Aggressive management and liver transplantation in Budd-Chiari syndrome secondary to Behçet's disease

Abstract

Budd-Chiari Syndrome (BCS) is a rare vascular disorder caused by hepatic venous outflow obstruction, often due to thrombosis, leading to liver congestion and portal hypertension. Behçet's Disease (BD), a chronic vasculitis, can cause BCS through inflammation-induced thrombosis. We report a 14-year-old male with BD who developed BCS. He initially presented with foot pain, uveitis, and a bilateral rash, later progressing to abdominal distension, jaundice, and hepatic dysfunction. Investigations revealed elevated transaminases and hyperbilirubinemia, with imaging confirming hepatic vein thrombosis. Management included corticosteroids, immunosuppressants, and anticoagulation. Despite treatment, liver function deteriorated, necessitating a transplant, after which he stabilized with significant symptomatic improvement. This case underscores the need for early recognition of BD-related vascular complications, timely intervention to prevent irreversible liver damage, and consideration of liver transplantation in severe BCS. Increased awareness of BD as a potential cause of BCS is crucial for prompt diagnosis and management.