Clinical aspects of congenital cystic dilatation of the common bile duct in adults

We reviewed the data for 155 patients with congenital cystic dilatation of the common bile duct (CCDB; Alonso-Lej Type I) treated at our institute in the past 12 years. Our definition of CCDB was segmental enlargement of the common bile duct or enlargement involving the proximal bile duct, visualized on cholangiograms. CCDB was subclassified as infant or adult type cyst, based on the extent of the enlargement and the clinicopathologic findings. The former is typically an enlargement of the common bile duct or an enlargement involving the common hepatic duct, while the latter is an enlargement of the entirety of the extrahepatic ducts or involves the intrahepatic bile ducts. Of the 155 patients, 5 (including 1 child) had infant type cysts and 150 (including 1 child) had adult type cysts; 125 patients had benign CCDB (3 with infant type cyst) and 30 exhibited CCDB biliary malignancy (2 with infant type). Anomalous arrangement of the pancreaticobiliary ductal system (AAPB) and congenital biliary stricture were frequently associated with CCDB and these features played an important role in the presenting symptoms of CCDB: AAPB was detected in 100 of the 155 patients, and stricture in 51. AAPB caused disturbance of the passage of bile from the terminal bile duct to the duodenum and cholestasis in the enlargement, with reciprocal regurgitation of bile into the pancreas and pancreatic juice into the bile duct, and the AAPB appeared to precede obstructive jaundice, acute pancreatitis, or biliary malignancies. Stricture was observed at one to all of six sites in the hepatic hilum. Intrahepatic stones were seen in 30 patients, and intrahepatic bile duct cancer was seen in 8 patients; in all these 8 patients, the cancer was associated with primary intrahepatic stones.     

Congenital dilatation of the common bile duct and pancreaticobiliary maljunction—clinical implications

Clinical conditions and diagnosis  Congenital dilatation of the common bile duct is a disease in which the extrahepatic bile duct, or both the extra and intrahepatic bile ducts, is dilated in various ways. Pancreaticobiliary maljunction is a disease in which the pancreatic duct meets the bile duct outside of the duodenal wall beyond the sphincter Oddi. Recently, these diseases have been thought to be closely related to each other but to be different malformations. Biliary tract carcinoma, especially bile duct carcinoma, is found in about 30% of patients with congenital dilatation of the bile duct. The concomitance of bile and pancreatic juice and their stasis in the biliary tract induce cellular proliferation and reproduction and stimulate genetic alterations in biliary epithelium, which may play an important role in carcinogenesis of the bile duct. Therapeutic strategies  Endoscopic retrograde cholangiopancreatography is useful for examining pancreaticobiliary maljunction. The operation is dilated bile duct resection and hepaticojejunostomy, which ensure that pancreatic juice and bile do not mix in the bile duct. Gallbladder carcinoma develops in more than 90% of pancreaticobiliary maljunction without bile duct dilatation.     

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.     

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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。     

Anomalous arrangement of pancreaticobiliary ductal system associated with cholangiocellular carcinoma

A 66-year-old man with congenital cystic dilatation of the common bile duct (CDB; Alonso-Lej Type I) and anomalous arrangement of the pancreaticobiliary ductal system (AAPB) associated with intrahepatic bile duct cancer (cholangiocellular carcinoma; CCC) underwent an extended right hepatic lobectomy. In the resected specimen, the CCC was located in Couinaud’s segment V, with invasion to segment IV and the right hepatic duct and right portal vein. However, there was no cancer involvement of the dilated extrahepatic bile ducts, except for histologic findings of chronic inflammatory cellular infiltration and intestinal metaplasia. It is presumed that the probable mechanisms underlying carcinogenesis in CCC developing from the epithelium of intrahepatic bile ducts are the same mechanisms as those operating in carcinoma of the extrahepatic bile duct in patients with AAPB, although AAPB associated with CCC is uncommon. AAPB appeared to be related to the development of the CCC.     

