bile cast syndrome ( bcs ) is a complication of orthotopic liver transplantation ( olt ) . we describe 2 patients with bcs who were successfully diagnosed with endoscopic retrograde cholangio pancreatography ( ercp ) and one of whom was successfully treated endoscopically . a 65-year - old female underwent olt 6 months prior to presentation for chronic hepatitis c and hepatocellular carcinoma . her intraoperative course during liver transplantation was complicated by a significant amount of blood loss from hepatic veins . she had a complicated postoperative course including hemorrhagic shock , abdominal compartment syndrome and acute renal failure which required hospitalization for 2 months after the transplantation . she developed an anastomotic bile leak which was treated with placement of a 10 fr stent . at 4 weeks and 8 weeks after initial ercp , the cbd stents were exchanged and secondary sclerosing cholangitis was suspected based on cholangiographic appearance . her immunosuppression regimen included tacrolimus 9  mg bid and mycofenolic acid 180  mg bid . laboratory data showed bilirubin of 1.1  mg / dl ( reference range : 0.2  mg / dl-1.3  mg / dl ) , alanine aminotransferase of 53  iu / l ( reference range : 13  iu / l-69  iu / l ) , aspartate aminotransferase of 60  iu / l ( reference range : 15  iu / l-46 iu / l ) , alkaline phosphatase of 257  iu / l ( reference range : 42  iu / l-140 iu / l ) , -glutamyl transferase of 466  iu / l ( reference range : 5  iu / l-55 balloon sweeps yielded a small amount of debris , but cholangiographic findings persisted that were concerning for filling defects or marked ductal irregularity in the region of the common hepatic duct and bifurcation . cholangioscopy ( fig .  2 ) was performed with the spyglass single operator biliary visualization system ( boston scientific , natick , ma , usa ) . a tubular biliary cast , in the shape of the bile duct , was seen in the donor bile duct extending from the anastomosis into the intrahepatic ducts . attempts to extract the cast with a snare , basket , mechanical lithotripter , balloon , spybite(boston scientific , natick , ma , usa ) biopsy forceps , and standard egd forceps were all unsuccessful due to inability to grasp the cast and tethering of the cast to intrahepatic extension . a 10 fr 12-cm cbd stent was placed . in subsequent months , it appears to contain filling defects which retrospectively are related to presence of biliary cast material in the right and left main hepatic ducts . a 58-year - old male with a history of hepatitis c and cirrhosis presented with fevers , chills , and a headache . the patient s immunosuppression regimen included mycophenolate 360  mg bid , prednisone 5  mg daily and tacrolimus 8  mg bid . laboratory data showed bilirubin of 2.2  mg / dl ( reference range : 0.2  mg / dl-1.3  mg / dl ) , alanine aminotransferase of 162  iu / l ( reference range : 13  iu / l-69  iu / l ) , aspartate aminotransferase of 159  iu / l ( reference range : 15  iu / l-46 iu / l ) , alkaline phosphatase of 1277  iu / l ( reference range : 42  iu / l-140  iu / l ) and white blood cell count of 8.17    10 cells / l . the left main hepatic duct was dilated with a 4-mm dilating balloon to facilitate clearance of the filling defects . cholangiogram prior to intervention shows diffuse stricturing of the left and right hepatic ductal system in patient 2 . b cholangiogram after removal of the biliary cast showing a widely patent left hepatic duct in patient 2 . subsequent to removal of the biliary cast , endoscopic treatment for the anastomotic stricture resulted in resolution of the stricture . a 65-year - old female underwent olt 6 months prior to presentation for chronic hepatitis c and hepatocellular carcinoma . her intraoperative course during liver transplantation was complicated by a significant amount of blood loss from hepatic veins . she had a complicated postoperative course including hemorrhagic shock , abdominal compartment syndrome and acute renal failure which required hospitalization for 2 months after the transplantation . she developed an anastomotic bile leak which was treated with placement of a 10 fr stent . at 4 weeks and 8 weeks after initial ercp , the cbd stents were exchanged and secondary sclerosing cholangitis was suspected based on cholangiographic appearance . her immunosuppression regimen included tacrolimus 9  mg bid and mycofenolic acid 180  mg bid . laboratory data showed bilirubin of 1.1  mg / dl ( reference range : 0.2  mg / dl-1.3  mg / dl ) , alanine aminotransferase of 53  iu / l ( reference range : 13  iu / l-69  iu / l ) , aspartate aminotransferase of 60  iu / l ( reference range : 15  iu / l-46 iu / l ) , alkaline phosphatase of 257  iu / l ( reference range : 42  iu / l-140 iu / l ) , -glutamyl transferase of 466  iu / l ( reference range : 5  iu / l-55 balloon sweeps yielded a small amount of debris , but cholangiographic findings persisted that were concerning for filling defects or marked ductal irregularity in the region of the common hepatic duct and bifurcation . cholangioscopy ( fig .  