Cruveilhier-Baumgarten disease in a patient with Turner syndrome: case report of a rare indication for liver transplantation

Liver Transpl. 2008 Mar;14(3):299-302. doi: 10.1002/lt.21374.

Abstract

Some chromosomal alterations can be associated with vascular abnormalities. For instance, Turner syndrome can be complicated by agenesis or hypoplasia of the portal venous system causing presinusoidal portal hypertension. Liver transplantation to treat this condition overcomes portal hypertension and reconstitutes the diminished hepatic function due to severe atrophy of the portal venous inflow.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Humans
  • Hypertension, Portal / complications
  • Hypertension, Portal / diagnosis
  • Hypertension, Portal / etiology
  • Hypertension, Portal / surgery*
  • Liver / blood supply
  • Liver Circulation
  • Liver Transplantation*
  • Middle Aged
  • Turner Syndrome / complications
  • Turner Syndrome / diagnosis
  • Turner Syndrome / surgery*