Exomphalos major: the Northern Ireland experience

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.