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.     

Introduction and evaluation of a novel multi-camera surface topography system

BackgroundSurface topography can be used for the evaluation of spinal deformities without any radiation. However, so far this technique is limited to posterior trunk measurements due to the use of a single posterior camera.Research questionPurpose of this study was to introduce a new multi camera surface topography system and to test its reliability and validity.MethodsThe surface topograph uses a two-camera system for imaging and evaluating the subjects front and back simultaneously. Inter- and intra-rater reliability was tested on 40 human subjects by two observers. For validation human, subjects were scanned by MRI and surface-topography. For additional validation we used a phantom with an anthropomorphic body which was scanned by CT and surface topography.ResultsInter- (0.97−0.99) and intra-rater reliability (0.81−0.98) testing revealed good and excellent results in the detection of the body surface structures and measurement of areas and volumes. CT based validation revealed good correspondence between systems in the imaging and evaluation of the phantom model (0.61–10.52 %). Results on validation of human subjects revealed good to moderate results in the detection and measurements of almost all body surface structures (1.36–13.34 %). Only measurements using jugular notch as a reference showed moderate results in validity (0.62–27.5%) testing.SignificanceWe have introduced a novel and innovative surface topography system that allows for simultaneous anterior and posterior trunk measurements. The results of our reliability and validity tests are satisfactory. However, in particular around the jugular notch region further improvements in the surface topography reconstruction are needed.     

EOS® imaging: Concept and current applications in spinal disorders

EOS® imaging is a proprietary imaging technology that was launched in 2007. Based on a gaseous particle detector with a multi-wire proportional chamber, it offers several advantages over other imaging modalities: low dose of radiation, ability to create 3D reconstructions, ability to conduct whole body imaging, high reproducibility in measuring various parameters of alignment and faster imaging time. EOS® imaging is slowly gaining widespread acceptance as its applications in various disorders continue to evolve. It has been found to be particularly useful and has opened up new avenues of research in the field of spinal deformities. This narrative review seeks to provide an overview of the proprietary technology behind EOS® imaging, compare it to existing imaging modalities, summarize its current applications in various spinal disorders and outline its limitations.     

Comment vieillit-on en institution avec une scoliose ? Impact de l’arthrodèse vertébrale sur la qualité de vie des paralysés cérébraux adultes

IntroductionQuality of life is now part of the discussion, when considering spinal arthrodesis in children and teenagers with severe cerebral palsy and scoliosis. From the subjective point of view of close caregivers, quality of life is significantly improved two to five years after surgery. Nevertheless information relevant to the long-term evolution of those patients, most of them institutionalized, is lacking. In this study the quality of life of 45 adult cerebral palsy subjects with scoliosis (20 with arthrodesis and 25 without), classified IV or V on the Gross Motor Classification System and living in institutions was questioned.Material and methodFour items: daily care, pain/comfort, sitting position and communication, were proposed to evaluation by professional caregivers, blind to the final goal of the study and asked to answer along a Lickert scale (−2 to +2).ResultsResults show that professional caregivers dont make any difference between operated and non operated patients when considering easiness of daily care, pain/comfort of the resident or ability to communicate, but judge sitting position to be slightly better for those with spinal arthrodesis. Satisfaction with sitting posture depends for one part on the degree of scoliotic curve but other factors (hip and knee rigidity, quality of the sitting orthosis) also seem to be relevant.ConclusionSpinal arthrodesis is beneficial for sitting posture, even in aging subjects with severe cerebral palsy and scoliosis living in institutions. But the study also points to the numerous other health challenges which negatively affect the quality of life of such patients.     

Evidence of impaired pain modulation in adolescents with idiopathic scoliosis and chronic back pain

BACKGROUND CONTEXT

Although 40% of adolescent idiopathic scoliosis (AIS) patients present with chronic back pain, the pathophysiology and underlying pain mechanisms remain poorly understood. We hypothesized that development of chronic pain syndrome in AIS is associated with alterations in pain modulatory mechanisms.

