Seminars in Pediatric Surgery 28 (2019) 89–94

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Seminars in Pediatric Surgery

journal homepage: www.elsevier.com/locate/sempedsurg

Immediate versus staged repair of omphaloceles

Erik D. Skarsgard
British Columbia Children’s Hospital, Department of Surgery, University of British Columbia, K0-110 ACB 4480 Oak Street, V6H 3V4 Vancouver,
British Columbia, Canada

ar t ic l e in f o abst r ac t
Keywords: Omphalocele is an abdominal wall defect involving the umbilical ring which results in visceral herniation
Omphalocele
of small and large intestine, liver, spleen and sometimes gonads. The covering of the herniated viscera by
Abdominal wall defect
a fused membrane consisting of peritoneum, Wharton’s jelly and amnion projects viscera from mechani-
Primary closure
Staged closure cal injury and exposure to chemical irritants in amniotic fluid.
Abdominal wall reconstruction Omphalocele is usually diagnosed before birth, is variable in size, and is frequently associated with
chromosomal and somatic anomalies, syndromes, and variable degrees of pulmonary hypoplasia which
can be lethal.
In this article we examine surgical closure options for omphaloceles ranging from early primary fas-
cial repair for small omphaloceles to a staged repair, often facilitated by an amnion preserving silo, which
may be necessary for giant omphaloceles that cannot be closed primarily. We also review some of the ad-
juncts to abdominal wall reconstruction including tissue expansion and mesh. Conservative management
(paint and wait) of giant omphaloceles is described elsewhere.
© 2019 Elsevier Inc. All rights reserved.

Omphalocele Characterization of risk associated with a

Gastroschisis and omphalocele (also referred to as exomphalos)
account for the vast majority of congenital abdominal wall defects.
In contrast to the paraumbilical nature of gastroschisis, omphalo-
cele is a true umbilical ring defect, characterized by herniation of
abdominal viscera including small and large intestine, liver, spleen,
and sometimes the gonads. Another distinguishing attribute of om-
phalocele is coverage of herniated viscera by a multi-layered mem-
brane, which includes peritoneum, Wharton’s jelly and amnion.
This membrane protects the viscera from exposure to amniotic
fluid as well as extra-abdominal mechanical irritation. The accu-
racy of contemporary prenatal diagnosis means that the majority
of cases of omphalocele are detected before birth, which allows for
planning of perinatal care, including surgical management of the
abdominal wall defect. Optimal decision making in management
of the defect frequently requires multi-disciplinary consideration
of a number of factors including defect size, presence of herniated
liver, presence and prognosis of associated somatic and syn-
dromic malformations, and the severity of associated pulmonary
hypoplasia.
E-mail address: eskarsgard@cw.bc.ca
diagnosis of omphalocele
In an era of contemporary prenatal diagnosis and multidisci-
plinary prenatal counselling, the pediatric surgeon plays a criti-
cal role in describing the options for defect closure after birth.
These discussions will usually be informed by an assessment of
risk factors known to have influence on outcome. These factors in-
clude whether the omphalocele is isolated or associated with other
prognosis-altering malformations, the size of the abdominal defect
and proportion of herniated liver, and the likelihood of associated
severe pulmonary hypoplasia. However, it is important to have in-
sight into the risk profile of an individual fetus and be aware that
omphalocele is a classic exemplar of the concept of hidden mortal-
ity associated with prenatal diagnosis. 1 Hence, the frequency and
lethality of associated anomalies in omphalocele may be quite dif-
ferent between a cohort detected by routine prenatal screening
versus a liveborn cohort. As an example, a recent study from a
national screening program in the Netherlands identified an 83%
rate of multiple associated anomalies, and of these, 57% had an
abnormal karyotype, most commonly trisomy 18.2 From the origi-
nal cohort of 141 prenatally detected fetuses, 61% underwent ter-
mination of pregnancy, and another 18% underwent spontaneous
miscarriage, leaving only 24 (17%) which were liveborn. Of these
24 liveborn infants, 12 (50%) had an isolated omphalocele. If one
analyzes a much larger liveborn cohort, one gets a better sense

