The role of bilateral talectomy in the management of bilateral rigid clubfeet

Severe clubfoot is a common sequela of arthrogryposis multiplex congenita and myelomeningocele. Talectomy is a practical surgical procedure to assist in the correction of severe, rigid equinovarus foot deformity. However, the results of talectomy may be less predictable in rigid bilateral equinovarus foot deformity than in the treatment of unilateral clubfoot. To determine the outcome of talectomy in the treatment of bilateral rigid clubfoot, we reviewed the cases of all patients at our center who underwent bilateral talectomy. Seven children were treated for bilateral talipes equinovarus with talectomy. The patient group consisted of 3 children with arthrogryposis, 3 with myelomeningocele, and 1 with Duchenne muscular dystrophy. The age at surgery averaged 6 years and ranged from 1 year and 10 months to 15 years and 7 months. The length of follow-up averaged 5 years and 6 months and ranged from 3 years to 12 years and 6 months. Three feet had a good and 7 feet a satisfactory correction of the deformity. Four feet had poor results. Bilateral talectomy was found overall to be a successful and predictable procedure, with low morbidity for the attainment of functional feet.     

The contributions of anterior and posterior tibialis dysfunction to varus foot deformity in patients with cerebral palsy

BACKGROUND: According to traditional teaching, the posterior tibialis is the main cause of varus foot deformity in patients with cerebral palsy. However, the relative frequency of anterior and posterior tibialis dysfunction has only been reported with use of dynamic electromyography in relatively small series of patients, with contrasting results. The purpose of the current study was to determine the relative prevalence of posterior and anterior tibialis dysfunction with use of gait analysis in a large group of patients with cerebral palsy and varus foot deformity. METHODS: The muscular contributors to varus foot deformity in seventy-eight patients (eighty-eight feet) who had cerebral palsy were evaluated with use of computerized motion analysis and dynamic electromyography. Data also were examined to identify any relationships between the timing of varus during gait and the contributing muscle. RESULTS: The muscular contributor to varus deformity was the anterior tibialis in thirty feet, the posterior tibialis in twenty-nine feet, both the anterior tibialis and the posterior tibialis in twenty-seven feet, and another contributor in two feet. Seventy feet had varus deformity during both stance phase and swing phase. Of these seventy feet, twenty-five exhibited dysfunction of the anterior tibialis, twenty exhibited dysfunction of the posterior tibialis, and twenty-three exhibited dysfunction of both muscles. Therefore, the timing of varus was not predictive of the contributing muscle or muscles. CONCLUSIONS: The current study demonstrated a higher prevalence of anterior tibialis dysfunction, both alone and in combination with posterior tibialis dysfunction, as a contributor to pes varus in patients with pes varus and cerebral palsy than had been reported previously. Dynamic electromyography provides clinically useful information for the assessment of such patients.     

Recurrent heel ulcers with calcaneal osteomyelitis in myelomeningocele: Treatment by partial calcanectomy and posterior transfer of tibialis anterior tendon

OverviewCalcaneus deformity of the foot is common in patients with myelodysplasia, mainly due to muscle imbalance. This deformity, especially in ambulatory patients, can result in gait problems and development of pressure sores, which can be complicated by calcaneal osteomyelitis.Material and methodsThis retrospective cross-sectional study included 12 patients (18 feet), with calcaneus deformity due to myelomeningocele, and presented with penetrating heel ulcers complicated by calcaneal osteomyelitis. The mean age of the included cases was 11 years. The ulcers were unilateral in six patients and bilateral in six. Sensation was absent on the plantar aspect of the foot in all cases. The treatment was done in two stages; The first stage was eradication of infection and obtaining good soft tissue coverage, and the second stage was obtaining motor balance to achieve a more plantigrade and braceable foot.ResultsThe average follow-up period was 19.2 months. In the final follow-up, twelve feet were graded as good, five as fair and one as poor according to Legaspi grading system.ConclusionThe combination of partial calcanectomy and subsequent transfer of tibialis anterior tendon, to improve the foot position and gait, can efficiently prevent ulcer recurrence in myelomeningocele patients with calcaneal deformity.Level of clinical evidence: 4Retrospective case series study.     

