- What Is Gastroschisis?
Gastroschisis is an abdominal wall defect that occurs in approximately 1 in 5,000 live births. This type of birth defect occurs very early in pregnancy and is characterized by a hole in the baby’s abdominal wall (Omphalocele).
In addition to the small abdominal wall defect, some of the baby’s intestines and other abdominal organs may protrude through the opening and spill out into the amniotic fluid around the fetus. The unprotected intestine then becomes irritated, causing it to swell and shorten.
If the hole is large enough, other organs such as the stomach and liver may be exposed and become affected as well.
The longer the exposure to amniotic fluid, the more opportunity for the abdominal organs to become damaged. As your baby continues to develop, the tight opening in the abdominal wall may also squeeze the blood supply to the intestine or cause it to twist around itself.
Intestinal blockage, loss or malfunction can occur and lead to long-term feeding and digestion problems for the baby.
In contrast to other birth defects affecting the abdominal organs, such as omphalocele, gastroschisis-associated abnormalities are confined to the GI tract and are not associated with chromosomal abnormalities.
Even when other abnormalities are present, they are not usually life-threatening. There is, however, an increased risk for intrauterine growth restriction (IUGR) and stillbirth in approximately 10 percent of cases.
In addition to the potential for damage to the exposed organs, babies with this condition may experience poor fetal growth and infants may experience problems with feeding, digestion and absorption of nutrients.
We understand the prospect of having a child with gastroschisis can be scary. But the good news is that most babies born with this defect achieve positive outcomes.
At Fetal Care Center Dallas, our multidisciplinary team of physicians works with your family to diagnose gastroschisis early and develop an appropriate plan for treatment after birth.
We also provide comprehensive counseling and guidance for parents whose unborn baby is diagnosed with this anomaly. Our physicians have years of experience in the diagnosis and treatment of this relatively uncommon condition.
- Prenatal Diagnosis of Gastroschisis
Gastroschisis is almost always located immediately to the right of the insertion of the umbilical cord. It may be detected after a routine blood test shows abnormally high alpha fetoprotein levels in the mother’s blood.
A high-definition level II ultrasound performed in the second trimester of pregnancy will most likely confirm the gastroschisis diagnosis and assess the impact on the baby’s intestine. If gastroschisis is not diagnosed before birth, it will be evident upon delivery.
- How We Treat Gastroschisis
Surgery after birth is the treatment of choice for babies with gastroschisis. Soon after the birth of your baby, a pediatric surgeon may perform a surgical repair procedure to place the organs back inside your baby’s belly and close the opening in the abdominal wall.
The extent of surgery required depends on how much of your baby’s intestines or other organs lie outside of his or her body at birth and if there is an associated blockage in the intestine.
More commonly, and if the opening is large and multiple organs are involved, the surgeon may cover them with a sterile dressing and perform the repair as a series of steps during which each organ will be returned to the abdomen one by one over a period of time.
Babies with severe gastroschisis often need additional treatment during this process, including:
- Intravenous nutritional supplements
- Antibiotics to prevent infection
- Close monitoring of body temperature
IV nutritional support is especially important during the treatment process as it often takes time for the GI tract to function well enough for your baby to be sustained through feedings alone.
Our staff will help prepare your family to care for your baby at home throughout this process.
- Long-Term Outcome for Babies with Gastroschisis
Modern surgical techniques allow most babies diagnosed with gastroschisis to live healthy lives. The level of ongoing care your baby may need varies depending on the severity of the defect and any associated symptoms.
The staff at Fetal Care Center Dallas will closely follow the condition of the intestine and your baby’s growth over time to promote optimal healing and recovery.
For Healthcare Providers
- Gastroschisis: Introduction
Gastroschisis is a congenital abdominal wall defect that is characterized by a full thickness defect to the right of the umbilical cord. The defect is present as early as the 6th week of gestation. There are several theories in regards to what causes gastroschisis. Duhamel (1963) suggested a discrete teratogenic insult to the somatopleural mesenchyme resulting in an isolated defect in differentiation. Another theory is that the physiologic hernia of the cord ruptures in utero, before closure of the umbilical ring (Shaw, 1975 and Glick et al. 1985). Others have suggested that in-utero regression of the right umbilical vein leaves a weakness on right side of the abdominal cord insertion, or that there is a disruption of the right omphalomesenteric artery again leading to a weakness predisposing to what we clinically recognize as gastroschisis (Hoyme et al., 1983 and Torfs et al., 1990).
At birth, the bowel may have a typical inflammatory matted abnormal appearance which is referred to as an intestinal “peel”. The peel is a layer of fibrin and collagen on the serosal surface of the bowel, which likely is caused by the combination of inflammatory reaction to constituents in the amniotic fluid and constriction of the bowel as it herniates through the abdominal wall defect. In addition, the bowel in fetuses with gastroschisis is often foreshortened (Amoury et al., 1977; Klein et al., 1983; Tibboel et al., 1986 a, b; Amoury et al., 1988; Langer et al., 1990, Moore, 1992).
Most cases of gastroschisis are detected prenatally. Ultrasound will often detect an abdominal wall defect at the time of the “dating” ultrasound, which is usually done around 20 weeks of gestation. Occasionally the abdominal wall defect is seen before 20 weeks gestation, if an ultrasound is obtained late in the first trimester. In the second trimester, maternal serum alpha-fetoprotein (MSAFP) screening will be elevated in most mothers pregnant with a fetus with gastroschisis, but the test is not specific for gastroschisis. MSAFP is also elevated in twin pregnancies, in neural tube defects, in omphalocele and in autosomal chromosomal anomalies.
