Search

Background: The treatment of type IV-A choledochal cyst is particularly difficult and remains a challenge because of the rareness and the various presentations of the disease involving not only the extrahepatic but also the intrahepatic biliary tract. The purpose of this study is to analyze our clinical experience for surgical treatment of type IV-A choledochal cyst, and compare between children and adults., Methods: During a 10-year period of time (2000-2010), clinical data of 81 consecutive patients with type IV-A choledochal cyst were retrospectively analyzed. We divided these patients into two groups, the child group (age ≤ 18 years) and the adult group (age >18 years). According to whether the patient received additional liver resection, patients were divided into a extrahepatic cystectomy (EHC) group and an additional liver resection (LR) group. The long-term outcomes after surgery were evaluated in two groups., Results: Of all 81 patients, there were 17 children and 64 adults; 16 children and 35 adults belonged to EHC group, one child and 29 adults belonged to LR group. The morbidity of biliary stricture and/or lithiasis in the adults was significantly higher than that in the children (p = 0.041 < 0.05). In the EHC group, the reoperation rate of adults was significantly higher than that of children (p = 0.019 < 0.05). For adult patients, the morbidity of biliary stricture and/or lithiasis and the reoperation rate in EHC group was significantly higher than that in LR group (p = 0.037 < 0.05 and p = 0.026 < 0.05 respectively). Five adults were found to have cholangiocarcinoma within a follow-up period, while no child was found to. However, for adult patients, no significant discrepancy was observed between EHC group and LR group (p = 0.366 > 0.05)., Conclusions: The present study suggests that the children have better outcomes than adults for patients with type IV-A choledochal cyst after EHC, while LR brings better outcomes than EHC for adult patients., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: This study was aimed to investigate etiology and clinical profiles of recurrent acute pancreatitis (RAP), particularly from the morphology of the pancreaticobiliary duct system., Material and Methods: Pancreaticobiliary morphology was examined in 230 of 381 patients with acute pancreatitis (AP) using endoscopic retrograde cholangiopancreatography. We analyzed factors associated with RAP including the pancreaticobiliary duct system., Results: RAP was diagnosed in 74 patients (19%). Major etiologies of RAP were alcoholic (38%), idiopathic (26%) and pancreaticobiliary malformation (22%). Patients with alcoholic RAP were significantly younger (47.2±11.6 years) than those with gallstone RAP (67.3±16.8; p<0.05). RAP with pancreaticobiliary malformation (male-to-female ratio: 1:4.3; p<0.01) and gallstone RAP (1:1.7; p<0.05) occurred predominantly in females in comparison with alcoholic RAP (1:0.2). Recurrence rate was 80% for AP with pancreaticobiliary malformation, significantly higher than for the others (p<0.01). Pancreas divisum was suspected as the etiology of mild RAP in 7 patients. Four RAP patients with pancreas divisum underwent endoscopic minor papilla sphincterotomy and improved. Pancreaticobiliary maljunction with biliary dilatation (choledochal cyst) was suspected as the etiology of mild RAP in 3 patients. The 3 RAP patients with choledochal cyst underwent prophylactic flow diversion surgery with complete resection of the dilated common bile duct, and achieved improvement. High confluence of pancreaticobiliary ducts was suspected as the etiology of mild RAP in 6 patients., Conclusion: Pancreaticobiliary malformation is one of the major causes of RAP. As some of them benefit from endoscopic or surgical treatment, morphology of the pancreaticobiliary duct system should be examined where possible in RAP patients.

