Budd–Chiari syndrome: a focussed and collaborative approach

Excerpt

Budd–Chiari syndrome (BCS) is a rare disorder caused by obstruction of the hepatic venous outflow tract at any level between the small hepatic veins and the right atrium, hence also known as hepatic venous outflow tract obstruction (HVOTO) (Fig. 1a–c). This obstruction leads to venous stasis resulting in congestive hepatopathy [1]. This congestive hepatopathy results in increased sinusoidal pressure with hepatic sinusoidal thrombosis as evidenced by fibrin deposition within sinusoids [2]. This sinusoidal thrombosis in turn causes reduced hepatic perfusion leading to ischemia and necrosis of hepatocytes especially in perivenular zones leading to fibrosis and portal hypertension [1, 2].

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