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SCORE School Pediatrics, Part 1 of 4
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SCORE School Pediatrics, Part 1 of 4
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Language: EN.
Segment:0 .
ALFRED CHAHINE: OK. Good afternoon everyone, and welcome to SCORE School. My name is Alfred Chahine. I'm the Assistant Editor of SCORE, and I will be your moderator today. A few housekeeping items before we start. Today is the first day when we switch from the Wednesday evening sessions to the Friday afternoon ones. We did this for a few reasons.
ALFRED CHAHINE: The two main ones are to be able to have the recording available at the beginning of the week for you all to use when reviewing the TWIS topics within your program. And the second reason is that when we surveyed the SCORE School users a couple of weeks ago, the time and day did not seem to be of tremendous importance to the majority. So we felt that Friday afternoon would be better.
ALFRED CHAHINE: As always, we encourage you to use the chat box to ask questions and make comments. Everyone has been muted to avoid unnecessary noise. You will see a QR code for attendance tracking displayed a few times throughout the presentation. You only need to submit it once, and you'll receive an email confirming your attendance. All of the SCORE School sessions are being recorded for later viewing on the SCORE website.
ALFRED CHAHINE: You should be aware that the names on the chat will be recorded as well. We encourage you to view these recorded sessions as your schedule allows. So today's session, the topic is pediatrics. The first out of four throughout the two-year cycle. And we'll be discussing a few modules. Some of them were discussed in the last pediatrics module of the previous cycle.
ALFRED CHAHINE: So we'll have a few redundancies, but that's OK. The topics the modules are Meckel's diverticulum, appendicitis, biliary atresia, choledochal cysts, branchial cleft anomalies, congenital diaphragmatic hernias, chest wall deformities, and neonatal small bowel obstruction. It is my pleasure to introduce to you our faculty presenter Dr. Jason Sulkowski. He is an Assistant Professor of Surgery and Pediatrics at the Virginia Commonwealth University in Richmond, Virginia.
ALFRED CHAHINE: He's a graduate of the Columbia University College of Physicians and Surgeons. He did his residency at SUNY Downstate Medical Center in Brooklyn, New York. During that time he spent two years doing research at Children's Hospital of Philadelphia and Ohio State. He completed a Pediatric Surgery Fellowship at Children's National Health system, where I had the privilege to work with him for two years.
ALFRED CHAHINE: A personal note on Jason is that he's the only fellow that I have worked with over a 22-year career who would go in and observe how faculty teach residents in the operating room. So that tells you about his dedication and his yearning to teach. He's a member of multiple organizations, and was honored with a Teaching Award as a resident while a resident in Brooklyn.
ALFRED CHAHINE: Thank you, Dr. Sulkowski, for lending your expertise to us and take it away, please.
JASON P SULKOWSKI: All right. Thank you very much. Thanks, Dr. Chahine and everyone else at SCORE for allowing me to give this presentation today. So let's just jump right into it. These are our topics for the day. Next slide. So we're going to start with a 3-year-old boy who has two days of bloody stools.
JASON P SULKOWSKI: What kind of differential does this case scenario make you think of?
ALFRED CHAHINE: Abbas, you're on.
JASON P SULKOWSKI: All right. So the differential for a child with bloody stools is pretty wide, and a lot of it's going to depend on what age the patient is. So you've got a neonate, you're going to be thinking about anal fissures, necrotizing enterocolitis, a milk protein allergy, or more concerning would be malrotation with volvulus. In an infant or a child, such as this patient, you're thinking intussusception, a Meckel's diverticulum, anal fissure, or infectious colitis.
JASON P SULKOWSKI: In an older patient, an adolescent, you'd be thinking inflammatory bowel disease. Again, colitis, a Meckel's, perhaps enteric duplication that's becoming symptomatic, or even hemorrhoids. Next slide. So taking into consideration this patient, two days of bloody stools. Imagine that the patient also is reporting no other symptoms.
JASON P SULKOWSKI: His abdomen has no pain whatsoever. What kind of imaging would you want to pursue? Next slide. So usually the first thing that we'll do in this sort of a patient is a Meckel's scan, because this is a pretty classic presentation for a Meckel's diverticulum.
JASON P SULKOWSKI: So we'll go right for the source. So we do a Meckel's scan, which is nuclear medicine study. The sensitivity isn't perfect. It ranges from about 60% to 85%, so there are a decent number of cases that go undetected by this study. The idea of the study is that it detects gastric mucosa. So as you can see on the image there on the right of the screen, the stomach is lighting up and then there's another area in the right lower quadrant that's lighting up.
JASON P SULKOWSKI: And that's consistent with heterotopic gastric mucosa in a Meckel's diverticulum. Next slide. So everyone should be familiar with the rule of twos for a Meckel's. This is a pretty commonly known mnemonic. They present in about 2% of the population. They are usually located two feet proximal to the ileocecal valve.
JASON P SULKOWSKI: They're usually two inches in length. They commonly have two types of ectopic mucosa, either gastric or pancreatic. About 2% of them become symptomatic over a patient's lifetime. And there's a 2 to 1 male to female ratio. Next slide. The embryology of a Meckel's is that it's a remnant of the omphalomesenteric duct.
JASON P SULKOWSKI: This duct is a communication in the fetus between the intestine and the extraembryonic yolk sac. The figure here on this slide shows all the different manifestations of a duct remnant. You can have a completely patent duct, which communicates the umbilicus to the bowel. You can just have umbilical anomalies like a polyp or a cyst. Or you can have a remnant cord or a Meckel's diverticulum.
JASON P SULKOWSKI: Next slide. Clinically, they can present a number of different ways. In younger children, about two or three years old, such as in the case that was presented, they most commonly will present with bleeding and no other symptoms. So we call it painless bleeding. In older children they're more likely to present with perforation of the Meckel's like you can see here in this picture.
JASON P SULKOWSKI: They can also present with obstruction. In older patients, neoplasm can arise from the ectopic tissue. And then they can even present with foreign body retention, because it's sort of like an open lumen, sort of like an appendix. And so you can get swallowed objects or fish bones or things like that occasionally. Next slide.
JASON P SULKOWSKI: So a patient who is discovered to have a Meckel's that's symptomatic requires an operation. This can usually be done laparoscopically, sometimes converted to open depending on what we find. If you find a Meckel's, such as in this case, and the base of the tissue is healthy, you can simply come across it tangentially with the GIA stapler. And you staple transverse to the direction of the intestine just like in this picture.
JASON P SULKOWSKI: If the base of the Meckel's is not healthy tissue, for example if there's a perforated ulcer or something like that, it's usually safer to do a segmental resection of that small segment of small intestine and perform a primary anastomosis. Next slide. Now what happens if you're doing an operation for another reason and you find a Meckel's in the patient? So removing a Meckel's comes at about a 4% risk of some sort of complication like a wound infection or a leak.
JASON P SULKOWSKI: However, the lifetime risk of symptoms developing from a Meckel's is approximately 3% to 6%. And then if you look at the number needed to prevent a death, you have to treat 800 patients in order to prevent one death. So that's a pretty significant number needed to treat ratio. There's some surgeons who would always remove a Meckel's that they discover because there is a slight risk of a neoplasm, but there is no specific right or wrong answer about this in particular.
JASON P SULKOWSKI: I think the best idea is to have a standard approach and just always follow the same rule. Next slide. OK. So now we have a 10-year-old female--
ALFRED CHAHINE: Dr. Sulkowski, can I stop you for a second?
JASON P SULKOWSKI: Yeah.
ALFRED CHAHINE: Can you speak to the-- so when you describe the operation, tangential stapling of the base, could you speak briefly about the idea and the concern about the ulcer and the bleeding being on the ileum, and whether you need to do a resection versus just a diverticulectomy?
JASON P SULKOWSKI: Yeah. So in the past the teaching was to all the time perform a bowel resection and in an anastomosis. However, more recently a number of studies have shown that in most cases you can safely staple just across the base of the Meckel's, sort of like doing an appendectomy. And as long as you do it in a transverse direction so you don't narrow the intestine, it is safe to do.
JASON P SULKOWSKI: If the tissue doesn't look healthy, like it's not going to hold the staples very well, then the safer thing to do would be a segmental resection in that case. A lot of the concern previously was that if there's bleeding or if there's Meckel's diverticulitis or something like that, that the tissue might not necessarily be able to hold staples pretty well.
JASON P SULKOWSKI: But unless it's very obviously ischemic-looking at tissue, it actually holds up pretty well.
ALFRED CHAHINE: Thank you.
JASON P SULKOWSKI: All right. So now we've got a 10-year-old girl who's coming in with one day of right lower quadrant pain, fever, and several episodes of vomiting. What could this be a presentation for?
ALFRED CHAHINE: Dr. Badami, You want to respond?
JASON P SULKOWSKI: OK. We can just go to the next slide.
ALFRED CHAHINE: I'm trying to, he says. OK. So it can be appendicitis.
JASON P SULKOWSKI: That's right. So I mean this can be a handful of things. Probably the classic presentation of appendicitis, but you do have to kind of go through your differential. The most common finding when it's not appendicitis is simple viral gastroenteritis or mesenteric adenitis. Obviously, there's appendicitis. In a female with right lower quadrant pain you have to think about ovarian torsion or an ovarian cyst. If this were a boy, testicular torsion can present with right lower quadrant pain.
JASON P SULKOWSKI: And then in this age group, a first episode of inflammatory bowel disease is always a possibility to consider as well. Next slide. So appendicitis is the most common reason that we're called to the pediatric emergency room for a consult, and it makes up anywhere from 5% to 10% of all pediatric emergency room visits.
JASON P SULKOWSKI: And abdominal pain alone is the single most common reason for any pediatric emergency room visit. I mean, it's a common problem. About 7% to 9% of people will end up developing appendicitis in their lifetime with a slightly increased risk in boys. Next slide. The pathophysiology of appendicitis is the appendiceal lumin becomes obstructed, commonly with a fecalith.
JASON P SULKOWSKI: And there can also be lymphoid hyperplasia, rarely a mass. This leads to increased pressure within the appendix, bacterial overgrowth which then causes venous outflow obstruction. And that leads to wall ischemia and bacterial invasion into the wall of the appendix. Ultimately, that wall will perforate which causes luminal contents to leak. And then if this isn't treated in time, a phlegmon and ultimately an abscess will form.
JASON P SULKOWSKI: Next slide. So on history you're going to get pretty much the standard story in many patients. They'll start out with generalized abdominal pain that will then be located in the right lower quadrant. However, in kids the variety of presentations can be quite wide, and so you've got to have a pretty high index of suspicion for appendicitis.
JASON P SULKOWSKI: It's often also associated with fever, nausea, vomiting, diarrhea, and painful urination. On physical exam they'll have a fever, sometimes they're tachycardic. Sometimes they look pretty well, sometimes they look pretty sick. It really varies. And then on exam they'll most commonly have pretty focal right lower quadrant tenderness.
JASON P SULKOWSKI: It may or may not have rebound, and they'll have a Rovsing sign, which is pain on the right side of the abdomen when you press on the left side. Labs are sometimes obtained, although not always in kids. You'll see an elevated white blood cell count with a left shift. And then the most common imaging to start out with is an ultrasound.
JASON P SULKOWSKI: That is user dependent of course, and so sometimes it's difficult to visualize the appendix. And then in most hospitals, the next step will be to get either a CT scan or an MRI. Here on the right, you can see a CT image with arrows pointing at two slices of the same appendix, and there's a little fecalith that you can see in the more cephalad portion. Next slide.
JASON P SULKOWSKI: And then here you can see an MRI image. And the appendix is kind of hard to make out, but there is a little measuring line on it that shows that it's one centimeter in length. And you can see it's a little bit more lit up and hyper compared to the surrounding tissue and bowel. Next slide. So initially, once you've made the diagnosis, all patients need to be given IV antibiotics right away.
JASON P SULKOWSKI: In most hospitals I think the more common treatment is ceftriaxone and flagyl, but it kind of varies based on local IV practice. And then for most patients they're going to head to the operating room for an urgent-- not an emergency, but urgent laparoscopic appendectomy. Some patients are treated non-operatively. It's been increasingly common to treat patients with early simple appendicitis with antibiotics alone.
JASON P SULKOWSKI: This is primarily done in research studies at academic centers. The success rate has been shown to be about 70% at one year. The other indication for non-operative management would be in a patient who has clearly perforated and has a phlegmon or some well-developed abscess. Patients in this case are treated with IV antibiotics until they are symptomatically better, and then they're usually treated with a complete course with oral antibiotics at home.
