The Mirizzi syndrome is a rare benign cause of obstructive jaundice. It is particularly interesting to surgeons because the surgery has to be carefully planned to avoid unnecessary damage to the common bile duct. Furthermore, it gives a differential diagnosis dilemma for surgeons as well as radiologist because there are no diagnostic procedures or clinical features that have a perfect access. As a result, the Mirizzi syndrome often has been mistaken for gallbladder cancer and cholangiocarcinoma. We experienced of a 76-year-old male patient, whose clinical symptoms were jaundice, epigastric pain and fever with chill and misdiagnosed as a cholangiocarcinoma with liver metastasis.
Aged ; Bile Duct Diseases ; Cholangiocarcinoma* ; Cholelithiasis ; Cholestasis ; Common Bile Duct ; Cystic Duct ; Diagnosis, Differential ; Fever ; Gallbladder Neoplasms ; Humans ; Jaundice ; Jaundice, Obstructive ; Liver ; Male ; Mirizzi Syndrome* ; Neoplasm Metastasis

BACKGROUND : Anomalous pancreaticobiliary ductal union (APBDU) is a congenital defect defined as a union of the pancreatic and the biliary ducts located outside the duodenal wall. Accordingly, this anomalous connection between the choledochus and the pancreatic duct is considered to be a factor in the development of biliary tract diseases such as choledochal cysts, pancreatitis, cholangitis, gallbladder cancer, and bile duct cancer. Our purpose is to analyze combined disease, especially biliary neoplasm, and to evaluate biliary tract microscopic changes in the biliary tract. METHODS : To study the clinical characteristics of APBDU, we reviewed 21 APBDU cases which were treated from June 1994 to January 1998. We studied the associated diseases, the surgical treatment, histological findings and the PCNA expression. We also analyzed the gallbladder epithelium in 10 control patients without APBDU. RESULTS : Among the 21 cases, 12 cases were accompanied by a choledochal cyst. A gallbladder carci noma occurred in 5 cases. Of the 13 operations, 4 involved a cholecystectomy only, and 9 involved a cholecystectomy, bile duct resection and hepaticojejunostomy. The histologic findings were reviewed in 13 cases. Bile duct metaplasia was found in 8 of the 9 cases with a bile duct resection, and gallbladder metaplasia was found in 8 of the 12 cases whereas metaplasia was detected in 1 of the 10 control patients. The PCNA expression was significantly high in APBDU group. CONCLUSIONS : The patients with APBDU showed a high incidence of gallbladder carcinoma and meta plasia in the epithelium of the gallbladder and the bile duct. This metaplasia in the gallbladder and the bile duct was thought to be related to the cancerous change.
Bile Duct Neoplasms ; Bile Ducts* ; Bile* ; Biliary Tract ; Biliary Tract Diseases ; Cholangitis ; Cholecystectomy ; Choledochal Cyst ; Common Bile Duct ; Congenital Abnormalities ; Epithelium ; Gallbladder Neoplasms ; Gallbladder* ; Humans ; Incidence ; Metaplasia ; Noma ; Pancreatic Ducts ; Pancreatitis ; Proliferating Cell Nuclear Antigen

Choledochal cyst is a relatively rare disease entity considered to be a congenital cyatic dilatation of the common bile duct. Since occurrence of malignant tumor in choledochal cyet was first reported by Irwin and Morrison in 1944, approximately 100 more cases of malignant tumors arising in congenital choledocal cyst have been reported in the world, and the risk of malignant tumor related to choledochal cyst have been reported 2.4-14%. Recently, we experienced a case of adenocarcinoma arising in choledochal cyst in 39 year-old woman who was diagnosed by ERCP and cholangioscopy with forceps biopsy, and was treated with en bloc resection of the choledochal cyst along with the pancreatic head and duodenum.
Adenocarcinoma ; Adult ; Bile Duct Neoplasms ; Biopsy ; Cholangiocarcinoma* ; Cholangiopancreatography, Endoscopic Retrograde ; Choledochal Cyst ; Common Bile Duct ; Dilatation ; Duodenum ; Female ; Head ; Humans ; Rare Diseases ; Surgical Instruments

Choledochal cyst is a relatively rare disease entity considered to be a congenital cyatic dilatation of the common bile duct. Since occurrence of malignant tumor in choledochal cyet was first reported by Irwin and Morrison in 1944, approximately 100 more cases of malignant tumors arising in congenital choledocal cyst have been reported in the world, and the risk of malignant tumor related to choledochal cyst have been reported 2.4-14%. Recently, we experienced a case of adenocarcinoma arising in choledochal cyst in 39 year-old woman who was diagnosed by ERCP and cholangioscopy with forceps biopsy, and was treated with en bloc resection of the choledochal cyst along with the pancreatic head and duodenum.
Adenocarcinoma ; Adult ; Bile Duct Neoplasms ; Biopsy ; Cholangiocarcinoma* ; Cholangiopancreatography, Endoscopic Retrograde ; Choledochal Cyst ; Common Bile Duct ; Dilatation ; Duodenum ; Female ; Head ; Humans ; Rare Diseases ; Surgical Instruments

