Accepted Manuscript

Advances in Prenatal and Perinatal Diagnosis and Management of

Gastroschisis

Megan C. Oakes MD , Manuel Porto MD ,

Judith H. Chung MD, PhD

PII: S1055-8586(18)30067-2
DOI: https://doi.org/10.1053/j.sempedsurg.2018.08.006
Reference: YSPSU 50768

To appear in: Seminars in Pediatric Surgery

Please cite this article as: Megan C. Oakes MD , Manuel Porto MD , Judith H. Chung MD, PhD , Ad-
vances in Prenatal and Perinatal Diagnosis and Management of Gastroschisis, Seminars in Pediatric
Surgery (2018), doi: https://doi.org/10.1053/j.sempedsurg.2018.08.006

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 ACCEPTED MANUSCRIPT

Advances in Prenatal and Perinatal Diagnosis and Management of Gastroschisis

Megan C. Oakes, MD; Manuel Porto, MD; Judith H. Chung. MD, PhD

Division of Maternal Fetal Medicine; Department of Obstetrics and Gynecology

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University of California Irvine Medical Center, Orange, California

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Corresponding Author:

Judith H. Chung, MD, PhD

Address: UC Irvine Health US

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Department of Obstetrics and Gynecology

333 City Boulevard West, Suite 1400

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Orange, CA 92868

Tel: 714-456-8695
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Fax: 714-456-7091
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Email: judithc@uci.edu
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ABSTRACT

Gastroschisis is a congenital, ventral wall defect associated with bowel evisceration. The

defect is usually to the right of the umbilical cord insertion and requires postnatal surgical

correction. The fetus is at risk for complications such as intrauterine growth restriction, preterm

delivery, and intrauterine fetal demise. In addition, complex cases, defined by the presence of

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intestinal complications such as bowel atresia, stenosis, perforation, or ischemia, occur in up to

one third of pregnancies affected by gastroschisis. As complex gastroschisis is associated with

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increased morbidity and mortality, research has focused on the prenatal detection of this high

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risk subset of cases. The purpose of this review is to discuss the prenatal, diagnostic approach to

the identification of gastroschisis, to describe potential signs of complex gastroschisis on

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prenatal ultrasound, to review current guidelines for antepartum management and delivery

planning, and to summarize results of both past and current intervention trials in fetuses with
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gastroschisis.
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Index Words: gastroschisis, sonography, prenatal, complex, bowel dilatation, antenatal.

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INTRODUCTION

Gastroschisis is a congenital, ventral wall defect, typically occurring to the right of the

umbilical cord insertion, and results in bowel evisceration.1 Current estimates of the prevalence

of gastroschisis demonstrate an uptrend, from 3.6 per 10,000 births during 1995–2005 to 4.9 per

10,000 births during 2006–2012.2 The reason for this is unclear, but younger maternal age,

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smoking, drug use, and maternal infection appear to be risk factors for gastroschisis.2

Furthermore, gastroschisis is not thought to be associated with an increased risk for

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chromosomal abnormalities above that conferred by maternal age alone. Pregnancies

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complicated by gastroschisis are at increased risk for intrauterine growth restriction (IUGR),

preterm labor, intrapartum fetal heart rate abnormalities, and increased hospital length of stay.3-7
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There is also an increased risk of intrauterine fetal demise (IUFD).8 Neonatal mortality may be as

high as 5-10%, which is mostly due to complex gastroschisis, defined by the presence of bowel
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atresia, stenosis, perforation, or ischemia.9-12 Therefore, much work has focused on the in utero

identification of those fetuses who may be at higher risk of complex gastroschsisis.9, 10, 13-20 In
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this review, we aim to discuss the prenatal diagnostic approach to gastroschisis, to describe
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potential signs of complex gastroschisis on antenatal ultrasound, to review current guidelines for

antepartum management and delivery planning, and to summarize results of both past and
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current intervention trials in fetuses with gastroschisis.

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Diagnostic Approach for Gastroschisis

The mainstay of prenatal diagnosis of gastroschisis is ultrasound. Gastroschisis typically

presents as a paraumbilical abdominal wall defect, to the right of the midline and with herniation

of free-floating bowel into the amniotic cavity.1 Figure 1 shows a transverse view of the fetal
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abdomen with a normal cord insertion. In comparison, in Figure 2, fetal gastroschisis is seen.

