Gastroschisis occurs in 1 in 4000 live births. An increased incidence in mothers younger than 21 years of age has been widely documented, although the reasons that led to the rising numbers remain unclear. While there was a significant increase in the prevalence of gastroschisis until 2008, there is evidence that this has decreased from 2008 to 2018 in the United States to a level noted in 1997. Preterm delivery is more frequent in infants with gastroschisis, with an incidence of 28% compared to 6% in babies without an AWD.

Embryologically, the abdominal wall forms during the fourth week of gestation when differential growth of the embryo causes infolding in the craniocaudal and mediolateral directions. During the sixth week, rapid intestinal and liver growth leads to herniation of the midgut into the umbilical cord. Elongation and rotation of the midgut occurs over the ensuing 4 weeks. By week 10, the midgut returns to the abdominal cavity, where the first, second, and third portions of the duodenum and the ascending and descending colon assume their fixed retroperitoneal positions. At the most basic level, an AWD involves an interruption of these embryologic processes and results in abnormal development. One theory suggests that gastroschisis results from failure of the mesoderm to form in the anterior abdominal wall. Currently, the ventral body folds theory, which suggests failure of migration of the lateral folds (more frequent on the right side), is most widely accepted. This implies that a gastroschisis occurs earlier in gestation, before an omphalocele might develop.

There are a variety of potential agents that have been implicated in the development of gastroschisis; however, no specific causal relationship has been established. A number of possible causative factors, including tobacco, certain environmental exposures (nitrosamines), cyclooxygenase inhibitor use (aspirin and ibuprofen), and decongestants (pseudoephedrine and phenylpropanolamine), have been suggested as etiologic agents. The well-known association of lower maternal age and low socioeconomic status with a higher incidence of gastroschisis has been linked with violence against women during gestation as a potential factor. Other factors such as change in paternity for gestations, nativity of women from Mexico, and geographic location near EPA (Environmental Protection Agency) sites have been suggested as potential causes. ^{, , ,}

Most pregnancies complicated by gastroschisis are diagnosed sonographically by 20 weeks’ gestation, with some being picked up even sooner. Routine prenatal ultrasonography (US) identifies abnormalities that are confirmed on higher-level US evaluation. Another indication for a detailed high-level US is an abnormal maternal serum α-fetoprotein (AFP) level, which is almost universally elevated in the presence of an AWD. ^, Findings that are pathognomonic of gastroschisis on prenatal US are bowel loops freely floating in the amniotic fluid and a defect in the abdominal wall to the right of a normal umbilical cord. Intrauterine growth restriction (IUGR) has been noted in a disproportionate number of these fetuses. ^, Occasionally, gastroschisis is not diagnosed prenatally and discovered at the time of delivery, which can result in challenges in neonatal management as these neonates must be transferred urgently to a center with the ability to care for them. Fortunately, these instances of previously unknown gastroschisis at delivery have become rare in the United States with improved prenatal care. Interestingly, despite recommendations to deliver at a tertiary care center, data from developed nations regarding outcomes of gastroschisis with inborn versus outborn babies does not suggest a worse outcome for either group as long as expeditious transport is available. ^,

The ideal prenatal test—in addition to diagnosing the AWD—would be able to accurately differentiate gastroschisis fetuses that are complex versus simple in nature. Despite extensive use of US in fetal gastroschisis and the identification of multiple potential predictors of an adverse outcome (such as complex gastroschisis), there is no consensus on their use in selecting fetuses for early delivery. ^{, , ,} Factors that maternal-fetal medicine experts consider important for gastroschisis are intraabdominal bowel dilation, extraabdominal bowel dilation, bowel-wall thickening, gastric dilation, severe IUGR, polyhydramnios, liver herniation, and urinary bladder herniation. ^{, ,} Two systematic reviews/meta-analyses evaluated studies on prenatal indicators and risk factors in gastroschisis, noting some of the same predictors listed earlier, with an emphasis on the intra- and extraabdominal bowel distention. ^, Both reviews concluded that, although in some cases a combination of two or more indicators may be used to identify fetuses at risk for complications, there were no completely reliable factors; gestational age, in particular, varied at the time of US. While the discrepancy in interpretation of these findings is improving with additional experience, prenatal US was still not able to accurately predict who would benefit from early delivery, and prospective studies were recommended. ^{, , ,}