(足母)外翻足外在肌功能的初步评价

目的: 应用肌电图测定足外在肌力变化, 初步探讨肌性因素在外翻成因及病变发展中的作用。方法: 外翻患者与正常足志愿者各 10足, 应用Keypoint四道程肌电诱发电位仪记录安静状态下胫前肌、胫后肌与腓骨长短肌的潜伏期与波幅变化。结果: 外翻足与正常足的腓骨长肌与胫后肌潜伏期与波幅的变化存在明显差异。结论: 外翻足患者存在足的外在肌功能紊乱, 可能在疾患发生、发展过程中发挥一定作用。     

Talectomy for equinovarus deformity in myelodysplasia

Nine patients with myelomeningocele (seventeen involved feet) had talectomy for the correction of equinovarus deformity. The age at surgery ranged from one year and eight months to seven years and four months old. The length of follow-up averaged seven years and four months and ranged from twenty-two months to twelve years. Fifteen feet had a good and two had a poor correction of the deformity of the hind part of the foot, the result being directly related to the intraoperative correction of the equinus deformity. The correction of the fore part of the foot was rated as good in eight, fair in one, and poor in eight feet. Residual deformity of the fore part of the foot compromised the functional result in six feet that had an acceptable correction of the deformity of the hind part.     

Posterior transfer of the anterior tibial tendon in children who have a myelomeningocele

Posterior transfer of the tendon of the anterior tibial muscle through the interosseous membrane to the calcaneus to prevent or correct a calcaneus deformity was performed in twenty patients (thirty-nine feet) who had a myelomeningocele. The average age of the patients at the time of the operation was 4.6 years, and they were followed for an average of six years. Satisfactory clinical and radiographic results were obtained in thirty-seven (95 per cent) of the thirty-nine feet. Two patients, one who was unable to walk and one who walked at home only, had a mild equinus deformity of the left foot. No patient had a clinical calcaneus deformity, but there was radiographic evidence of talipes calcaneus in one patient (two feet). The anterior tibial muscle functioned more consistently when the operation was performed after the patient was four years old and in patients who had a fifth lumbar or first sacral motor level.     

Experimental study of denervated rat muscle. Part I: Histochemical and electrophysiological observations of denervated rat muscle

Histochemical and electrophysiological observations were carried out on denervated rat muscles, mainly extensor digitorum longus muscle. The myofibrillar ATPase reaction was employed to classify the type of muscle fibers. Type 1 fiber, low ATPase activity, and type 2 fiber, high ATPase activity, were recognized. It is generally believed that denervation causes a preferential atrophy of type 2 fibers and these fibers may be more dependant on neural influence than type 1 fibers. But the result of this investigation revealed that the atrophy of type 1 fiber and the enlargement of type 2 fiber will be caused by denervation. The term of enlargement is not equivalent to hypertrophy. Enlargement means the increasing of diameter of muscle fiber reacting to the denervation in this experiment. It is deduced that type 1 fiber is affected more by neural control than type 2 fiber.     

遗传性运动和感觉神经性疾病的临床电生理学和组织病理学的研究

目的：评价骨矫形术治疗遗传性运动和感觉周围神经性疾病（HMSN）的疗效。方法：应用临床、电生理学和组织病理学的方法研究由HMSN V型所致进行性双足畸形。结果：HMSN V型临床表现为双足呈马蹄内翻畸形、腓骨肌萎缩并伴有脊髓椎体束征；电生理学检查下肢运动神经传导速度测不到、感觉神经传导速度减弱，上肢运动和感觉神经传导速度正常；腓肠神经光镜和电镜检查为髓神经纤维，其数量和无髓纤维的直径减少，神经轴索和髓鞘再生。结论：对该病的外科治疗是手术矫形。     

The results of transfer of the tibialis anterior to the heel in patients who have a myelomeningocele

