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PEDIATRIC SURGERY

ISBN: 978-0-323-07255-7
Volume 1 9996085473
Volume 2 9996085538

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Library of Congress Cataloging-in-Publication Data

Pediatric surgery. 7th ed. / editor in chief, Arnold G. Coran ; associate editors, N.
Scott Adzick . . . [et al.].
p. ; cm.
Includes bibliographical references and index.
ISBN 978-0-323-07255-7 (2 vol. set : hardcover : alk. paper)
I. Coran, Arnold G., 1938- II. Adzick, N. Scott.
[DNLM: 1. Surgical Procedures, Operative. 2. Child. 3. Infant. WO 925]
617.98dc23
2011045740

Editor: Judith Fletcher

Developmental Editor: Lisa Barnes
Publishing Services Manager: Patricia Tannian
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Designer: Ellen Zanolle

Printed in the United States of America

Last digit is the print number:

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3 2 1

Max Grob.1214 Toxic effects of mercurochrome were described later.1517 A modern version was introduced by Ein and
Shandling, who used an adhesive semipermeable artificial
membrane.18 Olshausen in 1887 first reported skin flap coverage of defects after removal of the membrane,19 and Gross
further demonstrated its effectiveness and popularized the technique.20 Staged closure of the resultant hernia could be difficult
because of failure of the abdominal cavity to grow without the
impetus of the intestines within it, and because of intestinal
adhesions to the skin flaps. For this reason, the skin was
sometimes closed over an intact omphalocele sac.21,22
In 1967 Schuster introduced staged reduction of large
omphaloceles with prosthetic material because he noted
that the abdominal cavity did not grow with skin closure
alone.23 The fact that no operative technique has achieved
universal success or acceptance is attested to by the many
ingenious methods that continue to be devised including skin
grafting,24,25 pneumoperitoneum and tissue expanders to
stretch the abdominal wall in preparation for closure,2628
partial hepatectomy,29,30 lateral relaxing incisions in the
fascia,31 and division of the rectus abdominis muscles.32

CHAPTER 75

Spectrum of Clinical Congenital

Abdominal Wall Defects
------------------------------------------------------------------------------------------------------------------------------------------------

Congenital Defects
of the Abdominal
Wall
Michael D. Klein

History
------------------------------------------------------------------------------------------------------------------------------------------------

Newborns with abdominal wall defects were reported in the

first century AD by Aulus Cornelius Celsus, a Roman physician, and then by Paulus Aegineta in the fifth century.1
Omphalocele is described in the sixteenth century printed
works of Ambrose Pare,2 and Lycosthenes may have been
the first to describe gastroschisis at about the same time.3
Taruffi introduced the term gastroschisis in 1894.4 For many
years gastroschisis was confused with an omphalocele that
had a torn sac.5 Moore and Stokes are credited with limiting
it to the specific clinical entity as we understand it today,6
although they give credit to earlier authors who used the term,
such as Bernstein,7 but not to the earliest collected series of
Massabuau and Guibal.8
The first successful repair of omphalocele was reported
by Hey in 1802.9 In 1873 Visick described the successful
repair of gastroschisis.10 Ahlfeld11 in 1899 described painting
an omphalocele sac with alcohol to produce an eschar
and awaiting contraction and epithelialization. The use of
mercurochrome for painting the sac was popularized by

The clinically important defects are all umbilical with intact

rectus abdominis muscles (Table 75-1). Omphalocele is a
large defect (>4 cm) covered by amniotic membrane that contains midgut and other abdominal organs including the liver
and often the spleen and gonad (Fig. 75-1). One unusual form
of omphalocele is the cephalic fold defect, or pentalogy of
Cantrell, in which the abdominal wall defect is supraumbilical
and the heart is in the sac through a defect in the pericardium
and the central tendon of the diaphragm.33 The other elements of the pentalogy are an intracardiac defect and a sternal
cleft. Ectopia cordis thoracis (when the heart is outside the
chest with no pericardial covering as opposed to being inside
the omphalocele sac) might be considered a form of a cephalic
fold defect (Fig. 75-2).
Another unusual omphalocele is the caudal fold defect,
cloacal exstrophy, in which the defect is infraumbilical and
accompanied by exstrophy of the bladder, epispadias, diastasis
of the pubic rami, and imperforate anus (see Chapter 120).
The ileum prolapses between the two halves of the exstrophied
bladder.
Gastroschisis is less than 4 cm in diameter, has no covering
membrane, and usually contains only the midgut with the stomach and possibly a gonad. It is almost always to the right of the
umbilical cord, although exceptions do occur (Fig. 75-3).34
Occasionally, a skin bridge may be present between the cord
and the defect, but the abdominal wall and its muscles are
normal. At birth the bowel can appear perfectly normal, but
more than 20 minutes after birth, the extruded intestine
may be thickened and covered with a fibrinous exudate matted
together so that individual loops cannot be distinguished.
There have been several reports of gastroschisis with a small
remnant of midgut appearing above a defect that has essentially closed, most likely caused by antenatal volvulus.35,36
Most authors recognize patients with gastroschisis and an
associated gastrointestinal condition such as atresia, perforation, necrosis, or volvulus as a separate entity with a poorer
973

974

PART VII

ABDOMEN

TABLE 75-1
Comparison of Congenital Abdominal Wall Defects
Defect

Site

Sac

Contents

Frequency

Associated Anomalies

Outcome

Omphalocelelateral fold

Umbilicus

Yes

Common

Chromosomal, cardiac

Omphalocelecephalic
fold (pentalogy of Cantrell)

Superior
umbilicus

Yes

Liver, intestine,
spleen, gonad
Liver, intestine

Rare

Omphalocelecaudal fold
(cloacal exstrophy)
Umbilical cord hernia
Gastroschisis

Inferior
umbilicus
Umbilicus
Right
umbilicus
Midline
sternum

Yes

Intestine

Rare

Yes
No

Intestine
Intestine

Unusual
Common

Cardiac, sternal cleft, pericardial

defect, central tendon diaphragm
defect
Bladder exstrophy, imperforate anus,
epispadias
Uncommon
Intestinal atresia

Good (depending on
associated anomalies)
Poor

Good
Good

No

Heart

Rare

Cardiac

Poor

Ectopia cordis thoracis

Fair

FIGURE 75-1 Omphalocele.

FIGURE 75-3 Gastroschisis.

FIGURE 75-2 Ectopia cordis.

FIGURE 75-4 Umbilical cord hernia.

outcome.37 This is usually called complicated gastroschisis.

