Experimental production of cystic dilatation of the common bile duct in neonatal lambs

A reliable experimental model for the study of cystic dilatation of the extrahepatic biliary system has been produced purely by ligation of the distal end of the common bile duct in the newborn lamb. Six of nine experimental lambs surviving the immediate postoperative period lived for periods ranging from 27 to 40 days. All six developed cystic dilatation of the common bile duct.     

Morphometrical evaluation of the extrahepatic bile ducts in reference to their structural changes with aging

Forty-three autopsy cases in which history of biliary diseases had not been demonstrated were submitted to histometrical evaluation of the inner diameter of common hepatic duct. In no case was there any postmortem evidence for hepatic or pancreatic diseases. The diameter D was found to be larger in aged individuals and there was a highly significant correlation between D and age. An upper rejection limit was calculated at 5% level based on the regression line of diameter against age, showing that the diameter did not exceed 4.8mm during the first two decades but the upper limit was elevated to 6.1mm and 7.5mm at 50 and 80 years respectively. It is thus necessary to take into consideration the age of the patient when we attempt to judge clinically whether or not bile ducts actually are dilated. The density in ductal wall of elastic fibers was determined by microscopic morphometry and was shown to correlate also well with both the ductal diameter and the age of patient. The dilatation of bile duct with aging appeared to be attributable to chronic tension load on its wall, which was responsible for hysteresis of mural connective tissues on one hand, and on the other, for reinforcement of ductal wall by compensatory formation of elastic fibers.     

Congenital bile duct dilation —Possibly an hereditary condition—

A female patient underwent resection of a large choledochal cyst and hepaticojejunostomy when she was 11 years old at Tokyo Medical and Dental University Hospital. Her mother, 48 years old, also had congenital bile duct dilatation (CBD) with cancer. The mother had an anomalous pancreatico-biliary ductal junction. To investigate the possibility of a genetic link, family members of another three consecutive CBD cases were examined for bile duct dilatation and anomalous pancreatico-biliary junction. Endoscopic retrograde choledocho-pancreatography (ERCP) revealed an anomalous pancreatico-biliary ductal junction, without bile duct dilatation in the mother of a 17-year-old CBD patient. Two familial occurrences of CBD had been reported in the Japanese literature. Their combinations were; a father—a daughter and two sisters, respectively. Considering that the sex ratio of CBD is one male to 2.6 females, our results suggest that CBD or anomalous pancreatico-biliary ductal junction may be 1) an X-linked dominant trait or 2) autosomal dominant trait with relatively low penetrance in males. Since anomalous pancreatico-biliary ductal junction does not necessarily develop into bile duct dilatation, more familial investigations on pancreatico-biliary ductal junction and bile duct dilatation are essential to clarify the genetic contribution to CBD.     

肝外胆管梗阻性疾病的MRCP诊断价值

目的探讨磁共振胆胰管成像技术对肝外胆管梗阻性疾病的诊断价值。方法对52例经病理或随访证实的肝外胆管梗阻性疾病患者的MRCP资料进行回顾性分析,总结良恶性肝外胆管梗阻的不同MRCP表现。结果52例中,MRCP均能够准确测定胆管扩张程度和梗阻的水平,其中良性梗阻27例,MRCP主要表现为胆管均匀扩张及逐渐狭窄,肝外胆管较肝内胆管扩张明显,肝内胆管呈“枯树枝状”;恶性梗阻25例,MRCP主要表现为胆管截断,胆胰管扩张,出现“双管征”,肝内外胆管扩张一致。恶性梗阻患者的肝内外胆管扩张程度明显大于良性梗阻者。结论MRCP作为一种无创的影像检查方法,对肝外胆管梗阻性疾病的诊断具有较高准确性,     

