Development of scoliosis in young children with osteogenesis imperfecta undergoing intravenous bisphosphonate therapy

We examined the relationship between scoliosis (Cobb angle  ≥ 10) and type of OI (Sillence classification: types I, III, and IV), physical mobility,Z-scores of bone mineral density in L2 –4 of the lumbar spine (L2–4 BMDZ-scores), age of patients at first treatment with PAM, pelvic frontal tilt and leg-length discrepancy. The prevalence of scoliosis was 23.5% in 34 young children with OI who underwent PAM therapy for a mean of 4.2 years. Lower L2 –4 BMDZ-scores, the presence of coronal and sagittal vertebral deformities and higher percentage of corrective osteotomy in the lower extremities were significant risk factors for the development of scoliosis. In patients with type III or IV OI, L2 –4 BMDZ-scores were significantly lower (p = 0.02) and the percentage of patients who started PAM therapy in early childhood was significantly lower in scoliosis group than in the non-scoliosis group (p = 0.01). Development of scoliosis depends on the severity of OI and has a strong relationship with bone fragility even under PAM therapy. Starting intravenous PAM therapy in infancy or early childhood has a potential to prevent the occurrence and progression of scoliosis associated with bone fr agility in young children with severe type III or IV OI.
Source: Journal of Bone and Mineral Metabolism - Category: Orthopaedics Source Type: research