Congenital absence of the portal vein associated with congenital hepatic fibrosis

Gocmen R. , Akhan O. , Talim B.

PEDIATRIC RADIOLOGY, vol.37, no.9, pp.920-924, 2007 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 37 Issue: 9
  • Publication Date: 2007
  • Doi Number: 10.1007/s00247-007-0533-y
  • Title of Journal : PEDIATRIC RADIOLOGY
  • Page Numbers: pp.920-924


The radiological features of a 7-year-old boy with congenital absence of the portal vein, pathologically proven congenital hepatic fibrosis, double inferior vena cava, ventricular septal defect, vertebral anomalies, crossed fused renal ectopia, and facial anomalies with pathological correlation are reported. This association between congenital absence of the portal vein and congenital hepatic fibrosis is unique.