University of California, Los Angeles, Department of Medicine, Los Angeles, CA, USA.
Ann Hepatol. 2009 Oct-Dec;8(4):379-83.
Primary sclerosing cholangitis (PSC) is a chronic, progressive, inflammatory and obstructive disease of the intra- and extra-hepatic bile ducts of unknown etiology. Currently, orthotopic liver transplantation (OLT) is the only definitive treatment for PSC-related end-stage liver disease. However, PSC has been known to recur in the grafted liver. Roux-en-Y hepaticojejunostomy is more commonly performed than choledochocholedochostomy for PSC, although choledochocholedochostomy has been found to be safe and efficacious for PSC if the distal common bile duct is uninvolved at the time of OLT. Our case is unique in that it describes a patient who developed de-novo cholangiocarcinoma in the remnant portion of the native common bile duct six years after OLT with choledochocholedochostomy for PSC-associated end-stage liver disease without having PSC recurrence. In conclusion, our case report indicates that choledochocholedochostomy may not be desirable in PSC due to an increased risk of developing cholangiocarcinoma in the native common bile duct. This risk exists as well with a Roux-en-Y hepaticojejunostomy in the remaining intra-duodenal and intra-pancreatic biliary epithelium, although in theory to a lesser extent. Therefore, the risk of developing cholangiocarcinoma in the recipient common bile duct can only be completely eliminated by performing a Whipple procedure at the time of OLT.
原发性硬化性胆管炎（PSC）是一种原因不明的肝内外胆管的慢性、进行性、炎症性和阻塞性疾病。目前，原位肝移植（OLT）是 PSC 相关终末期肝病的唯一确定性治疗方法。然而，PSC 已知会在移植肝中复发。与 PSC 相比，Roux-en-Y 肝肠吻合术更为常见，尽管如果在 OLT 时远端胆总管未受累，胆管空肠吻合术对于 PSC 是安全有效的。我们的病例很独特，它描述了一名患者在 OLT 后六年，因 PSC 相关终末期肝病而行胆管空肠吻合术，在没有 PSC 复发的情况下，在残留的原发性胆总管中出现新发胆管癌。总之，我们的病例报告表明，由于原发性胆总管中发生胆管癌的风险增加，胆管空肠吻合术在 PSC 中可能并不理想。在剩余的十二指肠内和胰内胆管上皮中，虽然理论上程度较轻，但 Roux-en-Y 肝肠吻合术也存在这种风险。因此，只有在 OLT 时行胰十二指肠切除术才能完全消除受体胆总管发生胆管癌的风险。
