Advances in Surgery for

A b d o m i n a l Wal l D e f e c t s
Gastroschisis and Omphalocele

Saleem Islam, MD, MPH

KEYWORDS
 Gastroschisis  Omphalocele  Prenatal diagnosis  Surgery  Neonatal

KEY POINTS
 Abdominal wall defects are comprised of 2 distinct entities, omphalocele and gastro-
schisis, which have very different management techniques and outcomes.
 Gastroschisis outcomes have improved dramatically over the past 4 decades, but still
comprise the largest group of patients needing bowel transplant.
 Omphalocele outcomes remain poor overall despite many advances in care.
 Large omphaloceles are one of the most difficult things to manage in pediatric surgery
and there is no standardized closure technique.
 There is a need to conduct multicenter prospective trials to better define groups of
neonates with poor prognosis and to develop improved techniques to manage them.

Abdominal wall defects (AWDs) are the most common congenital surgical problem in
fetuses and neonates. The incidence of these defects has steadily increased over the
past few decades due to rising numbers of gastroschisis. Most of these anomalies
are diagnosed prenatally and then managed at a center with readily available pediatric
surgical, neonatology, and high-risk obstetric support. While commonly lumped
together, omphaloceles and gastroschisis are distinct anomalies that have different
management and outcomes; therefore, they will be considered separately. There
have been several recent advances in the care of patients with AWDs, both in the fetus
and the newborn, which will be discussed in this article.

OMPHALOCELE
Termination and the Hidden Mortality
Omphaloceles have an incidence of 1 case per 1100 population in fetuses at around
14 to 18 weeks gestation, yet the number of live born is 1 case per 4000 to 6000 in-
fants.1,2 These defects have a very high rate of termination of pregnancy (30%–52%)

Pediatric Surgery, Department of Surgery, University of Florida College of Medicine, Post Office
Box 100119, 1600 SW Archer Road, Gainesville, FL 32607, USA
E-mail address: [email protected]

Clin Perinatol 39 (2012) 375–386

doi:10.1016/j.clp.2012.04.008 perinatology.theclinics.com
0095-5108/12/$ – see front matter Ó 2012 Elsevier Inc. All rights reserved.
376 Islam

due to the presence of associated anomalies in addition to attrition (fetal demise,

spontaneous abortion –(5%–10%) above and beyond termination.3,4 Some reviews
state that the termination request rate for omphaloceles may be as high as 83%.4
Most reports differentiate isolated omphalocele from those with other associated
defects, due to the fact that survival is considered to be highly dependent on the pres-
ence and severity of these anomalies (isolated omphalocele has a survival rate of as
high as 96%).5,6 Data suggest that prenatal ultrasound and karyotyping are able to
identify 60% to 70% of the associated defects that become apparent postnatally.6
In 2 recent series, survival for neonates with isolated omphaloceles and those with
nonisolated omphaloceles that were not picked up during prenatal evaluation were
found to be similar.7,8 However, these same studies showed that the overall survival
of fetuses with omphalocele was between 23% and 52%. Thus, when one analyzes
the survival in live born neonates, one is already dealing with a selected group of
patients, with improved likelihood of survival.

