急性胆源性胰腺炎和胰胆管合流异常的关系

目的 探讨急性胆源性胰腺炎(ABP)和胰胆管合流异常(APBDU)的关系.方法 选择ABP手术患者165例.发病时抽取静脉血栓测总胆红素(TB)、丙氨酸氡基转移酶(ALT)、天冬氨酸氨基转移酶(AST)、碱性磷酸酶(ALP),γ-谷氨酰转移酶(GGT),保守治疗后复查;术中胆道造影观察胰胆管合流情况.结果 165例胆道造影患者中,发现APBDU 41例.保守治疗后APBDU和NAPBDU组的TB、ALT、AST、ALP、GGT都明显降低,有统计学意义(P<0.05).治疗后TB、ALP都恢复正常.APBDU组仍高于NAPBDU组,但没有统计学意义(P>0.05);APBDU组的ALT、AST和GGT都高于NAPBDU组,有统计学意义(P<0.05).结论 APBDU是导致ABP的重要原因之一.     

磁共振胰胆管成像在胆源性胰腺炎中的价值

目的：评价磁共振胰胆管成像（MRCP）在胆源性胰腺炎中的应用价值。方法：对21例胆源性胰腺炎患者行MRCP检查，并对照手术结果进行分析。结果：MRCP对胆源性胰腺炎、胆道结石诊断准确率为95．2％，此技术可清楚显示胰胆管的形态。结论：MRCP可无创性地显示胆道系统，有助于胆源性胰腺炎是否行胆总管探查的决定。     

胰胆管合流异常与胆道肿瘤

胰胆管合流异常(anomalous Dancreaticobiliary ductal union，APBDU)于1969年由Babbitt等^[1]首次报道，随着内镜逆行性胆管胰管造影术(endoscopic retrograde cholangiopancreatography，ERCP)和磁共振胰胆管成像(magnetic resonance cholangiography，MRCP)等影像学检查的广泛应用，APBDU检出率有增多趋势。APBDU与     

胰胆管合流异常三例

胰胆管汇合异常是指胆总管与主胰管在十二指肠壁外汇合的一种少见的解剖异常 ,可引起许多胆胰系统疾病。我院 2 8例术后胆道造影患者中 ,有 8例出现主胰管显影 ,其中 3例清晰显示胆总管与主胰管在十二指肠壁外汇合 ,现对其作一报道。病历摘要例 1:男 ,5 7岁。无痛性黄疸 10ｄ以“肝门部胆管癌”住院。术中见 :肿瘤位于肝门部 ,直径 4ｃｍ ,固定。以胆道探子扩张右肝管 ,置入 6ｃｍ长镍钛记忆合金胆道支架 ,胆总管内置 1根引流管 ,肿瘤表面以钛夹标志。术后 10ｄ经胆管引流管行胆道造影 ,见胆总管直径 0 .8ｃｍ ,肝内胆管直径 1.0ｃｍ ,主胰…     

磁共振胰胆管成像对胆源性胰腺炎胆道病变的诊断价值

目的 评价磁共振胰胆管成像(MRCP)对胆源性胰腺炎胆道病变的诊断价值。方法 25例胆源性胰腺炎患者，临床检查发现总胆红素、ALT升高及部分患者B超检查提示胆总管扩张，疑有胆总管病变，施行MRCP检查。结果 19例发现有胆道病变，诊断率达76％(19／25)，明显高于B超的36％(9／25)。结论 在胆源性胰腺炎后行胆囊切除术前行MRCP检查，可避免胆总管结石的遗漏或不必要的胆总管探查。     

先天性胰胆管合流异常20例报告

胰胆管合流异常国内极少报告，临床上常被误诊或漏诊。为提高对本病的诊治水平，现就我院１９８５年以来收治的２０例总结报告如下。１临床资料本组２０例先天性胰胆管合流异常患者中男８例，女１２例。年龄５个月～２４岁，平均７岁零８个月。主要症状有腹痛、发烧、黄疸...     

