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Published on: December 4, 2023
Outcomes in the giant omphalocele population: A single center comprehensive experience
Heather R Nolan1, Monica L Wagner1, Todd Jenkins1
1Cincinnati Children's Hospital Medical Center, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH, USA.
Insights
Outcomes for giant omphalocele depend on multiple factors, including congenital anomalies and gestational age. Primary closure in survivors led to earlier repair and shorter hospital stays, but overall treatment superiority requires further study.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Congenital Anomalies
Background:
- Giant omphalocele presents complex challenges due to large abdominal wall defects and associated physiological issues.
- Congenital anomalies significantly complicate morbidity and mortality in neonates with giant omphaloceles.
Purpose of the Study:
- To investigate the impact of different congenital anomalies and surgical treatment types on outcomes in giant omphalocele patients.
- To identify predictors of favorable versus unfavorable outcomes in this population.
Main Methods:
- Retrospective chart review of 35 giant omphalocele patients from 2009-2018, excluding specific complex cases.
- Patients were categorized into favorable or unfavorable outcomes based on mortality and operative morbidity.
- Survivors were analyzed by closure type: staged, delayed, or primary.
Main Results:
- Unfavorable outcomes were linked to major congenital anomalies, sac rupture, and cardiac anomalies.
- Increased gestational age and birth weight were associated with significantly better outcomes.
- Primary closure in survivors correlated with younger age at repair and shorter hospital/NICU stays.
Conclusions:
- Predicting giant omphalocele outcomes necessitates evaluating multiple variables, including specific anomalies and birth parameters.
- Factors like cardiac anomalies, genetic diagnosis, sac rupture, and lower birth weight increase unfavorable outcomes.
- While primary closure offers benefits in survivors, further research is needed to establish the superiority of different operative treatments.
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.

