Ventral Abdominal Wall DefectsSara A. Mansfield, MD, MS,* Tim Jancelewicz, MD, MA, MS*

*Division of Pediatric Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, TN

Practice Gap

Clinicians should be aware of the strategies for prenatal and postnatal

management of infants with omphalocele and gastroschisis.

Objectives After completing this article, readers should be able to:

1. Recognize the implications and differences in management between

gastroschisis and omphalocele.

2. Review the initial stabilization and management of infants with

omphalocele and gastroschisis.

3. Review the surgical management options and rationale for infants with

ventral abdominal wall defects.

INTRODUCTION

Abdominal wall defects represent a wide spectrumof congenital anomalies. These

can range from lethal limb-body wall syndrome to benign umbilical cord hernias.

Gastroschisis and omphalocele are the 2 most common defects and are the focus

of this review. Although both of these diseases affect the umbilical area, they differ

widely in their underlying pathogenesis, genetics, and associated disorders.

Consequently, their management techniques and outcomes are quite different.

This article reviews the pathogenesis, genetics, diagnostics, and outcomes for

each disease, followed by an in-depth review of recent management updates.

OMPHALOCELE

PathogenesisOmphalocele (or exomphalos) is a herniation of the abdominal viscera through a

midline abdominal wall defect (Fig 1). This defect is located at the base of the

umbilical stalk, and herniated viscera are covered by a 3-layer membrane of

peritoneum,Wharton jelly, and amnion. This contrasts with umbilical cord hernias,

which are covered by intact skin and contain only a small protrusion of abdominal

contents, and gastroschisis, which has no covering at all and occurs to the right of

midline (Table). Omphaloceles are present in approximately 1 per 1,100 pregnan-

cies.However, there is a high rate of spontaneous abortion,making the incidence of

omphalocele per live birth approximately 1 per 4,000 to 1 per 6,000. (1)(2)

During the fourth week of gestation, the flat embryonic disc undergoes a series

of craniocaudal and lateral folding that creates a tubular, C-shaped embryo. Lateral

AUTHOR DISCLOSURE Drs Mansfield andJancelewicz have disclosed no financialrelationships relevant to this article. Thiscommentary does not contain a discussion ofan unapproved/investigative use of acommercial product/device.

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folding continues until the somatic mesoderm reaches the

midline, creating the future peritoneal cavity. As the gastroin-

testinal tract grows rapidly, it herniates through the umbilical

stalk during the sixthweek of gestation due to inadequate space

in the abdominal cavity. The midgut then undergoes 270°

counterclockwise rotation before returning to the abdomen

during gestational weeks 11 and 12. Omphalocele is thought to

occur due to failure of the abdominal viscera to return to the

abdomen after normal physiologic herniation. (3)

The herniated organs are variable and most commonly

include the small bowel but can include others, such as the

stomach, colon, bladder, and liver. (3)(4) In contrast to

gastroschisis, the overlying sac protects the abdominal

contents from the irritating effects of amniotic fluid expo-

sure. With an intact sac, infants with omphalocele typically

have normal gastrointestinal motility. However, ileus may

be seen in the setting of a ruptured sac. The severity of the

ileus is typically associated with the duration of exposure to

amniotic fluid or the external environment.

Most morbidity seen in infants with omphalocele is due to

associated congenital anomalies. More than 50% to 70% of

infants bornwithomphalocelewill haveother congenital defects.

Almost half of these infants will have a major congenital heart

defect. The long-termoutcomes aredirectly related to these other

anomalies rather than to the abdominalwall defect itself. Isolated

omphalocele has a survival rate of more than 95%. (5) Overall

survival has been difficult to assess given the high spontaneous

and elective termination rate. (6) Survival rates have been cited at

less than 20% to 50% in prenatally diagnosed cases, which

includes cases of termination. (1)(6)

Omphalocele is frequently seen as part of genetic disor-

ders and syndromes. Approximately 10% of infants with

Figure 1. A. Lateral view of a newborn with an omphalocele containing liver and intestine. B. Anterior view. C. Two-year-old girl with a giantomphalocele that was completely epithelialized. The defect contained the entire liver and intestines. D. First-stage closure of a giant omphalocele, withpartial reduction of contents due to lack of abdominal domain. E. Second-stage closure of a giant omphalocele, 3 months after the first stage. Primaryfascial closure was achieved with this approach.

