Volume 71, Number 9

OBSTETRICAL AND GYNECOLOGICAL SURVEY

Copyright 2016 Wolters Kluwer Health,
Inc. All rights reserved. CME REVIEW ARTICLE
CHIEF EDITOR'S NOTE: This article is part of a series of continuing education activities in this Journal through which a total of
24
36 AMA PRA Category 1 CreditsTM can be earned in 2016. Instructions for how CME credits can be earned appear on the last
page of the Table of Contents.

Gastroschisis: A Review of
Management and Outcomes
Rachel V. O'Connell, BS,* Sarah K. Dotters-Katz, MD,
Jeffrey A. Kuller, MD, and Robert A. Strauss, MD
*Medical Student, UNC School of Medicine, Chapel Hill, NC; Clinical Fellow, Division of Maternal Fetal Medicine,
Department of Obstetrics and Gynecology, University of North Carolina at Chapel Hill, Chapel Hill, NC; Professor, Division of
Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Duke University, Durham, NC; and Professor, Division of
Maternal Fetal Medicine, Department of Obstetrics and Gynecology, University of North Carolina at Chapel Hill, Chapel Hill, NC

We performed an evidence-based review of the obstetrical management of gastroschisis. Gas-

troschisis is an abdominal wall defect, which has increased in frequency in recent decades. There is
variation of prevalence by ethnicity and several known maternal risk factors. Herniated intestinal
loops lacking a covering membrane can be identified with prenatal ultrasonography, and maternal serum
-fetoprotein level is commonly elevated. Because of the increased risk for growth restriction, amniotic
fluid abnormalities, and fetal demise, antenatal testing is generally recommended. While many studies
have aimed to identify antenatal predictors of neonatal outcome, accurate prognosis remains challeng-
ing. Delivery by 37 weeks appears reasonable, with cesarean delivery reserved for obstetric indications.
Postnatal surgical management includes primary surgical closure, staged reduction with silo, or
sutureless umbilical closure. Overall prognosis is good with low long-term morbidity in the majority
of cases, but approximately 15% of cases are very complex with complicated hospital course, extensive
intestinal loss, and early childhood death.
Target Audience: Obstetricians, Maternal Fetal Medicine Providers, Certified Nurse Midwives, and Family
Medicine Providers.
Learning Objectives: After participating in this activity, the reader should be able to describe common preg-
nancy complications associated with gastroschisis; discuss options for prenatal and antenatal fetal surveillance;
counsel parents regarding prenatal predictors of neonatal outcome and long-term prognosis; and describe the
evidence-based recommendations for timing and mode of delivery.

BACKGROUND is a full-thickness right-sided periumbilical abdominal

Gastroschisis, derived from the Greek words gaster
meaning belly and schisis meaning cleft, was first de-
scribed in the literature by Calder in 1733.1 Gastroschisis
All authors and staff in a position to control the content of this CME
activity and their spouses/life partners (if any) have disclosed that they
have no financial relationships with, or financial interests in, any com-
mercial organizations pertaining to this educational activity.
Correspondence requests to: Sarah K. Dotters-Katz, MD, Division of
Maternal Fetal Medicine, Department of Obstetrics and Gynecology,
University of North Carolina at Chapel Hill, 3010 Old Clinic Building,
Campus Box 7516, Chapel Hill, NC 27599-7516. E-mail: sarah.
dotters-katz@duke.edu.
www.obgynsurvey.com | 537
wall defect that results in the herniation of abdominal
structures (bowel) out of the fetal abdominal cavity, ex-
posing the organs to amniotic fluid in utero.
Gastroschisis occurs early in embryologic develop-
ment, approximately 6 to 10 weeks after conception.
However, the exact pathogenesis is unknown and vari-
ous theories have been suggested. Some authors postu-
late that there is a failure of the mesoderm to form the
body wall,2 while others suggest that gastroschisis is
due to the rupture of amnion around the umbilical ring.3
Still others have surmised there is abnormal involution
of the right umbilical vein leading to weakening of the
body wall, and disruption of the right vitelline artery

Copyright 2016 Wolters Kluwer Health, Inc. All rights reserved.

