目的：探讨内镜逆行胰胆管造影（endoscopic retrograde cholangiopancreatography, ERCP）在诊断和治疗小儿胰胆管汇合异常（pancreaticobiliary maljunction, PBM）中的安全性和有效性。方法：回顾性分析本院ERCP确诊为PBM的74例患儿,分析临床资料和术后随访。结果：按照日本胆胰管合流异常研究组（Japanese Study Group on Pancreaticobiliary Maljunction, JSGPM）分型, A型32例,B型16例, C型23例,D型3例。MRCP检查的阳性诊断率仅8%。本研究所有患儿均行乳头肌切开和鼻胆管引流。其中69例（93.4%）行取石/蛋白酶栓,2例术后留置胰管支架。7例（9.5%）术后出现ERCP后轻型胰腺炎,无术后出血、贲门撕裂及穿孔等严重并发症发生。除7例（9.5%）PBM不合并胆道扩张的患儿外,其余术后均行肝外胆管切除+肝管空肠Roux-en-Y吻合术。结论：ERCP在小儿PBM的诊治过程中具有诊断效力强、疗效好、安全性高的优点,对早期确诊并干预PBM的病理进程具有重要意义。

Objective To investigate the safety and effectiveness of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis and treatment of pediatric pancreaticobiliary maljunction(PBM). Methods A total of 74 children with PBM diagnosed by ERCP were enrolled. The clinical data with postoperative follow-up were analyzed. Results There were 32 cases with type A, 16 cases with type B, 23 cases with type C and 3 cases with type D based on the classification of PBM proposed by Japanese Study Group on PBM. The positive diagnostic rate of PBM was only 8% using MRCP. All children underwent endoscopic sphincteropapillotomy with endoscopic nasobiliary drain of which 69 cases(93.4%) were preceded with extraction of pancreatic protease emboli or stones. Pancreatic stent was followed in 2 cases. Seven cases (9.5%) had mild pancreatits after ERCP. No bleeding or cardiac laceration and perforations were found. The children had resection of extrahepatic bile duct combined with hepatic duct jejunum Roux-en-Y anastomotisis except for 7(9.5%) cases without biliary dilatation. Conclusions ERCP could be performed safely with effective diagnosis and treatment in pediatric PBM, and is an important procedure for children with PBM during early stage.