Omphalocele and Gastroschisis With Dr. Foong-Yen Lim

Published: 2022-02-10

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Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Todd Ponski — host
Dr. Foong-Yen Lim — guest

Chapters

0:00Introduction and Anatomical Definitions — Introduction to abdominal wall defects with Dr. Fung Lim from Cincinnati Children's Hospital. Defines gastroschisis as full-thickness defect to the right of umbilicus without membrane, and omphalocele as midline defect with membranous cover and potential associated anomalies.
1:54Prenatal Diagnosis and Surveillance — Elevated alpha-fetoprotein as screening test, ultrasound for definitive diagnosis. Omphalocele requires additional MRI and echocardiogram due to associated anomalies. Monthly fetal growth tracking, biophysical profile, and non-stress testing due to risks of growth restriction and intrauterine fetal demise.
3:15Epidemiology and Risk Factors — Gastroschisis affects 1 in 2,200 live births, most common in young mothers, first pregnancies, with 60% premature and over 90% under 2,500 grams. Risk factors include pseudoephedrine and acetaminophen. Omphalocele associated with advanced maternal age and trisomy 13, 18, and 21 in 35-90% of cases.
5:02Immediate Postnatal Management and Closure Options — Initial focus on minimizing heat and fluid loss to prevent dehydration, acidosis, and pulmonary hypertension. Primary closure considered for pristine bowel with small defects and adequate abdominal domain. Staged closure favored for large defects or compromised bowel, including cases with atresia or perforation.
6:58Cincinnati Children's Techniques and Complex Scenarios — Sutureless closure technique using umbilical cord and dressing, allowing spontaneous skin growth. Management of small bowel atresia with tapering or resection. Sequential reduction for omphalocele using Duoderm silo and plastic clips, followed by delayed primary closure.

Key claims

0:55Gastroschisis 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 — Dr. Foong-Yen Lim
0:55Omphalocele is right through the middle of the umbilicus and has a membranous cover — Dr. Foong-Yen Lim
0:55Omphalocele is a congenital disorder and can have associated anomalies, but gastroschisis occurs in otherwise normal babies that have an intrauterine event during development — Dr. Foong-Yen Lim
1:54Very high level of alpha fetal protein is associated with gastroschisis or omphalocele — Dr. Foong-Yen Lim
1:54Ultrasound imaging is needed to definitively diagnose abdominal wall defects — Dr. Foong-Yen Lim
1:54For omphalocele, besides ultrasound, MRI and echocardiogram are routinely obtained because these patients may have other associated anomalies — Dr. Foong-Yen Lim
1:54Fetal growth is tracked monthly because there is concern for significant growth restriction — Dr. Foong-Yen Lim
2:56Biophysical profile and non-stress testing are needed because intrauterine growth restriction and intrauterine fetal demise can occur in these patients — Dr. Foong-Yen Lim
3:15Gastroschisis affects approximately one in every 2,200 live births — Todd Ponski
3:27Gastroschisis is most common among babies born to young mother of low gravity and usually first pregnancy, with 75% being first born — Dr. Foong-Yen Lim
3:27Nearly 60% of gastroschisis cases are premature — Dr. Foong-Yen Lim
3:27More than 90% of gastroschisis infants are born less than 2,500 grams in weight — Dr. Foong-Yen Lim
3:27Pseudoephedrine and acetaminophen have an odds ratio of 4.2 times for gastroschisis compared to aspirin alone at 2.7 — Dr. Foong-Yen Lim
3:27Other risk factors for gastroschisis include vitamin B deficiencies, drug use, and some genetic predispositions — Dr. Foong-Yen Lim
4:19Mothers with omphalocele are usually advanced in their age compared to the gastroschisis group, with advanced maternal age having an odds ratio of 3.3 — Dr. Foong-Yen Lim
4:19Trisomy 13, 18, and 21 occur in anywhere between 35 and 90% of patients with omphalocele — Dr. Foong-Yen Lim
5:02Minimizing heat loss and fluid loss in these babies is crucial immediately after birth, otherwise they can show up extremely dehydrated and cold — Dr. Foong-Yen Lim
5:02These babies can have significant acidosis and pulmonary hypertension — Dr. Foong-Yen Lim
5:34Primary closure is considered if bowel looks pristine, non-thickened, non-inflammatory, with only small amount on the outside and enough abdominal domain — Dr. Foong-Yen Lim
6:07Staged closure is favored if the defect is large or there's issue with the bowel — Dr. Foong-Yen Lim
6:07Some patients can have atresia, compromised bowel, or perforation — Dr. Foong-Yen Lim
6:07Patients can develop intestinal perforation after only two days to four days of enteral feeding — Dr. Foong-Yen Lim
6:34Majority of babies in the last four and a half years at Cincinnati Children's are being managed using a sutureless closure — Todd Ponski
6:58In 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 — Dr. Foong-Yen Lim
6:58With sutureless closure, skin will grow over and the umbilical defect can close spontaneously over time — Dr. Foong-Yen Lim
7:29For small bowel atresia, management options include tapering the dilated portion of bowel or resecting the bowel before tapering — Dr. Foong-Yen Lim
7:57For omphalocele with no significant respiratory issues, sequential reduction using meshes is the best option — Dr. Foong-Yen Lim
7:57Meshes are sewn to the edges of the fascia without interrupting the membrane — Dr. Foong-Yen Lim
8:17At 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 — Dr. Foong-Yen Lim
8:17After sequential reduction with plastic clips, the patient is taken to the operating room for delayed primary closure of the fascia and skin — Dr. Foong-Yen Lim

