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（NF-1）继发重度脊柱后凸侧凸患者接受多节段后路全脊椎截骨术（PVCR）后的临床疗效。方法 采用回顾性研究设计，纳入4例成年男性重度僵硬性脊柱畸形患者，平均年龄26.5岁，平均后凸角122°，侧凸角88°，通过临床分析与辅助检查完成病例回顾。结果 术后平均随访时长为27个月，平均切除椎骨3枚。所有病例均经CT证实脊柱融合术后椎间融合确切。矢状面与冠状面畸形矫正率分别为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（美国脊髓损伤协会（ASIA）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，其畸形矫正手术技术难度较高，且无法避免重大并发症风险。但鉴于此类畸形的矫正手段有限，多节段后路全脊椎截骨术已被证实为安全且有效的治疗技术。本研究证据等级为IV级；研究类型为病例系列研究。