Gastroschisis in the fetus is probably associated with intrauterine distress. Neonates with gastroschisis are more frequently
premature and commonly have respiratory problems. Even
term babies with gastroschisis are more likely to be small for
gestational age3840 and to have younger mothers.41
Umbilical cord hernia is least common. It is also less than
4 cm and contains only the midgut, but it is covered by a
membrane (Fig. 75-4). It is often confused with omphalocele.
The differences are that it contains only midgut, never liver,

and the abdominal wall above the defect is normal, with the
rectus muscles meeting in the midline at the xiphoid. Few associated anomalies are reported with this defect. Like all abdominal defects in which the midgut has not returned to
the abdominal cavity before birth to allow for rotation and fixation, these patients have malrotation, although it is not usually a cause of intestinal obstruction.
Umbilical hernia is distinguished from these anomalies by two features: (1) The defect is covered by normal
skin, and (2) it is only rarely present at birth, instead usually

CHAPTER 75

becoming apparent in the first weeks or months of life. In the

literature before 1970 and in the literature outside pediatric surgery even today, these three forms are often confused, particularly
omphalocele/gastroschisis and omphalocele/umbilical cord
hernia.
Infants with congenital abdominal wall muscular deficiency, or prune-belly, syndrome have all the normal layers
of the abdominal wall but little muscle in the loose areolar
tissue. Much of the morbidity is related to similar muscular
deficiency in the genitourinary and gastrointestinal tracts.
Other abdominal wall defects, often incompatible with
life,4244 have been described in humans45,46 including true
absence of abdominal wall structures with evisceration of
bowel.47,48

Embryology
------------------------------------------------------------------------------------------------------------------------------------------------

EMBRYOLOGY OF THE ABDOMINAL WALL

At 3 weeks gestation the flat disk of the embryo develops four
folds that will enclose the body cavities (Fig. 75-5, A and B).
Two lateral folds form the pleuroperitoneal canals once they

CONGENITAL DEFECTS OF THE ABDOMINAL WALL

975

meet anteriorly in the midline. The cephalic fold brings down

with it the developing heart, which actually began distal to the
brain, but now takes its place within the anterior chest wall.
It also carries the septum transversum, which continues
posteriorly and divides the pleuroperitoneal canals into the
pleural and peritoneal cavities. The caudal fold brings with
it the developing bladder or allantois, which started off distal
to the anus. During this process the gut tube has formed along
the length of the embryo with a communication at the umbilicus to the yolk sac; the yolk sac will eventually disappear,
sometimes leaving a vitelline duct remnant on the distal ileum.
At about 5 weeks gestation, this gut tube elongates and develops within the umbilical coelom (Fig. 75-5, C), a cavity in
the body stalk on the anterior surface of the embryo. At about
10 weeks gestation the gut returns from the space within the
umbilical stalk to the peritoneal cavity and undergoes rotation
and fixation.

EMBRYOGENESIS OF THE DEFECTS

Omphalocele represents a failure of the body folds to complete
their journey.49 Most omphaloceles are lateral fold defects and
are always at the umbilicus. The rectus muscles often insert far
apart on the costal margins and for this reason cannot be

Amnion
Neural tube

Mesentery Pleuroperitoneal space

Amnion

Heart
Intestine

Yolk sac

Midgut

Yolk sac
Umbilical vein

Umbilical coelom

Resorbed right
umbilical vein

Cecum
Coelom

Aorta
Allantois
Umbilical artery

Vitelline duct
and vessels

Urachus
Umbilical arteries

FIGURE 75-5 Embryology of the abdominal wall. A, Two-week embryo as a flat disk before folding to form the body cavities. B, At 4 weeks the folding is
complete. The gut tube is about to be pinched off the yolk sac. C, At 6 weeks the elongating midgut enters the umbilical coelom. D, A view from inside the
abdominal cavity showing the relatively unsupported right side of the umbilicus as a result of resorption of the right umbilical vein.

976

PART VII

ABDOMEN

brought entirely together in a repair. Whatever the insult may

be that causes it, this aberration occurs early in embryogenesis
and is thus likely to affect other organ systems as well, so children with omphalocele frequently have associated anomalies.
Cephalic fold defects result in ectopia cordis or the pentalogy
of Cantrell, whereas caudal fold defects cause bladder and
cloacal exstrophy. Gastroschisis, antenatal exposure of the
viscera, is probably caused by failure of the umbilical coelom
to develop.50 The elongating intestine then has no space in
which to expand and ruptures out the body wall just to the
right of the umbilicus, possibly because the right side of the
umbilicus is relatively unsupported as a result of resorption
of the right umbilical vein at about 4 weeks gestation
(Fig. 75-5, D).51 An alternate explanation that the yolk sac
and associated vitelline structures fail to incorporate into
the umbilical cord, thus allowing the midgut to exit the abdomen at the point they exit, is also reasonable.52 Thus gastroschisis has no covering membrane. In gastroschisis the bowel
is usually thickened, matted, edematous, and covered with a
fibrinous peel. Some have explained the latter appearance on
the basis of the change in amniotic fluid electrolyte composition with the onset of fetal kidney function.5356 Evidence
from studies in animals indicates that the peel forms in
utero5759 and that it is a postnatal event.60 Some investigators
have related the condition of the bowel to the presence of
meconium from the fetus in the amniotic fluid.19,61 In a recent
clinical study, neonates delivered with meconium staining
had a fibrinous peel, whereas those without staining had
none.55 Our clinical observation of more than 50 deliveries
indicates that the appearance of the bowel is most often a
postnatal event (Fig. 75-6). At the moment of birth, the bowel
in gastroschisis is usually quite normal. Twenty minutes later
it begins to acquire the characteristic changes. These changes
may be due to exposure to air, but more likely they are
related to mesenteric venous occlusion at the level of the
abdominal wall defect with resultant edema and transudation
of proteinaceous fluid.
Umbilical cord hernia is a simple failure of the midgut to
return to the peritoneal cavity at 10 to 12 weeks. Thus this
defect contains only midgut and is covered by a membrane.
Such hernias are much smaller than omphaloceles and have
a better outlook. Distinguishing umbilical cord hernia from
omphalocele both embryologically and clinically is important

FIGURE 75-6 Gastroschisis at delivery. The bowel does not appear

matted, edematous, and coated with a fibrinous peel.