急性胆源性胰腺炎

胆道与胰腺的解剖与功能互有联系 ,急性胰腺炎的发病胆道因素占有很大比例。在国外以胆囊结石、继发性胆管结石特别是小结石的自然排石过程常是其发生和复发的直接原因 ,因而将此统称之为胆石性胰腺炎。在我国原发性胆管结石、胆道蛔虫症及胆道感染等因素也占重要比重 ,因而较之更为复杂 ,所以称胆源性胰腺炎较为合理。胆道结石的嵌顿、梗阻、排石过程和胆道蛔虫感染这些刺激对壶腹括约肌的损伤引起胰胆出口的痉挛、充血、水肿 ,都导致胆胰管排空不畅和压力升高 ,加上胆胰结合部的不同方式 ,易致胆汁向胰液的返流 ,致使部分病人发生急性胰腺炎或慢性胰腺炎的反复急性发作 ,其中 ,胆胰管出口的排空不畅处于中心的地位 ,并易导致种种并发症的发生。内镜乳头切开引流常是有用的手段。如何认识、诊断和及时进行有效治疗 ,是应该进行充分讨论和达到共识的。现谨约请有关专家就此进行评述。———编者     

胰胆管合流异常合并胆道肿瘤的诊断与治疗

目的 阐述胰胆管合流异常这种解剖学异常的临床分型与其所导致胆道肿瘤的关系，探讨如何选择适当的、合理的手术治疗。方法 回顾性分析我院自1979年12月至2001年10月间所收治的64例胰胆管合流异常症病人的临床表现、影像学特点和分型与其合并胆道肿瘤的关系。结果 本组病人中：胰管型(P-C型)28例，合并胆道肿瘤11例；胆管型(C-P型)32例，合并胆道肿瘤8例；共同通道型4例，胆道肿瘤2例。结论 胰胆管合流异常同胆道肿瘤的发生关系密切。胰、胆管合流异常病人的临床分型不同，所导致的胆道肿瘤发生的可能性不同。     

急性胆源性胰腺炎诊治体会

急性胆源性胰腺炎（acute biliary pancreatitis．ABP）是指因各种胆道疾病诱发的急性胰腺炎。目前发病机制尚不明确，其诊断和治疗亦存在争议。2000年1月-2006年12月我科共收治ABP患者116例，报道如下。     

Advanced bile duct carcinoma in a 15-year-old patient with pancreaticobiliary maljunction and congenital biliary cystic disease

We report a case of advanced bile duct carcinoma arising in a 15-year-old female with pancreaticobiliary maljunction and congenital biliary cystic disease. Pancreaticoduodenectomy and partial resection of the liver was performed. Surgical and histopathological findings indicated advanced tubular adenocarcinoma, classified as final stage IVb according to the General rules for surgical and pathological studies on cancer of the biliary tract proposed by the Japanese Society of Biliary Surgery, 5th edition, and stage IV according to the American Joint Committee on Cancer (AJCC)/International Union Against Cancer (UICC), 6th edition. She underwent chemotherapy with gemcitabine HCl after discharge. She died of cachexia 14 months after the surgery. Although it is well known that biliary malignancies arise frequently in patients with pancreaticobiliary maljunction, it is uncommon for advanced bile duct carcinoma to occur in a 15-year-old female. We should pay attention to the possibility of biliary malignancy in patients with pancreaticobiliary maljunction and congenital biliary cystic disease, even when the patients are juveniles.     

Endoscopic sphincterotomy is a useful preoperative management for refractory pancreatitis associated with pancreaticobiliary maljunction

Background

Pancreatitis associated with pancreaticobiliary maljunction (PBM) is commonly treated nonoperatively before surgery. It is, however, sometimes uncontrollable, and little has been reported about the management.

Methods

Focusing on the preoperative management, we reviewed clinical courses of 4 PBM cases (ages 1 to 7 years old). Each had pancreatitis that was totally resistant to medical treatment and was applied endoscopic sphincterotomy (ES).

