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- 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