Cases reported "Bile Duct Diseases"

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1/16. Heterotopic gastric mucosa in intrahepatic bile duct, presenting with hemobilia: a case report.

    We present a 66-year-old man with unique heterotopic gastric mucosa in the intrahepatic bile duct causing hemobilia. Endoscopic retrograde cholangiography showed irregular stenosis of the left intrahepatic bile duct, and a provisional diagnosis of cholangiocarcinoma was made. Therefore, partial hepatic lobectomy and cholecystectomy were performed. Histological examination of the liver showed the presence of ectopic gastric mucosa in the intrahepatic bile duct containing mucous glands with parietal and chief cells and bile. Heterotopic gastric mucosa in the intrahepatic bile duct is a rare cause of hemobilia.
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2/16. Treatment of extrahepatic bile duct disease in infancy and early childhood.

    Extrahepatic bile duct disease is a rare finding in infancy and early childhood. However, there is an increasing number of patients operated at this age reported in the literature. This increase may have multifactorial reasons, e.g. real increase, better ability of detection as a result of better diagnostic techniques and knowledge of predisposing factors of extrahepatic bile duct disease in childhood, especially in early childhood and infancy. The following report describes three cases of extrahepatic bile duct disease in infancy and early childhood treated at the Department of Surgery of the University of technology in Aachen, germany. From 1986 to 1998 28 patients below 18 years were operated at our Department of Surgery. There was a recognizable increase of patients in 1996, 1997 and 1998. Whereas from 1986 to 1995 an average of 1.5 Cholecystectomies in pediatric patients were done, the years from 1996 to 1998 show an average of 5,33 patients operated per year. Every patient obtained a cholecystectomy -- 15 conventional open Cholecystectomies and 13 Laparoscopies, which were primarily performed in children in our clinic in 1991. Besides cholecystectomy in one case a Hepaticoenterostomy was necessary and in another case surgical treatment of the Papilla of Vater and the common bile duct was performed. In 22 patients symptomatic cholelithiasis was the indication for a cholecystectomy. Another Patient had a gallbladder polyp consisting of heterotopic Duodenal glands, two patients showed a shock gallbladder following trauma and cardiac operation and three patients had chronic cholecystitis without gallstones. Clinical data was collected and retrospectively reviewed. Additionally, we created a personal questionnaire to carry through a follow-up. Three patients were less than 3 1/2 years old. The youngest patient was only 5 months old and presented with cholelithiasis and choledocholithiasis. Another male patient, aged 2 years received a cholecystectomy and a Hepaticoenterostomy because of a choledochal cyst Type Ib (Todani-classification). And a 3-year-old-girl had a shock gallbladder caused by thromboembolism following cardiac operation nine days before.
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3/16. 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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4/16. Multiple hepatic peribiliary cysts with cirrhosis.

    Multiple hepatic peribiliary cysts were found in three autopsy cases of patients who had had underlying liver diseases and obstructive jaundice. Macroscopically, the cysts were visible and present exclusively in the hepatic hilum and larger portal tracts. Histologically, the cysts were of varying size and were lined by a single layer of cuboidal or flattened epithelial cells without atypia. Intimate association between the cysts and peribiliary glands was found in the walls of large bile ducts. All three cases were associated with liver cirrhosis in patients with portal hypertension, and two of the patients had also had hepatocellular carcinoma. These findings support the previous assumption that multiple hepatic peribiliary cysts may be closely related to a portal hypertensive condition. Although peribiliary cysts have been considered to be clinically asymptomatic in general, in one of our patients, the cystic dilatation appeared to have been responsible for the progression of obstructive jaundice.
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5/16. Peribiliary cysts can mimic Caroli's disease: a case report.

    Peribiliary cysts, otherwise known as cystic dilatation of the peribiliary glands, are uncommon, and are usually discovered incidentally at autopsy, or in explants following liver transplantation. Preoperative diagnosis is often difficult owing to their asymptomatic nature and small size. Exclusion of a premalignant or malignant cystic condition is mandatory. We report a case of peribiliary cysts, initially thought to represent Caroli's disease, and briefly discuss the management of this condition.
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6/16. Multicystic biliary hamartoma: a hitherto undescribed lesion.

