[Odontochondrodysplasia].

BACKGROUND

Dentinogenesis imperfecta is exceptionally associated to chondrodysplasia. The aim of this work is to present four cases of such an association.

CASE REPORT: These four children (three boys; one girl) suffered from growth retardation, ligamentous hyperlaxity, scoliosis. Main features were present since the first months of life. Dentinogenesis imperfecta was more marked on the first teeth. On X-rays, all patients had short tubular bones, more pronounced at the level of the middle segment of their limbs, with irregular metaphyses. Cone-shaped epiphyses were present on the hands. Iliac wings were square-shaped and vertebral bodies had a posterior wedging.

CONCLUSION

These four cases, including two previously published as Goldblatt's syndrome, share the same findings as another case described by this author. We propose the name of odontochondrodysplasia for this apparently unfortuitous association: dentinogenesis imperfecta and chondrodysplasia.