We report here on an extremely rare case of duplicated extrahepatic bile ducts that was associated with choledocholithiasis, and this malady was visualized by employing the minimum intensity projection images with using multi-detector row CT. The presence of duplicated extrahepatic bile ducts with a proximal communication, and the ducts were joined distally and they subsequently formed a single common bile duct, has not been previously reported.
Aged, 80 and over ; Bile Ducts, Extrahepatic/*abnormalities/pathology/radiography ; Choledocholithiasis/*complications/radiography ; Female ; Humans

Papillary tumor of the bile duct is characterized by the presence of an intraductal tumor with a papillary surface comprising innumerable frondlike infoldings of proliferated columnar epithelial cells surrounding slender fibrovascular stalks. There may be multiple tumors along the bile ducts (papillomatosis or papillary carcinomatosis), which are dilated due to obstruction by a tumor per se, by sloughed tumor debris, or by excessive mucin. Radiologically, the biliary tree is diffusely dilated, either in a lobar or segmental fashion, or aneurysmally, depending on the location of the tumor, the debris, and the amount of mucin production. A tumor can be depicted by imaging as an intraductal mass with a thickened and irregular bile duct wall. Sloughed tumor debris and mucin plugs should be differentiated from bile duct stones. Cystically or aneurysmally, dilated bile ducts in mucin-hypersecreting variants (intraductal papillary mucinous tumors) should be differentiated from cystadenoma, cystadenocarcinoma and liver abscess.
Bile Duct Neoplasms/*radiography ; *Bile Ducts, Extrahepatic ; *Bile Ducts, Intrahepatic ; Carcinoma, Papillary/radiography ; Cystadenoma, Mucinous/radiography ; Diagnosis, Differential ; Female ; Human ; Male ; Middle Age

Autoimmune pancreatitis is a distinct disease characterized by the presence of autoantibodies and hypergammaglobulinemia, inflammation of the pancreatic parenchyma, and irregular stricture of the pancreatic duct. The involvement of distal common bile duct is frequently observed, but intrahepatic bile duct involvement is very rare, which seem to have similar feature to primary sclerosing cholangitis. We report a case of the patient with autoimmune pancreatitis combined with extensive involvement of extrahepatic and intrahepatic bile duct, which had a favorable response to steroid therapy.
Aged ; Autoimmune Diseases/complications/*diagnosis/drug therapy ; Bile Ducts, Extrahepatic/*radiography ; Bile Ducts, Intrahepatic/*radiography ; Humans ; Male ; Pancreatitis/complications/*diagnosis/radiography ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed

The most commonly associated anomalies in patients with extrahepatic biliary atresia are cardiovascular, digestive and splenic defects. Of the cardiovascular anomalies, there are very few reports of biliary atresia with cardiomyopathy. We report the first case of a child with extrahepatic biliary atresia and restrictive cardiomyopathy. The patient was a 13-month-old boy diagnosed with extrahepatic biliary atresia at the age of 2 months, when he underwent laparotomy for definite diagnosis.Hepatic portoenterostomy was performed after confirmative cholangiogram. Recently, he developed severe cough and dyspnea, and his respiratory symptoms worsened. Chest radiograph showed cardiomegaly. Two- dimensional echocardiography showed marked biatrial enlargement. On M- mode echocardiogram, a slight increase in left ventricular dimension was seen in early diastole with a relatively good left ventricular function. Mitral inflow Doppler tracing showed an increased E-velocity (1.1 m/sec) with decreased deceleration time (75 m/sec), and increased E/A ratio (0.33). He was diagnosed as having restrictive cardiomyopathy with characteristic echocardiographic features.
Bile Ducts, Extrahepatic/*abnormalities ; Biliary Atresia/*complications/physiopathology/radiography/ultrasonography ; Cardiomyopathy, Restrictive/*complications/physiopathology/radiography/ultrasonography ; Human ; Infant ; Lung/radiography ; Male ; Radiography, Thoracic