Rarely, liver and stomach herniation may also occur. Gastroschisis is most commonly diagnosed

during the second trimester, although there have been case reports of diagnosis in the first

trimester.21, 22 Early diagnosis is possible, but the sonographer must keep in mind that normal,

physiologic midgut herniation occurs in the developing fetus during the first trimester and can be

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erroneously attributed to the presence of an abdominal wall defect.23, 24 According to Green and

Hobbins,25 up to 20% of fetuses will still demonstrate physiologic herniation of the bowel at 12

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weeks’ gestation; therefore, suspicion for gastroschisis by first trimester ultrasound must be

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interpreted with caution. Figure 3 shows a fetus in which gastroschisis was diagnosed at 12 5/7

weeks, which was subsequently confirmed by repeat ultrasound at 19 0/7 weeks (Figure 4).
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Although helpful for counseling, earlier diagnosis of gastroschisis does not change antenatal

management or outcomes in a continuing pregnancy.

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The differential diagnosis for bowel herniation should include other abdominal wall

defects such as omphalocele, in addition to cord herniation and limb-body wall complex.1 In
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particular, it is critical to differentiate gastroschisis from omphalocele, given that they can appear
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quite similar on ultrasound but have vastly different fetal and neonatal outcomes. In contrast to

gastroschisis, omphalocele occurs as a result of defective ventral wall infolding and is more
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commonly associated with genetic syndromes, as well as additional anomalies of the neurologic,

cardiac, pulmonary, and renal systems.26-28 Syndromes associated with omphalocele include
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Beckwith-Weidemann, cloacal exstrophy, fibrochondrogenesis, Marshall-Smith syndrome,

Meckel-Gruber syndrome, Trisomy 13, Trisomy 18, and triploidy.26-28

As shown in Figure 5, an omphalocele typically has a membranous sac that covers the

eviscerated bowel, although in utero rupture may occur.29, 30 Assessment of the umbilical cord
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insertion site also allows differentiation between omphalocele and gastroschisis, with the cord

insertion into the umbilical sac of an omphalocele versus insertion directly into the otherwise

intact abdominal wall with gastroschisis. Color flow Doppler may be helpful to more clearly

delineate the location of the umbilical cord insertion site into the fetal abdominal wall. Figures 6

and 7 show the umbilical cord insertion site in a fetus with gastroschisis. The addition of color

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flow Doppler in Figure 7 aids in the identification of the umbilical cord insertion site in relation

to the extraabdominal, free-floating bowel. Finally, an extracorporeal liver is much more

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common in omphalocele, as opposed to gastroschisis.

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Elevated maternal serum alpha fetoprotein (MSAFP), a component of the routine second

trimester genetic screen typically performed between 15 and 20 weeks’ gestation, can also be
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seen in gastroschisis and is thought to be due to free diffusion of AFP from the fetal circulation

into maternal circulation.31 Elevations of MSAFP, up to 7 multiples of the median, have been
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reported in conjunction with fetal ventral wall defects.31 MSAFP has proven to be more sensitive

in detecting gastroschisis than omphalocele, likely due to the intact, overlying membrane in
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omphalocele. The combination of ultrasound and second trimester serum screening detects over

90% of gastroschisis cases antenatally.32

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Types of Gastroschisis

Gastroschisis can generally be divided into simple and complex cases. In cases of simple
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gastroschisis, the condition is not associated with intestinal complications, whereas in complex

gastroschisis (CG), intestinal complications, such as bowel atresia, stenosis, volvulus,

perforation, or ischemia, are present. CG represents approximately 11-28% of all cases and

results in a significantly greater risk of morbidity and mortality.9-12, 33 The entity of closed or
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closing gastroschisis has also been described.33, 34 In this condition, the defect closes around the

prolapsed viscera, resulting in exit and/or entry intestinal atresia and ischemia.34 However, there

is thought to be a wide spectrum of presentations.33

Potential Signs of Complex Gastroschisis

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As previously mentioned, outcomes of gastroschisis are influenced by the presence or

absence of intestinal complications.10, 33 A recent meta-analysis by Bergholz et al demonstrated

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that neonates with CG, defined by the authors as gastroschisis plus intestinal atresia, perforation,

necrosis, or volvulus, have an eight-fold increased risk of death.10 Additionally, the affected
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infants were at increased risk for prolonged hospital stay, longer time to first and full enteral
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feeds, longer duration of parenteral nutrition, short gut syndrome, and necrotizing enterocolitis.