Gastroschisis is associated with a variable degree of inflammatory thickening of the visceral bowel walls, which results in the characteristic appearance of “matted” intestinal loops. The reason for this inflammatory “peel” is unclear, but the presence of elevated levels of cytokines (interleukin-6 [IL-6], IL-8, tumor necrosis factor [TNF]-α) in the amniotic fluid, in addition to the effects of fetal urine, is thought to cause the abnormal collagen deposition. One report noted a significant decrease in interstitial cells of Cajal (ICCs) in gastroschisis bowel in neonates compared with controls, further implicating the role of the proinflammatory state in utero. ^, Studies in animal models have shown that the duration of amniotic fluid exposure is correlated with the degree of the inflammatory peel and subsequent intestinal dysmotility. ^{, ,} Efforts to reduce this exposure by either amniotic fluid exchange or intrauterine furosemide treatment, which induces fetal diuresis, have shown to be beneficial in animal models. These animal studies spurred early human trials with amniotic fluid exchange in France and Italy, but these were inconclusive and are not routinely being performed in humans at this time.

The optimal mode and timing of delivery for a fetus with gastroschisis continues to be controversial and a source for debate. Proponents of routine cesarean delivery (C-section) argue that the process of vaginal birth results in injury or increased risks for infection and sepsis. However, the literature suggests that both vaginal delivery and C-section are safe. A meta-analysis failed to demonstrate a benefit of C-section in fetuses with gastroschisis, and therefore recommended vaginal delivery. Delivery mode should be at the discretion of the obstetrician and the mother, with C-section reserved for other obstetric indications or fetal distress.

The timing of delivery is also controversial. Preterm delivery has been advocated to limit exposure of the bowel to the amniotic fluid and reduce damage to the pacemaker cells and nerve plexi, which may contribute to the profound dysmotility and malabsorption seen in these infants. ^, Early delivery may theoretically mitigate these effects, but the data are not convincing, especially as preterm delivery adds potential complexities for the mother and newborn. A number of single-center retrospective studies have suggested that elective preterm delivery at 34–35 weeks’ gestation was beneficial and have reported an earlier time to closure, attaining full enteral feeds and a decreased length of stay; however, these investigators also utilized a protocolized postnatal care pathway that may have been responsible for some of the improved outcomes. A 2013 Cochrane review of preterm birth for gastroschisis found that there was not enough evidence to suggest a beneficial effect. A report from the Canadian Pediatric Surgery Network (CAPSNet) noted a linear relationship between increasing gestational age and decreased bowel matting, and strongly advocated delivery at near term (defined as 37 weeks). A randomized trial from the United Kingdom found no benefit after induced early delivery, with the only trends being an improvement in length of hospitalization and earlier initiation of feeding. Two systematic reviews of preterm induced delivery versus elective term delivery versus expectant management had conflicting results, but concluded that the evidence was not enough to support routine preterm delivery. ^,

Neonates with gastroschisis can have significant evaporative water losses from the open abdominal cavity and exposed bowel. Intravenous access should be immediately obtained, and fluid resuscitation initiated as soon as possible, although not in excess. Nasogastric (NG) decompression is important to reduce further gastric and intestinal distention. Routine endotracheal intubation is not considered necessary and is reserved for respiratory distress issues. The viscera should be wrapped in warm saline-soaked gauze and placed in a central position on the abdominal wall. The neonate should be positioned on the right side to prevent kinking of the mesentery and potential bowel ischemia. Viscera are covered by a plastic wrap (similar to that used in food service coverings) or the infant is placed partially in a plastic bag (“bowel bag”) to reduce evaporative losses and improve temperature homeostasis ( Fig. 47.2 ). Although gastroschisis most often is an isolated anomaly, thorough examination of the neonate is important. Bowel atresia is the most common associated anomaly in patients with gastroschisis, with rates ranging from 7% to 28% in several series ( Table 47.2 ). A review of the literature noted rare occurrences of anomalies in the cardiac, pulmonary, nervous, musculoskeletal, and genitourinary systems, as well as chromosomal abnormalities in babies with gastroschisis. ^, As mentioned previously, evidence suggests that excess fluid resuscitation is detrimental as it results in edema, an increase in time to closure, as well as an increased risk of abdominal compartment syndrome. ^,

Image of a neonate with gastroschisis who has been transported wrapped with a bowel bag. The bag has been untied to allow inspection of the herniated bowel.