- Incidence of Gastroschisis
The number of babies born with gastroschisis has increased over the last 2 decades. The reason for the increase is not known. Epidemiologic data have shown that young maternal age is associated with an increased risk of gastroschisis. Goldbaum et al (1990) studied infants with gastroschisis in the state of Washington and found a 4 fold increased risk in mothers less than 20 years of age.
Cigarette smoking has been associated with gastroschisis (Haddow et al., 1993). Medication and recreational drugs that can cause vascular constriction has been linked to an increased risk of gastroschisis (Van Allen, 1981; Colado et al., 1997; Plessinger, 1998)
- Sonographic Findings
It is difficult to diagnose gastroschisis in the first trimester because of the normal herniation into the umbilical cord (Cyr et al., 1986). The intestine returns to the abdomen by 11 weeks gestation. The earliest reported diagnosis is 13 weeks 3 days gestation (Guzman, 1990).
Ultrasound will typically demonstrate a full thickness abdominal wall defect, almost always, to the right of the umbilical cord. There is bowel floating freely in the amniotic fluid without a limiting membrane as seen in omphalocele. In omphalocele the bowel is contained within the omphalocele sac, which is comprised of the parietal peritoneum and the amnion with hyaluronic acid in between. Omphalocele can be confused with gastroschisis if the omphalocele sac ruptures which is a relatively rare event. The prenatal detection rate for gastroschisis is over 80% (Barisic et al., 2001). Gastroschisis and omphalocele were shown to be accurately distinguished in 79.3% of cases on initial diagnosis and in 84.5% of cases after referral for further evaluation (Walkinshaw et al., 1992).
A ruptured hernia of the cord can present late as gastroschisis, in these cases the gastroschisis may develop later in pregnancy and may not be present on ultrasound early in pregnancy (Knott and Colley, 1987).
- Differential Diagnosis
The differential diagnosis of gastroschisis should include omphalocele, ruptured omphalocele, hernia of the cord and limb-body wall complex. Omphalocele and gastroschisis are differentiated by the presence of a sac in omphalocele with cord insertion on the sac. In gastroschisis, the umbilical cord inserts into the abdominal wall with the abdominal wall defect immediately to the right of it’s insertion. In gastroschisis there is no sac and the intestine is floating freely in the amniotic cavity. In addition, the abdominal wall defect is small usually less than 4 cm in diameter evan at term. In contrast, the abdominal wall defect in omphalocele is quite large often with herniated liver and stomach which is not the case with gastroshcisis. Ruptured omphalocele may be mistaken for gastroschisis, however if the abdominal wall defect is sufficiently large that it allows the liver to herniate through the abdominal wall defect it is more likely to be omphalocele and not gastroschisis. Ruptured hernia of the cord will present later in pregnancy and may be impossible to differentiate from gastroschisis unless a prior ultrasound showed a hernia of the cord without an abdominal wall defect. Limb body wall complex is characterized by very severe limb defects and anterior wall defects, these can be of head, chest or abdomen usually not in the midline and with spinal abnormalities which together are rarely mistaken for gastroschisis.
- Prenatal Natural History
Chromosomal anomalies are rare in gastroschisis (Mayer et al., 1980; Mann and Ferguson-Smith, 1984; Sermer et al., 1987; Romero et al., 1988; Lewinsky et al., 1990; Sipes et al., 1990). Only in cases where sonographic abnormalities in addition to gastrointestinal abnormalities are seen, is chromosomal evaluation recommended.
In a study of 24 international birth defects registries including over 3300 cases of gastroschisis 10% were found to have associated major unrelated defects, with 2% part of a recongnized sundrome and cardiac abnormalities detected in 2-3% (Mastroiacovo et al 2007. A report from the Texas Birth Defects Registry found up to a third of cases of gastroschisis had associated anomalies (Benjamin et al 2014). However, in a prospectively collected database of 4700 infants with gastroschisis discharged from 348 neonatal intensive care units in North America found associated anomalies in only 8% of cases and cardiac defects in 1% (Corey et al 2014).
Gastroschisis has been classified into simple and complex has on the presence or absence of atresia, perforation, volvulus, and necrosis. Between 10 and 25% of cases of gastroschisis will be complex. The focus of prenatal diagnosis has been on trying to distinguish simple from complex gastroschisis in order to better understand the prenatal natural history of gastroschisis and better guide prenatal management that might influence the rate of intrauterine fetal demise, fetal distress, and prematurity. Simple gastroschisis has a survival of over 98% and low morbidity rates (Bradnoch et al 2011, Gamba et al 2014), but complex gastroschisis is associated with survival rates of 70 to 80% with prolonged hospital stay and higher long-term morbidity rates (Bradnoch et al 2011, Cowan et al 2012).
In utero bowel dilatation is one of the most commonly noted abnormalities on fetal ultrasound. Its significance for outcome has not been established. A large study from Canada including a 100 patients diagnosed prenatally with gastroschisis did not find that prenatal bowel dilatation over 18 mm was associated with a worse outcome (Skarsgaard et al., 2007). Piper and Jaksic (2006) reviewed the experience at Boston Children’s Hospital and found no difference in length of stay, time on TPN, mortality, or time in the NICU for babies who had bowel dilatation over 6 mm prenatally.
