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Congenital extrahepatic porto systemic shunt (CEPS) is a rare condition in which the porto mesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt. It is commonly associated with multiple congenital anomalies. A diagnosis of Abernethy malformation is made by non-invasive cross-sectional imaging techniques such as Doppler ultrasound, CT or MRI, which show the shunt and intrahepatic portal vein branches. We report a case presenting with psychological issues, systemic hypertension, upper extremity deformities, liver lesions and portal hypertension. Usually the patients are diagnosed in childhood, in our case the patient is a middle aged woman. This case report shows the importance of imaging in diagnosis of this condition and presentation of the patient with portal hypertension which is rare.

Abernethy Malformation and the Spectrum of Imaging Findings: A Case Report