Congenital hand differences

先天性手部差异
  • 文章类型: Journal Article
    显微外科无疑是手外科的巅峰之作。近年来的显着进步已经扩大了获得性和先天性手部缺陷中脚趾到手转移以恢复功能的适应症,美学,和运动,对供体部位的发病率最低。没有一个固定的显微外科转移技术,但外科医生的多功能性和创新使用什么可以节省,因为每个案例都是独特的。近年来,美学上的改进和减少捐赠部位的发病率已经占据了前列。我们提出了几个案例来提出资深作者的首选技术,并以此为目标。
    Microsurgery is undoubtedly the pinnacle of hand surgery. Significant advancement in recent years has stretched the indications for toe-to-hand transfer in both acquired and congenital hand defects to restore function, esthetics, and motion, with minimal morbidity to the donor site. There is no one fixed microsurgical transfer technique but a surgeon\'s versatility and innovation in using what one could spare because each case is unique. Esthetic refinements and reducing donor site morbidities have taken a front seat in recent years. We present a few cases to put forward the senior author\'s preferred techniques with this objective in mind.
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  • 文章类型: Journal Article
    目的:我们尝试了一种技术,用于先天性无名指小指掌骨滑脱症患者,在滑脱部位切开并牵引延长第五掌骨并矫正掌骨关节伸展挛缩后,同时进行插入同种异体骨移植。这项研究的目的是描述手术技术及其结果。
    方法:我们回顾了在我们研究所手术治疗的先天性无名指小指掌骨滑脱症患儿的医疗记录。六个平均年龄5.0岁的孩子的八只手(范围,1.7-9.3)年在切开截骨后通过同时插入同种异体骨移植进行治疗,分心延长,和极小指外展肌的肌腱切开术。我们从连续的X射线照片中测量了掌骨头与头的面积比,并根据年龄进行了分析。我们还测量了平均8.1(范围,1.4-16.8)年随访。将这些变化与平均年龄为8.1岁的5名儿童的7只手的变化进行了比较(范围,1.5-15.6)采用相同方法处理的年,但没有分心延长第五掌骨,随访平均12.1(范围,4.1-19.8)年,作为对照组。
    结果:所有患者在术后前2年内,术前掌骨头与头的面积比值异常恢复正常。IMA变化平均为39.8°,掌骨长度比变化了17%。对照组显示平均IMA变化36.6°,掌骨长度比率变化6%。
    结论:在关节滑膜裂开截骨术和第五掌骨牵张加长矫正掌关节外展挛缩后,同时插入同种异体骨移植可以恢复先天性无名指掌关节滑膜的影像学参数。第五掌骨头骨化的正常化表明外科手术可能是安全的。
    方法:治疗性V
    OBJECTIVE: We attempted a technique for patients with congenital ring-little finger metacarpal synostosis involving simultaneous interpositional allograft bone after split osteotomy of the synostosis site and distraction lengthening of the fifth metacarpal along with correction of the metacarpal joint abduction contracture. The purpose of this study was to describe the surgical technique and its outcomes.
    METHODS: We reviewed the medical records of children with congenital ring-little finger metacarpal synostosis treated surgically at our institute. Eight hands of six children with an average age of 5.0 (range, 1.7-9.3) years were treated by simultaneous interpositional allograft bone after split osteotomy, distraction lengthening, and tenotomy of abductor digiti minimi. We measured the metacarpal head-to-capitate area ratios from serial radiographs and analyzed them according to age. We also measured the change in the intermetacarpal angle (IMA) and metacarpal length ratio during an average of 8.1 (range, 1.4-16.8) years of follow-up. These changes were compared with changes in seven hands of five children with an average age of 8.1 (range, 1.5-15.6) years treated by the same method, but without a distraction lengthening of the fifth metacarpal and followed up for an of average 12.1 (range, 4.1-19.8) years, as a control group.
    RESULTS: Abnormal metacarpal head-to-capitate area ratio before surgery was normalized in all patients within the first 2 years after surgery. The IMA change averaged 39.8°, and the metacarpal length ratio changed by 17%. The control group showed an average IMA change of 36.6° and metacarpal length ratio change of 6%.