https://doi.org/10.1053/j.sempedsurg.2019.04.010
1055-8586/© 2019 Elsevier Inc. All rights reserved.
90 E.D. Skarsgard / Seminars in Pediatric Surgery 28 (2019) 89–94

of the risk profile that a pediatric surgeon should consider when

counselling beyond the second trimester. A pooled analysis of 2300
liveborn omphalocele cases from 12 state birth defect state reg-
istries identified an isolated omphalocele rate of 22%. 3 Thirty-two%
had congenital heart disease, but of these, 75% were either atrial
or ventricular septal defects. Twenty-two% had other non-cardiac,
non-lethal structural birth defects of the genitourinary, digestive,
craniofacial and musculoskeletal defects. Only 17% had chromoso-
mal abnormalities, of which the most common was trisomy 18.
The other important risk factor to be considered in selecting
treatment is size of the omphalocele. Although the designation gi-
ant omphalocele (GO) is widely quoted in the literature, no con-
sensus definition exists. A commonly used definition is an abdom-
inal wall defect ≥5 cm in diameter containing liver.4,5 Others have
defined GO as having an extra-abdominal liver volume of at least Fig. 1. Small omphalocele and the results of a primary closure of the defect.
50%. However, it is clear that any single definition based on a static
measurement may not capture the degree of viscero-abdominal
disproportion, causing some authors to discourage the concept of a After initial stabilization, an echocardiogram should be ob-
single unifying definition,6 or to apply the definition when closure tained, both to evaluate structural anatomy, but also to look for
without use of prosthetic material is mechanically impossible. 7 evidence of an abnormal circulatory transition that might suggest
Another factor which plays a role in selecting closure tech- the existence of significant pulmonary hypertension. An abdomi-
nique is the severity of associated pulmonary hypoplasia and pul- nal ultrasound should also be performed to look for evidence of
monary hypertension at birth, which diminishes tolerance of any renal abnormalities. The presence of unexplained dysmorphic fea-
increased intraabdominal pressure associated with surgical closure. tures should prompt a genetics consultation, particularly if a kary-
Several case series have reported that between 37 and 57% of otype was not obtained before birth. Interestingly, small central
patients with large, liver-containing omphaloceles develop clinical omphaloceles tend to have a higher incidence of chromosomal ab-
and echocardiographic evidence of pulmonary hypertension shortly normalities than do large omphaloceles. All of these investigations
after birth.8,9 Notably, the need for intubation and mechanical ven- should be obtained promptly to get as detailed and accurate an
tilation, as well as duration of mechanical ventilation, need for anti- assessment of non-omphalocele risk factors that might influence
pulmonary hypertension treatment, and length of stay and mortality treatment decisions.
were all correlates of a diagnosis of PHT in patients with a GO.
Surgical treatment
Perinatal management
The ultimate goal of surgical intervention in omphalocele is the
Initial management of the liveborn infant with a prenatally di- achievement of fascial and skin coverage, and the avoidance of a
agnosed omphalocele should take into consideration the size of physiologically intolerable increase in intraabdominal pressure. It
the omphalocele, the presence or absence of herniated liver, and is useful to think of surgical treatment in one of three domains:
the existence of associated malformations that could potentially af- (1) Immediate (primary) repair; (2) Staged repair with delayed pri-
fect initial resuscitation. Most omphalocele pregnancies can be ex- mary closure; and (3) Delayed repair (paint and wait). This review
pected to deliver at or near term. Small omphaloceles that do not will focus on the first two treatment options.
contain liver can be safely delivered vaginally, while any omphalo-
Immediate (primary) repair
cele that contains a significant volume of liver should be delivered
by caesarean section to avoid risks of hepatic injury or sac rupture.
Almost all small omphalocele defects that do not contain liver,
The delivery of babies with a GO should be attended by a neonatal
and carefully selected larger defects are amenable to primary re-
resuscitation team capable of dealing with respiratory insufficiency
