Posterior thoracolumbar fusion in a patient with Kleefstra Syndrome related scoliosis: The first case reported

Spine deformities could be considered a possible manifestation of the childhood hypotonia, typical feature of Kleefstra Syndrome (KS). There is a paucity of literature describing posterior spinal fusion in the Kleefstra syndrome patient. For patients who develop severe scoliotic curve, bracing is often ineffective and surgery is recommended. We report the first corrective surgery for scoliosis in one patient with KS.We describe a case of 13-year-old female with severe developmental scoliosis in KS. Preoperative examination showed a thoracolumbar scoliosis with left convex thoracic curve (T3-T9, 97°) and right convex thoracolumbar curve (T9-L3, 88°). Posterior correction, pedicle screw fixation and bone graft fusion T3-L5 was performed. Postoperatively, the thoracic curve was corrected to 33° while the thoracolumbar one to 26° and better standing posture was obtained. Six month follow-up images showed no loosening of the hardware. The patient is still in our follow-up program.Scoliosis seems to be a rare evenience of the severe hypotonia of patients with KS. We report the first case of scoliosis in KS treated successfully with surgery. Corrective surgery for spinal deformity, such as scoliosis, could help in posture and improve the quality of life especially in complicated patients such as syndromic ones.     

前路器械内固定手术矫治脊椎侧凸

作者从１９７９年开始使用戴瓦氏手术，１９８３年开始使用斯伍基氏手术，至１９９４年共做５７例（男性２８例，女性２９例），年龄为９～２８岁。手术无死亡。术后并发症戴瓦氏手术：１例截瘫，２例椎体骨折伴螺丝钉拔出，２例螺丝钉折断，２例金属缆折断；斯伍基氏手术，１例杆折断，２例切口感染。     

Three-dimensional spinal curvature in idiopathic scoliosis

Scoliosis is usually considered as a deformity of the spine in the frontal plane, without reference to curvatures in other planes. In this study, the three-dimensional shape of the spine of 104 patients with untreated idiopathic scoliosis (5-55 degrees Cobb) was studied by means of stereo radiographs to determine relationships between curvature of the spine in the frontal plane view, in the lateral view, and in the intermediate views. There was a weak but statistically significant correlation (r = 0.2) relating greater scoliosis with lesser kyphosis or greater lordosis. In the thoracic region, the sagittal plane spinal curvature was less than that measured in a population without scoliosis (mean difference, 7.72 +/- 9.9 degrees). Seventy-four of 76 scolioses in the upper region of the spine with lateral curvature greater than 5 degrees Cobb were kyphotic. Sixty-four of 84 curves greater than 5 degrees Cobb in the lower region were lordotic. Measuring curvatures in the plane of symmetry of the rotated apical vertebra altered these ratios to 69 of 76 kyphotic in the upper region and 68 of 84 lordotic in the lower region. The plane of maximum curvature of sections of the spine with scoliosis was not related to the plane of symmetry of the rotated apical vertebra, for in kyphotic regions of the spine the rotations of these two planes were in opposite directions. In all cases, the magnitudes of the rotations were quite different, i.e., by a factor of -0.22 for curves in thoracic region and by a factor of 0.24 for curves in the lumbar region. This implies that mechanical measures to correct this spinal deformity or to prevent progression should apply different rotations to the apex from those applied to the curve as a whole and, in opposite senses, in curves in kyphotic regions. There was no evidence of an abnormality of sagittal curvature of a magnitude to implicate it in the etiology or in the treatment.     

The relationship between jaw deformity and scoliosis

Objectives To analyze the relationship between lateral displacement of the mandible and scoliosis. Methods From April 2002 through July 2003, we examined posteroanterior cephalometric radiographs and chest X-rays from 85 patients with jaw deformities and a control group of 20 patients with no jaw deformities. To measure the lateral shift of the mandible, we drew a horizontal baseline (X axis) on the cephalogram connecting the intersection of the external margins of the orbits and the most lateral points of the greater wings of the sphenoid. A vertical baseline (Y axis) was then marked perpendicular to the X axis, intersecting the ethmoid crista galli. Then, we measured the lateral displacement of the mandibular mentum from the Y axis. Displacement to the right was designated positive; that to the left was designated negative. Cobb's method was used to measure scoliosis curves on chest X-rays; the direction of the curve was designated similarly. Results Of the 85 patients with jaw deformity, 23 (27.1%) had a Cobb angle exceeding 10°. None of the control group had scoliosis exceeding 10°. No correlation was found between the direction of mandibular displacement and the direction of scoliosis. Conclusion This study suggests a relationship between jaw deformities and scoliosis, as scoliosis was found in 27.1% of the patients with a main complaint of jaw deformity.     

旋转复位配合矫形鞋治疗退变性腰椎侧弯

退变性腰椎侧弯(degenerative lumbar scoliosis,DLS)是继发于腰椎间盘及腰椎骨关节退变的成人脊柱侧弯,较一般典型的腰椎骨性关节炎症状重,同时伴有明显旋转侧弯畸形,常规治疗方法疗效差。2003年9月-2005年9月采用脊柱(定点)旋转复位手法结合矫形鞋治疗77例DLS患者,近期效果满意,总结报告如下。1临床资料本组77例中,男29例,女48例;平均年龄56.5岁(45~76岁)。病程平均6个月(2个月~10年)。所有患者均有腰痛及下肢疼痛,同时伴有明显的腰脊柱侧弯,站立、行走疼痛明显,其中48例(82.3%)需要药物控制疼痛,间歇性跛行者26例(33.8%),直腿抬高试验…     

