Gastroschisis (Uptodate)

Authors:
Courtney D Stephenson, DO
Charles J Lockwood, MD, MHCM
Andrew P MacKenzie, MD
Section Editors:
Louise Wilkins-Haug, MD, PhD
Deborah Levine, MD
Deputy Editor:
Vanessa A Barss, MD, FACOG

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Sep 2019. | This topic last updated: Aug 07, 2019.

INTRODUCTIONGastroschisis is a full-thickness paraumbilical abdominal wall defect usually

associated with evisceration of bowel (picture 1) and sometimes other abdominal organs.

This topic will discuss issues related to prenatal diagnosis and management of pregnancies with fetal
gastroschisis. The other major fetal abdominal wall defect, omphalocele, occurs at the umbilicus and is
reviewed separately. (See "Prenatal diagnosis of omphalocele".)

CLINICAL SIGNIFICANCEBowel herniation may lead to a variety of intestinal abnormalities

because the mesenteric blood supply can become compromised and because prolonged exposure of the
bowel to the toxic environment of the amniotic fluid may result in inflammatory changes in the bowel
wall (see 'Associated anomalies and findings' below). In addition to bowel abnormalities, other
common potential sequelae of gastroschisis include growth restriction (30 to 60 percent of cases),
spontaneous preterm birth (30 to 50 percent), and fetal demise (3 to 6 percent) [1-8]. The mechanisms
causing these adverse outcomes are unclear. Deficiency in growth may be due to undernutrition from
loss of protein and fluid across the exposed bowel [9], but abnormal Doppler measurements have been
observed in a minority of cases [10,11]. The increased risk of fetal demise may be related to growth
restriction, cord compression, or other undefined factors.

After birth, the newborn requires special care because of the exposed bowel. The defect must be
closed, which can be difficult if the defect is large and other abdominal organs are also herniated;
however, the prognosis is generally very good. (See 'Neonate' below.)

PATHOGENESISSeveral hypotheses have been proposed to explain the pathogenesis of gastroschisis;

all involve defective formation or disruption of the body wall in the embryonic period, with subsequent
herniation of bowel [12]:

●Failure of mesoderm to form in the body wall

●Rupture of the amnion around the umbilical ring

●Abnormal involution of the right umbilical vein leading to weakening of the body wall

●Disruption of the right vitelline artery with subsequent body wall damage

Gene polymorphisms that interact with environmental factors, such as smoking, may play a role in
pathogenesis [13]. The maternal immune response to new paternal (fetal) antigens may also play a role
[14]. There is no high-quality evidence that any drug causes gastroschisis, but a possible association
has been reported for aspirin [15], ibuprofen [16], and vasoconstrictive agents (eg, pseudoephedrine)
[17]. Use of acetaminophen in the first trimester has been reported both to lower the risk of
gastroschisis [18] and to increase the risk [19].
The prevalence of gastroschisis appears to be higher in areas where surface water agricultural chemical
levels are high and when conception occurs in the spring, the time when agricultural chemicals (eg,
atrazine) are commonly applied [20-26]. The possible role of these chemicals in the pathogenesis of
gastroschisis requires further study as an association with adverse birth outcomes has not been reported
consistently [27,28].

Gastroschisis has not been associated with maternal deficiency of any micronutrient or macronutrient
[29].

INCIDENCE AND EPIDEMIOLOGYGastroschisis and omphalocele are the most common fetal
abdominal wall defects: the prevalence of each is approximately 3 to 4 per 10,000 live births/fetal
deaths/stillbirths/pregnancy terminations [1,30]. The incidence of gastroschisis is similar in male and
female fetuses [31-33], higher in singleton pregnancies than in twin pregnancies [34], and higher in
Hispanic and non-Hispanic white mothers than non-Hispanic black mothers [35].