TABLE. Defining Characteristics of Abdominal Wall Defects

CHARACTERISTIC OMPHALOCELE GASTROSCHISIS UMBILICAL HERNIA

Sac Peritoneum, Wharton jelly, amnion Absent Intact skin

Location of defect Umbilicus Right of umbilicus Umbilicus

Associated anomalies Common (50%) Uncommon (<10%) Uncommon

Atresias Uncommon 10% Uncommon

Prognosis Associated anomalies Bowel motility Excellent

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omphalocele will have Beckwith-Wiedemann syndrome

(macroglossia, gigantism, omphalocele, hypoglycemia).Genetic

abnormalities are identified in approximately one-third of

infants with omphalocele and include trisomy 12, 18, and 21.

Other conditions that result from incomplete folding of the

body wall folds during embryogenesis manifest as variants

of omphalocele. Infraumbilical omphalocele is often asso-

ciated with genitourinary abnormalities and includes blad-

der or cloacal exstrophy as part of OEIS association

(omphalocele, exstrophy of the bladder, imperforate anus,

and spinal defects) (Fig 2A).

Pentalogy of Cantrell is a rare lesion (5.5 per 1 million live

births) consisting of epigastric omphalocele, inferior sternal

defect, anterior diaphragmatic defect, and intracardiac and

pericardial defects (Fig 2B). The heart is positioned outside

the thoracic space within the epigastric defect (Video 1).

There is a spectrum of severity, and survival is related, in

general, to the type of cardiac defect; incomplete or partial

pentalogy tends toward more favorable outcomes than com-

plete pentalogy. (7) When associated with complete ectopia

cordis, survival is extremely poor (Fig 2C).

Prenatal Diagnosis and ManagementOmphaloceles are usually diagnosed prenatally in the latefirst

or second trimester. Elevated maternal serum a-fetoprotein

levels should raise suspicion of an abdominal wall defect.

Ultrasonography has a sensitivity of 75% to 80% and a

specificity of approximately 95% for both omphalocele and

gastroschisis. (1)(8) As mentioned previously, the midgut

undergoes physiologic herniation during the sixth week of

gestation and does not fully return to the abdomen until week

11 or 12. Therefore, a diagnosis of omphalocele should not be

made before the 12th week of gestation. Karyotype analysis is

indicated for all cases of suspected omphalocele. Often the

prenatal diagnosis is straightforward, allowing providers to

adequately counsel the patient and provide perinatal plan-

ning. However, it is important to note that prenatal imaging

and karyotyping may miss 30% to 40% of associated anom-

alies that are subsequently found after birth. (2)

In cases in which the diagnosis is not evident, further

imaging may be necessary. Fetal magnetic resonance imag-

ing (MRI) has been advocated to better delineate anatomy,

allowing for more accurate prenatal counseling. (9) Umbil-

ical cord hernia, which has an excellent prognosis and a low

likelihood of associated anomalies, can be difficult to dif-

ferentiate from gastroschisis and omphalocele on conven-

tional ultrasonography. However, in the second and third

trimesters, the intact skin seen in umbilical hernias be-

comes apparent on MRI. If an umbilical cord hernia is

present, knowledge of this finding should be communicated

to delivery room staff to avoid injury to the bowel when

clamping the umbilical cord.

When an omphalocele is diagnosed, prenatal evaluation

should focus on identifying associated cardiac and central

nervous system defects because these are the major deter-

minants of outcome. Although ultrasonography is highly

accurate at diagnosing omphalocele, (10) fetal MRI can add

more information to the prediction of postnatal morbid-

ity. (11)(12) The degree of pulmonary hypoplasia can be

Figure 2. A. Classic cloacal exstrophy with open cecum and prolapsed terminal ileum (TI); visible is 1 hemibladder (HB). B. Pentalogy of Cantrell withepigastric omphalocele, sternal defect, anterior diaphragmatic defect, and pericardial defect, allowing the heart (H) to be visible in the epigastrium; thischild was discharged from the hospital with staged repair planned at a later date. C. Complete ectopia cordis and pentalogy of Cantrell, with the heart(H) completely outside the chest; this newborn died within 24 hours of birth.

