Cleft

Cleft
  • 文章类型: Case Reports
    唇腭裂(CLP)是一种常见的先天性缺陷,这需要多学科的团队方法来实现令人满意的美学和功能矫正。横向差异是CLP案件的主要问题之一,上颌骨扩张已用于纠正下颌骨和上颌骨之间的骨骼和牙齿横向差异。在长期内,文献中引用了许多类型的扩展协议。本报告介绍了一名9岁单侧CLP患者的治疗方法,并强调了正畸和手术干预的结合。上颌扩张是通过棺材弹簧实现的,然后为功能和美学目的进行口鼻瘘闭合手术。
    Cleft lip and palate (CLP) represent a common congenital defect, which needs a multidisciplinary team approach for satisfactory aesthetic and functional correction. Transverse discrepancies are among the major problems in CLP cases, and maxillary expansion has been used to correct skeletal and dental transverse discrepancies between the mandible and maxilla. During the long period, many types of expansion protocols have been cited in the literature. This report presents the treatment of a nine-year-old patient with unilateral CLP and emphasizes the combination of orthodontic and surgical interventions. The maxillary expansion was achieved by Coffin spring, and then oronasal fistula closure surgery was done for both function and aesthetic purposes.
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  • 文章类型: Journal Article
    目的:不同手术技术对上颌骨生长的影响尚不清楚。这项研究调查了Furlow双相对Z成形术与直线修复(SLR)技术对中面生长和随后的正颌手术的长期影响。
    方法:回顾性队列研究。
    方法:三级儿童医院。
    方法:本研究评估了1994-2023年使用Furlow或SLR技术进行原发性腭成形术的患者。患者在最近的随访中年龄>14岁。
    方法:未进行干预。
    方法:主要结果是正颌手术和正颌手术纠正中面发育不全(MFH)的推荐率。在正颌手术推荐时进行头颅测量以验证MFH。
    结果:总计,1857例患者接受了腭成形术,其中335符合纳入标准(49SLR,286Furlow)。末次随访的平均年龄为18.5±2.6岁。接受Furlow和SLR的患者在正颌手术率(p=0.428)或正颌手术推荐率(p=0.900)方面没有显着差异。与不推荐进行正颌手术的患者相比,推荐进行正颌手术的患者具有更多的负ANB角度(p<0.001)和更小的SNA角度(p<0.001),表现为上颌骨发育不全.在多元回归时,VeauIII和IV裂患者对正颌手术的需求增加,分别为p=0.047和p=0.008。
    结论:我们的研究结果表明,更高的裂隙严重程度有助于未来的正颌手术。然而,腭成形术技术不影响正颌手术率。当外科医生考虑pal成形术技术对矢状生长限制的影响时,我们的结果提供了有价值的数据。
    OBJECTIVE: The influence of different surgical techniques on maxillary growth remains unclear. This study investigates the long-term impact of Furlow double-opposing Z-plasty versus straight-line repair (SLR) techniques on midface growth and subsequent orthognathic surgery.
    METHODS: Retrospective cohort study.
    METHODS: Tertiary children\'s hospital.
    METHODS: This study evaluated patients who underwent primary palatoplasty with Furlow or SLR techniques from 1994-2023. Patients were >14 years old at their most recent follow-up.
    METHODS: No interventions were performed.
    METHODS: Primary outcomes were orthognathic surgery and orthognathic surgery recommendation rates to correct midface hypoplasia (MFH). Cephalometrics at the time of orthognathic surgery recommendation were traced to validate MFH.
    RESULTS: In total, 1857 patients underwent palatoplasty, of which 335 met inclusion criteria (49 SLR, 286 Furlow). Average age at last follow-up was 18.5±2.6 years. Patients who underwent Furlow versus SLR showed no significant difference in orthognathic surgery rates (p=0.428) or recommendation for orthognathic surgery rates (p=0.900). Patients recommended to undergo orthognathic surgery had more negative ANB angles (p<0.001) and smaller SNA angles (p<0.001) than patients not recommended for orthognathic surgery, demonstrating maxillary hypoplasia. Upon multivariate regression, patients with Veau III and IV clefts had an increased need for orthognathic surgery, p=0.047 and p=0.008, respectively.