Because of the significantly increased morbidity and mortality among neonates with complex
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versus simple gastroschisis, research efforts have focused on the study of antenatal,

ultrasonographic findings that may be potential signs of CG. Some of these findings include
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bowel dilation, polyhydramnios, gastric dilation, and bowel wall thickness.

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Bowel Dilation

Assessment of bowel dilation has been the most extensively reviewed finding with
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respect to its utility in predicting cases of CG. Specific features of bowel dilation and their

relationship to CG have also been separately assessed in various studies. Such features include
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location of dilated bowel, extent of dilation, gestational age at onset of dilation, and change in

dilation.
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Location of bowel dilation

Figure 8 shows an example of fetal gastroschisis with intraabdominal bowel dilation

(IABD), while Figure 9 depicts a case of fetal gastroschisis with extraabdominal bowel dilation

(EABD). The specific location of bowel dilation as a predictor of CG and can be divided into

studies evaluating the presence of IABD, EABD, and/or bowel dilation of unspecified location.

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The conclusions are somewhat conflicting and may be due to the fact that the definition of bowel

dilation varies widely from study to study.35 With respect to IABD, a number of studies have

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shown a strong correlation between IABD and CG. Kuleva at al11 performed a single center

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retrospective review of 105 cases of gastroschisis, investigating antenatal ultrasound predictors

of CG, which was defined as atresia, stenosis, perforation, necrosis, or volvulus. IABD, defined
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as dilation > 6mm in this study, was found to be a consistent marker for CG with an odds ratio

(OR) of 4.13 and 95% confidence interval (CI) of 1.32-12.9. Other single center, retrospective
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cohort studies have yielded similar findings.14, 15, 17, 18 In addition, a meta-analysis of 9 studies

by D’Antonio et al35 similarly reported a positive association between IABD and bowel atresia
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(OR 5.48, 95% CI 3.1-9.8).

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In contrast, only a small number of studies have shown a positive association between

EABD and CG.13, 36 In one study by Sinkey et al,36 EABD was associated with a 12-fold
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increased risk of atresia or necrosis (OR 12.14, 95% CI 2.82-52.27). While this may seem

convincing, the overwhelming majority of studies have shown no association.11, 19, 35 In the
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study by Kuleva et al,11 EABD was seen in the vast majority of infants, and in fact, more infants

with simple gastroschisis (93%) had EABD, as compared to those with complex gastroschisis

(85%). In a meta-analysis of 10 studies, published by D’Antonio,35 EABD was not shown to

have a significantly increased odds of bowel atresia (pooled OR 1.34, 95% CI 0.8-2.3). Tower et
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al19 performed a systematic review of 10 studies between 1980 and 2007. Although this analysis

did not specifically use features of complex gastroschisis as end points, their findings suggested

that fetuses with gastroschisis and EABD, defined as 10-18mm, were not at increased risk for

IUFD, neonatal death, or bowel resection. A sigmoid colon distended with meconium is

commonly found in neonates with gastroschisis at birth, which may explain the frequent finding

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of inconsequential EABD on ultrasound.

When bowel dilation was collectively evaluated, without specification of location, no

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association with CG was found.20, 37, 38 Overcash et al37 performed a retrospective review of 191

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pregnancies complicated by gastroschisis within the five institutions of the University of

California Fetal Consortium from 2007-2012. IUGR, oligohydramnios, and bowel dilation were
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not associated with adverse outcomes, and 45% had evidence of bowel dilation on the last

ultrasound prior to delivery. Similarly, in the study by Wilson et al,20 the incidence of bowel
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dilation was common (54%), and the authors were not able to show an association between

bowel dilation and intestinal complications. The incidence of bowel dilation was even higher in
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a single center, retrospective review by Ghionzoli et al.38 In this study, 130 infants born with
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gastroschisis were examined in order to assess the association, if any, between antenatal bowel

dilation and diagnosis of intestinal atresia. The authors defined dilation as ≥18 mm, resulting in
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the diagnosis of fetal bowel dilation in 67% of infants. While its presence was not found to be

predictive of bowel atresia with a positive predictive value (PPV) of 17%, 95% CI 0.1-0.3, its
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absence was highly predictive of the absence of bowel atresia with a negative predictive value

(NPV) of 99%, 95 % CI 0.9-0.99. The absence of bowel dilation, therefore, appears to strongly

correlate with the absence of CG.