Over the course of the last three decades, realization that there was a subset of gastroschisis neonates that were at higher risk for morbidity and mortality has led to the development of risk stratification. ^{, ,} This risk was based on the presence or absence of any intestinal complication (atresia, ischemia, perforation, or development of necrotizing enterocolitis [NEC]) and is now classified as complex or simple gastroschisis (Figs. 47.3 and 47.4 ). Patients with complex defects have a higher in-hospital mortality rate, require multiple operative interventions, have prolonged hospitalization, increased rates of sepsis, and higher rates of prolonged cholestasis and need for intestinal transplantation due to intestinal failure. ^, This classification has allowed for better prenatal counseling, hospital planning, and transfers to centers with advanced capabilities for complex patients. ^{, ,} In addition, for the simple gastroschisis the degree of matting is considered to be important in prognosis, thus the creation of the “gastroschisis prognosis score.”

(A) This baby was born with gastroschisis and intestinal atresia. Note the atretic intestinal segment ( arrow ). The bowel was placed in a silo, and the baby underwent exploration and uneventful repair of the atresia at 6 weeks of age. (B) Example of a jejunal atresia associated with gastroschisis and a moderate peel. (C) Gastroschisis and a colonic atresia. The proximal dilated colon ( arrow ) resides in the surgeon’s hand. Note the massively dilated stomach ( asterisk ). A colostomy was created at the time of reduction of the intestine into the abdomen, with successful repair of the colonic atresia a few weeks later. Babies born with intestinal atresia or perforation are considered to have complex gastroschisis.

Images (B) and (C) courtesy Drs Pramod Puligandla and Jean-Martin Laberge.

This newborn presented with gastroschisis and intestinal perforation. Note the two lumens ( arrows ) in the exposed segment of intestine. This perforation was closed primarily, and the bowel was placed in a silo. The baby recovered uneventfully.

The primary goal is to expeditiously return the viscera to the abdominal cavity while minimizing the risk of damage due to intestinal injury or increased intraabdominal pressure. The two main surgical options are immediate closure/primary repair or silo assisted/delayed closure with serial reduction over a few days. In all cases, an immediate initial inspection of the bowel for obstructing bands, perforation, or atresia should be undertaken. Bands crossing the bowel loops must be divided before silo placement or primary abdominal closure to avoid subsequent intestinal obstruction. The choice of which method to undertake is dependent on the presentation of the viscera and the neonate, as well as surgeon and institutional preferences, and in some cases can be controversial. ^,

Primary Closure

Historically, immediate primary closure of gastroschisis was advocated in all cases, and when not possible, the neonate would not survive. This approach became less common with the use of the silastic silo, and eventually the preformed silo (which was easier to apply). ^, However, immediate closure was never fully supplanted and has returned in popularity for appropriate neonates, as well as in resource-limited locations where silos are not available. Primary closure is performed either at bedside (with or without intubation or general anesthesia), or in the operating room under general anesthesia. ^, Skin closure alone may be used, avoiding any increase in intraabdominal pressure, and is reported to have excellent long-term outcomes and, despite a fascial defect remaining, limited umbilical hernia formation. ^, Others have described the use of the umbilical stalk as an allograft. In these cases, care is taken to keep the umbilical stalk moist prior to the closure ( Fig. 47.5 ). A prospective randomized study comparing sutureless closure (using the umbilical cord) to sutured repair noted that the time to full feeds and length of stay was significantly longer in the sutureless group, despite no additional complications. A systematic review and meta-analysis of umbilical cord flap versus fascial closure found that flap repair was associated with equivalent or superior outcomes to fascial closure. In the past, most surgeons have excised the umbilicus during closure; however, preservation of the umbilicus has been shown to lead to an excellent cosmetic result. Therefore, now most surgeons will try to save it. It is safe to say that sutureless and bedside methods of gastroschisis[Q9] repair are safe and at least equivalent to fascial closure techniques and should be in the armamentarium of pediatric surgeons. Prosthetic fascial closure is very rare in gastroschisis; options include nonabsorbable mesh or bioprosthetic materials, such as porcine small intestinal submucosal mesh.

