Hepatolithiasis associated with anomalous biliary anatomy and a vascular compression. Hepatolithiasis and anomalous...

Biliary tract abnormalities occur in about one of every three people, usually being minor and of no clinical significance. Major abnormalities, however, may present in an unusual manner and provide a major hazard to the unsuspecting surgeon. A patient presenting with cholangitis without jaundice or abnormal liver function tests is reported. Endoscopic retrograde cholangiography failed to demonstrate any bile ducts in the right postero-lateral segments of the liver, the "naked segment sign". A percutaneous transhepatic cholangiogram demonstrated a stricture obstructing the right posterior segmental hepatic duct with hepatolithiasis above the stricture. At operation an anomalous vessel was found at the site of the stricture. This case highlights the unusual way in which biliary tract anomalies may present and the importance of adequate pre-operative investigation.