palatal development

  • 文章类型: Journal Article
    目标:首届克里特峰会旨在团结专家并促进跨学科合作,寻求对咽喉功能不全(VPI)管理的集体理解。
    方法:关于VPI管理的多学科唇裂护理团队之间的互动辩论和对话。
    方法:在为期四天的全面唇裂护理研讨会(CCCW)中进行了两个小时的讨论。
    方法:来自各种裂隙学科的32位全球领导者。
    方法:CleftSummit允许有意义的跨学科合作和知识交流。
    方法:能够就VPI管理的统一声明达成共识。
    结果:参与者一致认为,有显著VPI和动态膜的患者应该首先接受延长膜的手术,以优化患者的预后。一个全球性的,应该进行多中心前瞻性研究来检验这一假设。
    结论:第一届CletSummit通过迭代讨论成功地将全球专业知识提炼为可操作的最佳实践指南,促进跨学科合作,为VPI护理的变革性多中心前瞻性研究铺平道路。
    OBJECTIVE: The inaugural Cleft Summit aimed to unite experts and foster interdisciplinary collaboration, seeking a collective understanding of velopharyngeal insufficiency (VPI) management.
    METHODS: An interactive debate and conversation between a multidisciplinary cleft care team on VPI management.
    METHODS: A two-hour discussion within a four-day comprehensive cleft care workshop (CCCW).
    METHODS: Thirty-two global leaders from various cleft disciplines.
    METHODS: Cleft Summit that allows for meaningful interdisciplinary collaboration and knowledge exchange.
    METHODS: Ability to reach consensus on a unified statement for VPI management.
    RESULTS: Participants agreed that a patient with significant VPI and a dynamic velum should first receive a surgery that lengthens the velum to optimize patient outcome. A global, multicenter prospective study should be done to test this hypothesis.
    CONCLUSIONS: The 1st Cleft Summit successfully distilled global expertise into actionable best-practice guidelines through iterative discussions, fostering interdisciplinary collaboration and paving the way for a transformative multi-center prospective study on VPI care.
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  • 文章类型: Journal Article
    此病例报告显示了罕见的发现,患有晨光异常和Moyamoya病的小儿患者在先前修复的肺泡不完全裂隙中发现了pal膜错构瘤。口腔脑膜错构瘤极为罕见,仅描述了2例,并且在c裂或肺泡内均未出现。这些发现促使人们对具有脑膜亚分类的口腔错构瘤进行回顾。进一步的讨论描述了left裂发育背景下脑膜错构瘤的拟议起源之间的关系。
    This case report demonstrates a rare finding of a pediatric patient with Morning Glory anomaly and Moyamoya Disease with a palatal meningeal hamartoma discovered as a mass within a previously repaired incomplete cleft of the alveolus. Oral meningeal hamartomas are exceedingly rare with only two palatal cases described and none within a cleft palate or alveolus. These findings prompt a review of oral hamartomas with meningeal subclassification. Further discussion describes the relationship of the proposed origins of meningeal hamartomas within the setting of cleft palate development.
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  • 文章类型: Journal Article
    比较接受手术前功能性上颌骨矫形外科(FMO)治疗的单侧Cleft患者的上颌骨生长,以及接受外科手术而未使用过矫正器的患者的上颌骨生长。
    前瞻性研究,将患者分为2组。G1由12例患者组成,这些患者在6个月大时未接受手术前干预和唇鼻成形术,G2包括12例使用手术前FunctionalMaxilary骨科治疗的患者;他们在研究期间没有接受手术。测量进行了三次:在生命的第一个月之前,在6个月和9-12个月之间的年龄。裂口距离(前,中等和后部),上颌宽度(前,中和后)以及次要和主要节段宽度进行了分析。
    肺泡裂隙减少G1为79.82%,G2为52%。后裂痕减少,G1为24.1%,G2为41.77%。G1期患者的节段宽度增加为24.53%,G2期患者的节段宽度增加为37.47%。至于犬间宽度,发现G1减少5.16%,G2增加9.19%。中等拱顶宽度仅在G29.02%中以统计学显著的方式增加。
    手术允许关闭肺泡裂隙。FMO使得通过上颌段的生长来闭合前裂和后裂成为可能,增加上颌骨的横向生长,可以防止上颌骨塌陷。每个团队必须单独评估是否指示术前骨科的使用。
    Compare the maxillary growth of patients with Unilateral Cleft treated with pre-surgical Functional Maxillary Orthopedic (FMO) and that of who underwent a surgical procedure with no previous use of appliances.
