Open AccessClinical manifestations of Ellis-van Creveld syndrome
Author(s) -
C Vinay,
R Sudhakara Reddy,
KS Uloopi,
R. Chandra Sekhar
Publication year - 2009
Publication title -
journal of the indian society of pedodontics and preventive dentistry/journal of indian society of pedodontics and preventive dentistry
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.378
H-Index - 33
eISSN - 1998-3905
pISSN - 0970-4388
DOI - 10.4103/0970-4388.57663
Subject(s) - dental anomalies , polydactyly , autosomal recessive trait , medicine , ectodermal dysplasia , variable expression , dermatology , hypodontia , orthodontics , anatomy , genetics , biology , gene
Ellis-van Creveld syndrome (EVC) is a chondro-ectodermal dysplasia characterized by short ribs, polydactyly, growth retardation and ectodermal and heart defects. It is a rare disease complex and very few cases have been reported in dental literature. This condition is inherited as an autosomal recessive trait with variable expression. The present case report describes EVC in a 7-year-old girl, with all the tetrad of cardinal features. We found a rare dental aberration in form; appearance of single conical roots in primary molars. The management of children with EVC is multidisciplinary, with consideration for the high incidence of cardiac defects in these patients.