j o u r n a l o f s u r g i c a l r e s e a r c h 1 9 8 ( 2 0 1 5 ) 3 8 8 e3 9 2

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Association for Academic Surgery

Giant omphaloceles: surgical management and

perinatal outcomes

Adesola C. Akinkuotu, MD,a Fariha Sheikh, MD,a

Oluyinka O. Olutoye, MD, PhD,a,b,c Timothy C. Lee, MD,a,b
Cariciolo J. Fernandes, MD,a,c Stephen E. Welty, MD,a,c
Nancy A. Ayres, MD,a,c and Darrell L. Cass, MDa,b,c,*
a
Texas Children’s Fetal Center, Texas Children’s Hospital, Houston, Texas
b
Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas
c
Department of Pediatrics, Baylor College of Medicine, Houston, Texas

article info abstract

Article history: Background: The purpose of this study was to describe the current management and out-
Received 3 January 2015 comes of infants with omphalocele.
Received in revised form Methods: The medical records of all patients treated for omphalocele at a large children’s
9 March 2015 hospital from January, 2003eFebruary, 2014 were reviewed. Patients were classified as
Accepted 19 March 2015 having an isolated omphalocele or omphalocele with minor or major associated anomalies.
Available online 25 March 2015 Prenatal data collected included fetal magnetic resonance imaging-based observed-to-
expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in
Keywords: the omphalocele sac.
Prenatal diagnosis Results: Of 95 patients, 59 presented prenatally and had comprehensive fetal center eva-
Surgical outcomes luation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated
Giant omphalocele anomalies. No live-born baby with an isolated defect (n ¼ 19) died, whereas mortality was
41% and 17% for those with major and minor anomalies, respectively (P ¼ 0.006). Infants
with major anomalies had significantly longer median length of intubation (36 versus
0 versus 0 d; P ¼ 0.04) and hospital stay (157 versus 28.5 versus 18 d; P < 0.001) compared with
those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed
surgically by delayed closure with a median age at repair of 10 mo (range, 3.4e23.6 mo). Six-
month survival was 80%. None of the delayed repair patients required a later operative
revision, whereas 2 of 5 with early repair did.
Conclusions: The presence of associated anomalies is the strongest predictor of morbidity
and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed
closure is associated with good outcomes, but larger, prospective studies comparing
delayed to early closure are needed to determine the optimal timing of repair.
ª 2015 Elsevier Inc. All rights reserved.