    CONCLUSIONS: Our findings suggest that higher cleft severity contributes to future orthognathic surgery. However, palatoplasty technique did not influence orthognathic surgery rates. Our results provide valuable data when surgeons are considering the impact of palatoplasty technique on sagittal growth restriction.
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  • 文章类型: Case Reports
    在全世界的兽医物种和人类中已经报道了与遗传和致畸相关的心脏先天性缺陷。其中,异位(EC),其特征是通过裂隙外化的心脏,在绵羊中极为罕见。本报告介绍了新生儿羔羊中两例完整的胸部EC的诊断特征。羔羊的临床发现,除了欧共体,平淡无奇。两种动物都表现出心脏外裂,没有心包覆盖,在胸廓裂隙中由心包和邻近皮肤的纤维环划定。组织学上,两只羔羊的心外膜被纤维组织增厚,一只动物也表现出明显的水肿,出血,和嗜中性炎症浸润。本研究羔羊EC的预后较差,尽管尝试了手术矫正,但仍有致命的结果。
    Cardiac congenital defects related to inheritance and teratogenesis have been reported in veterinary species and humans worldwide. Among these, ectopia cordis (EC), characterized by an externalized heart through a cleft, is extremely rare in sheep. This report presents the diagnostic features of two cases of complete thoracic EC in newborn lambs. Clinical findings in the lambs, aside from the EC, were unremarkable. Both animals exhibited exteriorized hearts without pericardial coverage, delineated in the thoracic cleft by a fibrous ring of the pericardium and adjacent skin. Histologically, the epicardium was thickened by fibrous tissue in both lambs, with one animal also showing marked edema, hemorrhage, and neutrophilic inflammatory infiltration. The prognosis of EC in the lambs of this study was poor, with fatal outcomes despite attempts at surgical correction.
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  • 文章类型: Journal Article
    对于在一个或多个量表上得分低于第25百分位数的儿童,将与患者相关的患者报告的结果测量(PROM)结果格式化为图形显示。报告在多学科诊所进行了试点,提供者对此进行了审查,它们的影响被定性记录。图形化PROM报告告知讨论,导致治疗计划的改变,并提高了对未满足的社会心理需求的认识。由于这次质量改进试点的成功,目测性PROM报告将成为我们多学科唇裂护理的常规部分.更广泛地说,图形化的PROM数据显示有助于更好地了解患者的观点,并导致更多的知情访问。
    Cleft-related Patient Reported Outcome Measure (PROM) results were formatted into graphical displays for children scoring below the 25th percentile on one or more scales. Reports were piloted in a multidisciplinary clinic where providers reviewed them, and their impact was qualitatively recorded. Graphical PROM reports informed discussions, led to treatment plan changes, and raised awareness of unmet psychosocial needs. Because of the success of this quality improvement pilot, visual PROM reports will become a regular part of our multidisciplinary cleft care. More broadly, graphical PROM data display facilitates better understanding of the patient\'s perspective and leads to more informed visits.
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  • 文章类型: Journal Article
    背景:随着改进的产前检测的出现,一些面部裂痕患者在产前被诊断,而另一些患者在产后被诊断。关于产前诊断的实用性以及这如何影响面部裂痕患者的护理的数据有限。
    方法:进行回顾性图表回顾。人口统计数据不完整的儿童和有症状的儿童被排除在外。通过Fisher精确检验和Kruskal-Wallis检验分析数据(p<0.05)。
    结果:106例患者符合纳入标准。取决于面部裂隙的类型,在产前以不同的频率诊断面部裂隙-单独患有腭裂的患者不太可能在产前被识别(p<0.0001)。与出生后诊断的患者相比,产前诊断的患者年龄较早(0.27个月对0.7个月,p<0.001)。同样,与产前诊断者相比,产前诊断者在更年轻的年龄接受手术(中位数:3.6个月vs10.67个月,p<0.001),并经历了较短的滞后时间(中位数:3.4个月vs8.4个月,p=0.027)从咨询到手术。重要的是,产前诊断导致手术前治疗的频率高于出生后诊断的儿童(86%vs22.2%,p<0.001)。
    结论:我们的数据表明,产前诊断为面部裂痕的患者更有可能接受手术前治疗,在较早的时候被介绍给颅面专家,在较早的年龄接受了手术,并且在初次就诊和手术之间经历了较少的滞后时间。有必要进行更多的研究来改进产前诊断方案,以改善手术结果。
    BACKGROUND: With the advent of improved prenatal detection, some patients with facial clefting are diagnosed prenatally while others are diagnosed postnatally. There is limited data regarding the utility of prenatal diagnosis and how this affects care of patients with facial clefts.
