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 t-test showed significative differences (P < 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