Studies worldwide have consistently reported that young women (ie, under 20 years of age) have a
several-fold higher rate of offspring with gastroschisis than the general obstetrical population [1,36-
39]. This is likely related to lifestyle factors that characterize this population (eg, cigarette smoking,
use of recreational drugs, alcohol consumption, low body mass index, increased frequency of
genitourinary infection) [16,36,40-45].

Both resource-rich and resource-limited countries have reported an increasing incidence of

gastroschisis [35,46,47]. In a study of data from 25 population-based registries in 15 European
countries, maternal age-standardized incidence of gastroschisis increased almost fourfold, from 0.54
per 10,000 births between 1980 and 1984 to 2.12 per 10,000 births between 2000 and 2002 [32]. In the
United States, gastroschisis prevalence increased 10 percent from 2006–2010 to 2011–2015, with the
highest prevalence among mothers aged <20 years [35]. The prevalence was particularly high in
counties with high opioid prescription rates.

LABORATORY MARKERSAn increased possibility of gastroschisis, as well as other fetal anomalies,

should be considered in pregnancies with an elevated maternal serum alpha fetoprotein (MSAFP) level
as almost all cases of gastroschisis are associated with this finding [48-50]. In three series of
gastroschisis cases, the median MSAFP level was 9.42 multiples of the median (MoMs) [49], 7.0
MoMs [50], and 4.4 MoMs [51]. By comparison, the MSAFP in normal pregnancies and pregnancies
with open spina bifida and anencephaly are, on average, 1 MoMs, 4 MoMs, and 8 MoMs, respectively
[52].

An elevated MSAFP is an indication for thorough ultrasonographic examination of the fetus for
anatomic abnormalities. (See "Open neural tube defects: Risk factors, prenatal screening and diagnosis,
and pregnancy management", section on 'Alpha-fetoprotein'.)

PRENATAL DIAGNOSISPrenatal detection rates are high as a result of routine ultrasound screening
for fetal anomalies, as well as maternal serum alpha fetoprotein (MSAFP) assessment in some
screening protocols for open neural tube defects and Down syndrome [53]. The European network of
population-based registries for the epidemiologic surveillance of congenital anomalies (EUROCAT)
reported over 90 percent of cases were detected prenatally [52]. Most cases can be detected by the end
of the first trimester (11 to 14 weeks).

Ultrasound findings — On ultrasound examination, gastroschisis appears as a relatively small

(typically 2 to 5 cm) paraumbilical abdominal wall defect, usually to the right of the midline, with
visceral herniation. The umbilical cord insertion site is adjacent to and separate from the defect and
should be normal. Usually, the intestine is the only herniated organ, but the liver and stomach may also
herniate; other intra-abdominal organs rarely pass through the defect. The stomach is usually
malpositioned, even when intra-abdominal. The intestinal mass lacks a covering membrane and floats
freely in the amniotic fluid. Sonographic findings of gastroschisis are illustrated by the following
diagnostic images: gray-scale (image 1A-B), color Doppler (image 2), three-dimensional ultrasound
(image 3).
The exteriorized bowel appears cauliflower-like because fluid between adjacent bowel loops results in
acoustic interfaces at both near and far bowel walls. Visualization of the bowel is enhanced by the
highly echogenic bowel wall edema and inflammation that may occur in addition to the dilated lumen
created by multiple volvuli in the free-floating loops. Late in pregnancy, the eviscerated bowel often
appears thickened, matted, and mildly dilated due to chronic exposure to amniotic fluid. The stomach
and intra-abdominal loops of bowel also may become dilated due to obstruction [54].

Associated anomalies and findings — Associated gastrointestinal anomalies and problems (eg,
malrotation, atresia, stenosis, perforation, necrosis, volvulus) occur in up to 25 percent of cases [55-57]
and may be related to vascular disruption caused by herniated bowel. Disruption of the superior
mesenteric artery, for example, may lead to volvulus or to "apple peel" jejunal-ileal lesions. Meckel's
diverticulum and gallbladder atresia also occur but are less common. Bladder herniation has been
reported in 6 percent of cases (image 4) and may cause bowel or urinary tract dilation. A rare type of
complex gastroschisis has been termed "closed," "closing," or "vanishing" gastroschisis [58-62]. In
these cases, the defect closes around the viscera, which can cause intestinal atresia and ischemia or
midgut infarction resulting in short bowel syndrome.

