Diagnostic criteria for pancreaticobiliary maljunction 2013

Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall. The diagnostic criteria for pancreaticobiliary maljunction were proposed in 1987. The committee of The Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) for diagnostic criteria for pancreaticobiliary maljunction began to revise the diagnostic criteria from 2011 taking recently advanced diagnostic imaging techniques into consideration, and the final revised version was approved in the 36^th Annual Meeting of JSPBM. For diagnosis of pancreaticobiliary maljunction, an abnormally long common channel and/or an abnormal union between the pancreatic and bile ducts must be evident on direct cholangiography, such as endoscopic retrograde cholangiopancreatography, percutaneous transpehatic cholangiography, or intraoperative cholangiography; magnetic resonance cholangiopancreatography; or three‐dimensional drip infusion cholangiography computed tomography. However, in cases with a relatively short common channel, it is necessary to confirm that the effect of the papillary sphincter does not extend to the junction by direct cholangiography. Pancreaticobiliary maljunction can be diagnosed also by endoscopic ultrasonography or multi‐planar reconstruction images provided by multi‐detector row computed tomography. Elevated amylase levels in bile and extrahepatic bile duct dilatation strongly suggest the existence of pancreaticobiliary maljunction.     

The presence of a common channel and associated pancreaticobiliary diseases: A prospective ERCP study

BACKGROUND: There are few endoscopic retrograde cholangiographic studies dealing with the relationship between the presence of a common channel and associated pancreaticobiliary diseases. AIMS: To endoscopically determine the incidence of common channels and assess whether the anatomy of the pancreaticobiliary ductal drainage into the duodenum has any bearing on pancreaticobiliary diseases. PATIENTS AND METHODS: We prospectively examined a common channel formation in 354 endoscopic retrograde cholangiographic cases. Cases with a common channel were divided into three groups: pancreaticobiliary maljunction, high confluence of pancreaticobiliary ducts with a common channel > or =6 mm in which the communication was occluded with the sphincter contraction, and common channel < or =5 mm in length. RESULTS: A common channel was observed in 131 cases (37.0%) including 11 with pancreaticobiliary maljunction and 13 with high confluence of pancreaticobiliary ducts. In cases with a common channel, the incidences of associated gallbladder carcinoma and acute pancreatitis were both 11.5%, which were significantly higher than 1.8% and 4.9% seen in cases without a common channel. In pancreaticobiliary maljunction cases, incidence of associated gallbladder carcinoma was 72.7%. CONCLUSION: The presence of an obvious common channel was observed in 37.0%. A close relationship is suggested between the presence of a common channel and development of gallbladder carcinoma and acute pancreatitis.     

Recent advances and problems in the management of pancreaticobiliary maljunction: feedback from the guidelines committee

Clinical practice guidelines on how to deal with pancreaticobiliary maljunction (PBM) were made in Japan in 2012, representing a world first. Using a narrow definition, congenital biliary dilatation involves only Todani type I (except type Ib) and type IV‐A, both of which are accompanied by PBM in almost all cases. Prospective ultrasonographic study revealed that the maximum diameter of the common bile duct increased with age. Pathophysiological conditions due to pancreatobiliary reflux occur in patients with high confluence of the pancreaticobiliary ducts, a common channel ≥6 mm long and occlusion of communication during contraction of the sphincter of Oddi. Since PBM can be diagnosed by magnetic resonance cholangiopancreatography, multi‐planar reconstruction multi‐detector row computed tomography and endoscopic ultrasonography, the current diagnostic criteria should be revised to take these diagnostic imaging modalities into consideration. According to a nationwide survey, biliary cancer occurred in 21.6% of adult patients with PBM with biliary dilatation and 42.2% of patients with PBM without biliary dilatation. In biliary cancer associated with PBM without biliary dilatation, 88.1% were gallbladder cancer. Treatment for PBM with biliary dilatation is prophylactic flow‐diversion surgery, but further investigations and surveillance studies are needed to clarify the appropriate surgical strategy for PBM without biliary dilatation.     