JASON P SULKOWSKI: If there is a drainage collection then they can head to IR and have percutaneous tenuous drainage. Whether or not they go to the operating room for an interval appendectomy is controversial. Usually if there's a fecalith present, the best thing to do is remove the appendix in about 6 to 8 weeks after the patient's recovered from the initial episode. However, some people elect to just observe the patient afterwards.
JASON P SULKOWSKI: Next slide. So post-operatively after they go for an appendectomy, there are a wide variety of protocols for the duration of antibiotics. So pretty much across the board if someone has simple appendicitis, a non-perforated appendix, they won't get any additional antibiotics. And once they're eating and their pain is under control, they'll be able to go home.
JASON P SULKOWSKI: But for perforated appendicitis, most kids will stay on antibiotics and the duration is, like I said, there's a wide variety. There's really no one right protocol. The practice has been to decrease the duration over time, and most centers are treating approximately five to seven days of antibiotics for perforated appendicitis.
JASON P SULKOWSKI: So most patients are able to be discharged when they're afebrile, when they're tolerating a diet, and when their pain is under control.
ALFRED CHAHINE: Dr. Sulkowski, can I interrupt you for a second. We have two questions in the chat about appendicitis. Dr. Badami is asking, Dr. Sulkowski, I know you have done extensive research in treating appendicitis non-operatively with antibiotics. How has your approach changed over the years in practice? Do you still defer surgical management?
JASON P SULKOWSKI: So that's a great question. So now that I'm not involved specifically with the research study, my practice is typically to recommend to have an appendectomy for patients with early appendicitis. I think it's a reasonable option, and if families ask about it then I'm happy to go down that route. But my personal practice has been to perform appendectomies for early appendicitis.
JASON P SULKOWSKI: I think it's still being actively studied around the country and in other countries as well. And I think there's still a lot to learn about that particular treatment modality, but that's not my personal practice right now.
ALFRED CHAHINE: And then Dr. Joshi asks, is it still felt with each hour of delay between diagnosis and appendectomy the risk of rupture increases, or do IV antibiotics arrest that timeline? Very good question.
JASON P SULKOWSKI: Yeah, that's a great question. So it's felt that in most cases if you start antibiotics right away, that buys you some time to get to the operating room. So it's pretty uncommon to take a child to the operating room in the middle of the night for an appendectomy. There have been a number of studies that have shown that doing so does not increase the risk of perforation and it also does not increase the risk of post-operative infectious complications.
JASON P SULKOWSKI: Clearly if you wait several days, that sort of gets thrown out the window and you do have to get to the procedure urgently. But it's no longer the emergency that it used to be considered. OK. So now if you have a patient who is 5, 6 days out from surgery and they're still not quite turning the corner, they're having fevers, they're still having a lot of pain, or they're not eating well, that's a patient who might have developed a post-operative collection, which is definitely more common in kids with perforated appendicitis.
JASON P SULKOWSKI: So the inclination is you start getting kind of antsy a few days after surgery, and you want to go in and look for something, but you really should wait at least five days before reimaging. Because first of all, you'll minimize the number of additional studies done. Sometimes you'll find a really tiny collection that will get better just with antibiotics.
JASON P SULKOWSKI: And it also gives a little bit of extra time for a collection to organize into something that the interventional radiologists are able to drain. Next slide. Complications after an appendectomy. The most common by far is a surgical site infection. The risk is lower doing laparoscopic appendectomies than it used to be for open appendectomies for superficial and site infection.
JASON P SULKOWSKI: For a deep infection, the risk is only about 1% or 2% for simple appendicitis, but that rises to almost one in five patients with perforated appendicitis. The other risk is readmission. This is about 5% to 10% of patients. Most commonly they're getting readmitted because they've developed an abscess or some other infection at home. Rarely they come in with a bowel obstruction or an ileus or just uncontrolled pain.
JASON P SULKOWSKI: Next slide. There's an opportunity to get your QR code. Next slide. OK. So we've got a three-day-old infant who is in the NICU with vomiting and has just not been able to take any feeds.
JASON P SULKOWSKI: What could be going on?
ALFRED CHAHINE: Abbas is saying, pyloric stenosis, duodenal atresia.
JASON P SULKOWSKI: OK. All right. Next slide. OK. So when you're thinking about a child with a possible bowel obstruction, you've got to kind of think about the whole range of possibilities. And the most important distinction to make initially is whether or not the vomit is bilious or non bilious. So if it's non bilious emesis that means the most likely the obstruction is more proximal in nature.
JASON P SULKOWSKI: And so by proximal it means proximal to the pyloris and proximal to the ampulla of Vater where bile would be coming from. So two common explanations for this would be some sort of atresia, like esophageal atresia, or duodenal atresia. With bilious emesis and more distal obstruction, the first thing you have to think about and rule out is malrotation with volvulus.
JASON P SULKOWSKI: There could also be some other more distal intestinal atresia, either in the small bowel or in the colon. It just could be a meconium ileus, Hirschsprung's disease, imperforate anus, or some other sort of colonic cause of obstruction. Next slide. So when you first go to assess this patient, the first thing you have to answer is whether or not the infant looks sick or if they look OK.
JASON P SULKOWSKI: So you're going to want to look at their vital signs and see if they're tachycardic, if they're hypertensive, is the patient already intubated, are they on high vent settings, what kind of support is the baby already requiring. And then focusing on the abdominal exam. Are they soft? Does it seem like a relatively benign exam? Are they quite distended?
JASON P SULKOWSKI: Are you even noticing skin changes on the abdomen? Those are the concerning findings that would cause you to expedite your work up and perhaps get to the operating room a little bit faster. Next slide. So as long as there's not anything particularly alarming like a distended discolored abdomen, you're almost always going to start with a plain X-ray just to kind of get an idea of what's going on.
JASON P SULKOWSKI: So as you can see here, this is a classic film for an esophageal atresia with OG2 coiled up in the upper pouch and then air in the stomach consistent with a type C tracheoesophageal fistula with esophageal atresia. Next slide. You can also identify a duodenal atresia here. You can see an air-filled stomach and then an air-filled duodenum with no distal air whatsoever.
JASON P SULKOWSKI: So this is a classic double bubble found with duodenal atresia. Next slide. Intestinal atresia would show a little bit more gas-filled loops of bowel, but still a paucity of bowel gas. And you can clearly tell that there's no air in the colon and no air in the rectum.
JASON P SULKOWSKI: So this would be concerning for a jejunal atresia. Next slide. And then here you can see that there's quite a bit of air-filled intestine, but this is concerning for a distal obstruction like a colonic obstruction because you can tell that there is no air in the rectum. So there's definitely a distal obstruction in a film like this.
JASON P SULKOWSKI: Next slide. So if you've got a bilious emesis or the baby looks sick, you want to get an immediate upper GI to rule out volvulus. Here this upper GI shows a bird's beak and no contrast passing the proximal small intestine. So this is very concerning for a volvulus, and this baby needs to go immediately to the operating room for exploration.
JASON P SULKOWSKI: Next slide. Now if the upper GI looks normal and you see your normal seal up of the duodenum, then often you can at the same time get a contrast enema if you're thinking about the possibility of a more distal obstruction. This is particularly helpful for colonic issues. This film here is showing a classic image for meconium ileus.
JASON P SULKOWSKI: You can see that there is a diminutive left colon and rectum. And then in the small intestine you can see the filling defects which are consistent with the inspissated meconium in the terminal ileum. Next slide. So if the baby is sick or you suspect that there's a volvulus, the baby needs to go to the operating room right away.
JASON P SULKOWSKI: If you identify volvulus in the operating room, need to untwist the bowel until you start to see the bowel is profused again, and then you perform a Ladd procedure. I'm not going to go into specifics of that, because that's a separate topic. But that's sort of the basic of what you would do for that particular diagnosis. Next slide.
JASON P SULKOWSKI: Now if the baby doesn't look particularly sick and you've got your film, your management's going to be based more on your suspected diagnosis. And you've got a little bit more time to do additional studies like the contrast study, either an upper GI or contrast enema, in order to kind of pinpoint exactly the anatomic location. Pretty much all of them, if there's any kind of bowel obstructions, they're all going to require some sort of surgical intervention in order to fix the problem, with the exception being for meconium ileus.
JASON P SULKOWSKI: The management for that is frequently just to repeat contrast enemas, because that ends up freeing up that inspissated meconium and allowing motility to start. Next slide. So the takeaway for this module in this topic is really just the initial assessment and evaluation. Determine if the baby looks sick. Is the vomiting bilious.
JASON P SULKOWSKI: If either of those are going on, the baby needs an upper GI right away and probably needs to go to the operating room very quickly. And then we tend to use in pediatrics and especially with babies plain x-rays and contrast studies in order to pinpoint the location of the obstruction. You'll notice that what's really missing compared to how adults are usually treated, is a CT scan. It's very uncommon for us to have to get a CT scan for anything in the surgical treatment for a neonatal bowel obstruction.
JASON P SULKOWSKI: Next slide. All right. So new patient. We've got a one-month-old full term male who's presenting with jaundice, pale stools, and--
ALFRED CHAHINE: Dr. Sulkowski, sorry to interrupt you. We have a question, hold on, before we move on. Dr. Badami says, I had operated last week on a baby with intestinal atresia with Dr. [? Belichick-- ?] your mentor, Jason. Intraoperative evidence of extensive vascular compromise to small bowel, majority jejunum resected, the necrotic jejunum, because of the atretic bile we could not find intraluminal opening to create a jejunostomy.
ALFRED CHAHINE: Thoughts? How do you manage such cases?
JASON P SULKOWSKI: Oh, wow. I mean, that's a very complicated situation. I mean, it's hard to give specifics without being in the operating room with you. But I mean, sometimes if you've got-- you can have a case of intrauterine volvulus and you have very little bowel left. The baby has severe short bowel syndrome. And to the best of your ability you want to just find the most proximal bowel you can and bring it up as a stoma temporarily.
JASON P SULKOWSKI: And a lot of times these infants will require long-term TPN until you're able to get them back in the operating room and try to get them back into continuity. But that's a really difficult situation.
ALFRED CHAHINE: Sounds like it, yeah.
JASON P SULKOWSKI: All right. So back to this case. So now we've got a one-month-old full term male who is presenting with jaundice, pale stools, and dark urine. So what could be going on in this baby? Next slide. All right. So we've got a jaundiced baby.
JASON P SULKOWSKI: The differential for hyperbilirubinemia in infant is quite extensive, and only a handful really pertain to surgically correctable issues. So it includes obstructive problems like biliary atresia, choledochal cysts, cystic fibrosis, Alagile syndrome, gallstones. It also includes the hepatocellular issues like TORCH infections, idiopathic neonatal hepatitis, and metabolic disorders.
JASON P SULKOWSKI: One of the most important things you have to do is determine what component of the bilirubin is direct, and that'll raise your concern if that's elevated for biliary atresia or choledochal cysts, which are both the primary surgically correctable causes of cholestasis. Next slide. So biliary atresia in particular is a fortunately uncommon problem.
JASON P SULKOWSKI: It occurs in just under one in 10,000 live births. It presents most commonly as a sporadic condition without any associated anomalies. About 85% of babies are in that group. And then there are two different categories of syndromic biliary atresia. One is just associated with other anomalies, but they're not laterality defects. And then the other group is called the biliary atresia splenic malformation anomaly, and this includes malrotation in situs inversus in addition to the biliary atresia and splenic issues.
JASON P SULKOWSKI: Biliary atresia is thought to be caused by progressive injury to the bile duct epithelium. It typically starts in the extrahepatic ducts and then works its way retrograde into the enteropatic ducts. It's unclear at this time what the pathophysiology of the disease really is. Some people think that it could be due to a viral infection. It could have an autoimmune etiology.
JASON P SULKOWSKI: And then there's even the possibility of perinatal or prenatal toxin exposure as a cause. Biliary atresia if left untreated will progress to cholestasis, cirrhosis, portal hypertension, and ultimately end stage liver disease. Next slide. So the evaluation of an infant with jaundice would start with history, and jaundice is pretty common in infants.
JASON P SULKOWSKI: But usually beyond about two weeks of time that becomes abnormal. The baby will also have acholic stools and dark urine. The image on the right shows a stool color card that's often used in order to determine whether or not a baby's stool is normal or abnormal in color. On exam usually these babies are pretty much normal in weight. And actually pretty healthy appearing, at least early on. They'll obviously be jaundiced.
JASON P SULKOWSKI: If the disease has progressed, sometimes you can feel a firm palpable liver edge and enlarged spleen due to portal hypertension. On labs, as I mentioned, they'll have a direct hyperbilirubinemia. This usually plateaus at about four to six, which is in distinction from other causes of cholestasis which will frequently have even higher levels of direct bilirubin.
