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Herein we describe a case of obstructive jaundice and cholangitis originating from choledochal lithiasis secondary to a stricture of the common bile duct in a patient with cavernous transformation of the portal vein. In fact, portal cavernous transformation gives rise to many dilated pericholedochal and periportal collaterals that bypass the portal vein obstruction. Extrinsic compression of the common duct by dilated venous collaterals together with pericholedochal fibrosis from the inflammatory process causing portal thrombosis may lead to biliary stricture and dilatation of the proximal biliary tree. This condition sometimes causes the formation of secondary biliary stones and cholangitis. Treatment in our case could not be accomplished by a biliodigestive anastomosis because the patient suffered from a short bowel syndrome subsequent to extensive ileal resection for splanchnic venous thrombosis. We repeatedly attempted stone removal during endoscopic retrograde cholangiopancreatography (ERCP) but finally resorted to a percutaneous transhepatic approach, which permitted removal of the stones and treatment of the stricture by pneumatic dilatation. The patient remains well 3 years after the procedure and has shown no signs of jaundice or cholangitis.

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