Thirty-four years' experience with biliary atresia in Denmark: a single center study

Area of Science:

• Pediatric Surgery
• Hepatology
• Public Health

Background:

• Biliary atresia (BA) is a rare pediatric liver disease.
• Management strategies in small countries are debated.
• Centralizing BA care may optimize outcomes.

Purpose of the Study:

• To evaluate the efficacy of Kasai portoenterostomy (KP) for biliary atresia.
• To assess outcomes over a prolonged period with centralized care in Denmark.
• To determine the impact of a single referral center on BA patient results.

Main Methods:

• Retrospective cohort study.
• Analysis of 89 infants with BA referred to a national center (1976-2010).
• Evaluation of jaundice resolution, native liver survival, and liver transplant (LT) outcomes.

Main Results:

• Median age at KP was 59 days; 12% were over 100 days.
• 53% achieved jaundice resolution within 6 months post-KP.
• Jaundice resolution rates significantly increased over time (79% in the latest period).
• 58% of patients were alive at follow-up (median age 6.8 years).
• 39% had native livers, 18% survived following LT.
• 5- and 10-year native liver survival rates were 44% and 37%.

Conclusions:

• Centralizing pediatric surgical expertise, even in small countries, improves biliary atresia outcomes.
• Kasai portoenterostomy remains an effective primary surgical treatment for BA.
• Concentrated patient populations enhance surgical success and long-term survival.