Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst: A case report

A congenital absence of the portal vein (CAPV) is a rare malformation, whic h almost is always associated with other anomalies such as hepatic tumors a nd cardiac malformations. This case report describes a 3-year-old girl with a congenital absence of the portal vein, focal nodular hyper plasia (FNH) of the liver, and a congenital choledochal cyst (CCC). Angiography findings showed the mesenteric vein and splenic vein to be joined together to form a common trunk that entered the inferior vena cava directly above the liver . This is the first known reported case of CAPV with concurrent CCC. Copyri ght (C) 2001 by W.B. Saunders Company.