Video.due to the sternal defect, abdominal wall defect, anterior diaphragmaticdefect, and absent pericardium.

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Pentalogy of Cantrell. The beating heart is visible under the skin

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predicted by measuring the ratio between the lung and

thorax transverse area. Lung volume with an observed-to-

expected ratio less than 50% is associated with higher

morbidity. (11)(13) In addition, the ratio of the largest diam-

eter of the omphalocele to the head circumference can

predict postnatal respiratory insufficiency, as well as the

need for staged versus primary closure. (6) However, there

are no prospective studies addressing these models. MRI is

most valuable after 20 weeks’ gestation, at a time when

termination may no longer be an option. The utility of MRI

seems to be in the orchestration of the advanced care needed

at delivery (ie, extracorporeal membrane oxygenation). (14)

MRI has not been studied regarding earlier screening and

prognostic abilities.

After diagnosing an omphalocele, plans should be made

for delivery at a tertiary care center with neonatology and

pediatric surgical support. Full-term delivery is recom-

mended unless earlier delivery for other obstetric indica-

tions or fetal distress is warranted. The route of delivery

continues to be a topic of debate, and no study has dem-

onstrated a benefit of cesarean delivery over vaginal delivery.

Most retrospective studies have demonstrated that infants

with small omphaloceles can be safely delivered vaginally.

(15) Cesarean delivery should be performed only for obstet-

ric indications. (16)(17) Some advocate for cesarean delivery

of babies with giant omphaloceles due to the theoretical risk

of sac rupture during vaginal delivery and the risk of hepatic

trauma in infants with herniated liver. (18) There is a lack of

data on the topic, and further studies are needed to make

meaningful recommendations.

Postnatal ManagementAs mentioned previously herein, the main source of mor-

bidity to an infant with an omphalocele is cardiopulmonary

defects. Thus, the initial management of infants should

include thorough cardiac and respiratory evaluation with

cardiac support and intubation if necessary. Fluid resusci-

tation should follow because the hernia defect predisposes

to large fluid losses (although less than that seen with

gastroschisis). An orogastric tube should be placed for

gastric decompression. The sac should be kept moist and

protected with saline-soaked gauze. Infants with surgical

issues such as omphalocele typically receive 48 hours of

antibiotics and undergo an evaluation to rule out sepsis.

Antibiotics can then be discontinued if cultures show no

growth at 48 hours. Administering antibiotics may not be

necessary in infants with intact omphalocele and should

not be given for a long duration. (19) Once stabilized, a

thorough evaluation should be performed to assess associ-

ated anomalies. This includes echocardiography, renal

ultrasonography, and karyotype analysis. Definitive surgical

correction is not an emergency in infants with an intact sac,

allowing time for a thorough evaluation. If the sac does

rupture, the infants are treated in a more urgent manner to

cover the exposed viscera, similar to treating infants with

gastroschisis. Enteral feeding can be started after initial

stabilization in infants with unruptured omphalocele and

can be safely given while awaiting definitive closure of the

abdominal wall because gastrointestinal motility and func-

tion are not affected.

Surgical management of the abdominal wall defect is

dictated by the size of the defect, the presence of an intact

sac, and the underlying cardiopulmonary status of the

infant. For small defects, primary closure should be attempted.

After excising the omphalocele sac, the umbilical vessels

can be ligated. The fascial edges are identified after the

creation of skin flaps. After reduction of the abdominal

viscera, the fascia is transversely closed. The overlying skin

can be closed with a purse-string suture, recreating the

umbilicus. (20)

Schuster was the first to describe the use of a silastic silo

to gradually reduce the abdominal contents into the abdo-

men. After excising the omphalocele sac, the silo is sewn to

the fascia or to the full thickness of the abdominal wall.