    Prospective study, the patients were divided into 2 groups. G1, was composed of 12 patients who received no pre-surgical intervention and cheilorhinoplasty at 6 months of age and G2, included 12 patients treated using Pre-Surgical Funtional Maxilary Orthopedic; they underwent no surgery during the study. Measurements were taken at three times: before the first month of life, at 6 months and between 9-12 months of age. Cleft distance (anterior, medium and posterior), maxillary width (anterior, medium and posterior) and minor and major segment width were analyzed.
    Alveolar Cleft decrease was 79.82% in G1 and 52% in G2. Posterior Cleft decrease was 24.1% in G1 and 41.77% in G2. Greater Segment Width increase was 24.53% for the patients in G1, and 37.47% for the patients in G2. As for Inter Canine Width, a decrease of 5.16% in G1 and an increase of 9.19% in G2 were found. Medium Arch Width only increased in a statistically significant manner in G2 9.02%.
    Surgery allowed for the closure of the alveolar cleft. FMO made it possible to close the anterior and the posterior clefts through the growth of the maxillary segments, increased the transverse growth of the maxilla and could prevent maxillary collapse. Each team must individually evaluate whether to indicate or not the use of preoperative orthopedics.
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  • 文章类型: Journal Article
    目的:本研究的目的是评估唇腭裂团队的在线教育资源。
    方法:横断面研究。
    方法:国际,多机构研究。
    方法:所有美国腭裂和颅面协会批准的具有网站的团队。
    方法:无。
    方法:评估网站的患者教育和支持内容。还评估了材料的存在与美国新闻周刊前100家医院之间的联系。
    结果:包含187个团队。29.4%的网站有教育视频,教育信息图表占18.2%,书面材料占66.8%,围手术期指导占19.3%,诊断信息占34.8%,治疗信息占63.1%。77.0%的网站上提供了团队成员的信息,英语以外的语言资源占38.5%,和支持组资源占25.7%。唇腭裂诊断信息与地理区域显著相关,西部地区影响最大(P=0.03)。美国新闻周刊前100名医院的团队之间存在显着差异:前100名医院的教育信息图表含量更高,围手术期指导,定义,诊断,和治疗(P<0.01)。存在教育视频之间没有显着差异(P=0.37)。
    结论:虽然许多网站都有基本的教育材料,很少有关于围手术期计划的详细信息,以及其他形式的信息,包括视频,信息图表,和非英语语言。在线提供全面的患者教育材料是唇腭裂患者的重要补充,应由唇裂团队优先考虑。
    OBJECTIVE: The objective of this study is to evaluate online educational resources on cleft lip and palate teams.
    METHODS: A Cross-Sectional Study.
    METHODS: An International, Multi-Institutional Study.
    METHODS: All American Cleft Palate and Craniofacial Association-approved teams with websites.
    METHODS: None.
    METHODS: Websites were assessed for patient education and support content. Affiliation between presence of materials and U.S. Newsweek Top 100 Hospitals was also assessed.
    RESULTS: 187 teams were included. Presence of educational videos were available in 29.4% of websites, educational infographics in 18.2%, written materials in 66.8%, perioperative instructions in 19.3%, diagnosis information in 34.8% and treatment information in 63.1%. Information on team members were available on 77.0% of websites, resources in languages other than English in 38.5%, and support group resources in 25.7%. Cleft lip and palate diagnosis information was significantly associated with geographic region, with the largest impact from the West region (P = .03). There was a significant difference between teams affiliated with U.S. Newsweek Top 100 hospitals: Top 100 hospitals had a higher presence of educational infographics, perioperative instructions, definitions, diagnosis, and treatment (P < .01). There was no significant difference between presence of educational videos (P = .37).
    CONCLUSIONS: While many websites had basic educational materials, very few included detailed information on peri-operative planning, as well as additional forms of information including videos, infographics, and non-English languages. Providing comprehensive patient education materials online is an important supplement for patients with cleft lip and palate and should be prioritized by cleft teams.
