Surg Clin N Am 86 (2006) 249260

Gastroschisis and Omphalocele

Daniel J. Ledbetter, MDa,b,*
a

Department of Surgery, Division of Pediatric Surgery, University of Washington

b
Department of Surgery, Childrens Hospital and Regional Medical Center,
4800 Sand Point Way NE, P.O. Box 5371/G0035, Seattle, WA 98105-0371, USA

Thepresentations
newborn whoinhas
an abdominal
wall
defect
is one of the most
matic
and othe
ers
many
challenging
todrathe
pediatric
surgeon.
This medicine
article presents
basics
of the two problems
most common
abdominal
wall
defectsdgastroschisis
and
omphaloceledincluding
principles
and
options
of
prenatal,
postnatal,
and
surgical
management.
Although
textbooks
the twodientities
together,
they are separate
and distinct
and
have
manygroup
important
erences
in pathology
associated
conditions
that
explain
theand
didi
erences
in between
treatment
plans andand
outcomes.
Understanding
the similarities
erences
gastroschisis
and omphalocele
is acessential
for
patient
management;
therefore,
the
following
sections

rst
knowledge the similarities and then emphasize the dierences.
Denitions
a full-thickness
defect
in the abdominal
usuallywall.
just
to Gastroschisis
the right
aisnormal
insertion
of
theposition
umbilical
intowall
the
Rarely
is of
located
in aof
mirror-image
to cord
theparts
left
of
thebody
umbilical
cord.
A itvariable
amount
intestine
and
occasionally
ofcovering
other
abdominal
organs
are
herniated
outside
the
abdominal
wall
with
no
membrane or sac.
An omphalocele
(alsowith
known
exomphalos)
is a covered
midline abdominal
wall
defect
of variable
size,
the as
herniated
viscera
byouter
a membrane
consisting
of peritoneum
on the
surface,
amnion
on the
surface,
and Whartons
jellythe
between
theinner
layers.
The umbilical
vessels
insert
into
the
membrane
and not
body wall.
The hernia
contents include
a variable
* Department of Surgery, Childrens Hospital and Regional Medical Center, 4800 Sand
Point Way NE, P.O. Box 5371/G0035, Seattle, WA 98105-0371.
E-mail address: dan.ledbetter@seattlechildrens.org
0039-6109/06/$
- see front matter
doi:10.1016/j.suc.2005.12.003

2006 Elsevier Inc. All rights reserved. surgical.theclinics.com

250

LEDBETTER

amount
of may
intestine,
often parts
the liver,
and
occasionally
The
be important
centered
in
theofupper,
mid,management.
or lower
abdomenother
and organs.
its size
and defect
location
have
implications
for
Abdominal wall development and the pathology of omphalocele
and gastroschisis
The embryonic
abdominal folds.
wall isAsformed
by infolding
of
the cranial,
and two
lateral
the
abdominal
walloutside
is forming,
thecaudal,
rapid growth
of
the
intestinal
tract leads
toand
its into
migration
the
cavity
through
the
umbilical
ring10th
theweek,
umbilical
cordabdominal
during
the
sixth
week
of
gestation.
By
the
to
12th
the
abdominal
wall
is
well
formed
and results
the intestine
returns
to therotation
abdominal
cavityxat
inion
a stereotypical
pattern that
in normal
intestinal
and later
[1].
Gastroschisis
is
thought
to
result
from
an
ischemic
insult
to
the developing body wall.
The right
right paraumbilical
area
is an
areaomphalomesenteric
at risk because
it is
supplied
by
the
umbilical
vein
and
right
artery
until
involute.
If then
this ordered
development
andresult
involution
is
disturbed
in they
degree
orischemia
timing,
a body
wall
defecthypothesis
could
frommay
the
resulting
body
wall
[2,3].
An
alternative
that
account
for
some
cases
of
gastroschisis
is
that
the
defect
results
from
an
early rupture of a hernia of the umbilical cord [4].
thecord.
bowelThe
does
not sequence
return to of
theevents
abdomen
but remains
outInare
inomphalocele,
the
umbilical
exact
are not
but
presumed
to involve
a failure
of abdominalorgans
wall infolding
[1]. known
Aout
variable
amount
of midgut
and
other
intra-abdominal
are
herniated
of
the
defect
depending
on
its
size
and
relative
location
on
the
abdominal
wall.
Cranial
fold
de

cits
predominately
result
in
epigastric
omphaloceles
that
may
be
associated
with
additional
cranial
fold
abnormalities
such
as
anterior
diaphragmatic
hernia, sternal
pericardial
defects,asand
cardiac
defects.
When these
elements
occur clefts,
together,
they are known
pentalogy
of Cantrell
the infolding
decit
the caudalthefold,
the
omphalocele
may[5].beWhen
associated
with bladder
or involves
cloacal exstrophy.
Epidemiology
There
are regional
dierences
the incidence
ofomphalocele;
abdominal wall
defects
and
the
relative
proportions
of in
gastroschisis
and
however,
a
rough
estimate
isper
that10,000
worldwide,
the
incidence
of
gastroschisis
ranges
between
0.4
and
3
births
and
seems
to
be
increasing,
whereas
the
incidence
ofThe
omphalocele
ranges
between
1.5 and
3 defects
per 10,000
births and
is
stable
[68].
etiology
of
both
abdominal
wall
is
unknown
and
most
are sporadic,
there areand
rare
familial (and
possibly
genetically
determined)
cases of but
gastroschisis
omphalocele.
There
are distinctive

