Association of anomalous pancreaticobiliary ductal junction with gallbladder carcinoma in Chinese patients: an ERCP study

BACKGROUND: Anomalous pancreaticobiliary ductal junction, a rare congenital anomaly, is associated with various biliary and pancreatic diseases. The aim of this study was to determine the frequency of anomalous pancreaticobiliary ductal junction in Chinese patients with gallbladder cancer. METHODS: One thousand eight hundred seventy-six patients underwent ERCP between April 2000 and September 2001 with biliary and pancreatic duct opacification in 1082. Among the latter patients, those with proven gallbladder carcinoma were identified. Anomalous pancreaticobiliary ductal junction was defined as a common channel greater than 15 mm in length or a contractile segment totally distal to the union of the biliary and pancreatic ducts. When the common bile duct appeared to join the main pancreatic duct, the anomalous pancreaticobiliary ductal junction was denoted as B-P subtype; if the main pancreatic duct appeared to join the common bile duct, it was denoted P-B subtype. RESULTS: Fifty-four patients had gallbladder carcinoma, 7 of whom (3 men, 4 women) had anomalous pancreaticobiliary ductal junction (P-B subtype 6, B-P subtype 1). The mean (SD) length of the common channel was 21.0 mm (11.2 mm) with a range of 12 to 45 mm. One patient had early cystic dilation of bile duct. Three other patients had anomalous pancreaticobiliary ductal junction; 1 had an associated choledochal cyst and 2 a normal biliary tree. The overall frequency of anomalous pancreaticobiliary ductal junction was 0.9% (10/1082 cases). The frequency of anomalous pancreaticobiliary ductal junction was significantly higher in patients with gallbladder carcinoma (p < 0.001; OR, 50.7; 95% CI [12.7, 202.3]). CONCLUSIONS: Anomalous pancreaticobiliary ductal junction is strongly associated with gallbladder cancer among Chinese patients.     

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。     

内镜在胰胆管合流异常诊疗中的作用探讨

目的探讨ERCP在胰胆管合流异常中的诊断价值,评估内镜治疗的效果。方法16例胰胆管合流异常（PBM）患者,通过ERCP造影进行PBM分型,结合临床症状,分析引起相关疾病的机制、影像特点,根据合并的其它胰胆疾病,选择适当的内镜取石、扩张或引流等治疗,观察治疗效果。结果16例胰胆管合流异常患者多伴有腹痛、呕吐、黄疸等症状,及转氨酶和／或淀粉酶水平的升高。其中,Ⅰ型（B—P型）7例,Ⅱ型（P—B型）5例,Ⅲ型（复杂型）4例;合并胆总管囊肿扩张10例,无扩张者5例,胆管癌并狭窄1例;伴有胆管结石11例（4例为蛋白栓）、胰管结石2例（1例不伴胆管结石）。9例予内镜下胆管取石,2例胰管取石,术中置入胆道支架引流7例,行鼻胆管引流3例,胰管支架置入5例,胆道金属支架置人1例。术后临床症状均明显缓解。结论ERCP是一种可靠的诊断手段,其分型与PBM相关疾病表现有明显相关,选择性、暂时性的内镜治疗在外科术前是有效的、必要的。     

先天性胆胰管合流异常的内镜诊断与治疗

目的探讨ERCP对胆胰管合流异常（APBDJ）的诊断价值与临床处理原则。方法回顾分析近5年来接受ERCP诊疗的患者中,APBDJ的发生情况、分型特点、与合并胆胰疾病的关系,以及内镜下处理的情况。结果探讨ERCP对胆胰管合流异常（APBDJ）的诊断价值与临床处理原则。方法回顾分析近5年来接受ERCP诊疗的患者中,APBDJ的发生情况、分型特点、与合并胆胰疾病的关系,以及内镜下处理的情况。结论ERCP是APBDJ一种直接、可靠的诊断手段;APBDJ与胆囊癌、胆总管囊肿及慢性胰腺炎等症有非常密切的关联;APBDJ患者可根据合并症的具体情况,采取相应的内镜治疗措施。     

Precancerous conditions of biliary tract cancer in patients with pancreaticobiliary maljunction: reappraisal of nationwide survey in Japan

It is widely known that pancreaticobiliary maljunction (PBM), an anomalous arrangement of the pancreaticobiliary ductal system, is frequently associated with biliary tract cancer in patients with or without bile duct dilatation. In 1985, we surveyed patients with PBM who had been operated on at 133 Japanese institutions. A close relationship was shown between biliary tract carcinogenesis and PBM, according to the type of maljunction and age distribution: PBM patients with cystic dilatation had a high risk of bile duct cancer, even in those who were young (aged less than 20 years); the incidence of gallbladder cancer increased markedly in PBM patients over 40 years old with cystic dilatation, while it gradually increased with age in the PBM patients without cystic dilatation. Therefore, we recommend surgical treatment for patients with PBM even if they have no symptoms.     