Serial reductions are then used to return the contents to the

abdomen, with the goal of fascial closure. The omphalocele

sac itself can be used as a silo if the sac is free from the

underlying viscera, or a silo can be placed over the sac. The

sac is sequentially tightened until the viscera have reduced,

allowing surgical fascial closure. Some surgeons will leave

the sac intact at the time of fascial closure, known as the

amnion inversion technique. (21)

Babies with significant cardiopulmonary diseasemay not

tolerate supraphysiologic increases in abdominal pressure.

For these, initial nonoperative management is preferred,

with plans for delayed surgical closure. The “paint and wait”

technique is a nonoperative strategy for large defects or for

infants who are poor surgical candidates. (22) A thin layer

of silver sulfadiazene cream is applied to the omphalocele

sac and wrapped with sterile gauze. This is repeated daily

until the sac is fully replaced with granulation tissue (usually

3–4 weeks). (23) Silver sulfadiazene can be applied by the

parents at home, allowing the infant to be discharged

from the hospital until ready for staged abdominal wall

closure.

The term giant omphalocele is used for defects larger than

5 to 10 cm (Fig 1 C–E). There are several options for manage-

ment. The Gross technique uses a bridging mesh (biologic or

synthetic) with a planned hernia repair at a later date (24) or

placement of a temporary vacuum dressing. (6)(25)(26) Once

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adequate abdominal wall domain is obtained, the fascia can

then be closed (Fig 1E). (27) Many different techniques are

performed because no single method is uniformly applicable

or successful.

As discussed previously herein, the embryogenesis of

abdominal wall defects includes interruption of the normal

rotation of the intestines. This leads to abnormal or absent

rotation of the intestines in infants with omphalocele. There is

a risk of developingmidgut volvulus in infants with abdominal

wall defects. The risk is higher in infants with omphalocele,

particularly those bornwith an intact sac. This is possibly due to

fewer adhesions that are formed after reduction, which would

normally protect the bowel from volvulus. (28) Therefore,

some surgeons have recommended performing a Ladd pro-

cedure at the time of closure to decrease these risks. (29)

However, other studies have not supported this practice and

found that thesepatients are still at risk for volvulus, evenwith a

prophylactic Ladd procedure. (30)

OutcomesMortality rates of omphalocele range from 15.6% to 52.4% in

the neonatal period. As mentioned, survival varies depend-

ing on the presence of associated anomalies. A review from

the National Birth Defects Prevention network found mor-

tality of 28.7%, with 75% of deaths occurring within the first

month. Survival was better in neonates with isolated

omphalocele compared with those with chromosomal

defects (hazard ratio, 7.75; 95% confidence interval, 5.4–

11.1). (31) The 1-year survival of babies with isolated ompha-

locele is greater than 90%, compared with 27% in those

with chromosomal anomalies. (32)

GASTROSCHISIS

PathogenesisGastroschisis is a defect of the abdominal wall located 1 to 2

cm to the right of the umbilicus (Fig 3), and it has a very

different clinical picture from an omphalocele. Gastroschi-

sis is a herniation of the bowel without a membranous

covering (Table) and is typically an isolated finding, without

associated congenital anomalies. The morbidity seen in

gastroschisis is directly related to the gastrointestinal dys-

motility that results from exposure to amniotic fluid during

gestation. Intestinal atresias are seen in approximately 6%

to 28% of infants with gastroschisis. (33)(34)(35) This is

thought to be due to trauma of the bowel against the

abdominal wall or vascular compromise from segmental

volvulus.

