Portal hypertension due to portal cavernoma caused by protein S deficiency in a young infant

Abstract

The portal cavernoma is the most common cause of extrahepatic portal hypertension. The risk factors in the neonatal period are dominated by sepsis and umbilical venous catheterization. The inherited thrombophilias have been studied in children and adolescents in a few series in the literature. We report an infant who develop after neonatal severe perinatal asphyxia and sepsis at the age of three months an array of portal hypertension in relation to a portal cavernoma. The thrombophilia detects severe deficiency of protein S.