    METHODS: A retrospective chart review was performed. Children with incomplete demographic data and those with syndromic conditions were excluded. The data were analyzed via Fisher\'s exact tests and Kruskal-Wallis tests (p < 0.05).
    RESULTS: 106 patients met inclusion criteria. Facial clefting was diagnosed prenatally at different frequencies depending on type of facial cleft- patients with cleft palate alone were less likely to be identified prenatally (p < 0.0001). Patients diagnosed prenatally were seen by craniofacial specialists at an earlier age compared to those diagnosed after birth (0.27 months vs 0.7 months, p < 0.001). Similarly, those with prenatal diagnosis underwent surgery at a younger age compared to those who were diagnosed postnatally (median: 3.6 months vs 10.67 months, p < 0.001) and experienced shorter lag time (median: 3.4 months vs 8.4 months, p = 0.027) from consultation to surgery. Importantly, prenatal diagnosis resulted in pre-surgical therapy more often than in children diagnosed postnatally (86% vs 22.2%, p < 0.001).
    CONCLUSIONS: Our data suggests that patients with prenatal diagnosis of facial clefts were more likely to undergo pre-surgical therapy, presented to a craniofacial specialist at an earlier age, underwent surgery at an earlier age, and experienced less lag time between initial visit and surgery. More study is warranted to improve protocols for prenatal diagnoses to improve surgical outcomes.
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  • 文章类型: English Abstract
    眶上额叶裂是Tessier在1976年描述的罕见颅面裂之一,通常偶发。它们在这个分类中被编号为9、10和11,并分别位于横向,在轨道上部的中间和中间。它们的临床表现在软组织和骨骼上是可变的,与可能的参与分离。它们的范围从简单的美学缺陷到眼睛功能预后。在这种情况下,需要系统地进行CT扫描。他们的管理必须适应损害的多态性,并基于多学科方法。如果有眼部风险,眼睑重建是紧急情况。在所有其他情况下,治疗被推迟,但必须在早期进行,以确保孩子的健康发展。
    Superior orbital frontal clefts are one of the rare craniofacial clefts described by Tessier in 1976, and occur most often sporadically. They are numbered 9, 10 and 11 in this classification, and are located respectively laterally, in the middle and medially to the upper part of the orbit. Their clinical expression is variable on soft tissue and bone, with possible dissociation of involvement. They range from a simple aesthetic defect to an eyes functional prognosis. CT scans are systematically required in this context. Their management must be adapted to the polymorphism of the damage, and is based on multidisciplinary approach. In case of ocular risk, the eyelid reconstruction is an emergency. In all other cases, treatment is deferred, but must be carried out at an early stage to ensure the child\'s healthy development.
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  • 文章类型: Journal Article
    这项研究的目的是利用鼻塞来客观评估咽喉功能,特别是通过对nasalance的量化。对美国成年人进行了鼻压计的初始校准。这项研究的目的是验证使用鼻压计客观诊断唇腭裂的法国儿童的咽喉功能不全(VPI),并选择相关的言语刺激进行临床实践。
    方法:42名8至10岁儿童的鼻音评分,天生唇腭裂,收集并与50名对照儿童进行比较。然后对来自本研究创建的法语语料库(句子和音节)的31种言语刺激进行了评分分析。最相关的阈值由接收器工作特性曲线确定,表现出最高的敏感性和特异性。
    结果:结果显示,对于所有包含口头音素的言语刺激,对照组和裂隙组的平均鼻音评分存在统计学上的显着差异(p<0.05)。定义了具有良好诊断准确性的阈值,选择15种言语刺激用于临床实践。
    结论:本研究中定义的鼻部阈值可用于客观诊断咽喉功能不全(VPI)以及随后对8至10岁的法国儿童的监测,天生唇腭裂。
    The objective of this study is to utilize the Nasometer to objectively assess velopharyngeal competence, specifically through the quantification of nasalance. Initial calibration of the nasometer was conducted on American adults. The objective of this study was to validate the use of the nasometer for the objective diagnosis of velopharyngeal insufficiency (VPI) in French children born with a total cleft lip and palate and to select relevant verbal stimuli for clinical practice.