Purpose: Annular pancreas (AP) is usually associated with duodenal obstruction in neonates. Pancreatitis with AP occurs frequently in adults but is rare in children. This article describes pancreatitis in children with AP and pancreatobiliary anomalies and its surgical treatment., Patients and Methods: Six children who underwent duodenal bypass for AP subsequently developed recurrent pancreatitis. Three had trisomy 21. Duodenoduodenostomy had been performed in 5 patients and gastrojejunostomy in 1 patient for neonatal duodenal obstruction. We reviewed overall management, imaging, and surgical treatment in these children., Results: All children subsequently complained of recurrent abdominal pain. Pancreatitis developed in 6 children, and magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography (ERCP) revealed associated pancreatobiliary anomalies such as pancreas divisum, pancreatobiliary malunion, choledochocele, and intraluminal duodenal diverticulum. In 5 cases, surgery for recurrent or chronic pancreatitis was performed. The range of follow-up was 11 to 54 months, and all children who underwent surgery had excellent results., Conclusions: Children with AP occasionally require reoperation for recurrent pancreatitis because of associate pancreatobiliary anomalies. Magnetic resonance cholangiopancreatography and ERCP provide excellent images of pancreatobiliary anomalies. Intraoperative cholangiopancreatography is also essential for accurate depiction of the ductal structure and selection of the appropriate surgical procedure., (Copyright 2010 Elsevier Inc. All rights reserved.)

Background: Anomalous pancreaticobiliary ductal junction (APBDJ) is believed to be one of the risk factors for gallbladder carcinoma. The present study aims to delineate the gallbladder carcinoma characteristics associated with APBDJ., Patients and Methods: Patients with gallbladder carcinoma associated with APBDJ between August 1992 and February 2005 were retrospectively reviewed. Two types of APBDJ classifications were considered: right-angle type (C-P) and acute-angle type (P-C)., Results: Ten of 218 patients (4.6%) with gallbladder carcinomas were associated with APBDJ. All patients were female with a mean age of 55.4 years (range 41 to 72 years). Gallstones were absent in nine patients. Seven patients (70%) had the P-C type and three patients (30%) had the C-P type. Survival differences between the P-C type and the C-P type of gallbladder carcinomas were noted (P=0.0269). Patients with incidentally detected gallbladder carcinoma had superior survival (P=0.0316)., Conclusion: Gallbladder carcinomas associated with APBDJ were significantly related to relatively young female patients without gallbladder stones. Survival outcomes in these patients were not different from those of gallbladder carcinoma without APBDJ. In particular, the P-C type of APBDJ seemed to be more associated with relatively advanced gallbladder carcinomas, and patients with incidentally detected gallbladder carcinomas with APBDJ had superior survival.

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- existing intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.

Introduction: Bile duct perforation (BDP) with resultant biliary ascites in children is a rare clinical condition. The aetiopathogenesis is still an enigma, with increasing evidence suggesting anomalous union of pancreaticobiliary ductal (AUPBD) system as the prime causative factor., Clinical Picture: We report 2 cases of spontaneous perforation of the bile duct confirmed on histopathological examination as choledochal cyst, in a 6-month-old female child and a 4-year-old boy who presented with subtle clinical symptoms., Treatment and Outcome: Both patients were successfully managed by excision of the gall bladder and common bile duct and Roux-en- Y hepaticojejunostomy. This procedure was performed following initial cholecystostomy drainage in the second case., Conclusions: From the available literature and experience with our patients, BDP is not merely spontaneous but may be related to AUPBD and choledochal cyst.

Background/aims: The clinical features of pancreatitis in patients with an anomalous union of the pancreatobiliary duct (AUPBD) are unclear. This study analyzed the clinical features, pathogenic mechanisms, risk factors, and clinical courses after treatment in AUPBD patients., Methodology: The medical records and cholangiopancreatograms of 58 patients with AUPBD and a choledochal cyst diagnosed between 1982 and 2001 were retrospectively reviewed., Results: Sixteen (27.6%) out of the 58 AUPBD patients experienced pancreatitis. The incidence of pancreatitis was significantly higher in those patients with a long (>21mm) and wide (>5mm) common channel, a wide diameter of the proximal pancreatic duct (>2.5mm), the presence of a filling defect in the common channel, and the presence of a pancreatic duct anomaly (p<0.05). A bile duct diversion from the pancreatic duct by a choledochal cyst excision prevented the recurrence of pancreatitis in most cases. A duodenopancreatectomy was required in some cases., Conclusions: The morphological characteristics of the common channel and pancreatic duct contribute to the development pancreatitis in AUPBD patients and bile reflux into the pancreatic duct might be the main mechanism of pancreatitis in these patients.

