Liver transplantation in Ellis-van Creveld syndrome: a case report.

Abstract

This case represents a rare association of Ellis-van Creveld (EvC) syndrome, a chondroectodermal disorder, with congenital paucity of bile ducts. Sequential liver biopsies during the patient's childhood demonstrated progressive fibrosis that can occur in other chondrodysplastic malformations. However, this EvC case is the first report to demonstrate paucity of intra-hepatic bile ducts progressing to cirrhosis and subsequently requiring transplant.

• Authors: Dalliah Black, Jason Reutter, Mark Johnson, Jeffrey Fair, John Woosley, David Gerber
• Journal: Pediatric transplantation (Pediatr Transplant) Vol. 6 Issue 3 Pg. 255-9 (Jun 2002) ISSN: 1397-3142 [Print] Denmark
• PMID: 12100513 (Publication Type: Case Reports, Journal Article)

Topics

• Ellis-Van Creveld Syndrome (pathology, surgery)
• Humans
• Infant, Newborn
• Liver Transplantation
• Male