Prenatal Diagnosis, Counseling, and Therapy

Prenatal diagnosis in omphaloceles is usually made in the late first trimester to midsec-
ond trimester by ultrasound screening or as part of a positive triple test with elevated
maternal a fetoprotein.8 As opposed to gastroschisis, these fetuses undergo further
tests to look for associated anomalies or chromosomal abnormalities. Other anomalies
are found in up to 80% of fetuses with normal karyotype, while chromosomal defects are
noted in approximately 49% (mostly trisomy 13, 18, or 21).8 A recent study that collated
all recent publications detailing the associated defects in AWD patients noted anomalies
that involved every organ system, while a study from the Netherlands indicated that only
14% of omphaloceles were truly isolated.6,9 Prenatal screening in omphalocele fetuses
needs to have a detailed evaluation of the cardiac system (14%–47%) and central
nervous system (3%–33%), as severe defects may lead to a serious discussion on
termination of pregnancy.9 However, fully one-third of fetuses considered to be isolated
have multiple associated defects detected postnatally.6 This is important information for
discussions that high-risk pregnancy teams have with prospective parents with an
omphalocele fetus (which are centered on the key question of postnatal outcome and
morbidity). Recently, there has been some attention directed toward developing a reli-
able sonographic predictor of postnatal morbidity and survival.10–12 Previous estimates
have relied on defining a giant omphalocele; however, this has not been a reliable
predictor for a number of reasons. First, the definition of a giant omphalocele in a fetus
is variable, with some studies using a 4 cm diameter or 5 cm diameter criterion, and
others defining it based on the presence or absence of liver outside the abdomen.13
Second, these studies have shown no significant impact on the diagnosis of either extra-
corporeal liver or a giant omphalocele in postnatal survival and therefore have not been
useful in guiding families.13 In the past few years, some investigators have studied ratios
between the greatest omphalocele diameter compared with abdominal circumference
(O/AC), femur length (O/FL), and head circumference (O/HC), and attempted to corre-
late that with postnatal morbidity and mortality.13,14 Of these, potentially the most useful
may be the O/HC, which if 0.21 or greater had 84% sensitivity and 58% specificity at
predicting the need for staged versus primary closure and respiratory insufficiency.13
These data were obtained in retrospective studies and need to be verified prospectively
before becoming standards for predicting postnatal outcome. There are currently no
routine fetal interventions that are performed either experimentally or in people for
omphaloceles specifically other than amniocentesis for karyotype.
The timing of delivery of an omphalocele is not controversial, and most infants are
born at term unless there are complicating features such as polyhydraminos. Preterm
 Advances in Surgery for Abdominal Wall Defects 377

delivery is not recommended. The route of delivery, however, is not a settled issue, with
proponents of vaginal birth as well as cesarean section. In large defects in which most of
the liver is extra-abdominal, concern for hepatic injury during vaginal delivery prompts
a cesarean section.5 To date there has been no study that advocates 1 method over the
other, and there are numerous reports of safe delivery of omphalocele patients
vaginally.15

Management of the Large or Giant Defect

In general, the surgical management of omphaloceles for the small- or even medium-
sized (2–4 cm) defects is fairly standard and involves primary closure with good
surgical outcomes. In these cases, the survival and morbidity are dependent upon
the associated defects. Interestingly, small defects, especially those in the central
location (as opposed to hypogastric or epigastric locations), have a higher incidence
of associated anomalies and chromosomal issues.8 Most of the larger defects that
survive to birth do not have lethal associated defects, and present some of the
most challenging problems for pediatric surgeons and neonatologists.
In a recent survey performed by a Dutch group of surgeons, authors of reports dis-
cussing closure of giant omphaloceles (1967–2009) were asked to see if they are still
performing the same repair, or whether they have modified their techniques. They
concluded that over a 30 - year period there has been no completely accepted tech-
nique to treat giant omphaloceles and that, in general, 2 methods have persisted,
staged or delayed closure of the defect.16 This study did not address the issue with
the previous mentioned heterogeneity of the definition of a giant omphalocele, similar
to the prenatal ultrasonographic definition issues. Some authors use size alone; others
consider the presence or absence of liver, while others use an estimate of the amount
of intestinal contents (all or partial). Still others have used a combination of liver and
intestines.17,18 This has resulted in an inability to truly combine the literature and arrive
at a consensus.
Most data on the surgical aspects of omphalocele closure are from small single
institution studies or case reports detailing a specific technique that was used. As
mentioned previously, they may be broadly classified into non operative (delayed
repair), or staged methods.18 In addition to these methods, there are several reports
of primary closure of a giant omphalocele shortly after birth with good results.17,19
In a report from London, 12 of 24 cases of large defects had an immediate repair per-
formed without any mortality, and compared with the remaining cases, had a shorter
ventilator requirement and time to attain full feeds.19 However, this trial was not
prospective, and there was significant selection bias toward the full -term and normal
birth weight neonates for immediate repair. In addition, the definition of large was not
uniformly applied. Most cases of large defects are not considered amenable to imme-
diate repair due to the lack of abdominal domain.18
Nonoperative techniques have in common the use of an agent that allows an eschar to
form over the intact amnion sac, which epithelializes over time, leaving a ventral hernia
that will likely require repair later in life.18 This method has been employed when the
surgeon considers the defect too large to allow for safe primary repair, or if the neonate
has significant concurrent cardiac or respiratory issues that would preclude an attempt
at surgery. This is not a new concept, having evolved from the time of Gross, who
described using skin flaps in 1948, and others who used alcohol as a topical agent in
1899.20 Concerns in large isolated defects are that an initial repair without having
abdominal domain for the organs would result in potential life-threatening abdominal
compartment syndrome or inability to provide skin coverage and. Therefore, this
approach would not be ideal. Initial reports described mercurochrome, alcohol, and
378 Islam

