家族性肌萎缩侧索硬化动物模型脊髓侧角神经元特征

目的探讨脊髓侧角神经元在SOD1G93A转基因鼠发病过程中的变化特征。方法利用甲苯胺蓝染色及免疫组化的方法观察SOD1G93A转基因小鼠不同病变时期上、中、下胸髓侧角神经元的数量变化特征。结果甲苯胺蓝染色法定量研究发现不同时期SOD1G93A转基因小鼠上胸髓、下胸髓水平,症状前期和发病期神经元计数较终末期均有显著性差别(P<0.05);与对照组相比,终末期胸髓各段侧角神经元计数明显减少且有统计学意义;免疫组化发现SOD1G93A转基因小鼠上胸髓、中胸髓水平不同时期相比,症状前期和发病期乙酰胆碱酯酶(AchE)阳性神经元计数较终末期均有显著性差别(P<0.05);与对照组相比,发病期上胸髓及终末期中、下胸髓水平侧角AchE阳性神经元数量减少具有统计学意义;结论在SOD1G93A转基因鼠发病过程中,存在脊髓侧角神经元丢失,尤其是在疾病的终末期,上、中、下胸髓节段间无明显差异。     

涉及后柱胫骨平台骨折的不同治疗策略

目的探讨涉及后柱胫骨平台骨折的治疗策略。方法自2009-07—2014-10收集206例胫骨平台骨折的临床资料,研究涉及后柱的胫骨平台骨折的发病率。采用Schatzker法及罗从风三柱法分别对骨折进行分类,后柱再分为后内侧柱和后外侧柱,筛选出涉及后柱的胫骨平台病历。根据后柱骨折的类型,选择合适的手术方案。结果 206例获得随访平均15.6(12~36)个月。150例骨折涉及后柱,其中130例涉及后外侧柱,97例涉及后内侧柱。206例无一例出现血管、神经损伤、内固定物松动及断裂,术后12个月复查骨折均获得骨性愈合。术后1年复位不良者3例。结论涉及后柱的胫骨平台骨折发病率高,应根据骨折类型,采用合适的切口、内固定方式等,以获得良好治疗效果。     

后路全椎节切除、双轴旋转矫形手术治疗重度陈旧结核性后凸的疗效分析

目的:分析后路全椎节切除、双轴旋转矫形手术治疗重度陈旧结核性后凸的手术效果,探讨影响疗效的因素。方法:2004年5月~2011年9月,采用后路全椎节切除、双轴旋转矫形固定融合术治疗重度陈旧结核性后凸畸形患者33例。男15例,女18例;平均年龄34.7±14.1岁(11~63岁),其中年龄≥35岁18例,35岁15例;术前平均后凸角98.9°±18.2°(70.0°~130.0°),其中≥100°者17例,100°者16例;后凸顶点位于上中胸椎(T10及以上)19例,胸腰段及腰椎(T11及以下)14例。脊髓损伤Frankel分级A、B级各1例,C级5例,D级12例,E级14例。ODI术前平均19.5±9.4分。根据年龄、后凸顶点位置、后凸角度、术前Frankel分级、有无并发症等情况进行分组。对所有患者行手术前后及末次随访时的后凸局部和全脊柱正侧位X线摄片,测量后凸Cobb角,对合并侧凸的患者测量侧凸Cobb角。对手术前和随访时的患者进行脊柱矢状位平衡测量、实际身高测量、Frankel神经功能分级、Oswestry评分、VAS评分和手术PSI满意指数的评定。总结患者术中和术后并发症,并积极采取相应处理。结果:所有患者术前平均后凸Cobb角98.9°±18.2°,术后平均为30.9°±11.3°,矫正率为69.1%,末次随访时平均为34.8°±15.1°,矫正率为65.3%。患者脊柱矢状位平衡、身高测量、神经功能Frankel分级、生活质量Oswestry评分、腰背痛VAS评分均改善,手术总体满意率为87.9%。并发症发生率为54.5%,其中术中并发症12例、术后短期并发症2例、术后中远期并发症4例。所有并发症均及时采取相应处理。发生并发症者的总体后凸矫正率与未发生并发症者相当,但严重神经并发症发生者在神经功能恢复和生活质量改善方面均明显不如无并发症者。结论:后路全椎节切除、双轴旋转矫形手术是治疗重度陈旧结核性后凸畸形的有效方法,可以获得较好的手术效果,但需要尽量防止严重神经并发症的发生。     