Bedside sutureless closure technique. (A) After sterile preparation and in the absence of general anesthesia, the bowel is being gently reduced back into the abdomen. Retained meconium was expressed from the colon to provide more abdominal domain. (B) Once the bowel is reduced, the abdominal-wall defect is “plugged” with the umbilical cord and secured in place with adhesive strips or an adhesive clear dressing. This may leave a small umbilical hernia that can likely be managed without operation.

Images courtesy Drs Pramod Puligandla and Jean-Martin Laberge.

Intraabdominal pressure measured by either the bladder or stomach pressure has been used to guide the surgeon during reduction. ^, Pressures higher than 10–15 mmHg are often associated with decreased renal and intestinal perfusion, and a silo or patch may be needed, whereas pressures above 20 mmHg may correlate with organ dysfunction and complications. ^, Similarly, an increase in central venous pressure greater than 4 mmHg has been correlated with the need for silo placement or patch closure. ^, Splanchnic perfusion pressure, the difference between mean arterial pressure and intraabdominal pressure, has also been used to guide the reduction. A splanchnic perfusion pressure less than 44 mmHg implies a decrease in intestinal blood flow. While these measures have been used and may be available at some centers, the most common approach is to note the effect of closure on ventilatory parameters such as peak inspiratory pressure (using volume control settings) or tidal volumes (when pressure control is in use), both of which are readily available and easier to interpret. In cases where these parameters are significantly altered on closure, reevaluation of the repair may be considered.

Staged Closure

In the mid-1990s, a prefabricated silo was developed with a circular spring positioned under the fascial opening, without the need for sutures or general anesthesia. This has made it possible to insert the silo in the delivery room or at the bedside ( Fig. 47.6 ). After placement, the bowel is reduced daily into the abdominal cavity as the silo is shortened by sequential ligation. It is important to continuously assess the bowel viability while in the transparent silo to ensure the bowel is receiving adequate blood flow ( Fig. 47.7 ). When the contents are entirely reduced, fascial and skin closure are performed. This process usually takes between 1 and 14 days, with the majority within 5 and 6 days, depending on the condition of the bowel and the neonate.

The use of a spring-loaded prefabricated silo is shown in these images. (A) The gastroschisis defect is seen. (B) An appropriate-sized spring-loaded silo is then placed over the eviscerated intestine. (C) The ring of the silo has been positioned under the fascial defect and attached to an overhead support to keep the bowel from torquing, which may result in intestinal ischemia. (D) Gradual reduction of the silo is performed. (E) Finally, the bowel has been completely returned to the abdominal cavity and the neonate is ready for transport to the operating room for closure of the fascia and skin.

This image depicts the development of ischemic necrosis after the bowel was placed in a silo. Care must be exercised when placing the intestines in a silo to ensure the silo is not constricting the blood supply.

Image courtesy Drs Pramod Puligandla and Jean-Martin Laberge.

Definitive closure in the operating room is similar to that for primary closure with the choices for sutureless versus skin versus fascial closure; again, prospective trials and systematic reviews would suggest that sutureless repair is associated with a longer hospital stay. ^{, ,} Residual ventral hernia rates are reported to be 60%–84% in the sutureless repair technique, the majority of which close spontaneously. ^, Closure of the skin in a transverse direction may create a “keyhole” appearance with a horizontal scar to the right of the umbilicus. Some surgeons advocate for a vertical closure to allow for a central umbilicus. A purse-string type skin closure around the umbilicus can be performed to create a scar around the umbilical stump for improved cosmesis ( Fig. 47.8 ).