    METHODS: The nasalance scores of 42 children aged 8 to 10 years old, born with a cleft lip and palate, were collected and compared with 50 control children. The scores were then analyzed in relation to 31 verbal stimuli from the French corpus created for this study (sentences and syllables). The most relevant threshold values were determined by receiver operating characteristic curves, which exhibited the highest sensitivity and specificity.
    RESULTS: The results demonstrated statistically significant differences (p < 0.05) in the mean nasalance scores of the control and cleft groups for all verbal stimuli containing oral phonemes. Threshold values with good diagnostic accuracy were defined, and 15 verbal stimuli were selected for use in clinical practice.
    CONCLUSIONS: The nasalance threshold values defined in this study can be utilized for the objective diagnosis of velopharyngeal insufficiency (VPI) and the subsequent monitoring of French children aged 8 to 10 years old, born with a cleft lip and palate.
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  • 文章类型: Journal Article
    背景:TessierNo.0裂口,以双裂鼻子为特征,显著影响面部发育,给患者带来巨大的心理和经济负担。由于不同的介绍和有限的手术治疗文件,纠正缺乏共识,尤其是成年人。
    方法:局部软组织皮瓣可以解决轻微畸形,但是建立一个强大的骨骼框架对于永久矫正通常是至关重要的。
    方法:本文介绍了一种通过重建鼻中隔成形术解决这种异常的新颖有效的方法。
    BACKGROUND: Tessier No. 0 cleft, characterized by a bifid nose, significantly impacts facial development, imposing significant psychological and financial burdens on patients. Correction lacks consensus due to varied presentations and limited documentation of surgical treatments, notably in adults.
    METHODS: Loco-regional soft tissue flaps can address minor deformities, but establishing a robust skeletal framework is often crucial for permanent correction.
    METHODS: This article introduces a novel and effective approach through reconstructive septorhinoplasty for addressing this anomaly.
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  • 文章类型: Journal Article
    背景:唇裂伴或不伴腭裂(CL/P)是最常见的先天性面部畸形,源于多因素原因。
    目的:我们的研究旨在确定腭裂的患病率和特征,确定相关的危险因素,为早期干预提供预防和产前检测信息,并评估腭裂术后康复方案。
    方法:本研究采用回顾性描述和临床方法。
    方法:这项研究包括103名儿童和青少年头颈外科诊所治疗的腭裂患儿,临床儿科,Warmia和Mazury大学。
    方法:我们对记录进行了全面评估,考虑到诸如性别之类的变量,裂隙类型,产妇职业,父母教育,和裂缝的家族史。使用R软件版本GPL-3和有序逻辑回归分析进行数据分析。
    结果:值得注意的是,在怀孕期间经历了显著压力的母亲所生的孩子出现双侧腭裂的几率增加了9.4倍.相反,没有发现实质性证据支持孩子性别的影响,出生顺序,体重,产妇接触工作场所毒素,感染,或因变量的药物毒性。
    结论:我们的研究结果表明,父母有唇裂病史的儿童和母亲受教育程度较低的儿童更容易发生双侧腭裂。此外,母亲在怀孕期间经历压力所生的孩子面临双侧腭裂的风险增加.重要的是要注意,关于儿童各种left裂手术技术后康复的文献很少。
    BACKGROUND: Cleft lip with or without cleft palate (CL/P) stands as the most common congenital facial anomaly, stemming from multifactorial causes.
