Evaluation of oral problems in an osteogenesis imperfecta population.
OBJECTIVE
The incidence of craniofacial and dental anomalies in children with the more severe nonlethal forms of osteogenesis imperfecta was evaluated.


METHODS
The study evaluated 40 children (age range, 1-17.5 years) with types III and IV osteogenesis imperfecta. In each case, the dentition was evaluated for the presence of dentinogenesis imperfecta, attrition, and caries, as well as for radiographic appearance, dental development, and malocclusion.


RESULTS
The incidence of dentinogenesis imperfecta was greater than 80% in the primary dentition. Clinically, the color of the dentition was of predictive value in appropriate management of the primary dentition. Tooth discoloration and attrition did not occur to the same extent in the permanent dentition as in the primary dentition in either group. Class III dental malocclusion 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 osteogenesis imperfecta, whereas accelerated development was noted in 23% of the patients with type IV. In addition, ectopic eruption occurred in 13 patients.


CONCLUSIONS
In addition to dentinogenesis imperfecta, significant oral problems occur in types III and IV osteogenesis imperfecta. Other features that impact the dental management of this population are highlighted.
Clinical Research Core, National Institute of Dental Research, National Institutes of Health, Bethesda, MD 20892, USA.
A C
AC
O'Connell
J C
JC
Marini
D000293:Adolescent; D000848:Anodontia / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000209:etiology; D003730:Dental Care for Persons with Disabilities; D003731:Dental Caries / Q000209:etiology; D003811:Dentinogenesis Imperfecta / Q000209:etiology* / Q000503:physiopathology; D019229:Dentition, Permanent; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008310:Malocclusion / Q000209:etiology*; D010013:Osteogenesis Imperfecta / Q000150:complications*; D019217:Tooth Attrition / Q000209:etiology; D014075:Tooth Discoloration / Q000209:etiology; D014076:Tooth Diseases / Q000209:etiology*; D014078:Tooth Eruption; D014094:Tooth, Deciduous