Interventions in Budd-Chiari syndrome: an updated review

Budd Chiari syndrome is a potentially treatable disease, and imaging is the key to its diagnosis. Clinical presentations may vary, ranging from asymptomatic to fulminant disease. Subacute BCS is the most common type encountered in clinical practice, characterized by ascites, hepatosplenomegaly, dilated abdominal wall veins, and varicosities in the lower limb and scrotum. While hepatic vein thrombosis is the leading cause in the West, membranous and short segmental occlusion are predominant in the Asian populations. These geographical variations have an impact on the treatment algorithm in managing BCS. Anticoagulation alone often fails to prevent disease progression, demanding further interventional therapy. Interventional therapy carries a lower morbidity and mortality than surgery. Anatomical recanalization and portosystemic shunting form the basis of endovascular management. Membranous or short-segment occlusion are best treated by angioplasty, which restores the physiological venous outflow and possibly disease reversal. Suboptimal results with angioplasty require stenting. Transjugular intrahepatic shunt (TIPS) or direct IVC to portal vein shunt (DIPS) decompresses the portal pressure and reduces the sinusoidal congestion, which in turn diminishes hepatocellular damage and hepatic fibrosis. Despite its ability to modify the disease course, TIPS carries several procedure and shunt-related complications, mainly hepatic encephalopathy. Thus, anatomical recanalization precedes TIPS in the traditional step-up approach in managing BCS. However, this concept is challenged by some authors, necessitating future reseach. TIPS is a valid bridge therapy in BCS with acute live failure awaiting liver transplantation. Despite all, interventional therapies fail in a subset of BCS patients, leaving them with only option of liver transplantation.