in managing patients and reporting results. Margulies61 first translated the embryologic work of Pernkopf62 and Politzer and Sternberg63 into English, which permitted Benson, Penberthy, and
Hill64 to recognize it as a separate clinical entity.
Alternative explanations for these various defects, especially gastroschisis, have been presented, but none have been
generally accepted. Some have thought that gastroschisis must
represent failure of mesodermalization with actual absence
of abdominal wall components.65,66 Although such anomalies
have been reported, they are usually stillborn monstrosities.
Nearly all live-born children with abdominal wall defects
have intact abdominal walls with normal muscle layers. More
recently there has been speculation that decreased blood flow
in the omphalomesenteric artery might be a cause.67 This has
resulted in many studies on the use of vasoconstrictive agents
in the first trimester. Because the omphalomesenteric artery
supplies intestine and not the abdominal wall, it is difficult
to see how this has gained such credence.
There are many reports of abdominal wall defects induced
in animals by exogenous agents. In a comprehensive review of
the literature gastroschisis was induced by 22 teratogens,
omphalocele by 9, and umbilical cord hernia by 8.68 Such
studies raise the issue of whether the apparent increase in
incidence of gastroschisis since 1970 might be due to new
environmental teratogens. Studies of abdominal wall defects
in animals might not necessarily be relevant. One recent example is the superb work of Brewer and colleagues on the mouse
knockout model of congenital abdominal wall defects.69,70
These investigators demonstrate that the AP-2a transcription factor is important for the normal development of the
mouse abdominal wall. They provide beautiful illustrations
of the development of the normal abdominal wall and its
abnormal development in the knockout model. Yet their
careful description allows the clinician to conclude that the
defect presented does not represent any of the clinical defects
pediatric surgeons see.
Possible causes of abdominal wall defects have also been
investigated in clinical material that has recently been
reviewed.71 For gastroschisis, demographic risk factors for
which there is more than isolated evidence include young
maternal age, low socioeconomic status, absence of the maternal father, poor maternal prenatal care, and primigravida
status. Nutritional factors are low levels of glutathione and
a-carotene, high levels of nitrosamines, absence of supplemental vitamin intake during pregnancy, folic acid fortification, and general, poor nutrition. Maternal obesity was
actually found to be protective. Most, but not all, studies show
no familial or genetic risk factors. There is little evidence that
living near chemical plants, farms, landfills, or other specific
sites is a risk factor. Vasoconstrictive agents are repeatedly
reported as related risk factors. Many studies confirmed that
use of illegal drugs including cocaine, methamphetamine,
and marijuana during pregnancy was a risk factor. Risk factors
for omphalocele are different from those for gastroschisis.
Demographic factors include both advanced and very young
maternal age and maternal obesity. Nutritional factors are
failure to use multivitamins during pregnancy (especially
Vitamin B 12), lack of folic acid fortification, and alterations
in glycemic control. No strong evidence is found for medication, illegal drug, or lifestyle factors, although maternal history
of febrile illness and in vitro fertilization do appear to be
risk factors.

CHAPTER 75

GENETICS AND FAMILIAL OCCURRENCE

CONGENITAL DEFECTS OF THE ABDOMINAL WALL

977

Antenatal Considerations
------------------------------------------------------------------------------------------------------------------------------------------------

Although rare, reports of abdominal wall defects, mainly

omphaloceles, occurring in families and even in twins have
occurred.7274 No specific genes have been identified with gastroschisis, but omphalocele is associated with chromosomal
anomalies (especially Trisomy 18), and candidates have been
reported for specific genes including PITX2, CDKN1C, MTHFR,
and 677C-T (folate). OEIS is closely related to caudal fold
omphalocele and may be related to a specific gene.72 Many syndromes include abdominal wall defects. Beckwith-Wiedemann
syndrome (congenital abdominal wall defect, macroglossia, and
hypoglycemia with a propensity for the development of abdominal tumors later in life) is the most common.73,74 It is remarkable that the abdominal wall defect in Beckwith-Wiedemann
syndrome can be any of the three aforementioned entities,
and yet we presume that they all have a different embryogenesis.
This syndrome also appears to be associated with assisted
reproductive technologies.75 Other syndromes that include
omphalocele are displayed in Table 75-2.

ULTRASOUND
Today, the diagnosis is usually made antenatally by ultrasound
(US).76 Omphalocele can be distinguished from gastroschisis
by the presence of a sac and from umbilical cord hernia by
the presence of the liver in the defect. The diagnosis allows
for antenatal counseling, which given the generally good
prognosis, can be reassuring. In a report from 11 European
antenatal US registries in 2001, the sensitivity for detecting
omphalocele was 75% (range, 25% to 100%), and that for
gastroschisis was 83% (18% to 100%).77 The first age at which
omphalocele was detected was 18
6 weeks, and gastroschisis, 20 7 weeks. Only 41% of fetuses with omphaloceles
detected antenatally were live-born. Twenty-two percent were
fetal deaths, and in 37% the pregnancies were terminated.
Fifty-nine percent of fetuses with gastroschisis were live-born,
with 12% being fetal deaths and 29% terminations. Recent
reports show no significant changes.78,79

TABLE 75-2
Omphalocele Syndromes
Name

Description

Inheritance

OMIM #

Shprintzen omphalocele syndrome

Malformation syndrome that includes mildly

dysmorphic facies, omphalocele, scoliosis,
learning disabilities, and pharyngeal and
laryngeal hypoplasia
Lethal syndrome associated with uterus
bicornis in one case, uvula duplex and
hydrocephalus internus in another,
omphalocele, and cleft palate
Pediatric overgrowth disorder involving
a predisposition to tumor development.
The clinical presentation is highly variable;
some cases lack the hallmark features of
exomphalos, macroglossia, and gigantism.
Abdominal wall defects common, as well
as visceromegaly including liver, spleen,
pancreas, kidneys, and adrenals
Large/giant omphalocele containing
liver and intestines. Also associated with
diaphragmatic hernia and radial ray
defects
Unusual facies, polydactyly, cardiac
abnormality, large omphalocele in a
few cases

Autosomal dominant

182210

Omphalocelecleft palate syndrome

Beckwith-Wiedemann syndrome
(also known as exomphalosmacroglossia-gigantism syndrome
[EMG syndrome] and WiedemannBeckwith syndrome [WBS])

Gershoni-Baruch syndrome

C syndrome (Opitz trigonocephaly

syndrome, Trigonocephaly
syndrome)
Donnai-Barrow syndrome
(Faciooculoacousticorenal
syndrome)
Thoracoabdominal syndrome (THAS)

Manitoba oculotrichoanal syndrome

(MOTA) (Marles syndrome)

Craniosynostosismental retardation
syndrome of Lin and Gettig

Facial anomalies, ocular anomalies,

sensorineural hearing loss, and proteinuria.
Some cases include omphalocele as an
associated anomaly
Diaphragmatic and ventral hernias, hypoplastic
lung, cardiac anomalies, cleft palate,
omphalocele, sporadic pentalogy of Cantrell
Hypertelorism, unilateral eye malformations,
aberrant anterolateral scalp hairline, nasal
and anal anomalies. Omphalocele noted
in several cases
Midline craniosynostosis, agenesis of the
corpus callosum, severe mental retardation,
unusual face, contractures, camptodactyly,
hypospadias, hypogonadism, small
omphalocele, and multiple small bowel
atresias

