Successful liver transplantation in a patient with ulcerative colitis – Associated with primary sclerosing cholangitis, complicated by biliary sepsis

Introduction: Primary Sclerosing Cholangitis (PSC) is an idiopathic disorder, characterized by a progressive obliterative inflammatory fibrosis of the bile ducts.

Case report: A 29-year-old male patient was diagnosed to have ulcerative colitis and PSC in 1995. On mesalazine (500mg t.i.d) and UDCA (250mg t.i.d) therapy he was symptom-free during the next 8 years, although his cholestatic liver enzymes increased gradually.

Acute biliary pancreatitis manifested in April 2003. ERCP detected small stones in the common bile duct, many strictures and saccular dilatations of the intrahepatic bile ducts containing stones as well. After papillotomy and endoscopic clearance of the common bile duct pancreatitis healed. Two weeks later high fever and right upper abdominal pain occured. On repeated ERCP the bile was purulent. Culture of the bile yielded multiple pathogenic organisms. Despite combined antimicrobial therapy – according to the sensitivity data, continuous nasobiliary lavage and vigorous supportive therapy rapid progression toward biliary sepsis and liver failure developed. Although active infection regarded as a relative contraindication of liver transplantation, the patient was accepted, beeing the only rescue therapy. Ten weeks after the begining of the biliary sepsis, successful ortotopic liver transplantation was performed. His recovery was uneventful. Histology of the removed liver revealed a small focal cholangiocarcinoma. His tumor markers remain negative.

Conclusion: Liver transplantation can be a life-saving procedure in PSC associated liver failure even a patient with biliary sepsis.