Chondroectodermal dysplasia (Ellis-van Creveld syndrome): a case report.
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.
Aminabadi
Naser A
NA
Department of Pediatric Dentistry, School of Dentistry, Tabriz University (Medical Sciences), Tabriz, Iran. aslaminabadi@gmail.com
Ebrahimi
Akram
A
Oskouei
Sina G
SG
D000848:Anodontia / Q000175:diagnosis; D002675:Child, Preschool; D003731:Dental Caries / Q000175:diagnosis; D004613:Ellis-Van Creveld Syndrome / Q000175:diagnosis*; D005260:Female; D005881:Gingiva / Q000002:abnormalities; D006801:Humans; D006984:Hypertrophy; D007742:Labial Frenum / Q000002:abnormalities / Q000473:pathology; D008046:Lip / Q000002:abnormalities