A Review of Covered Abdominal Wall Defects: Cord Hernias Are Associated With Major Anomalies.

INTRODUCTION: Covered abdominal wall defects (CAWD) can be categorized into giant omphaloceles (GOs), nongiant omphaloceles (NGOs), and umbilical cord hernias (UCHs). We sought to evaluate differences in management and outcomes of the different CAWD, treated at a large tertiary children's hospital, with regards to survival and association with other major congenital anomalies.

METHODS: A retrospective review of CAWD patients between January 2010 and January 2021 was conducted. GO was defined as a fascial defect >5 cm or >50% liver herniation. UCH were defined as fascial defects ≤ 2 cm. All others were classified as NGO. Type of repair, time to fascial closure, index hospitalization length of stay (LOS), and survival rates were compared. Four major anomaly categories were identified: cardiac, midline, Beckwith-Weidemann Syndrome, and other genetic anomalies.

RESULTS: We identified 105 CAWD patients (UCH n = 40; GO n = 34; and NGO n = 31). Ninety percent of UCH underwent primary repair, 10% were never repaired. NGOs were repaired by primary or staged methods in 92.9% of cases and 7.1% by delayed repair. Primary or staged repair occurred in 32.4% of GOs and delayed repair occurred in 67.6%. The median days to repair was 181 [24,427] GO, 1 [1,3] NGO, and 1 [0,1] UCHs (P < 0.01). Index hospitalization median LOS (days) was 90 [55,157] GO, 23 [10,48] NGO, 9 [5,22] UCH, (P < 0.01). There were no statistical differences in survival rates, number of patients with major anomalies (GO 35.4%, NGO 51.5%, UCH 50%), or types of anomalies.

CONCLUSIONS: UCHs and omphaloceles have similar incidences of major associated anomalies. Thus, all patients with a covered abdominal wall defect should undergo workup for associated anomalies.