Most cases have no extraintestinal abnormalities. In a study of pooled data from 24 international birth
defects registries including over 3300 cases of gastroschisis, approximately 85 percent were isolated,
defined as gastroschisis alone or associated with only one or more of the following anomalies: any
intestinal defect, any deformation (eg, clubfoot and hip dysplasia, except arthrogryposis), any minor or
mild defect (eg, patent ductus arteriosus, patent foramen ovale, unspecified atrial septal defect,
hydronephrosis, or enlarged pelvis) [47]. The other 15 percent of gastroschisis cases were associated
with a chromosomal syndrome (mostly trisomy 18, 13, or 21, or sex chromosome aneuploidy), other
syndrome, or multiple congenital anomalies (one or more major defects unrelated to gastroschisis). A
study using a prospectively collected database including almost 4700 infants with gastroschisis
discharged from 348 neonatal intensive care units in North America reported associated anomalies in 8
percent and cardiac anomalies in 1 percent [2]. However, others have reported associated anomalies in
as many as one-third of cases [63]. The discordancy may be due to ascertainment bias [3,47,55,64,65].

Oligohydramnios is the most common amniotic fluid abnormality, but polyhydramnios may occur,
particularly in fetuses with reduced bowel motility or obstruction.

Differential diagnosis — Omphalocele is the major disorder to consider in differential diagnosis. The
membranous sac helps to distinguish omphalocele from gastroschisis; however, membranes
occasionally rupture in utero, in which case, other features need to be evaluated to make the correct
diagnosis. If the membrane ruptures, the locations of the liver and cord vessels and insertion site can
help to differentiate an omphalocele from gastroschisis. Omphalocele is often associated with an
extracorporeal liver, while the liver is typically intracorporeal in gastroschisis. The cord insertion site is
into the umbilical sac in omphalocele and paraumbilical onto an otherwise intact abdominal wall in
gastroschisis. If the sac ruptures, the cord vessels traverse the amniotic remnant in omphalocele and
enter the abdomen in the suprapubic region. Gastroschisis is associated with a lower rate of associated
defects than omphalocele (32 versus 80 percent in one large study [63] and 8 versus 35 percent in
another large study [2]) [2,63,66].

Other major abdominal wall defects in differential diagnosis (ectopia cordis, limb-body wall complex,
cloacal exstrophy, and urachal cyst) are rare (prevalence of each is less than 1 in 100,000 births).
Omphalocele and body-stalk defects are connected to the cord, ectopia cordis develops above and
bladder exstrophy below the cord insertion, and gastroschisis is paraumbilical (table 1).

PREGNANCY MANAGEMENT

Fetal genetic studies — The purpose of fetal genetic testing is to obtain information that has prognostic
significance and thus can impact parental decision-making with respect to continuing the pregnancy,
pregnancy management, and management of the newborn. The prevalence of chromosomal
abnormalities in fetuses with isolated gastroschisis (ie, no extraintestinal anomalies) is not increased
above that in the baseline population; thus, this anatomic finding alone is not a strong indication to
pursue invasive diagnostic fetal genetic testing (assuming ruptured omphalocele sac has been excluded)
[67]. Because the risk is higher when extraintestinal structural abnormalities are identified on
ultrasound, we suggest genetic amniocentesis in these cases [47]. Microarray molecular testing rather
than G-banding has been recommended whenever fetal structural anomalies are detected on prenatal
ultrasound examination [68], but the additional value in cases of gastroschisis has not been studied
extensively [69]. (See "Prenatal genetic evaluation of the fetus with anomalies or soft markers".)

