重视青少年特发性脊柱侧凸病因机制中的基因学研究

青少年特发性脊柱侧凸(adolescent idiopathic scoliosis,AIS)是发生于青春期发育前后的最常见的脊柱畸形,其病因不明.目前其病因学研究主要包括遗传学机制、激素的作用、结缔组织异常、生长不对称及生物力学作用等,但并无统一的病因学解释.     

青少年特发性脊柱侧弯研究国外进展

青少年特发性脊柱侧弯(adolescent idiopathic scoliosis,AIS)还有很多争论和未解决的问题,最困难的问题是病理发病机制不清楚.尽管支具治疗已经作为有曲线进展危险的侧弯患者标准的治疗方法,无论在功效和作用方面还不能经历严格的评价.随着对手术治疗的重视,技术大大改进,增加了手术安全性,保持脊柱的平衡,但是这些治疗方法长期的效果还不得知;外科手术可以减少畸形,防止曲线进一步进展,因此可以减少将来心肺功能出问题的危险,但是长期防止脊柱侧弯的其他负面作用还不清楚.本文讨论一下目前关于青少年特发性脊柱侧弯病理发病机制,自然病史,非手术治疗和手术治疗概念.     

单胸弯及单腰弯特发性脊柱侧凸的躯体生长参数研究

 目的 探讨单胸弯及单腰弯青少年特发性脊柱侧凸(adolescent idiopathic scoliosis, AIS) 女性患者的躯体生长发育特点。方法 研究对象包括 339例 AIS女性患者和 3914位同龄健康女孩, 实 足年龄 10~17岁。单胸弯 AIS女孩(端椎位于 T₁~T₁₂之间)220例, 单腰弯 AIS女孩(端椎位于 T₁₂~L₅之 间)119例;Cobb角为 32°±10°(15°~63°)。测量所有 AIS组和对照组女孩的身高、体重及臂长, 计算体重 指数(body mass index, BMI)及下肢长度, 并根据 Bjure校正公式计算 AIS女性患者的校正身高及校正 坐高。结果 单胸弯与单腰弯 AIS患者的身高及坐高在各年龄段间差异均无统计学意义;而两组 AIS 女性患者的校正身高及坐高均明显高于同龄对照组女孩。同时两组 AIS患者的臂长及下肢长度也显著 长于正常青少年。在 15岁年龄段, 单胸弯 AIS患者的体重显著低于正常青少年, 而单腰弯 AIS患儿的 体重在各年龄段均与正常青少年相似。结论 与同年龄段正常青少年相比, 单胸弯和单腰弯 AIS患者 可能具有共同的异常生长模式;此外, 与正常女孩及单腰弯 AIS女孩相比, 单胸弯 AIS患者体内脂肪含 量偏低的趋势更为明显。     

Selective fusion for adolescent idiopathic scoliosis: a review of current operative strategy

Selective fusion of thoracic and thoracolumbar/lumbar curves in adolescent idiopathic scoliosis is a concept critically debated in the literature. While some surgeons strongly believe that a more rigid and straighter spine provides predictably excellent outcomes, some surgeons recommend a mobile and less straight spine. This topic is a crucial part of surgical treatment of idiopathic scoliosis, particularly in young patients who will deal with the stress of the fusion mass at the proximal and distal junctions over many years. This study will review the literature on various aspects of selective fusion.     

Pathological mechanism of idiopathic scoliosis: experimental scoliosis in pinealectomized rats

The pathological mechanism of curve progression in idiopathic scoliosis is still obscure. In this study we investigated the pathological mechanism of idiopathic scoliosis in experimentally induced scoliosis in rats. A total 30 rats were divided into three groups: ten bipedal rats with a sham operation, which served as the control; ten quadrupedal rats with pinealectomy; and ten bipedal rats with pinealectomy. Scoliosis developed only in pinealectomized bipedal rats and not in pinealectomized quadrupedal rats. Cervicothoracic lordosis developed in bipedal rats with or without pinealectomy. These deformities of lordoscoliosis in pinealectomized bipedal rats were similar to human idiopathic scoliosis. Lordosis or lordotic tendency was sufficient to cause the spine to rotate to the side. Rotational instability of the spine with rotation of lordotic segment appears to produce a characteristic scoliotic deformity as a secondary phenomenon. Our findings suggest that lordosis may develop in bipedal rats, but pinealectomy is required for the development of lordoscoliosis. Balanced muscle tone controlled by the postural reflex is important to maintain normal posture with a straight spine in the bipedal condition. The disturbance of equilibrium and other postural mechanisms secondary to a deficiency of melatonin after pinealectomy may promote development of lordoscoliosis with vertebral rotation especially in the bipedal posture.     

