Budd-Chiari Syndrome Due to Protein C Deficiency: A Case Report

Budd-Chiari Syndrome (BCS), a rare vascular disorder of the liver characterized by obstruction of hepatic venous outflow, has an estimated prevalence of approximately 1 in 100,000 in the general population. Among its various etiologies, coagulation factor deficiencies are uncommon. This case, following Case Report (CARE) guidelines, describes a case of BCS associated with protein C deficiency resulting in a thrombus in the inferior vena cava. The patient was an unmarried Indian woman in her 40s who presented with progressive pedal edema and abdominal distension for 6 months. She was evaluated thoroughly, and other causes were excluded. Imaging showed classical features of BCS, including complete fibrotic occlusion of all three hepatic veins, intrahepatic venous and splenorenal collaterals, caudate lobe enlargement, and a 5.2 cm thrombus in the inferior vena cava. She was treated with interventional angioplasty along with anticoagulation and diuretic therapy. She is under regular follow-up and has been counseled for liver transplantation for liver cirrhosis. This case highlights the rare association of protein C deficiency with BCS and emphasizes the need for early recognition, multidisciplinary management, and supportive nursing care to improve outcomes and quality of life.