538 Obstetrical and Gynecological Survey

or yolk sac artery, with subsequent body wall damage.4
Finally, other theories include abnormal body wall fold-
ing, gene polymorphisms, maternal immune response
to new paternal antigens, and use of medications such
as aspirin or pseudoephedrine in the first trimester.5,6
The prevalence of gastroschisis, approximately 4 in
10,000 live births, has been steadily rising over recent
decades. However, this is believed to be due to more
accurate pregnancy screening rather a true increase in
occurrence rates.7,8 Prevalence varies by ethnicity,
and while some studies found a higher prevalence in
the Hispanic population compared with non-Hispanic
whites,911 other studies concluded the opposite.12 While
localized clustering of gastroschisis births has been ob-
served in areas such as the rural southern Piedmont of
North Carolina, even after controlling for major risk
factors,13 no difference in geographic risk factors have
been identified.14
Risk factors associated with gastroschisis include ma-
ternal age younger than 20 years, cigarette smoking,7,1517
recreational drug use such as cocaine, alcohol con-
sumption, low body mass index,18 and first trimester
genitourinary tract infections.19 Young maternal age is
one of the strongest associated risk factors, and accord-
ing to 1 large study, women younger than age 20 years
were approximately 7 times more likely to have an in-
fant with gastroschisis when compared with women
aged 25 to 29 years (95% confidence interval, 6.51
7.92).12 Women who smoke cigarettes have 2.07
(1.333.23) times the risk of having a pregnancy with
gastroschisis. In a study of mothers younger than 20
years, prior pregnancy loss was a significant risk factor
for gastroschisis (odds ratio [OR], 2.34; 1.373.97).20
In another study, women with an interpregnancy inter-
val less than 12 months were at increased risk for a
gastroschisis pregnancy (OR, 1.7; 1.12.5), and this as-
sociation was enhanced if the pregnancy was after a ter-
mination or miscarriage (OR, 2.5; 1.15.6) or if the
woman resided in northern study areas with winter/
fall conception (OR, 2.8; 1.35.9).21 In a large analysis
of data from the National Birth Defects Prevention
Study, paroxetine use was associated with gastroschisis
(2.5; 95% confidence interval, 1.24.8).22 The relation-
ship between most risk factors and the pathogenesis is
not known, with the exception of the vasoconstrictive
exposures, such as cocaine, amphetamines, deconges-
tants, and nicotine.23
Prenatal Diagnosis
When an abdominal wall defect is identified on ultra-
sound, gastroschisis and omphalocele are the 2 most
common etiologies. Physiologically, omphalocele dif-
fers from gastroschisis in that the herniation of internal
organs is enclosed in a sac made of 3-layers of perito-
neum. Other major differences between gastroschisis
and omphalocele are described in Table 1. The differen-
tial diagnosis of abdominal wall defect also includes
other less likely etiologies, including cloacal exstrophy,

TABLE 1
Comparison of Gastroschisis and Omphalocele

Gastroschisis Omphalocele
Definition Gaster = belly; schisis = cleft Omphalos = umbilicus; cele = hernia
Full thickness abdominal wall defect, to the right of the Herniation of internal organs through an umbilical defect,
umbilicus, with extruded intestinal loops without a contained in a 3-layer membrane
membranous covering
Usually contains small intestines, liver, spleen, colon,
sometimes gonads
Incidence 4 per 10,000 pregnancies 4 per 10,000 pregnancies
Risk factors Maternal young age, low BMI, smoking More common in women <20 or >40 y
Associated anomalies Rare24,25 Frequent2426
Pathogenesis Theories include failure of the mesoderm to form the Failure of bowel loops that do not return to abdominal
body wall, rupture of amnion around the umbilical ring, cavity after embyologic herniation of umbilical cord
abnormal involution of the right umbilical vein leading at weeks 610 gestation
to weakening of the body wall, and disruption of the
right vitelline artery, or yolk sac artery, with subsequent
body wall damage5
Ultrasound Findings Defect often to right of umbilical cord; herniated bowel Defect is centrally located, herniated loops of bowel
lacks membranous covering and sometimes liver, with covering membrane
If sac is ruptured, it may appear like gastroschisis but with
abnormal umbilical cord and a remnant omphalocele sac.
Genetics 0.1% have abnormal karyotype27 30%40% have abnormal karyotype
High rate of termination of pregnancy (30%50%) due to
associated anomalies
BMI, body mass index.

Copyright 2016 Wolters Kluwer Health, Inc. All rights reserved.

