Related Experiment Videos
Surgical and radiologic treatment of primary Budd-Chiari syndrome
A Pisani-Ceretti1, M Intra, F Prestipino
1Second Divisione di Chirurgia Generale, Ospedale Fatebenefratelli e Oftalmico, Milan, Italy.
Insights
Budd-Chiari syndrome (BCS) treatment varies by disease stage. Portasystemic shunts are effective for acute/subacute forms, while liver transplantation suits advanced disease. Interventional radiology aids poor-risk patients.
Area of Science:
- Hepatology
- Vascular Surgery
- Interventional Radiology
Background:
- Budd-Chiari syndrome (BCS) is a rare condition causing portal hypertension due to hepatic venous outflow obstruction.
- Etiologies include polycythemia vera, oral contraceptives, membranes, and sepsis, though often idiopathic.
- The study reviews treatment strategies for primary BCS over a 28-year period.
Purpose of the Study:
- To evaluate treatment outcomes for primary Budd-Chiari syndrome.
- To compare the efficacy of different therapeutic approaches based on disease stage.
Main Methods:
- Retrospective analysis of 19 primary BCS patients treated between 1969 and 1997.
- Interventions included transjugular/transhepatic stenting, side-to-side portacaval shunts (with grafts), and transfemoral caval stenting.
- Patient outcomes and survival rates were tracked.
Main Results:
- 18 of 19 patients survived the perioperative period.
- The 5-year survival rate was 83% with a mean follow-up of 66.7 months.
- Treatment success varied by BCS stage, with shunts effective for reversible injury and transplantation for irreversible damage.
Conclusions:
- Primary BCS management is stage-dependent.
- Portasystemic shunts are effective for acute/subacute BCS with reversible liver injury.
- Orthotopic liver transplantation is indicated for fulminant/chronic forms with irreversible hepatic damage; caval stenting may precede shunting if IVC obstruction is present.
Abstract:
Budd-Chiari syndrome (BCS) is an uncommon form of portal hypertension caused by obstruction of the hepatic venous outflow. From 1969 to 1997 we treated 19 patients (7 men, 12 women; mean age 37.6 years) affected by primary BCS. In most of the cases no etiologic factors were identified; in the remaining cases the etiology was associated with 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 via a transjugular or transhepatic approach. The remaining 16 patients underwent a side-to-side portacaval shunt, which required interposition of a graft in five cases. In two patients with a significant caval obstruction, 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 patient 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 BCS requires different therapies depending on the stage of the disease. The fulminant or chronic forms with irreversible hepatic damage require definitive treatment, such as orthotopic liver transplantation. For the acute or subacute forms, characterized by reversible hepatic injury, a portasystemic shunt represents the most effective treatment. The patients 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.