Biliary Cast Formation with Sclerosing Cholangitis in Critically Ill Patient: Case Report and Literature Review

Sclerosing cholangitis in critically ill patients (SC-CIP) is a rare condition that is not familiar to many radiologists. In addition, the associated imaging findings have not been described in the radiological literature. We report a case of biliary cast formation with SC-CIP and describe the radiological findings of CT, magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiography (ERC). A diagnosis of SC-CIP should be considered in intensive care unit (ICU) patients with persistent cholestasis during or after a primary illness. The typical CT, MRCP and ERC findings include new biliary casts in the intrahepatic duct with multiple irregular strictures, dilatations, and relative sparing of the common bile duct.

Biliary Cast Syndrome in Non-Liver Surgery Patients / 대한소화기학회지

Biliary cast describes the presence of casts within the biliary tree. It is resultant sequel of cholangitis and hepatocyte damage secondary to bile stasis and bile duct injury. Biliary cast syndrome was first reported in patient undergone liver transplantation. The pathogenesis of biliary cast is not clearly identified, but proposed etiologic factors include post-transplant bile duct damage, ischemia, biliary infection, or post-operative biliary drainage tube. Although biliary casts are uncommon, most of biliary cast syndrome are reported in the liver transplant or hepatic surgery patients. A few reports have been published about non-transplant or non-liver surgery biliary cast. We report two cases of biliary cast syndrome in non-liver surgery patients.

The therapeutic value of percutaneous transhepatic cholangioscopy for biliary cast after liver transplantation / 中华消化内镜杂志

Objective To investigate the value of percutaneous transhepatic cholangioscopy (PTCS) for diagnosis and treatment of biliary cast after liver transplantation. Methods Data of 11 patients with biliary cast after liver transplantation, who underwent PTCS from April 2008 to November 2010, were retrospectively analyzed. Results In 11 patients , one had biliary cast in common bile duct, 3 in right intra-hepatic bile duct, 4 in left intra-hepatic bile duct, and 3 distributed in intra- and extra-hepatic bile ducts. A total of 68 times of PTCS were performed in 11 patients, achieving significant decrease in levels of serum transaminase and bilirubin in 10. Occasional fever occurred in 1 patient after closure of drainage tube,which was managed by replacement with a thinner one. There were no severe complications such as biliary fistula or uncontrollable bleeding. Partial rupture of fistula occurred in 1 case. All patients were followed up for 10-30 months and were all in good condition except one patient died from other disease during the followup. Conclusion PTCS is a safe, effective and applicable method to treat the biliary cast after liver transplantation.

ERCP for diagnosis and management of biliary cast syndrome after liver transplantation / 中华消化内镜杂志

Objective To evaluate endoscopic retrograde cholangiopancreatography (ERCP) for diagnosis and management of biliary cast syndrome after orthotropic liver transplantation. Methods A total of 71 consecutive patients with abnormal liver function and MRCP findings after liver transplantation underwent ERCP for diagnosis and management. Their data were retrospectively reviewed. Results A total of 188 sessions of ERCP were carried out on the 71 patients, most of whom were found to have stenosis of anastomotic stoma and/or bile duct. Bile sludge was found and removed in all patients diagnosed within 3 months after liver transplantation, while pigmentoid stones were found and removed in patients diagnosed within 3-6 months and biliary casts in patients diagnosed at more than 6 months. Each patient underwent 2.6 sessions averagely. Biliary casts were formed at an average time of 22. 7 ± 15.6 months after transplantation. PostERCP complications included 2 cases of pancreatitis and 3 cholangitis, with an occurrence rate of 2. 6%(5/188), which were all controlled with conservative treatment. The follow-up data was available in 56 patients showing improvement in liver function after ERCP, among who 42 met the endoscopic criteria of cure,1 0 received second liver transplantation because of progressive sclerosing cholangitis and 4 died from diseases other than liver transplantation. Conclusion Therapeutic ERCP for the biliary cast syndrome after liver transplantation is feasible, safe and effective, and can be performed repeatedly with good short-term effect.

A Case of Biliary Cast Syndrome in a Non-liver Transplatation Patients / 대한소화기내시경학회지

The development of total biliary casts is very unusual, and especially in patients who have not undergone liver transplantation. There are only a few reports of total biliary casts in non-liver transplantation patients who have antiphospholipid antibody syndrome, B-cell non-Hodgkin's lymphoma, cholecystectomy or allogenic hematopoietic stem cell transplantation. Here we present the case of a previously well 77-year-old man who developed a total biliary casts without any risk factors and there was no obvious liver insult. The casts were managed endoscopically.

A Case of Biliary Cast Syndrome in a Non-liver Transplant Patient / 대한소화기내시경학회지

Biliary casts are uncommon but are typically seen in post-liver transplant patients, whereas very few cases have been reported in non-liver transplant patients. A 65-year-old man, who had not undergone a liver transplantation or other hepatobiliary surgery, presented with jaundice and fever. Radiological imaging studies showed diffuse thickening and enhancement of the intrahepatic duct with mild ductal dilatation and multiple linear filling defects. A percutaneous transhepatic cholangioscopy revealed severe bile duct inflammation and multiple biliary casts, which were removed with multiple percutaneous choledocoscopic procedures. The pathogenesis of the biliary casts in this patient was uncertain. However, we presumed that biliary tract infection with subsequent extensive cholangitis was an important predisposing factor in the cast formation. We report a case of biliary cast syndrome with no preexisting morbidity that was managed favorably with endoscopic removal.

