[Congenital atresia of the extrahepatic bile-ducts in two siblings (author's transl)].

Congenital atresia of the extrahepatic bile-ducts in two siblings (boy and girl) with parental consanguinity is reported. Diagnosis is based on liver biopsy. In one case (boy) laparotomy was performed. An attempt at reconstructive surgery of the biliary tract was unsuccessful and the boy died at the age of 13 months. Only a small number of cases with familiar congenital extrahepatic biliary atresia have been published to date.