大剂量氨甲环酸用于脊柱矫形手术的安全性和有效性

目的:评价大剂量氨甲环酸(TXA)应用于脊柱矫形手术尤其是经后路全脊椎切除术(PVCR)的安全性及有效性。方法:2009年2月~2010年10月收治脊柱畸形患者共66例,术前凝血功能检查异常及长期服用影响凝血功能药物的患者共7例被排除,最终59例患者纳入本研究。其中严重僵硬性脊柱畸形患者均接受一期PVCR,其余患者均接受一期单纯后路矫形融合术,所有手术均由同一位医师主刀完成。患者被分为TXA组和对照组。TXA组中8例接受PVCR,18例接受非PVCR矫形手术;对照组中9例接受PVCR,24例接受非PVCR矫形手术。TXA组患者在切皮前20min静脉输入100mg/kg剂量的TXA,随后给予维持量10mg·kg~(-1)·h~(-1)的TXA直到切口关闭;对照组给予等量的生理盐水替代。统计患者的术中失血量、真实失血量、输血量等数据,并监测患者围手术期的凝血功能、血红蛋白、红细胞比容等,同时监测药物相关并发症,包括潜在的肝、肾功能损伤,上/下肢静脉血栓,肺栓塞,心肌梗死及癫痫等。结果:TXA组中接受PVCR的患者术中失血量(4219±1386ml)、真实失血量[(134.2±36.4)%]及输血量(2986±1458ml)均少于对照组接受PVCR手术的患者[分别为9906±5251ml,(332.9±191.8)%,6255±3401ml,均P0.05]。TXA组中接受非PVCR矫形手术患者的术中失血量、真实失血量及输血量也均小于对照组非PVCR矫形手术患者(P0.05)。TXA组中PVCR术中失血量较对照组PVCR减少57.4%,而非PVCR手术术中失血量减少39.8%,大剂量TXA减少术中失血的作用在PVCR术中更为明显。TXA组及对照组围手术期肝、肾功能指标无明显差异(P0.05)。TXA组中未出现上/下肢静脉血栓、肺栓塞、心肌梗死、癫痫及急性肾功能衰竭。结论:大剂量TXA可有效减少脊柱矫形手术的术中失血与输血,尤其在PVCR手术中大剂量TXA的效果更为突出;大剂量TXA在脊柱矫形手术中的使用是安全的。     

双切口3至4块钢板固定治疗累及后内侧柱的复杂胫骨平台骨折15例疗效分析

目的 探讨双切口3至4块钢板固定治疗累及后内侧柱的Schatzker Ⅴ、Ⅵ型复杂胫骨平台骨折的临床疗效.方法 2013年1月至2015年12月采用前外侧联合后内侧倒L形双切口,外侧1块3.5 mm锁定钢板,内侧及后内侧2至3块3.5mm小钢板固定治疗累及后内侧柱的Schatzker Ⅴ、Ⅵ型复杂胫骨平台骨折患者15例,分析术后复位效果及随访时膝关节功能情况,评估手术临床疗效.结果 15例均获随访,随访时间18 ～30个月,平均24个月.术后骨折复位效果满意,末次随访时复位无丢失,骨折均愈合,膝关节活动度0 ～135°,平均115..膝关节功能采用HSS评分标准评定:优10例,良2例,可3例,差0例,优良率80％.结论 对于累及后内侧柱的SchatzkerⅤ、Ⅵ型复杂胫骨平台骨折,采用双切口,3至4块3.5 mm钢板固定,可以获得有效固定,膝关节功能恢复良好,并发症少,疗效满意.     

Arthroscopic Findings in Refractory Symptomatic Fourth and Fifth Tarsometatarsal Joints

Refractory pain to the fourth and fifth tarsometatarsal (TMT) joint can be a source of disability and functional impairment. While pain has been attributed to injury, post-traumatic arthritis, arthrofibrosis, the principal causes of pain in the absence of arthritis are not well elucidated. The purpose of this study is to characterize arthroscopic pathology associated with chronic refractory pain to the fourth and fifth TMT joints. We retrospectively examined 24 patients that underwent arthroscopic surgery of the fourth and fifth TMT joints for refractory pain at our academic institution between 2015 and 2019. We used the Outerbridge classification for chondral lesions, the Kellgren Lawrence radiographic classification for osteoarthritis, and described intraarticular pathologies as acute hypertrophic synovitis, chronic synovial fibrosis, hyaline bands, meniscoid bodies, loose joint bodies, arthrofibrosis. Approximately, 31 of 45 TMT joints (68.9%) presented with radiographic evidence of arthritis. Approximately, 14 of 45 TMT joints (31.11%) were absent of radiographic signs of arthritis. The frequency of soft tissue pathology seen in these patients without radiographic evidence of arthritis was arthrofibrosis (87.5%), chronic synovial fibrosis (75.0%), and acute hypertrophic synovitis (62.5%). This is the first study to report arthroscopic pathologies associated with refractory pain to the fourth and fifth TMT joints.     