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Extent of bowel dilation

Extent of bowel dilation has also been queried. Garcia et al39 performed a retrospective

review of 94 pregnancies. Extra-abdominal bowel transverse diameter was measured prior to

delivery, with bowel dilation varying from 6-25 mm at 34-36 weeks’ gestation. Bowel

complications occurred in 8/94 cases, of which 5 had bowel dilation ≥ 25 mm. Also, bowel

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dilation ≥ 25 mm was associated with a lower rate of successful primary closure, longer time to

full enteral feeds, longer length of stay and yielded a sensitivity of 28%, positive predictive value

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of 38%, and negative predictive value of 87% for IUFD, neonatal death, or bowel complications

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(necrosis, perforation, atresia, volvulus). Although their findings may lead one to consider early

delivery if bowel dilation ≥25 mm is detected, the authors suggest placing this finding in the
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context of gestational age.

Temporal onset of bowel dilation

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Lato et al40 did further work, evaluating the predictive value of the temporal onset of

small bowel dilation in the prediction of intestinal atresia and postnatal complications. In a
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retrospective observational study, 78 cases of gastroschisis with small bowel dilation were

assessed and divided into the following groups: ≥10 mm at < 27 weeks, ≥10 mm at 27-30 weeks,
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and ≥ 18 mm at ≥ 30 weeks. Bowel atresia was significantly correlated with bowel dilation in all
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groups. The best predictor of bowel atresia was ≥ 10mm at <30 weeks with sensitivity of 89%

and specificity of 79%.

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Change in bowel dilation

Change in bowel dilation may also be an indicator of CG. One report by Chung et al41

described a case in which intestinal rupture was suspected, as evidenced by sudden

decompression of previously dilated bowel and the presence of meconium on amniocentesis.

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These findings prompted induction of labor at 36 weeks’ gestation. The patient failed to achieve

active labor and was ultimately delivered via cesarean. The neonate was subsequently diagnosed

with severe bowel compromise and colonic atresia with gangrenous changes.

Bowel dilation in simple gastroschisis

While in utero bowel dilation has been extensively studied for its potential to detect

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postnatal cases of complex gastroschisis, it has also been evaluated in the context of simple

gastroschisis cases.42 In a study by Youssef et al, cases of simple gastroschisis were evaluated

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for antecedent bowel dilation on prenatal ultrasound. Its presence, among simple gastroschisis

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cases, was more likely to be associated with longer need for total parenteral nutrition (OR 2.06,

95% CI 1.31-3.24) and was more likely to result in longer hospital stay (OR 1.79, 95% CI 1.16-
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2.75). Of note, the location of the bowel dilation, whether intraabdominal or extraabdominal,

was not specified in this particular study.

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Other markers of bowel dilation

In a retrospective review by Martillotti et al,17 4/6 cases of volvulus were suspected

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prenatally based upon the ultrasound image of volvulus with a loop of dilated bowel showing a

“coffee bean sign,”43, 44 although this is not regularly reported in the literature as a consistent
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marker of CG.
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Polyhydramnios

The presence of polyhydramnios in pregnancies complicated by gastroschisis is thought

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to be the result of exposed bowel having reduced ability to reabsorb amniotic fluid. Figure 10

demonstrates polyhydramnios, with an amniotic fluid index (AFI) of 28.1 cm, in a fetus with

gastroschisis. In this case, the polyhydramnios was diagnosed at 35 1/7 weeks. Similar to bowel

dilation, studies examining the association of polyhydramnios and CG have yielded mixed
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findings. In a metanalysis by D’ et al,35 the presence of polyhydramnios was a positive

predictor of bowel atresia with an OR 3.76, 95% CI 1.7-8.3. Japarj et al16 performed a

retrospective review of pregnancies complicated by gastroschisis. Of the 45 patients, 6 had

severe bowel complications, defined as atresia, perforation, or necrosis, and polyhydramnios was

most significantly associated with these complications, with a likelihood ratio of 11.7 (p=.001).

