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Objective: The aim of the present study was to explore the surgical treatment and prognosis of 27 cases of neurofibromatosis type 1 with severe dystrophic kyphosis., Methods: We performed surgical treatment for scoliosis and kyphosis caused by dystrophic curves at Peking Union Medical College Hospital, Beijing, China from December 2015 to December 2017. The study included 21 patients with moderate to severe kyphosis, 12 males and 9 females, with an average age of 14.95 ± 6.05 years. All patients had kyphosis angles greater than 70° and had more than four skeletal developmental defects. A total of 6 patients with severe kyphosis, 2 males and 4 females, with an average age of 12.5 years, had more than five skeletal developmental defects with a kyphosis angle greater than 90° or a lumbar kyphosis angle greater than 40°. According to the patient's own situation, we adopted a low-grade surgery scheme (grades 1 or 2) or a high-grade surgery scheme (grades 3-6). The low-grade surgery was mainly lower articular surface resection or pontodestomy, and the high-grade surgery was mainly apical vertebral body or upper discectomy. All patients were followed up to determine their prognosis., Results: Statistical analysis showed that there was a significant difference in preoperative and postoperative scores between the two groups (P < 0.05), and scoliosis correction showed that surgical treatment had a significant effect on scoliosis kyphosis. The mean follow-up time was 66.7 months. Follow-up results showed that 50% of complications after internal fixation were related to high-level surgery. Complications included displacement of the titanium cage, removal of the lamina hook, formation of pseudoarthrosis, and internal fixation failure (with a rate of 7.7%-14.3%). In contrast, there were no associated symptoms for low-grade surgery. In addition, the results showed that gender, age, extent of resection, height, and body mass index had no significant effect on preoperative, postoperative, and prognostic indicators of patients (P > 0.05)., Conclusion: Early identification of dysplastic scoliosis-related deformities plays an important role in surgical planning and prognosis, and low-level surgical procedures are more favorable for patients' prognosis., (© 2020 The Authors. Orthopaedic Surgery published by Chinese Orthopaedic Association and John Wiley & Sons Australia, Ltd.)

Background: There have been many reports on the treatment of congenital kyphoscoliosis. However, congenital deformities in the cervicothoracic spine (C7-T1) have not been well described because of the rarity of these conditions., Methods: The medical records and imaging studies of 25 children who were treated with 360° osteotomy for congenital deformities in the cervicothoracic spine (C7-T1) at a mean age of 11.4 years were reviewed., Results: All 25 children presented with torticollis; 4 presented with neck pain; 10, with facial asymmetry; and 3, with preoperative neurological deficits. Twenty-three patients had congenital deformities in other regions of the spine. Six patients had a total of 8 intraspinal deformities. On average, the cervicothoracic curve was corrected from 53° preoperatively to 14° at the latest follow-up, the segmental kyphosis was corrected from 25° to 12°, and the head tilt improved from 25° to 5°. Nineteen patients had a total of 28 complications, including 1 transient cord injury together with a permanent C8 nerve root injury, 11 transient nerve root injuries, 1 transient Horner syndrome, 9 cases of decompensation of a compensatory curve, 2 implant failures, 2 cases of hemothorax, 1 dural tear, and 1 case of delayed wound-healing., Conclusions: Most congenital cervicothoracic deformities are fixed, and early surgical intervention may be needed. A 360° osteotomy is indicated for this type of rigid deformity and may provide satisfactory correction. However, 360° osteotomy in the cervicothoracic spine (C7-T1) is technically demanding with a higher risk of nerve root injuries, although most injuries tend to be transient. If the compensatory thoracic curve is severe and rigid, 1-stage or staged surgery in this region may be required., Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Study Design: A retrospective study., Objective: The objective of this study is to report the result of patients with neurofibromatosis type 1(NF-1), dystrophic scoliosis, and rib-head protrusion into the spinal canal who received posterior scoliosis correction surgery without rib-head resection., Summary of Background Data: A total of 124 patients with NF-1 and dystrophic scoliosis were treated at our institution during the study period. Eight patients with a median age of 12 years had rib-head protrusion into the spinal canal and received surgery and were included in the analysis., Methods: All 8 patients (6 male, 2 female) were treated from 2003 to 2013 and received posterior correction with a pedicle screw-rod 3-dimensional correction system or screw-hook hybrid system. Scoliosis correction rate and percentage of spinal canal occupied by the rib head were analyzed., Results: The median patient age, number of segments fused, and follow-up duration were 12 years, 10.5, and 22.5 months, respectively. There were no surgery-related complications, and symptoms in all patients improved after surgery. The median postoperative and 1-year follow-up sagittal kyphotic angles were significantly smaller as compared with the preoperative value (28.5 and 31 vs. 62.5 degrees, P=0.012). The median postoperative coronal Cobb angle of the main thoracic curve was significantly smaller compared with the preoperative value (29 vs. 64.5 degrees, P=0.012). The median percentage of the spinal canal occupied by the intraspinal rib was significantly lower at 1-year follow-up compared with the preoperative value (23.1% vs. 28.6%, P=0.018)., Conclusions: Posterior correction without rib-head excision can provide good outcomes for patients with NF-1 and dystrophic scoliosis and rib-head protrusion into the spinal canal.