Cases reported "Bile Duct Diseases"

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1/52. A case of bilioduodenal fistula treated with a self-expandable metallic stent.

    We report the case of a 72 year-old female patient who suffered from biliary fistulae. The biliobiliary and bilioduodenal fistulae appeared after an operation for biliary bleeding. Conventional therapy for biliary fistula would be the disconnection of the fistula by either conservative or operative treatment. In the present case, however, it was preferable to enlarge the fistula to drain bile juice into the duodenum, rather than to close the fistula because it would have been difficult to achieve a tight adhesion with this operation. The enlargement by a plastic tube stent failed to drain the bile juice into the duodenum, because the sludge made the tube stenotic. Therefore, a self-expandable metallic stent was applied in this case. An expandable stent was used because a large final caliber is necessary to prevent stenosis of the fistula by sludge and mucosal hyperplasia. After insertion of a self-expandable metallic stent by the percutaneous transhepatic biliary drainage route, the patient has not suffered from cholestasis and cholangitis for the last 30 months. It can therefore be concluded that enlargement of the fistula by a self-expandable metallic stent is a convenient therapy for such biliointestinal fistulae.
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ranking = 1
keywords = cholangitis
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2/52. Hepatic peribiliary cysts diagnosed by magnetic resonance cholangiography.

    We report a 76-year-old man with hepatic peribiliary cysts diagnosed by magnetic resonance cholangiopancreatography (MRCP). On his first admission, in 1991, the patient was misidentified as having localized dilatation of the left intrahepatic bile ducts, from the ultrasound (US) and computed tomographic (CT) findings, or primary sclerosing cholangitis from endoscopic retrograde cholangiopancreatography (ERCP). However, US and CT in 1998 suggested worsening of the lesions. MRCP was performed for the first time, revealing hepatic peribiliary cysts in the hepatic hilum and along the left hepatic ducts. Drip infusion cholangiography (DIC)-CT confirmed the extraluminal compression of the bile ducts, caused by the cysts, without an influx of contrast medium into the hepatic peribiliary cysts. Retrospective evaluation showed increases in the size and number of the cysts in 1998 compared with the findings in 1996.
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ranking = 1
keywords = cholangitis
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3/52. Delayed granuloma formation in a patient with vanishing bile duct syndrome 7 years post-liver transplantation.

    A patient was found to have numerous granulomata 7 years after orthotopic liver transplantation for primary sclerosing cholangitis (PSC) on a recent liver biopsy specimen. This histopathologic finding prompted a review of the literature to determine the commonality of this feature in the absence of the usual causes of granulomatous liver disease, none of which were found to be the cause of this patient's liver histopathologic state. The presence of posttransplantation granulomata is rare, and although previously reported to occur shortly after liver transplantation, this finding has not been reported previously with either PSC or vanishing bile duct syndrome. We are not aware of another case of granulomata associated with recurrent PSC or vanishing bile duct syndrome 7 years after liver transplantation.
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ranking = 1
keywords = cholangitis
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4/52. Cholangiojejunal fistula caused by bile duct stricture after intraoperative injury to the common hepatic artery.

    A 68-year-old man, admitted for the treatment of recurrent cholangitis after a pancreatoduodenectomy (PD) performed 3 years previously was diagnosed as having multiple hepaticolithiasis. On laparotomy, the hepatic artery was not recognized. The anastomosed common hepatic duct was obstructed, and a fistula had been formed between the right hepatic duct and the Roux limb of the jejunum. lithotripsy was performed from this fistula and it was reanastomosed. Angiography was performed postoperatively and it revealed common hepatic artery injury, most likely to have occurred during the previous PD. The patient's postoperative course was uneventful and he has been asymptomatic for 8 months after the operation, indicating that reanastomosis of the fistula can be an effective method. The stricture of the anastomosis was suspected to be mainly due to cholangial ischemia, because no episode of anastomotic leak or retrograde biliary infection had occurred during the PD perioperative period. There are several reports of late stricture of anastomosis 5 or more years after cholangiojejunostomy. This patient, therefore, requires further long-term follow up.
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ranking = 1
keywords = cholangitis
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5/52. Autoimmune hepatitis associated with bile duct injury resembling chronic non-suppurative destructive cholangitis.

    Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) are representative autoimmune liver diseases in which hepatocytes and intrahepatic bile ducts, respectively, are selectively damaged by autoimmune mechanisms. Bile duct injury and loss is characteristic of PBC and chronic non-suppurative destructive cholangitis (CNSDC), in particular, is a histological hallmark of PBC. In this report, we present an unusual case of AIH accompanied by CNSDC-like bile duct injury in a 46-year-old woman. The patient's serum aminotransferase level was abnormally high. The serum levels of alkaline phosphatase, gamma-GTP and IgG were also elevated, but the IgM level was within normal limits. The titer of antismooth muscle antibody (SMA) was 1:80, while antinuclear autoantibody (ANA) and the M2 fraction of antimitochondrial antibody (AMA) were both negative. Liver biopsy disclosed CNSDC-like bile duct injuries and severe interface hepatitis and lobular hepatitis with perivenular zonal necrosis were observed. The aggregate score of the International Autoimmune hepatitis Group corresponded to the category of probable AIH. Immunohistochemically, histocompatibility leukocyte antigen-DR, which is aberrantly expressed in the damaged bile ducts of PBC, was not found in the injured bile ducts of this case. Laboratory data were much improved by treatment with prednisone, but ursodeoxycholic acid was not effective. Although the possibility of an overlapping syndrome of AIH- and AMA-negative PBC could not be excluded, this case was diagnosed as AIH with CNSDC-like bile duct lesions.
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ranking = 5
keywords = cholangitis
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6/52. Percutaneous hepatolithotomy: the Northwestern University experience.