JASON P SULKOWSKI: Sometimes transaminases will also be elevated. You'd want to do a TORCH workup, check alpha-1 antitrypsin levels, and also check your coags because these babies will start having vitamin K issues and so they'll potentially be coagulopathic. Next slide. So as with almost every other condition in babies or in pediatrics, we'll start with an ultrasound as one of our first imaging studies.
JASON P SULKOWSKI: What you might find is the absence of a gallbladder or potentially a very tiny diminuitve gallbladder. Sometimes you can see what's known as the triangular cord sign, which is basically the scarred down portal plate. You'll see it at the bifurcation of the portal vein as you can see on the image on the right. You can also sometimes see cystic dilation of the common bile duct.
JASON P SULKOWSKI: There is an ideology of biliary atresia or cystic biliary atresia that can sometimes be confused for a choledochal cyst. Next slide. Another scan that's frequently obtained is a high dose scan which we're pretty familiar with for taking care of cholecystitis. In babies we typically will pre-treat them for five days with phenobarbital in order to increase the quality of the study.
JASON P SULKOWSKI: What we're looking for is failure of the tracer acid to excrete into the small intestine. If you see tracer going into the small intestine, then you know you've got a patent extrahepatic biliary system. And so you know you do not have biliary atresia. But as you can see on the study here on the slide, the liver is lit up but there's nothing lighting up the small intestine.
JASON P SULKOWSKI: So this would be concerning for biliary atresia. Next slide. And finally, the gold standard diagnosis is made with an interoperative cholangiogram. This can be done either open or laparoscopically. And what you want to see is contrast reflexing back up into the liver and then also going into the intestine. You can see here in the image the duodenum has contrast in it and then you can see where the right and left bile ducts are going approximately up into the liver.
JASON P SULKOWSKI: Next slide. Frequently, these patients will undergo percutaneous biopsy before they go to the operating room. This can sometimes help with the diagnosis. Early on in the course, in younger infants, this might lead to false positives. But it can be helpful in older kids, because they can determine the degree of hepatic fibrosis.
JASON P SULKOWSKI: And this can be very helpful information for prognosis in determining the course of treatment. On a biopsy with biliary atresia the classic findings are proliferation of bile ducts, portal fibrosis, and plugging of the canaliculi and the bile ducts. Next slide. So when biliary atresia is of concern, the next step is usually to go to the operating room to perform the interoperative cholangiogram.
JASON P SULKOWSKI: And if it's positive, the patient requires a Kasai portoenterostomy. So the Kasai procedure consists of resecting the atretic extrahepatic biliary system. So you would take the gallbladder if it's present and then the common bile duct. You'd resect this all the way up to the portal plate which is located, like I said, at the bifurcation of the portal vein. And then this is sharply transacted and that's going to get you to your biliary radicals where they're exiting the liver.
JASON P SULKOWSKI: You want to be careful not to use cautery here, because you can injure those small bile ducts. And then you perform a Roux-en-Y reconstruction with an end-to-side portoenterostomy. This surgery is most often done open. There have been laparoscopic Kasais done in the past. However, studies show that their outcomes were not quite as good, so it's currently not recommended. But there are certain places around the world, such as in East Asia where biliary atresia is more common and the surgeons are having more experience with that and getting better results.
JASON P SULKOWSKI: So time will tell. Next slide. So complications after a Kasai procedure. You can get a leak of one of your two anastomosies, either the portal jejunostomy or the jejunojejunostomy from the Roux-en-Y reconstruction. Sometimes infants can develop a lymphatic leak which will lead to chylous ascites.
JASON P SULKOWSKI: Patients with any kind of portoenterostomy are at risk for cholangitis. So frequently they're kept on Bactrim for prophylaxis for some time, although there's not really great evidence that this helps too much. Then there's obviously failure of the procedure. So progression of the cholestasis and portal hypertension. These patients can potentially be malnourished because of poor biliary circulation.
JASON P SULKOWSKI: They can develop variceal bleeding due to their portal hypertension and ascites. Outcomes are pretty directly correlated with the age of the patient at the time of their Kasai procedure. Usually patients at a younger age or with less fibrosis of the liver have improved survival of their native liver.
JASON P SULKOWSKI: If biliary atresia is diagnosed beyond 90 days of life, a Kasai is probably most likely futile in that situation, and most centers would probably recommend going straight to a liver transplant for that patient. However, if we're able to get to the diagnosis and do a Kasai before 45 days of life, those patients have a much better chance of maintaining their native liver for some time. So this is a rough approximation, but the numbers that I kind of remember for myself are that about one third of patients will have an unsuccessful Kasai and never really get good drainage.
JASON P SULKOWSKI: One third will have a successful Kasai and have good biliary drainage, but within a couple of years they'll start having progression of their liver disease and require a transplant. And then the final third will have a successful Kasai and will end up with long term survival of their native liver, sometimes making it out to 15, 20 years, doing pretty well.
ALFRED CHAHINE: Dr. Sulkowski, we have a question about the biopsy. Is it always required or is imaging diagnosis is sufficient to proceed with surgery?
JASON P SULKOWSKI: So a biopsy is-- That's a great question. Biopsy is not required to go to the operating room, because really the definitive way to make the diagnosis is through the cholangiogram and that has to be done in the operating room. The biopsy can provide some helpful information though about prognosis. And then even in the operating room, we always include a liver biopsy again for the same reason, to get an idea of how bad the damage to the liver is and help with prognosis.
JASON P SULKOWSKI: Next slide. OK. So we've got a prenatal ultrasound done on a 24-week gestational age fetus that is showing an upper abdominal cystic structure. So what can cause an abdominal cyst in a fetus?
JASON P SULKOWSKI: Next slide. So the most common cause of a cyst in the abdomen in a fetus is an ovarian cyst. And you wouldn't necessarily think that that would be in the upper abdomen, but sometimes it's kind of hard to tell on these prenatal ultrasounds and so on you never want to rule that out completely as a possibility.
JASON P SULKOWSKI: Other possibilities are an enteric duplication, a mesenteric cyst, an adrenal cysts which could be either the cause of intrauterine hemorrhage or neuroblastoma, a choledochal cyst, or cystic biliary atresia. So for this patient there's not much that you would do at this time, at 24 weeks gestational age, but you're going to recommend to continue doing serial ultrasounds until the baby's born.
JASON P SULKOWSKI: Next slide. Next slide. So this baby is going to continue getting serial ultrasounds every four weeks. And then after they're born, you'll do another ultrasound so you'll be able to get a better look and have a better idea where the cyst might be arising from. So in this baby, this is where the cyst actually appears.
JASON P SULKOWSKI: You can see the gallbladder is there and then the cyst is situated right behind it. So this is primarily concerning for a choledochal system, this situation. Perhaps an enteric cyst. Next slide. So a choledochal cyst can present a number of different ways. So it can present pre-natally, as we just talked about, as an abdominal cyst.
JASON P SULKOWSKI: It can present in an infant or a young child with biliary obstruction either due to choledocholithiasis. They could have jaundice. They could present with recurrent episodes of cholangitis. Sometimes a cyst gets so large that it can be a palpable mass in the right upper quadrant. But it's a relatively rare problem. It occurs in about one in 100,000 cases, although it is more common in East Asian countries.
JASON P SULKOWSKI: Next slide. There are five different main types of choledochal cysts, as you can see in the figure here on the right. A type one is the fusiform dilation of the extrahepatic bile duct. Type two is a diverticulum coming off of the bile duct. Type three is a choledochal seal that is present in the duodenal wall at the ampulla of Vater.
JASON P SULKOWSKI: Type four A is multiple cysts located both intra and extrahepatic. And type four B is multiple cysts located just in the extrahepatic bile duct. And then type five is Caroli's disease which is entirely intrahepatic dilations of the bile ducts. This can sometimes present throughout the entire liver or just in a single level of the liver. The pathophysiology is thought to be due to malunion of the pancreatic and biliary ducts during development.
JASON P SULKOWSKI: This leads to a long common channel which causes reflexive pancreatic fluid into the bile duct and that leads to damage occurring to the duct, which then allows it to start becoming dilated. A type 3 cyst is a little bit different. Most likely this is thought to be cause to dysfunction at the sphincter of Oddi. Next slide.
JASON P SULKOWSKI: So if you're concerned about a choledochal cyst after you've gotten your ultrasound, you want to check LFTs and look for the possibility of hyperbilirubinemia. Like I said, get an ultrasound. And then an MRCP is usually the next step to really get a good look at the anatomy. This is a great study to assess the extrahepatic and intrahepatic biliary anatomy.
JASON P SULKOWSKI: You can see an image here that you've got a fusiform dilation of the extrahepatic biliary system, so this is a type 1 choledochal cyst. ERCP can also be used. More commonly this will be done in an older patient. We typically don't do ERCPs on infants. When this is discovered, it's not really helpful by an ERCP because you have the ability to do interventions. It might be necessary if there's an obstructing stone or you need to do some sort of decompression for cholangitis.
JASON P SULKOWSKI: Next slide. So if a cyst is discovered, it needs to be resected. The management for a choledochal cyst is to resect it and do a reconstruction. So the reason to do this, is that this can lead to recurrent infections, recurrent episodes of cholangitis. There's also the risk of malignant degeneration of that cyst.
JASON P SULKOWSKI: It can become a cholangiocarcinoma. So for type one, two, and four cysts, the extrahepatic bile duct is excised in an hepaticoduodenostomy or hepaticojejunostomy with Roux-en-Y reconstruction is performed. You can see here in the surgical picture, this is a type one cyst. You can see the large dilation of the common bile duct. And then the lower image is the hepaticojejunostomy, the anastomosis of the bowel to the remnant bile duct.
JASON P SULKOWSKI: If you have a type three cyst, this can commonly be managed just with sphincterotomy which would be done in ERCP. You can also do a transduodenal excision of the choledochocele. And then for Caroli's disease, a type five cyst, you can do a liver resection if there's just a segment of the liver that's affected, but more commonly the entire liver is affected and a transplant must be done.
JASON P SULKOWSKI: Next slide. Complications from this surgery include a bile leak, stricture of the anastomosis, and recurrent cholangitis. Overall, the long-term outcomes tend to be excellent, but these patients do need to be followed up for development of cholangiocarcinoma. There are no formal guidelines in place, but it's probably just something to have checked on at a regular interval.
JASON P SULKOWSKI: Next slide. All right. So now we have a three-year-old who has come into your office with a neck mass, erythema, and tenderness. And you can see what it looks like here in the picture. Any thoughts?
JASON P SULKOWSKI:
ALFRED CHAHINE: Branchial cleft cysts, Dr. Badami says.
JASON P SULKOWSKI: That's good. All right. Next slide. So it can be a number of different things. This is a surgery lecture, so it's probably going to be a branchial cleft cyst, right? So it could be just simple lymphadenitis. It could have a dermoid cyst that's become infected, or a serbaceous cyst. Branchial cleft cysts are most likely going to present on the lateral aspect of the neck as in this image.
JASON P SULKOWSKI: If this were located in the midline, it would be a thyroglossal duct cyst potentially. You can also have a lymphatic malformation that can get infected or that can have hemorrhage into it and can present with erythema and pain. Next slide. So the embryology of branchial anomalies is relatively complicated, but the important thing to remember is that the branchial structures are comprised of ducts that are made out of ectoderm pouches which are endoderm and then arches which are mesoderm.
JASON P SULKOWSKI: And the failure of either a cleft or a pouch to obliterate results in a remnant cyst, sinus, or fistula. The opening superficially of a sinus or a cyst are going to be in a line just from interior to the tragus, and then down along the neck anterior to the sternocleidomastoid muscle. Internally, a fistula can open in a number of different places and it tends to depend on which cleft is involved. So if it's the first cleft, it's going to be the ear.
JASON P SULKOWSKI: The second will be the tonsil. Third cleft will be the pyriform sinus, and the fourth will open up in the esophagus. And there's a mnemonic to help you remember the number of syllables of the location of the fistula opening corresponds to the number of the cleft that's involved. Next slide. So by far the most common is a second branchial cleft cyst.
JASON P SULKOWSKI: These sinuses will typically present with either drainage or infection. If it's a cyst, it'll present with either a swelling or an infection. Next slide. If they present initially with infection, the first step is to treat it with antibiotics.
JASON P SULKOWSKI: If you need to, you can needle aspirate it. You really want to avoid doing an incision and drainage on these, because you can obscure the anatomy and make it more difficult to do a resection down the road. You can get an ultrasound to confirm the diagnosis if there's any question, but typically imaging is not always necessary. If you're suspicious that this is a first bronchial anomaly, you do want to do a good ear exam to make sure that there isn't a communication with the external auditory canal.
