Agenesis of gallbladder and multiple anomalies of the biliary tree in a patient with portal thrombosis: a case report.

A 55-year-old male patient was admitted to hospital because of splenomegaly. Abdominal ultrasonography and computed tomography failed to demonstrate the gallbladder. The diagnosis of right portal vein thrombosis was established by Doppler ultrasonography, splenoportography and computed tomography angiography. To investigate the biliary tree and evaluate the effect on the biliary tree of portal changes, endoscopic retrograde cholangiopancreatography was performed. Endoscopic retrograde cholangiopancreatography study revealed the absence of gallbladder, cystic duct and common bile duct together with the junction of the right and left hepatic ducts at the pancreatic head, with predominant left hepatic duct. To our knowledge, this is the first reported case of multiple congenital anomalies of the extrahepatic biliary tree associated with right portal vein thrombosis. The presence of these rare pathologies in two viscera running together in a patient with right portal vein thrombosis is a very rare condition.