The Ellis-van Creveld (EVC) syndrome is a chondroectodermal dysplasia and is characterized by the cardinal features of disproportionate short stature, polydactyly, hidrotic ectodermal dysplasia, and congenital heart malformations, along with other skeletal and dental abnormalities.
Oral manifestations of chondroectodermal dysplasia (Ellis-Van Creveld Syndrome).
It may also be associated with syndromes such as achondroplasia and chondroectodermal
Double teeth may also be part of syndromes such as achondrodysplasia and chondroectodermal
EVC syndrome or Chondroectodermal
dysplasia is a condition in which cartilage growth is affected causing stunted growth.
When present in Caucasians, post-axial polydactyly is associated with an autosomal recessive transmission and syndromic conditions such as Ellis-van Creveld and chondroectodermal
Increased first-trimester fetal nuchal translucency thickness in association with chondroectodermal
dysplasia (Ellis-van Creveld syndrome).
Differential considerations for this condition include multiple exostosis, enchondromatosis, multiple epiphyseal dysplasia, Turner's syndrome without LWD, achondroplasia, chondroectodermal
dysplasia, and hypoparathyroidism.
Syndromes commonly associated with double teeth are Wolf--Hirschhorn syndrome, achondroplasia, focal dermal hypoplasia, osteopetrosis, and chondroectodermal