A Case of Biliary Cast Syndrome after Cadaveric Liver Transplantation / 대한소화기학회지

We experienced one fatal case of biliary cast syndrome after cadaveric liver transplantation involving both intrahepatic ducts. A 58-year-old man underwent cadaveric liver transplantation because of hepatitis B virus related liver cirrhosis and concomitant hepatocellular carcinoma. Five weeks after the liver transplantation, postoperative course was complicated by development of acute cholangitis. Subsequent endoscopic retrograde cholangiography revealed diffuse intrahepatic bile duct strictures without filling defects. Percutaneous liver biopsy, which was done to exclude rejection, revealed biliary cast. Successful endoscopic removal was precluded due to its diffuse involvement. Because of the deterioration of patient's condition by refractory biliary obstruction and cholangitis, retransplantation from cadaveric donor was performed. Debridement of the biliary tree after graft removal yielded a near-complete cast of the intrahepatic ductal system. Biliary cast syndrome should be suspected when jaundice or cholangitis is associated with dilated ducts on abdominal imaging studies in cadaveric liver transplantation recipients. Initial therapeutic options include removal of biliary cast after endoscopic or percutaneous cholangiography. Although endoscopic retrieval of biliary cast by endoscopic retrograde cholangiopancreatography could be employed as a first-line management, other modalities such as endoscopic nasobiliary drainage, percutaneous transhepatic drainage, or retransplantation should be considered when complete removal is not feasible and the condition of the recipient deteriorates.

A Case of Biliary Cast Syndrome with a Biliary Stricture and Suppurative Cholangitis after Liver Transplantation / 대한소화기내시경학회지

Biliary complications after liver transplantation occur in 13~35% of patients. Biliary cast syndrome, cast formation of biliary sludge along the bile duct, can develop in 4~18% of liver transplant recipients, although the incidence rate is significantly decreasing due to the improvement of graft harvesting and preservation. It is very important that early diagnosis and effective management of biliary cast syndrome be performed when there is a bile duct stricture or dilatation associated with jaundice and cholangitis in the recipient after liver transplantation, due to the possibility of retransplantation and death of the patient from graft loss. We report a case of a biliary cast formed with suppurative cholangitis and extracted incidentally with a plastic biliary stent during an endoscopic procedure after cadaveric liver transplantation, in which the ERCP findings revealed a stricture at the anastomosis site of the common bile duct and cholangitis.

Biliary Cast Successfully Removed by Percutaneous Transhepatic Cholangioscopy, and This Developed in a Patient Who Received Orthotopic Liver Transplantation / 대한소화기내시경학회지

"Biliary cast syndrome" describes a cast formed from retained lithogenic material, and this cast is morphologically confined to the bile duct; this develops in 4~18% of liver transplant recipients. The pathogenesis of cast formation is not clearly understood. The proposing etiological factors for biliary cast syndrome include acute cellular rejection, a prolonged cold ischemic time, use of postoperative biliary drainage tubes and biliary infection. These casts are more likely to develop in the setting of hepatic ischemia and biliary stricture. Endoscopic and percutaneous cast extraction might achieve favorable results and this should be attempted before surgical therapy. We report here on a case of biliary cast syndrome that was secondary to orthotopic liver transplantation; this was successfully treated via percutaneous choledochoscopic removal. We also include a review of the literature.

Obstructive Jaundice due to Biliary Cast Syndrome Followed by Orthotopic Liver Transplantation / 대한소화기학회지

Biliary complication occurs in 6-34% of all liver transplant patients. Although bile leaks and strictures are relatively common, other biliary complications such as T-tube leak, choledocholithiasis, and biliary cast syndrome can also be observed. The biliary cast syndrome describes the presence of casts causing obstruction with its resultant sequelae of biliary infection, hepatocyte damage secondary to bile stasis and ductal damage, all contributing to cholangiopathy. Because the exact timing of cast formation after orthotopic liver transplantation is not consistent, it is difficult to define the true incidence of biliary cast syndrome without long-term follow-up data. Proposed etiological mechanisms include acute cellular rejection, prolongation of cold ischemic time, infection, biliary drainage tubes, and biliary obstruction. The diagnosis of biliary cast syndrome is usually confirmed by endoscopic retrograde cholangiopancreatography. There have been few published articles about biliary casts in Korea. Herein, we report a case of biliary cast syndrome followed by orthotopic liver transplantation.

A Case of Biliary Cast with a Characteristic Finding on ERCP / 대한소화기내시경학회지

Cast formation of biliary sludge leading to obstruction and cholangitis have been reported in patients who received orthotopic liver transplantation. The pathogenesis of biliary cast after orthotopic liver transplantation appeared to be multifactorial. Cold ischemic damage, immunologic attack to the bile duct and bile stasis may have played a role. On the other hand, only three cases of biliary cast have been reported in non-transplanted patients. We described the successful endoscopic removal of this complication in a 70-year-old man whose biliary sludge aggregated into firm casts occupying the extrahepatic ducts which has been developed after cholecystectomy and segmentectomy for intrahepatic cholangiocarcinoma. We speculated on the hemolysis and prolonged fasing as the initiating events but the exact pathogenesis of biliary cast remains to be clarified.

A Case of Biliary Cast with a Characteristic Finding on ERCP / 대한소화기내시경학회지

Cast formation of biliary sludge leading to obstruction and cholangitis have been reported in patients who received orthotopic liver transplantation. The pathogenesis of biliary cast after orthotopic liver transplantation appeared to be multifactorial. Cold ischemic damage, immunologic attack to the bile duct and bile stasis may have played a role. On the other hand, only three cases of biliary cast have been reported in non-transplanted patients. We described the successful endoscopic removal of this complication in a 70-year-old man whose biliary sludge aggregated into firm casts occupying the extrahepatic ducts which has been developed after cholecystectomy and segmentectomy for intrahepatic cholangiocarcinoma. We speculated on the hemolysis and prolonged fasing as the initiating events but the exact pathogenesis of biliary cast remains to be clarified.