    CONCLUSIONS: Simultaneous interpositional allograft bone after split osteotomy of the synostosis site and distraction lengthening of the fifth metacarpal with correction of metacarpal joint abduction contracture can restore the radiographic parameters in congenital ring-little finger metacarpal synostosis. The normalized ossification of the fifth metacarpal head indicates that the surgical procedure is probably safe.
    METHODS: Therapeutic V.
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  • 文章类型: Journal Article
    这项研究描述了心理健康和心理社会筛查过程,为患有上肢肌肉骨骼疾病的儿童提供护理和干预措施。对107名患有先天性手和上肢差异以及臂丛神经出生损伤的儿童的医疗保健专业人员进行了横断面电子调查研究。其中,41(38%)报告说,他们经常筛查心理健康和社会心理问题。很少(12%)报告使用标准化结果测量。总的来说,51(48%)的医疗保健专业人员报告说,他们的机构有一个等待心理健康服务的名单。总的来说,医疗保健专业人员对人员配备不满意,获得护理和可用干预措施的类型。报告的护理障碍包括对心理健康支持的需求日益增长,转诊后缺乏资源和护理连续性差。未来的研究应侧重于识别和验证心理健康筛查工具,并调查影响获得心理健康护理的过程。证据级别:IV。
    This study describes mental health and psychosocial screening processes, access to care and interventions provided to children with upper limb musculoskeletal conditions. A cross-sectional e-survey study was conducted of 107 healthcare professionals who work with children with congenital hand and upper limb differences and brachial plexus birth injuries. Of them, 41 (38%) reported that they routinely screen for mental health and psychosocial concerns. Few (12%) reported the use of standardized outcome measures. In total, 51 (48%) healthcare professionals reported that there was a waiting list for mental health services at their institution. Collectively, healthcare professionals were unsatisfied with the staffing, access to care and types of interventions available. Reported barriers to care included the growing need for mental health support, lack of resources and poor continuity of care after referrals. Future research should focus on identifying and validating a mental health screening tool and investigating the processes affecting access to mental health care.Level of evidence: IV.
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  • 文章类型: Journal Article
    该研究的目的是评估在线患者教育材料(PEM)对先天性手差异的可读性。
    在线排名前十,10种条件的英语PEM(多指,齐体,触发手指/拇指,clindactyly,Camptodactyly,合音尖顶,拇指发育不全,放射状发育不良,还原缺陷,和羊膜带综合征)按来源和国家进行汇编和分类。使用5种工具评估可读性:Flesch阅读轻松评分(FRES),Flesch-Kincaid等级(FKGL),GunningFogIndex(GFI),科尔曼-廖氏指数(CLI),和简单测量的步声指数(SMOG)。为了说明上述公式中每个条件名称的潜在影响,在用单音节单词替换名称后,重复了分析。
    100个PEM的平均可读性评分为FRES56.3,其中目标≥80,FKGL8.8,GFI11.5,CLI10.9和SMOG8.6,中位数评分为9.8,其中目标评分≤6.9。调整后,所有可读性评分均显著改善(P<.001)。调整后评分为FRES63.8,FKGL7.8,GFI10.7,CLI9.1和SMOG8.0,中位评分为8.6。使用所有工具,只有1个网页达到了目标水平。发表国(美国和英国)的双样本t检验表明,使用预调整CLI(P=.009)和中位数等级指标(P=.048),来自英国的PEM更易于阅读。单因素方差分析显示,条件或来源对可读性没有影响。
    大多数针对先天性手差异的在线PEM的书写水平都高于六年级的推荐阅读水平,即使是根据条件名称的影响进行了调整。
    UNASSIGNED: The aim of the study is to assess the readability of online patient education materials (PEMs) for congenital hand differences.
    UNASSIGNED: The top 10 online, English-language PEMs for 10 conditions (polydactyly, syndactyly, trigger finger/thumb, clinodactyly, camptodactyly, symbrachydactyly, thumb hypoplasia, radial dysplasia, reduction defect, and amniotic band syndrome) were compiled and categorized by source and country. Readability was assessed using 5 tools: Flesch Reading Ease Score (FRES), Flesch-Kincaid Grade Level (FKGL), Gunning Fog Index (GFI), Coleman-Liau Index (CLI), and Simple Measure of Gobbledygook Index (SMOG). To account for the potential effect of each condition\'s name in the aforementioned formulas, the analysis was repeated after replacing the name with a monosyllabic word/s.
