EUS diagnosis of ectopic opening of the common bile duct in the duodenal bulb： A case report

Among the various congenital anomalies of the biliary system, an ectopic opening of the common bile duct (CBD) in the duodenal bulb is extremely rare. ERCP is essential for diagnosing the anomaly. A 55-year-old male was admitted to hospital for severe right upper quadrant abdominal pain, followed by fever, chills, elevated body temperature and mild icterus. The diagnosis of ectopic opening of CBD in the duodenal bulb was established on endoscopic ultraso-nography (EUS), which clearly demonstrated dilated CBD, with multiple stones and air in the lumen, draining into the bulb. A normal pancreatic duct, which did not drain into the bulb, was also observed. This finding was confirmed on ERCP and surgery. As far as we know, this is the first case of this anomaly diagnosed by EUS. Ectopic opening of the CBD in the duodenal bulb is not an incidental finding, but a pathologic condition which can be associated with clinical entities such as recurrent or intractable duodenal ulcer, recurrent biliary pain, choledocholithiasis or acute cholangitis. Endoscopic ultrasonography features allow preoperative diagnosis of this anomaly and can replace ERCP as a first diagnostic tool in such clinical circumstances. Embryology of the anomalies of the extrahepatic biliary tree has been also reviewed.     

Recurrence of inflammatory pseudotumor in the distal bile duct： Lessons learned from a single case and reported cases

Inflammatory myofibroblastic tumors (IMTs) or inflammatory pseudotumors (IPs) have been extensively discussed in the literature. They are usually found in the lung and upper respiratory tract. However, reporting of cases involving the biliopancreatic region has increased over recent years. Immunohistochemical study of these lesions limited to the pancreatic head or distal bile duct seems to be compatible with those observed in a new entity called autoimmune pancreatitis, but usually intense fibrotic reaction (zonation) predominates producing a mass. When this condition is limited to the pancreatic head, the common bile duct might be involved by the inflammatory process and jaundice may occur often resembling adenocarcinoma of the pancreas. We have previously reported a case of IMT arising from the bile duct associated with autoimmune pancreatitis which is an extremely rare entity. Four years after Kaush-Whipple resection, radiological examination on routine follow-up revealed a tumor mass, suggesting local recurrence. Ultrasound-guided FNA confirmed our suspicious diagnosis. This present case, as others, suggests that persistent follow-up is necessary in order to prevent irreversible liver damage at this specific location.     

Clinicopathological features and prognosis of surgical resected cases of biliary cancer with pancreaticobiliary maljunction

To the Editor:Pancreaticobiliary maljunction(PBM)is a congenital anomaly in which the pancreatic and bile ducts join anatomically outside of the duodenal wall away from the Oddi’s sphincter.This condition causes the reflux of pancreatic juice into the bile duct under high pressure,resulting in various pathologic changes.The features of PBM patients are common bile duct dilatation,long common channel,and high amylase levels in bile juice.Among them,one of the most significant problems is the development of biliary cancer,including extrahepatic bile duct and gallbladder cancers[1].A nationwide survey in Japan reported biliary cancer in 21.6%of adult patients with PBM concomitant with congenital biliary dilatation,32.1%and 62.3%extrahepatic bile duct and gallbladder cancers,respectively[2].     

Diagnosis and clinical implications of pancreatobiliary reflux

The sphincter of Oddi is located at the distal end of the pancreatic and bile ducts and regulates the outflow of bile and pancreatic juice. A common channel can be so long that the junction of the pancreatic and bile ducts is located outside of the duodenal wall, as occurs in pancreaticobiliary maljunction （PBM）; in such cases, sphincter action does not functionally affect the junction. As the hydropressure within the pancreatic duct is usually greater than in the bile duct, pancreatic juice frequently refluxes into the biliary duct （pancreatobiliary reflux） in PBM, resulting in carcinogenetic conditions in the biliary tract. Pancreatobiliary reflux can be diagnosed from elevated amylase level in the bile, secretinstimulated dynamic magnetic resonance cholangiop ancreatography, and pancreatography via the minor duodenal papilla. Recently, it has become obvious that pancreatobiliary reflux can occur in individuals without PBM. Pancreatobiliary reflux might be related to biliary carcinogenesis even in some individuals without PBM. Since few systemic studies exist with respect to clinical relevance and implications of the pancreatobiliary reflux in individuals with normal pancreaticobiliary junction, further prospective clinical studies including appropriate management should be performed.     

