Omphalocele

  • midline anterior abdominal wall defect
  • solid viscera or bowel may herniate
  • extruded contents covered in a sac
  • large lesions: due to complete failure of fusion of lateral body folds
  • bowel only lesions: due to persistence of physiological herniation of gut after 10th week of fetal development
  • umbilical cord inserts at tip of defect
  • giant omphalocele: > 5 cm diameter or when liver is present
  • detected in second trimester
  • associated chromosomal and structural abnormalities
  • Beckwith-Wiedemann syndrome: omphalocele, macroglossia, gigantism
  • malrotation inevitable