2 ) was performed with the spyglass single operator biliary visualization system ( boston scientific , natick , ma , usa ) . a tubular biliary cast , in the shape of the bile duct , was seen in the donor bile duct extending from the anastomosis into the intrahepatic ducts . attempts to extract the cast with a snare , basket , mechanical lithotripter , balloon , spybite(boston scientific , natick , ma , usa ) biopsy forceps , and standard egd forceps were all unsuccessful due to inability to grasp the cast and tethering of the cast to intrahepatic extension . a 10 fr 12-cm cbd stent was placed . in subsequent months , it appears to contain filling defects which retrospectively are related to presence of biliary cast material in the right and left main hepatic ducts . a 58-year - old male with a history of hepatitis c and cirrhosis presented with fevers , chills , and a headache . he underwent deceased donor liver and kidney transplantation two months prior to his presentation . the patient s immunosuppression regimen included mycophenolate 360  mg bid , prednisone 5  mg daily and tacrolimus 8  mg bid . laboratory data showed bilirubin of 2.2  mg / dl ( reference range : 0.2  mg / dl-1.3  mg / dl ) , alanine aminotransferase of 162  iu / l ( reference range : 13  iu / l-69 iu / l ) , aspartate aminotransferase of 159  iu / l ( reference range : 15  iu / l-46 iu / l ) , alkaline phosphatase of 1277  iu / l ( reference range : 42 iu / l-140  iu / l ) and white blood cell count of 8.17    10 cells / l . the left main hepatic duct was dilated with a 4-mm dilating balloon to facilitate clearance of the filling defects . 4 ) . following extraction , cholangiograms revealed a widely patent left main duct ( fig . cholangiogram prior to intervention shows diffuse stricturing of the left and right hepatic ductal system in patient 2 . b cholangiogram after removal of the biliary cast showing a widely patent left hepatic duct in patient 2 . subsequent to removal of the biliary cast , endoscopic treatment for the anastomotic stricture resulted in resolution of the stricture . bile casts occur in 4  % to 18  % of olt recipients 1 . more than 70  % of casts are identified in the first 16 weeks after transplant 1 . bile casts are dark and hard in appearance and formed by lithogenic material confined to the bile duct dimensions 3 
 4 . although the exact mechanism of injury is unknown , ischemic injury to the biliary epithelium is most common 5 
 6 
 7 . other mechanisms including anastomotic and non - anastomotic strictures leading to accumulation of biliary sludge can lead to formation of casts 5 . bacterial infection and stones are also predisposing factors for biliary ductal injury and cast formation 6 . microcirculatory dysfunction is also suggested , given the higher prevalence of renal failure and need for renal replacement therapy in patients with bcs 7 . a combination of endoscopic interventions is considered including sphincterotomy , balloon and basket extraction , lithotripsy and stent placement . in one study , re - transplantation and 12-month mortality rates were significantly higher with bcs ( 30  % vs. 7  % ) and bcs patients also required a longer intensive care unit stay post - olt 7 . success rates with endoscopic therapy for bcs have been reported to be 70  % recently 7 . previously in a large study of post - olt adverse events , biliary cast syndrome occurred in 4/260 patients and a median of 3.5 ercp treatments attempted over a median of 21 weeks achieved a success rate of 25  % 8 . percutaneous drainage has also been advocated , especially in the setting of cholangitis or with roux - en - y choledochojejunostomy . percutaneous transhepatic choledocoscopy via a tract created with radiographic assistance is a novel approach used for management of bcs . when there is intrahepatic biliary system involvement , management becomes even more challenging . retransplantation is considered when complete removal is not feasible and if the patient s clinical condition deteriorates 2 
 7 . a combined endoscopic and percutaneous method was able to successfully clear the casts in 60  % of patients in one study 9 . surgical intervention including hepaticojejunostomy , choledochojejunostomy and revision of choledochocholedocostomy reportedly has had success rates above 85  % 10 .
background and study aims  bile cast syndrome ( bcs ) is a complication of orthotopic liver transplantation ( olt ) . 
 it occurs in 4  % to 18  % of olt recipients and can present as cholangitis and graft damage or loss 
 . twenty - two percent of patients with bcs require repeat olt . 
 the diagnosis and management of bcs can be challenging . 
 our aim is to share our experience with bcs and to briefly review the diagnosis and management of the condition .