* Corresponding author. Division of Pediatric Surgery, Department and Texas Children’s Fetal Center, Texas Children’s Hospital, 6701
Fannin St., Suite 1210, Houston, TX 77030. Tel.: þ1 832 822 3135; fax: þ832-825-3141.
E-mail address: dcass@bcm.edu (D.L. Cass).
0022-4804/$ e see front matter ª 2015 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.jss.2015.03.060
 j o u r n a l o f s u r g i c a l r e s e a r c h 1 9 8 ( 2 0 1 5 ) 3 8 8 e3 9 2
1. Introduction
Omphalocele is one of the most common congenital abdom-
inal wall defects, occurring in approximately 1 in 4000e7000
live births [1]. It is a midline abdominal defect in which
herniated abdominal contents are contained by a sac that is
in continuity with the umbilical cord. This defect is often
associated with other anomalies and/or chromosomal ab-
normalities that negatively impact outcomes [2]. Routine
prenatal screening has enabled early detection of an ompha-
locele and associated anomalies, and there is a high incidence
of elective termination of pregnancy or intrauterine fetal
demise [2,3]. Omphaloceles may be stratified into three groups
as follows: small, giant, or ruptured [4,5]. A giant omphalocele
(GO) is often described as those with an abdominal wall defect
size >5 cm or with more than 50 or 75% of the liver within
the sac [4,6].
The goal in the management of GO is reduction of
abdominal contents and closure of the abdominal wall defect
when the patient has been stabilized medically with good
supportive care. Patients with an omphalocele have a small
abdominal cavity, and trunk and early closure of the abdom-
inal wall may lead to a sudden increase in intra-abdominal
pressure and respiratory insufficiency due to diminished
lung excursion. This is of particular concern in patients with
GO, as these patients also tend to have pulmonary hypoplasia
that may cause respiratory insufficiency [5]. The timing of
operative repair for infants with GO remains controversial,
and there is limited published evidence to drive decision-
making for the pediatric surgeon. Delayed closure tech-
niques have been developed to mitigate the complications of
early surgical repair. One such approach is with the Gross
technique, which involves the development of skin flaps and
closure of skin over the sac without opening the sac [7].
Another technique is the gradual reduction of abdominal
contents using a prosthetic silo [8]. Finally, the use of topical
agents including sulfadiazine cream and povidoneeiodine
solution [9,10] are described as the so called “paint and wait”
approach [11].
Given the variations in surgical practice and management
of these patients, we sought to describe the current treatment
strategies and associated outcomes on infants with GO
managed at our institution.
2. Methods
The study was approved by the Institutional Review Board at
the Baylor College of Medicine, Houston, TX and is designed as
a retrospective cohort study.
2.1. Patient population
Patients diagnosed with omphalocele and managed at our
tertiary pediatric care center between January 2003 and
February 2014 had their medical records reviewed. GO was
defined as a defect with >50% of the liver in the omphalocele
sac. Patients were classified into three groups as follows: (a)
isolated omphalocele; (b) omphalocele with minor, or (c)
389
omphalocele with major associated anomalies. Major
anomalies were defined as cardiac, genitourinary, or ano-
rectal defects requiring immediate medical or surgical
management, Bochdalek-type congenital diaphragmatic
hernia, alveolar-capillary dysplasia, or chromosomal aneu-
ploidy or duplication. All other anomalies were classified as
minor.
2.2. Prenatal data
For patients who were diagnosed in utero, we collected gesta-
tional age at diagnosis and fetal magnetic resonance imaging-
derived observed-to-expected total fetal lung volumes. Fetal
lung volumes were obtained by dividing the total measured
fetal lung volume by the fetal lung volume expected for
gestational age, a validated technique described previously
[12,13].
2.3. Postnatal outcomes
Outcomes of interest included postnatal death, hospital
length of stay, days on mechanical ventilation, and use of
supplemental oxygen at 30 d of life.
2.4. Surgical technique
Early closure was performed using the Gross technique with
mobilization of skin flaps and suture repair with or without a
skin or fascia patch. In patients who underwent delayed
closure, the omphalocele was managed with daily dressing
changes with topical 1% silver sulfadiazine cream, followed
by primary fascial repair once there was complete epithelial-
ization and the omphalocele was reducible (at a later
admission).
2.5. Statistical analysis
Statistical analysis was performed using Student t-test and
ManneWhitney U tests for parametric and nonparametric
continuous variables, respectively. Chi-square analysis was
used for categorical variables. A P value of <0.05 was consid-
ered statistically significant. Statistical analysis was per-
formed using the IBM SPSS statistical software version 22 (IBM
Corporation, Armonk, NY).
3. Results
3.1. Patients
There were a total of 95 patients diagnosed with omphaloceles
during the study period; 59 patients (62.1%) were diagnosed
prenatally and received comprehensive fetal center evalua-
tion. The remaining 36 patients were diagnosed postnatally.
Thirteen prenatally diagnosed omphaloceles were excluded
from further analysis because of termination of pregnancy
(n ¼ 3), in utero fetal demise (n ¼ 7) or delivery at an outside
facility with immediate neonatal demise (n ¼ 3). Thus, a total
of 82 patients were included in our study.
390 j o u r n a l o f s u r g i c a l r e s e a r c h 1 9 8 ( 2 0 1 5 ) 3 8 8 e3 9 2

Table 1 e Outcomes of fetuses and neonates with omphalocele.