 Gastroschisis: Management and Outcomes CME Review Article
limb-body wall complex, ectopia cordis, ruptured
omphalocele, hernia of the cord, and urachal cyst.
However, the diagnosis is usually clear on ultrasound
examination.
Historically, the diagnosis of gastroschisis was often
suspected in the setting of an elevated maternal serum
-fetoprotein (msAFP) level due to increased passage
of -fetoprotein from the fetus into the amniotic fluid
via the abdominal wall defect. The msAFP tends to be
approximately 7.0 multiples of the median (3.5
13.5).28 However, as ultrasound has improved, the di-
agnosis is more commonly made with early ultrasound.
For women who do not have an early ultrasound and
present with an elevated msAFP level, gastroschisis
should be excluded. Ultrasound findings consistent
with this diagnosis include a paraumbilical abdominal
wall defect with intestinal loops protruding through
the defect. This defect is usually right of midline. The
stomach can be either dilated or herniated through the
abdominal wall defect.2931 The herniated intestinal
loops lack a covering membrane, and therefore float
freely in the amniotic fluid. Late in pregnancy, ultra-
sound findings may include thickened or matted bowel
wall due to chronic exposure to the amniotic fluid. Other
findings may include intra-abdominal bowel dilation
or extra-abdominal bowel dilation.32 Polyhydramnios
can be seen, and if it associated with bowel dilation, it
may suggest intestinal atresia.33
Chromosomal abnormalities are rare in gastroschisis,
with only 0.1% of infants found to have an abnormal
karyotype.27 Therefore, in the absence of nongastro-
intestinal structural abnormalities, fetal genetic evalua-
tion does not appear warranted. However, if a patient
with a major structural abnormality undergoes invasive
prenatal diagnostic testing, chromosomal microarray
analysis may be considered.34 However, very limited
data exist on the genetic profiling of gastroschisis.35
Associated congenital anomalies can occur; however,
variation from 8%24 to 32%25 has been reported. These
anomalies are most commonly gastrointestinal, includ-
ing malrotation, atresia or stenosis of the bowel, supe-
rior mesenteric artery disruption, Meckel diverticulum,
gallbladder atresia, and bladder herniation.2426 In
rare instances, gastroschisis has been associated with
amyoplasia,36 which may further support vascular
disruption theories, and with cases of Smith-Lemli-
Opitz syndrome. Recurrence risk in subsequent off-
spring has been reported as high as 2.4%.37
Natural History
Pregnancies complicated by gastroschisis are at
high risk for adverse perinatal outcomes, including
Copyright 2016 Wolters Kluwer Health, Inc. All rights reserved.
539
growth restriction, polyhydramnios, oligohydramnios,
spontaneous preterm delivery, and stillbirth. Gastroschisis
does not seem to be associated with an increased risk
of preeclampsia or gestational diabetes.
The rate of fetal growth restriction in gastroschisis is
high, ranging from 30% to 60%. Using traditional bi-
ometry, a fetus with gastroschisis will have reduced
abdominal circumference. Thus, making the diagno-
sis of fetal growth restriction in these fetuses difficult,
with both high false-positive and false-negative rates
reported in the literature.38 Chaudhury and colleagues
reported that the Hadlock formula systematically
underestimated birth weight because the formula
heavily weights the abdominal circumference. The
Siemer39 and Shepard40 formulas had the lowest per-
centage of error between estimated fetal weight and
birth weight.41 The Siemer formula does not include
abdominal circumference, the Shepard formula mod-
erately weights the abdominal circumference, and the
Hadlock formula heavily weights the abdominal cir-
cumference. Table 2 shows the various sensitivities,
specificities, and positive predictive values of these
formulas, as reported in the study by Chaudhury
et al.41 Girsen et al42 reported that accurate prediction
of fetal weight is important because infants with
gastroschisis who were small for gestational age at
birth had a 4-fold increase in odds for prolonged
length of stay, independent of gestational age. The
pathophysiology of fetal growth restriction in cases
of gastroschisis may not be related to placental insuf-
ficiency. An alternative mechanism may relate to loss
of fluid and protein across the exposed bowel. Dixon
and colleagues43 found that bowel atresia appears to be
protective against fetal growth restriction. They postu-
lated that the transmural loss of proteins may be greater
when the herniated bowel is patent rather than atretic
because patent bowel has an intact vascular supply
and greater surface area from which to lose protein.
Pregnancies complicated by gastroschisis appear to be
at increased risk of both oligohydramnios and poly-
hydramnios. Oligohydramnios affects 10% to 25%
of cases with gastroschisis.44,45 Polyhydramnios,
while less common than oligohydramnios, is associ-
ated with bowel atresia (OR, 3.76; 1.78.3).33
TABLE 2
Test Characteristics of Ultrasound Formulas for Predicting Fetal
Growth Restriction Less Than the 10th Percentile41 (n = 62)
Formula Sensitivity Specificity PPV NPV Accuracy
Hadlock 0.89 0.70 0.55 0.94 0.74
Shepard 0.78 0.84 0.67 0.92 0.82
Seimer 0.67 0.86 0.67 0.86 0.80
Accuracy = (sensitivity) (prevalence) + (specificity) (1 prevalence).
PPV, positive predictive value; NPV, negative predictive value.
