[Idiopathic ductopenia in adults as a cause of cryptogenic cirrhosis and complications of portal hypertension].

We describe a 56 years old male patient with long-term chronic liver disease of unknown etiology presenting with esophageal varices rupture. Prophylaxis of re-bleeding with propranolol and endoscopic sclerotherapy failed to prevent further haemorrhagic events and the placement of a transjugular intrahepatic portosystemic shunt (TIPS) was needed. The portal hemodynamic data revealed sinusoidal portal hypertension and the liver biopsy displayed ductopenic cholestasis. The patient met all criteria of idiopathic ductopenia. Subsequently, the jaundice worsened and the patient required liver transplantation.