258320

Inheritance of BWS is complex. Possible

patterns include autosomal dominant
inheritance with variable expressivity,
contiguous gene duplication at 11p15,
and genomic imprinting resulting from
a defective or absent copy of the
maternally derived gene

130650

Autosomal recessive hypothesis in one

case

609545

Autosomal recessive mostly, autosomal

dominant in a few cases; disruption of
the CD96 gene involved with encoding
a member of the immunoglobulin family
Autosomal recessive; mutation in the
LRP2 gene

211750

X-linked dominant

313850

Autosomal recessive

248450

222448

218649

Continued

978

PART VII

ABDOMEN

TABLE 75-2
Omphalocele Syndromescontd
Name

Description

Chromosome 9p deletion syndrome

Trigonocephaly, flattened occiput, prominent

forehead, broad flat nasal bridge, anteverted
nares, malformed external ears, hypertelorism,
hypertonia. Omphalocele rare anomaly
Agonadism, hypoplasia of the right pulmonary
artery, hypoplasia of the right lung, isolated
dextrocardia with complex cardiac
malformations, and diaphragm hernia or
omphalocele
Excessive birth weight, swollen globular
body with a thick neck, apparently short
limbs, polydactyly, craniosynostosis with
acrocephaly, omphalocele, and
abnormal face
Popliteal pterygium with a cord containing
nerves and vessels, synostosis of hand and foot
bones with digital hypoplasia and syndactyly,
facial clefts, ankyloblepharon and filiform bands
between the jaws, omphalocele, aplasia of
the urethra
Mental and physical growth retardation,
hypertelorism, facial clefting, urogenital
abnormalities, eye abnormalities, hearing
loss, omphalocele, caudal appendage,
umbilical hernia
Severe micrognathia, rib defects, mental
retardation, microcephaly, histologic anomalies,
omphalocele
Diaphragmatic hernia, abnormal face, distal
limb anomalies*
Omphalocele, bladder exstrophy, imperforate
anus, and spinal defects

PAGOD syndrome (agonadism with

multiple internal malformations)

Acrocephalopolydactylous dysplasia
(Elejalde syndrome)

Popliteal pterygium syndrome

(lethal type) (Bartsocas-Papas
syndrome)

Malpuech facial clefting syndrome

(Facial clefting syndrome, gypsy
type)

Cerebrocostomandibular syndrome

Fryns syndrome
OEIS complex

Inheritance

OMIM #
158170

202660

Most likely autosomal recessive

200995

263650

Autosomal recessive (kindred highly

inbred)

248340

Both autosomal dominant and autosomal

recessive have been described

117650

Autosomal recessive

229850
258040

Data from references 194198.

*Omphalocele is not reported in OMIM but is noted in several case reports. The first reference also points to other syndromes not noted by OMIM.

Antenatal US also detects associated anomalies.80 In gastroschisis these anomalies are usually intestinal atresias.81,82
In omphalocele one study reports a 25% incidence of the
detection of major associated anomalies.83 The frequency of
associated cardiac anomalies in omphalocele makes antenatal
echocardiography helpful.84
Given the poor outcome of all forms of ectopia cordis,
termination could be a reasonable alternative when US demonstrates the heart outside the chest. Some ultrasonographers
have attempted to correlate the bowel problems of gastroschisis with the antenatal appearance on US,85,86,90 but
this has not been successful in all centers including more
recent experience.82,91,92
Routine use of antenatal US has not been definitively
shown to improve perinatal morbidity or maternal outcome,
although there may be some survival benefit for a fetus with
a life-threatening anomaly. There may, however, be a cost
savings, if the mother chooses termination of pregnancy.
Such has not been the case for either omphalocele or gastroschisis,93 although termination rates for these treatable
anomalies can be high (63% in one study).94 A recent report
demonstrated that multidisciplinary prenatal care for mothers
carrying pregnancies with gastroschisis produced infants
with higher birthweights and greater gestational age, although
there was no difference in the outcome for gastroschisis or
the likelihood for a successful vaginal delivery.95

AMNIOTIC FLUID AND SERUM TESTS

Elevated alpha fetoprotein (AFP) in both maternal serum and
amniotic fluid and elevated amniotic fluid acetylcholinesterase
(AChE) have been correlated with abdominal wall defects
when there is no myelomeningocele.96 In a study of 23
pregnancies with gastroschisis and 17 with omphalocele,
second-trimester serum AFP was 9.42 times greater than normal in gastroschisis and 4.18 times normal in omphalocele.97
Another study found elevated amniotic fluid AFP in 100% of
pregnancies with gastroschisis and in only 20% of those with
omphalocele. AChE was elevated in 80% of pregnancies
with gastroschisis and 27% of those with omphaloceles.98

Obstetric Delivery
------------------------------------------------------------------------------------------------------------------------------------------------

Intuitively, it may seem appropriate to deliver these patients

by cesarean section to avoid injury to the bowel or tearing
of the omphalocele sac, and some reports claim a benefit
for cesarean section.99101 There is also, however, a report
of two patients with gastroschisis whose bowel was injured
during cesarean section delivery.102 The more recent obstetric
literature finds no benefit of cesarean section.103110 Some
reports even show no benefit with referral of the mother for
delivery in a pediatric surgery center.111,112 One must conclude that the mode of delivery is a decision to be made by

CHAPTER 75

the obstetrician on the basis of obstetric indications, not on the

presence of an abdominal wall defect.
The belief that the condition of the bowel in gastroschisis is
due to a relatively late (33 weeks) intrauterine event has led
some to recommend preterm delivery.113 Because we think
that the condition of the bowel is a postnatal event, we do
not believe that it is worth the risks of prematurity. It seems
most likely that the good results observed with preterm delivery by planned cesarean section are related to the fact that it
allows for immediate repair and avoids the venous congestion
of the mesentery and its effect on the intestine. Others have
also found no benefit of preterm delivery,99 and a recent study
shows increased morbidity with preterm delivery.114
Antenatal counseling and coordination with the obstetric
team are essential. Because the condition of the bowel in gastroschisis and distention of the bowel and size of the liver in
omphalocele are often related to the time between delivery
and repair, it is important to make arrangements for repair
as soon as possible after delivery.72

Clinical Features
------------------------------------------------------------------------------------------------------------------------------------------------

