Single-access liver floss technique with antegrade hepatic vein access and recanalization in Budd-Chiari syndrome

Abstract

B udd-Chiari syndrome encompasses a variety of conditions, all characterized by hepatic outflow obstruction but heterogeneous in etiology and morphology of venous occlusion as well as clinical manifestations of hepatic congestion. Prompt, appropriate treatment improves prognosis. A transjugular intrahepatic portosystemic shunt (TIPS) may be created to manage sequelae of portal hypertension and provide a bridge to transplant if significant hepatic injury has occurred. Liver transplant may be the sole treatment option if the disease is identified in late stages. When early in the disease process, however, parenchymal damage may be mitigated by reestablishing hepatic venous outflow which has classically been achieved by transjugular or percutaneous transhepatic access approaches when transjugular approaches prove infeasible. TECHNIQUEA 14-year-old male presented with a 6-month history of progressive exercise intolerance and abdominal distension. Evaluation at an outside hospital identified massive ascites and concluded with a provisional diagnosis of cirrhosis on the basis of ascites and the abnormal appearance of the liver on abdominal computed tomography (CT). The patient was subsequently transferred to our institution for further evaluation. On presentation, he was found to have a tense and distended abdomen without other signs of liver failure. Initial serologic workup including liver enzymes, ceruloplasmin, alpha-1 antitrypsin pi typing, autoimmune hepatitis serologies, and hepatitis B and C studies were negative, and he was referred to interventional radiology for paracentesis and transjugular liver biopsy.Right internal jugular vein access was obtained. Attempts to cannulate the hepatic veins were unsuccessful despite several catheter configurations. A single plane digital subtraction cavogram was acquired, notable for the absence of reflux into normal hepatic veins at the level of the venous confluence (Fig. 1a). Additional evaluation of the inferior vena cava (IVC) and tributaries was performed with axial intravascular ultrasound (Volcano Visions PV .035, Philips), demonstrating the absence of right (RHV), middle (MHV) and left hepatic veins (LHV) at the hepatic vein confluence. An engorged caudate vein was identified, connecting to the IVC via a small orifice. A 5 French (F) angled hydrophilic catheter (Glidecath, Terumo) and 0.035-inch hydrophilic guidewire (Glidewire, Terumo) were introduced and used to cannulate the identified orifice and were subsequently advanced into the right intrahepatic venous system. Planar digital subtraction venogram (Fig. 1b) I N T E R V E N T I O N A L R A D I O LO G Y T E C H N I C A L N OT EABSTRACT A 14-year-old boy presented with several months of increasing abdominal girth and fatigue. Imaging confirmed massive ascites and hepatic congestion secondary to central hepatic venous obstruction. Several large intrahepatic collateral veins were seen draining via caudate and emissary veins. After an unsuccessful attempt at retrograde recanalization utilizing intravascular ultr...