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J Pediatr Surg. 2001 Apr;36(4):622-5.

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

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First Department of Surgery, Ryukyu University School of Medicine, Nishihara-cho, Okinawa 903-0215, Japan.


A congenital absence of the portal vein (CAPV) is a rare malformation, which almost is always associated with other anomalies such as hepatic tumors and cardiac malformations. This case report describes a 3-year-old girl with a congenital absence of the portal vein, focal nodular hyperplasia (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. J Pediatr Surg 36:622-625.

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