pair. One issue of potential controversy that may be more rele-
that may require immediate intubation and ventilatory support.
vant from an embryologic rather than treatment perspective is the
Once the infant has been brought back to the neonatal inten-
differentiation of a small omphalocele from a congenital umbili-
sive care unit (NICU), supportive care appropriate to the baby’s
cal cord hernia (CUCH).12,13 An omphalocele results from varying
cardiorespiratory condition should be initiated. Precautions to
degrees of failure of infolding of the cephalad, caudal and lat-
both stabilize the omphalocele and protect the covering amnion
eral body folds, leading to abnormal formation of the umbilical
from mechanical injury or desiccation should be taken. This may
ring, while in CUCH the umbilical ring is normally formed, with a
involve covering the amnion with saline gauze or by placing the
“collar” of skin that extends from the abdominal wall onto the
whole lower half of the baby (to include the omphalocele) in a
neck of the sac and complete fusion of the rectus muscles in the
sterile “bowel bag”. Vascular access (preferentially avoiding the
midline. Management options for small omphaloceles and CUCH
umbilical vessels) should be established, with consideration of
include umbilical cord ligation, or formal sutured closure of the
the need for arterial access based on cardiorespiratory status.
defect, with or without umbilical cord preservation (Fig. 1). With a
Intravenous fluids with a goal of achieving and maintaining eu-
small omphalocele containing intestine, one may be tempted to re-
volemia should be initiated. Close glucose monitoring should be
duce the viscera (frequently be twisting the cord) and simply ligate
established in any baby with features of Beckwith-Weidemann
the omphalocele sac near its base, thereby avoiding a formal clo-
syndrome which occurs in approximately 15% of liveborn cases,
sure requiring general anesthesia. The risk of this approach is the
and disproportionately in association with small omphaloceles. 10
inadvertent ligation of a knuckle of intestine that may be adherent
These features include macroglossia, macrosomia, and hemihy-
to the peritoneum, and therefore does not completely reduce.14
pertrophy, or a history of polyhydramnios or placentomegaly in a
Determining eligibility for primary repair can be difficult, but
fetus that otherwise appears to have an isolated omphalocele. 11 A
a clinical assessment of tolerance of visceral reduction by careful
nasogastric or orogastric tube should be inserted.
manipulation of the sac contents at the bedside may be helpful,
particularly if the liver is fairly easily reduced. In the majority of
 E.D. Skarsgard / Seminars in Pediatric Surgery 28 (2019) 89–94
Fig. 2. A–C - Following midline fascial closure, dermal advancement sutures are used to approximate the skin edges to the cord, leading to a normal appearing neo-umbilicus.
cases of small omphalocele amenable to primary repair, this is
best carried out under general anesthesia in the operating room.
Preoperative antibiotics should be given. For small omphaloceles
not containing liver, the sac should be opened carefully, and the
viscera completely reduced. The options for closure are fascial
level ligation and division of the umbilical vessels, followed by
fascial closure (vertically, transversely or via purse-string) and
then creation of a neoumbilicus, usually by a dermal purse-string
of absorbable suture,15 or by a double purse-string technique
that creates an umbilical skin collar. 16 Alternatively, the umbilical
cord can be preserved by ligating the umbilical vessels 1–2 cm
above the fascia, and then closing the fascia around the vessels
in a purse-string manner. The vessels and redundant sac are then
centrally reconstituted into an umbilical cord, which is allowed to
desiccate and fall off in the usual manner. The resulting umbilicus
may be cosmetically superior based on retention of a cicatrix, that
is lacking in a purse-string umbilicoplasty.13
For moderate and large omphaloceles still amenable to primary
repair, but with a fascial or skin defect too large for purse-string
closure, the usual practice is a primary midline fascial closure with
umbilical cord preservation. If one is quite certain that a primary
closure can be safely achieved, it is reasonable to proceed with am-
nion excision and mobilization of skin flaps sufficient to allow mid-
line approximation of peritoneum and fascIa with reconstitution of