Purpose: To retrospectively analyze prospective magnetic resonance (MR) cholangiographic interpretations of findings and compare them with clinical outcome and to determine the accuracy of MR cholangiography in depicting extrahepatic biliary atresia and helping to distinguish it from other causes of neonatal jaundice., Materials and Methods: Twenty-six infants (15 male, 11 female; median age, 2 months) underwent MR cholangiography with a 1.5-T MR imaging unit. Original interpretations were compared with clinical outcome. Statistical analysis was performed to determine the accuracy of MR cholangiography in depicting extrahepatic biliary atresia. Equivocal cases and any cases lost to follow-up were excluded., Results: Findings in six of 26 infants were interpreted as normal, and none of five patients (one lost to follow-up) had biliary atresia or other surgical lesions; two were abnormal but not suggestive of biliary atresia (one false-negative finding); 12 were consistent with biliary atresia (three false-positive findings); four demonstrated a choledochal cyst; and two were equivocal. MR cholangiography accuracy was 82% (19 of 23); sensitivity, 90% (nine of 10); and specificity, 77% (10 of 13) for the detection of extrahepatic biliary atresia, with a positive predictive value of 75% (nine of 12) and a negative predictive value of 91% (10 of 11)., Conclusion: Results of this study found that MR cholangiography is 82% accurate, 90% sensitive, and 77% specific for depicting extrahepatic biliary atresia. Contrary to previous reports, false-positive and false-negative findings occur at MR cholangiography.

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.

Anomalous pancreaticobiliary junction often leads to biliary tract carcinoma but only rarely to pancreatic carcinoma. We report three cases of pancreatic carcinoma associated with anomalous pancreaticobiliary junction. All three were female with a mean age of 68 years. Carcinomas were located in the pancreatic head (n = 2) or body (n = 1). None had choledochal cyst and one had experienced recurrent acute pancreatitis. All carcinomas were at an advanced stage with a poor prognosis. No unique imaging or histologic findings of the carcinomas could be identified. Attention should be paid to the possibility of pancreatic carcinoma in patients with anomalous pancreaticobiliary junction, particularly in aged patients. Early diagnosis and treatment of anomalous pancreaticobiliary junction may prevent development of pancreatic carcinoma.

Congenital cysts of the biliary tract are rare anomalies generally observed in pediatric age, exceptionally in adults. The different extension and the morphology of cystic lesions make possible a classification in subtype. This disease, of Len asymptomatic, is characterized by high incidence of complications such as pancreatitis, cholangitis, and cancer. For these reasons, congenital cystic dilatation of bile duct should be radically treated by complete resection of the dilated extraepatic biliary tract. A review of the International Literature and a rare case of congenite dilatation of the intra and extraepatic biliary ducts in a female 54 years old, treated by choledochal resection with hepatico-jejunostomy on Roux en Y segment, are presented.

Background: The aim of this study was to establish an optimal management strategy for pancreatic disorders associated with anomalous pancreaticobiliary junction (APBJ)., Methods: In 64 adult surgical cases of APBJ (common channel 15 mm or longer) (43 with and 21 without choledochal cyst), associated pancreatic disorders, pancreatographic results, and treatment outcomes were analyzed., Results: Of 64 patients, 56 had pancreatobiliary symptoms. Twenty-four patients (38%) had pancreatic disorders: acute pancreatitis (n = 20), chronic calcifying pancreatitis (n = 2), and pancreatic carcinoma (n = 2). Twenty patients (31%) had abnormal pancreatograms. The incidence of acute pancreatitis was significantly higher in patients with an abnormal pancreatogram, particularly dilatation, protein plugs or stones of the common channel or main pancreatic duct, and coexisting pancreatic ductal anomaly. All patients with choledochal cyst underwent cyst excision and hepaticojejunostomy. Eleven patients without choledochal cyst or pancreatobiliary carcinoma underwent cholecystectomy alone. Protein plugs and pancreatic stones were extracted through the bile duct stump or by sphincterotomy. No patients experienced pancreatitis during a mean postoperative follow-up of 6.7 years., Conclusions: In managing APBJ, attention should be paid to the possibility of associated pancreatic disorders and an abnormal pancreatogram. APBJ with choledochal cyst requires cyst excision. Cholecystectomy alone may be adequate for APBJ without cyst.