    UNASSIGNED: The mean readability scores of the 100 PEMs were FRES 56.3, where the target was ≥80, FKGL 8.8, GFI 11.5, CLI 10.9, and SMOG 8.6, and the median grade score was 9.8, where the target grade was ≤6.9. Following adjustment, all readability scores improved significantly (P < .001). Postadjustment scores were FRES 63.8, FKGL 7.8, GFI 10.7, CLI 9.1, and SMOG 8.0, and the median grade score was 8.6. Only 1 webpage met the target level using all tools. Two-sample t test for country of publication (the United States and the United Kingdom) demonstrated that PEMs originating from the United Kingdom were easier to read using the preadjustment CLI (P = .009) and median grade metrics (P = .048). A 1-way analysis of variance revealed no influence of condition or source on readability.
    UNASSIGNED: Most online PEMs for congenital hand differences are written above the recommended reading level of sixth grade, even when adjusted for the effect of the condition\'s name.
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  • 文章类型: Journal Article
    目的:手术松解术是小儿手外科医师最常见的先天性手部手术之一。我们研究的目的是评估与联合释放相关的并发症,并确定与术后2年内较高并发症发生率相关的因素。
    方法:对2005年至2018年在单个儿科中心接受联合释放的患者进行了回顾性图表审查。如果患者被诊断为合并并接受了手术释放,并排除了手裂的诊断,不完整的手术文件,在外部机构进行的手术,或随访护理不超过第一次术后访视。使用Clavien-Dindo(CD)系统对并发症进行分类。
    结果:59例患者符合纳入标准,其中包括在85例手术中释放的143个网。85例手术共发生27例并发症。在23%的手术中,并发症的严重程度为CDI级或II级,最常见的是计划外的演员阵容变化,和CDIII级在8%的手术中。无CDIV级或V级并发症发生。CDIII级并发症包括6次再次手术。每次手术进行>1次联合松解术时,并发症发生率较高。对于经历>1次手术事件的患者,它也更高。与单次手术相比,多次手术的患者每次手术的并发症发生率相似。伴随诊断和并发症的复杂性与较高的并发症发生率无关。
    结论:手术并发症发生率为31%,其中44%的并发症与计划外的石膏更换相关,8%的并发症与入院或再次手术相关。合并释放后并发症的危险因素包括每次手术>1个web和>1个手术事件。
    方法:预后IV。
    OBJECTIVE: Syndactyly surgical release is one of the most common congenital hand surgeries performed by pediatric hand surgeons. The purpose of our study was to evaluate the complications associated with syndactyly release and determine factors that correlate with higher complication rates within the 2-year postoperative period.
    METHODS: A retrospective chart review was completed for patients who underwent syndactyly release at a single pediatric center between 2005 and 2018. Patients were included if they had a diagnosis of syndactyly and underwent surgical release, and excluded for a diagnosis of cleft hand, incomplete surgical documentation, surgery performed at an outside institution, or follow-up care that did not extend beyond the first postoperative visit. Complications were classified using the Clavien-Dindo (CD) system.
    RESULTS: Fifty-nine patients met the inclusion criteria, which included 143 webs released in 85 surgeries. A total of 27 complications occurred for the 85 surgeries performed. The severity of complications was CD grade I or II in 23% of surgeries, most commonly unplanned cast changes, and CD grade III in 8% of surgeries. No CD grade IV or V complications occurred. The CD grade III complications included 6 reoperations. The complication rate was higher when performing >1 syndactyly release per surgery. It also was higher for patients undergoing >1 surgical event. Rates of complication per surgery were similar between patients with multiple surgeries compared with those with a single surgery. Concomitant diagnoses and complexity of syndactyly was not associated with a higher complication rate.
    CONCLUSIONS: Syndactyly release was associated with a complication rate of 31% per surgical event with 44% of these complications related to unplanned cast changes and 8% of complications that required admission or reoperation. Risk factors for complications following syndactyly release include >1web operated on per surgery and undergoing >1 surgical event.
