P1.042: A Multi-institutional Characterization of Infants with Giant Omphalocele

Background: Omphalocele is one of the most common congenital defects of the abdominal wall. However, the overall incidence of giant omphaloceles (GO) is low and consensus is lacking on treatment. We hypothesized that there was wide variability of management of GO across the Midwestern Pediatric Surgery Consortium (MWPSC).

Methods: A multi-institutional retrospective review between 7/1/2012-5/31/2023 was performed of patients with GO (defined as fascial defect > 5 cm). Patients were identified via ICD codes and confirmed with chart review.

Results: Nine centers with 124 children were included. Center enrollment volume ranged from 2-27 (median 13.5). Two-thirds of patients (64, 69%) had additional congenital anomalies. Eleven patients (11%) had major cardiac anomalies, of which 3 had Pentalogy of Cantrell.
Most patients had an intact sac (116, 94%) with 5 grossly or partially ruptured sacs (4%) and the remaining 3 unknown. The sac contained bowel and liver in 52 (42%) patients, bowel only in 16 (13%), liver only in 18 (15%), and a combination of bowel, liver, stomach, bladder, and ovaries in the remaining patients. There was insufficient data on sac contents in 14 patients (11%). The median age at fascial closure was 6.1 months (IQR 0.87, 13.1).
Initial management data were available for 120 children (Table). A technique of paint and wait was used for 83 patients (69%), a DuoDerm silo for 12 (10%), an alternative compressive technique for 11 (9%), an operative silo for 11 (9%), and an alternative technique for the remaining 3. Age at fascial closure was lowest in the operative silo group (p = 0.003), and percentage of patients whose fascia was closed primarily was highest in the Duoderm silo group (p < 0.001).
Presence of ≥2 organs in the sac was associated with surgical repair at an older age (14.0 vs 7.9 months, p = 0.02). Presence of liver in the sac was associated with decreased likelihood of achieving primary fascial closure (62% vs 92% primary closure rate, p = 0.003). Skin was closed primarily in 102 patients (94%).
Forty-seven patients (38%) experienced complications secondary to GO reduction, most commonly respiratory (27, 22%), infectious (20, 16%), or worsening pulmonary hypertension (11, 9%). There was no difference in frequency of complication based on initial management strategy.

Conclusion: Giant omphalocele is a morbid condition, with half the infants in our cohort not achieving fascial closure until after 6 months of age. Additionally, over a third of patients faced complications related to the reduction process. Increased number of organs was associated with prolonged time to surgery and presence of liver in the sac associated with decreased likelihood of achieving primary fascial closure, indicating that these infants are particularly at risk. Further research is warranted to identify optimal management strategies for patients with GO.