胰胆管汇合异常病理解剖与胰胆系疾病关系研究

多层螺旋CT及图像后处理技术、MRI等的广泛应用,为胰胆管汇合部解剖学研究提供了重要手段.通过胰胆管汇合异常与胆道系统病变关系研究,可以提高对胆道系统病的发病机理、病理过程的认识,为预防性治疗提供依据.本文对胰胆管汇合异常的定义、分型、病理解剖及其与胆系疾病的关系、临床治疗进行综述.     

胰胆管汇合异常的诊疗进展

胰胆管汇合异常(PBM)为胰胆管在十二指肠壁外汇合的先天性疾病。PBM的临床症状不典型, 往往被一系列的胆胰疾病所掩盖;PBM也因反复发作的胰腺炎、胆管结石等胆胰疾病及胆道系统的恶性肿瘤而逐渐被关注。随着内镜微创技术的创新发展, 内镜微创治疗手段或许可以为PBM患者提供新的治疗方法和思路, 有望达到内镜微创介入纠正胰胆管汇合部结构异常的目标。本文就PBM的定义、内镜诊断、发病机制及内镜治疗进展等作一综述。     

胰胆管合流异常的病理特征与诊治进展

胰胆管合流异常 (pancreaticobiliary maljunction,PBM)是胰管和胆总管异常汇合的一种先天性畸形 [1 ]。从 1916年 Kizu-mi首先提出 PBM这一概念 ,至 1978年日本学者因此成立“胰胆管合流异常研究会”,PBM已日渐引起国内外广泛关注 ,对PBM基础及临床的研究正逐步深入。1　PBM的病理特征约 75 %的正常人胆总管与主胰管在十二指肠粘膜下汇合成共通管 ,并随着年龄逐渐增长。共通管周围及近端具有 Oddi氏括约肌 ,其中胆总管括约肌部分恒定存在 ,在神经与体液双重因素的调节下可控制胆汁的正常排出 ,亦可防止胰液逆流入胆管。据 1991年日…     

胆管非扩张型胰胆管合流异常诊治的进展

胆管非扩张型胰胆管合流异常 ,是否易引起胆管癌及胆囊癌 ?治疗上是否需要切除胆管、重建胆道的分流术 ?目前无统一的观点。笔者结合近年来的有关文献 ,就胆管非扩张型胰胆管合流异常诊治的进展做一综述。     

胰胆管合流异常与胰胆管疾病的关系

自Babbitt[1]1969年首次报道胰胆管合流异常(anomalouspancreaticobiliary ductal uion,APBDU)以来,人们发现APBDU与许多胆道及胰腺疾病密切相关。近年来,随着MRCP、ER-CP、PTC等影像学技术的发展和广泛应用,有关的报道逐渐增多[2],对APBDU又有了更进一步的认识。APBDU的基本概念解剖学上APBDU是指胰胆管共同通道在十二指肠壁外合流,造成共同通道过长,使十二指肠乳头括约肌的作用不能影响到整个合流部,胆汁、胰液相互逆流而引起胆道及胰腺的各种疾病。APBDU分为两型:①P-C型,即胰管注入胆总管型;②C-P型,即胆总管注入胰…     

胰胆管异常连接

胰胆管异常连接 (ａｎｏｍａｌｏｕｓｐａｎｃｒｅ ａｔｉｃｏｂｉｌｉａｒｙｄｕｃｔａｌｕｎｉｏｎ ,ＡＰＢＤＵ )是指主胰管和肝外胆管在十二指肠壁外提前汇合 ,胰胆共同管延长 ,失去了Ｏｄｄｉ括约肌的调控 ,引起胆汁和胰液混流 ,继而导致一系列胆系胰腺疾病乃至癌变 ,是一种先天性畸形 ,其发病率为 1.5 %～3.2 % [1] 。一般而言 ,正常人胰胆共同管长约 1～ 12ｍｍ (平均 4.5ｍｍ ) [2 ] 。如成人共同管长于 10ｍｍ ,小儿共同管长于 4ｍｍ ,即可认为存在着胰胆管异常连接[3] 。目前研究发现 ,胰胆管异常连接是一种病因学因素 ,常易合并…     

