ABERNETHY MALFORMATION PRESENTING WITH LIVER MASS : A RARE PRESENTATION OF A RARE ENTITY

Abstract

Abernethy malformation is a very rare congenital extrahepatic porto-systemic shunt (CEPS) that develops between portomesenteric vasculature and systemic veins (IVC / left renal vein / left iliac vein / left hepatic vein) and thus bypassing the liver and resulting in deranged metabolism of various metabolites. They have clinical features varying from that of hypergalactosemia to hyperbilirubinemia to hyperammonemia and pulmonary veins congestion, hepatopulmonary syndrome, hepatic encephalopathy. Another presentation in the spectrum is patient presenting with a hepatic mass lesion, which form as a liver's response to absent/reduced portal ow leading to regenerative nodular hyperplasia. These nodules with time can progress into hepatic tumors like adenoma ,focal nodular hyperplasia(FNH), hepatoblastoma and even hepatocellular carcinoma(HCC). Ultrasound (USG) is a useful tool for screening of congenital anomalies associated with the liver. Diagnosis of abernethy malformation requires a high index of suspicion and can easily be overlooked if not carefully evaluated. In the majority of cases, the complications and other secondary features often lead to diagnosis of abernethy malformation. Also,we should emphasize the search for an underlying abernethy malformation on the background of hepatic mass(as in our case). We report a case of abernethy malformation presenting with liver mass on screening USG and conrmed on contrast enhanced computed tomography (CECT).