Fetal monitoring — There is little consensus regarding the best approach to monitoring the fetus in
these pregnancies due to a lack of high-quality evidence to guide clinical practice [33,70].

Assessment of fetal growth and amniotic fluid volume — Assessment of fetal growth is typically
performed starting at 24 weeks and repeated every 3 to 4 weeks [70]. Growth restriction in fetuses with
abdominal wall defects is predictive of an increased risk of adverse neonatal outcome in some studies
[4,10] and may be associated with an increased risk of fetal demise [6,71]. (See 'Antepartum fetal
surveillance' below.)

Amniotic fluid volume (AFV) abnormalities frequently occur. Oligohydramnios may be related to fetal
growth restriction and is a risk factor for cord compression and its sequelae. Polyhydramnios is less
common but is an important finding because it is often caused by bowel atresia and is predictive of
severe bowel complications in the neonatal period [72]. (See "Oligohydramnios" and
"Polyhydramnios".)

We use a standard formula for estimating fetal weight, which was validated in one large study [73].
Because the most commonly used formulas for estimating fetal weight, such as Hadlock, rely heavily
on the measurement of abdominal circumference, others have reported that these formulas tend to
underestimate the weight of these fetuses [74]. Siemer and colleagues developed a specific formula for
estimating fetal weight in fetuses with abdominal wall defects using the biparietal diameter,
occipitofrontal diameter, and femur length measurements [75]. This formula appears to estimate fetal
weight in these fetuses more accurately than formulas using abdominal circumference and is used by
some clinicians [75-77].

Assessment of fetal bowel — We perform serial targeted sonographic evaluations of the stomach and
bowel (both intra- and extra-abdominal) to look for significant dilation or acute changes, such as
thickening and edema at the time of fetal growth scans (growth scans are every three to four weeks
starting after the anatomic survey), although some advocate for additional bowel assessment every 2
weeks after 32 weeks. If we observe these changes before 34 weeks, we administer a course of
glucocorticoids to enhance fetal maturation in the event that preterm delivery becomes indicated, but
we do not deliver solely on the basis of bowel wall thickening or dilation.

Gastric dilation, bowel dilation, and bowel wall thickening have been considered poor prognostic signs
by several investigators [78-83], but others have not found the presence of these findings sufficiently
predictive to influence clinical management [4,84-92]. For this reason, approximately 50 percent of
maternal-fetal medicine specialists do not measure bowel wall thickness at all, and the remainder
measure it weekly to monthly [70]. A 2009 systematic review including data from 10 observational
studies (n = 273 patients) concluded that there was no strong evidence that fetuses with isolated
gastroschisis and antenatal bowel dilation greater than 10 mm or greater than 18 mm were at increased
risk of intrauterine death, postnatal bowel resection, length of time to oral feeds, or length of time
hospitalized [93]. However, the small number of adverse events in these studies precluded making a
definite conclusion about the significance of prenatal bowel dilation in fetuses with gastroschisis.

We begin weekly antepartum fetal testing upon diagnosis of substantial dilation, rather than waiting
until 32 weeks (see 'Antepartum fetal surveillance' below). There is some evidence that substantial
extra-abdominal small bowel dilation >25 mm is associated with short-term prenatal complications,
including nonreassuring fetal testing and fetal death [82]. In one small series, the three fetuses with
external bowel diameters of 27 to 28 mm had these adverse outcomes, while 11 fetuses with diameters
of 5.0 to 24.5 mm did not [82]. Adverse prenatal sequelae may be related to umbilical cord
compression by the dilated bowel or severe uteroplacental insufficiency. Other series have observed
that intra-abdominal dilation was predictive of bowel obstruction [94-96] and associated with increased
neonatal morbidity when multiple, but not single, loops of bowel were dilated [83]. But data are far
from definitive; the threshold for dilation predictive of adverse outcome varies among studies, and
some studies have not observed adverse effects from either intra-abdominal or extra-abdominal bowel
dilation [97,98].