Spontaneous necrosis of solid gallbladder adenocarcinoma accompanied with pancreaticobiliary maljunction

A 71-year-old Japanese man with acute cholecystitis and an incarcerated gallbladder （GB） stone was admitted. Plain ultrasonography （US） incidentally detected a mass-like lesion in the fundus. Doppler US revealed that this elevated lesion had no blood flow. Computed tomography showed a relatively low-density mass, measuring 5 cm x 4 cm in diameter, with no positive enhancement. Magnetic resonance imaging showed a mass in the fundus with a slightly low intensity on Tl-weighted images and a slightly high intensity on T2-weighted images. We were agonized in making the qualitative diagnosis of mass-like lesions of the fundus, such as a benign tumor, cancer, or debris. We performed laparoscopic cholecystectomy, because the incarcerated GB stone clearly caused acute cholecystitis. Intra-operative cholangiography clearly revealed pancreaticobiliary maljunction. Amylase levels in the common bile duct and gallbladder were quite high. The elevated lesion in the fundus dearly showed severe necrosis. Although this necrotic nodule included non-viable adenocarcinoma cells, viable cancer cell nests were located in the muscularis propria and subcutaneous layer. Histopathological examination confirmed a solid adenocarcinoma. Thus, we diagnosed it as a gallbladder cancer, based on histopathological analysis of the resected specimen. We therefore undertook radical surgery, including wedge resection of the liver, radical dissection of regional lymph nodes, and resection of the extrahepatic bile duct. Histopathological findings revealed no cancer, hyperplasia or dysplasia in the additionally resected specimens. The patient was finally staged as T2, N0, H0, P0, M（-）, stage Ⅱ. We present the first case of spontaneous necrosis of solid gallbladder adenocarcinoma, with a review of previous studies.     

Magnetic resonance cholangiopancreatography study of pancreaticobiliary maljunction and pancreaticobiliary diseases

AIM:To discuss the imaging anatomy about pancreaticobiliary ductal union,occurrence rate of pancreaticobiliary maljunction(PBM)and associated diseases in a Chinese population by using magnetic resonance cholangiopancreatography(MRCP).METHODS:Data were collected from 694 patients who underwent MRCP from January 2010 to December2012.Three hundred and ninety-three patients were male and 301 patients were female.The age range was16-92 years old and the average age was 51.8 years.The recruitment indication of all cases was patients who had clinical symptoms,such as abdominal pain,jaundice,nausea and vomiting,which thus were clinically suspected as relative pancreaticobiliary diseases.All cases were examined by MRCP using single-shot fast spin-echo sequences.In order to obtain MRCP images,the maximum intensity projection was used.RESULTS:According to the anatomy of pancreaticobiliary ductal union based on our analysis of MRCP images,all cases were classified into normal type and abnormal type according to the position of pancreaticobiliary ductal union.The abnormal type could be further divided into P-B type,B-P type and the duodenum type.By analyzing the incidence of biliary stone and inflammation,pancreatitis,biliary duct tumors and pancreatic tumors between normal and abnormal types,significant differences existed.The abnormal group was more likely to suffer from pancreaticobiliary diseases.Comparing three different types of PBM that were associated with pancreaticobiliary diseases by using Fisher’s method,the result showed that there was no significant difference in the incidence of biliary stones,cholecystitis and pancreatic tumors.The incidence of pancreatitis in B-P type and P-B type was higher than that in duodenum type;the incidence of biliary duct tumor in B-P type was higher than that in P-B type;the incidence of biliary duct tumor in duodenum type was lower than that in P-B type.The incidence of congenital choledochus dilatation in normal type and abnormal type was similar,and there was no significant difference between the two types.CONCLUSION:Types of PBM are closely related to the occurrence of pancreaticobiliary diseases.MRCP has important clinical value in the early diagnosis and preventive treatment of pancreaticobiliary diseases.     

