Original article: Exomphalos defects : A review of 15 cases

Abstract

Background : The outcome after repair of exomphalos defects has improved significantly with refinements in surgical techniques, multidisciplinary management and monitoring of intra-abdominal pressures. Methods : A retrospective study of medical records of 15 cases with omphalocele was done. Results : Antenatal diagnosis was available in six cases. There were eight females and seven males with a mean birth weight of 2.2 kg. Nine babies had associated anomalies. There were nine major (defect size > 5 cm) and six minor defects. Immediate closure in neonatal period was carried out in 12 cases. Urinary bladder pressure (UBP) was measured to assess intra-abdominal pressure in cases where primary closure was difficult. Primary closure was omitted in the event of intravesical pressures exceeding 20 mm Hg (∼ 25 cms of water). Primary surgical closure was possible in five (56%) major cases. Two cases were subjected to silo repair followed by delayed primary closure whereas the other two required a Goretex mesh closure. Three minor defects could be repaired primarily whereas the remaining three were managed conservatively and closed at age of 9 to 12 months. There were no significant anaesthetic complications. Elective postoperative ventilation was required in one baby. There were three deaths at ages five, nine and ten months due to unrelated causes. Conclusion : Satisfactory outcome is possible in cases with exomphalos defects with intra-operative intravesical pressure assessment forming a convenient method for excluding abdominal compartment syndrome.