JASON P SULKOWSKI: And if you're concerned about a third or fourth branchial anomaly, you want to do laryngoscopy and esophagram to reveal communicating tracts. Next slide. So the management of these is surgical excision. So if they presented initially with an infection you want to make sure the infection is cleared first. You can use a probe or a catheter to follow the extent of the tracts.
JASON P SULKOWSKI: You can see an image here there's a probe going into the tract. And sometimes it extends pretty far and you can use stepladder incisions like here in order to follow the tract as it works its way proximally. It can extend into the pharynx, and knowledge of the anatomy is really key. These tend to be located pretty close to a number of important nerves and blood vessels, including the facial, vagus, hypoglossal, and glossopharyngeal nerves, and the carotid artery.
JASON P SULKOWSKI: Next slide. Overall, most patients with these do well after excision. There is a relatively high wound infection rate, up to 10%. Recurrence is more likely if some of the tracts or some of the cyst is left behind. That can occur in up to 20% of cases. If that does happen, reexcision or endoscopic cauterization of an internal opening that's present can be done.
JASON P SULKOWSKI: Next slide. As I mentioned, if this cystic structure was located in the midline and you notice that the cyst moved with swallowing, then you'd be particularly concerned about a thyroglossal duct cyst. These also have to be resected. The procedure for this is called a Sistrunk procedure, where you excise the entire cyst and the tract, including the mid portion of the hyoid bone, because the tract always goes through the hyoid rate if you don't resect that, there's a high rate of recurrence.
JASON P SULKOWSKI: These can extend all the way up to the base of the tongue. Rarely, there is no other thyroid tissue in the patient. And when you remove this cyst, you've removed all their thyroid tissue and they can present with hypothyroidism. So that's one reason that some surgeons will get a routine ultrasound of the neck prior to the surgical excision just to make sure that there is a normal thyroid gland present.
JASON P SULKOWSKI: Next slide. OK. So new topic. You've got a 15-year-old boy who's coming to your office with mild exercise intolerance and he's complaining about his sunken sternum. So there's really not much of a differential to this. This is basically a chest wall deformity known as pectus excavatum.
JASON P SULKOWSKI: Next slide. So there are several different main categories of chestal deformities. The most common is pectus excavatum, which is present in about 2% of all children. They're much more common in boys, but they do occur in girls as well. Next slide. The other main type is pectus carinatum, which is a protrusion of the sternum as opposed to a cavity.
JASON P SULKOWSKI: Again, these are much more commonly occurring in boys. Next slide. The thought is that these are a result of abnormal development of the costal cartilage which causes inward pressure on the sternum and that either causes a depression or an elevation of the sternum. Next slide. So when you're seeing a patient like this, you want to get a good history about whether or not they have any issues with exercise tolerance.
JASON P SULKOWSKI: Many patients won't have any complaints at all about being able to run and do sports, or will just have minimal symptoms. You want to look for any evidence of comorbidities, particularly connective tissue disorders like Marfan's or Ehlers-Danlos. And then your workup will proceed with a chest CT. You want to have good cross-sectional imaging, because there are a couple of indices that you want to calculate in order to A, determine that the diagnosis is correct, and B also, determine the extent of the defect.
JASON P SULKOWSKI: So one is called the Haller index which involves dividing the width of the chest by the distance between the vertebral body and the sternum. Any value greater than 3.2 of the Haller index is consistent with pectus excavatum and should be repaired. The other measure is the correction index, which basically tells you how much of an increase in the depth of the chest you need in order to get to a normal appearing chest.
JASON P SULKOWSKI: This is increasingly being used as a more accurate measure of really the degree of the pectus defect. Most patients will also be sent for pulmonary function tests and an echocardiogram in order to assess if there is some underlying cardiopulmonary effect from the defect, even if the patient is not reporting any symptoms. And then patients should also go for metal allergy testing.
JASON P SULKOWSKI: Because they may have an allergy to the metal that the bar is constructed out of, and you'd want to know that before implanting this device. If they do, then a premade titanium bar can be used instead. Next slide. So the treatment for pectus excavatum that's most commonly performed these days is a thoracoscopic Nuss procedure.
JASON P SULKOWSKI: So you start by doing the right sided thoracoscopy, and create a retrosternal window bluntly. And through this you pass a curved metal bar. It's inserted concave and then you flip it 180 degrees to make it convex and then this will elevate the sternum. Most patients are able to get by with one bar, but in more severe defects or in deeper defects sometimes two bars are necessary as this image. And the bars are left in place for about two to three years before they're removed.
JASON P SULKOWSKI: Next slide. The other procedure that's used for pectus excavatum is the Ravitch procedure. This is a much more invasive procedure. Involves longitudinal or transverse sternal incision. Bilateral skin flaps and pec major flaps are elevated. The perichondrium is incised sharply on either side of the sternum, and then the costal cartilage is resected.
JASON P SULKOWSKI: As you're doing that, a lot of times you'll see the sternum starts to flatten out automatically but sometimes the sternum is so abnormal that you have to create an osteotemy in order to help flatten. Next slide. Post-operatively, the main issue that most patients have is pain as you would expect.
JASON P SULKOWSKI: We typically use a multi-modal approach with pain controls. So basically every type of pain medicine you can think of is employed. So Tylenol and NSAIDS, muscle relaxants, gabapentin. Many patients are put on a PCA or given an epidural. And then some people perform an intercostal or paravertebral nerve block or even cryoablation. The main complications from this procedure. Obviously cardiac perforation is the one of most concern.
JASON P SULKOWSKI: If that were to occur, it mandates an immediate sternotomy and repair of the heart. The pericardium itself can be perforated. This doesn't require specifically repairing the pericardium, but you can't leave the bar within the pericardium. So you would have to reposition the bar in that case. Patients very frequently have a pneumothorax on one or both sides of the chest.
JASON P SULKOWSKI: This will often just improve on its own without any intervention. The bar can migrate, it can flip around 180 degrees, or it can just migrate to an abnormal position. This does require to be repositioned in the operating room. And then rarely patients will also have a bar infection or an allergic reaction to the bar. Often this can be treated just with medications, but it does sometimes require removal of the bar.
JASON P SULKOWSKI: Next slide. Nearly all patients end up with improved appearance after a Nuss procedure or a Ravitch. And many will also have the benefit of cardio pulmonary improved performance. They'll have improved FEV1 and their right heart function will be better on an echo. Many patients also described improved exercise tolerance.
JASON P SULKOWSKI: Next slide. I see Abbas has asked a question. Is there a particular age you wait to perform the procedure? The best time to do it is in the early teens, because much of the growth-- much of the linear growth has completed, but the bones are still a little bit more flexible.
JASON P SULKOWSKI: And so it's a good time to do it. If you wait until late teens or even into the 20s, it can still be done but you might not necessarily get as much of benefit. All right. This is our last case, I believe. So we've got a newborn infant who presents immediately after birth with respiratory distress. An immediate chest X-ray is obtained and this is what you see.
JASON P SULKOWSKI: Any thoughts? So this is consistent with a diaphragmatic hernia. You could clearly see that there is intestine in the left chest. So CDH is a congenital defect in the diaphragm that allows bowel or any other abdominal organs to permeate into the thoracic cavity. There are two main types. Morgagni and Bochdalek.
JASON P SULKOWSKI: The defect results in compression of the ipsolateral lung which causes pulmonary hypoplasia and pulmonary vascular hypertrophy and hypertension. Next slide. It occurs in about two in 10,000 live births. It's frequently associated with other defects and anomalies, such as trisomies, congenital heart disease, abdominal wall defects.
JASON P SULKOWSKI: And it's always associated with an intestinal rotational anomaly, since the bowel is in the chest and not able to fix normally into the abdomen. Next slide. So more and more commonly these are being diagnosed prenatally on ultrasound, and then with follow up MRI. This is helpful because we can determine the laterality of the defect, we can see if the liver is in the chest or in the abdomen, and we can calculate the lung head ratio, which is done by measuring the cross-sectional area of a lung divided by the head circumference.
JASON P SULKOWSKI: This is really helpful for prognosis. Next slide. So if the liver is down in the abdomen, survival rates are quite good. Greater than 90%. However, if the liver is up in the chest, the survival is less than 50%. And the lung head ratio is also prognostic. If this is found to be less than 0.8 or 1, this is considered to be associated with a poor prognosis.
JASON P SULKOWSKI: Also increasingly, we're using the observed to expected LHR which has been found to be a little bit more helpful. You can see if the O to E ratio is 10% the survival is pretty dismal. Whereas as the O to E ratio approaches 50%, then the survival is upwards of 3 in 4 patients. Next slide. So if the diaphragm hernia wasn't diagnosed prenatally, it can also most commonly be diagnosed shortly after birth with respiratory distress, as in the case presented.
JASON P SULKOWSKI: You'll also see a scaphoid abdomen, since much of the abdominal content is up in the chest. And then you will clearly see the X-ray findings show. Next slide. And the last way that a CDH can present is sort of in a delayed fashion. This is usually a Morgagni hernia. And sometimes they can present with vague abdominal symptoms, but it's often just identified with because a child is getting a chest X-ray for some other indication and you just happen to discover that there's bowel up in the chest.
JASON P SULKOWSKI: Next slide. So for the standard neonatal diaphragmatic hernia, the most important initial step is just maintaining cardiopulmonary stability. You really want to minimize iatrogenic injury from how you're managing your vent and your cardiopulmonary support. So we use pressure control ventilation and permissive hypercapnia.
JASON P SULKOWSKI: The key is to follow a strict protocol. There's great evidence that just following vent management protocol is much better than kind of improvising. Other adjuncts to management include using nitric oxide, using the oscillatory ventilator, and ECMO. So ECMO is used probably in about one in three cases of CDH. Indications include a preductal oxygen saturation of less than 85%, inability to improve the pH better than 7.2, persistent hypercarbia above 70, and persistent hypertension.
JASON P SULKOWSKI: Next slide. So the timing of repairs is critical for patients like this. You really want to wait until you've achieved some degree of physiologic stabilization with some normalcy to your vitals. Ideally you want to have your vent at a setting FiO2 less than 50%.
JASON P SULKOWSKI: You'd like your blood pressure to be within normal limits, and you want evidence of good tissue perfusion. So a normal urine output, a normalized lactate, and a normal pH. You also want to make sure on echo that your pulmonary arterial pressures are less than systemic pressures. On the other hand, if you've had to resort to ECMO it's very controversial whether or not you should do your repair on or off ECMO.
JASON P SULKOWSKI: Many different people use many different approaches to this. Some people will repair right away after going on ECMO. Other people will give the baby a chance to get off ECMO and if not, then do the repair. And then still others will wait until the ECMO run has ended and then do the CDH repair. There's really no one right approach. Next slide.
JASON P SULKOWSKI: So the approach to repair can be done either thoracoscopically or open. This is going to depend entirely on how sick the baby is. So a baby who has an obvious diaphragmatic hernia but is on room air and is very stable, you can easily get away with doing the thoracoscopic repair. On the other hand, if you're doing a repair on ECMO, really you're probably going to do that open. You can see there are four different categories of defects increasing in size from A through D.
JASON P SULKOWSKI: If you've got an A or a B defect, those are more often amenable to a thoracoscopic approach, and potentially doing a primary repair. If you have a C or D defect, then you really should be doing it open and they're more likely to require that you use a patch. Next slide.
JASON P SULKOWSKI: Common complications after CDH repair include bleeding, infection, you can get compartment syndrome after reducing all the abdominal contents back in their correct location. Chylothorax is a relatively common complication from CDH repairs. And then also recurrence of the hernia. Late complications include a risk for bowel obstructions and again, recurrence of the hernia which occurs in about 10% to 20% of cases.
JASON P SULKOWSKI: These babies are overall medically complex and are associated with a number of other long term sequelae, including gastroesophageal reflux. About one in four will end up getting a Nissen. There's significant morbidity from pulmonary hypoplasia and pulmonary hypertension. Neurodevelopmental delay is extremely common with about 2/3 of all patients having this at three years of age. And there are also significant musculoskeletal abnormalities due to tension on the diaphragm, the thoracotomy incision, and then also from the hypoplastic lungs.
JASON P SULKOWSKI: Next slide. And that is it. Are there any other questions that I can answer?
ALFRED CHAHINE: Wonderful, Dr. Sulkowski. This was a great review of very difficult topics. In the interest of time, I guess, Dr. Badami or any up with you do you have any questions? All right. So we'll see you next week, and thank you so much. Take care.