Antepartum fetal surveillance — The precise timing, choice of test, and frequency of testing is
arbitrary. A survey of maternal-fetal medicine specialists reported that 68 percent began antenatal
testing at 32 weeks, another 12 percent began at 28 weeks, and 8 percent began at 34 weeks [70].
Nonstress tests (NSTs), biophysical profile scores (BPPs), and amniotic fluid indexes were generally
performed weekly.

In the absence of growth restriction, oligohydramnios, or other complications warranting close fetal
surveillance (eg, substantial bowel dilation, preeclampsia), we suggest weekly NST and/or a BPP
beginning at 32 weeks of gestation for all fetuses with gastroschisis. This is because these pregnancies
may be associated with an increased risk for fetal demise late in the third trimester (pooled prevalence
4.48 per 100 gastroschisis pregnancies overall, 95% CI 3.48-5.76 [6]) [6,71]. Fetal surveillance is
increased to twice weekly after 36 weeks. Although fetal heart rate (FHR) abnormalities are common
in these pregnancies, the value of antepartum fetal surveillance is only supported by low-quality data
[99-103].

If growth restriction is suspected, we perform NSTs and/or BPPs and Doppler velocimetry of the
umbilical artery at least weekly from diagnosis until 28 weeks of gestation and then twice weekly
thereafter. If absent or reversed flow in the umbilical artery is detected at ≥34 weeks, delivery is
indicated. (See "Fetal growth restriction: Evaluation and management", section on 'Pregnancy
management'.)

Delivery — Ideally, delivery should occur in a facility with appropriate resources for caring for these
neonates as the body of evidence suggests inborn newborns have better outcomes than those who
require transfer after birth for treatment [104]. Coordinating delivery at a tertiary care center provides
optimal conditions for the neonate [105].

Timing — The decision on timing of delivery is based on a combination of factors, including

gestational age (probability of fetal lung maturity), ultrasound findings (fetal growth profile, AFV,
appearance of fetal bowel), and fetal testing results (NST, BPP, umbilical cord Doppler if fetal growth
restriction is present). We suggest consultation with a maternal-fetal medicine specialist, neonatologist,
and pediatric surgeon before delivery to discuss patient-specific factors in the timing of delivery. We
do not consider bowel dilation alone an indication for preterm delivery if fetal growth, AFV, and fetal
testing (BPP, NST) remain reassuring. In the authors' practices, delivery of fetuses with gastroschisis,
normal growth, normal AFV, and reassuring fetal testing is scheduled for 38+0 weeks of gestation.
Delivery before 38+0 weeks is performed for standard obstetric indications. It should be noted that the
mean gestational age at spontaneous labor in pregnancies complicated by gastroschisis is in the 36th
week of gestation [7,106,107]. This should be considered if patients are planning to relocate to deliver
at surgical site for repair.

We believe available data support this approach, which avoids the morbidity of preterm birth,
minimizes the risk of gastroschisis related neonatal morbidity (eg, sepsis, bowel damage [necrosis,
atresia, perforation, adhesion]), and eliminates any possibility of stillbirth at term (39 to 40 weeks)
[103,108,109]. One contributor to this topic (LWH) does not intervene until the 39 th week of gestation
if twice-weekly fetal surveillance (BPP, NST) is reassuring and the bowel is not significantly dilated,
but delivers earlier if bowel dilation >25 mm develops after 37 weeks of gestation. A survey of
maternal-fetal medicine specialists reported 43 percent delivered at 37 weeks and 29 percent delivered
at 39 weeks if gastroschisis was stable; 6 percent recommended delivery prior to 37 weeks [70].