Pancreaticobiliary reflux as a high-risk factor for biliary malignancy:Clinical features and diagnostic advancements

Pancreaticobiliary junction is composed of complex structure with which biliary duct and pancreatic duct assemble and go out into the ampulla of Vater during duodenum wall surrounding the sphincter of Oddi.Although the sphincter of Oddi functionally prevents the reflux of pancreatic juice,pancreaticobiliary reflux(PBR) occurs when function of the sphincter of Oddi halt.The anatomically abnormal junction is termed pancreaticobiliary maljunction(PBM) and is characterized by pancreatic and bile ducts joining outside of the duodenal wall.PBM is an important anatomical finding because many studies have revealed that biliary malignancies are related due to the carcinogenetic effect of the pancreatic back flow on the biliary mucosa.On the other hand,several studies have been published on the reflux of pancreatic juice into the bile duct without morphological PBM,and the correlation of such cases with biliary diseases,especially biliary malignancies,is drawing considerable attention.Although it has long been possible to diagnose PBM by various imaging modalities,PBR without PBM has remained difficult to assess.Therefore,the pathological features of PBR without PBM have not been yet fully elucidated.Lately,a new method of diagnosing PBR without PBM has appeared,and the features of PBR without PBM should soon be better understood.     

Ectopic pancreaticobiliary drainage accompanied by proximal jejunal adenoma： A case report

A patient with obstructive jaundice was examined by multidetector row helical computed tomography （MDCT） and magnetic resonance imaging （MRI）, and his common bile duct was observed to be leading into the distal portion of the horizontal duodenum with a pancreaticobiliary union outside the duodenal wall. A mass was also found in the proximal jejunum. All the above findings were confirmed by subsequent surgery, thus contrast-enhanced MDCT and MRI with appropriate image post-processing could provide non- invasive and accurate information regarding anatomy and lesions of the pancreaticobiliary duct and duodenal union, which may improve the feasibility of surgery and reduce postoperative complications,     

Importance of early diagnosis of pancreaticobiliary maljunction without biliary dilatation

AIM: To clarify the strategy for early diagnosis of pancreaticobiliary maljunction (PBM) without biliary dilatation and to pathologically examine gallbladder before cancer develops.METHODS: The anatomy of the union of the pancreatic and bile ducts was assessed by using endoscopic retrograde cholangiopancreatography (ERCP). Patients with a long common channel in which communication between the pancreatic and bile ducts was maintained even during sphincter contraction were diagnosed as having PBM. Of these, patients in which the maximal diameter of the bile duct was less than 10 mm were diagnosed with PBM without biliary dilatation. The process of diagnosing 54 patients with PBM without biliary dilatation was retrospectively investigated. Histopathological analysis of resected gallbladder specimens from 8 patients with PBM without biliary dilatation or cancer was conducted.RESULTS: Thirty-six PBM patients without biliary dilatation were diagnosed with gallbladder cancer after showing clinical symptoms such as abdominal or back pain (n = 16) or jaundice (n = 12). Radical surgery for gallbladder cancer was only possible in 11 patients (31%) and only 4 patients (11%) survived for 5 years. Eight patients were suspected as having PBM without biliary dilatation from the finding of gallbladder wall thickening on ultrasound and the diagnosis was confirmed by ERCP and/or magnetic resonance cholangiopancreatography (MRCP). The median age of these 8 patients was younger by a decade than PBM patients with gallbladder cancer. All 8 patients underwent prophylactic cholecystectomy and bile duct cancer has not occurred. Wall thickness and mucosal height of the 8 resected gallbladders were significantly greater than controls, and hyperplastic changes, hypertrophic muscular layer, subserosal fibrosis, and adenomyomatosis were detected in 7 (88%), 5 (63%), 7 (88%) and 5 (63%) patients, respectively. Ki-67 labeling index was high and K-ras mutation was detected in 3 of 6 patients.CONCLUSION: To detect PBM without biliary dilatation before onset of gallbladder cancer, we should perform MRCP for individuals showing increased gallbladder wall thickness on ultrasound.     

