3. History
• 1634 - Ambroise Paré (French barber surgeon) first
described Omphalocele.
• Derived from Latin word “Omphalos” meaning
prominence or navel.
• 1733 – James Calder (Scottish neonatal surgeon) first
described Gastroschisis.
• Derived from the Greek word “Gaster”(Gastro)
meaning belly and “schisis” meaning to tear or split
from "
4. Epidemiology
Gastroschisis
Incidence - 4 per 10,000
M:F is 1:1
• 10-15% association with
congenital anomalies such as
CHD(VSD), cleft palate and
intestinal atresia
• 40% are premature/SGA
Omphalocele
Incidence - 3 per 5,000
M:F is 1.5:1
>70% association with
congenital anomalies such
Bowel atresia, Imperforated
anus, Trisomies 13, 18, 21,
Beckwith-Wiedemann
Syndrome & Pentalogy of
Cantrell
5. Etiology
• Gastroschisis
o Congenital abdominal wall defect
towards the right side of the
umbilicus and protruded bowel is not
covered by a membrane.
o Failure of migration and fusion of the
lateral folds of the embryonic disc on
the 3rd-4th week of gestation.
o Disruption of the right
omphalomesenteric artery as midgut
returns to abdomen by the 10th week
causing ischemia of the abdominal
wall and weakness then herniation.
o Rupture of omphalocele
• Omphalocele
o Congenital abdominal wall defect with
protrusion of abdominal viscera
contained within a parietal peritoneum
and amniotic membranous sac with
Wharton’s jelly.
o Due to failure of the midgut to return
to abdomen by the 10th week of
gestation during midgut rotation.
6. Risk Factors
Omphalocele
• Increased maternal age
• Twins
• High gravida
• Consecutive children
Gastroschisis
• Young maternal age
• Low gravida
• Prematurity
• Low birth-weight
secondary to IUGR
11. Clinical Features
OMPHALOCELE
• central defect of the abdominal wall beneath the
umbilical ring.
• Defect may be 2-12 cm (Small-<5cm)(Large>8cm)
• Always covered by sac
• Sac is made of amnion, Wharton’s jelly and
peritoneum
• The umbilical cord inserts directly into the sac in an apical
or lateral position.
• Small contains intestinal loops only. Large may
involve liver, spleen and bladder, testes/ovary
• >50% have associated anomalies
GASTROSCHISIS
• Defect to the right of an intact umbilical cord allowing
extrusion of abdominal content
• Umbilical cord arises from normal place in abdominal wall
• Opening <=5 cm
• No covering sac (never has a sac )
• Evisceration usually only contains intestinal loops
• Bowels often thickened, matted and edematous
• 10-15% have associated anomalies
• 40% are premature/SGA
12. Diagnosis
• Alpha-feto-protein-synthesized in fetal liver and
excreted by fetal kidneys and crosses placenta by 12
weeks.
• Elevated maternal AFP - neural tube defects,
abdominal wall defects, duodenal or esophageal
atresia
• 40% false positive rate
• Fetal ultrasound after 14 weeks gestation is the
confirmatory test.
13. Prenatal Ultrasound
• Normal umbilical cord
insertion site
• Small bowel loops seen in the
amniotic cavity
• No covering membrane over
the loops of bowel
• Can include stomach and large
bowel
• Majority occur to the right of
the umbilical cord
Gastroschisis
14. Prenatal Ultrasound
• Umbilical cord
insertion is typically
midline on the mass
• Located centrally
• Contents are intestinal
loops and maybe liver,
spleen and gonads.
Omphalocele
16. Pre-operative Management
• ABC
• Heat Management
– Sterile wrap or sterile bowel bag
– Radiant warmer
• Fluid Management
– IV bolus 20 ml/kg LR/NS
– D10¼NS 2-3 maintenance rate
• Nutrition
– TPN (central venous line )
• Abdominal Distention
– OG/NG tube
– urinary catheter
• Infection Control
Broad-spectrum antibiotics - Ampicillin and Gentamycin
• Closure of the Defect
17. Omphalocele
• Conservative
1. Large omphalocele (10-12cm)
apply topical application -
Betadine ointment or silver
sulfadiazine to the intact sac.
2. Secondary eschar formation and
granulation.
3. Healing lasts for 12 months then
repaired as ventral hernia.
o Primary Closure
Small defects (<4cm)
excision of the sac and closure
of the fascia and skin over the
abdominal contents
o Mesh patch
Medium defects (6-8cm)
18. • Post operative care
o NICU
o Ventilation
o Feeding:
– Minimal volume
o 48 hrs Antibiotics
o Hernia dealt with at 1
yr old
19. Gastroschisis
• Primary closure
o If bowel easily reduced
• Staged closure
o Silo fashioning:
Sac excised
Silo sewn to rectus fascia/full thickness
20. • Post operative care
o NICU
o Feeding delayed for
weeks
o Oral stimulation/sucking
reflex
o Broad spectrum
antibiotics
21. Long Term Outcomes
Omphalocele
• Small - recover well
• Large:
– Gastro-oesophageal reflux - 43%
– Majority improve over time
– 20% pulmonary insufficiency
– Respiratory Infections
– Asthma
– Feeding difficulties;
• 60% with giant omphalocele
• May need gastrostomy for feeding
– Failure to thrive
Gastroschisis
• Generally excellent if no atresia
• NEC:
– 18.5% of neonates more with formula
– Bowel loss - short gut syndrome
• Cryptorchidism:
– 15-30%
– Due either being outside/prematurity
– Replacement and orchidopexy by 1 yr
• 60% have psychosocial stress if
umbilicus sacrificed
