Pseudarthrosis

假性关节病
  • 文章类型: Journal Article
    背景:重组人骨形态发生蛋白-2(rhBMP-2)在成人脊柱畸形(ASD)手术的总体成本效益方面并未显示出优异的优势。
    方法:回顾性目的:生成假关节风险评分,以告知rhBMP-2的使用情况,平衡成本与生活质量和并发症。
    方法:纳入ASD患者的3年数据。从ODI到SF-6D计算获得的生活质量,并转化为质量调整寿命年(QALYs)。使用PearlDiver数据库和CMS定义的并发症和合并症计算成本。通过逻辑回归为预测变量生成已建立的权重,以产生可解释虚弱的假关节的预测风险评分。糖尿病,抑郁症,ASA等级,胸腰椎后凸和三柱截骨术的使用。风险评分类别,通过条件推理树(CIT)导出的阈值对rhBMP-2使用的成本效用进行了测试,控制年龄,先前的融合,基线畸形和残疾。
    结果:64%的ASD患者接受rhBMP-2(308/481)。有17例(3.5%)患者发生假关节。rhBMP-2的使用并没有降低假关节的发生率(OR:0.5,[0.2-1.3]).每个风险类别的假关节发生率为:无风险(NoR)0%;低风险(LowR)1.6%;中等风险(ModR)9.3%;高风险(HighR)24.3%。接受rhBMP-2的患者总体QALYs与未接受rhBMP-2的患者相似(0.163vs.0.171,p=.65)。在LowR队列中,rhBMP-2的使用成本效用更差(p<.001)。在ModR患者中,rhBMP-2的使用具有模棱两可的成本效用(53,398美元与61,581美元,p=.232)。在HighR队列中,通过使用rhBMP-2降低了成本效用(98,328美元与211,091美元,p<.001)。
    结论:我们的研究表明,rhBMP-2对发生假关节的高危个体具有有效的成本效用。生成的评分可以帮助脊柱外科医生评估风险,并增强在适当的临床环境中战略使用rhBMP-2的合理性。
    方法:III.
    BACKGROUND: Recombinant human bone morphogenetic protein-2 (rhBMP-2) has not shown superior benefit overall in cost-effectiveness during adult spinal deformity (ASD) surgery.
    METHODS: Retrospective PURPOSE: Generate a risk score for pseudarthrosis to inform the utilization of rhBMP-2, balancing costs against quality of life and complications.
    METHODS: ASD patients with 3-year data were included. Quality of life gained was calculated from ODI to SF-6D and translated to quality-adjusted life years (QALYs). Cost was calculated using the PearlDiver database and CMS definitions for complications and comorbidities. Established weights were generated for predictive variables via logistic regression to yield a predictive risk score for pseudarthrosis that accounted for frailty, diabetes, depression, ASA grade, thoracolumbar kyphosis and three-column osteotomy use. Risk score categories, established via conditional inference tree (CIT)-derived thresholds were tested for cost-utility of rhBMP-2 usage, controlling for age, prior fusion, and baseline deformity and disability.
    RESULTS: 64% of ASD patients received rhBMP-2 (308/481). There were 17 (3.5%) patients that developed pseudarthrosis. rhBMP-2 use overall did not lower pseudarthrosis rates (OR: 0.5, [0.2-1.3]). Pseudarthrosis rates for each risk category were: No Risk (NoR) 0%; Low-Risk (LowR) 1.6%; Moderate Risk (ModR) 9.3%; High-Risk (HighR) 24.3%. Patients receiving rhBMP-2 had similar QALYs overall to those that did not (0.163 vs. 0.171, p = .65). rhBMP-2 usage had worse cost-utility in the LowR cohort (p < .001). In ModR patients, rhBMP-2 usage had equivocal cost-utility ($53,398 vs. $61,581, p = .232). In the HighR cohort, the cost-utility was reduced via rhBMP-2 usage ($98,328 vs. $211,091, p < .001).