Eosinophilic cholangitis with obstructive jaundice mimicking bile duct carcinoma

A 55-year-old male who presented with obstructive jaundice and radiographically documented extrahepatic biliary tract obstruction is reported. Eosinophilic infiltration of the gallbladder, common bile duct, intrahepatic bile ducts, and bone marrow was observed. Eosinophilic cholangitis, a rare inflammatory condition that clinically resembles a biliary malignancy, should be taken into consideration in the differential diagnosis in the evaluation of presumed neoplasm of the bile ducts.     

Idiopathic benign biliary stricture

A rare case of idiopathic benign biliary stricture is reported. A 50-year-old man with liver dysfunction underwent ultrasonography, which revealed dilation of the intrahepatic bile ducts, and endoscopic retrograde cholangiopancreatography, which revealed a short, ring-like stenosis at the junction of the left and right hepatic ducts. Although a benign stricture was suspected, radiologic tests alone were insufficient to make a firm diagnosis. Therefore, a cholecystectomy and resection of the extrahepatic biliary tract were performed. Microscopic examination of the resected specimen demonstrated no evidence of malignancy. The final diagnosis was mild, localized, chronic cholangitis. The patient had not had previous biliary tract surgery, choledocholithiasis, nor did he have a congenital abnormality of the biliary tract, bile duct carcinoma, or pancreatic disease. Since there was no evidence of primary sclerosing cholangitis, the stricture was considered to be idiopathic. Received for publication on May 30, 1997; accepted on April 13, 1998     

Intrahepatic cholangiojejunostomy for unresectable malignant biliary tumors with obstructive jaundice

We reviewed our experience with intrahepatic cholangiojejunostomy as a palliative therapy for patients with unresectable malignant diseases involving the ductal confluence or the common hepatic duct. Fifteen patients with malignant biliary obstruction were treated by cholangiojejunostomy at our hospital. Two patients had intrahepatic cholangiocarcinoma, 7 had gallbladder carcinoma, 5 had bile duct carcionoma, and 1 had pancreatic carcinoma. Segment III cholangiojejunostomies were performed in 14 patients and segment V cholangiojejunostomy in 1. Contraindications for surgical resection were locoregional invasion of tumors involving the proper and/or common hepatic artery and portal vein in 15 patients and the presence of hepatic metastases in 6 patients. Liver metastases were detected in 5 of the 7 patients with gallbladder carcinoma. Postoperative complications occurred in 2 patients (13%), but there was no leakage of the cholangioenteric anastomosis in our series. There was no operative mortality after cholangiojejunostomy. Of the 9 patients who survived for more than 6 months after surgery, 7 showed a significant improvement in performance status (PS) (82 ± 10%) 3 months after the surgery compared with the preoperative PS (70 ± 7%). Four of the 9 patients had recurrent cholangitis as a late complication, but 4 were completely free from jaundice. Median survival after cholangioenteric bypass was 9 months (range, 2–25 months). With respect to tumor location, the median survival time was 4 months (range, 2–25 months) in patients with gallbladder carcinoma and 15.5 months (range, 12–22 months) in those with bile duct carcinoma. While the median survival period after surgery was only 3 months (range, 2 to 8 months) in the 5 patients with hepatic metastases from gallbladder carcinoma, 2 patients without liver metastasis survived for 9 and 25 months after segment III cholangioenteric bypass. In conclusion, cholangiojejunostomy can provide useful palliation for malignant biliary obstruction when combined with careful patient selection. Received: September 5, 2000 / Accepted: November 8, 2000     

逆行经肝胆道引流在胆管结石手术中的应用

目的：探索可代替T管引流的手术方法。方法：对49例肝内外胆管结现人术中实施逆行经肝胆道引流术。其中37例逆行穿刺经右肝置管外引流;12例从肝胆管残端置管逆地引流。总胆管切口原位缝合关闭。结果：逆行穿刺引流术术后并发气胸及引流管出血各1例（5．4％）,术后平均第8天拔管。逆行经肝胆管残端置管引流乾术后残余结石4例,均经引流窦道取净。保留胆囊19例,术后造影,胆囊显影良好。术后随访6月－9年,B超检查无胆管狭窄及复发结石,保留的胆囊未形成结石。结果：逆行穿刺经肝胆道引流术后带管时间短,逆行经肝管残端引流对术后残余结石的处理较方便。两种引流术式对肝外胆管及保留的胆囊无不良影响。     

