The only treatment for biliary atresia is early surgical intervention. The standard operation is portoenterostomy, which was first introduced by Kasai more than 30 years ago. Numerous variations and modifications of this procedure have been published and the outcome for jaundice-free EHBA-patients after surgery has improved over the years. Nevertheless, surgical therapy is only symptomatic and, in spite of successful portoenterostomy, portal hypertension develops in most patients with EHBA.
Concerning the outcome for babies with EHBA, the rate of bile flow after portoenterostomy depends on the practical experience of the surgeon. Therefore, we would welcome a concentration of EHBA-patients in multidisciplinary centers with a lot of experience with biliary atresia.