Objective: Since choledochal cyst is frequently associated with the anomalous union of pancreaticobiliary duct (AUPBD), AUPBD has been regarded to be the etiologic factor of choledochal cyst. However, the clinical significance of AUPBD an patients with choledochal cyst has not been clearly defined. Therefore, to clarify the significance of AUPBD in choledochal cyst patients, we compared the clinical features of patients with choledochal cyst according to the presence or absence of AUPBD., Methods: Among 52 cases which were diagnosed as choledochal cyst out of 5,037 ERCP referrals between August 1990 and December 1996, we selected 44 cases, in which the pancreaticobiliary junction was clearly visualized on cholangio-pancreaticography. These cases were divided into AUPBD-present group (n = 28) and AUPBD-absent group (n = 16). Clinical features were compared between the two groups. Furthermore, in AUPBD-present group, clinical data were also analyzed according to Kimura's classification of AUPBD., Results: In our study, AUPBD was associated with choledochal cyst in 28 (64%) cases. AUPBD was found only in type I and IV according to Todani's classification of choledochal cyst. There were no significant differences between the AUPBD-present group and the AUPBD-absent group in the incidence of gallstone disease, while the incidence of acute inflammation was 93% (26/28) in the AUPBD-absent group (p < 0.01). Carcinoma developed only in the AUOBD-present group (9/28, 32%) (p < 0.05). Pancreatic disorders (i.e. pancreatic stone, pancreatitis or pancreatic cancer) occurred in 12 of 28 cases in the AUPBD-present group (43%), while only in 1 of 16 cases in the AUPBD-absent group (6%) (p < 0.05)., Conclusion: AUPBD associated with choledochal cyst may have implications not only as a possible etiologic factor but also as an important factor that may affect the clinical course, surgical planning and prognosis. In cases with choledochal cyst, we should make an effort to evaluate the presence of AUPBD.

Background: Excision is the treatment of choice for choledochal cyst, and free bile drainage is essential to avoid ascending cholangitis. However, anastomosis between the relatively narrow common hepatic duct and bowel (conventional anastomosis) in case of type IV-A cyst, co-existing biliary anomalies and anatomical variations may cause ascending cholangitis resulting from insufficient biliary decompression., Methods: One hundred and four patients with choledochal cyst were treated by cyst excision. Conventional anastomosis was performed in 22 patients and hilar anastomosis in 82., Results: An anastomotic stricture developed in nine of the 22 conventional anastomosis cases and all required reoperation. Of the 82 hilar anastomosis cases, only one required reoperation for a primary stricture. Co-existing biliary anomalies and anatomical variants were seen in 26 patients: (1) primary stricture in 18; (2) aberrant posterior duct in two; (3) low confluence of the hepatic ducts in two; (4) aberrant hepatic artery in two; and (5) very small bile duct in two. All 26 patients underwent widening of the ductal lumen (ductoplasty) or additional procedures., Conclusion: Complete removal of the extrahepatic bile duct and a wide hilar anastomosis is essential to prevent ascending cholangitis. Ductoplasty or additional procedure can be performed only at the hilum.

Further studies on image techniques and hepatobiliary scintigraphy for diagnosis of biliary diseases revealed details of the whole biliary ductal system and appeared some kinds of biliary malformation, which are clinically important and had been unknown yet, such as anomalous arrangement of the pancreatico-biliary ductal system, congenital biliary strictures and congenital dilatation of the intrahepatic bile duct associated with choledochal cyst. We had 42 cases with primary intrahepatic calculi for these 12 years and they had Alonso-Lej Type I cysts with dilatation of the intrahepatic bile duct and congenital biliary strictures in the-hepatic hilum. Furthermore, we had 30 cases with both the biliary malformations, but without intrahepatic stones. Consequently, coexistence of both these anomalies of the bile duct is thought to be the basis for the formation of primary intrahepatic calculi.

