Primary Hepatolithiasis, Recurrent Pyogenic Cholangitis, and Oriental Cholangiohepatitis-A Tale of 3 Countries
ABSTRACT Primary hepatolithiasis (HL), recurrent pyogenic cholangitis, and oriental cholangiohepatitis are terms commonly used in Japan, Hong Kong, and Korea respectively, and describing the different aspects of the same disease, with "HL" indicating the pathologic changes, "recurrent pyogenic cholangitis" emphasizing the clinical presentation and suppurative inflammation, and "oriental cholangiohepatitis" highlighting its ethnic preference and mysterious nature. HL is predominantly a disease of the far east and shows great regional differences in the incidence and the type of intrahepatic stones. Pathologically, it is characterized by pigmented calcium bilirubinate stones within dilated intrahepatic bile ducts featuring chronic inflammation, mural fibrosis, and proliferation of peribiliary glands, without extrahepatic biliary obstruction. Episodes of suppurative inflammation cumulate in sclerosing cholangitis in peripheral ducts and parenchymal fibrosis from scarring and collapse. Mass-forming inflammatory pseudotumor and neoplasms-like intraductal papillary neoplasms and cholangiocarcinoma are increasingly recognized complications. Bacterial infection and dietary factors are believed to be important in the formation of pigment stones within intrahepatic bile ducts, whereas parasitic infestation is likely coincidental. With improvement of environmental conditions and westernization of diet, the incidence of pigment stones has decreased. At the same time, cholesterol stones with milder clinical manifestations and pathologic changes are increasingly recognized, and for which stone dissolution therapy can be considered. Understanding the underlying pathology avoids confusion with other diseases more prevalent in the western world, and allows correct selection of the appropriate treatment.
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ABSTRACT: The aim of this study was to review the clinical, radiographic, and pathologic features of cases of benign segmental cholangiectasia in non-Asian US patients with clinical concern for cholangiocarcinoma and compare these features with cases of recurrent pyogenic cholangitis (RPC) in Asian patients. A total of 10 non-Asian US patients with benign segmental cholangiectasia were included in this study. Nine of them underwent partial hepatic resection due to cholangiographic findings of segmental cholangiectasia with mural thickening and/or proximal biliary stricture. One was found to have markedly dilated and thickened intrahepatic bile ducts at the time of autopsy. Clinical and radiographic findings were reviewed. Elastin stains and immunostains for immunoglobulin G4, cluster of differentiation (CD1a), and Langerin were performed. Six comparison cases of RPC in Asian US patients were also examined. Histologic examination of resection specimens revealed markedly dilated large intrahepatic bile ducts with variable degrees of mural fibrosis, periductal gland hyperplasia, inflammation, and liver parenchymal atrophy. These changes were not associated with a ductular reaction. There was no evidence of biliary dysplasia or biliary cirrhosis in any cases. No gross or microscopic feature definitively separated the Asian from non-Asian patients. The etiology of this disorder in non-Asian US patients is unclear. It does not appear to represent a localized variant of Caroli disease or primary sclerosing cholangitis. The high degree of similarity shared by these cases and classic RPC suggests a common pathogenic mechanism, although the pathologic features tend to be less well developed in the cases from the non-Asian US patients. Copyright © 2014 Elsevier Inc. All rights reserved.Human pathology 12/2014; 46(3). DOI:10.1016/j.humpath.2014.11.019 · 2.81 Impact Factor
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ABSTRACT: To investigate the feasibility, efficacy and safety of laparoscopic hepaticoplasty using gallbladder as subcutaneous tunnel and sphincter-of-Oddi preservation for hepatolithiasis. From January 2010 to July 2013, six patients with hepatolithiasis were treated at our institution. All the patients underwent laparoscopic surgery. The procedures included common hepatic duct exploration, stone clearance by fiberoptic choledochoscopy, hilar bile duct hepaticoplasty with preservation of the sphincter of Oddi, anastomosis between the hilar bile duct and neck of the gallbladder, and establishment of a subcutaneous tunnel with the gallbladder. Two patients underwent left lateral hepatectomy simultaneously. Clinical data including operation time, intraoperative blood loss, operative morbidity, hospital mortality, stone clearance, and recurrence rate were analyzed. All patients successfully completed laparoscopic surgery. The mean length of hospital stay was 4.5 ± 0.9 d (range: 3-6 d). The mean blood loss of the hepatectomy was 450 mL (range: 200-700 mL), and the blood loss of the other four was 137 ± 151 mL (range: 50-400 mL). The mean operative time was 318 ± 68 min (range: 236-450 min). The operative morbidity and hospital mortality were zero. The immediate stone clearance rate was 100%. All patients were followed up for an average of 17 mo (range: 7-36 mo). One of the six patients had abdominal mass with pain, and subcutaneous tunnel cholangiography showed severe gallbladder-biliary anastomotic stricture at 4 mo postoperatively. There was no stone recurrence and no cholangitis during follow-up. Laparoscopic hepaticoplasty using gallbladder with a subcutaneous tunnel and preserving the sphincter of Oddi is feasible, safe and effective for hepatholithiasis.World Journal of Gastroenterology 03/2014; 20(12):3350-5. DOI:10.3748/wjg.v20.i12.3350 · 2.43 Impact Factor
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ABSTRACT: Biliary tract carcinoma develops within the intrahepatic or extrahepatic biliary tree and gallbladder. Primary sclerosing cholangitis, hepatolithiasis, congenital choledochal cyst, liver fluke infection, pancreatobiliary maljunction, toxic exposures and hepatitis virus infection are risk factors for the development of human biliary carcinoma. The precise molecular abnormalities of biliary carcinogenesis are still unknown, but chronic inflammatory conditions induce the production of reactive oxygen or nitrogen species leading to DNA damage. Recent studies indicate that cholangiocarcinoma of the large bile duct may arise in premalignant lesions such as biliary intraepithelial neoplasm (BilIN) and intraductal papillary neoplasm of the bile duct (IPNB). BilIN and IPNB are generally confined to the large and septal-sized bile duct. BilINs are occasionally observed in non-biliary liver cirrhosis as well as chronic biliary disease. In contrast, the precursor lesion of intrahepatic cholangiocarcinoma of the small bile duct type remains unclear. We herein demonstrated the histological characteristics of different tumor development pathways from premalignant lesion to carcinoma in different sites of the biliary tree.Journal of Hepato-Biliary-Pancreatic Sciences 07/2014; 21(7). DOI:10.1002/jhbp.71 · 2.31 Impact Factor