Portal vein phlebolithiasis found post-liver transplantation in the native liver of a child with biliary atresia

Biliary atresia is defined as partial or total obliteration of the extra-hepatic bile ducts. In advanced cases, liver transplantation (LTx) is considered the most appropriate treatment. This report describes a female patient whose biliary atresia and subsequent cirrhosis required LTx at 1 yr of age. Macroscopic inspection of the hilar region of the native liver post-Tx revealed the formation of a pouch in the hepatic duct and a stone in the lumen of the portal vein. X-ray diffraction analysis showed that the stone was composed of cholesteryl cinnamate, gluconic acid phenylhydrazide, Na beta broma-allyl mercaptomethyl penicillinate, and Al2O3 crystals. While the cholesterol component is a known element of gallstones, we attributed the Na beta broma-allyl mercaptomethyl penicillinate to the patient's drug therapy. Our literature search revealed no previous record or crystallographic analysis of portal vein phlebolithiasis. In this report we describe this rare finding.