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Abdominal Wall Defects: When Bowel Develops Outside the Body

The episode's main topic retold as a plain-language walkthrough — what it is, why it matters, and what the speakers concluded. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Explainer · AI-written, human-reviewed

Why This Subspecialty Exists

Gastroschisis and omphalocele represent failures in the embryologic closure of the abdominal wall, leaving intestines and sometimes other viscera exposed at birth. These defects require immediate neonatal intervention, and — particularly in omphalocele — frequently coexist with chromosomal abnormalities and structural malformations that demand coordinated multidisciplinary care 3:15 4:19. Pediatric surgeons who manage these conditions navigate not only the technical challenge of returning organs to the abdomen but also the physiologic fragility of growth-restricted, premature infants at high risk for metabolic collapse.

The Core Clinical Problem

Gastroschisis is a full-thickness defect just to the right of the umbilicus through which intestines herniate without any covering membrane 0:55. It occurs as an intrauterine vascular event in otherwise chromosomally normal fetuses 0:55. Omphalocele, by contrast, is a midline defect through the umbilicus itself, covered by a membranous sac, and represents a congenital malformation associated with trisomy 13, 18, or 21 in 35 to 90% of cases 0:55 4:19.

The immediate postnatal threat is not the defect itself but the consequences of evaporative heat and fluid loss from exposed bowel. Without aggressive thermal protection and fluid resuscitation, these infants rapidly become hypothermic, dehydrated, acidotic, and develop pulmonary hypertension 5:02 5:02. The surgical problem is then twofold: return the herniated contents to an abdominal cavity that may be too small to accommodate them, and do so without compromising bowel perfusion or respiratory mechanics.

How the Approach Works

Prenatal diagnosis typically follows an elevated maternal serum alpha-fetoprotein, confirmed by ultrasound 1:54 1:54. For omphalocele, the workup extends to fetal MRI and echocardiography to screen for associated cardiac and central nervous system anomalies 1:54. Monthly ultrasound tracks fetal growth because intrauterine growth restriction is common, and biophysical profiles monitor for fetal compromise that might necessitate early delivery 1:54 2:56. Nearly 60% of gastroschisis infants are born prematurely, and more than 90% weigh under 2,500 grams 3:27 3:27.

At delivery, the priority is minimizing evaporative losses. The exposed bowel is covered, the infant is placed under a warmer, and fluid resuscitation begins immediately. The surgical decision then hinges on the appearance of the bowel and the size of the defect. If the bowel looks pristine — non-thickened, non-inflamed — and the defect is small with adequate abdominal domain, primary closure is feasible 5:34. The surgeon reduces the contents and closes the fascia and skin in a single operation.

