BINGHAM: So how do these ganglion cells progress through the colon? I've seen this asked, does it come proximally and go distally, does it start distally and go proximally?
DR. KNIERY: This is when you're really differentiating the 99th percentile, but that would be, I believe they go from, they go caudate, they go from proximal to distal.
DR. BINGHAM: Yeah so it's failure of progression caudally of these cells, treatment?
DR. KNIERY: So you have to resect the aganglionic colon and so you can actually do suction biopsies in the operating room to help determine, but many times you'll be able to see a differentiation in the caliber of the colon to help determine how high you have to resect and then you can either make an ostomy or make a coloanal anastomosis and that gets into complicated pediatric surgery there.
DR. BINGHAM: Okay, perfect, let's move on. We're getting into weeds with that stuff. Let's talk about bloody stools in a kid, so now we've talked about cause of emesis bilius/non-bilius. Let's talk about bloody stools. So Meg, what's under differential for bloody stools? [Dr. Kashyap] So just remember that the currant jelly stools, it's not necessarily bloody stools, but the currant jelly stools is intussusception but then the next big one in a neonate, especially a premature infant, I would think about necrotizing enterocolitis and then in older children I would think about a Meckel's diverticulum.
DR. BINGHAM: Perfect, okay, so that's a good differential for bloody stools, let's walk it back. We've already talked about intussusception, we don't need to talk about that again, let's move on to the next one. Necrotizing enterocolitis, how does that present? [Dr. Kashyap] So it's usually in that premature baby who's in the NICU and they started enteral feeding and they get paged about bloody stools, the infant may also have abdominal distension or be septic.
DR. BINGHAM: Perfect and how do you make the diagnosis? [Dr. Kashyap] So you can do plain film X-ray and you'll see various things, you can see pneumatosis, free air, portal venous gas, and then if you have a high suspension for NEC, and at that point you would do serial lateral decubitus films to check for perforation.
DR. MCCLELLAN: Okay, and important to note that pneumatosis, again, like I said not indication for surgery alone, as well as that seems a little counterintuitive to adult surgeons but it's important to know for necrotizing enterocolitis. How do you treat these? [Dr. Kashyap] So you'd like to manage this by resuscitation, keep the baby NPO, give some broad spectrum antibiotics, start TPN, avoid the enteral feeding, and then eventually if you have a lower concern for perforation, you can put in an OG tube.
DR. BINGHAM: Okay and when do you operate on these? [Dr. Kashyap] So like you said, you don't need to operate if they have pneumatosis but if they have free air, if they're peritonitic, if they're deteriorating, a big one is if they have erythema on their abdominal wall and then portal venous gas is a poor prognostic indicator but I've heard both ways about whether it is an indication for surgery or not.
DR. BINGHAM: Great. So yeah these can be very very challenging and often not great outcomes but let's say that, something I've seen asked before, is that you had a patient that had a NEC, got taken to the OR, ended up with an ostomy or two and now you're thinking about taking down those ostomies. What do you have to get before you do that? [Dr. Kashyap] Yeah, so this is the big mistake that people make is not doing the barium enema prior to taking down those ostomies because due to the colitis and infection they can get all these obstructions, and so to make sure that they don't have stenotic portions you get the barium enema.
DR. BINGHAM: Perfect. Yeah if you reconnect those ostomies if they have a distal obstruction, you're going to be in a world of hurt when that baby leaks. Okay let's move on to our next cause of bloody stools, Meckel's diverticulum. So John, what's Meckel's diverticulum, what causes it?
DR. MCCLELLAN: So it's due to a persistent vitelline duct or omphalomesenteric duct I believe, too, is another name for it.
DR. BINGHAM: Um-hum, how does it present?
DR. MCCLELLAN: So this is your number one cause of-- let's say you got a pediatric patient with a painless lower GI bleeding, it's your number one cause of this. And it's usually present on the antimesenteric border of the small intestine.
DR. BINGHAM: Okay there's the famous rule of twos when it comes to the Meckel's diverticulum. What's the rule of twos?
DR. MCCLELLAN: Yeah there's a lot of these two, so it's typically two inches long, two centimeters in diameter, it presents in kids less than two years old, there's a 2:1 male to female predominance, it's two feet from the IC valve, it presents in 2% of the population, 2% are symptomatic and there's two types of tissues, pancreatic and gastric, and gastric is obviously the one most symptomatic, and there's obviously two presentations, Meckel's diverticulitis and bleeding.
