Transjugular intrahepatic portosystemic shunt in a patient with Caroli’s disease and portal cavernoma

A 49 year old male, suffering from Caroli’s disease and long-standing portal vein thrombosis with cavernoma formation, presented with recurrent upper gastrointestinal bleeding from ectopic duodenal varices, uncontrolled by endoscopic and pharmacological management. Recent magnetic resonance imaging showed peri-oesophageal, perigastric (●) and periduodenal varices (*), portal cavernoma (∞) with a patent superior mesenteric vein, on a background of the known Caroli’s disease characterized by multiple parenchymal cystic dilatations of the biliary tree (ϕ) (Fig. 1). A transjugular intrahepatic portosystemic shunt (TIPS) creation was proposed after a multidisciplinary discussion. Portal cavernoma and Caroli’s disease, increasing the intraprocedural bleeding and infective risk, represent both relative contraindications for TIPS [ ]. As per routine TIPS creations in our centre, real-time ultrasound guidance was used for portal vein targeting and allowed for precise puncture of the portal vein remnant avoiding rupture of the multiple liver parenchymal cystic dilatations of the biliary tree. Subsequently two coaxial e-PTFE covered stents (Viatorr^®; GORE, Flagstaff, Arizona) were deployed reaching the porto-mesenteric confluence. The porto-caval pressure gradient decreased from 16 to 8 mmHg (Fig. 2). The procedure was performed without complications. The patient was discharged home 4 days after the procedure.