Delivery as early as 33 weeks of gestation has been suggested based on reports of an increased risk of
fetal demise in the third trimester [4,71,110,111] and a possible increase in risk of inflammatory bowel
changes from ongoing exposure to amniotic fluid. Not only is there no evidence that planned preterm
delivery decreases the need for silo closure, the time until enteral feeding can begin, or other
gastrointestinal problems [103,112,113], but most contemporary studies of pregnancies complicated by
gastroschisis have found that preterm delivery is the most important predictor of adverse outcome
[106,114], especially if performed before 34 weeks [115]. Thus, we believe preterm delivery to reduce
the risk of fetal demise or improve neonatal outcome is unwarranted if fetal growth, AFV, and fetal
testing (BPP, NST) are normal [103].

Route — Although high-quality evidence is not available, we recommend reserving surgical delivery
for usual obstetric indications. Labor and rupture of membranes have not been proven to adversely
affect outcome, and there is no evidence that cesarean delivery improves outcome in uncomplicated
gastroschisis [8,116-119]. In meta-analyses of observational studies evaluating the effect of mode of
delivery in fetuses with abdominal wall defects (gastroschisis or omphalocele), cesarean delivery was
not associated with improvement in any neonatal outcome (mortality, rate of primary fascial repair,
neonatal sepsis, necrotizing enterocolitis, secondary repair, short-gut syndrome, time until enteral
feeding, length of hospital stay) [120,121].

Some pediatric surgeons recommend cesarean delivery for gastroschisis with liver involvement,
especially when there is marked liver herniation, because of the theoretic risk of dystocia and trauma.
This is a relatively rare finding and may actually represent omphalocele complicated by rupture of the
surrounding amnio-peritoneal membrane. The delivery route in these rare cases should be
individualized based on patient specific factors.

Intrapartum fetal heart rate — Some, but not all, studies report a high frequency of nonreassuring FHR
tracings and amniotic fluid staining (meconium or bile [122]) leading to cesarean delivery [7,46]. FHR
abnormalities could be due to cord compression related to oligohydramnios or fetal hypovolemia
related to chronic loss of protein and water across the exposed bowel. There are no studies on the use
of intrapartum amnioinfusion in these cases. We do not use it because of concerns of injuring bowel
and lack of demonstrable benefit.

NEONATE

Clinical findings — The characteristic clinical finding in newborns is a full-thickness paraumbilical

abdominal wall defect, often associated with evisceration of bowel (picture 1). It is usually located to
the right of the umbilical cord insertion site and tends to be <4 cm in diameter [123,124]. There is no
covering membrane. Inflammation and fibrosis from chronic exposure to amniotic fluid result in
thickening and matting of the intestines, decreased bowel motility, and possibly luminal obstruction
[123]. Although amnioexchange procedures reduced the inflammatory process in animal studies, a
randomized trial in humans did not find beneficial effects on pregnancy outcome or complications
[125].

Delivery room care — Neonatal fluid losses are 2.5 times that of a healthy newborn in the first 24
hours of life [126]. The neonate is at risk for insensible heat and fluid losses from exposure of the
eviscerated bowel. In addition, third space fluid deficits from sequestration of intestinal fluid can be
significant.

The initial approach to management of these newborns includes [126,127]:

●Wrapping the bowel with sterile saline dressings covered with plastic wrap. This preserves body heat,
minimizes insensible fluid loss, and protects the bowel. In some centers, the infant is immediately
placed into a plastic bag covering its lower half to maximize temperature control and hydration while
allowing access to initial visual examination.

●Inserting an orogastric tube to decompress the stomach.

●Placement of peripheral intravenous access to provide fluids and broad-spectrum antibiotics that
cover maternal vaginal flora (eg, ampicillin and gentamicin). The maintenance fluid requirement is
increased two- to threefold because of losses from the exposed bowel.

●Ensuring a patent airway.

●Keeping the neonate in a thermoneutral environment.

●Providing respiratory support, if required.

Although breastfeeding or other oral feeding is not possible until after the gastroschisis has been
repaired, mothers are encouraged to pump and store breast milk for future use.