    OBJECTIVE: Our study aimed to ascertain the prevalence and characteristics of cleft palates, identify associated risk factors to inform prevention and prenatal detection for early intervention, and assess postoperative rehabilitation protocols for cleft palates.
    METHODS: This study employs a retrospective descriptive and clinical approach.
    METHODS: The study includes 103 children with cleft palates treated at the Department of Head and Neck Surgery Clinic for Children and Young Adults, Department of Clinical Pediatrics, University of Warmia and Mazury.
    METHODS: We conducted a thorough evaluation of records, considering variables such as sex, cleft type, maternal occupation, parental education, and family history of clefts. Data analysis was carried out using R software version GPL-3 and ordinal logistic regression analyses.
    RESULTS: Notably, children born to mothers who experienced significant stress during pregnancy exhibited a 9.4-fold increase in the odds of having bilateral cleft palates. Conversely, no substantial evidence was found to support the influence of the child\'s sex, birth order, body mass, maternal exposure to workplace toxins, infections, or drug toxicity on the dependent variable.
    CONCLUSIONS: Our findings suggest that children with parents who have a history of clefts and those with less educated mothers are more likely to develop bilateral cleft palates. Additionally, children born to mothers experiencing stress during pregnancy face an increased risk of bilateral cleft palates. It is important to note that there is a paucity of literature on rehabilitation following various cleft palate surgical techniques in children.
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  • 文章类型: Journal Article
    这项研究探讨了主持人的看法,马来西亚非综合征性唇腭裂(CL/P)学龄儿童的沟通障碍和策略,父母,和老师。14名7至12岁的非综合征性CL/P儿童,他们的父母(n=16),他们的教师(n=10)是通过目的性抽样招募的。参与者是根据弹性评分(RS-10;(Wagnild,2015)区分低(<34)和高弹性(35-40)(Wagnild,2015).个人深度访谈在网上进行,并进行定性评估。分析是利用Braun和Clarke(2006)的指导方针进行的。进行了归纳主题分析。儿童确定的交流促进者,父母和老师是孩子的个人属性,共同利益,帮助别人。被发现的障碍是不熟悉的对话伙伴,和不敏感的评论或问题。儿童使用被动(如忽略)和主动(如反驳,解释了条件)解决社会交往问题的策略。高弹性儿童的反应比低弹性儿童更积极。父母描述的策略包括接受孩子,赋予自己权力,根据裂缝管理计划,赋予孩子权力。教师采用了一般的方法,如给予鼓励,增强信心,教同理心。口罩的存在被视为促进者和沟通障碍。该研究提供了有关非综合征性CL/P儿童遇到的挑战和应对机制的信息,他们的父母和老师。这些知识可能有助于制定有针对性的干预措施,以支持非综合征性CL/P儿童在学校的交流。
    This study explores the perceptions of facilitators, barriers to communication and strategies in Malaysian school-aged children with non-syndromic cleft lip and palate (CL/P), parents, and teachers. Fourteen children with non-syndromic CL/P aged 7 to 12 years, their parents (n = 16), and their teachers (n = 10) were recruited via purposive sampling. Participants were selected based on resilience scores (RS-10; (Wagnild, 2015) distinguishing low (<34) from high resilience (35-40) (Wagnild, 2015). Individual in-depth interviews were conducted online and evaluated qualitatively. The analyses were conducted utilising the guidelines by Braun and Clarke (2006). Inductive thematic analysis was performed. Facilitators for communication identified by children, parents and teachers were the child\'s personal attributes, common interests, and helping others. Barriers that were identified were unfamiliar conversation partners, and insensitive comments or questions. Children used passive (e.g. ignored) and active (e.g. retorted, explained the condition) strategies to address social communication issues. Children with high resilience responded more actively than children with low resilience. Strategies that were described by the parents included accepting the child, empowering themselves, following the cleft management plan, and empowering the child. Teachers adopted general approaches such as giving encouragement, increasing confidence, and teaching empathy. The presence of face masks was perceived as both a facilitator and a barrier to communication. The study provides information on challenges encountered and coping mechanisms used by children with non-syndromic CL/P, their parents and their teachers. This knowledge may help the development of targeted interventions to support the communication of children with non-syndromic CL/P in school.
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