To study the long-term utility of transfer of the tibialis anterior tendon to the heel in patients who have a myelomeningocele, we reviewed the cases of twenty-five patients who had reached maturity. Forty-six transfers had been performed in these patients, and all of the patients had been followed for at least twelve years. At follow-up, four of the forty-six transferred muscles were functioning in a foot that was plantigrade, eleven were functioning but the foot had a persistent calcaneal deformity, and seventeen transferred muscles had never functioned. Of the fourteen remaining feet, eight had required a release of the transferred tendon because of progressive equinus deformity after the transfer and six had a minor degree of the same deformity but additional surgical treatment was not deemed necessary for them. Patients who had been operated on after the age of five years generally benefited more from the operation, in that additional surgical procedures were not necessary and the transfer was functional at the time of our review. Most of the patients in whom an equinus deformity developed after the procedure were later found to have spasticity in some of the muscles of the leg. It is likely that these patients originally had unrecognized spasticity in the transferred muscles.     

班布特罗与当归补血汤对失神经肌肉萎缩防治作用的实验研究

目的：研究班布特罗及当归补血汤对防治失神经骨骼肌萎缩的作用效果及有无协同作用。方法 SD大鼠40只，随机分组，每组10只，建立失神经骨骼肌萎缩的实验模型。术后4周处死大鼠，双侧腓肠肌及心脏称重，测定肌肉SOD含量，测肌纤维直径，检测细胞凋亡情况。结果 各用药组与对照组肌总蛋白含量、SOD含量、肌纤维直径均无显著性差异（P〉0．05）。在排除个体体重差异后肌肉湿重亦无显著性差异。两种药物之间无协同作用。各组均可见凋亡细胞，荧光显微镜下肉眼观测凋亡细胞的数量无显著性差异。结论 班布特罗与当归补血汤在4周时无明显预防肌萎缩作用及协同作用；细胞凋亡在失神经肌萎缩中发挥一定的作用；本实验为探索药物长期应用防治失神经骨骼肌萎缩的研究提供借鉴。     

MSCs静脉移植对周围神经再生的作用

目的检测间充质干细胞(mesenchymal stem cells,MSCs)经静脉移植后在周围神经损伤模型内的迁移、分布情况,并评价其对轴突、靶器官的保护作用。方法经尾静脉注射BrdU标记的MSCs入大鼠坐骨神经损伤模型内(实验组)。术后自神经断端、肌肉组织取材检测BrdU阳性细胞,评价坐骨神经指数(SFI)、肌纤维横截面积及肌细胞凋亡数量。结果术后4、8周从神经断端、肌肉组织内检测到BrdU阳性细胞。实验组的SFI、肌纤维横截面积明显高于对照组(不注入MSCs),细胞凋亡数量低于对照组,两组差异均有统计学意义(P<0.05,P<0.01)。结论MSCs静脉移植至体内能归巢到损伤的神经断端、肌肉组织,具有促进轴突再生、延缓肌萎缩的作用。     

Motor neurotization by segmental epineurectomy and implantation: lateral muscular neurotization

In this study, we aimed to develop a new muscular neurotization technique, in which transection of the donor nerve is avoided. We investigated the outcome of insertion of the donor nerve within the denervated muscle following segmentary epineurectomy (lateral muscular neurotization). Thirty-five male Wistar rats were evaluated in five groups, including sham control, denervation control, denervation combined with segmentary epineurectomy, direct muscular neurotization, and lateral muscular neurotization. Electromyography, muscle weight measurements, and histological evaluations were performed at postoperative months 2 and 3. The denervation group was statistically successful for denervation as compared with the sham control group for all parameters. The lateral neurotization group was successful in preventing muscle atrophy and gaining reinnervation in electromyographic, histological, and weight parameters. The direct neurotization group was also successful in histological and weight parameters. Lateral muscular neurotization is promising because it does not interrupt the fascicular integrity and is successful in reinnervation; therefore, it seems to be a good alternative for direct muscular neurotization.     