Results

The first case underwent percutaneous transhepatic catheter drainage (PTCD) primarily. In spite of daily lavage using the drainage tube for a week, plugs located in the common channel were not removed, and clinical findings were not improved. Therefore, ES followed by removal of protein plugs was performed to improve pancreatitis dramatically. Through this experience, 3 subsequent cases with refractory pancreatitis all underwent successful ES primarily soon after the medical treatments turned out to be ineffective. In all 4 cases, protein plugs were impacted in common channels, and ES could successfully remove the plugs that were impossible to remove by using PTCD. Improved preoperative pancreaticobiliary decompression by ES shortens the duration of recalcitrant acute pancreatitis associated with PBM allowing for a subsequent safe operation.

Conclusions

Endoscopic sphincterotomy is one of the useful preoperative managements for refractory pancreatitis associated with PBM.     

胰胆管合流异常合并胆囊床迷走胆管致术后胆汁漏

LG是治疗胆囊结石和慢性胆囊炎的有效方法。回顾性分析2012年8月解放军第八十八医院收治的1例因胰胆管合流异常以及胆囊床迷走胆管致LC术后胆汁漏、腹腔感染患者的临床资料,旨在探讨比类疾病的正确诊断和治疗方法。     

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 and p53 suppressor gene mutations, loss of heterozygosity of p53, and overexpression of the p53 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. Received: March 12, 2001 / Accepted: April 16, 2001     

Anomalous biliary drainage associated with pancreaticobiliary maljunction and nondilatation of the common bile duct

A case report is presented of an infant with jejunal atresia and hyperbilirubinemia with diagnosed pancreaticobiliary maljunction without choledochal dilatation. Common channel insertion was into the third portion of the duodenum.     

Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy

Background  It is widely accepted that congenital choledochal cyst is associated with pancreaticobiliary maljunction (PBM). But, PBM is an independent disease entity from choledochal cyst. PBM is synonymous with “abnormal junction of the pancreaticobiliary ductal system”, “anomalous arrangement of pancreaticobiliary ducts”, “anomalous union of bilio-pancreatic ducts”, etc. Cases with PBM not associated with biliary duct dilatation are often found, and these cases are frequently complicated gallbladder cancer. The Japanese Study Group of Pancreaticobiliary Maljunction was started in 1983, and defined diagnostic criteria and nationwide registration system of PBM cases was started. PBM is defined as a union of the pancreatic and biliary ducts which is located outside the duodenal wall. Bile and pancreatic juice reflux and regurgitate mutually. Biliary carcinogenesis  The most bothersome problem is biliary carcinogenesis. Gallbladder cancers arise in 14.8% and bile duct cancers arise in 4.9%. The incidence of the gallbladder carcinoma of PBM without bile duct dilatation is 36.1%. Many investigators have tried to clarify the carcinogenic process, from various aspects. The biliary epithelia are injured by harmful substances, and in the course of repair, multiple alterations of oncogenes and tumor suppressor genes are followed, and they lead to carcinoma through multistage interaction. In the biliary epithelia of PBM, incidence and degree of hyperplasia are characteristic. K-ras gene mutations are observed in the cancerous as well as noncancerous lesions of biliary tract of PBM patients. Mutations of p53 gene and overexpression of p53 protein are also found in the cancerous and noncancerous lesions. These changes are called “hyperplasia–carcinoma sequence”. Treatment  Total excision of the extrahepatic bile duct with gallbladder followed by hepaticojejunostomy, Roux-en-Y, or end-to-side hepaticoduodenostomy are treatment of choice, even for cases with not dilated bile duct, because the incidence of cancer in the nondilated bile duct is not negligible, and genetic changes are seen in a nondilated bile duct.     

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. Received: May 1, 2000 / Accepted: September 5, 2000     

Spontaneous necrosis of gallbladder carcinoma in patient with pancreaticobiliary maljunction

