radial longitudinal deficiency

径向纵向缺陷
  • 文章类型: Journal Article
    解决软组织和持续骨骼发育引起的变形应力对于在径向纵向缺陷中获得前臂腕部对齐的长期矫正至关重要。本研究的目的是报告儿童尺袖截骨术的中期随访结果。对总共17例患者(21例累及肢体)进行了回顾,平均随访66个月(范围50至96)。最终随访时手前臂角度的平均校正为51°。术前和最终随访时的平均手前臂位置分别为-1.1cm(SD0.9)和1.3cm(SD0.8),分别。在畸形矫正的整个原始阶段,这种干phy端截骨术放松了radial骨结构。在最后一次随访中,尺骨平均生长为对侧的62%。我们的技术可以为矫正提供可行的解决方案,并防止畸形的复发,同时在中长期保持尺骨生长。证据等级:III.
    Addressing the deforming stresses arising from soft tissue and continued skeletal development is crucial to obtain long-term correction of forearm carpal alignment in Radial longitudinal deficiency. The aim of the present study was to report the medium-term follow-up results of radialization with ulnar cuff osteotomy in children. A total of 17 patients (21 involved limbs) with a mean follow-up of 66 months (range 50 to 96) were reviewed. Mean correction of the hand forearm angle at the final follow-up was 51°. Mean hand forearm position preoperatively and at the final follow-up were -1.1 cm (SD 0.9) and +1.3 cm (SD 0.8), respectively. This metaphyseal osteotomy relaxed the radial structures throughout the original phase of deformity correction. The mean ulnar growth was 62% of the contralateral side at the final follow-up. Our technique may provide a feasible solution to the correction and prevent recurrence of deformity while maintaining ulnar growth in the medium to longer term.Level of evidence: III.
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  • 文章类型: Journal Article
    报告组织病理学,电子显微镜,和从radial纵向缺陷(RLD)患者获得的组织样本的免疫组织化学结果,并研究软组织异常作为RLD畸形的次要驱动因素的贡献。
    手术时(放射化或集中化),从14例16条四肢受Bayne3型和4型RLD影响的患者中获得了radial侧肌肉和肌腱的标本。使用光学显微镜对样品进行评估,电子显微镜,和免疫组织化学检查。
    在16个冷冻肌肉样本中,6(37%)显示正常肌肉,而10人(63%)显示存在萎缩性纤维。正常肌肉6例,纤维型分布未见异常。六名患者表现出1型肌纤维的优势。没有一个标本有肌成纤维细胞;16个标本中有4个有肥大细胞,16个标本中有9个显示存在血小板衍生生长因子阳性细胞。肌成纤维细胞的特征(基底层的存在,细胞间连接,或血小板囊泡)在电子显微镜上未在任何标本中发现。
    组织病理学,电子显微镜,和免疫组织化学结果,特别是肌成纤维细胞的缺失,在从RLD患者获得的组织样本中,不支持软组织异常作为RLD畸形的次要驱动因素的假设。
    这项研究为软组织在RLD畸形发展中的可能作用提供了初步见解。
    To report the histopathological, electron microscopic, and immunohistochemical findings of tissue samples obtained from patients with radial longitudinal deficiency (RLD) and investigate the contribution of abnormality in soft tissues as a secondary driver of deformity in RLD.
    Specimens from radial-sided muscles and tendons were obtained at the time of surgery (either radialization or centralization) from 14 patients with 16 limbs affected with Bayne type 3 and type 4 RLD. The specimens were evaluated using light microscopy, electron microscopy, and immunohistochemical examination.
    Among the 16 frozen muscle specimens, 6 (37%) showed normal muscle, while 10 (63%) showed the presence of atrophic fibers. The 6 cases with normal muscle showed no abnormality in fiber type distribution. Six patients showed predominance of type 1 muscle fibers. None of the specimens had myofibroblasts; 4 of 16 specimens had mast cells, and 9 of 16 specimens showed the presence of platelet derived growth factor-positive cells. Features of myofibroblasts (the presence of basal lamina, intercellular junctions, or pinocytic vesicles) were not identified in any specimen on electron microscopy.
    The histopathological, electron microscopic, and immunohistochemical findings, in particular the absence of myofibroblasts, in tissue samples obtained from patients with RLD, do not support the assumption of abnormality in soft tissues as a secondary driver of deformity in RLD.
    This study provides a preliminary insight into a possible role of soft tissues in the development of the deformity in RLD.
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  • 文章类型: Journal Article
    目的:调查在我院因先天性异常而进行的体检队列中的功能和美学结果。
    方法:从1987年到2016年,我们对32只1至8岁儿童的手进行了政治调查(中位数,2年)。我们随访了1至31年的31只手(中位数,手术后10年)。参与者及其护理人员通过视觉模拟量表和患者报告的结果测量(患者报告的结果测量信息系统儿科上肢;手臂残疾的简短版本,肩和手指标测量;和EQ-5D-3L)。我们检查了手的运动,力量,灵敏度,和功能。
    结果:有2例并发症和6例再次手术。轻度异常(径向纵向缺乏Bayne型N/0至2)的参与者的主观和客观手功能优于严重异常(径向纵向缺乏Bayne型3-4,尺骨dimelia,5手指手)。术前食指接近正常的手,在大多数情况下,良好的拇指反对和捏,两组的手都受益于圆柱握把的创造。握力和捏力低于队列研究中报道的,在队列研究中,进行了额外的胫骨成形术。
    结论:患有严重先天性畸形的手也从手术中获益。我们建议采用简化的随访计划,以确定在儿童成长过程中应考虑进行其他手术以增强力量的情况。
    方法:治疗IV。
    OBJECTIVE: To investigate the functional and aesthetic outcomes in a cohort with pollicizations performed due to congenital anomalies in our hospital.
    METHODS: From 1987 to 2016, we performed pollicizations in 32 hands of children aged 1 to 8 years (median, 2 years). We followed-up on 31 of the hands from 1 to 31 years (median, 10 years) after the procedure. The participants and their caregivers self-assessed their function and appearance with visual analogue scales and patient-reported outcome measures (Patient-Reported Outcomes Measurement Information System Pediatric Upper Extremity; the short version of the Disability of Arm, Shoulder and Hand Outcome Measure; and EQ-5D-3L). We examined the hands with regard to motion, strength, sensitivity, and function.
    RESULTS: There were 2 complications and 6 reoperations. Participants with mild anomalies (radial longitudinal deficiency Bayne type N/0 to 2) had better subjective and objective hand function than participants with severe anomalies (radial longitudinal deficiency Bayne type 3-4, ulnar dimelia, 5-finger hand). Hands with preoperatively near-normal index fingers had, in most cases, good thumb opposition and pinch, and hands in both groups benefited from the creation of a cylinder grip. Grip and pinch strength were lower than reported in cohort studies where an additional opponensplasty had been performed.
    CONCLUSIONS: Hands with severe congenital anomalies also benefited from the procedure. We recommend a simplified follow-up program to identify cases where additional surgeries to enhance strength should be considered during growth of the child.
    METHODS: Therapeutic IV.
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