Bowel dilatation and bowel thickening may indicate bowel damage, but the existing data does not provide evidence for changing the delivery time or mode if these features are present on ultrasound. In a more recent meta-analysis by D’Antonio et al of 2023 fetuses with gastroschisis found a positive association between intra-abmdominal bowel dilation, polyhydramnios and bowel atresia. In addition, prenatal gastric dilation was found to be associated with increased risk of neonatal death (D’Antonio et al 2018)
Intra uterine growth retardation (IUGR) is common and may affect as many as 77% of babies (Carpenter et al., 1984; Molenaar and Tibboel, 1993). A study by Royner and Richards (1977) found a large difference between predicted IUGR (43%) and actual IUGR at birth (23%). The weight of the fetus is often under estimated because the abdominal circumference is taken into account. In babies with gastroschisis the abdominal circumference is small because most of the bowel is outside the abdomen.
A few studies (Crawford et al.,1992; Burge and Ade-Ajayi, 1997) have reported an up to 10% rate of stillbirth in the third trimester in babies with gastroschisis. This is now thought to be an overestimate of the rate of intrauterine fetal demise in gastroschisis. The cause of death is thought to be mid-gut volvulus or cord compression. More recently, the CAPSNET data from Canada reported a fetal loss rate of only 1.4% among 700 cases of gastroschisis (www.CAPSNET.org).
Premature birth is common in pregnancies with gastroschisis. At least one third of babies are born prematurely, possibly the most important reason for premature labor is polyhydramnios (Mayer et al., 1980; Kirk and Wah, 1983; Carpenter et al., 1984; Caplan and McGregor, 1989; Molenaar and Tibboel, 1993). Also oligohydramnios is seen in gastroschisis (Bair et al., 1986; Crawford et al., 1992). Mercer et al. (1988) reported amniotic fluid staining in 73% of their series of 22 babies with gastroschisis. The significance of this is unclear but it may indicate fetal distress.
- Management of Pregnancy
Most stillbirths in pregnancies with gastroschisis occur late in the third trimester. Crawford et al. (1992) recommended weekly biophysical profile testing starting at 30-32 weeks gestation because of the association with intrauterine growth restriction and weekly ultrasounds to assess the bowel wall thickness, intra-and extra-abdominal bowel dilation and amniotic fluid volume. Sonographic changes to the bowel can be difficult to interpret but this can be a sign of a temporary change or development of atresia or necrosis. Since prematurity is associated with multiple problems, it is recommended to prolong pregnancy until the fetus is as close to term as possible. Most pregnancies are electively induced at 37 weeks gestation if spontaneous delivery has not taken place yet.
Gastroschisis is not an indication for cesarean section (C-section) as outcomes are similar for babies delivered via vaginal birth (Boutros et al., 2009). In a contemporary study from Canada 62% of babies with gastroschisis were delivered vaginally (Boutros et al., 2009). Studies from New Zealand (Abdel-Latif et al., 2008) and from Missouri (Snyder and StPeter, 2005) show a trend towards an increase in cesarean sections in pregnancies with gastroschisis. Since there is no evidence that cesarean sections improve outcomes in neonates with gastroschisis it is unclear why there is a trend towards more cesarean sections.
The approach at the Fetal Care Center of Dallas is to perform twice weekly antenatal testing and weekly ultrasound to monitor the amniotic fluid volume and Doppler velocimetry. The average gestational age at delivery with expectant management is 37 weeks’ (Baud et al 2013). If fetal growth, amniotic fluid volume, and antenatal testing are normal, the risk of intrauterine fetal demise is minimal and delivery prior to 37 weeks is hard to justify. It should be noted however, that fetuses that meet these criteria are in the minority. In general, we schedule patients for induction of labor at 37 weeks’ if they haven’t already delivered. There is no evidence that cesarean section delivery improves outcomes in uncomplicated gastroschisis and should be reserved for obstetrical indications (How et al 2000, Salihu et al 2004, Pugligandla et al 2004)
- Fetal Intervention
There is currently no fetal treatment that will improve outcomes in gastroschisis. Amnioexchange has been proposed as a possible fetal treatment for gastroschisis. The rationale is that there are two mechanisms that cause bowel damage: constriction of the abdominal wall defect with resultant hypo-perfusion of the bowel and various irritants in the amniotic fluid (Langer et al., 1989, 1990). In France Luton et al. (2003) performed a trial of amnioexchange, there was no significant improvement in outcomes with this intervention.
- Newborn Management
Immediately after delivery, the newborn with gastroschisis is placed in a plastic “bowel” bag. This is done to reduce evaporative fluid and heat losses and to help keep the baby warm. The baby should be placed on the right side and the bowel should be supported to prevent kinking of the mesenteric vessels.
An orogastric or nasogastric tube is placed into the stomach to decompress it. Intravenous (IV) access is important and necessary immediately after birth for the administration of crystalloid intravenous fluids and antibiotics. Newborn babies with gastroschisis have large ongoing fluid losses and will often need in excess of 10-30 ml/kg in IV fluid boluses.
A surgeon will soon after birth assess the bowel for any abnormalities and decide on the next steps in the surgical treatment.
- Surgical Treatment
Either a peripherally inserted central venous catheter (PICC) is placed in the NICU prior to surgery or a tunneled central venous catheter (broviac) is placed by the surgeon. The central line is critical for the newborn with gastroschisis, it allows for administration of IV nutrition and medications.
