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Abernathy syndrome, widely calledCongenital extra-hepatic portosystemic Shunt (CPES) is a rare condition with a prevalence of 1 in 30000 -50000 cases, where the portal venous system is diverted away from the liver into the systemic veins.
It is classified into (Type I) with absent portal vein in which there is no perfusion of liver by the portal vein and (Type II) in which few thin radicles of portal vein are present and portal blood gets partially shunted to the inferior vena cava.
Early diagnosis helps in preventing the harmful effects of bypassing of liver. Initially, patients present with symptoms note related to liver such as chronic/ recurrent abdominal pain and fatigue.
In later course of disease hepatic parenchymal abnormalities are seen including nodular liver lesions or tumors such as hepatoblastoma/ hepatocellular carcinoma and life- threatening conditions such as hepatopulmonary syndrome, hepatic encephalopathy.