Life-threatening esophageal variceal bleeding due to proteins C, S deficiency requiring esophagogastric devascularization: A case report

Proteins C and S are important for the prevention of intravascular blood clotting, and patients with deficiency of these proteins have a higher risk of developing diffuse venous thrombosis. However, the onset is usually in childhood and the thrombosis is rare in portal system. Here we report an adult with this rare disease causing life-threatening esophageal variceal bleeding due to thrombosis of the portal venous system. For treatment of such bleeding which has failed in response to pharmacological treatment and endoscopic sclerotherapy, esophagogastric devascularization, gastric stapling and splenectomy are required.