There are essentially three approaches to closing the gastroschisis abdominal wall defect. The surgeon’s preference, degree of viscero-abdominal disproportion the appearance of the bowel and how the newborn is doing clinically are some of the factors that will determine the surgical treatment.
Primary closure can be achieved in significant percentage of patients with gastroschisis. It is important that the baby is doing well and that the bowel is not very dilated and there is minimal viscero-abdominal disproportion to be able to close the defect primarily. This can be accomplished at the bedside, without intubation, general anesthesia and with minimal sedation (Bianchi et al 1998) using the umbilical cord as a flap in a suture-less closure (Sandler et al 2004).
This technique also requires a clinically well neonate and that the bowel is not very dilated, the difference is that the bowel is reduced and the defect covered with the umbilical stump and a Duoderm dressing. The sutureless closure avoids an operation, but it may increase the risk for an umbilical hernia which will require closure later in life.
If reduction of the bowel with primary flap closure is not possible without causing respiratory compromise due to pressure of the diaphragm then placement of a performed silo can be performed at the bedside. This will allow elevated of the bowel which facilitates the resolution of bowel wall edema and allows reduction of the loops into the abdominal cavity over time. This reduction can be encouraged by manual reduction of the loops of bowel tying umbilical tape around the silo to prevent re-herniation. This reduction can be repeated daily or twice daily as needed until all the bowel is reduced. The abdominal wall defect can then be either closed operatively or an umbilical cord flap can be used. The silo tends to stretch the fascial defect which may limit ability to do an umbilical cord flap in some cases.
The most common approach and the safest approach for babies who are in distress, has dilated bowel, or has complex gastroschisis is to place the bowel in a preformed silo bag and then sequentially reduce it. When all of the bowel has been reduced into the abdominal cavity, the fascia is closed with sutures or closed with a Duoderm dressing as described above.
Even in an infant with gastroschisis is known to have an associated bowel atresia the primary goal in gastroschisis is closure of the abdominal wall defect. Any attempt at resection of the bowel atresia at the time of delivery should be avoided as the bowel wall is edematous and inflamed and resection and primary anastomosis will result in anastomotic leak and sepsis. Once the bowel has been reduced into the abdomen the inflammatory peel begins to regress and by two weeks post-operatively the bowel is back to normal. At this point the atresia can be safely resected and a primary anastomosis safely performed.
- Postnatal Management
Postnatally the most common and problema affecting babies with gastroschisis is intestinal dysmotility and inability to absorb nutrients. In the first few weeks of life, all babies with gastroschisis will require total parenteral nutrition (TPN) as they slowly adjust to enteral feeds. During this time a nasogastric (or orogastric) tube will drain the secretions from the stomach until the baby has bowel function. The mean time to first enteral feed was 16 days in a Canadian study (Boutros et al., 2009) and the median time to full enteral feeds in babies with simple gastroschisis was 24 days and 81 days in complex gastroschisis in a cohort study from England and Ireland (Bradnock et al., 2011).
Bradnock et al. (2011) identified 11% with complex gastroschisis: atresia, necrosis or bowel perforation in their cohort study. Emil et al. (2011) identified 23% with complex gastroschisis in a smaller cohort. Boutros et al., found that 22% of the patients in the Canadian study required multiple operations, likely related to complex gastroschisis. This group of patients with gastroschisis has a significantly longer NICU stay, ranging from 84 days (Bradnock et al., 2011) to 104 days (Emil et al., 2011) and will more likely develop short bowel syndrome and be at risk for TPN induced liver failure.
- Associated Conditions
Intestinal failure may result from gastroschisis. It can either be secondary to short bowel syndrome as a result of loss of bowel length, or it can be a result of severe dysmotility and chronic intestinal pseudo-obstruction. Both conditions will require long-term TPN dependency, which can result in liver failure, sepsis and may require bowel and liver transplantation. Fortunately it is rare that patients born with gastroschisis end up needing bowel and liver transplantation.
Pneumatosis intestinalis is seen in up to 10% of infants with gastroschisis. Pneumatosis is a sign of necrotizing enterocolitis (NEC) in newborn babies, but in gastroschisis medical NEC is more common than surgical NEC which requires laparotomy to address perforation or medically refractory systemic illness. Medical NEC is managed with bowel rest and IV antibiotics for7-10 days until pneumatosis resolves.
Cryptorchidism is common in baby boys with gastroschisis, about a third of baby boys with gastroschisis will have cryptorchidism at birth and a third of these babies will need to undergo orchidopexy (Hill and Durham, 2011). Hernias, both inguinal and incisional are common in infants with gastroschisis which may not present for several months after discharge from the NICU.
Infants born with gastroschisis are often small typically < 5% for body weight at delivery and their small size tends to persists through the end of the first year of life. Most children will begin catching up in somatic growth after their first year of life.
- Long-Term Outcome
The duration of hospitalization is directly related to the degree of gastrointestinal compromise or presence of gastrointestinal atresia. Approximately 10% of patients with gastroschisis will have hypoperistalsis syndrome. These infants remain dependent on parenteral nutrition for an indefinite period, sometimes permanently. The average hospital stay following primary closure of gastroschisis is usually on the order of 7 to 14 days. Often feeding difficulties after repair of gastroschisis will delay discharge because of the need for gavage feedings. Although primary closure may be achieved and infants are weaned from mechanical ventilatory support, they often remain quite tachypneic, which impairs their ability to suckle. Once further abdominal-wall relaxation and accommodation has had time to occur, there is less tension and pressure on the diaphragm and the respiratory rate decreases. Once a respiratory rate of less than 60 breaths per minute is achieved, infants can suckle effectively and be weaned from supplemental gavage feeding.