Muscle study in experimental scoliosis in rabbits with costotransversectomy: evidence of ischemic process

Scoliosis involves the central nervous system diseases, ligaments, articulations and skeletal muscles, but there is no consensus on its pathogeny and progression of muscle abnormalities. In this study, we investigate the morphologic changes in the muscle of rabbit submitted to experimental scoliosis, with special emphasis on abnormalities related to blood supply. We studied 26 rabbits subjected to costotransversectomy by pulling out six transverse processes at thoracic level and six rabbits were used as controls. All the animals operated upon developed scoliosis showing an average angle of 29.1° on the 60th day, with its apices located at T4 and T12 when they were subjected to paraspinal muscle biopsy on both sides. The muscle biopsies were subjected to histological stains and histochemical reactions, as well as to a morphometric study. On the concave side, the changes were not statistically significant regarding the control group. On the convex side conjunctive tissue proliferation, infiltration by adipose tissue, central nucleus excess, inflammatory reaction, segmental fibrosis, type 1 fiber hypertrophy, type 2 fiber hypertrophy and atrophic angular dark fibers in the unspecific esterase were statistically significant. The segmental fibrosis reached a circumscribed muscle segment, compatible with an ischemic phenomenon. The histological diagnoses on the concave side of the animals had unspecific alterations (atrophy and hypertrophy) in 13, myopathy in 3, denervation in 3 and normal in 7. The convex side diagnoses were myopathy in 14, denervation in 8, mixed in 3 and normal in 1. The procedure determined morphologic changes on the convex side indicating possible denervation or myopathy of ischemic origin.     

大鼠失神经支配骨骼肌萎缩后肌细胞凋亡及Bcl-2、Bax表达的变化

目的 研究失神经支配骨骼肌萎缩后肌细胞凋亡及Bcl-2、Bax表达的变化规律.方法 实验以失神经支配腓肠肌为动物模型.选用体质量为200～250 g的雌性SD大鼠42只,随机分成2组,实验组36只,健康对照组6只.按术后取材时间的不同再将实验组分为6组,每组6只大鼠.分别运用Tunel法及免疫组织化学方法检测失神经后不同时间萎缩腓肠肌细胞凋亡及Bcl-2、Bax的表达变化,同时测量肌湿重比和肌纤维横截面积,透射电镜观察肌细胞的超微结构变化.结果 萎缩的腓肠肌湿重比在失神经4周内和第10周到第12周下降最快,而在第4周到10周和第12周到16周下降较慢,且变化差异无统计学意义(P＞0.05).Bcl-2仅在失神经早期表达增高,于第4周达高峰;而Bax分别于失神经第2周与第12周达高峰,增高幅度大于Bcl-2.Bcl-2/Bax的比值除对照组(1.522±0.215)和失神经第8周时(1.065±0.165)大于1外,其余各时间点均小于1.肌细胞凋亡率分别于失神经第4周和第12周达到高峰,其变化趋势与Bax相仿.结论 失神经腓肠肌萎缩过程呈现4个阶段;凋亡和凋亡相关基因Bax过量表达在失神经不可逆肌萎缩过程中发挥着重要的作用;靶向作用Bax基因,抑制Bax表达可能延缓失神经骨骼肌萎缩.     

Congenital paralytic vertical talus. An anatomical study.

Dissections of the feet of a three-month-old infant with paralytic congenital vertical talus secondary to lumbar myelomeningocele were compared with a dissection of a normal foot. The major differences appeared to be absence of the plantar intrinsic muscles and dorsal dislocation of the talonavicular joint. It is postulated that the pathological process begins as a failure of the intrinsic muscles to oppose the unbalanced, active dorsiflexion forces of the anterior crural muscles. This imbalance then allows disruption of the talonavicular joint, mechanically the least stable joint in the mid-part of the foot. All dorsiflexion forces acting on the ankle then become ineffective and plantar flexion forces serve only to pull the calcaneus and talus into equinus, causing a "vertical" talus. Treatment must be directed at reducing the talonavicular dislocation, correcting the equinus deformity of the hind part of the foot, and substituting for the undeveloped plantar intrinsic muscles.     

