Outcomes after surgical correction of severe scoliosis in patients with osteogenesis imperfecta: a prospective, 2-year minimum follow-up study with radiographic and patient-reported outcomes
摘要
To evaluate both radiographic and patient-reported outcomes after surgical correction of severe scoliosis in osteogenesis imperfecta (OI) patients with minimum 2-year follow-up. The main research question was whether modern multimodal surgical approaches translate into meaningful improvements in both deformity correction and quality of life measures.
MethodsThis retrospective analysis of prospectively collected data included 35 OI patients (mean age 12.2 ± 3.7 years) who underwent posterior spinal fusion for scoliosis at a single institution between 2013 and 2022. OI type 3 was most common (69%). Radiographic parameters and patient-reported outcomes (SRS-22, CP CHILD, PROMIS) were assessed preoperatively and at latest follow-up (mean 5.5 ± 3.1 years). Surgical technique included navigation-guided pedicle screw placement, cement augmentation at foundation levels, and perioperative bisphosphonate therapy.
ResultsMajor curve improved from 72.3 ± 15° to 32.2 ± 17° (56% correction, p < 0.001) and minor curve from 53.8 ± 14° to 24.1 ± 12° (55% correction, p < 0.001). Other radiographic parameters including apical vertebral translation, lowest instrumented vertebra tilt, pelvic obliquity, and thoracolumbar kyphosis showed significant improvement while maintaining thoracic kyphosis and lumbar lordosis. SRS-22 total score improved from 3.6 to 4.1 (p = 0.002), with function, pain, and self-image domains achieving minimal clinically important differences. CP CHILD total score improved, with significant improvement in the positioning/transferring/mobility domain. PROMIS showed significant improvements in fatigue and pain interference. Two complications (5.7%) occurred without infections or neurological deficits.
ConclusionModern surgical correction of severe scoliosis in OI patients achieved significant improvements in both radiographic parameters and patient-reported outcomes, demonstrating the effectiveness of current multimodal surgical strategies in this challenging population.