Relationship between an involvement of Oddi's sphincter in a choledochopancreatic junction and clinical appearances in 120 patients with anomalous arrangement of the pancreatico-biliary ductal system

We studied relationship between an involvement of Oddi's sphincter in a choledochopancreatic junction and clinical appearances in 120 patients with anomalous arrangement of the pancreaticobiliary ductal system. The 120 composed of 83 with a sphincteric mechanism at the junction of the common bile and pancreatic duct and 37 without the sphincteric mechanism. We found that influences of sphincteric mechanism at the junction closely related to the length of the common duct between an orifice of the papilla of Vater and a junction of the common bile and pancreatic duct. And in all 83 with a sphincteric mechanism at the junction the length of the common duct showed less than 1.5 cm, and in all 37 without a sphincteric mechanism at the junction the common duct showed more than 1.5 cm in length. There was no correlation between a distribution of the sphincter muscle and clinical appearances, association with congenital choledochal cyst, cholelithiasis and bile duct cancer in the 120 patients.     

Effect of cholecystectomy for gallstones on the surface of the papilla of Vater and the diameter of the common bile duct

BACKGROUND AND STUDY AIMS: To date, little is known about changes in the size of the papilla of Vater in patients with gallstones. Most of the research concerning these patients has investigated changes in the diameter of the common bile duct and pressure in the sphincter of Oddi region. The latest research has not confirmed the prevalent opinion that the common bile duct dilates after cholecystectomy; moreover, knowledge about changes in the form and size of the papilla of Vater remains poor. The aim of our research was to measure the surface of the papilla of Vater and the diameter of the periampullary pancreatic part of the common bile duct before surgery, then 3 months and 6 months after surgery, using endoscopic ultrasound. PATIENTS AND METHODS: In patients with symptomatic gallstones but with no evidence of cholestasis or stones in the common bile duct, the surface of the papilla of Vater and the diameter of the periampullary pancreatic part of the common bile duct were measured before surgery by endoscopic ultrasound. In the postoperative period, measurements were repeated in patients with an early occurrence of nausea, distension, bloating and pain in the upper abdomen and in patients without any symptoms in the same time period. The surface of the normal papilla of Vater and the diameter of the periampullary pancreatic part of the common bile duct were determined in healthy subjects. RESULTS: In all the patients included in our study, the surface of the papilla of Vater was enlarged before surgery and, after 3 months, was even larger. However, after 6 months, it was practically the same as before surgery. The diameter of the periampullary pancreatic part of the common bile duct before surgery and 6 months after surgery was within normal limits. However, a statistically significant and transient increase in the diameter was noticed in the third month after cholecystectomy. The results were almost the same for symptomatic and asymptomatic patients. CONCLUSION: In patients with symptomatic gallstones, the surface of the papilla of Vater is increased while the diameter of the periampullary pancreatic part of the common bile duct is normal. Cholecystectomy is an intervention that causes an additional transient increase in the surface of the papilla of Vater and a transient increase in the diameter of the periampullary pancreatic part of the common bile duct. The surface of the papilla of Vater does not reach normal values but the diameter of the periampullary pancreatic part of the common bile duct is normalized 6 months after surgery. There is no relation between clinical disorders and changes in the morphology of the papilla of Vater and the periampullary pancreatic part of the common bile duct after cholecystectomy.     

Villous tumor of the papilla of Vater presenting with hypopotassemia

We report a case of villous tumor of the papilla of Vater associated with hypopotassemia. The patient was a 73-year-old woman who presented with jaundice and fever. She had a history of diabetes mellitus and liver dysfunction. Laboratory studies revealed that levels of total bilirubin, alkaline phosphatase, and C-reactive protein, and the white blood cell count were elevated (suggestive of cholangitis) and that the serum potassium level was markedly reduced, to 1.9 mEq/l (normal value 3.5–5.0 mEq/l). Duodenoscopy showed a villous tumor arising in the papilla of Vater. Percutaneous transhepatic biliary drainage was performed. Approximately 700–1500 ml of bile with viscous mucoid fluid was drained daily. Percutaneous transhepatic cholangioscopy showed a papillary lesion in the distal common bile duct. Biopsied specimens from both percutaneous transhepatic cholangioscopy and duodenoscopy disclosed tubulovillous adenoma. Endoscopic ultrasonography showed that the tumor had spread to the main pancreatic duct as well as to the common bile duct. The patient underwent pylorus-preserving pancreaticoduodenectomy. Pathology examination disclosed well differentiated adenocarcinoma, carcinoma in situ, in tubulovillous adenoma. The cancer cells were observed at the bottom of the tumor spreading in the common bile duct. This is a rare case of a patient presenting with hypopotassemia associated with a tubulovillous tumor of the papilla of Vater that secreted mucoid material.     

