The growth-friendly surgical treatment of scoliosis in children with osteogenesis imperfecta using distraction-based instrumentation.

PURPOSE: The study was undertaken to determine the feasibility of growth-friendly distraction-based surgery in children with OI.

METHODS

Two multi-center databases were queried for children with OI who had undergone GR or VEPTR surgery. Inclusion criteria were a minimum 2-year follow-up and three lengthening procedures following the initial implantation. Details of the surgical techniques, surgical complications, and radiographic measurements of deformity correction, T1-T12 and T1-S1 elongation and growth were recorded.

RESULTS

Five patients were identified. There was one patient with type I OI and two patients each with type III and type IV. Four patients had GR constructs and one a VEPTR construct. The initial scoliosis deformity averaged 80° (70°-103°), and the subsequent corrections averaged 32% for initial correction, 48% at last follow-up, and 54% for the two patients that had a final fusion. The T1-T12 and T1-S1 growth averaged 31 mm and 44 mm respectively, and yearly growth averaged 4 mm and 6 mm, respectively. Growth was notably much less in those with more severe disease. There were 13 complications in 4 patients. Nine of the 10 surgical complications were anchor failures which were corrected in 7 planned and 2 un-planned procedures. Significant migration occurred in one patient with severe OI type III.

CONCLUSION

The results varied in this heterogeneous population. In general, satisfactory deformity corrections were obtained and maintained, modest growth was obtained, and complications were similar to those reported in other series of growth-friendly surgery. Limited growth and significant anchor migration are to be anticipated in this population.

LEVEL OF EVIDENCE: IV.