INCIDENCE AND ASSOCIATED CONDITIONS

Omphalocele
Before 1970, omphalocele was the most common of the
abdominal wall defects; it is now the second after gastroschisis. The overall incidence is 1 to 2.5 per 5000 live births8
with a male preponderance.115
Conditions associated with omphalocele are listed in
Table 75-3. Up to 45% of patients with omphalocele have
been reported to have a cardiac abnormality including ventricular septal defect, atrial septal defect, ectopia cordis, tricuspid atresia, coarctation of the aorta, and persistent pulmonary
hypertension of the newborn.116 Chromosomal abnormalities
can be found in up to 20%, and an association with Down
syndrome has also been reported.117 Patients with omphalocele are more likely to be large for gestational age (macrosomia
or > 4 kg in birth weight).118 Musculoskeletal and neural
tube defects are also reported in greater than expected incidence.119,120 Gastroesophageal reflux is more likely, with
43% being affected in one study.121
Gastroschisis
Gastroschisis has become the most common of the abdominal
wall defects over the past 30 years.122124 This may be related
to the increased incidence of prematurity and the increased
TABLE 75-3
Associated Conditions
System

Gastroschisis (%)

Omphalocele (%)

Cardiac
Respiratory
Central nervous system
Musculoskeletal
Gastrointestinal
Genitourinary
Facial
Chromosomal

2-12
<1
2-10
<1-10
5-40
3-10
1-3
<1-3

7-47
1-4
4-30
4-25
3-20
6-20
1-14
3-20

CONGENITAL DEFECTS OF THE ABDOMINAL WALL

979

survival of premature infants in general, or to the fact that it

was not until the 1970s that the distinction between gastroschisis and omphalocele was regularly made.44 The incidence
is about 2 to 4.9 per 10,000 live births,122,125127 with a male
preponderance.28,115
The anomalies associated with gastroschisis are usually
related to the midgut, with the most common being intestinal
atresia (see Table 75-3).128 In the first year of life infants with
gastroschisis are likely to have gastroesophageal reflux (16%)72
and undescended testicle (15%), although the latter often
corrects spontaneously.129,130 Many reports recognize congenitally short or dysmotile bowel with gastroschisis.131 Although
neither condition has been quantified in terms of severity
or incidence, they are certainly lower than the incidence of
atresia (<5%).
Omphalocele
Initial Care Although the bowel in omphalocele is protected by the sac, operation is still urgently needed to increase
the chance for primary closure. A nasogastric (NG) tube
should be placed early to decompress the intestines. A rectal
examination aids this with evacuation of meconium. Maintenance of body temperature is especially important. Ventilator
support and supplemental oxygen should be supplied
as needed.
Because of the frequency of associated heart defects, cardiology evaluation and echocardiography are in order, although
the results will not delay repair.132 Intravenous fluids are provided at maintenance rates, best through an upper extremity. If
not done at birth, 1 mg of vitamin K should be administered,
as well as prophylactic antibiotics
The value of primary closure has been debated. Some
believe that staged closure is so successful that it is preferable
to avoid the possible complications of increased abdominal
pressure, which include respiratory compromise, decreased
venous return with decreased urine output and cardiac output, compromise of the blood supply to the intestine, and
acidosis related to kinking of the hepatic veins as the liver
is reduced. Nonoperative initial treatment (painting the
sac with an antiseptic) is still useful when operative closure
is not possible.133
Operative Closure In the operating room the sac and abdomen are prepared with an antiseptic and excess cord is
removed. If the patient is hypoxic or unstable, we cannulate
and preserve the umbilical artery or vein (or both) for transplantation to the lateral abdominal wall for postoperative
monitoring, but this is rarely necessary.134 We always use plastic adhesive edge drapes to preserve heat. We first attempt to
reduce the abdominal contents with the sac intact, as a number of reports suggest.135137 Such reduction is often impossible either because the abdominal cavity is too small or
because the sac is adherent to the liver and falciform ligament.
We then incise the skin a few millimeters from the sac circumferentially around the defect and elevate skin flaps until the
rectus muscles can be identified. At this point the sac is
excised with ligation of the umbilical vessels and urachus.
Any sac adherent to the liver is divided such that some
remnants are left on the liver.
If the viscera cannot be reduced, it is helpful to stretch the
abdominal wall manually in a posterior-to-anterior direction.
The intestine is replaced first and then the liver. The liver

980

PART VII

ABDOMEN

effectively holds the intestine in place, and if skin flap closure

should be necessary, the secondary operation will be much
safer. For primary closure, mattress sutures are placed through
all layers of the abdominal wall except the skin. It is important
to place these sutures through the rectus abdominis muscles
and not just through the midline fascia, which may result in
a postoperative hernia. It will not be possible to appose the
rectus muscles in the upper portion of the incision because
they insert broadly on the costal margin. It is also not necessary because the liver fills this space. Turning anterior rectus
fascial flaps to cover this defect has been suggested, but we
have not found this to be necessary. It is best to place the
abdominal sutures without tying them and then pull alternate
sutures to either side to see how the patient will tolerate fascial
closure. If the anesthesiologist can ventilate the patient with
peak inspiratory pressures of less than 25 cm H2O, closure
is safe. The skin is closed with a running suture, and in most
patients this makes a scar that looks like an umbilicus. Several
methods have been described for performing a cosmetic
umbilicoplasty at the initial operation.138141 It often suffices
to close the skin incision in a subcuticular purse-string fashion
with absorbable suture material while taking every second or
third bite to the fascia. However, frequently not enough skin
is available for these procedures. In many cases the skin
will have a tenuous vascular supply and appear blanched.
Adhesive tape should not be used for a wound dressing.
Other methods have been suggested to determine whether
the fascial closure is too tight: a saphenous vein intravenous
line that will not drip by gravity or intravesical or NG tube
pressure greater than 20 cm H2O.32,142 Splanchnic perfusion
pressure (calculated as mean arterial pressure minus intraabdominal pressure measured either intragastric or intravesical) has been suggested as being an even better predictor.143
If the fascial closure is judged to be too tight, one can consider
only skin closure, but in our experience, if the closure is still
too tight after the maneuvers suggested, the skin closure will
also probably be too tight. In this case the viscera can be covered with a prosthetic silo that will allow slow reduction of the
abdominal viscera over a 1-week period. The simplest of these
devices is the preformed Silastic chimney with a spring-loaded
ring at the bottom, which is placed though the defect beneath
the edges of the abdominal wall (Fig. 75-7).144 The surgeon
can suture this ring to the abdominal wall to prevent the ring

FIGURE 75-7 Gastroschisis with bowel contained in a preformed

Silastic silo.

FIGURE 75-8 Gastroschisis with bowel contained in a hand-sewn

Silastic silo.

from extruding as pressure is applied to reduce the viscera.