silver nitrate as the eschar-producing agents, which were very effective, but associated
with toxicity and abandoned.20 Subsequently, there have been reports of a number of
agents used, including silver sulfadiazine, povidone–iodine solution, silver impregnated
dressings, neomycin, and polymixin/bacitracin ointments.20–24 The eschar and epithe-
lialization may take over 4 to 10 weeks to complete, and in some cases, patients
complete the process after discharge. There are also reports that combine the use of
an agent listed previously with compression dressing, which helps in reducing the
contents in the abdomen and facilitates closure.25 In some cases, there is no need for
surgical closure, as the defect contracts and closes similar to an umbilical hernia;
however, most patients will eventually require closure of a ventral hernia defect, which
is usually performed between 1 and 5 years of age. The repair is performed via either
primary fascial closure, autologous repair with component separation, or use of
a mesh repair.21,26,27 Each has been reported with success; however, the number of
patients in each report is small, and, without prospective studies, the failures are usually
not reported. In some cases, innovative techniques have been used to recreate the
abdominal domain including the use of tissue expanders in the abdomen that are grad-
ually increased in size.28 While the initial reports of the staged Gross operation had
significant mortality and morbidity, current results are much better, with very few deaths
reported.21
Staged closure in the neonatal period involves the use of a variety of different tech-
niques to obtain closure with multiple procedures. These can be classified into
methods that use the existing amnion sac with serial inversion and those in which
the sac is excised and replaced with mesh and then closed over time. Amnion inver-
sion allows gradual reduction of the sac and, when completely involuted, the sac is
excised and either a primary closure is performed or mesh is used.29 There are several
reports that detail different methods of mesh closure, each with the similar goal of
obtaining skin and fascial apposition. This may be achieved by excision of the mesh
sequentially, allowing for native fascial closure. Alternatively, mesh may be left in
situ with skin coverage on top.30 Some authors have advocated the use of biologic
mesh that becomes incorporated and may have less recurrent hernia formation.31,32
Vacuum-assisted closure of these defects has also been described, as has a novel
external skin closure system.33,34 Again, it is difficult to truly compare the series, as
the definition of size of the defect is not uniform.
A large, prenatally ruptured omphalocele is a special situation that represents one of
the most difficult problems in pediatric surgery. The goal in repair of these unique situ-
ations is to obtain coverage of the exposed abdominal viscera, which is challenging
and, aside from a few case reports, there is not much experience. There is 1 small
series that has good outcome in terms of survival, but there is a high incidence of
intestinal fistulas, sepsis, and pulmonary hypoplasia that can lead to poor outcomes.35