Endoscopic diagnosis of pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. As the action of the sphincter of Oddi does not functionally affect the junction in PBM patients, continuous pancreatobiliary reflux occurs, resulting in a high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst) and PBM without biliary dilatation (maximal diameter of the bile duct ≤ 10 mm). The treatment of choice for PBM is prophylactic surgery before malignant changes can take place. Endoscopic retrograde cholangiopancreatography (ERC P) is the most effective examination method for close obs ervation of the pattern of the junction site. When the communication between the pancreatic and bile ducts is maintained, despite contraction of the sphi ncter on ERCP, PBM is diagnosed. In these pat ients, levels of pancreatic enzymes in the bile are gene rally elevated, due to continuous pancreatobiliary reflux via a long common channel. Magnetic resonance cholangiopancreatography and 3D-computed tomography can diagnose PBM, based on findings of an anomalous union between the common bile duct and the pancreatic duct, in addition to a long common channel. Endoscopic ultrasonography and intraductal ultra sonography can demonstrate the junction outside the duodenal wall, and are useful for the diagnosis of asso ciated biliary cancer. Gallbladder wall thickness on ultra so nography can be a screening test for PBM.     

Carcinoma of the papilla of Vater following treatment of pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is frequently associated with biliary cancer due to reflux of pancreatic enzymes into the choledochus, and even after surgery to correct the PBM such patients still have a risk of residual bile duct cancer. Here, we report the case of a 59-year-old female with carcinoma of the papilla of Vater which developed 2.5 years after choledochoduodenostomy for PBM. During the postoperative follow-up period, computed tomography obtained 2 years after the first operation demonstrated a tumor in the distal end of the choledochus, although she did not have jaundice and laboratory tests showed no abnormalities caused by the previous operation. As a result, carcinoma of the papilla of Vater was diagnosed at an early stage, followed by surgical cure. For early detection of periampullary cancer in patients undergoing surgery for PBM, careful long-term followup is needed.     

Importance of early diagnosis of pancreaticobiliary maljunction without biliary dilatation

AIM: To clarify the strategy for early diagnosis of pancreaticobiliary maljunction (PBM) without biliary dilatation and to pathologically examine gallbladder before cancer develops.METHODS: The anatomy of the union of the pancreatic and bile ducts was assessed by using endoscopic retrograde cholangiopancreatography (ERCP). Patients with a long common channel in which communication between the pancreatic and bile ducts was maintained even during sphincter contraction were diagnosed as having PBM. Of these, patients in which the maximal diameter of the bile duct was less than 10 mm were diagnosed with PBM without biliary dilatation. The process of diagnosing 54 patients with PBM without biliary dilatation was retrospectively investigated. Histopathological analysis of resected gallbladder specimens from 8 patients with PBM without biliary dilatation or cancer was conducted.RESULTS: Thirty-six PBM patients without biliary dilatation were diagnosed with gallbladder cancer after showing clinical symptoms such as abdominal or back pain (n = 16) or jaundice (n = 12). Radical surgery for gallbladder cancer was only possible in 11 patients (31%) and only 4 patients (11%) survived for 5 years. Eight patients were suspected as having PBM without biliary dilatation from the finding of gallbladder wall thickening on ultrasound and the diagnosis was confirmed by ERCP and/or magnetic resonance cholangiopancreatography (MRCP). The median age of these 8 patients was younger by a decade than PBM patients with gallbladder cancer. All 8 patients underwent prophylactic cholecystectomy and bile duct cancer has not occurred. Wall thickness and mucosal height of the 8 resected gallbladders were significantly greater than controls, and hyperplastic changes, hypertrophic muscular layer, subserosal fibrosis, and adenomyomatosis were detected in 7 (88%), 5 (63%), 7 (88%) and 5 (63%) patients, respectively. Ki-67 labeling index was high and K-ras mutation was detected in 3 of 6 patients.CONCLUSION: To detect PBM without biliary dilatation before onset of gallbladder cancer, we should perform MRCP for individuals showing increased gallbladder wall thickness on ultrasound.     