Outcomes Isolated Minor Major P value

Total patient number 23 41 31 N/A

Live born 19 39 27 N/A
GO, n (%) 11 (52) 25 (61) 23 (74) N/A
Postnatal mortality, n (%) 0 (0) 7 (17) 11 (41) 0.006
Duration of mechanical ventilation, d median (IQR) 0 (0e2) 0 (0e5) 36 (22e89) 0.04
Hospital LOS, d median (IQR) 18 (9.5e38.5) 28.5 (13e32.5) 157 (48.5e185) <0.001
O2 at 30 DOL, n (%) 2/16 (12.5) 9/33 (27.3) 16/18 (88.9) <0.001

DOL ¼ days of life; IQR ¼ interquartile range; LOS ¼ length of stay; N/A ¼ not applicable; O2 ¼ oxygen.

3.2. Outcomes of all omphalocele patients
Of the 82 live-born infants at our institution, 40 (49%) had GO.
There were 21 infants (26%) with chromosomal anomalies, 25
(30%) had major associated anomalies and 19 patients (23%)
had an isolated defect. Overall, 18 infants (22%) died in the
postnatal period. Table 1 details the outcomes of live-born
omphalocele patients. There was a significant difference in
mortality rate among patients with an isolated defect (0%),
associated minor anomalies (17 %), and associated major
anomalies (41%; P ¼ 0.006). Infants who had omphaloceles
with associated major anomalies had significantly greater
pulmonary morbidity as evidenced by longer duration of me-
chanical ventilation and higher need for supplemental oxygen
at 30 d of life (Table 1). Patients with associated major anom-
alies also had a significantly longer hospital stay compared
with those with minor or no associated anomalies (Table 1).
3.3. Outcomes and characteristics of GO patients
Of the 40 patients with GO, 27 (66%) were diagnosed prena-
tally. The mean and median observed-to-expected total fetal
lung volumes were 45.2  14.7% and 48.4% (interquartile
range, 34.3%e53%), respectively. The majority of GO patients
(n ¼ 35) were managed by delayed surgical closure with topical
povidoneeiodine followed by abdominal wall closure at a
median age of 10 mo (range, 5.3e23.6 mo). The median length
of postoperative hospital stay in patients undergoing delayed
elective closure was 4 d. Table 2 summarizes the character-
istics and outcomes of GO patients managed by both strate-
gies. Two patients who were managed with delayed closure
developed complications during the “paint and wait” period
including necrotizing enterocolitis and spontaneous fistuli-
zation of bowel into the omphalocele sac. No patients who
underwent delayed surgical closure required an operative
revision of their abdominal wall, whereas 2 of the 5 patients
(40%) (P ¼ 0.028) who underwent early closure did. The oper-
ative revisions were a primary repair of a large ventral hernia
and a repair of a large omphalocele-like defect in a patient
who had undergone previous staged closure.
4. Discussion
In this study, we report the outcomes of infants diagnosed
with omphaloceles at our institution. We evaluated the cur-
rent management strategy of patients with GO and their
associated outcomes. In our cohort, we found a prenatal
mortality of 13.7%, which was significantly lower than previ-
ous studies that reported rates of elective terminations and
fetal demise ranging from 35%e77% [1,4]. This lower rate of
prenatal mortality is likely due to the availability of compre-
hensive prenatal care at our institution, and the inherent se-
lection bias in those families inclined toward termination of
pregnancy are less likely to seek consultation at a compre-
hensive fetal center. Conversely, patients contemplating
termination may be willing to continue the pregnancy after a
comprehensive evaluation and multidisciplinary counseling.
The postnatal survival rate of 78% in this cohort was similar to
previous studies reporting postnatal survival between 70 and
80% [9,14].
The majority of patients with omphaloceles in this study
(77% of live-born infants) had associated anomalies. We found
that the presence of associated anomalies, especially major

Table 2 e Characteristics and outcomes of patients with GO managed with early versus delayed closure.
Characteristics and outcomes Early repair (n ¼ 5) Delayed repair (n ¼ 35) P value