When the defect is large or the bowel is compromised — thickened, matted, or frankly perforated — staged closure is favored 6:07 6:07. Traditionally this involved placing a spring-loaded silo over the herniated bowel and progressively reducing it over days. At Cincinnati Children's Hospital, the majority of gastroschisis cases now undergo sutureless closure: after reducing the bowel, the umbilical cord stump is positioned over the defect and dressed, allowing skin to migrate centrally and close the defect spontaneously over weeks without a formal fascial repair 6:34 6:58 6:58. This approach avoids a return to the operating room and appears to yield acceptable cosmetic and functional outcomes.

Complications include intestinal atresia — present in a subset of gastroschisis cases — which may require resection or tapering of dilated proximal bowel 7:29. Late perforations can occur even after initial closure, sometimes manifesting only after enteral feeds are introduced within the first several days of life 6:07.

For omphalocele without respiratory compromise, sequential reduction using a mesh or synthetic silo sewn to the fascial edges allows gradual reduction of the sac contents without violating the membrane 7:57 7:57. Cincinnati's technique uses a Duoderm silo clipped to the skin with plastic clips, progressively tightened until the sac is flush with the abdominal wall, at which point the infant returns to the operating room for delayed primary fascial and skin closure 8:17 8:17.

Where Practice Remains Contested

The optimal timing of delivery, the role of cesarean section, and the threshold for primary versus staged closure all vary by center and by surgeon. The sutureless technique, while increasingly adopted, lacks long-term data on hernia rates and abdominal wall function. Management of intestinal atresia — whether to taper or resect — remains a matter of surgical judgment rather than protocol 7:29.

When to Involve This Team

Any fetus with an abdominal wall defect identified on prenatal ultrasound should prompt referral to a tertiary center with pediatric surgery and neonatal intensive care capabilities. Delivery should be planned at that center. Elevated maternal serum alpha-fetoprotein in the second trimester warrants anatomic ultrasound even if the initial screen was otherwise reassuring 1:54. For omphalocele, genetic counseling and karyotyping are essential given the high rate of aneuploidy 4:19. Postnatal transfer is appropriate only if the infant is stable, adequately resuscitated, and the bowel is protected — but outcomes are better when delivery occurs where definitive care is available.

Takeaways from this story

Gastroschisis occurs in chromosomally normal infants; omphalocele carries 35-90% risk of trisomy 13, 18, or 21.
Immediate postnatal priority is preventing hypothermia and dehydration, which rapidly lead to acidosis and pulmonary hypertension.
Primary closure is appropriate for small defects with pristine bowel; large or compromised defects require staged reduction.
Sutureless closure allows spontaneous skin migration over the defect, avoiding formal fascial repair in selected gastroschisis cases.
Intestinal perforation can occur after closure once enteral feeds begin, requiring vigilance during the early feeding period.

Surgical Decision-Making in Neonatal Abdominal Wall Defects

The episode's teaching points arranged as a structured lesson, building from the basics up to the finer points. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Teaching arc · AI-written, human-reviewed

Anatomic distinction drives everything downstream

Gastroschisis and omphalocele are not variations on a theme — they are fundamentally different lesions requiring different workups and different management 0:55 0:55 0:55. Gastroschisis is a full-thickness defect to the right of the umbilicus without membrane, occurring from an intrauterine event in otherwise normal babies 0:55 0:55. Omphalocele is midline through the umbilicus with membranous cover and represents a congenital disorder with associated anomalies 0:55 0:55. This distinction determines whether you order an echocardiogram and MRI (omphalocele) or proceed with ultrasound alone (gastroschisis) 1:54. The syndromic associations matter: trisomy 13, 18, and 21 occur in 35 to 90% of omphalocele patients 4:19, while gastroschisis carries no such burden but presents in a different demographic — 75% first-born to young mothers, with 60% premature and more than 90% under 2,500 grams 3:27 3:27 3:27.

Fetal surveillance targets the known failure modes

Monthly fetal growth tracking, biophysical profile, and non-stress testing are not protocol for protocol's sake — they exist because intrauterine growth restriction and intrauterine fetal demise are real risks in this population 1:54 2:56. If surveillance imaging becomes worrisome enough that you are concerned about fetal demise, the threshold for admission or emergent delivery has been crossed. The monitoring intensity reflects the stakes, not institutional preference.