    CONCLUSIONS: Our study shows rhBMP-2 demonstrates effective cost-utility for individuals at high risk for developing pseudarthrosis. The generated score can aid spine surgeons in the assessment of risk and enhance justification for the strategic use of rhBMP-2 in the appropriate clinical contexts.
    METHODS: III.
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  • 文章类型: English Abstract
    The reconstruction of long bone defects as a result of primary traumatic, secondary infection or tumor-related loss of substance continues to represent a surgical challenge. Callus distraction via segment transport, vascularized bone transfer and the induced membrane technique (IMT) are established methods of reconstruction. In recent decades IMT has experienced increasing popularity due to its practicability, reproducibility and reliability. At the same time, the original technique has undergone numerous modifications. The results are correspondingly heterogeneous. This overview is intended to explain the basic principles of IMT and to provide an overview of the various modifications and their complications.
    UNASSIGNED: Die Rekonstruktion langstreckiger Knochendefekte infolge von primär traumatischen oder sekundär infektions- oder tumorbedingten Substanzverlusten stellt nach wie vor eine chirurgische Herausforderung dar. Die Kallusdistraktion über Segmenttransport, der vaskularisierte Knochentransfer und die induzierte Membrantechnik (IMT) stellen etablierte Verfahren der Rekonstruktion dar. In den letzten Jahrzehnten erfreut sich die IMT aufgrund ihrer Praktikabilität, Reproduzierbarkeit und Zuverlässigkeit zunehmender Popularität. Gleichsam erfuhr die Originaltechnik eine Vielzahl von Modifikationen. Die Ergebnisse stellen sich als entsprechend heterogen dar. Diese Übersicht soll die wesentlichen Grundprinzipien der IMT darlegen und einen Überblick über die verschiedenen Modifikationen und ihre Komplikationen geben.
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  • 文章类型: Journal Article
    背景:椎弓根减影截骨术(PSO)可有效纠正脊柱排列不良,但并发症发生率高。不同PSO水平的生物力学效应尚不清楚。没有有限元(FE)分析比较L2-,L3-,L4-,和L5-PSO。
    目的:为了评估PSO水平对脊柱整体运动范围的影响,强调后部仪器,与前柱的载荷分担,和近端连接应力。
    方法:计算生物力学分析。
    方法:使用经过验证的3D脊柱骨盆FE模型(T10-Pelvis)在L2,L3,L4和L5进行PSO。每个模型都配备了来自T11-Pelvis的四杆配置(主杆直列卫星杆)。模拟包括两步分析;(1)对胸部应用300N,400N至腰部,和400N到骶骨,和(2)对T10椎体的顶端板施加7.5Nm的力矩。髋臼表面在所有自由度上都是固定的。运动的范围,骨盆脊髓参数(腰椎前凸(LL),骶骨斜坡(SS),骨盆发病率(PI),和骨盆倾斜(PT)),PSO部队,并测量了冯·米塞斯的应力。将所有模型与L3-PSO模型进行比较,并且捕获百分比差异。
    结果:与完整比对相比:LL增加了48%,45%,59%,在L2-中占56%,L3-,L4-,和L5-PSO模型;SS增加了25%,15%,11%,PT下降76%,53%,和45%在L2-,L3-,和L4-PSO(L5-PSO中SS和PT近似完整模型);腰椎截骨术不影响PI。与L3-PSO相比:L2-,L4-,L5-PSO显示高达32%,34%,全球ROM降低了34%。在L5-PSO中观察到最少的T10-T11ROM。每个模型中的左侧和右侧SIJROM大致相似。其中,L5-PSO模型在SIJ显示的ROM最少。与L3-PSO相比,L2-,L4-,L5-PSO模型显示高达67%,61%,在UIV处减少了78%的应力,分别。在L3-PSO模型中观察到UIV+处的最小应力。L2-和L3-PSO显示最大PSO力。L5-PSO模型显示,在所有运动中,主杆上的应力最低。
    结论:我们的有限元研究表明,L5-PSO导致腰椎前凸最大,全球最低,SIJ,T10-T11ROM和主杆上的应力,提示潜在的机械好处,以降低杆断裂的风险。然而,L4-和L5-PSO导致穿过截骨部位的力最小,这可能会增加假关节的风险。这些发现提供了生物力学见解,可以为手术计划提供信息,尽管进一步的临床研究对于确定最佳PSO水平并验证这些结果至关重要。
    结论:了解PSO水平的生物力学影响对于优化手术结果和降低术后并发症的风险至关重要。
    BACKGROUND: Pedicle subtraction osteotomy (PSO) is effective for correcting spinal malalignment but is associated with high complication rates. The biomechanical effect of different PSO levels remains unclear, and no finite element (FE) analysis has compared L2-, L3-, L4-, and L5-PSOs.