540 Obstetrical and Gynecological Survey
Gastroschisis is associated with a high rate of both
spontaneous and iatrogenic preterm birth. Rates of
spontaneous preterm birth range from 28% to 61%46,47
Fetal heart rate abnormalities in the third trimester have
been reported as high as 43%, contributing to a high
rate of iatrogenic preterm delivery.48
Fetal demise is more common in pregnancies compli-
cated by gastroschisis than nonanomalous pregnancies.
A recent meta-analysis reported a pooled prevalence
of 4.48 per 100 gastroschisis pregnancies49 compared
with 0.6 per 100 in the general population. Reports
from the early 1990s estimated stillbirth rates as high
as 12.5%, but more recent studies report rates approxi-
mately 4.5%.49 The meta-analysis concluded that the
overall risk of intrauterine fetal demise (IUFD) is greatest
before 36 weeks' gestation, and that once a fetus reaches
35 weeks' gestation, it has already accumulated most
of the risk of IUFD. In contrast, a more recent study
by Meyer and colleagues50 found that the weekly ongo-
ing risk of IUFD significantly increases after 38 weeks.
The cause of an increased risk of stillbirth among fe-
tuses with gastroschisis remains unclear. Some specu-
late either midgut volvulus or progressive cord
compression by eviscerated bowel may play a role.51
Many retrospective studies have been dedicated to
identifying clinically significant prenatal ultrasound
predictors of neonatal morbidity to allow for risk
stratification.29,30,5265 A recent meta-analysis of 2023
gastroschisis fetuses aimed to summarize this large
body of data, finding that intra-abdominal bowel dila-
tion and polyhydramnios detected on prenatal ultra-
sound were associated with bowel atresia. In addition,
gastric dilation on prenatal ultrasound was associated
with a higher chance of neonatal death.33 Bowel atresia
typically occurs at the level of the small bowel. The
subsequent accumulation of amniotic fluid causes
dilation of the proximal bowel and ultimately
polyhydramnios. Patients with bowel atresia are classi-
fied as complex and have a more complicated postna-
tal and postsurgical course.
Antenatal Management
When the diagnosis of gastroschisis is made, refer-
ral to a tertiary center with maternal fetal medicine,
genetic counseling, pediatric surgery, and neonatol-
ogy is recommended. Prenatal care can continue at
routine increments, though more frequent ultrasound
is recommended (see below). Because immediate
neonatal care is needed at the time of delivery, it is
helpful for families to interact with these pediatric
providers before delivery. Consultation with pediatric
surgery is also helpful to provide parents with
Copyright 2016 Wolters Kluwer Health, Inc. All rights reserved.
expectations surrounding the postnatal course for
their infant.
The approach to antenatal monitoring for the preven-
tion of IUFD in cases of gastroschisis is highly variable,
from daily home monitoring to twice-weekly office
testing.44,45,48 Given the increased risk of IUFD, some
form of antenatal testing is usually recommended.
However, antenatal surveillance protocols vary, with
some studies suggest starting testing at 28 weeks44
and others recommending daily fetal home monitoring
starting at 30 weeks. Our practice is to perform serial ul-
trasounds every 4 weeks for fetal growth beginning in
the third trimester. Antenatal testing is initiated at 32
to 34 weeks, or sooner if abnormal fetal growth or other
comorbidity is identified. This approach to antenatal
management and testing is based on expert opinion be-
cause, at this time, no specific antenatal testing regimen
has been shown to improve neonatal outcome or lower
the incidence of fetal death.
Because bowel compromise is commonly seen, likely
due to prolonged exposure of bowel to amniotic fluid as
well as the compression of bowel and vasculature near
the abdominal wall defect, some studies have evaluated
in utero interventions to preempt this. Amnioexchange
with warm saline has been studied; however, this did
not improve outcomes or reduce neutrophils or the in-
flammatory cytokines thought to contribute to bowel
compromise.66,67 At this time, there is no proven in
uterofetal therapy for gastroschisis and it is not recom-
mended. Suspicion of bowel compromise on prenatal
ultrasound does not warrant early delivery, as studies
of bowel parameters on ultrasound have poor correla-
tion with postnatal bowel function.68
Delivery Considerations
Delivery at a tertiary care center with neonatal inten-
sive care unit and pediatric surgery team is recom-
mended. Delivery outside a tertiary care center has
been associated with increased complication rates in in-
fants born with gastroschisis.69 Timing of delivery is a
long-debated topic in this population. The theoretical
benefits of elective preterm delivery include decreased
exposure of bowel to amniotic fluid thus minimizing
necrosis, atresia, or necrotizing enterocolitis and their
sequelae. However, the risks of elective preterm deliv-
ery include well-established prematurity-related com-
plications, such as respiratory distress syndrome, which
may complicated the postnatal course of an infant
who will need surgery.
Wide variations in practice surrounding the timing of
delivery exist, despite numerous studies aimed at deter-
mining the optimal timing of delivery. Studies on
 Gastroschisis: Management and Outcomes CME Review Article 541

