Late Ischemic Cholangiopathy After Liver Transplantation From a Deceased Donor With Situs Inversus Totalis: A Case...

Situs inversus totalis is a rare congenital anomaly in which the thoracic and abdominal viscera are arranged in mirror image. The use of liver grafts from deceased donors with situs inversus totalis is a challenge because of the profound anatomical and technical variations that complicate implantation. Approximately 11 such transplants have been reported worldwide. Here, we present a case of orthotopic liver transplant in a 66 -year -old man with hepatitis C -related cirrhosis and hepatocellular carcinoma in which the graft was from a 17 -year -old deceased donor with situs inversus totalis. The graft, which displayed mirror -image hilar anatomy, was implanted with a modified piggyback technique, orienting the donor right lobe anteriorly; direct duct -to -duct biliary reconstruction was performed. Graft function was preserved through most of the first postoperative year, after which the patient developed progressive cholestasis and diffuse biliary strictures. Allograft biopsies demonstrated ischemic changes of the bile ducts and parenchyma consistent with ischemic cholangiopathy in the absence of hepatic arterial thrombosis, without evidence of acute rejection but potential features of subtle chronic rejection. The patient was not a candidate for retransplant and died 14 months after transplant. This case illustrates a technically successful situs inversus totalis donor graft that was complicated by late, diffuse, and irreversible biliary obstruction.