Outcomes in Omphaloceles
As has been discussed, the outcomes in this defect may be looked at in 2 ways:survival
of fetuses diagnosed with omphalocele, or in those who are live born.17 Multiple studies
have documented survival of less than 20% to 50% in prenatally diagnosed cases
(including termination).8 A report from London indicated that 90% of cases with
prenatal diagnosis reached the point of undergoing surgical repair.36 Studies looking
at survival postnatally document a close association with the presence of defects
and chromosomal anomalies.5,36,37 Going beyond survival alone, there are several
issues that lead to continuing morbidity. Pulmonary hypoplasia can be severe in these
neonates and may require prolonged mechanical ventilation and tracheostomy.38
However, long-term follow-up in a cohort of older children documented normal lung
 Advances in Surgery for Abdominal Wall Defects 379

volumes and oxygen consumption.39 Gastroesophageal reflux is also common, as well

as failure to thrive, and these are significant issues that require long-term therapy and
a high incidence of fundoplication and nutritional support.37,40 One long-term concern
in patients with giant omphaloceles is the cosmetic appearance of their abdomen and
the missing umbilicus.41 Outside of these few studies, there are few data on the long-
term outcomes in infants with omphalocele, and the use of multicenter registries would
be beneficial in understanding the true impact of these defects and the cost associated
with them.

GASTROSCHISIS
Prenatal Therapy
Most gastroschisis patients are diagnosed and identified prenatally and are delivered at
a center with the ability to care for the neonate. Most are diagnosed in the early to mid-
second trimester, providing ample time for consideration of any prenatal interventions
designed to improve postnatal outcomes. The issues that lead to intestinal damage and
prolonged dysmotility are thought to be the result of interactions between the bowel
serosa and the amniotic fluid, and in particular the meconium and other waste products
in the fluid.42–44 To ameliorate the effects of this interaction, studies in small and large
animal models have suggested a benefit to altering the amniotic fluid environment.44–46
Three interventions have been attempted: (1) amniotic fluid removal and exchange with
physiologic fluid, (2) amniotic fluid supplementation in cases of oligohydramnios, and (3)
amniotic instillation of furosemide to induce fetal diuresis.43,47–49 Each of the interven-
tions showed promise in animal models, with reduction in the intestinal damage as
measured by the thickness of the peel, number of interstitial cells of Cajal, and inflam-
matory markers. However, it is unclear whether these interventions can result in any
long-term benefit. There have been a few instances of human amniotic fluid exchange
as well as amnio infusion for oligohydramnios in the setting of gastroschisis, but the
number of patients is too few to make any conclusions.50,51 In 1 small study of 10 gas-
troschisis patients compared with controls, there was a reduced hospital length of stay
when compared with controls, but no other variables were reported.52 Thus, prenatal
therapy holds promise, but with a current survival rate of over 90% in cases without
any intervention, safety issues may limit its usefulness and adoption.

Ultrasonographic Markers of Concern

Since a report in 2001 that recommended stratification of gastroschisis cases into
complicated and simple, there has been growing realization that these 2 classifica-
tions may actually be different disease conditions with different outcomes.53 The
authors defined complicated gastroschisis as one in which there is a bowel atresia;
perforation; ischemia or necrosis; or bowel loss that has occurred in utero. Efforts
have been directed at accurately predicting which fetuses will develop complicated
problems. Initial reports considered the appearance of the intestine and the stomach,
but recently focus has been directed to the diameter of the loops of intestine that are
contained within the abdomen. Alfaraj and colleagues54 made note that gastric dilation
predicted the presence of meconium-stained fluid, but no other adverse outcome.
Lato and the group from Dusseldorf found improved predictive capability of ultra-
sound when both intestinal dilation and gestational age were noted, and they were
able to correlate bowel atresia with dilation of greater than 10 mm noted before 30
weeks gestation.55 Contro and colleagues and Huh and colleagues identified intestinal
damage in 29% and 37% of fetuses respectively using greater than 6 mm diameter as
a cutoff.56,57 The studies are still plagued by inconsistent use of the measurement
380 Islam

(>6–>20 mm) and the time in gestation that it is noted; therefore, the usefulness is
controversial.58 Further work with larger multicenter prospective studies is needed
to refine the ability to predict intestinal damage and complex gastroschisis and
improve prenatal counseling.58

