Abernethy Malformation – Congenital Extra-hepatic Portosystemic Shunt Associated with Multiple Liver Adenomatosis: Case Report

In this article we report a case of Abernethy malformation, also known as congenital extrahepatic portosystemic shunt (CEPS). It is a rare vascular malformation in which the portal vein drains into a systemic vein, diverting it from its normal path to the liver. The clinical presentation is variable and most commonly the shunt is diagnosed during the propaedeutic for investigation of the symptoms. Discussion and diagnosis: R.B.S.R, 21 years old, male, with history of liver disease and previous diagnosis of multiple liver adenomatosis from 9 years of age, was hospitalized for pre-transplant liver evaluation. In the medical history, he presented delayed neuropsychomotor development, congenital scoliosis, neurogenic bladder and single kidney, and had also undergone two surgeries. His initial laboratory tests showed increased transaminases and canalicular enzymes. Ultrasonography, radiography, computed tomography (CT) and magnetic resonance imaging (MRI) were performed, which enabled us to identify and confirm important points for diagnosis. Conclusions: It is important that the radiologist recognize the findings early. CT and MRI are fundamental in the management of the syndrome, since they provide the information for diagnosis, planning, intervention and follow-up, as well as the identification of complications.