Endoscopic diagnosis of pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. As the action of the sphincter of Oddi does not functionally affect the junction in PBM patients, continuous pancreatobiliary reflux occurs, resulting in a high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst) and PBM without biliary dilatation (maximal diameter of the bile duct ≤ 10 mm). The treatment of choice for PBM is prophylactic surgery before malignant changes can take place. Endoscopic retrograde cholangiopancreatography (ERC P) is the most effective examination method for close obs ervation of the pattern of the junction site. When the communication between the pancreatic and bile ducts is maintained, despite contraction of the sphi ncter on ERCP, PBM is diagnosed. In these pat ients, levels of pancreatic enzymes in the bile are gene rally elevated, due to continuous pancreatobiliary reflux via a long common channel. Magnetic resonance cholangiopancreatography and 3D-computed tomography can diagnose PBM, based on findings of an anomalous union between the common bile duct and the pancreatic duct, in addition to a long common channel. Endoscopic ultrasonography and intraductal ultra sonography can demonstrate the junction outside the duodenal wall, and are useful for the diagnosis of asso ciated biliary cancer. Gallbladder wall thickness on ultra so nography can be a screening test for PBM.     

Endoscopic management of postoperative bile leaks

BACKGROUND: Significant bile leak as an uncommon complication after biliary tract surgery may constitute a serious and difficult management problem. Surgical management of biliary fistulae is associated with high morbidity and mortality. Biliary endoscopic procedures have become the treatment of choice for management of biliary Gstulae. METHODS: Ninety patients presented with bile leaks after cholecystectomy ( open cholecystectomy in 45 patients, cholecystectomy with common bile duct exploration in 20 and laparoscopic cholecystectomy in 25). The presence of bile leaks was confirmed by ERCP and the appearance of bile in percutaneous drainage of abdominal collections. Of the 90 patients with postoperative bile leaks, 18 patients had complete transaction of the common bile duct by ERCP and were subjected to bilioenteric anastomosis. In the remaining patients after cholangiography and localization of the site of bile leaks. therapeutic procedures like sphinctero-tomy, biliary stenting and nasobiliary drainage ( NBD ) were performed. If residual stones were seen in the common bile duct, sphincterotomy was followed by stone extraction using dormia basket. Nasobiliary drain or stents of 7F size were placed according to the standard techniques. The NBD was removed when bile leak stopped and closure of the fistula confirmed cholangiographically. The stents were removed after an interval of 6-8 weeks. RESULTS: Bile leaks in 72 patients occurred in the cystic duct (38 patients), the common bile duct (30 ), and the right hepatic duct (4). Of the 72 patients with post-operative bile leak, 24 had associated retained common bile duct stones and 1 had ascaris in common bile duct. All the 72 patients were subjected to therapeutic procedures including sphincterotomy with stone extraction followed by biliary stenting (24 patients), removal of ascaris and biliary stenting (1), sphincterotomy with biliary stenting (18), sphincterotomy with NBD (12), biliary stenting alone (12), and NBD alone (5). Bile leaks stopped in all patients at a median interval of 3 days (range 3-16 days) after endoscopic in- terventions. No difference was observed in efficacy and in time for the treatment of bile leak by sphincterotomy with endoprosthesis or endoprosthesis alone in patients with bile leak after surgery. CONCLUSIONS: Post-cholecystectomy bile leaks occur most commonly in the cystic duct and associated common bile duct stones are found in one-third of cases. Endoscopic therapy is safe and effective in the management of bile leaks and fistulae after surgery. Sphincterotomy with endoprosthesis or endoprosthesis alone is equally effective in the management of postoperative bile leak.     

Bilhemia after trans-jugular intra-hepatic porto-systemic shunt and its management with biliary decompression

Bilhemia or bile mixing with blood is a rare clinical problem. The clinical presentation is usually transient self-resolving hyperbilirubinemia, progressive and rapidly rising conjugated hyperbilirubinemia, or recurrent cholangitis. Endoscopic retrograde cholangiopancreatography （ERCP） plays an important role in diagnosis and management. Biliary decompression with endoscopic sphincterotomy is useful in treating these patients. If not recognized and treated in time, the condition can be fatal in a significant proportion of patients. This usually occurs after blunt or penetrating hepatic trauma due to a fistulous connection between the biliary radicle and portal or hepatic venous radical. Cases have been described due to iatrogenic trauma such as liver biopsy and percutaneous biliary drainage. However, the occurrence after trans-jugular intra-hepatic porto-systemic shunt （TIPS） is very rare. We report a case of bilhemia presenting as rapidly rising bilirubin after TIPS. The patient was managed successfully with ERCP and removal of a blood clot from the common bile duct.     

