Outcome of biliary atresia after kasai portoenterostomy at a single italian center (university of Padova, 1990–2011).

Background: Biliary Atresia (BA) is worldwide the leading cause of end-stage liver disease in children and the most common indication for pediatric liver transplantation (LT). National studies from several European, Asiatic and North American countries report a 48% median success rate (range 30−61%) of Kasai Portoenterostomy (KPE) and a median survival with native liver (SNL) of 37.9% (range 31−53) at 10 years after KPE. Young age at diagnosis (<45 days) and center experience (>5 cases/year) are the main prognostic factors associated to KPE success. No published data are available concerning BA epidemiology and outcome in Italy. Objective: The aim of this study is to describe the outcome of children with BA treated at a single University Center of Pediatric Hepatology and LT in Italy. Methods: Health records of all cases of BA diagnosed at the Department of Pediatrics of the University of Padova between January 1990 and December 2011 were retrospectively reviewed. Results: A total of 52 children were diagnosed with BA during the study period (mean 2.5/year) of which 20 in the last 5 years (mean 4/year). 51 patients underwent KPE at a median age of 66 days (range 19–115); a significant difference (p<0.001, unpaired t test) was observed concerning the mean age at KPE between children diagnosed during the 1990s (mean 49±5 days) and after 2000 (mean 72±3 days). Median age at last follow up was 5.6 years (0.7−22 years). KPE success (i.e. total bilirubin <20 umol/L at 6 months after KPE) was 31%. Among patients with unsuccessful KPE (n = 34): 64% underwent LT at a median age of 1.8 years (main indications consisted in end-stage liver disease, hepato-pulmonary syndrome/HPS, hepatocellular carcinoma); 8% died on the waiting list for transplant at a median age of 10 months (all due to uncontrolled variceal bleeding); 14.7% have compensated cirrhosis and are still on follow up; one patient is on the waiting list for liver transplantation and one was lost at follow up. Among children with successful KPE (n = 15): 4 have no clinical or biochemical signs of liver disease; 4 have signs of portal hypertension; 4 show signs of compensated chronic liver disease; 1 was transplanted for HPS at 9.5 years of age; 1 died for reasons unrelated to BA; one was lost at follow up. Data of two patients were untraceable. Overall the rate of NLS at 10 years after KPE was 38%. Conclusions: Despite advanced age at KPE and relatively low numbers of new cases per year, both the success rate of KPE and the 10 years SNL after this intervention are comparable with current international standards. As demonstrated in other countries, centralization of surgical care and the launch of a screening program to anticipate age at referral have the potential to further improve the outcome of children after KPE. The time to begin an Italian National Program for the management of BA has definitely come.