Hospitalization for infants with gastroschisis requiring staged closure is much longer, related to the need for gradual reduction, greater visceroperitoneal disproportion, and a second procedure to achieve fascial closure. The hospitalization in these infants may be prolonged by several weeks.
Inguinal hernias will develop in most infants with gastroschisis because of increased intraabdominal pressure. Occasionally, incisional hernias seen as bulging from attenuated fascia at the closure site will require remedial surgery months or years later. There are no long-term sequelae from gastroschisis if there is no associated hypoperistalsis syndrome.
- Genetics and Recurrence Risk
Gastroschisis has been generally considered a sporadic event, with a multifactorial cause, but there have been reports of familial recurrence (Hershey et al. 1989; Lowry and Baird 1982; Salinar et al. 1979). Torfs et al. (1991) described a 4.3% sibling recurrence rate in a pop-ulation-based study. A 4.3% recurrence risk implies a mixture of genetic predisposition with environmental factors. In 1 study, Torfs and Curry (1993) found only 6 published reports of familial occurrence of gastroschisis. A single-gene defect is unlikely for this condition. Families should receive genetic counseling regarding recurrence risk. They should be offered MSAFP testing and prenatal sonography in future pregnancies.
Amoury RA, Beatty EC, Wood WI, et al. Histology of the intestine in human gastroschisis relationship to intestinal malfunction: dissolution of the “peel” and its ultrastructural characteristics. J Pediatr Surg 1988;23:950–956.
Amoury RA, Holder TM. Gastroschisis complicated by intestinal atresia. Surgery 1977;82:373–381.
Bair JH, Russ PD, Pretorius DH. Fetal omphalocele and gastroschisis: a review of 24 cases. Am J Radiol 1986;147: 1047–1051.
Baird PA, MacDonald EC. An epidemiologic study of congenital malformations of the anterior abdominal wall in more than half a million consecutive live births. Am J Hum Genet 1981;33:470–478.
Bianchi A, Dickson AP: Elective delayed reduction and no anesthesia: “minimal intervention management” for gastroschisis. J Pediatr Surg 1998; 33: 1338-1340
Bond SJ, Harrison MR, Filly RA, et al. Severity of intestinal damage in gastroschisis: correlation with prenatal sonographic findings. J Pediatr Surg 1988;23:520–525.
Baud D, Lausman A, Alfaraj MA, et al. Expectant management compared with elective delivery at 37 weeks for gastroschisis. Obstet Gynecol 2013; 121:990
Bowerman R, Aulla N, Ginsberg H. High resolution sonographic identification of fetal midgut herniation into the umbilical cord: differentiation from fetal anterior abdominal wall defects. J Ultrasound in Med 1988;109(suppl):7.
Bradnock TJ, Marven S, Owen A et al: BAPS-CASS. Gastroschisis: one year outcomes from national cohort study. BMJ 2011; 343; d6749
Brock DJ, Barron L, Duncan P, et al. Significance of elevated mid-trimester maternal plasma AFP values. Lancet 1979;1:1281.
Bryant MS, Tepas JJ, Mollitt DL, et al. The effect of initial operative repair on the recovery of intestinal function in gastroschisis. Am Surg 1985;55:210
Calzolari E, Volpato S, Bianchi F, et al. Omphalocele and gastroschisis: a collaborative study of five Italian congenital malformation registries. Teratology 1993;47:47–55.
Caniano DA, Brokaw B, Ginn-Pease ME. An individualized approach to the management of gastroschisis. J Pediatr Surg 1990;25:287–300.
Caplan MS, MacGregor SN. Perinatal management of congenital diaphragmatic hernia and anterior abdominal wall defects. Clin Perinatol 1989;16:917.
Carlan SJ, Knuppel RA, Perez J, et al. Antenatal fetal diagnosis and maternal transport gastroschisis. Clin Pediatr 1990;29:378.
Carpenter MW, Curci MR, Dibbins AW, et al. Perinatal management of ventral wall defects. Obstet Gynecol 1984;64: 646.
Colombani PM, Cunningham MD. Perinatal aspects of omphalocele and gastroschisis. Am J Dis Child 1977;131:1386.
Colado ML, O’Shea E, Granados R, et al: A study of the neurotoxic effect of MDMA (‘ecstasy’) on 5-HT neurones in the brains of mothers and neonates following administration of the drug during pregnancy. Br J Pharmacol 1997;121:827–833
Coughlin JP, Drucker DE, Jewell MR et al. Delivery room repair of gastroschisis. Surgery 1993;114:822–827.
Cowan KN, Puligandla PS, Laberge JM et al: Canadian Pediatric Surgical Network. The gastroschisis prognostic score: reliable outcome prediction in gastroschisis. 2012; 47(6) 1111-1117
Crandall BF, Robinson L, Grau P. Risks associated with an elevated maternal serum alpha-fetoprotein level. Am J Obstet Gynecol 1991;165:581–586.
