The first report of living donor liver transplantation for abernethy malformation (congenital absence of the portal vein) in Vietnam

Abstract

Abernethy malformation, also known as Congenital absence of the portal vein (CAPV), is a very rare malformation. There are some reports of liver transplantation as a treatment for CAPV, and our report is the first case in Vietnam. A 10-year-old boy, diagnosed with Abernethy type 1b malformation (portal vein draining directly into the inferior vena cava) and liver tumor. Living donor liver transplantation was performed using a right liver graft from his mother. Histopathological examination of the native liver showed a huge mass lesions within the right liver and absence of portal vein in portal space. Postoperatively, the patient attained optimal liver function and at a 13-month follow-up has returned to normal life. Keywords: Congenital absence of the portal vein, Abernethy malformation, Liver transplantation