Background : IPNB is a rare neoplasm of biliary tract with high chances of malignant transformation as compared to its pancreatic counterpart IPMN. There are very few case reports in literature about its management and surgical technique

Methods : We present a 67-year-old female patient who presented with upper abdominal pain and elevation of hepatobiliary enzymes without jaundice. Contrast enhanced CT scan and magnetic resonance showed lesion at the hilum with ductal communication suggestive of IPNB. Patient was planned for surgery with initial plan of En-bloc extra-hepatic biliary tract excision and major right sided liver resection as a back-up in case of inability to procure negative margins on right-sided ducts.

Results : On intraoperative cholangiography, we found the biliary communication between the lesions with RASD, RPSD and LHD. En-bloc extra-hepatic biliary tract excision with triple cholangio-jejunostomy (RASD, RPSD and LHD) was performed. The operative time was 360 min, and the estimated blood loss was 200 ml. Patient was discharged on post-operative day 4 without any major complication such as bile leak. The final histopathological diagnosis was Intraductal papillary neoplasm of bile duct (type 2) with associated invasive carcinoma, pT1N0 with R0 resection. After 18 months of follow-up patient has no recurrence and normal liver function.

Conclusions : IPNB is usually a benign neoplasm but its propensity to undergo invasive carcinoma cannot be ignored as in our case. Proper surgical planning helps in achieving R0 resection with good long-term outcome.