Crawford RAF, Ryan G, Wright VM, et al. The importance of serial biophysical assessment of fetal well being in gastroschisis. Br J Obstet Gynaecol 1992;99:899–902.
Cyr DR, Mack LA, Schoenecker SA, et al. Bowel migration in the normal fetus: US detection. Radiology 1986;161: 119–121.
D’Antonio F, Virgone C, Rizzo G et al: Prenatal risk factors and outcomes in gastroschisis: A meta-analysis. Pediatrics 2018 136(1) 159-169
DeLorenzo M, Yazbeck S, Ducharme JC. Gastroschisis: a 15-year experience. J Pediatr Surg 1987;22:710–712.
deVries PA. The pathogenesis of gastroschisis and omphalocele. J Pediatr Surg 1980;15:245.
Duhamel B. Embryology of exomphalos and allied malformations. Arch Dis Child 1963;38:142.
Egenaes J, Bjerkedal T. Forekomst av gastroschisis og omfalocele i Norge 1967–1979. Tidsskr Nor Laegeforen 1982;102:172–176.
Ein SH, Rubin SZ. Gastroschisis: primary closure or silo pouch. J Pediatr Surg 1980;15:549.
Ein SH, Superina R, Bagwell C, et al. Ischemic bowel after primary closure for gastroschisis. J Pediatr Surg 1988;23: 728–730.
Emil S, Canvasser N, Chen T et al: Contemporary 2-year outcomes of complex gastroschisis. J Pediatr Surg 2012 47(8): 1521-1528
Ewigman BG, Crane JP, RADIUS Study Group. Effect of prenatal ultrasound screening on perinatal outcome. N Engl J Med 1993;329:821–827.
Fonkalsrud EW. Selective repair of neonatal gastroschisis based on degree of visceroabdominal disproportion. Surgery 1980;139:138.
Gamba P, Midrio P:Abdominal wall defects: prenatal diagnosis, newborn management and long-term outcomes. Semin Pediatr Surg 2014;23(5) 283-290
Glick PL, Harrison MR, Adzick NS, et al. The missing link in the pathogenesis of gastroschisis. Pediatr Surg 1985;20: 406–409.
Goldbaum G, Daling J, Milham S. Risk factors for gastroschisis. Teratology 1990;42:397–403.
Goldfine C, Haddow JE, Knight GJ, et al. Amniotic fluid alpha-fetoprotein and acetylcholinesterase measurements in pregnancies associated with gastroschisis. Prenat Diag 1989;8:697–700.
Gornall P. Management of intestinal atresia complicating gastroschisis. J Pediatr Surg 1989;24:522–525.
Green JJ, Hobbins JC. Abdominal ultrasound examination of the first-trimester fetus. Am J Obstet Gynecol 1988;159: 165–175.
Gutenberger JE, Miller DL, Dibbins AW, et al. Hypogammaglobulinemia and hypoalbuminemia in neonates with ruptured omphaloceles and gastroschisis. J Pediatr Surg 1973;8:353–359.
Guzman ER. Early prenatal diagnosis of gastroschisis with transvaginal ultrasonography. Am J Obstet Gynecol 1990;162:1253–1254.
Haddow JE, Palomaki GE, Holman MS. Young maternal age and smoking during pregnancy as risk factors for gastroschisis. Teratology 1993;47:225–228.
Hemmenki K, Saloniemi I, Kyronen P, et al. Gastroschisis and omphalocele in Finland in the 1970’s: prevalence at birth and its correlates. J Epidemiol Commun Health 1982;36:289–293.
Hershey DW, Haesslein HC, Marr CC, et al. Familial abdominal wall defects. Am J Med Genet 1989;34:174.
Hill LM, Breckle R, Gehrking WC. Prenatal detection of congenital malformations by ultrasonography. Am J Obstet Gynecol 1985;151:44–50.
Hoyme HE, Jones MC, Jones KL. Gastroschisis: abdominal wall disruption secondary to early gestational interruption of the omphalomesenteric artery. Semin Perinatol 1983;7: 294–298.
Killam WP, Miller RC, Seeds JW. Extremely high maternal serum alpha-fetoprotein levels at second-trimester screening. Obstet Gynecol 1991;78:257.
King DR, Savrin R, Boles ET. Gastroschisis update. J Pediatr Surg 1980;15:553.
Kirk EP, Wah RH. Obstetric management of the fetus with omphalocele or gastroschisis: a review and report of 112 cases. Am J Obstet Gynecol 1983;146:512.
Klein M, Kluck P, Tibboel D, et al. The effect of fetal urine on the development of the bowel in gastroschisis. J Pediatr Surg 1983;18:47.
Knott PD, Colley NV. Can fetal gastroschisis always be diagnosed prenatally? Prenat Diagn 1987;7:607–610.
Langer JC, Longaker MT, Crombleholme TM, et al. Etiology of intestinal damage in gastroschisis. I. Effects of amniotic fluid exposure and bowel constriction in a fetal lamb model. J Pediatr Surg 1989;24:992–997.
Langer JC, Bell JG, Castillo RO, et al. Etiology of intestinal damage in gastroschisis. II. Timing and reversibility of histological changes, mucosal function, and contractility. J Pediatr Surg 1990;25:1122–1126.
Langer JC, Khanna J, Caco C, et al. Prenatal diagnosis of gastroschisis: development of objective sonographic criteria for predicting outcome. Obstet Gynecol 1993;81:53–56.
