Biliary atresia: we still operate too late

Abstract

To analyze the age at surgery for children with biliary atresia and their survival periods without need for liver transplantation. The study was performed at Hospital de Clínicas de Porto Alegre, in Porto Alegre, state of Rio Grande do Sul, Brazil. The medical records of patients operated between 1982 and 2007 who were residents of the state of Rio Grande do Sul were reviewed. Of the 112 cases of children with biliary atresia studied, 38 (33.9%) occurred between 1982 and 1989, 46 (41.1%) between 1990 and 1999 and 28 (25.0%) after 2000. Portoenterostomy was not performed for 12 cases (10.7%). Age at surgery ranged from 25 to 297 days (median: 80.5; IQR25-75: 61.3-109.0 days); for 20.5% of cases, the age was below 60 days. There was no age difference at diagnosis for the three decades in the study. Patients from the countryside (median: 87.0; IQR25-75: 69.0-115.0 days) were referred significantly later (p = 0.007) than those living in Porto Alegre and the metropolitan area (median: 68.0; IQR25-75: 55.5-98.0 days). The ratio of patients younger than 60 days was significantly lower (p = 0.013) for those from the countryside. Survival periods with native liver for all patients was 46.2% in 2 years, falling continuously until reaching 15.3% in 20 years. Patients operated before reaching 60 days of age had longer survival periods with native livers (log rank < 0.0001). Late performance of portoenterostomy was a constant in the past 25 years, and this delay led to shorter survival periods with native livers for biliary atresia patients.