GA at birth (wk), mean  SD 37.9  2.2 36.4  3.1 0.306

Birth weight, g mean  SD 2693  540 2695  836 0.864
Fetal center evaluation, n (%) 1 (5) 25 (71.4) 0.043
Chromosomal anomalies, n (%) 0 (0) 4 (11.4) 1.00
Minor anomalies, n (%) 3 (60) 14 (40) 0.634
Major anomalies, n (%) 2 (40) 18 (51.4) 1.00
Length of intubation, days median (IQR) 12 (6e27) 0 (0e3) 0.932
Length of stay, d median (IQR) 73 (50e143) 33 (21e103) 0.857
Oxygen at 30 DOL, n (%) 2/5 (40) 16/29 (55.2) 1.00
Mortality at 6 mo, n (%) 0/5 (0) 8/34 (23.5) 0.563

DOL ¼ days of life; GA ¼ gestational age; IQR ¼ interquartile range; SD ¼ standard deviation.
 j o u r n a l o f s u r g i c a l r e s e a r c h 1 9 8 ( 2 0 1 5 ) 3 8 8 e3 9 2
anomalies, was associated with increased mortality, longer
durations of mechanical ventilation, and hospital length of
stay. Although a similar study by Heider et al. [15] found that
patients with major associated anomalies had significantly
higher mortality than those with no associated major mor-
tality, they did not find any differences in hospital length of
stay and days of mechanical ventilation. It is likely to have
differences in hospital length of stay and days of mechanical
ventilation because in the present study patients were further
divided into those without any associated anomalies and
those with minor anomalies. In another large series of pa-
tients with prenatally diagnosed omphalocele, mortality was
increased in patients with associated anomalies, and the
absence of associated anomalies was found to be a good
prognostic indictor of neonatal survival [3]. In a recent study
by Calvert et al. [2], in which the outcomes of prenatally
diagnosed omphalocele were evaluated, the authors found
that those with isolated omphalocele had a median length of
hospital stay of 20 d, which was slightly less than the median
length of stay of patients with isolated omphalocele in our
series. The findings of this study therefore support current
studies in the literature, which highlight the importance of
associated anomalies in the outcomes of patients with
omphaloceles.
More importantly, the findings of this study add to the cur-
rent body of literature pertaining to the management of GO in a
large, single-center series. The management of GO presents a
challenge to the pediatric surgeon, given the large abdominal
wall defect and concerns for associated respiratory dysfunction
related to a slender chest wall, pulmonary hypoplasia, and
possible ventilator-associated lung injury. It has been reported
that patients with GO are less likely to undergo management
with early closure compared with those with small omphalo-
celes [16]. Although associated anomalies are less common in
GO than in those with small-to-moderate omphaloceles [14],
the course is complicated in patients with anomalies because of
the intersection between optimal management of the ompha-
locele and the other anomalies. Because the impact of ompha-
locele size on the clinical course remains unclear, the timing of
GO closure varies with some practitioners advocating for early
closure, whereas others for delayed closure. In this study, we
show that delayed closure of GO does not lead to an increase in
morbidity or mortality. At our institution, the “paint and wait”
strategy of delayed closure is the preferred method of man-
agement for patients with GO. However, the final decision
regarding early compared with delayed closure is left to the
surgeon’s clinical judgment. As expected, we found that the
majority of patients (87.5%) were managed with delayed
closure. Interestingly, five patients were managed with early
closure. Further review of these five patients revealed that most
had been managed with early closure of the skin flaps based on
surgeon preference, whereas two patients were managed with
early closure due to concomitant repair of a cloacal exstrophy
and the presence of a congenital heart defect necessitating open
heart surgery in the immediate perinatal period. Previous
studies comparing delayed with early closure for GO have found
that infants managed with staged closure required longer
duration of ventilation and hospital stay than those who un-
derwent early closure [6]. Although similar studies looking at
staged repair of GO have demonstrated prolonged
391
mechanical ventilation and hospital stays [10,15,17,18], we
found that patients who were managed with delayed closure
had shorter median length of mechanical ventilation and
hospital stay, despite a higher percentage of major associ-
ated anomalies seen in this group. Interestingly, all patients
managed with early closure compared with 77% of those
managed with delayed closure were alive at 6 mo, a differ-
