A case of a rare anomaly of the common bile duct associated with an abnormal arrangement of the pancreaticobiliary ductal union

A 39 year-old Japanese female patient with a duplication of the distal portion of the common bile duct is presented herein. Moreover, an abnormal arrangement of the pancreaticobiliary ductal union, congenital biliary dilatation and carcinoma of the gallbladder were all demonstrated by cholangiographic and endoscopical studies. The patient underwent radical surgery for advanced adenosquamous carcinoma of the gallbladder, and her postoperative course was satisfactory. A reflux of pancreatic juice into the bile duct was demonstrated, but it was eliminated and considered to be a contributory etiologic factor of the gallbladder carcinoma.     

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.     

Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct

Background/purpose

For anomalous arrangement of the pancreaticobiliary duct (AAPBD) with nondilatation of the common bile duct (CBD), the optimal surgical procedure remains controversial. The authors investigated which procedure would be most effective for AAPBD with nondilatation of the CBD.

Methods

The authors encountered 60 children with AAPBD in our institution between 1979 and 2002. Six of the 60 were classified as the nondilated type (CBD diameter; less than 8 mm), whereas the other 54 were classified as the dilated type (CBD diameter; more than 9 mm). Amylase levels in serum, CBD, and gallbladder were examined. Cellular activity of the resected gallbladder was examined for the incidence of hyperplasia and Ki-67 labeling index (Ki-67 LI).

Results

The amylase level in the nondilated type was elevated as in the dilated type. Epithelial hyperplasia of the gallbladder was present in 4 of the 6 with the nondilated type (67%). 10 of the 20 with the dilated type (50%), and none of the 6 controls (0%). The Ki-67 LI of the dilated type was significantly higher than that of control.

Conclusions

A free reflux of pancreatic juice into the biliary system was found regardless of dilatation, and cellular proliferative activity of the gallbladder mucosa was increased in both the nondilated and dilated type. Therefore, excision of the extrahepatic bile duct including cholecystectomy is recommended for AAPBD with nondilatation of the CBD.     

A case of duplication of the common bile duct with anomaly of the intrahepatic bile duct

A 50-year-old man, with a complaint of fever and epigastralgia, was admitted to the Yamaga City Hospital. Choledocholithiasis and stenosis of the lower part of choledochus were pointed out. Cholecystectomy, choledocholithotomy and papilloplasty were performed and the choledochotomy closed over a T-tube. T-tube cholangiograms, after operation, revealed stenosis, negative shadow and abnormal branching of the left intrahepatic bile duct. Then, the lateral segmentectomy of the liver was done. But the negative shadow didn't disappear . With further examination, we could find the accessory bile duct in the retroperitoneal space. Cholangiogram, from this accessory bile duct, revealed patency of this bile duct into the left intrahepatic bile duct and the duodenum. A final diagnosis of duplication of the common bile duct was established. We collected Japanese cases of this anomaly and discussed a classification of the disorder.     

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

We report a 64-year-old woman with double cancer of the gallbladder and the common bile duct associated with anomalous junction of the pancreaticobiliary ductal system. Eleven previous cases have been reported to date. From analysis of the 12 cases reviewed, including our patient, double cancer of the gallbladder and the common bile duct accompanied by anomalous junction of the pancreaticobiliary ductal system is characterized by the predominance of well-differentiated and superficial cancer.     

Preduodenal portal vein with preduodenal common bile duct: an extremely rare anomaly

Preduodenal portal vein (PDPV) may occur as an isolated event and result in no symptoms, or it may found at autopsy as an incidental finding; associated preduodenal common bile duct (PDCBD) is an extremely rare event. To the 8 reported cases of PDPV with PDCBD, we add this rare case. Such a discovery is often incidental and of little import; however, it takes on major importance for hepatobiliary surgeons because the accidental damage of PDPV with PDCBD can lead to serious consequences. In addition to describing and illustrating this case, we discuss its relevant anatomy, embryology and associated malformations.     

A new case of cyst of the common bile duct with an anomaly of the bilio-pancreatic convergence

A case of common bile duct cyst operated upon in a 2 1/2 year old girl is used as a basic for discussing the forms associated with anomaly of convergence of common bile and wirsung's ducts. The latter, of dysembryoplastic origin, appears to be the cause of this type of cyst. These forms are atypical in that no mass is palpable and they are frequently complicated by a pancreatitis. They constitute the majority of Japanese cases when the anomaly is investigated. Its detection is possible by retrograde catheterization or more rarely during perioperative cholangiography, the only possibility in the very young infant. Different operative technics are outlined, taking into account principally the risk of secondary malignant changes and the possible performance of a hepatico-duodenal anastomosis without increasing the risk of an ascending angiocholitis.     

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.     

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.     

Septum formation of the common hepatic duct associated with an anomalous junction of the pancreaticobiliary ductal system and gallbladder cancer: Report of a case

We herein describe a 48-year-old woman who developed a septum formation of the bile duct combined with an anomalous arrangement of the pancreaticobiliary ductal system in conjunction with gallbladder cancer. A preoperative endoscopic retrograde cholangiogram demonstrated a filling defect in the common hepatic duct which was misdiagnosed to be an elevated lesion. Further exploration revealed a septum formation of the bile duct along with gallbladder cancer. A resection of the dilated bile duct and gallbladder along with a dissection of the regional lymph nodes was performed. A histological evaluation showed the septum to consist of a normal bile duct wall while the tumor in the gallbladder was poorly differentiated adenocarcinoma. The patient made an uneventful recovery. The septum formation was presumed to be congenital in origin.     