    METHODS: Prognosis IV.
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  • 文章类型: Journal Article
    小儿全醒局部麻醉无止血带技术(WALANT)手手术治疗小儿手部手术谱是可行的,其中可能包括外伤和先天性手部差异。成功的关键因素是为这项技术确定合适的患者,典型患者通常年龄>10岁。与患者的讨论和为最大效率的充分准备成为患者和外科医生舒适的关键。在这里,我们描述了一种为儿科人群成功执行这种有价值的技术的实用方法。
    Pediatric wide-awake local anesthesia no-tourniquet technique (WALANT) hand surgery is feasible for the treatment of the spectrum of pediatric hand surgeries, which can include traumatic injuries and congenital hand differences. The key component for success is identifying the appropriate patient for this technique, with the typical patient frequently being >10 years of age. The discussion with the patient and adequate preparation for maximum efficiency become key for patient and surgeon comfort. Here we describe a practical approach for successfully performing this valuable technique for the pediatric population.
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  • 文章类型: Journal Article
    背景:在资源受到挑战的国家,由于缺乏全国范围的监测系统,先天性手差异(CHD)的真实程度仍然未知.我们创建了一个基于医院的注册系统,旨在确定冠心病的患病率和分布,并比较Swanson/IFSSH(SI)和Oberg,Manske和Tonkin(OMT)分类。方法:从2018年1月至2021年12月,将0至18岁冠心病儿童的数据输入在线注册表,并根据SI和OMT分类进行分类。使用描述性分析总结了冠心病的患病率和分布以及家族史等危险因素。使用Fischer精确检验和比值比进一步分析了综合征和遗传关联。使用聚类列来比较两个分类系统。结果:研究期间共有307例冠心病患者,患病率为2.4/1,000。其中,164是单方面的,21人是近亲结婚(7.6%),10人(3.6%)有冠心病家族史。虽然没有统计学意义,与单方面参与相比,双边参与的遗传可能性是其两倍.在27例患者中,总共发现了10种不同的综合征,其中最常见的是Holt-Oram综合征。最常见的CHD是径向纵向缺乏(111)。共有82名(27%)儿童患有其他相关异常,无法归类为综合征。无法使用SI或OMT分类对两名患者进行分类。结论:在线注册建立了一种有效的方法来存储和分析与CHD相关的数据。它提供了有关其在印度南部流行的新信息,这与现有文献相似。大多数CHD可以分为SI和OMT分类。然而,仍然存在一些无法分类的条件。需要建立CDH的国家登记册,以进行有效管理,资金和研究。证据级别:IV级(流行病学)。
    Background: In resource challenged nations, the true magnitude of the congenital hand differences (CHD) remain unknown due to a lack of nation-wide surveillance system. We created a hospital-based registry system with the aim to determine the prevalence and distribution of CHD and compare the Swanson/IFSSH (SI) and Oberg, Manske and Tonkin (OMT) classifications. Methods: Data of children aged 0 to 18 years with CHD was entered into the online registry and classified based on the SI and OMT classifications from January 2018 to December 2021. The prevalence and distribution of CHD and risk factors like family history were summarised using descriptive analysis. Syndromic and heredity associations were further analysed using Fischer exact test and odds ratio. Clustered columns were used to compare the two classification systems. Results: A total of 307 patients with CHD presented during the study with a prevalence of 2.4/1,000 patients. Among them, 164 were unilateral, 21 were born of consanguineous marriages (7.6%) and 10 (3.6%) had a family history of CHD. Although not statistically significant, bilateral involvement was twice as likely to be hereditary compared to unilateral involvement. A total of 10 different syndromes were identified among 27 patients of which Holt-Oram syndrome was most common. The most common CHD was Radial longitudinal defificiency (111). A total of 82 (27%) children had other associated anomalies that could not be grouped as a syndrome. Two patients could not be classified using either SI or OMT classifications. Conclusions: The online registry established an efficient way to store and analyse data related to CHD. It provides new information on its prevalence in South India, which is similar to the existing literature. Most of the CHD can be grouped in both the SI and OMT classifications. However, there still remains some conditions that are unclassifiable. There is a need for a national registry of CDH for effective management, funding and research. Level of Evidence: Level IV (Epidemiological).