the umbilicus at the inferior margin of the incision. Alternatively,
one can raise skin flaps laterally until the medial edges of fascia
are encountered, and a primary fascial closure with amnion inver-
sion can be accomplished, with the advantage of non-violation of
the peritoneum and adhesion avoidance. A number of techniques
of skin-flap umbilicoplasty which target normal positioning and
elevation of the umbilicus in primarily closed omphaloceles have
been described.17–19 An example, with dermal advancement su- tures
used to approximate skin edges to a preserved umbilicus is shown
in Fig. 2.
Staged repair
One of the significant challenges facing the pediatric surgeon
and multidisciplinary neonatal team in the surgical management
of omphaloceles is the large omphalocele with a fascial defect of
5 cm or greater, where the options are a staged primary (with or
without mesh) repair, versus a conservative “paint and wait” ap-
proach with a delayed repair after complete epithelialization has
occurred. The “paint and wait” approach is described elsewhere
and is the default option for the truly giant omphalocele with ex-
ternal liver and severe viscero-abdominal disproportion, or in the
case of any large omphalocele associated with marked pulmonary
hypoplasia and hypertension. In all other babies with a large om-
phalocele, individualized, shared decision-making with the parents
91
should consider such factors as size, gestational age and cardiores-
piratory status, as well as the potential need for surgical treat-
ment of associated malformations (such as a diaphragmatic hernia,
esophageal atresia, intestinal atresia or a congenital heart defect).
This decision-making should also consider the anticipated trajec-
tory of care (for example a palliative approach, in the presence of
trisomy 13 or 18), and the preference of parents for a more ag-
gressive (and perhaps riskier) surgical approach that achieves ear-
lier abdominal closure, versus a conservative one that commits the
parents to a greater burden of dressing changes, outpatient visits
and a significantly delayed abdominal wall reconstruction.
Gradual visceral reduction techniques
In 1948, Gross described the first successful treatment of GO,
which involved widely undermined abdominal skin flaps, which
were stretched over the intact amnion and sutured in the mid-
line.20 Although, many of these children survived, they did so with
massive ventral hernias that were extremely difficult to repair. In
1963, Ravitch described the novel use of repeated needle peri-
toneum administered over many weeks to establish intraabdominal
domain following a skin flap closure. 21 Then in 1967, Schuster de-
scribed the use of prosthetic sheeting sewn into the shape of a silo,
with attachment to the fascial margins as a technique for achieving
gradual visceral reduction and delayed primary closure of a GO. 22
Since then, a number of techniques have evolved which all use the
basic principle of gradual reduction of the herniated viscera to al-
low sufficient recovery of abdominal domain that an eventual clo-
sure (primary fascia and skin, prosthetic and skin or skin alone), is
possible. Regardless of technique, one can anticipate that if gradual
visceral reduction of a GO containing liver is to be attempted, the
baby will certainly need mechanical ventilatory support, adequate
sedation with or without neuromuscular paralysis and a peripher-
ally inserted central catheter (PICC) for parenteral nutrition until
enteral feeds are fully established.
One critical decision to be made early in a planned silo reduc-
tion of a large omphalocele is whether or not to preserve the am-
nion. While some authors describe excision of the amnion to allow
direct suturing of the silo to fascia/skin, its value as a mechanical
and wound sepsis barrier during the visceral reduction process
cannot be underestimated. In addition, if attempts at reduction
are not tolerated due to increases in intraabdominal pressure, or if
the silo becomes infected or tears away from the defect margins,
amnion preservation still allows the option of conservative man-
agement; an option which does not exist if the amnion has been
excised. Silo placement with amnion preservation can be accom-
plished two ways: Mesh23 or silastic sheeting, 24,25 can be sewn
directly to skin at the amnio-cutaneous junction; or a skin flap can
be raised a few mm below the amnio-cutaneous junction, exposing
92 E.D. Skarsgard / Seminars in Pediatric Surgery 28 (2019) 89–94

