Cerebral Palsy

脑性瘫痪
  • 文章类型: Journal Article
    BACKGROUND: severe, rigid hip abduction deformity in individuals with cerebral palsy (CP) is an exceptionally uncommon condition. This posture hinders the positioning in the wheelchair and the completion of basic activities of daily living (ADL). Addressing such severe deformities can be quite challenging.
    METHODS: a 14-year-old male, with spastic-dystonic quadriplegic CP, developed rigid and severe flexion-abduction contractures in both hips, characterized by 90 degrees of flexion and 100 degrees of abduction. These contractures severely impeded his ability to comfortably use a wheelchair and even pass through doorways. Performing basic ADLs became a significant challenge for both the patient and his caregivers.
    RESULTS: the treatment approach involved a two-stage surgical procedure, one for each hip, with a two-month interval between them. An extensive release of the fascia latae, gluteus maximus, external rotators, and hip flexors; in combination with a proximal femur osteotomy were performed. To maintain the corrections achieved, long-leg casts connected with two bars were employed, followed by orthotic support and physiotherapy. Following the procedure, lower limb adduction was achieved, and the patient and caregivers were highly satisfied, as ADLs and basic caregiving had been greatly facilitated.
    CONCLUSIONS: while the available literature on the management of severe rigid abduction hip contractures in non-ambulatory CP patients is limited, and treatment options are often complex, the present case underscores the effectiveness of a comprehensive approach involving soft tissue release and bone surgery. Achieving a more favorable wheelchair positioning and facilitating basic ADLs and care represents a significant success for patients and families.
    UNASSIGNED: la deformidad severa y rígida en abducción de cadera en individuos con parálisis cerebral (PC) es una condición infrecuente. Esta postura dificulta el posicionamiento en la silla de ruedas y la realización de actividades básicas de la vida diaria (AVD). El tratamiento de estas deformidades tan severas puede ser todo un reto.
    UNASSIGNED: varón de 14 años, con PC tetrapléjica espástica-distónica, que desarrolló contracturas rígidas y severas de flexión-abducción en ambas caderas, caracterizadas por 90 grados de flexión y 100 grados de abducción. Estas contracturas impedían gravemente su capacidad para utilizar cómodamente una silla de ruedas e incluso pasar por las puertas. La realización de actividades básicas de la vida diaria se convirtió en un reto importante tanto para el paciente como para sus cuidadores.
    RESULTS: el tratamiento consistió en una intervención quirúrgica en dos fases, una para cada cadera, con un intervalo de dos meses entre ellas. Se realizó una amplia liberación de la fascia lata, el glúteo mayor, los rotadores externos y los flexores de la cadera; en combinación con una osteotomía proximal del fémur. Para mantener las correcciones conseguidas, se emplearon escayolas de pierna larga conectadas con dos barras, seguidas de soporte ortésico y fisioterapia. Tras la intervención, se consiguió la aducción de los miembros inferiores y el paciente y los cuidadores se mostraron muy satisfechos, ya que se habían facilitado en gran medida las AVD y los cuidados básicos.
    CONCLUSIONS: aunque la bibliografía disponible sobre el tratamiento de las contracturas rígidas graves de la cadera en abducción en pacientes no deambulantes con PC es limitada, y las opciones de tratamiento suelen ser complejas, el presente caso subraya la eficacia de un enfoque integral que incluye la liberación de los tejidos blandos y la cirugía ósea. Conseguir una posición más favorable en la silla de ruedas y facilitar las AVD básicas y los cuidados representa un éxito significativo para los pacientes y sus familias.
