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
Differential considerations for this condition include multiple exostosis, enchondromatosis, multiple epiphyseal dysplasia, Turner's syndrome without LWD, achondroplasia, chondroectodermal
dysplasia, and hypoparathyroidism.
Ellis Van Creveld syndrome (chondroectodermal
dysplasia),9 cyclopia, pachyonychia congenita, cleft lip and palate, Down's syndrome, Pierre-Robin, Pallister-Hall, short rib-polydactyly type II, Wiedeman-Rautenstrauch (neonatal progeria).10,5
Syndromes commonly associated with double teeth are Wolf--Hirschhorn syndrome, achondroplasia, focal dermal hypoplasia, osteopetrosis, and chondroectodermal
Further some systemic conditions or syndromes associated with such teeth are;--Hallerman-streiff syndrome (occlusomandibulodyscephaly with hypotrichosis), Ellis-Van creveld syndrome (chondroectodermal
dysplasis), Craniofacial dysostosis syndrome, Multiple steacystoma, Cleft palate, Pierre Robin syndrome, Adrenogenital syndrome, Epidermolysis bullosa simples etc are reported .
Although multiple supernumerary teeth, in most cases, are associated with different syndromes, such as cleidocranial dysplasia, familial adenomatous polyposis, chondroectodermal
dysplasia, trichorhinophalangeal syndrome, and Robinow syndrome (24-27), multiple supernumerary teeth without an accompanying syndrome were reported in previous studies (14, 22).
It is also known as chondroectodermal
dysplasia and mesoectodermal dysplasia; dysplasia is an abnormality in form or development .
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.
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