Congenital bile duct dilatation--possibly an hereditary condition

A female patient underwent resection of a large choledochal cyst and hepaticojejunostomy when she was 11 years old at Tokyo Medical and Dental University Hospital. Her mother, 48 years old, also had congenital bile duct dilatation (CBD) with cancer. The mother had an anomalous pancreatico-biliary ductal junction. To investigate the possibility of a genetic link, family members of another three consecutive CBD cases were examined for bile duct dilatation and anomalous pancreatico-biliary junction. Endoscopic retrograde choledocho-pancreatography (ERCP) revealed an anomalous pancreatico-biliary ductal junction, without bile duct dilatation in the mother of a 17-year-old CBD patient. Two familial occurrences of CBD had been reported in the Japanese literature. Their combinations were; a father--a daughter and two sisters, respectively. Considering that the sex ratio of CBD is one male to 2.6 females, our results suggest that CBD or anomalous pancreatico-biliary ductal junction may be 1) an X-linked dominant trait or 2) autosomal dominant trait with relatively low penetrance in males. Since anomalous pancreatico-biliary ductal junction does not necessarily develop into bile duct dilatation, more familial investigations on pancreatico-biliary ductal junction and bile duct dilatation are essential to clarify the genetic contribution to CBD.     

胆总管继发结石病人胆囊管形态的变化

目的 分析胆囊结石继发胆道结石病例胆囊管结构的特点,为腹腔镜联合胆道镜胆道探查术提供术式判断的指证.方法 回顾性分析108例腹腔镜联合胆道镜胆囊切除加胆管探查术病例的术前影像检查结果,同时结合术中胆囊管形态、直径和扩张的情况,分析胆囊管结构变化特点.结果 术前B超检查显示108例病人均有胆囊结石,胆总管扩张75例,胆囊管扩张21例,胆总管结石76例.磁共振胰胆管造影(MRCP)检查发现胆总管扩张81例,有明显结石图像的52例,胆囊管扩张36例,发现9例胆囊管异位走行.CT检查显示胆总管扩张45例,有明显结石影的42例,胆囊管扩张19例.胆囊管有明显扩张的病例中,胆囊管和胆总管之间的夹角多呈钝角(22/24例);此夹角呈锐角的病例胆囊管扩张的少(2/28).胆囊管扩张明显的病例胆囊管形态常短粗(24/24),胆囊管细长的病例中胆囊管扩张的少.结论 MRCP对胆囊管的形态和走行有明显的判断和指导作用.胆囊管和胆总管之间的夹角呈钝角,胆囊管短粗的病例,其胆囊管扩张明显.     

犬胆管壁的应力-应变性扩张构建宽口径胆管的实验研究

目的 探索短期内以持续渐增的局部胆管内压构建局部宽口径胆管的可行性及形态特征.方法 杂交犬12只随机分配到A、B两组中,A组仅在胆总管内放置胆管扩张器作胆汁外引流,B组在胆总管内放置胆管扩张器引流并分别在手术后的第1、8、15、22天向扩张球囊注水,注水容积分别为0.1、0.15、0.2、0.25 ml,扩张球囊以渐增的压力持续扩张1个月.观察两组手术前后血清总胆红素(TB)、丙氨酸转氨酶(ALT)、天冬氨酸转氨酶(ALT)及胆管直径(BDD)、胆管壁及肝组织学病理变化、术后X线胆道造影检查.结果 A、B两组扩张前后及组间比较在TB、ALT、AST及肝组织学方面差异不明显;BDD在A、B两组扩张前后及组间比较差异明显;B组胆管壁增厚,管腔增粗,管壁柔韧性好,肝外胆管以扩张处为中心向邻近胆管方向扩张,胆管壁特殊染色示胶原及弹力纤维排列致密,无断裂.结论 以持续渐增的局部胆管内压扩张局部胆管,构建的胆管管壁厚,管腔粗,胆管壁胶原及弹力纤维排列致密.扩张的局部胆管为犬胆管损伤早期胆肠重建及制备宽大的胆肠吻合口创造了条件.     

Dynamic study of pancreaticobiliary reflux using secretin-stimulated magnetic resonance cholangiopancreatography in patients with choledochal cysts

Background/Purpose

Refluxes through pancreaticobiliary maljunctions play an important role in the pathophysiology of choledochal cysts. Dynamic studies of the pancreaticobiliary tract were performed using secretin-stimulated magnetic resonance cholangiopancreatography.

