Oral findings in osteogenesis imperfecta

Area of Science:

• Dentistry
• Genetics
• Orthodontics

Background:

• Osteogenesis imperfecta (OI) is a group of genetic disorders characterized by brittle bones.
• Dental anomalies, including dentinogenesis imperfecta (DI), can occur in patients with OI.
• The prevalence and specific characteristics of dental findings in OI patients require further elucidation.

Purpose of the Study:

• To evaluate the dentitions of patients with autosomal dominant or sporadic osteogenesis imperfecta (OI).
• To determine the association between different types of OI and the presence of dentinogenesis imperfecta (DI).
• To assess the incidence of malocclusions and impacted teeth in OI patients.

Main Methods:

• Clinical examination of the dentitions of twenty-eight patients diagnosed with OI.
• Categorization of OI based on inheritance patterns (autosomal dominant vs. sporadic).
• Assessment of dental features including tooth wear, fractures, malocclusions, and impactions.

Main Results:

• Dentinogenesis imperfecta (DI) was diagnosed in all patients with dominantly inherited OI from three families, but not in those from three other families.
• Among sporadic OI cases, DI was present in 4 out of 12 patients.
• Abnormal tooth wear and spontaneous fractures were exclusively observed in patients with DI. The DMF ratio increased with age in OI type I and was higher in OI type III with DI. Class III malocclusions (66%) and impacted first and second molars were common.

Conclusions:

• The occurrence of dentinogenesis imperfecta (DI) in osteogenesis imperfecta (OI) varies significantly, even within families with dominant inheritance.
• Dental anomalies such as abnormal wear, fractures, malocclusions, and impacted teeth are frequent in OI patients.
• OI type and DI presence are associated with increased DMF index and specific orthodontic issues.