Pathotyping and Clinical Manifestations of Biliary Cast Syndrome in Patients After an Orthotopic Liver Transplant

Abstract

Objectives: To summarize the pathotyping and clinical manifestations of biliary cast syndrome in patients after an orthotopic liver transplant. Materials and Methods:The clinical manifestations, auxiliary examination, therapeutic regimen, and clinical efficacy of 103 biliary cast syndrome patients who underwent an orthotopic liver transplant were retrospectively analyzed. Patients were divided into 6 groups from type 1 to type 6, according to the injury level of the biliary duct epithelium. Results: Many biliary cast syndrome patients showed symptoms including jaundice, dark urine, argillaceous stool, itchy skin, and fever. Serum levels of alanine aminotransferase, γ-glutamyl transpeptidase, alkaline phosphatase, and total bilirubin were increased. In addition, total white cell counts in peripheral blood also were increased. T-tube cholangiography showed filling defects of various amounts. Optical fiber choledochoscope examination revealed that the biliary tract was filled with solid substances, and necrosis of the biliary tract epithelium was observed in some biliary cast syndrome patients. From type 1 to type 6 biliary cast syndrome patients, the probability of clinical symptoms and biliary tract stricture gradually increased, the time needed for supporting gradually prolonged after removal of the biliary cast, and T-tube cholangiography showed that the filling defects gradually expanded. Conclusions:Clinical manifestations and cholangiography presentations mainly depend on pathotyping.

Endoscopic Management of Biliary Cast Syndrome Post Hepatic Trauma

“…With regard to outcome, Zhu et al studied 103 biliary cast syndrome patients who underwent liver transplant. They found a total cure rate and total cure rate of 54% and 71% total improve rate (18). Interestingly, the relapsing rate was as high as 55% after endoscopic treatment [27] Mortality was 13.6%.…”

“…Systemic bacterial or fungal infection, which causes intrahepatic cholestasis may also be a predisposing factor to the formation of casts in the bile ducts after liver transplantation [14]. Finally, many others have been associated as risk factors for the formation of biliary casts, such as, hepatic artery stenosis or thrombosis, biliary strictures, renal replacement therapy, ischemia-reperfusion injury, prolonged cold preservation, prolonged warm ischemia, hemolysis, pregnancy or AIDS virus infection [15][16][17][18].…”

“…Systemic bacterial or fungal infection, which causes intrahepatic cholestasis may also be a predisposing factor to the formation of casts in the bile ducts after liver transplantation [14]. Finally, many others have been associated as risk factors for the formation of biliary casts, such as, hepatic artery stenosis or thrombosis, biliary strictures, renal replacement therapy, ischemia-reperfusion injury, prolonged cold preservation, prolonged warm ischemia, hemolysis, pregnancy or AIDS virus infection [15][16][17][18].Casts can also occur in non-transplant patients, although that is extremely unusual; only a few cases have been published to date. Table 1 summarizes the different scenarios in which the biliary cast syndrome has been described [19][20][21][22][23][24][25][26].…”

Biliary Cast Syndrome Following Liver Transplantation: A Challenging Case

“…The clinical presentation may vary from asymptomatic to a varying severity of the symptoms mentioned above. Despite a lack of clinical symptoms or laboratory index changes, intrahepatic biliary tract filling defects and bile casts may be seen in the patients on ERCP or other imaging techniques [12].…”

“…thrombosis and stenosis and biliary ischemia is controversial but recent literature does suggest a possible link between the two [9,12]. Unfortunately, the unfavorable anatomy of his hepatic vessels made him a non-candidate for re-transplantation.…”