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vater’s papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vater’s papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.     

"Duodenal intussusception" due to adenoma of the papilla of Vater

The case of a 55-year-old woman with a pedunculate adenoma of the papilla of Vater is presented. Diagnostic imaging modalities including ultrasonography, CT scan, magnetic resonance of cholangiopancreatography, simultaneous duodenography and cholangiography, and angiography showed a giant tumor protruding intraluminally and moving forward in the duodenum by peristalsis. It had a duodenal intussusception-like appearance, with remarkable left-lower deviation of the common bile duct and major pancreatic duct in the papilla of Vater as far as the left side of the aorta. Episodes of jaundice or ileus were absent, probably because the tumor was mobile in the duodenum. As biopsy specimens showed no malignancy and intraductal ultrasonography in the common bile duct revealed no intraductal invasion of the tumor in the papilla of Vater, the patient underwent transduodenal papillectomy with papilloplasty with pancreatic ductoplasty. Pathological diagnosis of consecutive specimens was a papillary adenoma with moderate atypia and occasional tubular structure. There seems to be an exceptional subtype of the tumor in the papilla of Vater, like this case, demonstrating the duodenal intussusception-like appearance without prominent clinical symptoms.     

Signet-ring-cell carcinoma of the ampulla of Vater: a case report

We encountered a rare variant of carcinoma of the ampulla of Vater in a 68-year-old man with postprandial abdominal pain and nausea. Ultrasonography and computed tomography showed a dilated common bile duct and main pancreatic duct. At duodenoscopy, the papilla of Vater was enlarged, and biopsy specimens taken from the papilla revealed signet-ring-cell carcinoma. Endoscopic ultrasonography showed a hypoechoic tumor without pancreatic invasion. Pylorus-preserving pancreatoduodenectomy was performed. Histologic examination of resected specimens indicated lymphatic and vascular invasion, but surgical margins were tumor-free. The patient is alive and disease-free 10 months after the operation.     

Gallbladder carcinoma with choledochoduodenal fistula: a case report with surgical treatment.

A 79 year-old man was admitted to our hospital because of upper abdominal pain and nausea. A mobile tumor was palpable in the right upper abdomen. Abdominal ultrasonography, computed tomography and celiac angiography revealed a gallbladder tumor. Endoscopic retrograde cholangiopancreatography revealed a fistula 1.5 cm oral to the orifice of the papilla of Vater, dilatation of the common bile duct, and a filling defect in the gallbladder. Pancreatoduodenectomy associated with reconstruction using Imanaga's method was performed under a pre-operative diagnosis of gallbladder carcinoma with choledochoduodenal fistula. The gallbladder contained a tumor and two bilirubin stones impacted in the orifice of the duodenal papilla. Histological studies confirmed that the gallbladder tumor was a mucinous adenocarcinoma and had not infiltrated the bile duct. We speculated that choledochoduodenal fistula stimulated the development of cancer due to chronic irritation from pancreatic juice reflux.     

Parapapillary choledochoduodenal fistula associated with cholangiocarcinoma

Parapapillary choledochoduodenal fistula is a rare disorder. We herein report a case of parapapillary choledochoduodenal fistula associated with cholangiocarcinoma. A 61‐year‐old woman was admitted to our hospital for further examination of a liver tumor. She had no clinical symptoms, but computed tomography scans showed an irregularly contoured liver tumor which was histologically confirmed to be adenocarcinoma, by a needle biopsy examination. Duodenal fiberscopy revealed a fistula orifice 1.0 cm proximal to the orifice of the papilla of Vater, and endoscopic retrograde cholangiography through the fistula showed a communication to the common bile duct. Hypotonic duodenography demonstrated reflux of contrast material into the choledochoduodenal fistula. The bile sample collected from the common bile duct showed extremely high levels of pancreatic enzymes, including amylase, phospholipase‐A2, and elastase‐I. Furthermore, Helicobacter DNA was detected in bile by polymerase chain reaction (PCR) analysis. This experience suggests to us that parapapillary choledochoduodenal fistula may be a risk factor for biliary tract carcinoma, and surgical management is the treatment of choice for this rare condition, even when the patient has no significant clinical symptoms.     