GASTROSCHISIS AND OMPHALOCELE

251

maternal
factors
for the dierent
abdominal
wall
defects.
Gastroschisis
has
a veryrisk
strong
young
maternal
age,
with most
of these
mothers
age association
20
years ortowith
younger
[6].smoking,
In
addition,
gastroschisis
has
been
linked tobeing
maternal
exposure
cigarette
illicit
drugs, vasoactive
over-the-counter
drugs
(such
as
pseudoephedrine),
and
environmental
toxins
[6].
These
associations
are
consistent
with
the
vascular
insuciency
of
the
abdominal
wall
theories
of
the
etiology
of
gastroschisis.
In
contrast,
omphalocele
is
associated
with
advancing
maternal
age,
with
most
of
these
mothers being over 30 years old [7].
Associated anomalies
Like are
all babies
who haverisk
birth
children
who have
abdominal
wall
defects
atofanassociated
increased
fordefects,
additional
anomalies,
but between
the relative
risk
and pattern
anomalies
is a esmajor
gastroschisis
and omphalocele.
These
dierenc
are dierence
very important
in clinical
management
and
long-term
prognosis.
In
gastroschisis,
the
incidence
of
associated
anomalies
is
between
10%
and
20%,
and
most
of
the
signi

ca
nt
anomalies
are have
in theintestinal
gastrointestinal
tract
[9]. About
10%
of babies
who have
gastroschisis
stenosis
or
atresia
that
results
from
vascular
insu

ciency
to
the
bowel
at
the
time
of
gastroschisis
development
or,
more
commonly,
from
later volvulus
or compression
the Other
mesenteric
vascular
pedicle
byanomalies
a narrowing
abdominal
wall testes,
ringof[10].
less
common
associated
include
undescended
Meckels
diverticulum,
and
intestinal
duplications.
Serious
associated
anomalies
outside
the
abdomen
or gastrointestinal tract, such as chromosomal abnormalities, are
unusual.
Intocontrast
to the relatively
low
riskin of
associated
anomalies
mainly localized
the have
gastrointestinal
tract
seen
patients
have
gastroschisis,
patients
who
omphalocele
have
a very
high
(upwho
to anomalies
50%70%)
incidence
associated
anomalies.
The
incidence
of
associated
is anomalies
lower of
in
liveborn
patients
because
those
who
have
multiple
and
serious
are more likely to be stillborn [11]. Chromosome anomalies, notably trisomy
13,
14,
15,
18,
and
21,
are
present
in
up
to
30%
of
cases.
Cardiac
defects
are
also
common,
being
to 50% of
cases. Multiple
anomalies
are
frequent
and may
be present
clusteredinsyndrome
in30%
syndromic
patterns.
One important
pattern
is
the [12].
Beckwith-Wiedemann
that may
be present
in up to 10%
of
cases
Beckwith-Wiedemann
syndrome
is
marked
by
macroglossia,
organomegaly,
early
hypoglycemia
(from
pancreatic
hyperplasia and
excess
insulin),
and
an
increased
risk
of
Wilms
tumor,
hepatoblastoma,
and
neuroblastoma
developing does
laternot
in directly
childhood.
The size
thepresence
abdominal
wall
defect
in omphalocele
withof
the
of
anomalies,
as demonstrated
by the
ndassociated
ingcorrelate
that small
defects
found
onother
prenataland
ultrasound
have a [13].
higher
riskof
chromosomal
abnormalities
cardiac defects

252

LEDBETTER

Prenatal diagnosis
Abdominal
wall defects
often diagnosed
by such
prenatal
ultrasoundandone
for
routine
screening
forare
obstetric
indications
as
evaluating
elevated
maternal
serum or
alpha
fetoprotein
(AFP).
AFP
isof
the
fetalinanalog
of
albumin,
and
maternal
serum
AFP
re

