Predictors of mortality in neonates with giant omphaloceles.

INTRODUCTION: This analysis performed a review of giant-omphaloceles to determine the predictors of mortality. EVIDENCE ACQUISITION: PubMed and KoBson databases were searched for terms "giant," "omphalocele," and "mortality." Primary end points included mortality correlation with gestational age (GA), birth weight (BW), eviscerated organs, associated anomalies and management. To calculate mean and median values IBM SPSS v. 23.0 was used. EVIDENCE SYNTHESIS: After de-duplication and review search revealed 42 articles of which 23 met the inclusion criteria with 396 giant-omphaloceles for this analysis. Median gestational age (GA) was 36 weeks for all neonates (range 21-41); 21 neonates were reported as premature with median GA 33.5 (range 21-36). Overall median birth weight (BW) was 3100 g (range 1100-4100 g). The diameter of abdominal wall defect was 4-15 cm with the average size of 7.6 cm except for non-giant giant omphaloceles (N.=7) where the defect was measuring between 2.7 and 4 cm. Amniotic sac contents beside intestines included liver (N.=154), stomach (N.=11), spleen (N.=2), pancreas (N.=1), gallbladder (N.=5), and 5 giant omphaloceles were reported to contain only liver; sac was ruptured in 22. Giant omphaloceles were associated with a variety of other anomalies, most often with cardiac anomalies (N.=93; 23.4%) and pulmonary hypoplasia and/or pulmonary hypertension (N.=39; 9.8%). Management included conservative treatment N.=264 (66.6%), primary closure (N.=17; 4.3%), staged closures (N.=98; 24.7%) primary or staged closure (N.=17; 4.3%). The most frequent complication was sepsis (N.=52). There were 90 (22.7%) lethal outcomes, 6 lethal outcomes in neonates even before final closure could be achieved and 12 in prematures. Leading cause of mortality was sepsis (N.=51; 56.6%), the cause of lethal outcome was not reported in 8 cases. CONCLUSIONS: Giant-omphaloceles have a lethal outcome in one-fifth of neonates. Predictors of mortality included pulmonary hypoplasia and respiratory failure with prematurity and ruptured sacs implicated within this group. Sepsis was the independent iatrogenic factor in mortality.