Abdominal Wall Defects:

Omphalocele vs. Gastroschisis

Joanna Thomson, MS3
Surgery Clerkship
March 13, 2007
 Embryology Review
 The Midgut gives rise to:
– Duodenum distal to the bile duct
– Jejunum
– Ileum
– Cecum
– Appendix
– Ascending colon
– Hepatic flexure of the colon
– Proximal two-thirds of transverse colon.
 Week 6
Physiological Umbilical Herniation
 As a result of rapid growth and
expansion of the liver, the
abdominal cavity temporarily
becomes too small to contain all
the intestinal loops.

 The intestinal loops enter the

extraembyronic cavity within the
umbilical cord during the sixth
week of development.

 As herniation occurs, the loop

undergoes a 90 degree
counterclockwise rotation around
the superior mesenteric artery.

Source: Langman’s Medical Embryology. Ninth Edition.

 Week 10
Return to Abdominal Cavity
 During 10th week of development,
herniated intestinal loops begin to
return to the abdominal cavity.

 Undergoes additional 180 degree

counterclockwise rotation about the
superior mesenteric artery.

 Factors responsible for this return

are not precisely known... It is
thought that regression of the
mesonephros (kidney), reduced
growth of the liver, and expansion
of the abdominal cavity all play
roles.

Source: Langman’s Medical Embryology. Ninth Edition.

 Omphalocele
 Herniation of abdominal viscera through an enlarged
umbilical ring.
– Failure of the bowel to return to the body cavity following
physiological umbilical herniation. Defective mesodermal growth
causes incomplete central fusion and persistent herniation of the
midgut.

 Extruded viscera may include LIVER, small and large

intestines, stomach, spleen, or bladder.

 Covered by amnion and peritoneum

– (May rupture before or at time of delivery)
Source: “Omphalocele and Gastroschisis.” eMedicine.
 Gastroschisis
 Herniation of intestinal loops through the anterior
abdominal wall.

 Defect lateral to the umbilicus (right>left)

– Abnormal involution of the right umbilical vein or vascular
accident involving the omphalomesenteric artery causes
localized abdominal wall weakness.

 No sac covers the extruded viscera.

– Having been bathed in the amniotic fluid and with compression
of the mesenteric blood supply at the abdominal defect, the
bowel wall may be inflammed and edematous. May appear to
have a thick, shaggy membrane and loops may appear
shortened and matted together.
Source: “Omphalocele and Gastroschisis.” eMedicine.
 Prenatal Diagnosis
 Elevated maternal serum alpha fetoprotein
 Ultrasound
Omphalocele Gastroschisis

Source: “Fetal Diagnosis and Treatment.” The Children’s Hospital of Philadelphia.

 Epidemiology
 Prevalence:
– Omphalocele: 1/5,000 births
– Gastroschisis: 1/10,000 births
 Increasing in frequency, especially in young women.

 Mortality:
– Omphalocele: 25%
 Related directly to presence of chromosomal and other
abnormalities
– Gastroschisis: <5%

Source: Langman’s Medical Embryology, Ninth Edition.

Omphalocele Associated Anomalies
 Chromosomal abnormalities (50%)
– Trisomies 13, 18, 21

 Congenital heart disease (50%)

 Neural tube defects (40%)
 Beckwith-Wiedemann syndrome
– LGA, hyperinsulinism, visceromegaly of kidneys, adrenal glands and pancreas,
macroglossia, hepatorenal tumors, cloacal extrophy

 Pentalogy of Cantrell
– omphalocele, ectopia cordis, anterior diaphragmatic hernia, intracardiac defect,
sternal cleft

Source: Langman’s Medical Embryology, Ninth Edition.

Gastroschisis Associated Anomalies
 Additional gastrointestinal problems (25%)
– Including atresia, volvulus, stenosis
– Loss of bowel secondary to ischemia
 Compromised bowel function

Source: Langman’s Medical Embryology, Ninth Edition.

 Initial Management
 Acute management aimed at maintaining
circulation to bowel and preventing infection while
stabilizing infant (temperature/fluids) :

– Cover the defect with sterile dressing soaked in warm

saline to prevent fluid loss

– Nasogastric decompression

– IV fluids with glucose

– Antibiotics
 Surgical Treatment
 Surgery performed to return the viscera to the
abdominal cavity and close the defect.

– Primary Surgical Closure: Success dependent on size

of the defect and size of the abdominal and thoracic
cavities.

– Staged Closure: Gradual reduction of the contents

into the abdominal cavity using an extra-abdominal
extension of the peritoneal cavity (termed a silo) and
using gentle pressure. Usually requires 1-3 weeks,
after which the defect is then primarily closed.
Source: “Omphalocele and Gastroschisis.” eMedicine.