Neues zur adjuvanten Therapie der Gallengangsatresie nach Kasai-Hepatoportoenterostomie

Biliary atresia is a rare cholangiopathy of unknown etiology and is the most common indication for a liver transplantation in childhood. In order to avoid or delay transplantation, Kasai-hepatoportoenterostomy (KPE) should be performed as early as possible.

Nevertheless, only 20–30% of patients achieve a long-term survival with their native liver. In order to further extend survival without a transplantation, numerous adjuvant treatments have been reported. The aim of these interventions is to improve postoperative biliary drainage, reduce the rate of cholangitis and ameliorate cholestasis-related symptoms; however, the evidence for advantages of the majority of these treatments is weak. Recent studies and international treatment protocols give an overview on the application of adjuvant treatment of this rare disease in the clinical practice. The use of antibiotics, probiotics, antiviral agents, ursodeoxycholic acid, steroids and further treatment options have been described in the adjuvant postoperative management of biliary atresia.