Larson JM, Pretorius DH, Budorick NE, et al. Value of maternal serum alpha-fetoprotein levels of 5.0 MoM or greater and prenatal sonography in predicting fetal outcome. Radiology 1993;189:77–81.
Lenke RR, Hatch EI. Fetal gastroschisis: a preliminary report advocating the use of cesarean section. Obstet Gynecol 1986;67:395.
Lenke RR, Persutte WH, Nemes J. Ultrasonographic assessment of intestinal damage in fetuses with gastroschisis: is it of clinical value? Am J Obstet Gynecol 1990;163: 995–998.
Lewinsky RM, Jonson JM, Lao TT, et al. Fetal gastroschisis associated with monosomy 22 mosaicism and absent cerebral diastolic flow. Prenat Diagn 1990;10:605–608.
Lewis DF, Towers CV, Garite TH, et al. Fetal gastroschisis and omphalocele: is cesarean section the best mode of delivery? Am J Obstet Gynecol 1990;163:773–775.
Lindham S. Omphalocele and gastroschisis in Sweden 1965–1976. Acta Paediatr Scand 1981;70:55–60.
Lowry RB, Baird PA. Familial gastroschisis and omphalocele. Am J Hum Genet 1982;34:517–518.
Luck SR, Sherman JO, Raffensperger JG, et al. Gastroschisis in 106 consecutive newborn infants. Surgery 1985;98:677.
Luton D, Guibourdenche J, Vuillard E et al: Prenatal management of gastroschisis: the place of the amnioexchange procedure. Clin Perinatol 2003 30(3): 551-572
McKeown T, McMahon B, Record RG. An investigation of 69 cases of exomphalos. Am J Hum Genet 1953;5: 168–175.
Mann L, Ferguson-Smith MA. Prenatal assessment of anterior abdominal wall defects and their prognosis. Prenatal Diag 1984;14:427.
Martinez-Frias ML, Prieto SL, Zaplana J. Epidemiological study of gastroschisis and omphalocele in Spain. Teratology 1984;29:337–382.
Mayer T, Black R, Matlak ME, et al. Gastroschisis and omphalocele. Ann Surg 1980;192:783.
Mercer S, Mercer B, D’Alton MA, et al. Gastroschisis: ultrasonographic diagnosis, perinatal embryology, surgical and obstetric treatment and outcomes. Can J Surg 1988; 31:25. Molenaar JC, Tibboel D. Gastroschisis and omphalocele. World J Surg 1993;17:337–341.
Molenaar J, Tibboel D: Gastroschisis and omphalocele. World J Surg 1993 17(3): 337-341
Moore KL, Persaud TVN. The developing human: clinically oriented embryology. 5th ed. Philadelphia: Saunders, 1993.
Moore T, Stokes GF. Gastroschisis. Surgery 1953;33:112–120.
Moore TC. Gastroschisis with antenatal evisceration of intestines and urinary bladder. Ann Surg 1962;157:263.
Moore TC. The role of labor in gastroschisis bowel thickening and prevention by elective pre-term and pre-labor cesarean section. Pediatr Surg Int 1992;7:256–259.
Moretti M, Khoury A, Rodriquez J, et al. The effect of mode of delivery on the perinatal outcome in fetuses with abdominal wall defects. Am J Obstet Gynecol 1990;163: 833–838.
Muraji T, Tsugawa C, Nishijima E, et al. Gastroschisis: a 17-year experience. J Pediatr Surg 1989;24:343–345.
Nicholls G, Upadhyaya V, Gornall P, et al. Is specialist center delivery of gastroschisis beneficial? Arch Dis Child 1993;69:71–73.
Nicolaides KH, Snijders RJM, Cheng HH, et al. Fetal gastrointestinal and abdominal wall defects: associated malformations and chromosomal abnormalities. Fetal Diagn Ther 1992;7:102–115.
Novotny A, Klein RJ, Boeckman CR: Gastroschisis: an 18-year review. J Pediatr Surg 1993;28:650–652.
Nyberg DA, Mahony BS, Pretorius DH. Diagnostic ultrasound of fetal anomalies. Yearbook Medical Publisher, Chicago, 1993; 385–432.
Oh KS, Dorst JP, Dominguez R, et al. Abnormal intestinal motility in gastroschisis. Radiology 1978;127:457–460.
Oldham KT, Coran AG, Drongowski RA, et al. The development of necrotizing enterocolitis following repair of gastroschisis: a surprisingly high incidence. J Pediatr Surg 1988;23:945–949.
O’Neill JA, Grosfeld JL. Intestinal malfunction after antenatal exposure of viscera. Am J Surg 1974;127:129–132.
Paidas M, Crombleholme TM, Robertson FM. Prenatal diagnosis and management of the fetus with an abdominal wall injury. Semin Perinatol 1994:18(3):182–195.
Palomaki GE, Hill LE, Knight GJ, et al. Second-trimester maternal serum alpha-fetoprotein levels in pregnancies associated with gastroschisis and omphalocele. Obstet Gynecol 1988;71:906.
Paulozzi LJ. Seasonality of omphalocele in Washington state. Teratology 1986;33:133–134.
Piper HG, Jaksin T: The impact of prenatal bowel dilation on clinical outcomes in neonates with gastroschisis. J Pediatr Surg 2006 41(5): 897-900
Plessinger MA (1998) Prenatal exposure to amphetamines. Risks and adverse outcomes in pregnancy. Obstet Gynecol Clin North Am 25: 119–38.
