Treacherous Pitfalls in Liver Transplantation for Budd-Chiari Syndrome: When the Devil Hides in the Shadows-A Case...

Budd-Chiari syndrome (BCS) is a rare vascular liver disorder characterized by obstruction of the hepatic venous outflow, involving the hepatic veins and/or the inferior vena cava (IVC). Etiology varies geographically, with thrombophilic conditions and myeloproliferative disorders predominating in Western countries, and membranous IVC obstruction being more frequent in Eastern countries. Liver transplantation (LT) remains the definitive treatment for patients with acute liver failure or advanced disease refractory to medical and interventional therapies, but it presents unique surgical challenges due to hepatomegaly, retroperitoneal fibrosis, and potential IVC stenosis or thrombosis.

We report a 33-year-old male with BCS who underwent deceased-donor LT using the piggyback technique. Intraoperatively, the retrohepatic IVC was fibrotic and narrowed; a diamond-shaped side-to-side cavocaval anastomosis was performed to optimize venous outflow. Despite an initially well-perfused graft, the patient developed primary graft nonfunction on the first postoperative day. Urgent retransplantation revealed severe venous outflow obstruction caused by an unrecognized stenosis of the native suprahepatic IVC near the right atrium. The first graft exhibited extensive congestion and coagulative necrosis. Retransplantation was successfully performed using conventional technique without veno-venous bypass, completely removing the recipient's IVC.

This case illustrates the potential for subtle anatomical anomalies, such as suprahepatic IVC stenosis, to cause acute post-transplant hepatic venous outflow obstruction in BCS. Awareness of these "Budd-Chiari pitfalls" is critical for surgical planning and intraoperative decision-making. LT remains a safe and effective therapy for carefully selected BCS patients, provided that meticulous attention is paid to venous anatomy and possible intraoperative challenges.