Orthotopic liver transplantation for portosystemic encephalopathy in an adult with congenital absence of the portal vein
Maciej Wojcicki, Elizabeth B. Haagsma, Annette S.H. Gouw, Maarten J.H. Slooff, Robert J. Porte – 30 August 2004 – Congenital absence of the portal vein (CAPV) is a very rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. There is no portal perfusion of the liver and no portal hypertension. This abnormality is usually coincidentally discovered in children, the majority of whom have no signs of encephalopathy and only slightly abnormal liver function tests.