ects
the
level
AFP
amniotic

u
id.
The
testing
was
developed
to
evaluate
the
fetus
for
chromosomal
abnormalities
and
neural
tube
defects,
but
AFP
is
also
usually
elevated
with
abdominal
wallgastroschisis
defects. The
and [14].
likelihood
of AFP maternal
elevation
varies
between
andmagnitude
omphalocele
In gastroschisis,
serum
AFPnine
is usually
markedly
abnormal,
with
an
average
elevation of
more
than
multiples
of
the
mean
(MoM).
In
contrast,
in
omphalocele,
AFP
is elevated
by an results
averageinof
only four
MoM,ofwith
a much
wider
range.
This
dierent
pattern
a lower
sensitivity
maternal
serum
AFP
for
omphalocele
than
for gastroschisis.
LikeFor
many
screening
tests,
the
sensidepends
on
the
cuto
value96%
chosen.
example,
if be
abnormal
isbut
detivity
only
ned
as
greater
than
3
MoM,
then
of
gastroschisis
would
detected
65% of omphaloceles [15].
Prenatal
ultrasound
is done identify
in most the
pregnancies
in themajority
United States
and,
when
done,
couldand
potentially
overwhelming
of abdominal
wall
defects
accurately
distinguish
omphalocele
from
gastroschisis.
This
identi

cat
ion
would
permit
an
opportunity
to
counsel
the
family
and
to
prepare
for
optimal ultrasound
postnatal care.
It is unfortunate,
however,
that the
accuracy by
of the
prenatal
forthe
diagnosing
abdominal
wall the
defects
is
aected
timing
andoperator.
goals of
study,
fetal
position,
and
experience
and
expertise
of
the
The
speci

c
ity
is
high
(more
than
95%),
but
the sensitivity
is only 60%
to 75%
for
identifying
gastroschisis
and with
omphalocele
[8,16].
Diagnostic
errors
may
result
because
of
(1)
confusion
other
rare abdominal
defects
awayomphaloceles
from the umbilicus,
not covered
by
a
membrane,
and
fatal);
(2)of(often
ruptured
mimic
a of
gastroschisis
because
of the
lack
a covering
membrane;
orthat
(3) rare
cases
gastroschisis
that
start
out
as
a
covered
defect
and
later
rupture
but
are
likely
and
commonly
simply defects.
missed during studies done for reasons other
than more
screening
for structural
Prenatal management
A fetus
with
an abdominal
wall defect isthere
a high-risk
pregnancy
on
levels.
For
gastroschisis
and(IUGR),
omphalocele,
an increased
risk
of many
intrauterine
growth
retardation
fetalultrasounds
death, isand
premature
delivery,
so
careful
obstetric
follow-up
with cases,
serial
and
other tests
of fetal
well-being
are
indicated.
In
both
there
is
some
controversy
regarding
the
timing and mode of delivery.
gastroschisis,
the the
diagnosis
of IUGR
can be
problematic
because
theIndi
culty measuring
torso, but
it probably
aects
30% to 70%
of of

GASTROSCHISIS AND OMPHALOCELE

253

fetuses.
The
fetal
growthlosses
failure
gastroschisis
unknown
but
presumed
to cause
be dueoftosupply
increased
of in
protein
from
the isexposed
viscera,
although
inadequate
of fetal
nutrients
isinjury
an
alternative
The exposed
bowel
is vulnerable
to midgut,
injury.
The
can
range hypothesis.
inintestinal
severity
from
volvulus
and
loss
of
the
entire
to
a
more
localized
atresia
and
stenosis,
to widespread
inammatory peel
or serositis
that
can
make
the develops
bowel
loops
indistinguishable
from
one
another.
in
ammatory
peel
after
30
weeksgestation
and
is presumed
toThe
be
due
to
bowel
wall
exposure
to
amniotic
u
id
or
to
intestinal
lymphatic
obstruction.
The
degree
of
the
in

am
matory
peel
is
di

cu
lt
to
quantify
on
prenatal
ultrasound
and
after
delivery,
so
it
has
been
di

cult
to
correlate
with
clinical outcome
variables
Because bowel
injury
is a major predictor
of
postnatal mortality
and[17].
morbidity,
improved
understanding
and
predictive
testing
would
point
thein way
toward
potential
interventions.
Oligohydramnios
is
also
common
gastroschisis,
being
present
inseverity
up to
25%
of
cases.
The
cause
is
unknown
and
it
is
usually
of
moderate
and associated
withated
IUGR,
fetal distress, and prompted
birth asphyxia.
Cases of
gastroschisis
with
investigation
of
amniotic
uidassocireplacement
witholigohydramnios
saline.
Observations
from
these
trials
experimental
models
supported
the hypothesis
thatbeen
amniotic
uid and
was
responsible
for
the
in

