Biliary atresia: the Brazilian experience

Objective: to evaluate epidemiological,clinical and prognostic characteristics of children with biliary atresia. methods: data regarding portoenterostomy,liver transplantation (ltx),age at last follow-up and survival were collected from the records of patients followed up in six brazilian centers (1982-2008) and compared regarding decades of surgery. results: of 513 patients,76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent ltx. in 69% of cases,ltx followed portoenterostomy,whereas in 31% of cases ltx was performed as the primary surgery. patients from the northeast region underwent portoenterostomy later than infants from southern (p = 0.008) and southeastern (p = 0.0012) brazil,although even in the latter two regions age at portoenterostomy was higher than desirable. over the decades,ltx was increasingly performed. overall survival was 67.6%. survival increased over the decades (1980s vs. 1990s,p = 0.002; 1980s vs. 2000s,p < 0.001; 1990s vs. 2000s,p < 0.001). the 4-year post-portoenterostomy survival,with or without ltx,was 73.4%,inversely correlated with age at portoenterostomy (80,77.7,60.5% for ≤ 60,61-90,> 90 days,respectively). higher survival rates were observed among transplanted patients (88.3%). the 4-year native liver survival was 36.8%,inversely correlated with age at portoenterostomy (54,33.3,26.6% for ≤ 60,61-90,> 90 days,respectively). conclusions: this multicenter study showed that late referral for biliary atresia is still a problem in brazil,affecting patient survival. strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life. copyright © 2010 by sociedade brasileira de pediatria.