DR. BINGHAM: Okay and how do you diagnose these if you don't diagnose it in the operating room?
DR. MCCLELLAN: So your Meckel scan is your best test for this but the scan will only pick up the gastric mucosa not the pancreatic mucosa.
DR. BINGHAM: Right, so your radionuclide Meckel scan is what you need to get, treatment?
DR. MCCLELLAN: The treatment for this is the resection of the diverticulum or you may need to do a segmental resection if they've significant diverticulitis, or it's involving the base of the diverticulum, or if the Meckel's diverticulum is greater than 1/3 the size of the bowel.
DR. BINGHAM: Okay that's a good general rule. We won't get into the minutia of doing a diverticulectomy versus doing a segmental resection, you do have to be sure you get that anomalous tissue there that could be causing problems, like bleeding, ulceration, that type of thing. Kevin let's move on to-- now we're moving out of our bloody stool segment and we're going to talk about some abdominal wall defects. It shows up a lot on tests.
DR. BINGHAM: The differentiating between gastroschisis and omphalocele, can you walk us through how you remember which is which and what characteristics fall under each category?
DR. KNIERY: Yeah, so omphalocele, I think of the "O' and I use the "O" of omphalocele as a kind of a completed sack and then it's also from the belly button, the "O" looks like a belly button, so it comes from the midline. And so the omphalocele is the more severe kind, though you don't have intestine kind of hanging in the breeze, it's covered by the peritoneal lining, it has more significant comorbidities with it, a lot of times there's cardiac and other abnormalities with this, whereas gastroschisis is kind of a little more traumatic in appearance as the bowel is kind of desiccated and not in the peritoneal sac and this is actually just to the right of the midline but the associations with this are less severe, generally just intestinal atresia, occasionally malrotation.
DR. KNIERY: And generally the patients with gastroschisis do very well once this is fixed and the etiology of gastroschisis is an in utero rupture of the umbilical vein.
DR. BINGHAM: Perfect, so like you say gastroschisis, in utero rupture of the umbilical vein, it's to the right of the midline and the intestine is not covered by a peritoneal sac, whereas omphalocele, failure of embryonal development does have a sac, is in the midline "O" like the belly button there, like the umbilicus as you were saying. And what's the initial treatment for these patients?
DR. BINGHAM: Both gastroschisis and omphalocele?
DR. KNIERY: Right, so it's generally you're going to use a saline soaked gauze and resuscitate the patients. You can put them either kind of in the Silo bag or a lot of times we will just reduce the intestine and do kind of an umbilical patch with the umbilical cord patch with the umbilical cord and then eventually do a primary closure.
DR. BINGHAM: Yeah, so you cover the bowel with the saline soaked gauze, resuscitate, sometimes you need TPN, NPO, replace the bowel, primary closure or do those, you know, the Silos to kind of gradually reduce the bowel and do a primary closure. Be sure you address the associated abnormalities, as Kevin alluded to and that's pretty much all you're going to see about those two I believe on the ABSITE so, let's move on to some more common things, some hernias in kids.
DR. BINGHAM: So Meg, umbilical hernia, what causes it, what do you do about it in children? [Dr. Kashyap] So umbilical hernias are pretty common in children because of a failure of the linea alba to close. So at birth it may still be open but they usually do close by the age of three, however, if they haven't then you typically like to repair by the time they're five before they enter school.
DR. BINGHAM: Great, how about comparing that to inguinal hernia, what causes it, how do you treat those? [Dr. Kashyap] So inguinal hernias in children are usually due to the persistent processus vaginalis and these actually do need to be repaired.
DR. BINGHAM: Okay, how do you repair them? [Dr. Kashyap] So it's a high ligation of the sac as opposed to adults where we do tissue repairs and mesh repairs, you just need to do a high ligation of the sac in children and then usually you want to do these repairs within 72 hours of reducing if it's incarcerated.
DR. BINGHAM: Okay, what about hydrocele in infants
DR. BINGHAM: Okay, what about hydrocele in infants similar but a little bit different, what are some different types of hydrocele and how do you approach those? [Dr. Kashyap] So hydroceles are similar in that it's also due to the patent processus vaginalis but there's no actual hernia sac extending into the internal ring. There's two types, one is communicating which is usually in the kids who have this waxing and waning bulge and swelling of their scrotum, and then non-communicating which usually resolve on their own because there's no communication, there's no fluid actively entering the hydrocele sac.
DR. BINGHAM: And treatment? [Dr. Kashyap] So this is also surgery if it's not resolved or if it's a communicating hydrocele, and you just resect the hydrocele and then you ligate the processus.
