Omphalocele occurs in approximately 1 in 5,000 births, is associated with advanced maternal age and chromosomal abnormalities in 30%.
Infants with omphalocele are at risk for cardiovascular anomalies and should undergo a thorough cardiovascular examination including echocardiography.
Infants with omphalocele are at risk for Beckwith-Wiedemann syndrome (hypoglycemia, macroglossia, gigantism, risk of malignancy, omphalocele) and therefore blood glucose levels should be checked urgently and then serially.
For defects small than 5 cm in diameter, primary closure is usually possible and should be attempted if the baby is healthy and without significant associated anomalies.
For giant defects (larger than 5 cm or containing a portion of the liver), it is best to use a staged approach (amnioinversion technique): a mesh is sutured securely to the fascia, the amnion is preserved, and the fascia reapproximated every other day until the edges are approximated.
For infants who are compromised due to cardiovascular or pulmonary disease, one might consider using a delayed closure approach, in which the amnion is coated with an antiseptic and allowed to epithelialize.