Biliary atresia (BA) is a rare disease in Denmark (population ~?5.5 million) and there has been some controversy on how smaller countries should manage such conditions to obtain the best possible outcomes. The aim of this study was to evaluate the efficacy of primary surgery (i.?e., Kasai portoenterostomy [KP]) over a prolonged period of time, during which one center has become the primary referral unit for the entire country.
A retrospective cohort study was carried out.
During the period 1976-2010, 89 infants were referred to Rigshospitalet, Copenhagen. The median age at KP was 59 days, with 11 (12%) older than 100 days. Overall, 47 (53%) resolved their jaundice and achieved normal bilirubin levels within 6 months of KP. There was a significant increase in resolution of jaundice over time (X2=8.8; p=0.03) with a rate of 79% in the latest period. Overall, 52 are still alive (58%); 37 (39%) with their native liver at a median age of 6.8 years (2 months - 34 years), and 15 (18%) are alive following LT with a median age of 12 years (33 months - 23 years). The 5- and 10-year native liver survival rates are 44 and 37%, respectively.
The overall results of this series reinforce our impression that even (or perhaps especially) in a small country, the same principles of concentrating patients and expertise will lead to better results.