Study

Design: Retrospective study.

Objective: To assess results of posterior instrumented fusion using pedicle screws in 12 children with osteogenesis imperfecta (OI) with spinal deformity at a single institution from 2001 to 2012. Summary of background data: This is the first case series of OI patients who underwent non-cement augmented screw-rod instrumented fusion published in the literature.

Methods: Of a total of 54 children with spinal deformity associated with OI, 12 (22.2%) were submitted to posterior spinal fusion with pedicle screws (80% density) because of severe spinal deformity. Here we reported the results in seven females and five males.

Results: Five thoracic (41.7%), five double (thoracic and lumbar 41.7%), and two lumbar (16.7%) curves were considered. The mean number of fused levels was 11.8 (range: 5 to 16). Mean age at surgery was 13 years 8 months. Mean follow-up was 7 years 11 months (range: 3 years 7 months to 16 years 1 month). The mean preoperative scoliosis angle was 75.6°, whereas the postoperative angle was 31.4° (58.5% correction rate). The mean preoperative kyphosis angle was 57.4° and the postoperative angle was 42.3°. We observed one superficial infection, one dural tear, and three cases of proximal junctional kyphosis; two patients required one revision surgery each (2 years and 4 months postoperatively on average).

Conclusions: To our knowledge, this is the first case series published in the literature regarding OI with instrumented fusion with non-cement augmented pedicle screws exclusively in children with spinal deformity. We found that posterior spinal fusion with the screw-rod system in OI deformity in children is feasible and reliable, and has acceptable clinical and imaging results in the long-term follow-up. Level of evidence: Level IV.