Prognostic factors for the outcome of congenital omphalocele

Background: The clinical course and prognosis of children born with congenital omphaloceles (CO) is highly variable hampering clinical management. Literature is sparse regarding factors predicting outcome of CO. Methods: Analysis of retrospective data of all newborn patients with CO treated in our hospital between 1987 and 2013 (N=39). Chest configuration has been determined radiologically as previously published (Hershenson, M.B. et al. Journal of Pediatric Surgery 1985; 20:348-353). Standard logistic regression models has been used to calculate odds ratios (OR) along with 95% confidence intervals (95% CI). Death during hospitalisation has been defined as the primary outcome. Results: Seven of 39 (18.0%) children died during hospitalisation with a median survival of 27 days (range 3 to 184). Associated lung malformations (OR 4.73, 95% CI 1.63 to 16.9), Apgar score 1 minute after birth (OR 0.44, 95% CI 0.21 to 0.75), thoracic width to height ratio (OR 0.92, 95% CI 0.82 to 0.99) and a liver containing (giant) CO were associated with death during hospitalisation. An additive score derived from these variables showed a negative predictive value of 96% (95% CI 80 to 100) for the low risk group and positive predictive value of 67% (95% CI 9 to 99) for the high risk group. Conclusion: Apgar 1 minute after birth, presentation with lung malformations, the size of the CO and chest configuration are associated with death during hospitalisation in children with CO.