Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess.

A 27-day-old boy had convulsion associated with brain abscesses and severe hypoxemia at the age of 3 months. Congenital absence of the portal vein (CAPV) and some associated anomalies were detected by radiological examinations. Brain abscess and hypoxemia were thought to be serious complications resulting from CAPV and were successfully corrected by living donor liver transplantation at the age of 4 months. This is the first report of a successful transplantation indicated for intrapulmonary shunt and brain abscess in an infant with CAPV.