Biliary Atresia in The Netherlands: Outcome of Patients Diagnosed between 1987 and 2008

Objective To examine the outcome of biliary atresia (BA) and to identify prognostic factors using a national database.

Study design All children born between January 1987 and December 2008 who underwent the Kasai surgical procedure for BA were retrieved from the Netherlands Study Group on Biliary Atresia Registry database. Outcomes were measured in terms of clearance of jaundice (bilirubin

Results Between January 1987 and December 2008, 214 patients underwent Kasai surgery for BA. In this series, the 4-year transplant-free survival was 46% +/- 4%, and 4-year overall survival was 73% +/- 3%. Clearance of jaundice, surgery within 60 days, and postoperative antibiotic prophylaxis use were independently associated with increased transplant-free survival. The yearly caseload per center (range, 0.5-2.1) was not correlated with transplant-free survival (r = 0.024; P = .73).

Conclusion During the past 2 decades, outcome parameters have remained constant and are comparable with those reported from other Western countries, despite a relatively low annual caseload per center. Timely surgical correction and postoperative antibiotic therapy were associated with a higher transplant-free survival rate. (J Pediatr 2012;160:638-44).