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  • 文章类型: Journal Article
    几种危险因素与胎儿窒息有关。这次回顾展的主要目的,分析,病例对照研究旨在确定辅助生殖技术(ART)是否可被视为这些因素之一.将162例胎儿窒息与361例未发生此事件的对照进行比较。我们包括32次ART怀孕,其中12个是通过卵子捐赠获得的。75%(24)的ART妊娠经历了胎儿窒息,提示ART使胎儿窒息的风险增加约7倍。这一发现与文献一致。ART妊娠中胎儿窒息的发病机制目前尚不清楚。因此,这个话题应该进一步研究。
    Several risk factors are associated with fetal asphyxia. The main aim of this retrospective, analytical, case-control study was to determine whether assisted reproductive technologies (ART) could be considered one of these factors. In total, 162 cases of fetal asphyxia were compared to 361 controls where this event did not occur. We included 32 ART pregnancies, of which 12 were obtained through egg donations. Overall, 75% (24) of ART pregnancies experienced fetal asphyxia, suggesting ART increases the risk of fetal asphyxia by about 7 times. This finding is consistent with the literature. The pathogenesis of fetal asphyxia in ART pregnancies is currently unknown. Accordingly, this topic should be further investigated.
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  • 文章类型: Journal Article
    目的:痉挛是脑瘫(CP)的一个具有挑战性的特征,可以通过选择性背根切断术(SDR)来治疗。尽管标准工作工具(SWTs)最近已被用来告知神经外科手术的护理标准,之前没有描述用于SDR的SWT。作者介绍了在其机构中使用的SDR的多学科方法SWT,以促进该领域的一致性并最大程度地降低并发症发生率。
    方法:使用多学科方法来定义SDR途径中的所有步骤。术前,术中,合成了术后工作流程,通过住院康复和减少感染来改善流动性。
    结果:SWTs已在两个机构实施了7年。一例3岁10个月的患者在29周时有早产史,痉挛-截瘫CP,右侧脑室周围白质软化,并介绍了接受L2-S1SDR的发育迟缓。
    结论:作者详细介绍了由多学科团队开发的SDR的SWT,并在患者路径的所有点进行了具体步骤。说明性案例强调,SWT可能有助于确保SDR的安全性,同时最大限度地提高其对CP患者的长期疗效。
    Spasticity is a challenging feature of cerebral palsy (CP) that may be managed with selective dorsal rhizotomy (SDR). Although standard work tools (SWTs) have recently been utilized to inform a standard of care for neurosurgical procedures, no SWTs for SDR have been previously described. The authors present the multidisciplinary approach SWTs for SDR used at their institutions to promote consistency in the field and minimize complication rates.
    A multidisciplinary approach was used to define all steps in the SDR pathway. Preoperative, intraoperative, and postoperative workflows were synthesized, with specific efforts to improve mobility through inpatient rehabilitation and minimize infection.
    The SWTs have been implemented at two institutions for 7 years. An illustrative case of a patient aged 3 years 10 months with a history of premature birth at 29 weeks, spastic-diplegic CP, right-sided periventricular leukomalacia, and developmental delay who underwent L2-S1 SDR is presented.
    The authors detail SWTs for SDR developed by a multidisciplinary team with specific steps at all points in the patient pathway. The illustrative case emphasizes that SWTs may help ensure the safety of SDR while maximizing its long-term efficacy for individuals with CP.
