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, 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.
First Department of Surgery, Ryukyu University School of Medicine, Nishihara-cho, Okinawa 903-0215, Japan.
T
T
Kinjo
H
H
Aoki
H
H
Sunagawa
S
S
Kinjo
Y
Y
Muto
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D000792:Angiography; D001707:Biopsy, Needle; D002675:Child, Preschool; D015529:Choledochal Cyst / Q000175:diagnosis*; D005260:Female; D020518:Focal Nodular Hyperplasia / Q000175:diagnosis* / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000473:pathology; D011169:Portal Vein / Q000002:abnormalities* / Q000196:embryology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome