Scoliosis In Osteogenesis Imperfecta A Single Centre Cross Sectional Study

Abstract

Purpose: Scoliosis has been frequently reported in patients with osteogenesis imperfecta (OI). We aim to show the differing prevalence of scoliosis among the types of OI, as well as characteristics and clinical implications of scoliosis among our ethnically Chinese cohort. Materials and Methods: In this single centre cross-sectional study, patients with OI and at least one radiograph of the spine were enrolled and classified into Sillence types I, III, IV, and V based upon clinical and radiological features. Cobb angle was measured, with severity and characteristics of curve noted according to the Scoliosis Research Society glossary. Results: One hundred and twenty-seven patients (75 males, 52 females; mean age, 9.6 years) were included. The overall prevalence of scoliosis among all patients was 53%. The prevalence of scoliosis was highest among type III 76%) compared to types V (60%), and IV (44%), while type I had the lowest (29%). Type III also had the highest prevalence of severe scoliosis (38%). Severity of scoliosis was positively correlated with age in OI types (type III, p<0.001; type IV, p<0.001; type V, p=0.004; respectively), apart from type I (p=0.222). Fifty-eight percent of all scoliotic curves were located in the thoracic region (T2–T11). Sixty-three percent of primary curves were right sided. Conclusion: Type III OI demonstrated the highest prevalence of scoliosis, both overall and severe. Given that scoliosis occurs mostly in the thoracic region (affecting cardiopulmonary function) and is correlated with age, early and aggressive intervention is appropriate. Meanwhile, type I has a relatively lower prevalence and no correlation with age, thus may be treated more conservatively.