Tense Ascites as a Presentaton of Protein S Defciency in a 9-Year-Old Boy

Ascites is not a usual finding in prehepatic portal hypertension, including portal vein thrombosis, but when portal vein thrombosis is acute and massive, ascites can be a presenting feature. We report a 9-year-old boy with tense ascites and portal and superior mesenteric vein thrombosis. A 9-year-old boy was evaluated for tense ascites which led to umbilical hernia since one month before admission. He did not have any clinical or laboratory stigmata of parenchymal liver disease. Imaging studies showed superior mesenteric and portal vein thrombosis. In laboratory tests for pre-thrombotic states, he suffered from significant protein S deficiency. Thrombophilic states like protein S deficiency predispose patient to vascular thrombosis. This vascular thrombosis can be present with signs and symptoms related to their territories. Prevention of thrombosis and rethrombosis with anticoagulant therapy is recommended.