JASON P SULKOWSKI: Thank you.
Segment:0 .
ALFRED CHAHINE: OK. Good afternoon everyone, and welcome to SCORE School. My name is Alfred Chahine. I'm the Assistant Editor of SCORE, and I will be your moderator today. A few housekeeping items before we start. Today is the first day when we switch from the Wednesday evening sessions to the Friday afternoon ones. We did this for a few reasons.
ALFRED CHAHINE: The two main ones are to be able to have the recording available at the beginning of the week for you all to use when reviewing the TWIS topics within your program. And the second reason is that when we surveyed the SCORE School users a couple of weeks ago, the time and day did not seem to be of tremendous importance to the majority. So we felt that Friday afternoon would be better.
ALFRED CHAHINE: As always, we encourage you to use the chat box to ask questions and make comments. Everyone has been muted to avoid unnecessary noise. You will see a QR code for attendance tracking displayed a few times throughout the presentation. You only need to submit it once, and you'll receive an email confirming your attendance. All of the SCORE School sessions are being recorded for later viewing on the SCORE website.
ALFRED CHAHINE: You should be aware that the names on the chat will be recorded as well. We encourage you to view these recorded sessions as your schedule allows. So today's session, the topic is pediatrics. The first out of four throughout the two-year cycle. And we'll be discussing a few modules. Some of them were discussed in the last pediatrics module of the previous cycle.
ALFRED CHAHINE: So we'll have a few redundancies, but that's OK. The topics the modules are Meckel's diverticulum, appendicitis, biliary atresia, choledochal cysts, branchial cleft anomalies, congenital diaphragmatic hernias, chest wall deformities, and neonatal small bowel obstruction. It is my pleasure to introduce to you our faculty presenter Dr. Jason Sulkowski. He is an Assistant Professor of Surgery and Pediatrics at the Virginia Commonwealth University in Richmond, Virginia.
ALFRED CHAHINE: He's a graduate of the Columbia University College of Physicians and Surgeons. He did his residency at SUNY Downstate Medical Center in Brooklyn, New York. During that time he spent two years doing research at Children's Hospital of Philadelphia and Ohio State. He completed a Pediatric Surgery Fellowship at Children's National Health system, where I had the privilege to work with him for two years.
ALFRED CHAHINE: A personal note on Jason is that he's the only fellow that I have worked with over a 22-year career who would go in and observe how faculty teach residents in the operating room. So that tells you about his dedication and his yearning to teach. He's a member of multiple organizations, and was honored with a Teaching Award as a resident while a resident in Brooklyn.
ALFRED CHAHINE: Thank you, Dr. Sulkowski, for lending your expertise to us and take it away, please.
JASON P SULKOWSKI: All right. Thank you very much. Thanks, Dr. Chahine and everyone else at SCORE for allowing me to give this presentation today. So let's just jump right into it. These are our topics for the day. Next slide. So we're going to start with a 3-year-old boy who has two days of bloody stools.
JASON P SULKOWSKI: What kind of differential does this case scenario make you think of?
ALFRED CHAHINE: Abbas, you're on.
JASON P SULKOWSKI: All right. So the differential for a child with bloody stools is pretty wide, and a lot of it's going to depend on what age the patient is. So you've got a neonate, you're going to be thinking about anal fissures, necrotizing enterocolitis, a milk protein allergy, or more concerning would be malrotation with volvulus. In an infant or a child, such as this patient, you're thinking intussusception, a Meckel's diverticulum, anal fissure, or infectious colitis.
JASON P SULKOWSKI: In an older patient, an adolescent, you'd be thinking inflammatory bowel disease. Again, colitis, a Meckel's, perhaps enteric duplication that's becoming symptomatic, or even hemorrhoids. Next slide. So taking into consideration this patient, two days of bloody stools. Imagine that the patient also is reporting no other symptoms.
JASON P SULKOWSKI: His abdomen has no pain whatsoever. What kind of imaging would you want to pursue? Next slide. So usually the first thing that we'll do in this sort of a patient is a Meckel's scan, because this is a pretty classic presentation for a Meckel's diverticulum.
JASON P SULKOWSKI: So we'll go right for the source. So we do a Meckel's scan, which is nuclear medicine study. The sensitivity isn't perfect. It ranges from about 60% to 85%, so there are a decent number of cases that go undetected by this study. The idea of the study is that it detects gastric mucosa. So as you can see on the image there on the right of the screen, the stomach is lighting up and then there's another area in the right lower quadrant that's lighting up.
JASON P SULKOWSKI: And that's consistent with heterotopic gastric mucosa in a Meckel's diverticulum. Next slide. So everyone should be familiar with the rule of twos for a Meckel's. This is a pretty commonly known mnemonic. They present in about 2% of the population. They are usually located two feet proximal to the ileocecal valve.
JASON P SULKOWSKI: They're usually two inches in length. They commonly have two types of ectopic mucosa, either gastric or pancreatic. About 2% of them become symptomatic over a patient's lifetime. And there's a 2 to 1 male to female ratio. Next slide. The embryology of a Meckel's is that it's a remnant of the omphalomesenteric duct.
JASON P SULKOWSKI: This duct is a communication in the fetus between the intestine and the extraembryonic yolk sac. The figure here on this slide shows all the different manifestations of a duct remnant. You can have a completely patent duct, which communicates the umbilicus to the bowel. You can just have umbilical anomalies like a polyp or a cyst. Or you can have a remnant cord or a Meckel's diverticulum.
JASON P SULKOWSKI: Next slide. Clinically, they can present a number of different ways. In younger children, about two or three years old, such as in the case that was presented, they most commonly will present with bleeding and no other symptoms. So we call it painless bleeding. In older children they're more likely to present with perforation of the Meckel's like you can see here in this picture.
JASON P SULKOWSKI: They can also present with obstruction. In older patients, neoplasm can arise from the ectopic tissue. And then they can even present with foreign body retention, because it's sort of like an open lumen, sort of like an appendix. And so you can get swallowed objects or fish bones or things like that occasionally. Next slide.
JASON P SULKOWSKI: So a patient who is discovered to have a Meckel's that's symptomatic requires an operation. This can usually be done laparoscopically, sometimes converted to open depending on what we find. If you find a Meckel's, such as in this case, and the base of the tissue is healthy, you can simply come across it tangentially with the GIA stapler. And you staple transverse to the direction of the intestine just like in this picture.
JASON P SULKOWSKI: If the base of the Meckel's is not healthy tissue, for example if there's a perforated ulcer or something like that, it's usually safer to do a segmental resection of that small segment of small intestine and perform a primary anastomosis. Next slide. Now what happens if you're doing an operation for another reason and you find a Meckel's in the patient? So removing a Meckel's comes at about a 4% risk of some sort of complication like a wound infection or a leak.
JASON P SULKOWSKI: However, the lifetime risk of symptoms developing from a Meckel's is approximately 3% to 6%. And then if you look at the number needed to prevent a death, you have to treat 800 patients in order to prevent one death. So that's a pretty significant number needed to treat ratio. There's some surgeons who would always remove a Meckel's that they discover because there is a slight risk of a neoplasm, but there is no specific right or wrong answer about this in particular.
JASON P SULKOWSKI: I think the best idea is to have a standard approach and just always follow the same rule. Next slide. OK. So now we have a 10-year-old female--
ALFRED CHAHINE: Dr. Sulkowski, can I stop you for a second?
JASON P SULKOWSKI: Yeah.
ALFRED CHAHINE: Can you speak to the-- so when you describe the operation, tangential stapling of the base, could you speak briefly about the idea and the concern about the ulcer and the bleeding being on the ileum, and whether you need to do a resection versus just a diverticulectomy?
JASON P SULKOWSKI: Yeah. So in the past the teaching was to all the time perform a bowel resection and in an anastomosis. However, more recently a number of studies have shown that in most cases you can safely staple just across the base of the Meckel's, sort of like doing an appendectomy. And as long as you do it in a transverse direction so you don't narrow the intestine, it is safe to do.
JASON P SULKOWSKI: If the tissue doesn't look healthy, like it's not going to hold the staples very well, then the safer thing to do would be a segmental resection in that case. A lot of the concern previously was that if there's bleeding or if there's Meckel's diverticulitis or something like that, that the tissue might not necessarily be able to hold staples pretty well.
JASON P SULKOWSKI: But unless it's very obviously ischemic-looking at tissue, it actually holds up pretty well.
ALFRED CHAHINE: Thank you.
JASON P SULKOWSKI: All right. So now we've got a 10-year-old girl who's coming in with one day of right lower quadrant pain, fever, and several episodes of vomiting. What could this be a presentation for?
ALFRED CHAHINE: Dr. Badami, You want to respond?
JASON P SULKOWSKI: OK. We can just go to the next slide.
ALFRED CHAHINE: I'm trying to, he says. OK. So it can be appendicitis.
JASON P SULKOWSKI: That's right. So I mean this can be a handful of things. Probably the classic presentation of appendicitis, but you do have to kind of go through your differential. The most common finding when it's not appendicitis is simple viral gastroenteritis or mesenteric adenitis. Obviously, there's appendicitis. In a female with right lower quadrant pain you have to think about ovarian torsion or an ovarian cyst. If this were a boy, testicular torsion can present with right lower quadrant pain.
JASON P SULKOWSKI: And then in this age group, a first episode of inflammatory bowel disease is always a possibility to consider as well. Next slide. So appendicitis is the most common reason that we're called to the pediatric emergency room for a consult, and it makes up anywhere from 5% to 10% of all pediatric emergency room visits.
JASON P SULKOWSKI: And abdominal pain alone is the single most common reason for any pediatric emergency room visit. I mean, it's a common problem. About 7% to 9% of people will end up developing appendicitis in their lifetime with a slightly increased risk in boys. Next slide. The pathophysiology of appendicitis is the appendiceal lumin becomes obstructed, commonly with a fecalith.
JASON P SULKOWSKI: And there can also be lymphoid hyperplasia, rarely a mass. This leads to increased pressure within the appendix, bacterial overgrowth which then causes venous outflow obstruction. And that leads to wall ischemia and bacterial invasion into the wall of the appendix. Ultimately, that wall will perforate which causes luminal contents to leak. And then if this isn't treated in time, a phlegmon and ultimately an abscess will form.
JASON P SULKOWSKI: Next slide. So on history you're going to get pretty much the standard story in many patients. They'll start out with generalized abdominal pain that will then be located in the right lower quadrant. However, in kids the variety of presentations can be quite wide, and so you've got to have a pretty high index of suspicion for appendicitis.
JASON P SULKOWSKI: It's often also associated with fever, nausea, vomiting, diarrhea, and painful urination. On physical exam they'll have a fever, sometimes they're tachycardic. Sometimes they look pretty well, sometimes they look pretty sick. It really varies. And then on exam they'll most commonly have pretty focal right lower quadrant tenderness.
JASON P SULKOWSKI: It may or may not have rebound, and they'll have a Rovsing sign, which is pain on the right side of the abdomen when you press on the left side. Labs are sometimes obtained, although not always in kids. You'll see an elevated white blood cell count with a left shift. And then the most common imaging to start out with is an ultrasound.
JASON P SULKOWSKI: That is user dependent of course, and so sometimes it's difficult to visualize the appendix. And then in most hospitals, the next step will be to get either a CT scan or an MRI. Here on the right, you can see a CT image with arrows pointing at two slices of the same appendix, and there's a little fecalith that you can see in the more cephalad portion. Next slide.
JASON P SULKOWSKI: And then here you can see an MRI image. And the appendix is kind of hard to make out, but there is a little measuring line on it that shows that it's one centimeter in length. And you can see it's a little bit more lit up and hyper compared to the surrounding tissue and bowel. Next slide. So initially, once you've made the diagnosis, all patients need to be given IV antibiotics right away.
JASON P SULKOWSKI: In most hospitals I think the more common treatment is ceftriaxone and flagyl, but it kind of varies based on local IV practice. And then for most patients they're going to head to the operating room for an urgent-- not an emergency, but urgent laparoscopic appendectomy. Some patients are treated non-operatively. It's been increasingly common to treat patients with early simple appendicitis with antibiotics alone.
JASON P SULKOWSKI: This is primarily done in research studies at academic centers. The success rate has been shown to be about 70% at one year. The other indication for non-operative management would be in a patient who has clearly perforated and has a phlegmon or some well-developed abscess. Patients in this case are treated with IV antibiotics until they are symptomatically better, and then they're usually treated with a complete course with oral antibiotics at home.
