Chondroectodermal dysplasia (Ellis-van Creveld syndrome): a case report.

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.

• Authors: Naser A Aminabadi, Akram Ebrahimi, Sina G Oskouei
• Journal: Journal of oral science (J Oral Sci) Vol. 52 Issue 2 Pg. 333-6 (Jun 2010) ISSN: 1880-4926 [Electronic] Japan
• PMID: 20587962 (Publication Type: Case Reports, Journal Article)

Topics

• Anodontia (diagnosis)
• Child, Preschool
• Dental Caries (diagnosis)
• Ellis-Van Creveld Syndrome (diagnosis)
• Female
• Gingiva (abnormalities)
• Humans
• Hypertrophy
• Labial Frenum (abnormalities, pathology)
• Lip (abnormalities)