ammatory
peel,
and
this
has
investigated
by
amniotic
but
uid additional
exchange expetransfusion.
reports
have results
been
promising,
rience
isPreliminary
needed
to con
these
because
of the
previously
noted
difculties
in
measuring
therm
degree
of bowel
injury
preand
postnatally
[18].
The
most
devastating
prenatal
complication
with
gastroschisis
is themidgut
uncom-volvulus
mon butorunpredictable
fetalcommonly
death. It may
be
caused
by an in utero
more
an
acute
compromise
of umbilical
blood
predictors
owprobably
by theofeviscerated
bowel.byIt[19].
is
unfortunate
that
there
are
no
reliable
this
complication
It
been presumed
that early
ultrasound
signs of fetus,
bowelbut
obstruction,
as has
increased
bowel
diameter,
indicate
a high-risk
whetherrisk
it such
isfor
a
higher
risk
for
fetal
death
and
intestinal
loss
or
only
a
higher
intestinal
stenosis
andfetal
atresia
ororinammatory
peel
is notintestinal
known. The
uncommon
but
tragic
death
thepatient
who
has major
loss
has
been
a
strong
motivating
force
for
the
early
delivery
of
the
fetus
with
gastroschisis
before
complications
[20]. It is still
unclear
whether
fetus
with asuch
high
riskof
prenatalhappen
complications
be reliably
identieda and
whether
the bene
ts outweigh
the risks of earlycan
delivery
[21,22].
Omphalocele
also has toanrelated
increased
incidence
of IUGR
(5%35%),
death
(usually
attributed
to severe
associated
anomalies),
and fetal
premature
labor
(5%60%)
[23].
Further
prenatal
diagnostic
studies
such
as
high-resolution
ultrasound looking
for often
structural
defects
(especially
cardiac
defects)
and
chromosomal
studies
are
done
to
diagnose
associated
anomalies
and
to
help
predict
prognosis.
Unlike
gastroschisis,
there
is
usually no reason to consider early delivery, although cesarean section is often

254

LEDBETTER

done with giant omphaloceles to prevent rupture or dystocia during labor

[24].
Newborn management
Thewith
initial
of newbornsand
whoafter
havethese
abdominal
wallassessed
defects
starts
the management
ABCs
of is
resuscitation,
have
been
and
attention
turned
to the
abdominal
defect.
Heatmainloss
is anastabilized,
important
problem, so
careprotecting
must
be taken
to drywall
the
baby and
tain
warm
environment
while
the
exposed
viscera.
Premature
birth
is the
frequently
associated
with both
conditions
and
must be considered
during
evaluation
and
treatment.
Checking
and
maintaining
serum
glucose
levels
partabdominal
of any neonatal
resuscitation
especially
important
in
babies
who ishave
wall defects
because but
ofofthe
associated
prematurity,
IUGR,
and
in
omphaloceles,
the
possibility
Beckwith-Wiedemann
syndrome.
Prematurity,
associated
pulmonary
hypoplasia,
or
the
signi

cant
heart
defects seen
in Gastric
omphalocele
may lead tois early
intubation
and mechanical
ventilation
[25].
decompression
important
to prevent
distention
of thefor
gastrointestinal
tract
and
possible
aspiration.
Vascular
access
is
obtained
intravenous

uids
and
broad-spectrum
prophylactic
antibiotics.
Babies
who
have
gastroschisis
in
particular
have
high

uid
losses
from evaporation
and
third-space
losses
and
may require
twice the
maintenance
volumes
of

uids
to
maintain
an
adequate
intravascular
volume.
A
bladder
catheter
is useful artery
to closely
output andif guide
thedurresuscitation.
The umbilical
and monitor
vein
mayurine
bemay
cannulated
needed
ing
resuscitation
but
in
omphalocele,
placement
be
di

cult
because
of
the
abnormal insertion
of to
thebe
vessels.
Evenduring
if umbilical
cannulas
are
successfully
placed, and
they course
may need
removed
the repair
of the
defect.
When
the and
ABCs
have been
accomplished,
the dierent
abdominalconsiderations
wall defect can
be
assessed
treated.
This
process
involves
in
gastroschisis
and is
omphalocele.
In gastroschisis,
themesenteric
exposed viscera
are
inspected
andiscare
taken
to avoid
twisting
of
the
vascular
pedicle.
If
there
vascular
compromise
because
the
abdominal
wall
opening
is
too
small,
then
the defect
should umbilical
immediately
be surgically
enlarged,
care
taken
to needs
avoid
thebeadjacent
vessels
and
mesentery.
Thewith
exposed
bowel
to
protected
and

uid
and
heat
losses
minimized.
The
easiest
method
is to place
thebowel
exposed
lower spehalf
the
baby
into
a transparent
plastic
bag.viscera
This
is and
fast, entire
requires
cial
skills
or experience,
and
allows
for can
ongoing
assessment
of clear
bowelnoplastic
perfusion.
Alternatively,
the
bowel
alone
be
wrapped
with
wrapping
but this
can bewith
technically
more di
cult. Finally,
moist
dressings
over the bowel
covered
a clear
plastic
wrapping
another
strategy
but
requires
judgment
on how
tight
to wrap
and
it hidesis the
bowel
from view.
Moist dressings
alone
should
be
avoided
because
of the
increased