JASON P SULKOWSKI: If there is a drainage collection then they can head to IR and have percutaneous tenuous drainage. Whether or not they go to the operating room for an interval appendectomy is controversial. Usually if there's a fecalith present, the best thing to do is remove the appendix in about 6 to 8 weeks after the patient's recovered from the initial episode. However, some people elect to just observe the patient afterwards.
JASON P SULKOWSKI: Next slide. So post-operatively after they go for an appendectomy, there are a wide variety of protocols for the duration of antibiotics. So pretty much across the board if someone has simple appendicitis, a non-perforated appendix, they won't get any additional antibiotics. And once they're eating and their pain is under control, they'll be able to go home.
JASON P SULKOWSKI: But for perforated appendicitis, most kids will stay on antibiotics and the duration is, like I said, there's a wide variety. There's really no one right protocol. The practice has been to decrease the duration over time, and most centers are treating approximately five to seven days of antibiotics for perforated appendicitis.
JASON P SULKOWSKI: So most patients are able to be discharged when they're afebrile, when they're tolerating a diet, and when their pain is under control.
ALFRED CHAHINE: Dr. Sulkowski, can I interrupt you for a second. We have two questions in the chat about appendicitis. Dr. Badami is asking, Dr. Sulkowski, I know you have done extensive research in treating appendicitis non-operatively with antibiotics. How has your approach changed over the years in practice? Do you still defer surgical management?
JASON P SULKOWSKI: So that's a great question. So now that I'm not involved specifically with the research study, my practice is typically to recommend to have an appendectomy for patients with early appendicitis. I think it's a reasonable option, and if families ask about it then I'm happy to go down that route. But my personal practice has been to perform appendectomies for early appendicitis.
JASON P SULKOWSKI: I think it's still being actively studied around the country and in other countries as well. And I think there's still a lot to learn about that particular treatment modality, but that's not my personal practice right now.
ALFRED CHAHINE: And then Dr. Joshi asks, is it still felt with each hour of delay between diagnosis and appendectomy the risk of rupture increases, or do IV antibiotics arrest that timeline? Very good question.
JASON P SULKOWSKI: Yeah, that's a great question. So it's felt that in most cases if you start antibiotics right away, that buys you some time to get to the operating room. So it's pretty uncommon to take a child to the operating room in the middle of the night for an appendectomy. There have been a number of studies that have shown that doing so does not increase the risk of perforation and it also does not increase the risk of post-operative infectious complications.
JASON P SULKOWSKI: Clearly if you wait several days, that sort of gets thrown out the window and you do have to get to the procedure urgently. But it's no longer the emergency that it used to be considered. OK. So now if you have a patient who is 5, 6 days out from surgery and they're still not quite turning the corner, they're having fevers, they're still having a lot of pain, or they're not eating well, that's a patient who might have developed a post-operative collection, which is definitely more common in kids with perforated appendicitis.
JASON P SULKOWSKI: So the inclination is you start getting kind of antsy a few days after surgery, and you want to go in and look for something, but you really should wait at least five days before reimaging. Because first of all, you'll minimize the number of additional studies done. Sometimes you'll find a really tiny collection that will get better just with antibiotics.
JASON P SULKOWSKI: And it also gives a little bit of extra time for a collection to organize into something that the interventional radiologists are able to drain. Next slide. Complications after an appendectomy. The most common by far is a surgical site infection. The risk is lower doing laparoscopic appendectomies than it used to be for open appendectomies for superficial and site infection.
JASON P SULKOWSKI: For a deep infection, the risk is only about 1% or 2% for simple appendicitis, but that rises to almost one in five patients with perforated appendicitis. The other risk is readmission. This is about 5% to 10% of patients. Most commonly they're getting readmitted because they've developed an abscess or some other infection at home. Rarely they come in with a bowel obstruction or an ileus or just uncontrolled pain.
JASON P SULKOWSKI: Next slide. There's an opportunity to get your QR code. Next slide. OK. So we've got a three-day-old infant who is in the NICU with vomiting and has just not been able to take any feeds.
JASON P SULKOWSKI: What could be going on?
ALFRED CHAHINE: Abbas is saying, pyloric stenosis, duodenal atresia.
JASON P SULKOWSKI: OK. All right. Next slide. OK. So when you're thinking about a child with a possible bowel obstruction, you've got to kind of think about the whole range of possibilities. And the most important distinction to make initially is whether or not the vomit is bilious or non bilious. So if it's non bilious emesis that means the most likely the obstruction is more proximal in nature.
JASON P SULKOWSKI: And so by proximal it means proximal to the pyloris and proximal to the ampulla of Vater where bile would be coming from. So two common explanations for this would be some sort of atresia, like esophageal atresia, or duodenal atresia. With bilious emesis and more distal obstruction, the first thing you have to think about and rule out is malrotation with volvulus.
JASON P SULKOWSKI: There could also be some other more distal intestinal atresia, either in the small bowel or in the colon. It just could be a meconium ileus, Hirschsprung's disease, imperforate anus, or some other sort of colonic cause of obstruction. Next slide. So when you first go to assess this patient, the first thing you have to answer is whether or not the infant looks sick or if they look OK.
JASON P SULKOWSKI: So you're going to want to look at their vital signs and see if they're tachycardic, if they're hypertensive, is the patient already intubated, are they on high vent settings, what kind of support is the baby already requiring. And then focusing on the abdominal exam. Are they soft? Does it seem like a relatively benign exam? Are they quite distended?
JASON P SULKOWSKI: Are you even noticing skin changes on the abdomen? Those are the concerning findings that would cause you to expedite your work up and perhaps get to the operating room a little bit faster. Next slide. So as long as there's not anything particularly alarming like a distended discolored abdomen, you're almost always going to start with a plain X-ray just to kind of get an idea of what's going on.
JASON P SULKOWSKI: So as you can see here, this is a classic film for an esophageal atresia with OG2 coiled up in the upper pouch and then air in the stomach consistent with a type C tracheoesophageal fistula with esophageal atresia. Next slide. You can also identify a duodenal atresia here. You can see an air-filled stomach and then an air-filled duodenum with no distal air whatsoever.
JASON P SULKOWSKI: So this is a classic double bubble found with duodenal atresia. Next slide. Intestinal atresia would show a little bit more gas-filled loops of bowel, but still a paucity of bowel gas. And you can clearly tell that there's no air in the colon and no air in the rectum.
JASON P SULKOWSKI: So this would be concerning for a jejunal atresia. Next slide. And then here you can see that there's quite a bit of air-filled intestine, but this is concerning for a distal obstruction like a colonic obstruction because you can tell that there is no air in the rectum. So there's definitely a distal obstruction in a film like this.
JASON P SULKOWSKI: Next slide. So if you've got a bilious emesis or the baby looks sick, you want to get an immediate upper GI to rule out volvulus. Here this upper GI shows a bird's beak and no contrast passing the proximal small intestine. So this is very concerning for a volvulus, and this baby needs to go immediately to the operating room for exploration.
JASON P SULKOWSKI: Next slide. Now if the upper GI looks normal and you see your normal seal up of the duodenum, then often you can at the same time get a contrast enema if you're thinking about the possibility of a more distal obstruction. This is particularly helpful for colonic issues. This film here is showing a classic image for meconium ileus.
JASON P SULKOWSKI: You can see that there is a diminutive left colon and rectum. And then in the small intestine you can see the filling defects which are consistent with the inspissated meconium in the terminal ileum. Next slide. So if the baby is sick or you suspect that there's a volvulus, the baby needs to go to the operating room right away.
JASON P SULKOWSKI: If you identify volvulus in the operating room, need to untwist the bowel until you start to see the bowel is profused again, and then you perform a Ladd procedure. I'm not going to go into specifics of that, because that's a separate topic. But that's sort of the basic of what you would do for that particular diagnosis. Next slide.
JASON P SULKOWSKI: Now if the baby doesn't look particularly sick and you've got your film, your management's going to be based more on your suspected diagnosis. And you've got a little bit more time to do additional studies like the contrast study, either an upper GI or contrast enema, in order to kind of pinpoint exactly the anatomic location. Pretty much all of them, if there's any kind of bowel obstructions, they're all going to require some sort of surgical intervention in order to fix the problem, with the exception being for meconium ileus.
JASON P SULKOWSKI: The management for that is frequently just to repeat contrast enemas, because that ends up freeing up that inspissated meconium and allowing motility to start. Next slide. So the takeaway for this module in this topic is really just the initial assessment and evaluation. Determine if the baby looks sick. Is the vomiting bilious.
JASON P SULKOWSKI: If either of those are going on, the baby needs an upper GI right away and probably needs to go to the operating room very quickly. And then we tend to use in pediatrics and especially with babies plain x-rays and contrast studies in order to pinpoint the location of the obstruction. You'll notice that what's really missing compared to how adults are usually treated, is a CT scan. It's very uncommon for us to have to get a CT scan for anything in the surgical treatment for a neonatal bowel obstruction.
JASON P SULKOWSKI: Next slide. All right. So new patient. We've got a one-month-old full term male who's presenting with jaundice, pale stools, and--
ALFRED CHAHINE: Dr. Sulkowski, sorry to interrupt you. We have a question, hold on, before we move on. Dr. Badami says, I had operated last week on a baby with intestinal atresia with Dr. [? Belichick-- ?] your mentor, Jason. Intraoperative evidence of extensive vascular compromise to small bowel, majority jejunum resected, the necrotic jejunum, because of the atretic bile we could not find intraluminal opening to create a jejunostomy.
ALFRED CHAHINE: Thoughts? How do you manage such cases?
JASON P SULKOWSKI: Oh, wow. I mean, that's a very complicated situation. I mean, it's hard to give specifics without being in the operating room with you. But I mean, sometimes if you've got-- you can have a case of intrauterine volvulus and you have very little bowel left. The baby has severe short bowel syndrome. And to the best of your ability you want to just find the most proximal bowel you can and bring it up as a stoma temporarily.
JASON P SULKOWSKI: And a lot of times these infants will require long-term TPN until you're able to get them back in the operating room and try to get them back into continuity. But that's a really difficult situation.
ALFRED CHAHINE: Sounds like it, yeah.
JASON P SULKOWSKI: All right. So back to this case. So now we've got a one-month-old full term male who is presenting with jaundice, pale stools, and dark urine. So what could be going on in this baby? Next slide. All right. So we've got a jaundiced baby.
JASON P SULKOWSKI: The differential for hyperbilirubinemia in infant is quite extensive, and only a handful really pertain to surgically correctable issues. So it includes obstructive problems like biliary atresia, choledochal cysts, cystic fibrosis, Alagile syndrome, gallstones. It also includes the hepatocellular issues like TORCH infections, idiopathic neonatal hepatitis, and metabolic disorders.
JASON P SULKOWSKI: One of the most important things you have to do is determine what component of the bilirubin is direct, and that'll raise your concern if that's elevated for biliary atresia or choledochal cysts, which are both the primary surgically correctable causes of cholestasis. Next slide. So biliary atresia in particular is a fortunately uncommon problem.
JASON P SULKOWSKI: It occurs in just under one in 10,000 live births. It presents most commonly as a sporadic condition without any associated anomalies. About 85% of babies are in that group. And then there are two different categories of syndromic biliary atresia. One is just associated with other anomalies, but they're not laterality defects. And then the other group is called the biliary atresia splenic malformation anomaly, and this includes malrotation in situs inversus in addition to the biliary atresia and splenic issues.
JASON P SULKOWSKI: Biliary atresia is thought to be caused by progressive injury to the bile duct epithelium. It typically starts in the extrahepatic ducts and then works its way retrograde into the enteropatic ducts. It's unclear at this time what the pathophysiology of the disease really is. Some people think that it could be due to a viral infection. It could have an autoimmune etiology.
JASON P SULKOWSKI: And then there's even the possibility of perinatal or prenatal toxin exposure as a cause. Biliary atresia if left untreated will progress to cholestasis, cirrhosis, portal hypertension, and ultimately end stage liver disease. Next slide. So the evaluation of an infant with jaundice would start with history, and jaundice is pretty common in infants.
JASON P SULKOWSKI: But usually beyond about two weeks of time that becomes abnormal. The baby will also have acholic stools and dark urine. The image on the right shows a stool color card that's often used in order to determine whether or not a baby's stool is normal or abnormal in color. On exam usually these babies are pretty much normal in weight. And actually pretty healthy appearing, at least early on. They'll obviously be jaundiced.
JASON P SULKOWSKI: If the disease has progressed, sometimes you can feel a firm palpable liver edge and enlarged spleen due to portal hypertension. On labs, as I mentioned, they'll have a direct hyperbilirubinemia. This usually plateaus at about four to six, which is in distinction from other causes of cholestasis which will frequently have even higher levels of direct bilirubin.