Association of gallbladder carcinoma and anomalous pancreaticobiliary ductal union

A total of 96 patients with gallbladder carcinoma in whom direct cholangiography clearly opacified the pancreaticobiliary ductal union and the common channel, and 65 patients with an anomalous union of these two duct systems at a distance greater than 15 mm from the papilla of Vater (normally less than 4.6 +/- 2.2 mm, mean +/- SD) were studied. It was found that this anomalous ductal union occurred in 16.7% of the patients with gallbladder carcinoma in comparison with an incidence of 2.8% among 641 consecutive patients with various hepatobiliary and pancreatic diseases studied by endoscopic retrograde cholangiopancreatography who did not have gallbladder carcinoma. It was also found that gallbladder carcinoma occurred in 24.6% of the 65 cases of anomalous ductal union in comparison with a 1.9% incidence of this cancer among 635 consecutive patients similarly studied and found to have normal ductal union (p less than 0.001). Thus, a close etiologic association was suggested between this anomaly in the terminal segment of the biliary tract and gallbladder carcinoma. Of the 65 patients with anomalous ductal union, 50 had the so-called congenital cystic dilatation of the common bile duct and 15 did not. Five of the 50 (10%) and 11 of the 15 (73.3%) had gallbladder carcinoma (p less than 0.01), and this carcinoma seems to be related to anomalous ductal union rather than to cystic dilatation of the common bile duct. As a tumorigenic factor in this anomaly, regurgitation of pancreatic juice has been stressed.     

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.     

Recent advances in pancreaticobiliary maljunction

The purpose of this review is to evaluate our current knowledge of the embryologic etiology of pancreaticobiliary maljunction (PBM), its diagnosis, clinical aspects, and treatment, and to clarify the mechanisms of PBM involvement in carcinogenesis. Although the embryologic etiology of PBM still awaits clarification, an arrest of the migration of the common duct of the biliary and pancreatic ducts inwards in the duodenal wall has hitherto been speculated to result in a long common channel in PBM. However, we propose the hypothesis that the etiology of PBM is caused by a disturbance in the embryonic connections (misarrangement) of the choledochopancreatic duct system in the extremely early embryo. That is, PBM is an anomaly caused by a misarrangement whereby the terminal bile duct joins with a branch of the ventral pancreatic duct system, including the main pancreatic duct. PBM is frequently associated with congenital bile duct cyst (CCBD). However, these two anomalies are thought to have different embryonic etiologies. The diagnostic criteria for PBM are the radiological and anatomical detection of the extramural location of the junction of the pancreatic and biliary ducts in the duodenal wall. However, in PBM patients with a short common duct (less than 1 cm in length), detection of the extramural location is difficult. The clinical features of PBM are intermittent abdominal pain, with or without elevation of pancreatic enzyme levels; and obstructive jaundice, with or without acute pancreatitis, while the clinical features of PBM patients with CCBD are primary bile duct stone and acute cholangitis. The optimum approach for the treatment of PBM is the prevention of the reciprocal reflux of bile and pancreatic juice in the pancreas and the bile duct system. To achieve these aims, the surgical approach is most effective, and complete biliary diversion procedures with bile duct resection (for example, choledochoduodenostomy or choledochojejunostomy of the Roux‐en‐Y type) are most useful. Recently, it has been recognized that the development of biliary ductal carcinoma is associated with PBM. That is, the development of gallbladder cancer occurs frequently in PBM patients without CCBD, and bile duct cancer originating from the cyst wall also occurs in PBM patients with CCBD. It is speculated that the pathogenesis of the bile duct or gallbladder cancer in PBM patients involves the reciprocal reflux of bile and pancreatic juice. Investigations of epithelial cell proliferation in the gallbladder of PBM patients, and of K‐ras mutations andp53 suppressor gene mutations, loss of heterozygosity ofp53, and overexpression of thep53 gene product in gallbladder cancer and noncancerous lesions in PBM patients have been carried out in various laboratories around the world. The results support the conclusion that PBM is a high risk factor for the development of bile duct carcinoma.     

