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 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.

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.

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.