Immediate postnatal management prevents the cascade

Minimizing heat loss and fluid loss is the first priority after delivery 5:02. These infants arrive dehydrated and hypothermic if you do not intervene, and from there they develop significant acidosis and pulmonary hypertension 5:02 5:02. The bowel exposure is obvious; the metabolic derangement that follows is what kills them. Wrap, warm, resuscitate — before you think about closure.

Closure strategy depends on bowel quality and abdominal domain

Primary closure is appropriate when the bowel looks pristine — non-thickened, non-inflammatory, small amount exteriorized, and adequate abdominal domain to accommodate reduction 5:34. If the defect is large or the bowel is compromised, staged closure is favored 6:07. Some patients present with atresia, compromised bowel, or perforation 6:07. Others develop intestinal perforation after only two to four days of enteral feeding 6:07. The decision is not about your comfort with primary closure; it is about whether the bowel and abdomen will tolerate it.

Sutureless closure eliminates an operation

The majority of gastroschisis cases at Cincinnati Children's over the last four and a half years have been managed with sutureless closure 6:34. After reducing the bowel, the umbilical cord is placed over the defect and dressed — no trip to the operating room, no sutures 6:58. Skin grows over, and the umbilical defect closes spontaneously over time 6:58. This is not a compromise; it is a deliberate strategy that avoids anesthesia and operative risk in a population already metabolically fragile.

Omphalocele with intact membrane allows sequential reduction

For omphalocele without significant respiratory issues, sequential reduction using meshes is the best option 7:57. Meshes are sewn to the fascial edges without interrupting the membrane 7:57. At Cincinnati Children's, a Duoderm silo is applied over the skin and sequentially clipped down using plastic clips until it is flush with the abdominal skin 8:17. After sequential reduction, the patient is taken to the operating room for delayed primary closure of fascia and skin 8:17. The membrane buys you time; use it.

Bowel atresia complicates but does not preclude closure

When small bowel atresia is encountered, the dilated proximal bowel must be addressed — either by tapering the dilated segment or resecting before tapering 7:29. The choice depends on the degree of dilation and the viability of the bowel. This is a judgment call, not a protocol, and it is made intraoperatively based on what you find.

Takeaways from this story

Gastroschisis and omphalocele require different workups: echo and MRI for omphalocele, ultrasound alone for gastroschisis.
Sutureless closure avoids operative risk — bowel reduced, umbilical cord placed over, skin grows over spontaneously.
Primary closure requires pristine bowel and adequate domain; staged closure for large defects or compromised bowel.
Heat and fluid loss drive acidosis and pulmonary hypertension — wrap and resuscitate before thinking about closure.
Intestinal perforation can develop after only two to four days of enteral feeding in these patients.

Abdominal Wall Defects: From Prenatal Diagnosis Through Closure Strategy

The episode's teaching points arranged as a structured lesson, building from the basics up to the finer points. Written by Kai from the episode transcript and reviewed before publishing.

For trainees · Teaching arc · AI-written, human-reviewed

Anatomy defines everything downstream

Location and membrane presence separate the two defects fundamentally. Gastroschisis is a full-thickness defect to the right of the umbilicus with exposed bowel and no membrane 0:55. Omphalocele sits midline through the umbilicus with a membranous sac intact 0:55. The distinction matters because omphalocele is a congenital disorder with associated anomalies, while gastroschisis occurs from an intrauterine event in otherwise normal babies 0:55. This difference drives every subsequent decision — from prenatal workup to closure timing to long-term prognosis.

Prenatal surveillance targets known failure modes

For omphalocele, the workup extends beyond the abdominal wall. Besides ultrasound confirmation, MRI and echocardiogram are routinely obtained because these patients may have other associated anomalies 1:54. Trisomy 13, 18, and 21 occur in anywhere between 35 and 90% of patients with omphalocele 4:19. For gastroschisis, ultrasound alone suffices for diagnosis, but both conditions require monthly fetal growth tracking because of concern for significant growth restriction 1:54. Biophysical profile and non-stress testing become necessary because intrauterine growth restriction and intrauterine fetal demise can occur 2:56. More than 90% of gastroschisis infants are born less than 2,500 grams in weight 3:27. The surveillance intensity reflects the risk: if imaging becomes worrisome enough for intrauterine fetal demise, the fetus needs continuous monitoring or emergent delivery.