    In this report, we presented 3 cases of unusual hamartomatous nodules of the liver. These nodules were located around hepatic capsule of the left hepatic lobe and characteristically protruded from the liver. Histologically, these nodular lesions consisted of ductal structures, periductal glands, and fibrous connective tissues containing blood vessels. Smooth muscle bundles focally surrounded ductal structures. Bile-like materials were observed within some ducts. Two cases were associated with xanthogranulomatous inflammation around bile-like materials, and this inflammatory process extended from ductal lumens to periductal connective tissues. In contrast, the remaining case, which was not associated with inflammation, showed a honeycomb appearance. Ductal epithelium and periductal glands resembled biliary epithelium and peribiliary glands, respectively, and they also expressed biliary-type cytokeratins such as cytokeratins 7 and 19. These nodules shared pathologic characteristics of ciliated hepatic foregut cysts, such as their location (around the falciform ligament) and periductal smooth muscle bundles, but did not fulfill the diagnostic criteria (no ciliated cells and multilocular lesions). These hamartomatous nodules of the liver did not fit into any of the described categories of hepatic nodular lesions. At present, we speculate that these lesions might be related to developmental abnormalities of the biliary tract or embryonal foregut.
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7/16. Abnormal low ratio of cholic acid to chenodeoxycholic acid in a cholestatic infant with severe hypoglycemia.

    We report a premature infant with severe hypoglycemia (serum glucose: 6 mg/dl) and cholestasis (serum total bile acids: 211.55 mumol/L) caused by hypoplasia of the interlobular bile ducts. This patient had developed intracranial hemorrhage and sepsis while undergoing treatment for hypoglycemia. As a result of endocrine evaluation, we made a diagnosis of idiopathic panhypopituitarism, congenital absence or hypoplasia of the pituitary gland. Moreover, we found abnormal bile acid profiles: The ratio of cholic acid to chenodeoxycholic acid was abnormally low in serum (0.04) and in biliary bile (0.33). However, 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestan-26-oic acid and bile alcohols were not detected. We therefore suspected that the severe cholestasis and abnormal bile acid profiles in the serum and biliary bile in this patient were related to physiologic immaturity of the enterohepatic circulation of bile acids and immaturity of hepatic 12 alpha-hydroxylation.
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8/16. Hepatobiliary alterations in massive biliary ascariasis. Histopathological aspects of an autopsy case.

    Hepatobiliary alterations found in an autopsy case of massive Biliary ascariasis, are reported on histological grounds. Severe cholangitis was the main finding, but other changes were also detected, such as pyloric and intestinal metaplasia, hyperplasia of the epithelial lining, with intraductal papillomas and adenomatous proliferation. Remnants of the worm were observed tightly adhered to the epithelium, forming microscopic intrahepatic calculi. Mucopolysaccharides, especially acid, showed to be strongly positive on the luminal border, and in proliferated glands around the ducts. The authors discuss the similarity between such findings and Oriental Cholangio-hepatitis, and suggest that inflammation and the presence of the parasitic remnants are responsible for the hyperplastic and metaplastic changes, similarly with what occurs in chlonorchiasis, fascioliasis and schistosomiasis.
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9/16. Morphological study of cholesterol hepatolithiasis. Report of three cases.

    Three cases of pure cholesterol intrahepatic stones are compared morphologically to those of calcium bilirubinate stones. cholesterol stones were found in the intrahepatic bile duct of the left lateral lobe in two cases and in both the left lateral and the right posterior lobe in one. Although the chronic inflammatory reaction and fibrous thickening of bile duct wall were similar in both types of hepatolithiasis, the proliferation of intrahepatic periductal glands and the production of mucin were rather mild, compared to that is the liner containing calcium bilirubinate stones. Multiple intramural cholesterol calculi and cholesterin granulomas (cholesterin crystals surrounded by foreign-body giant cells) were found within the cystically dilated small bile duct branches and/or conduits of periductal glands. The calculi and granulomas were characteristic for cholesterol hepatolithiasis. These findings suggest that the formation of the cholesterol stones differs from that of calcium bilirubinate stones; the perturbation of factors influencing cholesterol nucleation in the hepatic bile may be related to the changed microenvironment of the intrahepatic bile ducts, which is followed by the formation of cholesterol stones.
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10/16. Solitary cystic dilation of the intrahepatic bile duct: morphology of two autopsy cases and a review of the literature.

    Solitary cystic dilation of intrahepatic bile ducts with neither extrahepatic biliary anomalies nor renal lesions has been reported previously in only 11 cases. We report two cases which were found as a result of postmortem cholangiography of 149 livers at autopsy. Case 1 had a cystic dilation measuring 1.2 cm in diameter in the left lateral superior area duct. Case 2 showed a cystic dilation measuring 1.0 cm in diameter in the left lateral segment duct. Histologically, the walls of the dilated ducts consisted of fibrous wall with proliferation of periductal mucous glands in both cases. The dilated and adjacent bile ducts in case 2 also contained tiny brown pigment stones and biliary sludge, suggesting that this bile duct lesion is important in the formation of intrahepatic calculi by providing a site for bile stasis and mucous hypersecretion.
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