The concept of congenital biliary dilatation (CBD) was established by Alonso-Lej whose classification has been widely accepted and become a standard. Todani, Komi and several investigators then added additional subclassifications and/or made a proposal of new classification. The trias, abdominal pain, abdominal mass and jaundice, had been characteristically found in these cases, but recently asymptomatic cases are often found. Many reports have shown that the cases with cystic dilation of the biliary duct frequently associated with biliary carcinoma and pancreaticobiliary maljunction (PBM), an anomalous union of pancreatic duct with biliary tree since the Rabbit's first report. Whereas extracorporeal ultrasound and CT scan are minimally invasive diagnostic procedures for these cases, a direct cholangiography, ERCP and PTC are the diagnostic procedures that make a definite diagnosis for the appropriate treatment. Recently, endoscopic ultrasound (EUS), helical CT and magnetic resonance cholangiopan-creatography (MRCP) have drawn an attention since these methods are less invasive than those ever being used. In particular, MRCP will be suitable for the screening of preoperative examination. Whereas cystoduodenostomy used to be performed in the past for these cases, the resection of dilatted bule duct along with the diversion of bile from pancreatic juice should be performed to prevent biliary carcinoma. Cholecystectomy along with the resection of bile duct and the biliary diversion from pancreatic juice should also be performed for cases of PBM without CBD because mutagenicity of bile mixed with pancreatic juice and K-ras point mutation in noncancerous bile duct epithelium in cases of PBM without CBD were demonstrated.

Congenital dilatation of bile duct (CDBD) or choledochal cyst has been demonstrated to be associated with pancreatico-biliary maljunction (PBM), and its various clinical signs and symptoms have been shown to be closely related with the presence of PBM. In almost all of the patients who were less than one year of age, the disease was of the cystic type and patients presented with either a palpable mass or jaunce as the main symptom. In patients with more than one year of age, the disease was of either the cystic or cylindrical type. A history of episodes of abdominal pain accompanied by elevated levels of serum amylase was present in most patients with both types of diseases. Histologic sections from the patients showed glandular formation with chronic inflammation, a result of refluxed activated pancreatic juice (possibly phospholipase A2): in contrast, histologic sections from the remaining patients of all ages showed only thickening of the fibrous layer. Thus, such variable morphologic features and clinical signs and symptoms in CDBD are highly dependent on two factors-the age at oset and the reflux of pancreatic juice into the bile duct through the common channel.

Congenital dilatation of the bile duct (CDBD), or choledochalcyst, is often complicated by recurrent pancreatitis. Reflux of pancreatic juice into the bile duct through pancreaticobiliary maljunction (PBM), an anomaly commonly associated with CDBD, and ensuing activation of pancreatic enzymes could be involved in the pathophysiologic mechanism of recurrent pancreatitis. A study was undertaken to follow the time course of the activity of phospholipase A2 (PLA2) in animal models of PBM. The assay procedures for PLA2 were evaluated, as were the conditions for separating the active enzyme from its inactive proenzyme (pro-PLA2) by immunoblotting. A rat model was designed according to Block's method with some modifications. The kinetics of prophospholipase A2 (proPLA2) activation in bile was examined by measuring PLA2 activity and by immunoblotting using anti-rat pancreatic enzyme antibody after separating PLA2 from its zymogen under nonreducing conditions. Experimental animals were divided into three groups: group 1 (PBM group) in which bile and pancreatic juice were mixed with occlusion of the papilla; group 2, in which the papilla and hepatic hillus were occluded without mixing the two juices; and group 3, in which simple laparotomy was done. In group 1 animals, pro-PLA2 in bile was activated to its active form. In group 2 animals, where proPLA2 was predominant, there was only slight elevation of PLA2 activity in bile. In group 1 an immunohistologic study demonstrated localization of PLA2 around necrotic foci in the pancreatic parenchyma. These results suggest the involvement of activated PLA2 in the pathogenesis of choledochal cystassociated pancreatitis.