This image depicts an excellent cosmetic result after gastroschisis repair in which umbilicoplasty was performed after reduction of the intestine into the abdomen. This photograph was taken prior to the development of the sutureless closure technique.

The routine use of a preformed silo has increasingly come into favor, with the theory being that avoidance of high intraabdominal pressure will avoid ischemic injury to the viscera and allow earlier extubation. ^{, ,} However, it is still possible that the bowel can become ischemic in the silo and close observation and serial examinations are important to assess for the bowel’s viability (see Fig. 47.7 ). Two reports noted a similar time to full enteral feedings but found that primary closure was associated with higher mean airway pressures, oxygen requirement, vasopressor requirement, and decreased urine output, and a systematic review of 20 studies comparing silo to primary closure noted that there was a benefit to the use of a silo. ^{, , ,} However, other reviews have suggested that the trend to use a silo instead of immediate closure may have swung too far and that the use of a silo may result in a more costly care pathway. The NETS2G study analyzed data from CAPSNet as well as British and Irish cases with GS. They noted that for simple gastroschisis, silo use was associated with 75% fewer GI complications, while in complex cases primary closure had benefits. Analysis of the American College of Surgeons National Surgical Quality Improvement Program (NSQIP-Peds) experience suggests that time to closure is important. ^, However, these studies did not necessarily stratify patients. Data from a multicenter retrospective database with 566 gastroschisis cases noted that primary closure patients were essentially equivalent to those silo patients who were reduced within 5 days, suggesting that the more severely matted cases will require a longer delayed approach and should be considered separately. A prospective randomized trial comparing silo versus immediate closure noted a significantly reduced time on the ventilator with no other differences between the groups. This study was underpowered as it did not meet accrual numbers; however, analysis suggested that the differences were not in favor of either approach. Another similar RCT was attempted using length of stay as the main outcome measure. This study was also truncated prior to the target recruitment as interim analysis noted no differences in length of stay, time to enteral feeds, or ventilator duration.

Best current evidence suggests that there is no significant difference in outcome with either approach (silo vs. immediate closure) for patients with simple gastroschisis.

A “closing or vanishing gastroschisis” is when the defect size decreases prior to delivery ( Fig. 47.9 ). As the defect gets smaller, the blood supply to the viscera progressively diminishes and can result in an atresia with associated loss of bowel. In extreme cases, the intestine outside the abdominal cavity completely disappears and results in congenital short bowel syndrome ( Fig. 47.10 ). There is no prenatal US finding that can reliably distinguish this condition. However, intraabdominal bowel distention or dilation can occur, which can help in deciding to deliver early in some patients. Although this is a rare finding, it usually results in short bowel syndrome.

Closing/vanishing gastroschisis. In this infant, the defect is “closing” around the root of the small bowel mesentery and a finger cannot be introduced into the defect. This could potentially lead to vascular compromise of a significant length of bowel with necrosis and perforation. Note the edematous mesentery.

Image courtesy Drs. Pramod Puligandla and Jean-Martin Laberge.

(A and B) These two images show different neonates with the prenatal diagnosis of gastroschisis. In each instance, the herniated intestine has died. At exploration, each patient was found to have short bowel syndrome due to very little small intestine.

When primary closure has been performed, one must have a high index of suspicion for increased intraabdominal pressure. These effects may range from perturbations in ventilation, renal function, and gastrointestinal ischemia. If an abdominal compartment syndrome is suspected, prompt laparotomy and silo placement should be performed.

Gastroschisis is often associated with abnormal intestinal motility and absorptive function, both of which gradually improve in most patients. Introduction of enteral feeding may be delayed for weeks while awaiting return of bowel function. The exact cause of the prolonged dysmotility is poorly understood but may be linked to the diminished number of ICCs in the intestine and the degree of peel. During this waiting period, continued hospitalization, nasogastric decompression, and parenteral nutrition are required. When bowel activity begins, enteral feeds are initiated and slowly advanced. Early oral stimulation is recommended to prevent loss of the sucking reflex. There is also some controversy in the use of feeding protocols in gastroschisis cases. One retrospective study noted that in patients who underwent primary repair, protocolized feeding with 20 mL/kg/day feeding advancement resulted in faster full feeds and shorter length of stay. However, another larger report from the multicenter Midwest Consortium found that protocolized feeds led to no difference in time to full feeds, total parental nutrition duration, central line use, or length of stay.