Synopsis of surgical management — Primary closure, when feasible, is performed within a few hours
of birth [128]. In the operating room, the bowel is decompressed by aspirating stomach contents and
evacuating the large bowel through the rectum. The size of the defect is increased 1 to 2 cm to
minimize trauma to the bowel during reduction. The abdominal wall is manually stretched, and the
bowel is replaced, taking care to avoid creating intra-abdominal pressure that is too high [126,129].
Primary closure is successful in 70 percent of cases.

If primary closure is not feasible because of thickened, distended intestinal loops and a small
abdominal domain, a staged closure with a silastic silo can be used as in omphalocele cases. A
preformed silo with a spring-loaded ring can be placed at the bedside to cover the herniated intestine
quickly without suturing [130]. Some surgeons perform silo placement with staged closure in all
gastroschisis cases as some data suggest that outcomes are equivalent to primary closure [131].

A sutureless closure that stretches the umbilical cord across the defect without fascial suturing is a
promising management approach, but data are limited [132,133].

Prolonged postoperative dysmotility is a common problem and interferes with enteral feeding. Studies
in animal models suggest that dysmotility is due to delayed maturation of the enteric nervous system
[134,135], possibly as a result of prolonged exposure to amniotic fluid [136].

Prognosis — Gastroschisis has the most favorable prognosis of the abdominal wall defects because
most cases are not complicated by concomitant nongastrointestinal anomalies or aneuploidy [137]. The
overall survival rate for live-born infants with gastroschisis was 97.8 percent in a prospective study of
4420 neonates born at 175 North American centers [8]. Sepsis, which occurred in 8.6 percent of the
cohort, was the only significant independent predictor of mortality.

Gastroschisis in infants can be categorized as "simple" or "complex" based on the absence or presence
of intestinal atresia, stenosis, perforation, necrosis, malrotation, or volvulus, but this distinction is often
not possible to discern prenatally [57]. Up to 25 percent of cases are complex, and these infants have
significantly more gastrointestinal, respiratory, and infectious disease complications in the neonatal
period [138]. In a 2014 systematic review and meta-analysis of studies that compared the outcome of
complex versus simple gastroschisis, complex gastroschisis was associated with higher risks of in-
hospital mortality (risk ratio [RR] 5.4, 95% CI 2.4-12.0), short bowel syndrome (RR 12.0, 95% CI 6.3-
22.8), bowel obstruction (RR 2.2, 95% CI 1.4-3.6), necrotizing enterocolitis (RR 1.97, 95% CI 1.1-
3.7), and parenteral nutrition and tube feedings on discharge (RR 11.2, 95% CI 3.8-33.2 and 2.8, 95%
CI 1.5-5.5, respectively), but the rates of primary abdominal closure and silo bag placement were
similar for both groups [138]. The postsurgical course for these infants can be lengthy (in one study:
mean 53 days, range 8 to 307 days [92]).

Long-term outcomes are generally satisfactory. A study assessing neurodevelopment at 5 to 17 years of

age reported overall intellectual abilities were within a normal range [139]. Survivors are prone to the
typical problems related to intestinal adhesions [140,141]. Although a minority of children have short-
bowel syndrome, many can eventually discontinue parenteral nutrition [142-144]. Lastly, many
patients are bothered by their lack of an umbilicus [140]; these individuals can consider umbilical
reconstruction surgery.

RECURRENCE RISKThere is an increased risk of recurrence in families with a child with

gastroschisis, which suggests that genetic factors play a role in causation. A systematic review of 11
population-based studies (862 probands) reported a 5.7 percent risk of gastroschisis in other family
members, 3 percent adjusted for probands and 4.3 percent in subsequent siblings [145]. However,
nongenetic factors are also important, which suggests a multifactorial inheritance pattern.

SUMMARY AND RECOMMENDATIONS

●Gastroschisis is a full-thickness paraumbilical abdominal wall defect, usually on the right and
associated with evisceration of fetal bowel. There is no covering membrane. (See 'Clinical findings'
above.)