Fig. 3. (A) Dissection of fascia in subcutaneous plane. (B) Suturing silastic mesh to fascial edge (C) Final construction of a “custom” silo that will facilitate gradual visceral
reduction with amnion preservation.

the fascial margin, and the sheeting can be carefully sutured di-
rectly to fascia26 (Fig. 3). Then the sheeting is sutured to itself to
construct a “custom” silo. The viscera are gradually reduced by
daily silo compression (often by clamping and suturing or appli-
cation of a linear stapler at the silo apex), until a delayed closure
is achieved. At the time of closure, the amnion can be simply
inverted into the abdomen, which is particularly useful if mesh is
required to repair the fascia, or it can be excised if that facilitates
delayed primary fascial closure. Although it would seem intuitive
that a prosthetic silo would be required to achieve reduction, some
authors have described visceral reduction without use of a silo, but
rather through the use of a technique of sequential sac ligation.27
Physiologic monitoring of intraabdominal pressure during the
course of visceral reduction is advisable. The development of ab-
dominal compartment syndrome (ACS) and its feared hemody-
namic (reduced cardiac output, splanchnic hypoperfusion, lactic
acidosis, renal failure, intestinal ischemia) and ventilatory (hy-
poventilation) sequellae and associated mortality risk should be
avoided at all costs. 28 A combination of close surveillance for clin-
ical features of ACS and trended bladder or intragastric pressure
measurements during the closure process should prevent or allow
early recognition and treatment of ACS. Another potential risk that
may require monitoring during reduction of a giant omphalocele
containing liver is the risk of hepatic vein or inferior vena cava
Fig. 4. The use of multiple subcutaneous expanders to create skin for defect
(IVC) occlusion associated with acute angulation of the hepatic cover- age. Expanders can also be placed in subfascial and intraabdominal
vein/IVC junction. This can cause acute vascular outflow obstruc- compartments.
tion of the liver, which can be detected by the sudden congested
appearance of the liver, a sudden rise in serum transaminase levels
and confirmed by sonographic doppler interrogation of the hepatic Fascial closure techniques
vasculature through the silo.29
Once gradual visceral reduction has sufficiently established
Tissue expanders
intraabdominal domain, fascial closure can be attempted. It can
be challenging to predict whether primary fascial closure will be
The recognition of the value of tissue expansion as a means
achievable, and the surgeon needs to have a backup plan if it
of increasing soft tissue surface area has encouraged
is not. The alternatives to simple midline fascial closure include
collaboration between pediatric surgeons and their plastic
fascial advancement techniques such as component separation, 33,34
surgical colleagues in dealing with the challenges of abdominal
closure with mesh (prosthetic or biologic), or skin flap closure,
wall reconstruction associated with GO. A number of case
as in the original method of Gross.20 Whatever procedure is
reports have demonstrated success in intraabdominal tissue
chosen should be undertaken in the operating room under optimal
expansion, usually by placement of the expanders in lower
conditions, with intraoperative invasive blood pressure monitoring,
abdomen and pelvis,30,31 and then gradually expanding to achieve
bladder or intragastric pressure monitoring and pre-established
intraabdominal domain. Care must be taken during this process
ventilatory goals and blood gas targets. With the silo removed, and
to ensure that recovery of intra- abdominal domain does not
the amnion intact (assuming it was preserved) primary fascial clo-
compromise ventilation or cause inad- vertent visceral pressure
sure can be simulated to assess physiologic tolerance. If it appears
injury. Other potential tissue planes that can expanded as a
that a midline fascial closure is feasible and safe, consideration
primary strategy of increasing the available sur- face for soft
should be given to the benefit of removing the amnion to allow
tissue coverage are the intramuscular plane between transversus
additional intraabdominal domain. If the amnion is removed, care
abdominus and internal oblique 32 and the subcuta- neous plane
should be taken with the portion that is frequently adherent to
(Fig. 4). Because of the various options for expansion,
the liver capsule, which may be best left attached. In addition, the
consideration of its potential application in abdominal wall
abnormal positioning of the liver often results in the hepatic veins
reconstruction should encourage an early collaborative
