Biliary atresia: the King's College Hospital experience (1974-1995)

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.