Biliary cast syndrome is a complication that occurs after liver transplantation, where dark solid bilirubin casts develop in the biliary tree, causing biliary obstruction.
This should be differentiated from biliary sludge, which represents thickened bile that has not precipitated.
It occurs in about 4% to 18% of the liver transplantation, mainly in the first 6 months and most often in liver transplantation from donation after cardiac death donors (DCD). 1,8
The most common presentation is with fever since the transplanted liver is insensitive/denervated. Laboratory tests will show cholestatic findings.
The hard casts are composed of bilirubin (up to 50%) and of bile acids (10-15%).2 They are thought to result from cold ischemia during the transplantation, but this has been disputed and other factors include acute rejection on a cellular basis, biliary infections, and biliary obstruction. 3
Endoscopic retrograde cholangiopancreatography (ERCP)
Usually, the first line of imaging because it offers the ability to perform cholangioplasty if necessary. It can show multiple areas of stricture and dilatations, along filling defects that represent biliary casts. 4
Irregular bile duct dilatation. Bile casts can manifest as linear hyperdense material within bile ducts.
Biliary casts appear hyperintense on T1-weighted MR images.5
Treatment and prognosis
It carries a greater mortality risk and can result in the need for another liver transplantation 6.
They are usually treated during an ERCP with a sphincterotomy, cast removal, and bile duct stenting.
Alternatives include percutaneous bile duct drainage and irrigation or surgical cast removal.
History and etymology
It was originally described in 1975 by Waldram, Williams, and Calne. 7
- 1. Srinivasaiah N, Reddy MS, Balupuri S, Talbot D, Jaques B, Manas D. Biliary cast syndrome: literature review and a single centre experience in liver transplant recipients. (2008) Hepatobiliary & pancreatic diseases international : HBPD INT. 7 (3): 300-3. Pubmed
- 2. Shah JN, Haigh WG, Lee SP, Lucey MR, Brensinger CM, Kochman ML, Long WB, Olthoff K, Shaked A, Ginsberg GG. Biliary casts after orthotopic liver transplantation: clinical factors, treatment, biochemical analysis. (2003) The American journal of gastroenterology. 98 (8): 1861-7. doi:10.1111/j.1572-0241.2003.07617.x - Pubmed
- 3. Gor NV, Levy RM, Ahn J, Kogan D, Dodson SF, Cohen SM. Biliary cast syndrome following liver transplantation: Predictive factors and clinical outcomes. (2008) Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society. 14 (10): 1466-72. doi:10.1002/lt.21492 - Pubmed
- 4. Verdonk RC, Buis CI, Porte RJ, Haagsma EB. Biliary complications after liver transplantation: a review. (2006) Scandinavian journal of gastroenterology. Supplement. doi:10.1080/00365520600664375 - Pubmed
- 5. Kinner S, Umutlu L, Dechêne A, Ladd SC, Barkhausen J, Gerken G, Lauenstein TC, Hunold P. Biliary complications after liver transplantation: addition of T1-weighted images to MR cholangiopancreatography facilitates detection of cast in biliary cast syndrome. (2012) Radiology. 263 (2): 429-36. doi:10.1148/radiol.12111625 - Pubmed
- 6. Franco J. Biliary complications in liver transplant recipients. (2005) Current gastroenterology reports. 7 (2): 160-4. Pubmed
- 7. Waldram R, Williams R, Calne RY. Bile composition and bile cast formation after transplantation of the liver in man. (1975) Transplantation. 19 (5): 382-7. Pubmed
- 8. Foley DP, Fernandez LA, Leverson G, Anderson M, Mezrich J, Sollinger HW, D'Alessandro A. Biliary complications after liver transplantation from donation after cardiac death donors: an analysis of risk factors and long-term outcomes from a single center. (2011) Annals of surgery. 253 (4): 817-25. doi:10.1097/SLA.0b013e3182104784 - Pubmed