A collision tumor composed of cancers of the bile duct and ampulla of Vater--immunohistochemical analysis of a rare entity of double cancer

Collision cancer of the bile duct and the papilla of Vater is an extremely rare entity. This is the first report of a case of bile duct collision cancer. A 75-year-old man presented with jaundice. Computed tomography showed isodensity masses in the middle bile duct and the papilla of Vater. Magnetic resonance cholangiopancreatography showed a tuberous filling defect in the middle bile duct. Gastroduodenal endoscopy showed a tumor with ulceration at the papilla of Vater. The patient was diagnosed with cancers of the middle bile duct and the papilla of Vater, and a pylorus-preserving pancreatoduodenectomy was performed. On pathological examination, the tumor in the middle bile duct showed a well differentiated carcinoma that had spread to the proximal bile duct, whereas the tumor in the papilla of Vater showed a papillo-tubular carcinoma with a marked production of mucin, suggesting an intestinal type of ampullary cancer. These tumors were directly communicated by microscopic findings. Therefore, the immunohistochemical characteristics were analyzed, using several antibodies, to determine whether the origins of the 2 cancers were different or not. As a result, it was concluded that this was a case of collision cancer of the middle bile duct and the papilla of Vater.     

A long-term surviving patient with cancer of papilla of Vater after local resection

We report successful local resection for cancer of papilla of Vater in an 86-year-old woman. She was referred to our hospital because of right hypochondralgia. Abdominal ultrasonography and computed tomography showed marked dilatation of the common bile duct (CBD). Endoscopic retrograde cholangiography disclosed a small shadow defect in the terminal of the dilated CBD. Biopsy of the papilla revealed well-to-moderately differentiated adenocarcinoma. Considering her extreme old age and keeping in mind her quality of life after the operation, and the finding that the tumor was localized within the papilla and highly differentiated, we performed local resection. In addition, the intrapancreatic portion of the CBD and part of the main pancreatic duct (MPD) were further resected to secure a negative margin, confirmed by frozen section. The MPD was reapproximated to the duodenal mucosa and a choledocho-duodenostomy was performed for CBD reconstruction. Histopathological examination showed the tumor was papillary adenocarcinoma, 10 × 15 mm in size; there was no invasion beyond the sphincter of Oddi, it had partly infiltrated the CBD, but had not invaded to the pancreas or duodenum. The patient's postoperative course was not eventful and she has had good quality of life for the past 6 years since the operation, without any evidence of recurrence. Although radical pancreaticoduodenectomy is now the standard procedure in patients with malignant tumor of the papilla of Vater, local resection is a reasonable alternative for high-risk patients with highly differentiated, apparently localized carcinomas.     

Endoscopic manometry of common bile duct and the sphincter of Oddi in patients with biliary tract stones and in controls]

Endoscopic manometry using microtransducer was applied to evaluate the motor activity of the sphincter of Oddi (SO) and common bile duct (CBD) in patients with biliary tract stones and in controls. CBD pressure, SO pressure and the incidence of irregular phasic wave pattern (IRPWP) were relatively higher in patients with biliary tract stones than those in controls. There was no significant difference in CBD and SO pressure between patients with dilated CBD and patients with non-dilated CBD, however the incidence of IRPWP was significant higher in patients with dilated CBD than that in patients with non-dilated CBD. SO pressure and the incidence of IRPWP were significant higher in patients with papillitis than those in patients without it. Patients with valvular orifice of the papilla of Vater showed high incidence of IRPWP, and in such cases it was suggested that SO had different motor functions for CBD and pancreatic duct because these ducts were seen opening onto the papilla separately according to the findings of pancreatocholangiogram. We concluded that endoscopic manometry of CBD and the SO was useful to evaluate the motor activity of SO and provided a basis for the assessment of the motor function mechanism of SO. Our results indicated the motor activity of SO had close relationship with biliary tract stones, CBD dilatation and endoscopic findings of the papilla of Vater.     

