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  • Title: Chondroectodermal dysplasia (Ellis-van Creveld syndrome): a case report.
    Author: Aminabadi NA, Ebrahimi A, Oskouei SG.
    Journal: J Oral Sci; 2010 Jun; 52(2):333-6. PubMed ID: 20587962.
    Abstract:
    Ellis-van Creveld (EVC) syndrome is a form of skeletal and chondroectodermal dysplasia, occurring with and without systemic involvement. Taurodontism of permanent and primary molars and upper posterior supernumerary teeth are rarely associated with this syndrome. A 5-year-old girl presented with early childhood caries and hypodontia. She had labiogingival adhesion, labiogingival frenulum hypertrophy, accessory labiogingival frenula, and a serrated appearance of the gingiva. She was also short in stature. All major features of chondroectodermal dysplasia were present. EVC syndrome requires multidisciplinary therapeutic planning, and the dentist plays a fundamental role in management of the oral and dental manifestations.
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