Budd Chiari Syndrome: Presentation, Management, and Prognosis

Budd Chiari syndrome (BCS) is a rare condition characterized by hepatic vein obstruction causing liver congestion, dysfunction and rarely liver failure. BCS may present as acute, subacute, or chronic liver disease. It usually presents at a younger age as compared to other splanchnic thrombotic conditions. Prothrombotic conditions, myeloproliferative disorders, and oral contraceptives use are common risk factors. Additionally, lower socioeconomic status and malnutrition are associated with BCS. Many prognostic scores are available and they are useful for general guidance. A stepwise treatment strategy is recommended for the management of BCS, starting with anticoagulation followed by portosystemic shunting and liver transplantation for most patients with BCS, but such a strategy may not be applicable in those who present with acute liver failure. Post liver-transplant survival outcomes in BCS, including in those who present with acute liver failure and multiple organ failures, are comparable to other indications. Pregnancy is not contraindicated in those with BCS, and maternal outcomes are favorable, but fetal outcomes remain poor. Most patients with BCS, including those who had liver transplantation, require lifelong anticoagulation. There are limited data on chronic BCS and these patients are often diagnosed with complications of postal hypertension or liver cancer. In this review, we discuss our current understanding of etiologies, diagnostic options and prognostic scores, natural history including pregnancy outcomes, and treatment outcomes of BCS.