Abstract

Inferior vena cava (IVC) thrombosis at its hepatic portion (also known as obliterative hepatocavopathy [OH]), in the absence of systemic or local diseases such as vasculitis, coagulopathy, infection and malignancy, is a rare event. We report the case of a 25-year-old woman with progressive abdominal pain and leg edema after exercise. Imaging showed congestive liver and IVC occlusion at the intrahepatic portion. A liver biopsy demonstrated portal congestion without evidence of fibrosis; after unsuccessful percutaneous attempts for recanalization, consideration was given to liver transplantation with IVC reconstruction versus IVC bypass. Due to the presence of preserved liver function, an externally supported 16-mm ringed polytetrafluoroethylene graft was used to bypass from the suprarenal IVC to the suprahepatic IVC. At five years, she remains symptom-free, with normal liver function and a patent graft on systemic anticoagulation. This report highlights the successful surgical management of a patient with OH with a thick membrane. It supports other published proposals that this entity differs significantly from classic Budd-Chiari syndrome with thrombosis that affects only the hepatic veins and, thus, OH should be approached and managed differently.

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