Omphalocele and Gastroschisis are abdominal wall defects that develop in-utero and surgically treated post-birth. Join Dr. Foong-Yen Lim, Surgical Director of the Cincinnati Children’s Fetal Care Center, and Dr. Todd Ponsky to discuss the prenatal diagnosis and management of abdominal wall defects.
Type: podcast · 9 min · posted 2022-02-10 Canonical: https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006 ## Chapters - [0:00](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=0) Introduction and Anatomical Definitions - [1:54](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=114) Prenatal Diagnosis and Surveillance - [3:15](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=195) Epidemiology and Risk Factors - [5:02](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=302) Immediate Postnatal Management and Closure Options - [6:58](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=418) Cincinnati Children's Techniques and Complex Scenarios ## Statements - "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" — Foong-Yen Lim (clinical) [0:55](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=55) - "Omphalocele is right through the middle of the umbilicus and has a membranous cover" — Foong-Yen Lim (clinical) [0:55](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=55) - "Omphalocele is a congenital disorder and can have associated anomalies, but gastroschisis occurs in otherwise normal babies that have an intrauterine event during development" — Foong-Yen Lim (clinical) [0:55](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=55) - "Very high level of alpha fetal protein is associated with gastroschisis or omphalocele" — Foong-Yen Lim (clinical) [1:54](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=114) - "Ultrasound imaging is needed to definitively diagnose abdominal wall defects" — Foong-Yen Lim (clinical) [1:54](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=114) - "For omphalocele, besides ultrasound, MRI and echocardiogram are routinely obtained because these patients may have other associated anomalies" — Foong-Yen Lim (guideline) [1:54](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=114) - "Fetal growth is tracked monthly because there is concern for significant growth restriction" — Foong-Yen Lim (guideline) [1:54](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=114) - "Biophysical profile and non-stress testing are needed because intrauterine growth restriction and intrauterine fetal demise can occur in these patients" — Foong-Yen Lim (clinical) [2:56](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=176) - "Gastroschisis affects approximately one in every 2,200 live births" — Todd Ponsky (epidemiological) [3:15](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=195) - "Gastroschisis is most common among babies born to young mother of low gravity and usually first pregnancy, with 75% being first born" — Foong-Yen Lim (epidemiological) [3:27](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=207) - "Nearly 60% of gastroschisis cases are premature" — Foong-Yen Lim (epidemiological) [3:27](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=207) - "More than 90% of gastroschisis infants are born less than 2,500 grams in weight" — Foong-Yen Lim (epidemiological) [3:27](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=207) - "Pseudoephedrine and acetaminophen have an odds ratio of 4.2 times for gastroschisis compared to aspirin alone at 2.7" — Foong-Yen Lim (epidemiological) [3:27](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=207) - "Other risk factors for gastroschisis include vitamin B deficiencies, drug use, and some genetic predispositions" — Foong-Yen Lim (epidemiological) [3:27](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=207) - "Mothers with omphalocele are usually advanced in their age compared to the gastroschisis group, with advanced maternal age having an odds ratio of 3.3" — Foong-Yen Lim (epidemiological) [4:19](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=259) - "Trisomy 13, 18, and 21 occur in anywhere between 35 and 90% of patients with omphalocele" — Foong-Yen Lim (epidemiological) [4:19](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=259) - "Minimizing heat loss and fluid loss in these babies is crucial immediately after birth, otherwise they can show up extremely dehydrated and cold" — Foong-Yen Lim (clinical) [5:02](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=302) - "These babies can have significant acidosis and pulmonary hypertension" — Foong-Yen Lim (clinical) [5:02](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=302) - "Primary closure is considered if bowel looks pristine, non-thickened, non-inflammatory, with only small amount on the outside and enough abdominal domain" — Foong-Yen Lim (guideline) [5:34](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=334) - "Staged closure is favored if the defect is large or there's issue with the bowel" — Foong-Yen Lim (guideline) [6:07](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=367) - "Some patients can have atresia, compromised bowel, or perforation" — Foong-Yen Lim (clinical) [6:07](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=367) - "Patients can develop intestinal perforation after only two days to four days of enteral feeding" — Foong-Yen Lim (clinical) [6:07](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=367) - "Majority of babies in the last four and a half years at Cincinnati Children's are being managed using a sutureless closure" — Todd Ponsky (clinical) [6:34](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=394) - "In sutureless closure, after pushing the bowel back in, the umbilical cord is put over and then dressing over, without taking babies to the operating room to close with suture" — Foong-Yen Lim (clinical) [6:58](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=418) - "With sutureless closure, skin will grow over and the umbilical defect can close spontaneously over time" — Foong-Yen Lim (clinical) [6:58](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=418) - "For small bowel atresia, management options include tapering the dilated portion of bowel or resecting the bowel before tapering" — Foong-Yen Lim (opinion) [7:29](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=449) - "For omphalocele with no significant respiratory issues, sequential reduction using meshes is the best option" — Foong-Yen Lim (guideline) [7:57](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=477) - "Meshes are sewn to the edges of the fascia without interrupting the membrane" — Foong-Yen Lim (clinical) [7:57](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=477) - "At Cincinnati Children's, Duoderm silo is done on top of the skin using plastic clips to sequentially clip it down until it's flush to the abdominal skin" — Foong-Yen Lim (clinical) [8:17](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=497) - "After sequential reduction with plastic clips, the patient is taken to the operating room for delayed primary closure of the fascia and skin" — Foong-Yen Lim (clinical) [8:17](https://library.globalcastmd.com/watch/omphalocele-and-gastroschisis-with-dr-foong-yen-lim-5006?t=497) ## Transcript 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 umbilical is that umbilical 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 umbilical. 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 emphalaceal. 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 confirmed diagnosis and don't get additional imaging. However, for emphalaceal, 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? Inutero 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 2,200 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 2,500 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 amphalocele. Usually moms are advanced, um, in their age compared to, you know, the, the gastroschisis group. And you can see that, uh, AMA, uh, the odd ratio is 3.3. You can see that the major risk factor for amphalocele are trisomy 13, 18, and 21, which occur in anywhere between 35 and 90% of patients with amphalocele. 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, 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. But what about stage closure? We favor stage closure if the defect is large or, um, there's issue with the bowel. So some of these patients unfortunately can have, uh, atresia, can have, um, bowel, uh, being compromised, uh, or also perforation. We definitely have patients that after only two days to four days of, uh, enteral feeding that they develop, uh, 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, uh, four and a half years are now being managed using a suture-less closure. Dr. Lim, walk us through how the Cincinnati Children's team performs a suture-less closure. After you push the bowel back in, uh, 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, uh, although they may still have a small umbilical defect over time, uh, the umbilical defect can close spontaneously. 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, um, it's up to, you know, how you want to manage that dilated portion of the bowel. Um, some of us will taper, uh, that some of us will resect the bowel, um, before tapering. So we have a primary closure, a staged closure and a suture-less closure. But what if we have a baby with omphalose seal and no significant respiratory issues? What's the best option here? It's a sequential reduction using, uh, meshes. You can use different kind of meshes, um, to eventually push all of the content in. You will sew the meshes to the edges of the, um, the fascia without actually, uh, 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 this. So we form these are duoderm and we can form over the, the, um, um, emphalose and then just use the plastic clip and sequentially clip it down until it's flashed to the abdominal, uh, 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, the same patient from the previous picture, uh, to the operating room. And, and we finally did a primary closure, um, delay 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. --- Not medical advice · citation policy: https://library.globalcastmd.com/ai