GASTROSCHISIS AND OMPHALOCELE

255

evaporative
losses. After
the exposed bowel is covered, the entire mass is
stabilized
byheat
placing
the mesenteric
pediclethe
[26].baby with its right side down to prevent kinking of
With
omphalocele,
the
care
of that
the defect
and its membrane
contents is di
erent. The
defect
is inspected
to make
sure
covering
is intact,
nonadherent
dressings
aresac
applied
andthe
stabilized
to prevent
trauma
to and
the
sac.
If
the
omphalocele
is
ruptured,
then
exposed
bowel
should
be
treated as it is for gastroschisis.
Surgical management
In gastroschisis
and
omphalocele,
the ultimate
arethe
straightforward:
to
reduce
theaherniated
viscera
intowall
the with
abdomen
and goals
to close
fascia andwhile
skin
to
create
solid
abdominal
a relatively
normal
umbilicus
minimizing
risks
to
the
baby.
To
achieve
these
goals,
many
dierent
techniques
have
been
described.
varies
dependingproblems.
on the
size
and there
type
ofis the
defect,
the
sizeTreatment
of
the baby,often
andfavor
the
associated
Because
little
hard variation
scienti
c inevidence
to
one method
over another,
there
is
considerable
the
surgical
approach.
What
follows
is
the
authors
personal
approach
gastroschisis
and omphalocele, with
discussion
of some
but not
all of thetoalternative
methods.
In
gastroschisis,
the
ongoing

uid
and
heat
losses
of
exposed
bowel
and
the
subsequent
metabolic
derangements
make or
rapid
coverage
a high therepriority.
During
the initial
resuscitation
atSilastic
delivery
as placed
soon
asinpossible
after,
athe
prefabricated,
spring-loaded
silo is
thelosses,
defectpreto
cover
exposed
bowel.
This
practice
minimizes
evaporative
vents
additional
trauma,
and
allows
forthe
andelivery
ongoingroom
assessment
ofbedside
bowel
perfusion.
These
devices
can
be
placed
in
or
at
the
without
anesthesia
[27].
If the
thedefect
abdominal
defect under
is too local
small to
accommodate
the
device,
then
canplaced
bewall
enlarged
anesthesia
and
sedation.
If
device
cannot be
at the bedside,
then is
as
soon as
possible
after room
thethis
initial
resuscitation
and
stabilization,
theFormal
baby
taken
to
the
operating
for
primary
closure
or
silo
placement.
closure
in
the
delivery
room
isis an
intriguing
concept
that delivery
minimizes
time
and preoperative
trauma
but
only
possible
with
planned
of
a
known
defect
and
requires
a signi
cant commitment
of resources
[28]. Immediate
primary
repair
without
anesthesia
has
reported
for selected
cases andtraumatic
may be
the
most
dramatic
of been
minimally
invasive
and
minimally
surgery
[29].
It is notexample
known how
often this approach
is feasible.
After
the
placement
of
the
spring-loaded
silo,
the
baby
is
evaluated
further
and
cared for in from
the ICU.
diuresis, gastrointestinal
tract
decompression
aboveWith
and spontaneous
below,
and
resolution
of bowel in
walla
edema,
the
volume
of
the
exposed
bowel
in
the
bag
markedly
decreases
short
period
time.into
When
baby ishas
otherwise
and the
the baby
spontanereduction
of of
bowel
the the
abdomen
reached stable
a plateau,
is ous

256

LEDBETTER

taken
to the operating
room
for bedside
an attempt atbeen
delayed
primary but
closure.
Se- rial
reduction
device
at the
advocated,
the risk
of
displacing of
thethe
coverage
device
makes thishas
plan less appealing.
In
the
operating
room,
if
the
bowel
can
be
reduced
into
the
abdomen
and
the
defect
closed
primarily
(or
by
delayed
primary
repair),
then
it
should
be
done.
decision
ofbewhether
a baby
can tolerate
reduction and
repair
can
be
diThe
cultclosure.
and can
aidedofby
measuring
the Hg
intragastric
pressure
during
attempted
A pressure
less
than
20 mm
predicts
successful
closure
without
complications
of
excessive
intra-abdominal
pressure
[30].
Other
methods
reported
to help
in the
decision
close or not
to close and
are measuring
indioxide.
central
pressure,
intoventilatory
pressures,
in endtidalchanges
carbon
Ifvenous
the baby
is
when
the posterior
fascia
is closed,
then
an
umbilicus
should
be constructed
at stable
the methods
level
of the
iliac
crest
during
the
skin
closure.
Several
di

erent
have
been
described
that
achieve
an
acceptable
long-term
contour
by
avoiding
the
long-term
tendency
of
surgically
sutured
skin
folds
to

atten
[31].
Creation
of
an
umbilicus
can
alwaysSilastic
be deferred
a latertotime.
If primary
repair
is not possible,
then
a formal
silo di
is to
sutured
the fascia
andreduction
serial
reduction
is done
postoperatively.
Several
erent methods
of
serial
have been
described,
but
the
authors
preference
is
to
use
a
specially
designed
wringer
clamp
with
a
guard
that
allows
the
Silastic
sheets
to
be
approximated
while
pushing
the
bowel
down
and
away
from
the
roller
mechanism
[32].
The
incremental
reduction
steps
are
easy,
and aeasily
reversible
and can
be
done
multiple
times
during
thequick,
day
and
permit
gradual
reclamation
of with
abdominal
domain.
The
reduction
istodone
as tolerated
over
several days,
the
goal
to

