HighlightsAbernethy malformation is a rare congenital abnormality characterized by an extrahepatic portosystemic shunt.Doppler ultrasonography is usually the initial investigation.Computerized tomography (CT) scan or magnetic resonance imaging (MRI) scan is helpful in confirming the diagnosis.Preoperative angiography with balloon occlusion test is highly recommended to determine the most appropriate intervention.We propose guidelines for approaching and management this condition.
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