A Case of Intrahepatic Cholangiocarcinoma Associated with Type IV Choledochal Cyst / 대한소화기학회지

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.

A Case of Primary Neuroendocrine Carcinoma of the Gallbladder Associated with Anomalous Union of the Pancreaticobiliary Duct

Primary neuroendocrine carcinoma of the gallbladder is extremely rare because normal gallbladder mucosa does not contain neuroendocrine cells. Neuroendocrine cells can be detected at sites of intestinal metaplasia induced by chronic inflammation, which may be the initial step in the development of neuroendocrine tumor of the gallbladder. Anomalous union of the pancreaticobiliary duct (AUPBD) is an uncommon congenital anomaly that is frequently associated with choledocholithiasis, cholangitis, pancreatitis, and cancer of the gallbladder or bile duct. In AUPBD, cancers of the gallbladder and bile duct can be induced by chronic inflammation. We report herein a case of large-cell neuroendocrine tumor of the gallbladder associated with AUPBD.

An anomalous union of the pancreaticobiliary duct that was treated by drainage of the accessory ampulla / 대한내과학회지

An anomalous union of the pancreaticobiliary duct is defined as a condition in which the junction between the common bile duct and the pancreatic duct is located outside the duodenal wall. Therefore, the sphincter of Oddi does not influence the junction; there is always communication between these ducts, and so pancreatic juice flows into the biliary tract through the communication, which may cause pancreaticobiliary diseases. Pancreas divisum is a congenital variant of the pancreatic ductal anatomy, and the mechanism is that the dorsal and ventral pancreatic buds fail to fuse during the gestation. We report here on a 21 year old woman who previously had suffered with recurrent pancreatitis, and an anomalous union of the pancreaticobiliary duct associated with the incomplete type of pancreas divisum was revealed by ERCP. She was treated with minor papilla sphincterotomy and stent insertion.

Premalignant Lesions of Gallbladder Carcinoma and Treatment / 영남의대학술지

Carcinoma of the gallbladder is an uncommon but highly malignant tumor with a poor five year survival rate. Early detection is very important for successful treatment because this tumor is very hard to cure in cases where it has advanced beyond the reach of surgical treatment. The purpose of this review was to evaluate risk factors for carcinoma of the gallbladder and determine the best management approach.. Solitary polys, more than one cm are considered to be predisposing factors for gallbladder carcinoma when they are found to be echopenic, sessile, and with a high cell density. Anomalous union of the pacreato-biliary duct(AUPBD) without a choledochal cyst is also considered to increase risk for gallbladder carcinoma. A polyps size of more than one cm and an AUPBD are indications for prophylactic cholecystectomy. The presence of gallstones is a well-established risk factor for the development of gallbladder carcinoma; risk appears to correlate with the stone size and the duration of chronic cholecystitis. Metaplastic changes of the gallbladder epithelium present with chronic cholecystitis and may indicate a premalignant lesion. Abnormal forms of cholecystitis such as xanthogranulomatous or a porcelain gallbladder also have malignant potential; cholecystoenteric fistula as well as bacterial infection of the gallbladder(typhoid, helicobacter species) also has malignant potential. In this review, the risk factors associated with carcinoma of the gallbladder are summarized with special attention to gallstones, polyps,AUPBD, and chronic inflammation.

Premalignant Lesions of Gallbladder Carcinoma and Treatment / 영남의대학술지

Carcinoma of the gallbladder is an uncommon but highly malignant tumor with a poor five year survival rate. Early detection is very important for successful treatment because this tumor is very hard to cure in cases where it has advanced beyond the reach of surgical treatment. The purpose of this review was to evaluate risk factors for carcinoma of the gallbladder and determine the best management approach.. Solitary polys, more than one cm are considered to be predisposing factors for gallbladder carcinoma when they are found to be echopenic, sessile, and with a high cell density. Anomalous union of the pacreato-biliary duct(AUPBD) without a choledochal cyst is also considered to increase risk for gallbladder carcinoma. A polyps size of more than one cm and an AUPBD are indications for prophylactic cholecystectomy. The presence of gallstones is a well-established risk factor for the development of gallbladder carcinoma; risk appears to correlate with the stone size and the duration of chronic cholecystitis. Metaplastic changes of the gallbladder epithelium present with chronic cholecystitis and may indicate a premalignant lesion. Abnormal forms of cholecystitis such as xanthogranulomatous or a porcelain gallbladder also have malignant potential; cholecystoenteric fistula as well as bacterial infection of the gallbladder(typhoid, helicobacter species) also has malignant potential. In this review, the risk factors associated with carcinoma of the gallbladder are summarized with special attention to gallstones, polyps,AUPBD, and chronic inflammation.

A woman in her twenties with gallbladder cancer associated with an anomalous union of the pancreaticobiliary duct / 대한내과학회지

An anomalous pancreaticobiliary ductal union is a rare congenital anomaly occurring in the biliary tract. This anomaly has been implicated as a cause of a choledochal cyst, bile duct and gallbladder carcinoma, and recurrent pancreatitis. A case is herein reported of a 29 year-old woman who suffered from advanced gallbladder cancer associated with an anomalous pancreaticobiliary ductal union (AUPBD). We here emphasize the causative role of AUPBD in the development of gallbladder cancer with review of the Korean literatures on gallbladder cancer associated AUPBD.