Migrated endoclip and stone formation after cholecystectomy： A new danger of acute pancreatitis

Endoclip migration into the common bile duct following laparoscopic cholecystectomy (LC) is an extremely rare complication. Migrated endoclip into the common bile duct can cause obstruction,serve as a nidus for stone formation,and cause cholangitis. We report a case of obstructive jaundice and acute biliary pancreatitis due to choledocholithiasis caused by a migrated endoclip 6 mo after LC. The patient underwent early endoscopic retrog-rade cholangiopancreatography (ERCP) with endoscopic sphincterotomy and stone extraction.     

Successful treatment of complex cholangiolithiasis following orthotopic liver transplantation with interventional radiology

Bile duct stones are a serious and the third most common complication of the biliary system that can occur following liver transplantation.The incidence rate of bile duct stones after liver transplantation is1.8%-18%.The management of biliary stones is usually performed with endoscopic techniques;however,the technique may prove to be challenging in the treatment of the intrahepatic bile duct stones.We herein reporta case of a 40-year-old man with rare,complex bile duct stones that were successfully eliminated with percutaneous interventional techniques.The complex bile duct stones were defined as a large number of bile stones filling the intra-and extrahepatic bile tracts,resulting in a cast formation within the biliary tree.Common complications such as hemobilia and acute pancreatitis were not present during the perioperative period.The follow-up period was 20 mo long.During the postoperative period,the patient maintained normal temperature,and normal total bilirubin and direct bilirubin levels.The patient is now living a high quality life.This case report highlights the safety and efficacy of the percutaneous interventional approach in the removal of complex bile duct stones following liver transplantation.     

New cannulation method for pancreatic duct cannulation-bile duct guidewire-indwelling method

The patient was a 58-year-old male with symptomatic alcoholic chronic pancreatitis.Since a 10 mm calculus was observed in the pancreatic body and abdominal pain occurred due to congestion of pancreatic juice,endoscopic retrograde cholangiopancreatography was conducted for assessment of the pancreatic duct and treatment of pancreatic calculus.Pancreatogram was slightly and insuff iciently obtained by injecting the contrast media via the common channel of the duodenal main papilla.We tried to cannulate selectively into the pancreatic duct for a clear image.However,the selective cannulation of the pancreatic duct was difficult because of instability of the papilla.On the other hand,selective cannulation of the bile duct was relatively easily achieved.Therefore,after the imaging of the bile duct,a guidewire was retained in the bile duct to immobilize the duodenal papilla and cannulation of the pancreatic duct was attempted.As a result,selective pancreatic duct cannulation became possible.It is considered that the bile duct guidewire-indwelling method may serve as one of the useful techniques for cases whose selective pancreatic duct cannulation is diff icult("selective pancreatic duct diff icult cannulation case").     

Duplication of the common bile duct manifesting as recurrent pyogenic cholangitis: A case report

BACKGROUNDDuplication of the extrahepatic bile duct (DCBD) is an extremely rare congenital anomaly of the biliary system. There are five types of DCBD according to the latest classification. Among them, Type V is characterized by single drainage of the extrahepatic bile ducts. Reports on DCBD Type V are scarce.CASE SUMMARYA 77-year-old woman presented with recurrent epigastric pain but without fever or chills. Computed tomography revealed a dilated common bile duct (CBD) that harboured multiple choledocholithiasis. Endoscopic retrograde cholangio-pancreatography (ERCP) was performed, and the stones were extracted using a Dormia basket. She was discharged without any complications; however, she visited the emergency department a day after she was discharged due to epigastric pain and fever. Laboratory findings were suggestive of cholestasis. After urgent ERCP for stone removal, magnetic resonance cholangiopancrea-tography was performed to evaluate remnant choledocholithiasis. Magnetic resonance cholangiopancreatography revealed a DCBD Type Va and remnant choledocholithiasis in the right CBD. Both CBDs were accessed, and the stones were cleared successfully during a subsequent ERCP.CONCLUSIONIn this article, we report an extremely rare case of DCBD manifesting as recurrent pyogenic cholangitis. This case highlights the importance of recognizing DCBD because stones in the unrecognized bile duct could make the patient’s prognosis critical.     

