Severity of Ascites Is Associated with Increased Mortality in Patients with Cirrhosis Secondary to Biliary Atresia

Background Very few prior studies have investigated the presence of ascites as a prognostic factor in children with cirrhosis. To the best of our knowledge, there are no prior studies evaluating the relationship between severity of ascites and patient survival in children with biliary atresia and cirrhosis. Aims To evaluate the association between severity of ascites and survival of children with cirrhosis and biliary atresia. Methods All children with cirrhosis secondary to biliary atresia evaluated at our institution from 2000 to 2014 were included in this study. Patients were classified into four groups: NA = no ascites; A1 = grade 1 ascites; A2 = grade 2 ascites; and A3 = grade 3 ascites. The primary endpoint of the study was mortality within the first year after patient inclusion. Ninety-day mortality was also evaluated. Prognostic factors related to both endpoints also were studied. Results One-year patient survival for NA was 97.1%, versus 80.8% for A1, versus 52% for A2, versus 13.6 for A3 ( p < 0.001). The presence of ascites increased mortality by 17 times. In the multivariate analysis, clinically detectable ascites (HR 3.14, 95% CI 1.14–8.60, p = 0.026), lower sodium (HR 1.15, 95% CI 1.04–1.27, p = 0.006), higher bilirubin (HR 1.06, 95% CI 1.00–1.12, p = 0.023), and higher PELD score (HR 1.05, 95% CI 1.02–1.08, p = 0.001) were all associated with decreased survival. Lower serum sodium (HR 1.20, 95% CI 1.09–1.32, p < 0.001) and higher PELD score (HR 1.03, 95% CI 1.001–1.063, p = 0.043) were associated with increased 90-day mortality. Conclusions Clinically detectable ascites is associated with decreased 1-year survival of children with biliary atresia. These patients should be treated with caution and prioritized for liver transplantation.