DR. BINGHAM: Okay. While we're talking about urologic disorders in children, let's talk about undescended testicles. So John, tell us about undescended testicles So John, tell us about undescended testicles and how you treat them?
DR. MCCLELLAN: So undescended testicles are usually diagnosed with an ultrasound or on physical exam first. You have to remember that questions you'll see on this, there's a risk of testicular cancer even after orchiopexy and typically this presents a seminoma, and you also need to be concerned about a chromosomal disorder if there's undescended testicles bilaterally.
DR. BINGHAM: And you kind of mentioned it, but treatment?
DR. MCCLELLAN: So the treatment for this is orchiopexy and you can also divide the spermatic vessels because there's a vas deferens collateral, too if you need to bring them down.
DR. BINGHAM: Yeah, so if you need to divide a vessel in order to get length to bring them down do your orchiopexy, you can do that for the spermatic vessels because of the collaterals. Good, so okay, let's move out of this territory and moving into another topic that's pretty high yield but fortunately there's not a whole lot you need to know about these but these are abdominal masses in infants. So we're going to cover three main ones that are the most likely to show up, neuroblastoma, nephroblastoma and hepatoblastoma.
DR. BINGHAM: So Kevin let's start with you, so neuroblastoma, how do they present?
DR. KNIERY: So these would generally be asymptomatic, the patient can have hypertension, diarrhea, raccoon eyes from orbital mets, and then less likely would be an unsteady gait.
DR. BINGHAM: Okay, where are they most commonly? Neuroblastoma, where's the most common location in children?
DR. KNIERY: Yeah so they can occur anywhere along the sympathetic chain but generally it occurs around the adrenal.
DR. BINGHAM: Okay and how about age distribution?
DR. KNIERY: So most common these are in young kids, so you want to remember that neuroblastoma is the young abdominal mass and that if it does happen when they're young it has the best prognosis.
DR. BINGHAM: How do you make the diagnosis if you suspect a neuroblastoma in a child?
DR. KNIERY: So you want to look to see if they have almost like a FEO, you want to see if they have elevated catecholamines, metanephrines, VMA, you can start with the abdominal X-ray but probably go straight to a CT scan to evaluate for an abdominal mass.
DR. BINGHAM: Okay, there's a couple of things that will sometimes show up that are associated with a high risk or worse prognosis when it comes to neuroblastomas, what are those things?
DR. KNIERY: Yeah, it's just one of those annoying questions that you every once in a while see is the high risk, worse prognosis is the neuron specific enolase, elevated LDH, a diploid tumor or N-myc amplification.
DR. BINGHAM: Right, so those are all associated with worse prognosis and as Kevin says, it's annoying but sometimes that stuff gets asked. Treatment?
DR. KNIERY: So I think resection is the primary mode if it is resectable, if not you can start with neoadjuvant doxorubicin.
DR. BINGHAM: Okay. So resection is typically the treatment as Kevin said, neoadjuvant therapy with doxorubicin if it's unresectable. Okay moving on, so that's neuroblastoma, nephroblastoma otherwise known as Wilms' tumor. So Meg, Wilms' tumor, how do they present? [Dr. Kashyap] So they're usually asymptomatic but they'll present with this abdominal mass that's growing, they can have hematuria, they can have hypertension and it's usually in children who are in the younger age group, the average age is usually about three years old.
DR. BINGHAM: Yep, so average age, three years old, like you say, can be asymptomatic, hematuria, hypertension. It's important to know that these will frequently metastasize to the bone and lung and a whole long XRT can be done from this. So let's say, you suspect a nephroblastoma Meg, how do you diagnose? [Dr. Kashyap] So you do a CT scan similar to the neuroblastoma, the one big thing to differentiate because they can be in the similar areas is that the nephroblastoma is from the nephrons and so you're going to have replacement of the renal parenchyma and you won't have the displacement that you would see in a neuroblastoma.
DR. BINGHAM: Excellent, it can be very difficult to distinguish between these two, so that's a key distinction. When we talk about nephroblastoma and Wilms' tumor, we often talk about WAGR syndrome, what is that? [Dr. Kashyap] So it's W-A-G-R the W is for the Wilms', A is for aniridia, G is for GU malformations and the R is for mental retardation.
DR. BINGHAM: Okay, great. And treatment? [Dr. Kashyap] So you do a nephrectomy and the big thing is not to upstage the tumor so you don't want to rupture the tumor and I think if you rupture it it's like automatically stage three and they require more chemotherapy in the adjuvant setting.