重组慢病毒介导心脏营养素-1基因转染延缓小鼠失神经骨骼肌萎缩

目的 探讨重组慢病毒介导的心脏营养素-1(cardiotrophin-1,CT-1)基因转染延缓小鼠失神经骨骼肌萎缩的疗效.方法 将36只Swiss小鼠,随机分为实验组和对照组,每组18只,切断胫神经,建立右下失神经支配肌萎缩模型.于实验组和对照组失神经腓肠肌分别转染重组慢病毒载体Lenti-GFP-CT-1和Lenti-GFP溶液20μl(108TU/ml),转染后2、4周,每个时间点分别取3只小鼠,于荧光显微镜下观察绿色荧光蛋白(GFP)的表达、Western blot检测CT-1的表达;取6只小鼠行单次收缩力、强直收缩力的检测,测定肌湿重、肌纤维横截面积,并观察肌纤维超微结构的变化.结果 慢病毒转染2,4周,两组腓肠肌中均可见大量GFP表达.Western blot检测显示实验组失神经腓肠肌中有明显的CT-1表达(P均<0.01).转染2周,实验组肌肉单次收缩力恢复率、强直收缩力恢复率、肌湿重维持率和肌纤维横截面积分别为[(47.61±6.25)%,x-±s,下同]、(56.08±5.47)%、(63.02±5.23)%、(1372.42±149.73)μm2,均明显高于对照组,后者分别为(27.23±5.06)%、(30.78±4.67)%、(52.41±4.98)%、(1147.28±128.67)μm2(P均<0.01);4周时,实验组上述各项指标分别为(33.13±4.76)%、(36.59±5.67)%、(51.46±5.36)%、(1209.12±142.57)μm2,仍明显高于对照组,后者分别为(16.40±5.48)%、(15.35±4.08)%、(39.15±6.12)%、(989.45±136.12)μm2(P均<0.01).此外,实验组肌浆网的扩张程度明显减轻.结论 慢病毒介导的基因治疗有较高的转染效率,其介导的CT-1基因转染能有效延缓小鼠失神经骨骼肌的萎缩,其疗效至少可以维持4周.     

Changes in canine skeletal muscles during experimental tibial lengthening

In 24 beagles, lengthening of the right tibia was performed by callus distraction after osteotomy and application of a ring fixator. Distraction was started at the fifth postoperative day, with a distraction rate of 0.5 mm twice per day, and ended after 25 days. A control group of six additional dogs underwent tibial osteotomy and external fixation without distraction. Twelve animals with and three animals without leg lengthening were euthanized immediately after the distraction period of 25 days (Group A); the remaining 15 dogs were euthanized after an additional consolidation phase of another 25 days (Group B). From the distracted right leg and from the left control leg the tibialis anterior muscle, extensor digitorum longus muscle, peroneus longus muscle, and gastrocnemius muscle were removed and studied by means of routine histologic, histochemical, and immunohistochemical analyses, and electron microscopic examination. The muscles of the control group showed no differences between the right and left sides. However, in the other 24 dogs of Groups A and B, the authors saw marked alterations affecting only the lengthened muscles but not the muscles of the control limbs. These changes were highly significant and included muscle fiber degeneration and regeneration, target fibers, central cores, minicores, marked endomysial and perimysial fibrosis, and atrophy of Type 1 and Type 2 fibers. In the consolidation period (Group B) fiber type grouping indicated that reinnervation had occurred. In addition, an increase in satellite cells and myoblasts and proliferation of nuclei were observed. The findings of the current study indicate that leg lengthening results not only in muscle fiber degeneration followed by regeneration and reinnervation but also in formation of new muscle tissue.     

Reanimation of paralyzed laryngeal muscles by electrical stimulation synchronized with inspiration.