胆囊管异常汇合的诊断及其在腹腔镜胆囊切除术中的意义

目的 分析逆行胰胆管造影术（ERCP）在诊断胆囊管异常汇合的价值及其在预防腹腔镜胆囊切除术（LC）胆管损伤等并发症中的作用。方法 我院从1992年7月至1999年6月共行LC4 500余例,其中对780例LC患者术前行ERCP检查。选择胆囊管、肝总管和胆总管三者解剖关系均清楚的772例ERCP片,观测其胆囊管异常汇合情况,测量其长度和直径,并与100例胰胆管无异常的ERCP片对比。结果 在780例ERCP检查者中,发现胆囊管异常汇合者125例（16.03%）。该780例行LC中,中转开腹胆囊切除术35例（4.49%）,发生各种并发症6例（0.77%）。但无胆管损伤等严重并发症发生。结论 ERCP能准确诊断胆囊管的各种异常汇合,在预防LC胆管损伤等胆道并发症的发生中起重要作用。     

胆胰管异常汇合的诊断与治疗

胆胰管异常汇合的诊断与治疗刘胜利，陈峰，杨小庆，杨德同本文对我院发现的２７例胆胰管异常汇合（ａｎｏｍａ－ｌｏｕｓｊｕｎｃｔｉｏｎｏｆｐａｎｃｒｅａｔｉｃｏｂｉｌｉａｒｙｄｕｃｔ，简称ＡＪＰＢＤ）作了回顾性研究，并探讨其诊断和外科治疗的意义。１．临床资...     

胰胆管汇合异常的研究进展

胰胆管汇合异常(pancreaticobiliarymaljunction,PBM)是指在解剖学上胰胆管共同开口于十二指肠乳头之前,形成一过长的共同管,在十二指肠外汇合的一种先天性畸形[1]。在行ERCP的病人中,PBM的发现率约在1.56%~4.5%之间,男女比例约为1∶3,东方多于西方。PBM虽然是一种少见的先天性畸形,但可以导致多种胆胰疾病。一、PBM的诊断标准和分型1991年,日本胰胆管汇合异常学会提出的PBM诊断标准为:胆管与胰管在十二指肠壁外汇合;共同管大于15mm。某些后天因素如十二指肠乳头炎、胆管或者胰管结石、肿瘤等引起的异常不在此列。对于共同通道的长…     

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…     

肌球蛋白在胰胆管合流异常患儿胆总管平滑肌中表达的改变

目的:观察胰胆管合流异常(PBM)患儿胆总管平滑肌肌球蛋白重链(MHC)与磷酸化肌球蛋白20 kD轻链(P-MLC20)表达的情况.方法:收集16例儿童PBM胆总管标本,以16例新生儿尸体胆总管标本为对照,免疫组化染色后用图像分析系统检测分析两种组织中MHC和P-MLC20表达;用Western blot法检测两种组织中P-MLC20的蛋白表达.结果:MHC与P-MLC20蛋白表达免疫组化图像分析系统定量分析结果显示,除两者的平均光密度(MOD)组间无差异外(均P＞0.05),PBM组两者的平均阳性面积比例(MLI)与阳性染色得分(MQS)均明显高于对照组(均P＜0.05);Western blot显示PBM组中P-MLC20蛋白表达量较对照组明显增高(P＜0.05).结论:PBM患儿胆总管平滑肌MHC与P-MLC20蛋白表达增高,该改变可能导致了胆总管平滑肌的收缩力代偿性增加.     

胰胆管合流异常的诊断进展

胰胆管合流异常(pancreaticobiliary maljunction,PBM)在临床上与许多外科疾病关系密切.然而,PBM的诊断却是一个相当困难的临床问题,容易发生漏诊、误诊.临床外科医师对于PBM的认识还不够充分,没有引起高度的重视,一度使PBM成为“腹部外科被遗忘的角落”.这使PBM的诊断变得更加困难.本文就PBM的临床影像学检查现状做一综述.     

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     

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     

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.     

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.     

