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胰胆管合流异常:病理生理学、诊断、分类、管理及研究前景的多维度探索

Pancreaticobiliary Maljunction: A Multidimensional Exploration of Pathophysiology, Diagnosis, Classification, Management and Research Prospects.

作者信息

Huang Peng, Yang Hu, Kuang Houfang, Yang Jun, Duan Xufei, Bian Hongqiang, Wang Xin

机构信息

Department of General Surgery, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430016, Hubei, China.

出版信息

Dig Dis Sci. 2025 Apr 19. doi: 10.1007/s10620-025-09057-0.

DOI:10.1007/s10620-025-09057-0
PMID:40252147
Abstract

Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall, usually forming a markedly long common channel, which can cause reciprocal reflux between pancreatic juice and bile. Cholangiography, endoscopic ultrasonography, surgery, and autopsy can be used to diagnose pancreaticobiliary maljunction. Elevated amylase levels in bile and extrahepatic bile duct dilatation strongly suggest the existence of pancreaticobiliary maljunction. The regurgitation may lead to the development of various hepatobiliary and pancreatic disorders such as pancreatitis and biliary carcinoma. The pathogenesis of pancreaticobiliary maljunction is the result of a series of pathophysiological changes caused by reflux. Surgery is recommended for patients diagnosed with pancreaticobiliary maljunction irrespective of the presence or absence of symptoms because of its high biliary carcinogenicity, but the treatment strategy is quite different between adult patients with and without biliary dilatation.

摘要

胰胆管合流异常是一种先天性畸形,其中胰管和胆管在十二指肠壁外进行解剖学上的汇合,通常形成一条明显较长的共同通道,这可导致胰液和胆汁之间的相互反流。胆管造影、内镜超声检查、手术及尸检可用于诊断胰胆管合流异常。胆汁中淀粉酶水平升高及肝外胆管扩张强烈提示胰胆管合流异常的存在。反流可能导致各种肝胆和胰腺疾病的发生,如胰腺炎和胆管癌。胰胆管合流异常的发病机制是反流引起的一系列病理生理变化的结果。鉴于其较高的胆管致癌性,无论有无症状,建议对诊断为胰胆管合流异常的患者进行手术,但成年有胆管扩张和无胆管扩张患者的治疗策略有很大不同。

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本文引用的文献

1
Pancreaticobiliary Maljunction and Its Relationship with Biliary Cancer: An Updated and Comprehensive Systematic Review and Meta-Analysis on Behalf of TROGSS-The Robotic Global Surgical Society.胰胆管合流异常及其与胆管癌的关系:代表机器人全球外科学会(TROGSS)进行的最新全面系统评价和荟萃分析
Cancers (Basel). 2025 Jan 2;17(1):122. doi: 10.3390/cancers17010122.
2
Morphological changes in the biliary mucosa in pediatric patients with congenital biliary dilatation are more influenced by the duration of amylase exposure than by amylase levels in the gallbladder.先天性胆管扩张症患儿的胆道黏膜形态学变化受淀粉酶暴露时间的影响大于胆囊内淀粉酶水平的影响。
Pediatr Surg Int. 2024 Aug 5;40(1):215. doi: 10.1007/s00383-024-05799-6.
3
Effectiveness of gel-immersion endoscopy for examination for suspected pancreaticobiliary maljunction.
凝胶浸入式内镜检查对疑似胰胆管合流异常的检查效果
Endoscopy. 2024 Dec;56(S 01):E489-E490. doi: 10.1055/a-2325-4853. Epub 2024 Jun 7.
4
Efficacy and Safety of Endoscopic Retrograde Cholangiopancreatography in Children of Pancreaticobiliary Maljunction Without Obvious Biliary Dilatation.内镜逆行胰胆管造影术在无明显胆管扩张的胰胆管汇合不良儿童中的疗效和安全性。
J Pediatr Surg. 2024 Apr;59(4):653-659. doi: 10.1016/j.jpedsurg.2023.11.026. Epub 2023 Dec 14.
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Clinical presentations and outcomes of pancreaticobiliary maljunction in different pediatric age groups.不同年龄段小儿先天性胰胆管汇合异常的临床表现和结局。
BMC Pediatr. 2023 Aug 26;23(1):427. doi: 10.1186/s12887-023-04248-y.
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Development of gallbladder cancer during follow-up of pancreaticobiliary maljunction: a report of two cases.胰胆管合流异常随访期间胆囊癌的发生:两例报告
J Surg Case Rep. 2022 Dec 30;2022(12):rjac595. doi: 10.1093/jscr/rjac595. eCollection 2022 Dec.
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A Comprehensive Review on Collagen Type I Development of Biomaterials for Tissue Engineering: From Biosynthesis to Bioscaffold.I型胶原蛋白用于组织工程的生物材料开发综述:从生物合成到生物支架
Biomedicines. 2022 Sep 16;10(9):2307. doi: 10.3390/biomedicines10092307.
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Imaging Features of Premalignant Biliary Lesions and Predisposing Conditions with Pathologic Correlation.具有病理相关性的癌前胆道病变和易患条件的影像学特征。
Radiographics. 2022 Sep-Oct;42(5):1320-1337. doi: 10.1148/rg.210194. Epub 2022 Aug 5.
9
Stepwise correlation of TP53 mutations from pancreaticobiliary maljunction to gallbladder carcinoma: a retrospective study.从胰胆管合流异常到胆囊癌的 TP53 突变的逐步相关性:一项回顾性研究。
BMC Cancer. 2021 Nov 19;21(1):1245. doi: 10.1186/s12885-021-09000-2.
10
Bile Metabolites and Risk of Carcinogenesis in Patients With Pancreaticobiliary Maljunction: A Pilot Study.胆酸代谢物与胆胰管汇合异常患者致癌风险:一项初步研究。
Anticancer Res. 2021 Jan;41(1):327-334. doi: 10.21873/anticanres.14779.