Background and Objectives: Thoracolumbar kyphosis is one of the most frequent skeletal manifestations in patients with achondroplasia. Few papers have been published on the surgical treatment of this condition, especially in skeletally mature patients. With this study, we presented a retrospective case series of long-term surgical results for achondroplastic patients with severe thoracolumbar kyphosis. This study was conducted to evaluate the outcome of surgical treatment for thoracolumbar kyphosis in patients associated with achondroplasia presenting with paraparesis. Materials and Methods: Three patients with achondroplasia who developed neurologic deficits due to severe thoracolumbar kyphosis and underwent surgical treatment were evaluated (mean age 22.3 years; mean follow-up 9.3 years). All patients were treated with posterior vertebral column resection (p-VCR) of hypoplastic apical vertebrae with a cage and segmental instrumentation. Neurologic outcomes (JOA scores), correction of kyphosis, and operative complications were assessed. Results: All patients had back pain, neurological deficits, and urinary disturbance before surgery. The average preoperative JOA score was 8.3/11 points, which was improved to 10.7/11 points at the final follow-up (mean recovery rate 83%). All patients obtained neurologic improvement after surgery. The mean preoperative kyphotic angle was 117° (range 103°-126°). The postoperative angles averaged 37° (range 14°-57°), resulting in a mean correction rate of 67%. All patients had postoperative complications such as rod breakage and/or surgical site infection. Conclusions: The long-term results of p-VCR were acceptable for treating thoracolumbar kyphosis in patients with achondroplasia. To perform this p-VCR safely, spinal navigation and neuromonitoring are inevitable when resecting non anatomical fused vertebrae and ensuring correct pedicle screw insertion. However, surgical complications such as rod breakage and surgical site infection may occur at a high rate, making informed consent very important when surgery is indicated.
Keywords: achondroplasia; long-term follow-up; navigation; vertebral column osteotomy.