    OBJECTIVE: To assess the effects of PSO level on the spine\'s global range of motion, stresses on posterior instrumentation, load sharing with the anterior column, and proximal junctional stresses.
    METHODS: A computational biomechanical analysis.
    METHODS: A validated 3D spinopelvic FE model (T10-Pelvis) was used to perform PSOs at L2, L3, L4 and L5. Each model was instrumented with a four-rod configuration (primary rods + in-line satellite rods) from T11-Pelvis. Simulation included a 2-step analysis; (1) applying 300 N to thoracic, 400 N to lumbar, and 400 N to sacrum, and (2) applying a 7.5 Nm moment to the top endplate of the T10 vertebral body. Acetabulum surfaces were fixed in all degrees of freedom. The range of motion, spinopelvic parameters (lumbar lordosis (LL), sacral slope (SS), pelvic incidence (PI), and pelvic tilt (PT)), PSO force, and von Mises stresses were measured. All models were compared with the L3-PSO model and percentage differences were captured.
    RESULTS: Compared to the intact alignment: LL increased by 48%, 45%, 59%, and 56% in the L2-, L3-, L4-, and L5-PSO models; SS increased by 25%, 15%, and 11% while PT decreased by 76%, 53%, and 45% in L2-, L3-, and L4-PSOs (SS and PT approximated intact model in L5-PSO); Lumbar osteotomy did not affect the PI. Compared to L3-PSO: L2-, L4-, and L5-PSOs showed up to 32%, 34%, and 34% lower global ROM. The least T10-T11 ROM was observed in L5-PSO. The left and right SIJ ROM were approximately similar in each model. Amongst all, the L5-PSO model showed the least ROM at the SIJ. Compared to L3-PSO, the L2-, L4-, and L5-PSO models showed up to 67%, 61%, and 78% reduced stresses at the UIV, respectively. Minimum stress at UIV+ was observed in the L3-PSO model. The L2-and L3-PSOs showed the maximum PSO force. The L5-PSO model showed the lowest stresses on the primary rods in all motions.
    CONCLUSIONS: Our FE investigation indicates that L5-PSO results in the greatest lumbar lordosis and lowest global, SIJ, and T10-T11 ROMs and stresses on the primary rods, suggesting potential mechanical benefits in reducing the risk of rod breakage. However, L4- and L5-PSOs led to the least force across the osteotomy site, which may increase the risk of pseudarthrosis. These findings provide biomechanical insights that may inform surgical planning, though further clinical investigation is essential to determine the optimal PSO level and validate these results.
    CONCLUSIONS: Understanding the biomechanical impact of PSO level is crucial for optimizing surgical outcomes and minimizing the risks of post-operative complications.
