Outcomes in the giant omphalocele population: A single center comprehensive experience

Abstract

Background/purpose: Morbidity and mortality in the giant omphalocele population is complicated by large abdominal wall defects, physiologic aberrancies, and congenital anomalies. We hypothesized different anomalies and treatment types would affect outcomes.

Methods: A 2009-2018 retrospective chart review of giant omphaloceles was performed. Exclusions included cloacal exstrophy, transfer after 3 weeks, surgery prior to transfer, conjoined twins, or not yet achieving fascial closure. Thirty-five patients met criteria and mortality and operative morbidity categorized them into favorable (n = 20) or unfavorable (n = 15) outcomes. Odds ratios analyzed potential predictors. Survivors were stratified into staged (n = 11), delayed (n = 8), and primary closure (n = 6) for subgroup analysis.

Results: Unfavorable outcomes were associated with other major congenital anomalies, sac rupture, and major cardiac anomalies, but had significantly lower odds with increasing gestational age (p = 0.03) and birth weight (p < 0.001). In survivors, the primary group was younger at repair (p < 0.001) and had shorter length of stay (hospital p = 0.02, neonatal intensive care unit p = 0.005). There was no significant difference for sepsis, ventilator days, return to the operating room, or ventral hernia.

Conclusions: Predictions of overall outcomes in the giant omphalocele population require analysis of multiple variables. Our findings demonstrated increased odds of unfavorable outcomes in major cardiac anomalies, pulmonary hypertension, genetic diagnosis, other major anomalies, polyhydramnios, postnatal sac rupture, increasing omphalocele sac diameter, lower O/E TLV, lower gestational age at birth, lower birth weight, and repair other than primary. In those surviving to repair, surgical outcomes analyses demonstrated an earlier age of repair and a shorter length of stay for those patients able to be closed primarily; however further research is necessary to determine overall superiority between operative treatment types.

Level of evidence: Level III.

Keywords: Abdominal wall defect; Delayed omphalocele closure; Giant omphalocele; Staged omphalocele closure.