Resurgence of biliary cast syndrome

Authors

  • See Ching Chan,

    1. Centre for the Study of Liver Disease and Department of Surgery, the University of Hong Kong, Pokfulam, Hong Kong, China
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  • Chung Mau Lo,

    1. Centre for the Study of Liver Disease and Department of Surgery, the University of Hong Kong, Pokfulam, Hong Kong, China
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  • Chi Leung Liu,

    1. Centre for the Study of Liver Disease and Department of Surgery, the University of Hong Kong, Pokfulam, Hong Kong, China
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  • Sheung Tat Fan

    Corresponding author
    1. Centre for the Study of Liver Disease and Department of Surgery, the University of Hong Kong, Pokfulam, Hong Kong, China
    • Department of Surgery, the University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong, China
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    • Telephone: 852-2855-4703 Fax: 852-2818-4407


Biliary cast syndrome (BCS), also known as ischemic-type biliary lesion, is peculiar in deceased donor liver transplantation was first described three decades ago.1 Improvement of graft harvesting and preservation significantly reduced its incidence. Resurgence of BCS in current practice necessitates identification of etiologies not commonly considered. BCS is characterized by multiple intrahepatic biliary strictures, ductal dilatation, intrahepatic abscesses, and biliary anastomotic leakage.

Abbreviation:

BCS, biliary cast syndrome

Case

A 53-year-old male hepatitis B carrier underwent deceased donor liver transplantation in Mainland China for hepatocellular carcinoma. Liver graft cold ischemic time was 8 hours and warm ischemic time according to discharge summary was only 5 minutes. Immunosuppressive regimen included corticosteroid and tacrolimus. Lamivudine and hepatitis B immunoglobulin were administered for control of hepatitis B. In view of the persistently deranged liver functions, the patient was transferred to us for further management.

On admission, blood test results showed bilirubin 303 μmol/L, alanine aminotransferase 412 U/L, aspartate aminotransferase 251 U/L, alkaline phosphatase 676 U/L, and gamma-glutamyl transferase 1023 U/L. Bilirubin became elevated to more than 600 μmol/L over a 2-week period. Computed tomography showed edematous periportal tissues. Hepatic arteries and portal veins were patent. Liver biopsy revealed features of large duct obstruction. Endoscopic retrograde cholangiopancreatography showed distended intrahepatic bile ducts with extensive filling defects, features of BCS (Figure 1). Tight space between the cast and ductal wall precluded deployment of a stent.

Figure 1.

Endoscopic retrograde cholangiopancreatography revealing dilated common hepatic duct and intrahepatic ducts with filling defects caused by a single biliary cast.

On laparotomy, a collection was present in Morison's pouch. Exploration of the extrahepatic portion of the graft bile ducts that lacked a definable wall resurfaced a biliary cast in one piece. The biliary tract bilaterally and even the cystic duct of the liver graft were replicated by the cast (Figure 2). Choledochoscopy revealed irregular surface of the biliary system. The choledochotomy was closed over a 14F latex T-tube for external drainage. The patient's liver function slightly improved postoperatively. He continued receiving lamivudine 100 mg daily and ursodeoxycholic acid 250 mg three times a day. Liver biopsy 3 weeks later showed features of cholestasis and large duct obstruction. One-half year from liver transplantation, liver function tests remained abnormal with bilirubin levels higher than 400 μmol/L, yet with satisfactory synthetic functions (prothrombin time 12 seconds).

Figure 2.

Biliary cast replicating the intrahepatic and extrahepatic ductal systems including the cystic duct stump.

Discussion

BCS is uncommon nowadays. Its etiological factors include prolonged warm ischemic time,2 biliary strictures,1 cholangitis, biliary drainage tubes, and acute cellular rejection. The former tends to occur in non–heart-beating donors,3 while biliary complications are associated with cytomegalovirus infection.4 Severity of condition correlates with a chance of graft loss and mortality should re-transplantation not be feasible. Cholangiography, usually endoscopically, is the standard means of diagnosis.5 Retrieval of the cast by basket extraction by endoscopic retrograde cholangiopancreatography as first-line management has been reported.6, 7 Involvement of both extra- and intrahepatic bile ducts gives no chance to endoscopic therapy but open surgery or re-transplantation.8 Subsequent improvement of the liver function relies on recovery of the ductal system, whereas temporary drainage is maintained by a T-tube. Effective preparation and preservation of the liver graft should prevent its occurrence.

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