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Findings were similar in retrospective reviews by Ghionzoli et al38 and Andrade et al.13

However, in a retrospective study by Huh et al,15 there was no significant difference in the rate of

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polyhydramnios between infants with and without CG

Gastric dilation
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An example of gastric dilation in a fetus with gastroschisis is presented in Figure 11. At
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the time of diagnosis, the gestational age of the fetus was 32 5/7 weeks, and the fetal stomach

measured nearly 6 cm in its greatest dimension. In comparison to bowel dilation, fewer studies
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have evaluated the presence of gastric dilation as a potential marker for CG. Sinkey et al36

performed a single center retrospective cohort review of 154 cases and found that an abnormally
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dilated stomach or an abnormally positioned stomach was associated with perinatal loss with an
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of OR 11.2, 95% CI 2.1-58.33 for abnormal gastric bubble and an OR of 17.1, 95% CI 2.99-

97.85 for abnormal stomach location. In a single center retrospective study of 98 fetuses with
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gastroschisis by Alfaraj et al,45 32.7% of cases had gastric dilation, which predicted meconium-

stained amniotic fluid at delivery. However, it was not predictive for any other adverse perinatal
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outcomes. Finally, a meta-analysis by D’Antonio et al35 found a positive association between

gastric dilation and neonatal death (OR 5.58, CI 1.3-24.1) but not bowel atresia.

Bowel wall thickness

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In Figure 12, thickened loops of free-floating bowel in the amniotic cavity are shown in a

fetus with known gastroschisis at 34 1/7 weeks’ gestation. In a small retrospective review by

Heinig et al,46 small bowel thickness >25 mm was found in 2/17 cases, and both were associated

with adverse postnatal condition of the bowel with a PPV of 33.3%, 95% CI 1-90.5 and a NPV

of 91%, 95% CI 58.7-99.8%. Another small study by Langer et al47 evaluated bowel wall

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thickness and adverse neonatal outcomes. Although this study did not specifically evaluate its

association with CG, bowel wall thickness >3mm was associated with increased time to oral

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feeding, but this association was not statistically significant (p=0.12).

Guidelines for Delivery US

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Antepartum Surveillance

It is important to remember that delivery timing is subject to indicators of fetal well-

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being. In general, it is recommended that all fetuses with gastroschisis be monitored with serial

growth ultrasounds because of the risk for IUGR, defined as estimated fetal weight <10 %ile. In
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one study, the incidence of IUGR was reported to be approximately 34%.7 Since both IUGR and
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the presence of gastroschisis may be independent risk factors for adverse neonatal outcomes and

impact surveillance and timing of delivery, it is paramount to obtain accurate information on

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fetal growth.

Interestingly, there is a retrospective cohort review by Overcash et al37 in which growth

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restriction was not found to be associated with adverse fetal outcomes. These results may be due

to the fact that a small abdominal circumference in itself may be an indicator of IUGR.48

Therefore, there has been some concern that the use of the biometric parameter of abdominal

circumference may skew estimation of fetal weight in fetal gastroschisis and over-estimate the
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incidence of IUGR, since the abdominal circumference may be small as a result of the defect

itself. To address this question, a recent multicenter cohort study by Zaki et al49 ascertained that

amongst several different formulae used for estimating fetal weight, the Hadlock formula, even

with the inclusion of abdominal circumference in the calculation, was the most accurate for

identifying IUGR in fetuses with gastroschisis.

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In addition to serial growth ultrasounds, twice weekly antepartum fetal heart rate testing

is generally instituted at 32-34 weeks in the absence of other indications for earlier evaluation,

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such as IUGR or oligohydramnios. Showcasing the importance of antepartum surveillance of

fetal well-being, a retrospective, observational study by Perry et al50 demonstrated that delivery
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was expedited in 35.4% of pregnancies complicated by gastroschisis for suspected fetal
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compromise. Of the infants delivered before the intended delivery date, 36% were delivered

based on concerning growth ultrasound findings, 28.1% based on concerning fetal heart rate
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tracing, and 32.8% based on self-reported, decreased fetal movement.

