Chronic portal vein thrombosis due to combined deficiency of protein C and protein S

Portal vein thrombosis (PVT) is a rare disorder that is associated with a variety of underlying condition of which liver cirrhosis, malignancy and myeloproliferative disorders are the most common. It is of two types, acute and chronic portal vein thrombosis. Anticoagulation therapy is recommended for all patients with acute portal vein thrombosis. Chronic portal vein thrombosis is characterised by the development o f portal hypertension. Bleeding from ruptured varices is the main complication. In the absence of bleeding, continuous anticoagulation therapy should be considered for chronic portal vein thrombosis in whom an underlying prothrombotic factor is to be identified. Here in this report a 13-year-old girl presented with haematemesis. The spleen was hugely enlarged. Her Hb was 8.38 g/dl. Grade III oesophageal varices were found in oesophagogastroduodenostomy. CT abdomen showed portal cavernoma formation with increased splenic collateral. Protein C activity was 45% and protein S activity was 40%. She was treated with beta-blocker, endoscopic variceal ligation followed by low molecular weight heparin and warfarin.