Dilatation of the intrahepatic bile duct associated with congenital anomalous junction of the cystic duct--a new disease entity

We describe 3 children with dilatation of the intrahepatic bile duct, who had anomalous junctions of the cystic duct, 2 high and 1 low, without pancreaticobiliary maljunction. They were all male, and underwent excision of the gallbladder and the extrahepatic bile duct followed by a Roux-en-Y hepaticojejunostomy as a definitive surgery. Postoperatively, the dilated intrahepatic bile duct became normal in size. From these 3 cases, we propose a new disease entity—dilatation of the intrahepatic bile duct because of congenital anomalous junction of the cystic duct.     

Congenital biliary dilatation may consist of 2 disease entities

Background/Purpose

This study aims to establish the possible mechanisms of pathogenesis of congenital biliary dilatation and to classify the disease accordingly.

Methods

Radiologic features of congenital biliary dilatation and pancreaticobiliary malunion in 107 affected children were examined and correlated with laboratory results. Relative lengths/diameters were calculated to provide comparison between children of different ages. Intraluminal pressures of common bile duct (CBD) were measured intraoperatively.

Results

The minimal relative diameters of distal CBD negatively correlated with the maximal relative diameters/lengths of dilated CBD, the maximal relative diameters of common hepatic duct, and left/right hepatic ducts. The intraluminal pressure in patients with a stenotic distal CBD (stenotic group) was significantly higher than that in patients with a nonstenotic distal CBD (nonstenotic group). The narrower the distal CBD, the more deranged the liver function. Conversely, serum/bile amylase levels were more elevated in the nonstenotic group. Common channel protein plugs were only found in the nonstenotic group, whereas common hepatic duct strictures, intrahepatic duct dilatations, and calculi were detected more frequently in the stenotic group.

Conclusion

We propose to categorize congenital biliary dilatation into 2 subgroups: (1) cystic type with stenotic distal CBD associated with deranged liver function and common hepatic duct stricture and (2) fusiform type with nonstenotic distal CBD associated with pancreatitis and common channel protein plugs. Different underlying pathologies of each group require different operative strategies.     

Double cancer of the gallbladder and common bile duct associated with an anomalous pancreaticobiliary ductal junction without a choledochal cyst: Report of a case

We report herein the case of a 37-year-old woman found to have double cancer of the gallbladder and common bile duct associated with an anomalous pancreaticobiliary ductal junction (APBDJ) without a choledochal cyst (CC). Abdominal ultrasonography showed an isoechoic mass in the gallbladder, and percutaneous transhepatic biliary drainage tubography revealed incomplete obstruction in the upper portion of the common bile duct and APBDJ. The patient underwent cholecystectomy, partial hepatic resection, pancreatoduodenectomy, and portal vein reconstruction. Pathological examination of the tumors from the gallbladder and bile duct revealed papillary carcinoma and poorly differentiated adenocarcinoma, respectively, and direct continuity was not observed between the tumors. A review of the literature on six cases of multiple primary carcinoma of the biliary tract associated with APBDJ without CC is presented following this case report. Double cancer of the biliary tract was found synchronously in five patients and metachronously in one. Gallbladder cancer showed subserosal invasion in four patients, while bile duct cancer invaded the pancreas in one patient and reached the serosa in two patients. Considering the potential for cancer to arise in the biliary tract and the difficulties associated with monitoring it, cholecystectomy and resection of the extrahepatic common bile duct may be the most appropriate treatment for patients with an APBDJ without a CC.     

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.     