JASON P SULKOWSKI: Sometimes transaminases will also be elevated. You'd want to do a TORCH workup, check alpha-1 antitrypsin levels, and also check your coags because these babies will start having vitamin K issues and so they'll potentially be coagulopathic. Next slide. So as with almost every other condition in babies or in pediatrics, we'll start with an ultrasound as one of our first imaging studies.
JASON P SULKOWSKI: What you might find is the absence of a gallbladder or potentially a very tiny diminuitve gallbladder. Sometimes you can see what's known as the triangular cord sign, which is basically the scarred down portal plate. You'll see it at the bifurcation of the portal vein as you can see on the image on the right. You can also sometimes see cystic dilation of the common bile duct.
JASON P SULKOWSKI: There is an ideology of biliary atresia or cystic biliary atresia that can sometimes be confused for a choledochal cyst. Next slide. Another scan that's frequently obtained is a high dose scan which we're pretty familiar with for taking care of cholecystitis. In babies we typically will pre-treat them for five days with phenobarbital in order to increase the quality of the study.
JASON P SULKOWSKI: What we're looking for is failure of the tracer acid to excrete into the small intestine. If you see tracer going into the small intestine, then you know you've got a patent extrahepatic biliary system. And so you know you do not have biliary atresia. But as you can see on the study here on the slide, the liver is lit up but there's nothing lighting up the small intestine.
JASON P SULKOWSKI: So this would be concerning for biliary atresia. Next slide. And finally, the gold standard diagnosis is made with an interoperative cholangiogram. This can be done either open or laparoscopically. And what you want to see is contrast reflexing back up into the liver and then also going into the intestine. You can see here in the image the duodenum has contrast in it and then you can see where the right and left bile ducts are going approximately up into the liver.
JASON P SULKOWSKI: Next slide. Frequently, these patients will undergo percutaneous biopsy before they go to the operating room. This can sometimes help with the diagnosis. Early on in the course, in younger infants, this might lead to false positives. But it can be helpful in older kids, because they can determine the degree of hepatic fibrosis.
JASON P SULKOWSKI: And this can be very helpful information for prognosis in determining the course of treatment. On a biopsy with biliary atresia the classic findings are proliferation of bile ducts, portal fibrosis, and plugging of the canaliculi and the bile ducts. Next slide. So when biliary atresia is of concern, the next step is usually to go to the operating room to perform the interoperative cholangiogram.
JASON P SULKOWSKI: And if it's positive, the patient requires a Kasai portoenterostomy. So the Kasai procedure consists of resecting the atretic extrahepatic biliary system. So you would take the gallbladder if it's present and then the common bile duct. You'd resect this all the way up to the portal plate which is located, like I said, at the bifurcation of the portal vein. And then this is sharply transacted and that's going to get you to your biliary radicals where they're exiting the liver.
JASON P SULKOWSKI: You want to be careful not to use cautery here, because you can injure those small bile ducts. And then you perform a Roux-en-Y reconstruction with an end-to-side portoenterostomy. This surgery is most often done open. There have been laparoscopic Kasais done in the past. However, studies show that their outcomes were not quite as good, so it's currently not recommended. But there are certain places around the world, such as in East Asia where biliary atresia is more common and the surgeons are having more experience with that and getting better results.
JASON P SULKOWSKI: So time will tell. Next slide. So complications after a Kasai procedure. You can get a leak of one of your two anastomosies, either the portal jejunostomy or the jejunojejunostomy from the Roux-en-Y reconstruction. Sometimes infants can develop a lymphatic leak which will lead to chylous ascites.
JASON P SULKOWSKI: Patients with any kind of portoenterostomy are at risk for cholangitis. So frequently they're kept on Bactrim for prophylaxis for some time, although there's not really great evidence that this helps too much. Then there's obviously failure of the procedure. So progression of the cholestasis and portal hypertension. These patients can potentially be malnourished because of poor biliary circulation.
JASON P SULKOWSKI: They can develop variceal bleeding due to their portal hypertension and ascites. Outcomes are pretty directly correlated with the age of the patient at the time of their Kasai procedure. Usually patients at a younger age or with less fibrosis of the liver have improved survival of their native liver.
JASON P SULKOWSKI: If biliary atresia is diagnosed beyond 90 days of life, a Kasai is probably most likely futile in that situation, and most centers would probably recommend going straight to a liver transplant for that patient. However, if we're able to get to the diagnosis and do a Kasai before 45 days of life, those patients have a much better chance of maintaining their native liver for some time. So this is a rough approximation, but the numbers that I kind of remember for myself are that about one third of patients will have an unsuccessful Kasai and never really get good drainage.
JASON P SULKOWSKI: One third will have a successful Kasai and have good biliary drainage, but within a couple of years they'll start having progression of their liver disease and require a transplant. And then the final third will have a successful Kasai and will end up with long term survival of their native liver, sometimes making it out to 15, 20 years, doing pretty well.
ALFRED CHAHINE: Dr. Sulkowski, we have a question about the biopsy. Is it always required or is imaging diagnosis is sufficient to proceed with surgery?
JASON P SULKOWSKI: So a biopsy is-- That's a great question. Biopsy is not required to go to the operating room, because really the definitive way to make the diagnosis is through the cholangiogram and that has to be done in the operating room. The biopsy can provide some helpful information though about prognosis. And then even in the operating room, we always include a liver biopsy again for the same reason, to get an idea of how bad the damage to the liver is and help with prognosis.
JASON P SULKOWSKI: Next slide. OK. So we've got a prenatal ultrasound done on a 24-week gestational age fetus that is showing an upper abdominal cystic structure. So what can cause an abdominal cyst in a fetus?
JASON P SULKOWSKI: Next slide. So the most common cause of a cyst in the abdomen in a fetus is an ovarian cyst. And you wouldn't necessarily think that that would be in the upper abdomen, but sometimes it's kind of hard to tell on these prenatal ultrasounds and so on you never want to rule that out completely as a possibility.
JASON P SULKOWSKI: Other possibilities are an enteric duplication, a mesenteric cyst, an adrenal cysts which could be either the cause of intrauterine hemorrhage or neuroblastoma, a choledochal cyst, or cystic biliary atresia. So for this patient there's not much that you would do at this time, at 24 weeks gestational age, but you're going to recommend to continue doing serial ultrasounds until the baby's born.
JASON P SULKOWSKI: Next slide. Next slide. So this baby is going to continue getting serial ultrasounds every four weeks. And then after they're born, you'll do another ultrasound so you'll be able to get a better look and have a better idea where the cyst might be arising from. So in this baby, this is where the cyst actually appears.
JASON P SULKOWSKI: You can see the gallbladder is there and then the cyst is situated right behind it. So this is primarily concerning for a choledochal system, this situation. Perhaps an enteric cyst. Next slide. So a choledochal cyst can present a number of different ways. So it can present pre-natally, as we just talked about, as an abdominal cyst.
JASON P SULKOWSKI: It can present in an infant or a young child with biliary obstruction either due to choledocholithiasis. They could have jaundice. They could present with recurrent episodes of cholangitis. Sometimes a cyst gets so large that it can be a palpable mass in the right upper quadrant. But it's a relatively rare problem. It occurs in about one in 100,000 cases, although it is more common in East Asian countries.
JASON P SULKOWSKI: Next slide. There are five different main types of choledochal cysts, as you can see in the figure here on the right. A type one is the fusiform dilation of the extrahepatic bile duct. Type two is a diverticulum coming off of the bile duct. Type three is a choledochal seal that is present in the duodenal wall at the ampulla of Vater.
JASON P SULKOWSKI: Type four A is multiple cysts located both intra and extrahepatic. And type four B is multiple cysts located just in the extrahepatic bile duct. And then type five is Caroli's disease which is entirely intrahepatic dilations of the bile ducts. This can sometimes present throughout the entire liver or just in a single level of the liver. The pathophysiology is thought to be due to malunion of the pancreatic and biliary ducts during development.
JASON P SULKOWSKI: This leads to a long common channel which causes reflexive pancreatic fluid into the bile duct and that leads to damage occurring to the duct, which then allows it to start becoming dilated. A type 3 cyst is a little bit different. Most likely this is thought to be cause to dysfunction at the sphincter of Oddi. Next slide.
JASON P SULKOWSKI: So if you're concerned about a choledochal cyst after you've gotten your ultrasound, you want to check LFTs and look for the possibility of hyperbilirubinemia. Like I said, get an ultrasound. And then an MRCP is usually the next step to really get a good look at the anatomy. This is a great study to assess the extrahepatic and intrahepatic biliary anatomy.
JASON P SULKOWSKI: You can see an image here that you've got a fusiform dilation of the extrahepatic biliary system, so this is a type 1 choledochal cyst. ERCP can also be used. More commonly this will be done in an older patient. We typically don't do ERCPs on infants. When this is discovered, it's not really helpful by an ERCP because you have the ability to do interventions. It might be necessary if there's an obstructing stone or you need to do some sort of decompression for cholangitis.
JASON P SULKOWSKI: Next slide. So if a cyst is discovered, it needs to be resected. The management for a choledochal cyst is to resect it and do a reconstruction. So the reason to do this, is that this can lead to recurrent infections, recurrent episodes of cholangitis. There's also the risk of malignant degeneration of that cyst.
JASON P SULKOWSKI: It can become a cholangiocarcinoma. So for type one, two, and four cysts, the extrahepatic bile duct is excised in an hepaticoduodenostomy or hepaticojejunostomy with Roux-en-Y reconstruction is performed. You can see here in the surgical picture, this is a type one cyst. You can see the large dilation of the common bile duct. And then the lower image is the hepaticojejunostomy, the anastomosis of the bowel to the remnant bile duct.
JASON P SULKOWSKI: If you have a type three cyst, this can commonly be managed just with sphincterotomy which would be done in ERCP. You can also do a transduodenal excision of the choledochocele. And then for Caroli's disease, a type five cyst, you can do a liver resection if there's just a segment of the liver that's affected, but more commonly the entire liver is affected and a transplant must be done.
JASON P SULKOWSKI: Next slide. Complications from this surgery include a bile leak, stricture of the anastomosis, and recurrent cholangitis. Overall, the long-term outcomes tend to be excellent, but these patients do need to be followed up for development of cholangiocarcinoma. There are no formal guidelines in place, but it's probably just something to have checked on at a regular interval.
JASON P SULKOWSKI: Next slide. All right. So now we have a three-year-old who has come into your office with a neck mass, erythema, and tenderness. And you can see what it looks like here in the picture. Any thoughts?
JASON P SULKOWSKI:
ALFRED CHAHINE: Branchial cleft cysts, Dr. Badami says.
JASON P SULKOWSKI: That's good. All right. Next slide. So it can be a number of different things. This is a surgery lecture, so it's probably going to be a branchial cleft cyst, right? So it could be just simple lymphadenitis. It could have a dermoid cyst that's become infected, or a serbaceous cyst. Branchial cleft cysts are most likely going to present on the lateral aspect of the neck as in this image.
JASON P SULKOWSKI: If this were located in the midline, it would be a thyroglossal duct cyst potentially. You can also have a lymphatic malformation that can get infected or that can have hemorrhage into it and can present with erythema and pain. Next slide. So the embryology of branchial anomalies is relatively complicated, but the important thing to remember is that the branchial structures are comprised of ducts that are made out of ectoderm pouches which are endoderm and then arches which are mesoderm.
JASON P SULKOWSKI: And the failure of either a cleft or a pouch to obliterate results in a remnant cyst, sinus, or fistula. The opening superficially of a sinus or a cyst are going to be in a line just from interior to the tragus, and then down along the neck anterior to the sternocleidomastoid muscle. Internally, a fistula can open in a number of different places and it tends to depend on which cleft is involved. So if it's the first cleft, it's going to be the ear.
JASON P SULKOWSKI: The second will be the tonsil. Third cleft will be the pyriform sinus, and the fourth will open up in the esophagus. And there's a mnemonic to help you remember the number of syllables of the location of the fistula opening corresponds to the number of the cleft that's involved. Next slide. So by far the most common is a second branchial cleft cyst.
JASON P SULKOWSKI: These sinuses will typically present with either drainage or infection. If it's a cyst, it'll present with either a swelling or an infection. Next slide. If they present initially with infection, the first step is to treat it with antibiotics.
JASON P SULKOWSKI: If you need to, you can needle aspirate it. You really want to avoid doing an incision and drainage on these, because you can obscure the anatomy and make it more difficult to do a resection down the road. You can get an ultrasound to confirm the diagnosis if there's any question, but typically imaging is not always necessary. If you're suspicious that this is a first bronchial anomaly, you do want to do a good ear exam to make sure that there isn't a communication with the external auditory canal.
