Systematic review of mortality associated with neonatal primary staged closure of giant omphalocele.

INTRODUCTION

Surgical management of giant omphalocele has evolved at a slow pace, but evidence on the survival of patients who underwent primary staged closure is scattered and atomized.

OBJECTIVE

To analyze the studies about of mortality associated with neonatal primary staged closure of giant omphalocele.

METHODS

Systematic review in three databases using ex-ante search protocol and selection of studies following the phases suggested by PRISMA and MOOSE criteria. Reproducibility and evaluation of methodological quality were guaranteed by using CARE and STROBE.

RESULTS

Seven studies of clinical cases with nine patients, and six cross-sectional studies with 85 individuals were analyzed. These were conducted in the USA mainly, between 1985 and 2018. In the case studies, the death was 11.1% owing to hepatic necrosis and portal system angiomatosis. On the cross-sectional studies, mortality was registered in 18.8% of patients owing to coarctation of the aorta, heart, kidney, intestinal, respiratory or multiple organ failure, an anomaly of venous return, prematurity, ruptured omphalocele, pulmonary hypoplasia, trisomy 13, ARDS, sepsis, and septic shock. The main complication was wound infection with subsequent confection of the silo, found in 5.4% of patients.

CONCLUSION

Only a few studies on staged closure of giant omphalocele were found on a low number of patients. The high survival rate and the low percentage of complications on the 94 analyzed patients suggest the effectiveness and safety of the procedure.

LEVELS OF EVIDENCE

According to the Journal of Pediatric Surgery this research corresponds to type of study level II for retrospective studies, and level IV for case series with no comparison group.