CORRECTION OF SEVERE DYSTROPHIC SCOLIOSIS IN NEUROFIBROMATOSIS 1 WITH POSTERIOR VERTEBRAL COLUMN RESECTION IN MULTIPLE LEVELS

ABSTRACT Objective To evaluate the results after multiple posterior vertebral column resection (PVCR) in patients with severe kyphoscoliosis secondary to neurofibromatosis type 1 (NF-1). Methods Retrospective study of 4 adult male patients, mean age of 26.5 years, with severe rigid deformity, mean kyphosis of 122º and scoliosis of 88º, through clinical analysis and complementary exams. Results The mean postoperative follow-up was 27 months. Three vertebrae were resected on average. Interbody consolidation of arthrodesis was confirmed by CT in all cases. The correction rates of sagittal and coronal deformities were 54.09% and 70.45%, respectively. Prior to surgery, all patients were neurologically intact and as a post-surgical complication one patient had motor deficit (ASIA D) and pneumothorax also occurred in two patients. Conclusions The correction of severe kyphoscoliosis in adult patients with NF-1 is technically demanding and is not free of major complications. However, in view of the limited possibility of correction of these deformities, multiple-level PVCR has proved to be a safe and effective technique. Level of evidence IV; Case Series.摘要 目的：评估1型神经纤维瘤病（neurofibromatosis type 1, NF-1）继发重度脊柱后凸侧凸畸形患者接受多次后路脊柱截骨术（posterior vertebral column resection, PVCR）后的手术疗效。 方法：本研究为回顾性研究，纳入4例成年男性患者，平均年龄26.5岁，均患有重度僵硬性脊柱畸形，术前平均后凸角度为122°，侧凸角度为88°，通过临床分析与辅助检查完成研究。 结果：患者术后平均随访时长为27个月，平均截除脊柱椎体3个。所有病例均经CT证实椎间融合节段获得骨性融合。矢状位与冠状位畸形的矫正率分别为54.09%与70.45%。术前所有患者神经功能均完好；术后并发症方面，1例患者出现运动功能障碍（美国脊柱损伤协会（American Spinal Injury Association, ASIA）分级D级），另有2例患者发生气胸。 结论：针对1型神经纤维瘤病继发重度脊柱后凸侧凸的成年患者，畸形矫正手术技术难度较高，且无法避免严重并发症的发生。但鉴于此类畸形的矫正手段有限，多节段后路脊柱截骨术已被证实为安全有效的治疗技术。 证据等级：IV级；病例系列研究。