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  • 文章类型: Journal Article
    目的:单水平选择性背根切断术(SDR),通常适用于非卧床患者,对于患有粗大运动功能分类系统(GMFCS)IV或V级的严重痉挛型脑瘫(CP),是一个有争议的话题。本病例系列和系统文献综述的目的是概述非卧床患者的姑息性SDR的适应症和结果CP和GMFCSIV和V级,重点是改善痉挛状态以及患者和护理人员报告的生活质量评估。
    方法:介绍了在作者机构接受单级SDR的CP和GMFCSIV或V级患者的回顾性病例系列。此外,搜索了两个数据库(PubMed和Embase),并根据术语“选择性背根切断术”进行了系统评价,并使用搜索字符串进行了系统评价,脑瘫,进行了“和”结果“。主要结果是根据改良的Ashworth量表(MAS)减少痉挛。次要结果是粗大运动功能测量值-66(GMFM-66)的变化,评估患者报告的结果测量(PROMs),手术发病率,和死亡率。
    结果:纳入了11名25岁以下连续接受姑息性单一水平SDR的儿童。所有患者的MAS评分均降低(平均1.09±0.66分),未发生手术发病率和死亡率。对于我们案例系列的系统回顾结果,除了4份报告,共包括274名患者。在所有研究中都注意到基于MAS评分的痉挛减少(平均范围1.09-3.2分)。此外,在2项研究中,上肢痉挛也显示MAS评分降低(范围1.7-2.8分).72%的患者GMFM-66评分有所改善,78%的患者膀胱功能得到改善。基于PROM,92%的患者/护理人员对手术后的结果和生活质量感到满意。发生2例伤口感染(2.7%)和1例脑脊液漏(1.3%),虽然没有描述手术相关的死亡。
    结论:这项分析表明痉挛有所改善,日常护理,CP和GMFCS水平为IV和V的患者的舒适度。较大的队列分析姑息性单一水平SDR的结果,基于MAS,GMFM-66和PROM,仍然需要,应该是未来研究的重点。系统审查登记号。:CRD42024495762(https://www。crd.约克。AC.英国/繁荣/)。
    Single-level selective dorsal rhizotomy (SDR), typically indicated for ambulatory patients, is a controversial topic for severe spastic cerebral palsy (CP) with Gross Motor Function Classification System (GMFCS) level IV or V. The objective of this case series and systematic literature review was to outline the indication and outcome of palliative SDR for nonambulatory patients with CP and GMFCS level IV and V, focusing on improvement of spasticity and of patient and caregiver reported quality of life assessment.
    A retrospective case series of patients with CP and GMFCS level IV or V who underwent single-level SDR at the authors\' institution is presented. Furthermore, two databases (PubMed and Embase) were searched and a systematic review with a search string based on the terms \"selective dorsal rhizotomy,\" \"cerebral palsy,\" and \"outcome\" was conducted. The primary outcome was the reduction of spasticity based on the modified Ashworth scale (MAS). Secondary outcomes were change on the Gross Motor Function Measure-66 (GMFM-66), evaluation of patient-reported outcome measures (PROMs), surgical morbidity, and mortality.
    Eleven consecutive children under the age of 25 years undergoing palliative single-level SDR were included. All patients showed a reduction in MAS score (mean 1.09 ± 0.66 points) and no surgical morbidity and mortality occurred. For the systematic review results from our case series, in addition to 4 reports, 274 total patients were included. Reduction of spasticity based on MAS score was noted in all studies (mean range 1.09-3.2 points). Furthermore, in 2 studies spasticity of the upper extremities showed a MAS score reduction as well (range 1.7-2.8 points). The GMFM-66 score improved in 72% of the patients, while bladder function improved in 78% of the patients. Based on the PROMs, 92% of the patients/caregivers were satisfied with the outcome and their quality of life after the procedure. Two wound infections (2.7%) and one CSF leak (1.3%) occurred, while no surgery-related deaths were described.
    This analysis showed an improvement in spasticity, daily care, and comfort for patients with CP and GMFCS levels IV and V. Larger cohorts analyzing the outcome of palliative single-level SDR, based on the MAS, GMFM-66, and PROMs, are still needed and should be the focus of future studies. Systematic review registration no.: CRD42024495762 (https://www.crd.york.ac.uk/prospero/).
