To summarize and analyze the diagnosis and treatment experience of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B).

Clinical data of one patient with IPMN-B admitted to Yantai Yuhuangding Hospital Affiliated to Qingdao University in April, 2018 were retrospectively analyzed. The 71-year-old female patient was hospitalized due to paroxysmal pain in the right upper abdomen for 2 weeks complicated with systemic skin and scleral jaundice. Physical examination: slight tenderness in the right upper abdomen, no rebound tenderness and negative Murphy sign. WBC 15.2×10⁹/L, DB 112.1 μmol/L, ALP 653 U/L and CA19-9>1 000 kU/L. Enhanced MRI demonstrated evident dilatation of intrahepatic and extrahepatic bile duct, gallbladder enlargement and left hepatic parenchymal atrophy. Duodenal endoscopy showed the congestion of duodenal papilla. She was initially diagnosed with obstructive jaundice. The informed consent of the patient was obtained and the local ethical committee approval was received. Exploratory laparotomy was performed after active anti-infection treatment.

Intraoperatively, cholestasis, left liver atrophy, gallbladder enlargement with thickening wall and dilatation of the common bile duct with a diameter of approximately 2.5 cm were observed. A large amount of viscous jelly-like substance was noted in the extrahepatic bile duct. Hyperemia and swelling occurred in the bile duct mucosa of the left intrahepatic bile duct, with a size of approximately 1.5 cm×1.3 cm. The lesions were restricted to the left intrahepatic bile duct. Cholecystectomy + left hemihepatectomy + T-tube drainage were performed. The patient was recovered well and the jaundice was progressively relieved. Postoperative pathological examination demonstrated the left intrahepatic IPMN-B complicated with moderate dysplasia, and partial lesions were complicated with severe dysplasia.

Early detection, diagnosis and treatment play a key role in the treatment of IPMN-B. Early radical resection and management of the biliary obstruction can achieve a good clinical prognosis.