Congenital absence of the portal vein.

Absence of the terminal portion of the portal vein and its intrahepatic branches was demonstrated in a 22-year-old woman with focal nodular hyperplasia (FNH) of the liver. Various imaging studies and angiography showed that the portal vein entered into the inferior vena cava just above the renal veins. The clinical and radiological features of this patient and nine previously reported cases of this entity are herein reviewed.