Risk factor for extrahepatic bile duct cancer in patients with anomalous pancreaticobiliary ductal union

BACKGROUND/AIMS: Anomalous connection between the choledochus and pancreatic duct is considered to be a factor in the development of biliary tract diseases such as choledochal cyst, pancreatitis, cholangitis, gallbladder cancer, and bile duct cancer. Our purpose was analysis of combined disease, especially biliary neoplasm and evaluated microscopic changes of extrahepatic bile ducts. METHODOLOGY: To study the clinical characteristics of anomalous pancreaticobiliary ductal union (APBDU), we reviewed 14 APBDU cases from June 1994 to June 1998. We studied the associated disease, surgical treatment, and the histological findings of the extrahepatic bile ducts. RESULTS: Gallbladder cancer was identified in 5 out of 14 patients with APBDU. The incidences of metaplasia of gallbladder and bile duct with APBDU were higher than that of control gallbladder epithelium. The proliferating cell nuclear antigen-labeling index of the gallbladder in patients with APBDU was significantly higher than that in the control group. CONCLUSIONS: The patients with APBDU showed high incidence of gallbladder carcinoma and metaplasia in epithelium of gallbladder and bile duct. As this metaplasia in the gallbladder and bile duct is thought of as a precancerous condition, it is important to remove the place that causes bile stasis and to stop backflow of pancreatic juice into the bile duct in managing patients with this anomaly. In other words, prophylactic cholecystectomy and reconstruction of the biliary tract are both necessary.     

Biliary carcinogenesis in pancreaticobiliary maljunction

The coexistence of bile duct carcinoma in choledochal cysts is well known. With large number of cases of congenital bile duct dilatation and pancreaticobiliary maljunction (PBM), it is now apparent that gallbladder carcinoma is more frequent in PBM without bile duct dilatation. The incidence of Gallbladder cancer in choledochal cyst and bile duct cancer in PBM, regardless of the presence of bile duct dilatation is significantly higher than that in control patients with biliary cancer but without PBM. A recent survey shows that the incidence of bile duct cancer in PBM without dilatation is equivalent to that of gallbladder cancer and bile duct cancer in patients with choledochal dilatation. Pathology in PBM is a result of reflux of pancreatic juice and stasis of the mixture in the biliary system. Carcinogenic factors thus present in the biliary contents induce epithelial changes in the biliary tract. In the biliary contents, activated pancreatic enzymes and secondary or deconjugated bile acids are markedly increased and they irritate the biliary mucosa. Mutagens are proven to form in the bile of PBM. In the epithelia of PBM, hyperplasia, metaplasia, and dysplasia are often found, and proliferative activity is increased; furthermore, K-ras gene mutation and overexpression of p53 protein are demonstrated. As for treatment of PBM, whether it is symptomatic or not, an operative procedure, is necessary, to prevent carcinogenic changes in the gallbladder and bile duct. Regardless of whether dilatation is present or not, total excision of the extrahepatic bile duct, along with gallbladder, is the treatment of choice, followed by hepaticojejunostomy or hepaticoduodenostomy.     

Cancer of the extrahepatic biliary tracts and abnormality of the biliopancreatic junction

This retrospective study was undertaken to evaluate the incidence of an abnormal pancreaticobiliary ductal union (long common duct) in biliary tract carcinoma. Of 86 patients presenting with such a cancer, 67 had a pre- or intra-operative radiologic examination. An abnormal pancreaticobiliary ductal union was found in only one 60 year old woman having stones associated with invasive gallbladder carcinoma. Operative cholangiogram revealed an early and marked Wirsung opacification with a 1.5 cm long common duct. This low incidence in our study contrasts deeply with Japanese data and raises several questions about the correct evaluation of pancreaticobiliary ductal union, the true consequences of such an anomaly and possible interracial variations in incidence.     

