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胰胆管合流异常与胰腺分裂不全:胆总管梗阻的罕见病因

Pancreaticobiliary malunion and incomplete pancreas divisum: an unusual cause of common bile duct obstruction.

作者信息

Petrasek J, Hucl T, Spicak J

机构信息

Department of Hepatology and Gastroenterology, Institute for Clinical and Experimental Medicine, Prague, the Czech Republic.

出版信息

Adv Med Sci. 2008;53(1):6-10. doi: 10.2478/v10039-008-0002-3.

Abstract

PURPOSE

Pancreaticobiliary malunion (PBM) is a distinct disease entity of the pancreatic and biliary ductal system defined as a condition in which the junction of the pancreatic and biliary ducts occurs above the duodenal wall. PBM may be combined with a stenosis of the distal common bile duct and pathological changes in the common bile duct wall (congenital cyst of bile duct), being a potentially malignant condition. Pancreas divisum, resulting from a fusion failure of the ventral and dorsal pancreatic buds, and characterized by a dominant Santorine duct, is considered to be a predisposing factor to recurrent attacks of acute pancreatitis. In incomplete pancreas divisum, the ventral and dorsal pancreas are connected by a segmental branch.

MATERIAL AND METHODS

We report a case of a 33-year-old female patient with PBM associated with incomplete pancreas divisum, who had presented episodes of acute cholangitis due to a benign distal common bile duct stricture.

RESULTS

Treatment with choledochoduodenostomy and cholecystectomy provided thorough relief and resolution of symptoms.

CONCLUSION

This is the first report of coexistent PBM and incomplete pancreas divisum in a Caucasian patient with unusually late clinical manifestation.

摘要

目的

胰胆管合流异常(PBM)是胰胆管系统的一种独特疾病实体,定义为胰管和胆管的汇合发生在十二指肠壁上方的一种情况。PBM可能合并胆总管远端狭窄和胆总管壁的病理改变(先天性胆管囊肿),是一种潜在的恶性疾病。胰腺分裂是由于腹侧和背侧胰芽融合失败导致的,其特征是Santorini导管占优势,被认为是急性胰腺炎反复发作的一个易感因素。在不完全性胰腺分裂中,腹侧和背侧胰腺通过一个节段性分支相连。

材料与方法

我们报告一例33岁女性患者,患有PBM并伴有不完全性胰腺分裂,因良性胆总管远端狭窄出现急性胆管炎发作。

结果

胆总管十二指肠吻合术和胆囊切除术治疗后症状完全缓解。

结论

这是首例关于一名白种人患者同时存在PBM和不完全性胰腺分裂且临床表现异常延迟的报告。

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