Timing of delivery
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There is much debate as to the timing of delivery in pregnancies complicated by

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gastroschisis. A majority of the literature aims to address whether there is an indication for

preterm delivery (<37 weeks), and if not, is early term delivery (37-38 6/7 weeks) versus
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expectant management until full term (≥39 weeks) associated with better neonatal outcomes.51

Preterm delivery (<37 weeks)

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One randomized controlled trial by Logghe and colleagues52 evaluated the effect of

elective preterm delivery at 36 weeks versus expectant management. Although their study was

small (N=42), there was neither significant benefit nor adverse effects of elective preterm

delivery. Likewise, a prospective cohort study by Al-Kaff et al53 also demonstrated no benefit in
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planned delivery at 36-37 weeks as compared to planned delivery at ≥ 38 weeks. A large

retrospective cohort study by Carnaghan et al54 concluded that elective delivery before 37 weeks

was not associated with change in time to full enteral feeds but was instead associated with

increased incidence of neonatal sepsis and prolonged length of stay. Cain et al55 performed a

large, multi-institutional, retrospective cohort study including 1005 neonates with gastroschisis.

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Not surprisingly, they noted significantly decreased rates of jaundice and respiratory distress

syndrome when comparing those born preterm (<34 weeks) to those born early term (37-38

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weeks). As gestational age increased at time of birth, they also noted a decreased length of stay

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and lower inpatient medical cost. In contrast, a systematic review and meta-analysis by Landisch

et al,56 suggested that elective preterm delivery (<37 weeks) was associated with shorter time to
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first enteral feed and decreased risk of neonatal sepsis. However, this result was not seen in their

other cohort of those who either delivered spontaneously or had an indicated preterm delivery.
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Early term vs. full term delivery

Recent studies have focused on the precise timing of delivery at term, and if there is any
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benefit to an early term delivery (37 0/7-38 6/7 weeks) versus a full term delivery (39 0/7-40

6/7).51 A retrospective cohort review by Baud et al57 assessed outcomes in neonates whose
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mothers underwent induction of labor at 37 weeks versus expectant management. They found
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that there were more cases of bowel damage (atresia, stricture, perforation, necrosis) (18.4% vs

7.8%, p=0.05), neonatal sepsis (42% vs 25%, p=0.02) and composite outcome of neonatal death
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and bowel damage, defined as necrosis, atresia, perforation, adhesion (21% vs 8%, p=.02) in the

expectantly managed group. There was no significant difference in emergency cesarean delivery

rates, respiratory distress syndrome, or 5 minute Apgar <7 between the groups. Sparks et al58

performed a retrospective cohort study of singleton pregnancies affected by gastroschisis and

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evaluated the risk of stillbirth and infant death for delivery compared to expectant management

for each gestational age week beginning at 35 weeks. From 37-39 weeks, the relative risk of

death grew with each week of expectant management peaking at 1.9 (CI 1.73-2.08) at 39 weeks,

with a number needed to deliver of 17.49 (CI 15.34-20.32) to prevent one death. A recent cost-

effective analysis was performed by Harper et al59 indicating that delivery at 38 weeks may

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optimize the balance between the risk of respiratory distress syndrome, incurred with earlier

delivery, and the risk stillbirth, a potential complication of continued expectant management.

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Route of delivery

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Early studies suggested that cesarean delivery improved outcomes for gastroschisis.

Benefits of cesarean delivery were thought to include the potential for a reduced risk of bowel
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injury and/or contamination by vaginal flora during the labor process and the allowance for

better coordination between timing of delivery and postnatal care by the neonatal intensivists and
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pediatric surgeons.60, 61 However, subsequent studies have not shown benefit to cesarean

delivery with respect to neonatal outcomes.60, 61 In a systematic review published in 2001 by

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Segel et al, the authors examined the results of 27 studies in which neonatal outcomes for infants

with abdominal wall defects were reported with respect to mode of delivery.61 The review did
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not show an increased risk of complications with vaginal delivery, such as ischemic bowel, small
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bowel obstruction, necrotizing enterocolitis, or sepsis, and pediatric mortality was similarly

unaffected. Thus, the authors concluded that the evidence does not support a policy of cesarean
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delivery for all infants with abdominal wall defects.61

