Mar 27, 2011
Most common congenital Anterior abdominal wall defects are Omphalocele & Gastroschisis.Combined incidence is 1:2000.It occurs equally in males & females.
Omphalocele: Failure of lateral & caudal folds to unite (which forms anterior abdominal wall) causes intestines to remain outside the body wall.
Gastroschisis: Abnormal involution of right umbilical vein resulting in the mesenchymal defects at the junction of the body stalk & body wall.
Omphalocele: There is anterior midline defect at the base of the umbilicus through which abdominal viscera herniates into an avascular hernial sac composed of peritoneum, wharton's jelly & amnion.
Presence or absence of liver in the sac differentiates between large & small omphalocele respectively.
Gastroschisis: There is a defect in the abdominal wall either on right (commonest) or left side of umbilicus without membranous sac.
Maternal serum AFP
Amniocentesis & Chromosomal analysis
Postnatal: Clinical & Ultrasonography
1) Cantrell Pentalogy: Epigastric omphalocele, Anterior diaphragmatic hernia, Sternal cleft,
Ectopia cordis, Cardiac anomaly.
2) Beckwith Wiedemann syndrome: Exomphalos, macroglossia, gigantism
3) Trisomy syndrome
4) Prune belly Syndrome
Intestinal malrotation, Meckel's diverticulum, Treacheo-esophageal Fistula, Undescended testis,
Small bowel atresia.
Management: Conservative (omphalocele):
1) Topical application of escharotic agents
2) Delayed external compressive reduction of omphalocele
1) Primary closure of anterior abdominal wall defect
2) Staged silo closure
3) Skin flap closure( ventral hernia)
4) Repair using synthetic grafts