Il trattamento chirurgico nella sindrome di Budd-Chiari primitiva : una terapia in evoluzione

Abstract

Background. Budd-Chiari syndrome (BCS) is an uncommon form of portal hypertension caused by obstruction of the hepatic venous outflow. Methods. From 1969 to 1997, 19 patients (7 men and 12 women, with a mean age of 37.6 years) affected with primary BCS were treated. In most of the cases no etiologic factors were identified; in the remaining cases the etiology was associated to polycythemia vera, use of oral contraceptives, presence of endoluminal membranes and repeated episodes of sepsis. Three patients with membranous occlusion of the major hepatic veins were treated by percutaneous placement of a self-expanding metallic stent, inserted by means of a transjugular or transhepatic approach. The remaining 16 patients underwent a side-to-side porto-caval shunt, which required the interposition of a graft in 5 cases. In 2 patients with a significant caval obstruction a metallic vascular stent was placed into the narrowed tract of inferior vena cava, before shunting, by means of a transfemoral venous approach. Results. One patient died within the first 30 postoperative days. The 18 survivors were followed for a mean of 66.7 months. The 5-year survival rate was 83%. Conclusions. Primary BCS requires different therapies depending on the stage of disease. The fulminant or chronic forms with irreversible hepatic damage, need a definitive treatment, such as orthotopic liver transplantation. On the contrary, in the acute or subacute forms, characterized by reversible hepatic injury, the porto-systemic shunt represents the most effective treatment. The patients with bad hepatic risk can be treated by using the new procedures of interventional radiology. In both cases preliminary caval stenting is necessary if the syndrome is complicated by a significant obstruction of the inferior vena cava