title: Surgical Treatment of Dystrophic Spinal Curves Caused by Neurofibromatosis Type 1: A Retrospective Study of 26 Patients.
abstract: Dystrophic scoliosis in neurofibromatosis type 1 (NF-1) is difficult to treat. The purpose of this study was to review the clinical and radiological outcome of surgical treatment of dystrophic spinal curves in NF-1, for analyzing its efficacy, safety, and possible complications.This retrospective study consisted of 26 NF-1 patients with spinal deformities treated between 2003 and 2012 in our department. Preoperative X-ray, 3D-CT, and MRI were performed to evaluate the deformities of dystrophic scoliosis accurately. All patients were treated with posterior instrumented fusion alone using screws and hooks. According to the anatomical development situation of each patient's pedicles and the transverse processes, we chose different fixations and different fixed segments. The clinical and radiological outcomes of surgical correction were evaluated postoperatively.The average preoperative kyphosis was 43° (range 15-86°). The postoperative kyphosis had an average of 20° (range 10-39°) yielding 53% correction. At final follow-up, there was an average of 4.6% correction loss. The preoperative scoliosis Cobb angle had an average of 47° (range 35-96°). The postoperative scoliosis Cobb angle had an average of 21° (range 10-37°) yielding 55% correction. At final follow-up, there was an average of 6.6% correction loss. The apical vertebral body rotation was corrected by an average of 48%. At final follow-up, the score of the SRS-30 questionnaire ranged from 97 to 135 with an average of 109.In conclusion, the deformities of dystrophic scoliosis can be accurately determine through preoperative radiolographic evaluation, which plays an important role in guiding the correction of scoliosis program development. The results of this study demonstrate that satisfactory therapeutic effects can be achieved in the dystrophic scoliosis patients by preoperative meticulous surgical plans, intraoperative careful manipulation, and hybrid instrumentation.
authors.affiliation: From the Department of Orthopeadics, Xijing Hospital (XZ, LS, LY, ZXW, DWZ, WL, QJ), Department of Physiology, The Fourth Military Medical University, Xi'an, P.R. China (JL).
authors.forename: Xiong
authors.initials: X
authors.lastname: Zhao
authors.forename: Jun
authors.initials: J
authors.lastname: Li
authors.forename: Lei
authors.initials: L
authors.lastname: Shi
authors.forename: Liu
authors.initials: L
authors.lastname: Yang
authors.forename: Zi-Xiang
authors.initials: ZX
authors.lastname: Wu
authors.forename: Da-Wei
authors.initials: DW
authors.lastname: Zhang
authors.forename: Wei
authors.initials: W
authors.lastname: Lei
authors.forename: Qiang
authors.initials: Q
authors.lastname: Jie
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*; D011183:Postoperative Complications / Q000453:epidemiology / Q000209:etiology; D012189:Retrospective Studies; D012600:Scoliosis / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D013123:Spinal Fusion / Q000295:instrumentation; D016896:Treatment Outcome