n
ish
it
within
a
week
10
days
to
avoid
the
serious
complications
of
wound
infection
and
necrosis
that
result
in
separation
of
the
Silastic
sheets
away
from the
fascia,
an open wall,
abdomen.
After
the viscera
have
been
reduced
levelcreating
of
the baby
is returned
to
the operating
room to
forthe
removal
ofthe
theabdominal
silo and closure.
Babies
who
have
gastroschisis
and
associated
intestinal
atresia
pose
a
serious
Ifthe
bowel
is in
good condition
and
theofabdomen
can be
closedchallenge.
without
di
culty,
then
combined
primaryTherefore,
repair
bothan
defects
is
possible,
but
ideal
circumstances
are unusual.
when
atresia
is
present
orsuch
suspected,
thestaged
rst priority
is toThe
close
the isabdomen
by with
primary,
delayed
primary,
or
silo
repair.
baby
maintained
gastric
decompression
and
parenteral
nutrition
for
several
weeks
until
repeat
laparotomy
repair
of
the peel
intestinalresolve
atresia
[10].theThis
stagingcontents
of the repairs
allows and
the
in
amm
atory
and
herniated
to
gain
domain
of the
abdomen
beforetoopening
the bowel
and creating
a vulnerable
anastomosis.
For
omphalocele,
the
strategy
is
markedly
di

eren
t.
First,
if
the
covering
sac
is
intact, then
there is no the
urgency
to perform
operative
closure.for
So long as the
viscera
membrane,
complete
evaluation
defects are
cancovered
be donewith
and other
problemsa treated.
When
the baby isas- sociated

GASTROSCHISIS AND OMPHALOCELE

257

otherwise
stable
and if the
the defect
is relatively
small, then
a primary
repair
can
be
doneand
by
excising
omphalocele
membrane,
reducing
the
herniated
viscera,
closing
the excision
fascia
and
skin.
Membrane
overlying
the liver
that
might
be injured
during
can
be left
in place.but
When
primary
closure
is
not
likely
to
be
possible,
there
are
many
options,
the
authors
preference
is to treat theover
omphalocele
sac
withto
topical
silver
sulfadiazine
and allow
it
to epithelialize
ensuing
weeks
months
[33,34].
Enteral
feedings
are
usually
tolerated
afterthe
thehave
baby
recovers
from the
any
systemic
problems.
After
the
associated
problems
been
addressed,
family
can
be
taught
the
relatively
easy
wound
care,
and
the
baby
is
followed
in
the
outpatient
clinic.
When
the
sac is epithelialized
ordone
otherwise
sturdy bandages
enough to
withstand
external
pressure,
compression
is
with
elastic
and
serially
increased
until theareabdominal
areis epithelialized,
reduced. When
the
abdominal
reduced,
thecontents
membrane
the
baby
is within
well,contents
a6 ventral
hernia
repair
is
done.
This
canwaiting
usuallyeven
beand
accomplished
to
12
months,
but
there
is
little
risk
in
longer.
The fascial
defect
remains
roughlyeasy
thelate
same
size ofwhile
the giant
babyomphagrows
around
it.
This
permits
a relatively
closure
initially
loceles.
This
strategy
was
initially
adopted
for
onlybut
those
patients
who
giant
omphaloceles
or
serious
associated
problems
hasmethod
worked
so
wellhad
in
those
di

c
ult
cases
that
it
is
now
the
authors
preferred
for
any
defect
thatbreakdown,
cannot be infection,
closed. It and
avoids
problems
offeedings
pulmonary
compromise,
wound
delayed
enteral
that
are
encountered
withobtaining
a major operation
in a vulnerable
newborn.
It isofalso
useful
for
fascialstrategies
closure
in
epigastric
region
largeespecially
omphaloceles.
Many
alternative
forthe
omphalocele
closure
have
been
described,
including
skin
coverage
only,
staged
Silastic
silo
reduction
and
repair,
reduction
within
the
omphalocele
membrane,
amnion
inversion,
and
fascial
patches.
Each
technique
may
have
utility
in
certain
circumstances,
butdelayed
in the authors
topical ventral
treatment
withhas
silver
sulfadiazineeand
repair of experience,
the epithelialized
hernia
been
safe
and
ective.
Outcomes
The outcome
patients who
have
gastroschisis
depends
onwho
the
condition
of the of
vulnerable
bowel,
the outcome
of largely
patients
have
omphalocele
depends
largely
onwhereas
the
associated
anomalies
and medical
conditions.
Overall,
patients
who
have
gastroschisis
have
an
excellent
prognosis.
Survival is atbowel
least 90%
to 95%,and
with
of the deaths in patients
who
have catastrophic
loss,
themost
long-term
of short
bowel
syndrome.do
Patients
whosepsis,
have
atresia
and
relativecomplications
short bowel
syndrome
may
eventually
well despite
a long
initial
hospitalization
and prolonged
dependence
on
hyperalimentation.
Even
babies
who
have
intact
intestinal
tracts may require a prolonged hospitalization of weeks to months