Pancreas divisum in pancreaticobiliary maljunction

BACKGROUND/AIMS: Pancreaticobiliary maljunction (PBM) and pancreas divisum (PD) are congenital anomalies that develop in the embryo at an early stage. They are possibly the result of bile and pancreatic duct misarrangement. We investigated the configuration of the pancreatic duct in patients with PBM and its clinical implications. METHODOLOGY: In 84 PBM patients, the configuration of the pancreatic duct and the presence of biliary cancer were documented. Patency of Santorini's duct was determined fluoroscopically or by dye-injection endoscopic retrograde pancreatography. Bile amylase levels were measured in 10 patients. RESULTS: Incomplete PD was detected in 8 (9.5%) of the 84 PBM patients. All of the 8 patients had a patent Santorini's duct, and only 1 patient had gallbladder cancer. The frequency of associated gallbladder cancer and the bile amylase level were significantly lower in PBM patients with a patent Santorini's duct than in PBM patients with a nonpatent Santorini's duct. CONCLUSIONS: PBM is sometimes associated with incomplete PD. In PBM patients with an incomplete PD, the incidence of cancer of the biliary tract may be lower, since pancreatic juice reflux into the bile duct might be reduced by the flow of pancreatic juice into the duodenum through Santorini's duct.     

Biliary tract malignancy and abnormal pancreaticobiliary junction in a Western population

BACKGROUND AND AIM: Abnormal pancreaticobiliary junction is a rare congenital disorder in which the junction of biliary and pancreatic duct is located outside the duodenal wall and forms a long common channel. The disorder has been associated with a high incidence of gallbladder cancer in Japanese studies. The aim of this study was to assess the frequency of abnormal pancreaticobiliary junction in a Western population and its association with biliary tract malignancies. METHODS: All endoscopic retrograde cholangiopancreatography examinations of 82 consecutive patients with gallbladder carcinoma or biliary duct cancer treated at Baylor University during a period of 10 years were analyzed. A further 220 consecutive patients with normal films or non-malignant pancreaticobiliary disease acted as controls. RESULTS: Fifty-eight (70.7%) patients were found to have adequate imaging of junction and were included in the analysis. These included 37 patients with common bile duct carcinoma and 21 with gallbladder carcinoma. Abnormal pancreaticobiliary junction (common channel > or =8 mm) was observed in 44.8% patients with biliary tract carcinoma compared to 6.2% of controls (P < 0.01). Eighteen had type I abnormality and eight type II. Seven patients had a cystic dilatation of the common bile duct, all with type I abnormality. Mean overall survival was 9.5 months. Survival did not differ significantly between patients with normal and abnormal junctions (P = 0.1). CONCLUSIONS: The results suggest a close association between the anatomy of the distal ends of the common bile duct and main pancreatic duct and development of biliary tract carcinoma in Western populations.     

Endoscopic retrograde cholangiopancreatography in children with symptomatic pancreaticobiliary maljunction: A retrospective multicenter study

BACKGROUND Endoscopic retrograde cholangiopancreatography(ERCP) has been widely used in pediatric patients with cholangiopancreatic diseases.AIM To evaluate the efficacy, safety, and long-term follow-up results of ERCP in symptomatic pancreaticobiliary maljunction(PBM).METHODS A multicenter, retrospective study was conducted on 75 pediatric patients who were diagnosed with PBM and underwent therapeutic ERCP at three endoscopy centers between January 2008 and March 2019. They were divided into four PBM groups based on the fluoroscopy in ERCP. Their clinical characteristics, specific ERCP procedures, adverse events, and long-term follow-up results were retrospectively reviewed.RESULTS Totally, 112 ERCPs were performed on the 75 children with symptomatic PBM.Clinical manifestations included abdominal pain(62/75, 82.7%), vomiting(35/75,46.7%), acholic stool(4/75, 5.3%), fever(3/75, 4.0%), acute pancreatitis(47/75,62.7%), hyperbilirubinemia(13/75, 17.3%), and elevated liver enzymes(22/75,29.3%). ERCP interventions included endoscopic sphincterotomy, endoscopic retrograde biliary or pancreatic drainage, stone extraction, etc. Procedure-related complications were observed in 12 patients and included post-ERCP pancreatitis(9/75, 12.0%), gastrointestinal bleeding(1/75, 1.3%), and infection(2/75, 2.7%).During a mean follow-up period of 46 mo(range: 2 to 134 mo), ERCP therapy alleviated the biliary obstruction and reduced the incidence of pancreatitis. The overall effective rate of ERCP therapy was 82.4%; seven patients(9.3%) were lost to follow-up, eight(11.8%) re-experienced pancreatitis, and eleven(16.2%)underwent radical surgery, known as prophylactic excision of the extrahepatic bile duct and hepaticojejunostomy.CONCLUSION ERCP is a safe and effective treatment option to relieve biliary or pancreatic obstruction in symptomatic PBM, with the characteristics of minor trauma, fewer complications, and repeatability.     

