Liver transplantation for "mass-forming" sclerosing cholangitis after laparoscopic cholecystectomy

Introduction: Chronic biliary obstruction consequence of a bile duct injury may require liver transplantation (LT) in case of secondary biliary cirrhosis, intractable pruritus or reiterate episodes of cholangitis. "Mass-forming" sclerosing cholangitis leading to secondary portal vein thrombosis and pre-sinusoidal portal hypertension has not been reported so far.

Presentation of case: We present the case of a patient who underwent laparoscopic cholecystectomy for Mirizzi syndrome. The persistent bile duct obstruction due to a residual gallstone fragment was treated by a prolonged biliary stenting. Following repeated bouts of cholangitis, a fibrous centrohepatic scar developed, conglobating and obstructing the main branches of the portal vein and of the biliary tree. The patient developed secondary portal vein thrombosis and portal hypertension. After an extensive diagnostic work-up, including surgical exploration to rule out malignancy, the case was successfully managed by liver transplantation.

Discussion: Mass-forming sclerosis of the bile duct and biliary bifurcation may develop as a consequence of chronic biliary obstruction and prolonged stenting. Secondary portal vein thrombosis and pre-sinusoidal portal hypertension represents an unusual complication, mimicking Klatskin tumor.

Conclusion: A timely and proper management of post-cholecystectomy complications is of mainstay importance. Early referral to a specialized hepato-biliary center is strongly advised.