胰胆管合流异常（PBM）是一种罕见的先天性胆胰系统发育缺陷，其特征为胰胆管在十二指肠壁外的非正常部位汇合，形成延长的共同通道。这一解剖变异破坏了Oddi括约肌的正常生理功能，减弱了对胆胰液逆流的防御机制，从而诱发胆管和胰腺的一系列并发症。尽管PBM的发病率较低，但由于其临床症状隐匿，往往导致诊断延误，增加了治疗难度和不良预后的风险。针对胆管扩张明显的PBM患者，外科干预，尤其是胆囊切除联合肝外胆管切除及胆管空肠Roux-en-Y吻合术是目前的标准疗法。但针对胆管无明显扩张的PBM，相关治疗策略尚存争议，多数专家倾向于胆囊切除，但对于肝外胆管的管理仍缺乏统一意见，亟待进一步研究探索。内镜逆行胰胆管造影（ERCP）是目前评估胰胆管结构异常和诊断PBM的首选工具，不仅可以明确病变性质，还能实现胆汁采集和胆管组织的病理学分析，且具有介入治疗功能，如支架置入、扩张、引流，尤其利于合并胆系肿瘤的患者。但ERCP的侵袭性限制了其在大规模筛查中的应用，特别是在儿童群体中，技术实施更为复杂，且存在诱发多种并发症的风险。本文旨在阐述PBM的定义、分类、发病机制、流行病学特征以及当前诊断与治疗策略研究进展，以期为临床实践提供参考指导。

Pancreaticobiliary maljunction （PBM） is a rare congenital developmental defect of the biliary-pancreatic system characterized by a junction of the pancreatic and bile ducts outside the duodenal wall， forming an extended common channel. This anatomical anomaly compromises the normal function of Oddi’s sphincter， weakens defenses against reflux， and thus triggers a series of biliary and pancreatic complications. Although there is a relatively low incidence rate of PBM， its insidious clinical symptoms often lead to delayed diagnosis， which increases the difficulties in treatment and the risk of poor prognosis. For PBM patients with marked bile duct dilatation， surgical intervention， especially cholecystectomy combined with extrahepatic bile duct resection and bile duct-jejunum Roux-en-Y anastomosis， remains the standard treatment at present. For PBM without marked bile duct dilatation， there are still controversies over related treatment strategies， and most experts are in favor of cholecystectomy， while there is still a lack of consensus on the management of extrahepatic bile ducts， which requires further research and exploration. Endoscopic retrograde cholangiopancreatography （ERCP） is currently the gold standard for diagnosing PBM and assessing pancreaticobiliary abnormalities， and it can not only clarify the nature of lesion， but also collect the bile and biliary duct tissue for pathological examination. ERCP also has the function of interventional treatment， such as stenting， expansion， and drainage， thereby bringing benefits to patients comorbid with biliary neoplasms. However， the application of ERCP in screening is limited by its invasiveness， with increases in technique complexity and the risk of complications in the pediatric population. This article summarizes the definition， classification， pathogenesis， and epidemiological features of PBM and the research advances in current diagnosis and treatment strategies， in order to provide guidance for clinical practice.