DR. BINGHAM: Great, okay. Last one, hepatoblastoma. John, tell us about hepatoblastoma in children?
DR. MCCLELLAN: So it's the most common malignant liver tumor, usually presents with increased alpha-fetoprotein, you may have a kid who has multiple fractures and precocious puberty and usually just treat it with resection or doxorubicin chemotherapy if it's unresectable.
DR. BINGHAM: Okay, so let's move on. Let's talk about some common hepatobiliary issues in children and probably the most common thing that's discussed is choledochal cysts. So choledochal cyst is thought to be caused by reflux of pancreatic enzymes in utero that's the pathophysiology behind it. There's five types, most common is type I which is fusiform dilation of the common bile duct and treatment is resection, sometimes require hepaticojejunostomy or even liver transplant.
DR. BINGHAM: So type I is fusiform dilation, type II is outpouching, type III is a choledochocele, type IV is both intra and extrahepatic dilation and type V is intrahepatic dilation and the treatment is going to vary depending on what type. The most common type as we talked about that you see on the test is the type I, which is resection, followed by hepaticojejunostomy. If you're dealing with your type IV, type V, sometimes those require segmental liver resections or liver transplantation.
DR. BINGHAM: And the overall goal of this is, why you operate is to reduce the chance of malignancy down the road. So that pretty much covers what you need to know about choledochal cysts for the ABSITE. Moving on, another biliary pathology, let's talk about biliary atresia. So Kevin, biliary atresia in children tell us a little bit about that.
DR. KNIERY: Yeah, so it's the most common indication for liver transplant, these children will generally present with neonatal jaundice that will persist after two weeks and the way you diagnose this is with a liver biopsy and you're looking for periportal fibrosis, bile plugs, cirrhosis, and you also need some cholangiography to help confirm.
DR. BINGHAM: Okay and what treatment?
DR. KNIERY: Treatment is the Kasai procedure which is the hepaticoportojejunostomy. You're basically draining their biliary system with an anastomosis of the jejunum to the bile ducts, like the intrahepatic bile ducts. If it's too progressed then you have to transplant.
DR. BINGHAM: Great, and so a good general rule there is a third of people improve, a third progress to transplant, and a third dies. That's your third rule for biliary atresia in infants. Okay moving on, we're almost done. We're in the home stretch of pediatrics for the ABSITE. Let's move on to thoracic, something that will show up and oftentimes you won't have a lot of exposure to in your training is congenital lung disease. So Meg, what are the different types of congenital lung disease that we have to worry about in kids?
DR. BINGHAM: [Dr. Kashyap] Yeah, so there's a few types, the big ones are the pulmonary sequestrations which are just lung tissue that does not actually communicate, it's sequestered from the tracheobronchial tree, has its own blood supply, usually from the aorta and venous drainage can be systemic or it can be pulmonary and these usually--
DR. BINGHAM: Talk a little bit more about that, because that shows up and it often confuses people where we talk about the extralobar sequestration versus the intralobar, how do you distinguish between those two? [Dr. Kashyap] All right, so extralobar would have systemic venous drainage whereas intralobar has pulmonary venous drainage. So again, if you think about it, so it's sequestered, so it's getting vascular supply extrapulmonary from the aorta directly and then if it's also extralobar then it's draining systemically but if it's intralobar, it's draining into the pulmonary veins.
DR. BINGHAM: Okay, great. And how does that present? [Dr. Kashyap] They usually present with infection or an abdominal chest X-ray, these can also be diagnosed in utero these days.
DR. BINGHAM: Okay and how do you treat them? [Dr. Kashyap] So you ligate the arterial supply and then you do a lobectomy.
DR. BINGHAM: Great, ligate arterial supply, lobectomy. So, just kind of remember that extra/interlobar is based on the the venous drainage because that can trip some people up. Okay, John, let's talk about congenital cystic adenomatoid malformation. What is that, what's the treatment?
DR. MCCLELLAN: Yeah, so CCAM, so the difference between these and pulmonary sequestration is that it communicates directly with the tracheobronchial tree but they're treated very similarly with a lobectomy.
DR. BINGHAM: Yeah, that's a key distinction, communication with the tracheobronchial tree for CCAMs versus pulmonary sequestration. Again a lot of these things trip people up so it's just important to kind of go through them and understand the differences because that's what's going to be the heart of the question. Kevin, congenital lobar emphysema, what is that, how does it present, how do you treat it?