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  • 文章类型: Journal Article
    Background: Children with congenital differences who require prosthesis, have special needs due to their growth and psychosocial factors. Cosmetic or body-powered prosthesis provides basic functional needs but poses a financial burden on the family. Prostheses with advanced functions need to be deferred until adulthood. 3D printed prosthesis is a novel alternative being cost-effective for children. Since limited literature is available on the functionality of 3D printed prosthesis and none in the South Asian population, this study was done to assess its utility in congenital hand amputations. Methods: Fourteen children with congenital hand amputations were selected for a prospective observational study. Unilateral below Elbow test (UBET), Box and Block test and ABILHAND questionnaire were used for assessment with and without prosthesis after six months. Results: Eleven patients completed the follow-up. Box and Block test improved from a mean score of 24 to 35 with the prosthesis implying an improvement in manual gross dexterity (p -0.049). UBET (p -0.002) and ABILHAND questionnaire (p < 0.001) showed a decrease in score with the use of a prosthesis which suggested a lack of lateral and tripod pinch in the current design of a prosthesis. Patients with below elbow prosthesis performed better. Conclusions: 3D printed prostheses are an excellent option for use as a transitional prosthesis as they are inexpensive, serves to improve needs with respect to grasping activities at both wrist and elbow level amputation and customizable according to patients\' need and limb deficiency and replaceable as the child grows with age. Current designs are useful with respect to grasping activities.
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  • 文章类型: Journal Article
    The purpose of this article is to provide information about the changing landscapes in research, treatment, civil rights\' protection, disability awareness, and accepted terminology in the care of children with congenital upper limb differences. This knowledge can guide clinical and nonclinical conversations between patients and their families.
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  • 文章类型: Case Reports
    背景:产前超声检查检测胎儿结构异常是产前护理的重要组成部分。在评估期间,近端肢体畸形很容易诊断。远端肢体,尤其是数字,异常,然而,可能很难发现,特别是如果超声检查是在妊娠晚期进行的,畸形是单方面的和孤立的。
    方法:一名24岁的primigravida预约接受全科医生的产前护理,在妊娠12周时有流产的威胁,34周时,患者转诊至产科医生接受进一步护理和分娩.生长超声检查正常,但在妊娠40周时,她出现了不明原因的产前出血。她进行了剖腹产,并分娩了一个带有“基本”左手指的女婴(“孤立的共生指”)。父母接受了咨询,他们拒绝了手外科医生和临床遗传学家对婴儿的进一步评估。3岁时,婴儿发育正常,“即使没有手指,也在用手,“根据母亲的说法。
    结论:合作目标指导的产前护理,涉及不同类别的医疗保健专业人员,但特别是一位进行胎儿异常超声检查的认证声学家,对于先天性手部缺陷的检测至关重要。充分的咨询对于婴儿家庭的满意度至关重要。
    BACKGROUND: Prenatal ultrasonography for the detection of fetal structural anomaly is an important component of antenatal care. During the assessment, proximal limb deformities are readily diagnosed. Distal limb, especially digit, abnormalities, however, may be difficult to detect, particularly if the ultrasonography is performed in the third trimester, and the deformity is unilateral and isolated.
    METHODS: A 24-year-old primigravida booked for antenatal care with a general practitioner had threatened miscarriage at 12 weeks of gestation, and at 34 weeks was referred to an obstetrician for further care and delivery. The growth ultrasonographic examination was normal but at 40 weeks of gestation she developed antepartum haemorrhage of unknown origin. She had a caesarean delivery and a female baby with \"rudimentary\" left fingers (\"isolated symbrachydactyly\") was delivered. The parents were counselled and they declined further assessment of the baby by a hand surgeon and a clinical geneticist. At 3 years of age, the baby had normal development and \"is using her hand even without fingers,\" according to the mother.
    CONCLUSIONS: Collaborative goal-directed antenatal care that involves different categories of healthcare professionals, but particularly a certified sonologist who performs fetal anomaly ultrasonography, is essential for the detection of congenital hand defects. Adequate counselling is essential for the satisfaction of the baby\'s family.
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