In a population-based cohort study by Friedman et al,60 the percentage of women

undergoing attempted vaginal delivery appears to have increased from 2005 to 2013, suggesting

that the literature demonstrating no benefit to cesarean delivery among pregnancies complicated
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by gastroschisis may be diffusing into clinical practice.61-63 However, in spite of this apparent

trend, a recent analysis of gastroschisis outcomes in North America showed that cesarean

deliveries rates in patients with fetal gastroschisis remain high, particularly in the United States

(U.S.), where the rate of cesarean delivery was reported to be 60.9%, nearly two times the

Canadian rate.64 This may be due, in part, to the continued high rate of planned cesarean

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deliveries for pregnancies with fetal gastroschisis, despite a trend towards more attempted

vaginal deliveries.60 Further, although attempted vaginal delivery may be considered more

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frequently, the high rate of IUGR and intrapartum fetal heart rate abnormalities among fetuses

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with gastroschisis may contribute to the continued high cesarean rate, despite a greater number

of women undergoing a trial of labor.5-7 Even though cesarean deliveries rates remain high for
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pregnancies complicated by gastroschisis, planned cesarean delivery in the absence of the usual

obstetrical indications is not generally recommended, due to the associated maternal morbidity of
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the procedure. Rather, indications for cesarean delivery in pregnancies complicated by

gastroschisis should be the same as those with uncomplicated pregnancies.

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Fetal Interventions

To date, no large clinical trials of fetal therapy for pregnancies complicated by

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gastroschisis have been completed. However, several small human studies, animal studies, and

case reports have explored the role of fetal therapy.

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Fetoscopic surgery

Although no human studies exist, there have been several animal studies exploring the

feasibility and efficacy of fetoscopic surgery for the treatment of gastroschisis. In two studies

using sheep models,65, 66 herniated bowel was unable to be returned to the abdominal cavity.
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17

However, a small study by Stephenson et al67 demonstrated that repair of gastroschisis was

possible (in 2 out of 6 sheep models) and resulted in resolution of inflammatory changes that

were initially noted at time of repair.

Diuretic use

One theory of the mechanism of bowel damage is contact with meconium in the amniotic

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fluid. Hakguder et al68 theorized that injection of intraamniotic furosemide may lead to lower

intraamniotic meconium concentration and, thus, decrease bowel damage. In a rat model with

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iatrogenically-created gastroschisis, intraamniotic injection of furosemide was associated with

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decrease in intestinal serosal thickness. To date, this is the only study exploring this potential

therapy.
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Nitric oxide (NO)

Given NO involvement in wound healing, Goncalves et al69 explored the use of a NO (S-
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nitrosoglutathione, GSNO) donor group in treatment of gastroschisis. After inducing

gastroschisis in rats, varying concentrations of GSNO were applied to the bowel. GSNO (at low
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concentrations) significantly reduced intestinal diameter and recovery of the serosal layer.
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Stem cell therapy

In a study by Feng et al,70 transamniotic stem cell therapy with amniotic fluid
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mesenchymal stem cells mitigated, although did not prevent, bowel damage in a rodent model of

gastroschisis. Their findings were replicated in rabbit models.71

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Amniotic fluid exchange

Amniotic fluid exchange has been the most extensively studied maneuver, with several in

vivo case reports72, 73 and other studies. Results have been mixed. A case-control trial by Luton

at al74 evaluated the effect of exchange of amniotic fluid for normal saline. There were no
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clinically significant differences between the case and control groups in regards to duration of

mechanical ventilation, length of stay in the neonatal intensive care unit (NICU), total

hospitalization, or time to oral nutrition or full feeds. However, a case series of serial amniotic

fluid exchanges in the 3rd trimester by Midrio et al75 showed that the exchanges did not modify

biochemical or inflammatory status of fluid or prevent injury to herniated gut as evidenced by

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time to full feeds.

Current clinical trials

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A review of ClinicalTrials.gov reveals two ongoing clinical trials with respect to the

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antenatal care and management of gastroschisis. The first is a French trial, known as the Trial of

Amnioechange in Gastroschisis Affected Fetuses.76 This study aims to explore the benefit of
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performing an exchange of amniotic fluid for normal saline every two weeks, beginning at 30

weeks’ gestation with the primary outcome of duration of need for ventilatory support and
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duration of parenteral nutrition. The intended recruitment goal is 140 participants, but the status

of the trial is unknown, as trial information has not been updated on the ClinicalTrials.gov
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website since 2011.