Route and Timing of Delivery

The issues of when and how to deliver a fetus with an AWD, especially gastroschisis,
remain controversial despite many advances in the past few decades in the under-
standing of AWD.15 Most gastroschisis fetuses are born prematurely around 34 to
36 weeks gestation and are small for gestational age due to significant intrauterine
growth retardation (IUGR).59 There was significant interest in having these babies
delivered prematurely via induction or cesarean at 34 to 36 weeks to limit the duration
of time that the intestines were exposed to amniotic fluid, based on data from animal
studies. Additionally, by performing a scheduled delivery, the neonate could be deliv-
ered at a facility where appropriate resources were present to care for the newborn.
One prospective, randomized trial in the United Kingdom was designed to study
outcomes from elective preterm delivery at 36 weeks versus undergoing spontaneous
labor, using an intent-to-treat strategy, and found no benefit to preterm delivery.60
Opposing evidence came from a prospective, but nonrandomized study at the
Mayo clinic involving only 16 patients with elective delivery at 34 weeks, which
reported reduced total parenteral nutrition (TPN) duration, decreased length of
hospital stay, and a higher rate of primary closure of the gastroschisis compared
with a historical cohort.61 Another retrospective review from Mannheim noted that
elective cesarean section before 36 weeks led to earlier closure of the defect and
enteral feeding,62 while Vegunta and colleagues63 reported benefits from planned
cesarean section at 36 to 38 weeks with higher rates of primary repair. Two further
reports from Pittsburgh looking at 75 cases of gastroschisis, and Portugal with 65
patients, found that patients electively delivered early had no difference in the appear-
ance of the bowel compared with normal deliveries, but had a longer hospital length of
stay. As a result, these authors advocated against preterm delivery.64,65 The data are
mixed, but the evidence falls to the side of allowing spontaneous delivery close to term
as opposed to elective preterm birth in the setting of gastroschisis.
Abdel-Latif, and colleagues,66 reporting on behalf of the Australia New Zealand
Neonatal Network, compared the short term outcomes in babies with gastroschisis
born via vaginal versus elective/emergency cesarean section. Fifty-four percent were
delivered vaginally, and there was no difference in any parameter of short-term
outcome between the groups. The group from Montreal published retrospective series
and, again, found no benefit to cesarean delivery in gastroschisis.67 In 2000, How and
colleagues68 reported their experience with 102 infants with an AWD and recommen-
ded cesarean section for obstetric indications only. In another retrospective review,
authors from Kansas City noted a significant trend over time (30-year period) toward
cesarean section,69 despite a lack of evidence to suggest its benefit. Segel published
a systematic review regarding mode of delivery and AWD, and, again, found no advan-
tage to a cesarean section.15 The evidence would suggest that in the absence of
obstetric indications, most gastroschisis fetuses should be allowed to deliver vaginally.

Silo or Immediate Closure?

Another point of contention in the care of gastroschisis patients is the method used to
close the defect. Schuster first described the creation of a silo for patients in whom
there was lack of domain for the viscera (viscero abdominal disproportion). The silo
was sewn to the fascia and allowed a gradual return of the viscera to the abdomen.
 Advances in Surgery for Abdominal Wall Defects 381

