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Abdominal Wall Defects: Priscilla Joe, MD Children's Hospital and Research Center at Oakland
Abdominal Wall Defects: Priscilla Joe, MD Children's Hospital and Research Center at Oakland
Priscilla Joe, MD
Childrens Hospital and Research Center at
Oakland
Omphalocele
Membrane sac arising from the umbilical cord covers
intestines
Outer membrane layer consists of amnion and inner
lining of peritoneum
Size ranging from small->giant defects containing
liver, small and large bowel, stomach, spleen,
ovaries, and testes
Associated with foreshortened bowel and malrotation
Small abdominal cavity and pulmonary hypoplasia
Gastroschisis
No membrane covering
Abdominal wall defect typically 2-4cm diameter
Lateral to the right side of the umbilical cord
Usually contains midgut and stomach
Thickened, atretic, and possibly ischemic bowel
Associated with malrotation
Embryology of Gastroschisis
Failure of vascularization of the abdominal wall
due to abnormal involution of the right umbilical
vein or a vascular accident of omphalomesenteric
artery causes abdominal wall weakness and
subsequent rupture
Rupture of a small omphalocele with absorption of
the sac and growth of a skin bridge between the
abdominal wall defect and umbilical cord
Embryology of Omphalocele
Normally, midgut returns to the abdomen
by 10th week of gestation
Somatic layers of cephalic, caudal, and
lateral folds join to close abdominal wall
With omphalocele, folds fail to close
Gastroschisis
Increasing incidence
Associated with young maternal age and
low gravida
Associated with prematurity and low birth
weights
Omphalocele
imperforate anus,
Beckwith-Wiedemann
Pentalogy of Cantrell
Diagnosis
AFP synthesized in fetal liver and excreted by
fetal kidneys and crosses placenta by 12 weeks
Elevated maternal MSAFP in neural tube defects,
abdominal wall defects, duodenal or esophageal
atresia
40% false positive rate
Fetal ultrasound after 14 weeks gestation
Amniocentesis and fetal echocardiography
Treatment
Surgical Management
Operative repair within 2-4 hours of birth
Primary closure for smaller defects
Delayed primary closure for large defects
Avoid compromised ventilation and abdominal
compartment syndrome
Use of silo with sequential reduction of
abdominal contents
Later fascial closure
Mortality/Morbidity