Roentgenographic Evaluation of the Spine in Patients with Osteogenesis Imperfecta

Abstract

Background Context Osteogenesis imperfecta (OI) is a hereditary connective tissue disorder that leads to bone weakness and deformities, especially in the spine, which can lead to poor outcomes. Purpose The aim of this study was to find patterns and risk factors in spinal deformities in patients with OI. Study Design/Setting Retrospective study at Irmandade da Santa Casa de Misericórdia de São Paulo. Methods Radiographs of the spine were evaluated. Patient Sample Seventy patients with OI were selected. Outcome Measures We observed the presence or absence of the following changes: biconcave vertebrae, chest and vertebral deformities, unilateral rib, and thoracolumbar kyphosis. The greater curve was considered to be the primary one and the secondary curve was considered compensatory. Results In the study sample, we observed that the patients' ages ranged between 7 and 50 years, with the mean age as 13 years, and 76% had scoliosis. In 68% of the cases, the main curve in the thoracic region was observed with the convexity to the right. Conclusion The following are associated with the presence of scoliosis in patients with OI: biconcave vertebrae, vertebral and chest deformity, unilateral rib, and thoracolumbar kyphosis. The thoracolumbar kyphosis is associated with thoracic hypokyphosis in patients with OI.