Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney

• Published in: European journal of pediatrics Vol. 157; no. 4; pp. 287 - 290
• Main Authors: GUARISO, G, FIORIO, S, ALTAVILLA, G, GAMBA, P. G, TOFFOLUTTI, T, CHIESURA-CORONA, M, TEDESCHI, U, ZANCAN, L
• Format: Journal Article
• Language: English
• Published: Heidelberg Springer 01-04-1998; Berlin Springer Nature B.V
• Subjects: Biological and medical sciences; Cell Division - physiology; Child; Female; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Hyperplasia; Liver - abnormalities; Liver - pathology; Liver Regeneration - physiology; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Malformations; Medical sciences; Polycystic Kidney Diseases - congenital; Polycystic Kidney Diseases - pathology; Portal Vein - abnormalities; Portal Vein - pathology; Human; Kidney disease; Agenesis; Urinary system disease; Congenital; Polycystic kidney; Liver; Hyperplasia; Hepatic disease; Genetic disease; Case study; Cyst; Digestive diseases; Benign neoplasm; Portal vein; Child
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/s004310050812

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.