Babies born with abdominal wall defects like omphalocele and gastroschisis can often have potentially complex postnatal care, which is why today we're going to discuss some key knowledge points with Dr. Fung Lim, who's a pediatric surgeon at Cincinnati Children's Hospital and the surgical director of the Fetal Care Center. I'm Todd Ponski, and this is the Stay Current in Pediatric Surgery podcast. All right, so let's start out with a textbook image of gastroschisis. If you're listening to the audio version of this podcast, scroll down under the media player in the Stay Current app, and we'll give you the link to this and every other image that Dr. Lim will be discussing. So this is a full thickness defect in the abdominal wall that allows, as you can see here, the bowel to protrude. So you can see the umbilical cord right next to it, and that's the bowel coming through the hole. So gastroschisis is a full thickness abdominal wall defect just to the right of the umbilicus, in which peritoneal contents, most often intestines, protrude through the abdomen without any membranous cover. And on phalocele, it's right through the middle of the umbilicus, and it has a membranous cover. But the main difference between gastroschisis and umphalocele is that umphalocele is a congenital disorder and can have associated anomalies, but gastroschisis occurs in otherwise normal babies that have an intrauterine event during development. Let's compare this image to the next one, which shows umphalocele. The obvious difference is the membrane covering the herniated contents, but another difference, and majority of the times, uh, for large defects, you'll have, besides intestine, uh, a good amount of the liver is on the outside. So prenatal diagnosis is an important factor in identifying these conditions, but are the warning signs we should be looking for obvious? If you see a very high level of alpha fetal protein, think about um, gastroschisis or umphalocele. The alpha fetal protein or AFP is a great screening test, but you need ultrasound imaging to definitively diagnose these abdominal wall defects. Uh, for gastroschisis, uh, we normally only get ultrasound, confirm the diagnosis and don't get additional imaging. However, for umphalocele, uh, besides the ultrasound, we also routinely get MRI and echocardiogram. Because these patients may also have other associated anomalies. Now, luckily, once we get the imaging, we can then follow a protocol to follow it up. As you can see here, we track their fetal growth monthly because there is a concern for significant growth restriction. You also need to follow them, uh, using biophysical profile and non-stress testing. Okay, wait, why do we need to do biophysical profile and non-stress testing? In utero growth restriction as well as IUFD in utero fetal demise, uh, can occur in this patient. So, if the surveillance imaging becomes worrisome enough that you're concerned about intrauterine fetal demise, the mother and the fetus need to be admitted for continuous monitoring or deliver the fetus emergently. Let's change gears and talk about epidemiology. Gastroschisis affects approximately one in every 2200 live births. But which babies are at highest risk for gastroschisis? It's, uh, most common among babies born to young mother of low gravity and usually first pregnancy. And 75% of that is the first born. So 25% is in the second or subsequent. Nearly 60% are premature. Unfortunately, intrauterine growth restriction is another big concern with more than 90% of these infants born less than 2500 grams in weight. There are other identified risk factors, including some common over-the-counter medications. It's actually as simple as pseudofed. Uh, pseudofed that contains, uh, pseudoephrin, uh, and acetaminophen. The, the odd ratio is actually 4.2 times compared to aspirin alone 2.7. And then there are other risk factors like vitamin B deficiencies, drug use, and some genetic predispositions. All right, now let's talk about the risk factors for omphalocele. Usually moms are advanced, um, in their age compared to, you know, the, the gastrosis group. And you can see that, uh, AMA, uh, the odd ratio is 3.3. You can see that the major risk factor for omphalocele are trisomy 13, 18, and 21, which occur in anywhere between 35 and 90% of patients with omphalocele. All right. So now that we're caught up on the prenatal diagnosis, anatomy, and epidemiology, let's go through a case. So we are outside of a tertiary pediatric center and we come across a congenital domino wall defect. What are the crucial procedures that we have to take immediately after birth? Minimizing heat loss and fluid loss in these babies. Otherwise they can show up, uh, extremely dehydrated as well as, um, being cold. These babies can have a significant acidosis and pulmonary hypertension. The severity of either of these diagnoses at birth can range widely, which is why it's key to take these initial non-operative steps before moving onto the closure. So closing these abdominal wall defects can be performed as either a primary closure or a stage closure. Dr. Lim, in which patients do you consider primary closure? If you have a bowel that look pretty pristine, non-thickened, non-inflammatory, and only small amount of them being on the outside, and that's enough abdominal domain, you can actually push them all back very quickly and perform primary closure. Okay. So if the bowel appears pristine, we could potentially do a primary closure. In fact, these bottom two images show how small the abdominal wall defects look over time following primary closure. Dr. Lim, in which patients are not being able to prevent the bowel from the bowel. But what about stage closure? Dr. Lim, we favor stage closure if the defect is large or there's issue with the bowel. So some of these patients unfortunately can have atresia, can have bowel being compromised, or also perforation. We definitely have patients that after only two days to four days of enteral feeding that they develop intestinal perforation. Okay, so if the bowel looks perfect, you could consider primary closure. If the defect is too large, consider a staged approach. But how are things done at Cincinnati Children's Hospital? Majority of our babies in the last four and a half years are now being managed using a sutureless closure. Dr. Lim, walk us through how the Cincinnati Children's Team performs a sutureless closure. Dr. Lim, walk us through how the bowel is in the middle of the bowel. After you push the bowel back in, you don't take these babies to the operating room to close that with suture, but rather put the umbilical cord over and then the dressing over. And a lot of times skin will grow over. Although they may still have a small umbilical defect, over time, the umbilical defect can close spontaneously. Dr. Lim, walk us through the bowel. Wow, so that was really helpful. But now let's talk about another challenging scenario. What do you do if you encounter a small bowel atresia? So in this particular case, it's up to, you know, how you want to manage that dilated portion of the bowel. Some of us will taper that. Some of us will resect the bowel before tapering. So we have a primary closure, a staged closure, and a sutureless closure. But what if we have a baby with omphalocele and no significant respiratory issues? What's the best option here? It's a sequential reduction using meshes. You can use different kinds of meshes to eventually push all of the content in. You will sew the meshes to the edges of the fascia without actually interrupting the membrane. You may have heard of the pitcher clamp procedure, and many of you may have even done it. But at Cincinnati Children's, they do it a little different. Duoderm silo can be done just on top of the skin of the patient, such as these. So we form, these are duoderm, and we can form over the emphalcia and then just use the plastic clip and sequentially clip it down until it's flashed to the abdominal skin. So they sequentially reduce the bowel with plastic clips until the baby is ready for that last step. And then take this patient, this is the same patient from the previous picture to the upper room and we finally did a primary closure, delayed primary closure of the fascia and skin. Thanks, Dr. Lim. The management of congenital abdominal wall defects can be complex. If you liked this episode, be sure to follow us on social media, subscribe to our YouTube channel and download the Stay Current in Pediatric Surgery app. It's in the Apple App Store and the Google Play Store. But until next time, remember knowledge should be free.