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  • 文章类型: Journal Article
    一名五岁男性,患有痉挛性四肢瘫痪脑瘫和表达性沟通障碍,因为家庭希望饮食自由化。由于在床边吞咽评估中发现的缺陷,饮食由固体和液体组成;由于严重的手术焦虑,尚未获得进一步的吞咽困难评估。采取了涉及丁螺环酮预处理的综合方法,脱敏,分心,和积极的强化。荧光镜吞咽研究成功完成,患者的饮食被升级为包括中等稠度的液体。
    特殊人群的程序性焦虑管理没有得到很好的研究。缺乏关于这些问题的明确建议增加了管理这些患者的难度。此案例突出了一种使用广泛可用的药理学和环境技术来解决个人需求的成功方法。此外,这种情况下,有必要确定潜在的原因,程序焦虑,并涉及一个跨学科的团队。
    应尝试确定驱动程序焦虑的因素。在与病人和家属讨论之后,相关信息应传达给工作人员,并邀请他们提出想法。虽然并非所有医院都有同等的资源,与不熟悉有关的担忧,潜在的焦虑,控制源可以通过有限的资源利用来解决,正如在这种情况下所证明的那样。
    UNASSIGNED: A five-year-old male with spastic quadriplegia cerebral palsy and an expressive communication disorder presented because the family desired liberalization of diet. The diet consisted of pureed solids and no liquids due to deficits identified on bedside swallow evaluation; further dysphagia assessment had not been obtained due to significant procedural anxiety. Comprehensive approaches were taken involving premedication with buspirone, desensitization, distraction, and positive reinforcement. The fluoroscopic swallow study was successfully completed, and the patient\'s diet was upgraded to include moderately thickened liquids.
    UNASSIGNED: Procedural anxiety management in special populations is not well-researched. The lack of definitive recommendations regarding these issues increases the difficulty of managing these patients. This case highlights one successful approach to addressing individual needs using widely-available pharmacologic and environmental techniques. Additionally, this case reinforces the need to identify underlying causes for procedural anxiety and involve an interdisciplinary team.
    UNASSIGNED: Attempts should be made to identify factors driving procedural anxiety. After discussing with the patient and family, relevant information should be relayed to staff with an open-ended invitation to propose ideas. While not all hospitals have equivalent resources, concerns related to unfamiliarity, underlying anxiety, and locus of control can be addressed with limited resource utilization, as demonstrated in this case.
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  • 文章类型: Case Reports
    方法:一名患有粗大运动功能分类系统5脑瘫和神经肌肉脊柱侧凸的17岁青少年男孩接受了从T3到骨盆的后路脊柱融合术和节段性脊柱器械。术后几个月他出现了右坐骨压迫伤,尽管采取了非手术措施,但仍然存在。随后,他在脊柱手术后16个月接受了同侧短肢截骨术,以治疗残留的骨盆倾斜和坐骨压力损伤,完全治愈了。在为期一年的随访中,没有进一步的压力伤害迹象。
    结论:本病例报告描述了短臂缩短截骨术是一种可行的治疗方法,用于治疗因骨盆固定倾斜引起的非愈合性坐骨压力损伤。
    METHODS: A 17-year-old adolescent boy with Gross Motor Function Classification System 5 cerebral palsy and neuromuscular scoliosis underwent posterior spinal fusion and segmental spinal instrumentation from T3 to the pelvis. He developed a right ischial pressure injury a few months postoperatively, which persisted despite nonoperative measures. He subsequently underwent an ipsilateral transiliac-shortening osteotomy 16 months after spinal surgery to treat his residual pelvic obliquity and the ischial pressure injury, which healed completely. At the 1-year follow-up visit, there were no further signs of pressure injury.
    CONCLUSIONS: This case report describes transiliac-shortening osteotomy as a viable treatment option for non-healing ischial pressure injuries secondary to fixed pelvic obliquity.
