Oral findings in osteogenesis imperfecta

Abstract

The dentitions of twenty-eight patients, each of whom had either an autosomal dominant or a sporadic osteogenesis imperfecta (OI) syndrome, were evaluated. The diagnosis of dentinogenesis imperfecta (DI) could be established in all seven patients with dominantly inherited OI in three families, while all eight persons with dominant OI in three other families had normal teeth. Of the thirteen remaining patients with OI, twelve had no family history of the disorder; four had DI and eight had normal teeth. One person had a family history of OI and DI. All patients with abnormal tooth wear and spontaneous tooth fractures had DI. The DMF ratio increased with age in all patients with OI type I and was higher among the patients with OI type III and DI. Class III malocclusions were found in 66% of the patients. A statistically significant high incidence of impacted first and second molars was noted.