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  • 文章类型: Journal Article
    目的:报告接受腰椎融合手术的患者样本的融合率,并评估基于计算机断层扫描(CT)的评估融合参数的评估者间可靠性。
    方法:回顾性分析2017年至2021年所有接受腰椎融合手术的成年患者。通过电子病历的图表审查收集患者的人口统计学和手术特征。CT扫描由两名主治脊柱外科医生和两名脊柱研究员独立审查。融合定义为(1)后外侧沟槽中任何一个骨桥接的证据,(2)刻面,或(3)任何CT视图上的椎体间(适用时)。螺钉晕的证据表明骨不连。使用科恩的kappa确定评分者间的可靠性。之后,参与者之间就融合的每个组成部分达成了共识.
    结果:所有手术的总融合率为63/69(91.3%)。总体22/25(88.0%)TLIF,16/19(84.2%)PLDF,3/3(100%)LLIF,和22/22(100%)的圆周融合经历了成功的融合。椎间融合的评分者可靠性良好(k=0.734),所有其他措施均中等(后外侧融合的k=0.561;小平面融合的k=0.471;螺钉封口的k=0.458)。总的来说,评估者对患者是否有融合或不愈合的可靠性中等(k=0.510).
    结论:在评估腰椎融合状态的大多数影像学检查中,仅有中等的评估者间可靠性。在评估体间融合的存在时,可靠性最高。大多数融合发生在小关节上。
    OBJECTIVE: To report the rate of fusion in a sample of patients undergoing lumbar fusion surgery and assess interrater reliability of computed tomography (CT)-based parameters for the assessment of fusion.
    METHODS: All adult patients who underwent lumbar fusion surgery from 2017 to 2021 were retrospectively identified. Patient demographics and surgical characteristics were collected through chart review of the electronic medical records. CT scans were reviewed independently by two attending spine surgeons and two spine fellows. Fusion was defined as evidence of bone bridging in any one of (1) posterolateral gutters, (2) facets, or (3) interbody (when applicable) on any CT views. Evidence of screw haloing was indicative of nonunion. Interrater reliability was determined using cohen\'s kappa. Afterwards, a consensus agreement for each component of fusion was reached between participants.
    RESULTS: The overall fusion rate among all procedures was 63/69 (91.3%). Overall 22/25 (88.0%) TLIF, 16/19 (84.2%) PLDF, 3/3 (100%) LLIF, and 22/22 (100%) circumferential fusions experienced a successful fusion. Interrater reliability was good for interbody fusion (k = 0.734) and moderate for all other measures (k = 0.561 for posterolateral fusion; k = 0.471 for facet fusion; k = 0.458 for screw haloing). Overall, interrater reliability as to whether a patient had a fusion or nonunion was moderate (k = 0.510).
    CONCLUSIONS: There was only moderate interrater reliability across most radiographic measures used in assessing lumbar fusion status. Reliability was highest when evaluating the presence of interbody fusion. The majority of fusions occurred across the facet joints.
