The management of exomphalos.

BACKGROUND/PURPOSE: The management of exomphalos in the authors' department over a 26-year period is reported together with a technique for delayed closure of the ventral hernia resulting from conservative treatment of exomphalos major.

METHODS: Patients were classified into exomphalos minor and major. Exomphalos minor was treated by early surgical closure. Exomphalos major was treated preferentially conservatively with delayed repair of the ventral hernia.

RESULTS: There were 104 patients (68 boys and 36 girls; exomphalos minor, 45; exomphalos major, 59). Forty-two patients with exomphalos minor underwent operation. Three patients died before surgery, and 9 others postoperatively of overwhelming sepsis. Fifteen babies with exomphalos major needed early operation (skin closure only in 3 and prolene mesh repair in 12), there were 2 preoperative and 4 postoperative deaths. Forty-two patients were treated conservatively, among these, 8 died of sepsis. Thirty-four children had closure of the ventral hernia (prolene mesh, 7 and native tissue, 27); there was no morbidity. Two children died after laparotomy for adhesive intestinal obstruction.

CONCLUSION: Mortality rate was related to sepsis, complications of delayed presentation, and severe congenital anomalies. There were no ill effects attributable to mercury or iodine absorption. Delayed ventral hernia repair by double breasting of the fibrous tissue sheet underlying the skin was found to be a reliable technique with low morbidity.