BACKGROUND/AIMS: The biliary tract often shows various morphologic abnormalities. Thus various anomalies and variations of the cystic duct have been extensively studied via cadeveric or intraoperative dissections and operative cholangiograms. The knowledge of the junction of cystic and common hepatic duct is essential for endoscopic management of biliary tract disease. But no large series identifying this critical cystico-hepatic junction(CHJ) by duodenoscopy has been reported. METHODS: To know the anomaly and variant of CHJ and to evaluate its clinical significence, we retrospectively reviewed 434 cases of endoscopic retrograde cholangiography performed at Korea University Hospital from 1992 through 1993. The CHJ was categorized as lateral or medial only for unequivocal angulation in that direction, and spiral when there is overlap of the CHJ with the bile duct in the posteroanterior view. The CHJ was further modified as parallel if the course of the two ducts was closely adherent for 1 cm or more. In addition to identifying the radial takeoff, the level of the CHJ along the length of the extrahepatic biliary tree was determined. The distance from the ampulla to the junction was divided by the distance from the ampulla to the bifurcation and expressed as proximal, middle, distal, respectively. RESULTS: The level of CHJ was mainly middle in 68.4% followed by distal in 16.4%, prximal in 15.2%. The radial orientation of CHJ was mainly lateral in 51.6%, followed by spiral in 32.3%, medial in 16.1%. The cystic duct runs parallely in 11.5%, mainly in the distally inserted, medially oriented cystic duct. The incidence of abnormalous CHJ was 5 case(1.2%), which included 1 case of cystic duct entering the right hepatic duct, 3 case of cystic duct entering at the junction of both extrahepatic duct(trifurcation), and 1 case of accessory hepatic duct entering cystic duct. The most common biliary disease by ERCP was gallbladder stone(25.5%), followed by common bile duct stone(19.5%), intrahepatic duct stone(7.2%), cystic duct obstruction(4.5%), cystic duct stone(2.9%), and others(bile duct cancer, gallbladder cancer, Mirizzi's syndrome). According to the level of the CHJ, there were low incidence of gallbladder stone in distal CHJ, low incidence of common bile duct stone in proximal CHJ and high incidence of Mirrizi's syndrome and gallbladder cancer in distal CHJ. There were no difference in the incidence of biliary disease according to the radial orientation and course of the CHJ. Practically, during therapeutic ERCP there were some tdchnical difficulties in extraction of biliary stone or selective bile duct cannulation in the presence of distally inserted, parallel course of the cystic duct. CONCLUSIONS: The anatomy of the CHJ by ERCP is more variable than that of previous surgical and autopsy reports and the endoscopist should be aware of this variants for safe and effective intervention in bile duct disease.
Autopsy ; Bile Duct Diseases ; Bile Ducts ; Biliary Tract ; Biliary Tract Diseases ; Catheterization ; Cholangiography* ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct ; Cystic Duct ; Duodenoscopy ; Gallbladder ; Gallbladder Neoplasms ; Hepatic Duct, Common ; Incidence ; Korea ; Retrospective Studies

What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

BACKGROUND/AIMS: Endoscopic retrograde cholangiopancreatography (ERCP) is difficult to perform in patients with a Billroth II anastomosis due to the anatomical alterations. This study evaluated retrospectively the efficacy and safety of endoscopic balloon dilatation after a partial infundibulotomy with a needle knife in patients who had undergone a Billroth II operation. METHODS: Between January 1, 2004 and December 31, 2005, 11 patients, who had undergone a prior Billroth II gastrectomy, underwent ERCP because of a suspicion of pancreatobiliary diseases. Infundibulotomy was performed using a needle knife sphincterotome. After a partial infundibulotomy, a dilation balloon was passed over a prepositioned guidewire and placed in the biliary orifice. Under endoscopic and fluoroscopic control, the balloon was then inflated with diluted contrast over a period of 1 to 2 minutes. RESULTS: A total of 10 patients (7 male and 3 female) were enrolled in this study. Eight had common bile duct stones, 1 bile duct cancer, and 1 pancreatic cancer. The common bile duct was dilated by a balloon. The calculi were removed in 6 patients, and a common bile duct stent was implanted in two cases via endoscopy. No complications such as acute pancreatitis or apparent infections of the biliary tracts were encountered in these cases except for mild fever, temporary high serum levels of aminotransferase and amylase after ERCP in 3 cases. No severe bleeding or perforation occurred in patients undergoing balloon dilatation. CONCLUSIONS: An infundibulotomy with balloon dilatation is a valuable alternative to endoscopic sphinterotomy in the treatment of patients with bile duct diseases, who have undergone prior Billroth II gastrectomies.
Amylases ; Bile Duct Diseases ; Bile Duct Neoplasms ; Biliary Tract ; Calculi ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct ; Dilatation* ; Endoscopy ; Fever ; Gastrectomy ; Gastroenterostomy* ; Hemorrhage ; Humans ; Male ; Needles ; Pancreatic Neoplasms ; Pancreatitis ; Retrospective Studies ; Stents