Prokinetic medications may be helpful in the postoperative period. In a rabbit model of gastroschisis, cisapride improved contractility of newborn intestine whereas erythromycin improved motility in control adult tissue only. However, a randomized controlled trial of erythromycin versus placebo found that enterally administered erythromycin did not improve time to full enteral feedings. A similar randomized trial examining the use of cisapride in postoperative neonates, most of whom had gastroschisis, showed a beneficial effect, but this drug is no longer available in the United States. Currently, there is no evidence to conclusively recommend use of prokinetics. The role of probiotics on the intestinal microbiome and function in gastroschisis was examined in a pilot randomized study with Bifidobacterium longum that altered the stool microbiome. However, it is unclear if that will lead to functional effects. ^,

Postoperative NEC has been seen in full-term infants with gastroschisis in higher-than-expected numbers (up to 18.5%), although there is a paucity of more recent data. Significant bowel loss from NEC can predispose to intestinal failure and its associated hepatic and infectious complications. On the other hand, another review noted that the clinical course of babies with gastroschisis who developed NEC often followed an uncomplicated course. ^, There are reports suggesting that infants with gastroschisis who were fed breast milk had a lower incidence of NEC than those who were fed formula. ^{, ,}

The incidence of omphalocele seen at 14–18 weeks’ gestation has been reported to be as high as 1 in 1100 fetuses, but drops to 1 in 4000–6000 at birth. As opposed to gastroschisis, the incidence and prevalence of omphalocele has remained stable in the United States. Thus, there is a considerable “hidden” mortality for a fetus with an omphalocele resulting from spontaneous loss of the fetus or termination. One review noted that requests for termination of pregnancy in omphalocele cases were as high as 83%.

The current understanding of the etiology for an omphalocele suggests that this defect is not from a failure in body-wall closure or migration. Rather, because the umbilical cord is attached to the sac, it is thought that an omphalocele develops due to a failure of the viscera to return to the abdominal cavity. Defects in the FGF, HOX, and SHH pathways are implicated in the development of omphalocele using an animal model. Multiple intraabdominal viscera including liver, bladder, stomach, ovary, and testis can also be found in the omphalocele sac. The sac itself consists of the covering layers of the umbilical cord and includes amnion, Wharton jelly, and peritoneum. The location of the defect is most often in the midabdominal or central region but may occur in the epigastric or hypogastric regions as well.

As opposed to gastroschisis, an omphalocele has a relatively high incidence of associated defects. These can range from chromosomal abnormalities—trisomy 13, 18, 21, and 45 X—to syndromes and nonsyndromic organ system anomalies (e.g., Beckwith–Weidemann, pentalogy of Cantrell), which can affect the cardiac, renal, GI, and musculoskeletal systems. ^, The severity and number of these associated issues determine the outcomes in omphalocele, allowing a classification based on whether they are “isolated” defects.

Elevation of maternal serum AFP is also present in many pregnancies complicated by omphalocele, although not as common as in gastroschisis. The diagnosis of omphalocele can be made by two-dimensional US at the time of the normal 18-week US evaluation for dates. Early first-trimester detection is with three-dimensional US.

US evaluation is very useful for the detection of associated anomalies. This is important as an isolated omphalocele has a survival rate of over 90%, but those with other defects (esp. cardiac/genetic) are much less likely to survive. ^, Prenatal US and karyotyping are able to identify about 60%–70% of the associated defects that are found postnatally. One study that reviewed associated defects in omphalocele infants noted anomalies that could potentially involve every organ system, whereas another report found only 14% of omphaloceles were truly isolated. Prenatal screening in a fetus with an omphalocele requires a detailed evaluation of the cardiac (14%–47% incidence of anomalies) and central nervous (3%–33% anomalies) systems as these data are critical in prenatal counseling conversations. ^, Therefore, developing a reliable sonographic predictor of postnatal morbidity and survival has been a priority. Unfortunately, prenatal findings of “giant” omphalocele based on size or contents have not been accurate in predicting outcomes. Ratios between the greatest omphalocele diameter compared with abdominal circumference (O/AC, or omphalocele ratio), the femur length (O/FL), and the head circumference (O/HC) have been studied, and the most useful may be the O/HC or the O/AC.