The incidence of gastroschisis has nearly doubled during

the past few decades, with a current rate of approximately 2

to 5 per 10,000 live births. (36)(37) The most significant risk

factor seems to be young maternal age, with a sevenfold

Figure 3. A. Gastroschisis with moderate bowel edema and fibrinous exudate from amniotic exposure. B. The bowel was placed into a silo to allow slowreduction over 2 to 3 days. C. Complex gastroschisis with herniated liver (L) and at least 1 intestinal atresia with visible mucosa (M); this child requiredvery slow reduction along with stoma creation, which was very difficult due to lack of abdominal wall surface. D. Sutureless (nonoperative) closure ofgastroschisis: (i) initial presentation with minimal bowel edema; (ii) successful reduction without a silo, showing the residual defect; (iii) coiling of theumbilical cord over the defect, followed by application of an occlusive dressing; (iv) appearance of the defect after 1 week of coverage; and (v)appearance of the umbilicus at 1 year of age (no umbilical hernia in this case).

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incidence among teenage mothers. Tobacco, (38)(39) recre-

ational drug, (40) and certain decongestant (41)(42) use have

also been implicated. These risk factors have been the basis

for embryologic theories. The association with sympatho-

mimetic agents such as smoking, cocaine, amphetamines,

and decongestants supports a vascular accident hypothe-

sis. Theories regarding the embryogenesis of gastroschisis

include failure of lateral body wall folding, localized para-

umbilical tissue weakness associated with regression of the

right umbilical vein, and vascular accidents of the vitelline

artery. (43)

In some cases, the abdominal wall defect may close

before birth. If bowel is present in the defect, this can result

in strangulation, necrosis, and possible amputation. This

phenomenon is referred to as “vanishing gastroschisis.”

Occasionally the bowel will be seen emerging from the

abdomen (Fig 4). The proximal bowel is typically dilated

and dysmotile. In severe situations, a large mass of bowel is

compromised and can lead to devastating short bowel

syndrome. Smaller abdominal wall defects are associated

with complex gastroschisis (vanishing gastroschisis, atresia,

necrosis, perforation, etc). (44)

Prenatal Diagnosis and ManagementUnlike omphalocele, intrauterine demise is uncommon

with gastroschisis. Whereas infants with omphalocele are

typically born at term, those with gastroschisis have an

average gestational age of 34 to 37 weeks. (18) Gastroschisis

is associated with higher maternal a-fetoprotein levels than

an omphalocele (7–9 times normal versus 4 times normal).

On ultrasonography, the umbilical cord insertion site is

normal, but the free-floating bowel is seen outside the

abdomen. The defect can be seen in a paramedian location,

usually to the right of the umbilicus. Gastroschisis most

commonly occurs as an isolated finding, but it is important

to look for additional malformations. Approximately 10% of

fetuses with gastroschisis will have an additional anomaly.

Serial ultrasonography is important to monitor bowel

viability. Bowel loops may appear thickened as irritation

from amniotic fluid worsens. Bowel dilation can be seen,

and if severe should be a warning of an underlying atresia.

Worsening dilation and echogenicity should raise the con-

cern of threatened, ischemic bowel. Some experts recom-

mend early delivery when signs of bowel compromise

develop. Survival for infants with gastroschisis decreases

to 70% if ischemia, perforation, or atresias are present at

birth. A recent meta-analysis reported correlations between

sonographic evidence of intra-abdominal bowel dilation and

polyhydramnios with the presence of bowel atresia, as well

as a correlation between gastric dilation and neonatal death.

(45)

As with omphalocele, vaginal delivery is preferred unless

an indication for cesarean delivery is present for obstetric

reasons or fetal distress. Currently, there is no difference in

outcomes between cesarean and vaginal delivery in the

literature. A recent meta-analysis of available observational

studies found no difference in overall mortality, ability to

undergo primary repair of the abdominal wall defect, inci-

dence of necrotizing enterocolitis, sepsis, time to full feeds,

or duration of hospital stay. (46) There has been debate over

the timing of delivery. Some groups advocate that preterm

delivery is associated with shorter duration of exposure to

amniotic fluid and, thus, less bowel dysmotility. Multiple

studies, including a Cochrane review, showed no difference

or higher rates of adverse outcomes associated with delivery

before 36 weeks, which exposes the infant to undue risks of

prematurity. (47)(48)(49)Most centers wait until the natural

onset of labor.