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  • 文章类型: Journal Article
    目的:颈椎前路椎间盘切除融合术(ACDF)后骨不连和明显下沉与不良的临床结局有关,偶尔会导致翻修手术。同种异体移植物和聚醚醚酮(PEEK)笼是用于ACDF的两种最常用的椎体间间隔装置。尽管已经进行了研究来比较这两种椎体间材料的功效,问题仍然是关于一个比另一个优越。因此,作者进行了系统评价和荟萃分析,以比较骨不连,沉降,使用同种异体移植物和PEEK笼作为体间设备的ACDF后的再手术率。
    方法:在本系统综述和荟萃分析中,作者系统地搜索了MEDLINE,EMBASE,和CochraneLibrary数据库,用于2023年11月之前发表的研究,比较了同种异体移植物和PEEK笼对ACDF的疗效和安全性。汇总分析旨在确定骨不连的差异,沉降,以及两个体间设备之间的再手术率。
    结果:涉及1462例患者的十项研究(同种异体移植,852名患者;PEEK笼,610名患者)被包括在内。汇总分析表明,与PEEK笼相比,同种异体移植的骨不愈合率显着降低(OR0.33,95%CI0.14-0.79;p=0.01)。此外,PEEK笼与同种异体移植相比,由于骨不连引起的再手术率明显更高(OR0.28,95%CI0.11-0.71;p<0.01),而由于整体原因导致的再手术率没有显着结果(OR0.38,95%CI0.11-1.29;p=0.12)。显著沉降的发生率(OR0.66,95%CI0.28-1.55;p=0.34)和平均沉降量(标准平均差0.03,95%CI-0.42至0.47;p=0.90)在同种异体移植和PEEK笼之间没有显着差异。
    结论:总体而言,目前的荟萃分析表明,同种异体移植优于用于ACDF的PEEK笼,由于提高了融合率和最小化的修订风险,没有增加沉降的风险。
    OBJECTIVE: Nonunion and significant subsidence after anterior cervical discectomy and fusion (ACDF) are associated with poor clinical outcomes, which occasionally lead to revision surgery. Allograft and polyetheretherketone (PEEK) cages are the two most commonly used interbody spacer devices for ACDF. Although studies have been conducted to compare the efficacies of these two interbody materials, the question remains regarding the superiority of one over the other. Therefore, the authors conducted a systematic review and meta-analysis to compare nonunion, subsidence, and reoperation rates after ACDF using allograft and PEEK cages as interbody devices.
    METHODS: In this systematic review and meta-analysis, the authors systematically searched the MEDLINE, EMBASE, and Cochrane Library databases for studies published prior to November 2023 that compared the efficacy and safety of allograft and PEEK cages for ACDF. A pooled analysis was designed to identify differences in nonunion, subsidence, and reoperation rates between the two interbody devices.
    RESULTS: Ten studies involving 1462 patients (allograft, 852 patients; PEEK cage, 610 patients) were included. The pooled analysis demonstrated that allograft had a significantly lower rate of nonunion compared to that of PEEK cages (OR 0.33, 95% CI 0.14-0.79; p = 0.01). Furthermore, the reoperation rate due to nonunion was significantly higher with PEEK cages compared to that with allograft (OR 0.28, 95% CI 0.11-0.71; p < 0.01), whereas the reoperation rate due to overall causes did not display significant results (OR 0.38, 95% CI 0.11-1.29; p = 0.12). The incidence of significant subsidence (OR 0.66, 95% CI 0.28-1.55; p = 0.34) and the mean amount of subsidence (standard mean difference 0.03, 95% CI -0.42 to 0.47; p = 0.90) did not demonstrate significant differences between allograft and PEEK cages.
    CONCLUSIONS: Overall, the current meta-analysis suggests the advantages of allograft over PEEK cages used for ACDF, due to an enhanced fusion rate and minimized revision risk, with no increase in the risk of subsidence.
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  • 文章类型: Case Reports
    胸肋不愈合导致的前胸壁不稳定是一种罕见的并发症,但可引起无效的疼痛和心律失常。经过改良的Ravitch手术后,一名40多岁的妇女因前胸壁疼痛和不稳定而被转诊给我们。观察到胸肋假性关节病,其中进行了多次手术,并伴有低度感染。在手术中使用了针对患者的三维建模和打印假体,既可以抬起胸骨进行胸肌矫正,又可以重新连接胸骨和胸骨肋部,以恢复前胸壁的稳定性。
    Anterior chest wall instability as a result of sternocostal non-union is a rare complication but can give rise to invalidating pain and cardiac arrhythmias. A woman in her 40s was referred to us with anterior chest wall pain and instability after a modified Ravitch procedure. Sternocostal pseudoarthrosis was seen for which multiple operations were performed which were complicated by low-grade infections. A patient-specific three-dimensional modelled and printed prostheses was used in an operation to both lift the sternum for pectus correction and to reconnect the sternum and the sternal costal junction to regain anterior chest wall stability.
