Budd-Chiari Syndrome (BCS) is a rare disease with an annual incidence of 0.1 to 10 per million people caused by the impaired venous outflow from the liver, primarily through the hepatic veins and inferior vena cava. BCS can be classified based on its etiology (primary or secondary), clinical course (acute, chronic, acute or chronic lesion), and morphology. Hypercoagulable conditions, myeloproliferative diseases, anatomical variability of the inferior vena cava, and environmental conditions are all etiological factors. Survival rates in treated patients range from 42 to 100%, depending on the etiology and presence of risk factors such as Child-Pugh score parameters, sodium and creatinine plasma levels, and treatment choice. Without treatment, 90% of patients die within three years, primarily from complications of liver cirrhosis. The recommended therapeutic approach for BCS is based on a stepwise algorithm that includes medical attention, interventional therapies to reinstate vessel stability (angioplasty, stenting, and local thrombolysis), Trans Jugular Porto Systemic shunt (TJPS) placement, and orthotropic liver transplantation as a last resort rescue treatment.
Published Date: 2022-05-27; Received Date: 2022-04-26