Postnatal ManagementImmediately after birth, the bowel must be protected from

the environment to conserve humidity and temperature.

The “bowel” bag, or Lahey bag, is the most common option.

The lower half of the infant is placed into the sterile bag,

Figure 4. A. Appearance of vanishing gastroschisis at birth with infarcted intestine and a tiny residual gastroschisis defect. B. Appearance of the proximaldilated small intestine and resected infarcted segment in the same patient at laparotomy. A primary serial transverse enteroplasty procedure wasperformed on this patient, and she became independent of parenteral nutrition within a year of birth.

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which is then loosely tied around the chest. The bowel

should be positioned in a way that protects the mesentery

from twisting or kinking against the abdominal wall. The

patient is laid right side down such that venous drainage

from the bowel is not compromised by kinking against the

fascial ridge. Judicious resuscitation is guided by vital signs,

capillary refill, urine output, and acid-base status. Resusci-

tation with additional fluid boluses beyond that indicated by

clinical parameters can lead to a need for mechanical venti-

lation due to pulmonary edema. (50) Intravenous access

should be obtained in anticipation of starting total parenteral

nutrition soon after birth.

Primary surgical closure of the defect is the preferred

method if the abdomen is amenable without causing

abdominal compartment syndrome. This condition occurs

when there is insufficient abdominal domain to accommo-

date reduction of the viscera and fascial closure, leading to

impaired venous return, decreased pulmonary compliance,

and organ injury (renal failure, bowel ischemia). The most

common closure method is suturing of the abdominal wall

fascia. Most recently, a “sutureless” closure is gaining

popularity. (51) With this approach, the bowel is reduced,

and the umbilical cord remnant is positioned over the

abdominal wall defect (Fig 3). A tight adhesive dressing is

placed over this and changed every few days until the fascial

defect has closed. Some infants may initially develop an

umbilical hernia, but many will close over time. Sutureless

closure can be performed at the bedside without general

anesthesia, a benefit over sutured fascial closure; (52) there

is also a lower rate of required mechanical ventilation.

(53)(54) The only randomized controlled trial on the matter

showed no benefit of sutureless (n¼19) compared with

classic sutured (n¼20) closure in length of stay or time

to full enteral feeding. (55)

If there is too much eviscerated bowel or if the bowel is

too edematous to immediately reduce, a preformed silastic

silo is placed (Fig 3B). This can be performed in the NICU.

The silo is then tightened once or twice daily until the

contents are fully reduced. These reductions can also be

performed at the bedside with a small amount of sedation.

Constant attention is given to the hemodynamics of the

infant during reduction. Once fully reduced, the defect can

then undergo surgical closure or sutureless closure. With

any of the closure methods, the bowel should be inspected

for the presence of intestinal atresias before definitive

closure.

In babies with complex gastroschisis (atresia, perfora-

tion, necrosis), a bowel resection or ostomymay be required.

The bowel is typically too edematous to safely repair intes-

tinal atresias immediately at birth. Therefore, these are

addressed after 4 to 6 weeks, when an anastomosis is

feasible. A contrast enema is usually performed to confirm

the level of the lesion(s).

The bowel is nonrotated in babies with gastroschisis.

However, the degree of intestinal adhesions in these infants

is usually significant enough that volvulus is a rare event.

(28)

OutcomesAfter surgical correction, infants with gastroschisis are still

faced with significant ileus and bowel dysmotility, result-

ing in hospitalization for weeks tomonths. Total parenteral

nutrition should be started soon after birth in anticipation

of lack of enteral nutrition, as ileus averages 2 to 3 weeks.

(43) After correction, the amount and character of the

nasogastric tube output is monitored before initiation of

enteral nutrition. Enteral feeds are then slowly advanced,

and the infant is monitored for tolerance. Standardization

of care may help with day-to-day management of these

infants. (56)(57) Overall survival in infants with gastro-

schisis is more than 90%. Interdisciplinary intestinal

failure teams should be involved to aid in long-term

dysmotility management.

References for this article are at http://pedsinreview.aappub-

lications.org/content/40/12/627.

