MACHADO, Cíntia de Vasconcellos; PASTOR, Iandira Maria Oliveira; TELLES, Paloma Dias da Silva    ROCHA, Maria Celina Barreiros Siquara da. Osteogenesis imperfecta associated with dentinogenesis imperfecta: case report. RFO UPF []. 2012, 17, 3, pp. 336-341. ISSN 1413-4012.

Introduction: Osteogenesis imperfecta is a rare genetic condition, which the main clinical manifestation is bone fragility with different degrees of severity. This disease may be associated with dentinogenesis imperfecta, in which the dentin tissue is altered. This study was conducted in order to familiarize health professionals with possible systemic manifestations of osteogenesis imperfecta associated with dentinogenesis imperfecta, as well as the oral aspects of this condition, since the severity of the disease is quite variable. Case report: this study describes a case of a 7 year-old girl, patient of the ambulatory of the pediatric dentistry discipline of the Dentistry School of the Federal University of Bahia, who carried the disease. The diagnosis of osteogenesis imperfecta type III was performed based on clinical and radiographic findings along with the patient's previous medical history. Typical manifestations of the disease were found, such as: bone fragility, limited mobility, and deformities in upper and lower limbs. Among oral aspects, the brown staining of the teeth, partial obliteration of the root canal of lower incisors, as well as the displacement of enamel in some teeth confirmed the presence of dentinogenesis imperfecta. Conclusion: Despite the severe systemic issues and limitations that may be present in patients affected by this disease, it is of utmost importance that care with the oral cavity are not overlooked, thus promoting the patient a better quality of life.

: Osteogenesis imperfecta; Dentinogenesis imperfecta; Pediatric dentistry.