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) o...

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Bibliographic Details
Published in:Journal of pediatric surgery 2001-04, Vol.36 (4), p.622-625
Main Authors: Kinjo, Tsukasa, Aoki, Hiromitsu, Sunagawa, Hiroki, Kinjo, Saneo, Muto, Yoshihiro
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - diagnosis
Angiography
anomalies
Biological and medical sciences
Biopsy, Needle
Blood and lymphatic vessels
Cardiology. Vascular system
Child, Preschool
Choledochal Cyst - diagnosis
congenital choledochal cyst
Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous
Female
focal nodular hyperplasia
Focal Nodular Hyperplasia - diagnosis
Focal Nodular Hyperplasia - surgery
Follow-Up Studies
Gastroenterology. Liver. Pancreas. Abdomen
Humans
Liver - pathology
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Medical sciences
Portal vein
Portal Vein - abnormalities
Portal Vein - embryology
Tomography, X-Ray Computed
Treatment Outcome
Tumors
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Description
Summary: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. Copyright © 2001 by W.B. Saunders Company.
ISSN:0022-3468
1531-5037
DOI:10.1053/jpsu.2001.22303