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  • 文章类型: Journal Article
    目的:本研究旨在评估以表现为重点的游泳计划对先前未经训练的脑瘫和高支持需求青少年(CPHSN)运动功能的影响,并确定CPHSN青少年的典型运动衰退是否发生在这些游泳者身上。
    方法:多基线,单病例实验设计(MB-SCED)研究包括五个阶段和30个月的随访。参与者是两名男性和一名女性,所有15岁的人,未经训练和CPHSN。干预是一项为期46个月的游泳训练计划,专注于提高性能。结果是游泳表现(速度);训练负荷(感知最小/周的运动等级;游泳距离/周)和粗大运动功能测量66项目集(GMFM-66)。使用中断时间序列模拟分析对MB-SCED数据进行分析。使用GMFM-66评分对46个月的运动功能进行建模(广义加法模型),并与预测的运动衰退模型进行比较。
    结果:对训练的反应GMFM-66得分的改善是显著的(p<0.001),和两个训练期各自导致显著的运动下降(p≤0.001)。参与者运动功能在研究期间保持在基线水平以上,and,重要的是,参与者没有出现其他CPHSN青少年典型的运动衰退.每周训练量也与世卫组织建议的身体活动水平相称。
    结论:研究结果表明,CPHSN青少年通过参加竞技游泳符合体力活动指南,可以防止运动衰退。然而,这个人群在临床上很复杂,为了安全,有效参与竞技体育,应优先考虑制定由熟练的多专业团队提供的方案。
    背景:ACTRN12616000326493。
    OBJECTIVE: This study aims to evaluate the effect of a performance-focused swimming programme on motor function in previously untrained adolescents with cerebral palsy and high support needs (CPHSN) and to determine whether the motor decline typical of adolescents with CPHSN occurred in these swimmers.
    METHODS: A Multiple-Baseline, Single-Case Experimental Design (MB-SCED) study comprising five phases and a 30-month follow-up was conducted. Participants were two males and one female, all aged 15 years, untrained and with CPHSN. The intervention was a 46-month swimming training programme, focused exclusively on improving performance. Outcomes were swim performance (velocity); training load (rating of perceived exertion min/week; swim distance/week) and Gross Motor Function Measure-66-Item Set (GMFM-66). MB-SCED data were analysed using interrupted time-series simulation analysis. Motor function over 46 months was modelled (generalised additive model) using GMFM-66 scores and compared with a model of predicted motor decline.
    RESULTS: Improvements in GMFM-66 scores in response to training were significant (p<0.001), and two periods of training withdrawal each resulted in significant motor decline (p≤0.001). Participant motor function remained above baseline levels for the study duration, and, importantly, participants did not experience the motor decline typical of other adolescents with CPHSN. Weekly training volumes were also commensurate with WHO recommended physical activity levels.
    CONCLUSIONS: Results suggest that adolescents with CPHSN who meet physical activity guidelines through participation in competitive swimming may prevent motor decline. However, this population is clinically complex, and in order to permit safe, effective participation in competitive sport, priority should be placed on the development of programmes delivered by skilled multiprofessional teams.
    BACKGROUND: ACTRN12616000326493.
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  • 文章类型: Case Reports
    几种外科手术用于治疗脑瘫的前臂动态内旋位置和手腕屈曲畸形。探讨了旋前转转位的术后结果,而专门设计的术后物理治疗及其结局有限。在这里,我们介绍了一个病例,在该病例中,在旋前肌改道后,评估了肌电生物反馈(EMG-BF)训练的结果,并评估了肱臂肌腱向桡侧腕骨短伸肌腱转移联合旋转截骨术的结果.峰值增加,而干预后肌肉的静息值下降。运动范围,手功能,手动能力,功能独立,生活质量水平得到改善。总之,EMG生物反馈训练可能对旋臂和肱臂的神经肌肉控制具有积极作用。自由使用上肢和改善的动手能力对患者的活动和生活质量有积极影响。
    Several surgical procedures are used to treat dynamic pronation position of the forearm and flexion deformity of the wrist in cerebral palsy. Postoperative results of pronator teres rerouting were explored, while specially designed postoperative physiotherapy and its outcomes were limited. Herein, we present a case in whom the outcomes of electromyographic biofeedback (EMG-BF) training were assessed after pronator teres rerouting and brachioradialis tendon to extensor carpi radialis brevis tendon transfer combined with derotation osteotomy. The peak value increased, while the resting value decreased for the muscles after the intervention. Range of motion, hand function, manual ability, functional independence, and quality of life levels were improved. In conclusion, EMG biofeedback training may have a positive effect on neuromuscular control of pronator teres and brachioradialis. Free use of the upper extremity and improved manual ability positively affect the activity and quality of life of the patients.
