Omphalocele and Other Congenital Abdominal Wall Defects

Posted by e-Medical PPT Saturday, August 3, 2013
CASE PRESENTATION
Baby – male, 4000gm, 39 week gestation
Mother – 26 yo G4P2 with inadequate prenatal care at an outside institute
Prenatal sonogram suggestive of omphalocele. s/p amniocentesis- normal karyotyping
Normal vaginal delivery

Soon after the birth
No acute distress
Macroglossia, macrosomia, ear creases
3.5 cm defect in abdominal wall at the umbilicus with bowel covered by a sac
Labs – Hypoglycemic FSG 30 mg/dL

Abdominal wall defects – first century AD by Aulus Cornelius Celsus, fifth century by Paulus Aegineta

Omphalocele
16th century by Ambrose Par
First successful repair by Hey in 1802
Stage reduction with prosthetic material by Schuster in 1967

Gastroschisis
16th century by Lycosthenes
Confused with ruptured omphalocele
Moore and Stokes described it as a separate entity
First successful repair by Visick in 1873

INCIDENCE
1 in 2000 live births
Omphalocele – 1-2.5 per 5000 births, M > F
Gastroschisis – 1 in 4000 births
Increased detection with increasing antenatal U/S, ms AFP testing

EMBRYOGENESIS OF DEFECTS
OMPHALOCELE
Failure of extra embryonic gut to return into abdominal cavity – simple omphalocele
Failure of lateral body folds to migrate medially – larger defect with liver
Covered by 2 layer amniotic peritoneal membrane
Defect at the umbilicus
Occurs early in embryogenesis

GASTROSCHISIS
In-utero vascular accident
Right umbilical vein involution  necrosis in abdominal wall defect
Premature right vitelline artery involution  weakening of abdominal wall rupture a/w intestinal atresia
Not covered by membranes
Defect to the right of the umbilicus
Intestinal damage later in pregnancy..
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