●The pathogenesis of gastroschisis is unknown. All theories involve defective formation or disruption
of the body wall in the embryonic period, with subsequent herniation of bowel. (See 'Pathogenesis'
above.)

●There is an inverse association between maternal age and incidence of fetal gastroschisis, with the
highest prevalence in whites and births to women under age 20 years. (See 'Incidence and
epidemiology' above.)

●Maternal serum alpha fetoprotein (MSAFP) is elevated in virtually all pregnancies with gastroschisis.
(See 'Prenatal diagnosis' above.)

●Prenatal diagnosis is based on sonographic visualization of a paraumbilical abdominal wall defect,

usually to the right of the midline, with visceral herniation (image 1A-B). The adjacent umbilical cord
insertion site is normal. The herniated intestines lack a covering sac and float freely in the amniotic
fluid. The combination of ultrasound examination and MSAFP screening detects at least 90 percent of
cases. (See 'Prenatal diagnosis' above.)

●Approximately 10 percent of cases are associated with malformations outside of the gastrointestinal
tract; additional gastrointestinal problems (eg, intestinal atresia, stenosis, perforation, necrosis,
malrotation, volvulus) are present in up to 25 percent of cases. The karyotype is abnormal in 1 percent
of cases, usually in the setting of associated abnormalities. (See 'Associated anomalies and findings'
above.)

●Pregnancy complications include increased risk of intrauterine growth restriction, fetal demise,
spontaneous preterm birth, and bowel thickening and dilation. (See 'Fetal monitoring' above.)

●We suggest offering invasive testing for fetal microarray molecular testing when gastroschisis is
associated with additional nongastrointestinal structural abnormalities. Isolated gastroschisis does not
appear to be associated with an increased risk of aneuploidy. (See 'Fetal genetic studies' above.)

●We obtain serial ultrasound examinations to follow fetal growth and amniotic fluid volume at two- to
four-week intervals since these fetuses are at risk of growth impairment and amniotic fluid
abnormalities. We also obtain targeted views of the intra- and extra-abdominal bowel to look for
significant dilatation, thickening, or edema. If such changes are observed prior to 34 weeks, we
administer a course of glucocorticoids to enhance fetal maturation in the event that preterm delivery
becomes indicated, but we do not intervene solely on the basis of bowel wall thickening or dilation.
(See 'Fetal monitoring' above.)

●Given the increased risk of fetal demise late in pregnancy, we suggest antepartum fetal surveillance
(nonstress test, biophysical profile) (Grade 2C). (See 'Fetal monitoring' above.)

●Ideally, delivery should occur in a facility with appropriate resources for caring for these neonates. In
the absence of standard obstetric indications for abdominal delivery, we suggest a trial of labor rather
than scheduled cesarean birth for most patients (Grade 2C). Cesarean delivery is reasonable if the liver
is significantly herniated because of the theoretic risk of dystocia and trauma. In the authors' practices,
delivery of fetuses with gastroschisis, normal growth, normal AFV, and reassuring fetal testing is
scheduled for 38+0 weeks of gestation; with earlier delivery for standard obstetric indications. This
minimizes neonatal morbidity and mortality and avoids the possibility of term (39 to 40 weeks)
stillbirth; however, there is no consensus on the optimum timing of delivery of these pregnancies. (See
'Delivery' above.)

●In the delivery room, the bowel is wrapped with sterile saline dressings covered with plastic wrap to
preserve heat and minimize insensible fluid loss. In some centers, the infant is immediately placed into
a plastic bag covering its lower half to maximize temperature control and hydration while allowing
access to initial visual examination. In addition, an orogastric tube is placed to decompress the
stomach, peripheral intravenous lines are inserted, and the airway is stabilized. (See 'Neonate' above.)

●Overall survival is over 90 percent. Approximately 10 percent of cases are complex, and these infants
have significantly more gastrointestinal, respiratory, and infectious disease complications in the
neonatal period. (See 'Prognosis' above.)

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