Surgical Ligation for the Treatment of an Unusual Presentation of Type II Abernethy Malformation.

Abernethy malformation is a very rare congenital vascular malformation consisting of diversion of portal blood away from liver, and it is commonly associated with multiple congenital anomalies. Here, we present a case of a male from China with nonspecific abdominal pain associated with an unusual pattern of type II Abernethy malformation, whose was diagnosed with a portosystemic shunt via a giant portal-inferior vena cava fistula (17.22 mm in diameter). The patient underwent a surgical ligation of the portocaval shunt and recovered well. We believe that this is the first case of a type II Abernethy malformation presenting as a portosystemic shunt via the giant portal-inferior vena cava fistula.