DR. KNIERY: So these can be quite concerning if you get consults on one of these in the NICU, it looks basically like a tension pneumothorax on a chest X-ray in an infant with difficulty breathing. This is due to the failure of cartilage development in the lung.
DR. BINGHAM: Okay and as you said, it presents similar to a tension pneumothorax. What's the treatment and most importantly, what's not the treatment?
DR. KNIERY: Exactly, what is not the treatment is to be the night float guy who puts a chest tube in, you need to do a lobectomy on these patients.
DR. BINGHAM: Correct, so if you see an infant with this, what looks like a tension pneumothorax on an X-ray, you got to be concerned about congenital lobar emphysema and the answer is not chest tube. Okay and finally, wrapping up our congenital lung disease, Meg, bronchogenic cysts, what are they, how do they present, how do you treat them? [Dr. Kashyap] So these are another extrapulmonary cyst, they're of the bronchial tissue and cartilage. They usually present as a mediastinal mass and in the operating room they can have some milky liquid in them and you treat them with resection.
DR. BINGHAM: Yeah, so it's kind of easy all the congenital lung disease, you resect it, it's the treatment for most of these. So it's easy to remember. Okay to wrap up thoracic, congenital diaphragmatic hernia. A couple of different types, John, tell us what those are.
DR. MCCLELLAN: So you have two types your Bochdalek and Morgagni hernias. The Bochdalek is the most common one, it's usually posterior, and Morgagni is more of the rare of the two and is presented anteriorly. I don't really have a great way of remembering that but I usually think of Morgagni, more anterior, is the best way to remember it.
DR. BINGHAM: Bochdalek is back into the left.
DR. MCCLELLAN: Oh yeah, that works too.
DR. BINGHAM: Okay, so most commonly left-sided, liver protects on the right, and usually both lungs are dysfunctional when you have these congenital diaphragmatic hernia. What are some different associations with having congenital diaphragmatic hernia, John?
DR. MCCLELLAN: Yeah, so the associates would be severe pulmonary hypertension which kind of goes along with both lungs are dysfunctional. You can also have cardiac and neural tube defects and also, like I mentioned there's a 20% association with malrotation earlier.
DR. BINGHAM: Okay and most of these are these days diagnosed on prenatal ultrasounds or you know, you get a chest X-ray in an infant and see bowel in the chest. Treatment, Kevin?
DR. KNIERY: The treatment for these is ventilation, the primary concern here is obviously oxygenating them, so you can use inhaled nitric oxide. Some of these kids actually will require ECMO. And once they're stabilized you take them to the operating room and repair the defect.
DR. BINGHAM: Yeah it's actually, it's not so much the diaphragmatic hernia itself that's causing the problem, it's that severe pulmonary hypertension so ventilation is definitely the big issue with treatment of these. Okay, let's move away from the thorax, let's move up into the neck. There's just very few things you need to know, we'll talk about the difference between branchial cysts, thyroglossal duct cyst, and cystic hygromas which are pretty much the only things I've seen asked about the neck in a kid.
DR. BINGHAM: So Meg, branchial cleft cysts, where do these come from and how do they present, what's the treatment? [Dr. Kashyap] So there's multiple different types based on embryologic origin of these branchial clefts but most commonly there's the second branchial cleft cyst which is in the middle of the anterior SCM so each of these kind of travel along the sternocleidomastoid and the treatment for them is resection and so I think the big thing in question stems is always when they talk about a cyst in the lateral portion of the neck.
DR. BINGHAM: So again, the sternocleidomastoid versus the thyroglossal cysts that are midline.
DR. BINGHAM: So John, moving on, thyroglossal duct cysts, tell us a little bit about those, how they're formed and what the treatment is.
DR. MCCLELLAN: Yeah so these are like Meg already mentioned, they're midline, they typically go through the hyoid bone. They're formed due to abnormal descent of the thyroid gland but also may be the patient's only thyroid tissue as well, it tracks down from the kind of posterior tongue. The treatment for this is the Sistrunk procedure which is the incision of the cyst, of the track, and then if the hyoid bone is significantly involved you may also need to resect that as well.
DR. BINGHAM: Great, and lastly, Kevin, cystic hygroma, how does it present, what is it, how do you treat it?
DR. KNIERY: So this is the lateral neck that's in the posterior triangle, so you know behind the sternocleidomastoid. This would be soft cystic multiloculated, they get kind of recurrent infections. It's lymphatic malformation connecting sometimes to the internal jugular. The treatment for these is eventually to resect them.
