The problem of "giant" omphalocele

"Giant" omphalocele implies an abdominal wall defect that is 5 cm or more in diameter with the liver in a central position. Giant omphalocele is often associated with other significant anomalies. Technically it is often difficult to close the abdominal wall defect and a staged repair utilizing prosthetic materials may be necessary. The morbidity and mortality associated with this entity remain significant despite advances in management techniques. Hospitalization is often prolonged and costly. For the best outcome in managing patients with giant omphalocele early attention to hypothermia and other metabolic requirements and long-term attention to nutritional needs are important. Techniques of closing the omphalocele should be adapted to the individual characteristics of the defect, but mobilization and stretching of the abdominal muscles should be begun as a newborn. The term hepatomphalocele implying "liver-containing omphalocele" is suggested to refer to this particular entity.