Similar to GS, route of delivery of a fetus with an omphalocele should be dictated by obstetric considerations as C-section has not been shown to be advantageous. Pregnancies are usually allowed to come to term, and spontaneous labor with vaginal delivery is preferred. However, many neonates with giant omphaloceles continue to be delivered by C-section in the United States because of the fear of fetal liver injury.

After delivery, the neonate is taken to the ICU and stabilized. The sac is kept intact and covered with a moist, warm gauze while a systematic and thorough search for associated anomalies is carried out. All neonates should undergo an echocardiographic evaluation. Renal abnormalities should be assessed by abdominal US. Neonatal hypoglycemia can be associated with Beckwith–Weidemann syndrome. Blood samples for genetic evaluation should be obtained as well.

In preparing infants with omphalocele for transport, risks arising from associated anomalies should be specifically addressed. Infants with an omphalocele do not have as severe fluid and temperature losses as those with gastroschisis, but these losses are still higher than those with an intact abdominal wall. The sac itself can be covered with saline-soaked gauze and an impervious dressing to minimize these losses. A nasogastric tube should be inserted and placed to suction.

Risk assessment in omphalocele is not as clearcut as in gastroschisis, in which there is a relatively straightforward division into simple and complex types. One categorization is based on the presence or absence of associated anomalies—isolated is one category in which there are no other abnormalities, or they are relatively minor and will have a favorable prognosis. Omphaloceles may also be classified based on where they are in relation to the abdomen—hypogastric, central, and epigastric. Cloacal exstrophy is associated with a hypogastric defect, whereas an epigastric location tends to have a higher incidence of cardiac anomalies and is associated with pentalogy of Cantrell. Finally, there is also a size-based classification that separates omphaloceles into hernias of the cord, small, medium, large, and giant defects. There are data that suggest increasing size has a direct correlation with worse outcome, but the exact definition of these sizes is lacking, which makes it challenging to compare in the literature.

Given the variability in omphalocele location, size, and severity of associated anomalies, multiple options for repair exist. In a survey, authors of reports from 1967 to 2009 discussing closure of giant omphaloceles were asked to see if they were still using the same approach or whether they had modified their techniques, and 42% no longer used the approach they favored in the original article. They concluded that there is currently no single accepted technique to treat giant omphaloceles in particular and that two methods were used the most: staged closure and delayed closure. In a systematic review of the literature for repair of a giant omphalocele, the data favored initial nonoperative scarification. The definition of a giant defect is variable as some surgeons use size alone, others consider the presence or absence of liver herniation, others use an estimate of the amount of intestinal contents, and still others have used a combination of the amount of liver and intestine in the sac as well as size. ^, This lack of an accepted definition is part of the reason for an inability to arrive at a consensus for management. The following sections will discuss surgical management based on the type of repair, which is typically based on the size as well as severity of other (mostly cardiac) anomalies.

Immediate Primary Closure

Defects that are less than 1.5 cm in diameter are referred to as a hernia of the cord and are repaired shortly after birth if there are no major associated anomalies. Larger defects may also be closed primarily if they have minimal loss of abdominal domain. Primary closure involves excision of the sac and closure of the fascia and skin over the abdominal contents. It is not unusual for an omphalomesenteric duct remnant to be associated with a small omphalocele; therefore, it should be evaluated ( Fig. 47.11 ). When dealing with a larger or medium-sized omphalocele, care must be taken when excising the portion of the sac covering the liver, because the hepatic veins may be located just under the epithelium/sac interface in the midline and can be injured. The inner portion of the sac is often adherent to the liver capsule, and significant hemorrhage can result from tears in it; therefore, it is usually best to leave the peritoneal part of the inner sac on the liver and not try to remove it. The inferior portion of the sac covering the bladder can be quite thin, and caution is needed to avoid bladder injury. In the larger defects, intraabdominal pressure can become elevated during reduction and repair, leading to abdominal compartment syndrome, and the surgeon should be aware of this potential; communication with the anesthesia team and possibly direct measurement of pressures as discussed in gastroschisis repair should be obtained.

