Abstract

Portal vein thrombosis (PVT) is an important cause of extrahepatic portal hypertension in children. A majority of children with PVT of unknown etiology have functional protein C deficiency or abnormally elevated levels of anti-cardiolipin antibodies. We present an 8-year-old boy with portal hypertension with protein C deficiency, who underwent mesocaval shunt surgery due to recurrent episodes of bleeding esophageal varices and subsequently developed a block in the mesocaval shunt due to a thrombotic tendency.

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