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Teeth of seven patients from the Brandywine isolate who had dentinogenesis imperfecta (DI) type III were evaluated by clinical, radiologic, and scanning electron microscopic techniques. The deciduous and permanent teeth were opalescent, and there was marked attrition. Enamel pitting was present on some permanent teeth. Anterior open bites were found in all persons with complete permanent dentitions. Pulps of developing teeth were larger than normal during early development but rapidly became almost completely obliterated. There was increased constriction at the cementoenamel junctions. While radiolucencies were noted at the apices of teeth which had pulp exposures due to attrition, several patients had similar radiolucencies which could not be attributed to caries or attrition. Scanning electron microscopy showed a significant reduction in the number of dentin tubules on fractured dentin surfaces; calcospherites at the calcification front were either irregularly shaped or absent. A single tooth from a patient with DI type II was studied and had similar abnormalities on scanning electron microscopy, although tubules were easier to find and calcospherites at the calcification front were more regular than in DI type III. The findings in DI type III of enamel pitting, enlarged pulps early in tooth development, and radiolucencies at the apices of teeth without pulp exposures support the hypothesis that DI type II and DI type III are different disorders.

The rare hereditary disease, dentinogenesis imperfecta, is a disturbance of dentin formation in both the deciduous and permanent dentitions. It may be associated with osteogenesis imperfecta, though it is probably that the two diseases are carried by different genes. This association was recognized in a 19-year-old man. Dentinogenesis imperfecta had been diagnosed at the age of 6 and had been regarded as a mutation; 11 years later, an atypical form of osteogenesis imperfecta developed. The case is atypical because of the apparent absence of dentinogenesis imperfecta in the patient's family. The dental manifestations may have heralded the bone disease.

Odontodysplasia is a rare developmental anomaly affecting the tooth structures in both deciduous and permanent dentitions. The enamel is thin and uneven in thickness, and the detinal tissue surrounds very large pulp chambers. Denticles are present in the pulp organ. The maxilla is involved twice as frequently as the mandible. Most of the affected teeth are in the anterior segments; however, all other teeth can be affected. The cause is unknown. Because of the tendency of the affected teeth to develop abscesses, the most common treatment is extraction. Two additional cases are reported, and the literature is reviewed.