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  • 文章类型: Journal Article
    本文报告烟台业达医院耳鼻咽喉科、烟台毓璜顶医院耳鼻咽喉科、烟台芝罘医院耳鼻咽喉科等三所医院从2008年2月至2023年8月收治的7例茎突舌骨韧带骨化假关节型的病例,介绍了茎突舌骨韧带骨化假关节类型的常见及特殊症状、体征、影像学特点,临床诊断要点等。7例患者中有4例接受手术治疗;3例拒绝手术治疗,采用咽部、颈部封闭及药物治疗。4例接受手术治疗的患者中有3例行经颈外进路手术,1例行经口内进路手术,术后3例患者症状消失,1例好转。3例保守治疗的患者中治疗后症状消失者1例,明显减轻者2例。.
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  • 文章类型: Journal Article
    由于关节固定术的可靠率,采用侧块螺钉的后颈椎融合术(PCF)是改善有症状的假关节的有利治疗选择;然而,这种技术增加了伤口感染和再入院的风险。涉及关节面固定器械的保留组织的PCF方法可降低术后并发症的发生率,同时稳定症状水平以实现关节固定术;然而,这些结局仅限于来自个别外科医生的小型研究队列,这些外科医生通常具有混合治疗适应症.
    150例病例来自美国6个地点的7名外科医生进行的回顾性图表审查。所有病例均在颈椎前路椎间盘切除术和融合术(ACDF)后从C3到C7的一个或多个级别进行了PCF翻修。PCF是使用小平面器械的组织保留技术进行的。涉及额外补充固定的病例,如侧块螺钉,棒,电线,或其他硬件被排除在外。人口统计,操作注释,术后并发症,医院再入院,和随后的手术干预被总结为一个完整的队列,并根据以下风险因素:年龄,性别,修订的级别数,体重指数(BMI),和尼古丁使用史。
    PCF翻修时患者的平均年龄为55±11岁,63%为女性。平均BMI为29±6kg/m2,有19%的人报告有尼古丁使用史。术后随访的中位数为68天(四分位距=41-209天),从修订PCF开始。有91个1级,492级,83级,24±水平PCF翻修病例。平均手术时间为52±3分钟,估计失血量为14±1.5cc。参与者在手术后平均1±0.05天出院。多级治疗导致更长的手术时间(单次=45分钟,multi=59min,P=0.01),但不影响估计的失血量(P=0.94)。通过多级治疗,医院的总夜晚增加了0.2个夜晚(P=0.01)。性,年龄,尼古丁病史,BMI对记录的围手术期结局无影响.有一次因深静脉血栓而再次住院,用ACDF治疗的1例修订水平的持续性假关节,和四个相邻节段疾病的实例。在最初接受多水平ACDF治疗的患者中,修订最常见于尾部水平(修订水平的48%),其次是颅骨(43%),最不经常处于中等水平(9%)。
    此围手术期和安全性结果的图表回顾提供了证据,支持保留组织的PCF与小关节器械治疗ACDF后有症状的假关节。需要翻修的最常见位置是尾和颅骨水平。与开放式替代方案相比,手术持续时间和估计的失血量是有利的。术后没有伤口感染,大多数患者在手术后第二天出院。
    UNASSIGNED: Posterior cervical fusion (PCF) with lateral mass screws is a favorable treatment option to revise a symptomatic pseudarthrosis due to reliable rates of arthrodesis; however, this technique introduces elevated risk for wound infection and hospital readmission. A tissue-sparing PCF approach involving facet fixation instrumentation reduces the rates of postoperative complications while stabilizing the symptomatic level to achieve arthrodesis; however, these outcomes have been limited to small study cohorts from individual surgeons commonly with mixed indications for treatment.
