胆管导管内乳头状黏液性肿瘤的诊治并文献复习

Diagnosis and treatment of intraductal papillary mucinous neoplasm of the bile duct: report of one case and literature review

目的总结分析胆管导管内乳头状黏液性肿瘤(IPMN-B)的诊治经验。方法回顾性分析2018年4月青岛大学附属烟台毓璜顶医院收治的1例IPMN-B患者临床资料。患者女,71岁,因"右上腹阵发性隐痛2周,全身皮肤、巩膜黄染"入院。查体:右上腹轻压痛,无反跳痛,Murphy征阴性。WBC 15.2×10~9/L,DB 112.1μmol/L,ALP 653 U/L,CA19-9>1 000 kU/L。增强MRI示肝内外胆管明显扩张,胆囊增大,左肝实质萎缩。十二指肠镜检查示十二指肠乳头充血。初步诊断为梗阻性黄疸。患者签署知情同意书,符合医学伦理学规定。予积极抗感染治疗后行剖腹探查。结果术中见肝脏淤胆,左肝萎缩,胆囊胀大壁厚,胆总管扩张,直径约2.5 cm。肝外胆管内大量黏稠胶冻样物质,肝左内叶胆管黏膜充血隆起,大小约1.5 cm×1.3 cm,病变局限于左肝内胆管,行胆囊切除+左半肝切除+T管引流。患者术后恢复顺利,黄疸进行性下降。术后病理检查示左肝内IPMN-B伴中度异型增生,局灶伴重度异型增生。结论 IPMN-B治疗的关键是早发现、早诊断、早治疗。早期根治性切除病灶,解除胆道梗阻可获得良好预后。

Objective To summarize and analyze the diagnosis and treatment experience of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B). Methods 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 15.2×10^9/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. Results 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. Conclusions 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.