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.     

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.     

Clinical and experimental studies on the etiology of congenital biliary dilatation (CBD)--with special reference to relationship between elastic fibers in the wall of the bile duct and type of CBD

The purposes of this study are to clarify the correlation between the morphological differences in congenital biliary dilatation (CBD) and elastic fibers in the wall of the bile duct. Using forty-three patients with CBD and fifty-seven autopsy cases in which history of biliary disease had not demonstrated, histopathological studies have been performed. In addition, experimental models of anomalous choledocho-pancreatico ductal junction were created in twenty-two young sheep and nine neonatal lambs, and the differences in morphological change were studied in two groups. The results are as follows. 1) Elastic fibers were not seen in the wall of the common bile duct of neonate or infant. So it suggests that the ductal wall was extremely weak in this period and increased intraluminal pressure of the biliary system, induced by distal obstruction, caused the cystic dilatation easily. 2) Fusiform dilatation of the extrahepatic bile duct was produced in all experimental models. The reason why cystic dilatation was not caused in these models is that elastic fibers were abundant in just ductal wall of neonatal lambs. From these results, aging factors may play an important role in the formation of CBD.     

Mechanism of the bile duct dilatation in congenital dilatation of the common bile duct

Radiographic studies including operative cholangiography, endoscopic retrograde cholangiopancreaticography and percutaneous transhepatic cholangiography were performed in 44 patients with congenital dilatation of the common bile duct. The relation between the maximum transverse diameter of the common bile duct (CBD) as an indicator of the degree of dilatation and various possible factors causing dilatation of CBD was studied. Among these factors, the length of the narrow segment (X) was closely related with the maximum transverse diameter (Y). The relation of these two was expressed by a simple equation; log Y = 0.068X + 1.06 (r = 0.934, p less than 0.001, n = 19) Dilatation of CBD has been successfully produced by narrowing the ductal lumen with a small plastic tube encirculating the distal common bile duct in dogs. Experimental dilatation was quite similar with congenital dilatation. In addition, the relation between the maximum transverse diameter (Y') and the length of the narrow segment (X') was also expressed in the following simple equation; log Y' = 0.060X' + 0.88 (r = 0.972, p less than 0.001, n = 18) The present studies did not support the hypothesis that abnormal junction of the pancreatico-biliary system might be the primary cause of congenital dilatation of CBD. They suggest that the stenosis of the distal CBD in the fetal life may determine dilatation of CBD.     

Ball-valve gastric tumor associated with anomalous junction of the pancreatico-biliary ductal system and a right-sided round ligament: Report of a case

We report the case of a ball-valve gastric tumor associated with anomalous junction of the pancreatico-biliary ductal system (AJPBDS) and a right-sided round ligament, misdiagnosed preoperatively as advanced gastric cancer with pancreatic head invasion. A 72-year-old woman presented with chest pain, but laboratory data showed only anemia. Gastroscopy revealed a bleeding polypoid gastric tumor in the anterior wall of the stomach, herniating into the duodenum (ball-valve syndrome), and a Bormann type-2 tumor in the posterior wall. Ultrasonography showed gallbladder stones, dilatation of the intrahepatic bile duct and pancreatic duct, and a left-sided gallbladder (attributed to a right-sided round ligament with anomalous branches of the portal veins). Laparotomy revealed that the gastric tumors were not advanced cancer invading the pancreatic head. Intraoperative cholangiography showed an AJPBDS, causing dilatation of the intrahepatic bile duct and pancreatic duct. We performed distal gastrectomy and cholecystectomy without biliary diversion. Microscopy revealed that the polypoid tumor was a hyperplastic polyp.     

Anatomic observations and etiologic and surgical considerations in choledochal cyst.

Twenty-five operative and postoperative cholangiograms in children with choledochal cysts indicated an intimate relationship between these cysts and anomalous pancreatico-biliary ductal junction. In all of these patients the distal main pancreatic duct (MPD) is excluded from the sphinctor of Oddi mechanism and free reflux of pancreatic juice up the biliary tree occurs. This leads to chronic cholangitis, which is presumed to be responsible for the formation of the choledochal cysts. Abrupt and intense exposure of the common bile duct (CBD) to pancreatic reflux may cause perforation and bile peritonitis. Long-standing exposure may induce malignant transformation in the choledochal cyst. Total cyst excision and separation of the biliary system from exposure to pancreatic reflux is suggested as the procedure of choice. Long-term results and metabolic and digestive consequences of this operation are yet to be determined.     