    UNASSIGNED: One hundred and fifty cases were identified from a retrospective chart review performed by seven surgeons across six sites in the United States. All cases involved PCF revision for a pseudarthrosis at one or more levels from C3 to C7 following anterior cervical discectomy and fusion (ACDF). PCF was performed using a tissue-sparing technique with facet instrumentation. Cases involving additional supplemental fixation such as lateral mass screws, rods, wires, or other hardware were excluded. Demographics, operative notes, postoperative complications, hospital readmission, and subsequent surgical interventions were summarized as an entire cohort and according to the following risk factors: age, sex, number of levels revised, body mass index (BMI), and history of nicotine use.
    UNASSIGNED: The average age of patients at the time of PCF revision was 55 ± 11 years and 63% were female. The average BMI was 29 ± 6 kg/m2 and 19% reported a history of nicotine use. Postoperative follow-up visits were available with a median of 68 days (interquartile range = 41-209 days) from revision PCF. There were 91 1-level, 49 2-level, 8 3-level, and 2 4±-level PCF revision cases. The mean operative duration was 52 ± 3 min with an estimated blood loss of 14 ± 1.5cc. Participants were discharged an average of 1 ± 0.05 days following surgery. Multilevel treatment resulted in longer procedure times (single = 45 min, multi = 59 min, P = 0.01) but did not impact estimated blood loss (P = 0.94). Total nights in the hospital increased by 0.2 nights with multilevel treatment (P = 0.01). Sex, age, nicotine history, and BMI had no effect on recorded perioperative outcomes. There was one instance of rehospitalization due to deep-vein thrombosis, one instance of persistent pseudarthrosis at the revised level treated with ACDF, and four instances of adjacent segment disease. In patients initially treated with multilevel ACDF, revisions occurred most commonly on the caudal level (48% of revised levels), followed by the cranial (43%), and least often in the middle level (9%).
    UNASSIGNED: This chart review of perioperative and safety outcomes provides evidence in support of tissue-sparing PCF with facet instrumentation as a treatment for symptomatic pseudarthrosis after ACDF. The most common locations requiring revision were the caudal and cranial levels. Operative duration and estimated blood loss were favorable when compared to open alternatives. There were no instances of postoperative wound infection, and the majority of patients were discharged the day following surgery.
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  • 文章类型: Journal Article
    先天性前臂骨假关节(CPFBs)很少见,只有106例报告病例,并且经常与神经纤维瘤病(NF)有关。大约5%的NF患者会出现假关节,50%的假关节患者患有NF。先天性假关节很难实现骨愈合。已经尝试了许多方法,包括铸造,有或没有移植的内固定,和电刺激,但是失败是经常发生的。自1975年以来,游离的血管化腓骨皮瓣(FVF)已用于桥接长骨缺损,自1979年以来已用于胫骨假关节。在CPFB中,FVF在实现联合方面比其他方法更成功,并且是当前选择的治疗方法。这里,我们介绍了3例前臂假关节用FVF治疗,回顾了关于CPFB的文献,并讨论了FVF治疗的一些技术问题。应用游离腓骨皮瓣治疗3例先天性假关节,诊断为7岁(尺骨),15个月(半径),和9年(桡骨和尺骨)。两个皮瓣用髓内钢丝和后期固定,一个有压缩板。一名持续性骨不连接受了翻修的非血管化骨移植和钢板治疗。所有患者在索引手术后11个月均达到愈合。血管化腓骨重建是治疗的选择,因为它提供了最高的公开愈合率和良好的功能效果。完全切除受影响的骨并稳定固定,后期用压缩板是成功的关键。手术技术要求很高,并发症很常见。可能需要二次手术,但是结果是有利的。证据级别:IV.