SummaryOmphalocele and gastroschisis are 2 of the most commoncongenital abdominal wall defects. The 2 disorders vary widely inpresentation, embryologic origin, and management.• Based on some research evidence as well as consensus (Level B),outcomes with omphalocele are excellent in the absence of anysignificant associated anomalies. (31)(32)

• There is good evidence (Level A) that survival in gastroschisisexceeds 90%, with morbidity primarily in those with intestinalinjury or atresia. (45)(46)

• Based largely on mid-level evidence and a few randomized trials(Level B), there is general consensus regarding management ofaffected infants. (6)(17)(18)

• Based on Level B evidence, a multidisciplinary, standardizedapproach to treatment can lead to excellent long-term outcomes.(57)

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1. You are called to the hospital for the impending delivery of a 37-week neonate withprenatally diagnosed omphalocele. You discuss the planwith the delivery team and explainto the medical students on the team the anatomy of this condition. In your discussion,you explain that omphalocele is a herniation of the abdominal viscera through a defect inwhich of the following anatomical structures?

A. Abdominal wall.B. Amnion.C. Peritoneum.D. Umbilical stalk.E. Wharton jelly.

2. You are providing postnatal care for a term neonate with omphalocele. The baby was bornat term to a 22-year-old primigravida mother who received prenatal care. Prenatalultrasonography provided the diagnosis of abdominal wall defect along with severalcongenital anomalies. Pregnancy was complicated by oligohydramnios. You consultpediatric surgery and genetics. In considering the sources of greatest risk for morbidity inthis patient, which one of the following factors accounts for most of the potential cause ofmorbidity in this patient?

A. Associated congenital anomalies.B. Gastrointestinal dysmotility.C. Irritating effects of amniotic fluid exposure.D. Limited pulmonary capacity.E. Young maternal age.

3. The mother of a patient in your pediatric practice is pregnant with her second child. Shecontacts you to get your opinion on the result of her prenatal ultrasonography, which raisedconcern for omphalocele. Her serum a-fetoprotein levels were elevated. Which one of thefollowing diagnostic tests is indicated for all cases of suspected omphalocele based onultrasonography findings?

A. Biophysical profile.B. First trimester fetal magnetic resonance imaging.C. Karyotype analysis.D. Repeated serum a-fetoprotein level.E. Weekly nonstress testing.

4. The mother in question 3 is at 35 weeks’ gestation with a smooth pregnancy course. She isreferred to the nearby tertiary care hospital, where she will be delivered by her obstetrician.The neonatology team will be present at the delivery. Which of the following is the mostappropriate step to be considered in the peripartum and postnatal care of this infant?

A. Cesarean delivery.B. Emergent definitive surgical correction at day 0 after birth.C. Fluid restriction to avoid pulmonary edema.D. Induction of labor at 36 weeks’ gestation.E. Orogastric tube placement immediately after delivery.

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5. A newborn male infant with gastroschisis is born at 37 weeks’ gestation. The baby isadmitted to the NICU where he is stabilized. Surgical correction is planned in the nextfew weeks. The family asks how long they should anticipate their baby to remain in thehospital after surgical correction. Which of the following best describes the expectedinpatient stay after surgical correction for gastroschisis in this patient?

A. At least 72 hours, until oral feeds are tolerated.B. On average 10 days, until an antibiotics course is completed.C. Several days postoperatively, until the first bowel movement.D. Up to 3 weeks, until weight gain is established.E. Weeks to months, due to significant dysmotility.

Vol. 40 No. 12 DECEMBER 2019 635 at Bibliotheque Univ Paris V on January 22, 2020http://pedsinreview.aappublications.org/Downloaded from

DOI: 10.1542/pir.2018-02532019;40;627Pediatrics in Review 

Sara A. Mansfield and Tim JancelewiczVentral Abdominal Wall Defects

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DOI: 10.1542/pir.2018-02532019;40;627Pediatrics in Review 

Sara A. Mansfield and Tim JancelewiczVentral Abdominal Wall Defects

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