We experienced one fatal case of biliary cast syndrome after cadaveric liver transplantation involving both intrahepatic ducts. A 58-year-old man underwent cadaveric liver transplantation because of hepatitis B virus related liver cirrhosis and concomitant hepatocellular carcinoma. Five weeks after the liver transplantation, postoperative course was complicated by development of acute cholangitis. Subsequent endoscopic retrograde cholangiography revealed diffuse intrahepatic bile duct strictures without filling defects. Percutaneous liver biopsy, which was done to exclude rejection, revealed biliary cast. Successful endoscopic removal was precluded due to its diffuse involvement. Because of the deterioration of patient's condition by refractory biliary obstruction and cholangitis, retransplantation from cadaveric donor was performed. Debridement of the biliary tree after graft removal yielded a near-complete cast of the intrahepatic ductal system. Biliary cast syndrome should be suspected when jaundice or cholangitis is associated with dilated ducts on abdominal imaging studies in cadaveric liver transplantation recipients. Initial therapeutic options include removal of biliary cast after endoscopic or percutaneous cholangiography. Although endoscopic retrieval of biliary cast by endoscopic retrograde cholangiopancreatography could be employed as a first-line management, other modalities such as endoscopic nasobiliary drainage, percutaneous transhepatic drainage, or retransplantation should be considered when complete removal is not feasible and the condition of the recipient deteriorates.
Bile Duct Diseases/*diagnosis/etiology/pathology ; Bile Ducts, Extrahepatic/pathology ; Bile Ducts, Intrahepatic/pathology ; Cholangiopancreatography, Endoscopic Retrograde ; Fatal Outcome ; Humans ; Jaundice, Obstructive/etiology ; *Liver Transplantation ; Male ; Middle Aged ; Postoperative Complications/*diagnosis/pathology/radiography ; Tomography, X-Ray Computed

We report a case of small cell carcinoma of extrahepatic bile duct presenting with jaundice and hemobilia. A 59-year-old woman was admitted due to right upper quadrant pain and jaundice. An abdominal computed tomography revealed a 2 cm sized mass in the extrahepatic bile duct. Endoscopic retrograde cholangiopancreatography revealed bloody discharge coming out of the papillary orifice in endoscopic view and a dilated extrahepatic bile duct with multiple irregular filling defects in cholangiogram. A coronal T2-weighted image revealed a hyperintense mass at extrahepatic bile duct. Laparotomy was performed, and pathologic examination of resected specimen showed tumor cells having round to oval nuclei with coarsely granular chromatin and scanty cytoplasm, which were immunoreactive for synaptophysin and chromogranin A, compatible with the diagnosis of small cell carcinoma. The small cell carcinoma of bile duct, despite its rarity, should be considered in differential diagnosis of the causes for obstructive jaundice and hemobilia.
Bile Duct Neoplasms/*diagnosis/pathology/radiography ; Bile Ducts, Extrahepatic/*pathology/radiography ; Carcinoma, Small Cell/*diagnosis/pathology/radiography ; Cholangiopancreatography, Endoscopic Retrograde ; Chromogranin A/metabolism ; Female ; Hemobilia/complications/*diagnosis ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Synaptophysin/metabolism ; Tomography, X-Ray Computed

Primary non-Hodgkin's lymphoma arising from the bile duct is extremely rare and the reported imaging features do not differ from those of cholangiocarcinoma of the bile duct. We report a case of a patient with extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue (MALT), who presented with obstructive jaundice and describe the distinctive radiologic features that may suggest the correct preoperative diagnosis of primary lymphoma of the bile duct. Primary MALT lymphoma of the extrahepatic bile duct should be considered in the differential diagnosis when there is a mismatch in imaging findings on computed tomography or magnetic resonance imaging and cholangiography.
Bile Duct Neoplasms/complications/*diagnosis/radiography ; *Bile Ducts, Extrahepatic ; Cholangiocarcinoma/diagnosis ; Cholangiography ; Diagnosis, Differential ; Humans ; Jaundice, Obstructive/complications/diagnosis ; Lymphoma, B-Cell, Marginal Zone/complications/*diagnosis/radiography ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed

No abstract available.
Bile Duct Diseases/*diagnosis/ultrasonography ; Bile Ducts, Extrahepatic ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Humans ; Hypertension, Portal/diagnosis ; Male ; Middle Aged ; Portal Vein/radiography ; Tomography Scanners, X-Ray Computed