    Congenital pseudarthrosis of the forearm bones (CPFBs) is rare, with only 106 reported cases, and is frequently associated with neurofibromatosis (NF). Approximately 5% of patients with NF develop pseudarthrosis, and 50% of patients with pseudarthrosis have NF. Achieving bone union is difficult in congenital pseudarthrosis. Many methods have been attempted, including casting, internal fixation with or without grafting, and electrical stimulation, but failure is frequent. Free vascularized fibular flaps (FVFs) have been used to bridge long bone defects since 1975 and in tibial pseudarthrosis since 1979. In CPFB, FVF is more successful than other methods in achieving union and is the current treatment of choice. Here, we presented three cases of forearm pseudarthrosis treated with FVF, reviewed the literature on CPFB, and discussed some technical aspects of FVF treatment. Three cases of congenital pseudoarthrosis were treated with free fibula flaps, diagnosed at ages of 7 years (ulna), 15 months (radius), and 9 years (radius and ulna). Two flaps were stabilized with intramedullary wires and latterly, one with compression plates. One persistent nonunion received revision nonvascularized bone grafting and plating. All patients achieved union by 11 months after index surgery. Reconstruction with vascularized fibula is the treatment of choice because it offers the highest published union rates and good functional results. Complete resection of the affected bone and stable fixation, latterly with compression plates are critical to success. Surgery is technically demanding, and complications are common. Secondary surgery may be required, but outcomes are favorable. LEVEL OF EVIDENCE: IV.
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  • 文章类型: Journal Article
    先天性胫骨假关节(CPT)是一种严重的病理特征是自发性骨折无法愈合,导致纤维状骨不连。一半的CPT患者受到NF1肿瘤抑制基因突变引起的多系统遗传性疾病1型神经纤维瘤病(NF1)的影响,RAS-丝裂原活化蛋白激酶(MAPK)信号通路的负调节因子。这里,我们分析了CPT和Prss56-Nf1基因敲除小鼠的患者,以阐明CPT相关的纤维骨不连的致病机制,并探索了治疗CPT的药理学方法.我们确定了病理性骨膜中NF1缺陷的雪旺氏细胞和骨骼干/祖细胞(SSPC)为驱动纤维化的受影响细胞类型。而缺乏NF1的SSPC采用了纤维化的命运,NF1缺陷的雪旺氏细胞产生关键的旁分泌因子,包括转化生长因子-β,并诱导野生型SSPC的纤维化分化。为了抵消NF1缺陷的施万细胞和SSPC中RAS-MAPK信号传导的升高,我们使用MAPK激酶(MEK)和Src同源2含蛋白酪氨酸磷酸酶2(SHP2)抑制剂。在Prss56-Nf1基因敲除小鼠模型中,体内联合抑制MEK-SHP2可预防纤维骨不连,为CPT中骨不愈合的治疗提供了一种有前途的治疗策略。
    Congenital pseudarthrosis of the tibia (CPT) is a severe pathology marked by spontaneous bone fractures that fail to heal, leading to fibrous nonunion. Half of patients with CPT are affected by the multisystemic genetic disorder neurofibromatosis type 1 (NF1) caused by mutations in the NF1 tumor suppressor gene, a negative regulator of RAS-mitogen-activated protein kinase (MAPK) signaling pathway. Here, we analyzed patients with CPT and Prss56-Nf1 knockout mice to elucidate the pathogenic mechanisms of CPT-related fibrous nonunion and explored a pharmacological approach to treat CPT. We identified NF1-deficient Schwann cells and skeletal stem/progenitor cells (SSPCs) in pathological periosteum as affected cell types driving fibrosis. Whereas NF1-deficient SSPCs adopted a fibrotic fate, NF1-deficient Schwann cells produced critical paracrine factors including transforming growth factor-β and induced fibrotic differentiation of wild-type SSPCs. To counteract the elevated RAS-MAPK signaling in both NF1-deficient Schwann cells and SSPCs, we used MAPK kinase (MEK) and Src homology 2 containing protein tyrosine phosphatase 2 (SHP2) inhibitors. Combined MEK-SHP2 inhibition in vivo prevented fibrous nonunion in the Prss56-Nf1 knockout mouse model, providing a promising therapeutic strategy for the treatment of fibrous nonunion in CPT.
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