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Dental Abnormalities in Osteogenesis Imperfecta: A Systematic Review.
Laura Ventura1,2,3,4, Sara J E Verdonk3,4,5, Lidiia Zhytnik1,2,3,4
1Department of Human Genetics, Amsterdam UMC Location Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
Osteogenesis imperfecta (OI) patients frequently experience dental issues like dentinogenesis imperfecta (DI) and malocclusions. Severe OI types show more dental abnormalities than milder ones, highlighting the need for dental care.
Area of Science:
- Genetics
- Oral Medicine
- Pediatric Dentistry
Background:
- Osteogenesis imperfecta (OI) is a rare genetic disorder causing bone fragility and deformities.
- Dental abnormalities, including dentinogenesis imperfecta (DI), malocclusions, and unerupted teeth, are common in individuals with OI.
Purpose of the Study:
- To comprehensively review and assess the prevalence of dental abnormalities in the Osteogenesis imperfecta (OI) population.
- To explore variations in dental issues across different OI clinical types and pathogenic variants.
Main Methods:
- A systematic literature search was conducted following PRISMA guidelines across PubMed, Embase, and Web of Science up to June 2024.
- 34 studies were included out of 672 screened articles.
Main Results:
- Dental abnormalities are prevalent in OI, with dentinogenesis imperfecta (DI) affecting 20-48% of patients.
- Severe OI types (III/IV) present more dental issues than milder types (OI I); OI type V typically lacks DI.
- Malocclusion prevalence varies (Class I: 14.8-50%, Class II: 0-37.5%, Class III: 4.1-84%), with Class III malocclusion being more common than in the general population. Open bites, cross-bites, and unerupted/missing teeth are frequent in OI types III and IV.
Conclusions:
- Comprehensive dental examinations are crucial for individuals with Osteogenesis imperfecta (OI) due to the high prevalence of dental abnormalities.
- There is a need for clearer diagnostic guidelines for dentinogenesis imperfecta (DI) in the context of OI.
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