258

LEDBETTER

because
ofmanifested
slow tolerance
of enteral feeding
[9]. Aonform
of necrotizing
enterocolitis
by pneumatosis
intestinalis
abdominal
radiograph
is
a unique
form ofrepair
intestinal
that
occurs
in the
postoperative
period
after
gastroschisis
wheninjury
feedings
are
being
advanced.
Feedings
are
often
complicated
by
gastroesophageal
re

ux
that
may
be
severe
[35].
Long-term
isobstruction.
usually good,
although
there have
is a
5%
to 10% gastrointestinal
long-term
risk
offunction
adhesive
Occasional
patients
inexplicable
long-term
intolerance
of
enteral
feedings.
The
outcome
of
babies
who have
omphalocele is much harder to generalize, but most
mortality
abdominaland
wallmorbidity
defect. is related to associated anomalies rather than the
Summary
Although
gastroschisis
anderence
omphalocele
are distinct abdominal
wall defects
with many
important
s,principles
an understanding
of the normal
development
of
themanagement
abdominaldiwall
and
ofreconstruction
intra-abdominal
pressure
evaluation
and
and
abdominal
wall
are
important
for
both
conditions.
Gastroschisis
is
likely
the
result
of
a
discrete
abdominal
wall bowel
ischemic
event
most ofof
the morbidity is may
a result
of acquired
in utero
injury.
Theand
correction
be accomplished
primary, delayed
orgeneral
stagedgastroschisis
repair;
the exact
method
is
largely by
determined
by the primary,
babys
medical
condition
and
capacity
of
the
abdominal
cavity
relative
to
the
herniated
viscera.
The
long-term
prognosis
is excellent.
Omphaloceleandis is
likely
the result
of aa much
more
general
defect
of
body
wall
development
associated
with
higher
inci-straightforward;
dence of serious
anomalies.
Thea surgical
correction
of small
defects
larger
defects,
strategy
of
topical
treatment
of
the ismembrane
withforsilver
sulfadiazine
and
late
repair
after
epithelialization
and
external
reduction
of
the
hernia
contents
has
simpli
ed
management
and
optimized
outcomes.
Rather
than being isrelated
to the
abdominal
wall
defect,
the
long-term
outcome
in
gastroschisis
mainly
related
to
the
degree
of
associated
intestinal
injury,
and
the
long-term
outcome
in
omphalocele is mainly related to the associated anomalies.
References
[1] Vermeij-Keers
NG,
van der WerSemin
JF. Embryonic
development
of the ventral
body wall and C,
its Hartwig
congenital
malformations.
Pediatr Surg
1996;5(2):829.
[2] deVries PA. The pathogenesis of gastroschisis and omphalocele. J Pediatr Surg 1980;15(3):
24551.
[3] Hoyme HE, Higginbottom MC, Jones KL. The vascular pathogenesis of gastroschisis: intrauterine interruption of the omphalomesenteric artery. J Pediatr 1981;98(2):22831.
[4] Glick PL, Harrison MR, Adzick NS, et al. The missing link in the pathogenesis of gastroschisis. J Pediatr Surg 1985;20(4):4069.