Proprioception in idiopathic scoliosis

Proprioception testing and gait analysis was performed on a group of 17 patients with idiopathic scoliosis. All curves had documented progression. The average curve was 26.8 degrees, all primary curves being right thoracic. The average age was 14.8 years. Twelve, healthy, age-matched controls underwent identical testing. Results showed that scoliotic subjects had asymmetry in their ability to reproduce angles between their two knees, as well as an absolute deficit in their ability to reproduce angles as compared with controls (P less than 0.01). The threshold of detection of change in angle at the knee was also quantitatively higher than controls (P less than 0.05), although asymmetry was not statistically significant. No significant differences were measured in gait parameters between scoliotics and controls. These joint position tests have been applied previously to young and elderly adults, athletes, and total joint patients. In no group has any asymmetry of response been demonstrated, both extremities showing virtually identical performance in each case. The tests administered measure sensory modalities, which are conducted through the posterior columns. Although the site of damage in the neural pathway cannot be specifically localized by these tests, results of this study support the presence of a neurologic deficit in idiopathic scoliosis.     

Adolescent idiopathic scoliosis

Conservative treatment with a brace continues to remain controversial. A recent study on the efficacy of brace treatment by the Scoliosis Research Society Study Group found it to be efficient, however, and recommends it. Surgical treatment of adolescent idiopathic scoliosis must be tailored to each individual need, type of curve, and surgeon's expertise. A straighter curve does not necessarily mean better for each curve. Recent advances in pedicle screw instrumentation, although powerful in the rate of correction achieved, carry a definitive increase in neurologic risk and must be used only when justified and by experienced teams.     

Review of current technologies and methods supplementing brace treatment in adolescent idiopathic scoliosis

Purpose

To report on the current technologies and methods supplementing brace treatment in adolescent idiopathic scoliosis.

Methods

A comprehensive literature review was performed to determine the effectiveness of bracing, to report on imaging techniques that can assist in the assessment of bracing, to understand the roles of the biomechanical treatment concepts on bracing and to address the importance of the quality of life of the brace wearers.

Results

The effectiveness of bracing still remains controversial. Many technologies are still in development to improve the bracing process and quantify the effects of bracing. Imaging techniques with decreased or no radiation are promising in providing more frequent data on curve progression for patients. Computer-assisted design models have been used for both fitting and manufacturing the brace to patient contours. Ultrasound has been developed as a new means of diagnosing scoliosis and determining the effects of a brace on a patient’s spine in real time. The brace treatment outcomes are correlated to the quantity and the quality of brace usage. Compliance monitors and force sensors have been developed to track the quality of brace usage. Improvements to brace wear also require consideration of patient quality of life. Surveys have been developed to describe the effects of family influence and self-image on bracing effectiveness of patient quality of life.

Conclusions

Bracing remains a highly qualitative process, relying on the empirical judgment of the physicians and orthotists, along with buy-in with the patient. The suggested improvements will help to push bracing into a more evidence-based practice.     

Infantile idiopathic scoliosis

This review of infantile idiopathic scoliosis highlights the clinical features, etiology, epidemiology, and treatment considerations that clearly distinguish this entity from the more common diagnosis of juvenile and adolescent idiopathic scoliosis. A comprehensive understanding of infantile idiopathic scoliosis provides the basis for reliable prediction of those curves that are likely to spontaneously resolve and those that will relentlessly progress if left untreated.     

青少年特发性脊柱侧凸患者心脏结构和功能的研究

目的 总结AIS患者心脏结构和功能的特点.探讨其影响因紊.方法 回顾分析2003年1月～2005年7月北京协和医院收治的104例主弯为胸弯的患者经全脊柱X线片、CT三维重建以及术中确诊的AIS患者的术前心脏彩色超声资料,其中男性21名,女性83例,年龄10～20岁,平均14.63岁.根据顶椎旋转度、侧弯方向、冠状面角、矢状面角以及侧弯分型分组,对反映AIS患者心脏结构和功能的各项指标进行比较.结果 左侧弯患者与右侧弯患者之间舒张末期左室内径(45.36±3.23 vs 42.02±3.74)、收缩末期左室内径(29.21±3.12 vs 26.49±2.85)以及主肺动脉径(19.71±1.49 vs 18.67±1.80)存在显著性差异(P＜0.05);冠状面角＜45.组与冠状面角＞80°组患者之间的右室内径(17.34±3.71 vs 20.55±4.74)以及冠状面角介于45.一80.组患者与冠状面角＞80.组患者之间的收缩末期左室内径(26.42±3.22 vs 28.73±4.03)存在显著差异(P＜0.05);矢状面角＜20°组与矢状面角＞40°组患者之间的舒张末期左房内径(27.00±3.47 vs 30.30±3.81)以及矢状面角＜20°组患者与其他两组患者之间的主动脉根径(23.89±2.06 vs 25.70±2.60,25.85±2.11)存在显著差异(P＜0.05);冠状面角＞80°组患者与其他两组患者之间的左室射血分数(62.62±5.79 vs 66.30±4.05,67.96±5.12)以及冠状面角＞80°组患者与冠状面角介于45°～80°组患者之间的左室短轴缩短率(33.82±4.17 vs 37.67±4.08)存在显著性差异(P＜0.05).顶椎旋转度、侧弯方向、矢状面角以及侧弯分型不同的患者之间,心脏射血分数及左室短轴缩短率没有显著性差异.结论 虽然AIS患者心脏结构和功能参数整体处于正常范围,但侧弯方向、冠状面角以及矢状面角仍然对AIS患者的心脏结构和功能产生影响.