Choledochocele associated with superficial spreading cancer with cholesterolosis of the bile duct

A 74-year-old woman, who had suffered from acute cholangitis, was referred to our department for further evaluation of the biliary tree. A diagnosis of choledochocele was made by endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography. A small flat elevation with cholesterolosis was observed in the lower bile duct, and circumferential wall thickening was apparent in some parts of the upper and middle bile duct by intraductal ultrasonography and peroral cholangioscopy. With the diagnosis of choledochocele associated with superficial spreading cancer and cholesterolosis of the bile duct, pylorus-preserving pancreaticoduodenectomy was carried out. Microscopically, the tumor had spread extensively from the lower to the upper bile duct. Its invasion was limited to the mucosa for the most part, with microinvasion of the subserosa in the upper bile duct. In the lower bile duct, foamy cells were located beneath the neoplastic epithelium of the elevation. The number of cases of choledochocele associated with biliary cancer is becoming higher than previously reported. This anomaly may play some role in the development of biliary malignancy. Histological examination seems to be mandatory before making a diagnosis of cholesterolosis of the bile duct, since this condition may occasionally accompany cancer.     

An analysis of the relationship between bile duct stones and periampullary duodenal diverticula

Previous studies have suggested a relationship between bile duct stones and periampullary duodenal diverticula. The aim of the present study was to examine this association in more detail, including step-wise logistic regression to identify independent predictors for the presence of diverticula. Clinical, endoscopic and radiological data were analysed from 794 consecutive subjects with bile duct stones; 44 with primary duct stones (without gall-bladder stones) and 750 with bile duct stones associated with current or previous gall-bladder stones. Frequencies of diverticula in subjects with bile duct stones were compared with those in age-matched controls who had undergone upper gastrointestinal endoscopy using a side-viewing endoscope (n = 100) or who had undergone endoscopic retrograde cholangiopancreatography (ERCP) for pancreatic cancer (n = 100). The frequencies of diverticula in subjects with primary duct stones (70%) and bile duct stones with current or previous gall-bladder stones (25%) were significantly higher than in both control groups (7 and 8%, respectively). When subjects with bile duct stones were analysed by step-wise logistic regression, age and bile duct diameter were independent predictors of the presence of diverticula, but diverticula were unrelated to gender, mode of presentation, number of bile duct stones and outcome after endoscopic sphincterotomy. A significant association exists between duodenal diverticula and bile duct stones, perhaps because diverticula predispose to stone formation through the combined effects of bile stasis and bacterial contamination of bile.     

Carcinosarcoma of the extrahepatic bile duct

A rare case of a carcinosarcoma of the extrahepatic bile duct demonstrating interesting features is described. A 75-year-old woman with a history of choledocholithotomy presented with acute obstructive suppurative cholangitis. Ultrasonography and computed tomography showed a thickened choledochal wall, with calcification. Percutaneous transhepatic and endoscopic retrograde cholangiography revealed a round filling defect accompanied by an irregular obstruction in the common bile duct. Carcinosarcoma was diagnosed from a protruding lesion in the common bile duct obtained by intraoperative frozen sectioning, and pylorus-preserving pancreatoduodenectomy was performed. Histological examination by light microscopy showed a transition between the carcinomatous and sarcomatous components and positive immunoreactivity for epithelial markers in the sarcomatous component. The patient died of a local recurrence 2 years after the surgery. Polypoid growth and ossification in the tumor could be representative features of carcinosarcoma of the extrahepatic bile duct.     

内镜扩约肌切开术治疗胆总管继发性结石

目的评价逆行胰胆管造影术（ＥＲＣＰ）和内镜括约肌切开术（ＥＳＴ）在腹腔镜胆囊切除前后诊断和治疗胆总管继发结石中的作用．方法采用ＥＲＣＰ和ＥＳＴ在ＬＣ术前或术后诊断和治疗胆总管继发结石２２８例,其中包括ＬＣ术前发现的１８５例和术后确诊的４３例．常规ＥＲＣＰ检查,证实胆总管内有结石后行ＥＳＴ．然后根据结石形态、大小和数目不同采取不同方法处理结石．①自然排石,适合于直径在０３ｃｍ～０８ｃｍ的结石;②取石网篮取石,适合于直径在０９ｃｍ～１５ｃｍ的结石;③碎石篮碎石,适宜直径大于１５ｃｍ以上的结石．结果全部２２８例患者中,ＥＳＴ成功２１７例（９５２％）,胆总管结石完全排出２０９例（９１７％）,发生各种并发症１９例（８８％）,主要并发症为急性胰腺炎、急性胆管炎和Ｏｄｄｉ扩约肌切口渗血,全部经非手术治疗愈合,无死亡病例．结论ＥＲＣＰ和ＥＳＴ是ＬＣ术前和术后诊治胆总管结石安全有效的方法之一．     