BACKGROUND/AIMS: Endoscopic retrograde cholangiopancreatography (ERCP) is difficult to perform in patients with a Billroth II anastomosis due to the anatomical alterations. This study evaluated retrospectively the efficacy and safety of endoscopic balloon dilatation after a partial infundibulotomy with a needle knife in patients who had undergone a Billroth II operation. METHODS: Between January 1, 2004 and December 31, 2005, 11 patients, who had undergone a prior Billroth II gastrectomy, underwent ERCP because of a suspicion of pancreatobiliary diseases. Infundibulotomy was performed using a needle knife sphincterotome. After a partial infundibulotomy, a dilation balloon was passed over a prepositioned guidewire and placed in the biliary orifice. Under endoscopic and fluoroscopic control, the balloon was then inflated with diluted contrast over a period of 1 to 2 minutes. RESULTS: A total of 10 patients (7 male and 3 female) were enrolled in this study. Eight had common bile duct stones, 1 bile duct cancer, and 1 pancreatic cancer. The common bile duct was dilated by a balloon. The calculi were removed in 6 patients, and a common bile duct stent was implanted in two cases via endoscopy. No complications such as acute pancreatitis or apparent infections of the biliary tracts were encountered in these cases except for mild fever, temporary high serum levels of aminotransferase and amylase after ERCP in 3 cases. No severe bleeding or perforation occurred in patients undergoing balloon dilatation. CONCLUSIONS: An infundibulotomy with balloon dilatation is a valuable alternative to endoscopic sphinterotomy in the treatment of patients with bile duct diseases, who have undergone prior Billroth II gastrectomies.
Amylases ; Bile Duct Diseases ; Bile Duct Neoplasms ; Biliary Tract ; Calculi ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct ; Dilatation* ; Endoscopy ; Fever ; Gastrectomy ; Gastroenterostomy* ; Hemorrhage ; Humans ; Male ; Needles ; Pancreatic Neoplasms ; Pancreatitis ; Retrospective Studies ; Stents

PURPOSE: Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. METHODS: From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and MRCP. The final diagnosis was based on the operative and pathologic findings with biopsy specimen including clinical and laboratory findings. RESULTS: A total of 70 patients, consisting of 31 males and 39 females, with a mean age of 2.6+/-3.3 years were studied. The final diagnosis was biliary atresia in 25, neonatal cholestasis in 18, choledochal cyst without anomalous pancreatobiliary duct union(APBDU) in nine, choledochal cyst with APBDU in seven, cholestatic hepatitis in five, chronic recurrent pancreatitis in three, sclerosing cholangitis in two, and secondary biliary cirrhosis in one case. The overall diagnostic accuracy of abdominal ultrasonography was 75.7% and that of MRCP was 97.1%. The sensitivity and specificity of MRCP were 100% and 98% for biliary atresia, 87.5% and 100% for choledochal cyst with APBDU, 100% and 100% for choledochal cyst without APBDU, sclerosing cholangitis and chronic recurrent pancreatitis, respectively. CONCLUSION: MRCP is a fast, non-invasive and reliable method for diagnosing pancreaticobiliary diseases in children and will be the standard diagnostic procedure in the future.
Biliary Atresia ; Biopsy ; Child* ; Cholangitis, Sclerosing ; Choledochal Cyst ; Cholestasis ; Diagnosis ; Female ; Hepatitis ; Humans ; Jaundice, Obstructive ; Liver Cirrhosis, Biliary ; Male ; Pancreatitis ; Sensitivity and Specificity ; Ultrasonography

The diagnostic accuracy of percutaneous transhepatic cholangioscopy (PTCS) was compared to that of three radiologic modalities in distal common bile duct (CBD) strictures for the evaluation of clinical application. Ninety-five patients who underwent PTCS for the evaluation of distal CBD strictures (35 malignant and 60 benign) whose masses were not obvious from radiologic imagings were included. Confirmative diagnosis could not be reached by endoscopic retrograde cholangiopancreatography (ERCP) or radiologic findings in all cases. Specific findings on the computed tomography (CT), magnetic resonance cholangiopancreatography (MRCP) and direct cholangiography were analyzed among 68 (25 malignant and 43 benign) out of the 95 patients in order to determine the sensitivity and specificity of three radiologic studies for the diagnosis of malignant distal CBD strictures, and to compare those results with those by a combination of PTCS-guided biopsy and tumor vessel observation on cholangioscopy. The sensitivity/specificity of CT, MRCP and direct cholangiography including ERCP in diagnosing malignant distal CBD strictures were 42.9%/65.8%, 53.3%/58.3%, and 70.8%/47.6% respectively, while it was 96%/100% for the combination of PTCS-guided biopsy and tumor vessel. PTCS is a useful method for differential diagnosis of distal CBD strictures, particularly when it is difficult to distinguish benign from malignant strictures by radiologic studies and when peroral approach is not feasible.
Aged ; Biopsy ; Cholangiopancreatography, Magnetic Resonance ; Common Bile Duct Diseases/*diagnosis/pathology ; Common Bile Duct Neoplasms/*diagnosis/pathology ; Constriction, Pathologic ; Endoscopy, Digestive System/*methods ; Endosonography ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Sensitivity and Specificity