Methods

Six patients with choledochal dilation were recruited for this study. Four patients exhibited cystic and 2 exhibited fusiform dilatation of the common bile duct (CBD). Magnetic resonance cholangiopancreatography images were obtained every minute during the 15-minute period after secretin stimulation. The sequential morphological changes in the biliary trees, pancreas, and duodenum were assessed, and the total pixel values of these organs were measured for each image, then plotted as a ratio against the baseline image.

Results

In 2 cases involving cystic dilatation, the intensity of bile duct images continued to rise after secretin stimulation. In a case involving fusiform dilatation, a transitory elevation in CBD intensity was observed. In 3 cases involving fusiform or cystic dilatation, the intensity of CBD did not change notably. In all cases, the duodenum was filled well after secretin stimulation.

Conclusions

The sustained elevation in bile duct intensity after secretin stimulation indicates reflux and bile stasis. Transitory elevation may indicate reflux without stasis. This method allows assessment of the dynamics of pancreatic and bile fluid under more physiologic condition.     

Familial occurrence of congenital bile duct dilatation

The occurrence of congenital bile duct dilatation (CBD) in both a mother and her daughter was recently experienced at Niigata University Hospital and Niigata Shimin Hospital. Bile duct dilatation with anomalous pancreaticobiliary ductal junction (AP-BDJ) was disclosed in both. Intrahepatic bile duct dilatation was only in the mother. Removal of dilated bile duct, cholecystectomy, and hepaticojejunostomy were performed in both. Fourteen cases of CBD from seven families were collected from literature and discussed.     

Right posterior branch draining into intra-pancreatic common duct: the triple duct sign

Simultaneous dilatation of pancreatic duct (PD) and common bile duct (CBD) is often seen on radio imaging in pancreatic head malignancy or chronic pancreatitis. This is called double duct sign. However, dilatation of aberrant bile duct along with PD and CBD resulting in triple duct sign on radio imaging has not been reported in literature till date. We report a case of 45 years old male with surgical jaundice due to pancreatic head mass. Computed tomography (CT) scan revealed three dilated ducts in the head of pancreas. Besides PD and biliary duct, the third duct was pre-operatively thought to be long cystic duct with low insertion in common hepatic duct (CHD). Intra-operative findings revealed an unusually long and dilated aberrant right posterior sectoral duct with low insertion into intra-pancreatic CHD. Patient underwent Whipple’s procedure with two separate biliary anastomosis besides pancreaticojejunostomy. Histopathology of specimen revealed chronic pancreatitis. Retrospective analysis shows that Magnetic Resonance Cholangiopancreatogram (MRCP) should have been done for correct pre-operative delineation of this anatomical anomaly. In conclusion, pre-operative detection of triple duct dilatation on CT scan should be further investigated with MRCP for anatomical variations of the biliary tract. This can guide operative planning and prevent inadvertent biliary injuries.     

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     

术中微切开胆道镜检查的可行性研究

目的探讨经胆囊管汇入部微切开入路胆道镜检查后一期缝合的可行性和安全性。方法对具有胆道探查指征的病人,施行胆囊管汇入部切开2～3mm,然后经此切开处置入胆道镜进行检查和治疗。探查完成后,不放置T管,直接(一期)缝合胆囊管及汇入部微切口。结果自1996年12月至2003年8月已施行此术式42例。发现肝外胆管结石26例54枚,术中结石取净率为100%(26/26),阴性探查16例。胆道镜检查或协助取石时间在5～20min,平均用时10min,无一例胆瘘发生。术后住院时间7～10d,平均为8d。38例得到随访,随访时间为3～24个月,平均为14个月。经B超和CT证实,无一例有胆道狭窄表现。结论经胆囊管汇入部微切开入路进行胆道镜检查,与传统的经胆总管前壁切开入路相比具有创伤小,病人术后反应小,安全、方便等优点,免除了病人术后的带T管痛苦,减少了术后并发症,缩短了术后住院时间,并且适于大部分胆道结石的病人,值得推广应用。     

Resected cystadenoma of the common bile duct

Biliary cystadenoma in the extrahepatic bile ducts is a very rare tumor. A 62-year-old woman with jaundice was admitted to our hospital. Imaging studies revealed a 4-cm cystic lesion around the hepatic hilum, compressing the common bile duct (CBD). When laparotomy was performed, a cystic tumor was detected in the hepatic hilum, filling the lumen of the CBD. Bile duct resection that included the tumor was performed, followed by biliary reconstruction. Microscopically, the cyst wall was lined by a single layer of cuboidal epithelial cells, covering an ovarian-like stroma. The degree of atypia was low and warranted the diagnosis of cystadenoma.     