腹腔镜胆道探查取石术应用体会

目的探讨腹腔镜胆道探查取石术的可行性。方法回顾性分析38例腹腔镜胆道探查取石病人的临床资料,对手术适应证、手术方式及并发症的预防进行总结。结果38例病人中,胆总管结石36例,胆总管扩张未见结石2例。同时合并胆囊结石34例,合并有肝内胆管结石2例,合并肝囊肿2例。术前全部病人均行B超和MRCP检查。37例（97．4％）完成腹腔镜胆道探查取石术,1例因致密粘连中转开腹手术,36例同时行腹腔镜胆囊切除,2例同时行肝囊肿开窗引流术。14例行胆总管一期缝合,24例行胆总管T管引流。手术时间为1～4h,平均1．5h。住院时间3～12d,平均5．5d。术后发生胆瘘2例,胆总管下端残余结石1例,胸腔积液1例。结论腹腔镜胆道探查术安全、微创、美观,可明显缩短住院时间,是一种可选择的治疗方法。     

复杂性肝内胆管结石的外科治疗

目的　总结复杂性肝内胆管结石的外科治疗方法及效果。 方法　 总结分析１９９２ ～１９９８ 年外科治疗复杂性肝内多段胆管结石并多处胆管狭窄３５ 例的手术方法,总结显露与切开肝内狭窄段胆管、取出结石、解除狭窄的经验和体会。 结果　３５ 例无手术死亡,术后近期并发感染、胆漏、肝功能不全或消化道出血共７ 例,均治愈;残留结石９ 例,术后经胆道镜取净结石７ 例。随访６ 个月至５ 年６ 个月２４ 例,优良２１ 例（８８ ％ ） ,好转２ 例（８ ％ ） ,无效１ 例（４ ％ ） 。 结论　复杂肝内胆管结石外科治疗的关键是显露和切开肝内各叶段胆管的狭窄段,取出结石、建立通畅的胆流通道。经肝门区或肝方叶可以显露和切开肝门胆管、左右肝管和左内叶、右前叶胆管,经肝膈面切开肝实质进路,可以显露和切开右肝内各叶段胆管。     

The reliability and clinical limitations of sonographic scanning of the biliary ducts.

Sonographic scanning of the biliary ducts has been successfully used as a screening test to distinguish between patients with surgical and medical jaundice, with an accuracy of 90%. However, there is no consensus in the literature on what numerically defines a dilated biliary duct. To clarify this problem a prospective study of 102 consecutive patients was initiated to determine the sonographic size range of bile ducts in patients with and without extrahepatic ductal obstruction. The ultrasonic measurements were compared with direct measurements of the common bile duct, at surgery. The extrahepatic ductal system was visualized sonographically in 62% of the patients, while the intrahepatic ducts were found in 81% of the population. Direct measurements at operation agreed with the ultrasonic measurements in 84% of the patients. Analysis of the size range of the biliary ducts in patients with and without extrahepatic obstruction, by chi square analysis and the Student's t-test, allowed the following guidelines to be established. Extrahepatic bile duct obstruction was present if the extrahepatic bile ducts was 1 cm or wider (p less than 0.001) or if the intrahepatic bile duct was in excess of 0.5 cm (p less than 0.001). Similarly if the extrahepatic bile duct measured less than 0.8 cm sonographically, and the intrahepatic bile duct was 0.4 cm or less than bile duct, obstruction was not present (p less than 0.001).     

Clinicopathological studies of anomalous arrangement of the pancreaticobiliary ductal system with pancreas divisum

We examined the morphological aspects of the pancreaticobiliary ductal system in 13 patients with both anomalous arrangement of the pancreaticobiliary ductal system (AAPB) and associated pancreas divisum (PD), and compared their clinicopathological findings with those of patients with either AAPB or PD alone. PD is classified into three types, i.e., separate pancreas, nonfusion of the ventral and dorsal pancreatic ducts, and partial fusion of the ventral and dorsal pancreatic ducts. Of the 13 patients with AAPB and associated PD, 5 were male and 8 female; their mean age was 57 years. Nine of the 13 had clinical symptoms. Five of the 13 had gallbladder cancer, 3 had cholecystolithiasis, and 2 choledochal cyst. One patient showed nonfusion of both pancreatic ducts and the others showed partial fusion of the ducts. The length of the common duct from the orifice of the papilla of Vater to the junction of the common bile duct with the ventral pancreatic duct and the location of the union varied. The presence of both AAPB and PD made the arrangement of the pancreaticobiliary ducts very complicated. Clinical aspects were similar to those of AAPB and the patients were likely to be treated for a condition other than PD. However, since relapsing acute pancreatitis and intermittent epigastric pain are typical symptoms in patients with AAPB as well as in those with PD, we should carefully follow up those patients with both AAPB and PD.     

