Wilde P H, Upadhyay S S, Leong J C
Duchess of Kent Children's Hospital, Hong Kong.
Spine (Phila Pa 1976). 1994 Jun 1;19(11):1264-70. doi: 10.1097/00007632-199405310-00013.
Of 28 patients with dystrophic spinal deformity resulting from neurofibromatosis who were treated surgically since 1968, 25 were studied regarding the outcome of spinal deformity at a mean follow-up of 9.7 years after surgery (mean age at follow-up, 21.5 years).
This study sought factors that contribute to curve deterioration despite successful spinal fusion for dystrophic spinal deformity in patients with neurofibromatosis. SUMMARY OF BACKGROUND DATA/METHODS: All patients had the following radiographs: standing anteroposterior and lateral, lateral bending, flexion and extension views of the spine, and radiographs of both tibiae. Patients who had large peripheral neurofibromata or evidence of long-bone deformity at other sites had radiographs of these anatomic regions. Spinal radiographs obtained pre-operatively, 6-12 months post-operatively, and at final follow-up were measured for spinal curvatures, vertebral height, and vertebral and disc wedging.
For analysis, the patients were divided into three groups according to deformity: 1) scoliosis, 2) ky-phoscoliosis (with kyphosis > 50 degrees) and 3) hyperkyphosis (with kyphosis > 50 degrees and sharply angulated over three vertebrae). Mean deterioration was: scoliotic group, 12 degrees (range, 0 degree-52 degrees); kyphoscoliotic group, 7 degrees (range, 0 degree-35 degrees); hyperkyphosis group, 38 degrees (range, 20 degrees-60 degrees). All patients in the latter group had anterior and posterior spinal fusion.
Spinal deformity in these patients tended to progress despite the achievement of spine arthrodesis in those with hyperkyphosis and short curves. The study shows that vertebral subluxation, disc wedging, and peripheral skeletal dystrophy are additional prognostic features that predict the progression of deformity after arthrodesis of the spine.
自1968年以来接受手术治疗的28例神经纤维瘤病所致营养不良性脊柱畸形患者中，对25例进行了研究，评估术后平均9.7年（随访时平均年龄21.5岁）的脊柱畸形结局。
本研究旨在寻找尽管神经纤维瘤病患者的营养不良性脊柱畸形成功进行了脊柱融合，但仍导致侧弯恶化的因素。背景数据/方法总结：所有患者均拍摄了以下X线片：站立位前后位和侧位、脊柱侧弯、屈伸位片以及双胫骨X线片。有大型周围神经纤维瘤或其他部位长骨畸形证据的患者拍摄了这些解剖区域的X线片。测量术前、术后6 - 12个月及末次随访时获得的脊柱X线片的脊柱曲度、椎体高度、椎体和椎间盘楔形变。
为进行分析，根据畸形情况将患者分为三组：1）脊柱侧凸，2）后凸脊柱侧凸（后凸>50度）和3）严重后凸（后凸>50度且在三个椎体上有明显成角）。平均恶化程度为：脊柱侧凸组12度（范围0度 - 52度）；后凸脊柱侧凸组7度（范围0度 - 35度）；严重后凸组38度（范围20度 - 60度）。后一组所有患者均进行了脊柱前后融合术。
尽管严重后凸和短节段曲线患者实现了脊柱融合，但这些患者的脊柱畸形仍有进展倾向。该研究表明椎体半脱位、椎间盘楔形变和周围骨骼营养不良是预测脊柱融合术后畸形进展的额外预后特征。