Bile duct carcinoma associated with an anomaly in the bile duct.

This paper reports a case of bile duct carcinoma associated with an extra-hepatic bile duct anomaly. In the present case, the anomaly induced an uncorrect preoperative diagnosis and obliged to change the surgical procedures during operation. A correct preoperative diagnosis was difficult to make in the present case, when the anomaly in the biliary system was not kept in mind.     

New insight into bile flow dynamics in anomalous arrangement of the pancreaticobiliary duct

BACKGROUND: In an anomalous arrangement of the pancreaticobiliary duct (AAPBD), two-way regurgitation, including pancreaticobiliary reflux and biliopancreatic reflux (BPR), might occur. Although pancreaticobiliary reflux has been shown by high levels of pancreatic enzymes in bile, there have been few reports of clinical evidence of BPR in AAPBD. Recently, spiral computed tomography combined with intravenous infusion cholangiography (IVC-SCT) has been reported to be useful for visualizing the biliary anatomy, which may allow a new insight into bile flow dynamics in AAPBD. METHODS: Fifteen paediatric patients with AAPBD were examined before operation using both IVC-SCT and endoscopic retrograde cholangiopancreatography. Visualization of the main pancreatic duct on IVC-SCT defined the presence of BPR. Blood samples were also analysed when the initial symptom was strongly present. RESULTS: BPR was observed in six of 15 patients. Pancreatic enlargement and hyperamylasaemia were significantly more frequent in patients without BPR than in those with BPR. Levels of enzyme markers of cholestasis were also higher in the former group. CONCLUSION: The present study, using IVC-SCT, has identified clinical evidence of BPR in AAPBD. Further studies are necessary to determine the correlation between BPR and clinical manifestations.     

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.     

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.     

胰胆管合流异常患者胆汁对人胆管癌细胞生长的影响

目的　研究胰胆管合流异常患者 (APBDU)胆汁对人胆管癌细胞QBC93 9生长的影响。方法　应用四唑氮蓝比色 (MTT)法检测QBC93 9增殖状况 ,应用流式细胞仪测定细胞周期和凋亡 ,采用酶联免疫吸附试验 (ELISA)检测前列腺素E2 (PGE2 )含量。结果　APBDU胆汁与正常对照组胆汁比较明显促进QBC93 9细胞增殖 (P <0 .0 5 ) ,这种促癌活性可被环氧合酶 2 (COX 2 )选择性抑制剂Celecoxib拮抗。用APBDU患者胆汁处理 2 4h的QBC93 9细胞增殖指数显著上升(P <0 .0 5 ) ,S期细胞比例为 ( 3 2 .2 4± 1.3 3 ) % ,比正常对照组 ( 10 .5 6± 1.47) %明显增高 (P <0 .0 5 ) ,G0 /G1期细胞比例为 ( 4 9.72± 4.13 ) % ,比正常对照组 ( 74.81± 5 .10 ) %明显降低 (P <0 .0 5 )。结论　APBDU胆汁具有潜在的促癌活性 ,这种促癌活性依赖COX 2和PGE2 途径。     

Cholecystolithiasis causing Mirizzi's syndrome with a rare anomaly of the extrahepatic bile duct in a child. Report of a case

A 14 year-old girl, who was admitted to our hospital due to increasing jaundice, intermittent right upper quadrant pain and fever, underwent operation based on the diagnosis of the Mirizzi's syndrome. Calculi filled the cystic duct and compressed the right hepatic duct. The right and left hepatic ducts lay closer to the duodenum than usual. The operation was limited to cholecystectomy alone. The calculi showed laminar cut surfaces and were composed of bilirubin lime. In addition to reporting our case, the literature dealing with this particular entity is reviewed.     

A case of biliary atresia with duplication of the common bile duct

The authors report a case of biliary atresia (BA) with duplication of the common bile duct. A 1-month-old girl was referred for investigation of persistent jaundice. BA was suspected and confirmed with intraoperative cholangiography. A portoenterostomy was performed at 50 days of age. During dissection of the fibrous remnant of the common bile duct, a separate fibrous bile duct remnant running in parallel on the left, was identified. Histologically, there was bile duct proliferation at the porta hepatis of the left bile duct remnant. A diagnosis of BA with duplicated common bile duct was made. Postoperative course was uneventful, and at a follow-up assessment at 7 months, she was jaundice free, and her progress has been unremarkable.     

腹腔镜胰十二指肠切除术治疗胆总管下段癌 (附一例报告)

目的:探讨腹腔镜胰十二指肠切除术治疗胆总管下段癌的安全性与可行性。方法:就我科于2005年1月用腹腔镜进行的一例胆总管下段癌所作胰十二指肠切除术,研究其手术技术、手术安全性、术后恢复情况和随访结果。结果:手术顺利,无术中严重并发症;手术时间6.5h,术中出血约50ml。术后恢复良好,术后第3天胃肠道功能恢复,第4天下床,第6天开始进流质。术后有少量胰漏,经单纯吸引后痊愈;无胃肠、胆肠吻合口漏等严重并发症;术后30d出院。出院后1个月,随访情况良好。结论:具丰富腔镜手术经验的专业医师施行腹腔镜胰十二指肠切除术具有可行性与安全性。本例的长期疗效有待进一步随访观察。本手术方法有待更多经验积累及随机临床论证。