DR. BINGHAM: Okay, perfect. Okay, so, that does it, that's pediatric surgery for the ABSITE. We're going to do some quick hits here real quick and then we'll be done. So let's go through these real quick, so Meg, quick hit number one, which immunoglobulin crosses the placenta? [Dr. Kashyap] IgG.
DR. BINGHAM: IgG crosses placenta, right. John, which immunoglobulin is transferred through breast milk?
DR. MCCLELLAN: IgA.
DR. BINGHAM: IgA, okay. Most commons. Okay so our most common malignancy in kids, Kevin?
DR. KNIERY: Leukemia, specifically ALL.
DR. BINGHAM: Okay, most common solid tumor, Meg? [Dr. Kashyap] They are CNX tumors.
DR. BINGHAM: Okay, John, most common abdominal tumor?
DR. MCCLELLAN: Your neuroblastomas.
DR. BINGHAM: Your neuroblastomas, right. Kevin, most common lung tumor?
DR. KNIERY: Carcinoid.
DR. BINGHAM: Carcinoid tumor is the most common lung tumor in children. Meg, most common tumor of the pediatric larynx is? [Dr. Kashyap] It's the laryngeal papillomatosis and this is the tumor that actually involutes after puberty.
DR. BINGHAM: Interesting. Okay, John you have a one month old with an elevated AFP and beta hCG, what's the diagnosis?
DR. MCCLELLAN: So it's sacrococcygeal teratoma and those are usually malignant at this age.
DR. BINGHAM: No, they're usually malignant after two months old.
DR. MCCLELLAN: Oh, sorry.
DR. BINGHAM: So malignant after two months old, so elevated AFP, beta hCG, sacrococcygeal teratoma, after two months usually malignant.
DR. MCCLELLAN: Clear.
DR. BINGHAM: Of course. Kevin, most common anterior mediastinal mass?
DR. KNIERY: Teratoma.
DR. BINGHAM: Terrible teratoma, okay. Meg, most common overall mediastinal mass? [Dr. Kashyap] These are the neurogenic tumors which are usually in the posterior mediastinum.
DR. BINGHAM: Correct. John, you have an infant with intermittent respiratory distress and poor suckling, what are you thinking?
DR. MCCLELLAN: So, I think a choanal atresia and it will most likely need surgical correction.
DR. BINGHAM: Yep, surgical correction. Kevin, most common airway obstruction in infants?
DR. KNIERY: Laryngomalacia.
DR. BINGHAM: Okay, and what is that?
DR. KNIERY: It's like when the larynx isn't fully formed and it's kind of floppy, for lack of better words, this will go away as the epiglottic cartilage matures.
DR. BINGHAM: Perfect. Meg, a two-year-old continues to have wheezing and recurrent infection, the tracheal rings are elliptical on bronchoscopy. [Dr. Kashyap] So that's the buzzword for tracheomalacia and you usually treat it with aortopexy, so that opens up the trachea.
DR. BINGHAM: Great. John, six months old with red lesion growing on face and scalp?
DR. MCCLELLAN: Yeah, these are usually hemangiomas and most of them resolve by when they're about eight years old otherwise, if they persist or relatively large you can use steroids or laser treatment.
DR. BINGHAM: Okay. Kevin, last quick hit, a 13-year-old boy has a large sunken area of the chest, what's the diagnosis, what's the treatment?
DR. KNIERY: This is a pectus excavatum, you can treat this with a Nuss bar and then the pectus carnitatum (sic), [chuckle in the background] pectus carnitum (sic)-- [Dr. Kashyap] Carinatum.
DR. KNIERY: How do you say it?
DR. MCCLELLAN: Carinatum.
DR. KNIERY: Well, John you can't say intestine right, so--
DR. MCCLELLAN: Yeah, that's true. [chuckles]
DR. KNIERY: So maybe just you, you do that one, I can't say it. [Dr. Kashyap giggles] Now I'm really confused, so and then there's also pectus carinatum which is the pigeon chest and you don't actually repair this you can just do chest braces.
DR. BINGHAM: Perfect, okay, that does it for our quick hits, that does it for our pediatric ABSITE review, thanks everybody for listening to Behind the Knife's ABSITE review. As we mentioned earlier join our mailing list, follow us on Facebook, we have a Youtube channel that has a lot of educational videos. Find us on Youtube and follow, continue pressing on, continue studying and dominate the ABSITE.
DR. BINGHAM: [narrator] Until next time, dominate the day. [upbeat outro music]