Pokorny WJ, Harberg, FJ, McGill CW. Gastroschisis complicated by intestinal atresia. J Pediatr Surg 1981;16:261.
Redford RHA, McNay MB, Whittle MJ. Gastroschisis and exomphaloceles: precise diagnosis by mid-pregnancy ultrasound. Br J Obstet Gynaecol 1985;92:54.
Roeper PJ, Harris J, Lee G, et al. Secular rates and correlates for gastroschisis in California (1968–1977). Teratology 1987;35:203–210.
Romero R, Pilu G, Jeanty P, et al. The abdominal wall: prenatal diagnosis of congenital anomalies. Norwalk, CT: Appleton & Lange, 1988.
Rosendahl H, Kivinen S. Antenatal detection of congenital malformations by routine ultrasonography. Obstet Gynecol 1989;73:947.
Royner BD, Richards D. Growth retardation in fetuses with gastroschisis. J Ultrasound Med 1977;16:13–16.
Salinar CF, Bartoshefsky L, Othersen HB, et al. Familial occurrence of gastroschisis. Am J Dis Child 1979;133: 514–517.
Sandler A, Lawrence J, Meehan J et al: A “plastic” surtureless abdominal wall closure in gastroschisis 2004 39(5): 738-741J Pediatr Surg
Sawin R, Glick P, Schaller R, et al. Gastroschisis wringer clamp: a safe, simplified method for delayed primary closure. J Pediatr Surg 1992;27:1346–1348.
Schmidt D, Rose E, Greenberg F. An association between fetal abdominal wall defects and elevated levels of human chorionic gonadotropin in mid-trimester. Prenat Diag 1993;13:9–12.
Schuster S. A new method for the staged repair of large omphaloceles. Surg Gynecol Obstet 1967;125:261–266.
Sermer M, Benzie RJ, Pitson L, et al. Prenatal diagnosis and management of congenital defects of the anterior abdominal wall. Am J Obstet Gynecol 1987;156:308–312.
Shah R, Woolley MM. Gastroschisis and intestinal atresia. J Pediatr Surg 1991;26:788–790.
Shaw A. The myth of gastroschisis. J Pediatr Surg 1975;10: 235–244.
Sipes SL, Weiner CP, Sipes DR, et al. Gastroschisis and omphalocele: does either antenatal diagnosis or route of delivery make a difference in perinatal outcome? Obstet Gynecol 1990a;76:195–199.
Sipes SL, Weiner CP, Wiliamson RA, et al. Fetal gastroschisis complicated by bowel dilation: an indication for imminent delivery? Fetal Diagn Ther 1990b;5:100.
Snyder CL, St Peter SD: Trends in mode of delivery of gastroschisis infants. Am J Perinatol 2005 22(7)391-396
Stiller RJ, Haynes, de Regt R, et al. Elevated maternal serum alpha-fetoprotein concentration and fetal chromosomal abnormalities. Obstet Gynecol 1990;75:994.
Stoodley N, Sherma A, Noblett H, et al. Influence of place of delivery on outcome of babies with gastroschisis. Arch Dis Child 1993;69:71–73.
Stringel G. Large gastroschisis: primary repair with Gore-Tex patch. J Pediatr Surg 1993;28:653–655.
Stringel G, Filler RM. Prognostic factors in omphalocele and gastroschisis. J Pediatr Surg 1978;14: 515–519.
Tibboel D, Raine P, McNee M, et al. Developmental aspects of gastroschisis. J Pediatr Surg 1986a;21:865–869.
Tibboel D, Vermey-Keers C, Kluck P, et al. The natural history of gastroschisis during fetal life: development of the fibrous coating on the bowel loops. Teratology 1986b;33: 267–272.
Timor-Tritsch IE, Warren WB, Peisner DB, et al. First trimester midgut herniation: a high-frequency transvaginal sonographic study. Am J Obstet Gynecol 1989;161: 831–833.
Torfs C, Curry C, Roeper P. Gastroschisis. J Pediatr 1990; 116:1.
Torfs CP, Curry CJR. Familial cases of gastroschisis in a population-based registry. Am J Med Genet 1993;45: 465–467.
Van Allen MI. Fetal vascular disruptions: mechanisms and some resulting birth defects. Pediatr Annu 1981;10:31–50.
Walkinshaw SA, Renwick M, Hebisch G, et al. How good is ultrasound in the detection and evaluation of anterior abdominal wall defects? Br J Radiol 1992;65:298–301.
Werler MM, Mitchell AA, Shapiro S. First trimester maternal medication use in relation to gastroschisis. Teratology 1992b;45:361–367.
Yaster M, Scherer TLR, Stone MM, et al. Prediction of successful primary closure of congenital abdominal-wall defects using intraoperative measurements. J Pediatr Surg 1989;24:1217–1220.
Zivkovle SM. Repair of gastroschisis using umbilical cord as patch. J Pediatr Surg 1991;26:1179–1180.
Figure 1. Newborn infant with gastroschisis demonstrating characteristic “peel.”
Figure 2. Color flow doppler image in fetus with gastroschisis, demonstrating small defect with herniated midgut. See color plate.
Figure 3. Prenatal ultrasound demonstrating loops of intestine free-floating in aminotic fluid.
Figure 4. Intraoperative view during creation of silo for staged closure of gastroschisis.