timing of delivery and neonatal outcomes reveal sig- intervention must occur as soon as possible to reduce
nificantly different information. Mesas Burgos and these losses.
colleauges70 observed the best outcome for patients
born between 35 and 36.9 weeks and recommend Newborn Management
planned cesarean delivery at 35 weeks, while Overcash
Although an extensive discussion of the methods
and colleagues52 found that delivery before that was as-
of abdominal wall closure is beyond the scope of this
sociated with adverse neonatal outcomes. Baud and
article, a simple understanding may be helpful in
colleagues71 found that induction of labor at 37 weeks
counseling patients. There are 3 categories of surgi-
was associated with reduced risk of sepsis, bowel dam-
cal management: operative primary fascial closure,
age, and neonatal death when compared with expectant
silo placement with staged reduction and delayed
management beyond 37 weeks' gestation. In contrast,
closure, and sutureless umbilical closure. Operative
Carnaghan and colleagues72 found that delivery before
primary fascial closure was the dominant closure
37 weeks was associated with prolonged time to
method in the 1990s, until the advent of preformed
achieve full enteral feeds and longer hospital stay. Cain
Silastic silos. When preformed Silastic silos became
and colleagues73 found that birth at early term (37 0/7 to
available in the early 2000s, pediatric surgeons could
38 6/7 weeks) or later term (39 0/7 weeks or greater)
promptly place a silo at the bedside in the neonatal in-
was associated with improved perinatal outcomes when
tensive care unit. The optimum method of closure is
compared with delivery before 37 weeks. Youssef
a topic of on ongoing debate in the pediatric surgery
et al74 found that for every week in utero, the percent-
community, and data remain unclear about the overall
age of patients with severe bowel matting, defined as
benefit of using preformed silos over alternate
a disruption of the normal smooth bowel wall contour,
therapy.9096 While 1 meta-analysis provided evi-
decreases by 3.6%, which contradicts the theoretical
dence that operative primary fascial closure was asso-
benefit of delivery to decrease bowel exposure to amni-
ciated with more favorable outcomes,97 another meta-
otic fluid. In contrast, Nasr et al75 found that delivery
analysis concluded that use of a preformed silo was
after 38 weeks was associated with increased bowel
associated with shorter duration of mechanical venti-
matting. Based on the available evidence, we generally
lation, but longer duration of parenteral nutrition.98
recommend delivery at 37 weeks, or sooner if other
Pediatric surgeons report that their decision to choose
obstetrical indication.
a particular closure technique is influenced by defect
There have been conflicting reports and recommen-
size, bowel appearance, concern for development of
dations on the optimal mode of delivery for pregnancies
necrotizing enterocolitis, and patient stabilitiy.99
complicated by gastroschisis. Some authors recom-
Stanger and colleagues found that patients who
mend delivery by cesarean delivery to better optimize
underwent attempted operative primary fascial clo-
resources for perinatal care.70 However, multiple au-
sure were more likely to have been born at a pediatric
thors have shown that labor and ruptured membranes
surgery center, have daytime admissions, and have
have not been associated with worse outcome.76,77 Sev-
higher Score for Neonatal Acute Physiology-II
eral studies have found no association between mode
(SNAP-II) scores.100
of delivery and rate of primary repair, morbidity, neo-
Enteral feeding is delayed for weeks to accommodate
natal sepsis, or length of hospital stay.78 At our centers,
the abnormal intestinal motility and poor nutrient ab-
we reserve cesarean delivery for typical obstetric
sorption. The prediction of readiness for enteral nutri-
indications.71,76,7988
tion after closure is subjective, and 1 study found that
At the time of delivery, the neonatal intensive care
the best outcomes are observed when enteral feeding
team should be present. Once the fetus is delivered,
is started at 7 days after closure.101 Infants fed human
the extruded bowel should be wrapped with sterile sa-
milk exclusively after gastroschisis repair had a shorter
line dressing and an impermeable barrier to minimize
time to achieve full enteral feeds and had a shorter time
insensible fluid losses. Immediate postnatal care may
to discharge.102
include insertion of an orogastric tube to decompress
the stomach and placement of a peripheral intravenous
Neonatal Outcomes
line to provide fluids and broad-spectrum antibiotics
that cover maternal vaginal flora. In the first 24 hours While the overall postnatal survival estimates are ap-
of life, fluid losses are 2.5 times that of a healthy new- proximately 90% to 95%,49 survival and the severity of
born due to insensible heat and fluid losses from expo- the neonatal course depends on the complexity of the
sure of eviscerated bowel plus third spacing of fluid abdominal wall defect.103 Patients can be stratified as
from compromised bowel.89 Some form of surgical either low-risk (simple) or high-risk (complex).

