Exomphalos major: the Northern Ireland experience

Abstract

In exomphalos major (EM), closure of the defect in the abdominal wall presents a challenge. The aim of this study is to evaluate a single centre experience of EM. A 15-year retrospective case-note review; data presented as median (range). Fourteen infants (7 female) were born with EM: birth weight 2.9 (1.2-3.8) kg, gestational age 38 (31-39) weeks. One infant died in utero and one within the first hour of life. Severe pulmonary hypoplasia was present in 7/13 (54%), and there was a mortality of 6/13 (46%) live births. Infants were treated non-operatively primarily. Two infants underwent early surgery: one infant, born with a ruptured sac, had a surgical silo constructed on day 1 and closure on day 8, while a second infant had partial closure (skin only) on day 11. Ten infants had application of silver sulphadiazine to the sac 2-3 times per week. Enteral feeds were established soon after birth. They were discharged from hospital to allow granulation. Ventral hernia closure was performed on a subsequent admission. Exomphalos major can be successfully treated non-operatively, allowing immediate enteral feeding and early discharge while granulation takes place. In this series, exomphalos major has an incidence of 1 in 26,000, mortality is 46% and severe pulmonary hypoplasia is present in 54% of infants.