Antibiotic ointment is applied around the edges as a dressing.
If this device is not available, one can use 0.007-inch-thick
Dacron-reinforced Silastic. We suture one piece to each side
of the abdominal wall so that the knots are on the outside
and the edges of both the fascia and Silastic face out. We then
separately suture up the inferior and superior edges and over
the top (Fig. 75-8).
We do not perform a Ladd procedure or appendectomy. If a
testicle is present in the sac, it can be placed in the abdominal
cavity because in most cases it will be in the scrotum in a year.
If an obvious atresia is present, we may bring out a single
stoma through normal abdominal wall or simply leave the
atresia in the abdomen and wait at least 6 weeks before
reoperating.
Most pediatric surgeons agree that giant omphalocele is a
separate category of defect that is particularly difficult to treat
and which has a much poorer outcome than the usual lateral
fold defect.145 As eloquently described by Campos and colleagues, there is no standard definition of this entity in terms
of size or amount of herniated viscera.146 It simply consists
of a degree of visceroabdominal disproportion that defies
treatment with fascial or even skin closure whether applied
primarily or after a staged closure. A recent report from the
Childrens Hospital of Philadelphia147 defines both in utero
and newborn giant omphalocele as one with 75% or more
of the liver in the sac. Unfortunately they did not present a
technique for determining the volume of the liver, so this
may also be open to individual interpretation. It is often
accompanied by respiratory symptoms.148 The Philadelphia
series is valuable in documenting 31 patients in a 6-year
period with 25 survivors. Seventy-one percent required
intubation in the first hour of life, and 40% of survivors
had chronic lung disease. More than half of the survivors
had associated anomalies, more than half had neurodevelopmental disability at 1 year of age, and three fourths had
feeding problems.
Giant omphalocele requires some imagination and creativity to treat.149 Suggestions have included painting the
sac with antiseptic,150 the use of skin flaps with grafting to
the open areas remaining,151 use of tissue expander,152 and
split-thickness skin grafting.153 We have tried on several
occasions to use Gore-Tex and then place skin flaps or grafts
over the Gore-Tex, with failure on each occasion as a result of

CHAPTER 75

sepsis and sloughing. However, it has been possible to use

flaps or grafts on the granulating surface that occurs after
removing the Gore-Tex, and the underlying pseudocapsule
seems to stabilize the abdominal wall. Using an absorbable
material covered by a VAC sponge dressing is an appealing
alternative.154,155 The use of tissue expanders to either expand
the abdominal cavity or obtain more skin in older infants is
also an option.28,156
Postoperative Care With primary closure, most patients
require assisted ventilation initially, but over a period of several
days the abdominal wall accommodates the abdominal contents. Intravenous fluids are administered at 150 mL/kg/hr or
more to maintain urine output at 1 mL/kg/hr. A central intravenous line is placed for parenteral nutrition. Short-term antibiotics are administered if primary closure has been performed,
but antibiotics are continued until completion of staged closure with a prosthesis. The wound is managed with antibiotic
ointment, and the sutures are removed at 3 weeks. We keep
a 10-French sump NG tube on low intermittent suction
until there is evidence of bowel function including stooling,
decreased distention, and reduced NG tube output. The tube
is then removed, and 12 hours later feedings are begun gradually. It is not unusual for the appearance of bowel function to
be delayed. If intestinal function has not resumed in 3 weeks,
a small bowel contrast study may be indicated.
If a prosthesis has been used, it is usually appropriate to
initiate reduction of the abdominal contents on postoperative
day 1. Reduction is accomplished simply by manual manipulation, which can then be secured in many ways: applying
long clamps suspended by umbilical tape to the overhead
warmer, applying a TIA 90 stapler, running a suture back
and forth, or using umbilical cord clamps (Fig. 75-9). A particularly ingenious method is a ringer mechanism,157 but it is
not commercially available. Simply tying the prosthetic sac
with umbilical tape leaves a larger circular defect than closing
it side to side does. One might consider adding several sutures
to each side outside the prosthesis when it is applied. These
sutures can then be tied once the prosthesis is flat to further
appose the edges.

FIGURE 75-9 Reduction of intestine contained in a preformed

Silastic silo.

CONGENITAL DEFECTS OF THE ABDOMINAL WALL

981

Despite Schusters original hypothesis, it is unlikely that the

abdominal wall grows during reduction.23 It is more likely
that the edema of the bowel wall resolves and the intestinal
contents are emptied while the abdominal wall is simply
stretched.
The several methods of measuring intra-abdominal pressure discussed earlier were originally used to monitor primary
closure and then reduction of a prosthesis. The most important lesson learned from these studies is that we had been
reducing the abdominal contents too slowly, usually every
other day. It is important to be aggressive. Most often the
prosthesis can be removed and the abdominal wall closed
in 7 days. If it is not performed by 14 days, the prosthesis
may begin to separate from the abdominal wall. Recently, Jona
suggested that active reduction may not be necessary.158 In his
small series no pressure was applied to the silo, and the
abdominal contents spontaneous reduced by day 8.
If skin flaps have been used, the definitive repair can be
done at any time, depending on the patients general condition. If the intestine begins to grow into a large skin sac instead
of stretching the abdominal cavity, an abdominal binder can
be used to direct forces inward. When it is time to bring
the fascia together, the skin can be dissected off the liver
with little trauma other than some mild bleeding that will
stop with pressure. If the edges of the muscular abdominal
wall cannot be brought together, a prosthetic mesh patch
can be used as long as it can be entirely covered by healthy
skin. If a part of it must be left exposed, it is unlikely to be
incorporated and will become infected or extruded. If one
can predict that skin flap closure will not be possible initially,
it is probably best to avoid dissecting flaps so that that tissue
will be healthier when it is time for the final repair.
If a postoperative hernia occurs, it can usually be repaired at
1 year of age without the use of a prosthetic patch. In occasional
patients the hernia resolves spontaneously.
Ectopia Cordis Thoracis and Cephalic Fold Defect
Operative repair of ectopia cordis thoracis and cephalic fold
defects is especially challenging. It is difficult to replace the
heart in the thoracic cavity without kinking the great vessels
or the pulmonary veins. Coverage with soft tissue to gain time
can result in cardiac tamponade, so wide intrathoracic dissection around the heart and great vessels has been advocated to
increase the space within the chest. The fascia of the abdominal wall is closed once cardiorespiratory stability has been
achieved. In ectopia cordis thoracis, it is also important to provide a rigid covering for the heart. This has been performed
with rib struts between the sternal halves and with prosthetic
material. Reported repairs of these difficult clinical problems
emphasize creative use of available tissue and individual
anatomic features.159162 In the cephalic fold defect (pentalogy of Cantrell), the sternal cleft and pericardial defect need
no special treatment. We have used immediate skin closure
of the omphalocele and allowed the patient to grow and have
the intracardiac defect treated later. A Gore-Tex patch can
then be used to close the central tendon of the diaphragm
with no tension so that the heart can extend somewhat into
the abdomen. With growth the patch becomes taut and
elevates the heart slowly into the chest, which can grow to
receive it. A valuable recent review of the literature has been
published.163

