Exomphalos (syn. Omphalocele): A Review of 45 Cases

Abstract

A retrospective study of the case notes of 45 infants with exomphalos had led to the arrangement of those in which the sac was intact into 3 groups according to the size of the exomphalos. Two additional groups were distinguished by reason of rupture of the sac or because the exomphalos formed part of a complex anomaly vesico-intestinal fissure. The survival rate was best when the exomphalos was small or medium-sized, and when the liver was not present in the sac. In such infants a primary repair of the abdominal wall was usually possible and the result satisfactory. Additional serious malformations, chiefly cardiac, were present in 35 of the 45 infants, and they were the direct or indirect cause of death in 21 (approximately 43%). Less serious associated malformations were found in 14 infants. Malrotation and malfixation were recorded in only 8 infants, but mid-gut volvulus and/or duodenal obstruction did not occur in any patient. A diaphragmatic hernia or eventration was found in 3 infants and the question is raised whether it is advisable to explore the abdomen for remediable conditions rather than close skin over the intact sac, which is desirable when a 2-stage repair is contemplated. There were only 3 survivors in a group of 13 infants in whom the abdominal defect exceeded 5 cm in diameter; in 11 instances the sac contained part of the liver. In 2 of the 3 survivors the skin alone was closed at the first operation and repair was satisfactorily completed in 2 stages. A study of the 10 deaths in this group showed that cardiac malformations were responsible for 6, and in retrospect there seems to be a place for a non-operative method of treatment in this type of patient especially when born prematurely and when pulmonary atelectasis is present. Factors considered to determine the outcome in an infant with exomphalos are the size of sac and the defect in the abdominal wall, and the presence of the liver within it; pre-partum rupture of the sac; coexistent malformations; prematurity; and early operation.