The high incidence of biliary tract carcinoma in patients with anomalous pancreaticobiliary ductal junction (APBDJ) with or without choledochal cyst (CC) has been well documented. Twenty-two patients with APBDJ were divided into three groups: Group A, four patients not associated with CC and biliary tract carcinoma; Group B, 13 patients with CC but without biliary tract carcinoma; and Group C, five patients with biliary tract carcinoma (four with and one without CC). Profiles of bile acids in the gall-bladder and/or common bile duct were analysed in these patients and compared with those in the control patients with cholecystlithiasis to examine the hypothesis that the levels of deoxycholic acid (DCA) and lithocholic acid (LCA) are elevated in patients with APBDJ because these secondary bile acids are mutagenic. Bile acids were quantified by gas-liquid chromatography. Total bile acid concentration in the gall-bladder bile was significantly lower in any group with APBDJ than that of controls. In the gall-bladder, increased proportion of chenodeoxycholic acid (CDCA) in Group A and B, decreased proportion of DCA in Group B and increased proportion of cholic acid (CA) in Group C were found in bile. In the bile duct, total bile acid concentration and proportion of DCA were significantly low in bile from Group C and decreased proportion of DCA and increased proportion of CDCA were found in bile from Group B. In both the gall-bladder and hepatic bile, proportion of LCA was not significantly different between any intergroups.(ABSTRACT TRUNCATED AT 250 WORDS)

A pair of monozygotic twins, 2-year-old Japanese girls, discordant for both anomalous pancreaticobiliary ductal junction and congenital biliary dilatation, are reported. The pathogenesis of the disease is discussed in relation to the occurrence of discordant monozygotic twins.

In a 61-year-old woman, villous adenocarcinoma of the gallbladder was associated with a congenital choledochal cyst and anomalous pancreaticobiliary ductal junction. The patient had a history of congenital choledochal cyst treated by cystoduodenostomy 27 years previously. The condition was demonstrated by intraoperative cholangiography and histologically confirmed. Extended cholecystectomy, regional lymph node dissection, excision of the cyst and hepaticojejunostomy were performed successfully.

Congenital dilatation of the bile duct (CDBD) or choledochal cyst has been demonstrated to be associated with an anomalous junction of the pancreaticobiliary ductal system. Multifarious clinical signs and symptoms of CDBD have been shown to be closely related with the presence of this anomalous junction. In the present study, 100 instances of CDBD treated surgically at our institutions during a 30 year period were classified into two types according to the morphologic features of dilatation of the bile duct; there were 77 instances of the cystic type and 23 of the cylindric type. Morphologic features of the lesion, clinical signs and symptoms and laboratory findings in these 100 instances were clinically analyzed. In almost all of the patients who were less than one year of age, the disease was of the cystic type and patients presented with either a palpable mass or jaundice as the main symptom. In patients more than one year of age, the disease was of either the cystic or cylindric type. A history of episodes of characteristic abdominal pain accompanied by elevated levels of serum amylase was present in 70 of the patients with the cystic type of disease and in all of the patients with the cylindric type. Histologic sections from the patients showed glandular formation with chronic inflammation, possibly a result of refluxed activated pancreatic juice; in contrast, histologic sections from the remaining patients of all ages showed only thickening of the fibrous layer. Thus, such variable morphologic features and clinical signs and symptoms in CDBD are highly dependent on two factors--the age at onset and the reflux of pancreatic juice into the bile duct through the common channel.

Pancreatic enzymes in the bile of congenital choledochal cyst with anomalous pancreaticobiliary ductal union were analyzed. Almost all pancreatic proteases were already activated in the bile aspirated immediately after insertion of PTCD tube. However, inactive proteases gradually increased after continuation of drainage by PTCD. Pancreatic protease activities appeared again after clamping the PTCD tube. In vitro study of trypsin activities in the bile containing no initial trypsin activity, active type of trypsin did not appear throughout the experimental period. It is suggested that continuous reflux of pancreatic juice into the bile and certain incubation time are necessary for activation of pancreatic protease in bile of congenital choledochal cyst.