Telomerase activity and Bcl-2 expression in gallbladders of pancreaticobiliary maljunction patients: a preliminary study

Background The congenital anomaly pancreaticobiliary maljunction (PBM) is considered to be a precancerous disease. PBM carcinogenesis is believed to be an accumulation of gene abnormalities, but the early events causing PBM carcinogenesis are still unclear. In the present study, telomerase activity and Bcl-2 expression in the gallbladder mucosa of PBM and non-PBM gallbladders were investigated.Methods The operative gallbladder materials were from five control cases, two cases of non-PBM gallbladder cancer, three of PBM gallbladder cancer, and three of non-neoplastic PBMs. Multiple sampling was performed from each gallbladder. The studies performed were: (1) immunohistochemistry of p53, Ki-67, and Bcl-2; (2) survey of k-ras point mutations; and (3) measurement of telomerase activity in each sample.Results In the cases of non-PBM cancer, abnormalities from the above studies were detected only in the cancerous lesions. Normal-appearing mucosa did not show Bcl-2 expression or telomerase activity. However, in the cases of PBM cancer, normal-appearing mucosa showed telomerase activity and Bcl-2 expression, but did not show p53, Ki-67, or k-ras abnormalities. In the non-neoplastic PBM, all samples showed Bcl-2 expression, and many showed telomerase activity.Conclusions Bcl-2 expression and activation of telomerase are probably early events causing carcinogenesis of the PBM gallbladder mucosa. They might be important factors causing carcinogenesis associated with chronic inflammation.     

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     

胰胆管合流异常与先天性胆总管囊肿

目的:探讨胰胆管合流异常(PBM)致先天性胆总管囊肿(CCC)的可能机制。方法:将21例经B超诊断为胆总管囊肿患者行ERCP检查，检查时经选择性胆管插管抽取胆汁检测其中淀粉酶(AMS)及C反应蛋白(CRP)浓度。结果: 21例PBM均合并CCC，其中13例为胆管合流型(C-P型)，8例为胰管合流型(P-C型) PBM。21例胆汁中AMS及CRP浓度均较对照组显著升高(P<0.01)，C-P型较P-C型升高更明显(P<0.05)。 结论:PBM可能是CCC形成的解剖学基础，胰液返流至胆管，被激活的高浓度胰酶破坏胆管壁，则可能是CCC形成的促发因素。     

Diagnostic impact of computed tomography cholangiography and magnetic resonance cholangiopancreatography on pancreaticobiliary maljunction

Background/Purpose

The aim of this study was to investigate the diagnostic potential of computed tomography cholangiography (CTC) and magnetic resonance cholangiopancreatography (MRCP) in children with pancreaticobiliary maljunction (PBM).

Methods

Fifty-three children with PBM were consecutively treated between 1997 and 2009. Among them, the patients who underwent CTC and/or MRCP preoperatively were enrolled in this study. Computed tomography cholangiography was examined after infusion of meglumine iodoxamate with subsequent 3-dimensional rendering. The visualization of the biliary and pancreatic duct systems was evaluated and compared with that visualized with MRCP. The findings of direct cholangiography were used as the standard of reference.

Results

Of the 53 cases with PBM, 17 cases were examined by CTC, 10 cases by MRCP, and 17 with both. The extrahepatic bile tract was visualized in 32 (94.1%) of 34 patients in CTC and in all 27 patients in MRCP. The intrahepatic bile duct was more frequently demonstrated by MRCP than by CTC (96.3% vs 70.6%, P = .02). Pancreaticobiliary maljunction was noted in 13 (38.2%) of 34 with CTC and in 12 (44.4%) of 27 with MRCP. The minimum age for visualization of PBM was at 10 months in CTC and at 1 year and 11 months in MRCP, respectively. The main pancreatic duct was more frequently visualized by MRCP than by CTC (81.5% vs 8.8%, P < .001).

Conclusions

Magnetic resonance cholangiopancreatography provides superior visualization of the intrahepatic duct and the pancreatic system when compared with CTC. However, it is still challenging to perform a good-quality examination in young infant. The great advantage of CTC is its ability to produce high-quality images without respiratory artifacts and that it allows accurate assessment of the presence of PBM equivalent to MRCP.