- 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 fetues that meet these criteria are in the minority. In general, we schedule patients for induction of labor at 37 weeks’ if they haven’t alresdy 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 pateints 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.
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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.