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  • 文章类型: Journal Article
    目的:检查非综合征性腭裂患者的腭提肌组成,并探讨Veau类的影响。
    方法:前瞻性队列研究。
    方法:三级护理学术医院。
    方法:招募13例非综合征性腭裂患者。
    方法:在原发性腭成形术期间,切除了一个提肌palatini肌肉样本,并准备进行组织学分析。
    方法:用油红和天狼星红染色测定脂肪和胶原蛋白含量,分别,而肌纤维横截面积是根据H&E染色的样本计算的,使用组织形态计量学方法进行分析。进行肌球蛋白重链同工型的免疫荧光染色。
    结果:患者在10.8个月大(四分位距[IQR]10.2-12.9)时接受了修复。两组的提上肌脂肪含量均较低,从0%到5.2%不等。胶原蛋白含量范围为8.5%至39.8%;脂肪和胶原蛋白含量均未显示与Veau类相关。平均肌纤维横截面积随着Veau类的增加而减少,从VeauII的808µm2(范围692-995µm2)到VeauIII的651µm2(范围232-750µm2)(P=.02)。随着Veau等级的增加,I型肌纤维的比例也没有显着下降(VeauII中的44.3%[范围31.4%-84.4%]与VeauIII中的35.3%[范围17.4%-61.3%])。
    结论:与VeauII相比,VeauIII裂隙中的上提肌肌纤维面积减少。这些差异对咽喉功能障碍的影响需要对更大的队列进行进一步分析。
    OBJECTIVE: To examine levator veli palatini muscle composition in patients with nonsyndromic cleft palate and investigate the impact of Veau class.
    METHODS: Prospective cohort study.
    METHODS: Tertiary care academic hospital.
    METHODS: Thirteen patients with nonsyndromic cleft palate were recruited.
    METHODS: During primary palatoplasty, a sample of levator veli palatini muscle was excised and prepared for histological analysis.
    METHODS: Fat and collagen content were determined utilizing Oil Red and Sirius red stains, respectively, while muscle fiber cross-sectional areas were calculated from H&E-stained samples, with analysis using histomorphometric methods. Immunofluorescent staining of myosin heavy chain isoforms was performed.
    RESULTS: Patients underwent repair at 10.8 months of age (interquartile range [IQR] 10.2-12.9). Fat content of the levator veli palatini muscle was low in both groups, ranging from 0% to 5.2%. Collagen content ranged from 8.5% to 39.8%; neither fat nor collagen content showed an association with Veau classes. Mean muscle fiber cross-sectional area decreased with increasing Veau class, from 808 µm2 (range 692-995 µm2) in Veau II to 651 µm2 (range 232-750 µm2) in Veau III (P = .02). There was also a nonsignificant decrease in proportion of type I muscle fibers with increasing Veau class (44.3% [range 31.4%-84.4%] in Veau II vs 35.3% [range 17.4%-61.3%] in Veau III).
    CONCLUSIONS: Muscle fiber area in levator veli palatini muscles decreases in Veau III clefts in comparison to Veau II. The impact of these differences in velopharyngeal dysfunction requires further analysis of a larger cohort.
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  • 文章类型: Journal Article
    背景:这项研究描述了宽裂隙患者可能丢失的绒毛长度,并通过局部皮瓣重建来挽救这种区域丢失,提供解剖学证据支持在pal成形术期间软腭的主要延长。
    方法:对所有腭裂宽度至少为10mm的患者进行了回顾性研究,在2年的时间内,在18个月大之前接受了颊侧皮瓣的原发性腭成形术。所有患者均接受了水平横切鼻粘膜的原发性腭成形术,这是在鼻粘膜修复后进行的,但在肌肉重建之前.测量所得的腭延长,并用颊瓣重建粘膜缺损。
    结果:在22例患者中,3(13.6%)有皮埃尔·罗宾序列的历史,5例(22.7%)有相关综合征。没有病人有VeauI裂口,7名(31.8%)患有VeauII,12(54.5%)有VeauIII,和3(13.6%)有VeauIV裂。所有患者在初次腭成形术期间均有右颊瓣。后鼻棘平均裂隙宽度为10.6±2.82mm,水平横切鼻粘膜闭合后平均延长膜为10.5±2.23mm。有2个(9.1%)瘘管,1(4.5%)伤口裂开,1(4.5%)30天再入院,没有出血并发症.
    结论:宽腭裂患者可能损失1cm的绒毛长度。颊侧皮瓣可以挽救腭长度区域的缺失,并有可能改善腭运动。
    This study characterizes the potential loss of velar length in patients with a wide cleft and rescue of this loss of domain by local flap reconstruction, providing anatomic evidence in support of primary lengthening of the soft palate during palatoplasty.
    A retrospective review was conducted of all patients with a cleft palate at least 10mm in width, who underwent primary palatoplasty with a buccal flap prior to 18 months of age over a 2-year period. All patients underwent primary palatoplasty with horizontal transection of the nasal mucosa, which was performed after nasal mucosa repair, but prior to muscular reconstruction. The resulting palatal lengthening was measured and the mucosal defect was reconstructed with a buccal flap.