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The second trial, the Gastroschisis Outcomes of Delivery (GOOD) Study, is currently

undergoing recruitment.77 This is an ambitious study, aiming to prospectively enroll 800

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participants from multiple centers in North America. The purpose is to determine the optimal

gestational age for the delivery of uncomplicated gastroschisis cases and will test the hypothesis
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that delivery at 35 0/7 - 35 6/7 weeks is superior to observation and expectant management with

a goal of delivery at 38 0/7 - 38 6/7 weeks.

Conclusion
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In conclusion, fetal gastroschisis can be suspected in the antenatal period by elevation in

maternal serum alpha fetal protein on 2nd trimester genetic screening and characteristic

ultrasound findings. Ultrasound in the detection of fetal gastroschisis is most reliable in the

second trimester, although diagnosis in the first trimester has been reported. CG cases, defined as

the presence of bowel atresia, stenosis, perforation, or ischemia, have higher risks of postnatal

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complications and death. Although there are no clear-cut guidelines by which to elucidate CG

prior to delivery, ultrasonographic markers such as location, extent, and onset of bowel dilation,

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changes in bowel dilation, gastric dilation, and bowel wall thickness may be used to alert the

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pediatric surgery team as to the level of clinical concern for CG. Of the sonographic markers

studied, IABD seems to be the most reliable for the antenatal detection of CG, whereas the most
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convincing marker for ruling out CG is the absence of any bowel dilation. Antenatal

surveillance includes serial growth ultrasound, due to the increased risk of IUGR, and the
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institution of antepartum fetal heart testing by 32-34 weeks’ gestation. With respect to timing of

delivery, current consensus appears to suggest delivery in the early term gestational period (37
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0/7 – 39 0/7 weeks), but preterm birth may be warranted for evidence of fetal compromise.
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Regarding route of delivery, vaginal delivery is recommended, with cesarean generally reserved

for the usual obstetrical indications. However, cesarean delivery rates remain increased for
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gastroschisis cases, likely due to a continued high rate of planned cesarean deliveries and high

risk of IUGR and intrapartum fetal heart rate abnormalities.

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Figure Legends

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Figure 1. Transverse view of the fetal abdomen with a normal cord insertion

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Figure 2. Transverse view of the fetal abdomen with gastroschisis

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Figure 3. Sagittal view of a fetus with suspected gastroschisis at 12 5/7 weeks’ gestation
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Figure 4. Sagittal view of the same fetus in Figure 3, now at 19 0/7 weeks’ gestation. Previously

suspected gastroschisis was confirmed.

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Figure 5. Transverse view of the fetal abdomen in a fetus with omphalocele. An abdominal wall

defect is seen that is enclosed by a membranous sac. The fetal stomach is noted to be pulled into
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Figure 6. Transverse view of the fetal abdomen in a fetus with gastroschisis without the use of

color flow Doppler.

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Figure 7. Transverse view of the fetal abdomen in the same fetus with gastroschisis that was

shown in Figure 6. In this image, color flow Doppler is used to delineate the umbilical cord
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insertion and the adjacent, free-floating extraabdominal bowel. Color flow Doppler also show

the umbilical arteries as they travel on either side of the fetal bladder, ultimately exiting at the

umbilicus.
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Figure 8. Gastroschsisis with intra-abdominal bowel dilatation.

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Figure 9. Gastroschisis with extra-abdominal bowel dilatation.

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Figure 10. Fetus with gastroschisis at 35 1/7 weeks’ gestation with polyhydramnios and

amniotic fluid volume of 28.1 cm. Debris was noted in the fluid, most prominently shown in
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quadrant 4 (Q4), which was suggestive of meconium. Meconium-stained amniotic fluid was

confirmed at time of delivery.

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Figure 11. Fetus with gastroschisis at 32 5/7 weeks’ gestation. The fetal stomach was noted to

be distended, measuring 5.59 cm in the greatest dimension.

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Figure 12. Fetus with gastroschisis at 34 1/7 weeks’ gestation. Thickened loops of free-floating

bowel in the amniotic cavity were noted.

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