@entity3181 ( @entity3181 ) is an uncommon form of portal @entity101 caused by @entity3181 . From 1969 to 1997 we treated 19 @entity1 (7 @entity1 , 12 @entity1 ; mean age 37.6 years) affected by primary @entity3181 . In most of the cases no etiologic factors were identified; in the remaining cases the etiology was associated with @entity6582 vera, use of oral contraceptives, presence of endoluminal membranes, and repeated episodes of @entity295 . Three @entity1 with membranous occlusion of the major hepatic veins were treated by percutaneous placement of a self-expanding metallic stent inserted via a transjugular or transhepatic approach. The remaining 16 @entity1 underwent a side-to-side portacaval shunt, which required interposition of a graft in five cases. In two @entity1 with a significant @entity3885 , a metallic vascular stent was placed in the narrowed tract of the inferior vena cava, before shunting, by means of a transfemoral venous approach. One @entity1 died within the first 30 postoperative days. The 18 survivors were followed for a mean of 66.7 months. The 5-year survival was 83%. Primary @entity3181 requires different therapies depending on the stage of the disease. The fulminant or chronic forms with irreversible @entity99 require definitive treatment, such as orthotopic liver transplantation. For the acute or subacute forms, characterized by reversible @entity99 , a portasystemic shunt represents the most effective treatment. The @entity1 at poor hepatic risk can be treated by interventional radiology. In both cases preliminary caval stenting is necessary if the syndrome is complicated by significant obstruction of the inferior vena cava.

Surgical and radiologic treatment of primary XXXX .

@entity1 :: ('9606', 'Species') :: ['women', 'patients', 'patient', 'men'] @entity6582 :: ('MESH:D011086', 'Disease') :: ['polycythemia'] @entity295 :: ('MESH:D018805', 'Disease') :: ['sepsis'] @entity101 :: ('MESH:D006973', 'Disease') :: ['hypertension'] @entity3885 :: ('MESH:D008173', 'Disease') :: ['caval obstruction'] @entity3181 :: ('MESH:D006502', 'Disease') :: ['obstruction of the hepatic venous outflow', 'Budd-Chiari syndrome', 'BCS'] @entity99 :: ('MESH:D056486', 'Disease') :: ['hepatic damage', 'hepatic injury']
@entity3181 :: (MESH:D006502,Disease) :: ['Budd-Chiari syndrome']