    Of the 22 patients included, 3 (13.6%) had a history of Pierre Robin sequence, and 5 (22.7%) had an associated syndrome. No patients had a Veau I cleft, 7 (31.8%) had a Veau II, 12 (54.5%) had a Veau III, and 3 had (13.6%) a Veau IV cleft. All patients had a right buccal flap during primary palatoplasty. The mean cleft width at the posterior nasal spine was 10.6  ±  2.82mm, and mean lengthening of the velum after horizontal transection of the nasal mucosa closure was 10.5  ±  2.23mm. There were 2 (9.1%) fistulas, 1 (4.5%) wound dehiscence, 1 (4.5%) 30-day readmission, and no bleeding complications.
    Patients with a wide cleft palate have a potential loss of 1cm velar length. The buccal flap can rescue the loss of domain in palatal length, and potentially improve palatal excursion.
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  • 文章类型: Case Reports
    背景:腭发育涉及通过复杂的分子机制进行的遗传调控,该机制可能会受到环境因素的干扰,导致受损的融合和腭裂形成。由于背舌错构瘤而遇到的left裂病例促使我们进行了这项系统审查。
    目的:回顾1例腭裂合并舌错构瘤的临床特点和治疗方法。
    方法:在PubMed中使用与left裂和舌错构瘤相关的关键词进行了系统的文献检索,Scopus,MEDLINE,和Scielo数据库到2021年12月,没有时间或语言限制。
    方法:研究报告了腭裂和舌错构瘤患者。
    方法:与临床概况有关的信息,诊断测试,组织病理学,管理,并提取结果。
    到目前为止,共发现了14篇相关出版物,报告了16例病例。其中,13例患者为女性(81.25%),男性3人(18.75%)。介绍的年龄从出生到19岁不等。口腔-面部-数字综合征(II型)是最常见的相关综合征。
    先天性舌错构瘤伴腭裂是一种罕见的表现,可以表现为孤立的实体或综合症的一部分。基因评估是有必要的,特别是对于多发性错构瘤性病变。优选的治疗是立即切除错构瘤,同时遵循用于腭成形术的标准时间表。
    Palate development involves a genetic regulation through a complex molecular mechanism that may be disrupted by environmental factors, resulting in impaired fusion and cleft palate formation. An encounter with a case of cleft palate due to dorsal tongue hamartoma prompted us to perform this systematic review.
    To review the clinical profile and management approach for a case with cleft palate and tongue hamartoma.
    A systematic literature search was conducted using keywords related to cleft palate and tongue hamartoma in PubMed, Scopus, MEDLINE, and Scielo databases through December 2021, with no time or language restrictions.
    Studies reporting patients with cleft palate and tongue hamartoma were included.
    Information related to clinical profile, diagnostic tests, histopathology, management, and outcomes were extracted.
    Fourteen relevant publications were identified with 16 cases reported so far. Among them, thirteen patients were females (81.25%), and 3 were males (18.75%). The age of presentation varied from birth to 19 years. Oral-facial-digital syndrome (type II) was the most commonly associated syndrome.
    Congenital tongue hamartoma with cleft palate is a rare presentation, which can present as an isolated entity or part of a syndrome. Genetic evaluation is warranted, particularly for multiple hamartomatous lesions. The preferred treatment is immediate excision of hamartoma while following a standard timeline for palatoplasty.
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  • 文章类型: Case Reports
    鼻旁窦全发育不全(TAPS)极为罕见,虽然部分发育不全很常见。到目前为止,TAPS在文献中似乎仅限于5例病例报告。我们介绍了一名29岁的患者,该患者具有综合征性面部外观,但偶然发现了TAPS。上颌,蝶骨,筛骨,额叶窦完全是再生障碍性的.此外,观察到倾斜和高拱形腭。患者的外貌与综合征脸一致,因为在检查中发现了一些发现,如过度端粒,睑裂缩短,突出而宽阔的鼻底,高拱形腭和颧骨发育不全。患者的资料比其他综合征更适合教师-柯林斯综合征,然而,某种诊断不是遗传的。据我们所知,这是首次报道的TAPS与综合征之间的关联.
