Abstract
Congenital absence of portal vein (CAPV) with complete diversion of portal blood into the vena cava, also referred as Abernethy malformation type 1, is a rare malformation of the mesenteric vasculature in which visceral venous blood bypasses the liver, completely draining into systemic circulation through a congenital porto-systemic shunt. This condition is often complicated by hepatic encephalopathy, hepatopulmonary syndrome or hepatic tumours; however the clinical features and the long term prognosis of this disorder are poorly known.
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