982

PART VII

ABDOMEN

Caudal Fold Defect

This complex problem is more completely dealt with in
Chapter 120. Initial management might consist of closure of
the omphalocele and creation of a colostomy. Preservation
of intestinal length including salvage of any colon that might
be attached to the bladder plate before creating a colostomy
is especially important. Lund and Woo have reviewed this
topic well.164,165
Gastroschisis
Initial Care Two features of gastroschisis make initial care
somewhat different from that of omphalocele. The patient is
frequently premature, and closer attention must be paid to
heat preservation, respiratory support, and the large surface
area of exposed intestine. The latter is also responsible for
increased fluid needs and heat loss. Perhaps the best way to
control this problem is to place most of the infant immediately
in a plastic drawstring bowel bag to control evaporative heat
and fluid loss.166 Because in most cases the intestine will be
perfectly normal immediately after delivery whether the delivery is vaginal or cesarean, the faster the bowel can be reduced,
the more likely primary closure can be achieved and the less
bowel wall edema and fibrinous coating will accumulate.
Previously we chose to deal with this issue by operating
immediately after delivery167 because our obstetricians preferred cesarean delivery for all children with abdominal wall
defects. As more obstetricians become convinced by the literature that the route of delivery does not affect outcome in
gastroschisis, fewer cesarean sections are scheduled. A review
of our more recent data indicates that rapid transfer from the
delivery room to the operating room (<1 hour) will also lead
to a greater likelihood of primary closure.167a When the delivery room and the pediatric operating room are in the same
hospital, this is much easier to arrange.
Operative Technique Time is taken to wipe the vernix caseosa carefully from most of the baby to facilitate many of
the subsequent maneuvers. If there is no immediate respiratory distress, an intravenous line is started, monitoring
equipment is applied, and the patient is intubated. It is helpful
to evacuate meconium from the rectum by anal dilatation. It
has generally been assumed that patients with gastroschisis
will have a volume deficit because of the large surface area
of the mass of eviscerated intestine. Yet a recent Canadian
study suggests that limiting preoperative fluid resuscitation
will improve outcomes.168 While the anesthesiologist is
involved with these activities, the surgeon holds the umbilical
cord up and, loop by loop, reduces the intestine. In most
cases, by the time that the patient is ready for skin preparation,
the bowel has been totally reduced. Other operative maneuvers and postoperative care are similar to that described for
omphalocele. A satisfactory cosmetic closure can be obtained
for gastroschisis by leaving the entire umbilicus intact during
the initial stages of the procedure (Fig. 75-10). The vessels and
urachus can be divided at the level of the peritoneum after
trimming the cord. A subcuticular skin closure is then
performed with the umbilicus being the left edge of the
midportion of the incision, or often the entire incision,
because the defect can often be quite small with gastroschisis
or umbilical cord hernia.

FIGURE 75-10 Gastroschisis with cosmetic closure obtained by preserving

the umbilicus.

In several centers the spring-loaded preformed Silastic

pouch (Bentec bag) is frequently placed at the bedside on arrival of the patient.169,170 Reduction can then be performed
also at the bedside, and the patient is taken to the operating
room only for final closure. Kidd and colleagues have found
that with this technique, they perform fewer primary closures
(34% vs. 84%) and have a somewhat longer length of stay
(38 days vs. 30 days), but the mortality (3% excluding those
with lethal associated anomalies) and time until full feeding
are no different.171 They did, however, report fewer ventilator days, infections, strictures, reoperations, and episodes
of necrotizing enterocolitis. Bianchi and colleagues have also
shown that waiting several hours to reduce the intestinal mass
may not be as deleterious as has been supposed.172,173 In a
2008 multicenter prospective trial comparing bedside placement of the silo to primary closure, there was a trend to fewer
days on the ventilator for the bedside silo group, but no
difference in days on TPN, length of stay, incidence of sepsis,
or incidence of necrotizing enterocolitis.174 A study from
Stanford showed good results with sutureless closure, which
is performed either initially with reduction of the bowel or secondarily after reduction of the Bentec bag.175 The umbilical
cord is laid over the small residual defect and held in place
with an adhesive, microporous dressing.
Management of complicated gastroschisis must be individualized.128 Often an atresia can simply be placed in the abdominal cavity and repaired 6 weeks after the abdomen has
been closed with native tissue, whether skin or fascia. A stoma
can also be brought out through normal abdominal wall lateral
to a prosthesis. This usually requires sewing on Silastic sheets
because the spring-loaded ring of the Bentec bag will interfere
with stoma placement. The most shrunken nubbins of bowel
can be surprisingly useful 2 to 3 months after residence in the
abdominal cavity.176 If they cannot be exteriorized, perforations and other complex wounds can be treated with a VAC
sponge dressing.177
Umbilical Cord Hernia
This defect is easily reduced by holding the sac upward
and gently massaging the bowel into the peritoneal cavity.
The fascia can always be closed primarily, and a cosmetic
umbilicoplasty is nearly always feasible.

CHAPTER 75

Complications
------------------------------------------------------------------------------------------------------------------------------------------------

Complications may be related to prematurity (in gastroschisis),

associated anomalies (in omphalocele), gastrointestinal tract
anomalies (in gastroschisis), and a closure that is too tight.
Mention has already been made of the increased fluid needs
and delayed recovery of bowel function. It is important to
be prepared to treat the problems of prematurity including
heat loss, respiratory failure, hyperbilirubinemia, hypoglycemia
and hyperglycemia, and hypocalcemia. In managing respiratory
distress it is important to obtain blood for capillary blood gas
analysis from the upper extremities because the lower extremities are likely to be edematous and congested. Patients with
gastroschisis and ruptured omphalocele are frequently hypovolemic. Philippart and colleagues studied fluid resuscitation in
patients with both gastroschisis and omphalocele by using
muscle pH as an indication of adequate perfusion and resuscitation.178 They found that all infants needed at least 25% of
estimated blood volume during surgery (17 to 80 mL/kg in
45 to 120 minutes) and required 82 to 312 mL/kg in the first
24 hours of life. Urine output is a good monitor of volume in
neonates. Treatment of most of the gastrointestinal tract anomalies can be delayed. In omphalocele, the bowel may be normal
enough to sustain an anastomosis, but edema accumulates in all
tissues and the liver expands with the length of the operation.
A closure that is too tight can lead to ventilatory compromise, decreased venous return and low cardiac output, and
oliguria. The remedy is to return the patient to the operating
room to remove the fascial sutures and perform skin closure
only. One may need to add a prosthesis. If a prosthesis has
been used initially, it may be possible to open it at the bedside
and reclose it more loosely. In omphalocele, postoperative
metabolic acidosis can develop as a result of kinking of the hepatic veins from reduction of the liver. In this case, removal
and refashioning of the prosthesis may be necessary.
All these patients tend to have a slow onset of bowel function,
no matter how quickly the defect is reduced or how normal the
bowel appears. Bowel function seems to return faster in patients
with omphalocele than in those with gastroschisis.179