Immediate postnatal management prevents cascade failures

Minimizing heat loss and fluid loss is crucial immediately after birth, otherwise these babies can show up extremely dehydrated and cold 5:02. The exposed bowel in gastroschisis or the membranous sac in omphalocele creates massive insensible losses. These babies can have significant acidosis and pulmonary hypertension 5:02. The initial non-operative steps — warming, fluid resuscitation, covering the defect — must precede any discussion of closure strategy. A baby who arrives acidotic and hypothermic has already lost the physiologic reserve needed for primary closure.

Closure strategy follows bowel quality and abdominal domain

Primary closure is considered if bowel looks pristine, non-thickened, non-inflammatory, with only small amount on the outside and enough abdominal domain 5:34. The decision is made at the bedside or in the operating room based on direct inspection. Staged closure is favored if the defect is large or there's issue with the bowel 6:07. Some patients can have atresia, compromised bowel, or perforation 6:07. The risk of perforation persists even after initial management — patients can develop intestinal perforation after only a few days of enteral feeding 6:07.

Sutureless closure eliminates one operative exposure

The majority of babies at Cincinnati Children's in the last several years are being managed using a sutureless closure 6:34. 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 6:58. Skin will grow over and the umbilical defect can close spontaneously over time 6:58. This approach trades a formal fascial closure for spontaneous epithelialization — acceptable when the defect is small enough and the bowel quality permits immediate reduction. For omphalocele with no significant respiratory issues, sequential reduction using meshes is the best option 7:57. Meshes are sewn to the edges of the fascia without interrupting the membrane 7:57. At Cincinnati Children's, a 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 8:17, followed by delayed primary closure of the fascia and skin in the operating room 8:17.

Atresia complicates the timeline

For small bowel atresia, management options include tapering the dilated portion of bowel or resecting the bowel before tapering 7:29. The choice depends on how much proximal dilation exists and whether the bowel wall quality permits tapering without devascularization. Atresia discovered at initial closure forces a decision: address it immediately and accept a longer operation, or stage the atresia repair after the abdominal wall is closed. The discussants emphasized that this remains a judgment call shaped by the baby's overall stability and the surgeon's assessment of bowel viability.

Takeaways from this story

Omphalocele requires MRI and echo because 35-90% have trisomy 13, 18, or 21; gastroschisis needs only ultrasound.
Over 90% of gastroschisis infants weigh under 2,500g at birth; monthly growth tracking and non-stress testing prevent fetal demise.
Primary closure requires pristine bowel and adequate domain; staged closure is favored for large defects or compromised bowel.
Sutureless closure avoids the OR by placing umbilical cord and dressing over reduced bowel, allowing spontaneous skin closure.
Intestinal perforation can develop within days after starting feeds, even after successful initial closure.

Topic overview

Discussion of omphalocele and gastroschisis, two congenital abdominal wall defects with distinct characteristics. Gastroschisis is a full-thickness defect to the right of the umbilicus without membranous cover, occurring in otherwise normal babies from an intrauterine event, while omphalocele is a midline defect through the umbilicus with membranous cover and associated with chromosomal anomalies in 35-90% of cases. Management approaches include primary closure for small defects with pristine bowel, staged closure for large defects or compromised bowel, and sutureless closure techniques. Prenatal surveillance is critical due to risks of intrauterine growth restriction and fetal demise.

Key takeaways

Gastroschisis occurs in otherwise normal babies; omphalocele has 35-90% chromosomal anomaly association (trisomy 13/18/21). (0:55)
Immediate postnatal priority: minimize heat/fluid loss to prevent severe dehydration, acidosis, and pulmonary hypertension. (5:02)
Primary closure suits small defects with pristine bowel; staged closure for large defects or compromised/perforated bowel. (5:34)
Sutureless closure (umbilical cord + dressing) allows spontaneous skin closure without OR trip—now majority approach at Cincinnati. (6:34)
Monthly fetal growth tracking essential: gastroschisis/omphalocele risk intrauterine growth restriction and fetal demise. (1:54)

Keywords

gastroschisis omphalocele intrauterine growth restriction intrauterine fetal demise acidosis pulmonary hypertension small bowel atresia intestinal perforation trisomy 13 trisomy 18 trisomy 21 ultrasound MRI echocardiogram biophysical profile

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Transcript

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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.

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