A case of an anomalous union of the pancreaticobiliary duct associated with incomplete pancreas divisum / 대한내과학회지

There are many pancreatic ductal anomalies in which there are clinically significant anomalies associated with acute and chronic pancreatitis or pancreatic carcinoma. Many anomalies of pancreatic duct are diagnosed at clinical evaluation of causes of recurrent acute pancreatitis. ERCP is the gold standard method of diagnosis. Noninvasive method such as MRCP or EUS may also establish the diagnosis. There are many reports for the clinical significance and management of pancreas divisum and anomalous union of the pancreaticobiliary duct. There have been rare cases with the coexistence of a pancreas divisum and anomalous union of the pancreaticobiliary duct in the world and only one case in Korea. The case of a 33 year old man with epigastic pain and fever was recently experienced. It was diagnosed to be the coexistence of a pancreas divisum and anomalous union of the pancreaticobiliary duct by ERCP, PTC and MRCP. We report this case with review of the anomalies.

Clinical Study of Choledochal Cyst

Congenital dilatation of the common bile duct (choledochal cyst) is an uncommon disease. Although the etiology is unknown yet, various theories such as distal obstruction of the common bile duct, congenital weakness of the duct and anomalous union of the pancreaticobiliary duct have been offered to explain the occurrence of choledochal cyst. Thirty - six cases of choledochal cyst over 22 years were analyzed clinically and classified according to Todani`s classification and Kimura's anomalous union of pancreaticobiliary duct type. Todani type I consisted with 22 cases which were subdivided into 19 cases of type Ia, 1 case of type Ib and 2 of type Ic. Type IVa consisted with 14 cases including one case of Caroli's disease. There were 25 type BP cases and 10 type PB cases and 1 normal pancreatobiliary junction. Serum alkaline phosphatase was increased significantly in almost all cases. Seven patients (19.4%) had associated congenital anomalies such as double gallbladder, left - sided gallbladder, common bile duct web, biliary atresia, accessory hepatic duct, heterotopic pancreas, cleft lip and 2 cases of intestinal malrotation. All patients underwent cyst excision and Roux - en - Y hepaticojejunostomy and cholecystectomy. There was one death due to methicillin resistant Staphylococcus aureus sepsis.

A Case of an Anomalous Union of the Pancreatobiliary Duct Associated with Pancreas Divisum / 대한소화기내시경학회지

An anomalous union of the pancreatobiliary ductal system is defined as the junction between the common bile duct and the pancreatic duct outside the duodenal wall and beyond the influence of the sphincter of Oddi. This anomaly may relate to some pancreatobiliary lesions such as choledochal cysts, biliary tract carcinomas, or pancreatitis. Pancreas divisum is the most common congenital variant of the pancreatic ductal anatomy, and when it occurs, the ductal system of the dorsal and ventral pancreatic bud, fail to fuse during the second month of gestation. There have only been four cases of the coexistence of an anomalous union of the pancreatobiliary duct and pancreas divisum, reported until now. The case of a 29 year old man with epigastric pain and fever was recently experienced. An ERCP revealed an anomalous union of the pancreatobiliary duct, complete pancreas divisum and common bile duct stone. The case is herein reported.

Choledochal cyst associated the with anomalous union of pancreaticobiliary duct (AUPBD) has a more grave clinical course than choledochal cyst alone

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.

Clinical Analysis of Anomalous Pancreaticobiliary Ductal Union

BACKGROUND: With improvements in the performance of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing hepatobiliary and pancreatic diseases, anomalous unions of the pancreaticobiliary duct (AUPBD) have come to our attention in recent years. Such unions are thought to be a factor in the development of carcinomas of the pancreaticobiliary system. The purpose of the present study was to evaluate the clinical analysis of AUPBD for proper treatment. METHODS: During the past 4 years from January 1, 1993, to December 31, 1997, 28 adult patients with AUPBD, in whom the pancreaticobiliary ductal union and terminal biliary tract were opacified by ERCP, were seen at Asan Medical Center. We divided the types of AUPBD according to Kimura's classification. RESULTS: The ages of the patients (20 women and 8 men) with this anomaly ranged from 16 to 68 years. The patients' main complaints were abdominal pain (16 cases), indigestion (10 cases), jaundice (4 cases), and fever (2 cases). The common channel measured on direct cholangiograms was 15 mm to 42 mm long. All of the patients whose anomalies were confirmed by ERCP had combined diseases (benign diseases 20 cases, malignant diseases 8 cases). Of the 20 patients with benign diseases, the 15 patients who underwent operative treatments had no recurrent findings or symptoms, but all of the 5 patients who underwent conservative treatments had recurrent pancreatitis or indigestion. These 5 patients will still need to be followed up. For the 8 patients with malignant diseases, the 3 who did not undergo operations died of far-advanced cancer. Among the remaining 5 patients who underwent operative treatments, 1 patient died of cancer recurrence, but 4 patients have been alive since their operations. CONCLUSIONS: We think that all ERCP examinations have to be carried out with AUPBD in mind. It is important to remove the place that causes bile stasis and to stop backflow of pancreatic juice intothe bile duct in managing patients with this anomaly. Therefore, a prophylactic cholecystectomy and reconstruction of the biliary tract are both necessary in treating AUPBD.

Choledochal Cyst and Anomalous Union of Pancreaticobiliary Duct in the Adult / 대한소화기내시경학회지

Background/Aims: Choledochal cyst is a congenital dilatation of extra- or intrahepatic bile duct. Recently, anomalous union of pancreaticobiliary duct(AUPBD) is supposed to be one of the major causes of choledochal cyst. The aims of this study are to assess the association ratio of AUPBD in choledochal cyst and whether the AUPBD in choledochal cyst have a clinical significance. Methods: The study subjects were 35 adult patients who were diagnosed by ERCP in Asan Medical Center from August, l990 to July, 1995. We have classified the type of choledochal cyst and AUPBD according to Todani and Kimura, respectively.