Biliary Tract IPMN Successfully Treated with Roux-en-Y Hepaticojejunostomy

Abstract

A 72 year-old lady presented with complaints of jaundice, fatigue, pruritis and anorexia. Her medical history was notable for chronic abdominal pain, GERD and previous cholecystectomy. Admission lab studies revealed a total bilirubin of 7.4, alkaline phosphatase of 194 and an AST of 82. CT and MR imaging showed dilated intra and extrahepatic biliary ducts and suspicion for an ampullary mass. The pancreatic duct was radiographically normal. Initial ERCP revealed haziness in the CBD; two stents were placed after the duct was swept for gelatinous fluid which was cytologically negative for malignancy. She was discharged home but returned two weeks later with persistent complaints of refractory jaundice. Repeat serum studies yielded an increased total bilirubin of 14.1, an alkaline phosphatase of 342 and a stable AST of 84. She underwent repeat EUS/ERCP and intraductal cholangioscopy (with the Spyglass system) which showed a lesion at the hilar and right main hepatic duct suspicious for intraductal papillary mucinous neoplasm (IPMN). She had surgical resection of the extrahepatic bile duct with right hepatectomy, re-excision of the left hepatic duct margin and Roux-en-Y hepaticojejunostomy. The postoperative course was complicated by the development of ascites and septicemia which were successfully treated with diuretics and antibiotics. Excised tissue sent for pathology confirmed primary right hepatic duct IPMN consistent with carcinoma in situ and high grade dysplasia. Furthermore, right partial hepatectomy revealed extensive IPMN involving major and minor ducts and obstructive cholangiopathy of the liver adjacent to the bile duct. Compared to its pancreatic counterpart, biliary tract-IPMN (BT-IPMN) is an uncommon phenomenon and is even rarer when isolated to the intra and extra-hepatic hilar ducts. BT-IPMN is more often associated with invasive features compared to similar pancreatic neoplasms. Patients typically present with abdominal pain, jaundice and elevated total bilirubin and alkaline phosphatase. Historically, BT-IPMNs have been frequently misdiagnosed (as adenocarcinomas, cholangiocarcinomas and cystic neoplasms); there is an emergence of literature substantiating the need for clear diagnostic definitions for this rare diagnosis. BT-IPMN is defined as an intraluminal neoplasm with papillary cyto-architecture and mucin hypersecretion often leading to marked dilatation of affected bile ducts. Treatment is ultimately resection of the involved tissue.