3D打印模型在儿童先天性脊柱侧凸治疗中的应用研究

目的探讨3D打印模型在儿童先天性脊柱侧凸治疗中的应用疗效。方法回顾性分析2015年12月至2016年12月于首都医科大学附属北京儿童医院接受手术治疗的83例先天性脊柱侧凸患儿临床及影像学资料,根据是否使用3D打印模型分为试验组(n=44)和对照组(n=39),比较两组手术时间、术中透视次数、置钉准确性、矫形效果及并发症情况。结果本研究共纳入83例先天性脊柱侧凸患儿,其中男童47例,女童36例,平均手术年龄5.98 (1.5~13)岁。行半椎体切除术40例,Ponte截骨术31例,经椎弓根截骨术8例,全脊椎切除术4例。随访时间24~35个月。试验组患儿手术时间短于对照组[(180.92±16.74) min (201.51±27.60) min],差异有统计学意义(t=2.798,P<0.05),试验组术中出血量[(340.23±89.52) mL vs.(392.64±100.41) mL]及术中透视次数(4.36±0.89 6.05±1.28)明显少于对照组,差异有统计学意义(P<0.05);试验组置钉准确率高于对照组(93.55%vs 79.91%)(X^2=218.00,P<0.05),平均置钉时间明显短于对照组[(4.24±1.05) min vs.(8.35±2.29) min],差异有统计学意义(t=10.71,P<0.05)。两组患儿矫形率无明显差异(t=-1.135,P=0.272)。试验组与对照组患儿术后胸膜损伤、硬膜损伤发生率无明显差异(P>0.05)。结论 3D打印模型能清晰、直观显示先天性脊柱侧凸患儿的脊柱结构及形态,为制定手术计划及手术置钉提供帮助,从而提高置钉准确率,减少术中透视次数,缩短手术时间,减少术中出血量。     

石膏矫形治疗幼儿特发性脊柱侧弯的临床研究

目的介绍简易石膏床方法行石膏矫形治疗幼儿特发性脊柱侧弯,并进行效果评价。方法选取2014年9月至2016年9月于河北省儿童医院住院治疗的25例幼儿特发性脊柱侧弯病例作为研究对象,平均年龄29.7个月(17~53个月),其中男童10例,女童15例,共行石膏矫形105次。应用去旋转石膏矫形方法并结合我院实际情况采用简易石膏床对患儿进行石膏矫形,测量并记录石膏矫形前后脊柱正位X线片上Cobb角改变情况,并对所有患儿按照性别及年龄是否≤2岁进行分组分析。结果 25例患儿石膏矫形治疗前脊柱侧弯Cobb角平均角度为(39.1±9.1)°,经过第1次石膏矫形后平均角度为(22.3±5.0)°,末次随访时平均角度为(20.3±4.3)°。第1次石膏矫形后Cobb角与矫形前相比差异有统计学意义(t=8.825,P<0.05)。随访终点时Cobb角与矫形前相比差异有统计学意义(t=9.653,P<0.05)。不同性别分组随访终点时Cobb角矫正度数差异无统计学意义(t=0.070,P=0.945)。不同年龄分组随访终点时Cobb角矫正度数差异无统计学意义(t=-0.733,P=0.471)。结论我们采用的简易石膏床方法结合去旋转技术进行石膏矫形可以纠正幼儿特发性脊柱侧弯患儿的Cobb角,性别和年龄因素未对矫正效果产生影响。     

高海拔地区脊柱侧凸患者心肺功能变化

目的评估高寒缺氧环境对脊柱侧凸患者心肺功能的影响。方法回顾分析2006年1月~2012年12月本院收治的高海拔地区特发性脊柱侧凸患者31例,并选取同时期来自平原地区的其他特征(年龄、性别、侧凸角、后凸角)相似特发性脊柱侧凸患者31例作配对研究。收集并分析62例患者术前站立位全脊柱正侧位X线片、术前肺功能和心脏彩超检查结果。结果高海拔地区脊柱侧凸患者心脏彩超结果中每搏输出量(stroke volume,SV)、射血分数(ejection fraction,EF)、左房内径(left atrial internal diameter,LAID)、左室内径(left ventricular internal diameter,LVID)、右房内径(right atrial internal diameter,RAID)以及肺功能检查中肺活量(vital capacity,VC)、肺总量(total lung capacity,TLC)、用力肺活量(forced vital capacity,FVC)、一秒率(FEV1/FVC%)大小与平原地区脊柱侧凸患者检查结果相比差异无统计学意义(P0.05)。而高海拔地区与平原地区脊柱侧凸患者的右室内径(right ventricular internal diameter,RVID)、第一秒用力呼气容积(forced expiratory volume in one second,FEV1)实测值相比,差异具有统计学意义(P0.05)。结论高海拔地区与平原地区脊柱侧凸患者的心肺功能总体上无明显差异,在进行术前评估、准备和围手术期处理上,高海拔地区脊柱侧凸患者与平原地区脊柱侧凸患者相比并无特殊。     

