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Improving outcomes of biliary atresia: French national series 1986-2009
Christophe Chardot1, Chantal Buet, Marie-Odile Serinet
1Observatoire français de l'atrésie des voies biliaires, Hôpital Necker - Enfants malades, Université Paris Descartes, Paris, France. christophe.chardot@nck.aphp.fr
Insights
Biliary atresia (BA) patients in France have an 89% life expectancy, with early Kasai operation improving outcomes and reducing the need for liver transplantation (LT). About 30% of BA patients can reach adulthood without LT.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Surgical Outcomes Research
Background:
- Biliary atresia (BA) is a severe neonatal liver disease.
- Liver transplantation (LT) has become a standard treatment for end-stage BA.
- Assessing BA prognosis in the LT era is crucial for understanding outcomes.
Purpose of the Study:
- To analyze the long-term prognosis of biliary atresia (BA) in France.
- To evaluate the impact of liver transplantation (LT) availability on BA patient survival.
- To determine factors influencing survival with native liver (SNL) and post-LT outcomes.
Main Methods:
- Retrospective chart review of 1107 BA patients born in France between 1986-2009.
- Patients categorized into three birth cohorts (1986-1996, 1997-2002, 2003-2009).
- Analysis of Kasai operation, jaundice clearance, survival with native liver (SNL), LT rates, and overall survival.
Main Results:
- Overall 5-year survival for BA patients improved significantly across cohorts (72% to 89%).
- Survival with native liver (SNL) at 20 years was 30%, influenced by early Kasai operation (<3 months).
- Post-LT survival at 5 years improved from 75% to 89% across cohorts, with decreased mortality without LT.
Conclusions:
- Biliary atresia patients in France now have an 89% life expectancy.
- Approximately 30% of BA patients can reach adulthood without requiring liver transplantation.
- Early Kasai operation, regardless of age, is key to reducing the need for LT and improving long-term outcomes.
Background & Aims:
This study analyses the prognosis of biliary atresia (BA) in France since liver transplantation (LT) became widely available.
Methods:
The charts of all BA patients living in France and born between 1986 and 2009 were reviewed. Patients were divided into 3 cohorts according to their years of birth: 1986-1996, 1997-2002, and 2003-2009.
Results:
1107 BA children were identified, 990 born in metropolitan France (incidence 1/18,400 live births). Kasai operation was performed in 1044 (94%), leading to complete clearance of jaundice (total serum bilirubin ≤ 20 μmol/L) in 38% of patients. Survival with native liver (SNL) after Kasai operation was 40%, 36%, and 30% at 5, 10, and 20 years, stable in the 3 cohorts. Median age at Kasai operation was 59 days, unchanged over time. Twenty-year SNL was 39%, 32%, 28%, and 19% after Kasai operation performed in the first, second, third months of life or thereafter (p=0.0002). 588 children underwent 692 LTs. Mortality without transplantation decreased over time: 16%, 7%, and 4% in the 3 cohorts (p<0.0001). Survival after transplantation was 83%, 82%, and 77% at 5, 10, and 20 years in the whole series. Five-year post-transplant survival was 75%, 90%, and 89% in the 3 cohorts (p<0.0001). In the whole series, overall BA patient survival was 81%, 80%, and 77% at 5, 10, and 20 years. Five-year BA patient overall survival increased over time: 72%, 88%, and 89% in the 3 cohorts (p<0.0001).
Conclusions:
BA patients currently have an 89% live expectancy, and a 30% chance to reach adulthood without transplantation. Early Kasai operation, without age threshold, reduces the need for liver transplantation until adulthood.