特发性脊柱侧凸患者椎旁肌中神经营养素3的表达及意义

目的:研究特发性脊柱侧凸(IS)患者椎旁肌组织中神经营养素3(Neurotrophin-3,NT-3)在核酸水平(mRNA)的表达情况。方法:随机选择19例IS患者,年龄12～19岁,平均14.3岁,Cobb角42°～80°(平均54°)。手术中取顶椎(T7～T10)凸侧、凹侧椎旁肌肉组织,采用半定量RT-PCR法检测NT-3 mRNA表达水平。取4例相同年龄段的椎间盘突出患者手术切口头端非病变部位椎旁肌肉组织作为对照。结果:对照组椎旁肌中NT-3的mRNA表达阳性率50%,相对表达量为0.0237±0.0158,IS组患者椎旁肌中表达阳性率63.16%,相对表达量为0.1213±0.0939,较对照组表达量增加(P=0.051)。9例Cobb角>50°的IS患者表达阳性率为44.44%,相对表达量0.0431±0.0359,和对照组无明显差异;10例Cobb角≤50°的IS患者中表达阳性率为80%,相对表达量为0.1604±0.0895,与对照组和Cobb角>50°的IS患者比较有明显增加(P<0.01)。结论:正常椎旁肌组织中NT-3在核酸水平微量表达,特发性脊柱侧凸患者椎旁肌组织中NT-3 mRNA表达增加,尤其在小角度IS患者中增加明显,提示IS患者中存在神经营养因子表达异常。     

前后路联合三维矫形手术治疗青少年特发性僵硬型胸椎侧凸

目的:评价前路松解联合后路三维矫形治疗青少年特发性僵硬型胸椎侧凸的效果,探讨术中、术后并发症的预防措施。方法:青少年特发性僵硬型胸椎侧凸34例,男5例,女29例;年龄8～21岁,平均14.7岁。均为右侧凸。均采用经前路松解植骨融合联合后路三维矫形内固定治疗,其中前路经胸入路26例,胸腔镜前路松解植骨融合矫形术8例。随访时均摄全脊柱X线片。术前、术后所摄X线片进行以下各项测量指标比较:Cobb角、顶椎的旋转度、下融合椎旋转度、躯干偏移距离。随访时观察有无脊柱失平衡、假关节形成及其他并发症的发生。结果:全部病例均获随访,时间12～39个月,平均22个月。所有患者Cobb角术后平均矫正率80.62%,其中18例患者出现矫正度数丢失,丢失度数3°～10°,平均4.3°(术后平均14.2°,1年后随访平均18.5°)。所有患者术后胸椎生理性后凸得到重建。术后胸椎融合弯顶椎的旋转改善率62.91%,下融合椎旋转改善率47.60%,躯干偏移平均距离3.3mm,未见脊柱失平衡现象。围手术期并发症包括低氧血症1例,胸腔积液2例,均经保守治疗后痊愈。术后6个月肺功能检查,未见有明显下降。脊柱胸弯融合段未发现假关节形成。结论:前路松解联合后路矫形内固定植骨融合治疗青少年特发性僵硬型胸椎侧凸可明显改善顶椎的旋转度,得到满意的三维矫形效果。术前积极准备、合理手术设计,术中严格操作,术后重视预防并发症能减少手术并发症的发生。     

前路半椎体切除截骨后路矫形固定治疗严重先天性脊柱侧凸

目的探讨分期前路半椎体切除截骨后路矫形手术治疗严重先天性脊柱侧凸的临床疗效。方法严重先天性脊柱侧凸14例,男4例,女10例;年龄8～13岁,平均11.2岁。其中形成缺陷2例,分节缺陷6例,混合型6例。半椎体位于T53例,T2、T8、T10、T11、L1、L4各1例。胸弯12例,胸腰弯2例。术前冠状面Cobb角63°～95°,平均72.1°。胸椎后凸减小或前凸8例,胸腰段后凸4例。一期采用前路半椎体切除、多节段椎体间楔形截骨及分节不全松解;二期采用后路椎弓根钉棒系统三维矫形固定。前后路手术均植骨,平均截骨5.5个节段。结果一期前路术后冠状面Cobb角48°～60°,平均51.5°;矫正率19.6%～37.8%,平均28.6%。二期术后冠状面Cobb角5°～45°,平均30.5°;矫正率52.6%～87.5%,平均62.5%。8例存在胸椎后凸减小或前凸的患者均恢复生理性后凸,4例胸腰段后凸患者3例达到矢状面矫正。全部病例随访8～30个月,平均12.1个月。无断钉、断棒及明显的矫正度丢失,植骨融合良好。发生并发症2例,椎弓根钉帽松动1例,T1神经根激惹1例。结论一期前路半椎体切除、多节段椎体间楔形截骨、分节不全松解,二期后路矫形固定及前后路植骨治疗先天性脊柱侧凸能够达到较满意的矫形效果,适合于8～12岁的青春期前患者。     

Scofix系统治疗特发性脊柱侧弯18例

青少年特发性脊柱侧弯是临床上比较常见的一种脊柱畸形 ,自 1998年 4月 - 2 0 0 2年 4月 ,我们采用Scofix内固定加植骨融合术治疗 18例 ,经随访 1～3年 ,疗效满意 ,现报告如下。1　临床资料　　本组 18例 ,男 8例 ,女 10例 ;年龄 11～ 16岁 ,病程 4个月～ 12年。按照国际脊柱侧弯研究会推荐的King方法分型[1] :Ⅰ型 2例 ;Ⅱ型 4例 ;Ⅲ型 7例 ;Ⅳ型 5例。脊柱侧弯及旋转按Cobb角及More法定量评估并计算弹性指数 :Cobb角为 4 0°～ 70° ,平均 5 2° ;旋转 0°～ 3° ,平均 2° ;弹性指数 10 %～ 38% ,平均2 9 %。所有病例手术均一次完成。2…