A Case of Hepatocellular Carcinoma in Abernethy syndrome

Abstract

Abernethy malformation is a rare type of congenital malformation of the hepatic portal system. It can take one of three forms and is classified as an extrahepatic portosystemic shunt between the portal vein and a large systemic vein, usually the inferior vena cava (IVC). Here, we present at 47-year-old male who was admitted with upper abdominal pain. His laboratory investigations showed a raised alkaline phosphatase, alanine aminotransferase and bilirubin but were otherwise within normal range. Ultrasound scan showed an indeterminate mass in the right lobe of the liver which was conformed with a CT scan. An anomalous portal vein drainage system was also identified on CT scan. Following MDT discussion, the decision for surgical exploration was made to assess the resectability and potential functional liver remnant. At exploration, resection was precluded by the complete absence of portal veins with no viable reconstruction option due to the size of the tumour and the potential of inadequate functioning residual liver following resection. Both the splenic vein and SMV were visualised intraoperatively and seen to form a short convergence before draining directly into the IVC. This abnormality is in line with type 1b abernethy malformation. Biopsies were also taken which confirmed a diagnosis of well differentiated HCC. Further treatment options including TACE and TARE have been deemed inappropriate due to the lack of portal vein as well as the size of the tumour. Considering this the patient has been referred for systemic treatment.