(A and B) In patients with small omphaloceles, it is not unusual for an omphalomesenteric duct remnant to be found. (C) The diverticulum was excised primarily, and the fascia and skin closed. This neonate recovered uneventfully.

There are a few reports of primary closure of even a giant omphalocele shortly after birth with good outcomes. In a report from London, 12 of 24 babies with a large defect had an immediate repair without any mortality. Compared with other similar infants, these patients had a shorter ventilator requirement and time to full feeding. However, these data should be interpreted with caution as the cases were carefully selected, and other data notes there is no difference in cost of care in staged versus immediate repair of giant defects.

Staged Neonatal Closure

In many cases, the large loss of domain in the peritoneal cavity prevents a single stage primary closure ( Fig. 47.12 ). Multiple methods have been proposed to obtain primary abdominal-wall closure in these babies.

This neonate was born with a large omphalocele. As is evident, the abdominal cavity is quite small. Primary closure is not possible in such a patient.

Staged closure in the neonatal period involves the use of different techniques. These can be classified into methods that utilize the amnionic sac with serial inversion and those in which the sac is excised and replaced with mesh and then closed over time. Amnion inversion allows gradual reduction of the sac followed by sac excision and primary or mesh closure. However, with the development of multiple biologic mesh options, this technique has mostly fallen out of favor with only a few series reporting its use. ^, Methods involving primary repair with any mesh require removal of the amnionic sac, with the mesh used to bridge the fascial gap and overlying skin closure. Repeat procedures to excise central portions of the mesh allow for gradual increase of abdominal domain and native fascial closure, or the mesh may simply be left in situ with skin over it. Multiple reports advocate for the use of biologic mesh that allows vascular and tissue ingrowth, as well as being potentially more resilient in the setting of an infection. Utilizing vacuum-assisted closure has also been described as has a novel external skin closure system, as well as the use of intramuscular botulinum toxin. ^, A recently described option is the construction of a bedside silo with an adhesive hydrocolloid dressing (Duoderm), and subsequent sequential reduction. In a series from South America of 40 infants with giant omphalocele (defined as an abdominal-wall defect greater than 5 cm in diameter and/or that contains more than 50% of the liver within the sac), the average time to closure was slightly more than 2 weeks, with excellent results overall.

Understanding the different choices are critical in dealing with giant defects as multiple options may be needed in the same case.

Escharification (“Paint and Wait”)

Nonoperative techniques have in common the use of an agent that allows an eschar to develop over the intact amnion sac. This eschar epithelializes over time, leaving a ventral hernia that will likely require repair later in life. This approach is employed when the surgeon considers the defect too large to allow for a safe primary repair, or if the neonate has significant cardiac or respiratory issues. This is not a new concept, having evolved from the time of Dr. Robert Gross, who described using skin flaps in 1948. ^, The primary concern in a baby with a large omphalocele is that an attempt at initial repair will result in potential life-threatening abdominal compartment syndrome or the inability to provide skin coverage. Initial reports described mercurochrome, alcohol, and silver nitrate as the eschar-producing agents that were very effective but were associated with toxicity ( Fig. 47.13 ). ^, Subsequently there have been reports of a number of substances, including silver sulfadiazine, povidone-iodine solution, silver-impregnated dressings, neomycin, and polymyxin/bacitracin ointments. ^, A randomized study from Africa compared povidone-iodine and Acacia nilotica, a naturally occurring plant substance, for escharification, and noted that there was a trend to earlier oral feeding and discharge with Acacia. Manuka honey use also has been described in a report from Africa, indicating that a vast number of substances may be used with local adaptation. The eschar and epithelialization may take 4–10 weeks, and the method often will include the use of compression dressings to facilitate abdominal domain. In some cases, operative repair may not be needed as the defect contracts and gradually closes, similar to an umbilical hernia, but most will require closure of the ventral hernia between 1 and 5 years of age ( Fig. 47.14 ).

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