Omphalocele and Beckwith-Wiedemann Syndrome

This chapter discusses omphalocele, which is a neonatal abdominal wall defect through which abdominal visceral content herniates. Although omphalocele is similar in gross appearance to gastroschisis, it is more frequently associated with chromosomal abnormalities such as trisomies 13, 15, 18, and 21, and syndromes such as Beckwith-Wiedemann, Rieger’s, and prune belly syndrome. It is also more frequently associated with anomalies in other organ systems. Management of infants with Beckwith-Wiedemann syndrome and omphalocele can be challenging due to the concurrent risk of difficult airway and aspiration. Associated comorbidities also need to be addressed to avoid secondary insults in the perioperative period.