While gallbladder carcinoma is occasionally associated with pancreaticobiliary maljunction, spontaneous necrosis of carcinoma is extremely rare. We herein present a case of spontaneous necrosis of gallbladder carcinoma associated with direct invasion of viable cancer cell nests to the muscularis propria and subserosal layer located beneath the primary nodules. A 65-year-old Japanese man was admitted to a local hospital, complaining of repeated discomfort in the right hypochondrium. Ultrasonography and computed tomography scanning revealed cholecystitis associated with gallstones. Cholecystectomy was performed, and operative cholangiography demonstrated pancreaticobiliary maljunction. The resected gallbladder showed multiple mixed stones filled with necrotic debris and bile sludge. Scrutiny of the mucosal surface revealed multiple small necrotic nodules in the fundus, which were histologically confirmed to be necrotic remnants of a cancerous glandular structure. Small nests of papillary adenocarcinoma were found beneath the nodules in the muscularis propria and in the venous structure located in the connective tissues next to the divided margin of the gallbladder bed. Resection of S4a and S5 of the liver and resection of the extrahepatic bile duct was then performed to remove the remaining cancerous tissues and/or micrometastasis in the liver and bile duct. The biliary tree was reconstructed with a hepaticoduodenostomy. No cancer nests or any precancerous lesions were found in the additionally resected specimens. This case indicates a unique morphological feature of gallbladder carcinoma associated with pancreaticobiliary maljunction, which provides some insight into the pathogenesis of spontaneous necrosis of gallbladder carcinoma. Received: August 8, 2000 / Accepted: October 26, 2000     

K-ras and p53 gene mutations in noncancerous biliary lesions of patients with pancreaticobiliary maljunction

Background/Purpose: We investigated the molecular mechanisms of carcinogenesis in the biliary epithelium in patients with pancreaticobiliary maljunction. Methods: Point mutations of the K-ras gene and the p53 gene, and the overexpression of p53 gene products were examined in the cancerous and noncancerous biliary epithelium of 37 patients with pancreaticobiliary maljunction, with or without biliary dilatation. Results: In the gallbladder epithelium of 5 patients with pancreaticobiliary maljunction associated with biliary carcinoma, K-ras gene mutations were detected in 3 (60%), p53 gene mutations in 3 (60%), and the overexpression of p53 gene products in 4 (80%), while in the bile duct epithelium of these patients, these features were found in 2 of 3 (66.7%), in all of 3 (100%), and none of 3 (0%) specimens, respectively. In the gallbladder epithelium of patients with pancreaticobiliary maljunction without biliary carcinoma, K-ras gene mutations were detected in 8 of 24 (33.3%) specimens, p53 gene mutations were detected in 16 of 27 specimens (59.3%), and the overexpression of p53 protein was detected in 5 of 27 (18.5%) specimens, while in the bile duct epithelium of these patients, these features were found in 10 of 25 (40%) specimens, 14 of 25 (56%) specimens, and 6 of 24 (25%) specimens, respectively. Conclusions: These results suggest that noncancerous lesions of the biliary epithelium in patients with pancreaticobiliary maljunction have mutations of the K-ras gene and/or the p53 gene, which provides genetic evidence that biliary epithelium has high carcinogenic potential. Received: June 7, 2001 / Accepted: August 21, 2001     

Metachronous double cancer of the gallbladder and pancreas associated with pancreaticobiliary maljunction

A 50-year-old Japanese woman complained of abdominal and back pain. Ten years previously she had undergone cholecystectomy, choledochectomy, and Roux-en-Y choledochojejunostomy for gallbladder cancer associated with pancreaticobiliary maljunction without bile duct dilatation. On the present admission, ultrasonography (US) and computed tomography (CT) demonstrated a large mass, 60 mm in size, in the pancreatic tail. Endoscopic retrograde cholangiopancreatography (ERCP) showed obstruction of the main pancreatic duct in the tail of the pancreas and revealed that the pancreatic duct was joined to the bile duct 25 mm above the papilla of Vater. The patient underwent distal pancreatectomy, splenectomy, left adrenalectomy, and partial gastrectomy. Histological examination revealed moderately differentiated ductal adenocarcinoma that had invaded to the proper muscle of the stomach. Double cancer of the gallbladder and pancreas in a patient with pancreaticobiliary maljunction is rare. Although the etiology of cancer of the pancreas associated with pancreaticobiliary maljunction is unclear, we should pay close attention to the pancreas as well as the biliary tract during the long-term follow-up of patients with pancreaticobiliary maljunction after they have undergone a choledochojejunostomy.