Primary Budd-Chiari syndrome due to lupus anticoagulant positive antiphospholipid syndrome

Budd-Chiari syndrome (BCS) is an uncommon but potentially life-threatening vaso-occlusive disorder of the hepatic venous outflow tract. In children, primary BCS is often associated with thrombotic abnormalities and may present with non-specific symptoms such as abdominal pain, ascites, jaundice and vomiting. A delayed diagnosis can lead to severe complications, including liver cirrhosis and portal hypertension. Imaging modalities such as Doppler ultrasonography, CT, MRI and liver elastography play a pivotal role in early detection. We present the case of an adolescent male diagnosed with primary BCS secondary to lupus anticoagulant-positive antiphospholipid syndrome. The condition was successfully managed initially with inferior vena cava angioplasty with stenting and later with transjugular intrahepatic portosystemic shunt procedure, along with anticoagulation therapy. This case highlights the importance of timely recognition, multidisciplinary management and long-term follow-up in improving clinical outcomes.