The clinical presentations of ectopic biliary drainage into duodenal bulbus and stomach with a thorough review of the current literature

Abstract

BackgroundEctopic biliary drainage is a rare congenital anomaly on which we have scarce data in the current literature.MethodsThe data were collected from the records of 400 endoscopic retrograde cholangio-pancreatography (ERCP). In this report, we present 10 cases (male/female: 9/1, mean age 54 years, range 38-74) with ectopic biliary openings into the duodenum and/or stomach diagnosed by endoscopic retrograde cholangio-pancreatography (ERCP).ResultsIn our series, the frequency of ectopic biliary drainage is 2% (10 out of 400 ERCPs). Recurrent attacks of cholangitis and complicated ulcer formation in the distal stomach and bulbar duodenum were the most common signs in the present series. The sites of ectopic biliary drainage were the stomach in 1 case, the duodenum bulbus in 7 cases and the postbulbar duodenum in 2 cases. Bulbar ulcer, deformed pylorus and bulbus were present in 7 cases, apical bulbar stricture in 2, gastric ulcer in 1, pyloroplasty and/or gastroenterostomy in 3 cases. One case had had previous bleeding episode. Some of them had undergone previous surgeries for gall-stone disease (cholecystectomy in 5 cases, bile duct operation in 3 cases) and ulcer complications (pyloroplasty/gastroenterostomy in 3 cases). ERCP revealed dilatation of the biliary tree and hook shaped distal choledochus in all cases, choledocholithiasis in 7 and Mirizzi syndrome in 1. Endoscopic balloon dilatations for gastric outlet obstruction, extraction of bile stones after balloon dilating the ectopic site, surgery for difficult cases with large bile duct stones or with gastric outlet obstruction were preferred methods in this series of patients.ConclusionWith this report, we have to remind that ectopic biliary drainage must be considered in the differential diagnosis when the clinician faces cases with gastric outlet obstruction due to peptic ulcer formation accompanied by cholangitis/cholestasis.

Ectopic Opening of the Common Bile Duct Accompanied by Choledochocele and Pancreas Divisum

“…Some previous reports described the presence of the major papilla in the second portion of the duodenum (1, 6, 7). In other reports, the pancreatic duct was visualized in fewer than half of the total cases (2)(3)(4)(5)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), which suggests the possibility of another orifice joining with the pancreatic duct. Moreover, from the viewpoint of the embryonic process, the presence of the major papilla is consistent with Boyden's theory (30), as described above.…”

“…Previous reports have suggested that the incidence of PD in autopsy cases is about 9% (21,22). To our knowledge, this is the first report of an ectopic opening of the CBD into the duodenal bulb accompanied by choledochocele and PD (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). Choledochocele concomitant with PD is also rare, with only three case reports having been published (25)(26)(27); therefore, our case is extremely rare.…”

“…The diagnostic criteria of ectopic openings of the CBD have not yet been established. In previous reports, such a diagnosis was often described as being made based on the following findings: 1) an orifice was observed in the stomach, bulb, or third portion of the duodenum by duodenoscopy or upper endoscopy, and the bile duct and/or the pancreatic duct were directly visualized radiographically when contrast medium was injected via this opening; 2) there was no evidence of any other drainage into the digestive tract on cholangiography; and 3) there was no evidence of a papilla-like structure in the second portion of the duodenum on duodenoscopic examination (3,4,8,12,15). In our case, the major papilla was observed at the normal location in the second portion of the duodenum, and the ventral pancreatic duct was joined to the major papilla.…”

“…Ectopic opening of the common bile duct (CBD) is a rare congenital biliary anomaly, with the CBD emptying into anomalous sites such as the third or fourth portion of the duodenum, stomach, or duodenal bulb (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). Choledochocele is also a biliary anomaly, with cystic dilatation of the intraduodenal segment of the CBD (18)(19)(20).…”

Difficult ERCP with Aberrant Papilla A Report of Three Cases

“…So in difficult ERCP where the papilla cannot be found in its usual location ectopic biliary drainage and abnormal situation of papilla must be considered and searched. The frequency of ectopic biliary drainage by ERCP is 2% (10 out of 400 ERCP) 8 .…”