Surgical treatment of severe cervical dystrophic kyphosis due to neurofibromatosis Type 1: a review of 8 cases

Object The goal in this study was to retrospectively investigate the clinical efficacy of surgical treatment for cervical dystrophic kyphotic deformity due to neurofibromatosis Type 1. Methods Between January 1998 and July 2008, 8 patients with cervical dystrophic kyphotic deformity due to neurofibromatosis Type 1 (mean Cobb angle of 58.5°) were surgically treated in the authors' department. The mean age at surgery was 19 years (range 12–38 years). Among these patients, 1 with a Cobb angle of 52° and good flexibility underwent single anterior correction, whereas the other 7 patients with severe deformity and poor flexibility received combined anterior and posterior cervical osteotomy. Motor-evoked potential studies were used intraoperatively for spinal cord monitoring. Radiographic assessment and Japanese Orthopaedic Association scoring were used to evaluate the clinical outcome. Results No severe neurological complications were noted. Two patients complained of persistent neck and shoulder pain after combined anterior and posterior correction, which alleviated after conservative treatment half a year later. All patients were followed up for a mean of 21.1 months (range 6–36 months). All patients had a solid bone fusion at the latest follow-up, with Japanese Orthopaedic Association scoring improving from 11.5 preoperatively to 14.1 postoperatively (p < 0.01) at the final follow-up. The kyphotic deformities improved significantly, with average Cobb angles of 2.5° postoperatively and 4.1° at final follow-up. Conclusions The deformity of neurofibromatosis with cervical kyphosis is severe, and surgery carries a high risk of failure. Although premature fusion may be performed, the deformity may still progress, and this situation may lead to failure of surgery. The successful management of this disease requires early recognition and a more aggressive and reliable intervention to prevent disastrous worsening of the deformity. Meticulous preoperative evaluation, appropriate surgical strategy, and skilled technique were essential for successful surgical treatment and good clinical results.