Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney.
Congenital absence of portal vein is a rare malformation. To date, 16 cases have been reported--all in association with other anomalies, i.e. benign or malignant hepatic neoplasms in 6 cases and cardiac malformations in 12. This case report described a girl with congenital absence of portal vein, focal nodular hyperplasia of the liver and cystic kidney dysplasia. Angiography showed the splenic vein and superior mesenteric vein joining to form a common trunk that entered the inferior vena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are discussed.
Guariso
G
G
Department of Pediatrics, University of Padova, Italy.
Fiorio
S
S
Altavilla
G
G
Gamba
P G
PG
Toffolutti
T
T
Chiesura-Corona
M
M
Tedeschi
U
U
Zancan
L
L
D002455:Cell Division / Q000502:physiology; D002648:Child; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008099:Liver / Q000002:abnormalities* / Q000473:pathology; D008115:Liver Regeneration / Q000502:physiology; D007690:Polycystic Kidney Diseases / Q000151:congenital* / Q000473:pathology; D011169:Portal Vein / Q000002:abnormalities* / Q000473:pathology