A study of concomitant chronic pancreatitis in forty-one cases of adult congenital biliary dilatation

Forty-one cases of adult congenital biliary dilatation (CBD) were studied for concomitant chronic pancreatitis. Of the 41 cases, 8 had had such previous operations as choledochojejunostomy or choledocho-duodenostomy without biliary diversion (reoperated cases) and the other 33 had not received previous operations (primary cases). All of the 36 cases whose type of pancreatico-biliary junction could be examined revealed anomalous pancreatico-biliary junction. Four cases (10%) of CBD (1 of the primary and 3 of the reoperated cases) were complicated by chronic pancreatitis. On examining the elapsed time between the first and the final operation of the reoperated cases, in the 5 cases whose periods were less than 13 years, none was complicated by chronic pancreatitis. On the contrary, in the 3 cases whose periods of time were more than 17 years, all were complicated by chronic pancreatitis. The results suggest that choledochoenterostomy without biliary diversion enhances pancreatic damage in cases with anomalous pancreatico-biliary junction.     

Gall Bladder Tumour,Choledochal Cyst and an Anomalous Pancreatico-Biliary Junction

This report highlights the association of a tumour in an acalculous gall bladder with an anomalous pancreatico-biliary junction (PBJ) and a type IVa choledochal cyst. Cholecystectomy and Rouxen- Y hepatico-jejunostomy (RHJ) was performed after division of the common bile duct (CBD) and excision of the dilated segment. The details of the case are presented and the role of an abnormal PBJ in gall bladder carcinogenesis is discussed.     

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     

A malformation of the pancreatico-biliary system as a cause of perforation of the biliary tract in childhood

Three cases of biliary tract perforation are presented. They had in common an anomalous arrangement of the pancreatico-biliary system, a cylindrical dilatation of the biliary tree, and an inflammatory thickening of the wall of the bile duct. The etiological importance of the anomalies is discussed. Bypass procedures are necessary at the final operation.     

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.     

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.     

Common channel syndrome--diagnosis with endoscopic retrograde cholangiopancreatography and surgical management

Six patients with intermittent bouts of vomiting, fever, abdominal pain, and jaundice beginning in infancy or early childhood were all demonstrated by endoscopic retrograde cholangiopancreatography to have an anomalous junction of the pancreaticobiliary ductal system with the formation of a characteristic long common channel. A varying degree of dilatation of the bile duct also was noted. Resection of choledochus followed by hepaticoduodenostomy was performed with satisfactory results invariably in all cases. The existence of a pathologic entity that might reasonably be designated "common channel syndrome" is discussed with some comments on its relationship with dilatation of the bile duct (choledochal cyst) as well as on the recommendable method of surgical treatment.     

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.     

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.     

Cylindrical dilatation of the choledochus: a special type of congenital bile duct dilatation

Cylindrical dilatation of the choledochus develops in 20% of patients with congenital bile duct dilatation and usually has acute-angled unions of the pancreatobiliary ductal system. Symptoms generally develop in patients over 1 year of age. The patients frequently complain of abdominal pain, vomiting, and fever as in those with acute pancreatitis. Ultrasonography and infusion cholangiography are the most useful tools in making a correct diagnosis. A high amylase level in the bile caused by the refluxing of pancreatic juice through anomalous ductal unions is commonly observed. This is responsible for biliary perforation in infancy and possibly carcinoma arising in the bile duct. The amylase concentration in the serum at the time of epigastric pain often is high, which leads to the diagnosis of acute pancreatitis. However, evidence of pancreatic inflammation is seldom noted. Accordingly, amylase in the bile may enter the circulating blood through the denuded epithelium or sinusoids of the liver. Excision of the whole extrahepatic duct along with hepaticoenterostomy would be essential for the treatment of cylindrical dilatation of the bile duct, especially when an anomalous ductal union is present.     

Surgical treatment of congenital dilatation of bile duct (choledochal cyst) with technical considerations

Since the concept of an anomalous junction of the pancreaticobiliary ductal system was introduced, there has been rapid progress in the study of the pathophysiology and the surgical treatment of choledochal cyst, or congenital dilatation of the bile duct. Because of its various disadvantages, cystoenterostomy as a routine procedure for treatment of this condition has been superseded by hepaticoenterostomy after removal of the devastated bile ducts with blocking of communication with the pancreatic ducts. This article describes the technical details of the surgical correction of choledochal cyst.     

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.     

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.     

胰胆管合流异常致胆总管扩张症的诊断治疗

目的探讨胰胆管合流异常致胆总管扩张症的诊断和治疗措施,减少胆道肿瘤的发生率。方法回顾分析13例,从发病原因、临床症状及手术方法进行探讨。结果反复发作胆管炎是主要临床表现,ERCP检查发现胰胆管合流部压力高,胆汁中淀粉酶>10 000 U/L可确诊,治疗应彻底切除扩张之胆总管,胆肠吻合术。结论胰胆管合流异常致胆总管扩张症是潜在恶变发生率较高的疾病,本组病理证实为38.4%,临床诊断和治疗应高度重视。