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Biliary atresia: the King's College Hospital experience (1974-1995)
M Davenport1, N Kerkar, G Mieli-Vergani
1Department of Paediatric Surgery, King's College Hospital, London, England.
Insights
Portoenterostomy surgery offers long-term success for about half of infants with biliary atresia. Early surgery age doesn't significantly impact survival, suggesting it shouldn't dictate treatment decisions.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Hepatology
Background:
- Biliary atresia is a severe neonatal liver disease requiring surgical intervention.
- Early diagnosis and treatment are crucial for improving outcomes in infants with biliary atresia.
- Portoenterostomy is a primary surgical procedure for biliary atresia.
Purpose of the Study:
- To analyze the long-term survival outcomes of infants with biliary atresia treated with portoenterostomy.
- To evaluate the impact of age at surgery on survival rates in biliary atresia.
- To assess the effectiveness of portoenterostomy as a long-term treatment for biliary atresia.
Main Methods:
- Retrospective analysis of 338 infants with biliary atresia treated between 1973 and 1995.
- Infants were stratified into three groups based on birth decade (1970s, 1980s, 1990s).
- Surgical outcomes including survival, need for liver transplantation, and age at surgery were analyzed.
Main Results:
- Overall survival at last follow-up was 50%, with 23% undergoing liver transplantation and 26% mortality.
- Five-year actuarial survival improved from the 1980s (50%) to the 1990s (60%).
- A significant decrease in median age at surgery was observed over the decades, but age at surgery did not significantly predict 5-year outcomes for infants treated since 1980.
Conclusions:
- Portoenterostomy is an effective long-term procedure for 40-50% of infants with biliary atresia.
- The remaining 50-60% of infants will likely require liver transplantation, often within 2 years of age.
- Age at surgery has limited predictive value for survival after portoenterostomy and should not dictate primary treatment.
Unlabelled:
The survival experience of 338 infants born with biliary atresia between January 1973 and December 1995 was analyzed. All the infants had their initial surgery at a single UK centre. These infants were divided into three groups based on year of birth; group 1 (1970s, n = 38); group 2 (1980s, n = 182), and group 3 (1990s, n = 118). The data from group 1 were incomplete and selected, and comparisons with the remaining groups were therefore restricted. However, all infants who had been treated since 1980 underwent portoenterostomy or hepaticojejunostomy and were included.
Results:
In the whole cohort there were 89 deaths (26%), 79 children (23%) who underwent liver transplantation and 170 children (50%) who were alive at last follow-up. The 5- and 10-year actuarial survival for group 2 was 50% and 41%, respectively and the 5-year actuarial survival for group 3 was 60%. Overall, 57 children have survived to 10 years after surgery for biliary atresia. There has been a progressive fall in the age at surgery from a median of 77 days in group 1, through 69 days in group 2 to 56 days in group 3 (P < .0001). However, there was no significant difference in outcome to 5 years between the age cohorts (< 40 days, 41 to 60 days, 61 to 99 days, and > or = 100 days; P > .1) for the infants treated since 1980 (n = 200).
Conclusions:
Portoenterostomy is an effective long-term procedure for biliary atresia in about 40% to 50% of infants. The remaining 50% to 60% will require transplantation mostly within 2 years of age, although there is also a continuing need beyond 5 and 10 years. The age at surgery has limited usefulness as a predictor of survival after portoenterostomy and certainly should not be used to dictate primary treatment.