Endoscopic findings of the duodenal papilla in patients with pancreaticobiliary maljunction

Background: To our knowledge this is the first report describing the relation between the form of the duodenal papilla and the pancreaticobiliary maljunction (PBM). Methods: The duodenal papilla was studied endoscopically in 46 patients with PBM and in 80 patients without PBM. Results: The duodenal papilla was classified into three types by three independent endoscopists. Conclusion: The oral protrusion of the duodenal papilla in patients with PBM was shorter or absent compared with the duodenal papilla in patients without PBM.     

Carcinoma of the cystic duct associated with pancreaticobiliary maljunction

Received: February 4, 2000 / Accepted: May 26, 2000     

Metachronous double cancer of the gallbladder and pancreas associated with pancreaticobiliary maljunction

A 50-year-old Japanese woman complained of abdominal and back pain. Ten years previously she had undergone cholecystectomy, choledochectomy, and Roux-en-Y choledochojejunostomy for gallbladder cancer associated with pancreaticobiliary maljunction without bile duct dilatation. On the present admission, ultrasonography (US) and computed tomography (CT) demonstrated a large mass, 60 mm in size, in the pancreatic tail. Endoscopic retrograde cholangiopancreatography (ERCP) showed obstruction of the main pancreatic duct in the tail of the pancreas and revealed that the pancreatic duct was joined to the bile duct 25 mm above the papilla of Vater. The patient underwent distal pancreatectomy, splenectomy, left adrenalectomy, and partial gastrectomy. Histological examination revealed moderately differentiated ductal adenocarcinoma that had invaded to the proper muscle of the stomach. Double cancer of the gallbladder and pancreas in a patient with pancreaticobiliary maljunction is rare. Although the etiology of cancer of the pancreas associated with pancreaticobiliary maljunction is unclear, we should pay close attention to the pancreas as well as the biliary tract during the long-term follow-up of patients with pancreaticobiliary maljunction after they have undergone a choledochojejunostomy.     

Synchronous double cancers of the common bile duct

We report an extremely rare case of synchronous double cancers of the common bile duct without pancreaticobiliary maljunction. Only two similar cases have been reported in the English literature. Endoscopic re-trograde cholangiopancreatography showed a tuberous filling defect in the middle and superior parts of the common bile duct, and mild stenosis in the inferior duct. Computed tomography (CT) showed a well enhanced mass in the middle and superior parts of the common bile duct. A single cancer of the middle and superior bile duct was suspected and extra-hepatic bile duct resection was performed. CT eleven months after surgery revealed enhanced inferior bile duct wall and a slightly enhanced tumor within it. Retrospective review of the CT images taken before first surgery showed enhanced inferior bile duct wall without intrabiliary tumor only on the delayed phase. The inferior bile duct tumor was suspected to have originally co-existed with the middle and superior bile duct tumor. Pancreaticoduodenectomy was performed subsequently. Histopathological examination revealed that the middle and superior bile duct tumor was a moderately differentiated tubular adenocarcinoma while the inferior bile duct tumor was a papillary adenocarcinoma. The two tumors were separated and had different histological findings and growth patterns, further suggesting that they were two primary cancers.     