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  • 文章类型: Case Reports
    脑瘫(CP)通常会导致严重的髋关节问题,由于脱臼和挛缩等问题而破坏肌肉骨骼发育和活动,因痉挛和肌肉张力增强而加剧。虽然CP患者需要全髋关节置换术(THA),由于担心错位和磨损,该程序具有很高的风险。这项研究探索了一种术中导航方法,以精确执行术前策略,以进行脊柱骨盆对准和最佳杯放置。我们讨论了一例22岁的男性CP患者,患有双侧髋关节脱位,并经历了明显的不适,阻碍行动并影响他作为残奥会赛艇运动员的表现。他接受了双侧髋关节置换手术,在术前步态分析和成像指导下,导航帮助准确的髋臼组件放置和纠正过度的股骨前倾使用模块化的茎。患者在站立和划船姿势中均获得了出色的稳定性。总的来说,计算机导航通过促进术中数据收集和术前计划的精确执行来增强复杂的髋关节修复。这种方法可能会延长假体的寿命,特别是通过基于脊柱骨盆对齐原理实现精确的髋臼组件放置,从而为接受THA的CP患者提供显著的益处。
    Cerebral palsy (CP) often results in severe hip issues, disrupting musculoskeletal development and mobility due to problems such as dislocations and contractures, aggravated by spasticity and heightened muscular tone. While total hip arthroplasty (THA) is required in CP patients, the procedure carries high risks due to concerns about dislocation and wear. This study explores a method of intraoperative navigation to precisely execute preoperative strategies for spinopelvic alignment and optimal cup placement. We discuss a case of a 22-year-old male CP patient with bilateral hip dislocations who experienced significant discomfort, impeding mobility and affecting his performance as a Paralympic rower. He underwent bilateral hip replacement surgeries, guided by preoperative gait analysis and imaging, with navigation aiding in accurate acetabular component placement and correction of excessive femoral anteversion using a modular stem. The patient achieved excellent stability in both standing and rowing postures. Overall, computer navigation enhances complex hip repair by facilitating intraoperative data collection and precise execution of preoperative plans. This approach may extend the lifespan of prostheses, particularly by achieving precise acetabular component placement based on spinopelvic alignment principles, thereby offering significant benefits for CP patients undergoing THA.
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  • 文章类型: Case Reports
    无脑积水患者中恶性肿瘤的发展极为罕见。我们描述了第一例睾丸癌,该睾丸癌在无脑积水的长期幸存者的未降睾丸中发展。
    一名32岁男子因无脑积水导致严重脑瘫,被转诊到我们部门评估右下腹皮下肿块。他是无脑积水的长期幸存者。在确认右睾丸癌的诊断后,起源于他的未降睾丸,进行了手术切除。病理检查显示为混合型生殖细胞肿瘤。
    治疗恶性肿瘤的决策过程,比如睾丸癌,在患有严重脑瘫的成年人中可能具有挑战性。临床伦理咨询有助于避免治疗延误。
    UNASSIGNED: The development of malignant tumors in patients with hydranencephaly is extremely rare. We describe the first case of testicular cancer that developed in the undescended testes of a long-term survivor of hydranencephaly.
    UNASSIGNED: A 32-year-old man with severe cerebral palsy due to hydranencephaly was referred to our department for the evaluation of a subcutaneous lump in the lower right abdomen. He was a long-term survivor of hydranencephaly. After confirming the diagnosis of right testicular cancer originating in his undescended testes, surgical resection was performed. Pathological examination revealed a mixed-type germ cell tumor.
    UNASSIGNED: The decision-making process for treating malignant tumors, like testicular cancer, in adults with severe cerebral palsy can be challenging. Clinical ethics consultation could be helpful in avoiding treatment delays.
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