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Congenital Abdominal

Wall Defects
Omphalocele vs.
Gastroschisis
Definition

Gastroschisis Omphlocele
Defect of the Herniation of the
anterior abdominal abdominal contents
wall in which the through the
viscera extrudes and umbilical ring,
is not covered by covered by a sac
amnion or a with an outer layer
peritoneal of amnion and and
membrane inner layer of
peritoneum.
Epidemiology
Gastroschisis Omphalocele
Incidence 1 per 6,000 Incidence 1 per 2500
M:F 1.5:1
M:F 1:1 70% or more association with
10-15% association congenital anomalies (cardiac,
GI, CNS, musculoskeletal,
with congenital genitourinary)
anomalies like Trisomies 13, 18,21.
CHD(VSD), cleft CHARGE (coloboma, heart,
palate and intestinal atresia choanae, retardation,
genitourinary, and ear
atresia
abnormalities)
40% are premature or Beckwith-
SGA Wiedemann( gigantism,
hemihypertophy, macroglossia_
Pathogenisis
Gastroschisis Omphalocele
Several hypotheses
Failure of the
Failure of the mesoderm to
form the body wall
lateral folds to
Rupture of the amnion around migrate and fuse
the umbilical ring appropriately at the
Abnormal involution of the umbilical ring,
right umbilical vein leading to resulting in
weakening of the body wall continued
Disruption of the right vitelline herniation of the
artery with subsequent body midgut.
wall damage
Abnormal folding of the body
wall resulting in a ventral
body wall defect
Risk Factors
Gastroschisis Omphalocele
Young maternal Increased
age <20 maternal age,
Low gravida Twin gestation
Prematurity High gravida
Low birth-weight Obesity
Diagnosis
Alpha-feto-protein: synthesized in the fetal liver and
excreted with fetal kidneys crosses the placenta by 12
weeks
Elevated alpha-fetoprotein on maternal triple-screen
evaluation.
Also associated with neural tube defects, duodenal or
esophageal atresia
Sensitivity is higher for gastroschisis.
40% false positive rate.

Maternal US after 12 weeks GA can identify abdominal


wall defects since the midgut usually returns to the
abdominal cavity.
Gastroschisis:
Right of the intact
umbilical cord.
No Covering.
Opening</= 5cm
Thickened edematous
bowels
Omphalocele
Central defect beneath the
umbilical ring
Covered by Sac of amnion
Whartons jelly and peritoneum
2-12 cm
Umbilical cord directly into the
sac either apically or laterally
Large ones can involve liver,
spleen, bladder
Approach
Multidisciplinary approach is recommended with
gentic counseling, radiology, obstetrics,
neonatologist, and pediatric surgery
Parents should be counseled as soon as the
diagnosis is made, particularly if lethal
anomalies are identified as well.
Serial ultrasounds should be performed over the
course of the pregnancy, particularly due to risk
of rupture of omphalocele and consequential
growth retardation and pulmonary hypoplasia.
Management
ABC at delivery
Create a warm aseptic environment with
warm sailed soaked gauze/ sterile bowel bag
Nasogastric or oral gastric tube for
decompression of stomach and viscera.
IV access for vascular support and antibiotics
should be established
Might require mechanical ventilation based
on pulmonary status.
Management
Gastroschisis Omphalocele
Timely primary Identify other possible
closure due to anomalies
increased evaporative Primary closure for small and
fluid loss and risk of
medium sized.
infection
Large needs staged closure:
Careful examination
of intestinal atresia Silo in the first 24 hrs that is
before closure gradually reduced over 3-7
days in the NICU and then final
Primary closure
closure of the abdominal wall
success rate of 70%,
in the OR.
if unsuccessful then a
staged closure can be
used.
Complications
Atresia:
Intestinal atresia in 10% of cases with gastroschisis.
If discovered at delivery can be reduced with bowel and
decompressed with nasogastric suction.
Can also explore abdomen 2-4 weeks after the reduction for
definitive repair once the inflammation has reduced.
If not initially discovered consider it possibility in infants with
prolonged feeding intolerance.

Abdominal Compartment Syndrome:


Risk of bowel ischemia after closure if its too tight or if
positioning caused vascular compromise.
Pressure should be monitored intra operatively and post op
Post op pressure greater than 20mmHG requires surgical
intervention and is an emergency.
Risk of short bowel syndrome if not treated timely.
Prognosis
Gastroschisis Omphalocele
Survival rate 90- Survival rate:
100% 70-90%
Mortality related
more to cardiac
anomalies.
Resources
Stephenson, Courtnery. "Gastroschisis." Gastroschisis. Uptodate, 5
Jan. 2017. Web. 26 Jan. 2017.

Stephenson, Courtney. "Omphalocele." Omphalocele. Uptodate, 30


Nov. 2016. Web. 26 Jan. 2017.

Bishop, Warren P. Pediatric Practice. New York: McGraw-Hill


Medical, 2010. 201-17. Print.

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