AN UNEXPECTED CAUSE OF CHOLANGITIS

A 57-year-old man with unremarkable previous medical history presented with acute cholangitis (bilirubin T/D 4,5/3,8 mg/dL, ALP 805 UI/I, GGT 203 UI/I). CT abdomen showed liver cirrhosis along with hypodense irregular filling defects in a dilated common hepatic duct up to the confluence, with upstream dilation of the intrahepatic ducts. A subsequent ERCP with IDUS and direct peroral cholangioscopy was performed, and the findings supported the suspicion of biliary papillomatosis. Intraductal biopsies showed papillary proliferations with focal high grade dysplasia and no signs of stromal invasion, thus confirming the final diagnosis of intraductal papillary neoplasm (IPN) BillN-3. Biliary IPN is a very rare disease, characterized by the presence of intraluminal papillary tumors of the intra- and/or extra hepatic bile ducts, with a distinctive papillary proliferation of billiary epithelial cells around a slender fibrovascular stalk. It potentially affects any site of the biliary tree, including the gallbladder. It is more common in men (M: F 2: 1) in the 6 – 7th decade, with a slow progression but with a high rate of malignant transformation (approximately 41%). The most common clinical manifestations are recurrent abdominal pain or discomfort, repeated relapsing cholangitis and cholestasis. The therapy is mainly surgical, with resection in localized disease; diffuse or recurrent disease requires pancreatoduodenectomy or liver transplantation in selected cases.

After a multidisciplinary discussion, the patient has now been referred and selected for orthotopic liver transplantation (due to the concomitant findings of liver cirrhosis and biliary IPN) in our transplant center.