Evaluation of oral problems in an osteogenesis imperfecta population

Objective. The incidence of craniofacial and dental anomalies in children w ith the more severe nonlethal forms of osteogenesis imperfecta was evaluate d. Study design. The study evaluated 49 children (age range, 1-17.5 years) wit h types III and IV osteogenesis imperfecta. In each case, the dentition was evaluated for the presence of dentinogenesis imperfecta, attrition, and ca ries, as well as for radiographic appearance, dental development, and maloc clusion. Results. The incidence of dentinogenesis imperfecta was greater than 80% in the primary dentition. Clinically, the color of the dentition was of predi ctive value in appropriate management of the primary dentition. Tooth disco loration and attrition did not occur to the same extent in the permanent de ntition as in the primary dentition in either group. Class III dental maloc clusion occurred in 70% to 80% of this osteogenesis imperfecta population, with a high incidence of anterior and posterior cross bites and open bites. A delay in dental development was observed in 21% of patients type III ost eogenesis imperfecta, whereas accelerated development was noted in 23% of t he patients with type IV. In addition, ectopic eruption occurred in 13 pati ents. Conclusions. In addition to dentinogenesis imperfecta, significant oral pro blems occur in types III and IV osteogenesis imperfecta. Other features tha t impact the dental management of this population are highlighted.