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Surgical experience in children with biliary atresia treated with portoenterostomy
1Department of Pediatric Surgery, Ruhr-University of Bochum, University of Bochum, Germany. sbittmann@web.de
Insights
Portoenterostomy surgery offers a 68% 5-year survival for biliary atresia patients. Early cirrhosis presence indicates a poorer prognosis, suggesting potential need for timely liver transplantation.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia is a severe fibrosing inflammatory condition of bile ducts, leading to cirrhosis and portal hypertension, with a 100% mortality without intervention.
- Portoenterostomy (Kasai procedure) is a critical surgical intervention, but its long-term efficacy varies, with many patients eventually needing liver transplantation.
Purpose of the Study:
- To evaluate the long-term outcomes of portoenterostomy in patients with biliary atresia.
- To identify factors influencing the success rate of portoenterostomy and patient survival.
Main Methods:
- Retrospective analysis of 30 consecutive patients who underwent portoenterostomy for biliary atresia.
- Assessment of 5-year actuarial survival, complications, and the impact of cirrhosis and intrahepatic biliary hypoplasia on outcomes.
Main Results:
- The 5-year actuarial survival rate was 68%.
- Successful portoenterostomy occurred in 65% of patients without cirrhosis versus 35% with cirrhosis (p = 0.0148).
- Liver cirrhosis was more prevalent in patients with intrahepatic biliary hypoplasia (66%) compared to those with only extrahepatic biliary atresia (29%).
Conclusions:
- Portoenterostomy remains the primary surgical treatment for extrahepatic biliary atresia.
- The presence of cirrhosis is associated with a poorer prognosis and may warrant consideration for early liver transplantation.
- Intrahepatic biliary hypoplasia, often co-occurring with cirrhosis, may contribute to lower portoenterostomy success rates.
Objective:
Biliary atresia is the result of a fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Without surgical intervention, mortality reaches 100%. The 5-year survival rate after portoenterostomy ranges from 13% to 60%, with approximately 60% of patients requiring liver transplantation at a later stage because of insufficient bile flow.
Methods:
This retrospective analysis includes 30 consecutive patients undergoing portoenterostomy for biliary atresia at our hospital.
Results:
The 5-year actuarial survival of the 30 patients was 68%. Thirteen patients (43.3%) died 3 days to 7 years after portoenterostomy. Four patients (13.3%) underwent liver transplantation 3 to 24 months after the Kasai procedure with a 100% survival. In 65% of patients without presence of cirrhosis, the portoenterostomy was successful, compared with 35% of cases with liver cirrhosis (p = 0.0148). Liver cirrhosis with extrahepatic biliary atresia alone was present in 5 of 17 patients (29%) as compared with 8 of 12 patients (66%) with intrahepatic biliary hypoplasia in addition to extrahepatic biliary atresia and cirrhosis.
Conclusions:
Portoenterostomy remains the treatment of choice for patients with extrahepatic biliary atresia. However, the presence of cirrhosis portends a poorer prognosis and may be an indication for early transplantation. Cirrhosis is more commonly present in the setting of intrahepatic biliary hypoplasia and may account for the lower success rates of portoenterostomy in this group of patients. Five-year survival of the female patients was 88% as compared with 55% of the male patients.
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