Comprehensive Treatment of a Patient with Osteogenesis Imperfecta and Congenital Hemivertebra: A Case Report

Osteogenesis imperfecta (OI) is a rare hereditary connective tissue disorder characterized by extreme bone fragility, making the surgical management of associated severe scoliosis exceptionally challenging due to the high risk of instrumentation failure. The coexistence of OI and a congenital hemivertebra in a single patient is exceedingly rare and further complicates spinal deformity correction. We report the case of a 17-year-old boy with OI who presented with progressive, severe thoracolumbar scoliosis and worsening lumbar pain. Imaging revealed major Cobb angles of 85° and 95°, a fully segmented L3 hemivertebra, and markedly reduced bone mineral density with a total lumbar Z-score of –5.8. To optimize surgical safety and outcomes, a comprehensive, multidisciplinary treatment strategy was implemented. This included preoperative halo-gravity traction to improve spinal flexibility and alignment, alongside bisphosphonate therapy to enhance bone mass. The patient underwent a posterior L3 vertebrectomy and spinal fusion from T4 to S1. Postoperative radiographs demonstrated satisfactory deformity correction. Although asymptomatic pedicle screw breakage was observed at the 6-month follow-up—highlighting the immense biomechanical stress posed by the inherent bone fragility in OI—the patient maintained stable spinal alignment without significant loss of correction at 12 months. This case underscores that while meticulous perioperative optimization (including traction and pharmacological therapies) combined with advanced surgical planning can achieve successful deformity correction in patients with OI and congenital scoliosis, continuous long-term monitoring remains vital due to the persistent risk of hardware fatigue. Trial registration Not applicable. This study is a case report and not a clinical trial.