ence that did not reach statistical significance. The mortality
in the delayed group in our series is likely due to complica-
tions from associated anomalies rather than specific com-
plications related to an unrepaired GO.
In assessing the outcomes of patients as related to the timing
of surgery, it is important to note the definition of delayed
closure in the literature varies largely such that time to opera-
tion ranges from hours to days. In a study by Na et al. [19], early
repair was defined as repair immediately after birth, whereas
late repair was defined as repair occurring 3 h after birth. In
our cohort, patients who underwent delayed closure had their
definitive operation at a median age of 10 mo of life, which is
significantly longer than those established by others. One of the
major advantages of a delay in operative repair is that it permits
time for thoracic and abdominal growth, which may limit res-
piratory complications associated with the abdominal wall
reconstruction and enable a more successful repair. In our se-
ries, those with GO that had delayed closure had smooth
postoperative recoveries, as indicated by a 4-d median length of
stay. Furthermore, the delayed closure likely allowed time for
an increase in abdominal domain, allowing for decreased ten-
sion at the closure and less risk of recurrent hernias. In support
of the issue of recurrent hernias, in a recent single-institution
cohort study, 2 of 20 (10%) GO patients managed with delayed
closure had recurrent ventral hernia. In the present study, none
of those who underwent delayed closure had operative revision,
whereas 2 of 5 who had early closure developed recurrent
ventral hernias. Furthermore, delayed abdominal wall closure
does not preclude the neonate from being discharged home if
otherwise stable. The early discharge therefore provides
improved family-centered care in which the infant can be
united in their home environment at an early age.
The major limitations of our study are related to its retro-
spective nature and the lack of agreement on the definition of
GO. For the purposes of this study, we defined GO as >50% of
the liver in the omphalocele sac. The definition of GO varies
with some defining it as a defect of >5 cm, whereas others
define it as a defect of >6 cm. However, most resources define it
by the presence of liver within the sac [5,17]. This limitation
points to the need for standardization with respect to defects
that are considered small, moderate, or giant so that in-
terventions studied in this patient population would be per-
formed in as uniform a population as possible. Finally, there is
potential bias in our cohort given that most patients were
managed with delayed closure. This highlights the preference
toward delayed closure in patients with GO at our institution, a
preference shared by other institutions [6,16]. The manage-
ment of these complicated patients based on institutional or
surgeon preference, however, poses a challenge as it prevents
proper evaluation of the various techniques used in omphalo-
cele closure. To truly demonstrate potential benefits of one
management strategy over another, larger prospective studies
are warranted.
392 j o u r n a l o f s u r g i c a l r e s e a r c h 1 9 8 ( 2 0 1 5 ) 3 8 8 e3 9 2
5. Conclusions
In conclusion, our study confirmed that outcomes of ompha-
loceles are associated with the presence of concomitant
anomalies. Specifically, the presence of major anomalies
including cardiac anomalies requiring surgery in the neonatal
period, congenital diaphragmatic hernia, and chromosomal
anomalies was associated with significantly increased mortality
and pulmonary morbidity. We also described the successful
management of GO with delayed closure without an increase in
adverse outcomes. Management of GO with topical agents such
as povidoneeiodine has the potential benefit of both reducing
pulmonary embarrassment and allowing patients with GO to be
discharged from the hospital under the care of well-counseled
parents or guardians with plans for definitive closure at a
later time. Randomized clinical trials comparing early versus
delayed closure are needed to delineate the more effective
method of managing these complicated patients.
Acknowledgment
Authors’ contributions: A.C.A. and D.L.C. conceptualized and
designed the study and drafted the initial article. A.C.A.,
D.L.C., F.S., O.O.O., T.C.L., C.J.F., S.E.W., and N.A.A. reviewed
and revised the article and approved the final article as sub-
mitted.
Disclosure
The authors report no proprietary or commercial interest in
any product mentioned or concept discussed in this article.
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