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Living donor liver transplantation for congenital absence of the portal vein with situs inversus

Received March 10, 2009; accepted May 17, 2009.Congenital absence of the portal vein (CAPV) is a raredisorder that may lead to the development of hepaticneoplasms, hepatic encephalopathy, pulmonary hyper-tension (PH), and hepatopulmonary syndrome (HPS).The portal vein develops by selective involution of thevitelline venous system, and associated abnormalitiesmay result in CAPV. Some patients with CAPV are di-agnosed at the time of neonatal screening for hyper-galactosemia.