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*

Aged ; Bile Duct Diseases ; complications ; diagnostic imaging ; pathology ; Bile Duct Neoplasms ; diagnosis ; Carcinoma, Hepatocellular ; complications ; diagnostic imaging ; pathology ; Diagnosis, Differential ; Humans ; Jaundice, Obstructive ; etiology ; Klatskin Tumor ; diagnosis ; Liver Neoplasms ; complications ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Thrombosis ; complications ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/diagnosis ; Bile Ducts, Intrahepatic/pathology/ultrasonography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/complications/*diagnosis/drug therapy ; Common Bile Duct/pathology/ultrasonography ; Humans ; Immunoglobulin G/*blood ; Immunohistochemistry ; Male ; Pancreatitis/complications/diagnosis ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed

Hepatic peribiliary cysts (HPCs) are characterized by cystic dilatations of the peribiliary glands located throughout the branches of the biliary systems. Specifically, they are mainly located along the hepatic hilum and major portal tracts. The natural history and prognosis of HPCs are uncertain. In fact, almost all HPCs have been discovered incidentally during radiological examination or autopsy, and they are considered to be clinically harmless. Recently, several cases of HPCs associated with obstructive jaundice or liver failure were reported in patients with pre-existing liver disease in several studies. However, until now there have been no reports of such a case in Korea. Herein, we report a case of HPCs that show a disease course with a poor prognosis. These HPCs developed in a 47-year-old man with progressive alcoholic liver cirrhosis.
Bile Duct Diseases/complications/*diagnosis/radiography ; Cholangiopancreatography, Magnetic Resonance ; Cysts/*complications/radiography ; Humans ; Jaundice, Obstructive/etiology ; Liver Cirrhosis, Alcoholic/complications/*diagnosis/radiography ; Male ; Middle Aged ; Tomography, X-Ray Computed

Biliary drainage in patients with malignant biliary obstruction relieves jaundice and prevents the development of cholangitis or hepatic failure from biliary obstruction. Therefore, this may result in better quality of life along with survival prolongation. Biliary stent placement is an effective and safe measure for biliary decompression and is preferred than bypass surgery in high risk patients. Entero-biliary perforation-communication is one of the rare complications of biliary stent. We herein report a case of duodeno-biliary perforation-communication in patient with distal cholangiocarcinoma who presented with duodenal ulcer and obstruction, occurring 4 years later from the metallic biliary stent insertion. Patient was managed with a pyloric metal stent and conservative care.
Aged, 80 and over ; Bile Duct Neoplasms/complications/*diagnosis ; Bile Ducts, Intrahepatic/pathology ; Biliary Fistula/*diagnosis/etiology/pathology ; Cholangiocarcinoma/complications/*diagnosis ; Duodenal Diseases/*diagnosis/etiology/pathology ; Female ; Humans ; Intestinal Fistula/*diagnosis/etiology/pathology ; Stents/*adverse effects ; Tomography, X-Ray Computed

Allopurinol-induced hypersensitivity syndrome is characterized by an idiosyncratic reaction involving multiple-organs, which usually begins 2 to 6 weeks after starting allopurinol. In rare cases, the adverse reactions to allopurinol are accompanied by a variety of liver injury, such as reactive hepatitis, granulomatous hepatitis, vanishing bile duct syndrome, or fulminant hepatic failure. Here we report a case with granulomatous hepatitis and ductopenia. A 69-year-old man with chronic renal failure, hyperuricemia, and previously normal liver function presented with jaundice, skin rash, and fever 2 weeks after taking allopurinol (200 mg/day). In histopathology, a liver biopsy specimen showed mild spotty necrosis of hepatocytes, marked cholestasis in parenchyma, and some granulomas in the portal area. There were vacuolar degeneration in the interlobular bile ducts and ductopenia in the portal tracts. Pathologic criteria strongly suggested the presence of allopurinol-induced granulomatous hepatitis with ductopenia and cholestasis. The patient fully recovered following the early administration of systemic corticosteroid therapy.
Aged ; Allopurinol/*adverse effects/therapeutic use ; Antimetabolites/*adverse effects/therapeutic use ; Bile Duct Diseases/*chemically induced/diagnosis/pathology ; Bile Ducts, Intrahepatic/*drug effects/pathology ; Cholestasis/*chemically induced/diagnosis/pathology ; Drug Eruptions/pathology ; Granuloma/*chemically induced/pathology ; Hepatitis, Toxic/*pathology ; Humans ; Kidney Failure, Chronic/complications/drug therapy ; Male

A "biloma" is a loculated collection of bile located outside of the biliary tree. It can be caused by traumatic, iatrogenic or spontaneous rupture of the biliary tree. Prior reports have documented an association of biloma with abdominal trauma, surgery and other primary causes, but spontaneous bile leakage has rarely been reported. A spontaneous infected biloma, without any underlying disease, is a very rare finding. We recently diagnosed a spontaneous infected biloma by abdominal computed tomography and sonographically guided percutaneous aspiration. The patient was successfully managed with percutaneous drainage and intravenous antibiotics. We report here a case of infected biloma caused by spontaneous rupture of the intrahepatic duct, and review the relevant medical literature.
Aged ; *Bile ; Bile Duct Diseases/*diagnosis/microbiology/*therapy ; Bile Ducts, Intrahepatic/*radiography ; Cholangiography ; Drainage ; Escherichia coli Infections/*complications ; Female ; Humans ; Rupture, Spontaneous ; Tomography, X-Ray Computed

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

No abstract available.
Bile Duct Neoplasms/complications/*diagnosis/pathology ; *Bile Ducts, Intrahepatic/pathology ; Cholangiocarcinoma/complications/*diagnosis/pathology ; Female ; Humans ; Lithiasis/*etiology/pathology/surgery ; Liver Diseases/*etiology/pathology/surgery ; Middle Aged

Inflammatory pseudotumor is an uncommon mass which develops most frequently in the lung of young adults. It is characterized by localized fibrous proliferations with chronic inflammatory cell infiltration. Due to its rarity and similarity in radiologic appearance with malignant hepatic tumors, hepatic inflammatory pseudotumor (HIPT) is often misdiagnosed and resected accidentally. We report a case of HIPT which was unnecessarily resected due to synchronous small peripheral cholangiocarcinoma located on the other segment of liver.
Bile Duct Neoplasms/complications/*diagnosis/pathology ; *Bile Ducts, Intrahepatic ; Cholangiocarcinoma/complications/*diagnosis/pathology ; Fatal Outcome ; Granuloma, Plasma Cell/complications/*diagnosis/pathology ; Humans ; Liver Diseases/complications/*diagnosis/pathology ; Male ; Middle Aged ; Tomography, X-Ray Computed