    Total aplasia of paranasal sinus (TAPS) is extremely rare, although the fact that partial aplasia is very common. TAPS seems to be limited to only 5 case reports in the literature until now. We present the case of a 29-year-old patient who has a syndromic face appearance but whose TAPS was detected incidentally. The maxillary, sphenoid, ethmoid, and frontal sinuses were totally aplastic. Furthermore, clinodactyly and high-arched palate were observed. The patient\'s appearance was consistent with a syndromic face because of some findings on inspection such as hypertelorism, shortening of the palpebral fissure, protruded and wide nasal base, high arched palate and zygomatic hypoplasia. The patient\'s profile was more suitable for Teacher-Collins syndrome than other syndromes, however, a certain diagnosis was not made genetically. To the best of our knowledge, this is the first reported association between TAPS and a syndromic condition.
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  • 文章类型: Journal Article
    该病例系列详细介绍了2例男性新生儿唇腭裂(CLP)的异常病例,后来发展为配方耳漏。两名患者均接受了双侧鼓膜切开术,并插入了压力平衡(PE)管治疗慢性渗出性中耳炎(OME)。PE管插入后发生与进食相关的慢性耳漏,后来证实耳漏是由于配方奶回流所致。患者接受抗生素滴耳剂治疗,常规耳部清洁,抗反流药物,和反流预防措施。在明确的腭裂修复后,配方耳漏完全解决。当CLP患者在放置PE管后出现慢性OME或耳漏时,应考虑配方液回流到中耳,并进行相应处理。
    The case series details 2 unusual cases of male newborns with cleft lip and palate (CLP) that later developed formula otorrhea. Both patients underwent bilateral myringotomies with the insertion of pressure equalizing (PE) tubes for chronic otitis media with effusion (OME). Chronic otorrhea associated with feeding occurred post-PE tube insertion and the otorrhea was later confirmed to be due to reflux of formula. Patients were treated with antibiotic ear drops, routine ear cleaning, anti-reflux medication, and reflux precautions. After definite cleft palate repair, formula otorrhea completely resolved. When patients with CLP develop chronic OME or otorrhea following PE tube placement, reflux of formula into the middle ear should be considered and treated accordingly.
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  • 文章类型: Journal Article
    年龄在6至12岁之间的标准骨移植已成为牙槽裂的首选治疗方法。鉴于手术时机在完全性腭裂修复中的重要性,重要的是要确定任何有延迟牙槽骨移植风险的人群.这项研究的目的是确定在牙槽骨移植的时机上全国范围内是否存在种族差异。
    回顾性分析设置:参加美国外科医生学会国家外科质量改善计划(ACS-NSQIP)的医院。
    纳入标准涉及2012年至2019年期间接受牙槽骨移植的患者,这些患者通过当前程序术语(CPT)代码42210确定。根据以下参数按手术时的年龄对患者进行分层:早期植骨(6岁之前),标准植骨(6至12岁),和晚期植骨(12岁后)。
    牙槽骨移植时患者年龄的种族和种族差异。
    总的来说,20.28%的队列在12岁后接受了牙槽骨移植。非洲裔美国人(29.33%)和西班牙裔美国人(24.42%)患者接受晚期牙槽骨移植的频率高于其他种族和族裔(P<0.001)。
    完全性腭裂患者接受晚期牙槽骨移植的频率存在种族和种族差异。鉴于与标准牙槽骨移植相比,晚期手术效果欠佳,因此重要的是进一步研究这些差异的驱动因素。
    Standard bone grafting between ages 6 and 12 has become the preferred treatment of choice for alveolar clefts. Given the importance of surgical timing in complete cleft palate repairs, it is important to identify any populations at-risk for delayed alveolar bone grafting. The purpose of this study is to identify whether a racial disparity is present nationally in the timing of alveolar bone grafting.
    Retrospective analysis Setting: Hospitals participating in the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP).
    Inclusion criteria involved patients who underwent alveolar bone grafting identified by current procedure terminology (CPT) code 42210 between years 2012 and 2019. Patients were stratified by age at time of operation based on the following parameters: early bone grafting (before 6 years of age), standard bone grafting (between 6 and 12 years of age), and late bone grafting (after 12 years of age).
    Racial and ethnic differences in the age of patients at the time of alveolar bone grafting.
    Overall, 20.28% of the cohort received alveolar bone graft after 12 years of age. African American (29.33%) and Hispanic (24.42%) patients received late alveolar bone grafting more frequently than other racial and ethnic groups (P < .001).
    Racial and ethnic disparities are present in the frequency at which patients receive late alveolar bone grafting for complete cleft palates. Given the suboptimal surgical results of late compared to standard alveolar bone grafting it is important to further investigate the driving factors of these disparities.
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