GASTROSCHISIS AND OMPHALOCELE

259

[5] Cantrell
Haller
JA, Ravitch
MM.pericardium,
A syndrome and
of congenital
involving
abdominal JR,
wall,
sternum,
diaphragm,
heart. Surgdefects
Gynecol
Obstetthe
1958;
107(5):60214.
[6] Curry JI, McKinney P, Thornton JG, et al. The aetiology of gastroschisis. Br J Obstet
Gynaecol 2000;107(11):133946.
[7] Tan KH, Kilby MD, Whittle MJ, et al. Congenital anterior abdominal wall defects in England and Wales 198793: retrospective analysis of OPCS data. BMJ 1996;313(7062):9036.
[8] Rankin J, Dillon E, Wright C. Congenital anterior abdominal wall defects in the north of
England, 19861996: occurrence and outcome. Prenat Diagn 1999;19(7):6628.
[9] Molik KA, Gingalewski CA, West KW, et al. Gastroschisis: a plea for risk categorization.
J Pediatr Surg 2001;36(1):515.
[10] Snyder CL, Miller KA, Sharp RJ, et al. Management of intestinal atresia in patients with
gastroschisis. J Pediatr Surg 2001;36(10):15425.
[11] Hwang PJ, Kousse BG. Omphalocele and gastroschisis: an 18-year review study. Genet
Med 2004;6(4):2326.
[12] Nicolaides KH, Snijders RJ, Cheng HH, et al. Fetal gastro-intestinal and abdominal wall defects: associated malformations and chromosomal abnormalities. Fetal Diagn Ther 1992;
7(2):10215.
[13] Nyberg DA, Fitzsimmons J, Mack LA, et al. Chromosomal abnormalities in fetuses with
omphalocele. Signicance of omphalocele contents. J Ultrasound Med 1989;8(6):299308.
[14] Palomaki GE, Hill LE, Knight GJ, et al. Second-trimester maternal serum alpha-fetoprotein
levels in pregnancies associated with gastroschisis and omphalocele. Obstet Gynecol 1988;
71(6 Pt 1):9069.
[15] Saller DN Jr, Canick JA, Palomaki GE, et al. Second-trimester maternal serum alpha-fetoprotein, unconjugated estriol, and hCG levels in pregnancies with ventral wall defects. Obstet
Gynecol 1994;84(5):8525.
[16] Walkinshaw SA, Renwick M, Hebisch G, et al. How good is ultrasound in the detection and
evaluation of anterior abdominal wall defects? Br J Radiol 1992;65(772):298301.
[17] Japaraj RP, Hockey R, Chan FY. Gastroschisis: can prenatal sonography predict neonatal
outcome? Ultrasound Obstet Gynecol 2003;21(4):32933.
[18] Luton D, Guibourdenche J, Vuillard E, et al. Prenatal management of gastroschisis: the
place of the amnioexchange procedure. Clin Perinatol 2003;30(3):55172 [viii].
[19] Salomon LJ, Mahieu-Caputo D, Jouvet P, et al. Fetal home monitoring for the prenatal
management of gastroschisis. Acta Obstet Gynecol Scand 2004;83(11):10614.
[20] Fitzsimmons J, Nyberg DA, Cyr DR, et al. Perinatal management of gastroschisis. Obstet
Gynecol 1988;71(6 Pt 1):9103.
[21] Moir CR, Ramsey PS, Ogburn PL, et al. A prospective trial of elective preterm delivery for
fetal gastroschisis. Am J Perinatol 2004;21(5):28994.
[22] Puligandla PS, Janvier A, Flageole H, et al. The signicance of intrauterine growth restriction is dierent from prematurity for the outcome of infants with gastroschisis. J Pediatr
Surg 2004;39(8):12004.
[23] Hsieh TT, Lai YM, Liou JD, et al. Management of the fetus with an abdominal wall defect:
experience of 31 cases. Taiwan Yi Xue Hui Za Zhi 1989;88(5):46973.
[24] How HY, Harris BJ, Pietrantoni M, et al. Is vaginal delivery preferable to elective cesarean
delivery in fetuses with a known ventral wall defect? Am J Obstet Gynecol 2000;182(6):
152734.
[25] Hershenson MB, Brouillette RT, Klemka L, et al. Respiratory insuciency in newborns with
abdominal wall defects. J Pediatr Surg 1985;20(4):34853.
[26] Langer JC. Gastroschisis and omphalocele. Semin Pediatr Surg 1996;5(2):1248.
[27] Minkes RK, Langer JC, Mazziotti MV, et al. Routine insertion of a silastic spring-loaded
silo for infants with gastroschisis. J Pediatr Surg 2000;35(6):8436.
[28] Coughlin JP, Drucker DE, Jewell MR, et al. Delivery room repair of gastroschisis. Surgery
1993;114(4):8226 [discussion: 67].

260

LEDBETTER

[29] Bianchi
A, Dickson
AP,and
Alizai
NK. Elective
delayed
midgut
anesthesia for
gastroschisis:
selection
conversion
criteria.
J Pediatr
Surgreductiondno
2002;37(9):13346.
[30] Yaster
M, Scherer
TL, Stone
et al. Prediction
of successful
primarySurg
closure
of congen- ital
abdominal
wall defects
usingMM,
intraoperative
measurements.
J Pediatr
1989;24(12):
121720.
[31] Itoh Y, Arai K. Umbilical reconstruction using a cone-shaped ap. Ann Plast Surg 1992;
28(4):3358.
[32] Sawin R, Glick P, Schaller R, et al. Gastroschisis wringer clamp: a safe, simplied method for
delayed primary closure. J Pediatr Surg 1992;27(10):13468.
[33] Hatch EI Jr, Baxter R. Surgical options in the management of large omphaloceles. Am J Surg
1987;153(5):44952.
[34] Nuchtern JG, Baxter R, Hatch EI Jr. Nonoperative initial management versus silon chimney
for treatment of giant omphalocele. J Pediatr Surg 1995;30(6):7716.
[35] Beaudoin S, Kieer G, Sapin E, et al. Gastroesophageal reux in neonates with congenital
abdominal wall defect. Eur J Pediatr Surg 1995;5(6):3236.