Cruveilhier-Baumgarten disease in a patient with Turner syndrome: case report of a rare indication for liver transplantation.
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.
Aucejo
Federico
F
Department of General Surgery, Cleveland Clinic, Cleveland, OH 44195, USA. faucejo@hotmail.com
Ibrahim
Zuhaib
Z
Hashimoto
Koji
K
Quintini
Cristiano
C
Kelly
Dympna
D
Vogt
David
D
Eghtesad
Bijan
B
Fung
John
J
Miller
Charles
C
Tuthill
Ralph
R
D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D008099:Liver / Q000098:blood supply; D008102:Liver Circulation; D016031:Liver Transplantation; D008875:Middle Aged; D014424:Turner Syndrome / Q000150:complications / Q000175:diagnosis / Q000601:surgery*