New clues for the developing human biliary system at the porta hepatis

The human biliary system is formed from the hepatic diverticulum, a structure which develops from the embryonic foregut in the fourth week of gestation. The cephalic portion of the hepatic diverticulum lies within the septum transversum, and gives rise to entodermal cells which become the primitive hepatocytes. The caudal part of the hepatic diverticulum is molded by mesenchyme to form the gallbladder, cystic duct, and extrahepatic bile duct. The gallbladder is initially tubular in shape, and undergoes morphological changes to become saccular during the 11th week of gestation. The extrahepatic bile duct elongates and widens as gestation progresses, and intramural mucus glands develop. There is no solid stage during the development of the extrahepatic bile duct. The extrahepatic bile duct is a well-defined tubular structure by the 6th week of gestation, whereas the intrahepatic biliary system during this period of gestation is represented by the primitive ductal plate. The ductal plate undergoes structural changes from the 11th week of gestation, beginning at the porta hepatis and progressing through gestation to the periphery of the liver. This remodeling process shapes the ductal plate from a flat sheath of biliary epithelium surrounding the portal vein branches into a network of interconnecting tubular structures. Mesenchyme plays an important role in ductal plate remodeling. The intrahepatic biliary system is in luminal continuity with the extrahepatic bile duct throughout gestation at the porta hepatis. The major bile ducts at the porta hepatis are fully formed by the 16th week of gestation. Received: September 30, 2000 / Accepted: January 10, 2001     

Left-sided gallbladder (sinistroposition) with duplication of the common bile duct.

A left-sided gallbladder sinistroposition is a rare finding. These gallbladders are situated left of the falciform ligament and are located under the left lobe of the liver, between segments III and IV. Common bile duct duplication is also rare, and its cause is not yet fully understood. A 55-year-old woman presented to our hospital with symptoms of chronic cholecystitis. During the laparoscopic cholecystectomy, it was discovered that not only was her gallbladder to the left of the falciform ligament, but she also had a duplication of her common bile duct. Although cases of left-sided gallbladders have been reported in the past, and there has been a report of a sinistroposition of both gallbladder and common bile duct, we believe this is the first reported case of left-sided gallbladder sinistroposition with the complete duplication of the common bile duct. As we learn more about various anatomical anomalies of the gallbladder through the use of laparoscopic cholecystectomies, surgeons encountering a left-sided gallbladder should be aware of the potential for associated anomalies.     

A case of benign bile duct stricture causing difficulty in differential diagnosis from bile duct carcinoma

We report a patient with benign bile duct stricture causing difficulty in differential diagnosis from bile duct carcinoma. A 66-year-old woman consulted a local physician because of general fatigue. Blood biochemical tests showed increased levels of biliary tract enzymes. Abdominal ultrasonography (US) revealed tapering and blockage of the midportion of the bile duct and dilation of the intrahepatic bile ducts. Magnetic resonance cholangiopancreatography (MRCP) demonstrated obstruction of the midportion of the bile duct. Later, because a marked increase in biliary tract enzymes and jaundice appeared, percutaneous transhepatic biliary drainage (PTBD) was performed. Post-PTBD cytological examination of bile was negative for cancer. A third biopsy showed slight hyperplasia with no malignant findings. Recholangiography, performed through PTBD, suggested gradual improvement of bile duct stricture, but could not completely exclude the possibility of malignancy; thus, resection of the bile duct including the stricture site was performed, and the resected specimen was submitted for intraoperative frozen section examination. Histopathological diagnosis did not reveal malignant findings. After cholecystectomy and bile duct resection, hepaticojejunostomy (Roux-en-Y) was performed. Because only erosion and desquamation of the mucosal epithelium and mild submucosal inflammatory cell infiltration and fibrosis were observed, chronic cholangitis was diagnosed histopathologically. Surgical resection of the bile duct should be considered for potentially malignant stricture of the bile duct.