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 malfor mations in 12. This case report described a girl with congenital absen ce of portal vein, focal nodular hyperplasia of the liver and cystic k idney dysplasia. Angiography showed the splenic vein and superior mese nteric vein joining to form a common trunk that entered the inferior v ena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are dis cussed.