Outcome
------------------------------------------------------------------------------------------------------------------------------------------------

SHORT TERM
The survival rate for gastroschisis is 90% in most series.121,180
In a registry study in Texas, the survival rate of infants with
gastroschisis was 93% in 1995-1997.181 In a study from
Manchester, United Kingdom, a 94% gastroschisis survival
rate was obtained.182 Of the seven patients who died, five died
of overwhelming sepsis. Primary fascial closure was achieved
in 80%. The median time to feeding was 30 days (range, 5 to
60 days), and the median length of stay was 42 days (range, 11
to 183 days). Those who required a silo or had associated intestinal atresia (8 of 91 patients) required more time until
feeding and had a greater length of stay, but no increased mortality. In a 2010 report based on 2490 patients in the Pediatric
Health Information System (PHIS) database created by the
Child Health Corporation of America (Kansas City, Mo.) the
overall survival was 96.4%. Associated conditions included
cardiovascular 15%, intestinal resection 12.5%, intestinal
atresia 11%, ostomy formation 8.3%, and pulmonary 5%.

CONGENITAL DEFECTS OF THE ABDOMINAL WALL

983

Survival rates for omphalocele range from 70% to 95%,

with most of the mortality being related to the associated
cardiac and chromosomal anomalies.180 In a report from
Los Angeles, there was no difference in mortality from omphalocele for birth weight, size of the defect, or type of initial closure.183 They also reported no significant change in mortality
from 1960-1970 (23%) to 1970-1980, when it was 19%.
Mortality was mainly related to associated anomalies. For
their patients with gastroschisis, survival rates did increase
in the more recent decade to 91%, and mortality was attributed to prematurity, bowel complications, and Candida sepsis
associated with total parenteral nutrition.
Few patients survive any form of ectopia cordis,184,185
but Groner does report one normally active 12-year-old
who wears a plastic shield to cover the as yet unreconstructed
bony defect.186 OGorman and colleagues reported seven
patients with pentalogy of Cantrell, of whom four survived
and three were free of a ventilator.187 Nearly all patients with
caudal fold defects survive, although bowel and urinary tract
function vary.165

LONG TERM
In a report from The Netherlands in 2009 on 111 patients
with omphalocele treated between 1971 and 2004, 20% of
the patients died, almost all related to associated congenital
anomalies.188 Readmission was required at some time in
70%. The most frequent later operations were inguinal hernia,
tonsillectomy, adenoidectomy, myringotomy tubes, fundoplication, bowel obstruction, malrotation, orchidopexy, and cosmetic revision of abdominal scars. Thirty percent of patients
were still taking medication of some sort. Only 3 patients ever
had the feeling that omphalocele interfered with their choices
of activities or professions, and 10 reported that omphalocele
did affect some social relationships (teasing).
Davies and Stringer interviewed 25 of 35 patients who
underwent surgery for gastroschisis between 1972 and
1984 and survived longer than 1 year.44 Their median age
was 16 years. Ninety-six percent were in good health and
experienced normal growth, and 35% required further surgery related to gastroschisis, two for small bowel obstruction
and three for scar revision. Fifty-seven percent reported
that absence of an umbilicus caused them some distress
during childhood. In 25 school-aged children with gastroschisis reported from Oregon, 7 were held back a grade or
enrolled in special classes, but all participated in normal
physical activities.180 Eighty-four percent of these patients
reported normal bowel movements. Those with abdominal
complaints were usually evaluated as nonspecific or functional. Of 22 patients who required bowel resection at the
initial operation, 10 had bowel complications, whereas only
2 of the 68 without a bowel resection had such complications.
Ten percent of all patients underwent further surgery for
abdominal wall hernia, scar revision, or undescended testis.
In 2009 the Canadian Paediatric Surgical Network reported
a contemporary survival of 95%.109
In a combined series of omphalocele and gastroschisis
reported from Oklahoma City, 94 patients had an 88% survival rate.189 There was long-term follow-up in 61 patients
for a mean of 14.2 years. Nineteen needed 31 reoperations,
mainly for abdominal wall hernia and intestinal atresia. Eighty
percent described their quality of life as good, but 40% were

984

PART VII

ABDOMEN

concerned about their height and felt inadequate in sports and

social activities. Many also expressed concern about the
absence of an umbilicus. Another combined series with a
mean age at follow-up of 8.8 years was reported by Lindham
from Sweden.190 He noted several episodes of bowel obstruction in the first year of life, recurrent abdominal pain without
a specific abnormality, and some concern in girls about the
scar and absence of an umbilicus. Growth and development,
however, were normal.
In a study from the United Kingdom, patients with gastroschisis averaged the 32nd percentile for weight at 5 years of
age and the 52nd percentile after that.191 With complicated
gastroschisis (such as gastroschisis associated with intestinal
atresia), they reached only the 25th percentile. The group
from Stanford192 found that most survivors of omphalocele
and gastroschisis had poor weight gain. None had gastrointestinal or metabolic problems at 3 years of age on the basis of
imaging studies, fecal fat excretion, and serum chemistry.
One third had IQ lower than 90, and this was related to the
length of hospitalization and nongastrointestinal anomalies.
Koivusalo and colleagues from Finland sent detailed questionnaires to 75 patients older than 17 years with congenital
abdominal wall defects (16 with omphalocele, 11 with gastroschisis) and received a response from 76%.193 The only illness
found more frequently than in the general population was
rheumatoid arthritis in 7%. Thirty-seven percent reported
some morbidity related to the scar and absence of the umbilicus, 51% had functional gastrointestinal disorders, and 12%
had low self-esteem. Still, 88% reported that they were in good
health, and their quality of life and educational levels were no
different from that of the general population.
Adverse late cardiorespiratory and pulmonary effects
are seldom found in patients with either omphalocele or

gastroschisis.179 Many children do express concern later in

life about the absence of an umbilicus. Although both the
short-term and long-term outlook for patients with ectopia
cordis and both cephalic fold and caudal fold omphaloceles
is guarded, patients with lateral fold omphalocele, umbilical
cord hernia, and gastroschisis have an excellent survival rate
and long-term prognosis. Most problems are related to
associated conditions, not to the abdominal wall defect or
its repair.
The complete reference list is available online at www.
expertconsult.com.
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