Concomitant regional odontodysplasia and hydrocephalus

The case of a patient with concomitant regional odontodysplasia, hydrocephalus, and mental retardation is presented. Tooth eruption was retarded, probably because of the presence of a cementum-like substance on the enamel surface as well as the presence of discontinuous odontogenic epithelium that surrounded the affected teeth. Neural damage during intrauterine life is suggested as a likely cause of the dental abnormalities. This hypothesis is supported by the fact that both primary and permanent teeth in the area were affected. It is further supported by the finding of dysplastic dentin.