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. Liver Transpl 14:299–302, 2008. © 2008 AASLD.