Safety and efficacy of posterior instrumentation for patients with congenital scoliosis and spinal dysraphism

Objective: Instrumentation and correction of severe congenital scoliosis, particularly in patients with spinal dysraphism, has been reported to cause a high potential rate of neurological compromise after instrumentation. The aim of this study was to evaluate the safety and efficacy of posterior instrumentation and correction of congenital scoliosis with accompanying spinal dysraphism. Level of Evidence: Level IV therapeutic studies. Methods: Retrospective x-ray measurements to analyze the efficacy and the evaluation of hospital charts to document the intraoperative and postoperative complications were performed for a consecutive patient series. Scoliosis Research Society-22 questionnaire was used to analyze the health-related quality of life. Results: Twenty-two patients (18 girls and 4 boys) formed the basis of the study. The average age was 12 years (range, 7-18 years) and the average follow-up period was 3.2 years (range, 2-10 years). The types of spinal dysraphism were diastematomyelia in 20 patients and syringomyelia with tethered cord in 2 patients. Twelve patients had previous surgery and 3 patients had simultaneous surgeries for spinal dysraphism. Posterior instrumentation with/without anterior release and fusion was performed in all patients. Major curve was corrected from an average of 71 degrees to 40 degrees (correction rate, 43.6%). The compensatory curve was corrected from an average of 47 degrees to 25 degrees (correction rate, 46.8%). The average loss of correction at final follow-up was 2.2 degrees for major curve and 3.5 degrees for the compensatory curve. The average scores for the 5 domains of Scoliosis Research Society-22 questionnaire were 3.5 for function, 3.9 for pain, 3.5 for self-image, 3.6 for mental health, 3.9 for satisfaction, and 3.6 for total. Neurological monitoring was conducted by using the wake-up test in all patients. The overall complication rate was 31%, including neurological compromise in 2 patients (9%). Conclusions: Spinal instrumentation was effective for the control of deformity with a relatively higher rate of complications. However, with respect to high complication rate, the ideal solution for managing the congenital cases is still to prevent the progression of the curve with early intervention by using the optimal surgical approach for that particular patient.