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Biliary atresia: the Canadian experience
Richard A Schreiber1, Collin C Barker, Eve A Roberts
1Department of Pediatrics, University of British Columbia, Vancouver, British Columbia. rschreiber@cw.bc.ca
Insights
Outcomes for Canadian children with biliary atresia were similar across two eras, with overall 4-year survival at 81%. Early Kasai operation significantly improves native liver survival, highlighting the need for timely diagnosis and intervention in pediatric liver disease.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Surgical Outcomes
Background:
- Biliary atresia is a severe neonatal liver disease requiring timely intervention.
- Liver transplantation is a critical treatment option for end-stage biliary atresia.
Purpose of the Study:
- To evaluate the long-term outcomes of Canadian children diagnosed with biliary atresia.
- To compare outcomes between two distinct treatment eras, considering the advent of liver transplantation.
Main Methods:
- Retrospective review of health records for infants diagnosed with biliary atresia between 1985-2002.
- Analysis of patient survival, native liver survival, and liver transplantation rates.
Main Results:
- The 4-year survival rate was 81%, with no significant difference between the two study eras (ERA I: 74%, ERA II: 82%).
- Kasai operation performed at or before 30 days of age resulted in significantly higher 4-year native liver survival (49%) compared to later interventions (36-23%).
- The 4-year liver transplantation survival rate was 82%, with similar outcomes across both eras.
Conclusions:
- Outcomes for biliary atresia in Canadian children are comparable to international standards.
- Late referral for diagnosis and treatment remains a significant challenge, necessitating improved policies for early detection.
- While liver transplantation has improved survival, optimizing the timing of the Kasai operation is crucial for preserving native liver function.
Objective:
To determine the outcomes of Canadian children with biliary atresia.
Study Design:
Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed.
Results:
349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age < or = 30, 31 to 90, and > 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS).
Conclusions:
This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.