Clinical implications of accessory pancreatic duct

The accessory pancreatic duct (APD) is the main drainage duct of the dorsal pancreatic bud in the embryo,entering the duodenum at the minor duodenal papilla (MIP).With the growth,the duct of the dorsal bud undergoes varying degrees of atrophy at the duodenal end.Patency of the APD in 291 control cases was 43% as determined by dye-injection endoscopic retrograde pancreatography.Patency of the APD in 46 patients with acute pancreatitis was only 17%,which was significantly lower than in control cases (P < 0.01).The terminal shape of the APD was correlated with APD patency.Based on the data about correlation between the terminal shape of the APD and its patency,the estimated APD patency in 167 patients with acute pancreatitis was 21%,which was signif icantly lower than in control cases (P < 0.01).A patent APD may function as a second drainage system for the main pancreatic duct to reduce the pressure in the main pancreatic duct and prevent acute pancreatitis.Pancreatographic f indings of 91 patients with pancreaticobiliary maljunction (PBM) were divided into a normal duct group (80 patients) and a dorsal pancreatic duct (DPD) dominant group (11 patients).While 48 patients (60%) with biliary carcinoma (gallbladder carcinoma,n=42;bile duct carcinoma,n=6) were identified in PBM with a normal pancreatic duct system,only two cases of gallbladder carcinoma (18%) occurred in DPD-dominant patients (P < 0.05).Concentration of amylase in the bile of DPD dominance was signifi cantly lower than that of normal pancreatic duct system (75 403.5 ± 82 015.4 IU/L vs 278 157.0 ± 207 395.0 IU/L,P < 0.05).In PBM with DPD dominance,most pancreatic juice in the upper DPD is drained into the duodenum via the MIP,and reflux of pancreatic juice to the biliary tract might be reduced,resulting in less frequency of associated biliary carcinoma.     

Endoscopic retrograde cholangiopancreatography in children with symptomatic pancreaticobiliary maljunction: A retrospective multicenter study

BACKGROUND Endoscopic retrograde cholangiopancreatography(ERCP) has been widely used in pediatric patients with cholangiopancreatic diseases.AIM To evaluate the efficacy, safety, and long-term follow-up results of ERCP in symptomatic pancreaticobiliary maljunction(PBM).METHODS A multicenter, retrospective study was conducted on 75 pediatric patients who were diagnosed with PBM and underwent therapeutic ERCP at three endoscopy centers between January 2008 and March 2019. They were divided into four PBM groups based on the fluoroscopy in ERCP. Their clinical characteristics, specific ERCP procedures, adverse events, and long-term follow-up results were retrospectively reviewed.RESULTS Totally, 112 ERCPs were performed on the 75 children with symptomatic PBM.Clinical manifestations included abdominal pain(62/75, 82.7%), vomiting(35/75,46.7%), acholic stool(4/75, 5.3%), fever(3/75, 4.0%), acute pancreatitis(47/75,62.7%), hyperbilirubinemia(13/75, 17.3%), and elevated liver enzymes(22/75,29.3%). ERCP interventions included endoscopic sphincterotomy, endoscopic retrograde biliary or pancreatic drainage, stone extraction, etc. Procedure-related complications were observed in 12 patients and included post-ERCP pancreatitis(9/75, 12.0%), gastrointestinal bleeding(1/75, 1.3%), and infection(2/75, 2.7%).During a mean follow-up period of 46 mo(range: 2 to 134 mo), ERCP therapy alleviated the biliary obstruction and reduced the incidence of pancreatitis. The overall effective rate of ERCP therapy was 82.4%; seven patients(9.3%) were lost to follow-up, eight(11.8%) re-experienced pancreatitis, and eleven(16.2%)underwent radical surgery, known as prophylactic excision of the extrahepatic bile duct and hepaticojejunostomy.CONCLUSION ERCP is a safe and effective treatment option to relieve biliary or pancreatic obstruction in symptomatic PBM, with the characteristics of minor trauma, fewer complications, and repeatability.     

A case of gallbladder carcinoma associated with pancreatobiliary reflux in the absence of a pancreaticobiliary maljunction： A hint for early diagnosis of gallbladder carcinoma

A 62-year-old man with progressive thickening of the gallbladder wall visited our outpatient clinic. The biliary amylase level in the common bile duct was 19900 IU/L and that of the gallbladder was 127000 IU/L, although endoscopic retrograde cholangiopancreatography revealed no pancreaticobiliary maljunction. Histology demonstrated a moderately differentiated adenocarcinoma of the gallbladder. Pancreatobiliary reflux and associated gallbladder carcinoma were confirmed in the present case, in the absence of a pancreaticobiliary maljunction. Earlier detection of the pancreatobiliary reflux and progressive thickening of the gallbladder wall might have led to an earlier resection of the gallbladder and improved this patient's poor prognosis.