退变性腰椎侧凸的手术治疗及疗效

目的探讨退变性腰椎侧凸的后路手术方法和手术疗效。方法回顾性分析2007~2012年本院手术治疗并获得随访的32例退变性腰椎侧凸患者的手术方法及疗效。患者均接受腰椎后路减压椎间融合器植骨融合内固定术,术后随访6个月~6年。采用下腰痛Oswestry功能障碍指数(Oswestry disability index,ODI)对患者手术前后的临床症状进行评分和疗效评价;通过手术前后冠状位和矢状位Cobb角度的比较,了解患者手术治疗的侧凸矫正率和腰椎前凸的恢复情况。结果患者ODI术前(52.30±13.65)%、术后(9.62±6.41)%,术前术后比较差异有统计学意义(P0.05),术后疼痛改善率为81.6%。32例患者中,24例疗效为优,6例疗效为良,2例疗效为可,术后疗效评价的优良率为93.8%。患者冠状位Cobb角术前21.99°±8.97°、术后6.84°±5.32°,术前术后比较差异有统计学意义(P0.05),侧凸矫正率为69.0%。患者腰椎前凸Cobb角术前13.80°±15.99°、术后24.95°±12.86°,术前术后比较差异有统计学意义(P0.05)。结论退变性腰椎侧凸采用腰椎后路减压椎间融合器植骨融合椎弓根螺钉内固定术治疗效果较理想,术后疗效满意。手术的关键是彻底减压、正确选择融合节段、重建退变节段椎间高度、矫正前凸减少和后凸畸形、纠正椎体侧方移位和侧凸畸形以稳定椎体。     

马方及类马方综合征脊柱侧凸与青少年特发性脊柱侧凸患者肺功能障碍的差异性比较

目的:比较马方和类马方综合征脊柱侧凸(Marfan syndrome and Marfanoid scoliosis,MMS)患者与青少年特发性脊柱侧凸(adolescent idiopathic scoliosis,AIS)患者肺功能的差异及影响因素。方法:1999年9月~2013年4月我院收治的以胸弯(冠状面)为主的马方及类马方综合征脊柱侧凸患者共85例,其中年龄为11~19岁且临床资料完整的患者共40例(MMS组),男14例,女26例;收集患者术前肺功能指标,包括第1秒最大呼气容积(FEV1)、用力肺活量(FVC)、最大用力呼气峰流量(PEF),数值采用实测值占预计值的百分比,分析肺功能指标与年龄、冠状面Cobb角、胸后凸Cobb角、胸弯柔韧度之间的关系。并与同期住院行脊柱侧凸矫形内固定术的相匹配的80例AIS患者(AIS组)的术前肺功能参数进行比较。采用曼-惠特尼U检验比较两组间的差异,并用Pearson相关性分析对两组患者肺功能指标与患者年龄及相关脊柱侧凸指标进行相关性分析。结果:MMS组患者中肺功能处于中、重度损害的比例(11/40)显著高于AIS组(5/80)(P0.05)。MMS组患者FEV1、FVC均明显小于AIS组患者(P0.05);PEF两组间差异无统计学意义。MMS组患者FEV1、FVC与冠状面Cobb角呈显著性负相关(r=-0.444、-0.524,P0.05);FEV1、FVC、PEF与年龄之间呈正显著性相关(r=0.363,0.326,0.348,P0.05);FVC与胸弯冠状面柔韧度之间呈显著性正相关(r=0.321,P0.05);FEV1、FVC与胸椎后凸角均无显著相关性。AIS组患者FEV1、FVC、PEF等指标与冠状面Cobb角呈显著性负相关(r=-0.338、-0.293、-0.253,P0.05);FEV1、PEF与年龄之间呈显著性正相关(r=0.286、0.341,P0.05);FEV1与胸后凸Cobb角之间呈显著性正相关(r=0.238,P0.05)。两组患者肺功能指标与相关指标间的相关性存在差异。结论:MMS患者肺功能损害较AIS患者严重,其肺功能主要受胸弯冠状面Cobb角、年龄共同影响。