Motor pattern of the sphincter of Oddi in patients with juxtapapillary diverticula

In 15 patients with duodenal diverticula close to the papilla of Vater we evaluated the motor activity of the sphincter of Oddi by endoscopic biliary manometry. Both basal pressure and phasic activity were similar to those in nine patients without biliopancreatic disease and in 60 patients with common bile duct stones. The anatomical relationship between the papilla and diverticula did not lead to any change in the motor pattern. If juxtapapillary diverticula are associated with a higher prevalence of biliopancreatic disease, dysfunction of the sphincter of Oddi does not seem to play a pathogenic role.     

胰管内乳头状黏液瘤的临床研究

目的 通过探讨胰管内乳头状黏液瘤（IPMT）的临床、逆行胰胆管造影（ERCP）表现及病理特征,提高对IPMT的认识。方法 对9例IPMT患者的临床、影像学表现及病理资料作回顾性分析。结果 9例中男女之比为2：1,年龄为37-76岁,平均68.4岁。上腹痛为最常见症状。肿瘤主要位于胰头部。ERCP可见十二指肠乳头肿大开口扩大,见大量黏液流出及主胰管扩张。病理表现多样性,可表现为腺瘤和腺癌。结论 IMPT是胰腺肿瘤的一种,其预后较一般的胰腺癌好,临床上应将其与其他胰腺肿瘤加以区别。     

Pancreatoscopy assisted by endoscopic sphincter dilation

To investigate tumor extension to the main pancreatic duct in patients with intraductal papillary mucinous tumors, we conducted pancreatoscopy assisted by endoscopic sphincter dilation (ESD). Two patients with intraductal papillary mucinous tumors were included in this study. The endoscopic sphincter dilation was performed using an Olbert balloon-tipped catheter. A mother-baby scope system was used for endoscopic examination. After dilation of the orifice, the babyscope was inserted smoothly. Tumorous lesions (especially the typical salmon-roe appearance of the tumors) were clearly observed in the main pancreatic duct in one patient, while no tumorous lesions were identified in the other patient. The ESD procedure may be applicable for assisting insertion of the endoscope through the papilla of Vater into the pancreatic duct. ESD is useful both for treating and for diagnosing pancreatic lesions. Received: June 19, 2001 / Accepted: February 22, 2002 Reprint requests to: N. Ueno, Present address: Third Department of Internal Medicine, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama 236-0004, Japan     

A case of adenosquamous carcinoma of the papilla of Vater]

Adenosquamous carcinoma of the papilla of Vater is a rare tumor and only a few cases have been reported so far. Here, we report a case of adenosquamous carcinoma in a 76-year-old male who presented with jaundice and right upper quadrant abdominal pain. Ultrasonography and enhanced abdominal CT scans showed dilated common bile duct (CBD) and intrahepatic bile duct (IHD) with a suspicious obstructing mass in distal CBD. On endoscopy, obstructing and ulcerated mass was noted on the papilla of Vater. Histopathological inspection of the biopsied specimens from mass showed adenosquamous cell carcinoma of the papilla of Vater. Since the patient refused operation, we inserted a self-expandable metallic stent in distal CBD. This is the first case report on adenosquamous carcinoma of the papilla of Vater in Korea.     

Chronic recurrent pancreatitis secondary to a submucosal ampullary tumor in a patient with neurofibromatosis

Summary We report a 45-year-old nonalcoholic patient with Von Recklinghausen's disease and chronic recurrent pancreatitis. On endoscopy, a benign-appearing submucosal tumor surrounded the papilla of Vater. Cannulation of the pancreatic duct showed a dilated ductal system with subsequent delayed drainage. Endoscopic manometry revealed elevated sphincter of Oddi pressures. Endoscopic sphincterotomy resulted in immediate normal drainage of the pancreatic duct and in subsequent clinical improvement.     