JASON P SULKOWSKI: And if you're concerned about a third or fourth branchial anomaly, you want to do laryngoscopy and esophagram to reveal communicating tracts. Next slide. So the management of these is surgical excision. So if they presented initially with an infection you want to make sure the infection is cleared first. You can use a probe or a catheter to follow the extent of the tracts.
JASON P SULKOWSKI: You can see an image here there's a probe going into the tract. And sometimes it extends pretty far and you can use stepladder incisions like here in order to follow the tract as it works its way proximally. It can extend into the pharynx, and knowledge of the anatomy is really key. These tend to be located pretty close to a number of important nerves and blood vessels, including the facial, vagus, hypoglossal, and glossopharyngeal nerves, and the carotid artery.
JASON P SULKOWSKI: Next slide. Overall, most patients with these do well after excision. There is a relatively high wound infection rate, up to 10%. Recurrence is more likely if some of the tracts or some of the cyst is left behind. That can occur in up to 20% of cases. If that does happen, reexcision or endoscopic cauterization of an internal opening that's present can be done.
JASON P SULKOWSKI: Next slide. As I mentioned, if this cystic structure was located in the midline and you notice that the cyst moved with swallowing, then you'd be particularly concerned about a thyroglossal duct cyst. These also have to be resected. The procedure for this is called a Sistrunk procedure, where you excise the entire cyst and the tract, including the mid portion of the hyoid bone, because the tract always goes through the hyoid rate if you don't resect that, there's a high rate of recurrence.
JASON P SULKOWSKI: These can extend all the way up to the base of the tongue. Rarely, there is no other thyroid tissue in the patient. And when you remove this cyst, you've removed all their thyroid tissue and they can present with hypothyroidism. So that's one reason that some surgeons will get a routine ultrasound of the neck prior to the surgical excision just to make sure that there is a normal thyroid gland present.
JASON P SULKOWSKI: Next slide. OK. So new topic. You've got a 15-year-old boy who's coming to your office with mild exercise intolerance and he's complaining about his sunken sternum. So there's really not much of a differential to this. This is basically a chest wall deformity known as pectus excavatum.
JASON P SULKOWSKI: Next slide. So there are several different main categories of chestal deformities. The most common is pectus excavatum, which is present in about 2% of all children. They're much more common in boys, but they do occur in girls as well. Next slide. The other main type is pectus carinatum, which is a protrusion of the sternum as opposed to a cavity.
JASON P SULKOWSKI: Again, these are much more commonly occurring in boys. Next slide. The thought is that these are a result of abnormal development of the costal cartilage which causes inward pressure on the sternum and that either causes a depression or an elevation of the sternum. Next slide. So when you're seeing a patient like this, you want to get a good history about whether or not they have any issues with exercise tolerance.
JASON P SULKOWSKI: Many patients won't have any complaints at all about being able to run and do sports, or will just have minimal symptoms. You want to look for any evidence of comorbidities, particularly connective tissue disorders like Marfan's or Ehlers-Danlos. And then your workup will proceed with a chest CT. You want to have good cross-sectional imaging, because there are a couple of indices that you want to calculate in order to A, determine that the diagnosis is correct, and B also, determine the extent of the defect.
JASON P SULKOWSKI: So one is called the Haller index which involves dividing the width of the chest by the distance between the vertebral body and the sternum. Any value greater than 3.2 of the Haller index is consistent with pectus excavatum and should be repaired. The other measure is the correction index, which basically tells you how much of an increase in the depth of the chest you need in order to get to a normal appearing chest.
JASON P SULKOWSKI: This is increasingly being used as a more accurate measure of really the degree of the pectus defect. Most patients will also be sent for pulmonary function tests and an echocardiogram in order to assess if there is some underlying cardiopulmonary effect from the defect, even if the patient is not reporting any symptoms. And then patients should also go for metal allergy testing.
JASON P SULKOWSKI: Because they may have an allergy to the metal that the bar is constructed out of, and you'd want to know that before implanting this device. If they do, then a premade titanium bar can be used instead. Next slide. So the treatment for pectus excavatum that's most commonly performed these days is a thoracoscopic Nuss procedure.
JASON P SULKOWSKI: So you start by doing the right sided thoracoscopy, and create a retrosternal window bluntly. And through this you pass a curved metal bar. It's inserted concave and then you flip it 180 degrees to make it convex and then this will elevate the sternum. Most patients are able to get by with one bar, but in more severe defects or in deeper defects sometimes two bars are necessary as this image. And the bars are left in place for about two to three years before they're removed.
JASON P SULKOWSKI: Next slide. The other procedure that's used for pectus excavatum is the Ravitch procedure. This is a much more invasive procedure. Involves longitudinal or transverse sternal incision. Bilateral skin flaps and pec major flaps are elevated. The perichondrium is incised sharply on either side of the sternum, and then the costal cartilage is resected.
JASON P SULKOWSKI: As you're doing that, a lot of times you'll see the sternum starts to flatten out automatically but sometimes the sternum is so abnormal that you have to create an osteotemy in order to help flatten. Next slide. Post-operatively, the main issue that most patients have is pain as you would expect.
JASON P SULKOWSKI: We typically use a multi-modal approach with pain controls. So basically every type of pain medicine you can think of is employed. So Tylenol and NSAIDS, muscle relaxants, gabapentin. Many patients are put on a PCA or given an epidural. And then some people perform an intercostal or paravertebral nerve block or even cryoablation. The main complications from this procedure. Obviously cardiac perforation is the one of most concern.
JASON P SULKOWSKI: If that were to occur, it mandates an immediate sternotomy and repair of the heart. The pericardium itself can be perforated. This doesn't require specifically repairing the pericardium, but you can't leave the bar within the pericardium. So you would have to reposition the bar in that case. Patients very frequently have a pneumothorax on one or both sides of the chest.
JASON P SULKOWSKI: This will often just improve on its own without any intervention. The bar can migrate, it can flip around 180 degrees, or it can just migrate to an abnormal position. This does require to be repositioned in the operating room. And then rarely patients will also have a bar infection or an allergic reaction to the bar. Often this can be treated just with medications, but it does sometimes require removal of the bar.
JASON P SULKOWSKI: Next slide. Nearly all patients end up with improved appearance after a Nuss procedure or a Ravitch. And many will also have the benefit of cardio pulmonary improved performance. They'll have improved FEV1 and their right heart function will be better on an echo. Many patients also described improved exercise tolerance.
JASON P SULKOWSKI: Next slide. I see Abbas has asked a question. Is there a particular age you wait to perform the procedure? The best time to do it is in the early teens, because much of the growth-- much of the linear growth has completed, but the bones are still a little bit more flexible.
JASON P SULKOWSKI: And so it's a good time to do it. If you wait until late teens or even into the 20s, it can still be done but you might not necessarily get as much of benefit. All right. This is our last case, I believe. So we've got a newborn infant who presents immediately after birth with respiratory distress. An immediate chest X-ray is obtained and this is what you see.
JASON P SULKOWSKI: Any thoughts? So this is consistent with a diaphragmatic hernia. You could clearly see that there is intestine in the left chest. So CDH is a congenital defect in the diaphragm that allows bowel or any other abdominal organs to permeate into the thoracic cavity. There are two main types. Morgagni and Bochdalek.
JASON P SULKOWSKI: The defect results in compression of the ipsolateral lung which causes pulmonary hypoplasia and pulmonary vascular hypertrophy and hypertension. Next slide. It occurs in about two in 10,000 live births. It's frequently associated with other defects and anomalies, such as trisomies, congenital heart disease, abdominal wall defects.
JASON P SULKOWSKI: And it's always associated with an intestinal rotational anomaly, since the bowel is in the chest and not able to fix normally into the abdomen. Next slide. So more and more commonly these are being diagnosed prenatally on ultrasound, and then with follow up MRI. This is helpful because we can determine the laterality of the defect, we can see if the liver is in the chest or in the abdomen, and we can calculate the lung head ratio, which is done by measuring the cross-sectional area of a lung divided by the head circumference.
JASON P SULKOWSKI: This is really helpful for prognosis. Next slide. So if the liver is down in the abdomen, survival rates are quite good. Greater than 90%. However, if the liver is up in the chest, the survival is less than 50%. And the lung head ratio is also prognostic. If this is found to be less than 0.8 or 1, this is considered to be associated with a poor prognosis.
JASON P SULKOWSKI: Also increasingly, we're using the observed to expected LHR which has been found to be a little bit more helpful. You can see if the O to E ratio is 10% the survival is pretty dismal. Whereas as the O to E ratio approaches 50%, then the survival is upwards of 3 in 4 patients. Next slide. So if the diaphragm hernia wasn't diagnosed prenatally, it can also most commonly be diagnosed shortly after birth with respiratory distress, as in the case presented.
JASON P SULKOWSKI: You'll also see a scaphoid abdomen, since much of the abdominal content is up in the chest. And then you will clearly see the X-ray findings show. Next slide. And the last way that a CDH can present is sort of in a delayed fashion. This is usually a Morgagni hernia. And sometimes they can present with vague abdominal symptoms, but it's often just identified with because a child is getting a chest X-ray for some other indication and you just happen to discover that there's bowel up in the chest.
JASON P SULKOWSKI: Next slide. So for the standard neonatal diaphragmatic hernia, the most important initial step is just maintaining cardiopulmonary stability. You really want to minimize iatrogenic injury from how you're managing your vent and your cardiopulmonary support. So we use pressure control ventilation and permissive hypercapnia.
JASON P SULKOWSKI: The key is to follow a strict protocol. There's great evidence that just following vent management protocol is much better than kind of improvising. Other adjuncts to management include using nitric oxide, using the oscillatory ventilator, and ECMO. So ECMO is used probably in about one in three cases of CDH. Indications include a preductal oxygen saturation of less than 85%, inability to improve the pH better than 7.2, persistent hypercarbia above 70, and persistent hypertension.
JASON P SULKOWSKI: Next slide. So the timing of repairs is critical for patients like this. You really want to wait until you've achieved some degree of physiologic stabilization with some normalcy to your vitals. Ideally you want to have your vent at a setting FiO2 less than 50%.
JASON P SULKOWSKI: You'd like your blood pressure to be within normal limits, and you want evidence of good tissue perfusion. So a normal urine output, a normalized lactate, and a normal pH. You also want to make sure on echo that your pulmonary arterial pressures are less than systemic pressures. On the other hand, if you've had to resort to ECMO it's very controversial whether or not you should do your repair on or off ECMO.
JASON P SULKOWSKI: Many different people use many different approaches to this. Some people will repair right away after going on ECMO. Other people will give the baby a chance to get off ECMO and if not, then do the repair. And then still others will wait until the ECMO run has ended and then do the CDH repair. There's really no one right approach. Next slide.
JASON P SULKOWSKI: So the approach to repair can be done either thoracoscopically or open. This is going to depend entirely on how sick the baby is. So a baby who has an obvious diaphragmatic hernia but is on room air and is very stable, you can easily get away with doing the thoracoscopic repair. On the other hand, if you're doing a repair on ECMO, really you're probably going to do that open. You can see there are four different categories of defects increasing in size from A through D.
JASON P SULKOWSKI: If you've got an A or a B defect, those are more often amenable to a thoracoscopic approach, and potentially doing a primary repair. If you have a C or D defect, then you really should be doing it open and they're more likely to require that you use a patch. Next slide.
JASON P SULKOWSKI: Common complications after CDH repair include bleeding, infection, you can get compartment syndrome after reducing all the abdominal contents back in their correct location. Chylothorax is a relatively common complication from CDH repairs. And then also recurrence of the hernia. Late complications include a risk for bowel obstructions and again, recurrence of the hernia which occurs in about 10% to 20% of cases.
JASON P SULKOWSKI: These babies are overall medically complex and are associated with a number of other long term sequelae, including gastroesophageal reflux. About one in four will end up getting a Nissen. There's significant morbidity from pulmonary hypoplasia and pulmonary hypertension. Neurodevelopmental delay is extremely common with about 2/3 of all patients having this at three years of age. And there are also significant musculoskeletal abnormalities due to tension on the diaphragm, the thoracotomy incision, and then also from the hypoplastic lungs.
JASON P SULKOWSKI: Next slide. And that is it. Are there any other questions that I can answer?
ALFRED CHAHINE: Wonderful, Dr. Sulkowski. This was a great review of very difficult topics. In the interest of time, I guess, Dr. Badami or any up with you do you have any questions? All right. So we'll see you next week, and thank you so much. Take care.
JASON P SULKOWSKI: Thank you.
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