胰胆管合流异常对胰腺损伤的实验研究

目的 探讨胰胆管合流异常(APBDU)对胰腺的损伤.方法 选择健康杂种猫10只,在胆管、胰管近十二指肠处纵行切开4～6 cm,然后间断吻合,以建立胰胆管合流异常的模型.手术造模时及术后6个月分别取胰腺组织行病理检查、电镜观察及胰腺组织丙二醛(MDA)水平检测.结果 7只猫生存6个月.术后6个月胰腺呈暗红色,充血、水肿,其中3只猫胰腺表面可见扩张的胰管.光镜下见3只猫胰腺有炎性细胞浸润,4只猫胰腺有间质血管增生、白细胞附壁.电镜下见胰腺腺泡细胞粗面内质网扩张,有的呈池状;线粒体数量增多、体积增大、崩解;高尔基复合体发达.手术前胰腺组织MDA水平为(1.23±0.7)nmol/mg prot,术后6个月升高达(2.90±1.9)nmol/mg prot,相差显著(F=4.80,P=0.0215).结论 胰胆管合流异常可导致胰腺损伤,抗氧化可能有助于减轻该损伤.     

Double cancer of gallbladder and bile duct associated with anomalous junction of the pancreaticobiliary ductal system

We report a case of double cancer of the gallbladder and the common bile duct associated with anomalous junction of the pancreaticobiliary ductal system, and review the literature of similar case reports. A 66-year-old woman was admitted to an associated hospital complaining of upper abdominal pain, and was diagnosed as having pancreatitis. Abdominal imaging revealed an irregularly protruding mass at the body of the gallbladder and an intraluminal protrusion at the lower third of the common bile duct. Endoscopic retrograde cholangiopancreatography also revealed anomalous junction of the pancreaticobiliary ductal system with congenital biliary dilatation of 14 mm in the largest diameter. She underwent surgical resection of the gallbladder, the extrahepatic bile duct and the gallbladder bed of the liver with a dissection of the regional lymph nodes for double cancer of the gallbladder and the bile duct associated with anomalous junction of the pancreaticobiliary ductal system. She is still alive 33 months after surgery without any signs of recurrence. There were 12 patients (including our case) reported in the literature who had double cancer of the gallbladder and the extrahepatic bile duct associated with anomalous junction of the pancreaticobiliary ductal system. Only 33% of these 12 patients had jaundice. Tumors of the 12 patients were commonly early-stage cancer both in the gallbladder (36%) and in the extrahepatic bile duct (73%). Therefore, we concluded that precise preoperative imaging of the total biliary tract should be required in order to detect early-stage cancer in patients with anomalous junction of the pancreaticobiliary ductal system before planning surgical procedures, and consideration should be given to the possibility of multiple occurrences of biliary tract cancers.     

Recent advances and problems in the management of pancreaticobiliary maljunction: feedback from the guidelines committee

Clinical practice guidelines on how to deal with pancreaticobiliary maljunction (PBM) were made in Japan in 2012, representing a world first. Using a narrow definition, congenital biliary dilatation involves only Todani type I (except type Ib) and type IV‐A, both of which are accompanied by PBM in almost all cases. Prospective ultrasonographic study revealed that the maximum diameter of the common bile duct increased with age. Pathophysiological conditions due to pancreatobiliary reflux occur in patients with high confluence of the pancreaticobiliary ducts, a common channel ≥6 mm long and occlusion of communication during contraction of the sphincter of Oddi. Since PBM can be diagnosed by magnetic resonance cholangiopancreatography, multi‐planar reconstruction multi‐detector row computed tomography and endoscopic ultrasonography, the current diagnostic criteria should be revised to take these diagnostic imaging modalities into consideration. According to a nationwide survey, biliary cancer occurred in 21.6% of adult patients with PBM with biliary dilatation and 42.2% of patients with PBM without biliary dilatation. In biliary cancer associated with PBM without biliary dilatation, 88.1% were gallbladder cancer. Treatment for PBM with biliary dilatation is prophylactic flow‐diversion surgery, but further investigations and surveillance studies are needed to clarify the appropriate surgical strategy for PBM without biliary dilatation.