Simultaneous retrohepatic inferior vena cavoplasty and side-to-side portacaval shunt for recurrent thrombosed mesoatrial shunt in the Budd-Chiari syndrome

The Budd-Chiari syndrome is an unusual and often fatal form of portal hypertension caused by hepatic vein occlusion. It comprises less than 5% of surgically correctable causes of portal hypertension and can be one of the most difficult to treat. Recurrent Budd-Chiari syndrome associated with a thrombosed mesoatrial shunt can be an even more vexing problem because of the patient's debilitated condition, hypercoagulable state, and altered anatomy from the previous thoracic and abdominal operations. Although the literature describes numerous surgical methods of treating the Budd-Chiari syndrome, no report specifically addresses the reoperative management of a recurrent thrombosed mesoatrial shunt. We report a patient with a recurrent thrombosed mesoatrial shunt, tightly stenotic retrohepatic inferior vena cava, and occluded hepatic veins with severe portal hypertension. A simultaneous inferior vena cavoplasty and a side-to-side portacaval shunt have produced excellent results with 26-month follow-up. To the best of our knowledge, this method has not been previously described. Other reported surgical methods of treating the Budd-Chiari syndrome are discussed, with emphasis on their relative applicability to the reoperative management of this condition. We submit that this one-stage patch cavoplasty and side-to-side portacaval shunt are the best direct surgical methods to provide immediate and long-term benefit for patients with this most challenging problem.