In the past couple of decades, the preformed, spring-loaded silo has made this method
easier and has become popular as a form of initial closure.70 The debate is whether
patients have improved outcomes with either immediate reduction and closure or
a delayed closure after a silo and gradual reduction. Retrospective reports have argued
the benefits of each method, citing benefits with regard to ventilator time, time to full
feeds, length of stay, and complication rates such as abdominal compartment
syndrome, bowel ischemia, and necrotizing eterocolitis (NEC).71–74 Pastor and col-
leagues75 performed a multicenter, randomized, prospective trial comparing outcomes
between silo and immediate closure. While they could not meet their accrual goals,
they showed no significant difference between either group for most outcomes, other
than duration of ventilation, which tended to be less in the silo group. A Canadian Asso-
ciation of Pediatric Surgeons network (CAPSnet) study published in 2008 reviewed the
experience in the first 99 cases of gastroschisis in Canada and noted that there was no
difference in overall outcome in silo versus immediate closure other than an increased
length of stay and duration of TPN usage in the silo group that likely reflected the obser-
vational nature of the study.76 In 2011, a cooperative, observational trial from the United
Kingdom that captured 77% (301 of 393) of all gastroschisis births over a 17-month
period noted that patients who had a silo placed took 5 days longer to reach full enteral
feeds and had a higher risk of intestinal failure when compared with immediate
closure.77 However, this was a nonselected group of patients, and the authors
cautioned in overinterpreting these data, as patients who had a silo were likely to
have had more viscera outside the abdomen. They did suggest performing a prospec-
tive study to address this question. Final closure technique has also been debated
recently, with groups reporting successful nonoperative and sutureless final closure
for either the immediate reduction or silo patients.78,79 Techniques for this have
involved the use of the umbilical cord tissue and dressing changes and Steri Strips
with vacuum-assisted closure.80,81 Advantages of this method are the avoidance of
anesthesia for bedside closure as well as improved cosmetic results.

Management of Complicated Gastroschisis

Molik and colleagues53 published an article in 2001 in which they strongly advocated for
risk adjustment in gastroschisis to allow better comparison of patients and outcomes.
This was borne out by 2 administrative database reviews from the United States that
supported and validated the classification of gastroschisis into complex and simple
based on the presence of bowel atresia, perforation, ischemia, or prenatal loss of intes-
tine with short bowel syndrome.82,83 Outcomes are quite different between these 2
groups, and long-term morbidity is almost entirely confined to the complex group
and associated short bowel syndrome, liver failure due to prolonged parenteral nutri-
tion, and sepsis. Most reviews estimate 5% to 15% of gastroschisis cases are complex,
and these patients predictably have a longer length of stay, increased TPN usage, and
increased costs as compared with the simple cases.84 The most common type of
complex gastroschisis is a concomitant bowel atresia, seen in up to 15% of patients.
Bowel atresia management depends upon the condition of the bowel and the infant.85
In most cases, the thickness and extent of the peel will not allow immediate anasto-
mosis. Therefore, the options are to reduce the viscera and perform repair in 3 to 5
weeks, allowing the intestines to normalize, or in more distal atresias, to create an
ostomy that would allow for enteral feedings before definitive repair.86 The prolonged
time on parenteral nutrition is responsible for the morbidity due to sepsis and chole-
static liver disease. Modern management of patients with short bowel at specialized
centers may help improve survival for this group of patients. Efforts at prenatal detec-
tion of this group of patients, as discussed previously, would help in counseling parents.
382 Islam

Outcomes
Outcomes in gastroschisis have changed dramatically in the past 4 decades, with the
advent of improved neonatal intensive care unit, surgical, obstetric, and nutritional
care. Overall, survival went from 50% to 60% in the 1960s to greater than 90%
currently. Long-term issues are rare in patients without bowel injury, and most infants
catch up in growth with their non-AWD cohorts within a few years.2 IUGR in gastro-
schisis does not have significant impact on outcome. Despite having malrotation,
the incidence of volvulus is rare in gastroschisis, and a Ladd procedure is rarely
required. Undescended testicles are noted in 15% to 30% of males with gastroschisis,
with a large number having extra-abdominal gonads at birth. Fifty percent or more of
these testicles will descend spontaneously by 12 months of age, while the remainder
will require an orchiopexy at that point.87 Simply replacing the testicle in the abdomen
at the time of final closure is recommended. The improved survival and diminished
morbidity from gastroschisis may be noted from the fact that up to 60% of children
will report psychological stress at the absence of a normal umbilicus, and this may
be the most prevalent long-term issue requiring reconstruction in some cases.88

SUMMARY

AWDs are comprised of 2 distinct entities, omphalocele and gastroschisis, that have
very different management techniques and outcomes. Overall, survival has improved
considerably, especially for gastroschisis. However, there is a need to conduct multi-
center prospective trials to better define groups of neonates with poor prognosis and
to develop improved techniques to manage them.

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