胫骨平台骨折的手术治疗策略分析

目的探讨分析手术治疗胫骨平台骨折的策略。方法回顾性分析我院在2011年3月至2013年10月收治的30例胫骨平台骨折患者的手术治疗情况,其中13例患者行双柱支撑固定植骨手术治疗,17例患者行单柱支撑固定植骨手术治疗。结果本组所有患者术后经过1²年时间随访,参照Rasmussen膝关节功能评分,结果治疗优良者26例,优良率为86.7%,其中优17例,良10例,可2例,差1例。结论在确保骨折部良好血运的情况下,首先解剖复位胫骨平台,并进行支撑固定植骨手术治疗的手术治疗效果显著,固定更加持久、稳固,是一种安全有效的手术方法,值得在临床上进一步推广。     

The risk factors of neurologic deficits of one-stage posterior vertebral column resection for patients with severe and rigid spinal deformities

Purpose

To determine the risk factors of neurologic deficits during PVCR correction, so as to help improve safety during and after surgery.

Methods

A consecutive series of 76 patients with severe and rigid spinal deformities who were treated with PVCR at a single institution between October 2004 and July 2011 were included in our study. Of the 76 patients, 37 were male and 39 female, with an average age of 17.5 years (range 10–48 years). There were 52 adolescent patients (with an age <18 years) and 24 adult patients (with an age ≥18 years). Preoperatively, postoperatively and 6 months after surgery, we performed systemically neurologic function evaluations of each patients through meticulous physical examination. Any new abnormality or deterioration in evaluation of neurologic function than preoperative is reckoned postoperative neurologic deficits. Ten variables that might affect the safety of neurologic deficits during PVCR procedures, including imaging factors, clinical factors and operational factors, were analyzed using univariate analysis. Then the variables with statistical difference were analyzed by using multi-factor unconditional logistic regression analysis.

Results

No patient in this series had permanent paraplegia and nerve root injury due to operation. Change of neurologic status was found in six patients after surgery. Results of single-factor comparison demonstrated that the following seven variables were statistically different (P < 0.05): location of apex at main curve (X ₃), Cobb angle at the main curve at the coronal plane (X ₄), scoliosis associated with thoracic hyperkyphosis (X ₅), level of vertebral column resected (X ₆), number of segmental vessels ligated (X ₇), preexisting neurologic dysfunction (X ₈), and associated with intraspinal and brain stem anomalies (X ₉). The multi-factor unconditional logistic regression analysis revealed that X ₈ (OR = 49.322), X ₉ (OR = 18.423), X ₅ (OR = 11.883), and X ₆ (OR = 8.769) were independent and positively correlated with the neurologic deficit.

Conclusions

Preexisting neurologic dysfunction, associated with intraspinal and brain stem anomalies, scoliosis associated with thoracic hyperkyphosis and level of vertebral column resected are independent risk factors for neurologic deficits during PVCR procedure.     

Clinical,hematological, and imaging observations in a 25-year-old woman with abetalipoproteinemia

Abetalipoproteinemia is an uncommon cause of ataxia and retinitis pigmentosa (RP). Most of the neurological and ocular manifestations occur secondary to deficiency syndromes that is consequent to fat malabsorption from the small intestine. In this report, we have described the phenotype of a young adult female who manifested with recurrent diarrheal illness in her first decade, followed by anemia, RP, and neurological involvement with progressive deafness, cerebellar and sensory ataxia, and subclinical neuropathy in her second decade of life. While RP and sensory ataxia due to vitamin E deficiency are well-recognized features of abetalipoproteinemia, deafness is rarely described. In addition, we have highlighted the abnormal posterior column signal changes in the cervical cord in this patient. Early recognition avoids unnecessary investigations and has a potential to retard the disease progression by replacing some of the deficient vitamins.     

脊柱外周型原始神经外胚层肿瘤影像学表现(7例报告)

目的观察脊柱外周型原始神经外胚层肿瘤(pPNET)的影像学表现。方法回顾性分析7例经病理证实的脊柱pPNET患者的影像学资料。结果 7例脊柱pPNET病例中,男性4例,女性3例。病变位于颈段(C2~7)1例,颈胸段(C7~T2)1例,胸段(T10~12)1例,腰段(L3~4、L4~5)2例,腰骶段(L1~S2)1例,骶段(S1~4)1例;其中,髓外硬膜内1例,硬膜外6例。MR(n=7)和CT(n=4)检查表现为硬膜内或硬膜外肿块,信号/密度不均,T1WI为等或稍低信号,T2WI为稍高信号,CT为等密度,增强扫描呈不同程度不均匀强化。肿瘤位于髓外硬膜内者可伴有多发蛛网膜下腔转移,硬膜外pPNET肿瘤的主体部分多位于椎骨或椎旁组织,肿块椎管内部分累及多个椎体平面,并通过椎间孔、骶孔与椎旁部分沟通。结论脊柱pPNET可表现为髓外硬膜内或硬膜外肿瘤,确诊依赖病理及免疫组化检查。