Surgical treatment of spinal deformities in Marfan syndrome: Long-term follow-up results using different instrumentations.

Background: Scoliosis is the most frequent spinal deformity related to Marfan syndrome (MFS). Treatment with a brace is often ineffective, and surgical treatment is very challenging; many instrumentations were used along the years. Our retrospective study has the purpose of identifying the reliability of different devices in three-dimensional correction of the spine deformities in MFS.

Materials And Methods: We reviewed retrospectively the records of patients surgically treated, in a single institution between 1999 and 2016, for spinal deformities in MFS. X-rays were reviewed for analyzing the magnitude of the curves in preoperative time (T0), the amount of correction in the immediate after surgery period (T1), and it's stability at follow-up (FU) (T2). The clinical outcomes were also evaluated with the Scoliosis Research Society 24.

Results: A total of 21 patients with a mean age at surgery of 16 years met inclusion and exclusion criteria. Four different construct types were identified: hooks with sublaminar wires (G1), hooks and pedicle screws (G2), pedicle screws (G3), and pedicle screws with sublaminar wires (G4). The mean FU time was 8 years. The average major scoliosis curve had a mean value of 63.48° at T0 and was corrected to 28.81° at T2. Furthermore, minor curve, thoracic lordosis, and lumbar kyphosis (when associated to scoliosis) were also corrected. Student -test showed significative differences ( < 0.05) for all curves between T0-T1 and T0-T2 while between T1 and T2, no differences were found. We also evaluated separately the results of each instrumentation, and G3 obtained the best performances.

Conclusions: Our results shows that screws may guarantee a better correction of the deformities.

Level Of Evidence: III.