Oral-Facial Aspects of Osteogenesis Imperfecta

The axial skeleton is affected to a variable degree in the different forms of osteogenesis imperfecta (OI). The pleiotropic effect also often involves the dentition and craniofacies, with approximately half of those with OI also having dentinogenesis imperfecta (DI). The incidence of DI is greater in the more severely affected viable class types (VI and III), which also tend to express the more severe effect on craniofacial growth. There are other dental abnormalities in addition to DI, including impacted teeth. The alteration in craniofacial growth starts with the cranial base, and extends to hypoplasia of the maxilla and relative prognathism of the mandible. This typically develops into a negative anterior overjet and a Class III malocclusion. Posterior open-bites also occur, which are extremely resistant to treatment. Orthognathic surgery is possible to treat the skeletal malocclusion on a case-by-case basis. Bisphosphonate use may slow orthodontic tooth movement, but appear to present a very low risk of osteonecrosis of the jaws if dental extractions are indicated.