Objective: To expose the severity of problems surrounding orthodontic treatment of patients with Osteogenesis Imperfecta (OI).
Subject and Method: Our patient is a girl with OI type III, who has been in orthodontic treatment for 5 years.OI is a clinically, biochemically and genetically heterogeneous group of generalized conective tissue diseases, with a prevalence rate of 1:5000 - 1:10,000.Clinically, there are four forms of OI, focusing on the general number of fractures, presenile hearing loss, and involvement of the blue sclerae.A further subclassification includes Dentinogenesis Imperfecta (DI) as a manifestation of OI in some patients.The presence of additional orthodontic malocclusions has been documented: Class III, maxillary hypoplasia, anterior and posterior crossbite and open bite.Type III is the most severe form of OI in those who survive.There are multiple fractures, progressive limb and spine abnormalities, associated with short statue.81% of the OI type III patients have DI.
Results: Our orthodontic treatment was several times interrupted due to severe bone fractures.We started with Frankel functional appliance to achieve good anteroposterior position of the incisors in the mixed and early permanent dentition.Because of the abnormal dentin associated with DI banding is recommended.We used stainless steel crowns with brackets attached.Careful presurgical consultation is indicated with the oral surgeon and anesthesiologist.
Conclusions: A review of the literature reveals that there is very little published about OI patients having orthodontic treatment.Because of the extreme variability of expression seen in OI each case represents a potentially variable outcome.