Clinical experience of biliary tract carcinoma associated with anomalous union of the pancreaticobiliary ductal system

Between 1978 and 1988, 15 patients with gallbladder cancer and 2 patients with bile duct cancer were seen among 49 patients with anomalous union of the pancreaticobiliary ductal system. Radiographic findings revealed two types of this anomalous condition: one in which the pancreatic duct entered the common bile duct(type 1) and one in which the common bile duct entered the pancreatic duct (type 2). In gallbladder cancer, the common bile duct presented no dilatation, or in some patients, mild dilatation, and type-1 anomalous union was frequently found among these patients. In contrast, the two patients with bile duct cancer had cystic dilatation of the common bile duct and type-2 anomalous union. The bile amylase level, which was determined in seven patients, was extremely high in all the patients. Histopathologically, the tumors in most patients showed papillary to papillo-tubular proliferation in the mucosal layer while atypical epithelial hyperplasia was noted in the vicinity of the tumor area. These findings suggest that this congenital anomaly in both ducts results in a loss of the normal sphincteric mechanism of the doudenal papilla, and that chronic relapsing cholecystitis or cholangitis, caused by the reflux of pancreatic juice into the biliary tract, can induced progressive changes to atypical epithelial hyperplasia which may develop into carcinoma.     

先天性胆总管囊肿胰胆合流共同管开口位置及其相关因素的分析研究

目的 探讨胰胆合流共同管开口异位及其相关因素在胆总管囊肿发病机制中的作用。方法 对116例显影清晰的先天性胆总管囊肿患者的ERCP或术中胆道造影的正位X线片进行有关数据的测量分析，并以6例遗传性球形红细胞增多症及7例新生儿肝炎综合征患儿胆道造影的X线片作为对照组，对测量数据进行统计学处理并加以比较。结果 对照组Vater壶腹均开口于十二指肠降段，而实验组开口于十二指肠降段者占31．1％，开口于降段以远者占68．9％，且共同管开口位置越远，共同管、胆总管越长，胰管直径及胰胆汇合角度越大。囊肿型囊肿的胆总管直径与长度及胰胆汇合角度与梭型比较，差异有非常显著性意义，且前者合并胰腺分离及其它肝内外胆管畸形的发生率明显增高。结论 影像学测量下，Vater壶腹开口异位的存在有可能是先天性胆总管囊肿的发病原因，而胰胆合流异常、胆总管远端狭窄及肝内胆管的畸形则是本病通常并发的病理改变，但也可能互为因果，有待进一步研究。     

Clinical experience of biliary tract carcinoma associated with anomalous union of the pancreaticobiliary ductal system

Between 1978 and 1988, 15 patients with gallbladder cancer and 2 patients with bile duct cancer were seen among 49 patients with anomalous union of the pancreaticobiliary ductal system. Radiographic findings revealed two types of this anomalous condition: one in which the pancreatic duct entered the common bile duct (type 1) and one in which the common bile duct entered the pancreatic duct (type 2). In gallbladder cancer, the common bile duct presented no dilatation, or in some patients, mild dilatation, and type-1 anomalous union was frequently found among these patients. In contrast, the two patients with bile duct cancer had cystic dilatation of the common bile duct and type-2 anomalous union. The bile amylase level, which was determined in seven patients, was extremely high in all the patients. Histopathologically, the tumors in most patients showed papillary to papillo-tubular proliferation in the mucosal layer while atypical epithelial hyperplasia was noted in the vicinity of the tumor area. These findings suggest that this congenital anomaly in both ducts results in a loss of the normal sphincteric mechanism of the duodenal papilla, and that chronic relapsing cholecystitis or cholangitis, caused by the reflux of pancreatic juice into the biliary tract, can induced progressive changes to atypical epithelial hyperplasia which may develop into carcinoma.     