being located just beneath the amnio-cutaneous junction in the
discussion with the plastic surgical team so that its use is
superior midline, making them susceptible to inadvertent injury.
optimally timed.
 E.D. Skarsgard / Seminars in Pediatric Surgery 28 (2019) 89–94
The decision to use a mesh should consider the size and loca-
tion of the residual fascial defect. If the defect is small and central,
and particularly if there is residual covering amnion that would
prevent acute herniation of bowel through the defect, it may be
best to avoid mesh altogether and accept a small ventral hernia
that can be closed later. Large sub-xiphoid defects associated with
Pentalogy of Cantrell are usually associated with deficient fascia
extending up to the costal margins, and these may be best treated
with mesh. The reported experience of non-absorbable meshes
such as TeflonTM ProleneTM (Ethicon, Cincinnati, OH) and Gore-
TexTM (Gore and Associates, Flagstaff, AZ) includes a
heterogeneous group of small case series of patients who have
undergone pri- mary, staged and delayed closures with or without
preservation of amnion, in which prosthetic material is used to close
the fascial defect.23,35,36 The limited follow up data suggests a fairly
high (∼25%) rate of mesh-related complications, mostly
infectious, that eventually require mesh removal weeks to months
later. The experience with biological mesh has been more
contemporary and has included a number of biomaterials including
porcine small intestinal submucosa (SIS TM, Cook Medical,
Bloomington, IN),37–39 human acellular dermis (AlloDermTM,
LifeCell Corporation, Bridge- water, NJ)39 porcine, un-crosslinked
dermal collagen (StratticeTM, LifeCell Corporation, Bridgewater,
NJ)40and porcine, crosslinked dermal collagen (Permacol TM,
Medtronic, Fridley, MN).41,42 In these small cases series, the
complication profile associated with biologic mesh appears to be
quite favorable, with low rates of infection, and very infrequent
need for patch removal due to infection. In one case series of 9
omphalocele patients treated with Surgisis, 5 patients
experienced 6 recurrences within a median follow up of 60
months. An experimental study compared the histologic
inflammatory responses between surgisis and alloderm mesh in a
rabbit model of omphalocele. 39 This study noted a reduced inflam-
matory response and greater biocompatibility with surgisis, which
was in part attributed to its absorption, which was complete by 16
weeks. This may account for the high comparative recurrence rate
observed in humans in comparison to the acellularized dermal
collagen products which are not resorbed.
The discussion of ruptured omphalocele is covered extensively
in another article in this issue.
Management of rotational abnormalities in
omphalocele
The disruption of the normal embryologic events of rotation
and fixation of the intestine predisposes infants with omphalocele
to malrotation and its potential consequences. However, the over-
all rates of malrotation and the proportion of patients who develop
midgut volvulus is difficult to ascertain.
In a combined institutional series of 414 abdominal wall de-
fect patients, of which 115 (28%) had omphalocele, the rates of
post-closure volvulus occurrence were higher for omphalocele pa-
tients (4.4%) compared to gastroschisis (1.0%), and were equally
distributed amongst those patients with small or GO. 43 However,
in a national administrative cohort study of omphalocele patients,
the performance of a pre-emptive Ladd procedure in 42 (vs 1027
who did not undergo Ladd procedure) was associated with a sig-
nificantly increased risk of subsequent volvulus for (9.1% vs
0.1%).44
Conclusions
The surgical closure of omphalocele presents a number of op-
tions that must be carefully tailored to the size of the defect,
the severity of viscero-abdominal disproportion and the presence
and severity of associated comorbidities, particularly pulmonary
hypoplasia. The emergence of a variety of novel reconstructive
options which include autologous tissue expansion and biologic
meshes represent significant advances that offer greater choice and
93
flexibility in the management of omphaloceles not amenable to
primary closure. When faced with a GO that appears amenable to
either a primary or delayed primary fascial closure, amnion preser-
vation maintains the default option of a “paint and wait” approach,
if closure attempts are ultimately unsuccessful. Finally, a dedicated
multidisciplinary team with expertise in antenatal diagnosis, post-
natal management of physiologic impairment caused by either pul-
monary hypoplasia or a tight abdominal closure, as well as surgical
expertise is critical to optimizing the short and long-term outcome
of these patients.
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