    Obstruction of intrahepatic ducts by calculi can lead to abdominal pain, cholestasis, abscesses, and cholangitis. patients with stones recalcitrant to extraction using endoscopic retrograde cholangiopancreatography (ERCP) have traditionally been referred to a general surgeon for open stone extraction or hepatic lobectomy despite its great potential morbidity. Borrowing techniques, instrumentation, and experience in performing percutaneous nephrolithotomy, we describe our experience with percutaneous hepatolithotomy (PHL), a minimally invasive, safe, and effective alternative to open surgery for recalcitrant biliary stones.
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ranking = 1
keywords = cholangitis
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7/52. Portal biliopathy.

    Herein we describe a case of obstructive jaundice and cholangitis originating from choledochal lithiasis secondary to a stricture of the common bile duct in a patient with cavernous transformation of the portal vein. In fact, portal cavernous transformation gives rise to many dilated pericholedochal and periportal collaterals that bypass the portal vein obstruction. Extrinsic compression of the common duct by dilated venous collaterals together with pericholedochal fibrosis from the inflammatory process causing portal thrombosis may lead to biliary stricture and dilatation of the proximal biliary tree. This condition sometimes causes the formation of secondary biliary stones and cholangitis. Treatment in our case could not be accomplished by a biliodigestive anastomosis because the patient suffered from a short bowel syndrome subsequent to extensive ileal resection for splanchnic venous thrombosis. We repeatedly attempted stone removal during endoscopic retrograde cholangiopancreatography (ERCP) but finally resorted to a percutaneous transhepatic approach, which permitted removal of the stones and treatment of the stricture by pneumatic dilatation. The patient remains well 3 years after the procedure and has shown no signs of jaundice or cholangitis.
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ranking = 3
keywords = cholangitis
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8/52. Prolonged cholestasis and ductopenia following gold salt therapy.

    Hepatotoxicity, predominantly cholestatic, is a rare adverse effect of gold salt therapy, which usually completely resolves within a few months. We report the case of a female patient treated for rheumatoid arthritis, who had gold salt overdose, and in whom acute cholestatic hepatitis occurred three weeks after beginning of therapy. Evolution of gold concentration was followed in plasma and urine, as well as in cutaneous and liver dry tissue. Liver biopsy showed marked inflammatory changes of interlobular bile ducts that evolved towards ductopenia, which was responsible for prolonged cholestasis still present 15 months later. In addition, sialadenitis with sicca syndrome was noted six months after onset of the disease. The mechanism of hepatotoxicity was probably immunoallergic since liver lesions were associated with hypersensitivity syndrome including dermatitis and blood and tissue eosinophilia. This is the first report of gold salt hepatotoxicity with histological demonstration of cholangitis followed by ductopenia.
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ranking = 1
keywords = cholangitis
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9/52. Marked diffuse dilations of the biliary tree associated with intrahepatic calculi, biliary sludges and a mucinous cyst of the pancreatic head in a 99-year-old woman.

    A 99-year-old woman was admitted to Shizuoka Shimizu Municipal Hospital because of fever and anasarca. Imaging and laboratory tests showed pneumonia, urinary tract infection, and cardiac failure. The patient died 20 days after admission. An autopsy revealed marked diffuse dilations of the biliary tree ranging from the lower common bile duct to intrahepatic bile ducts. Intrahepatic calcium bilirubinate stones and biliary sludges were recognized within the dilated bile ducts. A unilocular cyst (2 cm in diameter) was present in the pancreatic head adjacent to the lower common bile duct, and it appeared to compress the common bile duct. Histologically, the walls of the dilated biliary tree showed proliferation of peribiliary glands, fibrosis, and infiltration of lymphocytes and neutrophils (cholangitis). The lumens of the dilated biliary ducts contained neutral and acidic mucins, fibrinous materials, bacteria, neutrophils, and aspergillus fungi, in addition to the calculi and sludges. The background liver showed atrophy (400 g). The pancreatic unilocular cyst was composed of mucous columnar cells with a few infoldings, and the pancreas also showed foci of mucinous duct hyperplasia and ectasia; the pathological diagnosis of the cyst was cystic dilations of a pancreatic duct branch (mucinous ductal ectasia or mucinous cyst). Other lesions included aspiration pneumonia, emaciation, atrophy of systemic organs, gastric leiomyoma, serous cystadenoma of the right ovary, and arteriosclerotic nephrosclerosis. The present case suggests that a mucinous cyst of the pancreas may compress the biliary tree and lead to marked diffuse dilations of the biliary tree. Alternatively, the dilations of the bile ducts may be associated with aging or may be of congenital origin. The dilated bile ducts may, in turn, give rise to bacterial and fungal cholangitis and formation of biliary sludges and intrahepatic calcium bilirubinate stones.
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ranking = 2
keywords = cholangitis
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10/52. Pericholangitis with ulcerative colitis following autoimmune hepatitis over 12 years.

    A 35-year-old woman was diagnosed as autoimmune hepatitis 12 years ago by abnormal findings of liver tests including lupus erythematosus (LE) cell phenomenon and liver biopsy. She was admitted in May 1990 with a history of lower gastrointestinal bleeding. colonoscopy with biopsy and barium enema revealed chronic ulcerative colitis along the entire colon. Since liver tests did not respond well to prednisolone treatment, liver biopsy was again performed and it revealed periductal inflammation with small duct proliferation, a finding compatible with pericholangitis. We herein report this patient who was initially diagnosed as autoimmune hepatitis and thereafter found to be pericholangitis associated with ulcerative colitis.
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ranking = 6
keywords = cholangitis
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