The influence of cholangitis on the prognosis of extrahepatic biliary atresia

The prognosis of extrahepatic biliary atresia (EHBA) depends first on the progression of the basically existing disease and second on the development of preoperative existing alterations of hepatic structure. In addition there are postoperative complications which have essential influence on the prognosis of the disease. In this context ascending cholangitis poses a very important problem for children who have undergone a portoenterostomy. Both the organism and the frequency of cholangitis may play a part. This study should outline the influence of cholangitis on the prognosis. Since 1972, 146 cases of extrahepatic biliary atresia have been treated and followed up postoperatively. Children with a liver fibrosis degree IV at surgery will develop a cirrhosis in 86% of cases without additional influence of cholangitis. Children with a liver fibrosis degree II/III will develop a cirrhosis in 20 % of cases without the influence of cholangitis after 15 years. But children with a fibrosis degree II/III with one or several episodes of cholangitis will develop a liver cirrhosis in 50 % of cases after 12.5 years. The difference is statistically significant. In addition the frequency of cholangitis is also an important factor: The more episodes of cholangitis the greater the probability of cirrhosis.