Mucin-Hypersecreting Tumor of the Pancreas with Mucin Extrusion through an Enlarged Papilla

Patient records, imaging films, macroscopic and microscopic features, and clinical follow-up data of seven Japanese patients with a mucin-hypersecreting tumor of the pancreas were reviewed. The mucin-hypersecreting tumor was defined as an enlarged major or minor papilla with a dilated orifice and visible mucin oozing on endoscopy. The series consisted of six men and one woman ranging from 47 to 79 yr old. The excretion of mucin through a patulous orifice of the enlarged ampulla of Vater was seen in six patients and of the enlarged minor papilla in the other patient with pancreas divisum. The main pancreatic duct was dilated in all patients. Amorphous mucin was seen in the dilated duct of five patients, and papillary nodules were present in two patients. Computed tomography and/or ultrasonography showed dilatation of the main pancreatic duct with multilocular cysts in six patients and with a solid tumor in the other patient. The tumor was located diffusely in the main pancreatic duct in one patient, whereas it was confined to branches in the head (four patients), body (one patient), or tail (one patient) of the pancreas in the six other patients. Histopathologic diagnosis was a cyst lined by hyperplastic mucus-secreting epithelium in one patient, mucinous cystadenoma in two, and mucinous cystadenocarcinoma in two. Five patients underwent resection and have survived for 1-46 months after the operation. The authors would like to emphasize this endoscopic syndrome because about half of mucin-hypersecreting tumors are malignant with a favorable prognosis.     

Temporary Pancreatic Duct Stenting to Prevent Pancreatitis Induced by Endoscopic Sphincter Dilation

Abstract: To investigate whether prophylactic temporary stenting of the main pancreatic duct would decrease the incidence of pancreatitis after endoscopic sphincter dilation (ESD), we conducted this procedure subsequent to ESD in 13 patients who had common bile duct stones. After ESD and extraction of stones, a stent was placed into the pancreatic duct across the papilla of Vater. The stent was removed endoscopically three days later. Stents were successfully placed in 12 (92.3%) patients. In 11 of the patients, there was no significant elevation of serum amylase values before and after the procedure. The remaining patient, whose stent was identified as occluded, demonstrated elevated serum amylase values. However, there were no other procedure-related complications. Although our report was only limited to 12 cases, our results suggest that temporary pancreatic stenting may help prevent postprocedual pancreatitis. (Dig Endosc 1999; 11:32–36)     

Undifferentiated carcinoma of the duodenal ampulla

This report demonstrates a case of undifferentiated carcinoma of the duodenal ampulla. A 74-year male experienced jaundice lasting for 3 weeks. An upper gastrointestinal series demonstrated a polypoid, ovoid filling defect in the second portion of the duodenum, and duodenoscopy disclosed a protruding mass involving the orifice of the papilla of Vater. Cholangiography demonstrated obstruction due to compression in the terminal common bile duct. Pylorus-preserving pancreatoduodenectomy was performed on the diagnosis of ampullary carcinoma. The gross specimen showed a polypoid mass, measuring 3.5 cm in diameter, in the ampulla, located mainly in the duodenal submucosal layer and invading the terminal common bile duct. Histologically, the tumor was small cell type, undifferentiated carcinoma, arising from the duodenal epithelium adjacent to the ampulla.     

Aberrant hepatic duct connected with the main pancreatic duct by anomalous pancreato-biliary ductal union: Case report

An aberrant hepatic duct directly connected to the main pancreatic duct with anomalous arrangement of the pancreato-biliary ductal system is reported here, the first report of such a case, to our knowledge. A 53-year-old woman was admitted to our hospital because of cholecystolithiasis with abdominal pain in the right upper quadrant. Endoscopic retrograde cholangiopancreatography (ERCP) showed that an aberrant hepatic duct, which independently drained the right posterior segment of the liver, connected to the main pancreatic duct at a high insertion site distal to the sphincter area of the major papilla. The common bile duct (containing stones), on the other hand, united with the main pancreatic duct in a normal fashion. Cholecystectomy and bile duct lithotomy were performed. The aberrant hepatic duct was separated from the main pancreatic duct just above the junction, and was anastomosed side-by-side to the common hepatic duct. The embryologic development of this lesion is not clear, but is discussed in this report.