Copyright 2016 Wolters Kluwer Health, Inc. All rights reserved.

542 Obstetrical and Gynecological Survey
Complex gastroschisis, found in approximately 10% to
15% of patients, is defined as gastroschisis with at least
one of the following features during primary evalua-
tion: intestinal atresia, volvulus, necrosis, or perfora-
tion.103,104 As Bergholz and colleagues104 point out,
these infants with complex gastroschisis can be ex-
tremely sick with extensive intestinal loss, short bowel
syndrome, liver failure, and early childhood death. A
large meta-analysis found that patients with complex
gastroschisis had a higher risk of sepsis, necrotizing en-
terocolitis, and short bowel syndrome. Newborns with
complex gastroschisis generally take longer to reach
full enteral feedings, and they have longer hospital
stays. They are more likely to be discharged home with
enteral tube feedings or parenteral nutrition when com-
pared with simple gastroschisis.104 Short bowel syndrome
is more common in cases of complex gastroschisis,
causing a higher chance of diarrhea, weight loss, and
sometimes long-term dependence on total parenteral
nutrition. Some have proposed using a prognostic score
correlating with bowel appearance at birth, based on the
presence of bowel matting, necrosis, atresia, or perfora-
tion evaluated within 6 hours of birth.105
Although the immediate neonatal course can be compli-
cated, most neonates with isolated gastroschisis have few
long-term complications. Some long-term consequences
may include gastrointestinal dysfunction such as abnor-
mal intestinal motility,106 gastroesophageal reflux,107
hiatal hernia,108 and cow's milk protein allergy.109
CONCLUSIONS
Obstetric providers should be aware of the diagnosis
and evidence for management of gastroschisis. A rise
in gastroschisis cases has been noted in recent decades.
Prenatal management includes serial ultrasound exami-
nations to monitor the pregnancy due to an increased
risk for growth restriction, amniotic fluid abnormalities,
and fetal demise. Intra-abdominal bowel dilation, poly-
hydramnios, and gastric dilation imply a higher risk of
developing postnatal complications. Delivery mode
and timing is debated in the literature. We recommend
delivery at 37 weeks, with cesarean delivery reserved
for obstetric indications. Neonatal management in-
cludes delivery at a tertiary center with multidisciplinary
care. Postnatal surgical management includes primary
surgical closure, staged reduction with silo, or sutureless
umbilical closure. Overall prognosis is good in the ma-
jority of infants, but the course can be devastating in
15% of cases.
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