Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare anatomic vascular malformation. Patients with Abernethy malformation may present with abdominal pain, abnormal liver function tests, hepatopulmonary syndrome, pulmonary hypertension, and/or portosystemic encephalopathy. Accurate identification of the shunt and portal vein and effective management of complications is vital in these patients. Routine imaging examinations are useful for the diagnosis and classification of Abernethy malformation. However, these examinations may miss some portal vein branches. Digital subtraction angiography is invaluable when routine imaging cannot precisely identify the hepatic portal vein in this situation.
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