OBJECTIVE: To determine the effects of electrical stimulation on denervated cat posterior cricoarytenoid (PCA) muscle. STUDY DESIGN AND SETTING: This study was conducted on six cats with PCA muscle denervation. All animals were sacrificed 12 weeks after surgery and the glottal area in the live animals and the fiber diameters of PCA muscle were obtained. RESULTS: Signals synchronized with inspiration were recorded and transmitted to stimulate PCA muscle. The abduction of the paralyzed vocal cord during inspiration was observed; this allows enough flow of air through the larynx to maintain the respiration. The stimulated fiber diameters of PCA muscle were different from that of nonstimulated (P < 0.01). Although all denervated muscles were degenerated, electrical stimulation was used to prevent muscular atrophy. CONCLUSIONS: This study indicates that electrical stimulation of the PCA muscle synchronized with inspiration could restore the abduction of a paralyzed vocal cord and prevent the denervated muscles from atrophying. SIGNIFICANCE: Electrical stimulation synchronized with inspiration may lead to reanimation of paralyzed laryngeal muscles.     

脊髓空洞源性脊柱侧凸椎旁肌超微结构的比较观察

目的 比较观察脊髓空洞源性脊柱侧凸患者椎旁肌超微结构病理改变的特征,探讨脊柱侧凸的发生机制。方法全部病例分为三组：脊髓空洞源性脊柱侧凸（SS）组12例,青少年特发性脊柱侧凸（AIS）组5例,非脊柱侧凸（NS）组2例。术中切取患者两侧骶棘肌组织,分别进行光镜和电镜观察。结果 SS组有2例患者出现小群性肌萎缩。1例发生周围神经脱髓鞘,7例可见靶纤维或靶样纤维。与AIS组相比,SS组患者骶棘肌的超微结构明显异常。表现为肌质网普遍扩张,线粒体肿胀形成髓鞘样结构,胞质内散在脂滴增多,肌原纤维间糖原聚集,糖原湖形成。结论脊髓空洞源性脊柱侧凸患者存在椎旁肌失神经支配现象,椎旁肌超徽结构的异常以胞浆中细胞器为主,两肌原纤维的病变较轻。SS患者椎旁肌超微结构的异常程度明显比AIS严重,这可能与其椎旁肌发生失神经支配有关。     

人体失神经萎缩骨骼肌蛋白质组学的初步研究

目的采用蛋白质组学分析方法研究人类失神经萎缩骨骼肌肌细胞的蛋白质组表达变化及其临床意义。方法以臂丛神经根性撕脱伤患者为对象,对失神经萎缩的肱二头肌、小鱼际肌及其健侧胸锁乳突肌肌纤维组织中的蛋白质进行二维电泳和PD-Quest分析,对差异蛋白质用MALDI-TOF质谱仪进行Trypsin酶解指纹图测定。结果(1)初步建立了人体骨骼肌蛋白质组学研究的技术平台,建立了失神经萎缩肱二头肌、小鱼际肌以及健侧胸锁乳突肌的凝胶图像的Matchset;(2)在失神经萎缩肌肉中800个蛋白质共有32个发生了变化,表达变化蛋白质主要集中在pI4~8,Mr相对分子质量为30000~50000范围内;(3)发现了肌肉萎缩部分的可能相关蛋白,在萎缩组共有6个点的表达量增加,24个点的表达量降低,两者差异有统计学意义(P<0.05);其中7个蛋白质在肱二头肌和小鱼际肌中的表达量是不同的。被鉴定的7个蛋白质可归类为代谢蛋白、分子伴侣蛋白、收缩性蛋白。结论该结果证实使用蛋白质组学分析骨骼肌萎缩相关蛋白质是可行和有效的,并首次提供了人类健康骨骼肌组织蛋白质2-D图谱。研究了失神经萎缩骨骼肌蛋白质组学的结果表明,细胞内蛋白质合成、防止自由基团的攻击、蛋白质错误折叠的修复以及细胞中蛋白质分选均与人类臂丛损伤后失神经骨骼肌萎缩的病程相关。