Association of extrahepatic bile duct duplication with pancreaticobiliary maljunction and congenital biliary dilatation in children: a case report and literature review

We herein report a case of cystic-type congenital biliary dilatation (CBD) in whom an extremely rare anomalous duplication of the common bile duct and pancreaticobiliary maljunction were diagnosed intraoperatively by meticulous surgical manipulations via conventional open surgery. By performing a dissection at the outer epicholedochal layer of the cyst, a thin cord-like structure shown to be the distal part of the common bile duct was identified. A further exploration revealed that the most distal (extra- and intrapancreatic) part of the common bile duct was duplicated, and each branch of the duct was connected to the main and accessory pancreatic ducts. The experience with our case and a literature review showed that extrahepatic bile duct duplication is generally associated with pancreaticobiliary maljunction and CBD. We conclude that an extremely careful exploration with delicate and meticulous surgical manipulation is essential to identify these morphological anomalies and prevent intraoperative and postoperative complications of CBD, such as pancreatic duct injury or pancreatitis.     

Extracystic biliary carcinoma associated with anomalous pancreaticobiliary junction and cysts

Anomalous pancreaticobiliary junction with cystic dilatation of the biliary tract is usually associated with carcinoma arising from the cyst wall. We report an extracystic location of biliary carcinoma in the presence of anomalous pancreaticobiliary junction and cysts in a patient with obstruction of the origin of the left hepatic duct who underwent hepatobiliary resection. Cholangiocarcinoma was found to have arisen in a noncystic left hepatic duct, in conjunction with cystic dilatation involving both the cystic and common bile ducts. The present case supports a relationship between anomalous pancreaticobiliary junction and biliary carcinogenesis that may affect the extracystic biliary tree.     

Epithelial cell proliferation activity of the biliary ductal system with congenital biliary malformations

Congenital biliary malformations such as anomalous arrangement of the pancreaticobiliary ductal system (AAPB), congenital cystic dilatation of the common bile duct (CCDB), and congenital biliary strictures at the hepatic hilum (CBSH) are newly designated disease entities and are frequently found in adult patients with biliary malignancy such as gallbladder carcinoma, common bile duct carcinoma, and intrahepatic bile duct carcinoma. In the present study, the relationship of these malformations and biliary malignancy was investigated. We studied 61 gallbladders of patients with AAPB and 56 gallbladders of patients without AAPB; 16 common bile ducts of patients with CCDB (12 with AAPB and 4 without AAPB) and 11 gallbladders of patients without CCDB; and 17 intrahepatic bile ducts of patients with CBSH and 6 intrahepatic bile ducts of patients without CBSH. Tissue sections from the mucosa of the gallbladder, common bile duct, and intrahepatic bile duct were stained for proliferating cell nuclear antigen (PCNA). The PCNA labeling indexes of patients with these malformations were significantly higher than those of patients without these malformations (P < 0.05). Cell proliferation of the epithelia in the biliary ductal system in patients with these congenital biliary malformations was accelerated. Consequently, these congenital malformations appear to be an important risk factor for the occurrence of biliary malignancy. Received for publication on Feb. 15, 1999; accepted on March 29, 1999     

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先天性胆管扩张症是指肝内、外胆管单独或联合的先天性发育异常,多发生于女性,常在婴幼儿期发现。随着医学影像检查在临床应用,少数于成年期发现确诊。先天胆管扩张症多发生于胆总管,并呈梭形扩张,最多见者为I型(先天性胆总管囊肿)。扩张的胆管内胆汁淤滞潴留,可并发胆管炎、结石、穿孔,少数囊壁发生恶变。先天性胆总管囊肿于成年期一经确诊,无论症状轻重,如无手术禁忌证均应手术治疗。目前主张,除非有特殊的适应证,不宜采取内引流或外引流术。彻底切除囊肿,重建肝外胆道是根治先天性胆总管囊肿的标准术式。