Pancreaticobiliary ductal anatomy in Thai People

The complex anatomy of the pancreaticobiliary duct was studied to demonstrate basic data in Thai people. Fresh specimens of the pancreas, common bile duct, and duodenum were obtained en bloc from the autopsies of 103 patients who had died of causes not related to trauma or disease of these organs. The length of the pancreas, the location of the pancreatic ducts in relation to the surface of the pancreas, the length and types of the common channels, as well as the anatomy of the ampulla, were studied, using methylene blue infusion via the pancreatic duct cannulation and careful dissection. Ninety-three male and 10 female patients were included. Their ages ranged from 15 to 76 years (mean 31.38 ± 12.98 years). The length of the pancreas ranged from 10.9—19 cm (mean, 15.60 ± 1.80 cm). The intrapancreatic portion of the common bile duct showed patterns of three types; most common (90/103; 87.38%) was type A, in which the anterior surface of the common bile duct was totally covered, while its posterior surface was partially covered, by the pancreatic parenchyma. On dissection of the accessory duct of Santorini in the pancreatic substance, the accessory duct was traceable to the duodenal wall in 59 specimens (57.26%). The anatomy of the Wirsung-choledochus confluence was grouped into five different types. The common channel (junction of the common bile duct and pancreatic duct) was found in 76.70% of specimens and its length varied from just a common junction (so-called “V-type” anatomy) to 15 mm (Y-type-b). Separate papillae (so-called “II-type”) were found in 12.62% of specimens. Separate openings in the same papilla (so-called “U-type”) were found in 10.68% of specimens. The Wirsung duct at the pancreatic neck was most often located posterior and superior in relation to the surface of pancreas. This study demonstrated several important points regarding the anatomy of the pancreaticobiliary junction and pancreatic ductal system in a Thai population. Some of these data were different from those reported in the literature for other population groups.     

无先天性胆管囊性扩张型的胰胆管汇流异常与胆胰疾病的关系

目的：研究无先天性胆管囊性扩张型的胰胆管汇流异常（APBJ）及其与与胆胰疾病的关系。方法：分析1995年1月－2002年1月间逆行胰胆管造影（ERCP）、术中胆道造影（IOC）、磁共振胰管造影（MRCP）等影像学检查的2150例胆胰疾病中的52例无先天性胆管囊性扩张型APBJ及其与胆胰疾病的关系。结果：本组APBJ的发生率为2．4％，APBJ的检查发现率为ERCP2．9％，IOC2．4％、MRCP2．0％，在52例无先天性胆管囊性扩张型APBJ中有P－C型（胰管注入胆总管）32例、C－P型（胆总管汇入胰管）20例。胆囊上皮增生的发生率为51．9％，胆囊结石的发生率为73．1％，胆囊息肉样病变的发生率为26．9％，急性胰腺炎的发生率为34．6％，结论：APBJ与胆囊疾病关系密切，无先天性胆管囊性扩张型APBJ的胆囊上皮增生和胆囊息肉样病变发生率高，是胆囊癌发生的一个高危因素，对此类型APBJ病人主张预防性胆囊切除，APBJ是胰腺炎发病的一个重要因素，C－P型APBJ的胰腺炎发生率高，在反复胰腺炎发作而无明显原因时，应考虑有APBJ的存在。     

Technical aspects in the laparoscopic management of complicated common bile duct stones

The management of common bile duct (CBD) stones traditionally required open laparotomy and bile duct exploration. With the advent of endoscopic and laparoscopic technology in the latter half of last century, endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic cholecystectomy (LC) has become the mainstream treatment for CBD stones and gallstones in most medical centers around the world. However, in certain situations, ERCP cannot be feasible because of difficult cannulation and extraction. ERCP can also be associated with potential serious complications, in particular for complicated stones requiring repeated sessions and additional maneuvers. Since our first laparoscopic exploration of the CBD (LECBD) in 1995, we now adopt the routine practice of the laparoscopic approach in dealing with endoscopically irretrievable CBD stones. The aim of this article is to describe the technical details of this approach and to review the results from our series.     

Common Bile Duct Duplication: The more the murkier

Congenital duplication of the common bile duct is an extremely rare anomaly of the biliary tract, which putatively represents failure of regression of the embryological double biliary system. Depending on the morphology of the duplicated bile duct, the anomaly can be classified into five distinct subtypes as per the modified classification (proposed by Choi et al). Among the five subtypes of bile duct duplication, type V duplication is considered to be the least common with only two previous cases of type Va variant reported in medical literature prior to the current report.