Treatment of scoliosis in osteogenesis imperfecta: Experience at a single institution

Abstract

Background: Spinal deformities are frequently seen in osteogenesis imperfecta (OI). We investigated contemporary spinal fusion techniques including pedicle screw fixation with or without cement augmentation in OI patients with scoliosis. Materials and Methods: OI patients with posterior-only scoliosis surgery were reviewed retrospectively (2005–2017). Preoperative and postoperative clinical status was compared. The radiographic review included pelvic obliquity, major curve magnitude, coronal balance, apical vertebral translation (AVT), lowest instrumented vertebrae (LIV) tilt angle, proximal and distal junctional angle, T1–S1 distance, and T1-pelvic angle. Results: Sixteen patients were included in the study. The mean age at surgery was 14 years (range, 6–19). The average follow-up period was 80 ± 40 months (range, 24–148). Mean preoperative curve magnitude of 76° ± 19° was significantly larger than the initial (31° ± 16°) and final (32° ± 17°) postoperative curve magnitudes (58% correction; P < 0.001). Mean preoperative AVT and LIV tilt angle were significantly higher than the initial and final postoperative measurements (P < 0.001 and P < 0.001, respectively). There was no difference between the measurements of coronal balance, pelvic obliquity, and T1–S1 distance among the preoperative, initial postoperative, and final follow-up measurements (P = 0.479, P= 0.125, and P= 0.05, respectively). There was no proximal junctional failure but one distal junctional failure led to revision surgery. Ambulatory status was unchanged in all patients, but an improvement in subjective self-reported clinical complaints was observed. Conclusion: Pedicle screw instrumentation with or without cement augmentation provided stability with few complications and improved clinical outcomes. Although preoperative activity level did not change compared with postoperative activity, there was an improvement in self-reported clinical complaints.