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Liver transplantation in Ellis–van Creveld syndrome: A case report
Author(s) -
Black Dalliah,
Reutter Jason,
Johnson Mark,
Fair Jeffrey,
Woosley John,
Gerber David
Publication year - 2002
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1034/j.1399-3046.2002.01085.x
Subject(s) - medicine , liver transplantation , cirrhosis , congenital hepatic fibrosis , transplantation , congenital disorder , pathology , gastroenterology , surgery , portal hypertension
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.